Unoperated Double Inlet Left Ventricles : Is There an Anatomical Disposition that Facilitates Favourable Blood Streaming?

Meyer, S.L. (Sophie Laura) (2015) Unoperated Double Inlet Left Ventricles : Is There an Anatomical Disposition that Facilitates Favourable Blood Streaming? thesis, Medicine.

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Abstract

Introduction: Double inlet left ventricle (DILV) patients are currently palliated with the Fontan Procedure. The data on long-term survival with a Fontan circulation are still dire. Natural survivors of DILV, on the other hand, have been reported to live to the sixth decade without surgical intervention. It has been hypothesized that unpalliated patients depend on favourable streaming patterns for separation of deoxygenated and oxygenated blood streams. The aim was to investigate unoperated DILV hearts with a focus on the anatomical relationships facilitating streaming patterns that make certain variants of DILV hearts superior to Fontan palliated hearts regarding function and survival. Methods: A literature review of all (28) unpalliated DILV patients, a retrospective data collection of three patients at the University Medical Centre Groningen and an examination of 42 post-mortem specimens was conducted. Results: Natural survivors presented at a mean age of 46 years, partly due to the historic nature of this cohort. Pulmonary stenosis was present in 18 out of 28 (64,3%) and proved to have a protective effect against pulmonary hypertension. In the ex vivo hearts, the pulmonary artery diameter was on average larger than the aortic diameter. The most common anatomic variant in both the natural survivors and the ex vivo hearts was DILV hearts with a discordant ventriculo-arterial (VA) connection, a left-sided right ventricle and left anteriorly located aorta. In half of the ex vivo hearts the inflow and outflow tracts were arranged in an angular fashion between 10˚ and 80˚. Conclusion: Overall, the anatomy of DILV hearts was very heterogenetic. It was possible to group these hearts according to certain anatomical features, most importantly the VA connection, the location of the right ventricle and the location of the aorta. The relationship of the inflow- and outflow tracts might play a crucial role in keeping the blood streams separated in vivo. Further research into these mechanisms, especially in an in vivo setting, are imperative.

Item Type:	Thesis (Thesis)
Supervisor name:	Ebels, Professor Tjark and Department of Cardiothoracic Surgery and University Medical Centre Groningen
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:42
Last Modified:	25 Jun 2020 10:42
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/354

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