Adult Congenital Heart Disease

Dr Amit Misri
Congenital heart disease is a type of defect in  which one or more structures of the heart or blood vessels that occur before birth. The heart structures, or vessels, do not form as they should during pregnancy. The incidence is about 8-10 case per 1000 live births. Many of these lesions are life threatening and if not treated at the right time cause death within the first year of life. On the other hand there are other heart defects which are not so serious and remain undetected till adulthood.
To make the general population aware about the occurrence of Congenital Heart Disease (CHD), its symptoms, and treatment, a full week Feb 7 to Feb 14 is  celebrated as CHD awareness week.
This article predominantly is dedicated to adolescent and adult patients who are having some form of congenital heart disease.
What is the incidence of Adult CHD?
Adult congenital heart disease is not uncommon. 1 in 150 adults are expected to have some form of congenital heart disease in US. 800,000 adults in the United States have grown into adulthood with congenital heart disease. This number increases by about 20,000 each year. In India the prevalence of CHD in adults has been estimated to be 2.4 per 1000 population. This difference from the western world may be attributed to lack of awareness about the disease and availability of limited screening tests.
Which group reaches Adulthood?
The mortality rates of CHD if untreated is about 30-40% during first year of life. But there are many forms of CHD which either remain asymptomatic or cause limited symptoms till adulthood. These include small holes in heart or milder varieties of valvular or vascular obstructions. With increase in activity the demands of body increase and thereby symptoms tend to manifest. On the other hand there are people who are diagnosed cases of CHD but cannot afford treatment. They tend to modify their lifestyle according to the symptoms. Then there is other group which has received timely  treatment in form of surgery or cardiac intervention and thus has been able to reach adulthood.
What are the symptoms?
Unlike infants whose symptoms remain mainly cyanosis, inability to feed and fast breathing adults present with myriad of symptoms. These can be exertional breathlessness, exertional fatigue, chest pain, syncope, haemoptysis, palpitations and cyanosis. Obstruction to aorta (artery supplying the whole lower body) can present as hypertension or just exertional pain in calf muscles.
What are the risks patients with Adult CHD face?
The most life threatening risk is that of sudden death. This is predominantly because of arrhythmias (irregular heart beats) secondary to effect of CHD on heart muscle. Apart from that, infection of heart is not uncommon and could be difficult to treat. Patients with hypertension secondary to obstructed  vessels can lead to brain hemorrhage. Patients who have mixing of oxygenated and deoxygenated blood have chance of developing intracranial abscess because impure blood can reach brain which otherwise would have been filtered in lungs.
What are the management possibilities?
The foremost important step is to consult a doctor who deals with congenital heart disease and is fully aware of the hemodynamics in such situations .A correct diagnosis and the natural history of the specific CHD is important in formulating a management plan. While some CHDs like small holes or milder form of valvular obstruction may just need a regular follow up and certain precautions, other more significant lesions will require surgery or intervention in cath lab to correct the defect.
Issues related to adult CHD?
Adults having congenital heart disease face many issues. These are related to job hunting, pregnancy and contraception, insurance, requirement of non cardiac surgical procedures. There is a lot of apprehension in general population and physicians regarding management of such persons. As far as pregnancy is concerned most of the treated cases and females with non significant cardiac lesions can safely give birth. However it is imperative to have a multi disciplinary approach involving the obstetrician and cardiologist having knowledge of adult CHD. In certain types of adult CHD oral contraceptives may be contra indicated as they can lead to coagulation disorders. Getting medical insurance may be an uphill task and most do not cover cardiac interventions for CHD. Jobs involving aviation or deep sea diving are more or less prohibited. Anesthesia for non cardiac surgeries in such patients is also challenging.
Future road ahead?
With advancement in technology, diagnosis and treatment has become much easier. More and more patients are being diagnosed early and receive timely appropriate treatment. In near future we are going to see more treated cases of adult CHDs than untreated cases as is already happening in western countries. Multi interdisciplinary  approach involving cardiologist having knowledge of CHD, general physician, obstetrician, endocrinologist is going to be the way ahead. With small life style adjustments and regular follow up this group of population is going to live a near normal life and enjoy it to the fullest
(The author is Dr Amit Misri Sr. Consultant Division of Pediatric Cardiology (Medanta Heart Institute)Gurgaon Haryana.