Echocardiographic-morphologic correlations in tricuspid atresia

Area of Science:

• Congenital heart disease
• Pediatric cardiology
• Cardiac morphology

Background:

• Traditional views of tricuspid atresia often describe an imperforate valve.
• Alternative theories suggest tricuspid atresia may resemble double-inlet left ventricle.
• Clarifying the underlying anatomical substrate is crucial for diagnosis and management.

Purpose of the Study:

• To elucidate the anatomical basis of tricuspid atresia.
• To correlate autopsy findings with two-dimensional echocardiographic data.
• To refine the understanding of this congenital heart defect.

Main Methods:

• Examination of 39 autopsy specimens diagnosed with tricuspid atresia.
• Review of 24 two-dimensional echocardiograms from patients with tricuspid atresia.
• Comparative analysis of anatomical features and imaging findings.

Main Results:

• 37 of 39 autopsy specimens showed a muscular floor, indicating absent right atrioventricular (AV) connection.
• Only two specimens had an imperforate tricuspid valve.
• Echocardiograms consistently showed an echo-dense band representing the absent right AV connection.

Conclusions:

• Tricuspid atresia is predominantly characterized by the morphological absence of a right AV connection.
• The ventricular mass typically consists of a dominant left ventricle and a rudimentary right ventricle.
• Findings challenge traditional definitions and emphasize the spectrum of anatomical variations.