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Transposition of the great arteries occurs rarely in patients with mirror image dextrocardia and situs inversus, while the combination with severe coarctation of the descending aorta (CoA) makes the anatomy even more unusual. Therefore, it is not surprising that a case with such unusual and complicated anatomy presents unique problems when a primary definitive correction is attempted. We report a patient with situs inversus totalis and complex congenital heart disease including transposition of the great arteries and severe CoA who underwent successful complete, single-stage, anatomic correction.

A 63-year-old man bearing most signs and symptoms (facial pigmentation, degenerative arthritis, and dark urine) pertinent to his known history of alkaptonuria underwent aortic valve replacement for critical aortic stenosis. Although rare, aortic stenosis is the most common cardiac manifestation of alkaptonuric ochronosis.

Junctional ectopic tachycardia is usually a limited but potentially life-threatening postoperative arrhythmia. We describe a case of malignant postoperative junctional ectopic tachycardia in a 13-month-old child who had undergone transatrial and transpulmonary repair of tetralogy of Fallot resistant to all conventional therapeutic measures and complicated by recurrent ventricular tachycardia. The arrhythmia was finally treated by open surgical cryoablation of the atrioventricular node and the implantation of a permanent pacemaker. The patient made an uneventful recovery followed by good long-term outcome.

Objective: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation., Methods: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded., Results: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis., Conclusions: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

Objectives: Postcardiotomy acute severe heart failure cannot be managed by medical treatment alone and most often requires some form of mechanical support. In this study we evaluate the efficacy of postoperative extracorporeal membrane oxygenation (ECMO) support following surgery for congenital heart disease (CHD) in infants and children., Methods: Over a 6-year period from October 1997 to October 2003, 10 patients aged 5 days to 28.5 months (median 3 months) who underwent surgical procedures for CHD received postoperative mechanical support for failing cardiac function despite optimal medical therapy. In 3 patients ECMO was instituted in the operating room (OR) and in 7 patients this was introduced in the intensive care unit (ICU) 2 to 48 (median 20) hours postoperatively., Results: Four patients (40%) were successfully weaned, while support was withdrawn in the remaining 6 due to irreversible vital organ damage. Following successful weaning, one of the survivors died 8 hours later from barotrauma and intrapulmonary hemorrhage, and another died 4 months later from persistent heart failure. The other two patients remain well in NYHA class II., Conclusions: Despite the adverse effects of ECMO, the methodology provided the necessary support and allowed the failing heart to recover in a number of patients where inotropic support alone proved inadequate.