Abstract
Two cases of congenital tricuspid stenosis complex were described. In this report, we stressed that both two-dimentional and contrast echocardiography were very useful in differentiating congenital tricuspid stenosis from tricuspid atresia, and that accurate anatomic and functional evaluation was a prerequisite for the selection of the surgical procedure.
Case 1. A 14-year-old girl was first admitted to our hospital in April, 1983. Cadiac catheterization performed elsewhere at age 4 revealed “tricuspid atresia”.
On admission, her weight was 34kg (-2.3 SD). The liver's edge was palpable 4 cm below the right costal margin. Hemoglobin was 19g/dl, hematocrit 62%, PaO2 39 mmHg and SaO2 70%. A mean frontal QRS axis of ECG was -36 degrees. The roentgenography revealed cardiac enlargement with a cardiothoracic ratio of 0.67 and diminished pulmonary vasculature.
The two-dimensional echocardiography revealed the complex consisted of a small tricuspid orifice, an atrial septal defect, a ventricular septal defect, a dilated left ventricle with hypokinesis and severe pulmonary stenosis. On the pressure tracing, “a” wave in the right atrium was 11 mmHg and interatrial gradient across the atrial septum was 0.7 mmHg. The cardiac index was reduced at 1.9L/min/m. The left ventricular end-diastolic pressure was 10mmHg.
Angiography demonstrated a narrow orifice of the tricuspid valve (50% normal in diameter), a hypoplastic right ventricle (27% normal in RVEDV). Blalock operation was performed. Postoperatively, she made a satisfactory recovery with improvement of cyanosis and exercise tolerance.
Case 2. A 2-year-old boy was admitted to our hospital in May, 1983, for evaluation of progressive congestive heart failure and pulmonary hypertension.
On admission, his weight was 9.5kg. The liver's edge was palpable 2.5cm below the right costal margin. A harsh systolic murmur of grade 3/6 intensity was heard and the inspiratory increase in intensity of the diasotlic murmur was recognized along the lower left sternal border. Hemoglobin was 14.7g/dl, PaO2 46 mmHg and SaO2 82%.
A mean QRS axis of ECG was -24 degrees. The roentgenography revealed the heart to be increased in size and with a convex pulmonary conus.
The lung fields were hypervascular. The two-dimensional echocardiography revealed a small tricuspid orifice, an atrial septal defect, a ventricular septal defect, normally plastic right ventricle and dilated pulmonary artery. Tricuspid a tresia was excluded by the contrast echography showing flow through the tricuspid orifice. On the pressure tracing, mean interatrial gradient across the atrial septum was 1.0 mmHg. Left ventricular end-diastolic pressure was 5mmHg. Pulmonary hypertension but without pulmonary vascular disease was confirmed by oxygen inhalation method. Angiography showed a narrow tricuspid annulus (46% normal in diameter) and well developed right ventricle (122% normal in RVEDV).
On the basis of these findings, banding of the pulmonary artery was indicated for this case.
