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We report a rare case of a successful cardiac transplantation in a patient suffering from cardiomyopathy and complex mitochondrial disease. The patient presented with severe heart failure and malignant ventricular arrhythmias requiring implantation of a defibrillator and advanced medical treatment. The patient was listed for urgent heart transplantation and received a donor heart after 36 days. One yr post-operatively, the patient has completely recovered.

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INTRODUCTION: Heart transplantation (HTx) has increasingly become a therapeutic option for end-stage heart failure of any origin in children. Short- and mid-term results are promising. However, long-term outcome has been a matter of concern because of acute or chronic rejection and side effects of immunosuppression. We performed a retrospective study of up to 15-years of follow-up on this patient entity. METHODS: Between 1988 and 2004, 58 HTx were performed in 55 children (cardiomyopathy (DCM) 32, congenital heart disease (CHD) 23, Re-HTx 3). Mean age was 9.1 +/- 7.2 years (4 days - 17.9 years). Twenty-nine patients had a total of 51 previous operations. RESULTS: Operative mortality was 4/58 (6.8 %) due to primary graft failure. Late mortality was 7/54 (12.1 %) due to acute rejection (2), pneumonia (2), intracranial hemorrhage (1), suicide (1) and lymphoma (1). Mean follow-up was 5.2 +/- 4.2 years. One-, 5-, and 10-year survival was 86 %, 80 % and 80 %, respectively, and improved significantly after 1995 (92 % and 92 %; p = 0.04). Survival was comparable for DCM and CHD patients (1-year: 88 % vs. 82 %; p = 0.19; 5-years: 84 % vs. 77 %; p = 0.12). Three patients with therapy resistant rejection and assisted circulation required retransplantation and are alive. Freedom from acute rejection was 46 % with primary cyclosporine immunosuppression and 63 % with tacrolimus. Ninety-eight percent of the survivors are at home and in excellent cardiac condition. CONCLUSION: Pediatric heart transplantation is a curative treatment for DCM and CHD with excellent clinical mid-term results. However, further follow-up is necessary to evaluate long-term side effects of immunosuppressants. Donor shortage remains a problem.

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Evaluating complex cardiac defects in small children preoperatively requires multiple diagnostic procedures including echocardiography, but also the invasive methods such as cardiac catheterisation, computer-tomography and magnetic resonance imaging. We tried to assess the complex anatomy of the atrioventricular valves in atrioventricular septal defect using bedside real-time three-dimensional echocardiography and comparing these results to the anatomic findings at the time of operation.

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BACKGROUND: Patients with hypoplastic left heart syndrome (HLHS) and associated malformations undergo Norwood palliation or potentially a two-ventricle repair. METHODS: Since 8/99, 8 patients with typical HLHS and two with DILV underwent Norwood/Fontan palliation (group I). Three other patients (group II) had two-ventricle repair. Anatomy was: aortic atresia, coarctation, unrestrictive VSD (patient 1), hypoplastic mitral and aortic valve, arch and LV, coarctation (patients 2 and 3). Surgical procedures were Norwood arch reconstruction with either Rastelli operation (patient 1) or ASD-closure (patients 2 and 3). RESULTS: Operative mortality in group I was 1/8 (day 22; RV-failure). Two patients died before Glenn (sepsis, RV-failure). Six patients underwent Glenn procedure successfully. No patient died in group II. Echocardiography after 13 +/- 7.4 months showed mild homograft dysfunction (patient 1) and an LVOT-gradient of 20 mmHg (patient 3). Clinical condition of all survivors in both groups is good. CONCLUSION: Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two-ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.

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Three-dimensional echocardiography is increasingly available. We report our experience with 3D reconstructions of ventricular septal defects: Unique images of all different types of ventricular septal defects are reconstructed from left and right ventricular en-face views. The images are discussed according to pathological classifications. The images shown in this article include all landmarks used in pathological classifications of ventricular septal defects to distinguish the different types of the defects and their extension directions. The possibility of visualizing the whole circumference prevents the examiner from under- or overestimating the size of a defect and gives the interventionalist a useful tool for patient and device selection for interventional VSD closure.