1 Grown –Up Congenital Heart Disease (GUCH) Mohammad Saifur Rohman, MD. PhD Interventional Cardioloy Consultant Lab. Cardiology and Vascular Medicine Faculty

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Grown –UpCongenital Heart Disease

(GUCH)

Mohammad Saifur Rohman, MD. PhDInterventional Cardioloy Consultant

Lab. Cardiology and Vascular MedicineFaculty of Medicine

University of Brawijaya

Definition

• Adults with congenital heart defects are a group of patients which is fast growing in numbers, due to advances in cardiac surgery and intensive care in the 1970s and 80s.

• Congenital heart disease (CHD) : Cardiac lesions present from birth

• CHD in adult: Small defect, no correction, partial correction, post correction

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Causes of congenital heart disease

• No one cause can explain all cases. Many factors :

• Genetic

• Environmental

Affect cardiac development in the uterus;

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• Maternal rubella- in addition to cataracts, deafness, and microcephaly, cause patent ductus arteriosus (PDA) and pulmonary stenosis

• Fetal alcohol syndrome- associated with cardiac defects (as well as microcephaly, micrognathia, microphthalmia, and growth retardation)

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• Maternal systemic lupus erythematosus – associated with fetal complete heart block (due to transplacental passage of anti-Ro antibodies)

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Genetic associations with congenital heart disease

• Trisomy 21- endocardial cushion defects, atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy of Fallot.

• Turner’s syndrome (X0)- coarctation of the aorta

• Marfan syndrome- aortic dilatation and aortic and mitral regurgitation

• Kartagener’s syndrome- Brochiectasis,chronic sinusitis, situs inversus

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Cardiac malformations

• Ventricular septal defect (VSD)• Atrial septal defect (ASD)• Patent ductus arteriosus (PDA)• Pulmonary stenosis – causes cyanosis if severe • Coarctation of the aorta• Aortic stenosis• Tetralogy of Fallot – causes cyanosis• Transposition of the great arteries – causes cyanosis• Other causes of cyanotic congenital heart disease-

pulmonary atresia, hypoplastic left heart, severe Ebstein’s anomaly with ASD

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Classification of cyanotic congenital heart disease Classification of cyanoctic congenital heart disease by the amount of pulmonary blood flow seen on chest x ray. Increased Pulmonary Blood Flow Normal or Decreased Pulmonary Blood Flow

Tricuspid atresia with large VSD Tricuspid atresia with restrictive VSD

Total anomalous pulmonary venous return Pulmonary atresia with intact ventricular septum

Truncus arteriosus Ebstein’s anomaly

D-transposition of the great arteries D-transposition of the great arteries with pulmonary stenosis

Taussig-Bing anomaly Double outlet right ventricle with pulmonary stenosis

Tetralogy of Fallot with minimal right ventricular outflow tract obstruction

Tetralogy of Fallot

Tetralogy of Fallot with pulmonary atresia and increased collateral flow

Tetralogy of Fallot wit pulmonary atresia

Single ventricle without pulmonary stenosis Single ventricle with pulmonary stenosis

Interrupted aortic arch with PDA Vena cava to left atrium communication

Hypoplastic left heart syndrome ASD with Eisenmenger’s syndrome

VSD with Eisenmenger’s syndrome

PDA with Eisenmenger’s syndrome

2 groups:Left to right shunting (Septa l defects) - PDA (Patent ductus Arteriosus)– VSD (Ventricular septal defect)– ASD (Atrial Septat defect)Obstruction lesion in left or right heart

without shuntAS (Aortic stenosis) CoA (Coarctation of Aorta)PS (Pulmonary stenosis)

Non Cyanotic Heart Disease

Acyanotic heart disease

Blood flow to lung

Normal Increased

RVH-PS-MS

LVH-MI-AS-KoA

RVH-ASD-PAPVR-PVOD

LVH/BVH-VSD-PDA-AVSD

Chest X ray

EKG

Echocardiography, cardiac catheterization

Non cyanotic heart disease with left to right shunting

Shunting via defectShunting via defect

Overflow to pulmonaryOverflow to pulmonaryOverflow to pulmonaryOverflow to pulmonary

size - locationof defect

size - locationof defect

Pulmonary vascular resistance

Pulmonary vascular resistance

•Dyspnea•Recurent respiratory infection• Fail to thrive• Heart failure

• asymptomaticasymptomatic• symptomaticsymptomatic

Depend on Pulmonary vascular resistance

Pulmonary blood Pulmonary blood flow overloadflow overload

Non Cyanotic Heart Disease Non Cyanotic Heart Disease SymptomsSymptoms

asymptomatic

Symptomatic

Clinical features

• Neonates: – Small VSD: asymptomatic– Large VSD left ventricular failure :

• Failure to thrive, feeding difficulty, sweating on feeding

• Tachypnea and intercostal recession• Hepatomegaly

• Adult:– Asymptomatic– Dyspnea due to PH or Eisenmenger syndrome

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•Volume overload of LV•Big left to right shunt•Dilatation of LA, LV dan PA•Congestive heart failure

A. Small VSD A. Small VSD • Holosistolic murmur

Left to right shunt

B. Moderate VSDB. Moderate VSD• Holosistolic murmur• Early diastolic murmur

Flow through mitral valve

C. Big VSD : C. Big VSD : PVR < SVRPVR < SVR• Loud S2 (P2) • Ejection systolic murmur

Flow through RV outflow tract

D. VSD besar : D. VSD besar : PVR PVR >> SVRSVR

• Load S2 (P2)• No Shunting (-)

Cardiomegaly LA and LV dilatation RV dilatation (PH) Increased pulmonary

vascularization (pletora)

Pulmonary hypertension in adult

Echocardiography

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Echocardiography

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Management

• 30% of cases close spontaneously, mostly by the time the child is 3 years of age.

• Some do not close until the child is 10 years old.

• Defects near the valve ring or near the outlet of the ventricle do not usually close

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Management

• Operative closure is the treatment of choice and is recommended for all lesions that have not undergone spontaneous closure

• VSD risk factor for infective endocarditis appropriate prophyalctic measures should be taken

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PDA

• In the fetus most of the output of the right ventricle bypasses the lungs via the ductus arteriosus

• This vessel joins the pulmonary trunk (artery) to the descending aorta distal to the left subclavian artery.

• The ductus arteriosus normally closes about 1 month after birth in full-term infants and takes longer to close in premature infants

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•Overload of LA and LV•Big left to right shunting•Dilatation LA, LV, AO and PA•Congestive heart failure

Pulmonary hypertension in adult

Clinical features

• The factors that determine the nature of clinical features are the same as in VSD and ASD i.e the size of the defect, the presence of PH, the development of Eisenmenger’s syndrome

• A patent PDA is more likely in babies born at high altitude, probably due to low atmospheric oxygen concentration; it may also occur In babies who have fetal rubella syndrome

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History

• Small PDA: asymptomatic

• Large PDA: large left-to-right shunt left ventricular failure with pulmonary edema causing failure to thrive and tachypnea

• Adults with undiagnosed PDA may develop PH and present with dyspnea

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Small PDA Continuous murmur – machinery murmur

Left to right shunting during systolic and diastolic

Big PDA – PH pulsus celler S2 (P2) Loud Continuous murmur

Left to right shunt fase sistolik dan diastolik

Systolic murmur Left to right shunt fase sistolik

Murmur (-) Early Diastolic murmur

Mitral flow

Management

• Pharmacological closure in neonates – indomethacin may induce closure if given early

• Operative closure – this can be performed as an open procedure in which the PDA is ligated or divided

• Or using ADO (Amplatzer ductal occluder) by cardiac catheter

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• LIGTION PDA• ADO

(BW> 8 kg)

• LIGTION PDA• ADO

(BW> 8 kg)

SYMPTOMATICSYMPTOMATICHeart failure

• digitalis• diuretika• vasodilator

PH – PVR ?

Management

• PDA is a risk fractor for infective endocarditis antibiotic prophylaxis is required for all patients before operative procedures.

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Shunting depend upon• Defect size• Compliant of RV < LV

Left to right shunt

3 types of Atrial septal defect

• (1) Septum primum (ostium primum ASD)- this defect lies adjacent to atrioventricular valves, which are often also abnormal and incompetent

• (2) Septum secundum (ostium secundum ASD)- the most common form of ASD, it is midseptal in location

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3 types of Atrial septal defect

• (3) Sinus venosus ASD – this lies high in the septum and may be associated with anomalous pulmonary venous drainage (in which one of the pulmonary veins drains into the right atrium instead of the left.

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History

• Early life: asymptomatic

• Adult life: dyspnea, fatigue, recurrent chest infections

• As time goes by, the increased pulmonary blood flow results in pulmonary hypertension and eventually reversal of the shunt and Eisenmenger syndrome

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•Volume overload in RV•Dilatation of RA, RV and PA•Pulmonary Hipertension

sindroma Eisenmenger

S2 S2 wide fixed splittingwide fixed splitting• pengosongan RV lama –

katup pulmonal terlambat menutup

• tidak berubah dengan respirasi

Sistolic ejection murmur Sistolic ejection murmur •Flow through RVOT and

PADiastolic murmurDiastolic murmur

•Flow through trikuspid valve

Examination

• The second heart sound is widely split because closure of the pulmonary valve is delayed due to increased pulmonary blood flow.

• The splitting is fixed in relation to respiration because the communication between the atria prevents the normal pressure differential between right and left sides that occurs during respiration.

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Examination

• The increased pulmonary blood flow causes a mid systolic pulmonary flow murmur.

• If PH has developed reduction of the left-to-right shunt, the pulmonary flow murmur disappears; there is a loud pulmonary component to the second heart sound

• If Eisenmenger’s syndrome occurs centrally cyanosed, finger clubbing

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Investigations

• EKG– Ostium secundum ASD: right axis deviation– Ostium primum defect: left axis deviation

• Chest radiography– Pulmonary arteries: dilated, its branches are

prominent– Enlarged right atrium, enlarged right ventricle

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RA dilatation RV dilatation

prominent pulmonary segment

Increased pulmonary vaskularisation (pletora)

PH Wide of pulmonal and

hilus Ischemic of peripheral

pulmonary vascular (pruning)

Echocardiography

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Echocardiography

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Cardiac catheterization

• To reveal ASD, because the catheter can be passed across it.

• Serial oxygen saturation measurements are made at different levels from the superior vena through the atrium and the right ventricle into the pulmonary artery.

• At the level of the left-to-right shunt there will be a step up increase of the oxygen saturation as blod flow from the left side enters the right.

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ASD CLOSUREFR (Qp/Qs) > 1,5• SURGERY•ASO

(BW > 8 kg)

ASD CLOSUREFR (Qp/Qs) > 1,5• SURGERY•ASO

(BW > 8 kg)

ASYMPTOMATICASYMPTOMATIC usia pra sekolah

(3 – 4 tahun)

SYMPTOMATICSYMPTOMATICHEART FAILURE

• digitalis• diuretika• vasodilator

PH : PVR ?

Complications of congenital heart disease

• Cyanosis – the presence of more than 5g/dL of reduced hemoglobin in arterial blood

• Congestive heart failure – this occurs due to the inability of the heart to maintain sufficient tissue perfusion as a result of the cardiac lesion

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• Pulmonary hypertension-this occurs as a result of an abnormal increase in pulmonary blood flow due to left-to-right shunt (e.g ASD, VSD, PDA)

• Infective endocarditis – congenital heart disease may result in lesions prone to bacterial colonization

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Pulmonary Hypertension

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Infective Endocarditis

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• Heart failure

• Sudden death – this may be due to arrhythmias (more common in these disorders) or outflow obstruction as seen in aortic stenosis

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Eisenmenger’s syndrome

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• Refers to the situation in which a congenital cardiac abnormality initially causes acyanotic heart disease, but cyanotic heart disease develops as a consequence of raised pulmonary pressure and shunt reversal

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• These clinical features are also seen in patients who have cyanotic congenital heart disease

• Cyanosis develops when the level of reduced hemoglobin is over 5 g/dL.

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Complications of Eisenmenger’s syndrome

• Clubbing fingers and toes• Polycythemia and hyperviscosity- with

resulting complications of stroke and venous thrombosis. Regular phlebotomy is the treatment of choice

• Cerebral abscesses-especially in children• Paradoxical emboli- emboli from venous

thrombosis may pass across the shunt and give rise to systemic infarcts

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Clubbing fingers and toes

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Documents

1 Grown –Up Congenital Heart Disease (GUCH) Mohammad Saifur Rohman, MD. PhD Interventional Cardioloy Consultant Lab. Cardiology and Vascular Medicine Faculty