The Desk of Chairperson Dr P S Sreemathi Panel of Pediatric Cardiologists and Cardiac Surgeons Peadiatric Segment E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 Defects that form abnormal pathways for the flow of blood in the heart are a few of the problems that occur when children are born with heart diseases.

 Parents with children afflicted with such heart diseases have so many doubts about their children lives and future.

Advances in treatment have been so profound, resulting in various methods of treatment and management that it becomes difficult to choose the right method. Panel Cardiologist and Cardiac surgeon present the existing knowledge of surgical and interventional methods of treatment.

 Dr Muthukumar Paediatric Cardiologist and Dr Neville Solomon Paediatric Cardiac Surgeon both authorities in their respective fields answer the questions based on the methods of management they can offer i.e interventional cardiological methods and Cardiac surgical methods.

Following are few of the questions that might arise in the minds of those interested. If you have further doubts you may ask either of them!

 SEPTAL DEFECTS

 What are Septal Defects?

 The heart consists of 4 chambers- right atrium, right ventricle, left atrium and left ventricle. The 2 atria are separated by the interatrial septum and the 2 ventricles are separated by the interventricular septum. Any defect in these structures that allows blood to pass is called septal defect. There are various kinds of atrial septal defect and ventricular septal defects. Their nomenclature depends on their location in the septa and their clinical significance depends on their size and effect on neighboring structures.

 What are the effects on the heart?

 The result of a septal defect is there is an increased blood flow at very either very high flow or pressure to the lungs. The right heart structures are accordingly affected either by dilatation or thickening of the right ventricle or both. There is increased pulmonary blood flow with damage to pulmonary vasculature which is initially reversible but subsequently is not. The increased blood flow into the lungs translates as increased return of blood into the left heart with dilatation of left atrium and left ventricle.

What are the consequent symptoms

 Symptoms depend on size and location of the defect. Infants with large VSD present early in 2nd-4th month with severe heart failure. Many develop pneumonia and can die from this. They do not gain weight as heart failure prevents them from feeding well. Children with ASD are not as acutely ill and present later.

 Babies with large VSD have to be operated as early as 3-4 months if echocardiography reveals no sign of spontaneous closure of the VSD and the child is symptomatic and failing to gain weight. Some types of VSD, particularly doubly committed subarterial can cause the neighbouring aortic valve to leak. This is an additional indication to close a VSD even if it is small. An ASD is closed by 3-5 years of age.

 What is the treatment?

 Diagnosis is confirmed by echocardiography. Cardiac catheterization is indicated when the child presents late and there are concerns the pulmonary artery pressures are too high to operate.

 The defect in infants is closed by open heart surgery. Infants may require longer in-hospital stay to build up their nutritional status. However, once corrective surgery is done, the infants show a dramatic improvement in heart failure, feeding and weight gain. The infant can be discharged 6-7 days postoperatively.

 The defect is closed with artificial material like Gore-tex (Poly tetra fluro ethylene PTFE) or Dacron or treated pericardium. Most children will have a lifelong cure and excellent quality of life. Complications include residual VSD and complete heart block (1-2 %) requiring permanent pacemaker.

 ATRIAL SEPTAL DEFECTS

 What is an Atrial Septal Defect (ASD)?

 Atrial septal defect is a common form of congenital heart disease wherein there is a communication between the right and left atrial chambers of the heart allowing blood to flow left to right.

 What are its effects on the heart?

 The basic consequence of this heart defect is there is an increased blood flow to the lungs with as blood preferentially flows from the left atrium to the right atrium and right ventricle and into the lungs. The right atrium and right ventricle dilate and as the pulmonary blood pressure increases, the right ventricle hypertrophies.

 What are the consequent symptoms?

 Many do not have symptoms and present as adults. The commonest symptoms are easy fatigability, recurrent respiratory tract infections, failure to thrive and as adults with atrial fibrillation/ flutter.

 If the ASD does not undergo timely intervention, some develop Eisenmenger’s syndrome with severe pulmonary hypertension.

 Some types of ASD, particularly ostium primum can have coexisting problems including mitral regurgitation (leaky mitral valve).

 How can the suspicion be confirmed?

 Diagnosis is confirmed by echocardiography.

 What is the treatment?

 The defect (ostium Secundum) may be closed by surgery or device. All other types require open heart surgery.

 Surgery may be through median sternotomy (anterior) or right thoracotomy (side- cosmetic)

 Surgery is low-risk and requires in-hospital stay of 5 days. Complications with ostium secundum ASD are uncommon. Residual mitral regurgitation and complete heart block can occur with osium primum ASD. SVC and/ or pulmonary venous obstruction can occur with sinus venosus ASD.

 

 

 

What are the survival results?

 

 

 

These patients do very well after surgery. They are very much like normal subjects. They can study well; take part in sports and competitions. They grow to adulthood and can pursue their desired careers.

 

 

 

VENTRICULAR SEPTAL DEFECT

 

 

 

What is a Ventricular Septal Defect (VSD)?

 

 

 

Ventricular septal defect is the commonst form of congenital heart disease wherein there is a defect in the septum between the right and left ventricular chambers of the heart allowing blood to flow left to right at high pressure.

 

 

 

What are the effects on the heart?

 

 

 

The basic consequence of this heart defect is there is an increased blood flow at very high pressure to the lungs. This lesion has much worse consequences compared to ASD. The right ventricle undergoes pressure hypertrophy and left atrium and left ventricle dilate due to increased pulmonary blood returning to the left heart.

 

 

 

What are the consequent symptoms?

 

 

 

Symptoms depend on size and location of the defect. Infants with large VSD present early in 2nd-4th month with severe heart failure. Many develop pneumonia and can die from this. They do not gain weight as heart failure prevents them from feeding well.

 

 

 

When does the doctor suspect that the child has VSD?

 

 

 

He hears sounds (murmurs) other than normal sounds

 

 

 

How is the suspicion of the doctor confirmed?

 

 

 

Echocardiography

 

 

 

How do symptoms affect treatment?

 

 

 

Unlike a patient with ASD, pulmonary hypertension develops very early in babies with large VSD. Hence, they should be operated on as early as 3-4 months if echocardiography reveals no sign of spontaneous closure of the VSD and the child is symptomatic and failing to gain weight.

 

 

 

Some types of VSD, particularly doubly committed subarterial can cause the neighbouring aortic valve to leak. This is an additional indication to close a VSD even if it is small.

 

 

 

What is the Treatment?

 

 

 

Diagnosis is confirmed by echocardiography. Cardiac catheterization is indicated when the child presents late and there are concerns the pulmonary artery pressures are too high to operate.

 

 

 

The defect in infants is closed by open heart surgery. Infants may require longer in-hospital stay to build up their nutritional status. However, once corrective surgery is done, the infants show a dramatic improvement in heart failure, feeding and weight gain. The infant can be discharged 6-7 days postoperatively.

 

 

 

The defect is closed with artificial material like Gore-tex or Dacron or treated pericardium. Most children will have a lifelong cure and excellent quality of life. Complications include residual VSD and complete heart block (1-2 %) requiring permanent pacemaker.

 

 

 

What is their rate of survival and quality of life?

 

 

 

They live long with a good quality of life.

 

 

 

PATENT DUCTUS ARTERIOSUS

 

 

 

What is a Patent Ductus Arteriosus (PDA)?

 

 

 

Why do symptoms occur when a child has PDA?

 

 

 

The ductus arteriosus is a structure that is essential for the survival of the fetus. This should close when the baby is born, and when this persists, the condition is labelled as patent ductus arteriosus.

 

 

 

What are the predisposing factors?

 

 

 

Preterm babies and mothers who have rubella during pregnancy

 

 

 

What are the effects on the heart ?

 

 

 

The basic consequence of this heart defect is there is an increased blood flow at very high pressure to the lungs. This lesion has much worse consequences compared to ASD. The right ventricle undergoes pressure hypertrophy and left atrium and left ventricle dilate due to increased pulmonary blood returning to the left heart.

 

 

 

What are the symptoms?

 

 

 

Symptoms depend on size of the duct. Preterm infants with large PDA may be stuck on a ventilator and cannot be weaned off. These require urgent surgery,

 

 

 

Others present early in 2nd-4th month with severe heart failure. Many develop pneumonia and can die from this. They do not gain weight as heart failure prevents them from feeding well. The presentation is similar to VSD and very often both lesions can coexist.

 

 

 

Unlike a patient with ASD, pulmonary hypertension develops very early in babies with large PDA. Hence, they should be operated on as early as 3-4 months if echocardiography reveals no sign of spontaneous closure of the PDA and the child is symptomatic and failing to gain weight. They may be operated at the end of 1 month if symptoms of heart failure such as breathlessness continues in spite of medical management.

 

 

 

What investigations are done to prove the presence of PDA?

 

 

 

Diagnosis is confirmed by echocardiography. Cardiac catheterization is indicated when the child presents late and there are concerns the pulmonary artery pressures are too high to operate.

 

 

 

What are the methods of treatment?

 

 

 

The duct in infants is closed by closed heart surgery or by the interventional cardiologist. Infants may require longer in-hospital stay to build up their nutritional status. However, once corrective surgery is done, the infants show a dramatic improvement in heart failure, feeding and weight gain. The infant can be discharged 4-5 days postoperatively.

 

 

 

The defect is approached through left thoracotomy and the PDA is either divided or tied off. The surgery is straightforward provided there is no intraoperative bleeding. Most children will have a lifelong cure and excellent quality of life. Complications include residual VSD and recurrentlaryngeal nerve palsy manifesting as hoarseness, and chylothorax. They are treatable.

 

 

 

ACKNOWLEDGEMENT

 

 

 

Congenital Heart Disease and Repair

 

 

 

Second Edition

 

 

 

Allen D.Everett, MD

 

 

 

D.Scott lim, MD

 

 

 

Illustrated by Paul and Jasper Burns.