tricuspid atresia

Tricuspid atresia is a cyanotic congenital cardiac anomaly which is characterized by agenesis of the tricuspid valve and right ventricular inlet. There is almost always an obligatory intra-atrial connection through either an ASD or patent foramen ovale (PFO) in order for circulation to be complete . A small VSD is often also present. In a proportion of cases, tricuspid atresia may also be associated with transposition of great arteries (TGA).

Clinical presentation

ECG

• giant (>5 mm) P waves
  • with peaked morphology in lead II
  • exaggeration of right atrial enlargement morphology
  • referred to as "Himalayan P waves"
• extreme axis deviation
• high left ventricular voltage (HLVV)
  • left ventricular hypertrophy voltage criteria

Pathology

It results from an unequal atrioventricular canal division and the right ventricle is typically very hypoplastic.

Associations

Recognized extra-cardiac associations include:

• right-sided aortic arch
• absent spleen: asplenia

Radiographic features

Plain radiograph

Chest radiographic features may vary depending on the presence and extent of a VSD or TGA. May demonstrate decreased pulmonary vascularity (i.e. oligaemic appearance). Cardiac size may be normal or enlarged.

Echocardiography/ultrasound

Usually the 1^st line imaging modality in utero. It allows direct visualization of the anomaly.

CT and MRI

Allows direct visualization of the anomaly and may typically show a fatty and/or muscular separation of the right atrium from the right ventricle. Cine MRI can offer functional information in addition to anatomy.

Siehe auch:

• Atriumseptumdefekt
• rechts descendierende Aorta
• Transposition der großen Arterien
• Single Ventricle
• Trikuspidalklappenstenose
• cyanotic congenital cardiac anomaly
• Ventrikelseptumdefekt

und weiter:

• Herzfehler
• Varianten der Herzanatomie
• fetal cardiomegaly
• Pulmonalatresie
• Trikuspidalklappe
• Zyanotischer Herzfehler