Search

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. (Level of Difficulty: Intermediate.)

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

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. (Level of Difficulty: Beginner.)

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.g., aortic stenosis, hypertension, aortic regurgitation). Clinical chart review showed none of the risk factors for dilated aorta, and echocardiography did not reveal any abnormalities. Of these 3,213 patients, 153 controls were matched to cases by age and sex by propensity score. Dilated aorta was defined according to clinical, Goldstein, and Lang's criteria. Results: The prevalence of a dilated sinus of Valsalva was 9 times higher in hypertrophic cardiomyopathy patients than controls (OR = 9.4, P = 0.003). The 9-fold higher prevalence in hypertrophic cardiomyopathy patients persisted after adjusting for height, weight, and aortic pathology. Association of dilated mid-ascending aorta with hypertrophic cardiomyopathy was significant after adjustment for height and body surface area but became borderline insignificant after adjusting for weight and aortic valve pathology. Conclusion: Hypertrophic cardiomyopathy appears to be associated with a dilated sinus of Valsalva, even after adjusting for height, weight, and aortic valve pathology.

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.

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. Patient characteristics and clinical outcomes were obtained from medical records. Backward stepwise logistic regression analyses were used to identify independent risk factors of ICU admission and in-hospital mortality. Cox proportional hazards models were used to evaluate relationships between ICU admission and in-hospital mortality. Results: Overall, 9647 patients were analyzed. Mean age was 64.6 ± 18 years. A linear decrease was observed for hospitalization duration (0.13 days/week, R2 = 0.71; P < 0.0001), ICU admissions (0.35%/week, R2 = 0.44; P < 0.001), and hospital mortality (0.16%/week, R2 = 0.31; P < 0.01). Bacterial co-infections, male sex, history of chronic lung and heart disease, diabetes, and Hispanic ethnicity were identified as independent predictors of ICU admission (P < 0.001). ICU admission and age of ≥ 65 years were the strongest independent risk factors associated with in-hospital mortality (P < 0.001). The in-hospital mortality rate was 8.3% (27.4% in ICU patients, 2.6% in non-ICU patients; P < 0.001). Conclusions: Results indicate that, over the pandemic’s first 10 months, COVID-19 carried a heavy burden of morbidity and mortality in older patients (> 65 years), males, Hispanics, and those with bacterial co-infections and chronic comorbidities. Although disease severity has steadily declined following administration of COVID-19 vaccines along with improved understanding of effective COVID-19 interventions, these study findings reflect a “natural history” for this novel infectious disease in the U.S. Midwest.

BackgroundCardiac 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.Case summaryA 74-year-old woman presented for evaluation of fatigue. The initial electrocardiogram and echocardiogram showed features of apical hypertrophic cardiomyopathy. The patient reported a lesion on her right forearm that had been present for many years, leading to its biopsy, which showed melanoma. Further evaluation with a chest-computed tomography (CT) scan showed left lung nodules and nodular thickening of the left ventricular apex. Positron emission tomography showed an increased uptake of fluorodeoxyglucose in the left lung nodule and left ventricular apex, suggestive of metastatic spread of the melanoma. A CT-guided biopsy of the left lung nodule revealed melanoma. The patient was treated with ipilimumab initially, followed by paclitaxel with poor response to treatment, and later passed under hospice care.ConclusionMetastatic tumors involving the left ventricular apex should be considered in the differential diagnosis of apical hypertrophic cardiomyopathy, especially in patients with a history of melanoma, and advanced cardiac imaging, including cardiac magnetic resonance imaging, CT, and/or positron emission tomography (PET) may help with narrowing down the differential diagnosis.

BackgroundLeft ventricular (LV) mechanics are impaired in patients with severe aortic stenosis (AS). We hypothesized that there would be differences in myocardial mechanics, measured by global longitudinal strain (GLS) recovery in patients with four subtypes of severe AS after transcatheter aortic valve replacement (TAVR), stratified based upon flow and gradient.MethodsWe retrospectively evaluated 204 patients with severe AS who underwent TAVR and were followed post-TAVR at our institution for clinical outcomes. Speckle-tracking transthoracic echocardiography was performed pre- and post-TAVR. Patients were classified as: (1) normal-flow and high-gradient, (2) normal-flow and high-gradient with reduced LV ejection fraction (LVEF), (3) classical low-flow and low-gradient, or (4) paradoxical low-flow and low-gradient.ResultsBoth GLS (−13.9 ± 4.3 to −14.8 ± 4.3, P < 0.0001) and LVEF (55 ± 15 to 57 ± 14%, P = 0.0001) improved immediately post-TAVR. Patients with low-flow AS had similar improvements in LVEF (+2.6 ± 9%) and aortic valve mean gradient (−23.95 ± 8.34 mmHg) as patients with normal-flow AS. GLS was significantly improved in patients with normal-flow (−0.93 ± 3.10, P = 0.0004) compared to low-flow AS. Across all types of AS, improvement in GLS was associated with a survival benefit, with GLS recovery in alive patients (mean GLS improvement of −1.07 ± 3.10, P < 0.0001).ConclusionsLV mechanics are abnormal in all patients with subtypes of severe AS and improve immediately post-TAVR. Recovery of GLS was associated with a survival benefit. Patients with both types of low-flow AS showed significantly improved, but still impaired, GLS post-TAVR, suggesting underlying myopathy that does not correct post-TAVR.

Excessive cardiac fibrosis, characterized by increased collagen-rich extracellular matrix (ECM) deposition, is a major predisposing factor for mechanical and electrical dysfunction in heart failure (HF). The human ventricular fibroblast (hVF) remodeling mechanisms that cause excessive collagen deposition in HF are unclear, although reports suggest a role for intracellular free Ca2+ in fibrosis. Therefore, we determined the association of differences in cellular Ca2+ dynamics and collagen secretion/deposition between hVFs from failing and normal (control) hearts. Histology of left ventricle sections (Masson trichrome) confirmed excessive fibrosis in HF versus normal. In vitro, hVFs from HF showed increased secretion/deposition of soluble collagen in 48 h of culture compared with control [85.9±7.4 µg/106 cells vs 58.5±8.8 µg/106 cells, P

Inferior vena cava (IVC) syndrome is commonly caused by deep vein thrombosis or a mass effect. We present an unusual case of IVC kinking and obstruction detected by echocardiography and confirmed by cardiac magnetic resonance and angiography. Management of marked tortuosity of the IVC secondary to a paralyzed hemidiaphragm is discussed. (Level of Difficulty: Beginner.)

This is a case of a 43-year-old man who in 2014 was diagnosed with oral squamous cell carcinoma involving the tongue. He underwent extensive surgery that involved right tongue cancer resection and reconstruction with a free flap graft from his right forearm. He then was started on chemotherapy and radiation. Surveillance computed tomography in December 2016 showed a cardiac lesion in the left ventricular apex, which was confirmed by further echocardiography and cardiac magnetic resonance imaging. A biopsy of the mass revealed metastatic squamous cell carcinoma. He was deemed to not be a surgical candidate and continued on palliative chemotherapy. The patient had a very poor prognosis and eventually succumbed to the disease, highlighting the importance of surveillance imaging in such cases. A high index of suspicion on the part of the physician is needed to help in the early identification of these patients.

Purpose: Excessive fibrosis has been suggested to result from persistence of fibroblasts in injured tissue due to impaired apoptosis, but signaling pathways are not fully defined. Methods: Suppression of apoptotic cell death following transforming growth factor-β1 (TGF-β1) exposure was studied using the culture of NIH/3T3 mouse embryonic fibroblasts. Caspase-3 activity, propidium iodide staining and annexin V binding induced by Fas-ligand (FasL) in NIH/3T3 fibroblasts in the absence and presence of TGF-β1 was determined, and relative contribution of signaling through Smad2/3 and noncanonical Erk1/2 and Akt pathways was dissected by assessing phosphorylation status of these kinases and caspase activity in the absence and presence of specific inhibitors (SB431542, PD0325901 and LY294002), respectively. Results: TGF-β1 treatment suppressed FasL-mediated fibroblasts apoptosis with a greater than threefold reduction of caspase-3 activity (from 894 ± 186 to 195 ± 56 nmol AFC/min/106 cells at 250 ng/mL of FasL) and reductions in cleaved caspase-8 and caspase-3 by 3.2-fold and 4.3-fold, respectively. The reduction in caspase activation was accompanied by a decrease in annexin V-positive cells by ~80%. TGF-β1 treatment phosphorylated Smad2/3, Erk1/2 and Akt, which were reduced by their selective inhibitors. Inhibition of Smad2/3 and Erk1/2 alone partially reduced the protective effect of TGF-β1 on caspase-3 activation, whereas inhibition of the Akt pathway had no significant effect. Concomitant inhibition of Smad2/3 and Erk1/2 completely reversed the protection by TGF-β1. Conclusions: TGF-β1-mediated suppression of apoptosis in fibroblasts involves both Smad2/3 and Erk1/2 pathways, but not the Akt pathway. A combined approach inhibiting Smad2/3 and Erk1/2 pathways can completely reverse the protective effect of TGF-β1 on apoptosis. These findings are proof of concept to help define strategies to reduce progression of fibrosis and resultant morbidities associated with conditions causing excessive fibrosis, including but not limited to keloid formation, transplant fibrosis and aging-associated fibrosis of the heart.

The increasing prevalence of stroke, with an estimated annual cost of $71.5 billion, has made it a major health problem that increases disability and death, particularly in patients with atrial fibrillation. Although advanced age and atrial fibrillation are recognized as strong risk factors for stroke, the basis for this susceptibility are not well defined. Aging or associated diseases are accompanied by changes in rheostatic, humoral, metabolic and hemodynamic factors that may contribute more to stroke predisposition than rhythm abnormality alone. Several thromboembolism-predisposing clinical characteristics and serum biomarkers with prognostic significance have been identified in patients with atrial fibrillation. Although anticoagulation decreases the risk of thromboembolism, management in the elderly remains complex due to major concerns about bleeding. New anticoagulants and nonpharmacologic strategies are helpful to reduce the risk of bleeding, particularly in older-elderly patients. Herein, we review the pathogenesis and management of select issues of thromboembolism in the elderly with atrial fibrillation.

Purpose: Outcomes in patients with atrial fibrillation who develop acute mesenteric ischemia, and the impact of anticoagulation on complications, are not defined. Methods: Patients admitted with acute mesenteric ischemia in the National Inpatient Sample from 2007, with and without atrial fibrillation, were compared for in-hospital outcomes using multivariate regression, and the impact of prior anticoagulation determined. Results: Of 48,872 patients with acute mesenteric ischemia, 8,306 had atrial fibrillation, with 680 patients also on anticoagulation. Atrial fibrillation patients were more likely to be older and have hypertension, heart failure, or chronic lung or renal disease. After adjusting for potential confounders, atrial fibrillation remained independently associated with higher mortality (P < 0.001). Patients on anticoagulation were less likely to have intestinal resections or shock, less likely to need intubation and had lesser hospital stay and hospital charges compared to those not on anticoagulation. Conclusions: In patients with acute mesenteric ischemia, presence of atrial fibrillation increases mortality, but prior anticoagulation reduces incidence of complications, length of stay and hospital charges.

As the number of individuals, particularly the elderly, using herbal products with prescription drugs continues to grow, the risk for adverse interactions increases but remains poorly recognized. The true incidence and nature of adverse herb reactions or herb-drug interactions remains unknown since no postmarketing surveillance mechanism exists. Adverse events are greatly underreported, and information regarding safety mainly comes from case reports and suboptimally conducted studies in a limited number of healthy young volunteers or patients with limited comorbidities. Therefore, convincing evidence for the safety of herbal products in the elderly is lacking, and the true magnitude of problems that herb-drug interactions pose to public health, particularly in elderly patients with cardiovascular diseases, is not known. Since cardiovascular diseases themselves are life threatening, necessitate use of multiple medications and occur in a population with extensive comorbidities, the risk of herb-drug and herb-disease interactions is not minor and cannot be ignored. This review addresses these concerns in an effort to raise awareness about the use of herbal medicine by the elderly and its potential adverse impact on the efficacy of prescription medications that can increase predisposition to catastrophic events such as major bleeding, inadequate anticoagulation leading to undesired clotting, transplant organ rejection and life-threatening cardiac arrhythmias.

Over 1,500 gene mutations are known to cause hypertrophic cardiomyopathy (HCM). Previous studies suggest that cardiac β-myosin heavy chain (MYH7) gene mutations are commonly associated with a more severe phenotype, compared to cardiac myosin binding protein-C (MYBPC3) gene mutations with milder phenotype, incomplete penetrance and later age of onset. Compound mutations can worsen the phenotype. This study aimed to validate these comparative differences in a large cohort of individuals and families with HCM. We performed genome-phenome correlation among 80 symptomatic HCM patients, 35 asymptomatic carriers and 35 non-carriers, using an 18-gene clinical diagnostic HCM panel. A total of 125 mutations were identified in 14 genes. MYBPC3 and MYH7 mutations contributed to 50.0% and 24.4% of the HCM patients, respectively, suggesting that MYBPC3 mutations were the most frequent cause of HCM in our cohort. Double mutations were found in only nine HCM patients (7.8%) who were phenotypically indistinguishable from single-mutation carriers. Comparisons of clinical parameters of MYBPC3 and MYH7 mutants were not statistically significant, but asymptomatic carriers had high left ventricular ejection fraction and diastolic dysfunction when compared to non-carriers. The presence of double mutations increases the risk for symptomatic HCM with no change in severity, as determined in this study subset. The pathologic effects of MYBPC3 and MYH7 were found to be independent of gene mutation location. Furthermore, HCM pathology is independent of protein domain disruption in both MYBPC3 and MYH7. These data provide evidence that MYBPC3 mutations constitute the preeminent cause of HCM and that they are phenotypically indistinguishable from HCM caused by MYH7 mutations.