A complex case of congenital heart disease is presented which features isomerism of the left atrial appendages combined with bronchoatrial discordance and an anteriorly placed non-interrupted inferior caval vein draining to the left-sided atrium. This combination of anomalies has, as far as we are aware, not been described before. It emphasizes the importance of direct confirmation of atrial arrangement by atrial angiography or transesophageal echocardiography.

Division of the left atrium is a rare congenital lesion, but one which is amenable to complete surgical correction. We describe twin brothers, demonstrated to be monozygotic, who presented in infancy with cardiac failure, pulmonary congestion and mild cyanosis. Both were found to have systemic and pulmonary venous return to a common atrial chamber, separated from the left atrial appendage and the vestibule supporting the mitral valve by a typical muscular diaphragm with a single small orifice. In one twin, there was also persistence of the left superior caval vein, while, in the other twin, a remnant of the primary atrial septum was present. Excision of the diaphragm and neoseptation of the atrial chambers was performed in both infants. At 10 years after surgery, there was normal growth and development in both twins, with no evidence of recurrent obstruction.

The role of the pathologist is changing with regard to the assessment of postoperative congenital heart disease. Whereas, in the past, the access to autopsied hearts provided the opportunity for detailed description and classification, nosologic details of cardiac malformations are now largely agreed. While a detailed analysis of the lesion remains an integral part of the pathologist's task, future advances will now largely be dictated by careful analysis of the effects of cardiac surgery. Such analysis can be divided into early and late phases. When assessing the specimen from a death occurring in the operating room or in the immediate postoperative period, the pathologist must examine carefully the operative incisions, and the correctness of the surgical procedure. Proper assessment of the procedure will necessitate removal of patches and pathways constructed by the surgeon, having documented carefully the location of sutures used to secure the foreign materials. The autopsy must also include assessment of the organs in the rest of the body, particularly the lungs, and must not ignore the immediate effect of the operative procedure on myocardial status. In the late phase, while again assessing in detail the access to the heart and the procedures employed during surgical correction, the pathologist will take care to evaluate the long-term effects of the procedure on the valves of the heart, the myocardium, the pulmonary vasculature and the other organs. At this stage, care should also be taken to ensure that late scarring does not involve the conduction tissues. Particular care will be needed when assessing the efficacy and durability of prosthetic materials inserted into the heart. Most of these steps will be undertaken intuitively by the pathologist, but interest in cardiac pathology as a specialty seems to be waning. The clinician can combat this trend by ensuring that all cases dying after operative procedures are obtained for autopsy, and by encouraging the pathologist to become involved in this fascinating area.

A five day old infant underwent repair of coarctation by resection and end-to-end anastomosis of the proximal and distal segments of the arch. Four weeks later, cross-sectional Doppler echocardiography showed a tight recoarctation proximal to the origin of the left subclavian artery. A dilated proximal left subclavian artery was demonstrated, with continuous flow from it into the descending aorta. These findings were confirmed at catheterization. This “vertebral arterial steal” disappeared following transcatheter balloon angioplasty.

Congenital heart disease occurs in roughly 0.7% of live-births. Approximately one-half of these defects are severe, requiring surgical correction or palliation. In a large percentage of such procedures in children, moderate or profound hypothermic cardiopulmonary bypass is employed. Because, nowadays, early correction of the defect rather than a palliative procedure is more frequently pursued, the surgeon, anesthesiologist, and perfusionist are called upon to manage younger and smaller children undergoing longer and more complex operations which necessitate longer times on bypass. Due to the particular underlying heart defect, the pathway of flow and viscosity of the blood are frequently different from those encountered in the adult with atherosclerotic coronary arterial or valvar heart disease. Thus, it is important for those caring for children undergoing cardiac surgery utilizing bypass to be well informed about its possible detrimental effects on the various organs. The purpose of this review will be to focus on the effects of bypass on flow to, and metabolism of, the organs and to highlight what is known with regard to these features in children rather than adults.

We examined the number of elastic lamellae in the wall of the proximal aortic arch, aortic isthmus and descending aorta in patients with coarctation of the aorta. In the proximal aortic arch, the number of elastic lamellae was significantly lower in patients with coarctation compared to those with normal hearts without aortic anomalies and those with intracardiac defects but without aortic anomalies. The isthmus also showed a significantly lower number of elastic lamellae in the presence of preductal coarctation. In the descending aorta, the number of elastic lamellae was not significantly different between the different groups. There is doubt about the etiology of coarctation. Recent investigations showed that cells from the cardiac neural crest contribute to the formation of the arch arteries and the media of the arch. A developmental error of the neural crest might be responsible for the abnormal mural structures found in patients with aortic coarctation.

We report echocardiographic findings of an intracardiac mass in a hydropic fetus who died after four weeks of follow-up. Within that period, the left ventricular myocardium had developed extensive echo-dense patterns suggesting tumorous infiltration. Autopsy revealed extreme calcification of the mitral valve, which had mimicked a tumorous mass on fetal echocardiography. The histological results were consistent with viral myocarditis.

We reviewed the echocardiographic features of 16 children with prominent Eustachian valves, six of whom had associated pathology of the right heart, while eight had other anomalies, primarily atrial and ventricular septal defects. The hearts of two patients were structurally normal. Prolapse of the membrane through the orifice of the tricuspid valve or through the atrial septum was present in seven patients, two of whom were symptomatic. One presented with severe cyanosis which resolved after surgical removal on the basis of the echocardiographic findings. The other presented with atrial arrhythmias which did not improve with removal. The size or behavior of the membrane, including prolapse, did not correlate with either age or presence of pathology of the right heart. The echocardiographic appearance of prominent Eustachian valves is variable. Rarely, when prominent, the valves can cause symptoms associated with obstruction to the right heart, particularly cyanosis or an arrhythmia.

Two neonates are described presenting in severe cardiac failure within a few hours of birth. Echocardiography and the demonstration by color-coded Doppler of retrograde diastolic flow in the proximal descending aorta suggested the presence of large cerebral arteriovenous malformations. Such lesions were demonstrated by cranial ultrasound. The early diagnosis permitted early intervention, albeit with less than satisfactory outcome.

We describe the unusual association of a divided left atrium (cor triatriatum) with an atrioventricular septal defect with separate atrioventricular orifices in an eight-year-old asymptomatic girl. The diagnosis had been made by cross-sectional echocardiography combined with Doppler technology and color-flow mapping. These methods together demonstrate both the anatomic and hemodynamic findings.

The activation of inflammatory systems as a consequence of cardiopulmonary bypass represents one possible means by which inj ury to tissues and associated dysfunction of organs may occur in the postoperative cardiac patient, both pediatric and adult. Kirklin and colleagues demonstrated in the early 1980's that complement activation occurs as a consequence of blood exposure to the extracorporeal circuit. Subsequent studies demonstrated this to be true in systems using both bubble and membrane oxygenators. The anaphylatoxin C5a and its metabolite C5a des arg are produced during activation of the complement cascade. They are potent stimulants which may induce activation of neutrophils. This activation may be manifested as changes in shape, decreased cellular deformability, and changes in adhesive and secretory function. Activation of neutrophils by these fragments of complement could contribute to neutrophilmediated damage to tissues by promoting retention of neutrophils in vascular beds and secretion of cytotoxic substances. Recent studies demonstrating the release of neutrophil granular enzymes into the plasma following cardiopulmonary bypass are evidence that activation of neutrophils is taking place in this setting.

Doppler echocardiography revealed a characteristic pattern of flow in four neonates with postductal coarctation of the aorta. There was a continuous left-to-right shunt at the level of the patent arterial duct with simultaneous measurement of a large gradient across the site of coarctation with a pandiastolic gradient and prolonged pressure half-time. Infusions of prostaglandin produced no hemodynamic improvement, and urgent surgical correction was performed.

Cardiopulmonary bypass makes a mess of homeostasis. Extracorporeal perfusion produces pulseless flow without physiologic controls, showers microemboli throughout the body, and brings blood diluted with additives to an enzymatic boil. Much of the morbidity associated with open heart surgery is due to contact of blood with the synthetic surfaces of the extracorporeal perfusion circuit. Bypass temporarily impairs function of every organ, alters concentrations of over 25 vasoactive substances within the plasma, and causes massive retention of fluid. In short, cardiopulmonary bypass makes patients sick.

Formation of aortic aneurysms is an important and not infrequent sequel of surgical repair of aortic coarctation. Detection is often haphazard and fatal ruptures are reported. We report a series of cases where resonance imaging identified aneurysmal dilatation of the thoracic aorta after either patch aortoplasry or subclavian flap repair of aortic coarctation. In one, rupture of the aneurysm occurred five months following the initial resonance study. Repeat imaging confirmed the clinical suspicion of rupture and successful surgical resection was subsequently performed. Magnetic resonance is a practical and reproducible imaging technique which is particularly valuable in the identification of abnormalities within the thoracic aorta following surgical aortoplasty or interventional procedures for aortic coarctation. As a method of routine surveillance in these groups of patients, it is superior to any alternative technique.

Systemic vascular malformations are a rare cause of congestive heart failure in the infant and newborn infant. Clinical diagnosis is often problematic. Accordingly, this study presents the clinical, radiographic, ultrasound imaging, pulsed and color Doppler features of systemic vascular malformations in six infants. In all cases, color Doppler provided vital information regarding size, location, source of feeding arteries and venous drainage essential for appropriate management of these patients. Color flow Doppler ultrasonography is an accurate and sensitive modality for detecting and evaluating vascular malformations.

Isomerism of the left atrial appendages is often associated with cyanosis due to intracardiac structural abnormalities such as common atrium and/or anomalous pulmonary or systemic venous connections. We report two cases of children with cyanosis and left isomerism but no other lesions within the heart. In these cases, multiple small pulmonary arteriovenous malformations were found bilaterally, resulting in intrapulmonary right-to-left shunting and systemic desaturation.

Pulmonary atresia with abnormal ventriculoarterial connections in the presence of an intact ventricular septum is rare even in postnatal life. Two cases are described in which this combination of lesions was diagnosed in previable fetuses at autopsy. Both pregnancies were terminated for gross structural abnormalities discovered on antenatal ultrasound scans. In the first case, the pulmonary atresia was associated with intact ventricular septum, a double outlet left ventricle and hypoplasia of the tricuspid valve, right ventricle and arterial duct, a combination of lesions that would appear to be unique. In the second case, there was complete transposition and a hypoplastic left ventricle. The severity of the cardiac lesions in both fetuses was such that it is unlikely that either would have survived to term. In the first case no other malformation was present, but in the second there was also a large encephalocoele secondary to early amnion rupture, and a single umbilical artery. The association of the cardiac lesion with early amnion rupture in this second fetus would appear to be a chance event.

Discrete subaortic stenosis is an acquired lesion that is rare in infancy. We report an infant in whom the lesion developed within four months after cross-sectional echocardiography had revealed an apparently normal outflow tract. Surgical repair was successfully undertaken.