Seminar congenital cardiac disorders (pda,TA and AP Window)

SEMINAR ON

PDA,TRUNCUS ARTERIOSIS,AP WINDOW

PRESENTED BY,UMADEVI.K

THE OXFORD COLLEGE OF NURSINGBENGALURU

PATENT DUCTUS ARTERIOSUS(PDA)

Patent dustus arteriosus is a congenitalo disorder in the heart wherein a neonates ductus arteriosus (blood vessel connecting pulmonary artery to proximal descending aorta) fails to close after birth.

PATENT DUCTUS ARTERIOSUS

PHYSIOLOGY

DUCTUS ARTERIOSUS is the blood vessel connecting pulmonary artery to proximal descending aorta in the fetal circulation

Normally,soon after the birth the ductus arterteriosus gets closed as a result of constriction of smooth muscles in its vessel wall by release of bradykinin.

When it remains open,it results in patent ductus arteriosus

As pulmonary resistance falls,the pulmonary artery pressure drops and blood with higher pressure from aorta is shunted to the pulmonary artery

CLINICAL MANIFESTATIONS

Tachycardia Dyspnea Heart murmer Cardiomegaly Bounding pulse Poor growth

DIAGNOSIS

Echocardiography Electrocardiography Chest x ray (cardiomegaly) Pda murmer is heard on physical

examination

TREATMENT

NSAIDS such as indomethacin or ibuprofen is given which helps to close PDA(Pgs are responsible for ductus patency,NSAIDS act as inhibitors for pgs)

Surgical correction by division and ligation of patent vessels and done at 1-2years of age.

AP WINDOW(AORTO PULMONARY WINDOW)

AP Window is the rare(0.1% of all congenital defects) congenital defect in which there is a hole connecting the a major artery(aorta) and pulmonary artery.

ETIOLOGY:- Aortopulmonary window represents a failure

of the conotruncus to differentiate into the aorta and pulmonary artery.

No genetic associations or environmental risk factors are known.

The 2 competing embryologic theories are › aortopulmonary window is part of a spectrum of

conotruncal abnormalities, which includes truncus arteriosus at one end of the spectrum, and

aortopulmonary window is unrelated to truncus arteriosus because the lesions associated with each defect are so dissimilar

CLINICAL MANIFESTATION:-

Minimal cyanosis present Symptoms of heart failure appear

during early infancy The defect is usually large, and the

cardiac murmur is systolic with a mid-diastolic rumble as a result of increased blood flow across the mitral valve.

PHYSIOLOGY

Normally blood flows through PA into lungs where it picks up oxygen.then blood travels back to heart by PV

With AP window blood from aorta flows into pulmonary artery

Large amount of blood flow to pulmonary artery results in pulmonary hypertension and heart failure

DIAGNOSTIC STUDIES:- Electrocardiogram- shows either left ventricular or

biventricular hypertrophy. Radiographic- shows cardiac enlargement and

prominence of the pulmonary artery and intrapulmonary vasculature.

Echocardiography- shows enlarged left-sided heart chambers.

Magnetic resonance angiography (MRA)- can also be utilized to visualize the defect.

Cardiac catheterization- reveals a left-right shunt at the level of the pulmonary artery, as well as hyperkinetic pulmonary hypertension, because the defect is almost always large.

Selective aortography- injection of contrast medium into ascending aorta demonstrates the lesion, and manipulation of the catheter from the main pulmonary artery directly to the ascending aorta is also diagnostic 

Medical Therapy:

Medical therapy is focused on preoperative stabilization.

Surgical correction is the only effective treatment for aortopulmonary window (APW).

Intravenous prostaglandins (e.g., alprostadil) may be required to maintain patency of the ductus arteriosus in patients with interrupted aortic arch in order to provide blood flow to the lower half of the body.

Digoxin and furosemide are frequently administered to treat the heart failure and volume overload associated with this lesion.

Inotropic agents (e.g., dopamine, dobutamine) may also be required for infants with significant heart failure and low cardiac output associated with myocardial dysfunction.

SURGERY

TRUNCUS ARTERIOSUS

Truncus arteriosus is the rare congenital heart disease in which the embryonical structure known as truncus arteriosus fails to properly divide pulmonary trunk and aorta.

TRUNSUS ARTERIOSUS

PHYSIOLOGY

With truncus arteriosus large blood vessel leads out of heart

Mixing of blood

Circulatory problems

TYPES Type I : one pulmonary artery and two

lateral pulmonary arteries

TYPE II :Two posterior or posteriolateral arteries

TYPE III: To lateral pulmonary arteries

CLINICAL MANIFESTATION:-

Vary with age and depend on the level of pulmonary vascular resistance.

In immediate new born period, › signs of heart failure usually absent› murmur and minimal cyanosis

In older infants,

› Pulmonary blood flow is torrential› Clinical picture dominated by heart failure› Cyanosis is minimal› Wide pulse pressure and bounding pulses- runoff

blood from the truncus to the pulmonary circulation› Enlarged heart and the precordium is hyperdynamic› S2 is loud and single

› A systolic ejection murmur, accompanied by a thrill, generally audible along the left sterna border

DIAGNOSTIC STUDIES:-

Electrocardiogram- shows right, left or combined ventricular hypertrophy, cardiac enlargement, prominent shadow that follows the normal course of the ascending aorta and aortic knob; the aortic arch is to the right in 50% of patients.

Echocardiography- shows the large truncal artery overriding the VSD and the pattern of origin of the branch pulmonary arteries.

Pulsed and colour Doppler- used to evaluate truncal valve regurgitation.

Cardiac catheterization- shows left to right shunt at the ventricular level, with right-left shunting into the truncus. Angiography reveals the large truncus arteriosus and more precisely defines the origin of the pulmonary arteries.

MEDICAL CARE: Medical care before surgical repair depends

on clinical presentation.

Most neonates with truncus arteriosus display some evidence of congestive heart failure; they are usually treated with digitalis and diuretic medicines.

Intravenous prostaglandin is often administered in patients with truncus arteriosus upon presentation because the differential diagnosis includes numerous anomalies with duct-dependent systemic or pulmonary blood flow. However, it is beneficial only in patients with associated interruption of the aortic arch or aortic coarctation.

SURGERY

Surgical repair shud be done with in 2 months as it is fatal.

RASTELLI REPAIR

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