Heart transplantation in pediatric population and in adults with congenital heart disease: long-term follow-up, critical clinical analysis, and perspective for the future.

BACKGROUND: Heart transplantation is a treatment option for children as well as for adults with congenital heart disease. OBJECTIVE: To report the experience of a tertiary center with heart transplant program in pediatric population and in adults with congenital heart disease. PATIENTS AND METHODS: The study consisted of the evaluation of pediatric as well as adult patients undergoing heart transplantation for congenital heart disease. We evaluated the following indication and complications such as renal dialysis, graft vascular disease, tumors and survival. RESULTS: From October 1992 to November 2013, 134 patients had transplantation, and there were 139 transplantations and 5 retransplantations. The immunosuppression regimen is based on calcineurin inhibitors and cytostatic drugs. The type of heart disease indicated for transplantation was cardiomyopathies in 70% and congenital heart disease in 30%. Of these 134 patients, 85 patients were alive. Actuarial survival is 77.4%, 69.6%, 59.3% at 1, 5, and 10 years after transplantation. Three patients underwent renal transplantation, 1 patient is in renal dialysis, and 8.2% of patients had post-transplant lymphoproliferative disease. Two patients had retransplantation for graft vascular disease; 1 of them required a simultaneous kidney transplant and died 30 days after the procedure and 1 patient is clinically well 2 years after retransplantation. CONCLUSION: Heart transplantation in children and in adults with congenital heart disease is a promising therapeutic option and enables long-term survival for these patients.

The impact of tacrolimus as rescue therapy in children using a double immunosuppressive regimen after heart transplantation.

BACKGROUND: Organ transplant recipients with refractory rejection or intolerance to the prescribed immunosuppressant may respond to rescue therapy with tacrolimus. We sought to evaluate the clinical outcomes of children undergoing heart transplantation who required conversion from a cyclosporine-based, steroid-free therapy to a tacrolimus-based regimen. METHODS: We performed a prospective, observational, cohort study of 28 children who underwent conversion from cyclosporine-based, steroid-free therapy to a tacrolimus-based therapy for refractory or late rejection or intolerance to cyclosporine. RESULTS: There was complete resolution of refractory rejection episodes and adverse side effects in all patients. The incidence rate (×100) of rejection episodes before and after conversion was 7.98 and 2.11, respectively (P ≤ .0001). There was a 25% mortality rate in patients using tacrolimus after a mean period of 60 months after conversion. CONCLUSION: Tacrolimus is effective as rescue therapy for refractory rejection and is a therapeutic option for pediatric patients.

Congenital atresia of the ostium of the left coronary artery. Diagnostic difficulty and successful surgical revascularization in two patients.

We report two cases of congenital atresia of the ostium of the left coronary artery. Case 1: a six-month-old infant presenting with serious cardiac insufficiency. A noninvasive diagnosis of dilated myocardiopathy was established and the clinical picture was pharmacologically compensated. When the patient was nine months of age, a hemodynamic study was performed that revealed congenital atresia of the ostium of the left coronary artery; the infant immediately underwent a successful anastomosis of the internal mammary artery with the left coronary artery. Case 2: an eleven-year-old asymptomatic boy with a history of heart murmur from the age of six months on, was referred for surgery with a diagnosis of anomalous origin of the left coronary artery from pulmonary trunk. A definitive diagnosis of atresia of the left coronary ostium was only established during surgery. Successful surgical revascularization with the left internal mammary artery, and left ventricular aneurysmectomy were performed.

Intraventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect: advantages of multiple patches.

OBJECTIVE: The objective of this paper is to report our experience with biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect by means of multiple patches that simplify and render feasible the intraventricular correction of this complex anomaly. METHODS: From April 1987 to April 1999, in 18 patients with double-outlet right ventricle and noncommitted ventricular septal defect, a technical modification that used multiple patches of bovine pericardium was used to construct an intraventricular tunnel connecting the left ventricle to the aorta. Ages ranged from 2 months to 13 years (mean age 4.73 +/- 3.41 years). RESULTS: The early mortality was of 11.1% (2 patients). Surviving patients were followed up for a mean of 2.65 years. Three late deaths (16.6%) occurred: 5 months, 7 months, and 7 months after the operation. All but 1 patient are in New York Heart Association class I. CONCLUSION: The use of multiple patches for biventricular correction of this anomaly simplifies and renders feasible the intraventricular repair in cases in which the 1-patch technique was deemed impossible.

Anomalous origin of the left coronary artery from the right pulmonary artery with intramural aortic trajectory. Clinicosurgical diagnostic implications.

OBJECTIVE: Anomalous origin of the left coronary artery from the right pulmonary artery (AOLCARPA), is a rare entity that is usually associated with other defects. Of the 20 cases of AOLCARPA reported in the literature, 14 (70%) had associations. We describe four patients with AOLCARPA without associated defects, but with a peculiar intramural aortic trajectory. METHODS: Fifty-five patients with anomalous origin of the left coronary artery were operated upon at INCORFMUSP. Four of the patients had the anomalous origin from the right pulmonary artery (RPA) without associated defects but with intramural aortic trajectory. Clinical and laboratory examinations were analyzed, as well as surgical findings. RESULTS: All patients had congestive heart failure (CHF) and 3 also had angina pectoris. Two patients had a murmur of mitral regurgitation, signs of myocardial infarction on the ECG and cardiomegaly. The shortening fraction varied from 9% to 23%. The hemodynamic study confirmed the diagnosis of anomalous origin of the coronary artery, but the intramural trajectory and the origin from the RPA were established only at surgery. In 3 patients, the technique of side-to-side anastomosis was performed with a good outcome. One patient, who underwent end-to-side anastomosis, died 6 months after the surgery. CONCLUSION: Association with other defects usually occurs in the AOLCARPA, and the intramural aortic trajectory is difficult to clinically diagnose but easy to surgically correct.

Transxiphoid approach without median sternotomy for the repair of atrial septal defects.

BACKGROUND: Interest in minimally invasive procedures has recently increased because it results in less surgical trauma, decreased patient discomfort, short hospital stay, reduced costs, and better cosmetic appearance. Based on these facts, we have been using the transxiphoid process approach without sternotomy for the correction of atrial septal defects. METHODS: From July 1996 to January 1997, the xiphoid process window approach was performed in 10 patients with ostium secundum atrial septal defect. Ages ranged from 6 months to 14 years (mean, 5.3 years). In all patients, extracorporeal circulation was carried out by means of cannulation of the femoral artery and both caval veins and of aortic cross-clamping. Videothoracoscopy was used to improve visualization of the aorta. RESULTS: There were no intraoperative or postoperative complications, and in all but 1 patient, extubation was possible while in the operating room. CONCLUSIONS: The xiphoid process window, with no median sternotomy, permitted closure of the atrial septal defects with good results and could be used as a less invasive technique for their correction.