New approach to multiple ventricular septal defect closure with intraoperative echocardiography and double patches sandwiching the septum

ObjectiveThis was an evaluation of a new approach to the management of multiple muscular ventricular septal defects. The defects were located with epicardial echocardiography, then transfixed with a guide wire inserted directly through the right ventricular free wall. They were closed with a custom-made multilayered double-patch device under cardioplegic arrest through a standard right atriotomy.MethodsThis was a retrospective study of 14 consecutive patients.ResultsThe median age and body weight at repair were 40 days (range 1 week–8 years, 3 months) and 4.1 kg (2.8-24 kg), respectively. Five patients (36%) had undergone at least one previous sternotomy; 11 patients (78%) had associated cardiac lesions. Closure of the multiple septal defects was successful in 12 patients (85%). Failure to localize all defects led to pulmonary artery banding in 2 patients. One patient had the residual septal defect closed with a percutaneous device 6 months later, and in the second patient the residual defect was closed with a conventional approach 11 months afterward. Two patients had permanent pacemaker insertion. In 279 patient-months of follow-up, there was 1 cardiac arrest on day 1 and no early or late deaths; all children but one are free of cardiac medications, and no significant residual left-to-right shunts were demonstrated in any patient.ConclusionThe reported management of multiple ventricular septal defects has been successful in this series, even in neonates and infants with complex associated cardiac lesions. It appears safe, simple, and effective

Surgical management of pulmonary artery sling in children

ObjectivesPulmonary artery (PA) sling is a rare vascular anomaly associated with congenital tracheal stenosis. The natural history is poor and these patients often require early surgical intervention. We describe our experience with repair of this condition.MethodsFrom 1984 to 2011, 21 patients with PA sling underwent repair at the Royal Children's Hospital (median age, 5.9 months). PA sling was associated with compression of the trachea in all patients. Tracheal surgery was required in 12 (57.1%) patients. All patients had an echocardiogram, and concomitant repair of coexisting cardiac anomalies was performed in 6 (28.6%, 6/21) patients.ResultsOperative mortality was 14.3% (3/21), occurring at 19 days, 4.4 months, and 5 months after surgery. Operative mortality for the first 10 years was 22.2% (1984-1993; 2/9), the next 10 years was 14.3% (1994-2003; 1/7), and 0% for the most recent 7 years (2004-2011; 0/5). All deaths occurred in patients requiring tracheal repair (25%, 3/12). No deaths have occurred since 2004 with introduction of the slide tracheoplasty technique. One (5.6%, 1/18) late death occurred at 8 months after repair. After tracheal repair, intervention for excessive granulations and tracheomalacia was necessary in 6 (50%, 6/12) patients. Median follow-up was 8 years (mean, 8.6 ± 6.4 years; range, 5 months to 20.6 years), and all survivors (100%, 17/17) remain asymptomatic.ConclusionsChildren with PA sling who do not require tracheal surgery have excellent outcomes. Mortality is determined by the need for tracheal surgery. However, with the advent of the slide tracheoplasty technique, mortality can be reduced. Survival beyond 1 year after surgery offers excellent prognosis

Propensity score matched analysis of partial atrioventricular septal defect repair in infancy

Partial atrioventricular septal defect (pAVSD) is usually repaired between 2 and 4 years of age with excellent results. Repair during infancy has been associated with poorer outcomes. However, most infants in reported series had heart failure or significant left atrioventricular valve (LAVV) regurgitation. The impact of surgery during infancy on outcomes remains unclear.All children at three institutions who underwent repair of pAVSD from 1975 to 2015 were included. Infants (age

Abnormal left ventricular diastolic function at late follow-up after repair of total anomalous pulmonary venous drainage: The impact of altered ventricular loading in utero

BackgroundAssessment of diastolic function has not been described after repair of total anomalous pulmonary venous drainage (TAPVD), but studies of exercise capacity demonstrate impaired performance in this population despite normal systolic function. We postulated that diastolic impairment might contribute to this finding.MethodsWe analyzed echocardiographic variables from 28 patients with repaired TAPVD and compared these with data from 32 healthy controls (normals) and 21 subjects with repaired transposition of the great arteries (TGA).ResultsLeft ventricular (LV) end-diastolic volumes were smaller in the TAPVD group (median, 50 mL/m2 compared with a median of 64 mL/m2 in TGA and 67 mL/m2 in normals; P < .001 in each case). LV diastolic function in the TAPVD group was impaired. Mitral early to late ratio was increased (median, 2.7 in TAPVD compared with a median of 1.9 in TGA [P = .047] and 2.1 in normals [P = .021]). LV isovolumic relaxation time was reduced (median, 50 milliseconds in TAPVD compared with a median of 70 milliseconds in both TGA and normals; P < .001 in each case). Late diastolic and systolic tissue Doppler velocities were lower and the E/e′ ratio was higher in the TAPVD group.ConclusionsPatients with repaired TAPVD are usually regarded as having excellent outcomes, but the finding of LV diastolic dysfunction in this population warrants more careful follow-up. We postulate that the diastolic impairment in these patients is the result of relative unloading of the LV during early cardiac development. These findings may also have implications in considering therapeutic approaches for hypoplastic ventricles in attempting to achieve biventricular repair