Accurate diagnosis of apical hypertrophic cardiomyopathy using explainable advanced electrocardiogram analysis.

AIMS: Typical electrocardiogram (ECG) features of apical hypertrophic cardiomyopathy (ApHCM) include tall R waves and deep or giant T-wave inversion in the precordial leads, but these features are not always present. The ECG is used as the gatekeeper to cardiac imaging for diagnosis. We tested whether explainable advanced ECG (A-ECG) could accurately diagnose ApHCM. METHODS AND RESULTS: Advanced ECG analysis was performed on standard resting 12-lead ECGs in patients with ApHCM [n = 75 overt, n = 32 relative (<15 mm hypertrophy); a subgroup of which underwent cardiovascular magnetic resonance (n = 92)], and comparator subjects (n = 2449), including healthy volunteers (n = 1672), patients with coronary artery disease (n = 372), left ventricular electrical remodelling (n = 108), ischaemic (n = 114) or non-ischaemic cardiomyopathy (n = 57), and asymmetrical septal hypertrophy HCM (n = 126). Multivariable logistic regression identified four A-ECG measures that together discriminated ApHCM from other diseases with high accuracy [area under the receiver operating characteristic (AUC) curve (bootstrapped 95% confidence interval) 0.982 (0.965-0.993)]. Linear discriminant analysis also diagnosed ApHCM with high accuracy [AUC 0.989 (0.986-0.991)]. CONCLUSION: Explainable A-ECG has excellent diagnostic accuracy for ApHCM, even when the hypertrophy is relative, with A-ECG analysis providing incremental diagnostic value over imaging alone. The electrical (ECG) and anatomical (wall thickness) disease features do not completely align, suggesting that future diagnostic and management strategies may incorporate both features.

Usefulness of ECG to differentiate apical hypertrophic cardiomyopathy from non-ST elevation acute coronary syndrome.

BACKGROUND: Apical hypertrophic cardiomyopathy (ApHCM) is a phenotypic variant of nonobstructive HCM. ApHCM is characterized by left ventricular hypertrophy involve the distal apex. The electrocardiographic character of ApHCM can mimic non-ST elevation acute coronary syndrome (NSTEACS), triggering a series of studies and treatments that may be unnecessary. This study aimed to clarify the electrocardiogram (ECG) differences between the two diseases. METHODS: Initial ECG recordings of 41 patients with ApHCM and 72 patients with NSTEACS were analyzed retrospectively. We analyzed the voltage of negative T (neg T) and R wave, the change of ST-segment as well as the number of leads with neg T wave in the 12-lead ECGs. RESULTS: Across the 12-lead ECGs, the magnitude of R wave significantly differed between ApHCM and NSTEACS in 10 leads excluding leads aVR and V1. ApHCM was associated with a greater maximal amplitude of R wave in lead V5 (3.13 ± 1.08 vs. 1.38 ± 0.73 mV, P <  0.001). The magnitude of T wave significantly differed between ApHCM and NSTEACS in 10 leads excluding leads II and V1. ApHCM was associated with a greater maximal amplitude of neg T wave in lead V4 (0.85 ± 0.69 vs. 0.35 ± 0.23 mV, P <  0.001). The frequency of giant neg T (1mv or more) wave was higher in ApHCM (36.5% vs. 0%, P <  0.001). The magnitude of ST-segment deviation significantly differed between ApHCM and NSTEACS in 10 leads excluding leads aVF and V2. ApHCM was associated with a greater maximal amplitude of ST-segment depression in lead V5 (0.19 ± 0.07 vs. 0.03 ± 0.06 mV, P <  0.001). The number of leads with neg T wave also differed between ApHCM and NSTEACS (6.75 ± 1.42 vs. 6.08 ± 1.51, P = 0.046). The sum of R wave in lead V5, neg T wave in lead V6 and ST-segment depression in lead V4 > 2.585 mV identified ApHCM with 90.2% sensibility and 87.5% specificity, representing the highest diagnostic accuracy. CONCLUSIONS: Compared with NSTEACS patients, ApHCM patients presented higher R and neg T wave voltage as well as a greater ST-segment depression in the 12-lead ECG. The ECG characteristics can help to differentiate ApHCM from NSTEACS in clinical setting.

Echocardiographic Evaluation of Left Atrial Function to Discriminate Non-Valvular Atrial Fibrillation Development in Patients with Apical Hypertrophic Cardiomyopathy.

BACKGROUND: Data is limited on baseline left atrial (LA) myocardial mechanics between apical hypertrophic cardiomyopathy (ApHCM) patients who develop non-valvular atrial fibrillation (NVAF) during follow-up and those who do not. METHODS: This retrospective study investigated the clinical outcomes of consecutive patients newly diagnosed with ApHCM between August 2011 and July 2014 who were followed-up for at least 3 years. The patients underwent 12-lead surface electrocardiography and/or 24-hour Holter electrocardiography at least once a year. The patients were divided into two groups, namely those who did or did not exhibit NVAF during follow-up, respectively. The baseline clinical and echocardiographic data of the two groups were compared. RESULTS: Twenty patients were studied, five of whom were lost to follow-up. Of the remaining 15 ApHCM patients, seven developed NVAF. No differences were observed in the clinical characteristics of the two groups. However, for the echocardiographic data, the NVAF development group exhibited a larger LA volume and impaired LA reservoir, conduit and booster functions. The NVAF development group also showed lower peak LA strain and stiffer left atrium. The LA volume, function, global strain and stiffness were all statistically associated with NVAF development. Among these parameters, a LA conduit function of ≤ 24.9% was found to be the best parameter to discriminate NVAF development. CONCLUSIONS: The baseline LA function was impaired in the ApHCM patients who subsequently developed NVAF during follow-up. A LA conduit function of ≤ 24.9% was strongly associated with NVAF development.

Left ventricular apical hypertrophy in a transplanted heart: a case report.

BACKGROUND: Left ventricular apical hypertrophic cardiomyopathy is a rare presentation of hypertrophic cardiomyopathy associated with thickening of the apical segment of the left ventricle. It was initially described in Japan in 1976 and is characterized by electrocardiogram findings showing giant T wave inversions in the precordial leads as well as a spade shaped appearance of the apical cavity on imaging (Abugroun et al., Cardiol Res 8:265-268, 2017). In this case, we present a patient with a heart transplant with a stable post-transplant course who was found to have apical hypertrophic cardiomyopathy. There have been a few cases of apical hypertrophy in a transplanted heart documented in the literature. Making this case even more unique is that this presentation is evident 17 years after heart transplantation. CASE PRESENTATION: Fifty-four year-old male with a history of orthotropic heart transplant in 2001 on immunosuppressive therapy presented with palpitations and associated lightheadedness. He had a blood pressure of 184/89 mmHg on arrival but otherwise had stable vital signs and physical examination. Cardiac biomarkers revealed a CK of 59 U/L and a troponin of 0.11NG/ML(normal < 0.04NG/ML). B type natriuretic peptide was 371 PG/ML(normal 0-100PG/ML). Routine laboratory studies demonstrated normal sodium, magnesium, serum creatinine, and a potassium of 3.3 mmol/L(normal 3.5-5.1 mmol/L). His hemoglobin and hematocrit were normal. His EKG showed sinus rhythm with old T wave inversions in the anterior and lateral leads. Echocardiogram revealed a left ventricular ejection fraction of 55-65%, left posterior wall of 1.3 cm and interventricular septal wall 1.2 cm, thickened trabeculated apex, with severely dilated left atrium. He had a stress test that showed mild inferior wall thinning and a cardiac MRI performed to further evaluate apical hypertrophy revealed prominent apical hypertrophy of the left ventricle with near obliteration of the apical cavity. He had no events on cardiac monitoring and was discharged with close followup with the transplant team. CONCLUSION: While there are many etiologies of ApHCM, it has not been well described in transplanted patients who are on chronic immunosuppressive therapy. It is unclear if these groups of patients are at an increased risk of developing this condition. The literature suggests that ApHCM is associated with a being prognosis but there is new data suggesting increased mortality in a subset of patients with this condition.

Electrocardiographic predictors of myocardial fibrosis and apical hypertrophic cardiomyopathy.

BACKGROUND: Electrocardiography (ECG) may be an efficacious diagnostic and prognostic tool in hypertrophic cardiomyopathy (HCM). This study aimed to investigate association between deep T-wave inversion (TWI) and apical HCM, and between fragmented QRS (fQRS) complex and myocardial fibrosis in patients with HCM. METHODS: Patients with documented HCM by cardiac magnetic resonance imaging (CMR) during 2005-2015 were studied. The 12-lead ECG and CMR were performed on the same day. All patients underwent CMR for the assessment of cardiac structure, function, and late gadolinium enhancement (LGE). LGE was used to detect myocardial fibrosis. RESULTS: One hundred forty-four HCM (mean age 66 ± 15.8 years, 60.4% male) were included. Twenty-nine (20.14%) subjects had deep TWI, and apical HCM was found in 76 (52.78%). Deep TWI was associated with apical HCM with the Odds ratio (95%CI) of 5.82 (2.07, 16.04) and p < 0.001 in univariate analysis model. The association was still significant in multivariate analysis with adjusted Odds ratio (95%CI) of 9.86 (3.17, 30.66), p < 0.001. Forty-seven (32.64%) subjects had fQRS complex, and myocardial fibrosis was detected in 101 (70.14%). fQRS complex was found to be associated with myocardial fibrosis in univariate analysis with the Odds ratio (95%CI) = 2.75 (1.16, 6.54), p = 0.019. However, the association cannot be demonstrated in the multivariate analysis. CONCLUSION: Deep TWI is independently associated with apical HCM, but the relationship between fQRS complex and myocardial fibrosis did not survive multivariate analysis.

The evolution of apical hypertrophic cardiomyopathy: Development of mid-ventricular obstruction and apical aneurysm 11 years after initial diagnosis.

Asian hypertrophic cardiomyopathy (AHCM) is a rare variant of hypertrophic cardiomyopathy (HCM) that is more prevalent in the Asian population. There is significant overlap between AHCM, mid-cavitary obstruction, and apical aneurysms. Although more benign compared to HCM, the course of AHCM is not clearly defined. We present an interesting case of an African American male with known AHCM who develops symptomatic mid-cavitary obstruction and apical aneurysm 11 years after initial diagnosis.

Apical hypertrophic cardiomyopathy: an uncommon cause of exertional chest pain in children.

Apical hypertrophic cardiomyopathy is an uncommon morphologic variant of hypertrophic cardiomyopathy, which is rarely diagnosed in childhood. To date, very few cases of asymptomatic children younger than 18 years have been reported in the literature. To the best of our knowledge, this is the first case of paediatric apical hypertrophic cardiomyopathy presenting with exertional chest pain, with characteristic electrocardiographic, echocardiographic, MRI, and cardiac angiography findings.

Long-term outcome of catheter ablation for atrial fibrillation in patients with apical hypertrophic cardiomyopathy.

INTRODUCTION: To assess the long-term outcome of catheter ablation in patients with hypertrophic cardiomyopathy (HCM), especially in patients with apical HCM (ApHCM). METHODS AND RESULTS: From 9,249 AF ablation cases, 97 patients (28 with ApHCM and 69 with non-ApHCM) were enrolled. Another 97 patients matched by age, AF type, AF duration, and left atrial diameter were selected as the control group. After a mean follow-up of (44.3 ± 29.6) months, success rate after a single procedure was 42.9% in the ApHCM patients (P  =  0.725), 36.2% in the non-ApHCM patients (P  =  0.136) versus 50.5% in the control group. After multiple procedures, success rate both in the ApHCM group (50%, P  =  0.047) and in the non-ApHCM group (50.4%, P  =  0.017) were lower than in the controls (68.0%). More patients in the ApHCM and in the non-ApHCM group suffered very late recurrence beyond 1 year after the index procedure. Left atrial diameter (hazard ratio [HR] 1.04, 95% confidential interval [CI] 1.01-1.08, P  =  0.018) and AF duration (HR 1.01, 95% CI 1.00-1.01, P  =  0.005) were independent predictors of recurrence after the index ablation. There was no difference in thromboembolic events between the HCM group and the control group (8.2% vs. 3.1%, P  =  0.082). CONCLUSIONS: Patients with ApHCM or non-ApHCM had similar success rate of AF ablation after single procedure and lower success rate after multiple procedure compared with the control group.

Clinical Outcomes of Myocardial Bridging versus No Myocardial Bridging in Patients with Apical Hypertrophic Cardiomyopathy.

OBJECTIVE: To determine the prevalence and clinical effects of myocardial bridging (MB) in patients with apical hypertrophic cardiomyopathy (AHCM). METHODS: Angiograms from 212 AHCM patients were reviewed to identify MB. The patients were classified into 2 groups: AHCM with and AHCM without MB. We reviewed patient records on cardiovascular (CV) risk factors, symptoms, CV events, and CV mortality. RESULTS: In all, 60 patients with MB and 100 without MB were included. Rates of angina (61.7 vs. 40%; p = 0.008), mimicking non-ST-segment elevation myocardial infarction (15 vs. 3%, p = 0.013), and Canadian Cardiovascular Society class III/IV angina (18.3 vs. 4%; p = 0.003) were higher in patients with MB than in those without. Mean follow-up periods (65.5 ± 50.5 vs. 64.4 ± 43.6 months, p = 0.378) and CV mortality (3.3 vs. 1%; p = 0.652) were similar in the 2 groups. Kaplan-Meier estimates demonstrated that CV event-free survival rates were lower in patients with MB than in those without (71.7 vs. 88%; p = 0.022). MB, late gadolinium enhancement, and female sex were independent risk factors for CV events in a multivariate Cox regression analysis adjusted for other risk factors. CONCLUSION: More serious symptoms and a higher risk of CV events were observed in AHCM patients with MB than in those without MB. CV mortality was similar in these 2 groups.

Apical Hypertrophic Cardiomyopathy: A Concerning Electrocardiogram in the Emergency Department.

BACKGROUND: Apical hypertrophic cardiomyopathy is a rare, complex phenotypic variant of the classically taught hypertrophic cardiomyopathy. It is highly variable in its pathophysiology as well as its clinical course, spanning the spectrum from a healthy asymptomatic lifestyle to sudden cardiac death and severe diastolic dysfunction. The hallmark electrocardiographic findings of unusually large T-wave inversions, in conjunction with the most common presenting symptom being chest pain, makes this disease entity concerning in the emergency department (ED) setting. CASE REPORT: A 61-year-old man with a history of hypertension presented to the ED with chest pain. His electrocardiogram exhibited a biphasic T wave in lead V2 with ST depressions in leads V3-V6 with deep symmetrical T-wave inversions in these leads as well. His troponin was negative and the patient was taken for cardiac catheterization. Catheterization revealed no coronary artery disease; however, it revealed a "spade like" filling pattern of the left ventricle, suggestive of an apical variant of hypertrophic cardiomyopathy. Subsequent cardiac magnetic resonance imaging confirmed the diagnosis of apical hypertrophic cardiomyopathy and the patient was started on a beta-blocker and discharged with cardiology follow-up. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Electrocardiographic interpretation is a critical skill of the emergency physician. Awareness of the syndrome and its specific electrocardiogram findings may help facilitate further testing that will aid in timely diagnosis and interventions.

[Electrocardiographic differences between apical hypertrophic cardiomyopathy and apical non-ST segment myocardial infarction]. / Diferencias electrocardiográficas en miocardiopatía hipertrófica apical e infarto apical sin elevación del segmento ST.

Apocal hypertrophic cardiomyopathy (AHCM) is a phenotypic variant within hypertrophic cardiomyopathies, in which ventricular repolarization alterations are present. These electrocardiographic disturbances can mimic an anterior infarction which triggers a series of studies and treatments that may be unnecessary. The aim of this study was to describe and compare electrocardiographic differences in a series of patients with AHCM and apical non-ST segment elevation myocardial infarction in patients (NSTEMI) with T-wave changes. We conducted an observational and retrospective study, including patients with diagnosed AHCM (N = 19) and apical NSTEMI (N = 19) with negative T waves in V1 and V6 lead of the EKG. Those with AHCM presented higher T-wave voltage (7 mV vs. 5 mV, p = 0.001) and peak voltage (29 mV vs. 17 mV, p = 0.003), higher R-waves (25 mV vs. 10 mV, p = 0.0001), and a maximum voltage of R and T sum (R + T) significantly higher (33 vs. 14, p = 0.00001). They also showed a greater T-wave asymmetry, with a TiTp / TpTf ratio > 1. At a cut-off value of 26.5 mV for the R + T variable, 68% sensitivity and 100% specificity were obtained to diagnose AHCM. This study shows the existence of major differences in electrocardiographic presentation of AHCM and apical NSTEMI.

[A Case of Apical Hypertrophic Cardiomyopathy With Rare Anomaly of Coronary Arteries Origin].

A clinical case of apical hypertrophic cardiomyopathy (HCM) in 44­years old man is presented. In this patient exercise ECG testing and 24­hour ECG monitoring revealed exercise-induced ST depression in the angiographically confirmed absence of coronary atherosclerosis. The uncommonness of this observation was the combination of HCM with a rare anomaly of coronary arteries origin.

Cardiac CT angiography in the emergency room: Apical hypertrophic cardiomyopathy presenting as acute coronary syndrome.

A 59-year-old male presented to the emergency room with symptoms of chest tightness and palpitations. Following conversion of atrial fibrillation to sinus rhythm, he had deep symmetrical T-wave changes on his electrocardiogram. Symptoms resolved almost immediately, and his initial troponin was negative. He underwent cardiac CT angiography utilizing an emergency room triage protocol which resulted in a diagnosis of nonobstructive coronary artery disease and apical hypertrophic cardiomyopathy. Following a hospital stay of less than 24 hours, he was discharged to outpatient follow-up on medical management and has remained asymptomatic over 6 months. This case presentation illustrates an example of the diversity of pathology that presents in emergency rooms with symptoms consistent with acute coronary syndrome.

Long-Term Outcome of Catheter Ablation for Atrial Fibrillation in Patients With Apical Hypertrophic Cardiomyopathy.

INTRODUCTION: Atrial fibrillation (AF) is a common manifestation in cases of hypertrophic cardiomyopathy (HCM). Catheter ablation (CA) for AF in patients with asymmetric septal HCM (SeHCM) is selectively effective and often needs a repeat procedure. Apical HCM (ApHCM) has a better prognosis than SeHCM. However, the outcome of CA for AF in patients with ApHCM is unclear. METHODS AND RESULTS: Eighteen patients with ApHCM (ApHCM group) and 13 SeHCM patients (SeHCM group) underwent CA for AF. Ninety sex-, age-, and AF type-matched non-HCM patients who underwent CA for AF were selected as controls (5 controls for each ApHCM patient). During a median follow-up of 44.7 ± 30.8 months, 50% of the patients remained free from AF/atrial tachycardia (AT) in the ApHCM group. The ApHCM patients displayed enlarged left atrial (LA) diameter (47.1 ± 6.0 mm vs. 42.4 ± 5.5 mm, P = 0.006) and increased E/Ea ratio (13.5 ± 4.4 vs. 9.1 ± 3.1, P < 0.001) as compared to the control group. In contrast, the mean LA diameter and E/Ea ratio of the ApHCM group were not different than those of the SeHCM group. The overall freedom from AF/AT in the ApHCM group was significantly worse than in the control group (log rank P = 0.028), but there was no difference between the ApHCM and SeHCM groups (P = 0.831). High LA diameter index ≥25 mm/m(2) (HR 12.8, 95% CI [1.2-142.1]; P = 0.037) was an independent predictor of AF/AT recurrence among patients with ApHCM. CONCLUSION: Long-term outcome of CA for AF was worse in patients with ApHCM, as compared to controls, but was similar to patients with SeHCM.

The Incremental Value of Magnetic Resonance Imaging for Identification of Apical Pouch in Patients with Apical Variant of Hypertrophic Cardiomyopathy.

BACKGROUND: The development of a left ventricular (LV) apical pouch in patients with apical hypertrophic cardiomyopathy (aHCM) has been thought to be the transition point that can become an apical aneurysm, which is linked to higher risk of adverse events. In our study, we sought to compare the ability of transthoracic echocardiography (echo) and cardiac magnetic resonance imaging (cMRI) to accurately identify the presence of an apical pouch or aneurysm in patients with aHCM. METHODS: We retrospectively reviewed the charts of all consecutive patients that had features of aHCM on imaging. Data from cMRI and echo examinations were abstracted, and the ability of these diagnostic modalities to identify the presence of a LV apical pouch and aneurysm was analyzed. RESULTS: Of 31 patients with aHCM, 17 (54.8%) had an apical pouch and 2 were found to have apical aneurysm (6.5%) on cMRI. Echo with and without perflutren contrast was able to accurately identify both aneurysms, but only 47.1% (8/17) of apical pouches seen by cMRI. Two patients had apical thrombus that was identified by cMRI, but not by echo. CONCLUSION: Our findings indicate that cMRI is superior to echo in identifying apical pouches in patients with aHCM. Our results also suggest that in patients undergoing echo, the use of perflutren contrast for LV opacification increases the diagnostic yield. Further study is necessary to delineate whether earlier identification of an apical pouch will be of clinical benefit for patients with aHCM by altering clinical management and avoiding adverse cardiovascular events.

Myocardial fibrosis evaluated by Look-Locker and late gadolinium enhancement magnetic resonance imaging in apical hypertrophic cardiomyopathy: association with ventricular tachyarrhythmia and risk factors.

PURPOSE: To evaluate the distribution and extent of myocardial fibrosis identified by either contrast-enhanced Look-Locker or late gadolinium enhancement magnetic resonance imaging (LGE MRI) and their relationships between ventricular tachyarrhythmia or risk factors in apical hypertrophic cardiomyopathy (APH). MATERIALS AND METHODS: Twenty-five APH patients were examined using a 3.0 T or 1.5 T instrument. We used MRI to evaluate myocardial T1 values and scar. We compared the myocardial fibrosis assessed by contrast-enhanced Look-Locker or LGE MRI with ventricular tachyarrhythmia or risk factors for hypertrophic cardiomyopathy. RESULTS: Myocardial scar was present in 17 of the 25 patients with APH. Myocardial scar was distributed predominantly in the apical myocardium (P < 0.01), whereas myocardial T1 values did not differ between the apical, midventricular, and basal septum. The extent of myocardial scar according to 16-segment model and ejection fraction were related to ventricular tachyarrhythmia or risk factors in APH (P < 0.05 for both). The myocardial T1 value was not associated with the tachyarrhythmia or risk factors. CONCLUSION: In APH, the extent of myocardial scar on LGE MRI is associated with ventricular tachyarrhythmia and risk factors. Quantification of the myocardial T1 value is not necessary for its risk stratification.

Evolutionary change mimicking apical hypertrophic cardiomyopathy in a patient with takotsubo cardiomyopathy.

In this report, we introduce a case of thickening of the involved left ventricular apical segment on echocardiography and deep T-wave inversions in precordial leads on electrocardiography transiently seen in the course of recovery from biventricular takotsubo cardiomyopathy, mimicking apical hypertrophic cardiomyopathy. This result suggests that the echocardiographic finding of transient myocardial edema can be identified by cardiac magnetic resonance imaging in takotsubo cardiomyopathy. Additionally, it persisted a few weeks after full functional recovery. We believe that this case will contribute in part toward clarifying the pathophysiology of takotsubo cardiomyopathy.

Invasive Cardiac Hemodynamics in Apical Hypertrophic Cardiomyopathy.

BACKGROUND: Symptomatic limitations in apical hypertrophic cardiomyopathy may occur because of diastolic dysfunction with resultant elevated left ventricular filling pressures, cardiac output limitation to exercise, pulmonary hypertension (PH), valvular abnormalities, and/or arrhythmias. In this study, the authors aimed to describe invasive cardiac hemodynamics in a cohort of patients with apical hypertrophic cardiomyopathy. METHODS AND RESULTS: Patients presenting to a comprehensive hypertrophic cardiomyopathy center with apical hypertrophic cardiomyopathy were identified (n=542) and those who underwent invasive hemodynamic catheterization (n=47) were included in the study. Of these, 10 were excluded due to postmyectomy status or incomplete hemodynamic data. The mean age was 56±18 years, 16 (43%) were women, and ejection fraction was preserved (≥50%) in 32 (91%) patients. The most common indication for catheterization was dyspnea (48%) followed by suspected PH (13%), and preheart transplant evaluation (10%). Elevated left ventricular filling pressures at rest or exercise were present in 32 (86%) patients. PH was present in 30 (81%) patients, with 6 (20%) also having right-sided heart failure. Cardiac index was available in 25 (86%) patients with elevated resting filling pressures. Of these, 19 (76%) had reduced cardiac index and all 6 with right-sided heart failure had reduced cardiac index. Resting hemodynamics were normal in 8 of 37 (22%) patients, with 5 during exercise; 3 of 5 (60%) patients had exercise-induced elevation in left ventricular filling pressures. CONCLUSIONS: In patients with apical hypertrophic cardiomyopathy undergoing invasive hemodynamic cardiac catheterization, 86% had elevated left ventricular filling pressures at rest or with exercise, 81% had PH, and 20% of those with PH had concomitant right-sided heart failure.

Apical hypertrophic cardiomyopathy: pathophysiology, diagnosis and management.

Since the first description of apical hypertrophic cardiomyopathy (ApHCM) in 1976, contrasting information from all over the world has emerged regarding the natural history of the disease. However, the recommended guidelines on hypertrophic cardiomyopathy (HCM) pay a cursory reference to ApHCM, without ApHCM-specific recommendations to guide the diagnosis and management. In addition, cardiologists may not be aware of certain aspects that are specific to this disease subtype, and a robust understanding of specific disease features can facilitate recognition and timely diagnosis. Therefore, the review covers the incidence, pathogenesis, and characteristics of ApHCM and imaging methods. Echocardiography and cardiovascular magnetic resonance imaging (CMR) are the most commonly used imaging methods. Moreover, this review presents the management strategies of this heterogeneous clinical entity. In this review, we introduce a novel transapical beating-heart septal myectomy procedure for ApHCM patients with a promising short-time result.

Interventricular Septal Involvement Is Associated with More Impaired Ventricular Function and Mechanics in Apical Hypertrophic Cardiomyopathy.

BACKGROUND: The interventricular septum has an important role in bi-ventricular performance. We hypothesized that septal involvement in apical hypertrophic cardiomyopathy (ApHCM-Mixed) adversely impacts ventricular structure and function when compared with isolated apical hypertrophy (ApHCM-Pure). METHODS: A total of 72 patients (ApHCM-Mixed = 36, ApHCM-Pure = 36) with serial 2D and speckle-tracking echocardiographic analyses were identified. Ventricular function and mechanics were characterized by left (LV) and right (RV) ventricular global longitudinal strain (GLS), RV free wall strain, and LV myocardial work indices, and clinical events were adjudicated. RESULTS: Clinical characteristics were similar between groups (mean age, 66 ± 15 years; 49% female; LV ejection fraction, 68 ± 11%). The ApHCM-Mixed group had larger LV mass indexes (141 ± 39 vs. 111 ± 30 g/m2, p < 0.001), worse LV (-9.6 ± 3.1 vs. -14.4 ± 3.4%, p < 0.001) and RV GLS (-14.3 ± 6.7 vs. -19.2 ± 5.2%, p = 0.001), impaired RV free wall strain (-18.5 ± 7.4 vs. -22.4 ± 6.3%, p = 0.02), and lower LV myocardial work indices including global work index (938 ± 306 vs. 1272 ± 339 mmHg%, p < 0.001), when compared with the ApHCM-Pure group. At a mean follow-up of 3.9 years, these differences all persisted. Five deaths were observed, all occurring in the ApHCM-Mixed group (14% vs. 0, p = 0.05), and with four being cardiac-related. This subgroup had a mean LV ejection fraction of 63%, LV GLS of -8.7%, an LV global work index of 875 mmHg%, and RV free wall strain of -15.9%, indicating significant subclinical bi-ventricular dysfunction. CONCLUSIONS: ApHCM-Mixed represents a distinct morphology in hypertrophic cardiomyopathy associated with more impaired ventricular function and mechanics when compared with ApHCM-Pure.