Mechanical Support as Failure Intervention in Patients with Cavopulmonary Shunts (MFICS): Rationale and Aims of a New Registry of Mechanical Circulatory Support in Single Ventricle Patients

Department of *Pediatrics, The Cardiac Center, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pa.
Congenital Heart Disease (Impact Factor: 1.2). 03/2013; 8(3). DOI: 10.1111/chd.12053
Source: PubMed

ABSTRACT It is now recognized that a majority of single ventricle patients, those with functionally univentricular hearts, who have survived palliative cavopulmonary connection will experience circulatory failure and end-organ dysfunction due to intrinsic inadequacies of a circulation supported by a single ventricle. Thus, there are an increasing number of patients with functional single ventricles presenting with failing circulations that may benefit from mechanical circulatory support (MCS). The paucity of experience with MCS in this population, even at high volume cardiac centers, contributes to limited available data to guide MCS device selection and management. Thus, a registry of MCS in this population would be beneficial to the field. A conference was convened in January 2012 of pediatric and adult cardiologists, pediatric cardiac intensivists, congenital heart surgeons, and adult cardiothoracic surgeons to discuss the current state of MCS, ventricular assist device, and total artificial heart therapy for patients who have undergone cavopulmonary connection, either superior cavopulmonary connection or total cavopulmonary connection. Specifically, individual experience and challenges with VAD therapy in this population was reviewed and creation of a multiinstitutional registry of MCS/ventricular assist device in this population was proposed. This document reflects the consensus from the meeting and provides a descriptive overview of the registry referred to as Mechanical Support as Failure Intervention in Patients with Cavopulmonary Shunts.

  • [Show abstract] [Hide abstract]
    ABSTRACT: Improved survival, recognition, and management of patients with congenital heart disease have increased the number of pediatric patients with ventricular dysfunction. Many of these patients require mechanical support to bridge to transplant or recovery. Development of pediatric ventricular assist devices has lagged behind adult devices. Until recently, adult devices were used in pediatric patients, with suboptimal results in the smaller patient population. Extracorporeal membrane oxygenation can be life saving, but is a poor choice for long-term support. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) is a paracorporeal device with a variety of pumps for patients of all sizes. The PumpKIN Trial will compare this device to the Infant Jarvik 2000 (Jarvik Heart, New York, NY, USA) in a prospective, randomized study. The single ventricle population presents unique challenges to placement and proper functioning of assist devices. Data are anecdotal and mortality is high. A registry has been developed to assist in caring for this challenging population.
    Artificial Organs 01/2015; 39(1). DOI:10.1111/aor.12439 · 1.87 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Ventricular assist devices (VADs) have revolutionized heart failure management in adults. Recently, VADs have similarly taken a prominent role in the management of end-stage heart failure in children. The purpose of this review is to describe the indications for VADs in children, types of devices available, current outcomes, and future directions of VAD therapy. There has been a dramatic increase in VAD utilization in children over the last decade. For small children, paracorporeal pneumatic pulsatile pumps (e.g., Berlin Heart EXCOR VAD, Berlin Heart GmbH, Berlin, Germany) are most commonly utilized for long-term support. In older children, intracorporeal continuous flow devices (e.g., HeartMate II Left Ventricular Assist System, Thoratec Corporation, Pleasanton, California, USA and HeartWare Ventricular Assist System, HeartWare Incorporated, Framingham, Massachusetts, USA) have been used and allow the possibility of destination therapy. Other devices, such as the total artificial heart, can be utilized for selected patients. Although overall outcomes of pediatric VADs are favorable, complication rates remain high. The utilization of VADs in complex circulations, such as single ventricle patients, remains infrequent and is associated with a high rate of adverse outcomes. VADs are well-established treatment for end-stage heart failure in children. Further investigation is needed to refine patient selection criteria, minimize complications, and develop additional pediatric-specific devices.
    Current opinion in cardiology 11/2013; DOI:10.1097/HCO.0000000000000030 · 2.59 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Mechanical circulatory support has seen numerous advances in the recent years, with important observations made to guide patient selection for the therapy, indications for use, and management of devices after implantation. There is rapid growth in the use of left ventricular assist device therapy (LVAD) for advanced heart failure, with a movement to pursue device intervention earlier in the disease spectrum before comorbidities escalate. With this increase in LVAD use have come new challenges, including unanticipated adverse events and high readmission rates. Simultaneously, complications encountered during LVAD support and an increased number of patients supported with a goal for transplant have had an important effect on the allocation of cardiac allografts. Still, the field continues to evolve and address these challenges in systematic fashion to provide novel solutions and meet the needs of a growing population with advanced heart failure. This has led to an extensive body of literature, ranging from case reports to multicenter clinical trials, which will enhance the future of LVAD technology and patient outcomes. This review summarizes important publications in mechanical circulatory support during the past 24 months.
    The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 02/2014; DOI:10.1016/j.healun.2014.02.014 · 5.61 Impact Factor