Bilimsel olaylar ve yazım kuralları kimlik sayfa sayısına eklenmeli !!!

BBAA EEDDTTRR - EDITOR IN CHIEF

Dr. N. Krad TOKEL, Trkiye

BBAA EEDDTTRR YYAARRDDIIMMCCIISSII - ASSOCIATE EDITOR IN CHIEF

Dr. Nazmi NARN, Trkiye

EEDDTTRR YYAARRDDIIMMCCIILLAARRII - ASSOCIATE EDITORS

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DDLL EEDDTTRR -- LLAANNGGUUAAGGEE EEDDIITTOORR

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YYAAYYIINN KKUURRUULLUU - EDITORIAL BOARD

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TRK PEDATRK KARDYOLOJ VE KALP CERRAHS DERNEADINA SAHBDr. Nazmi NARN

SORUMLU YAZI LER MDRDr. Nazmi NARN

YNETM YERTrk Pediatrik Kardiyoloji ve Kalp Cerrahisi Dernei

Hodere Caddesi No: 180/4 ankaya, ANKARA

Tel : (0312) 212 02 00

Faks : (0312) 212 02 00

web : www.turkpedkar.org.tr

e-posta : [email protected]

YAYIN PERYODU VE TRPediatric Heart Journal 3 ayda bir olmak zere ylda 4 say yaynlanr (Mart-

Haziran-Eyll-Aralk).

Yerel sreli yayn.

ADRES DEKLKLERDerginin yaynland tarihlerden en az 15 gn nce dernek yazma adresine

bildirilmelidir. Zamannda yaplmayan bildirimler nedeniyle derginin aboneye ula-

mamasndan yaync sorumlu tutulamaz.

YAYIN HAKKIPediatric Heart Journalde yaynlanan yazlar, resim, ekil ve tablolar yayncnn yazl

izni olmadan ksmen veya tamamen herhangi bir vasta ile baslamaz, oaltlamaz.

Bilimsel amala kaynak gstermek kaydyla zetleme ve alnt yaplabilir.

BASILDII YER-BASIMCI-YAYIMCIOrtadou Reklam Tantm Yaynclk Turizm Eitim naat Sanayi ve TicaretA.. (Trkiye Klinikleri)Trkoca Cad. No:30 06520 Balgat/Ankara/Trkiye

Tel : 0 312 286 56 56

Faks : 0 312 220 04 70

e-posta : [email protected]

web : www.turkiyeklinikleri.com

Basma verili tarihi: 06.04.2016

ISSN: 2148-4910

Online ISSN: 2458-7591

THE OWNER ON BEHALF OF TURKISH PEDIATRIC CARDIOLOGY AND CARDIAC SURGERY SOCIETYDr. Nazmi NARN

MANAGING CLERICAL DIRECTORDr. Nazmi NARN

ADDRESS FOR MANAGEMENTTurkish Pediatric Cardiology and Cardiac Surgery Society

Hodere Caddesi No: 180/4 ankaya, ANKARA

Phone : (0312) 212 02 00

Fax : (0312) 212 02 00

web : www.turkpedkar.org.tr

e-mail : [email protected]

PUBLICATION TYPE AND PERIODSPediatric Heart Journal is published 4 times a year (March-June-September-

December).

Local periodic publication.

CHANGE OF ADDRESSNotify the subscription office for the change of address at least 15 days before thedate of issue. The publisher will not be responsible from any lost resulting from anaddress change if not informed.

COPYRIGHTAll articles, images, figures and tables published in this journal are protected byCopyright. No material published in this journal may be reproduced or duplicatedpartially or totally without the written permission from the copyright holder. Permis-sion is not required to cite or summarize the publications for scientific purposes inthe condition that a full reference to the source is shown.

PUBLISHING HOUSE-PUBLISHEROrtadou Advertisement Presentation Publication TourismEducation Construction Industry and Trade Co. (Trkiye Klinikleri)Trkoca Cad. No:30 06520 Balgat/Ankara/Turkey

Phone : +90 312 286 56 56

Fax : +90 312 220 04 70

e-mail : [email protected]

web : www.turkiyeklinikleri.com

ORJNAL ARATIRMALAR

169 EEffffeeccttss ooff aa CCoommbbiinnaattiioonn ooff SSppiirroonnoollaaccttoonnee,, FFuurroosseemmiiddee aanndd CCaappttoopprriill oonn BBlloooodd EElleeccttrroollyyttee LLeevveellss ooff IInnffaannttss wwiitthh CCoonnggeessttiivvee HHeeaarrtt FFaaiilluurree DDuuee ttoo CCoonnggeenniittaall HHeeaarrtt DDiisseeaassee Doutan Kalp Hastal Nedeniyle Konjestif Kalp Yetersizlii Olan Bebeklerde Spironolakton, Furosemid, Kaptopril Kombinasyonunun Kan Elektrolit Dzeylerine EtkileriAlev ARSLAN, Nazan ZBARLAS, Sevcan ERDEM, Osman KKOSMANOLU

174 EEvvaalluuaattiioonn ooff CCaarrddiiaacc FFuunnccttiioonnss iinn CChhiillddrreenn wwiitthh FFaammiilliiaall MMeeddiitteerrrraanneeaann FFeevveerrAilevi Akdeniz Atei Olan ocuklarda Kardiyak Fonksiyonlarn DeerlendirilmesiPelin AYYILDIZ HACIMEROLU, Yonca AIKGZ, Taner KASAR, Erkut ZTRK,

brahim Cansaran TANIDIR, smail LEK

182 YYeenniiddooaannddaa KKaarrddiiyyoolloojjii KKoonnssllttaassyyoonnuu:: KKaarrddiiyyaakk AAnnoommaallii SSkkll vvee EEttiiyyoolloojjiikk FFaakkttrrlleerrCardiology Consultation in Neonates: The Incidence of Cardiac Anomaly and the Ethiological FactorsSaadet ELK CENGZ, Ali Rahmi BAKLER, Ula KARADA, Kay ELAIK, ule DEMR,

Buket DORUSZ, Esra ARUN ZER

189 ffrrmmll ooccuukkllaarrnn DDeeeerrlleennddiirriillmmeessiiEvaluation of Children with Cardiac MurmurNurdan EROL, Yusuf zzet AYHAN, Fatma Tuba ALTIN COKUN, Merve KOLU

OLGU SUNUMLARI

194 LLeefftt VVeennttrriiccuullaarr OOuuttflflooww TTrraacctt OObbssttrruuccttiioonn aanndd AAoorrttiicc IInnssuufffificciieennccyy CCaauusseedd bbyy AAcccceessssoorryy MMiittrraall VVaallvvee:: CCaassee RReeppoorrttAksesuar Mitral Kapan Neden Olduu Sol Ventrikl k Yolu Obstrksiyonu ve Aort YetersizliiTaner KASAR, sa ZYILMAZ, Mehmet Bedir AKYOL, Serta HAYDN, Alper GZELTA

NDEKLER

CCiilltt 22 SSaayy 44 YYll 22001155

197 CCoonnttrraallaatteerraall SSeeqquueessttrraattiioonn iinn RRiigghhtt--SSiiddeedd SScciimmiittaarr SSyynnddrroommee PPrreesseennttiinngg wwiitthh IInnflflaammmmaattoorryy MMyyoofifibbrroobbllaassttiicc TTuummoorr:: CCaassee RReeppoorrtt Kar Taraf Sekestrasyondan Kaynakl nflamatuar Miyofibroblastik Tmr ile Birlikte Grlen Sa Taraf Scimitar Sendromusa ZYILMAZ, Taner KASAR, brahim Cansaran TANIDIR, Serglen DERVOLU, Alper GZELTA, Ender DEM

200 PPrroommiinneenntt LLeefftt VVeennttrriiccuullaarr NNoonnccoommppaaccttiioonn iinn aa PPaattiieenntt wwiitthh PPuullmmoonnaarryy AAttrreessiiaa--IInnttaacctt VVeennttrriiccuullaarr SSeeppttuumm:: CCaassee RReeppoorrtt Pulmoner Atrezi ve ntakt Ventrikler Septumu Olan Bir Hastada Belirgin Sol Ventrikl NonkompaksiyonuEyp ASLAN, Pelin AYYILDIZ, brahim Cansaran TANIDIR, Erkut ZTRK, Yakup ERGL, Alper GZELTA

203 AA RRaarree CCoommpplliiccaattiioonn AAfftteerr PPuullmmoonnaarryy AArrtteerryy BBaannddiinngg:: PPsseeuuddooaanneeuurryyssmm ooff tthhee PPuullmmoonnaarryy AArrtteerryy:: CCaassee RReeppoorrtt Pulmoner Artere Bant Uygulamas Sonras Oluan Nadir Bir Komplikasyon: Pulmoner Arter PsdoanevrizmasFatma Sevin ENGL, Ahmet RDEM, Aysel TRKVATAN, Ersin EREK, Alper GZELTA

207 DDoouubbllee OOrriifificcee MMiittrraall VVaallvvee iinn aa CChhiilldd wwiitthh VVeennttrriiccuullaarr SSeeppttaall DDeeffeecctt;; CCaassee RReeppoorrtt aanndd RReevviieeww ooff tthhee LLiitteerraattuurreeVentrikler Septal Defektli Bir ocuk Olguda ift Orifisli Mitral Kapak: Olgu Sunumu ve Literatrn Gzden GeirilmesiFiliz EKC, Zehra Diyar TAMBURACI USLU, Salih ZOBANOLU, Mehmet Halil ERTU,

Frat KARDELEN, Gayaz AKURN

211 RReeppaaiirr ooff aann IIaattrrooggeenniicc AAoorrttiicc VVaallvvee LLeeaaflfleett IInnjjuurryy:: CCaassee RReeppoorrtt yatrojenik Aort Kapak Yaralanmasnn OnarmMurat KO, mer Faruk EK, Sercan TAK, Onur IIK, Vehbi DOAN, Hakan AYDIN, Ali KUTSAL

214 MMoonnoozzyyggoottiicc TTwwiinnss wwiitthh WWiilllliiaammss SSyynnddrroommee:: CCaassee RReeppoorrttMonozigotik kizlerde Williams SendromuSelcen YAROLU KAZANCI, Mehmet Bedir AKYOL

218 UUzzuunn QQTT SSeennddrroommuu vvee iittmmee KKaayybb:: JJeerrvveellll vvee LLaannggee--NNiieellsseenn SSeennddrroommlluu kkii KKzz KKaarrddee Long QT Syndrome and Hearing Loss: Two Sisters with Jervell and Lange-Nielsen SyndromeFunda AKPINAR, Dolunay GRSES, zlem GL, Oya UYGUNER, Fsun DZCAN

BLMSEL TOPLANTILAR

Pediatr Heart J 2015;2(4) 169

Effects of a Combination of Spironolactone,Furosemide and Captopril on Blood ElectrolyteLevels of Infants with Congestive Heart Failure

Due to Congenital Heart Disease

AABBSS TTRRAACCTT OObbjjeeccttiivvee:: To investigate blood electrolyte levels when spironolactone is administered concomi-tantly with furosemide and captopril to infants with moderate to severe heart failure due to congenital heart dis-ease. MMaatteerriiaall aanndd MMeetthhooddss:: Thirty infants with congenital heart defects and moderate to severe heart failure,which could not be controlled by the use of captopril and furosemide for at least one month, were enrolled in thisstudy. Serum potassium, serum sodium, blood urea nitrogen (BUN) and serum creatinine levels were determinedat the initiation of the treatment and first week controls. Data from patients in Group A (2mg/kg/day spirono-lactone + furosemide + captopril; n=16) and Group B (>2 mg/kg/day spironolactone + furosemide + captopril;n=14) were analyzed for differences. RReessuullttss:: Hyponatremia was detected in 15 patients (50%) on the first weekcontrols after initiation of spironolactone treatment. There were 10 patients with mild and 5 patients with mod-erate hyponatremia. Three of them had moderate dehydration and were hospitalized, hydrated intravenouslyand spironolactone was discontinued. Eight of them had mild dehydration; spironolactone dosage was reducedand hydrated orally. Spironolactone dosage was reduced in only 4 cases who were in better clinical status and notdehydrated. Two days later, hydration status and electrolyte levels were improved in all patients. None of the pa-tients experienced hyperkalemia. According to the 1st week control results, there was a statistically significantdifference between blood sodium levels in all patients (p=0,005) and this difference mainly resulted from GroupB patients. When groups were compared for electrolyte levels before and after spironolactone treatment, a sta-tistically significant decrease in blood sodium levels and an increase in blood potassium levels was noted inGroup B (p=0.009, p=0.016; respectively). There were no significant differences between the groups in termsof hyponatremia frequency or severity at the 1st week controls. Serum creatinine levels at the first week con-trol were higher in Group B patients; this was statistically significant when compared with Group A (p=0.002).CCoonncclluussiioonn:: Occurrence of hyponatremia, hyperkalemia and dehydration in patients receiving spironolactone isinfluenced by its dosage when used concomitantly with captopril and furosemide. Even at recommended doses,it is essential to always monitor serum sodium and potassium levels.

KKeeyy WWoorrddss:: Heart failure; congenital heart disease; spironolactone; hyperkalemia; hyponatremia

ZZEETT AAmmaa:: Doutan kalp hastalna bal orta-ar iddette kalp yetersizlii olan, furosemid ve kaptopril te-davisi alan bebeklerde spironolakton eklendiinde kan elektrolit dzeylerinin deerlendirilmesi amaland. GGeerreevvee YYnntteemmlleerr:: En az 1 ay sre ile kaptopril ve furosemid kullanmna ramen kontrol altna alnamayan orta-ariddette kalp yetersizlii olan 30 bebek almaya alnd. Her hastann tedavi balanmadan nce ve tedavinin bi-rinci haftasnda serum potasyum, serum sodyum, kan re azotu (BUN), serum kreatinin deerlerine bakld. GrupA (16 hasta) (2 mg/kg/gn spironolakton+furosemid+kaptopril) ve Grup B (14 hasta) (>2 mg/kg/gn spirono-lakton+furosemid+kaptopril) olarak rastgele belirlendi. BBuullgguullaarr:: Spironolakton sonras 15 hastada (%50) birincihafta kontrollerinde hiponatremi saptand. On hastada hafif, be hastada orta dzeyde hiponatremi mevcuttu. Buhastalarn nde orta derecede dehidratasyon gelitii iin spironolakton kesildi, intravenz hidrasyon saland.Sekiz hastada ise hafif dehidratasyon mevcuttu,; spironolakton dozu azaltld ve oral hidrasyon saland. Hafif hi-ponatremisi olan, dehidratasyonu olmayan drt hastada ise sadece spironolakton dozu azaltld. Onbe hastannda iki gn sonraki sodyum dzeyi ve hidrasyon durumu normaldi. Hastalarn hibirinde hiperkalemi gelimedi.Tm hastalarda spironolakton ncesi ve sonras hiponatremi geliimi karlatrldnda istatistiksel olarak anlamlfark saptand (p=0,005), bu fark asl olarak Grup B hastalarndan kaynaklanmaktayd. Gruplar aras birinci haftasonundaki elektrolit dzeyleri karlatrldnda, sodyum dzeylerindeki d (p=0,009), potasyum dzeyle-rindeki art (p=0,016) Grup Bde istatistiksel olarak anlamlyd. Birinci hafta kontrolnde gruplar arasnda hipo-natremi skl veya ciddiyeti karlatrldnda istatistiksel olarak anlaml fark saptanmad. Birinci hafta kontrolserum kreatinin dzeyleri Grup B hastalarda daha yksekti, Grup A hasta sonularyla karlatrldnda ista-tistiksel olarak anlaml fark saptand (p=0,002). SSoonnuu:: Kaptopril ve furosemid ile e zamanl spironolakton kul-lanan hastalarda hiponatremi, hiperkalemi, dehidratasyon gelimesi doza baldr. nerilen dozlardakullanldnda bile serum sodyum ve potasyum dzeylerinin takibi gerekmektedir.

AAnnaahh ttaarr KKee llii mmee lleerr:: Kalp yetersizlii; doutan kalp hastal; spironolakton; hiperkalemi; hiponatremi

PPeeddiiaattrr HHeeaarrtt JJ 22001155;;22((44))::116699--7733

Alev ARSLAN,a

Nazan ZBARLAS,a

Sevcan ERDEM,a

Osman KKOSMANOLUa

aDivison of Pediatric Cardiology,ukurova University Faculty of Medicine,Adana

Ge li Ta ri hi/Re ce i ved: 06.07.2015Ka bul Ta ri hi/Ac cep ted: 15.12.2015

Ya z ma Ad re si/Cor res pon den ce:Alev ARSLANBakent University Faculty of Medicine,Adana Teaching and Medical Research Center,Division of Pediatric Cardiology, Adana,TRKYE/[email protected]

Copyright 2015 by Trk Pediatrik Kardiyoloji veKalp Cerrahisi Dernei

ORJNAL ARATIRMA

ongestive heart failure is the state of sys-temic and pulmonary congestion resultingfrom inability of the heart to pump as much

blood as required for the adequate metabolism ofthe body.. The most common reason of congestiveheart failure (CHF) in early infancy is congenitalheart defects with large left-to right shunts (VSD,PDA, AVSD).1 The goals of medical treatment forheart failure are to reduce the preload, enhancecardiac contractility and reduce the afterload.Heart failure results from excessive fluid retentionin large left-to-right shunts and diuretics are usedprimarily to control symptoms of pulmonary andperipheral congestion. Treatment paradigm in chil-dren has been adapted from reports acquired inadults with heart failure. Afterload reduction is themainstream of heart failure therapy; its efficacy inheart failure is related to disruption of activation ofthe renin-angiotensin axis and to reduction of car-diac adrenergic drive. Aldosterone-blocking agentsare beneficial not only due to their diuretic effect,but rather due to blockade of aldosterone.2 In pa-tients with severe heart failure, which cant betaken under control, and particularly if there isneed for higher doses of diuretics, aldosterone an-tagonists can be added to the combination.3 The al-dosterone antagonists (most commonly used one isspironolactone) act as potassium-sparing agents andalso have antifibrotic and antiremodelling effectson the myocardium.4,5 The side effects of drugs aremore intense in infancy due to specific reasonssuch as the premature structure of the kidneys, im-plementation of inappropriate medication times,dosage adjustments and dilution of the tablet formsof the drugs. The hydration status, kidney func-tions and blood electrolyte levels need to be care-fully controlled particularly when spironolactoneis used with angiotensin converting enzyme in-hibitors (ACEi). The aim of this study is to evaluatethe effect of spironolactone, furosemide and capto-pril combination on blood electrolyte levels of in-fants with congenital heart disease and congestiveheart failure, which cant be taken under control.

MATERIAL AND METHODS

Thirty infants aged less than 1 year who had con-genital heart defects with CHF and could not be

controlled by the use of captopril and furosemide atleast one month were included in the study (all pa-tients were discussed for early corrective surgery).The heart failure score was made according to themodified Ross scoring system (Table 1).6 Infantsscored as moderate and severe congestive heart fail-ure were included in the study. Furosemide mainte-nance dosage was 1 mg/kg/day given as a single doseand captopril dosage was 0.5 mg/kg/dose every 12hours for all patients. They were given spironolac-tone with 2 doses of 1-3 mg/kg/day. The bloodsodium, potassium and creatinine levels, and hydra-tion status were evaluated before and at the firstweek of the combination treatment (summarized inTable 2). Dosage of spironolactone was 2 mg/kg/dayin Group A and >2mg/kg/day in Group B infants. Attheir 1st week control, the spironolactone dosageswere arranged according to their electrolyte levelsand hydration statuses. Mild hyponatremia is definedas a serum sodium concentration between 130 and135 meq/L, moderate hyponatremia as a concentra-tion between 125 and 129 mEq/L, and severe hy-ponatremia as a concentration less than 125 mEq/L.

Written informed consent was obtained fromall families and the study was conducted in accor-

Alev ARSLAN et al. EFFECTS OF A COMBINATION OF SPIRONOLACTONE, FUROSEMIDE AND CAPTOPRIL...

Pediatr Heart J 2015;2(4)170

0 +1 +2

History

Diaphoresis Head only Head and body, Head and body,

at exertion at rest

Tachypnea Rare Several times Frequent

Physical examination

Breathing Normal Retractions Dyspnea

Respiratory rate (breaths/min) (years)

0-1 year 60

1-6 years 45

7-10 years 35

11-14 years 28

Heart rate (beats/min) (years)

0-1 year 170

1-6 year 115

7-10 year 100

11-14 year 90

Hepatomegaly size (cm) 3

TABLE 1: Modified Ross classification of heart failure.

CHF: Congestive heart failureTotal score: 0-2 (no CHF), 3-6 (mild CHF), 7-9 (moderate CHF), 10-12 (severe CHF).

dance with the Helsinki Declaration. Parents wereinformed about the drugs, dosages, administrationtimes, amount of feeding, daily diaper consump-tion, urine output and absolute clinical signs of de-hydration and electrolyte imbalance.

STATISTICAL ANALYSIS

Statistical analyses were performed using SPSS soft-ware version 17. The variables were investigatedusing analytical methods (Kolmogorov-Smirnovand Shapiro-Wilks tests) to determine whether ornot they were normally distributed. Descriptiveanalyses were presented using mean SD, me-dian (minmax value), and percentages were ex-pressed where appropriate. The Wilcoxon testwas used to compare the change in blood sodiumand potassium levels before and after spironolac-tone treatment. The Mann-Whitney U test wasused to compare blood sodium levels afterspironolactone treatment between groups. A p-value of less than 0.05 was considered to be sta-tistically significant.

RESULTS

Thirty patients were included in the study. Sixteenof the patients were indicated as Group A and 14were indicated as Group B. There were 11 patientsdiagnosed as complete atrioventricular septal de-fect (AVSD) (7 of them were Down Syndrome), 1as partial AVSD (large primum atrial septal defect,mitral cleft and severe mitral insufficiency), 5 aslarge perimembranous outlet ventricular septal de-fect (VSD), 8 as perimembranous inlet VSD, 3 asmoderate VSD + patent ductus arteriosus and 2 aslarge aortopulmonary window. The average age ofthe patients was 4.20.3 months (range 2-10months), average weight was 4.81.5 kg (range 3-8.4 kg). Their average sodium level was 1372.6mEq/L (range 131-142 mEq/L) and average potas-sium level 4.60.4 mEq/L (range 3.5-5.4 mEq/L) be-fore the spironolactone. The mean age of Group Apatients was 4.92.1 months (range 1-10 months)and Group B was 4.52.1 months (range 2-9months). The mean weight of the Group A patientswas 5.21.5 kg (range 3-7.9 kg) and Group B was4.81.2 kg (range 3-8.4 kg). There was no statisti-cally difference between the groups with regard toweight and age. The mean spironolactone dose was1.70.3 mg/kg (range 1.1-2 mg/kg) in Group A and2.30.2 mg/kg (range 2.1-2.8 mg/kg) in Group B.The pre-treatment and 1st week control values ofelectrolyte levels, BUN and creatinine results ofthe groups have been summarized in Table 2 andFigure 1.

EFFECTS OF A COMBINATION OF SPIRONOLACTONE, FUROSEMIDE AND CAPTOPRIL... Alev ARSLAN et al.


GroupA (n:16) Group B (n:14)mean SD mean SD(min-max) (min-max) P values

Pretreatment Na+ (mEq/L) 1362.6 1382.4 0.120

(131-141) (134-142)

First week control (mEq/L) 1354.2 1333.7* 0.060

blood Na+ (126-141) (128-141)

Pretreatment K+ (mEq/L) 4.60.6 4.60.2 0.700

(3.5-5.4) (4.3-5)

First week control blood 4.50.5 4.80.3** 0.250

K+ (mEq/L) (3.4-5.2) (4.3-5.4)

Pretreatment BUN (mg/dL) 10.12.6 10.13.7 >0.05

(6.4-14.7) (4.8-20)

First week control BUN 11.53.9 12.37.5 >0.05

(mg/dL) (6.4-20) (4.3-29)

Pretreatment Cr (mg/dL) 0.290.17 0.30.12 >0.05

(0.2-0.9) (0.2-0.6)

First week control Cr (mg/dL) 0.240.1 0.35 0.002

(0.2-0.5) (0.2-2.7)

TABLE 2: Blood Na+ and K+ values before and after spironolactone treatment.

* Significant decrease in blood Na levels after spironolactone in Group B (p=0.009)** Significant increase in blood potassium levels after spironolactone in Group B(p=0.016) descriptive analysis was presented using median (min-max) due to non-normal distribution)

FIGURE 1: Blood Na+ values before and after spironolactone treatment.

In the 1st week of the treatment, hyponatremiawas detected in 15 of all patients (50%), which wasstatistically significant (p=0.005). There were 10patients with mild and 5 with moderate hypona-tremia (Table 3). In the first week control, 3 ofthem were hospitalized for moderate dehydra-tion, hydrated intravenously and spironolactonewas discontinued. Eight cases with mild dehy-dration were hydrated orally and spironolactonedosage was reduced. In 4 cases without dehydra-tion and better clinical statuses, only spironolac-tone dosage was reduced. Two days later, hydrationstatuses and electrolyte levels were improved in allpatients.

According to the first week control results,there was a statistically significant difference be-tween blood sodium levels in all patients (p135 mEq/L 11 (68.8%) 4 (28.6%) 15 (50%)

130-135 mEq/L (mild hyponatremia) 3 (18.8%) 7 (50%) 10 (33.3%)

125-129 mEq/L (moderate hyponatremia) 2 (12.5%) 3 (21.4%) 5 (16.7%)

TABLE 3: Blood sodium levels and hyponatremiaseverity per groups.

EFFECTS OF A COMBINATION OF SPIRONOLACTONE, FUROSEMIDE AND CAPTOPRIL... Alev ARSLAN et al.

standard deviations and narrow data ranges, mini-mal changes could make the difference statisticallysignificant.

The hormone aldosterone affects glomerulardistal tubules and increases reabsorption of sodiumand water. Spironolactone is generally a weak na-triuretic agent with diuretic activity related to thelevel of aldosterone; however, when it is combinedwith loop diuretics, its diuretic effect becomes potentiated. The combination of aldosterone bloc-king agents, ACEi and a loop diuretic can cause ex-cessive diuresis and natriuresis. Development ofhyperkalemia may be prevented with the effects ofloop diuretics.9

In the current study, hyponatremia was ob-served in both patient groups at different spirono-lactone dosages; however, their clinical statusrecovered quickly after reduction or cessation ofspironolactone. Therefore, if this triple combina-tion is going to be used, even at the spironolactonedose of 1 mg/kg/day, the serum sodium and potas-sium levels should be monitored carefully. Therecommended spironolactone dose when com-

bined with loop diuretics should not exceed 2 mg/kg/day unless mandatory.

CONCLUSION

Close clinical and laboratory monitoring is neces-sary in the combination treatment of heart failure.Excessive diuresis, hyponatremia and hyperkalemiamay worsen the patients clinical condition andheart failure. It has been determined that, when aspironolactone range of 1-3 mg/kg/dose is to beadded to a combination of furosemide and ACEi,the potassium levels are not affected but hypona-tremia can develop in short-term electrolyte mon-itoring. Therefore, close electrolyte monitoring isnecessary. Parents should be counselled about theamount of feeding, daily diaper consumption, urineoutput and absolutely clinical signs of dehydrationand electrolyte imbalance.

AAcckknnoowwlleeddggeemmeennttssThe authors gratefully acknowledge to ala Sartrk forreviewing the statistical analyses and zgr Sandal whochecked for language.


1. Scott DJ, Rigby ML, Miller GA, ShinebourneEA. The presentation of symptomatic heartdisease in infancy based on 10 years experi-ence (1973-82). Implications for the provisionof services. Br Heart J 1984;52(3):248-57.

2. Shaddy RE, Tani LY. Chronic heart failure inchildren. In: Allen HD, Driscoll DJ, Shaddy RE,Feltes TF, eds. Moss and Adams' Heart Disease in Infants, Children, and Adolescents.8th ed. Vol 2. Philadelphia: Lippincott Williams& Wilkins; 2013. p.1565-78.

3. Blume ED, Freed MD, Colan SD. Congestiveheart failure. In: Keane JF, Lock JE, Fyler DC,eds. NADAS Pediatric Cardiology. 2nd ed.Philadelphia: Saunder Elsevier; 2006. p 83-97.

4. Pitt B, Zannad F, Remme WJ, Cody R, Castaigne A, Perez A, et al. The effect ofspironolactone on morbidity and mortality in patients with severe heart failure. Ran-domized Aldactone Evaluation Study Inves-tigators. N Engl J Med 1999;341(10):709-17.

5. Ezekowitz JA, McAlister FA. Aldosteron block-ade and left ventricular dysfunction: a sys-temic review of randomized clinical trials. EurHeart J 2009;30(4):469-77.

6. Ross RD. The Ross classification for heart fail-ure in children after 25 years: a review and anage-stratified revision. Pediatr Cardiol 2012;33(8):1295-300.

7. Randomized Aldactone Evaluation Study In-vestigators. ACE inhibitor co-therapy in pa-tients with heart failure: Rationale for theRandomized Aldactone Evaluation Study(RALES). Am J Cardiol 1996;78(8):902-7.

8. Saito M, Takada M, Hirooka K, Isobes F, Ya-sumura Y. Serum concentration of potassiumin chronic heart failure patients administeredspironolactone plus furosemide and eitherenalapril maleate, losartan potassium or can-desartan cilexetil. J Clin Pharm Ther 2005;30(6):603-10.

9. Dedieu N, Burch M. Understanding and treat-ing heart failure in children. Paediatrics andChild Health 2013;23(2):47-52.

REFERENCES


Evaluation of Cardiac Functions inChildren with Familial Mediterranean Fever

AABBSS TTRRAACCTT OObbjjeeccttiivvee:: Familial Mediterranean Fever (FMF) is a chronic inflammatory disease characterized byrecurrent attacks of serositis. Colchicine treatment is known to prevent clinical attacks. This study aimed to eval-uate cardiac functions of children with FMF and compare with those of healthy controls, and secondly, to revealthe difference in cardiac indices between patients on regular colchicine treatment versus those recently startedon colchicine treatment. MMaatteerriiaall aanndd MMeetthhooddss:: 45 patients and 24 healthy controls were evaluated. Patientswere divided into two subgroups. Group I consisted of patients who were on regular colchicine treatment andGroup II consisted of patients who just started using colchicine. Demographic features, two dimensional (2D), M-mode, pulsed wave (PW) and tissue Doppler imaging (TDI) echocardiography findings of the patients and the con-trols were evaluated. RReessuullttss:: Although the demographic features and conventional (2D, M-mode and PW)echocardiographic parameters of FMF patients were not significantly different, the TDI parameters significantlydiffered between the patients and the controls. The Am values were significantly higher in Group I (0.090.02cm/s), and Group II (0.080.01 cm/s) versus the control group (0.070.02 cm/s), (p=0.001, p=0.026; respectively).Em/Am values of only Group I was significantly lower than the control group (1.930.37, 2.20.36, respectively;p=0.023). Although lower in Group II, the difference of Em/Am values did not reach significance in betweenGroup II and controls (p=0.130). IVRT was significantly shorter in Group II than Group I and the control group(Group I, II and control groups 43.697.71 ms, 33.2513.65 ms and 41.9611.54 ms, respectively; p=0.01). CCoonn--cclluussiioonn:: Although conventional systolic and diastolic echocardiographic parameters were in normal ranges, someof the diastolic functions demonstrated by TDI echocardiography were impaired in FMF patients with or with-out colchicine treatment. While only late diastolic filling was found to be affected in children with an initial di-agnosis, the lower Em/Am values also reached a statistical significance in FMF patients on regular colchicinetreatment when compared to the control group. We suggest that effective amounts of colchicine treatment whichare sufficient for prevention of overt inflammatory attacks and amyloidosis do not prevent deterioration of dias-tolic indices.

KKeeyy WWoorrddss:: Familial Mediterranean Fever; cardiac diastolic functions; colchicine

ZZEETT AAmmaa:: Ailevi Akdeniz Atei (AAA) tekrarlayan serozit ataklar ile karakterize kronik inflamatuar bir has-talktr. Kolisin tedavisinin klinik ataklar nledii bilinmektedir. Bu almada AAA tans ile izlenmekte olanhastalarn kardiyak fonksiyonlarnn deerlendirilmesi ve salkl kontrollerle karlatrlmas ve ikinci olarak d-zenli kolisin tedavisi alan ve kolisin tedavisi yeni balanan hastalarn kardiyak fonksiyonlarnn karlatrl-mas planland. GGeerree vvee YYnntteemmlleerr:: Krkbe hasta ve 24 salkl kontrol deerlendirildi. Hastalara iki grubaayrld. Grup I dzenli kolisin tedavisi kullanan hastalardan ve Grup II kolisin tedavisi yeni balanan hastalar-dan oluturuldu. Hastalarn ve salkl kontrollerin demografik zellikleri, iki boyutlu (2D), M-mod, pulsed Dopp-ler (PW) ve doku Doppler (DD) ekokardiyografi bulgular deerlendirildi. BBuullgguullaarr:: AAA hastalar ve kontrollerinarasnda demografik bulgular ve konvansiyonel ekokardiyografi bulgular (2D, M mode ve PW), asndan anlamlfark saptanmamakla beraber DD parametreleri kontrollerden anlaml derecede farkl idi. Grup I ve II Am deerleri(srasyla 0,090,02 cm/s, 0,080,01 cm/s) kontrol grubundan (0,070,02 cm/s) anlaml derecede yksek idi(srasyla p=0,001, p=0,026). Sadece Grup Iin Em/Am deerleri kontrol grubundan anlaml derecede dk idi(1,930,37, 2,20,36; p=0,023). Grup IIde daha dk olmakla beraber Grup II ve kontrol grubu Em/Am deer-leri arasnda istatistiksel olarak anlaml fark saptanmad (p=0,130). IVRT Grup II de Grup I ve kontrol grubundananlaml derecede ksayd (Grup I, II ve kontrol grubu srasyla 43,697,71 ms, 33,2513,65 ms, 41,9611,54 ms;p=0,01). SSoonnuu:: Kolisin tedavisi kullanan ya da kullanmayan FMF hastalarnn konvansiyonel sistolik ve diasto-lik fonksiyonlar normal olmakla beraber, baz DD diastolik ekokardiyografi bulgularnda farkllk saptanmtr.Yeni tan alan hastalarda sadece ge diastolik dolum etkilenmi grlmekle beraber, dzenli kolisin tedavisi alanhastalarn Em/Am deerlerinde de kontrollere gre anlaml dklk saptanmtr. Klinik inflamatuar ataklarve amiloidozu engellemek iin kullanlan kolisin dozunun, diastolik kardiyak fonksiyonlarn bozulmasn en-gellemediini dnyoruz.

AAnnaahh ttaarr KKee llii mmee lleerr:: Ailevi Akdeniz Atei; kardiyak diastolik fonksiyonlar; kolisin


Pelin AYYILDIZ HACIMEROLU,a

Yonca AKIKGZ,b

Taner KASAR,a

Erkut ZTRK,a

brahim Cansaran TANIDIR,a

smail LEKc

aDepartment of Pediatric Cardiology,Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center and Research Hospital, stanbulbDepartment of Pediatric Nephrology,Samsun Education and Research Hospital,SamsuncDepartment of Pediatric Rheumatology, mraniye Education and Research Hospital, stanbul


This study was presented as a poster in 12th National Pediatric Cardiology and Cardiovascular Surgery Congress, 1-5 May 2013, Fethiye-Mula, Turkey.

Ya z ma Ad re si/Cor res pon den ce:Pelin AYYILDIZ HACIMEROLUMehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center and Research Hospital, Department of Pediatric Cardiology,stanbul,TRKYE/[email protected]


ORJNAL ARATIRMA

EVALUATION OF CARDIAC FUNCTIONS IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER Pelin AYYILDIZ HACIMEROLU et al.


amilial Mediterranean Fever (FMF), alsocalled recurrent polyserositis, is an autosomalrecessive disease characterized by recurrent

attacks of serositis with accompanying fever, whichmostly affects people of Mediterranean descent.Nonsense or missense mutations in the MEFV genewere shown to be the cause in most cases.1 In pa-tients with FMF, there is a lack of inhibiting theactivity of chemotactic factors which leads toepisodes of inflammation of serosal tissues.2 Apartfrom renal involvement, clinical or subclinical car-diac involvement has also been reported in patientswith FMF.3-5 Colchicine treatment is known to pre-vent clinical attacks and amyloid development inmost patients; hoever, it has been stated that sub-clinical inflammation continues during the attack-free period.6

Cardiac functions of FMF patients were stud-ied in detail with conventional echocardiograpy,which was a partially subjective method as it isload-dependent. Tissue Doppler imaging is re-ported to provide more accurate means of quanti-fying both ventricular functions than conventionalechocardiography and to be more sensitive forevaluating subclinical cardiac abnormalities.7 Thereare few studies in the pediatric population evalu-ating cardiac functions, but to our knowledge,there is no study evaluating the cardiac functions atthe time of initial diagnosis and after a regulartreatment of colchicine.

This study aimed to evaluate cardiac functionsof children with FMF in comparison with cardiacfunctions of normal children and secondly to com-pare cardiac functions of two groups of childrenwith FMF: patients who were on regular colchicinetreatment and those who were only recently diag-nosed and put on colchicine treatment.

MATERIALS AND METHODS

Data of 45 FMF patients and 24 healthy controlswere evaluated retrospectively from patient files.All the patients fullfilled the clinical criteria forFMF.8 Patients were divided into two subgroupsaccording to the duration of colchicine treatment.Group I consisted of patients who were oncolchicine treatment for more than 6 months with

normal renal function tests and Group II consistedof patients who were just diagnosed and put oncolchicine. Group III (Control group) consisted ofchildren who were referred to cardiology for eval-uation for sports or innocent murmur.

Evaluation of the study population was per-formed during an attack-free period. Patients withacute infection, acute attack related to disease, pa-tients with other chronic diseases, valve abnor-malities and those unresponsive to colchicinetreatment were excluded. Body mass index was cal-culated as the ratio of weight in kilograms to thesquare root of height in meters. Blood pressure wasmeasured after 10 minutes of rest with a mercurysphygmomanometer and an appropriate sized cuff.

The study was approved by local ethics com-mittee, written informed consent was taken fromparents of all patients and healthy controls.

Echocardiographic studies were performed bya commercially available ultrasound system with3S and 6S sector transducers (VIVID 7 pro, GE,Milwaukee, WI, USA). An electrocardiogram wassimultaneously recorded in all examinations. Allexaminations were performed by the same experi-enced pediatric cardiologist using standard viewsand techniques according to the guidelines of theAmerican Society of Echocardiography, subjectslying in supine position without any sedation.9

The measurements were calculated by averaging3 consecutive cardiac cycles. The corrected andweighted kappa statistics for intra-observer vari-ability demonstrated good intra-observer agree-ment (kappa=0.72).

The standardized examinations included 2-di-mensionally guided M-mode echocardiograms andselected 2-dimensional and Doppler recordings.The tricuspid and mitral inflow velocities for dias-tolic functions were recorded from the apical 4chamber view, with the sample volume placed atthe tip of the leaflets of the valves during diastole.10

Measured parameters were: peak velocity of earlydiastolic filling wave (E), measured as the height ofmaximal deflection during the first half of diastole;peak velocity of the atrial filling wave (A), meas-ured as the height of maximal deflection after the

Pelin AYYILDIZ HACIMEROLU et al. EVALUATION OF CARDIAC FUNCTIONS IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER


plateau phase of the early filling period; the earlymitral flow deceleration time (DTE), measured asthe time taken from the maximum E point to base-line. Ratio of early to atrial peak velocity (E/A), de-fined as peak velocity of the early diastolic fillingwave divided by peak velocity of atrial filling wave,were also recorded. Myocardial performance index(MPI) was calculated as the sum of isovolumic con-traction time (IVCT) and isovolumic relaxationtime (IVRT) divided by left ventricular ejectiontime (LVET).11 Diastolic functions were also evalu-ated by tissue Doppler for left and right ventricleswhile the sample volume was at the lateral annulusof mitral valve and tricuspid valve, respectively, inthe apical 4-chamber view.

Statistical analyses were performed using theNumber Cruncher Statistical System (NCSS 2007Statistical Software, Utah, USA) statistical packprogramme. Data were expressed as the means andstandard deviations. Groups were compared withone-way analysis of variance (One way ANOVA),subgroup comparisons were done with Tukey mul-tiple comparison test. Chi-square and Fischer real-ity tests were used to compare qualitative data.Values for p less than 0.05 were considered sig-nificant.

RESULTS

There were no statistically significant differencesfor age, sex, weight, height, body mass index, sys-tolic and diastolic blood pressures between the

groups (p>0.05). The acute phase reactants [C reac-tive protein (CRP), erythrocyte sedimentation rate,fibrinogen] were within normal ranges in allgroups. There were no significant differences foracute phase reactants between groups (p>0.05),except CRP, which was significantly higher inGroups I and II than that of Group III (p=0.019)(Table 1).

All patients were started recently or alreadyreceiving colchicine at a dosage of 0.05-0.075mg/kg/day. The duration of treatment was 3.172.86 (0.6-13) years in Group I.

All measured cardiac and left ventricle M-mode indices (left atrium/aort ratio, interventricu-lar septum thickness, left ventricular end-diastolicand end-systolic dimensions, left ventricular pos-terior wall thickness), left ventricular ejection frac-tion and fractional shortening and heart rate werein normal ranges and did not differ between groups(p>0.05) (Table 2). Peak mitral and tricuspid earlyand late diastolic velocities measured by standardpulse wave Doppler, mitral valve early diastolicflow deceleration time, E/A values, MPI valueswere similar in all groups (p>0.05) (Table 3).

The tissue Doppler imaging data are given inTables 4 and 5. There were statistically significantdifferences in Am and Em/Am ratio betweengroups (p


Group I Group II Group III pHeart rate/min 89.9615.05 91.0610.86 9215.01 0.874

LA diameter (cm) 2.370.47 2.230.36 2.30.34 0.549

Aort diameter (cm) 2.330.39 2.130.4 2.290.38 0.285

LA/Ao 1.020.14 1.060.16 1.020.16 0.708

IVSd (cm) 0.820.18 0.760.22 0.840.2 0.490

IVSs (cm) 1.020.24 1.060.2 1.010.22 0.808

LVEDd (cm) 3.680.51 3.730.62 3.680.52 0.943

LVIDs (cm) 2.280.28 2.280.42 2.340.38 0.807

LVPWd (cm) 0.80.18 0.730.16 0.770.23 0.522

LVPWs (cm) 1.080.3 10.25 10.19 0.478

EF (%) 68.656.17 69.884.9 67.225.76 0.359

FS (%) 37.815.08 38.883.84 36.744.5 0.363

TABLE 2: M-mode echocardiographic data.

Data were expressed as meanSD; LA: Left atrium; LA/Ao: Left atrium/aort; IVS: Interventricular septum; LVEDD: Left ventricular end diastolic dimension; LVESD: Left ventricular endsistolic dimension; LVPW: Left ventricular posterior wall; EF: Ejection fraction of the left ventricle; FS: Fractional shortening of the left ventricle.

Group I Group II Group III pMitral inflow

E (m/s) 1.040.14 0.970.22 1.050.15 0.275

A (m/s) 0.580.11 0.550.09 0.580.09 0.584

E/A 1.850.3 1.790.41 1.850.38 0.867

DT Em (ms) 148.450.84 141.1428.67 149.139.28 0.813

MPI (%) 0.40.07 0.420.09 0.410.06 0.462

Tricuspid inflow

E (m/s) 0.750.11 0.730.12 0.750.1 0.827

A (m/s) 0.420.07 0.40.06 0.430.07 0.471

E/A 1.80.3 1.830.32 1.790.34 0.912

TABLE 3: Standard Pulsed Wave Doppler echocardiographic data.

Data were expressed as meanSD; E: Peak early diastolic velocity; A: Peak late diastolic flow velocity; DT Em: Early diastolic flow deceleration time; MPI: Myocardial performance index.

Group I Group II Group III pMitral annulus

Em (cm/s) 0.160.03 0.160.02 0.150.03 0.249

Am (cm/s) 0.090.02 0.080.01 0.070.02 0.001

Em/Am 1.930.37 1.970.33 2.20.36 0.023

DT Em (ms) 88.7718.81 87.839.08 84.0116.39 0.553

IVRT (ms) 43.697.71 33.2513.65 41.9611.54 0.01

Tricuspid annulus

Et (cm/s) 0.180.02 0.170.03 0.180.02 0.633

At (cm/s) 0.110.02 0.110.02 0.110.02 0.629

Et/ At 1.590.27 1.620.41 1.650.36 0.775

TABLE 4: Pulsed Wave Tissue Doppler Echocardiographic data. Data were expressed as meanstandard deviation.

Em peak vel: Early diastolic myocardial peak velocity of mitral annulus; Am peak vel: Late diastolic myocardial peak velocity of mitral annulus; DT Em: Early diastolic myocardial peakvelocity deceleration time; IVRT: Isovolumic relaxation time; Et peak vel: Early diastolic myocardial peak velocity of tricuspid annulus; At peak vel: Late diastolic myocardial peak ve-locity of tricuspid annulus.




Groups I and II (p=0.838). Em/Am values of thecontrol group were significantly higher than GroupI (p=0.023). Although Em/ Am values of the con-trol group were higher than Group II, differencewas not statistically significant (p=0.130). Therewas a statistically significant difference of IVRT pa-rameters between groups (p=0.01) where IVRT val-ues of Group II were significantly shorter thanthose of Group I and the control group (p=0.009,p=0.039, respectively). There was no statisticallysignificant difference in IVRT values betweenGroup I and the control group (p=0.838).

DISCUSSION

In this study, cardiac functions of patients with fa-milial Mediterranean fever (FMF) were evaluatedwith conventional and tissue Doppler echocardio-graphy and compared with healthy controls. Wedemonstrated that while systolic and conventionaldiastolic functions were in normal ranges, some ofthe tissue Doppler parameters were impaired inFMF patients. Secondly, the cardiac functions ofpatients who were on regular colchicine treatmentand who were only recently diagnosed and startedcolchicine treatment were evaluated in two sub-groups. We found impairment in some of the dias-tolic parameters in recently diagnosed patientswithout colchicine treatment (significantly higherpeak A velocity, lower but not statistically signifi-cant Em/Am values) and subclinical diastolic dys-function in the patient group on regular colchicinetreatment (significantly lower Em/Am values)when compared with controls.

Echocardiographic assessment of ventriculardiastolic functions relied on Doppler patterns of

mitral and tricuspid inflow. Reflecting the pressuregradient between the atrium and ventricle, trans-valvular velocities are directly related to atrial pres-sure (preload) and inversely related to ventricularrelaxation. The principle of detection of the shiftin frequency of ultrasound signals reflected frommoving blood cells in conventional Doppler tech-niques, are used to quantify the higher amplitude,lower-velocity signals of myocardial tissue motionin TDI. The annulus initially moves away from theapex during diastole and then back towards theapex during atrial contraction and these values arecomparable with those of transmitral flow.12

The E peak arises due to early diastolic filling.Approximately 70-75% of the ventricular filling isduring this phase. The A peak arises due to atrialcontraction, pumping 20-25% of stroke volumeinto the ventricle. The A wave includes flow oc-curring during atrial systole.13 Matsuda et al. statedthat a wave of left atrial systolic pressure is not asimple wave produced by left atrial systole, it in-cludes a component of reflection associated withincreased left ventricular end diastolic pressure.14

The E/A ratio is a marker of the function ofthe ventricle of the heart. The abnormalities in theE/A ratio suggest that the ventricle cannot fill prop-erly in the period between contractions.13

There are few previous studies demonstratingthe subclinical changes in diastolic cardiac func-tions in children with FMF. zdemir et al evalu-ated both right and left ventricular functions andfound right ventricular diastolic dysfunction inFMF patients. They suggested that right ventriclemight be affected first due to the difference in mor-phology of the right ventricle when compared toleft ventricle.15 On the other hand, Sari et al eval-uated 44 adult FMF patients (median age: 30, range:19-47 years) who were on regular daily colchicinetreatment and measurements of the study wereperformed during the attack-free period and sug-gested that subclinical myocardial involvement ispresent in relatively young FMF patients who werealso free of classical cardiovascular risk factors.16

Tavil et al reported impaired left ventricular dias-tolic indices with tissue Doppler imaging in adultFMF patients.17 Likewise Baysal et al studied car-

Group 1- Group 1- Group 2-p values Group 2 Group 3 Group 3

Am (cm/s) 0.838 0.001 0.026

Em/ Am 0.924 0.023 0.130

IVRT (ms) 0.009 0.838 0.039

TABLE 5: p values in between groups.

Em peak vel: Early diastolic myocardial peak velocity of mitral annulus; Am peak vel: Late diastolic myocardial peak velocity of mitral annulus; IVRT: Isovolumic relaxation time.


diac functions in children with FMF with bothconventional and tissue Doppler echocardiographyand reported significant changes in left ventriclediastolic indices of FMF patients when comparedwith controls while systolic functions were simi-lar.4 Nearly all of the studies mentioned abovefound, significant or not, a lower peak E velocity, ahigher peak A velocity and lower Em/Am param-eters in patients with FMF.4,16,17 Baysal et al.claimed that the increase of peak A velocity in chil-dren with FMF was probably due to increased leftventricular chamber stiffness caused by FMF.4 Sup-porting these studies, the present study foundhigher peak A velocity values in patients with FMFboth with and without colchicine treatment, alsolower but not significant Em/Am ratios in initiallydiagnosed patients and significantly lower Em/Amvalues in FMF patients on regular colchicine treat-ment.

Isovolumic relaxation time is an interval inthe cardiac cycle, from the end of the aortic ejec-tion to the onset of filling by opening of the mitralvalve. Two different opposing processes of IVRTcan be defined with left ventricular disease. Pro-longation by the disease process as in left ventric-ular hypertrophy, diabetes, or coronary arterydisease or shortening as left atrial pressure rises.18 Avery short IVRT is a sign of a raised left atrial pres-sure. If it is taken into consideration how a patientmay improve clinically while at the same time di-astolic measurements become more abnormal, itshould be remembered that the most common wayfor diastolic measurements to normalize is for leftatrial pressure to rise.19 In the present study, theIVRT values of FMF patients without colchicinetreatment were significantly shorter than FMF pa-tients on regular colchicine treatment and the con-trol group. The shortening of IVRT might beexplained by the attempt to normalize diastolicmeasurements in untreated FMF patients by in-creasing left atrial pressure.

Previous studies reported the potential forchronic inflammatory diseases like systemic lupuserythematosus or rheumatoid arthritis to impairvascular and cardiac functions.20-22 The main patho-physiology claimed for developing cardiac impair-

ment in those disorders is the inflammation, whichmay accelerate the development of atherogenesis,thrombosis and congestive heart disease. Systemicinflammation is also claimed to be an importantfactor for development of amyloidosis in whichdeposition of these amyloid fibrils in the cardiac in-terstitium and conducting system leads to restric-tive cardiomyopathy, arrhythmias, eventuallybiventricular heart failure and death.23 Supportingthese studies, presence of subclinical athereoscle-rosis was reported in FMF patients demonstratedwith increased carotid intima media thickness.3,24

Likewise, it was reported that subclinical inflam-mation occurs over prolonged periods in patientswith FMF and infrequent clinically overt attacksrepresent the tip of the iceberg in FMF patients.23

Yalnkaya et al evaluated 36 FMF patients andtheir 39 healthy parents (obligate heterozygotes)and reported that acute phase reactants in FMF pa-tients during attack-free periods and their rela-tives were significantly higher than healthycontrols, indicating the presence of continuingsubclinical inflammation during the attack-freeperiods in patients with FMF receiving regularcolchicine therapy.25 Similar observations havebeen made by other authors like Dzova andLanchmann et al.23,26 Similarly, Baysal et al. foundhigher CRP and serum amyloid A levels in FMF pa-tients when compared to controls which supportedan ongoing subclinical inflammation.4 In anotherstudy, coronary flow reserve were found impairedin patients with FMF and they found positive cor-relation between the severity of the impairmentand CRP levels, which also supports the findingthat cytokine levels remain elevated even in the at-tack-free period.27,28 Our results also demonstratedsignificantly higher, albeit within normal limits, ofacute phase reactant levels in FMF patients receiv-ing regular colchicine therapy supporting ongoingsubclinical inflammation in the attack-free periodas in the previous reports.

Colchicine is an alkaloid drug that is effec-tively used in several inflammatory diseases likegout, Behets disease and it is in use since 1970s toprevent the acute attacks of FMF.29 Regular pro-phylactic treatment with colchicine at a dose of 1




2 mg daily was reported to prevent or substantiallyreduce the clinical manifestations of FMF in at least90% of cases.6 There are many reports demon-strating the efficacy of colchicine in prevention ofinflammatory attacks and amyloid developmentalong with studies reporting ongoing subclinical in-flammation in FMF patients despite colchicinetreatment.6,29-31

Our results demonstrated diastolic dysfunctionin patients with FMF despite colchicine treatment.To the best of our knowledge, none of the reportsabout the cardiac functions of FMF patients evalu-ated the cardiac functions at the beginning of thediagnosis before a regular colchicine treatment.Furthermore, some of the authors noted as a limi-tation of their study that all of their study patientswere on regular colchicine treatment and theycould not compare the results of colchicine re-sponders with nonresponders and claimed thatcolchicine probably protects patients from theharmful effects of inflammation and its inverse ef-fects on the heart.4,15 We suggest that effectiveamounts of colchicine treatment that is reported tobe sufficient for prevention of overt inflammatoryattacks and amyloidosis does not prevent deterio-ration of diastolic indices, thus, impairment insome of the diastolic parameters is present from thebeginning of the symptoms or probably much ear-

lier and maintained despite regular colchicinetreatment.

Although total number of patients was com-parable to some of the previous studies evaluatingcardiac functions of children and adults withFMF, the small number of subgroups and short-ness of duration of the disease were the limita-tions in this study. The results of this studysuggested that larger scale prospective longitudi-nal studies are needed before definitive conclu-sions can be made and recent study might be aprelude for this purpose.

In conclusion, although conventional systolicand diastolic echocardiographic parameters werein normal ranges, some of the diastolic functionsdemonstrated by TDI were impaired in FMF pa-tients with or without colchicine treatment. Theseresults and previous studies support that the longerthe duration of the disease, the worse the diastoliccardiac indices, probably due to ongoing subclini-cal inflammation which is claimed to accelerate thedevelopment of atherogenesis, thrombosis inchronic inflammatory diseases like FMF despiteregular colchicine treatment. Therefore, life-longfollow-up by noninvasive screening methods canbe recommended for these patients for early un-masking of cardiac deterioration as in other chronicinflammatory diseases.

1. Apostolidou E, Kambas K, Chrysantho-poulou A, Kourtzelis I, Speletas M, Ritis K, etal. Genetic analysis of C5a receptors in neu-trophils from patients with familial Mediter-ranean fever. Mol Biol Rep 2012;39(5):5503-10.

2. Touitou I. The spectrum of Familial Mediter-ranean Fever (FMF) mutations. Eur J HumGenet 2001;9(7):473-83.

3. Peru H, Altun B, Doan M, Kara F, Elmaci AM,Oran B. The evaluation of carotid intima-mediathickness in children with familial Mediter-ranean fever. Clin Rheumatol 2008;27(6):689-94.

4. Baysal T, Peru H, Oran B, Sahin TK, Koksal Y,Karaaslan S. Left ventricular diastolic functionevaluated with tissue Doppler imaging in chil-dren with familial Mediterranean fever. ClinRheumatol 2009;28(1):23-8.

5. Bilginer Y, Ozaltin F, Basaran C, Duzova A,Besbas N, Topaloglu R, et al. Evaluation of intima media thickness of the com-mon and internal carotid arteries with inflammatory markers in familial Mediter-ranean fever as possible predictors for atherosclerosis. Rheumatol Int 2008;28(12):1211-6.

6. Onen F. Familial Mediterranean Fever.Rheumatol Int 2006;26(6):489-96.

7. Choong CY, Herrmann HC, Weyman AE,Fifer MA. Preload dependence of Doppler-derived indexes of left ventricular diastolicfunction in humans. J Am Coll Cardiol1987;10(4):800-8.

8. Livneh A, Langevitz P, Zemer D, Zaks N, KeesS, Lidar T, et al. Criteria for the diagnosis offamilial Mediterranean fever. Arthritis Rheum1997;40(10):1879-85.

9. Sahn DJ, De Maria A, Kisslo J, Weyman A.The committee on M-mode standardization ofthe American Society of Echocardiography:results of echocardiographic measurements.Circulation 1978; 58:1072-83.

10. Quiones MA, Otto CM, Stoddard M, Wag-goner A, Zoghbi WA. Recommendations forquantification of Doppler echocardiography: areport from the Doppler Quantification TaskForce of the Nomenclature and StandardsCommittee of the American Society ofEchocardiography. J Am Soc Echocardiogr2002;15(2):167-84.

11. Tei C, Ling LH, Hodge DO, Bailey KR, Oh JK,Rodeheffer RJ, et al. New index of combinedsystolic and diastolic myocardial performance:a simple and reproducible measure of cardiacfunction--a study in normals and dilated car-diomyopathy. J Cardiol 1995;26(6):357-66.

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13. Galderisi M. Diastolic dysfunction and diastolicheart failure: diagnostic, prognostic and ther-apeutic aspects. Cardiovasc Ultrasound 2005;3:9.

14. Matsuda Y, Toma Y, Matsuzaki M, Moritani K,Satoh A, Shiomi K, et al. Change of left atrialsystolic pressure waveform in relation to leftventricular end-diastolic pressure. Circulation1990;82(5):1659-67.

15. Ozdemir O, Agras PI, Aydin Y, Abaci A, HizliS, Akkus HI, et al. Assessment of cardiacfunctions using tissue Doppler imaging in chil-dren with familial Mediterranean fever. CardiolYoung 2012;22(2):188-93.

16. Sari I, Arican O, Can G, Akdeniz B, Akar S,Birlik M. [Assessment of aortic stiffness andventricular functions in familial Mediterraneanfever]. Anadolu Kardiyol Derg 2008; 8(4):271-8.

17. Tavil Y, Ureten K, Ozturk MA, Sen N, KayaMG, Cemri M, et al. The detailed assessmentof left and right ventricular functions by tissueDoppler imaging in patients with familialMediterranean fever. Clin Rheumatol 2008;27(2):189-94.

18. Mattheos M, Shapiro E, Oldershaw PJ, Sacchetti R, Gibson DG. Non-invasive as-sessment of changes in left ventricular re-laxation by combined phono-, echo-, and

mechanocardiography. Br Heart J 1982;47(3):253-60.

19. Gibson DG, Francis DP. Clinical assessmentof left ventricular diastolic function. Heart2003;89(2):231-8.

20. Selzer F, Sutton-Tyrrell K, Fitzgerald S, TracyR, Kuller L, Manzi S. Vascular stiffness inwomen with systemic lupus erythematosus.Hypertension 2001;37(4):1075-82.

21. Wong M, Toh L, Wilson A, Rowley K,Karschimkus C, Prior D, et al. Reduced arte-rial elasticity in rheumatoid arthritis and the re-lationship to vascular disease risk factors andinflammation. Arthritis Rheum 2003;48(1):81-9.

22. Ozkan M, Emel O, Ozdemir M, Yurdakul S,Koak H, Ozdoan H, et al. M-mode andDoppler echocardiographic study in 65 pa-tients with Behcets syndrome. Eur Heart J1992;13(5):638-41.

23. Lachmann HJ, Sengul B, Yavuzsen TU, BoothDR, Booth SE, Bybee A, et al. Clinical andsubclinical inflammation in patients with FMFand heterozygous carriers of MEFV mutations.Rheumatology (Oxford) 2006;45(6):746-50.

24. Akdogan A, Calguneri M, Yavuz B, Arslan EB,Kalyoncu U, Sahiner L, et al. Are familialMediterranean fever (FMF) patients at in-creased risk for atherosclerosis? Impaired en-dothelial function and increased intima mediathickness are found in FMF. J Am Coll Cardiol2006;48(11):2351-3.

25. Yalinkaya F, Cakar N, Acar B, Tutar E, GrizH, Elhan AH, et al. The value of the levels ofacute phase reactants for the prediction of fa-milial Mediterranean fever associated amyloi-dosis: a case control study. Rheumatol Int2007;27(6):517-22.

26. Duzova A, Bakkaloglu A, Besbas N, TopalogluR, Ozen S, Ozaltin F, et al. Role of A-SAA inmonitoring subclinical inflammation and incolchicine dosage in familial Mediterraneanfever. Clin Exp Rheumatol 2003;21(4):509-14.

27. Caliskan M, Gullu H, Yilmaz S, Erdogan D,Unler GK, Ciftci O, et al. Impaired coronary mi-crovascular function in familial Mediterraneanfever. Atherosclerosis 2007;195(2):e161-7.

28. Gang N, Drenth JP, Langevitz P, Zemer D,Brezniak N, Pras M, et al. Activation of the cy-tokine network in familial Mediterranean fever.J Rheumatol 1999;26(4):890-7.

29. Markel G, Imazio M, Brucato A, Adler Y. Pre-vention of recurrent pericarditis with colchicinein 2012. Clin Cardiol 2013;36(3):125-8.

30. Cabili S, Zemer D, Pras M, Aviram A, Sohar E,Gafni J. The prevention of amyloidosis in fa-milial Mediterranean fever with colchicine.Proc Eur Dial Transplant Assoc Eur RenAssoc 1985;21:709-11.

31. Sevoyan MK, Sarkisian TF, Beglaryan AA,Shahsuvaryan GR, Armenian HK. Preventionof amyloidosis in familial Mediterranean feverwith colchicine: a case-control study in Arme-nia. Med Princ Pract 2009;18(6):441-6.



Yenidoanda Kardiyoloji Konsltasyonu:Kardiyak Anomali Skl ve

Etiyolojik Faktrler

ZZEETT AAmmaa:: Bu alma, hastanemiz yenidoan nitesine yatan hastalar arasnda ocuk kardiyolojisi kon-sltasyonu istenenlerde konjenital kalp hastalklarnn (KKH) skl ve dalm ile etyolojik faktrlerinideerlendirmek amacyla yapld. GGeerree vvee YYnntteemmlleerr:: almamz, 4553 hasta arasndan ocuk kardiyo-lojisi konsltasyonu istenen 573 hasta prospektif olarak incelenerek gerekletirildi. Olgularn antenataltan ve bulgular, antenatal risk faktrleri (diyabet, preeklampsi, erken membran rptr, enfeksiyon, oli-gohidramnios, polihidramnios, uyuturucu madde ve ila kullanm), ekokardiyografi yaplma nedenleriile elde edilen tanlar arasndaki ilikiye bakld. BBuullgguullaarr:: almaya alnan 573 hastann130unda (%22,6)KKH tespit edildi. Konjenital kalp hastal tespit edilen 130 hastann %8,4 siyanotik, %91,6s asiyano-tik KKHye sahipti. Asiyanotik KKHler arasnda en sk ventrikler septal defekt (%42,3,), atriyal septal de-fekt (%35,3), patent duktus arteriyozus (%9,2), pulmoner stenoz (%7) grlrken, siyanotik KKHler iindeFallot tetralojisi (%3) ile byk arter transpozisyonunun (%1,5) en sk olduu tespit edildi. Konjenitalkalp hastal tans alan olgularn %96,1inin kardiyovaskler sistem d nedenlerle hastaneye yatrldgrld. ocuk kardiyolojisi konsltasyonu isteme sebeplerinin banda en sk takipne (n=183), saturas-yon dkl (n=100) ve frm (n=73) vard. Konjenital kalp hastal grlme sklnn zellikle e-itli anomalilere sahip ve sendromlu olgularda artt ortaya konarak genetik sendrom tanl ya dasendromik zellikleri olan bu olgularn %50sinde KKH olduu tespit edildi. Down sendromlu olgularn%71inde KKH tespit edilmi olup bu KKHlerin %57sinin atriyoventrikler septal defekt olduu grld.SSoonnuu:: Konjenital kalp hastalklarnn yenidoan dneminde asemptomatik olabilecei gibi, konjestif kalpyetersizlii bulgular, siyanoz ile ortaya kabilecei zellikle eitli anomalilere sahip ve sendromlu ol-gularda sklnn artt bir kez daha ortaya kondu.

AAnnaahh ttaarr KKee llii mmee lleerr:: Yenidoan; kardiyoloji konsltasyonu; doumsal kalp hastal

AABBSS TTRRAACCTT OObbjjeeccttiivvee:: This study is performed in order to detect congenital heart disease incidence andthe etiological factors that contributed in patients who were hospitalized in neonatal care unit in our hos-pital and consulted to pediatric cardiology. MMaatteerriiaall aanndd MMeetthhooddss:: Our study was performed with 573 of4553 patients who were consulted to pediatric cardiology prospectively. The antenatal diagnosis and find-ings of the patients, the antenatal risk factors (diabetes, preeclampsia, early membrane rupture, infection,oligohydramnios, polyhydramnios, narcotic and drug usage), the reason of echocardiographic evaluationand the disease that were found were evaluated and the relationship between the findings were evaluated.RReessuullttss:: Congenital heart failure was found in 130 (%22,6) of 573 patients who were enrolled to the study.%8,4 were cyanotic and %91,6 were non-cyanotic in these 130 patients. While ventricular septal defect(%42,3), atrial septal defect (%35,3), patent ductus arteriosus (%9,2) and pulmonary stenosis (%7) weremost frequently seen in non-cyanotic patients, tetralogy of Fallot (%3) and big artery transposition (%1,5)were most frequently in cyanotic patients. The %96.1 of patients with congenital heart disease were hos-pitalized because of other reasons rather than cardiac diseases. Tachypnea (n: 183), desaturation (n: 100),and murmur (n: 73) were the most frequent consultation to cardiology reasons. The incidence of con-genital heart disease is again found to be increased in patients with several congenital anomalies and syn-dromes and %50 of these patients had congenital heart disease. Congenital heart disease was found to be%71 of the patients with Down syndrome and %57 was atrioventriculer septal defect. CCoonnlluussiioonn:: In neona-tal period, it may be either asymptomatic or congestive heart failure and cyanosis may be seen. The inci-dence of congenital heart disease is again found to be increased in patients with several congenitalanomalies and syndromes.

KKeeyy WWoorrddss:: Newborn; consultation to cardiology; congenital heart disease


Saadet ELK CENGZ,a

Ali Rahmi BAKLER,b

Ula KARADA,b

Kay ELAIK,a

ule DEMR,a

Buket DORUSZ,b

Esra ARUN ZERc

aocuk Sal ve Hastalklar Klinii,bocuk Kardiyolojisi Klinii,cYenidoan Klinii,zmir Tepecik Eitim ve Aratrma Hastanesi, zmir


Ya z ma Ad re si/Cor res pon den ce:Ula KARADAzmir Tepecik Eitim ve Aratrma Hastanesi,ocuk Kardiyolojisi Klinii, zmir,TRKYE/[email protected]


ORJNAL ARATIRMA

YENDOANDA KARDYOLOJ KONSLTASYONU: KARDYAK ANOMAL SIKLII... Saadet ELK CENGZ ve ark.


onjenital kalp hastalklar, doutan olananomalilerin iinde en sk rastlanlandr.Bir yenidoann konjenital kalp hastalkl

doma riski 1000 canl doumda 8-12dir (yaklak%1).1-3

Konjenital kalp hastalklarnn ou olgudanedeni bilinmemekle birlikte, genetik ve evre-sel faktrlerin birlikte etkileimine bal olduudnlr. Yenidoan dneminde KKHlerin kli-nik bulgular anatomik bozuklua gre deikenlikgsterir. Solunum zorluu, siyanoz, ok tablosu gibiar belirtilerden veya asemptomatik olarak fizikmuayenede tek bana frm saptanmasndan yada genetik bir sendromun bulgularndan yola k-larak yaplan ekokardiyografik deerlendirmedetespit edilebilir.4,5

Tannn erken konularak tedavi planlanmas,konjenital kalp hastalkl ocuklarn morbidite vemortalitesinin azaltabilmesi asndan nemlidir.Ancak klinik bulgular erken dnemde ortaya k-mayabilir. Tandaki gecikmeler nedeniyle erken te-davi ve giriimlerin uygulanamamas durumundabu hastalklar lmcl seyredebilir. En erken tanise, prenatal dnemde yaplan fetal ekokardiyografiile konulabilir.6-8

Bu alma, hastanemiz yenidoan nitesineyatan hastalar arasnda ocuk kardiyolojisi konsl-tasyonu istenmi olanlarda KKHnin skl ve da-lm ile etiyolojik faktrlerini deerlendirmekamacyla yapld.

GERE VE YNTEMLER

almamz, 1 Kasm 2012-30 Haziran 2014 tarih-leri arasnda zmir Tepecik Eitim ve AratrmaHastanesi yenidoan nitesinde yatrlan 4553hasta arasndan ocuk kardiyolojisi konsltasyonuistenen 573 hasta prospektif olarak incelenerek ger-ekletirildi. alma ncesi hastanemiz etik kurulizni alnd. Hastanemiz blge hastanesi olup yeni-doan nitesi 3. dzey ve 50 yatak kapasitelidir.

almaya alnan olgularn hastanede yatlarsrasnda gebelik ya, doum arl, cinsiyeti,doum ekli, postnatal ya, akraba evlilii varl,anne ve baba ya, varsa antenatal tan ve bulgular,antenatal risk faktrleri (diyabet, preeklampsi,

erken membran rptr, enfeksiyon, oligohidram-nios, polihidramnios, uyuturucu madde ve ilakullanm), yat anndaki tans, fizik muayenebulgular, ekokardiyografi yaplma nedeni, ekokar-diyografi yaplma srasnda postnatal ya, ekokar-diyografi sonular kaydedildi.

Ekokardiyografik inclelemeler tm hastalaraayn pediatrik kardiyoloji uzman tarafndan, So-nosite M-turbo ekokardiyografi cihaz ile yapld.Her iki atriyum arasnda renkli Doppler ile restrik-tif olmayan soldan saa ant oluturan defektler se-kundum tip atriyal septal defekt (ASD) olarak kabuledildi. Restriktif gei oluturanlar patent foramenovale (PFO) kabul edilerek bu olgular almayaalnmad. Prematre bebeklerde periferik vasklerdirencin d hz ve duktusun oksijene verdiiyanta bal olarak ak kalma sresi uzamaktadr.9

Bu nedenle patent duktus arteriyozusa (PDA) sahiptm prematr bebekler ile izlemde PDAs kapananmatr bebekler almaya alnmad.

almada, KKHnin skl ve dalm deer-lendirildi. Olgularn antenatal tan ve bulgular, an-tenatal risk faktrleri (diyabet, preeklemsi, erkenmembran rptr, enfeksiyon, oligohidromnioz,polihidromnioz, uyuturucu madde ve ila kulla-nm), ekokardiyografi yaplma nedenleri ile eldeedilen tanlar arasndaki ilikiye bakld.

statiksel analizler SPSS 21.0 International Bu-siness Machines Corporations bilgisayar programile yapld. Sonular ortalama-standart sapma, or-tanca ve yzdelik deerler eklinde belirtildi.

BULGULAR

alma grubuna dahil edilen 573 hastann 314(%54) erkek, 259u (%46) kz olup196s (%34)matr, 377si (%66) prematr bebek olup; alma-daki hastalarn ortalama gebelik sresi 33,55,3hafta, doum arlklar 21461075 gr idi. Hastala-rn kardiyoloji konsltasyonlar ortanca olarakpostnatal 3 gnde (1-130 gn) gerekletirildi.Hasta bebeklerin ebeveynlerinin 96snda (%17)akraba evlilii mevcuttu. Ortalama anne ya286,1, baba ya 316,6 ya idi.

almaya alnan 573 hastann130unda(%22,6) KKH tespit edildi. Bu 130 olgunun 57si

Saadet ELK CENGZ ve ark. YENDOANDA KARDYOLOJ KONSLTASYONU: KARDYAK ANOMAL SIKLII...


(%44) erkek, 73 (%56) kz olup; gebelik sresi35,54,7 hafta, doum arlklar 2445962 gr idi.Postnatal ortanca tan ya ise 3 (1-120 gn) gnd.Hasta bebeklerin ebeveynlerinin 23nde (%17,6)akraba evlilii mevcuttu. Ortalama anne ya27,26,5, baba ya 30,66,6 ya idi.

Konjenital kalp hastal tespit edilen 130 has-tann %8,4 siyanotik, %91,6s asiyanotik KKHyesahipti. Asiyanotik KKHler arasnda en sk ventri-kler septal defekt (VSD) (%42,3) grlrken, si-yanotik KKHler iinde Fallot tetralojisininin (%3)en sk olduu tespit edildi (Tablo 1).

almamzda asiyanotik KKHli hastalarn%61i kz iken siyanotik KKHli hastalarn%27sinin kz olduu grld. Atriyal septal defekt(ASD) (%54 kz), VSD (%66s kz) ve pulmonerstenoz (PS) (%60 kz) kzlarda daha sk grlr-ken atriyoventrikler septal defektin (AVSD)(%56s erkek) erkeklerde daha sk olduu tespitedildi. Patent duktus arteriyozus ise kz ve erkekolgularda eit orandayd.

Konjenital kalp hastalklar iin antenatal riskfaktr saptanan 139 olgunun annelerinin 50sindediyabet (%36), 60nda (%43) preeklampsi (8 olgudadiyabet ve preeklapmsi birlikte idi), 14nde (%10)

erken membran rptr, 11inde (%7,9) oligohid-ramnios, 2sinde (%1,5) polihidramnios, 1inde(%0,75) antikonvlsan ila kullanm, 1inde(%0,75) uyuturucu madde kullanm tespit edildi.Risk faktrlerine gre KKH saptanma oranlar vetipleri Tablo 2de gsterildi.

Konjenital anomaliler nedeniyle sendrom d-nlen ancak kesin tans konamam, Down sen-dromu gibi kesin tans olan ve orta hat defekti ileanorektal malformasyonu olan hastalarda KKHninsaptanma oranlar ve dalm ise Tablo 3te gste-rildi.

ocuk kardiolojisi konsltasyonu istem sebep-leri ve ekokardiyografide KKH saptanma sklTablo 4te gsterildi. frm (n=73) nedeniyle eko-kardiyografi (EKO) yaplarak KKH tespit edilen 41(%56) olgunun 20sinde (%49) VSD, 10unda(%24,5) ASD, 2sinde (%5) aort koarktasyonu (AK),2sinde (%5) Fallot tetralojisi ve 7sinde (%17) PStespit edildi. Desaturasyon (n=100) nedeniyle EKOyaplarak KKH tespit edilen 23 (%23) olgunun8inde siyanotik KKH (bu hastalarn 2si Fallot tet-ralojisi, 2si byk arter transpozisyonu (BAT), 2sitrunkus arteriyozus (TA), 1i hipoplastik sol kalpsendromu ve 1i tek ventrikl idi) tespit edildi.

Olgu says KKH iinde oran (%)Asiyanotik tan

VSD 55 42,3

ASD 46 35,3

PDA 12 9,2

PS 10 7

AVSD 9 6,9

AK 5 3,8

Kesintili arkus aorta 1 0,7

Siyanotik tan

Fallot tetralojisi 4 3

BAT 2 1,5

Pulmoner atrezi 1 0,76

Hipoplastik sol kalp 1 0,76

Trunkus arteriozus 1 0,76

ift kl sa ventrikl 1 0,76

Tek ventrikl 1 0,76

TABLO 1: Asiyanotik ve siyanotik konjenital kalphastalklarnn dalm.

ASD: Atriyal septal defekt; AK: Aort koarktasyonu; BAT: Byk arter transpozisyonu;AVSD: Atriyoventrikler septal defekt; PDA: Patent duktus arteriyozus; PS: Pulmonerstenoz; VSD: Ventrikler septal defekt.

Risk faktrleri (n) KKH Olgu says % Diyabet (50) Hipertrofik 2 4

kardiyomiyopati

ASD 2 4

VSD 1 2

PS 1 2

Preeklampsi (60) ASD 3 5

VSD 5 8,3

Bikspit aorta 1 1,6

Fallot tetralojisi 3 5

PA 1 1,6

AVSD 1 1,6

Diyabet ve Hipertrofik 1 12,5

preeklampsi (8) kardiyomiyopati

Erken membran rptr (14) AVSD 1 16,6

Oligohidramnios (11) VSD 1 9

ASD 2 18

Antikonvlsan ila (1) VSD 1 100

Uyuturucu (1) Normal 1 0

TABLO 2: Risk gruplarna gre konjenital kalp hastalklar ve oranlar.


Dier hastalarn 8inde (%35) ASD, 6snda (%26)VSD, 1inde (%4) kesintili arkus aorta olduu g-rld. Hidrops fetalis (n=10) nedeniyle EKO yap-larak KKH tespit edilen olgularn 1inde musklerVSD, 1inde muskler VSD ve bikspit aorta bir-likte saptand. Fetal EKOsunda patolojik bulgu ol-mas (n=7) nedeniyle EKO yaplan olgularn 6snda(%85) KKH saptanm olup bu hastalarn 2sindeVSD, 1inde sekundum ASD, 1inde trunkus arte-riyozus, 1inde kesintili aortik ark, 1inde intra-kardiyak kitle tespit edildi. Aritmi nedeniyledeerlendirilen 39 olgunun 7sinde supraventrik-

ler taikardi (SVT), 2sinde erken atrial vuru, 1indeerken ventrikler vuru, 5inde bradikardi ve24nde sinzal taikardi tespit edilmi olup SVTliolgulardan 1inde (%14) sekundum ASD, bradikar-disi olan 1 olguda sekundum ASD ve taikardisiolan 1 olguda AVSD saptand.

TARTIMA

Konjenital kalp hastalklarnn gerek skln sy-leyebilmek iin bu hastala sahip olan olgularntmne tan konulmas gerekmektedir. Ancak bubaz nedenlerden dolay mmkn deildir. Bu ne-denlerin banda, hastalklarn doru tansn koy-mak iin gerekli tbbi donanm ve deneyimlipediatrik kardiyoloun henz birok lkede yeterlidzeyde olmamas gelmektedir. Bunlarn dndaFallot tetralojisi ve geni VSD gibi hemen klinikbulgu veren ve tannan kardiyak patolojiler ya-nnda minimal PS, kk VSD ve ASD gibi lez-yonlar ou zaman bulgu vermedikleri gibi rutinfizik muayenede de gzden kaabilirler. Ayrca do-umdan hemen sonra oluan yenidoan lmleri-nin bir ksmndan da KKH sorumlu olup buhastalarda tan konmas iin iin yeterli sre ola-mamaktadr. Tm bu nedenlerden dolay bildirilensklklarn gerek sklklardan daha az olduu d-nlmektedir.10,11

almamzda ocuk kardiyolojisi konsltas-yonu istenen 573 olguda KKH skl %22,6 (130olgu) olarak bulundu. Bulut ve ark.nn alma-snda, takiplerde PDAs kapanan matr yenido-anlar, izole PDA saptanan prematreler ve izolePFO saptanan hastalar almaya dahil edilmedi-inde bizim almamzdaki gibi KKH skl 367yenidoanda %28 olarak bildirilmitir.12 Gven veark.nn 201 yenidoan ile yaptklar almada iseKKH saptanma sklnnn %76 gibi yksek biroranda olduu grlmtr.13 almalar arasndakioranlarn bu kadar farkl olmasnn sebebi, yenido-an hekimlerinin semptomlara duyarllnn farklolmas nedeniyle ocuk kardiyolojisi konsltas-yonu isteme sklndaki farkllk ve almaya al-nan hastalarn klinik zellikleri (dismorfik hastalar,kromozom bozuklular olan hastalar, ekstrakardi-yak anomalileri olan hastalar vb) gibi baka neden-ler ile aklanabilecei dnlmtr.

Tan Ekokardiyografi sonucu/n %Konjenital anomali (n=18) Normal (n=16) 89

Sekundum ASD (n=1) 5,5

Muskuler VSD (n=1) 5,5

Down sendromu (n=14) Normal (n=4) 29

AVSD (n=8) 57

VSD (n=1) 7

VSD + ASD (n=1) 7

Tracher-Collins sendromu (n=1) Sekundum ASD (n=1) 100

Pierre-Robin sendromu (n=2) Normal (n=2) 0

Konj. kistik adenomatz mal. (n=1) Normal (n=1) 0

Koolen de Vries (n=1) Sekundum ASD (n=1) 100

Bohring-Opitz sendromu (n=1) Sekundum ASD (n=1) 100

Smith-Lemli-Opitiz sendromu (n=1) Sekundum ASD (n=1) 100

Amniyotik band sendromu (n=1) Normal (n=1) 100

Dandy-Walker sendromu (n=2) Normal (n=2) 0

Ambigious genitale (n=1) Normal (n=1) 0

Anorektal malformasyon (n=1) Sekundum ASD (n=1) 100

TABLO 3: Konjenital anomalili ve sendromlu olgulardakonjenital kalp hastalklarnn dalm

Endikasyon Olgu says KKH skl n/%frm 73 41/56

Saturasyonda dme 100 23/23

Takipne 183 33/18

Dolam bozukluu 55 9/16

Non-immn hidrops fetalis 10 2/20

Konjenital anomali 83 17/20

Maternal diyabet 50 9/18

Fetal EKOda patoloji 7 6/85

Disritmi 39 3/7,6

TABLO 4: Ekokardiyografi endikasyonlar ve konjenital kalp hastal skl.


Saadet ELK CENGZ ve ark. YENDOANDA KARDYOLOJ KONSLTASYONU: KARDYAK ANOMAL SIKLII...


Siyanotik olmayan KKHler iinde en sk VSD(%25-30) grlrken, siyanotik KKHler iinde ensk BAT (%5-6) grlmektedir.14 Bulut ve ark.nnyapt almada VSD (%34,3) en sk asiyanotikKKH iken BATn (%6,6) en sk siyanotik KKH ol-duu grlmtr.12 Ayn almada PDA %14,3,ASD %10,5, AK %6,6, AVSD %5,7, Fallot tetralo-jisi %4,8, PS %4,8, PA %3,8, hipoplastik sol kalpsendromu %2,9, tek ventrikl %1,9 sklkta bil-dirilmitir. Bizim almamzda da asiyanotikKKHler iinde en sk (%42,3) VSD tespit edilirken,siyanotik KKHler iinde Fallot tetralojisi %3, BATise %1,5 orannda grlmtr. Konjenital kalphastalklarnn dalmna bakldnda literatrleuyumlu olarak VSD (%42,3), ASD (%35,3), PDA(%9,2) ve PS (%7) en sk tespit edilen KKHler ikenalmamzda siyanotik KKH orannn dier al-malara gre daha dk olduu grlmtr. Budurumun hastanemizde henz yenidoan kalp cer-rahisi yaplamamasna bal olduu dnlm-tr.

almamzda cinsiyet dalmna bakldndaKKHli olgularn %56s kz idi. Siyanotik KKHyesahip olgularn %73 erkek iken asiyanotikKKHye sahip olgularn ise %61i kzd. Bulut veark.nn yapt almada da benzer oranlar bildi-rilmi olup KKHli olgularn %54,3 kz olup siya-notik KKHli olgularn %64nn erkek olduugrlmtr.13 Literatrde de ciddi KKHlerin er-keklerde daha sk olduu, ASD ve PDAnn kzlardadaha sk grld bildirilmektedir.9,15,16 Bizim a-lmamzda da ASDli olgularn %54 kz olupPDAl olgularda cinsiyet fark saptanmamtr.

Konjenital kalp hastalklarnn geliiminde ge-netik ve evresel faktrlerin neden olduu multi-faktriyel bir kaltm sz konusudur.17 Annedekidiyabetes mellitus, romatoid artrit ve sistemiklupus eritematozus gibi kollajenozlar, fenilketo-nri, koaglopati ve sferositozis gibi hematolojikbozukluklar ile annenin hamilelikte ila (lityum,talidomid, warfarin, alkol, antikonvlsanlar vb) al-mas, doumsal rubella gibi durumlarn ocuklardakonjenital kalp hastal grlme skln artrdbilinmektedir.18,19 almamzda diyabetik anne be-beklerinde hipertrofik kardiyomiyopati (HKMP)grlme skl %6 tespit edilmitir. Preeklampsili

anne bebeklerinin %35inde KKH saptanmtr.Ayrca saylar az olmasn ramen, erken memeb-ran rptr ve oligohidramniosa sahip annelerinbebeklerinde de KKH sklnn artt grlm-tr. Preeklampsinin, erken membran rptrnnve oligohidramniosun konjenital kalp hastal ge-liimini ve skln etkileyip etkilemedii konu-sunda literatrde yeterli alma bulunmamaktadr.Bu konuda yorum yaplabilmesi iin ok saydahastay ieren vaka-kontrol almalarnn yapl-mas gerektii dnlmtr.

Birok genetik sendrom ve dier sistem mal-formasyonlar ile KKH birliktelii bilinmektedir.9

KKHlerin %5-8i de kromozom anomalileri ile be-raber grlmektedir. rnein Down sendromu(trizomi 21) ile ilikili konjenital kalp defektleri iyitanmlanm ve bu sendroma sahip ocuklarn yak-lak %40nda aikar kalp hastal (zellikleAVSD) gsterilmitir. Atriyoventrikler septal de-fektli ocuklarn da %75inin Down sendromlu ol-duu tespit edilmitir.16 almamzda genetiksendrom olabilecei dnlen tans konmamanomalili ve tans konmu sendromik olan 34 ol-gunun %50sinde KKH saptanmtr. Down sen-dromlu olgularn %57sinde AVSD tespit edilmitir.Bulgularmzn literatrle uyumlu olduu d-nlmtr.

Konjenital kalp hastal olan yenidoanlarzellikle yaamn ilk gnlerinde asemptomatikolabileceinden ounlukla kalp damar sistemidndaki nedenlerle hastaneye yatrldn d-nmekteyiz. Bulut ve ark.nn yapt almadaKKH tans alan hastalarn %83,9unda, Gven veark.nn yapt almada KKH tans alan hastala-rn %59unda hastaneye yat nedeninin kardiyo-vaskler sistem d nedenlerden kaynaklandbildirilmitir.12,13 Bizim almamzda ise bu orann%96,1 olduu grlmtr. almamzda bu ora-nn yksek olmas hastanemizde henz yenidoankalp cerrahisinin yaplamamasna balanmatr.

frm varl genellikle KKH ile ilgili olsa dafrm olan bebeklerin ounda yapsal lezyonyoktur ve KKH grlen bebeklerin hepsinde f-rm tespit edilmeyebilir.20 frmn saptanmasayrca muayene eden kiinin yetenek ve deneyi-


mine, zamanlama, frekans ve muayenenin hangikoullar altnda yapldna baldr.20 Bir almada7204 yenidoan bebein 46snda frm saptana-rak ekokardiyografi uygulanm, bu olgularn13nde kalp normal olarak deerlendirilmi, 8 be-bekte frm fizyolojik nedenlere balanm (fiz-yolojik hafif periferik pulmoner stenoz gibi) olup25 bebekte (%54) frmn kardiyak malformas-yona bal olduu tespit edilmitir. Bu hastalarnda tm asemptomatik olup 15inde VSD, 3ndeAK, 3nde Fallot tetralojisi, 2sinde ASD, 1inde PSve 1inde aort kapak stenozu saptanmtr.21 al-mamzda takipne ve desaturasyondan sonra f-rm 3. en sk konsltasyon isteme sebebi olupfrm tespit edilen olgularn %56snda KKH tes-pit edilmitir.

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