We evaluated the clinical significance of the configuration of right ventricular pressure curves after balloon valvoplasty in 35 patients with pulmonary valvar stenosis. Right ventricular pressures were measured with a fluidfilled catheter. We divided the subjects into two groups according to the pressure curves seen after balloon valvoplasty. In eight patients, two peaks were found in the curves, with the higher peak occurring at late systole. The remaining 27 patients had a single peak observed during early to mid systole. In all patients in the group with a single peak, the ratio of ventricular pressures decreased by more than half, and no residual narrowing was seen in right ventricular outflow tract, the diameter after valvoplasty increasing by more than half over the diameter before the procedure. In contrast, in five of eight patients in the group with double peaks in the pressure curves, the ratio between ventricular pressures remained higher than 0.5, and the diameter of the right ventricular infundibulum was reduced to less than half the diameter prior to balloon valvoplasty. In three of these patients with double peaked pressure contours, to whom propranolol was administered intravenously, the pressure configuration changed to one with a single peak and the ventricular pressure ratio fell to below 0.5. The degree of obstruction of the right ventricular infundibulum also decreased. These data suggest that a high right ventricular pressure and two peaks in the pressure curve with the higher peak at late systole after balloon valvoplasty indicate, first, the presence of a significant narrowing in the right ventricular outflow tract and, second, effective balloon valvoplasty.

We describe a patient with pulmonary atresia and intact ventricular septum in whom the right atrium was divided by a vascular aneurysm located in the right atrioventricular groove. We postulate that the structure represents an aneursymally dilated right coronary artery taking anomalous origin from the pulmonary trunk, with fistulous communication to the right atrium. We discuss the findings relative to concepts of development of the coronary arteries in normal hearts and in pulmonary atresia with an intact ventricular septum.

An infant is described with congenitally corrected transposition and Ebstein's malformation. Banding of the pulmonary trunk had been previously performed because of a muscular ventricular septal defect. The patient underwent the double-switch procedure with the intention of unloading the morphologically right ventricle and the malformed tricuspid valve. This resulted in prompt postoperative functional and haemodynamic improvement.

We report a 12-year-old boy with hypertrophic cardiomyopathy presenting with syncope. His electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block, conduction delay in the anterior fascicle of the left bundle branch, and intermittent complete atrioventricular block. We detected hypertrophic cardiomyopathy in the mother. The relationship between the diseases is discussed, and interpreted in the light of previous descriptions.

The anatomic and pathophysiologic features of tricuspid valvar dysplasia are discussed in the light of two cases observed during fetal life which progressed with an unexpectedly favorable course. Some fetal echocardiographic features are proposed to explain this outcome. First, absence of associated cardiovascular abnormalities; second, clear separation between well represented right ventricular walls and the tricuspid valvar leaflets; third, normal proximal insertion of the tricuspid leaflets and, fourth, normal dimensions of the pulmonary trunk. We recommend karyotyping because of the possible association of this malformation with an aneuploid karyotype. We also describe transitory functional atresia of the pulmonary valve at birth, as far as we know for the first time, in a neonate that had normal forward flow of blood as seen on Doppler sampling in fetal life.

Implantation of the Rashkind double umbrella device for closure of the persistently patent arterial duct is now routine in many units. The use of this device in non-ductal positions, however, has been reported in only a limited number of patients, and its impact on the subsequent clinical course has not been established. A retrospective review of all such occlusions, therefore, was undertaken to address the early results and impact on clinical management. From 9/1988 through 2/1994, implantation of a double umbrella device was attempted in 21 patients (eight female, 13 male) with cardiovascular communications other than a persistent arterial duct excluding the intentional fenestrations made in the modified Fontan procedure. Median age at implantation was 2.6 years (three months-12.4 years) and weight was 11.6 kg (4.1–45) kg. Lesions included four ventricular septal defects, an aortopulmonary window, an aortopulmonary collateral artery, an atrial septal defect, four Gore-tex® shunts, and a residual persistent communication between the superior caval vein and the right atrium following either a bidirectional cavopulmonary anastomosis, or a classical Glenn operation. Eight patients underwent occlusion of unintentional residual right-to-left (six) or left-to-right (two) communications following the Fontan procedure. Implantation was accomplished in all without mortality, utilizing either 12 mm (13) or 17 mm (eight) devices. Surgical removal of the device was required in one patient four weeks subsequent to implantation. At latest follow-up (1–66 months, median four months), color-flow Doppler studies revealed complete occlusion in 17 (77%) patients. Implantation of the double umbrella in sites other than the duct is, therefore, feasible and simplified technically with a modification of the delivery system. Such novel applications of transcatheter intervention using double umbrella devices may avoid or complement subsequent surgery.

Because left ventricular mass may be important in judging feasibility of Fontan type of palliation in tricuspid atresia, this study was undertaken to generate data on left ventricular mass, volume and mass to volume ratio in newborns and infants with tricuspid atresia, native pulmonary stenosis and concordant ventriculoarterial connections prior to any surgical palliation to obtain values for “normal” left ventricular dimensions in tricuspid atresia. The left ventricle was evaluated in the apical two and four chamber view. From these two perpendicular imaging planes, we calculated mass as difference between epicardial and endocardial volume x 1.05 (specific gravity of heart muscle). Mass divided by volume at end-diastole yields the index: mass to volume. Data from 23 newborns and infants with tricuspid atresia were compared to data from 30 age-matched controls with normal hearts. In both patient and control groups, growth of the left ventricle was not linear but related to the 1.4 (volume) or 1.25 (mass) power of body surface area. The equation best describing relation between left ventricular volume and body surface area in normals is volume = 60.7(body surface area)1.4−0.2 ml; in patients with tricuspid atresia volume calculates as 96.7(body surface area)1.4+0.9 ml. In relation to normal infants, infants with tricuspid atresia had a left ventricular volume of 167 (135–206)% of normal and a left ventricular mass of 163 (132–201)% of normal. Left ventricular mass assessed in normals calculates as mass = 59.9(body surface area)1.25+1.3 grams; in patients with tricuspid atresia it calculates as mass= 117.5 (body surface area)1.25−0.9 grams. The index of left ventricular mass to volume in patients with tricuspid atresia measured 1.32, not significantly different from controls, in whom this index calculates as 1.34.

Atrial correction for complete transposition involves a potential risk of later development of right ventricular dysfunction. To determine the reasons contributing to such systemic ventricular failure, radioisotope first-pass and SPECT perfusion studies at rest after one dose of Technetium-99m MIBI were performed in 26 asymptomatic patients, 5.9±2.7 years after a Mustard or Senning repair. Mean age at operation was 4.3±4.3 years. All patients were followed with 24-hour Holter monitoring and echocardiography. Normal perfusion was observed in 11 patients with a mean age at operation of 2.25±1.8 years and mean right ventricular ejection fraction of 49.0±8.7%. Perfusion defects were found in 15 patients with a mean age at operation 5.8±4.8 years (p<0.05) and mean right ventricular ejection fraction of 43.2±9.3% (NS). In the group of older patients, 10 had extensive perfusion defects with significantly lower right ventricular ejection fraction (39.7±9.3%, p<0.05) and even older mean age at the surgery (7.1±5.6 years p<0.02). Mild or moderate tricuspid regurgitation was found significantly more frequently in those patients with perfusion defects (73%) than in the group of younger patients (36.3%), p<0.05. There was no difference in the frequency and type of arrhythmias, nor in the extent of follow-up, between the two groups. We conclude that, in patients undergoing surgery at an older age, it is more common to find perfusion defects and, often, lower right ventricular ejection fraction. Perfusion abnormalities found in such asymptomatic patients may be a result of several factors: impaired right ventricular function as a consequence of pressure and volume overload, enhanced myocardial fibrosis, or long-lasting preoperative hypoxemia and tricuspid valvar dysfunction secondary to ventricular dilation. We suggest that the perfusion defects observed in this study may be a better indicator of systemic ventricular impairment.