Computed Tomographic Angiography Provides Reliable Coronary Artery Evaluation in Infants With Pulmonary Atresia Intact Ventricular Septum.

Evaluate the use of coronary CTA as an initial assessment for determining Right Ventricle Dependent Coronary Circulation (RVDCC) in neonates with Pulmonary Atresia with Intact Ventricular Septum (PA IVS). Retrospective review of cases with coronary CTA and compare with available catheter angiography, pathology, surgical reports, and outcomes from Mar 2015 to May 2022. In our cohort of 16 patients, 3 were positive for RVDCC, confirmed by pathologic evaluation, and there was concordance for presence or absence of RVDCC with catheter angiography in 5 patients (4 negatives for RVDCC, 1 positive). Clinical follow up for the 8 patients that underwent RV decompression had no clinical evidence of myocardial ischemia. Our findings suggest that coronary CTA is reliable as first-line imaging for determination of RVDCC in neonates with PA IVS. These findings, if supported by further prospective study, may reserve invasive coronary angiography for cases with diagnostic uncertainty or at the time of necessary transcatheter interventions.

Neonatal heart transplant outcomes: A single institutional experience.

OBJECTIVE: Neonatal orthotopic heart transplantation was introduced in the 1980s as a treatment for complex congenital heart disease. Progress in single-ventricle palliation and biventricular correction has resulted in a decline in neonatal heart transplant volume. However, limited reports on neonatal heart transplants have demonstrated favorable outcomes. We report the long-term outcomes of patients with neonatal heart transplants at our institution spanning nearly 30 years. METHODS: A retrospective analysis of neonatal heart transplants and neonates listed for transplant was performed at Children's Hospital Colorado. Primary outcomes were early and late survival. Secondary outcomes were rejection episodes, retransplantation, and development of cardiac allograft vasculopathy or post-transplant lymphoproliferative disease. RESULTS: A total of 21 neonates underwent orthotopic heart transplantation at our institution. Among these, 10 neonates were transplanted from 1991 to 2000, 8 neonates were transplanted from 2001 to 2010, and 3 neonates were transplanted from 2011 to 2020. The average age of these patients was 17 days, and the average weight was 3.43 kg. Early survival was 95.2%. Survival at 1 and 5 years was 85.7% (confidence interval [CI], 61.9%-95.2%) and 75% (CI, 45.6%-85.5%), respectively. Of eligible patients, the 10-year and 20-year survival was 72.2% (CI, 45.1%-85.3%) and 50% (CI, 25.9%-70.1%), respectively. CONCLUSIONS: Our institution reports favorable outcomes of neonatal heart transplantation. These results should be considered within the context of outcomes for patients awaiting transplant and the limited donor availability. However, the successful nature of these procedures suggest it may be necessary to reevaluate the indications for neonatal heart transplantation, particularly where risk of mortality and morbidity with palliative or corrective surgery is high.

Aortic stiffness in adolescent Turner and Marfan syndrome patients.

OBJECTIVES: Turner syndrome (TS) and Marfan syndrome (MFS) are partially characterized by aortopathies with a risk of developing severe aortic dilation, stiffness and consequent dissection and aneurysm formation. The incidence of a bicuspid aortic valve (BAV) is also increased in TS. We investigated aortic stiffness in teenage TS and MFS patients and evaluated to what degree stiffness in TS patients is augmented by the presence of a BAV. METHODS: Fifty-seven patients with TS (n = 37) and MFS (n = 20), as well as 22 controls with similar age and size distribution underwent evaluation of thoracic aortic stiffness using phase-contrast magnetic resonance imaging. Calculated stiffness indices including pulse wave velocity (PWV), distensibility and relative area change (RAC) were collected to characterize the ascending aorta and descending aorta. PWV was also determined to evaluate global aortic arch stiffness. RESULTS: Patients with TS had reduced distensibility (0.43 vs 0.58%/mmHg, P < 0.05) and RAC (21 vs 29%, P < 0.01) in the ascending aorta when compared with normal controls. Similarly, patients with MFS had reduced ascending aortic distensibility (0.39 vs 0.58%/mmHg, P < 0.05) and RAC (22 vs 29%, P < 0.05). There were no differences in measured PWV in the ascending aorta. Patients with TS had significantly elevated PWV measured in the aortic arch when compared with controls (2.7 vs 1.9 m/s, P < 0.05). Patients with MFS had more prominent elevation in aortic arch PWV (4.2 vs 1.9 m/s, P < 0.01). The descending aortas had decreased distensibility (0.36 vs 0.55%/mmHg, P < 0.05) and RAC (18 vs 25%, P < 0.01) only in MFS patients. Additionally, 18 TS patients with a BAV were compared with 19 TS patients with a trileaflet aortic valve, without significant differences observed in any of the considered stiffness indices. CONCLUSIONS: TS and MFS teenage patients display evidence of increased aortic stiffness. In TS patients, this is focused in the ascending aorta and is independent of the presence of a BAV. MFS patients display a generalized reduction in compliance of the entire aorta.

Pediatric ECMO outcomes: comparison of centrifugal versus roller blood pumps using propensity score matching.

Centrifugal blood pumps are being increasingly utilized in children supported with extracorporeal membrane oxygenation (ECMO). Our aim was to determine if survival and ECMO-related morbidities in children supported with venoarterial (VA) ECMO differed by blood pump type.Children aged less than 18 years who underwent VA ECMO support from 2007 to 2009 and reported to the Extracorporeal Life Support Organization registry were propensity score matched (Greedy 1:1 matching) using pre-ECMO characteristics.A total of 2,656 (centrifugal = 2,231, roller = 425) patients were identified and 548 patients (274 per pump type) were included in the propensity score-matched cohort. Children supported with centrifugal pumps had increased odds of hemolysis (odds ratio [OR], 4.03 95% confidence interval [CI], 2.37-6.87), hyperbilirubinemia (OR, 5.48; 95% CI, 2.62-11.49), need for inotropic support during ECMO (OR, 1.54; 95% CI, 1.09-2.17), metabolic alkalosis (blood pH > 7.6) during ECMO (OR, 3.13; 95% CI, 1.49-6.54), and acute renal failure (OR, 1.61; 95% CI, 1.10-2.39). Survival to hospital discharge did not differ by pump type.In a propensity score-matched cohort of pediatric ECMO patients, children supported with centrifugal pumps had increased odds of ECMO-related complications. There was no difference in survival between groups.

Outcomes of neonates undergoing extracorporeal membrane oxygenation support using centrifugal versus roller blood pumps.

BACKGROUND: Advances in centrifugal blood pump technology have led to increased use of centrifugal pumps in extracorporeal membrane oxygenation (ECMO) circuits. Their efficacy and safety in critically ill neonates remains unknown. Blood cell trauma leading to hemolysis may result in end-organ injury in critically ill neonates receiving centrifugal pump ECMO. We hypothesized that neonates undergoing ECMO support using centrifugal pumps were at increased odds of hemolysis and subsequent end-organ injury. METHODS: Children 30 days of age or younger who received support with venoarterial ECMO and were reported to the Extracorporeal Life Support Registry during 2007 to 2009 underwent propensity score matching (Greedy matching 1:1) using pre-ECMO support characteristics. RESULTS: A total of 1,592 neonates receiving ECMO (centrifugal pump = 163 and roller pump = 1,492) were identified. Significant differences in demographic, presupport, and cannulation variables were present before matching. One hundred seventy-six neonates who were supported using either centrifugal (n = 88) or roller pumps (n = 88) were matched using propensity scoring. No significant differences in demographic, presupport, or cannulation variables were present after matching. Neonates undergoing support using centrifugal pumps had increased odds of hemolysis (odds ratio [OR], 7.7 [2.8-21.2]), hyperbilirubinemia (OR, 20.8 [2.7-160.4]), hypertension (OR, 3.2 [1.3-8.0]), and acute renal failure (OR, 2.4 [1.1-5.6]). Survival to discharge was not different between pump types. CONCLUSIONS: Use of ECMO using centrifugal pumps is associated with increased odds of hemolysis that likely contributes to other end-organ injury. Research into the optimal use of centrifugal pumps and strategies to prevent support-related complications need to be investigated.

Repair of congenital heart disease: a primer--Part 2.

This review, the second of two parts, describes the repair of aortic arch anomalies, left-to-right shunts, valvular disease, tetralogy of Fallot, and truncus arteriosus. Cardiac transplantation is also discussed. Advances in the surgical management of congenital heart disease have led to improved patient survival and quality of life. Improvements in technology in computed tomography and magnetic resonance imaging have resulted in increasing utilization of cross-sectional imaging in these patients. Perioperative care necessitates that radiologists have a basic understanding of the surgical treatment and the resultant postoperative anatomy. Because many patients with treated congenital heart disease are being followed up into the 4th and 5th decades of life, this is information that will fall within the domain of all radiologists who interpret cross-sectional images of the thorax.

Repair of congenital heart disease: a primer-part 1.

Advances in the surgical management of congenital heart disease have led to enhanced patient survival and quality of life. Improvements in technology in computed tomography and magnetic resonance imaging have resulted in increasing use of cross-sectional imaging in these patients. Perioperative care in these patients requires that radiologists have an understanding of the surgical treatment and the resultant postoperative anatomy. Because many of these patients with treated congenital heart disease are being followed into the 4th and 5th decades of life, this is information that will fall within the domain of the radiologist who deals with adults. This review, which is presented in two parts, covers the major surgical procedures used for the treatment of congenital heart disease, and will be presented in two parts. In part 1, median sternotomy and its complications, palliative procedures, and complex repairs are discussed.

Left main coronary artery-to-pulmonary artery fistula with severe aneurysmal dilatation.

Coronary artery fistulas (CAF) are rare abnormalities that can be symptomatic or asymptomatic. Most drain into the right ventricle or pulmonary artery, though a variety of other drainage sites have been reported. We report the results of the surgical closure of a symptomatic left coronary-to-pulmonary artery fistula associated with a giant 10-cm aneurysm and discuss the management of coronary artery fistulas.