Interdiscip Cardiovasc Thorac Surg
. 2024 Jul 22:ivae136.
doi: 10.1093/icvts/ivae136. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/39037933/
Giant Morgagni Hernia and Aorto-Pulmonary Collaterals in a Loeys-Dietz Patient Undergoing Surgery for Aortic Root Aneurysm and Mitral Valve Prolapse
Federica Lo Presti 1, Giuseppe Palmiero 2, Giuseppe Limongelli 2, Alessandro Della Corte 1
Affiliations expand
- PMID: 39037933
- DOI: 10.1093/icvts/ivae136
Free article
Abstract
The case of a Loeys-Dietz Syndrome patient undergoing mitral valve repair and composite aortic root and valve replacement is here described: pre-operative CT scan unraveled a previously misdiagnosed Morgagni hernia (anterior diaphragmatic), containing omentum only, compressing the right ventricle. Intra-operatively, an abnormal oxygenated blood backflow into the left ventricle was observed, post-operatively found to be caused by major aorto-pulmonary collateral arteries. This is the first case of Morgagni hernia and systemic-pulmonary shunt ever reported associated with Loeys-Dietz Syndrome. These congenital features may be important in both phenotyping and surgical management.
Keywords: Loeys-Dietz Syndrome; Morgagni hernia; aortic root aneurysm; aorto-pulmonary collateral arteries; mitral regurgitation.
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.
