Prognostic role of cardiopulmonary exercise testing in wild-type transthyretin amyloid cardiomyopathy patients treated with tafamidis.

Objectives: Tafamidis has demonstrated survival benefits in transthyretin amyloid cardiomyopathy (ATTR-CM), yet variability in therapeutic response underscores the need for reliable tools to predict outcomes. This study evaluates the prognostic utility of cardiopulmonary exercise testing (CPET) in this population.

Methods: This retrospective study included tafamidis treated wild-type (ATTRwt) CM patients who completed CPET.

A mutation in transthyretin gene in a Mexican patient leading to hereditary amyloidosis: a case report.

Background: Transthyretin is a protein produced by the liver, and when normal, it carries out the role of transporting thyroid hormone and vitamin A in the body. Mutations in the gene that codes for this protein can cause it to misfold. A misfolded protein can not carry out its functions and can also build up in different organs leading to a group of diseases known as amyloidosis.

Evaluating Heart Transplant Outcomes Using the SherpaPak Heart Storage System.

The SherpaPak Cardiac Transport System (SCTS) is a novel hypothermic organ transport device which maintains an optimal temperature range of 4-8°C during donor heart transport. Its use in many major transplant centers has increased over the last several years. We retrospectively examined 120 heart transplant patients, 60 using SCTS and 60 using traditional cold storage on ice (TCS), at the University of Kansas Medical Center (KUMC) between June 2020 and June 2023.

Non-ST elevation myocardial infarction from aortic root thrombus after LVAD placement.

Aortic root thrombosis (ART) is a rare but serious complication following the placement of continuous-flow left ventricular assist devices (CF-LVAD). We present the case of a patient with idiopathic non-ischaemic cardiomyopathy who developed a non-ST elevation myocardial infarction (NSTEMI) secondary to ART after CF-LVAD placement. The patient was treated conservatively with antiplatelet therapy and increased international normalized ratio (INR) goal with resolution of thrombus on repeat imaging.

Rare Variant of Hereditary Amyloid Transthyretin Cardiomyopathy Secondary to Ser97Tyr Mutation.

Hereditary transthyretin amyloidosis (hATTR) is an autosomal dominant, adult-onset disease that stems from point mutations in the TTR gene encoding the protein transthyretin. The disease is progressive and life-threatening and is associated with amyloid deposits in multiple organs including the heart, kidney, skin, eyes, nervous system, and gastrointestinal tract. Genotypic and phenotypic heterogeneity is a characteristic hallmark of hereditary transthyretin amyloidosis.

Paradoxical septic embolism in an Ebstein's anomaly patient leading to brain abscess: A case report.

Unlabelled: Ebstein's anomaly (EA), a congenital cardiac anomaly, is characterized by apical displacement of the tricuspid valve leaflet(s) into the right ventricle. We present the case of a 61-year-old female with a history of EA, Wolff-Parkinson-White syndrome, and patent foramen ovale (PFO), who presented with worsening hypoxia and confusion, in the setting of left lower extremity cellulitis and abscess. The computed tomography (CT) scan of the head showed a cerebellar infarct with hemorrhagic conversion.

Comprehensive review of statin-intolerance and the practical application of Bempedoic Acid.

Statin-intolerance (SI) has prevalence between 8.0 % and 10 %, and muscular complaints are the most common reason for discontinuation. Bempedoic acid (BA), an ATP citrate lyase inhibitor, decreases hepatic generation of cholesterol, upregulates low-density lipoprotein (LDL) receptor expression in the liver, and eventually clears circulating LDL-cholesterol from the blood.

Role of cardiac MRI in predicting the risk of right heart failure in patients who underwent left ventricular assist device implantation.

Despite the advancement in the left ventricular assist devices (LVADs), right heart failure (RHF) remains a challenging adverse event after LVAD implantation and is associated with increased morbidity and mortality. In this study, we sought to assess the role of cardiac magnetic resonance-derived right ventricular ejection fraction (CMR-RVEF) in predicting the risk of post-LVAD RHF. Overall baseline characteristics and clinical outcomes were compared between the patients who developed post-LVAD RHF and those who did not.

Transcatheter aortic valve replacement for aortic insufficiency in a patient with aortic root Thrombus and left ventricular assist device: A risk worth taking?

Unlabelled: A 61-year-old man with end-stage ischemic cardiomyopathy post HeartMate 3 (Abbott laboratories, Chicago, Illinois, USA) left ventricular assist device (LVAD) implant was hospitalized after he had recurrent ventricular tachycardia requiring implantable cardioverter-defibrillator shocks. His transthoracic echocardiogram and computed tomography angiography of the chest showed presence of trace aortic insufficiency (AI) and aortic root thrombus (ART) of non-coronary cusp without obstruction of right or left coronary artery ostium despite therapeutic international normalized ratio. He presented again 3 months later with worsening heart failure signs and symptoms.

Novel Nebulized Milrinone Formulation for the Treatment of Acute Heart Failure Requiring Inotropic Therapy: A Phase 1 Study.

Background: Nonintravenous inotropic-delivery options are needed for patients with inotropic-dependent heart failure (HF) to reduce the costs, infections and thrombotic risks associated with chronic central venous catheters and home infusion services.

Methods: We developed a novel, concentrated formulation of nebulized milrinone for inhalation and evaluated the feasibility, safety and pharmacokinetic profile in a prospective, single-arm, phase I clinical trial. We enrolled 10 patients with stage D HF requiring inotropic therapy during a hospital admission for acute HF.

Cytomegalovirus infection in heart transplant patient presenting as appendicitis.

Unlabelled: Cytomegalovirus (CMV) may manifest in various ways. While immunocompetent hosts may be asymptomatic or present with a mononucleosis-like illness, immunocompromised patients can have organ-specific disease capable of significant morbidity and mortality. CMV appendicitis is a particularly rare presentation.

Trends and Outcomes of Cardiogenic Shock in Patients With End-Stage Renal Disease: Insights From USRDS Database.

Background: There is a paucity of data regarding epidemiology, temporal trends, and outcomes of patients with cardiogenic shock (CS) and end-stage renal disease (chronic kidney disease stage V on hemodialysis).

Methods: This is a retrospective cohort study using the United States Renal Data System database from January 1, 2006 to December 31, 2019. We analyzed trends of CS, percutaneous mechanical support (intraaortic balloon pump, percutaneous ventricular assist device [Impella and Tandemheart], and extracorporeal membrane oxygenation) utilization, index mortality, 30-day mortality, and 1-year all-cause mortality in end-stage renal disease patients.

Endomyocardial biopsy-proven hydroxychloroquine-induced cardiomyopathy in a patient with rheumatoid arthritis.

Hydroxychloroquine is a disease-modifying antirheumatic drug used for various rheumatological conditions. Its long-term use is well-known to have toxic effects on cardiac muscle cells. We present a biopsy-proven case of hydroxychloroquine-induced cardiotoxicity with detailed histopathological and imaging findings.

Impact of Biopsy Proven Liver Fibrosis on Patients Undergoing Evaluation and Treatment for Advanced Heart Failure Surgical Therapies.

There is a paucity of data regarding the impact of liver fibrosis on patients with stage D heart failure (HF). We conducted a retrospective study (January 1, 2017 to December 12, 2020) in patients with stage D HF who underwent liver biopsy as part of their advanced HF therapy evaluation. Baseline characteristics and 1-year outcomes were compared between no- or mild-to-moderate-fibrosis (grade 0 to 2) and advanced-fibrosis (grade 3 to 4) groups.

Loperamide-induced cardiogenic syncope: a case report of a life-threatening presentation of an over-the-counter drug.

Background: Loperamide at supratherapeutic doses can cause cardiac toxicity, presenting as cardiogenic shock, prolonged QT, malignant arrhythmias, or in severe cases sudden cardiac death. Surreptitious loperamide use is difficult to diagnose. We present an interesting case of loperamide use presenting with polymorphic ventricular tachycardia, cardiogenic shock.

Early and aggressive presentation of wild-type transthyretin amyloid cardiomyopathy: A case report.

Background: Wild-type transthyretin amyloidosis (ATTRwt) is the most common form of transthyretin amyloid cardiomyopathy, occurring mostly over age of 60 years (mean age of 80 years). Mean survival without treatment is 3.6 years, making early detection imperative.

Rare case of chronic Q fever myocarditis in end stage heart failure patient: A case report.

Background: Q fever myocarditis is a rare disease manifestation of Q fever infection caused by . It is associated with significant morbidity and mortality if left untreated. Prior studies have reported myocarditis in patients with acute Q fever.

Emergency department use and hospital admissions among adult orthotopic heart transplant patients.

Objective: To study the demographics, clinical presentations, and outcomes of emergency department (ED) visits of patients with heart transplantation (HT) in the United States.

Methods: We performed a secondary analysis of the National Emergency Department Sample database from 2016 to 2018. All ED visits of patients with HT aged ≥ 18 years were identified using International Classification of Diseases, Tenth Revision codes.