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Query: UMLS:C0020672 (hypothermia)
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Infant orthotopic cardiac transplantation has been recently applied to various forms of congenital heart disease with encouraging short-term results. Between June 1986 and September 1987 we evaluated 16 infants for orthotopic cardiac transplantation. Fourteen had hypoplastic left heart syndrome, one had endocardial fibroelastosis with aortic atresia, and one had anomalous pulmonary arterial origin of the left main coronary. Eight families accepted the treatment program and eight families refused (two because of associated anomalies and six on philosophical grounds). Of the eight patients who were candidates for orthotopic cardiac transplantation, one died 6 hours after diagnosis, one was allowed to die after 60 days because of acquired neurologic complications, and another had congenital cytomegalic virus infection. The remaining five patients (four with hypoplastic left heart syndrome, one with anomalous pulmonary arterial origin of the left main coronary) had orthotopic cardiac transplantation. The operation was performed with absorbable polydioxanone suture with deep hypothermia and circulatory arrest in four neonates for hypoplastic left heart syndrome (average time 47 minutes) and bicaval cannulation and continuous bypass in one 11-month-old infant for anomalous origin of the left main coronary. In-house retrieval was used in all. One neonate died of complications as a result of pretransplant donor heart dysfunction and size discrepancy, whereas the remaining three neonates and one infant survived and are home 23 months, 12 months, and 8 months (the patients with hypoplastic left heart syndrome) and 17 months (the patient with anomalous origin of the left main coronary) postoperatively. Triple-drug immunosuppression included cyclosporine, azathioprine, and prednisone. Rejection was diagnosed by clinical evaluation of child activity and monocyte cell cycle analysis from peripheral blood samples without myocardial biopsies. Routine echocardiograms, electrocardiograms, and chest x-ray films were not helpful. Six episodes of rejection were successfully treated in four patients. Twelve-month postoperative catherization in one patient (hypoplastic left heart syndrome) showed appropriate graft growth, no aortic or pulmonary anastomotic strictures, normal right and left ventricular function, and no coronary artery disease. We conclude that infant orthotopic cardiac transplantation is an acceptable procedure for severe forms of untreatable congenital heart disease. The excellent short-term results warrant continued application of orthotopic cardiac transplantation.
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PMID:Infant orthotopic cardiac transplantation. 247 96

Open valvotomy was successfully performed in neonate with critical aortic stenosis using cardiopulmonary bypass. The baby was referred to our hospital at the age of 24 days with very grave state, and needed intensive care including endotracheal intubation and inotropic support. Critical valvular aortic stenosis was confirmed by echocardiography. Poststenotic dilatation and enough size of short axis LV dimension were reported, and aortic annulus was measured 6 mm in diameter. Without catheterization and angiography, open valvotomy was performed with moderate hypothermia and ischemic arrest using single dose of cold cardioplegia at the age of 29 days. Bicuspid aortic valve was thick and dysplastic with thick gelatinous cusp edge, however commissurotomy was applicable in two direction. The diameter of aortic opening was enlarged from 2 mm to 7 mm. Total bypass and aortic cross clamp time were 78 and 28 minutes respectively. The baby recovered uneventfully and there was no evidence of significant AS or aortic regurgitation in echocardiography 7 months after surgery. Sorts of reoperation for restenosis or regurgitation were reported. The results of reoperation for regurgitation were reported to be poor, especially in young infants who should be performed aortic valve replacement. However, residual AS could be manipulated with re-valvotomy, PVB, apico-aortic conduit or AVR. As the choice of first relief of critical AS without other anatomical disadvantages including hypoplastic left ventricle, endocardial fibroelastosis, and mitral stenosis, it would be crucial for late results to prevent progression of aortic regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case report of successful open valvotomy in neonate with critical aortic stenosis]. 830 70

Successful open aortic valvotomy and end-to-end anastomosis were performed to the patient with critical aortic stenosis and CoA on the first day of life. A fetus was diagnosed as aortic stenosis and coarctation by fetal echocardiography at 29 weeks of gestation. The mother was transferred to our hospital at the onset of labor and delivered vaginally at 40 weeks of gestation. Soon after the birth, the newborn, birth weight 2630 gram, female, underwent echocardiography by pediatric cardiologists which demonstrated the aortic orifice of 5.1 mm in diameter and thickened cusps with poor mobility. Transaortic pressure gradient measured by Doppler echocardiography was 111 mmHg and the left ventricular wall motion was hyperdynamic without any signs of endocardial fibroelastosis. Prostaglandin E1 administration was started to maintain systemic circulation and the hemodynamic status has been stable before surgery. On her first day of life, the operation was performed using cardiopulmonary bypass with moderate hypothermia. Isolated cerebral and myocardial perfusion technique was applied during the repair of coarctation of the aorta. The open aortic valvotomy with resection of myoxomatous nodules on leaflet edges effectively released pressure gradient across the aortic valve without regurgitation. Post-operative course was uneventful and she discharged at 28th day after surgery. We conclude early diagnosis including fetal echocardiography and early repair would lead the better surgical outcome to the neonates with critical aortic stenosis.
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PMID:[Successful repair of critical aortic stenosis with coarctation on the first day of life]. 975 Apr 48