There is a clear and present danger for the patient considered for Fontan's operation in the presence of excessive ventricular hypertrophy and/or mass. Such ventricular hypertrophy and/or mass probably reflects singly, or in combination, banding of the pulmonary trunk and subaortic stenosis, as well as other factors. While one can construct a number of therapeutic algorithms for patients seen at various ages with a restrictive ventricular septal defect and subaortic stenosis in the setting of double inlet left ventricle and discordant ventriculoarterial connections, the optimal management should be to minimize the potential risks for development of subaortic stenosis and excessive ventricular hypertrophy and/or mass.

Follow-up cardiac catheterization and angiography was performed in 23 patients after complete, staged repair of pulmonary atresia with ventricular septal defect and multiple systemic-to-pulmonary collateral arteries. Time interval from repair varied from 0.2 to 89 months (mean 26 months). Major residual anomalies of arborization of the pulmonary arteries, with or without pulmonary vascular disease, were present in six patients, two of whom had die patch on the ventricular septal defect perforated because of systemic or suprasystemic right ventricular pressure in the early postoperative period after repair. Both of these children later died, while the other four patients were found to have moderately to severely elevated ventricular pressure ratios and pulmonary arterial pressure measurements, though they are all alive at follow-up intervals from 20 months to 6.8 years. The remaining 17 patients all had hemodynamically satisfactory or good results, with pulmonary arterial mean pressures ranging from 8 to 30 mm Hg (mean 22 mm Hg) and pressure ratios between the ventricles from 0.24 to 0.75 (mean 0.43)—the higher ratios generally reflecting gradients across the conduit valve. Angiography demonstrated that the hypoplastic pulmonary arteries increased in size to normal dimensions, during staging, in most patients, though abnormalities of arborization persisted in several. Analysis of the data from the entire cohort of 58 patients, who were accepted for staging towards repair, showed a significant association between early age at entry to the program and likelihood of complete repair being achievable.

Lung biopsies were taken from 30 children aged three months to 15 years (median, 11 months) who had pulmonary hypertensive congenital heart disease and were living at an altitude of 1750 meters. They had either a ventricular septal defect and/or patency of the arterial duct, atrioventricular septal defect or complete transposition with a ventricular septal defect. Biopsies were studied using quantitative morphometric light microscopic techniques. All patients with a ventricular septal defect with or without patency of the arterial duct showed a significant increase in mean percentage arterial medial thickness of both pre- and intraacinar pulmonary arteries compared with those of normal children of similar age living at sea level (p<0.001 for both pre- and intraacinar vessels) and with children with a ventricular septal defect living at sea level (p<0.001 for both pre- and intraacinar vessels). Extension of muscle to more peripheral pulmonary arteries was also greater. Intimal proliferation and fibrosis was seen in 10 patients, in three of whom it was severe. Intimal proliferation occurred more frequently than in children with a ventricular septal defect living at sea level. The findings were similar in patients with atrioventricular septal defect and complete transposition with ventricular septal defect. These findings suggest that patients with congenital heart disease who live at a relatively high altitude develop pulmonary vascular disease more rapidly than do those living at sea level.

The diameter of the pulmonary outflow tract was measured from the right ventricular cineangiogram at different phases of the cardiac cycle prior to balloon valvoplasty in 54 patients with congenital pulmonary valvar stenosis. Measured at the level of the hinge point of the valvar leaflets, the pulmonary outflow tract was found to be largest at end-diastole and smallest at end-systole. The mean increase in diameter from systole to diastole was 25.3% (range 6–53%) for 47 patients with typical valvar stenosis, and 24.3% (range 14–41%) for seven patients with dysplastic valvar leaflets (p>0.5). Dilatation using balloons less than 1.2 times the size of the maximal end-diastolic diameter produced a mean reduction in the pressure gradient from right ventricle to pulmonary trunk of 71.1%, compared to 76.2% with bigger balloons (p>0.3). Two patients with an initial ratio between balloon and outflow tract of 0.9 required a second dilatation. The timing of measurement of the diameter of the pulmonary outflow tract should be standardized. Oversized balloons probably are not necessary for pulmonary valvoplasty.

A ventricular septal defect is, almost always, an integral part of double outlet right ventricle and has been classified into the subaortic, subpulmonary, doubly committed and non-committed varieties. This study was performed to correlate the cross-sectional imaging characteristics of such ventricular septal defect in double outlet right ventricles using pathological specimens. The extent and the orientation of the outlet septum were the most important in the differentiation of the four varieties of ventricular septal defect. In the subaortic variety, the outlet septum fused with the left anterior margin of the defect, this being marked by the anterior limb of the septomarginal trabeculation. In the subpulmonary variety, the outlet septum fused with the right posterior margin of the defect, this being the posterior limb of the septomarginal trabeculation. The outlet septum was vestigial in case with doubly committed defects. In those with non-committed defects, the defect was not shown in those images or sections which demonstrated the outlet septum.

We examined the echocardiograms of 40 patients with double oudet right ventricle, proven either at autopsy or during surgery. By way of the subcostal approach, it proved possible to determine the insertion of the outlet (infundibular) septum and to categorize the malformations within the classification proposed by de la Cruz and her colleagues. It was also possible to ascertain the relationships of the atrioventricular valves with one or with both infundibulums, and these features could be correlated with infundibular interrelationships. The interrelationship of the great arteries, in contrast, was not always in accordance with the infundibular interrelationships, indicating that the arterial interrelationship may not prove to be a good parameter for surgical decision-making. Of greatest importance was the visualization and echocardiographic appraisal of the spatial position and size of the ventriculoinfundibular fold, since this anatomic feature was the key to determining the optimal surgical approach. This is so because the ventriculoinfundibular fold constitutes one of the borders of the ventricular septal defect, and separates it from the plane of the leaflets of the arterial valves. Furthermore, the fold frequendy contributes to infundibular obstruction. Our echocardiographic studies, based on a better anatomic knowledge, permitted us to choose the most suitable surgical technique in our patients.

We studied the morphology of the aortic valves of 1,036 Syrian hamsters belonging to two families subjected to high endogamous pressure. Most (n=955) specimens were examined using a corrosion-cast technique. In the remaining 81 specimens, valvar morphology was assessed by means of a stereomicroscope, and in 18 of these cases a histologic study was also performed. In one of the families, a high proportion (30.5%) of hamsters had aortic valves with two leaflets, the aortic sinuses being oriented ventrodorsally. The percentage of such anomalous valves was found to be positively correlated with the inbreeding coefficient of specimens. In the other family, the occurrence of an aortic valve with two leaflets was a sporadic event. Stereoscopic and histologic observations demonstrated that, in 52 of 63 aortic valves with three leaflets, the ventral commissure between the right and left leaflets was partially fused, while in 10 other cases this commissure was completely fused. In addition, in nine of 18 aortic valves with two leaflets, there was a more or less well-developed raphe located in the ventral aortic sinus, approximately where the true ventral commissure would have been. Our observations suggest the existence of a continuous spectrum of aortic valvar morphology, ranging from a valve with three leaflets and no fusion of the ventral commissure to an aortic valve with two leaflets devoid of any raphe. This spectrum seems to be the result of the interaction of genetic and intrauterine environmental factors. Results of crosses between both related and unrelated hamsters suggest that the observed morphology in individuals is the result of quantitative inheritance.

Embolization of major aortopulmonary collateral arteries by coils introduced via catheterization is a well-established procedure for the control of excessively high pulmonary blood flow in patients with tetralogy of Fallot and pulmonary stenosis or atresia. Under special circumstances, this procedure can be life-saving. Between October 1987 and February 1992, four patients with tetralogy of Fallot and pulmonary atresia and one patient with pulmonary stenosis rather than atresia underwent transcatheter embolization of major aortopulmonary collateral arteries as an emergency. Their ages ranged from 41 days to 13 years, with a mean of 45 months. Two patients had recently undergone surgical repair of tetralogy of Fallot while one had been palliated by enlarging the right ventricular outflow tract without closing the ventricular septal defect. All three had been dependent on mechanical ventilation in the postoperative period with signs of pulmonary congestion or intraparenchymal hemorrhage. Eight embolizations were attempted with 0.025 or 0.038 inch coils; seven collateral arteries were completely occluded and one was partially occluded. Thirteen coils were used in total. The systemic saturation rose in one postsurgical patient, with radiological evidence of decreased pulmonary congestion and regression of hemorrhage. In the other cases, the systemic saturation was unchanged. Peak pulmonary arterial pressures dropped in two patients but remained unchanged in three. Our experience suggests that embolization of major aortopulmonary collateral arteries can be beneficial in critical patients, thus avoiding the risks of surgical reexploration.

Isolated fetal pericardial effusion is generally held to have a good prognosis. We report a study of seven cases identified from a total of 504 fetuses scanned and followed with serial fetal scans.Our results show that it was associated with a poor fetal prognosis in six of the seven pregnancies. Probably due to the high incidence of mid-trimester abortion and the association with other abnormalities only evident postnatally, this has not, hitherto, been reported. We would advocate close follow-up and a guarded prognosis when isolated pericardial effusions are found on fetal scans.

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.

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.

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.

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.

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.