Double inlet left ventricle: Comprehensive echocardiographic anatomy evaluation with special focus on the morphology of atrio-ventricular valves and ventricular septal defect: Case report and literature review
1 Pediatric and Adult Cardiology, Prakash Heart Station, Nirala Nagar, Lucknow, UP, India.
2 Cardiac Technician, Prakash Heart Station, Nirala Nagar, Lucknow, UP, India.
Research Article
World Journal of Biology Pharmacy and Health Sciences, 2024, 18(03), 334–353
Article DOI: 10.30574/wjbphs.2024.18.3.0365
Publication history:
Received on 09 May 2024; revised on 23 June 2024; accepted on 25 June 2024
Abstract:
In double inlet left ventricle (DILV), the ventricle most frequently has left ventricle (LV) morphology, although right ventricle (RV), mixed, indeterminate, or undifferentiated morphologies have been reported. The main ventricle is largely a morphological LV with an outlet chamber connected to it which has a RV morphology. Ventricular septal defect (VSD) or bulboventricular foramen size and anatomy may be variable. The AV valves may be normal, or one of them may be hypoplastic, stenotic, or even atretic. The great arteries are most frequently transposed and the aorta arises from the hypoplastic RV, and the pulmonary artery comes off the main LV chamber. L-transposition of great arteries (L-TGA) happens more often than d-transposition of great arteries (D-TGA). The great vessels are normally related in 30% of cases. Double-outlet right ventricle (DORV) may be seen, in which both great vessels arise from the rudimentary RV. Pulmonary stenosis (PS) is present in two thirds of patients and such stenosis is seen irrespective of the great artery relationship. Rarely subaortic obstruction maybe present in patients with transposition of the great arteries.
Here, we are presenting a case of complex cyanotic congenital cardiac defect of DILV with VSD complicated by L-TGA and severe tricuspid regurgitation (TR) in a 3-year-old male toddler.
Keywords:
Double inlet left ventricle; Single ventricle; Univentricular heart; L-transposition of great arteries; Noncommitted VSD; Remote VSD; Subaortic stenosis.
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