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Transcatheter occlusion of a hepatic vein-to-left atrium fistula: Should we close venovenous collateral vessels following Fontan operation?

ABSTRACT: Right-to-left shunt due to abnormal systemic venous drainage to the left heart is among the causes of hypoxemia following Fontan operation. There are conflicting data regarding the closure of the venovenous collaterals (VVCs) post-Fontan, showing decreased survival in older patients. In a child with visceral heterotaxy, we describe a rare fistula draining a right-sided hepatic vein into hepatic venous plexus and a right-sided pulmonary venous atrium. The patient presented with severe hypoxemia post-Fontan and underwent fistula occlusion with AMPLATZER™ Vascular Plug II, successfully improving hemodynamic status with resolution of the hypoxemia. Younger patients with cyanosis due to VVCs may benefit from percutaneous occlusion post-Fontan.

SUBMITTER: El-Khouri M

PROVIDER: S-EPMC10015390 | biostudies-literature | 2022 Jul-Aug

REPOSITORIES: biostudies-literature

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Transcatheter occlusion of a hepatic vein-to-left atrium fistula: Should we close venovenous collateral vessels following Fontan operation?

El-Khouri Maya M Arabi Mariam M El Rassi Issam I Bulbul Ziad Z Sawaya Fadi F Bitar Fadi F

Annals of pediatric cardiology 20220701 4

Right-to-left shunt due to abnormal systemic venous drainage to the left heart is among the causes of hypoxemia following Fontan operation. There are conflicting data regarding the closure of the venovenous collaterals (VVCs) post-Fontan, showing decreased survival in older patients. In a child with visceral heterotaxy, we describe a rare fistula draining a right-sided hepatic vein into hepatic venous plexus and a right-sided pulmonary venous atrium. The patient presented with severe hypoxemia p ...[more]

PMID: 36935823

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Project description:Myocardial deformation analysis by cardiac MRI (CMR) yielding global circumferential and longitudinal strain (GCS and GLS) is an increasingly utilized method to accurately quantify systolic function and predict clinical events in patients with Fontan circulation. The purpose of this study was to use principal component analysis (PCA) to investigate myocardial temporal deformation patterns derived from strain-time curves to learn about latent strain features beyond peak values. We conducted the study with specific attention to dominant single left or right ventricle (SLV and SRV) morphologies. Methods and Results: Patients remote from Fontan operation who underwent follow-up CMR were analyzed for standard volumetric and function hemodynamics including myocardial deformation parameters including GCS and GLS. We applied PCA to investigate in an unbiased fashion the strain-time curve morphology and to calculate patient specific shape scores. All variables were subjected to single variable Cox regression analysis to detect composite clinical outcome including death, heart transplant, protein losing enteropathy and plastic bronchitis. A total of 122 patients, (SLV = 67, SRV = 55) with a mean age of 12.7 years underwent comprehensive CMR analysis. The PCA revealed 3 primary modes of strain-curve variation regardless of single ventricle morphology and type of strain investigated. Principle components (PCs) described changes in (1) strain-time curve amplitude, (2) time-to-peak strain, and (3) post-systolic slope of the strain-time curve. Considering only SLV patients, GCS was only CMR variable predictive of clinical events (HR 1.46, p = 0.020). In the SRV group, significant CMR predictors of clinical events were derived indexed end-diastolic (HR 1.02, p = 0.023) and end-systolic (HR 1.03, p = 0.022) volumes, GCS (HR 1.91, p = 0.003) and its related first component score (HR 1.20, p = 0.005), GLS (HR 1.32, p = 0.029) and its third component score (HR 1.58, p = 0.017). CMR derived global strain measures are sensitive markers of clinical outcomes in patients with Fontan circulation, particularly in patients with the SRV morphology. Myocardial strain-time curve morphology specific to SLV and SRV patients inspired by unbiased PCA technique can further aid with predicting clinical outcomes.

Dataset Information

Transcatheter occlusion of a hepatic vein-to-left atrium fistula: Should we close venovenous collateral vessels following Fontan operation?

Publications

Transcatheter occlusion of a hepatic vein-to-left atrium fistula: Should we close venovenous collateral vessels following Fontan operation?

Similar Datasets

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