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At least 361 records · Page 20Linked to original sources

[Surgical correction of interventricular communication with pulmonary stenosis in a hemophiliac].

Open heart surgery was performed in a 5 year-old boy with severe hemophilia A and large ventricular septal defect with pulmonary stenosis. High doses of Factor VIIIC superconcentrates delivered as small transfusion volumes allowed the use of extracorporeal circulation with heparin. Transfusions began 5 hours before surgery and were stopped 21 days later. No adverse reaction was observed. Thus major surgery is possible in severe hemophiliacs provided strict rules for replacement therapy are followed.

Child, Preschool↗

Lecompte procedure for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis.

From December 1988 to February 1993, 19 patients were treated by the Lecompte procedure for complete transposition of the great arteries associated with a ventricular septal defect and pulmonary stenosis. The mean age at operation was 3.1 +/- 0.8 years (mean +/- standard error). This technique consisted of resecting the outlet septum, constructing a tunnel that connected the left ventricle to the aorta, closing the proximal pulmonary arterial stump, pulling the distal pulmonary artery down to the right ventriculotomy site directly, and covering anteriorly with the fixed autologous pericardium. Operative mortality was 5.3%. The mean follow-up was 24.2 +/- 3 months, with no late death. One reoperation was performed because of residual right ventricular outflow tract obstruction. All survivors were studied by echocardiography at intervals of 6 months to 1 year. In all survivors (except for 1 child who underwent reoperation), the estimated pressure gradient between the right ventricle and the pulmonary artery, the structure of the left ventricular outflow tract, left ventricular function, and right ventricular contractility were all satisfactory.

Abnormalities, Multiple↗

Numerical evaluation of facial pattern in children with isolated pulmonary stenosis.

A facial contouring technique, using light sectioning (Cobb, 1972), was modified (Ainsworth and Joseph, 1977) and used in a numerical study of children with isolated pulmonary stenosis (PS) to test the hypothesis that the facial pattern in this condition differs from the normal. Measurements were compared between a group of 20 normal children and a group of 20 children with PS between ages 6 and 10 1/2 years. A distinctive facial pattern emerged. Many anteroposterior measurements were much greater in the PS group, indicating that the tissues were more prominent in the maxillary region. Altogether, 29 measurements showed significant differences (P less than 0.05) between the two groups. Discriminant analyses were carried out to discover which, if any, might be used to predict the group to which an individual should belong. Depending on the variables chosen, between 34 and 37 individuals from the total of 40 were assigned to the correct group--PS or control.

Child↗

Hereditary cholestasis combined with peripheral pulmonary stenosis and other anomalies.

A syndrome consisting of chronic intrahepatic cholestasis with retention of bile acids but with normalization of bile pigment excretion ahd blood lipids, peripheral pulmonary stenosis, vertebral anomalies and a characteristic facies is described in six patients, including a father and his daughter. An autosomal dominant mode of inheritance is suggested.

Abnormalities, Multiple↗

Arterial duct angioplasty as an adjunct to dilatation of the valve for critical pulmonary stenosis.

OBJECTIVE: To assess the efficacy of arterial duct angioplasty in maintaining adequate ductal patency in neonates with critical pulmonary valve stenosis. PATIENTS: Two neonates presenting with cyanosis due to critical pulmonary valve stenosis with severe right ventricular hypoplasia underwent percutaneous balloon dilatation of the pulmonary valve. Despite successful dilatation, both remained cyanosed while receiving prostaglandin E2 infusions. RESULTS: Angioplasty of the arterial duct resulted in increased arterial oxygen saturations. Adequate arterial duct patency was maintained for three months during which time right ventricular dimensions increased enough to allow sufficient pulmonary circulation without the duct. CONCLUSIONS: Arterial duct angioplasty is an effective additional treatment for patients with critical pulmonary valve stenosis whose right ventricle is too small to provide adequate forward flow after balloon dilatation of the valve. Arterial duct angioplasty sustains adequate patency of the duct while the right ventricle grows sufficiently to undertake the pulmonary circulation.

Angioplasty, Balloon, Coronary↗

Chylothorax in a polysplenia infant with cor triatriatum, pulmonary stenosis and sick sinus syndrome.

An infant with chylothorax and polysplenia is described. Her chylothorax was refractory and related to right-sided heart failure derived from pulmonary stenosis, cor triatriatum, and sick sinus syndrome. In addition to Brock operation and cardiac pacing, thoracic duct ligation was performed, but it resulted in an only transient amelioration of the chylothorax. Eventually, the chylothorax resolved when total cardiac repair was performed for cor triatriatum. We propose that, in a patient with chylothorax and right-sided heart failure caused by compound cardiac malformations, as in this patient early surgical treatment for the underlying disease of right-sided heart failure should be considered.

Chylothorax↗

Transposition with ventricular septal defect and pulmonary stenosis: the Rastelli operation.

A total of 52 Rastelli operations have been performed at the Mayo Clinic for transposition of the great arteries with ventricular septal defect and pulmonary stenosis. Of the 43 patients over four years of age, two died (5%). A much higher mortality among younger children, in our earlier experience, led to a policy of early palliation, if required, by a Blalock-Taussig shunt, with later correction. The technique which evolved is described. The results of the operation thus far suggest that its success is comparable to that for the repair of the tetralogy.

Abnormalities, Multiple↗

Comparison of efficacy of medium-sized cutting balloons versus standard balloons for dilation of peripheral pulmonary stenosis.

The efficacy of medium-sized cutting balloons (CBs) 5 to 8 mm in diameter was compared with that of standard balloons (SBs) in the dilation of peripheral pulmonary stenosis (PPS). Nine vascular lesions in 7 patients were primarily dilated by CBs, and 13 vascular lesions in 11 patients were dilated by SBs alone. The efficacy of balloon dilation was evaluated by angiography and intravascular ultrasound (IVUS). There was no significant difference between the 2 groups in the success rate (66% vs 62%), the percentage increase in minimal lesion diameter (MLD; median 71% vs 52%) on angiography, and the number of new tears (2 vs 1) on IVUS. However, in the subgroup of vascular lesions with MLDs of <4.0 mm, for which CBs with diameters >200% of the MLD could have been applied, the percentage increase in MLD (95% vs 53%, p <0.05) and the number of tears (2 vs 1, p <0.05) with CBs were significantly greater than with SBs, requiring significantly smaller maximum balloon pressure (10 vs 13 atm, p <0.05). CBs are as effective as conventional balloons in the dilation of PPS and may be more effective when larger balloons are available.

Catheterization↗

Doppler estimation of pressure gradient in pulmonary stenosis: maximal instantaneous vs peak-to-peak, vs mean catheter gradient.

We undertook a study to identify the hemodynamic significance of a Doppler-derived gradient across a stenotic pulmonary valve. Furthermore, we attempted to define the optimal plane for velocity data acquisition. A total of 17 children with valvar pulmonary stenosis were evaluated using Doppler echocardiography. Flow-velocity profiles were obtained from both the parasternal and subxiphoid windows. Ten of 17 patients were studied before and after balloon valvotomy. Therefore, 27 different transvalvar gradients were assessed by Doppler and these data were compared with the catheter-derived maximal instantaneous, peak-to-peak, and mean pressure gradients. The maximal Doppler gradient correlated well with the catheter-derived peak-to-peak pressure gradient (r = 0.95) and catheter maximal instantaneous pressure gradient (r = 0.95). Although these correlation coefficients were similar, the Doppler maximal gradient consistently overestimated the peak-to-peak catheter gradient by as much as 25%-40%. Such an overestimation was not observed when we compared the maximal Doppler gradient with the catheter-derived maximal instantaneous gradient. Moreover, the regression line of the latter comparison closely approximated the line of identity. The correlation coefficient between Doppler mean and catheter mean gradients was only 0.91. Doppler velocities were best derived when multiple transducer positions were employed to interrogate pulmonary artery velocity.

Blood Pressure↗