Coronary sinus rhythm in the polysplenia syndrome.
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An initial series of 180 patients subjected to definitive repair of cardiac malformations during infancy is presented. The operations were performed with profound hypothermia and circulatory stasis. Statistics relating to the entire series of patients are presented and discussed, after which the results among eleven individual groups of patients categorized by type of cardiovascular lesion are analyzed. There was 113 survivors, representing an over-all mortality rate of 37 per cent. The mortality rate was highest among patients severely ill preoperatively and among those with complex malformations. Deaths were also more frequent among infants under 6 months of age and less than 5 kilograms in weight. Analysis shows a steady increase in percentage of patient survival over the years reported from December, 1967, to April, 1975.
The patient was a one-year-old boy, who underwent surgery with a diagnosis of atrial septal defect (ASD). During operation, aorto-pulmonary window (A-P window) which had not been detected by the preoperative examinations, was found. Therefore, the A-P window was divided prior to closing ASD. The patient is in good condition six months after the operation. The causes of the inaccurate preoperative diagnosis were discussed.
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A case of Ebstein's anomaly and partial atrioventricular canal associated with double orifice mitral valve is reported. Patch closure of the primum defect, plication of the atrialized ventricle, and tricuspid valve replacement with a St. Jude Medical valve were carried out successfully, and the patient is doing well 2 years and 9 months after the operation. To our knowledge, this combination of congenital heart defects has not been reported in the literature.
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The authors report a case of type A interruption of the aortic arch with the subclavian arteries arising from the descending aorta which was of special interest because of the absence of a patent ductus arteriosus. Therefore, the systemic circulation was assured from the carotid arteries which perfused the vertebral arteries retrogradely via the circle of Willis. The association of a partial endocardial cushion defect and hypoplasia of the ventricle explained the surgical failure. A review of the literature of interruption of the aortic arch without a ductus confirmed that this was a rare condition and, paradoxically, well tolerated in the absence of associated malformations. On the other hand, the left ventricular lesions, usually present in the classical forms with patent ductus arteriosus, operated in the neonatal period, increase the operative risk.
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Partial Atrioventricular Canal Defect ( PAVC ) The operative results of this anomaly is generally acceptable. Between 1978 and 1982, 32 patients with PAVC underwent intracardiac repair. Two operative deaths occurred in 41 and 42 years old patients who were in NYHA class 4 preoperatively. Postoperative mitral insufficiency was either nil or mild except in one patient with mitral valve prolapse which was left unrepaired. Complete Atrioventricular Canal Defect ( CAVC ) Our operative procedures for the repair of CAVC are as follows, 1) all of accessory chordae are cut off and the bridging leaflet is incised into mitral and tricuspid components. 2) Ventricular septum, atrioventricular valve and atrial septum are constructed with our designed endocardial cushion prosthesis (Dacron prosthesis with pericardial brim of 3-4 mm width in each side). Between 1978 and 1982, 20 patients with CAVC containing 16 infants less than 2 years old underwent intracardiac repair using this prosthesis. Thirteen patients had no severe associated anomalies and only one death occurred in this group. Three patients were combined with tetralogy of Fallot (one died) and two patients with double outlet right ventricle (one died). The other two patients had either coarctation of the aorta (died) or interruption of the aortic arch (survived).
In order to obtain better operative results with less postoperative atrioventricular valve regurgitation, we have developed a new method of repair for the complete atrioventricular canal with a composite prosthetic patch called an endocardial cushion prosthesis, in which horizontal wings are attached to a vertical patch on both sides to compensate for the defective atrioventricular valve tissue. Ten patients ranging in age from 8 months to 8 years (mean 28.9 months) have been operated on with this technique since 1975. All patients except one showed severe preoperative pulmonary hypertension (over 50 mm Hg) in mean pulmonary artery pressure. Six patients had trivial-to-moderate mitral regurgitation before surgery. Nine patients survived the operation (operative mortality 10%). Postoperative mitral regurgitation was found in three of seven patients who underwent cardiac catheterization and angiocardiographic examination at an average of 4.4 months after surgery. The grades of mitral regurgitation were trivial in all. Mild-to-moderate tricuspid regurgitation was observed in five patients and there was a correlation between the grades of tricuspid regurgitation and pulmonary/systemic peak pressure ratio (r = .757, p less than .05). This method appears to be advantageous in preventing postoperative mitral regurgitation with low operative mortality. However, tricuspid regurgitation was not well avoided among the patients with persisting pulmonary hypertension.
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