Publications by authors named "Muhammad A K Nuri"
Increased utilization of the hybrid procedure is not associated with improved early survival for newborns with hypoplastic left heart syndrome: a single-centre experience.
Eur J Cardiothorac Surg
March 2024
Article Synopsis
- - The study aimed to compare the Hybrid and Norwood procedures for treating patients with hypoplastic left heart syndrome and assess their impact on hospital mortality from 1984 to 2022.
- - Over the years, 1,899 patients underwent the Norwood procedure while 82 had the Hybrid procedure, with Hybrid usage rising to 30% by the last analysis period, but it was linked to higher hospital mortality after adjusting for risk factors.
- - Despite the increased adoption of the Hybrid procedure, overall mortality rates have remained stable, suggesting the need for further evaluation of the Hybrid approach in terms of its efficacy.
We report a case of hypoplastic left heart syndrome and with subsequent aortopathy and then found to have hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome due to a germline SMAD4 pathologic variant. The patient's staged palliation was complicated by the development of neoaortic aneurysms, arteriovenous malformations, and gastrointestinal bleeding thought to be secondary to Fontan circulation, but workup revealed a SMAD4 variant consistent with hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome. This case underscores the importance of genetic modifiers in CHD, especially those with Fontan physiology.
View Article and Find Full Text PDFBackground: Children undergoing orthotopic heart transplant (OHT) may require complex reconstruction of superior vena cava (SVC) anomalies. SVC anatomy and mode of reconstruction are potential risk factors for SVC obstruction.
Methods: A retrospective single-center review was conducted of patients undergoing initial OHT between January 1, 1990, and July 1, 2021.
Parkes Weber syndrome is a fast-flow and slow-flow vascular anomaly with limb overgrowth that can lead to congestive heart failure and limb ischemia. Current management strategies have focused on symptom management with focal embolization. A pediatric case with early onset heart failure is reported.
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