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Clarifying the Cardiovascular Morphology and Associated Abnormalities in Patients with Double Inlet Left Ventricle
Niraj Nirmal Pandey1, Mansi Verma2, Sheetal Sharma2
1Department of Cardiovascular Radiology & Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, 110,029, India. nirajpandey2403@gmail.com.
Insights
Double inlet left ventricle (DILV) is a congenital heart defect often accompanied by other cardiovascular issues. Multidetector CT angiography effectively visualizes these complex intracardiac anomalies, aiding surgical planning.
Area of Science:
- Cardiovascular Imaging
- Congenital Heart Disease
- Pediatric Cardiology
Background:
- Double inlet left ventricle (DILV) is a complex congenital heart defect characterized by a functionally single ventricle.
- Associated cardiovascular anomalies are common in DILV and significantly impact patient management and surgical outcomes.
Purpose of the Study:
- To evaluate the intracardiac morphology and associated cardiovascular anomalies in patients with DILV using multidetector CT angiography.
- To assess the utility of multidetector CT angiography in the detailed anatomical evaluation of DILV and its related abnormalities.
Main Methods:
- Retrospective review of cardiac CT angiography scans from January 2014 to January 2023.
- Systematic evaluation of intracardiac morphology and cardiovascular abnormalities in 97 patients with DILV, excluding those with a common atrioventricular valve.
Main Results:
- All 97 patients exhibited a hypoplastic right ventricular cavity.
- Transposition of great arteries was the most frequent ventriculo-arterial configuration (61.9%).
- Pulmonary outflow tract obstruction (46.4%) and aortic arch anomalies (11.3%) were commonly observed associated anomalies.
Conclusions:
- DILV is frequently associated with diverse cardiovascular anomalies, notably transposition of great arteries and pulmonary outflow tract obstruction.
- Multidetector CT angiography is a valuable tool for comprehensive intracardiac anatomical assessment and identification of associated anomalies in DILV, guiding surgical strategies.
Abstract:
The present study sought to evaluate the intracardiac morphology and associated cardiovascular anomalies in patients with double inlet left ventricle (DILV) on multidetector CT angiography. A retrospective search of the electronic departmental database was conducted from January 2014 to January 2023 to identify patients with a diagnosis of DILV on cardiac CT angiography. Patients with a common atrioventricular valve were excluded. The scans were systematically reviewed in a sequential segmental fashion for evaluating the intracardiac morphology and associated cardiovascular abnormalities. DILV was identified in 97 patients (74 males; mean age: 5.15 years). A hypoplastic right ventricular cavity was seen in all patients. The most prevalent ventriculo-arterial configuration was transposition of great arteries seen in 60/97 (61.9%) patients. Concordant ventriculo-arterial connections (Holmes heart) were observed in 17/97 (17.5%) patients while double outlet right ventricle and origin of aorta from right ventricle with pulmonary atresia were seen in 8/97 (8.2%) patients respectively. Aortic arch anomalies were seen in 11/97 (11.3%) patients. Type A interrupted aortic arch was present in 1 (1%) patient, and 2 (2.1%) patients had aortic stenosis. Some form of pulmonary outflow obstruction was seen in 45/97 (46.4%). Pulmonary atresia was observed in 11/97 (11.3%) patients out of which 1 had non-confluent pulmonary arteries. Pulmonary venous anomalies were observed in 4/97 (4.1%) patients. A single coronary artery was seen in 6/97 (6.2%) patients. DILV is a congenital heart disease with functionally single ventricle and is associated with a wide variety of cardiovascular anomalies, most commonly transposition of great arteries and pulmonary outflow tract obstruction. Multidetector CT angiography is an excellent modality for detailed intracardiac anatomical evaluation and depiction of associated cardiovascular anomalies which may aid in deciding the surgical approach.
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