Subclinical Hypertrophic Cardiomyopathy in Elite Athletes: Knowledge Gaps Persist.

Subclinical hypertrophic cardiomyopathy (HCM) is a phenotypic entity that has emerged from the increased use of cardiovascular magnetic resonance imaging in the evaluation and family screening of patients with HCM. We describe the case of a competitive athlete with a sarcomere gene mutation and family history of HCM who was found to exhibit the subclinical HCM phenotype on cardiovascular magnetic resonance imaging in the absence of left ventricular hypertrophy. We discuss the clinical uncertainties in her management. (Level of Difficulty: Advanced.).

Danon Cardiomyopathy: Specific Imaging Signs.

Danon disease (DD) is a rare, X-linked genetic disorder caused by LAMP2 deficiency. Clinical phenotype involves early cardiomyopathy development along with pre-excitation, skeletal myopathy, retinopathy, and cognitive impairment. We highlight how a noninvasive diagnostic approach based on clinical and imaging red flags for DD can be employed to raise high clinical suspicion for DD, which was confirmed by genetic testing results. (Level of Difficulty: Intermediate.).

Aortic arch calcification and risk of all-cause mortality and cardiovascular disease: The Guangzhou Biobank Cohort Study.

Background: There were no reports on the associations of aortic arch calcification (AAC) measured by chest X-ray with all-cause mortality and cardiovascular disease (CVD) in older general population. Moreover, previous studies of hemodialysis patients showed that AAC was correlated with left ventricular hypertrophy (LVH) and predicted CVD jointly. Whether the effects remained in the general population is unknown. We examined the associations of AAC with all-cause mortality and CVD in general population and the risk associated with the coexistence of AAC and LVH. Methods: Presence and severity (grades 0-2) of AAC were measured by chest X-ray, and LVH was identified by 12-lead electrocardiogram in 27,166 Chinese aged 50+ years free of CVD from Guangzhou Biobank Cohort Study. Multivariate Cox regressions were used to examine associations of AAC and LVH with outcomes. Findings: During an average follow-up of 14·3 years, 5,350 deaths and 4,012 CVD occurred. Compared to those without AAC at baseline, those with AAC had higher risks of all-cause mortality (HR 1·24, 95% CI 1·17-1·31) and CVD (HR 1·22, 95% CI 1·14-1·30), with dose-response relationship (P ≤ 0·001). Furthermore, those with coexistence of AAC and LVH had higher risks of all-cause mortality (HR 1·72, 95% CI 1·37-2·15) and CVD (HR 1·80, 95% CI 1·40-2·32) than those without AAC and LVH. Interpretation: As chest X-ray has been performed commonly for health screening and in hospital patients when first admitted, AAC measured by chest X-ray can be further applied to assist cardiovascular risk stratification in the community and clinical settings. Funding: The Natural Science Foundation of China (No. 81941019).

Acute Myocarditis Mimicking Hypertrophic Cardiomyopathy in Marfan Syndrome and Morphologically Abnormal Mitral Valve.

A 40-year-old man with Marfan syndrome presented with chest pain and troponin elevation. Urgent echocardiography was suggestive of hypertrophic cardiomyopathy, but cardiovascular magnetic resonance identified features of acute myocarditis. Repeated imaging 4 months later showed resolution of septal thickness, confirming acute myocarditis. (Level of Difficulty: Intermediate.).

Cardiac Arrest in a Softball Player Following a Collision: Catching the Correct Diagnosis.

Commotio cordis is a rare cause of sudden cardiac arrest from blunt chest trauma; however, it is a diagnosis of exclusion. We present a case of sudden cardiac arrest in a collegiate athlete initially attributed to commotio cordis but in whom further history and workup revealed another rare condition. (Level of Difficulty: Advanced.).

Echocardiographic Investigation of Low-Flow State in a Hispanic/Latino Population.

Objective: To assess the prevalence of low-flow state (LFS) with left ventricular (LV) stroke volume index of less than 35 mL/m2 and the demographics, clinical and echocardiographic characteristics associated with LV remodeling and function in a Hispanic/Latino population. Participants and Methods: The study included 1346 asymptomatic participants from the Hispanic Community Health Study/Study of Latinos with normal LV ejection fraction (≥55%) and no valvular heart disease. LV volume, mass and left atrial volume, LV ejection fraction, global longitudinal strain, and myocardial contraction fraction were measured by echocardiography. The participants were divided into LFS or normal flow state (NFS: stroke volume index ≥35 mL/m2). Demographics, clinical and echocardiographic characteristics, and measures of LV remodeling and function were compared between the LFS and NFS groups. Results: The prevalence of LFS was 41%. In comparison with NFS, the LFS had lower LV mass index (77.2±0.96 g/m2 vs 84.6±0.86 g/m2; P<.001), left atrial volume index (20.6±0.35 mL/m2 vs 23.5±0.37 mL/m2; P<.001), global longitudinal strain (-16.8±0.16% vs -17.7±0.17%; P<.001), and myocardial contraction fraction (43.3±0.63% vs 55.7±0.64%; P<.001). There was no significant difference in the relative wall thickness (LFS: 0.40±0.004 vs NFS: 0.40±0.005; P=.57). The LFS group had significantly higher hemoglobin A1c (6.18±0.07% vs 5.97±0.04%; P=.01) than the NFS group. Conclusion: A high prevalence of LFS associated with echocardiographic characteristics reflecting unfavorable LV remodeling and function was observed in a Hispanic/Latino population. Further studies of the prognostic significance of LFS in a large multiethnic population are warranted.

Flecainide Toxicity Leading to Loss of Pacemaker Capture and Cardiac Arrest.

An 86-year-old man with paroxysmal atrial fibrillation on flecainide, a class IC antiarrhythmic, presented with cardiac arrest. The patient had extremely wide QRS complexes with inconsistent pacemaker capture on electrocardiography. Due to cardiac failure and renal failure, the patient developed progressive flecainide toxicity, which led to pacemaker failure, and ultimately, death. (Level of Difficulty: Beginner.).

Unmasking Hydroxychloroquine Cardiotoxicity in a Patient With Heart Failure and Chronotropic Incompetence.

Chronic use of hydroxychloroquine can result in cardiomyopathy and conduction disturbances. Here, we describe a case of hydroxychloroquine cardiotoxicity in a patient with heart failure with preserved ejection fraction and severe chronotropic incompetence. (Level of Difficulty: Intermediate.).

Short-Segment Type B Interrupted Aortic Arch Presenting With Subarachnoid Hemorrhage With Subsequent Primary Percutaneous Repair.

This report describes a young adult man presenting with subarachnoid hemorrhage secondary to an intracranial aneurysm who was found to have a short-segment type B interrupted aortic arch. We describe the clinical presentation, evaluation, and management of this patient and highlight imaging findings and percutaneous repair of the aneurysm and interrupted aortic arch. (Level of Difficulty: Intermediate.).

Thick Heart, Wide QRS, Broad Differential.

Routine electrocardiogram in a middle-aged man with left ventricular hypertrophy showed sinus rhythm, a short PR interval, and delta wave, confirming ventricular pre-excitation. Pre-excitation was fixed after a premature atrial complex and in atrial fibrillation, features diagnostic of a fasciculo-ventricular pathway. Genetic testing confirmed a diagnosis of hypertrophic cardiomyopathy. (Level of Difficulty: Intermediate.).

A Pleasant Surprise in the Face of ST-Segment Elevation.

ST-segment elevation is a dreadful finding in the emergency department because it is often associated with myocardial infarction and demands a prompt and definitive treatment. However, the clinical and echocardiographic assessment of a patient with electrocardiographic changes trumps any electrocardiology expert and should always lead to a clinical decision. (Level of Difficulty: Intermediate.).

Lowering the Recommended Maximal Wall Thickness Threshold Improves Diagnostic Sensitivity in Asians With Hypertrophic Cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is defined as left ventricular end-diastolic maximal wall thickness (WTMax) ≥15.0 mm, without accounting for ethnicity, sex, and body size. It is well-established that Asians have smaller hearts than do Caucasians. Objectives: This study aims to examine the implications of this single absolute WTMax threshold on the diagnosis of HCM in Asians. Methods: The study consisted of 360 healthy volunteers (male: n = 174; age: 50 ± 12 years) and 114 genetically characterized patients with HCM (male: n = 83; age: 52 ± 13 years; genotype-positive, n = 39). All participants underwent cardiovascular magnetic resonance. WTMax was measured semiautomatically at end-diastole according to the standard 16 myocardial segments. Results: Healthy male volunteers had increased WTMax compared with that of female volunteers (8.4 ± 1.2 mm vs 6.6 ± 1.1 mm, respectively; P < 0.001). Conversely, WTMax was similar between male and female patients with HCM (15.2 ± 3.4 mm vs 14.7 ± 3.0 mm, respectively; P = 0.484) and between those with and without a pathogenic gene variant (P = 0.828). Using the recommended diagnostic threshold of 15.0 mm, 56 patients with HCM had WTMax <15.0 mm and no healthy volunteers had WTMax >15.0 mm (specificity of 100% and sensitivity of 51%). Lowering WTMax thresholds to 10.0 mm in female patients and 12.0 mm in male patients did not affect specificity (100%) but significantly improved sensitivity (84%). Despite lower left ventricular mass, female patients with HCM demonstrated more features of adverse cardiac remodeling than did male patients: increased myocardial fibrosis, higher asymmetric ratio, and disproportionately worse myocardial strain. Conclusions: The study highlights cautious application of guideline-recommended WTMax to diagnose HCM in Asians. Lowering WTMax to account for ethnicity and sex improves diagnostic sensitivity without compromising specificity.

Unusual Manifestations of Coral Reef Aorta Complicated Severe Valvular Heart Disease in a Dialysis Patient.

We report the unusual case of a 68-year-old man with coral reef aorta complicated with severe calcified valvular heart disease who has been undergoing dialysis for 21 years. This report highlights the etiology and the unusual manifestations of coral reef aorta in a long-term dialysis patient. (Level of Difficulty: Intermediate.).

The Use of Machine Learning for the Care of Hypertension and Heart Failure.

Machine learning (ML) is a branch of artificial intelligence that combines computer science, statistics, and decision theory to learn complex patterns from voluminous data. In the last decade, accumulating evidence has shown the utility of ML for prediction, diagnosis, and classification of hypertension and heart failure (HF). In addition, ML-enabled image analysis has potential value in assessing cardiac structure and function in an accurate, scalable, and efficient way. Considering the high burden of hypertension and HF in China and worldwide, ML may help address these challenges from different aspects. Indeed, prior studies have shown that ML can enhance each stage of patient care, from research and development, to daily clinical practice and population health. Through reviewing the published literature, the aims of the current systemic review are to summarize the utilities of ML for the care of those with hypertension and HF.

Adult height and incidence of atrial fibrillation and heart failure in older men: The British Regional Heart Study.

AIMS: Taller stature has been associated with increased risk of atrial fibrillation (AF). AF and heart failure (HF) often co-occur but the association between height and risk of HF in older adults has not been well studied. We have examined the association between height and incident AF and incident HF in older adults. METHODS: Prospective study of 3346 men aged 60-79 years with no diagnosed HF, myocardial infarction or stroke at baseline (1998-2000) followed up for a mean period of 16 years, in whom there were 294 incident HF cases and 456 incident AF. Men were divided into 5 height groups: <168.2, 168.2-172.5, 172.6-176.9, 177.0-183.0 and >183.0 cms based on the 25th, 50th, 75th and 95th centiles distribution of height. RESULTS: CVD risk factors tended to decrease with increasing height but a positive association was seen between height and electrocardiographic QRS duration and incident AF. Both short stature (<168.2 cm) and tall stature (>183.0 cm) was associated with significantly increased risk of HF in age-adjusted analysis compared to those in the second height quartile [HR (95 %CI) = 1.62 (1.15, 2.26) and 2.04 (1.23, 3.39) respectively]. In short men the increased risk remained after adjustment for adverse CVD risk factors; in tall men the association was largely associated with AF and QRS duration. CONCLUSION: Tall stature is associated with significantly increased risk of AF leading to increased risk of HF. Short stature was associated with increased HF risk which was not explained by known adverse CVD risk factors.

Dynamic Left Ventricular Outflow Tract Obstruction Post-Transcatheter Aortic Valve Replacement.

We describe the first case of successful management of left ventricular outflow tract obstruction developing late after transcatheter aortic valve replacement with right ventricular apical pacing. The possible mechanisms of obstruction resolution are described. (Level of Difficulty: Advanced.).

Hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. The disease is characterized by marked variability in morphological expression and natural history, ranging from asymptomatic to heart failure or sudden cardiac death. Left ventricular hypertrophy and abnormal ventricular configuration result in dynamic left ventricular outflow obstruction in most patients. The goal of pharmacological therapy in HCM is to alleviate the symptoms, and it includes pharmacotherapies and septal reduction therapies. In this review, we summarize the relevant clinical issues and treatment options of HCM.

A Rare Case of Lead-Induced Cardiomyopathy.

A 39-year-old man, painter by profession, presented with symptoms of heart failure. His work up was unrevealing except for elevated blood lead levels (BLL). He was started on guideline-directed medical therapy and was referred to occupational therapy. No improvement in his ejection fraction was noted until his BLL decreased. (Level of Difficulty: Intermediate.).

Acute myocardial infarction in young newbie bodybuilder using multiple steroid and protein supplements.

Coronary artery disease (CAD), the major reason of deaths worldwide is generally known as a disease of the elderly, however it is grasping the youth too. The most common etiology of young CAD is lifestyle changes, smoking, and development of other comorbid conditions such as diabetes and hypertension at an early age. There has been an upward trend in youngsters regarding consciousness about their body build and thus use of various protein supplements and anabolic steroids for faster results. The present case reports a young patient presenting with severe retrosternal left-sided chest pain for 15-20 min to the emergency department. His electrocardiogram was suggestive of acute anterolateral wall ST segment elevation myocardial infarction for which he underwent urgent coronary angiography and percutaneous coronary intervention. His personal history revealed a significant use of steroids, proteins, and other supplements in supraphysiological doses for instant body building efforts without any other significant past medical, surgical, or family history. He showed good recovery and was strongly recommended to stop steroids and protein supplements. In conclusion, supraphysiological doses of protein supplements, anabolic steroids, and other nutritional products bear a risk factor for CAD. .

Evaluating Suspected Cardiac Amyloidosis.

A 71-year-old-woman presented with breathlessness, general tiredness and orthopnea. Echocardiography and electrocardiogram were suspicious for cardiac amyloidosis. This case illustrates contemporary evaluation to confirm the diagnosis and distinguish between different types of amyloid. (Level of Difficulty: Beginner.).