Jonathan Eaton, MD
Jonathan Eaton, MD
LSU Health Shreveport, United States
Disclosure information not submitted.
Ajit Alexander, MD
CRITICAL CARE CLINICAL FELLOW
Louisiana State University Health Sciences Center, United States
Disclosure information not submitted.
Title: Inoperable CTEPH with decompensated right heart failure and awake VA ECMO as a bridge to transplant
Case Report Body:
Introduction: Inoperable CTEPH is infrequent in the younger population. Management is challenging when patients develop intractable heart failure. Use of awake VA ECMO as a bridge to transplant may be lifesaving.
Description: A 28-year-old African American female with an 11-year history of CTEPH, not a candidate for pulmonary endarterectomy (PEA) with chronic calcification of the right PA, was admitted for shortness of breath and lower extremity edema. She was NYHA class III with 6-meter walk distance of 342 m. She had chronic corpulmonale on triple therapy with selexipag riociguat, bosentan, and warfarin. She had acute on chronic right sided heart failure. Her ECHO showed a dilated hypokinetic right ventricle and paradoxical septal motion with bowing into the left ventricle. She was diuresed but progressed to needing CVVH. She was maintained on triple therapy. Milrinone and norepinephrine were started, and she was monitored with volume assessment, point of care ultrasound, NT proBNP and lactic acid. Despite treatment, she had increasing pressor requirements, atrial arrhythmias, and worsening hypoxia on high-flow nasal prongs. Although previously hesitant for transplant evaluation, she was now in agreement. She was cannulated for venoarterial extracorporeal membrane oxygenation while awake. With local anesthesia only, peripheral access was obtained through her left femoral artery and right femoral vein. Due to complete occlusion of her right pulmonary artery, it was technically difficult to place a PA catheter. IV epoprostenol was initiated with discontinuation of selexipag. Norepinephrine and vasopressin were weaned off. CVVH was continued for volume removal. She was accepted and transferred to a transplant center for evaluation.
Discussion: CTEPH is rare. In decompensated RHF, mechanical support may be the only life-saving option. The primary way to provide hemodynamic support in patients with RV failure with pulmonary hypertension is VA ECMO. Access is usually obtained peripherally with a drainage cannula in the femoral vein and a return cannula in the femoral artery. Central or upper-limb cannulation can also be done. ECMO should be considered if the patient has been evaluated for transplant or has a realistic chance of receiving organs. Awake ECMO is preferred and is associated with better outcomes.