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Congenital mitral valve (MV) dysplasia is a relatively rare and highly complex cardiac disease. We sought to provide a comprehensive analysis of the current surgical techniques for treating mitral valve insufficiency and the results of mitral valve repair at our institution. Between 1972 and 2017, 104 consecutive patients underwent surgical repair of congenital MV dysplasia-insufficiency at our institution. Among these, 59 patients presented with MV insufficiency (or prevalent MV insufficiency) and were part of the study. There was 1 early (1.7%) and 1 late death (1.7%). Survival at 5, 10 and 20 years was 98%, 98% and 94%, respectively. Eight patients (14%) required MV replacement for residual significant MV insufficiency. Freedom from re-intervention for MV dysfunction at 5, 10 and 20 years were 89%, 84% and 79%, respectively. Surgical techniques for treating mitral valve insufficiency must be tailored for each patient with the main goal of achieving a proper valve function, rather than a "normal" anatomy. The mechanism underlying valve dysfunction in congenital mitral valve insufficiency is multifactorial and requires the application of a variety of surgical techniques in each case., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Surgery for congenital valve anomalies in children is a challenging topic. We aim to assess early and late functional outcomes of CorMatrix scaffold after repair of aortic and pulmonary valves (PV) in congenital heart disease in a prospective nonrandomized clinical study on children with congenital aortic (Group 1) or PV (Group 2) disease. Primary endpoints were reoperation or reintervention on semilunar valves and echocardiographic evidence of regurgitation or stenosis greater than mild. Results of PV repair in tetralogy of Fallot were compared with a control group of patients who underwent PV repair with polytetrafluoroethylene. A total of 22 consecutive selected patients with complex congenital heart disease were included: PV repair in 18 and aortic valve repair in 4. At discharge bidimensional echocardiography, semilunar valve regurgitation was mild in 50% of all patients. At a median follow-up of 23 months (4.3-51.3), reoperation for aortic valve replacement was necessary in 2; right ventricular outflow tract obstruction occurred in 3, requiring interventional treatment in 2. Pulmonary regurgitation degree worsened in most patients of Group 2 at follow up. When comparing patients with tetralogy of Fallot in Group 2 with patients who underwent PV repair with polytetrafluoroethylene, there were no significant differences in PV function at follow up. We conclude that CorMatrix scaffold for semilunar valve reconstruction does not present with significant advantages to traditional materials at mid term follow up. In addition, when used for pulmonary valve reconstruction, CorMatrix seems to show gradual functional deterioration in the mid term., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Routine early transatrial-transpulmonary repair of tetralogy of Fallot (TOF) has been performed at our institution since 1990. A transatrial-transpulmonary approach implies a minimal or no ventriculotomy and therefore it is expected to improve the right ventricular performance in the long term. Early transatrial-transpulmonary repair has been used for avoiding the deleterious effects of preoperative chronic cyanosis and spells, surgery for systemic-to-pulmonary artery shunts and their consequences, chronic right ventricular systolic pressure, and myocardial compensatory hypertrophy, with the aim of achieving better preservation of the right ventricular function in the long term. In our experience, transatrial-transpulmonary repair of TOF has been feasible at any age with immediate results that are comparable to those performed later in infancy.

Congenital heart diseases are currently treated in the pediatric age. However, a conspicuous number of patients requires treatment in the adult age. This study has been undertaken by members of the European Congenital Heart Surgeons Association with the aim of evaluating the impact of cardiac surgery in this particular age group. We have collected data from 1,247 patients who underwent 1,287 operations during a 5-year period between January 1, 1997 and December 31, 2001. Patients were divided into three groups: (1) palliative procedures (4.4%), operation performed to improve patients' clinical status without restoring normal anatomy or physiology; (2) repair (79.3%), operation performed to achieve an anatomic or physiologic repair with separation of the pulmonary from the systemic circulation (included in this group are also Fontan-type repair and one and a half ventricle repair); (3) reoperation (16.3%), all the reoperations performed after repair (either anatomic or physiologic). Hospital mortality (within 30 days) was 2.4% (range, 0% to 15.3% in different centers). Kaplan-Meier estimates shows a 94% survival at 4 years, which is higher for repair (95%) as compared with reoperations (92%) or palliations (88%). Surgery for congenital heart disease in the adult age is a safe, beneficial, and low-risk treatment that modifies patients' natural history by improving their clinical status.