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CONGENITAL HEART DISEASE

 

What is congenital heart disease?

 

Congenital heart disease comprises a spectrum of defects of the heart present at birth. These defects occur during development of the normal heart in the womb even before babies are born. The crucial period of heart development is in the first 4 weeks of the heart development in womb when it changes from a simple tube like structure to form a complex efficient structure with a pump like action, valves and tubes that carry blood to the entire body and lungs. Although present at birth, on some occasions, congenital heart disease is picked up in later life.

 

 

How common is it?

 

It is one of the commonest birth defect – approximately 1 in 100 babies born in UK have congenital heart disease. About 1 in 4 of those with congenital heart disease require surgery.

 

 

What are the types of congenital heart disease?

 

These can vary from simple defects such as holes in the heart (atrial septal defect or ventricular septal defect, patent ductus arteriosus), diseases of the valve (aortic valve stenosis, pulmonary valve stenosis, bicuspid aortic valve), narrowing of arteries (coarctation of aorta, pulmonary artery stenosis) or complex abnormalities of connection (Tetralogy of Fallot, transposition of great arteries, double outlet right ventricle, total anomalous pulmonary venous drainage) and severe anomalies (tricuspid atresia, hypoplastic left heart syndrome, single ventricle circulations, isomerism with unbalanced ventricles).

 

 

What causes congenital heart disease?

 

Some factors are known to increase the risk of congenital heart disease however no obvious cause can be identified in most cases. Maternal diabetes, alcohol intake, infections during the first trimester are all high risk factors for congenital heart disease. Presence of genetic defects in babies such as Down syndrome, Williams syndrome, Noonan’s syndrome, Di George syndrome all have increased risk of congenital heart disease. The risk increases in siblings or children of those with congenital heart disease also suggesting a genetic basis.

 

 

How is it detected?

 

Congential heart disease can be diagnosed in the womb by fetal echocardiography performed between 18 to 22 weeks. Congenital heart disease can be diagnosed quickly and comprehensively with a detailed consultation and clinical examination. Dr Khambadkone will perform an echocardiography and  electrocardiography personally and give a diagnosis and management plan at the end of the consultation. Rarely, additional tests such as X ray of the chest, Heart Rhythm monitoring, exercise testing, CT angiogram or Cardiac Magnetic Resonance Imaging may be necessary.

 

 

What are the symptoms?

 

Babies born with severe congenital heart disease can have symptoms soon after birth or could be completely well for a few days due to the presence of a connecting tube between the main body and lung arteries (patent arterial duct or patent ductus arteriosus) that can compensate for most severe congenital heart disease. The ductus arteriosus is programmed to shut down by nature within the first few hours or days after birth and this can make babies very sick in a very short time. Symptoms after birth may be poor feeding, sleepiness, cold hands and feet with low oxygen levels and blue colour (cyanosis) or pale appearance (shock). Babies could have heart failure manifest as poor feeding, rapid breathing with indrawing of ribs, sweaty head or body and poor weight gain.

 

 

What is the treatment of congenital heart disease?

 

Depending upon the heart defect, the treatment can either be with key hole procedures called cardiac catheterization - closing holes in the heart (atrial septal defect, patent arterial duct, ventricular septal defect), opening narrow valves (balloon aortic and pulmonary valvotomy), improving narrowing of arteries (balloon angioplasty and stent angioplasty of coarctation of aorta or pulmonary artery stenosis) and implanting heart valves without open heart surgery (percutaneous pulmonary valve implantation). Some heart conditions require open heart surgery that can only be done with heart lung bypass.

 

Dr Khambadkone works very closely with renowned congenital cardiac surgeons to offer excellent surgical outcomes across the wide spectrum of simple and complex congenital heart disease. The long term survival after surgery for congenital heart surgery is one of the great successes in modern medicine. Close to 90% of children operated would survive beyond 18 years. Long term follow up of these adults is important and is undertaken by specialists in Adult Congenital Heart Disease.

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