Ventricular Assist Device Bridge to Heart Transplantation in a Child with Homocystinuria
We present a case of a 15-year-old boy with uncontrolled HCU, who developed ischemic cardiomyopathy and underwent left ventricular assist device placement and subsequently cardiac tra nsplantation.
Amyloid transthyretin (ATTR) cardiac amyloidosis (CA) is an increasingly recognized cause of restrictive cardiomyopathy and associated heart failure with preserved ejection fraction (HFpEF). Despite improved diagnostic techniques to identify this condition, many patients experience delayed diagnosis. Interpretation of routine cardiac biomarkers (i.e. troponins and brain natriuretic peptide (BNP)) and echocardiography in ATTR-CA is limited, particularly in relation to pyrophosphate (PYP) scintigraphy.
Guideline directed medical therapy with beta blockers, ACEi/ARBs, and aldosterone antagonists with the goal of affecting cardiac reverse remodeling (CRR) has previously been the cornerstone of management of patients with heart failure with reduced ejection fraction (HFrEF). More recently, PARADIGM-HF demonstrated the superiority of sacubitril-valsartan to enalapril in reducing HF hospitalizations as well as cardiac and all-cause mortality. Other studies demonstrated that ARNI therapy may enhance CRR to a greater degree than ACEI or ARB therapy alone.
Functional mitral regurgitation (FMR) in non-ischemic cardiomyopathy (NICM) may be partly related to the dysfunction of subvalvular apparatus. Thus, we sought to investigate the correlation between papillary muscle viability and FMR changes in NICM.
Patients with dilated cardiomyopathy (DCM) who achieved left ventricular reverse remodeling (LVRR) have a favorable prognosis, but it is still difficult to precisely predict LVRR in the clinical setting.
The underlying mechanisms of right ventricular (RV) dysfunction in patients with non-ischemic dilated cardiomyopathy (NICM) remain poorly defined. We evaluated a potential correlation between local mechanical dyssynhcrony of interventricular septum (IVS) and RV function in these patients.
Although a significant proportion of cardiomyopathies are determined by genetic factors, the genetic testing of these patients has not been introduced routinely into clinical practice. The aim of this study is the genotyping of patients with advanced cardiomyopathy who have been bridged for heart transplantation or have been already transplanted, in order to evaluate the usefulness of genetic testing in the early diagnosis of the disease, and in the clinical management of these patients and their relatives.
We sought to better characterize differences in transplant outcomes between patients with alcoholic cardiomyopathy and those with other forms of cardiomyopathy in a national cohort.
Marfan syndrome (MS) is an autosomal dominant connective tissue disorder that causes a defect in the gene fibrillin-1. MS patients develop advanced cardiomyopathies, in which previous studies have demonstrated the presence of a discrete cardiomyopathy associated with MS. Given the vascular complications associated with MS and unclear impact immunosuppression may have on systemic vascular disease, little evidence guides suitability of patients with MS for heart transplant. We sought to investigate long-term heart transplant outcomes of patients with MS listed in the UNOS registry.
Postpartum cardiomyopathy (PPCM) is a prevalent cause of end-stage heart failure in women, from which about 10% will require heart transplantation. We aimed to further evaluate long-term heart transplant (HT) outcomes in this cohort of patients and compare them to all other heart transplant recipients.
Approximately half of patients undergoing heart transplant (HTX) have a primary cardiomyopathy. There is growing evidence for a genetic basis in many primary cardiomyopathies, with HFSA guidelines recommending genetics evaluations for many of these. We sought to assess the frequency of genetic disease in a single HTX center.