Anomalous origin of the left coronary artery from the pulmonary artery, scimitar syndrome, and aortic coarctation.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) represents one of the most common causes of myocardial ischemia in infants and if left untreated results in a high mortality rate. When ALCAPA coexists with other congenital malformations, particularly those associated with pulmonary hypertension, the initial presentation can be quite confusing and is often misinterpreted. We report an infant with ALCAPA associated with scimitar syndrome and aortic coarctation whose clinical course illustrates the complexities and difficulties of management with a successful outcome.

[Surgery for coarctation of the aorta in infants younger than three months]. / Hirursko lecenje koarktacije aorte kod odojceta uzrasta do tri meseca.

INTRODUCTION: Recurrent coarctation is a complication which is seen at a consistent rate following repair for coarctation of the aorta in young infants. OBJECTIVE: This retrospective analysis was carried out to compare the results between resection with end-to-end anastomosis (ETE), and resection with extended end-to-end anastomosis (E-ETE), in this age group during late follow-up period. The role of ductus arteriosus is not clearly defined and the second objective of this study was to analyze intimal thickening in aortic coarctation. MATERIAL AND METHODS: From 1999 to 2003, 45 patients less than 3 months of age underwent repair of aortic coarctation. Mean age was 24 days (2-89 days), average weight was 3.5 +/- 0.6 kg (2.4-5.2 kg). The method of repair was ETE in 14 (31.1%) patients, E-ETE in 29 (64.4%) patients and other techniques were applied in 2 cases. Demographic, morphometric, clinical and operative variables were analyzed for correlation with recurrent arch obstruction. In order to characterize the components of intimal thickening in coarctation, narrowed segments of aorta resected from 16 neonates during surgery were examined immunocytochemically and by electron microscopy. For light microscopy, the specimens were dehydrated in graded ethanol (70-100%), cleared in xylol and embedded in paraffin. Immunocytochemical staining was performed in 5 microm sections from formaldehyde-fixed paraffin-embedded blocks, using a labeled streptavidin-biotin method with an LSAB kit (Dako). RESULTS: Early mortality was 6.7% (CI 95%, 2.9%-10.4%). All early deaths (3 patients) occurred in infants with associated ventricular septal defects (p<0.05). The mean follow-up for all patients was 30 +/- 21 months (range 1.5-63 months). During mean follow-up of 2 months, recurrent arch obstruction was diagnosed in 9 patients (21.4%). Two patients with associated complex heart defects died before re-intervention, one had mild gradient on catheterization (20 mm Hg) and one is waiting for catheterization. Five patients were reoperated and the mean time to re-intervention was 4 months (range 2.6-6 months). Kaplan-Meier freedom from recoarctation was 78.1 +/- 6.4% at 5 years in the whole group. Freedom from recoarctation was 60.6 +/- 15.4% at 25 months in ETE group and 86.2 +/- 6.4% at 60 months in E-ETE group (p=0.062). Factors associated with recoarctation, obtained by univariable Cox regression, included abnormal right subclavian artery (p=0.003), hypoplastic proximal transverse aortic arch (Z < or = -2, p=0.025) and weight at operation < or = 3 kg (p=0.02). Abnormal origin of the right subclavian artery was the only independent predictor of recoarctation obtained by multivariable Cox regression analysis. DISCUSSION: All examined specimens had intimal thickening of the posterior aortic wall, with accumulation of smooth muscle cells (SMC) with alpha smooth muscle actin (alpha-SMA) and vimentin-immunoreactivity (but not desmin and MHC) and also expressed PCNA and S-100. In the inner media of the anteromedial wall of the aorta, all specimens had large number of SMC expressing desmin and MHC. SMC in the inner media exhibit contractile phenotype and their origin could be ductal. CONCLUSION: Both procedures are effective for coarctation repair in young infants. Risk of recoarctation is a function of the complex anatomy of the arch, while residual ductal tissue may play a significant role.

[Results of treatment of cyanotic heart defects in children]. / Rezultati lecenja cijanogenih mana srca kod dece.

Cyanotic heart diseases are relatively rare, but they are severe and heterogeneous congenital heart diseases, which require complex surgery. Development of different advanced surgical procedures, such as arterial switch operation (ASO), Fontan and its modifications, Norwood etc. operations, as well as better perioperative care significantly improved survival rate and quality of life of these children. The study group included 308 children treated for cyanotic heart disease in Yugoslavia, in the period January 2000 to July 2004. Some of them (239, 77.6%) were treated at the University Children's Hospital in Belgrade, and others (69, 22.4%) in different institutions abroad. The age of the operated patients varied between 1 day and 19 years (median 12 months). The patients (pts) were divided into four groups, according to the disease and type of the operation. In the whole group of 308 patients treated due to cyanotic heart disease, there were 232 (75.3%) cases with open heart surgery and 76 (24.7%) with closed procedures. The mortality rate was significantly different between disease/operation groups, and age groups. Average mortality rates differed from 11.8% for palliative procedures to 12.5% for complete corrections. Mortality rate and achieved surgical results in treatment of children with cyanotic heart diseases were significantly worse than those published by leading cardiac surgery centers in the world. However, there is a clear tendency in introducing new surgical procedures, lowering the age at which the operation is done and decreasing the mortality rates.