Branch Pulmonary Artery Regurgitation in Repaired Tetralogy of Fallot: Correlation with Pulmonary Artery Morphology, Distensibility, and Right Ventricular Function

Background: The aim was to determine the effect of pulmonary artery (PA) morphology on the branch pulmonary artery-regurgitation fraction (BPA-RF), the relationship of pulmonary insufficiency (PI) to BPA-RF and PA-distensibility, and factors (BPA-RF and PA-distensibility) associated with right ventricular function (RVF) in repaired tetralogy of Fallot (rTOF). Methods: A total of 182 rTOF patients (median age 17.1 years) were analyzed for length, angle of PA, BPA-RF, PI, and PA-distensibility, using magnetic resonance imaging. Results: The left PA had a significant greater RF than the right PA (median (interquartile range)): LPA 43.1% (32.6–51.5) and RPA 35.2% (24.7–44.7), p < 0.001. The LPA was shorter with a narrower angle than the RPA (p < 0.001). The anatomy of the branch-PA was not a factor for the greater LPA-RF (odds ratio, 95% confidence interval: CI, p-value): length 0.44 (0.95–2.00), p = 0.28; angle 0.63 (0.13–2.99), p = 0.56. There was a strong positive correlation between PI and BPA-RF-coefficients (95% CI), p-value: LPA 0.78% (0.70–0.86), p < 0.001; RPA 0.78% (0.71–0.84), p < 0.001 and between BPA-RF and distensibility-coefficients (95%CI), p-value: LPA 0.73% (0.37–1.09), p < 0.001; RPA 1.63% (1.22–2.03), p < 0.001, respectively. The adjusted BPA-RF did not predict RVF, RPA (p = 0.434), LPA (p = 0.268). Conclusions: PA morphology is not a significant factor for the differential BPA-RF. The vascular wall in rTOF patients responds to chronic increased intravascular volume by increasing distensibility. BPA-RF is not a determinant of RVF.

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.

Isolated partial anomalous origin of a branch pulmonary artery from the descending aorta

The condition of partial anomalous origin of a branch pulmonary artery from the descending aorta could be found in several diseases and should be carefully differentiated. We report an unusual case of anomalous systemic arterial supply to normal basal segments of the left lower lung and another case of intralobar pulmonary sequestration. These two cases were treated successfully by transarterial embolisation using the Amplatzer Vascular Plug. We also set up a diagnostic algorithm to differentiate these diseases from anomalous systemic arterial supply to the pulmonary region. It is possible to make the correct diagnosis using the step-by-step diagnostic algorithm and careful interpretation of chest computed tomography angiography.

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.