Left pulmonary artery originating from the right coronary artery in a patient with pulmonary atresia and ventricular septal defect.

We describe a patient with pulmonary atresia and ventricular septal defect in whom the left pulmonary artery was originating from the dilated right coronary artery. Successful division of the left pulmonary artery from the right coronary artery and the connection with the right ventricular outflow tract was carried out with closure of the end of the origin. The preoperative accurate diagnosis of this anomaly with ascending aortography is the most important in the surgical management of patients with pulmonary atresia and ventricular septal defect.

Da Silva's cone repair for Ebstein's anomaly: effect on right ventricular size and function.

OBJECTIVES: Da Silva's cone repair is a novel technique for surgical reconstruction of the tricuspid valve and the right ventricle (RV) in Ebstein's anomaly. The technique consists of extensive leaflet mobilization, longitudinal plication of the atrialized ventricle and cone-shaped reconstruction of the tricuspid valve, allowing for leaflet-to-leaflet coaptation. We evaluated the influence of Da Silva's cone repair on tricuspid competency, right ventricular size and function. METHODS: From February 2010 until July 2013, 20 patients (median age 30.0 years, range 6.6-68.3 years) underwent Da Silva's cone repair. A 4- to 6-mm interatrial communication was left in all patients. Echocardiographic studies and magnetic resonance imaging (MRI) before and after the repair were evaluated. RESULTS: Median follow-up was 11 (0.5-36) months. There were 2 early deaths and no late death. Echocardiography at follow-up revealed mild or absent tricuspid regurgitation in 16 patients. Two patients showed moderate tricuspid insufficiency. MRI studies showed that the mean functional RV end-diastolic volume decreased after surgery (pre 334 [135-656] ml; post 175 [115-404] ml, P < 0.001). The mean RV ejection fraction decreased (pre 47 ± 10%; post 35 ± 13%, P = 0.001), and the mean antegrade net stroke volume of the RV increased (pre 65 ± 28 ml; post 75 ± 30 ml, P = 0.057). CONCLUSIONS: Da Silva's cone repair for Ebstein's anomaly creates excellent valve function in all patients. Consecutively, the size of the RV decreases and the antegrade net stroke volume increases 6 months after the operation.

Independent risk factors for cardiac operations in adults with congenital heart disease: a retrospective study of 543 operations for 500 patients.

Adults with congenital heart disease (CHD) are an increasing population requiring cardiac operations. To date, the perioperative risk factors for this group have not been identified. This study aimed to identify clinical, morphologic, and hemodynamic risk factors for an adverse outcome. This study retrospectively analyzed a cohort of 500 patients (ages >16 years) who underwent 543 operations between January 2004 and December 2008 at a single center. The composite end point of an adverse outcome was in-hospital death, a prolonged intensive care exceeding 4 days, or both. The composite end point was reached by 253 of the patients (50.6%). Of the 500 patients, 13 (2.6%) died within 30 days after the operation. After logistic regression analysis, the following eight items remained significant: male gender (P = 0.003; odds ratio [OR] 1.8; 95% confidence interval [CI] 1.2-2.6), cyanosis (P > 0.006; OR 3.7; 95% CI 1.5-9.4), functional class exceeding 2 (P = 0.004; OR 2.2; 95% CI 1.3-3.7), chromosomal abnormalities (P = 0.004; OR 3.3; 95% CI 1.4-7.7), impaired renal function (P = 0.019; OR 3.8; 95% CI 1.2-11.5), systemic right ventricle (RV) in a biventricular circulation (P = 0.027; OR 3.3; 95% CI 1.1-9.5), enlargement of the systemic ventricle (P = 0.011; OR 1.7; 95% CI 1.1-2.6), and operation with extracorporeal circulation (P = 0.002; OR 4.3; 95% CI 1.7-11.4). Early mortality in the current adult CHD population is low. Morbidity, however, is significant and influenced by the patients' conditions (male gender, chromosomal abnormalities), history (cyanosis, New York Hospital Association [NYHA] class), and underlying morphology (systemic RV). This information for a large cohort of patients could help progress toward more adequate counseling for adults with a congenital heart defect.

Clinical, echocardiographic and histopathologic findings in nine patients with surgically explanted ASD/PFO devices: do we know enough about the healing process in humans?

BACKGROUND: Atrial septal defects (ASD) and persistent foramen ovale (PFO) are managed in increasing numbers by catheter interventions as an attractive alternative to surgery. Early complications have been described in clinical series whereas late complications are rare. No series are reported with clinical, echocardiographic and histological data. METHODS AND RESULTS: We collected clinical, echocardiographic, and histolological data of nine patients with surgically explanted devices. Occlusion devices were explanted after a mean interval of 3.4 ± 2.4 years (range 0.9-8.3). Indications were recurrent thromboembolic events in five, residual shunt/dislocation in three, and growing mass on echocardiography despite oral anticoagulation in one patient. Two patients suffered potentially live threatening events due to coronary embolism. One of them had to be resuscitated due to ventricular fibrillation. Histologically, residues of superficial thrombus formation could be demonstrated in two of the devices. In another patient, hyperplastic tissue formation was related to a local inflammatory process but not to a thrombus as suspected by echocardiography. CONCLUSION: Late complications after device implantation may occur up to 8 years after device implantation and may be potentially live threatening. Echocardiographic controls should be prolonged beyond the first year after implantation and every explanted device should be histologically worked up in an experienced center. Up to now, the mechanisms of late thrombogenesis are not fully understood.