Isolated hypoplasia of right ventricle with atrial septal defect: a rare form of cyanotic heart disease.

Isolated hypoplasia of right ventricle is a rare kind of congenital heart disease that can present with cyanosis in childhood. We evaluated the clinical profile, diagnosis and management strategy of isolated hypoplasia of right ventricle in children. During 1993-1997, six children were diagnosed to have isolated right ventricular hypoplasia in our institution. Two patients were referred because of cyanosis, while cardiac murmur was the reason for referral in the remaining four. Besides clinical evaluation, all these patients had chest radiography, electrocardiography and echo-doppler studies. At echocardiography the valve diameters were measured and the degree of hypoplasia was quantified as standard deviation units. Cardiac catheter studies and angiography, and surgical intervention were carried out where indicated. Both operated and unoperated patients were followed up for 3-5 years. Cyanosis (severe--2, mild--4) and a soft ejection systolic murmur at the left sternal border were present in all patients. The second sound was normal. On two-dimensional echocardiography, all had hypoplasia of right ventricular (trabecular portion) and bi-directional shunt across atrial septal defect. Cardiac catheterisation was performed in four patients, which confirmed the echo findings and revealed normal right heart pressures. These four patients underwent surgical procedures. Simple closure of atrial septal defect was sufficient in two patients. Two others required bi-directional cavopulmonary anastomoses, and atrial septal defect closure was tolerated only by one of these two patients. Complete correction is not always feasible and adequacy of right ventricle to receive the entire venous return should be accurately assessed prior to, as well as during surgery.

Two dimensional and Doppler echocardiographic evaluation of double chambered right ventricle.

Two-dimensional and Doppler echocardiographic findings in 20 patients with double chambered right ventricle are described. All patients had the diagnosis established by cardiac catheterisation and confirmed at operation. Echocardiographic evaluation was done prior to surgical correction. Anomalous muscle bands in right ventricular cavity were detected in 16 patients. Doppler flow velocities in the right ventricular cavity suggested infundibular obstruction to blood flow at a low level in all 17 patients studied by Doppler echocardiography. Ventricular septal defects (11 patients), pulmonary stenosis (3 patients), and aortic regurgitation (3 patients) were detected accurately before operation by echocardiographic examination. Failure to detect the anomalous muscles in right ventricular cavity may occur in adult patients with poor anterior resolution and in those with severe right ventricular outflow obstruction and myocardial hypertrophy. Two dimensional echocardiography with Doppler flow analysis is useful in the evaluation and differential diagnosis of right ventricular outflow obstructions prior to invasive studies and surgical intervention.

Two-dimensional and Doppler echocardiographic study of coronary arteriovenous fistulas.

Sixteen patients with coronary arteriovenous fistula (CAVF) were studied by two-dimensional echocardiography (2DE). Of these 12 had Doppler studies. In all, the diagnosis of CAVF was confirmed by aortic root or selective coronary angiography. In 8 patients, the 2DE findings suggested CAVF. Five patients had dilated main coronary arteries and in 4 patients abnormal fistulous channels were identified. Two had diastolic flutter of the tricuspid valve and one had spontaneous contrast in the right atrium. In 11 out of 12 patients, Doppler analysis picked up a continuous flow (to the right atrium in 5, right ventricle in 4 and to both ventricles in 2 patients) prior to the cardiac catheterisation. We conclude that noninvasive diagnosis of CAVF can be made with the combined use of two-dimensional and Doppler echocardiography in most of the patients.