Day 2 :
Transplant Surgeon Papworth Hospital, United Kingdom
Time : 09:30-10:15
Marius Berman is a Consultant Cardiothoracic and Transplant Surgeon at Royal Papworth Hospital, Cambridge, UK. His main interests are management of acute cardiogenic shock, and inter-hospital transfer of patients on VA ECMO as bridge to recovery or therapy.
In an era of PPCI, mortality due to AMI has fallen substantially over the past three decades. Nevertheless, patients with post-infarction ventricular septal defect (PIVSD) carry a grim prognosis and resource demanding. The management of PIVSD is complicated, and requires substantial critical care, imaging, interventional, and surgical expertise. It is therefore advisable, when clinically feasible, to transfer these patients to regional centers with adequate individual experience in the care of these patients. Traditionally, the main stream of treatment was surgery, pending hemodynamically stability and size of left to right shunt. There is no clear evidence to guide the surgical management of patients who are in shock, as all approaches have shown extremely high mortality. Possible strategies include emergency surgery, a period of mechanical circulatory support in the form of IABP or ECMO, prior to a delayed surgical or percutaneous intervention, or emergency placement of a percutaneous closure device to reduce the shunt. Often, there is a natural selection when pathway chosen was optimized, with those surviving a healing phase proceeding to therapy. Percutaneous closure may also be a viable option for patients in the sub-acute to chronic period whose comorbidities preclude surgical repair, and whose septal anatomy is favorable to device placement. We encounter incidences of percutaneous closure post-surgical closure where the patch dehisced due to further progression of the ischemic insult. We favor the establishment of a multidisciplinary PIVSD team, including interventional cardiologist, cardiac surgeon, anesthetist and radiographer in order to tailor patient specific treatment based on presenting symptoms and co-morbidities.
University of Mississippi Medical Center, USA
Charles H Gaymeswas has completed his graduation from High School with top honors and attended the University of the West Indies Medical School, graduating class of 1978. He completed Residency in Pediatrics at the University of Mississippi Medical Center in 1989 and was “Outstanding Resident” in 1989. He completed his Pediatric Cardiology Residency at MUSC, South Carolina in 1992. He has been on faculty at UMMC 1992 – Present. He was “Outstanding Pediatric Faculty Member” in 1995. Currently, he practices as a Professor of Pediatrics/Cardiology and Director of Children Arrhythmia Services.
Introduction: Ninety-five percent of hypertrophic cardiomyopathy are related to defects present in four genes MYH7, MYBPC3, TNNT2 and TNN13. The cause of sudden cardiac arrest (SCA) in hypertrophic cardiomyopathy has not been determined. The guidelines for risk stratification for SCA and AICD implant from the ACC and AHA are not based on any large studies in children. Anecdotal cases do not support their usefulness. We report our data base analysis of a cohort of patients with HCM followed at a single center from 1995-2017 and found no consistent risk factors for SCA.
Methods: We reviewed our database for all hypertrophic cardiomyopathy patients followed by pediatric cardiac electrophysiology. Data was tabulated for presenting symptoms, EKG finds, ambulatory monitoring, and echocardiogram measurements. In decreased patients, autopsy results were also compiled and tabulated.
Results: Total of 57 candidates (37 male and 20 female) of age 1-29 (mean 19) years with 3 syncope 6 SCA as first symptom (3 resuscitated) and palpitations 20/57 were included in the study. 7/57 had family history of HCM 3/57 (SCA). 16/57 had no sustained VT (ambulatory monitor). 5/47 AICD received appropriate therapy (only 1/5 met guidelines for AICD) 2/47 patients inappropriate therapy. 1/47 with AICD died from ventricular fibrillation (patient within guidelines for AICD).
Conclusions: We could not identify specific criteria for risk stratification of SCA/AICD implantation in our population of patients with hypertrophic cardiomyopathy. The current guidelines are not sensitive or specific enough in children to guide AICD implant. The risk for SCA likely resides in the cellular dysfunction and may be related to the genetics. Until larger studies could better risk stratify SCA the decision for AICD should be discussed with the patient and a decision made even if the guidelines are not met.