Transcatheter coil occlusion of hepatic arteriovenous malformation in a neonate.

A 3-day-old neonate was diagnosed to have severe congestive heart failure due to a large shunt through a hepatic arteriovenous malformation. Percutaneous transcatheter delivery of two detachable coils resulted in complete abolition of the shunt. The patient showed dramatic clinical improvement and resolution of the heart failure, which was sustained on follow-up. This case represents a novel use of detachable occluding spring coils designed primarily for occluding patent ductus arteriosus.

Transcatheter closure of a large coronary artery fistula with Amplatzer duct occluder: a new approach.

We report a new retrograde approach for the successful closure of a large right coronary artery fistula in a 27-year-old man using the Amplatzer duct occluder. The device was deployed through a coronary angioplasty-guiding catheter that had been advanced through the aorta and the dilated right coronary artery into the fistula. This method simplified the procedure by eliminating the need for making a femoral artery-to-femoral vein wire loop.

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.

Assessment of severity of aortic stenosis by continuous wave Doppler echocardiography.

Fifty consecutive patients with aortic stenosis were evaluated by continuous wave Doppler echocardiography for assessment of the transaortic gradient. The Doppler derived gradients were compared with the gradients measured at cardiac catheterisation. Excellent correlation was found between the Doppler and catheterisation findings for the maximum instantaneous gradient (r = 0.92) and the mean systolic gradient (r = 0.84). The maximum, midsystolic and late systolic Doppler gradients also showed a good correlation with the peak to peak catheter gradient. The maximum Doppler velocity however, showed overestimation of the peak to peak gradient in the presence of mild aortic stenosis (predictive accuracy 86%). The midsystolic Doppler velocity showed the highest predictive accuracy (94%) for the detection of severe aortic stenosis. No case of severe aortic stenosis was missed by Doppler using either the maximum or midsystolic Doppler velocity. These findings indicate that continuous wave Doppler ultrasound provides a reliable estimate of the gradient in patients with aortic stenosis.

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.

Double chambered right ventricle with anomalous muscle bundles and false tendons in the left ventricle.

A patient with double chambered right ventricle, with anomalous muscle bundles and false tendons in the left ventricle, is described. This combination of anomalies may represent a failure of regression of foetal trabeculations.