Clinical features of Noncompaction Cardiomyopathy

Noncompaction cardiomyopathy (NCCM) is recognized as a separate disease entity since the first report in 1984 of a rare case with persistent myocardial sinusoids and a series of 8 pediatric and adolescent patients in 1990 with increased trabeculation of the left ventricular endocardium. Given the cumulative evidence since then, NCCM is considered as a primary, predominantly genetic disorder of the myocardium. The classic clinical presentation includes severe heart failure, malignant ventricular arrhythmias, thrombo-embolic events and sudden cardiac death. But the complete spectrum comprises also asymptomatic to minor cardiac complaints like atypical chest pain and nonspecific palpitations. The noncompacted myocardium is characterized by an excessively prominent trabecular meshwork and deep intertrabecular recesses, as seen early in human embryogenesis. Therefore, the primary patho-physiological hypothesis is that incomplete compaction of the loose myocardial meshwork during gestation results in a noncompacted, heart, prone to heart failure, arrhythmias and intracardiac thrombus formation. The diagnostic criteria proposed by Jenni et al. include abnormally thickened ventricular walls with a two-layered structure, consisting of thickened, noncompacted (NC) endocardial myocardium and a thin compacted (C) epicardial myocardium (maximal end-systolic ratio NC/C 2 at parasternal short axis view). The diagnosis is established by imaging of the ventricular walls and cavities, classically by two-dimensional transthoracic echocardiography with color Doppler flow. The abnormal structures of NCCM could be identified as deep intertrabec

Patient-reported outcomes in left ventricular assist device therapy:a systematic review and recommendations for clinical research and practice

Background-Technological advancements of left ventricular assist devices (LVAD) have created today's potential for extending the lives of patients with end-stage heart failure. Few studies have examined the effect of LVAD therapy on patient-reported outcomes (PROs), such as health status, quality of life, and anxiety/depression, despite poor PROs predicting mortality and rehospitalization in patients with heart failure. In this systematic review, we provide an overview of available evidence on the impact of LVAD therapy on PROs and discuss recommendations for clinical research and practice. Methods and Results-A systematic literature search identified 16 quantitative studies with a sample size >= 10 (mean +/- SD age=50.1 +/- 12.6 years) that examined the impact of LVAD therapy on PROs using a quantitative approach. Initial evidence suggests an improvement in health status, anxiety, and depression in the first few months after LVAD implantation. However, PRO scores of patients receiving LVAD therapy are still lower for physical, social, and emotional functioning compared with transplant recipients. These studies had several methodological shortcomings, including the use of relatively small sample sizes, and only a paucity of studies focused on anxiety and depression. Conclusions-There is a paucity of studies on the patient perspective of LVAD therapy. To advance the field of LVAD research and to optimize the care of an increasingly growing population of patients receiving LVAD therapy, more well-designed large-scale studies are needed to further elucidate the impact of LVAD therapy on PROs. (Circ Heart Fail. 2011;4:714-723.

Perioperative Levosimendan Infusion in Patients With End-Stage Heart Failure Undergoing Left Ventricular Assist Device Implantation

Left ventricular assist device (LVAD) therapy has been instrumental in saving lives of patients with end-stage heart failure (HF). Recent generation devices have short-to-mid-term survival rates close to heart transplantation. Unfortunately, up to 1 in 4 patients develop a life-threatening right-sided HF (RHF) early post LVAD implantation, with high morbidity and mortality rate, necessitating prolonged ICU stay, prolonged inotropic support, and implantation of a right-ventricular assist device. Pre-operative optimization of HF therapy could help in prevention, and/or mitigation of RHF. Levosimendan (LEVO) is a non-conventional inotropic agent that works by amplifying calcium sensitivity of troponin C in cardiac myocytes, without increasing the intra-cellular calcium or exacerbating ischemia. LEVO acts as an inodilator, which reduces the cardiac pre-, and after-load. LEVO administration is associated with hemodynamic improvements. Despite decades long of the use of LVAD and more than two decades of the use of LEVO for HF, the literature on LEVO use in LVAD is very limited. In this paper, we sought to conduct a systematic review to synthesize evidence related to the use of LEVO for the mitigation and/or prevention of RHF in patients undergoing LVAD implantation