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Surgical treatment of absent pulmonary valve syndrome.

The absent pulmonary valve syndrome includes agenesis of the pulmonary valve, annular stenosis, and pulmonary insufficiency. The pulmonary arteries are aneurysmal and usually compress the tracheobronchial tree. These features are associated with a ventricular septal defect and right ventricular hypertrophy. Nineteen children aged 5 days to 11 years were treated between 1976 and 1983. Nine were younger than 1 year. Intractable respiratory symptoms and heart failure were the main features in the infant group; five required preoperative assisted ventilation. Older children had decreased exercise tolerance and repeated respiratory infections. Several surgical techniques were used in this series. The best results were achieved when the size of the main, right, and left pulmonary arteries was reduced by extensive anterior resection; the ventricular septal defect was closed; and an aortic homograft was interposed between the right ventricle and the pulmonary artery. Four infants and four children treated in this way survived the operation. Five of the nine operated infants died (56%); all older children survived the operation. At follow-up, six children were asymptomatic and one was in New York Heart Association Class II. All four infants were progressing satisfactorily although all had some residual pulmonary symptoms. Closure of the ventricular septal defect, reduction of the size of the pulmonary arteries, and insertion of an aortic homograft was the optimal treatment in our series.

Aneurysm↗

[A case of mitral valve replacement with autologous pulmonic valve in congenital mitral stenosis].

A one-year-old infant with congenital mitral stenosis and pulmonary hypertension underwent by mitral valve replacement with his pulmonic valve autograft and pulmonary tract reconstruction with heterogeneous pericardial conduit. His postoperative hemodynamics data showed that left atrial pressure decreased and pulmonary hypertension continued. Echocardiography showed that the implanted autograft functioned properly. On the seventh postoperative day, he died of pulmonary hypertension. In case with congenital mitral stenosis with the small mitral annulus and the small left ventricular cavity, it is difficult to perform mitral valve replacement by commercially available mechanical or tissue valves. Because these valves are not suitable for the small mitral annulus. The mitral valve replacement with pulmonic valve autograft is recommended in such a case with the congenital mitral stenosis.

Female↗

[Effect of the respiratory phases on the right jugular vein pulsation curve and pulmonary hemodynamics in mitral valve stenosis].

In patients with mitral stenosis the main and early changes in the pulsation curve of the right jugular vein consist in an elevation of the y-collapse. The height of the y-collapse correlates with the degree of venous congestion in the lungs, i.e. a parallelism exists between the signs of venous congestion in the pulmonary and general circulation systems. The degree of venous congestion in the lungs of patients with mitral stenosis varies with the phases of respiration. An elevation of the v-collapse in mitral stenosis cases is observed at early stages of the disease, simultaneously with the disorders in venous circulation in the upper lobes. The mechanism of circulatory disorders in the veins of the general circulation system may be due to not only pulmonary hypertension and right ventricular insufficiency, but also to the changes in the intrathoracic pressure secondary to the changes in pulmonary parenchyma.

Hemodynamics↗