Successful repair of double-outlet right ventricle with bilateral conus, 1-transposition of great arteries (S,D,L), and subpulmonary ventricular septal defect.

Surgical correction was carried out successfully in a severely cyanotic 3-year-old Japanese girl who had a very rare type of double-outlet right ventricle. The malformation was associated with bilateral conus, 1-transposition of the great arteries, and subpulmonary ventricular septal defect without significant pulmonary stenosis in situs solitus. A large amount of subaortic conal musculature which separated the aortic valve from the subpulmonary ventricular septal defect was removed, as was the anterior rim of the ventricular septal defect. A tunnel, constructed with a woven Teflon prosthesis, was inserted in such a manner as to direct blood from the left ventricle through the defect and out to the aorta. The pulmonary outflow tract was reconstructed with a Teflon patch lined with pericardium. The patient's postoperative recovery was uneventful, and she was doing well 3 months postoperatively. To our knowledge, no identical case with a similar type of surgical correction has previously been reported.

Lung pathology in infants with severe pulmonary hypertension and cardiac disease.

Lung biopsy specimens were taken from 39 infants younger than 12 months of age with congenital heart disease and severe pulmonary hypertension (Pp/Ps greater than or equal to 0.75) accompanied by respiratory distress. The pathological change in lung specimens and clinical courses were compared. These 39 infants underwent surgical treatment of patent ductus arteriosus (PDA), seven patients; ventricular septal defect (VSD), 13 patients; and complex heart anomaly, 19 patients. The common pathological findings of the lung specimens taken from these infants were lymphoid cellular infiltration and thickening of the alveolar septum, which we have called "septitis" in the present study. In most cases pulmonary vascular obstructive change was within Grade 2 of the Health-Edwards criteria. Septitis was classified into three categories: mild, moderate, and severe. Only three of the 19 infants with severe septitis survived postoperatively, whereas 10 of the 12 infants with moderate septitis and all eight with mild septitis could be successfully weaned. The cause of septitis remains unidentified. We have found the patient's age and pulmonary hypertension to be closely related to the grade of septitis in this study. Septitis plays a much more important role than pulmonary vascular obstructive change in the prognosis of pulmonary hypertensive heart disease in early infancy.

Characterization and potential application of purified aldehyde oxidase from Pseudomonas stutzeri IFO12695.

The molecular weight of purified aldehyde oxidase from Pseudomonas stutzeri IFO12695 was estimated to be 160 kDa by a gel filtration method. SDS-PAGE showed that the enzyme consisted of three non-identical subunits with molecular weights of 18, 38, and 83 kDa. The enzyme exhibited an absorption spectrum with maxima at 277, 325, 365, 415, 450, 480, and 550 nm and possessed molybdenum, CMP, iron, sulfur, and FAD as its cofactors, indicating that it belonged to the xanthine oxidase family. A variety of aliphatic and aromatic aldehydes were oxidized; and among them n-hexylaldehyde gave the most rapidly action. When 10 mM formaldehyde was treated with the aldehyde oxidase in the presence of catalase for 240 min, the formaldehyde concentration was reduced to 0.8 mM, suggesting this enzyme might be effective for the removal of formaldehyde contained in wastewater.

Pulmonary changes in congenital heart disease of childhood: histometrical and histological studies.

One hundred and fifty lungs from the cases below 15 years of age with various congenital heart diseases and 80 controls were used for histometrical and histological studies. Cases with congenital heart disease were divided into two groups of the increased and the decreased pulmonary blood flow. In the former group, the thickness of the pulmonary arterial media was the same as that of controls in the neonatal period, and through the wall thickness gradually decreased in a pattern seen in controls, the thickness was constantly larger than that of controls. In some cases, the media increased gradually within 6 months after birth. Pneumonia and massive pulmonary hemorrhage were seen in a higher incidence in autopsy cases. Pneumonia in younger infants was histologically characteristic and possibly more correlated to their death. In the latter group, most of the cases were with the thinner medias of the pulmonary arteries. Massive pulmonary hemorrhage was not common in the latter group.

Surgical treatment of idiopathic hypertrophic subaortic stenosis in children.

Five children with idiopathic hypertrophic subaortic stenosis (IHSS) were operated upon during the past 7 years and all survived. Their ages ranged from 4 years and 5 months to 14 years (mean 8 years and 5 months). The operations performed were 1 right ventricular myectomy and 4 transaortic left ventricular myotomies. Whether surgery performed in childhood would change the natural history or not is still unknown, however, according to our results and many available reports, we have so far reached the following conclusions; 1. Transaortic left ventrlcular myotomy or myectomy is a safe and effective operation for localized muscular subaortic stenosis in children. 2. Because the disease is progressive, operation in childhood may be worth to be considered, when the resting or provocative pressure gradient is excessive. 3. For those who have a marked increase in left ventricular outflow pressure gradient by the provocation, operation would be considered even though the gradient at rest is less than 50 mmHg.

The effect of phenoxybenzamine on the pulmonary vascular bed in surgically corrected ventricular septal defect associated with pulmonary hypertension.

From 1971 to 1977, a total of 43 cases with ventricular septal defect associated with pulmonary hypertension (VSD + PH), ranging from 3 months to 6 years of age, underwent VSD closure using cardiopulmonary bypass with a prophylactic dose of phenoxybenzamine (POB) 1 mg/Kg. Twenty of these cases are included in the present study of the effect of POB on pulmonary hypertension. The ratios of pulmonary to systemic arterial pressures (Pp/Ps) and vascular resistances (Rp/Rs) were measured before, immediately after and 1 month after VSD closure. In all cases, the Pp/Ps before VSD closure was larger than 0.75. Both Pp/Ps and Rp/Rs markedly decreased immediately after VSD closure but rose again 1 month thereafter. These results suggest the possibility of pulmonary vasodilating effect of POB, which could be potentially useful in relieving the right ventricular load in the early postoperative period. The over all mortality was 3 out of 43 cases including 2 late deaths.