Congenital heart disease: the original heart failure syndrome.

Heart failure is characterised by a triad comprising cardiac abnormality, exercise limitation and neurohormonal activation. The 2% of the adult population who suffer with heart failure are known to derive both symptomatic and prognostic benefit from exercise and pharmacologic neurohormonal antagonism. The existence of heart failure has traditionally been considered in the context of ischaemic, hypertensive, valvular and myopathic disease but in this article we develop the argument that patients with congenital heart disease also manifest all the pathophysiological criteria that constitute the chronic heart failure syndrome. We discuss the inherent limitations to a purely anatomical approach to congenital heart disease, bring attention to the large and rapidly growing population of adults with this condition and conclude by calling for a therapeutic approach to congenital heart disease that is based on the heart failure paradigm.     

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 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 and pulmonary hypertension

Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardiac catheterization is crucial to confirm diagnosis and facilitate treatment. Bosentan is the only medication to date to be compared with placebo in a randomized controlled trial specifically targeting congenital heart disease-associated PAH. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation is reserved for recalcitrant cases.     

Congenital heart disease and the liver

There are approximately 1 million adult patients with congenital heart disease (CHD) in the United States, and the number is increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion or decreased cardiac output resulting from the underlying cardiac disease or as a result of palliative cardiac surgery; transfusion or drug-related hepatitis may also occur. The unique physiology of Fontan circulation is particularly prone to the development of hepatic complications and is, in part, related to the duration of the Fontan procedure. Liver biochemical test abnormalities may be related to cardiac failure, resulting from intrinsic liver disease, secondary to palliative interventions, or drug related. Complications of portal hypertension and, rarely, hepatocellular carcinoma (HCC) may also occur. Abnormalities such as hypervascular nodules are often observed; in the presence of cirrhosis, surveillance for HCC is necessary. Judicious perioperative support is required when cardiac surgery is performed in patients with advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patients with CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combined heart-liver transplantation may be required in those with decompensated liver disease or HCC, but experience is limited in the presence of significant CHD. The long-term sequelae of many reparative cardiac surgical procedures are not yet fully realized; understanding the unique and diverse hepatic associations and the role for early cardiac transplantation in this population is critical. Because this population continues to grow and age, consideration should be given to developing consensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population. (HEPATOLOGY 2012;56:1160-1169).     

冠心病和心力衰竭

冠心病心力衰竭（简称冠心病心衰）顾名思义是指由于冠心病引起的心力衰竭,据统计大约65％的心力衰竭由冠状动脉疾病引发的。冠心病心衰在临床上分急性和慢性两种,急性心衰主要由急性心肌梗死和急性冠脉缺血诱发的心肌收缩或舒张功能异常所致,慢性心衰主要是心肌梗死后心肌重塑和心肌的血供长期不足,心肌组织发生营养障碍和萎缩,以致纤维组织增生所致。由于冠心病导致心衰的成因不同,因此治疗上的侧重点就会有所不同,下面就对冠心病心衰发病机制及诊治作一浅谈。     

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 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 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 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,heterotaxia and laterality

Congenital heart disease occurs in about 0,8% of all newborns. Many cardiac malformations occur among relatives and have a polymorphic presentation. The origin of most congenital heart disease is thought to be multifactorial, implying both anomalous expression of genes and the influence of epigenetic factors. However, in a small number of cases, the origin of congenital heart disease has been directly related to chromosomal anomalies or to defects in a single gene. Curiously, defects in a single gene can explain a polymorphic presentation if the anomalous gene controls a basic embryonic process that affects different organs in time and space. Some of these genes appear to control the establishment of laterality.The establishment of the left-right asymmetry starts at the Hensen node. Here, the initial embryonic symmetry is broken by cascades of gene activation that confer specific properties on the left and right sides of the embryo. Although there are variations between species, some basic patterns of gene expression (Nodal, Pitx2) appear to be maintained along the phylogenetic scale. Anomalous expression of these genes induces the heterotaxia syndrome, which usually courses with congenital heart disease. The development of heart malformations is illustrated with the mouse mutant iv/iv, which is a model for the heterotaxia syndrome and the associated congenital heart disease.     

Congenital heart disease: Cor biloculare

The interesting features of this case, aside from its great rarity, are the presence of the four auriculoventricular valves which represent the primitive endocardial cushions, the hyperplastic eustachian valve, the common pulmonary vein and the unusual venous circulation of the lung. An examination of the roentgenogram of the chest revealed only a single large artery going upward and descending to the right.