Transcatheter device closure has emerged as the preferred treatment for ostium secundum atrial septal defects with adequate margins, occasionally requiring surgical intervention to manage complications. We report a case of a young female patient who presented with systemic desaturation and exertional dyspnoea 5 years after device closure. Evaluation demonstrated anterior malposition of the septal occluder, with the right atrial rim of the device diverting the blood from the inferior vena cava to the left atrium. She underwent surgical extraction of the device and pericardial patch closure of the atrial septal defect.

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 present a patient with primary pulmonary hypertension who had unusually high pulmonary arterial pressure prior to double-lung transplantation. Obstruction of the right ventricular outflow tract developed after transplantation and progressed over the subsequent two years.

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.

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.

Double-chambered left ventricle is a rare CHD that is usually asymptomatic and managed conservatively but can present with ventricular arrhythmias or heart failure. It is important to differentiate from acquired diseases such as post-infarct pseudoaneurysm that need surgical treatment, and cardiac MRI offers an excellent diagnostic tool.