(Saturday Board 52) Venovenous extracorporeal membrane oxygenation and late ductal stenting for infant with transposition of the great arteries, pulmonary stenosis, and SARS-CoV-2 pneumonia

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Abstract:

Introduction: The SARS-CoV-2 pandemic has affected medical decision-making in all practice areas, including the pediatric cardiac intensive care unit (CICU), sometimes necessitating the use of innovative management strategies. Venovenous extracorporeal membrane oxygenation (VV-ECMO) and, particularly, late ductal stenting are infrequently applied interventions in the CICU. Here we present the case of a critically ill infant with d-transposition of the great arteries (d-TGA), ventricular septal defect (VSD), pulmonary stenosis (PS), and patent ductus arteriosus (PDA), in which VV-ECMO and late ductal stenting were utilized successfully in the setting of active SARS-CoV-2 infection to treat worsening PS and pulmonary venous desaturations, thereby delaying surgical intervention and its associated risks during active infection.

Case Description: A 3 month old male with history of d-TGA, VSD, and PS, initially managed with a balloon atrial septostomy at birth, was admitted to the CICU after presenting to the emergency department with respiratory distress and hypoxemia. He was found to be SARS-CoV-2 positive, requiring only nasal cannula for respiratory support at time of admission. Initial echocardiogram demonstrated known d-TGA, VSD, severe pulmonary stenosis (peak gradient 95-110mmHg), and unrestrictive atrial communication with preserved systolic function. An incidental finding of a tiny, hemodynamically insignificant PDA was also noted. While admitted, the patient exhibited intermittent, severe desaturations with escalating respiratory support. In light of his tiny PDA and desaturation episodes, he was started on a prostaglandin infusion to promote additional pulmonary blood flow through the PDA, limiting the severity and frequency of desaturations. However, the patient became severely hypoxemic, despite multiple interventions including invasive mechanical ventilation, fluid administration, vasoactive infusions, and neuromuscular blockade. Despite being severely hypoxemic, the echocardiogram demonstrated preserved ventricular function, so the decision was made to escalate care to VV-ECMO therapy. Following ECMO cannulation, the patient’s hypoxemia quickly resolved, and he remained hemodynamically stable. Given the persistence of his small PDA and the desire to avoid the risks of cardiac surgery in the setting of acute COVID infection, percutaneous intervention to augment pulmonary blood flow was attempted. Despite its diminutive size, his PDA was able to be successfully cannulated and stented the day after his ECMO cannulation. After ductal stent placement, he was able to be quickly weaned from ECMO support and was decannulated the following day. He was subsequently extubated and ultimately discharged home with planning for definitive surgical intervention underway.

Discussion: Here we present an interesting case of an infant with d-TGA, VSD, PS, and PDA in which VV-ECMO and PDA stenting were successfully applied to treat acute hypoxemia in the setting of SARS-CoV-2 infection and severe pulmonary stenosis. These therapies may be considered in appropriate patients for whom the risks of cardiac surgery are significant.