Total anomalous pulmonary venous connection: Results of surgical repair of 100 patients at a single institution

ObjectiveSurgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome.MethodsOur cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990–2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0–4 years), and the median weight was 3.5 kg (range, 1.3–15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy.ResultsThere were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 ± 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0–281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4–753 days).ConclusionPatients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection

Breaking the Rules: Left Common Carotid Artery from the Main Pulmonary Artery in an Infant with a Vascular Ring.

Anomalous left common carotid artery is a unique and rare abnormality. Most aortic arch anomalies can be explained by the totipotential aortic arch. Rare anomalies may not fit traditional arch regression models. Echocardiography is key for arch anomaly diagnosis and monitoring. Identification of this very rare anomaly carries significant clinical implications

sj-docx-1-pch-10.1177_21501351231224323 - Supplemental material for Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis

Supplemental material, sj-docx-1-pch-10.1177_21501351231224323 for Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis by Xander Jacquemyn, Tajinder P. Singh, Jeffrey G. Gossett, Konstantin Averin, Shelby Kutty, Liesl J. Zühlke, Leila H. Abdullahi and Aparna Kulkarni in World Journal for Pediatric and Congenital Heart Surgery</p

sj-pdf-2-pch-10.1177_21501351231224323 - Supplemental material for Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis

Supplemental material, sj-pdf-2-pch-10.1177_21501351231224323 for Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis by Xander Jacquemyn, Tajinder P. Singh, Jeffrey G. Gossett, Konstantin Averin, Shelby Kutty, Liesl J. Zühlke, Leila H. Abdullahi and Aparna Kulkarni in World Journal for Pediatric and Congenital Heart Surgery</p

ISHLT consensus statement on donor organ acceptability and management in pediatric heart transplantation

The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted

Prevalence, predictors, and outcomes of cardiorenal syndrome in children with dilated cardiomyopathy: a report from the Pediatric Cardiomyopathy Registry

BACKGROUND: The association of cardiorenal syndrome (CRS) with mortality in children with dilated cardiomyopathy (DCM) is unknown. METHODS: With a modified Schwartz formula, we estimated glomerular filtration rates (eGFR) for children ≥1 year old with DCM enrolled in the Pediatric Cardiomyopathy Registry at the time of DCM diagnosis and annually thereafter, and defined CRS as an eGFR <90 mL/min/1.73 m(2). Children with and without CRS were compared on survival and serum creatinine concentrations (SCr). The association between eGFR and echocardiographic measures was assessed with linear mixed-effects regression models. RESULTS: Of 285 eligible children with DCM diagnosed at ≥1 year of age, 93 were evaluable. CRS was identified in 57 (61.3%). Mean (SD) eGFR was 62.0 (22.6) mL/min/1.73 m(2) for children with CRS and 108.0 (14.0) for those without (P<0.001); median SCr concentrations were 0.9 and 0.5 mg/dL, respectively (P<0.001). The mortality hazard ratio of children with CRS vs. no CRS was 2.4 (95% CI: 0.8-7.4). eGFR was positively correlated with measures of left ventricular function and negatively correlated with age. CONCLUSIONS: CRS in children newly diagnosed with DCM may be associated with higher 5-year mortality. Children with DCM, especially those with impaired left ventricular function, should be monitored for renal disease