Three-dimensional printed heart models showed potential utility, especially in understanding the relationship between intraventricular communications and great vessels, as well as in simulation for creating intracardiac pathways.

Objective: Surgical outcomes of biventricular repair for hearts with hypoplastic left ventricle with congenital mitral valve stenosis are described. Serial changes of left ventricular geometry and clinical features after biventricular repair were reviewed. Methods: Eight patients with hypoplastic left ventricle and congenital mitral valve stenosis who underwent first surgical intervention for biventricular circulation in neonatal or infantile period between 2001 and 2014 comprise the study population. Serial change in left ventricular end-diastolic diameter, left ventricular mass index, and relative wall thickness after biventricular repair were evaluated by two-dimensional echocardiography. Results: The median Z-scores of left ventricular end-diastolic diameter and mitral valve diameter before the first surgical intervention were À3.0 (range, À4.8 to À2.0) and À1.0 (À2.9 to 2.1), respectively. Mitral valves were surgically treated in five patients; they were replaced in two and repaired in three patients. Left ventricular end-diastolic diameter Z-score at five years after biventricular repair was 0.1 (À3.0 to 1.0), which was significantly larger than before first surgical intervention (P ¼ .005). Left ventricular mass index, on the other hand, did not change, but relative wall thickness significantly decreased (P ¼ .009). Postoperative catheter study showed pulmonary hypertension with high left ventricular end-diastolic pressure in more than half of survivors. Conclusions: Left ventricle increased in size after the biventricular repair with appropriate mitral valve procedures before progression of pulmonary hypertension. Left ventricular mass, however, did not accompany the increase. Some patients may have suffered from mild, but certain restrictive left ventricular physiology and subsequent pulmonary hypertension as the result of abnormal remodeling process of the myocardium.

Background:The surgical treatment of congenital heart disease is reported to be associated with a high prevalence of scoliosis, although the detailed etiology is unknown. Surgical interventions involving the rib cage are considered to increase the risk of scoliosis. However, whether the cardiac condition or the procedure performed makes patients more susceptible to the development of spinal deformity is controversial.Methods:The present study included 483 patients who underwent surgery for the treatment of congenital heart disease with use of procedures involving the immature rib cage (sternotomy and/or thoracotomy) during the first year of life, followed by the evaluation of standing chest radiographs at ≥10 years of age. Patients with congenital spinal deformity and potential neuromuscular disease were excluded. The prevalence of and predictive factors for scoliosis were evaluated. The presence of scoliosis (Cobb angle ≥10° to <20°, ≥20° to <30°, ≥30° to <45°, ≥45°), the convex side of the curve, and the location of the curve were evaluated radiographically. Potential predictive factors that were analyzed included the age at the time of surgery, surgical approach, use of cardiopulmonary bypass, postoperative heart failure and/or cyanosis, New York Heart Association (NYHA) class, cardiomegaly, and age at the time of radiography.Results:The mean age at the time of surgery was 112 days, and the mean age at the time of radiography was 14.4 years. The prevalence of scoliosis was 42.4%, and the prevalences of ≥10° to <20°, ≥20° to <30°, ≥30° to <45°, and ≥45° scoliosis were 31.7%, 5.8%, 2.5%, and 2.5%, respectively. Three patients underwent surgery for the treatment of progressive scoliosis. Multivariate analysis indicated that the predictive factors were female sex, left thoracotomy, bilateral thoracotomy, NYHA class, and age at the time of radiography for ≥10° scoliosis; cardiomegaly, NYHA class, and age at the time of radiography for ≥20° scoliosis; cardiomegaly, number of surgical procedures, and age at the time of radiography for ≥30° scoliosis; and cardiomegaly for ≥45° scoliosis. Age at the time of radiography was a predictor of <45° scoliosis; however, the relative association was small.Conclusions:Surgery for the treatment of congenital heart disease during the first year of life was associated with a high prevalence of scoliosis (≥40%). While female sex was one of several predictors of ≥10° scoliosis, cardiomegaly was the sole predictor of ≥45° scoliosis.Level of Evidence:Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Regardless of a significant reduction in systemic ventricular volume, DKS anastomosis concomitant with a BDG procedure shows promise for a nonobstructive systemic ventricular outflow tract after a Fontan operation.

mediate-term clinical outcomes and can also serve as a predictor of need for reintervention regardless of patient age and disease complexity. 4,5 Methods Patients and Surgical Procedure The National Cerebral and Cardiovascular Center Institutional Review Board approved this retrospective study, and opt-out consent was obtained instead of individual written informed consent (R19043-2). A total of 159 consecutive patients undergoing repair of TOF primarily or after systemic to pulmonary shunt between 2001 and 2015 were enrolled (Table 1). Patients with major aortopulmonary collateral arteries, atrioventricular septal defect, or absent pulmonary valve were excluded. Mean age and body weight at repair were 12.7±6.6 months and 7.9±1.7 kg, respectively. Nineteen patients (11.9%) had pulmonary atresia, and 77 patients (48.4%) had undergone palliative systemic to pulmonary shunt through lateral thoracotomy. TOF repair was performed at between 2 months and 2 years of age following preoperative cardiac catheter evaluation. The patients with pulmonary atresia (11.9%) under

No abstract

This study aimed to review clinical features and surgical outcomes of coronary sinus orifice atresia (CSOA). From 2003 to 2015, 6 patients were diagnosed with CSOA. Median age at diagnosis was 2 years (range 0.2-73). CSOA was preoperatively diagnosed in 2 patients, intraoperatively in 3, and postoperatively in 1. Coronary venous blood flow drained into innominate vein through the left superior vena cava (LSVC) in 2, into the atrium through Thebesian veins in 2, and into both in 2. Three patients required surgical treatment. The first patient with a congenitally corrected transposition of great arteries had undergone ligation of the LSVC and a cutback of the proximal LSVC into the functional right atrium during the double-switch operation. The second patient with tricuspid atresia gradually developed hypoxia after the Fontan operation due to the increase in veno-venous shunt blood flow from the Fontan pathway to the right atrium through the LSVC and Thebesian veins, so that the LSVC was ligated and the coronary sinus was unroofed 6 years after Fontan operation. The other functional single ventricle patient had undergone ligation of the LSVC and a cutback of the proximal LSVC into the left atrium during the Fontan operation. There were no mortalities at the median follow-up of 9 years (1-11). CSOA should be suspected when the LSVC and usual-sized innominate vein were presented. Surgical treatment is required if the postoperative hemodynamics would affect coronary venous perfusion, like single ventricular palliation (244 words).

OBJECTIVES The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit. METHODS Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. The left ventricle was dominant in 26 patients (72.2%). Median age at conversion and interval from initial Fontan operation to conversion were 24.1 years (interquartile range 18.9–29.2) and 17.8 years (15.4–20.9), respectively. Surgical cryoablation was concomitantly performed in 32 patients (88.9%). Cardiac catheter examination was performed preoperatively (36 patients, 100%) and at 1 year (31 patients, 86%), 5 years (25 patients, 69%) and 10 years (13 patients, 36%) after TCPC conversion. Symptom-limited treadmill exercise with expired gas analysis was performed preoperatively (32 patients, 88.9%) and at 1 year (27 patients, 75.0%), 5 years (20 patients, 55.6%) and 10 years (12 patients, 33.3%) after conversion. RESULTS All patients received follow-up; the mean follow-up period was 8.2 ± 4.8 years. Actuarial survival rate, protein-losing enteropathy-free survival rate and rate of survival with sinus rhythm maintenance at 10 years were 79.2%, 67.8% and 48.5%, respectively. The survival curve declined steeply when the duration of Fontan circulation exceeded 25 years. New cases of protein-losing enteropathy developed postoperatively in 2 patients. Permanent pacemakers were implanted in 12 patients (33%), but atrial tachyarrhythmia was not sustained in any of the remaining patients. Pulmonary arterial pressure (11.0 ± 3.1 to 9.5 ± 3.6 mmHg, P = 0.003), pulmonary vascular resistance (2.1 ± 0.7 to 1.3 ± 0.5 WU/m2, P &lt; 0.0001) and cardiac index (2.0 ± 0.3 to 2.9 ± 0.6 l/min/m2, P &lt; 0.0001) significantly improved from preoperative evaluation to 1 year after the conversion, and these improvements were maintained during the entire follow-up period. Peak oxygen uptake remained unchanged from the preoperative evaluation (49.7 ± 11.5% predicted) to 1 year (52.5 ± 12.0%), 5 years (56.2 ± 9.6%) and 10 years (51.2 ± 9.4%) after conversion (P = 0.19). CONCLUSIONS Owing to its anti-arrhythmic effect and Fontan pathway recruitment effect, TCPC conversion with an extracardiac conduit prevented the natural decline of exercise tolerance that is seen in classic Fontan patients.