Single ventricle

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Classification according to ICD-10
Q20.4 Double inlet ventricle.
Singular ventricle
ICD-10 online (WHO version 2019)

In medicine, a single ventricle or single-chamber heart is a form of congenital heart malformations in which only one functioning heart chamber ( ventricle ) has formed during fetal development . It encompasses very diverse forms, which also have different effects on the circulatory system and the therapeutic approach. What all forms have in common is that there really is only one heart chamber or a main chamber that receives its blood from both atria with a "secondary chamber" that receives its blood from the main chamber. Another criterion is the access via the heart valves and thus the inflow and outflow of the chamber.

to form

A distinction is made between two basic forms, depending on whether the right ventricle ( pulmonary circulation ) or the left ventricle ( body circulation ) has not developed sufficiently. After the ventricle, these are then referred to as single ventricles with a functional left ventricle or a functional right ventricle.

After this initial assignment, the actual causes of development are also named as diagnoses:

  • Single ventricle with functional left ventricle ( the right ventricle is not sufficiently developed )
    • Double inlet left ventricle (= DILV) - Both AV valves or a common AV valve open into the left ventricle.
    • Double outlet left ventricle (= DOLV) - the aorta and pulmonary artery leave the functionally effective left ventricle completely or more than 50%
    • Pulmonary atresia
    • Tricuspid atresia
  • Single ventricle with functional right ventricle ( the left ventricle is not sufficiently developed )

therapy

Surgical therapy in the form of palliative interventions and an operation that improves the situation depends on the exact anatomical constellation and is decided individually for each child.

  • Fontan operation for malformations of the right ventricle, especially closure of the tricuspid and / or pulmonary valve
  • Norwood surgery for malformations of the left ventricle, especially hypoplastic left heart syndrome
  • Heart transplantation as an option if both of the aforementioned operations are not promising and the child's condition requires it.

literature