Transthyretin Cardiac Amyloidosis.

Purpose Of Review: Transthyretin (TTR)-related cardiac amyloidosis is a progressive infiltrative cardiomyopathy that mimics hypertensive, hypertrophic heart disease and may go undiagnosed. Transthyretin-derived amyloidosis accounts for 18% of all cases of cardiac amyloidosis. Thus, the study's purpose is to provide a comprehensive review of transthyretin cardiac amyloidosis.

Light-chain cardiac amyloidosis.

Cardiac amyloidosis is an underrecognized condition, in which delays to diagnosis have great implications on management options, prognosis, and morbidity. Once cardiac tissue is infiltrated by amyloid fibrils, there is a cascade of pathologic changes that can display an array of clinical manifestations, from impaired relaxation of the ventricular myocardium to severe restrictive disease or even progressive systolic heart failure. Management is guided not only by recognizing the subtype of amyloidosis (primary, hereditary, and wild-type transthyretin amyloidosis), but also the clinical stage of the disease.

Impact of INTERMACS Profile on Clinical Outcomes for Patients Supported With the Total Artificial Heart.

Background: Insufficient data delineate outcomes for Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile 1 patients with the total artificial heart (TAH).

Methods: We studied 66 consecutive patients implanted with the TAH at our institution from 2006 through 2012 and compared outcome by INTERMACS profile. INTERMACS profiles were adjudicated retrospectively by a reviewer blinded to clinical outcomes.

Transthyretin Cardiac Amyloidosis in Black Americans.

Transthyretin-related cardiac amyloidosis is a progressive infiltrative cardiomyopathy that mimics hypertensive and hypertrophic heart disease and often goes undiagnosed. In the United States, the hereditary form disproportionately afflicts black Americans, who when compared with whites with wild-type transthyretin amyloidosis, a phenotypically similar condition, present with more advanced disease despite having a noninvasive method for early identification (genetic testing). Although reasons for this are unclear, this begs to consider the inadequate access to care, societal factors, or a biological basis.

New Right Bundle Branch Block as a Criterion for Emergent Coronary Angiography.

Context: ST-segment elevations in two or more contiguous leads or new left bundle branch block (LBBB) on electrocardiography (ECG) in a patient with acute onset chest pain are diagnostic criteria for acute myocardial infarction (AMI) and generally warrant urgent coronary angiography and cardiac catheterization. However, the significance of new right bundle branch block (RBBB) without other acute ECG changes is unclear and is currently not considered a criterion.

Case Report: We present a patient with chest pain, positive biomarkers of myocardial necrosis and isolated new right bundle block on ECG.

Pulmonary Hypertension After Heart Transplantation in Patients Bridged with the Total Artificial Heart.

Pulmonary hypertension (PH) among heart transplant recipients is associated with an increased risk of mortality. Pulmonary hemodynamics improves after left ventricular assist device (LVAD) implantation; however, the impact of PH before total artificial heart (TAH) implantation on posttransplant hemodynamics and survival is unknown. This is a single center retrospective study aimed to evaluate the impact of TAH implantation on posttransplant hemodynamics and mortality in two groups stratified according to severity of PH: high (≥3 Woods units [WU]) and low (<3 WU) baseline pulmonary vascular resistance (PVR).

Dobutamine induced eosinophilic myocarditis and right heart failure requiring emergent biventricular assist device implantation.

Dobutamine is a rare and unrecognized cause of eosinophilic myocarditis (EM). It is even more unique for it to cause significant clinical deterioration. Patients with end stage cardiomyopathy have very little cardiac reserve and dobutamine induced myocarditis may cause enough harm to require urgent mechanical circulatory support.