Mavacamten Safety and Efficacy in a Heart Transplant Patient Exhibiting Obstructive Hypertrophic Cardiomyopathy Phenotype.

Hypertrophic cardiomyopathy is the most common inherited cardiac disease, exhibiting diverse phenotypes. Obstructive hypertrophic cardiomyopathy occurs in about two-thirds of cases and carries a worse prognosis. Mavacamten use in heart transplant recipients is limited.

Massive Left Ventricular Hypertrophy With Acromegaly: Hypertrophic Obstructive Cardiomyopathy Phenocopy or Phenotype?

Acromegaly-a rare endocrine disorder-results when a growth hormone-secreting somatotroph pituitary adenoma leads to increased insulin-like growth factor 1 production. Acromegaly is known to cause left ventricular hypertrophy. We present a case of acromegaly with massive left ventricular hypertrophy that was determined to be coexistent with gene-positive hypertrophic obstructive cardiomyopathy.

Myocardial work indices in bileaflet mitral valve prolapse patients.

Aims: Mechanisms underlying left ventricular dysfunction and arrhythmogenesis in bileaflet mitral valve prolapse (BMVP) patients are not well defined. Myocardial work index (MWI) is a non-invasive assessment that correlates with myocardial oxygen consumption. We aimed to compare global and regional MWI in BMVP patients with normal controls.

Hypertrophic Cardiomyopathy-Septal Perforator Arteries Doppler Flow Dynamics.

Background: Patients with hypertrophic cardiomyopathy (HCM) have been shown to exhibit abnormal diastolic vessel flow; however, flow pattern profiles and their possible association with different grades of diastolic dysfunction have not been studied. Color Doppler two-dimensional echocardiography permits visualization of the septal perforator arteries, and pulsed-wave Doppler allows recording of diastolic septal artery flow (SAF). Through routine visualization of the septal perforator arteries and acquisition of SAF, we noticed 3 patterns of SAF in patients with HCM.

Challenge in mitral valve intervention: Super-elongated anterior and diminutive posterior mitral valve.

Diminutive posterior mitral valve leaflet with an extremely elongated anterior leaflet is a rare phenomenon. We share such a case discovered in an 82-year-old patient with severe, symptomatic mitral regurgitation. LA, left atrium; LV, left ventricle; MR, mitral regurgitation.

Large Symptomatic Ventricular Fibromas: A Surgical Challenge.

• Cardiac fibromas are benign clinical entities that are rarely diagnosed in adulthood. • Echocardiography is considered the first imaging modality used for evaluation. • Multimodality imaging is critical for diagnosis and perioperative intervention.

Barlow's mitral valve with a dancing chorda.

We present a case of a ruptured mitral valve chorda visualized using the high temporal and axial resolution of transthoracic M-mode echocardiography.

Prevalence of dilated mid-ascending aorta in individuals 15 years and older: In search of optimal diagnostic criteria and their effect on the burden of disease.

Background: Controversy regarding the definition of the upper limit of normal (ULN) for dilated mid-ascending aorta (mAA) stems from variation in criteria, based on several small-sized studies with small datasets of normal subjects (DONS). The present study was carried out to demonstrate this variation in the prevalence of mAA dilation and to identify the optimal definition by creating the largest DONS.

Methods: Echocardiographic studies of patients ≥ 15 years of age performed at a large tertiary care center over 4 years ( = 49,330) were retrospectively evaluated.

Age-Based Outcomes in Patients Who Underwent Septal Reduction Therapy.

There are no national data on age-based outcomes of septal reduction therapy. Using the National Inpatient Sample, we included all adult patients who underwent septal myectomy (SM) or alcohol septal ablation (ASA) from 2005 to 2019. The primary objective was to evaluate the in-hospital mortality and new permanent pacemaker (PPM) placement after SM and ASA in 3 age groups.

Myocardial Work in Apical Hypertrophic Cardiomyopathy.

Background: Pressure-strain loop analysis is a novel echocardiographic technique to calculate myocardial work indices that has not been applied to patients with apical hypertrophic cardiomyopathy (ApHCM). We hypothesized that myocardial work indices differ between patients with ApHCM and those with non-ApHCM. This study aimed to (1) evaluate myocardial work indices in patients with ApHCM compared with those with non-ApHCM, (2) describe associations with relevant clinical variables, and (3) examine associations with significant late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging.

Never Too Old: A Nonagenarian With Apical Hypertrophic Cardiomyopathy.

• We present the oldest living (age, 93 years) asymptomatic patient with marked ApHCM. • ApHCM has a variable prognosis, and the elderly can have a benign course. • Metastatic tumor to the apex should be considered in the differential diagnosis.

Survival Into the Seventh Decade of Life Following Mustard Repair.

Atrial switch procedures (Senning and Mustard) for transposition of the great arteries have largely been abandoned for arterial switch procedures. The number of surviving patients who have undergone atrial switch procedures is declining. We present a case of the oldest known survivor (aged 67 years) of the Mustard procedure.

Hypertrophic cardiomyopathy is associated with dilated sinus of Valsalva: A case-control study.

Background: We aimed to test the hypothesis that there is an association between hypertrophic cardiomyopathy and dilated aorta in a case-control, matched-design fashion.

Methods: Of 65,843 studies done from November 2011 to December 2015, we found, after detailed evaluation by a single author, 153 cases of hypertrophic cardiomyopathy and 3,213 controls who were classified as normal clinically and echocardiographically. Controls were defined as normal patients referred to the echocardiography laboratory with no diagnoses and no known risk factors for dilated aorta (e.

Myocardial Work in Echocardiography.

Myocardial work is an emerging tool in echocardiography that incorporates left ventricular afterload into global longitudinal strain analysis. Myocardial work correlates with myocardial oxygen consumption, and work efficiency can also be assessed. Myocardial work has been evaluated in a variety of clinical conditions to assess the added value of myocardial work compared to left ventricular ejection fraction and global longitudinal strain.

Regional Disparities in the Use of Septal Reduction Therapy and Associated Outcomes in the United States (from a Real-World Database).

The regional differences in the use of septal reduction therapies and the associated outcomes in patients with Hypertrophic obstructive cardiomyopathy (HOCM) are unknown. The primary objective of our study was to evaluate the regional disparities in the use of septal reduction therapies, including septal myectomy and alcohol septal ablation, in patients with HOCM. The secondary objective was to analyze the regional differences in the outcomes in these patients.

Case report: Metastatic melanoma masquerading as apical hypertrophic cardiomyopathy.

Background: Cardiac tumors are usually metastatic. Melanoma is the tumor with the highest rate of cardiac metastasis. Clinicians need to be aware of the metastatic involvement of the left ventricular apex as a differential diagnosis of apical hypertrophic cardiomyopathy.

Refining the upper limit of normal for the ascending aorta: In search of optimal criteria -- a large database study of normal individuals.

Objectives: The cutoff for dilated mid-ascending aorta (mAA) is controversial and has several definitions. The present study was carried out to determine the prevalence of mAA dilation based on published definitions and to identify the optimal cutoff.

Methods: Echocardiographic studies of patients >15 years of age performed at a large tertiary care center over 4 years, = 49,330, were retrospectively evaluated.

Five-Chambered Heart With Double-Chambered Left Ventricle Diagnosed by Multimodality Imaging.

• Left ventricular (LV) diverticulum is a rare cardiac anomaly. • Echocardiographic examination of accessary LV chambers is critical to diagnosis. • Multimodality imaging can aid in ruling out additional congenital heart defects.

Monckeberg calcification of coronary arteries: a string of pearls.

Coronary artery calcium scores are derived from cardiac-gated noncontrast computed tomography scans that are used in cardiac risk stratification. However, an elevated calcium score does not always translate to coronary artery luminal obstruction. Our case demonstrates an extremely high coronary artery calcium score despite nonobstructive coronaries on angiogram.

Clinical Characteristics, Risk Factors, and Outcomes Among a Large Midwestern U.S. Cohort of Patients Hospitalized With COVID-19 Prior to Vaccine Availability.

Purpose: The COVID-19 pandemic posed unprecedented demands on health care. This study aimed to characterize COVID-19 inpatients and examine trends and risk factors associated with hospitalization duration, intensive care unit (ICU) admission, and in-hospital mortality.

Methods: This retrospective study analyzed patients with SARS-CoV-2 infection hospitalized at an integrated health system between February 2, 2020, and December 12, 2020.