This case study presents an urgent intervention involving Right Ventricular Outflow Tract (RVOT) stenting in a 1-month-old infant diagnosed with Double Inlet Left Ventricle (DILV) and Double Outlet Right Ventricle (DORV), successfully addressing severe cyanosis.
Case Summary
A 1-month-old infant presented with severe cyanosis, exhibiting an oxygen saturation of approximately 50%. Clinical assessment and diagnostic imaging confirmed a complex congenital heart defect: Double Inlet Left Ventricle (DILV) with Double Outlet Right Ventricle (DORV). A critical finding was the presence of only a “trickle of forward flow” through the Right Ventricular Outflow Tract (RVOT), indicating a severe obstruction to pulmonary blood flow.
Diagnosis
The primary diagnosis was a combination of two complex congenital heart anomalies:
- Double Inlet Left Ventricle (DILV): This is a rare condition where both atria (the upper chambers of the heart) connect to a single, dominant left ventricle (the lower pumping chamber). This means the heart functions essentially as a single-ventricle system.
- Double Outlet Right Ventricle (DORV): In this defect, both great arteries (the aorta and the pulmonary artery) originate entirely or predominantly from the right ventricle. This can lead to various physiological challenges depending on the relationship of the great arteries to the VSD.
The most critical functional issue in this case was the “trickle of forward flow through the RVOT”. This indicated a severe obstruction or hypoplasia of the RVOT, which is the pathway for blood to exit the right ventricle and reach the pulmonary arteries for oxygenation. This severe obstruction directly led to profound cyanosis due, to insufficient blood flow to the lungs.
Line of Treatment
Given the life-threatening severe cyanosis caused by the inadequate pulmonary blood flow, the immediate decision was to perform RVOT stenting. This interventional cardiology procedure aimed to create a more adequate and stable pathway for blood to flow from the single ventricle to the pulmonary arteries.
The procedure involved:
- RVOT Stenting: A coronary stent was carefully deployed within the Right Ventricular Outflow Tract. The purpose of the stent was to mechanically open and maintain the patency of the obstructed outflow tract, thereby increasing pulmonary blood flow.
Results
Following the RVOT stenting, the child’s oxygen saturation significantly increased, indicating a successful improvement in pulmonary blood flow and alleviation of the severe cyanosis. This immediate palliation stabilized the infant’s critical condition.
The patient is now planned for a Glenn shunt, which represents the next crucial stage in the staged surgical palliation for univentricular hearts. The successful initial palliation with the RVOT stent has made the child a more suitable candidate for this subsequent procedure, which will further optimize the separation of systemic and pulmonary circulations.