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Clarifying the cardiovascular morphology and associated abnormalities in patients with tricuspid Atresia using
Damandeep Singh1, Niraj Nirmal Pandey2, Mansi Verma1
1Department of Cardiovascular Radiology & Endovascular Interventions, All Institute of Medical Sciences, New Delhi, 110029, India.
Insights
This study analyzed 404 patients with tricuspid atresia, revealing common cardiovascular anomalies like septal defects and pulmonary obstruction. Multidetector CT angiography is crucial for comprehensive pre-surgical evaluation of these complex congenital heart defects.
Area of Science:
- Cardiology
- Radiology
- Congenital Heart Disease
Background:
- Tricuspid atresia is a severe congenital heart defect characterized by the absence of the tricuspid valve.
- Associated cardiovascular anomalies are common and significantly impact patient management and outcomes.
Purpose of the Study:
- To assess the intracardiac morphology and associated cardiovascular anomalies in patients with tricuspid atresia.
- To evaluate the utility of multidetector CT angiography in characterizing these complex cardiac lesions.
Main Methods:
- Retrospective review of multidetector CT angiography datasets from 404 patients diagnosed with tricuspid atresia.
- Detailed analysis of intracardiac morphology, including atrial and ventricular septal defects, ventricular hypoplasia, and great vessel relationships.
- Classification of tricuspid atresia types based on ventriculo-arterial morphology.
Main Results:
- All patients had an atrial septal defect and hypoplastic right ventricle; 99.8% had a ventricular septal defect.
- Common anomalies included pulmonary stenosis (73.26%) and pulmonary atresia (13.6%).
- Other findings included patent ductus arteriosus (24.8%) and right-sided aortic arch (9.4%).
Conclusions:
- Tricuspid atresia frequently presents with a spectrum of cardiovascular anomalies, notably septal defects and pulmonary outflow tract obstruction.
- Multidetector CT angiography provides a comprehensive morphological assessment crucial for surgical and interventional planning in patients with tricuspid atresia.
Abstract:
The present study sought to assess the intracardiac morphology and associated cardiovascular anomalies in patients with tricuspid atresia using multidetector CT angiography. CT angiography datasets of all patients diagnosed with tricuspid atresia at our institution between November 2014 and December 2021 were retrospectively reviewed. The type of tricuspid atresia and associated cardiovascular structural anomalies detected on CT angiography were evaluated. Tricuspid atresia was identified in 404 patients (276 males [68.3%]; mean age at diagnosis: 4.54 ± 6.61 years [range: 9 months-40 years]). Right isomerism was observed in 12/404 (3%) patients followed by situs inversus in 9/404 (2.2%) and left isomerism in 1/404 (0.2%) patients respectively. All patients (404/404; 100%) had an atrial septal defect and a hypoplastic right ventricle while a ventricular septal defect was seen in 403/404 (99.8%) patients. The most common type based on ventriculo-arterial morphology was Type I, with normally related great vessels (78.5%) followed by Type II, with transposed great arteries (21%) and Type III, with common arterial trunk (0.5%). Pulmonary stenosis and pulmonary atresia were seen in 296/404 (73.26%) and 55/404 (13.6%) patients respectively. A right-sided aortic arch with mirror image branching pattern was seen in 38/404 (9.4%) patients. A patent ductus arteriosus was seen in 100/404 (24.8%) patients. Coronary artery anomalies were observed in 34/404 (8.4%) patients. Tricuspid atresia is a congenital heart defect associated with an array of cardiovascular lesions, most commonly septal defects, conotruncal anomalies and pulmonary outflow tract obstruction. A comprehensive evaluation of cardiovascular morphology using CT angiography may thus prove crucial prior to surgery/interventions in these patients.
