Search

Background The aim of this study was to evaluate the incidence and risk factors for the development of right ventricular outflow tract obstruction (RVOTO) after the arterial switch operation (ASO). Methods Between 1983 and 2014, a total of 688 patients underwent ASO. RVOTO was defined as any obstruction of the right ventricular outflow tract (RVOT) requiring reintervention. Results RVOTO developed in 79 patients (11%) at amedian time of 3.8 years (range, 1 day-23.6 years) after ASO. Freedom from RVOT reintervention was 96 ± 1, 89 ± 1, and 83 ± 2% at 1, 10, and 25 years, respectively. Independent risk factors for the development of RVOTO in a Cox's regression model were side-by-side great arteries (p < 0.001), aortic arch anomalies (p < 0.001), use of a pericardial patch for augmentation of the coronary buttons (p < 0.001), and a peak gradientmore than 20 mm Hg over the RVOT at discharge (p < 0.001). Conclusion The incidence of RVOTO after ASO is not negligible. Complex morphology, such as side-by-side great arteries and aortic arch anomalies influences the development of RVOTO. [ABSTRACT FROM AUTHOR]

Background Systemic-to-pulmonary artery shunt placement is an established palliative procedure for congenital heart disease. Although it is thought to be a simple operation, it is associated with significant morbidity and mortality. Methods Data for all neonates who underwent surgery for a systemic-to-pulmonary artery shunt between 2000 and 2016 were reviewed. The study endpoints were shunt failure and shunt-related mortality. Shunt failure was defined as a shunt dysfunction because of thrombosis or stenosis requiring intervention or reoperation; shunt mortality was defined as death because of a shunt dysfunction. Results A total of 305 shunts (central shunt, n = 135; Blalock-Taussig shunt, n = 170) were implanted in 280 patients. The median patients' age at the time of surgery was 9 days (1-31 days). Themedian shunt size was 3.5 mm(3-4mm). Twentyfour patients (8%) were diagnosed with a shunt failure, with a median time of 7 days (0-438 days). Freedom from shunt failure at 1 year was 91.6% ± 2%. A shunt-related mortality was ascertained for 12 patients (4%). Freedom from shunt-related mortality at 1 year was 96% ± 1%. Perioperative platelet transfusion (p = 0.01), central shunt (p = 0.02), 3-mm shunt size (p = 0.02), and postoperative extra corporeal membrane oxygenation (ECMO) (p < 0.01) were identified as risk factors for shunt failure. Platelet transfusion (p = 0.04) and postoperative ECMO (p < 0.01) were further identified as risk factors for shunt mortality. Conclusion Based on these data, we recommend implanting a modified Blalock-Taussig shunt of at least 3.5 mm in neonates. Perioperative platelet transfusion and postoperative ECMO increase the risk of shunt failure. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Our aim was to evaluate outcomes following a total cavopulmonary connection (TCPC) in patients with preoperatively impaired ventricular function (VF). METHODS: Of 483 consecutive TCPC patients, 44 (9.1%) had impaired VF (ejection fraction <50%, Group A), and 439 patients had normal VF (ejection fraction >_50%, Group B). We compared the clinical outcomes between the groups. RESULTS: The median age at TCPC was 2.8 (interquartile range 1.9-8.3) years in Group A and 2.3 (1.8-3.5) years in Group B (P = 0.025). An atrioventricular valve (AVV) operation prior to (38.6 vs 27.1%, P < 0.001) and concomitant with (31.8 vs 12.1%, P < 0.001) the TCPC was performed more frequently in Group A. The median intensive care unit stay (7.0 vs 7.0 days, P = 0.737) and 30-day survival (97.7 vs 98.4%, P = 0.737) were not significantly different between groups. Freedom from death, transplantation (P = 0.115) and catheter intervention (P = 0.603) showed no difference between groups. However, freedom from cardiac reoperation was significantly lower in Group A (P < 0.001). VF was resolved in 22 of the 39 (56.4%) survivors in Group A. The recovered patients had a lower incidence of AVV reoperation (0 vs 6, P = 0.002) and pacemaker rhythm (0 vs 5, P = 0.006). CONCLUSIONS: In patients planned for TCPC, impaired VF is often associated with AVV regurgitation. TCPC can be performed with low risk and comparable clinical results except for cardiac reoperation in patients with impaired VF when compared to patients with normal VF. Following TCPC, VF recovers in half of the survivors. A competent AVV and sinus rhythm are prerequisites for recovery. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. METHODS: Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. RESULTS: Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on longterm follow-up magnetic resonance imaging. CONCLUSIONS: Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. METHODS: Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC <18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. RESULTS: Median age at TCPC was 1.4 (interquartile ranges: 1.3–1.5) years in group A and 2.5 (1.9–4.5) years in group B. Duration of intensive care unit stay (6 vs 7 days), hospital stay (20 vs 20 days), and 30-day survival (100 vs 98%) was not significantly different between groups. Estimated survival (95.3 vs 92.1%), freedom from reoperation (93.7 vs 86.3%), freedom from catheter intervention (60.1 vs 77.0%), and freedom from protein losing enteropathy (97.6 vs 93.8%) at 10 years was not significantly different between groups. At last follow-up, no patient in group A but 13 patients in group B exhibited reduced ventricular function (P = 0.035). Exercise-capacity testing showed that both peak oxygen uptake (36.4 vs 28.6 ml/kg/min; P = 0.026) and its percentage of predicted value (82.9 vs 70.0%; P = 0.004) were significantly higher in group A (n = 6, mean postoperative period: 8.9 years) than in group B (n = 119, mean postoperative period: 8.7 years). CONCLUSIONS: Fontan completion for TCPC can be performed at an early age with a low perioperative risk and good intermediate results. Earlier unloading of the systemic ventricle and earlier elimination of chronic cyanosis by staged cavopulmonary connection might be advantageous for preservation of long-term ventricular function. [ABSTRACT FROM AUTHOR]