Hypoplastic left heart syndrome with an anomalous origin of the left coronary artery.

Abnormal origin of the coronary artery in children with hypoplastic left heart syndrome is an extremely rare defect. We describe a newborn with concomitant hypoplastic left heart syndrome and abnormal origin of the left coronary artery arising from the right pulmonary artery. A Norwood procedure and direct reimplantation of the left coronary artery to the ascending aorta was performed.

[Results of surgical treatment of congenital heart defects in infants below 2500 grams]. / Wyniki operacyjnego leczenia wrodzonych wad serca u dzieci z masa ciala ponizej 2500 g.

We present a review of our recent experience of operating on infants below 2500 g suffering from congenital heart disease. A retrospective review was performed in 73 children who had undergone cardiac operations at our institution from 1990 to 1999. There were 43 (59%) females and 30 (41%) males; the mean age at operation was 28 days (range 4 to 92). The mean gestational age was 34.7 weeks (range 24 to 41), mean birth weight--1899 g (range 700 to 2450) and mean weight at operation--2013 g (range 640 to 2500). Cardiac diagnoses included patent ductus arteriosus (PDA) (N = 21, 28.7%), ventricular septal defect (VSD) (N = 9, 12.3%), transposition of great arteries (TGA) (N = 10, 13.7%), tetralogy of Fallot (TOF) (N = 7, 9.6%), double-outlet right ventricle (DORV) (N = 4, 5.5%), truncus arteriosus communis (TAC) (N = 4, 5.5%), hypoplastic left heart syndrome (HLHS) (N = 4, 5.5%), coarctation of aorta (CoAo) (N = 6, 8.2%), total anomalous pulmonary venous return (TAPVR) (N = 2, 2.7%), aortic stenosis (AoVS) (N = 3, 4.1%), interrupted aortic arch (IAA) (N = 1, 1.4%), pulmonary atresia (PA) (N = 1, 1.4%) and common atrioventricular canal (CAVC) (N = 1, 1.4%). Hospital mortality was 16.4%. There were 6 early deaths (8.2%) and 6 late deaths (8.2%). A higher mortality rate was noted in premature children and in children who had undergone palliative procedures. At a 1-116-month follow-up (mean 39 months), of 57 (93.4%) patients, 46 (80.7%) were in NYHA class I and 11 (19.3%) in NYHA class II. In conclusion the authors believe that early intervention can be performed in infants below 2500 g, and the associated mortality and morbidity rates are low.

[Operative outcome in children with total anomalous pulmonary venous connection]. / Wyniki operacyjnego leczenia dzieci z calkowitym nieprawidlowym polaczeniem zyl plucnych.

The authors present their experience in surgical treatment of children with total anomalous pulmonary venous connection. A retrospective analysis was carried out in 42 patients operated on in the years 1979-1999. The group included 30 boys aged 7 days to 7 years (mean 170 +/- 480 days) and 12 girls aged 14 days to 7 months (mean 86 +/- 80 days). Diagnostic studies allowed to determine that 24 patients (57%) had supracardiac type (type I), nine (21%)--cardiac (type II), five (12%)--infracardiac (type III) and four (10%)--mixed (type IV). The diagnosis was based on clinical examinations and echocardiography, supplemented in 17 patients (40%) by hemodynamic studies and angiocardiography. Preoperative pulmonary hypertension was noted in 34 children (81%), including 15 patients (36%) diagnosed by hemodynamic studies and 19 children (45%) by echocardiography. All children were operated on in deep hypothermia with circulatory arrest. In the early postoperative period (30 days), seven children (17%) died, while four more (9.5%) died in the latter period. The former were characterized by significantly lower birth weight values (p = 0.023). The total mortality rate was higher in children with type II and III anomalies. No statistically significant association was found between preoperative pulmonary hypertension and early mortality. Thirty-one children (74%) remain in late follow-up. All of them are in NYHA (New York Heart Association) class I and develop normally. Total anomalous pulmonary venous connection is a rare congenital anomaly that can be fully repaired with improving early and late results.

Primary reconstruction of interrupted aortic arch--surgical management and results.

Type B interrupted aortic arch associated with other extracardiac and intracardiac congenital lesions is a lethal defect. Surgical management with a one- or two-stage approach is complex, difficult and associated with high morbidity and mortality. Between January 1996 and December 1999, 5 consecutive patients with type B interrupted aortic arch were operated on. The group included 3 girls and 2 boys weighing from 2.4 kg to 3.5 kg (mean 2.84 kg) and aged from 4 to 35 days (mean 15 days). All patients underwent a primary reconstruction of the aortic arch and one-stage repair of the associated heart defects. Two patients developed symptoms of mild aortic arch narrowing 7-12 months postoperatively. Balloon angioplasty of the obstructed site was successfully carried out in one of the patients. In the second child, angioplasty was not successful and surgery was used. All the patients survived and are being followed-up. This experience supports a strategy of primary one-stage repair of IAA type B, including direct anastomosis for aortic arch reconstruction, and all associated heart anomalies in the neonatal period.

Effects of cyclosporin A on contractile activity and cytoskeleton in chick embryo cardiomyocytes.

The effects of cyclosporin A (CsA), a clinically used immunosupressive drug, on contractile activity of chick cardiomyocytes grown as small aggregates or explants suspended on a network of elastic glass fibres or cultured in a monolayer were analysed in vitro with computer-aided image cytometry methods. At therapeutic concentrations (200-1500 ng/mL), CsA induced changes in the frequency and amplitude of the beating activity of cardiomyocytes 15 min after application. Longer treatment of cardiomyocytes, for 20-24 h, additionally induced changes in their shape and cytoskeleton organization (F-actin and alpha-actinin distribution). These results indicate that CsA is able to affect directly the contractile activity, morphology, and cytoskeleton architecture of heart cells.