Multimodality Approach to a Complex Scimitar Syndrome: How Advanced Diagnostics Can Guide Therapeutic Strategies.

We report an unusual association of scimitar syndrome with right diaphragmatic hernia, left-sided aortic arch with "aberrant right subclavian artery" in a 2-year-old boy who underwent stepwise transcatheter occlusion of a significant aortopulmonary collateral followed by surgical treatment for the repair of the diaphragmatic hernia and esophageal compression. (Level of Difficulty: Advanced.).

HeartWare Explant After Recovery 6 Years After Implant in a 3-Year-Old Child: Has the Game Changed?

The use of continuous-flow mechanical circulatory support in preschool children remains anecdotal. This case report describes the sequel to the implantation with a HeartWare HVAD system (Medtronic, Minneapolis, MN) in a 3-year old child. A 3-year-old boy with myocarditis-related cardiomyopathy underwent implantation with a HeartWare device. After an uncomplicated postoperative course, the patient was discharged home. Serial echocardiography showed progressive left ventricular recovery. After 6 months, the device was decommissioned, and the outflow graft was tied off. Six years after insertion, the device was explanted uneventfully. The HeartWare ventricular assist device offers viable long-term mechanical circulatory support in selected children that results in sustainable care and good quality of life.

High-risk Fontan completion patients achieve low perioperative risk and benefit from cavopulmonary connection 7 years out†.

OBJECTIVES: Our unit has pursued Fontan completion in all patients except those with immobility or combined poor ventricular function and high pulmonary artery pressures. We assessed retrospectively whether conventional high-risk criteria would predict patients with a poorer outcome. METHODS: One hundred and thirty-three consecutive children who underwent extracardiac Fontan completion (2004-2012) had their outcomes recorded (mean follow-up of 7 years). Three groups were analysed: those with 1 of 6 historical risk factors (outside 6 commandments), those with 1 of reduced systemic ventricular function or pulmonary artery pressure >15 mmHg (outside 2 commandments) versus those with no contraindications. The Fischer's exact test examined frequency differences, with the χ2 test to look for outcome associations. RESULTS: There were no differences in postoperative complication rates between the outside 6 commandments (n = 105) or outside 2 commandments (n = 49) versus the low-risk no-contraindication group (n = 28): arrhythmias [18% (P = 0.3) or 18% (P = 0.3) vs 25%], infection [22% (P = 0.6) or 33% (P = 0.2) vs 21%], cerebrovascular accident [6% (P = 0.5) or 10% (P = 0.3) vs 4%], length of stay [20 days (P = 0.4) or 23 days (P = 0.2) vs 21 days] and duration of chest drainage (P = 0.5). There was 1 predischarge mortality in each group. Long term, the majority of patients in each group had suitable haemodynamics for fenestration closure [95% (P = 0.7) or 95% (P = 0.7) vs 92%]. Long term, there was no difference in the rate of arrhythmias [11% (P = 0.5) or 12.5% (P = 0.3) vs 7%], protein-losing enteropathy [1% (P = 0.1) or 2% (P = 0.3) vs 7%] or moderate or more ventricular dysfunction on echocardiography [2% (P = 0.7) or 4% (P = 0.7) vs 4%]. Notably, there was a higher rate of catheter reinterventions in the high-risk groups [22% (P < 0.05) or 24% (P < 0.05) vs 7%]. CONCLUSIONS: The medium-term benefits of Fontan completion can be achieved for high-risk patients, suggesting that historical selection criteria should be re-examined.

Long-term outcomes following partial atrioventricular septal defect (AVSD) repair in Ireland.

BACKGROUND: We describe the long-term results of partial atrioventricular septal defect (AVSD) repair in a single centre encompassing a 22-year period. Described are rates of survival, reoperation and complications. METHODS: We performed a retrospective review of 556 patients undergoing AVSD repair to identify the 51 patients who underwent partial AVSD repair in Our Lady's Children's Hospital, Crumlin, Ireland, between 1993 and 2015 with long-term follow-up where available. RESULTS: A total of 29 (56.8%) of patients were male and mean age at operation was 3.32 years. Mean weight was 13.2 kg. Trisomy 21 was present in 29 (56.8%). Five patients (9.6%) had undergone prior surgery. Mean cardiopulmonary bypass time was 89 ± 36 min and mean aortic cross-clamp time was 57 ± 28 min. One patient underwent partial AVSD repair and concomitant tracheal resection and extracorporeal membrane oxygenation decannulation. One patient was managed with suture atrial septal defect (ASD) closure, the remainder with patch repair of ASD and mitral cleft closure. The length of hospital stay was 9 ± 5 days. Median follow-up was 6.06 years (IQR, 1.65-10.2 years). There were no early mortalities. One patient died 1 year following surgery (1.9%). One patient required reoperation at an interval of 2 years for severe mitral regurgitation (1.9%). CONCLUSIONS: Short- and long-term survival following partial AVSD repair in Ireland revealed excellent results compared with other published series. Reoperation incidence also compared excellently with other reports published in the literature.

Expectation and quality of life after aortic valve replacement over 85 years of age match those of the contemporary general population.

BACKGROUND: In the transcatheter aortic valve implantation (TAVI) years, very elderly patients with aortic stenosis (AS) are referred to surgery with reluctance despite excellent hospital outcomes. A poorly assessed outcome of discharged survivors might further overlook the actual efficacy of the surgical strategy in this cohort. We thus evaluated life-expectancy and functional results in discharged survivors over 85 years operated on for AS. METHODS: Between January 2001 and December 2013, 57 consecutive patients aged ≥85 years underwent aortic valve replacement (AVR) with or without concomitant procedures at our institution. Late survival rate (SR), New York Heart Associaion (NYHA) functional class and quality of life (RAND SF-36) were assessed. SR and quality of life (QoL) were than compared to the contemporary general population matched for age and gender, as calculated by the Italian National Institute of Statistics. RESULTS: Overall in-hospital mortality was 8.8% (5 pts). In patients without concomitant coronary artery bypass grafting (CABG), in-hospital mortality was 2.9%. Survival at 5 and 9 years was 57.7 ± 8.4% and 17.9 ± 11.4%, respectively. No predictors of late mortality including concomitant CABG were identified at Cox analysis. The mean NYHA class for long-term survivors improved from 3.1 to 1.6 (p<0.001). Survivors reported better QoL-scores compared to the age- and gender-matched contemporary general population in 4 RAND SF-36 domains. Life-expectancy resulted comparable to that predicted for the age and gender-matched general population. CONCLUSIONS: Isolated AVR in patients aged ≥85 years can be performed with acceptable risk. Survivors improve in NYHA class and, when compared to age- and gender-matched individuals, show a similar life expectancy and a no lower QoL.