Management of tricuspid regurgitation in congenital heart disease: is survival better with valve repair?

OBJECTIVE: Tricuspid valve (TV) regurgitation in congenital heart disease includes a heterogeneous group of lesions, and few series have documented the outcomes. METHODS: We reviewed the records of 553 patients with congenital heart disease who had undergone TV surgery for tricuspid regurgitation from January 1993 to December 2010. Patients with Ebstein malformation were excluded. Their mean age was 32 ± 21 years, and 300 were female (54%). The most common diagnoses were conotruncal anomaly in 216 patients (39%), previous ventricular septal defect closure in 83 (15%), atrioventricular septal defect in 77 (14%), and pulmonary atresia with an intact ventricular septum in 11 (2%). Preoperative right-sided heart failure was present in 124 patients (22%), and 55 patients (10%) had pulmonary hypertension. RESULTS: TV repair was performed in 442 (80%) and TV replacement in 111 (20%) patients. Repeat sternotomy was performed in 415 patients (75%). Previous TV repair was present in 44 patients (8%); of these, 17 (38.6%) underwent repeat TV repair. The overall early mortality was 3.1% (17 patients) and was 2.5% for TV repair and 5.4% for TV replacement (P = .001). The mean follow-up period was 4.5 ± 4.1 years (maximum, 18). The overall survival at 1, 5, and 10 years was 97%, 93%, and 85%, respectively. Survival was better for patients with repair than with replacement. TV repair was an independent predictor of better survival (P = .001). CONCLUSIONS: Important tricuspid regurgitation can occur with a variety of congenital diagnoses. Early mortality is low and late survival is superior with tricuspid repair than with valve replacement. Surgical treatment of tricuspid regurgitation in congenital heart disease should be performed before the onset of heart failure.

Late outcome of repair of congenital coronary artery fistulas--a word of caution.

OBJECTIVES: We reviewed our experience with repair of congenital coronary artery fistulas. METHODS: From June 1983 to December 2009, 46 patients (median age, 59 years; range, 1-84 years) underwent surgical repair. The presenting symptoms included angina in 16 patients (35%), congestive heart failure in 11 (24%), and bacterial endocarditis in 5 (11%). Preoperatively, 9 patients (20%) had at least moderate tricuspid regurgitation. Coronary artery dominance was right in 38 patients (83%). Coronary artery aneurysms were found in 8 patients (17%). The most common pattern was right coronary artery-to-coronary sinus fistula (18 patients, 39%); 11 patients had (23%) more than 1 fistula. One patient had undergone previous coil embolization. RESULTS: Cardiopulmonary bypass was used in 39 patients (85%), with extracardiac and intracardiac repair performed in 30 (65%) and 16 (35%), respectively. The most common associated procedures were coronary artery bypass in 13 patients (28%). Early mortality occurred in 1 patient (2%). Postoperative myocardial infarction occurred in 5 patients (11%); 4 of these patients underwent simple ligation or division of their fistulas. The mean follow-up was 6 ± 5.8 years (maximum, 22 years). Late mortality occurred in 11 patients (24%). Two patients underwent reoperation for severe tricuspid regurgitation. Survival was significantly reduced compared with the age- and gender-matched population (P = .03). Residual fistulas were detected in 3 patients (6%), with no reintervention needed. CONCLUSIONS: Perioperative myocardial infarction is an important complication of ligation of coronary artery fistulas and can contribute to reduced late survival. The tricuspid valve should be evaluated carefully at repair because of the relatively high rate of residual regurgitation in survivors.

Incidence of aortic complications in patients with bicuspid aortic valves.

CONTEXT: Bicuspid aortic valve (BAV), the most common congenital heart defect, has been thought to cause frequent and severe aortic complications; however, long-term, population-based data are lacking. OBJECTIVE: To determine the incidence of aortic complications in patients with BAV in a community cohort and in the general population. DESIGN, SETTING, AND PARTICIPANTS: In this retrospective cohort study, we conducted comprehensive assessment of aortic complications of patients with BAV living in a population-based setting in Olmsted County, Minnesota. We analyzed long-term follow-up of a cohort of all Olmsted County residents diagnosed with definite BAV by echocardiography from 1980 to 1999 and searched for aortic complications of patients whose bicuspid valves had gone undiagnosed. The last year of follow-up was 2008-2009. MAIN OUTCOME MEASURE: Thoracic aortic dissection, ascending aortic aneurysm, and aortic surgery. RESULTS: The cohort included 416 consecutive patients with definite BAV diagnosed by echocardiography, mean (SD) follow-up of 16 (7) years (6530 patient-years). Aortic dissection occurred in 2 of 416 patients; incidence of 3.1 (95% CI, 0.5-9.5) cases per 10,000 patient-years, age-adjusted relative-risk 8.4 (95% CI, 2.1-33.5; P = .003) compared with the county's general population. Aortic dissection incidences for patients 50 years or older at baseline and bearers of aortic aneurysms at baseline were 17.4 (95% CI, 2.9-53.6) and 44.9 (95% CI, 7.5-138.5) cases per 10,000 patient-years, respectively. Comprehensive search for aortic dissections in undiagnosed bicuspid valves revealed 2 additional patients, allowing estimation of aortic dissection incidence in bicuspid valve patients irrespective of diagnosis status (1.5; 95% CI, 0.4-3.8 cases per 10,000 patient-years), which was similar to the diagnosed cohort. Of 384 patients without baseline aneurysms, 49 developed aneurysms at follow-up, incidence of 84.9 (95% CI, 63.3-110.9) cases per 10,000 patient-years and an age-adjusted relative risk 86.2 (95% CI, 65.1-114; P <.001 compared with the general population). The 25-year rate of aortic surgery was 25% (95% CI, 17.2%-32.8%). CONCLUSIONS: In the population of patients with BAV, the incidence of aortic dissection over a mean of 16 years of follow-up was low but significantly higher than in the general population.

Outcome of caval division techniques for partial anomalous pulmonary venous connections to the superior vena cava.

BACKGROUND: Correction of a high partial anomalous pulmonary venous (PV) connection to the superior vena cava (SVC) may be complicated by sinus node dysfunction, or SVC or PV obstruction. We have used the caval division technique when the anomalous PV insert high above the cavoatrial junction and report our long-term outcome. METHODS: Between 1990 and 2009, 178 consecutive patients underwent operations for a partial anomalous PV connection. Caval division was used in 41 patients (23%). Median age was 34 years (range, 1.3 to 76 years). Thirty patients (73%) had an atrial septal defect. Eighteen patients (44%) underwent Warden procedure in which the SVC was divided, the upper end was connected to the right atrial appendage, and the remaining SVC served as a conduit for PV drainage to the left atrium through the atrial septal defect. A modified Warden was performed in 17 patients (41%) using a short ringed interposition graft between the SVC and the right atrial appendage. Six patients (15%) with persistent left SVC underwent right SVC division without reimplantation. RESULTS: No early deaths occurred. One Warden group patient (5%) required early reoperation for SVC obstruction. One patient (2%) with atrial fibrillation underwent a concomitant Maze procedure and permanent pacemaker implantation for tachybrady syndrome. All other patients remained in sinus rhythm at follow-up. Follow-up was complete in all patients (mean, 2±2.6 years; maximum, 14 years). One noncardiac-related death occurred at 14 years. SVC obstruction developed in 3 Warden group patients (17%) and was managed with angioplasty and stenting. No patient had pulmonary vein stenosis on follow-up. CONCLUSIONS: Caval division techniques appear to be effective, with low morbidity and mortality. The modified Warden procedure may decrease the risk of SVC obstruction. Late arrhythmias or PV obstruction are uncommon.