Race, Genotype, and Prognosis in Black Patients With Transthyretin Cardiac Amyloidosis.

Previous studies suggest worse outcomes in patients with variant transthyretin cardiac amyloidosis (ATTR-CA) because of valine-to-isoleucine substitution at Position 122 (V122I) (ATTRv-CA) compared with patients with wild-type (WT) disease (ATTRwt-CA). Given V122I is almost exclusively found in Black patients, it is unclear if this is attributable to the biology of genotype or racial differences. Patients with ATTR-CA diagnosed between January 2001 and August 2021 were characterized into 3 categories: (1) White with ATTRwt-CA (White-WT); (2) Black with V122I ATTRv-CA (Black-V122I), and (3) Black with ATTRwt-CA (Black-WT).

A Review of Current and Evolving Imaging Techniques in Cardiac Amyloidosis.

Purpose Of Review: Establishing an early, efficient diagnosis for cardiac amyloid (CA) is critical to avoiding adverse outcomes. We review current imaging tools that can aid early diagnosis, offer prognostic information, and possibly track treatment response in CA.

Recent Findings: There are several current conventional imaging modalities that aid in the diagnosis of CA including electrocardiography, echocardiography, bone scintigraphy, cardiac computed tomography (CT), and cardiac magnetic resonance (CMR) imaging.

Multicenter registry and test bed for extended outpatient hemodynamic monitoring: the hemodynamic frontiers in heart failure (HF) initiative.

Background: Hemodynamic Frontiers in Heart Failure (HF) is a multicenter academic research consortium comprised of 14 US institutions with mature remote monitoring programs for ambulatory patients with heart failure (HF). The consortium developed a retrospective and prospective registry of patients implanted with a wireless pulmonary artery pressure (PAP) sensor.

Goals/aims: HF registry collects demographic, clinical, laboratory, echocardiographic (ECHO), and hemodynamic data from patients with PAP sensors.

Changes in Left Ventricular Ejection Fraction and Clinical Trajectories of Transthyretin Cardiac Amyloidosis with Systolic Dysfunction.

Background: Transthyretin cardiac amyloidosis (ATTR-CM) is classically thought of as a progressive disease with preserved systolic function. The longitudinal clinical trajectories of ATTR-CM with impaired left ventricular ejection fraction (LVEF) remain unclear.

Methods: This is a single-center retrospective cohort study of consecutive patients with ATTR-CM who underwent two or more echocardiograms with baseline LVEF < 50%.

Convolution Neural Network Algorithm for Shockable Arrhythmia Classification Within a Digitally Connected Automated External Defibrillator.

Background Diagnosis of shockable rhythms leading to defibrillation remains integral to improving out-of-hospital cardiac arrest outcomes. New machine learning techniques have emerged to diagnose arrhythmias on ECGs. In out-of-hospital cardiac arrest, an algorithm within an automated external defibrillator is the major determinant to deliver defibrillation.

Impact of exercise on pulmonary artery pressure in patients with heart failure using an ambulatory pulmonary artery pressure monitor.

Background: In this multicenter prospective study, we explored the relationship between pulmonary artery pressure (PAP) at rest and in response to a 6-min walk test (6MWT) in ambulatory patients with heart failure (HF) with an implantable PAP sensor (CardioMEMS, Abbott).

Methods: Between 5/2019 and 2/2021, HF patients with a CardioMEMS sensor were recruited from seven sites. PAP was recorded in the supine and seated position at rest and in the seated position immediately post-exercise.

Multicenter validation of a machine learning phase space electro-mechanical pulse wave analysis to predict elevated left ventricular end diastolic pressure at the point-of-care.

Background: Phase space is a mechanical systems approach and large-scale data representation of an object in 3-dimensional space. Whether such techniques can be applied to predict left ventricular pressures non-invasively and at the point-of-care is unknown.

Objective: This study prospectively validated a phase space machine-learned approach based on a novel electro-mechanical pulse wave method of data collection through orthogonal voltage gradient (OVG) and photoplethysmography (PPG) for the prediction of elevated left ventricular end diastolic pressure (LVEDP).

Identifying novel phenotypes of elevated left ventricular end diastolic pressure using hierarchical clustering of features derived from electromechanical waveform data.

Introduction: Elevated left ventricular end diastolic pressure (LVEDP) is a consequence of compromised left ventricular compliance and an important measure of myocardial dysfunction. An algorithm was developed to predict elevated LVEDP utilizing electro-mechanical (EM) waveform features. We examined the hierarchical clustering of selected features developed from these EM waveforms in order to identify important patient subgroups and assess their possible prognostic significance.

Cardiac amyloidosis: an update on diagnosis, current therapy, and future directions.

Purpose Of Review: This review aims to serve as a practical guide for differentiating the two most common forms of cardiac amyloidosis, as well as reviewing the approach to diagnosis and management, particularly as it pertains to transthyretin cardiac amyloidosis.

Recent Findings: Emerging literature continues to unravel new understandings and challenges in the field of cardiac amyloidosis. Although cardiac amyloidosis has historically been thought of as a 'zebra diagnosis', current evidence has shown that this is a common cause of heart failure.

Framework for Digital Health Phenotypes in Heart Failure: From Wearable Devices to New Sensor Technologies.

Consider these 2 scenarios: Two individuals with heart failure (HF) have recently established with your clinic and followed for medical management and risk stratification. One is a 62-year-old man with nonischemic cardiomyopathy due to viral myocarditis, an ejection fraction (EF) of 40%, occasional rate-limiting dyspnea, and comorbidities of atrial fibrillation and hypertension. The other is a 75-year-old woman with ischemic cardiomyopathy, an EF of 35%, a prior hospitalization 6 months ago, and persistent symptoms of edema and orthopnea.

Cardiac Amyloidosis Screening at Trigger Finger Release Surgery.

Cardiac amyloidosis is often preceded by orthopedic manifestations such as carpal tunnel syndrome, and 10% of patients who underwent idiopathic carpal tunnel release surgery will have biopsy-confirmed amyloid deposits in the tenosynovial sheath. Trigger finger is also commonly reported in patients with amyloidosis and involves the same tendon sheath as carpal tunnel syndrome, but the prevalence of amyloid deposition is unclear. This prospective cross-sectional study enrolled 100 patients aged ≥50 years at the time of surgery for idiopathic trigger finger.

Disparities in heart and lung transplantation.

Purpose Of Review: Cardiothoracic transplantation is the definitive therapy for end-stage heart and lung disease. In service to this population, disparities in access and care must be simultaneously understood and addressed.

Recent Findings: There are sex, race, geographic, age, and underlying disease disparities in both heart and lung transplantation.

A Critical Review of Hemodynamically Guided Therapy for Cardiogenic Shock: Old Habits Die Hard.

Purpose Of Review: Here, we review the importance of using hemodynamic data to guide therapy and risk stratification in cardiogenic shock as well as the various definitions of this syndrome that have been used in prior studies. Furthermore, we provide perspective regarding the controversy surrounding pulmonary artery (PA) catheter use as well as current society guidelines and scientific statements. Lastly, we review the technical aspects for accurate interpretation of data of cardiogenic shock.

Predicting cardiac disease from interactions of simultaneously-acquired hemodynamic and cardiac signals.

Background And Objective: Coronary artery disease (CAD) and heart failure are the most common cardiovascular diseases. Non-invasive diagnostic testing for CAD requires radiation, heart rate acceleration, and imaging infrastructure. Early detection of left ventricular dysfunction is critical in heart failure management, the best measure of which is an elevated left ventricular end-diastolic pressure (LVEDP) that can only be measured using invasive cardiac catheterization.

Rare survival of left ventricular free wall rupture following myocardial infarction.

Myocardial free wall rupture is a rare but usually fatal complication of acute myocardial infarction (MI) especially if it occurs out of hospital and occurs in 2-4% of patients who suffer from acute MI. Rapid diagnosis is essential but not always easy as diagnostic tests may be inconclusive. In this case report authors examine a rare and unique patient survival after left ventricular free wall rupture following MI.

The Most Expensive Drug in the World: To Continue or Discontinue, That Is the Question.

Tailoring treatment based on genetics and medical history may be preferable for patients with atypical hemolytic uremic syndrome who face lifelong therapy on an expensive medication and increased risk of infection.