Congenital heart disease in Ceylon

An analysis of 555 consecutive cases of congenital heart disease in Ceylon has shown that for the whole series interatrial septal defect is the commonest cardiac lesion; next is interventricular septal defect, followed by persistent ductus arteriosus. Interventricular and interatrial septal defects are equally common among all the patients below 16 years of age, but interventricular septal defects are commonest among those below 11 years. Persistent truncus arteriosus is unusually common, while coarctation of the aorta and congenital aortic stenosis are rare, as compared with series reported from other places.     

Congenital heart disease

Dr. Frederick K. Heath: Congenital heart disease includes a wide variety of anatomic defects, about one-half of which produce significant physiologic abnormalities. This half, representing about 1 per cent of all organic heart disease and about 2 per cent of that seen in children, recently has become the center of great physiologic and surgical interest. For advances in vascular surgery have made it possible to achieve dramatic and often life-saving results in this group of patients. Coincidently, investigation by means of the intravenous catheter had progressed so far that precise diagnostic and postoperative follow-up information could be obtained. This was important since it soon became obvious that clinical appraisal based only on physical findings and statistics gave neither accurate diagnosis nor quantitative information regarding cardiac function. Important, too, is the use of radiographic technics whereby the abnormality may be visualized.The four cases presented serve to illustrate present day technics and their limitations, how data obtained by catheterization technics are interpreted, and representative results of surgical therapy. They also emphasize the frequent occurrence of multiple defects.By means of cardiac catheterization with simultaneous measurement of the blood oxygen content and pressures in the various chambers it is possible by application of the Fick principle to calculate cardiac output, to define the systemic and pulmonary circulation in terms of volume flow per unit of time, and to estimate, when present, the direction of shunt flow. In connection with pressure measurements it is to be remembered that an increase in pressure may result from obstruction to flow, e.g., stenosis of the pulmonary artery, in which case the rate of flow will be decreased; or on the other hand, it may simply be due to an increased rate of flow per se. It is clear that information both as to pressure and rate of flow are important for interpretation.Accurate anatomic and physiologic estimations are of invaluable aid in deciding whether or not operative interference should be helpful. Thus in simple patent ductus arteriosus an excellent result is to be expected. Of twenty-six uncomplicated cases of this condition operated upon by Dr. Humphreys only one death occurred postoperatively and only one proven recurrence was noted. When the patent ductus arteriosus is complicated by other defects, the outlook depends upon the nature of these complications. In this group the operative mortality and results are decidedly less favorable.When indicated, i.e., cyanotic heart disease, the creation of an artificial ductus, as carried out by Dr. Blalock, may relieve cyanosis but it also increases the pulmonary blood flow and the work of the heart. Whether eventual cardiac hypertrophy and failure, pulmonary congestion and polycythemia may be postoperative sequelae is not yet clear. These findings were observed in one patient thought to have a tetrad of Fallot plus a patent ductus arteriosus from birth; yet in patients receiving an artificial ductus the disappearance of polycythemia has been encountered.     

Congenital heart disease in pregnancy

Care of pregnant patients with congenital heart disease requires understanding of the specific congenital defect, the nature of previous surgical correction, and the residua and sequelae. General risks and principles can be adduced in management decisions. In addition, lesion- and patient-specific details are important. There are only a few conditions that place patients at a high enough risk to advise that pregnancy be avoided under all circumstances (pulmonary vascular obstructive disease, Marfan syndrome with dilated aortic root, severe aortic stenosis, and severe systemic ventricular dysfunction). Preconception counseling, optimization of status, and meticulous multidisciplinary management during pregnancy and the postpartum period will improve outcomes.     

Congenital heart disease in pregnancy

Heart disease is a main cause of maternal mortality in the United States and the United Kingdom. Most deaths are from acquired conditions. However, due to the increased survival of children born with congenital heart disease (CHD) over the past 30 years, the population of adults with congenital heart disease in the U.S. now exceeds 1 million. Thus, there are now more adults with CHD than children with CHD. Many of these adult survivors of pediatric heart disease are of childbearing age and are considering pregnancy. This article reviews the literature concerning pregnancy and CHD.     

Congenital heart disease and heart failure

The syndrome of heart failure in adult non-congenital heart disease patients includes myocardial disease and ventricular dysfunction. In the presence of congenital abnormalities the cause of heart failure is often multi-factorial and can be a result of the underlying anomaly, surgical intervention, or ventricular dysfunction. Despite the possible clinical similarities, the two conditions are fundamentally different. In congenital heart disease the neurohormonal system is already abnormal even in the absence of clinical manifestations of heart failure and, in many cases, exercise intolerance is related to cyanosis. The approach to heart failure management in the two etiologies might be similar. Preventative attempts to preserve ventricular function in coronary or valve disease parallels early reparative therapy in congenital heart disease Pharmacological therapy is common for the two conditions, despite the limited number of evidence-based recommendations for congenital diseases. In drug-resistant patients, cardiac electrical resynchronization is an established therapy for treating ventricular asynchrony in non-congenital heart failure sufferers, but has only recently been adopted in selected congenital cases. Due to this, congenital heart disease patients are managed in highly specialized unites in close cooperation with cardiologists and surgeons. The ideal follow-up protocol for such patients remains to be determined, particularly in those individuals with subclinical signs of residual cardiac dysfunction. Heart Fail Monit 2008;6(1):2-8.     

Congenital heart disease in the adult

On the basis of literary data and of their own series of 264 adult patients, who were in 1978-1982 subjected to invasive haemodynamic examination for congenital heart disease, 162 patients operated on, and 128 patients followed up for long-term periods after surgical treatment of congenital heart disease in adult age, the authors analyse the causes of late recognition of the disease, operative complications and long-term operative results. It is pointed out that a haemodynamically serious congenital heart disease is indicated for surgical correction even in adult age, and that age itself does not represent a limitation to cardiosurgical intervention. In adult age, it is necessary to anticipate a greater number of complications and a more severe post-operative course, but the long-term results are very good.     

Congenital heart disease and pregnancy

In Europe and North America, a dramatic fall in the incidence of rheumatic fever and rheumatic heart disease has coincided with advances in medical and surgical management of congenital heart disease and has resulted in a shift in the relative incidence of these two categories of cardiac disorders in women of child-bearing age. This review deals with pregnancy and congenital heart disease—unoperated and operated. Central to this topic is the intricate interplay between maternal circulatory and respiratory physiology and maternal congenital heart disease, and the effects of this interplay upon the fetus which is exposed to risks that threaten its intrauterine viability and to risks that subsequently express themselves as developmental defects or transmitted congenital malformations of the heart or circulation.     

Congenital heart disease in Down's syndrome.

Eighty cases of Down's syndrome were found among 1504 children with congenital heart disease under the age of 15 years. The most common cardiac anomaly, ventricular septal defect, was found in 49 per cent of the 80 cases studied, while the second most frequently encountered anomaly, common atrioventricular canal, was found in 15%. Haemodynamic investigations of 24 cases of ventricular septal defect showed Eisenmenger's syndrome to be present in 10 cases; this seemed to appear at an earlier age in mongoloid children than in other children with ventricular septal defect. Right-to-left shunt was found in 5 out of 9 cases of common atrioventricular canal. Pulmonary hypertension was found in all of 24 cases of ventricular septal defect and in 7 of 9 cases of common atrioventricular canal. The cumulative survival up to 10 years was 64% for girls and 49% for boys. Death was most commonly the result of pulmonary complications which occurred in 22 out of the 34 patients who died.     

Congenital heart disease in Down's syndrome.

Eighty cases of Down's syndrome were found among 1504 children with congenital heart disease under the age of 15 years. The most common cardiac anomaly, ventricular septal defect, was found in 49 per cent of the 80 cases studied, while the second most frequently encountered anomaly, common atrioventricular canal, was found in 15%. Haemodynamic investigations of 24 cases of ventricular septal defect showed Eisenmenger's syndrome to be present in 10 cases; this seemed to appear at an earlier age in mongoloid children than in other children with ventricular septal defect. Right-to-left shunt was found in 5 out of 9 cases of common atrioventricular canal. Pulmonary hypertension was found in all of 24 cases of ventricular septal defect and in 7 of 9 cases of common atrioventricular canal. The cumulative survival up to 10 years was 64% for girls and 49% for boys. Death was most commonly the result of pulmonary complications which occurred in 22 out of the 34 patients who died.