Hypertrophic Cardiomyopathy With Left Ventricular Apical Aneurysm in Brothers

2011 ◽  
Vol 108 (4) ◽  
pp. 612-613 ◽  
Author(s):  
Chetan Shenoy ◽  
Martin S. Maron
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular ◽  
Apical Aneurysm

Left Ventricular Apical Aneurysm in a Patient with Hypertrophic Cardiomyopathy Diagnostic and Management Dilemmas

2019 ◽  
Vol 03 (03) ◽  
Author(s):  
Parthena Theodoridou ◽  
Despoina Masmanidou ◽  
Panagiotis Kousidis ◽  
Panagiotis Roumelis ◽  
Anastasios Tsarouchas ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular ◽  
Apical Aneurysm

Abstract 13943: Hypertrophic Cardiomyopathy with Left Ventricular Apical Aneurysm Represents a High-Risk Subgroup Necessitating Aggressive Preventive Therapy: A Long-term Follow-Up Study

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Tammy S Haas ◽  
John R Lesser ◽  
Mark S Link ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Death ◽  
Left Ventricular ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Apical Aneurysm

Background: Increasing penetration of high spatial resolution cardiovascular magnetic resonance (CMR) imaging into routine cardiovascular practice has resulted in more frequent identification of a subset of hypertrophic cardiomyopathy (HCM) patients with thin-walled, scarred left ventricular (LV) apical aneurysms. Prior experience involved relatively small numbers of patients with short follow-up and therefore the risk associated with this subgroup remains incompletely defined. Therefore, we assembled a large HCM cohort with LV apical aneurysms and long-term follow-up in order to clarify clinical course and prognosis. Methods and Results: Of 2,400 HCM patients, 60 (2.5%) were identified by CMR with LV apical aneurysm, 24 to 86 years of age, including 19 (32%) <45 years old; 70% male, and followed for 5.6 ± 3.5 years. Over the follow-up period, 24 patients experienced 31 adverse disease-related complications including: appropriate implantable cardioverter-defibrillator discharge for VT/VF (n=11), received or listed for heart transplant (n=6), heart failure death (n=5), nonfatal thromboembolic events (n=4), resuscitated out-of-hospital cardiac arrest (n=3), and sudden death (n=2). In addition, an intracavitary thrombus was identified in the apical aneurysm in 9 patients without a thromboembolic history. Combined HCM-related death and aborted life threatening event rate was 8.6% per year, nearly 6-fold greater than the 1.5% annual mortality rate reported in the general HCM population. Conclusions: Patients with LV apical aneurysms represent a high-risk subgroup within the diverse HCM spectrum, associated with substantial increased risk for disease-related morbidity and mortality, including advanced heart failure, thromboembolic stroke and sudden death. Identification of this unique HCM phenotype should prompt consideration for primary prevention ICD, and anticoagulation for stroke prophylaxis.

scholarly journals Midventricular Hypertrophic Cardiomyopathy with Apical Aneurysm: Potential for Underdiagnosis and Value of Multimodality Imaging

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Archana Sivanandam ◽  
Karthik Ananthasubramaniam
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
African American Woman ◽  
Multimodality Imaging ◽  
American Woman ◽  
Implantable Defibrillator ◽  
Left Ventricular ◽  
Apical Region ◽  
St Segment Elevation ◽  
St Segment ◽  
Apical Aneurysm

We illustrate a case of midventricle obstructive HCM and apical aneurysm diagnosed with appropriate use of multimodality imaging. A 75-year-old African American woman presented with a 3-day history of chest pain and dyspnea with elevated troponins. Her electrocardiogram showed sinus rhythm, left atrial enlargement, left ventricular hypertrophy, prolonged QT, and occasional ectopy. After medical therapy optimization, she underwent coronary angiography for an initial diagnosis of non-ST segment elevation myocardial infarction. Her coronaries were unremarkable for significant disease but her left ventriculogram showed hyperdynamic contractility of the midportion of the ventricle along with a large dyskinetic aneurysmal apical sac. A subsequent transthoracic echocardiogram provided poor visualization of the apical region of the ventricle but contrast enhancement identified an aneurysmal pouch distal to the midventricular obstruction. To further clarify the diagnosis, cardiac magnetic resonance imaging with contrast was performed confirming the diagnosis of midventricular hypertrophic cardiomyopathy with apical aneurysm and fibrosis consistent with apical scar on delayed enhancement. The patient was medically treated and subsequently underwent elective implantable defibrillator placement in the ensuing months for recurrent nonsustained ventricular tachycardia and was initiated on prophylactic oral anticoagulation with warfarin for thromboembolic risk reduction.

scholarly journals ELECTROCARDIOGRAPHIC CHARACTERISTICS IN HYPERTROPHIC CARDIOMYOPATHY WITH LEFT VENTRICULAR APICAL ANEURYSM

2020 ◽  
Vol 75 (11) ◽  
pp. 369
Author(s):  
Nandini Mehra ◽  
Anthony Kashou ◽  
Christopher DeSimone ◽  
Alexander C. Egbe ◽  
Rick A. Nishimura ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular ◽  
Apical Aneurysm

Electrocardiographic evolution in patients with hypertrophic cardiomyopathy who develop a left ventricular apical aneurysm

2015 ◽  
Vol 48 (5) ◽  
pp. 818-825 ◽  
Author(s):  
Ermelinda Pennacchini ◽  
Maria Beatrice Musumeci ◽  
Maria Rosa Conte ◽  
Claudia Stöllberger ◽  
Francesco Formisano ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular ◽  
Apical Aneurysm

scholarly journals Imaging systolic and diastolic heart murmurs in hypertrophic cardiomyopathy

2014 ◽  
Vol 1 (1) ◽  
pp. 1
Author(s):  
Andrea Barison ◽  
Giancarlo Todiere ◽  
Pier Giorgio Masci ◽  
Giovanni D Aquaro
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Mitral Regurgitation ◽  
Specific Treatment ◽  
Left Ventricular ◽  
Early Diastole ◽  
Apical Aneurysm ◽  
Free Precession Imaging ◽  
Worse Prognosis ◽  
Left Ventricular Base

Muscular obstruction in hypertrophic cardiomyopathy can occur not only in systole, but also in diastole. Here we report a case of hypertrophic cardiomyopathy in a middle-aged lady: echocardiography and cardiovascular magnetic resonance confirmed the diagnosis of hypertrophic cardiomyopathy with significant systolic and diastolic midventricular obstruction, corresponding to the murmurs audible in mid-systole and early-diastole respectively. Moreover, the mid-systolic murmur was clearly distinguishable from the mitral regurgitation murmur, which was audible throughout systole (pan-systolic) and likely related to systolic anterior motion of the mitral chords; similarly, the early-diastolic heart murmur due to diastolic obstruction was  better audible at the apex and clearly distinguishable from common diastolic murmurs due to aortic regurgitation. Echocardiography (with Doppler recordings) and magnetic resonance (with turbulence flows at steady state free precession imaging) allowed a detailed visualization of each audible murmur (systolic obstruction, mitral regurgitation, diastolic obstruction). In particular, while systolic obstruction has been extensively studied in hypertrophic cardiomyopathy, only very few reports about diastolic obstruction exist, describing either a normodirected midventricular diastolic obstruction (forward flow) similar to this case or a paradoxical midventricular diastolic obstruction (backward flow) in patients with an apical aneurysm emptying during early diastole into the left ventricular base through a narrow midventricular channel. Whether diastolic obstruction implies worse prognosis or requires specific treatment, deserves further research.

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