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The neuro-cutaneous syndrome tuberous sclerosis is commonly associated with rhabdomyomas in various organs including the heart. We are reporting a rare case of a 7-month old male child with congenitally corrected transposition of the great arteries associated with tuberous sclerosis and cardiac rhabdomyomas. To our knowledge, this rare association has not been reported so far.

Objective: We report the mid-term follow-up of patients, who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA) with intact ventricular septum and left-ventricular outflow tract obstruction (LVOTO) over a 10-year period from 2000 to 2009., Methods: Thirteen TGA patients (3.9% of our ASO cohort) with intact ventricular septum and LVOTO underwent ASO. LVOTO was defined as pulmonary valve z-score ≤ -2.0 (n=3) or peak LVOT gradient ≥40 mmHg with (n=7) or without (n=3) anatomic subvalvar stenosis on echocardiography. Median age and weight were 14 days (range, 7-130 days) and 3.2 kg (range, 2.1-4.6 kg). The LVOT abnormalities included fibromuscular narrowing (n=5) and atrioventricular valve-related findings (n=5). LVOT clearance was achieved by resection of accessory mitral tissue (n=2) only., Results: Follow-up was 100% complete. There were no early or late deaths. Freedom from re-operation for neo-aortic valve regurgitation and/or LVOTO was 100% at a median follow-up of 38 months (range, 6-115 months). All patients had functional status appropriate for their age. Three patients had mild aortic regurgitation. The median Doppler estimated LVOT systolic gradient was 12 mmHg (range, 0-18 mmHg) for the entire cohort at the latest follow-up., Conclusions: Mid-term outcomes of ASO for a highly selected group of patients with pulmonary valve annulus z-score ≤ -2.0 ≥ -0.4, resectable organic LVOTO, and dynamic peak LVOT gradient ≥40 mmHg remain satisfactory, with a need for long-term follow-up., (Copyright © 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Objectives: One-stage repair of transposition of great arteries (TGA) and aortic arch obstruction (AAO) is currently advocated, but carries formidable surgical challenges. This report presents our experience and re-interventions for residual lesions over the last 10 years., Methods: Twenty-two patients (19.5 ± 42.4 days; range 2-206; median 10 days, 3.5 ± 0.6 kg) diagnosed with TGA (nine patients) or double outlet right ventricle (DORV) (13 patients) and AAO underwent one-stage repair. Of the nine TGA patients (two with intact ventricular septum), AAO were: two patients hypoplastic arch, one patient discrete coarctation, four patients hypoplastic arch with coarctation and two patients interrupted aortic arch. The 13 DORV patients were all of Taussig-Bing type and one showed multiple ventricular septal defects (VSDs). The degree of AAO ranged from hypoplastic arch in five patients, coarctation two patients, combined four patients and interrupted aortic arch (IAA) two patients. Arterial switch with Lecomte ± VSD repair was performed during cooling, and aortic arch repair was performed under deep hypothermic circulatory arrest (DHCA) (35 ± 14 min at 16.9 ± 0.7 °C). Our preference was to use homograft patch-plasty for arch and direct end-to-side anastomosis for coarctation repair. Aortic-cross-clamp time was 124 ± 24 min and cardiopulmonary bypass (CPB) time 215 ± 84 min., Results: Early survival was 19/22 (86%) up to 30 days without mortality in the second half of our series. Three patients required extracorporeal membrane oxygenation (ECMO) support and renal support was needed in three and preferred permanent pace maker (PPM) implantation in two. Length of stay was 21.9 ± 22.1 days. There was one late death and overall survival was 18/22 (82%) for the follow-up period of 4.8 years (0.2-9.8 years). Eight patients (44%) required re-intervention for re-coarctation. Four patients required right ventricular outflow tract (RVOT)/pulmonary artery re-interventions. At follow-up, there was no requirement for aortic valve replacement, residual VSD closure and no evidence of ventricular dysfunction., Conclusions: One-stage repair of TGA/DORV and AAO can be performed safely with a good survival rate. Three important lessons that we have learnt are as follows: (1) the subpulmonary VSD may have a perimembraneous component, (2) late re-coarctation is not infrequent and (3) late residual right-sided cardiac lesions remain an issue in complex TGA repair., (Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)