CICU Nurse Practitioner The Children's Hospital of Philadelphia PHILADELPHIA, Pennsylvania, United States
Abstract:
Introduction: Infants with single ventricle (SV) circulation remain among the most challenging patients to support with ventricular assist devices (VADs). As these patients are increasingly being considered for heart transplantation (HT), identifying effective methods for VAD support is needed. We report a case of a high-risk SV patient status post hybrid palliation successfully bridged to HT with use of the Berlin Heart and a unique cannulation strategy.
Case Description: A patient prenatally diagnosed with Turner’s syndrome, transverse arch hypoplasia and severe coarctation was born at our hospital. She was started on a prostaglandin infusion and transferred to the Cardiac Intensive Care Unit (CICU). Postnatal imaging showed aortic and mitral valve hypoplasia, small left ventricular size, worsening mild to moderately diminished right ventricle (RV) function and moderate to severe tricuspid regurgitation (TR). She had magnetic resonance imaging showing anomalous pulmonary venous drainage and abnormal lymphatics on T2 imaging. After multidisciplinary review, she underwent hybrid palliation with pulmonary artery (PA) banding and patent ductus arteriosus stenting. Despite optimizing medical therapy, she had persistent respiratory failure and limited improvement in RV function and TR. She was listed for HT at 3 months old. With progressive end organ dysfunction and continued respiratory failure, she was taken for VAD placement with a 15 mL Berlin Heart. In the operating room, there was a 25 mmHg reverse coarctation from the lower to upper extremities. To ensure adequate VAD output to the upper and lower extremities, and to mitigate the effects of arch obstruction, a Hemashield graft was placed from the right innominate artery to the main PA and the Berlin Heart outflow cannula was placed into the graft, with the inflow from the right atrium. She recovered well after VAD placement with normalizing renal and hepatic labs and feeding tolerance. A tracheostomy was also placed to allow for rehabilitation and a decrease in ICU therapies. She underwent HT with PA plasty and anomalous pulmonary vein repair 198 days after being listed and 119 days after her VAD placement.
Discussion: This patient was at high risk for a poor outcome after VAD and HT based on her SV physiology, anomalous pulmonary venous drainage, lymphatic abnormalities, and genetic syndrome. The effective use of a VAD with hybrid circulation was further complicated by a hypoplastic arch, making it difficult to ensure adequate VAD output to the upper and lower extremities. The novel cannulation strategy in which a central shunt graft was placed between the right innominate artery and main PA and cannulated with the Berlin Heart outflow cannula bypassed the effects of arch obstruction. This allowed for improved end organ function, preserved neurologic status and a successful HT.
