Longevity in the setting of tetralogy of Fallot: survival to the 84th year.

A patient with tetralogy of Fallot suffered minimal disability until the age of 45 years, when she developed bacterial endocarditis complicated by hemiplegia. She remained well, but became markedly polycythaemic and, at the age of 50 years, underwent surgical correction to reduce the risk of further thromboembolic incidents. She continued in remarkably good health until her death from lobar pneumonia in her 84th year.

Preductal aortic coarctation and patent ductus arteriosus in a Sumatran tiger (Panthera tigris sumatrae) cub.

Preductal aortic coarctation and patent ductus arteriosus are described in a neonatal Sumatran tiger, Panthera tigris sumatrae. Eight days postpartum, the cub appeared weak, and it was separated from the dam for hand rearing. On examination it was dehydrated and hypothermic.

Congenital atresia of the orifice of the left coronary artery.

A 3-month-old infant developed signs of cardiac failure, which was initially attributed to cardiomyopathy. At 8 months, further investigations showed evidence of myocardial ischaemia with reversal of the flow of blood in the left coronary artery, which received no demonstrable inflow from the aorta. An anomalous connection of this artery with the pulmonary trunk was diagnosed but, at surgery, it was found that the arterial orifice was completely atretic, although the main stem was of normal size.

Unusual origin and course of the left pulmonary artery.

An infant with a cardiac murmur was found to have a patent arterial duct and an anomalous left pulmonary artery. The duct was surgically ligated at the age of 8 months, and she remained free of cardiac or respiratory symptoms up to her death at 6 years from an unrelated intestinal condition. An anomaly was discovered at post mortem examination; the left pulmonary artery arose from the right pulmonary artery and passed behind the trachea to enter the left lung.

Rupture of pulmonary aneurysms in association with long-standing Waterston shunts.

Anastomosis of the ascending aorta to the right pulmonary artery, the so-called Waterston shunt, was undertaken as a palliative procedure for children with cyanotic congenital heart disease due to obstruction of the pulmonary outflow tract with reduced pulmonary blood flow. We present the clinicopathological correlations in two patients who underwent construction of Waterston shunts as neonates, and subsequently died of ruptured pulmonary aneurysms in adult life. Rupture should, therefore, be recognized as a late complication of this procedure, and be considered in the long-term follow-up of such patients, especially when the shunted lung is hypertensive.

Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases.

We present two cases in which there was isomeric arrangement of the left atrial appendages in association with multiple spleens and complete heart block. In both of these, the venous connections were normal. In one case the arrangement of the bronchial tree and the lungs was normal, the liver was left-sided, and the stomach was right-sided.

The anatomy of a collection.

An old collection of 81 congenitally malformed hearts was examined in detail using the system of sequential segmental analysis. The specimens were almost entirely from infants and young children who had died during the two decades between 1954 and 1973. There had been surgical intervention in 26 cases, and most of these patients died during or shortly after the operation.

A postmortem review of congenital cardiac malformations in a series of 180 adults, over the age of 16 years, born between 1865 and 1980.

The evolution of diagnosis and treatment of congenital heart malformations can be traced through patients surviving into adulthood. We reviewed the heart specimens from 180 patients aged 16 to 86 years and considered the morphological features, the nature of any interventional procedures, and the events leading to death. Based on the mode of clinical presentation, 33 cases were considered covert, and the remaining 147 cases were known or suspected to have a cardiac abnormality during life.

Morphology of the elastic pulmonary arteries in pulmonary hypertension: a quantitative study.

Recent studies of intravascular ultrasound of the pulmonary arteries suggest that the technique can detect intimal and medial thickening in patients with pulmonary hypertension, potentially providing a method of assessing severity of pulmonary vascular disease in life. A major drawback of the technique is that only the elastic pulmonary arteries are accessible to current ultrasound catheters. The aim of this study was to determine whether morphological changes in vessels accessible to intravascular imaging reflect severity of pulmonary vascular disease and are of a sufficient degree to be detectable by current ultrasound catheters.

A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification.

This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years.

Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death.

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistuious communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present.

Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum.

An apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum.

The Brock procedure (closed infundibular resection) for Fallot's tetralogy: 43 years later.

Innovative cardiac surgery ('blind' or indirect infundibular resection) for tetralogy of Fallot on a child of 4 years was followed by survival for 43 years without further surgery. The patient remained well until about one year before death, when he developed clinical features of progressive biventricular failure associated with pulmonary hypertension and incompetence of the pulmonary and tricuspid valves. Postmortem examination showed severe damage to one of the leaflets of the pulmonary valve, interpreted as due to inadvertent avulsion during the original surgical procedure.

Anatomical conundrum: unusual position of the aortic arch.

At autopsy on a 72-year-old woman, the aortic arch showed an unusual arrangement, passing behind the trachea and esophagus and descending on the left side. The heart was normal, and death was due to unrelated causes. The nature of this anomaly is considered in respect of embryological development.

Anatomic conundrum in a case of complete transposition of the aorta and pulmonary trunk.

An unusual cord-like structure was found on postmortem examination of the heart of a 30-year-old woman who had complete transposition of the great arteries. This extended from the posterior aspect of the right side of the base of the aorta to the posterior wall of the distal part of the arch immediately proximal to the insertion of the arterial ligament. The nature and origin of this is discussed.

Bilateral Ebstein's Malformation Associated with Multiple Orifices in the Atrioventricular Valves.

Ebstein's malformation involving both atrioventricular valves is a very rare entity. We report two cases in which the atrioventricular valves were also associated with multiple orifices. The first was an adult with known congenital heart disease.

Fibrous matrix of ventricular myocardium in tricuspid atresia compared with normal heart. A quantitative analysis.

Background: The collagen matrix is a small component of the myocardium, but it provides a supportive framework. An increase in collagen in the pressure-overloaded ventricle is known to cause myocardial stiffness. However, little is known about the collagen matrix in the volume-overloaded ventricle, particularly in relation to congenital heart disease.

Covert congenital cardiovascular malformations discovered in an autopsy series of nearly 5,000 cases.

The objective of this study was to assess the prevalence of covert congenital cardiac malformations. A retrospective analysis was made of a personal series of 4,953 consecutive postmortem examinations performed over 35 years (1946-1980) in a mixed urban and rural region of North Lincolnshire, England. The subjects were 4,210 adults and 743 children (under the age of 16 years).