Congenital Nephrotic Syndrome and the Heart: Lest We Forget!

We report a 2-month-old girl, presenting with anasarca in the early infancy, and was diagnosed to have congenital nephrotic syndrome. In view of a systolic murmur, echocardiography was done which revealed ostium secundum atrial septal defect and branch pulmonary artery stenosis. Genetic analysis was suggestive of single base pair duplication, resulting into frame shift mutation of NPHS1 (nephrin) gene.

A stent for branch pulmonary artery stenosis after double-lung transplantation in a patient with COVID-19: a case report

Background COVID-19 continues to be a pandemic worldwide. Lung transplantation is the last option to increase life expectancy of end-stage COVID-19 patients. Branch pulmonary artery stenosis (PAS) is a rare complication after lung transplantation with an extremely poor prognosis. The current trend in the management of branch PAS is percutaneous balloon angioplasty and/or stent implantation, rather than high-risk reoperation with a lower success rate. Case summary The subject was a 54-year-old male with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection who underwent a double-lung transplantation. He suffered hypoxemia and right heart dysfunction following the operation. Right cardiac catheterization and pulmonary angiography examination revealed severe stenosis of the right branch pulmonary artery. Due to immunosuppression and reduced coagulation function, the patient underwent pulmonary artery balloon dilatation and stent implantation, and ultimately recovered well. Discussion The combination of balloon dilatation and stent implantation is a good alternative to reoperation for patients with COVID-19.

Combined Liver Kidney Transplant in Adult Patient With Alagille Syndrome and Pulmonary Hypertension

In this article, we describe a case of a 33-year-old female with Alagille syndrome complicated by bilateral branch pulmonary artery stenosis resulting in moderate pulmonary hypertension, end-stage liver disease complicated by portal hypertension, and chronic renal disease who presented for combined liver-kidney transplant. Alagille syndrome is an autosomal dominant disease affecting the liver, heart, and kidneys. Multidisciplinary preoperative evaluation was performed with a team consisting of a congenital heart disease cardiologist, a cardiac anesthesiologist, a nephrologist, and a transplant surgeon. We describe Alagille syndrome and our intraoperative management. To our knowledge, this is the first description of a combined liver-kidney transplant in an adult patient with Alagille syndrome.

Abstract 16377: Cardiovascular Computed Tomography Can Predict Branch Pulmonary Artery Stenosis in Neonates With Ductal Dependent Pulmonary Blood Flow

Background: A subpopulation of neonates with ductal dependent pulmonary blood flow (PBF) are at risk for branch pulmonary artery (PA) stenosis in the PA that receives the patent ductus arteriosus (PDA). Identification of this population is important in planning initial and future interventions. The objective of this study was to identify novel cardiovascular computed tomography (CCT) measures in the preoperative period that are associated with the need for future branch PA surgical or catheter-based intervention. Methods: In this single center retrospective study, we identified neonates (< 28 days) with ductal dependent PBF who underwent CCT prior to initial intervention from 11/2003-12/2019. A single reviewer made conventional and novel measurements of the branch PAs as well as PDA size and configuration. The primary outcome was reached if there was any initial or subsequent surgical or catheter-based intervention on the branch PA that received the PDA. Results: A total of 47 neonates (16 female, 31 male) were included. 30 (64%) reached the primary outcome. Upon multivariable regression analysis, PDA origin at the aortic isthmus [OR 7.13 (95% CI 1.01-50.4), p = 0.049] and distance to the largest diameter (DLD) (Figure 1) [OR 1.24 (95% CI 1.01-1.51), p = 0.040] were independently associated with the primary outcome. DLD is the distance of the largest diameter of the PA with the PDA from the bifurcation in confluent PAs or from the insertion of the PDA in discontinuous PAs. On receiver operating characteristic analysis, area under the curve for DLD = 0.75, p = 0.01. A DLD > 6 mm displayed 82% sensitivity and 67% specificity in identifying patients who met the primary outcome. Conclusions: PDA origin at the aortic isthmus and DLD can risk stratify patients at high risk for requiring surgical or catheterization-based branch PA interventions. Further analysis is needed to determine if intervention strategies based on these measurements improve patient outcomes.