clinical approach to congenital heart disease

Clinical Approach to Congenital Heart Disease

Ahmad Hariz 071303074Group D2

Epidemiology

• 8/1000 live born children have significant cardiac malformations

• 1 in 10 stillborn infants have a cardiac anomalies

Acyanotic Heart Disease

• Defined as:– A congenital disorder manifested with left to right

shunting and obstructive lesions. – Clinical signs are not always apparent at birth,

they manifest anytime during infancy or early childhood.

• Incidence & classification– Left to right shunt• Ventricular septal defect – 30%• Patent Ductus Arteriosus – 12%• Atrial Septal Defect – 7%

– Outflow obstruction• Pulmonary stenosis – 7%• Aortic stenosis – 5%• Coarctation of aorta – 5%

Ventricular septal defect

Incidence and Pathophysiology:

●VSDs account for approximately 25% of all CHDs. ●VSD is the most common congenital cardiac lesion

and is often accompanied by other cardiac defects. ●The lesion consists of an abnormal opening between

the right and left ventricles which may vary in size from a miniscule hole to complete absence of the septum, resulting in a common ventricle.

Ventricular septal defect

Ventricular septal defect

Manifestations:●Signs and symptoms vary with the size of the defect

and the presence of associated cardiac lesions. Clinical symptoms are usually not seen at birth because of continued high pulmonary vascular resistance in the newborn. Infants with moderate to large defects will become symptomatic within the first few weeks of life.

●Children with small defects will remain asymptomatic.

Ventricular septal defect

• Clinical manifestations– Tachypnea, dyspnea– Poor growth– Palpable thrills– Systolic murmur at left lower sternal border– Shortness of breath – Failure to gain weight – Fast heart rate – Pounding heart – Frequent respiratory infections

Ventricular septal defect

• Complications– Congestive heart failure. – Growth failure, especially in infancy. – Bacterial endocarditis– Irregular heartbeat or rhythm– Pulmonary artery hypertension

Patent ductus arteriosus

• Pathophysiology– It is normally closed shortly after birth in term

infant– In PDA, it failed to close by a month post term,

due to defect in the constriction mechanism– The flow of blood is from aorta to pulmonary

artery

Patent ductus arteriosus

Patent ductus arteriosus

• Clinical features– Symptoms depends on amount of extra blood flow

to lungs– Usually asymptomatic in small PDA– CHF symptoms in moderate to large shunt– On physical examination:• Widened pulse pressure• Collapsing/ bounding pulse

Patent ductus arteriosus

• Differential cyanosis (cyanosis of lower limb but upper limb pink)– Upper limb supplied by brachiocephalic trunk and left

subclavian artery (before PDA junction)

• Left infraclvicular/upper left sternal edge continous murmur

Atrial septal defect

Incidence and Pathophysiology:● ASD accounts for approximately 10% of all CHDs. It is seen more

frequently in females than males. ● The lesion consists of an abnormal opening between the atria

Types of Lesions:1. Ostium Secundum – located at the middle of the atrial septum

(fossa ovalis), the most common type.2. Ostium Primum – located low in the atrial septum, results from a

defect in endocardial tissue formation and is often associated with a left mitral valve malformation.

3. Sinus Venosus – which is located high in the septum close to the SVC

Atrial septal defect

Atrial septal defect

Manifestations: Most infants and children are asymptomatic but over years

to decades may experience:

1. Fatigue and SOB2. Palpitations or atrial dysrhythmias – result of atrial

enlargement3. Recurrent respiratory infections can occur when there is a

large amount of pulmonary blood flow4. Systolic murmur is produced by increased blood flow across

the pulmonary valve.

Atrial septal defect

5. Diastolic murmur is present with large shunts6. Stroke or major organ damage can occur

because of embolization of thrombus, air or other materials – PARADOXIMAL EMBOLISM

7. Tachypnea, tachycardia and enlarged liver from heart failure

Aortic stenosis

• Aortic valve leaflets are partly fused together, giving restrictive exit from left ventricle

• Often associated with mitral stenosis and coarctation of aorta

Aortic stenosis

Aortic stenosis

• Clinical features– Mild to moderate cause no symptoms– Severe : causes easy fatigue, exertional chest pain

and syncope– Systolic ejection murmur, maximum at upper right

sternal edge, radiating to neck

Aortic stenosis

– Small volume, slow rising pulse– Carotid thrills– Apical ejection click (if valvular stenosis)

Pulmonary stenosis

• Pulmonary valve leaflets partly fused together, giving restrictive exit from right ventricle

Pulmonary stenosis

Pulmonary stenosis

• Clinical features– Most are asymptomatic– Moderate to severe • Exertional dyspnea and easily fatigability

– Severe stenosis• Cyanosis

Pulmonary stenosis

– Ejection systolic murmur in upper left sternal edge, thrill may present

– Soft or absent P2– Valvular stenosis will result in a click

Coarctation of aorta

• During the development of aortic arch, area near the insertion of ductus arteriosus failed to develop correctly

• Results in narrowing of aortic lumen• Always juxtaductal in position

Coarctation of aorta

• Clinical features– Asymptomatic– Always systemic hypertension in right arm– Ejection systolic murmur at upper sternal edge– Collaterals at the back– Radio femoral delay• Due to blood bypassing the obstruction via collateral

vessels