Tricuspid atresia

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Classification according to ICD-10
Q22.4 Congenital tricuspid valve stenosis
ICD-10 online (WHO version 2019)

The tricuspid atresia is the lack of conditioning ( atresia ) of the tricuspid valve between the right atrium and right ventricle (right ventricle). It is a congenital heart malformation that belongs to the single ventricle . In the place of the tricuspid valve there is an impermeable membrane. The venous blood can only enter the left atrium through an atrial septal defect. The malformation results in an insufficient supply of oxygen to the body and thus a cyanotic heart defect is present. Tricuspid atresia is one of the congenital cyanotic heart defects.

Malformations and Consequences

The right ventricle is usually considerably underdeveloped and there is a ventricular septal defect .
The blood from the inferior and superior vena cava flows through an atrial septal defect into the left atrium. From there it goes into the enlarged left ventricle, which usually works like a single chamber together with the not completely separated right ventricle. The newborns have cyanosis because venous and arterial blood mixes. In addition, a can Pulmonalstenose present which limits the flow to the lungs. If pulmonary stenosis does not exist and it does not develop over time, there is a risk of developing pulmonary hypertension because the large left ventricle supplies the aorta and pulmonary artery with the same high pressure.

In about 20% of cases there is also a transposition of the large arteries , which is used for typing.

Rashkind maneuvers for tricuspid atresia IB

Types

The basic classification is based on the position of the large arteries:

  • Type I: normal position of the great arteries
  • Type II: d -transposition of the great arteries
  • Type III: l -Transposition of the great arteries
  • Type IV: Truncus arteriosus communis

In addition, there is a supplementary secondary typing, which indicates the flow status to the lungs:

Diagnosis

therapy

Initial

Additionally

  • If pulmonary atresia is present or pulmonary stenosis restricts the inflow to the lungs too much, the creation of an aorto-pulmonary shunt ( e.g. a Blalock-Taussig anastomosis ) can improve the situation.
  • If there is a risk of "pulmonary hypertension" because there is no pulmonary stenosis, pulmonary banding may be necessary.

Finally

  • The Fontan operation with its modifications is the surgical procedure used today. If there is a previous operation, an aorto-pulmonary shunt is then lifted again or a "pulmonary banding" is released.

Long-term expectations

After the Fontan operation, the children are no longer cyanotic. The everyday resilience is usually quite good. Cardiac arrhythmias can occur in the long term .
Regular check-ups and compliance with endocarditis prophylaxis are necessary for life.

literature

  • Reinhard Larsen: Anesthesia and intensive medicine in cardiac, thoracic and vascular surgery. (1st edition 1986) 5th edition. Springer, Berlin / Heidelberg / New York et al. 1999, ISBN 3-540-65024-5 , pp. 377-380.

Individual evidence

  1. Keck; Hausdorf (Ed.): Pediatric Cardiology . 5th edition. Urban & Fischer, Munich; Jena 2002, ISBN 3-437-21960-X , p. 227 .
  2. Thomas Borth-Bruhns, Andrea Eichler: Pediatric cardiology . 1st edition. Springer, Berlin 2004, ISBN 978-3-540-40616-7 , pp. 191 ff .