Cardiopulmonary support in critically ill children

林盈瑞 醫師

Lung vs. Heart – presentation

Cyanosis

Tachypnea

Etiology of cyanosis

• CNS

• Respiratory disease

• Cardiovascular disease

• Methemoglobinemia

• Other – hypoglycemia, adrenogenital syndrome, polycythemia, blood loss,sepsis

Approach of cyanosis

+

History PE

Lab

Hyperoxitest

PGE1

Cyanosis in newborn

• TGA – most common• TOF with pulmonary atresia – 1.5-2% of CHD• Tricuspid atresia –1-3 % of CHD• Pulmonary atresia with intact ventricular septum – 1% of

CHD• Persistent Truncus arteriosus – < 1%of CHD• Single ventricle -- <1%,varies degree of cyanosis• TAPVR with and without pulmonary venous obstruction –

1% of CHD• Ebstein’s anomaly – in severe case,cyanosis and CHF d

uring first few days and improving after pulmonary vascualr resistance decrease

氧氣對心血管影響

• 下降肺血管阻力 右心 afterload 下降 血液更容易到肺部

• 提高血液中氧氣濃度 減少心臟做功及增加冠狀動脈氧氣濃度

Ventilator 使用對心跳的影響

• PPV 增加 lung volume reflex bradycardia

Ventilator 使用對右心室功能的影響

• PPV intrathoracic pressure ↑ RA pressure ↑ RV preload ↓ RV output ↓

• PVR 的影響 :End-expiratory lung volume < Functional residual cap

acity hypoxic pulmonary constriction and the tortuous course of large to medium-sized pulmonary vessels PVR ↑

End-expiratory lung volume > Functional residual capacity alveoli hyperexpansion compression of the pulmonary capillaries PVR ↑

Ventilator 使用對左心室功能的影響

• LV preload ↓ RV volume ↓ RV pressure ↑ LV compression

• LV afterload change↓

肺高壓 (Pulmonary hypertension)

定義 – 平均肺動脈壓在休息時 > 25 mmHg 或運動時大於 30 mmHg

肺高壓原因 (1)• Related to Hypoxic Pulmonary Hypertension

– Long-term high altitude dwelling – Restrictive respiratory dysfunction

• Obesity • Kyphoscoliosis • Neuromuscular disorders • Severe pleural fibrosis • Lung resection

– Chronic upper airway obstruction • Congenital webs • Enlarged tonsils • Obstructive sleep apnea

– Chronic lower airway obstruction • Chronic bronchitis • Asthmatic bronchitis • Bronchiectasis • Cystic fibrosis • Emphysema

– Chronic diffuse parenchymal disease • Interstitial fibrosis • Pneumoconioses • Granulomatous disease • Alveolar filling disorders • Connective tissue disorders (scleroderma, rheumatoid lung)

肺高壓原因 (2)

• Related to Arterial Obstructive Pulmonary Hypertension– Thrombotic disease

• Sickle cell disease • Coagulation disorders

– Embolic disease • Chronic thromboemboli • Tumor emboli • Schistosomiasis

– Connective tissue disorders/vasculitis • Lupus • Systemic sclerosis

– Chronic liver disease (portal hypertension) – Hematologic problems (myeloproliferative abnormalities)

肺高壓原因 (3)• Related to Pulmonary Venous Hypertension

– Aortic • Coarctation • Supravalvular aortic stenosis

– Left ventricle • Aortic stenosis • Aortic regurgitation • Congenital subaortic stenosis • Hypertrophic cardiomyopathy • Constrictive pericarditis • Restrictive cardiomyopathy • Dilated cardiomyopathy • Mitral stenosis • Mitral regurgitation

– Left atrium • Ball-valve thrombus • Myxoma • Cor triatriatum

– Pulmonary veins • Congenital pulmonary vein stenosis • Mediastinitis • Mediastinal fibrosis • Mediastinal neoplasm • Radiofrequency ablation for atrial fibrillation

肺高壓原因 (4)

• Related to Pulmonary Hypertension due to Left-to-Right shunts– Patent ductus arteriosus – Aortopulmonary window – Rupture of aortic sinus – Ventricular septal defect – Atrial septal defect

肺高壓原因 (5)

• Idiopathic Pulmonary Arterial Hypertension

• Familial Pulmonary Arterial Hypertension

臨床表現• Right side heart failure – 水腫 , 肝脾腫大 , 心率不整 , 昏倒 , cyanosis

• Left side heart failure – 因左心 preload 下降及右心室壓迫左心室造成 – 昏倒 , 咳血 , 胸悶 , 頭痛 , 易疲倦

治療•去除 underline disease

矯正心臟疾病矯正上呼吸道阻塞問題矯正肺部疾病

治療• 避免上升肺壓及處理併發症

避免劇烈運動 , 避免到高山區 視需要給予氧氣 利尿劑 控制心衰竭 給予避免血小板凝集藥物 (如 aspirin) 治療心率不整 打流感疫苗 避免增加肺血管收縮因子 – 如 alveolar hypoxemia, acidosis, hypercapnia, vasoconstrictor drugs, fat emulsions, hypothermia, and high PEEP

治療• 使肺壓下降

改善 O2, Hyperventilation inotropic agent vasodilator High-dose Ca-block + anticoagulants benefit in ad

ult patients NO useContinuous IV or inhalation prostacycline (PGI2) Bosentan transplantation

如何使肺高壓下降• PH↑

• PaCO2↓

• PaO2 and PAO2↑

• 適當的 intrathoracic pressure (PEEP)

• Inhaled NO

肺壓對心臟病人的影響• L-to-R shunt – ASD, VSD, PDA 肺壓下降會使分流增

加 心衰竭情況可能加劇• Left side obstruction 的 PDA dependent CHD – Crit

ic CoA, interrupted aorta, hypoplastic left heart 肺壓下降會使血流不易從肺動脈經動脈導管到周邊 周邊組織灌流不足

• Parallel flow 的 PDA dependent CHD – TGA 肺壓下降會使左心面對低的後負荷 久了可能造成左心室心肌退化之後無法接受全矯正

• Right side obstruction 的 PDA dependent CHD – TOF, Pulmonary atresia, Tricuspid atresia 肺壓下降會使肺血流增加 Cyanosis 改善

ECMO(Extracorporeal Membrane Oxygenato

r)

體 外 循 環 機( 葉 克 膜 )

What is ECMO?

• First from 1971

• Oxygenation outside the body

• Support heart and/or lung function

• Similar to bypass used in the operating room but can be used for longer periods of time

How ECMO Works ?

• Pumping blood out of the body; oxygen is added to the blood and carbon dioxide is removed before it is returned to the patient.

• Pumping a steady amount of blood through the ECMO machine each minute. We call this the ECMO flow rate.

• As the patient improves, we may decrease the flow rate and let their heart and lungs do more of the work.

Are there different types of ECMO?

• veno-arterial or VA ECMO

• veno-venous or VV ECMO

VA ECMO

可取代心臟及肺臟功能

VV ECMO

只能取代肺部功能

Babies will usually have a

cranial ultrasound study: bleeding around the brain?

cardiac ultrasound study: heart function which type of ECMO to use (VA or VV).

What is the goal of ECMO?

•確定身體有足夠氧氣應供應• ECMO 並不能治療心臟及肺臟疾病本身 ,只是爭取時間來恢復其功能

•使用期間可降低呼吸器設定條件以避免肺部再受傷害

•可降低強心藥物的劑量及種類

Why ECMO?

• Severe pulmonary hypertension - high blood pressure in the lungs

• Pneumonia

• Respiration failure from trauma or severe infections

• Heart failure

體外膜肺之使用參考： (91/12/01 生效 )

一、 心因休克（一） 心臟手術重建後，造成右心室衰竭併可回復性肺高壓危機。（二） Bridge ：為準備心臟手術或心室輔助器或心臟移植，而暫代之。（三） 心臟手術後暫時左心室功能喪失（ stunned heart ）常見於 prolong bypass 。 (四） 可回復性的心肌病變，如心肌炎、冠狀動脈暫時性痙攣。（五） 先天性心臟病疾病手術重建後，心輸出量不足造成單側或雙側心衰竭。（六） 肺栓塞或肺梗塞（ pulmonary embolism or infarction ）。

二、 呼吸衰竭（一） ARDS （成人呼吸窘迫症候群）1. Qsp/Qs>30﹪（ intrapulmonary R to L shunt ）： normal<5﹪2. Peak airway pressure>45cmH2O3. TSLCs<30ml/ cmH2O （ Total Static Lung Compliance ）4. ARDS Typical X-ray and/or CT scan5. FiO2： 1.0, PaO2<50mmHg, PEEP> 5cmH2O

（二） 小兒及新生兒1. 吸入性胎便肺炎症候群（ MAS； Meconium aspiration syndrome ）2. 呼吸窘迫症候群（ Hyaline membrane disease ）3. 先天性橫膈膜疝氣（ CDH； Congenital diaphragm hernia ）

4. 新生兒頑固性肺高壓（ PPHN ； persistent pulmonary hypertension of neonate ）

（一） 外科手術或外傷後 24小時內（二） 頭部外傷併顱內出血 72小時內（三） 缺氧致腦部受損（四） 惡性腫瘤患者（五） ARDS 併 COPD（六） 病患在放置 ECMO前有明顯不可逆轉之病況（七） 已證實不可逆轉之腦傷害（八） 持續進展之退化性全身性疾病

禁忌症

Exclusion Criteria:

• Lethal chromosomal anomaly

• <34 weeks gestational age

• <2000gms

• Coagulopathy that is ongoing/uncorrectable

• Mechanical ventilation for > 14 days

• Severe neurologic insult or asphyxiaUniversity of Minnesota Department of Pediatrics

Risk of ECMO

•出血•感染 •輸血反應•血栓•血管傷害•中風•機器故障

What happens on ECMO?

• Turn the ventilator settings down to "rest" settings.

• Turn down many of the heart medicines.

• Daily routine; chest x-ray each morning, weight and bath, blood samples (labs) and constant monitoring of vital signs.

• Let the patients rest as much as possible

Medications

• Pain medication • Heparin • Antibiotics prophylaxis • Dopamine, Dobutamine….• TPN or enteral nutrition when possible

測 ABG ：– Pulse oximeter放右手，右手的 SaO2 反應病人自己的心肺功能；左手的 SaO2 比較反應出 ECMO 之 SaO2。

– VV mode ：一般不用調整設定， FiO2: 1.0， gas flow: 10L/min 。

– VA mode ：在調整穩定後，只要 ECMO SaO2 沒有變化，不用測 ECMO ABG 。

–病人之 ABG ：依醫囑測。

病例介紹•年齡 : 13 歲小女孩•到院主訴症狀 :意識障礙

最近 1~2 天有輕微感冒現象入院前 1天有胸痛情形在學校出現全身無力、冒冷汗等症狀且合併意識障礙而被送至本院急診

過去病史• 無個人心臟方面疾病• 無家族心臟方面疾病• 無服用藥物病史

住院過程•急診心電圖顯示心室頻脈 •對電擊 , 給藥及心臟按摩沒反應•予以插管及裝上體外心肺循環機• 3 天後順利移除體外心肺循環機及插管•目前病人恢復狀況良好

95-08-30 08:32

95-08-30 17:10

95-09-04 11:52

95-09-01

95-09-04

題目 1•下列所述何者不是新生兒發紺 (Cyanosis) 的原因 ?

(A) 先天性肺炎 (B) 法洛氏四合症 (C) 腦部損傷 (D) 以上皆會造成發紺

題目 2•針對使肺壓下降的方法 ,下列何者為非 ? (A) Hyperventilation (B) NO 使用 (C) 調高 PEEP (D) 使 PH值上昇

題目 3• 太高的正壓呼吸對心臟的影響下列何者為非 ?

(A) 會使心跳變慢 (B) 使右心室輸出血量減少 (C) 使左心室前負荷下降 (D) 以上皆對

題目 4• 對於 ECMO 之敘述何者為非 ? (A) ECMO 可取代肺部功能 (B) ECMO 可取代心臟功能 (C) ECMO 可治療心肌炎 (D) ECMO 使用期間可降低 ventilator setting 條件

題目 5•下列何種病人不適合使用 ECMO (A) 猛爆性心肌炎 (B) Duchenne muscular dystrophy 病人合併心肌病變所致之心臟衰竭

(C) 成人呼吸窘迫症候群 (D) 新生兒頑固性肺高壓

History

maternal history( drugs, DM…), prenatal history( PROM, polyhydramnios oligohydramnios,CS or NSD, Meconium aspiration ,asphysia ), family history, intermittent or persistent cyanosis or sudden onset.

Physical examination

• central or peripheral cyanosis, respiratory distress ( RR, retraction, nasal flaring,grunting ) or not,airway patency, heart murmur, differential of cyanosis or not, BT, vital signs, abdomen, neurologic

Lab

CxR, ABG, pulse oximeter, CBC/DC, sugar

methemoglobin level, EKG, hyperoxic test, Brain echo, echocardiography

HyperoxitestFiO2=0.21

PaO2(%Sat)

FiO2=1.00

PaO2(%Sat)

PaCO2

Normal 70 (95) >200(100) 40

Pul. disease 50(85) >150(100) 50

Neurologic disease 50(85) >150(100) 50

Methemoglogulinemia 70(95) >200(100) 35

Cardiac disease

Parallel circulation

Restricted PBF

Complete mixing without restricted PBF

<40(<75)

<40(<75)

50-60(85-93)

<50(<85)

<50(<85)

<150(<100)

35

35

35

PPHN

PFO,no R-to-L shunt

PFO,with R-to-L shunt

Preductal Postductal

70(95) <40(<75)

<40(<75) <40(<75)

Variable

Variable

35-50

35-50

PGE1

• Starting dose : 0.05-0.1 ug/kg/min

• Therapeutic effective ( PaO2 15-20 mmHg)

• If no effect double dose, upto 0.4 ug/kg/min

• Adverse reaction – fever (14%), Apnea (12%), flush ( 10%),hypotension, seizure, arrythmia