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Book

Single Ventricle

Joseph Heaton  1 , Mohamed H. Alahmadi  2 , Laith Rhabneh  3 , Daniel Heller  4
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
.
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Book

Single Ventricle

Joseph Heaton et al.
Free Books & Documents

Excerpt

A functionally univentricular heart, or single ventricle, as defined by Jacobs and Anderson, refers to a condition where 1 ventricle cannot adequately support either systemic or pulmonary circulation. Various anomalies, including hypoplastic left heart syndrome (HLHS) and tricuspid atresia, fall under this classification. The International Pediatric and Congenital Cardiac Code provides standardized terminology for these conditions. The anomalous structure often leads to the mixing of oxygenated and deoxygenated blood through various mechanisms. The condition is typically attributed to genetic factors, though environmental influences can contribute to malformations.

With congenital cardiothoracic surgery advancements, children born with a single ventricle can now live for decades to reach adulthood and beyond. The surgically created univentricular anatomy and physiology enable an orphan ventricle to support systemic circulation as pulmonary blood flows passively in the lungs, a configuration known as the Fontan circulation.

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Conflict of interest statement

Disclosure: Joseph Heaton declares no relevant financial relationships with ineligible companies.

Disclosure: Mohamed Alahmadi declares no relevant financial relationships with ineligible companies.

Disclosure: Laith Rhabneh declares no relevant financial relationships with ineligible companies.

Disclosure: Daniel Heller declares no relevant financial relationships with ineligible companies.

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