Total artificial heart in the pediatric patient with biventricular heart failure
Approximately 1 in 100,000 children are diagnosed each year with symptoms of cardiomyopathy in the USA. Dilated cardiomyopathy (DCM) is the primary indication, accounting for 65% of heart transplants in children 11-17 years of age. Unfortunately, children awaiting a heart transplant are subject to the highest waiting list mortality in solid-organ transplant medicine in the USA. The Total Artificial Heart has been approved as a bridge to transplant in patients with severe biventricular heart failure. It is the only device that eliminates the symptoms and source of end stage biventricular heart failure. There have been more than 1,200 implants of the Total Artificial Heart, accounting for more than 335 patient years of life on the device.
This case report outlines how surgeons in Phoenix, Arizona, successfully implanted a temporary Total Artificial Heart into a 14-year-old boy with heart failure. The multidisciplinary team determined the heart was the only available choice for the patient because of his conditions. “In selected pediatric patients, the Total Artificial Heart is the assist device of choice for decompensating biventricular heart failure. Commonly encountered VAD (ventricular assist devices) issues, such as valve-related problems, left ventricular clot formation, right heart failure, arrhythmias and need for inotropes, are eliminated with the Total Artificial Heart,” the paper said in its conclusion.
Mechanical circulatory support emerged for the pediatric population in the late 1980s as a bridge to cardiac transplantation. The Total Artificial Heart (TAH-t) (SynCardia Systems Inc., Tuscon, AZ) has been approved for compassionate use by the Food and Drug Administration for patients with end-stage biventricular heart failure as a bridge to heart transplantation since 1985 and has had FDA approval since 2004. However, of the 1,061 patients placed on the TAH-t, only 21 (2%) were under the age 18. SynCardia Systems, Inc. recommends a minimum patient body surface area (BSA) of 1.7 m2, thus, limiting pediatric application of this device.
This unique case report shares this pediatric institution’s first experience with the TAH-t. A 14-year-old male was admitted with dilated cardiomyopathy and severe biventricular heart failure. The pati rapidly decompensated, requiring extracorporeal life support. An echocardiogram revealed severe biventricular dysfunction and diffuse clot formation in the left ventricle and outflow tract. The decision was made to transition to biventricular assist device. The biventricular failure and clot formation helped guide the team to the TAH-t, in spite of a BSA (1.5 m2) below the recommendation of 1.7m2. A computed tomography (CT) scan of the thorax, in conjunction with a novel three-dimensional (3D) modeling system and team, assisted in determining appropriate fit. Chest CT and 3D modeling following implantation were utilized to determine all major vascular structures were unobstructed and the bronchi were open. The virtual 3D model confirmed appropriate device fit with no evidence of compression to the left pulmonary veins. The postoperative course was complicated by a left lung opacification. The left lung anomalies proved to be atelectasis and improved with aggressive recruitment maneuvers. The patient was supported for 11 days prior to transplantation. Chest CT and 3D modeling were crucial in assessing whether the device would fit, as well as postoperative complications in this smaller pediatric patient.
SS Park, DB Sanders, BP Smith, J Ryan, J Plasencia, MB Osborn, CM Wellnitz, RN Southard, CN Pierce, FA Arabia, J Lane, D Frakes, DA Velez, SG Pophal, and JJ Nigro
Total artificial heart in the pediatric patient with biventricular heart failure Perfusion 0267659113496580, first published on July 18, 2013 doi:10.1177/0267659113496580