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Reconstruction of the right ventricular outflow tract in tetralogy of Fallot and pulmonary stenosis with a monocusp patch.

In order to reduce postoperative pulmonary insufficiency (PI) a transannular monocusp patch was implanted in 14 patients with severe tetralogy of Fallot and hypoplastic pulmonary valve ring (group A). The results of left and right heart catheterization, obtained within one year of the correction, were compared to those of 9 patients, who received a simple transannular pericardial patch (group B). The mean maximal systolic pressure ratio between the right and left ventricle (A = 0.43 +/- 0.03; B = 0.49 +/- 0.04), the mean maximal systolic pressure gradient between the right ventricle and the pulmonary artery (A = 10.38 +/- 0.52 mmHg; B = 12.2 +/- 2.5 mmHg), and the degree of PI (A = 24.7 +/- 3.4%; B = 22.0 +/- 3.0% of total stroke volume) were not significantly different (p greater than 0.05) for the 2 groups. Although optimally implanted, the available monocusp patches cannot prevent or reduce postoperative pulmonary insufficiency in patients with severe tetralogy of Fallot. This experience suggests the need for a simple transannular pericardial patch if the pulmonary valve ring requires enlargement.

Adolescent↗

Percutaneous transcatheter myectomy of subvalvar pulmonary stenosis in tetralogy of Fallot: a new palliative technique with an atherectomy catheter.

A male infant with tetralogy of Fallot was given a modified left Blalock-Taussig shunt at 11 days of age because of duct dependence. Twenty months later, because of increasing cyanosis and polycythaemia, he was given palliative treatment with balloon dilatation of the right ventricular outflow tract. Seven months later a right ventricular angiogram showed further progression of the infundibular stenosis with poor anterograde opacification of the pulmonary arteries. He underwent percutaneous transcatheter myectomy of the infundibulum with an atherectomy catheter. One month later he was well and less cyanosed.

Cardiac Catheterization↗

The surgical anatomy of tetralogy of Fallot with pulmonary atresia rather than pulmonary stenosis.

We examined the pertinent surgical features of the anatomy of 56 hearts having tetralogy of Fallot with pulmonary atresia instead of stenosis, or malformations with pulmonary atresia closely related to tetralogy. We took particular cognizance of the pulmonary arterial supply in 15 hearts in which this was derived through systemic-to-pulmonary collateral arteries, dissecting, as far as possible, the bronchopulmonary segmental distribution of the collateral arteries compared to the intrapericardial pulmonary arteries in 11 of these hearts. Two of the hearts had absence of intrapericardial pulmonary arteries, so that a solitary arterial trunk left the base of the heart. Evidence of an atretic subpulmonary infundibulum was found in 40 of the hearts, while such an infundibulum was lacking in the remainder. The pulmonary atresia was muscular in 43 hearts, valvar in 11, while the pulmonary trunk was absent in the other two hearts. In the hearts with collateral arteries, on average 2.6 collaterals were found in each case, varying from two to five per case. Only one of these arose from a brachiocephalic artery, the others all arising from the descending aorta. The distribution of collateral arteries in two cases was remarkably reminiscent of the arrangement of bronchial arteries. As far as could be judged, 16.5 bronchopulmonary segments on average were supplied in each heart, 5.1 exclusively by collateral arteries, 11.8 by intrapericardial pulmonary arteries and an average of 0.64 segments per case having a shared supply.

Collateral Circulation↗

Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect.

Clinical and morphological findings are described in 2 adults with combined valvular pulmonary and aortic stenosis with atrial septal defect, an extremely rare combination of lesions. The direction of the shunt via the atrial septal defect in this combination appears to be dependent on the relative degree of outflow obstruction on the right side compared with the left side of the heart. In 1 patient the degree of obstruction at the pulmonary valve was nearly three times that at the aortic valve and, therefore, the shunt was right to left and the patient was severely cyanotic. In the other patient, the degree of obstruction at the pulmonary valve was slightly less than that at the aortic valve and, therefore, the shunt was entirely left to right and the patient was acyanotic. Diagnosis of an associated obstructive lesion on the left side of the heart in a patient with an obstructive lesion on the right side of the heart is essential because operative relief of the right-sided obstruction without relief of the left-sided obstruction may lead to rapid, fatal pulmonary congestion.

Adult↗