Dr. Rakhi Maitrayee Bhowmic

Resident , Phase – ADr. Rumman Resident,

Phase – B Department of Neonatology

Outline:

Definition Incidence Development of Heart Foetal Circulation Classification Aetiology and Association with other

Congenital Anomalies When to suspect? What to do? Cyanotic Heart Disease Acyanotic Heart Disease

Definition

Congenital heart disease: A malformation of the heart, aorta, or other large blood vessels that is the most frequent form of major birth defect in newborns.

Source : www.medicinenet.com

Incidence

10 per 1000 live new born babies2/3 of these manifest in the neonatal periodOf these incidents 1/3 of the babies die during the 1st

week of lifeCardiac malformation may occur

as an isolated anomaly or

combination with other complex anomalies

e.g. VATER Syndrome

Incidence in Bangladesh:

Paediatric heart journal of Bangladesh published an article where they have shown Total 896 no of patient admitted in the year from 2009 to 2014.

It observed that…

1.Majority (59.8%) patients were male ,M:F=1.5:1

2.Majority patients were Acyanotic(82.47%) and rest (17.52%) were cyanotic

3.Distribution of diagnosis:

VSD is most common (28.9%) , ASD(25.1%), PDA(21.6%), TOF(15.3%), PS(13.8%), TR(11.5%)…..and rest are others.

07/05/16

Development of Heart -1

Development of Heart - 2

Foetal Circulation

Source: http://cardiovascularsystemud.weebly.com/

Classification of CHD

CHD

Acyanotic

With Shunt

With Obstruction

Cyanotic

↓PulmonaryBlood Flow

Parallel Circulationwith Poor Mixing Complete Mixing

• ASD• VSD• PDA• Endocardial

Cushion Defect

Left Sided**• Aortic Stenosis• COA• Interrupted

aortic arch• Hypoplastic leftheartRight Sided• Pulmonary

Stenosis• Ebstein Anomaly

• TOF• Tricuspid

Atresia• Critical

pulmonary stenosis

• Single ventricle with PS

• TGA with intact septum

• TGA with VSD

• Total Anomalous Pulmonary Venous connection

• Truncus arteriosus

• Single Ventricle without PS

Etiology

Most cases are multifactorial

07/05/16

Maternal disorders Maternal drugs

Rubella infection (30-35%)PDA, peripheral pulmonary stenosis

SLE (35%)Complete heart block

DM (2%)

Warfarin therapy (5%)PDA, pulmonary valve stenosis

Fetal alcohol syndrome (25%)

ASD, VSD, tetralogy of Fallot

Syndrome Association with CHDSyndrome Cardiac AnomaliesTrisomy 13(Patau Syndrome)

Over 80% have cardiac defect,VSD is the commonestPDA may occur

Trisomy 18( Edward Syndrome)

Over 95% have cardiac defectVSD is very commonPDA may be associated

Trisomy 21(Down Syndrome )

40 -50 % have cardiac defectCommon AV canal & VSD are most common TOF ASD, PDA and rarely complex anomalies are associated

Turner’s Syndrome(Monosomy X)

25 -45 % have cardiac defectCoarctation of aorta & bicuspid aortic valve are common

How to suspect?

If the followings are present:

1.Presence of cyanosis

2.Respiratory distress

3.Unexplained shock/acidosis

4.S/S of Heart failure

5.Presence of murmur.

Key Characteristics: LR shuntsExcessive sweating - Tendency for CCF

Frequent chest infections -Due to decreased lung compliance which leads to frequent respiratory tract infections

Failure to thrive - due to increased workload, persistent heart failure, and frequent respiratory infections with under nutrition

Precordial bulge

Cardiomegaly

Shunt & flow murmurs

Plethoric lung fields

Key Characteristics:obstructive lesions

Absence of frequent chest infections

Normal precordial shape

Forcible/heaving cardiac impulse, without cardiomegaly

Delayed S2

Ejection systolic murmur, with thrill

Normal sized heart with normal pulmonary vascularity

Chest pain- severe aortic stenosis lead to myocardial ischemia

What to do?1.Hyperoxia test to exclude cyanotic CHD

2.Record SpO2 in all four limbs

3.Record BP in all four limbs

4.ABG if not Hyperoxia test

5.CXR

6.ECG

7.Echo

07/05/16

Acyanotic Heart Lesions

Left to Right Shunting---extra volume in right side of heart--increased pulmonary blood flow---increased cardiac work load.

Obstructive Lesions---stenosis of valve or vessel---pressure rises behind obstruction and distal blood flow reduces/absent.

Decreased systemic perfusion and shock can be caused by obstructive lesions

Rarely present at birth

Mostly leads to CHF

PATENT DUCTUS ARTERIOSUS

Failure of closure of fetal ductus arteriosus with continued patency in postnatal life.

Factors assoc. with increased incidence

PrematurityRDSFluid overloadAsphyxiaCongenital syndrome-Rubella,

Trisomy 13,Trisomy 18

Factors associated with decreased incidence

Antenatal steroid administrationIUGRProlonged rupture of membranes

Clinical presentation

Initial presentation may be at birth, usually on days 1- 4 of life

The clinical features assoc with L to R shuntDepend on the magnitude of shunt and the

ability to handle extra volume.

Clinical Signs

Murmur

Hyperactive precordium

Bounding peripheral pulses

Increase in pulse pressure

Hypotension

Respiratory deterioration

Recurrent apnoea in ventilated infants.

Investigation

Echocardiography – direct visualization,

direction of flow,

secondary effect,

rule out alternative diagnosis

Chest X-ray- pulmonary plethora,

cardiomegaly

Management

• Respiratory support:

May need ventillatory support

Increased PEEP – Helpful for pulmonary edema

• Fluid restriction

• Maintain hematocrit

• Diuretics

• PG inhibitor: Indomethacin (PGE1 inhib)

Ibuprofen

Surgical correction

Haemodynamically significant PDA in whom medical treatment has failed or there is contraindication to use indomethacin.

Prognosis

72% spontaneous closure in premature infants <30 weeks.

Conservative treatment (medication) has a closure rate of 94% .

VSD 25% of all CHD Degree of shunt depends upon the size of shunt and

pressure difference . With age PVR decreases, increase blood flow to right and then to pulmonary artery.

With large VSD, pulmonary vessels are exposed to systemic pressure, leading to pulmonary hypertension and pulmonary vascular disease.

Large and moderate defects may present in first few weeks of life.

VSD contd.

CF: tachypnea, dyspnea, FTT, systolic murmur at Left Lower Sternal Border for small VSD, systolic/diastolic murmur if large VSD, pounding heart, frequent resp infections, CHF mostly with large VSD

Complications: CHF, FTT, Endocarditis, Arrhythmia, PAH, Eisenmenger syndrome.

CXR- Cardiomegaly ECG- LVH

VSD contd

Natural History and Management:20-80% closes spontaneouslyDigoxin, diuretics and ACE inhibitors for afterload reductionSmall VSD, mostly medical, 15-50% closes spontaneouslySurgery early in lifeSoft nipples to ease stress of feeding.

ASD 10% of all CHD, F>M

RV compliance lower than LV, leads to L to R shunting. Increased blood flow through ASD, enlarge RA, RV and inc. Pul blood flow.

CF: Most infants and children are asymptomatic.

- Fatigue and Shortness Of Breath

- Palpitations and atrial dysrythmias

- Recurrent pul infections

- Systolic murmur- inc flow across PV

- Wide fixed split S2

Diagnosis and Management

RV enlargement on CXR

RV ↑/RBBB on ECG

Surgery at age 2-5

Natural History:

- If not treated may lead to Atrial dysrrythmias and heart failure.

- Small secondum ASD may close spontaneously during first year of life

- Associated with Holt-Oram syndrome

CXR ASD

Pulmonary Stenosis

Usually associated with ASD, Valvular dysplasia with Noonan.

Hypertrophy of Rt ventricle with increased RV pressure

Arterial oxygen saturation remain normal even in severe stenosis unless Rt to Lt shunt through ASD/VSD/PDA exists.

Mild to moderate PS is usually asymptomatic.

Critical pulmonary stenosis: Severe PS in neonate leading to Rt to Lt shunting through PFO- cyanosis and CHF.

Treatment with balloon valvuloplasty

Bicuspid Aortic Valve/ASMostly an incidental finding

Calcification of bicuspid valve in later life

Leads to stenosis/insufficiency or aortic root dilatation.

Usually systolic murmur with opening snap

Treatment:

Stenosis: valvuloplasty, surgery

Insufficiency: surgery and vasodilators

Dilatation: ACE inhibitors

Associated with Turner’s, Williams, CoA

Coarctation Systemic bld flow obstruction, 98% time just below origin of Lt SCA and Ductus

M:F::2:1

Presents with shock like picture in first few days of life due to closing ductus

With severe CoA, RVH provide blood to descending aorta, femoral pulses are palpable, ductal Rt to Lt shunt present with differential cyanosis.

Murmur at infrascapular area

4 extremity BP

ECG: LVH

CXR: Rib notching

MRI: better visualization

Coarctation

Natural History: Persistent Hypertension and coronary artery disease if left untreated.

Associated with Turner’s, Bicuspid AV

May present with shock and diminished systemic perfusion, pulmonary congestion with decreased filling of failing left heart. Tx with ionotropes.

Treatment:

- Severe CoA, require PGE 1 in early infant life.

- Balloon angioplasty- dissection later

- Surgery is usual Tx

- Post-op rebound hypertension require medical management.

- Recoarctation may occur, require careful monitoring and revision.