Case presentation
The patient is a 56-year-old African American male former smoker with rheumatoid arthritis and pulmonary hypertension who was diagnosed with nonspecific interstitial pneumonia in 2011 after a lung biopsy. He required supplemental oxygen at home and was managed with an immunosuppressive regimen including azathioprine and prednisone. In early 2019 he was admitted with symptoms of congestive heart failure. Transthoracic echocardiogram (TTE) indicated a left ventricular ejection fraction (LVEF) of 45-55% and reduced right ventricular function. Heart catheterization noted an elevated pulmonary artery pressure of 68/31, pulmonary capillary wedge pressure (PCWP) of 23, and Fick cardiac index (CI) of 1.77. Cardiac MRI and TTE displayed pulmonary hypertension with evidence of right heart failure, severe tricuspid regurgitation, and reduced left ventricular function.
Elevated PCWP with depressed biventricular function and CI suggested the need for HLT. While undergoing additional pre-operative workup his clinical status acutely deteriorated despite maximal medical therapy. He was urgently taken to the operating room and successfully placed on peripheral femoral VA-ECMO with a percutaneous technique as a bridge to transplant (BTT). We used a 15 fr Bio-Medicus femoral arterial cannula (Medtronic), 27 fr Bio-Medicus venous femoral cannula (Medtronic), and a distal perfusion catheter with a 5 fr sheath (Cordis). The cannulas were connected to a Cardiohelp system (Maquet) with initial flows started at 3.5 L/min; there was immediate clinical improvement. The cannulas were secured to the skin with several size 0 silk sutures as well as two foley catheter anchoring device for each cannula, preventing any tension or movement.
He was extubated within 12 hours of cannulation. In order to improve and optimize his physical condition, his rehabilitation was focused on nutrition and ambulation A multidisciplinay team composed of perfusion specialists, nurses and physical therapists (PT) guided and monitored the patient during bed mobility, transfers and ambulation. Prior to ambulation, the patient was considered stable in his cardiopulmonary and mental status, without any bleeding and with adequate physical strength and activity tolerance. The cannulated leg was allowed a range of hip flexion from 0-90 degrees given stable ECMO flows. The patient was allowed to sit at the edge of the bed and in a recliner chair with hip flexion no more than 90 degrees as to encouarge posture and upright position throughout the day. Physical exercise included assisted active bed and chair exercises, sitting, standing and walking. Hemodynamics, comfortable respiratory status and safety were the highest priorities. He was standing with PT assistance the day after cannulation, stood for 10 minutes with contact guard assist (CGA), completed pre-gait activites (weight shifting) and sat in the recliner chair. He was also instructed in a seated exercise program. Five days later he needed only minimal assistance to transfer from bed to chair and started ambulating (walked 410 ft with walker and CGA ). Prior to transplant he was able to walk almost 700 ft (Fig.1). Of note, on admission before ECMO cannulation, he was walking just 300 ft, limited by shortness of breath. He did not develop any complications related to ambulation.
On ECMO day 11, a suitable donor became available (fig. 2). He was taken to the operating room for heart-lung block transplant, ECMO decannulation and femoral vessel repair. The chest was left open secondary to coagulopathy and closed the next day. Postoperatively, he was started our standard immunosuppressive regimen.
He was discharged from the hospital on postoperative day 21. The patient continues to do well, with normal hemodynamics on his most recent echocardiography and mild (1R) rejection on biopsy. He is stable on New York Heart Association functional class I status, exercises on a treadmill twenty minutes five days a week, and has resumed normal physical activity.