Jitendra J. Shah

Homograft replacement of mitral valve in children

BACKGROUND Recent reports have demonstrated successful early outcomes using mitral valve homografts in adults. We report our early results after homograft mitral valve replacement in 4 children with previous atrioventricular septal defects, previous placement of a prosthetic valve, and rheumatic valvular disease. METHODS Between May 1996 and June 1997, 4 children (ages 5, 11, 13, and 15 years) underwent mitral valve replacement with cryopreserved mitral valve homografts at our institution. Preoperative echocardiography confirmed moderately severe to severe mitral regurgitation, stenosis, or both in all 4 patients. RESULTS Successful homograft valve replacement was achieved in all 4 patients. Based on symptoms, physical examinations, and echocardiographic follow-up, all four homograft mitral valves are functioning well with normal hemodynamics. None of these patients are receiving warfarin. Follow-up has been limited to 10 months. CONCLUSIONS In children requiring mitral valve replacement, the use of mitral valve homografts offers advantages over prosthetic valves, such as the avoidance of complications associated with thrombosis and anticoagulation. Homograft mitral valve replacement is technically feasible in children with congenital and rheumatic heart disease and previous prosthetic valves.

The Ross-Konno Is a High-Risk Procedure When Compared With the Ross Operation in Children

BACKGROUND For children who require aortic valve replacement, the pulmonary autograft (Ross procedure) may be the ideal substitute. However, performing a modified Konno procedure at the time of autograft implantation (Ross-Konno) may be associated with significant morbidity and mortality. A retrospective study was undertaken to compare the outcomes of Ross-Konno (RK) and the Ross (R) procedures including the need for reinterventions and long-term survival. METHODS Between 1993 and 2011, 78 children (mean age, 11.1 ± 5.6 years; range, 1 week to 18 years) underwent the Ross procedure. Modified Konno-type enlargement of the left ventricular outflow tract was performed in 18 of those patients. RESULTS There was no statistically significant difference between the groups with respect to pathologic process, sex, concomitant procedures, and aortic gradient. Our data demonstrate that mean age (R, 12.9 years versus RK, 5.3 years; p < 0.001), mean size of allograft (R, 23.3 mm versus RK, 20.1 mm; p < 0.001), previous surgery (R, 51% versus RK, 83%; p = 0.05), and postoperative morbidity (R, 3% versus RK, 28%; p = 0.003) were significantly different between the groups. There were 3 hospital deaths (all RK with mitral valve anomalies). Actuarial survival at 10 years was significantly better for Ross patients than Ross-Konno (R, 96% versus RK, 72%; p = 0.001). Freedom from autograft, right ventricular outflow tract obstruction, and cumulative reoperations at 10 years were not significantly different between groups. CONCLUSIONS The risk of death and postoperative complications after the Ross-Konno procedure is higher than for the Ross procedure. Preoperative complexity (including mitral valve anomalies) is associated with significantly higher morbidity and mortality. Autograft insufficiency and right ventricular outflow tract obstruction are common postoperative complications, requiring reoperation in one quarter of patients, but these were not significantly different between the groups.

Autograft or Allograft Aortic Root Replacement in Children and Young Adults With Aortic Valve Disease: A Single-Center Comparison

BACKGROUND We analyzed the outcome of children and young adults (younger than 40 years) with aortic valve disease who underwent allograft or autograft aortic root replacement (ARR) in our institution and evaluated whether there is a preference for either valve substitute. METHODS One-hundred fifty patients younger than 40 years underwent ARR between January 1990 and July 2011. Forty-four patients, aged 18.8 ± 12.4 years, had ARR with allograft conduit (allograft group), whereas 106 patients, aged 17.9 ± 11 years (p = 0.63), had a Ross ARR during the same period of time (autograft group). Echocardiographic data were reviewed to evaluate valve performance. The 2 groups were similar with respect to age, gender, etiology, and previous and concomitant procedures. RESULTS Operative deaths were 3 in the autograft group. There were 6 late deaths in the autograft group and 5 in the allograft group. Survival was 92% and 84% at 5 and 15 years, respectively, in the allograft group versus 93% and 91% in the autograft group (p = 0.42). Freedom from any type of reintervention and from reoperation on aortic valve were similar (autograft, 64% and 72% versus allograft, 66% and 66%; p = not significant) at 15 years. Freedom from explantation were significantly better for Ross patients (autograft, 82% versus allograft, 66%; p = 0.05). CONCLUSIONS Aortic valve replacement with either the autograft or allograft provides good clinical results in children and young adults during an intermediate duration of observation. Survival early after ARR does not differ depending on the type of prosthesis. In patients with aortic valve disease, autograft and allograft ARR show comparable satisfactory early and long-term results, with the increasing reoperation risk in the second decade after operation remaining a major concern.

811-4 Use of resynchronization therapy in pediatric patients with congenital heart disease and complete heart block

Background: Biventricular pacing has been shown to be a beneficial therapy in adult patients with ventricular dysfunction and intraventricular conduction delay. However, no pediatric applications for this therapy have been tested. We hypothesized that pediatric congenital heart disease patients with complete heart block and poor ventricular function would benefit from conversion to biventricular pacing from traditional single site atrioventricular (DDD) pacing. Patients: A total of 6 patients with congenital heart disease, poor ventricular function and complete heart block were included in this study. Median age at the time of conversion to biventricular pacing was 11.1 months, ranging from 5.6 to 118 months. Diagnoses included complete atrioventricular canal (1), aortic stenosis/subaortic stenosis (4), antiRo, anti-La cardiomyopathy (1). Patients were all treated for heart failure prior to conversion to biventricular pacing, with digoxin (6/6), ACE-inhibitor (6/6), and carvedilol (2/6). Baseline ejection fraction with DDD pacing was 34 ± 12%. Median duration of followup after conversion to biventricular pacing was 2.6 months, ranging from 4 days to 5.8 months. Results: After conversion to biventricular pacing the ejection fraction increased to 57 ± 12%, p<0.004. In all subjects, the ejection fraction improved, with a minimum improvment of 12 EF units. Five patients also showed an improvement in weight for age, p=0.04. Other clinical improvement was noted, with 1 patient removed from the transplant list, and 1 patient tolerating reduction in medical support. Conclusions: Biventricular pacing appears to offer short-term benefit for selected pediatric patients with complete heart block and poor left ventricular function. Further studies looking at the long-term benefit of this therapy in the pediatric population are needed.

Repair of transposition of the great arteries with total anomalous pulmonary venous return

A 13-year-old Haitian girl presented with complex congenital heart disease consisting of transposition of the great arteries with total anomalous venous return. The patient exhibited clinical findings of long-standing congestive heart failure and cyanosis. She underwent complete repair with resolution of her symptoms.