Aneurysms In Hypertrophic Cardiomyopathy Research Articles

Clinical Course and Treatment of Patients With Apical Aneurysms Due to Hypertrophic Cardiomyopathy

BackgroundThere is controversy about risk of malignant arrhythmias and stroke in patients with apical aneurysms in hypertrophic cardiomyopathy (HCM). ObjectivesThe aim of this study was to estimate the associations of aneurysm size and major HCM risk factors with the incidence of lethal and potentially lethal arrhythmias and to estimate incidence of unexplained stroke. MethodsIn 108 patients (age 57.4 ± 13.5 years, 37% female) from 3 HCM centers, we assessed American Heart Association/American College of Cardiology guidelines risk factors and initial aneurysm size by echocardiography and cardiac magnetic resonance imaging and assessed outcomes after median 5.9 (IQR: 3.7-10.0) years. ResultsImplantable cardioverter defibrillator discharges or sudden cardiac death (SCD) occurred in 21 (19.4%) patients. Of patients with a risk factor, 55% subsequently had ventricular tachycardia (VT), ventricular fibrillation (VF), or SCD at follow-up, compared with 10% in those who did not (P < 0.001). The upper tercile of size had a 5-year cumulative risk of 35%, while the lower tercile had 5-year risk of 6% (P = 0.0046). In those with the smallest aneurysms <2 cm2 and also without risk factors VT, VF, or SCD occurred in only 2.5%. Clinical atrial fibrillation (AF) was prevalent, occurring in 49 (45%). Stroke was commonly associated with AF. Stroke without conventional cause had an incidence of 0.5%/year. Surgery in 19% was effective in reducing symptoms. VT ablation and surgery were moderately effective in preventing recurrent VT. ConclusionsRisk factors and aneurysm size were associated with subsequent VT, VF, or SCD. Patients with aneurysms in the lowest tercile of size have a low cumulative 5-year risk. Clinical AF occurred frequently. Stroke prevalence in absence of known stroke etiologies is uncommon and comparable to risk of severe bleeding.

Diagnosis and Clinical Implication of Left Ventricular Aneurysm in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a genetic disease with heterogeneous clinical presentation and prognosis. Within the broad phenotypic expression of HCM, there is a subgroup of patients with a left ventricular (LV) apical aneurysm, which has an estimated prevalence between 2% and 5%. LV apical aneurysm is characterized by an area of apical dyskinesis or akinesis, often associated with regional scarring. To date, the most accepted pathomechanism of this complication is, in absence of coronary artery disease, the high systolic intra-aneurysmal pressure, which, combined with impaired diastolic perfusion from lower stroke volume, results in supply-demand ischemia and myocardial injury. Apical aneurysm is increasingly recognized as a poor prognostic marker; however, the efficacy of prophylactic anticoagulation and/or intracardiac cardioverted defibrillator (ICD) in improving morbidity and mortality is not yet clearly demonstrated. This review aims to elucidate the mechanism, diagnosis and clinical implication of LV aneurysm in patients with HCM.

Apical Aneurysms and Mid–Left Ventricular Obstruction in Hypertrophic Cardiomyopathy

BackgroundApical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%. ObjectivesThe authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms. MethodsThe authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities). ResultsThere were 108 patients with a mean age of 57.4 ± 13.5 years; 40 (37%) were women. A total of 103 aneurysm patients (95%) had mid-LV obstruction with mid-LV complete systolic emptying. Of the patients with obstruction, 84% had a midsystolic Doppler signal void, a marker of complete flow cessation, but only 19% had Doppler systolic gradients ≥30 mm Hg. Five patients (5%) had relative hypokinesia in mid-LV without obstruction. Aneurysm size is not bimodal but appears distributed by power law, with large aneurysms decidedly less common. Comparing mid-LV obstruction aneurysm patients with all control groups, the short-axis (SAX) systolic areas were smaller (P < 0.007), the percent SAX area change was greater (P < 0.005), the papillary muscle (PM) areas were larger (P < 0.003), and the diastolic PM areas/SAX diastolic areas were greater (P < 0.005). Patients with aneurysms had 22% greater SAX PM areas compared with those with elevated LV velocities but no aneurysms (median: 3.00 cm2 [IQR: 2.38-3.70 cm2] vs 2.45 [IQR: 1.81-2.95 cm2]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying. ConclusionsThe great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.

Different characteristics of apical aneurysm in hypertrophic cardiomyopathy are related to difference in long-term prognosis

BackgroundData regarding long-term cardiac and cerebrovascular adverse events in patients with hypertrophic cardiomyopathy (HCM) and apical aneurysm (AAn) are scarce and specific treatment strategies that include the use of anticoagulants have not yet been established. We aimed to evaluate the prevalence and long-term prognostic implication based on characteristics of AAn in patients with HCM. MethodsA total of 458 consecutive patients diagnosed with HCM underwent cardiovascular magnetic resonance imaging and echocardiography from August 1, 2008 to December 31, 2015. AAn was classified into Grade 1 and Grade 2 based on size and morphology. The patients were followed up for a median duration of 6.3 years (range, 4.2–8.7 years) for major adverse cardiac and cerebral events (MACCEs); a composite of cardiac death, HCM-related hospitalization, cerebrovascular accident (CVA), heart transplantation, myocardial infarction, and implantable cardiac defibrillator/cardiac resynchronization therapy. ResultsAAn was detected in 9.2%. MACCEs developed more frequently in patients with AAn than in those without AAn (30.1% vs. 20.7%, P = 0.015), with the rate of CVA as the main difference (9.7% vs. 5.3%, P = 0.011). Grade 2 AAn group showed significantly higher MACCE than Grade 1 AAn group (41.8% vs. 21.9%, P < 0.001). In multivariate analysis, the presence of AAn was independently associated with increased risk of MACCEs (adjusted hazard ratio: 1.95; 95% confidence interval, CI: 1.16–3.28; P = 0.012). ConclusionsAAn is independently associated with increased risk of HCM-related adverse events, especially cerebral infarction, with significant relationship between aneurysm size and adverse events.

Clinical Characteristics and Prognostic Importance of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy

BackgroundLeft ventricular (LV) apical aneurysms in hypertrophic cardiomyopathy (HCM) are a recognized risk marker for adverse cardiovascular events. There is variable practice among clinicians and discordance between international guidelines regarding treatment recommendations and prognostication for this important phenotype. ObjectivesThe authors sought to describe the morphology, clinical course, and risk of adverse events in a large single-center cohort of HCM patients with LV apical aneurysms. MethodsThis study analyzed 160 HCM patients with an LV apical aneurysm who were evaluated in our dedicated HCM clinic between January 1997 and April 2021. ResultsMean age was 59.1 ± 13.6 years, and 71% of these patients were male. Mean aneurysm size was 1.77 ± 1.04 cm. Over 6.2 ± 4.8 years, 14 (9%) patients had a sudden cardiac death (SCD) event, including appropriate therapy from an implantable cardioverter-defibrillator (ICD) or resuscitation from cardiac arrest (annualized event rate 1.77%/y), 39 (24%) had either a thromboembolic stroke or apical thrombus formation (2.9%/y), and 14 (9%) developed LV systolic dysfunction with an ejection fraction (EF) <50% (1.28%/y). HRs for SCD, stroke or thrombus, and EF <50% per 1-cm increase in aneurysm size were 1.69 (P = 0.007), 1.60 (P = 0.0002), and 1.63 (P = 0.01), respectively. Aneurysm size ≥2 cm was associated with a 5-year SCD rate of 9.7%, compared with 2.9% for aneurysm size <2 cm (log-rank P = 0.037). This subgroup also had higher risk of stroke/thrombus formation (HR: 2.20; P = 0.002), with an annualized event rate of 2.7%/year. A total of 39 (24%) patients reached the combined end point of SCD, stroke, or LV dysfunction (2.12%/y) with an HR of 1.47/cm increase in aneurysm size (P = 0.003) and an HR of 2.22 for patients with aneurysm size ≥2 cm (P = 0.02). ConclusionsIncreasing aneurysm size confers poorer prognosis. Aneurysm size ≥2 cm should alert potential consideration for prophylactic anticoagulation and primary prevention ICDs.

Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy: Equivalent Detection by Magnetic Resonance Imaging and Contrast Echocardiography

Left ventricular (LV) apical aneurysm is a unique morphological entity and novel adverse risk marker existing within the broad phenotypic spectrum of hypertrophic cardiomyopathy (HCM). Its true prevalence in the HCM population is likely underestimated because of inherent limitations of conventional noncontrast echocardiography. The authors hypothesized that contrast echocardiography is a reliable imaging technique compared with cardiovascular magnetic resonance (CMR) for the detection of apical aneurysms. The aim of this study was to assess the effectiveness of contrast echocardiography in the detection of LV apical aneurysms in patients with HCM in comparison with the gold standard, CMR. One hundred twelve patients with HCM identified from an institutional clinical database, who underwent echocardiographic and CMR examinations within 12months and had LV apical aneurysms identified on either or both imaging modalities, were retrospectively analyzed. Discordant cases were reviewed by an expert panel, and a consensus was reached regarding the presence or absence of an apical aneurysm. The reason for any discrepancy was recorded. The mean age of the patients was 59±13years, and 73% were men. Sixty-four (57%) underwent contrast echocardiography. The median interval between echocardiography and CMR was 118days (interquartile range, 61-237days). Thirty-nine patients (35%) had discordance between echocardiographic and CMR findings, of whom 20 had aneurysms reported on echocardiography but not CMR and 19 vice versa. Upon reanalysis by the expert panel, aneurysms were initially missed on CMR in 16 patients (80%), largely because of interpretation error secondary to small aneurysms, with a mean aneurysm size of 0.82±0.38cm in these cases. Before secondary review by the expert panel, contrast echocardiography had sensitivity of 97% compared with 85% for CMR (P=.0198) and 64% for noncontrast echocardiography (P=.0001). After secondary review, contrast echocardiography had sensitivity of 98% compared with 67% for noncontrast echocardiography (P=.0001) and 97% for CMR (P=1.00). Contrast echocardiography has high sensitivity for detecting LV apical aneurysms and should beused routinely in the evaluation and risk stratification of patients with HCM.

Left ventricular aneurysm in hypertrophic cardiomyopathy with midventricular obstruction.

Left ventricular aneurysm in hypertrophic cardiomyopathy with midventricular obstruction.

Leftventricular apical aneurysm in hypertrophic cardiomyopathy as a risk factor for sudden death at any age.

In hypertrophic cardiomyopathy (HCM) aging has proved protective against sudden death (SD) risk and aggressive recommendations for prophylactic ICDs are uncommon in patients ≥60 years. Nevertheless, we present a patient with an unexpected but aborted sudden death event at the advanced age of 71 years due to a left ventricular apical aneurysm (LVAA) which has emerged as a novel SD marker. Subsequent reappraisal of the Tufts HCM database, specifically the 118 LVAA patients, showed that 36% of SD events occurred at ≥60 years. Of HCM patients ≥ 60 years, SD was 8-fold more common with aneurysm than without aneurysms (16%vs 2%; P<0.001). Risk in HCM with LVAA persists throughout life and senior LVAA patients should also be considered for primary prevention of SD with the ICD.

Left ventricular aneurysms in hypertrophic cardiomyopathy with midventricular obstruction: A systematic review of literature.

Hypertrophic cardiomyopathy (HCM) with or without left ventricular apical aneurysm (LVA) had been studied in the past. Midventricular obstruction associated with HCM and LVA is a unique entity that has not been distinguished previously as a separate phenotypic disease in HCM patients. A systematic review of Pubmed and Google Scholar was conducted from inception until September 2017 for all observational studies conducted on HCM with midventricular obstruction and LVA. A total of 94 patients from 39 studies were included in our analysis. The mean age of the patients was 58.05 ± 11.76 years with 59.6% being males. The most common electrocardiographic finding was T wave inversion occurring in 13.8% of the cases followed by ST elevation (9.5%). Maximal left ventricle (LV) wall thickness was reported 18.89 ± 5.19mm on transthoracic echocardiography and paradoxical jet flow was detected in 29.8% of patients. Beta-blockers (58.5%) were the most common drug therapy at baseline and amiodarone (10.6%) was the most common antiarrhythmic used for ventricular tachycardia (VT). The most common complication, VT, occurred in 39.3% of cases and the incidence of all-cause mortality was 13.8 % over 16 ± 20.1 months follow-up. Implantable cardioverter defibrillator (ICD) was used in 37.2% of patients; 25.7% of patients with ICD received appropriate shock therapy. HCM with LVA and midventricular obstruction is a unique entity that appears to be associated with high incidence of morbidity and mortality. Thus, early diagnosis and therapeutic intervention is recommended for management of this condition.

Clinical Profile and Prognosis of Left Ventricular Apical Aneurysm in Hypertrophic Cardiomyopathy

BackgroundHypertrophic cardiomyopathy with left ventricular apical aneurysm is a unique entity with diverse manifestations and varied prognoses among races. This study evaluated the prevalence, clinical characteristics and outcomes of apical aneurysm in Chinese patients with hypertrophic cardiomyopathy. MethodsConsecutive patients with apical aneurysm were recruited from 1,844 patients with HCM treated at our hospital from 2002-2013. Basic clinical data and follow-up data were collected and analyzed. ResultsApical aneurysm was identified in 24 patients (1.3%) (mean age: 52 ± 14 years). We identified an hourglass-shaped (71%) or distally hypertrophic (29%) left ventricle and found mural thrombi and nonsustained and sustained ventricular tachycardia in 11 (46%), 4 (17%) and 9 (38%) patients, respectively. During follow-up (5.0 ± 3.4 years [range: 1–14 years]), following were the clinical adverse events experienced by 14 patients (58%) (annual rate: 11.7%): sudden cardiac death (n = 4), appropriate discharge of an implantable cardioverter-defibrillator (n = 4), progressive heart failure (n = 4) or heart failure–related death (n = 1) and stroke (n = 4). The 4 patients who underwent aneurysmectomy had no adverse events. Patients with SCD had a lower ejection fraction (P = 0.004) and a larger left ventricular end-diastolic diameter (P < 0.001) than nonoperated survivors. ConclusionsApical aneurysm is not rare in patients with HCM and it confers an extremely poor prognosis. Early aggressive therapies should be considered for this entity and prophylactic aneurysmectomy may be an option.

Association of ST elevation with apical aneurysm in hypertrophic cardiomyopathy

ObjectivesApical aneurysms in patients with hypertrophic cardiomyopathy (HCM) represent an underrecognized but clinically important subset of HCM patients. However it may be frequently missed by echocardiography because of poor image quality of left ventricular apex. We aimed to compare electrocardiographic STE in HCM patients with and without apical aneurysm. MethodsWe developed this clinical review using an extensive MEDLINE review of the literature and data from our laboratories; and some electrocardiographic parameters including STE were analysed in HCM patients with and without apical aneurysm. ResultsThere were 29 HCM patients without apical aneurysm (Group 1; 52.6±17.7years, 69% male) and 28 HCM patients with apical aneurysm (Group 2; 59.6±13.2years, 57% male). The STE in V4-6 derivations were statistically more frequent in patients with apical aneurysm compared to those without aneurysm (93% vs 7%, p<0.001). There was a positive correlation between the presence of the STE in V4-6 derivations and the presence of the apical aneurysm (Spearman's ρ=0.895, p<0.001). ConclusionsClinicians and specifically echocardiographers must pay special attention on the electrocardiography to correctly detect the frequently overlooked apical aneurysm in HCM patients, and should be careful for apical aneurysm particularly in the presence of STE in V4-6 derivations.

Prevalence and characteristics of apical aneurysm in hypertrophic cardiomyopathy: a prospective analysis of cardiac magnetic resonance findings and clinical outcomes

Methods A total of 350 consecutive patients diagnosed with HCM (mean age 54 ± 12, 278 males) underwent cardiac magnetic resonance (CMR) and echocardiography. All enrolled patients were prospectively followed up for adverse clinical events including cardiac death, admission for heart failure and cerebrovascular accident. We divided the subjects into 4 phenotypes according to the location of hypertrophic segment; asymmetrical septal hypertrophy (ASH), apical, concentric and septal/apical type. On CMR, the LV volumetric parameters were measured, and the amount of late gadolinium enhancement (LGE) was calculated with grayscale thresholds of 6 SD above the mean signal intensity for normal remote myocardium. Echocardiographic evaluations included left atrial volume index, mitral inflow pattern, tissue Doppler of mitral annulus and LV dimension. Median follow up duration was 37 months. Results The prevalence of AAn on CMR was 14.3%, which was significantly higher compared to previously reported data. AAn was detected in all groups of HCM regardless of type (16.8% in ASH type, 15.3% in apical type, 17.9% in concentric type, and 9.1% in septal/apical type of HCM). Clinical manifestations and LV volumetric parameters on CMR did not differ between the HCM patients with and without AAn. The frequency and the amount of LGE were not different between two groups (frequency; 94% vs. 93.3%, p=1.00, extent; 11.7±8.9 vs. 13.0±10.3, p=0.43). During follow up, the frequency of adverse clinical events did not differ between patients with and without AAn (p=0.259).

Prevalence and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy

Prevalence and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy

Response to Letter Regarding Article, “Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy”

We thank Dr Giuseppe Ando et al for their interest in our description of left ventricular (LV) apical aneurysms occurring in patients with hypertrophic cardiomyopathy (HCM), a newly recognized and important subgroup within this heterogeneous disease spectrum.1 Ando et al have raised a number of important issues relative to nomenclature, mechanism, and the treatment of LV apical aneurysms in HCM patients. With respect to nomenclature, we agree that the term diverticula should be …

Letter by Andò et al Regarding Article, “Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy”

To the Editor: We read with great interest the article by Maron et al1 about the cohort of patients with hypertrophic cardiomyopathy (HCM) and left ventricular apical aneurysms. In the mid-1990s, the same authors drew our attention to a small group of patients with HCM and uncommon apical bulges regarded as diverticula .2 Cardiac magnetic resonance now sheds light on the structure of these apical bulges and the surrounding myocardium and demonstrates transmural myocardial fibrosis of the aneurysmal rim, largely extending in the contiguous ventricular walls.1 Apical aneurysm has been classically considered a complication of acute …

Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy

Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy

Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease characterized by a diverse clinical and phenotypic spectrum. This study reports the prevalence, morphology, clinical course, and management of an underrecognized subgroup of HCM patients with left ventricular apical aneurysms. Of 1299 HCM patients, 28 (2%) were identified with left ventricular apical aneurysms, including a pair of identical twins. Aneurysms were recognized at a wide age range (26 to 83 years), including 12 patients (43%) who were <or=50 years of age. Apical aneurysms varied considerably in size (maximum dimension, 10 to 66 mm), were dyskinetic/akinetic with thin rims, and were associated with transmural (and often more extensive) myocardial scarring identified by late gadolinium enhancement cardiovascular magnetic resonance. Apical aneurysms were recognized by echocardiography in only 16 of 28 patients (57%) but by cardiovascular magnetic resonance in the 12 patients undetected by echocardiography. Left ventricular chamber morphology varied; however, 19 patients (68%) showed an "hourglass" contour, with midventricular hypertrophy producing muscular narrowing and intracavitary gradients in 9 patients (74+/-42 mm Hg). Sarcomeric protein missense mutations known to cause other phenotypic expressions of HCM were present in 3 patients. Over 4.1+/-3.7 years of follow-up, 12 patients (43%) with left ventricular apical aneurysms experienced adverse disease complications (event rate, 10.5%/y), including sudden death, appropriate implantable cardioverter-defibrillator discharges, nonfatal thromboembolic stroke, and progressive heart failure and death. Patients with left ventricular apical aneurysms represent an underappreciated subset in the heterogeneous HCM disease spectrum with important clinical implications, often requiring a high index of suspicion and cardiovascular magnetic resonance for identification. Apical aneurysms in HCM are associated with substantial cardiovascular morbidity and mortality and raise novel treatment considerations.

Extensive Subepicardial Fibrosis in a Patient With Apical Hypertrophic Cardiomyopathy With Persistent ST-Segment Elevation Simulating Acute Myocardial Infarction

HomeCirculationVol. 112, No. 3Extensive Subepicardial Fibrosis in a Patient With Apical Hypertrophic Cardiomyopathy With Persistent ST-Segment Elevation Simulating Acute Myocardial Infarction Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessReview ArticlePDF/EPUBExtensive Subepicardial Fibrosis in a Patient With Apical Hypertrophic Cardiomyopathy With Persistent ST-Segment Elevation Simulating Acute Myocardial Infarction Jong-Won Ha, Byoung-Wook Choi, Se-Joong Rim, Seok-Min Kang, Yangsoo Jang, Namsik Chung, Won-Heum Shim and Seung-Yun Cho Jong-Won HaJong-Won Ha From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author , Byoung-Wook ChoiByoung-Wook Choi From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author , Se-Joong RimSe-Joong Rim From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author , Seok-Min KangSeok-Min Kang From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author , Yangsoo JangYangsoo Jang From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author , Namsik ChungNamsik Chung From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author , Won-Heum ShimWon-Heum Shim From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author and Seung-Yun ChoSeung-Yun Cho From Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, Seoul, Korea. Search for more papers by this author Originally published19 Jul 2005https://doi.org/10.1161/CIRCULATIONAHA.104.479386Circulation. 2005;112:e49–e50Apical hypertrophic cardiomyopathy is a unique form of hypertrophic cardiomyopathy (HCM), in which the hypertrophy of myocardium predominantly involves the apex of the left ventricle. The ECG in apical HCM typically shows repolarization changes in the anterolateral leads and sometimes giant negative T waves. Previous reports have shown that apical HCM may mimic myocardial infarction, although its underlying mechanism is unclear. This case report demonstrates typical echocardiographic and MRI features of apical HCM, but with unusual electrocardiographic features characterized by chronic ST-segment elevation in the precordial leads. Contrast-enhanced MRI showed unusual extensive subepicardial delayed hyperenhancement at the left ventricular apex, suggesting that subepicardial fibrosis may be a possible cause for this unusual ECG abnormality in patients with apical HCM. Because this condition can be misdiagnosed as acute infarction, resulting in unwarranted thrombolytic therapy or emergency angiography, we believe this case is important as a reminder that acute infarction is not the only cause of ST-segment elevation.In March 2003, a 44-year-old man presented with exertional dyspnea. He was normotensive and had never experienced chest pain or other symptoms suggestive of heart disease. A routine 12-lead ECG (Figure 1) revealed an ST-segment elevation in V3 to V6 precordial leads, which made us suspect possible acute myocardial infarction. Physical examination revealed no abnormal findings, and cardiac biomarkers were negative. Echocardiography and MRI (Figure 2 and Movies) revealed a severe hypertrophy confined to the left ventricular apex, suggesting apical HCM. Coronary angiography by MRI showed no significant luminal narrowing (Figure 3). A contrast-enhanced image obtained by MRI showed prominent delayed hyperenhancement localized at the subepicardial area of the left ventricular apex (Figure 4). Follow-up ECG obtained 1 year later showed persistent ST elevation in precordial leads without interval change (Figure 5). Download figureDownload PowerPointFigure 1. Initial ECG showing ST-segment elevation in V3 to V6 precordial leads, indicative of possible acute myocardial infarction.Download figureDownload PowerPointFigure 2. MRI revealed severe hypertrophy confined to left ventricular apex, suggesting apical HCM.Download figureDownload PowerPointFigure 3. Coronary angiography by MRI showed no significant luminal narrowing.Download figureDownload PowerPointFigure 4. Contrast-enhanced image obtained by MRI of 4-chamber view (left) and 2-chamber view (right) showed prominent delayed hyperenhancement localized at subepicardial area of left ventricular apex.Download figureDownload PowerPointFigure 5. Follow-up ECG 1 y later showed persistent ST elevation in precordial leads without interval change.The Data Supplement is available at http://www.circ.ahajournals.org/cgi/content/full/112/3/e49/DC1.FootnotesCorrespondence to Jong-Won Ha, MD, PhD, Cardiology Division, Yonsei University College of Medicine, Yonsei Cardiovascular Center, CPO Box 8044, Seoul, Korea. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By Wu H, Cao Y and Liang L (2021) Persistent ST-Segment Elevation in a Young Man, JAMA Internal Medicine, 10.1001/jamainternmed.2021.4432, 181:11, (1514), Online publication date: 1-Nov-2021. Ozeke O, Ertan C, Keskin G, Deveci B, Cay S, Ozcan F, Topaloglu S, Aras D, Demir A and Aydogdu S (2015) Association of ST elevation with apical aneurysm in hypertrophic cardiomyopathy, Indian Heart Journal, 10.1016/j.ihj.2015.05.019, 67:5, (434-439), Online publication date: 1-Sep-2015. July 19, 2005Vol 112, Issue 3 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.104.479386PMID: 16027264 Originally publishedJuly 19, 2005 PDF download Advertisement SubjectsCardiomyopathyComputerized Tomography (CT)