High-risk imaging characteristics in left ventricular apex for the life-threatening arrhythmic events in Japanese hypertrophic cardiomyopathy patients.

Left ventricular (LV) apical aneurysm is known to be associated with the life-threatening arrhythmic events in hypertrophic cardiomyopathy (HCM). However, the current 2014 ESC guideline has not included apical aneurysm as a major risk factor for sudden cardiac death and 2018 JCS guideline includes it only as a modulator, while it has been included as a new major risk marker in 2020 AHA/ACC guideline. Therefore, we sought to identify high-risk imaging characteristics in LV apex which is associated with a higher occurrence of ventricular tachycardia/fibrillation (VT/VF). In 99 consecutive Japanese HCM patients (median age, 65 years; 59 males) undergoing implantable cardioverter-defibrillator (ICD) implantation for primary prevention following cardiac magnetic resonance including late gadolinium enhancement (LGE), the occurrence of appropriate ICD interventions for VT/VF was evaluated for 6.2 (median) years after ICD implantation. Overall, appropriate ICD interventions occurred in 43% with annual rates of 7.0% for appropriate interventions. Kaplan-Meier analysis demonstrated that the presence of LV apical aneurysm was significantly associated with a higher occurrence of appropriate interventions (annual rates 18.9% vs. 6.4%, P = 0.013). Similarly, patients with high LV mid-to-apex pressure gradient (annual rates 14.9% vs. 6.2%, P = 0.022) and presence of apical LGE (annual rates 10.9% vs. 4.0%, P = 0.001) experienced appropriate interventions more frequently. An aneurysm, high-pressure gradient, and LGE in an apex are associated with VT/VF. These characteristics in apex should be kept in mind when implanting ICD in Japanese HCM patients as a primary prevention.

Additive prognostic significance of ejection fraction for ESC risk model in hypertrophic cardiomyopathy patients.

The European Society of Cardiology (ESC) clinical risk model is reported in predicting sudden death of hypertrophic cardiomyopathy (HCM). We examined the validity of this model and investigated the significance of ejection fraction (EF) in predicting the prognosis using ESC risk model in HCM patients. 305 HCM patients (198 males) were followed (median follow-up 4.8 years) for life-threatening arrhythmic events (sudden death, aborted sudden death, sustained VT/VF, appropriate ICD intervention for VT/VF) and were divided using ESC risk model into low- (Group L), intermediate- (Group I) and high- (Group H) risk groups. There was a significant difference in the events rate among the 3 groups (L, 0.9%/year; I, 3.9%/year; H, 6.8%/year; log-rank p < 0.001) in all study patients. Reduced EF (<50%) was identified in 27 (8.9%) cases. There was a significant difference in the events rate among the 3 groups in patients with reduced EF (L, 2.4%/year; I, 4.9%/year; H, 16.1%/year; log-rank p = 0.025). There was a significant difference in the events rate among 2 groups in patients stratified as Group H (preserved EF, 3.1%/year vs. reduced EF, 16.1%/year; log-rank p = 0.041). ESC risk model precisely predicts life-threatening events in patients with HCM. Adding EF to ESC risk model are useful for further risk stratification of life-threatening arrhythmic events.

High Signal Intensity on T2-Weighted Cardiovascular Magnetic Resonance Imaging Predicts Life-Threatening Arrhythmic Events in Hypertrophic Cardiomyopathy Patients.

BACKGROUND: The prognostic value of high signal intensity on T2-weighted cardiovascular magnetic resonance imaging (T2 high signal) in hypertrophic cardiomyopathy (HCM) patients in a single-center cohort was investigated.Methods and Results:A total of 237 HCM patients (median age, 62 years; 143 male) underwent T2-weighted, cine and late gadolinium enhancement (LGE) imaging, and were followed (median duration, 3.4 years) for life-threatening arrhythmic events. The clinical and magnetic resonance imaging characteristics were extracted, and predictors of life-threatening arrhythmic events were assessed on multivariate analysis. LGE was present in 180 patients (75.9%). Median LGE score was 3 in a left ventricle 17-segment model. T2 high signal was present in 49 patients (20.7%). The annual events rate was significantly higher in patients with extensive LGE (score ≥4) than in those without (3.0%/year vs. 0.5%/year, P=0.011). On multivariate analysis, extensive LGE (hazard ratio, 5.650; 95% CI: 1.263-25.000, P=0.024) as an independent predictor for life-threatening arrhythmic events. In patients with extensive LGE, the annual events rate was significantly higher in patients with T2 high signal than in those without (5.8%/year vs. 0.9%/year, P=0.008). CONCLUSIONS: Extensive LGE was an independent predictor of life-threatening arrhythmic events in HCM patients. Furthermore, T2 high signal is useful for the risk stratification of serious arrhythmic events in patients with extensive LGE.

Prognostic value of cardiovascular magnetic resonance imaging for life-threatening arrhythmia detected by implantable cardioverter-defibrillator in Japanese patients with hypertrophic cardiomyopathy.

Implantable cardioverter-defibrillator (ICD) is effective to prevent sudden death in HCM patients. We reviewed ICD records to analyze the relation between life-threatening arrhythmia and late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) in Japanese hypertrophic cardiomyopathy (HCM) patients. In 102 consecutive patients (median age 63 years, 63 males) implanted with an ICD after CMR with gadolinium enhancement (median follow-up 2.8 years), the outcome of life-threatening arrhythmic events (appropriate ICD interventions for ventricular tachycardia or ventricular fibrillation) was examined. Appropriate interventions rate were 10.3% per year for secondary prevention and 7.4% per year for primary prevention. The annualized ICD-related complication rate was 3.7%. 43/91 patients (47%) implanted ICD for primary prevention had maximum wall thickness ≥20 mm plus LGE in ≥4 of 17 left ventricular segments (cut-off value obtained from ROC curve); the appropriate ICD intervention rate was significantly higher in this group than in other patients group (annualized event rate, 11.1 vs. 4.6%; log-rank P = 0.038). A combination of myocardial hypertrophy and LGE is a useful outcome predictive factor for life-threatening ventricular arrhythmia in Japanese HCM patients.

Planimetry of the Orifice Area in Aortic Valve Stenosis Using Phase-Contrast Cardiac Magnetic Resonance Imaging.

Manual planimetry is a well-established method using transesophageal echocardiography (TEE) to assess the severity of aortic stenosis (AS). TEE, however, is a less than optimal approach in patients with calcified valves. Even when using cine-cardiac magnetic resonance (CMR), it is often difficult to evaluate the true border of the aortic orifice because of jet turbulence. With phase-contrast sequences of CMR, high flow signals at the aortic orifice can be clearly visualized, even in cases with severe calcification and jet turbulence. Therefore, the aims of the present study were to compare the utility of CMR using phase-contrast imaging with TEE and cine-CMR for the performance of planimetry of the aortic valve. The study cohort consisted of 30 consecutive patients with moderate or severe aortic valve stenosis documented by TEE who had undergone phase-contrast and cine-CMR for the evaluation of AS. Manual planimetry of the area of high flow signal was traced over the phase-contrast images at systolic peak, when the aortic valve is maximally opened. The results showed that the aortic valvular area (AVA) value derived from TEE correlated better with phase-contrast planimetry (r2 = 0.84, P < 0.05) than cine-mode planimetry (r2 = 0.57, P < 0.05). Bland-Altman plots indicated that the variation of measuring AVA was greater using the cine-mode method than the phase-contrast method. In conclusion, phase-contrast CMR offers a tool for evaluating the severity of aortic valve stenosis noninvasively. Phase-contrast CMR has the potential to become a routine clinical option as an alternative to TEE, at least in selected cases.

Extent of Late Gadolinium Enhancement on Cardiac Magnetic Resonance Imaging in Japanese Hypertrophic Cardiomyopathy Patients.

BACKGROUND: In addition to the presence of late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR), the extent of LGE is considered clinically important in hypertrophic cardiomyopathy (HCM). We evaluated the extent of LGE on CMR in a large series of Japanese HCM patients. METHODS AND RESULTS: CMR was performed in 317 HCM patients (147 male). The extent of LGE was scored as the sum of LGE-positive segments in a left ventricle (LV) 17-segment model. LGE was present in 246 patients (77.6%). LGE was detected in 3.5±3.1 segments on average. When the patients were divided according to maximum wall thickness (mild, <20 mm; moderate, 20-29 mm; severe, ≥30 mm), median LGE score increased as wall thickness increased (mild, 2 vs. moderate, 4 vs. severe, 5; P=0.000). When the patients were divided according to ejection fraction (EF) (reduced, <50%; low-normal, 50-65%; normal, >65%), median LGE score increased as EF decreased (reduced, 7 vs. low-normal, 4 vs. normal, 2; P=0.000). On multivariate analysis, reduced EF (OR, 0.947, P=0.015), pressure gradient <30 mmHg (OR, 0.359, P=0.000) and increased maximum wall thickness (OR, 1.236, P=0.000) were independent factors associated with extensive LGE. CONCLUSIONS: Progression of LGE was related to increased wall thickness, decreased contractility, and reduced intraventricular pressure gradient.

Prognostic value of late gadolinium enhancement on cardiac magnetic resonance imaging in Japanese hypertrophic cardiomyopathy patients.

BACKGROUND: The prognostic value of late gadolinium enhancement (LGE) on contrast-enhanced cardiovascular magnetic resonance (CMR) in Japanese hypertrophic cardiomyopathy (HCM) patients in a large, single-center cohort was investigated. METHODS AND RESULTS: A total of 345 HCM patients (mean age, 59±17 years; 214 male) underwent CMR with gadolinium enhancement, and were followed (mean duration, 21.8 months) for cardiovascular events. Patients were divided into event-positive and event-negative groups. The clinical and CMR characteristics were compared between the 2 groups, and predictors of cardiovascular events assessed on multivariate analysis. LGE was positive in 252 patients (73%). The annual cardiovascular events rate was significantly higher in patients with LGE than in those without (6.2%/year vs. 0.6%/year, P=0.003). On multivariate analysis, LGE (hazard ratio [HR], 7.436; 95% confidence interval [CI]: 1.001-55.228, P=0.050), increased myocardial mass index (HR, 1.013; 95% CI: 1.002-1.023, P=0.018), reduced left ventricular ejection fraction (HR, 0.965; 95% CI: 0.945-0.985, P=0.001), and atrial fibrillation (HR, 2.257; 95% CI: 1.024-4.976, P=0.043) were significantly associated with cardiovascular events. CONCLUSIONS: The presence of LGE, increased myocardial mass index, reduced left ventricular ejection fraction and atrial fibrillation were independent predictors of adverse prognosis in Japanese HCM patients.

High signal intensity on T2-weighted cardiac magnetic resonance imaging correlates with the ventricular tachyarrhythmia in hypertrophic cardiomyopathy.

Late gadolinium enhancement (LGE) with cardiac magnetic resonance (CMR) can predict ventricular arrhythmia and poor prognosis in hypertrophic cardiomyopathy (HCM) patients. Although myocardial T2-high signal has been reported to appear within LGE in those patients, its clinical significance remains unclear. We investigated the relationship between the T2-high signal and nonsustained ventricular tachycardia (NSVT) in HCM patients. Eighty-one HCM patients who underwent Holter ECG and CMR including T2-weighted and LGE imaging were retrospectively recruited. They were divided into NSVT-positive and NSVT-negative groups. We compared the clinical and CMR characteristics between both of the groups, and assessed predictors of NSVT with multivariate analysis. Myocardial T2-high signal was observed in 15/81 (18.5 %) patients. Each T2-high signal was localized within LGE. Significantly in the NSVT-positive group, the prevalence of atrial fibrillation [5/17 (29.4 %) vs. 2/64 (3.1 %), p = 0.0006] and T2-high signal [9/17 (52.9 %) vs. 6/64 (9.4 %), p < 0.0001] and the left ventricular (LV) end-systolic volume index (32.2 ± 15.9 ml/m(2) vs. 23.3 ± 14.9 ml/m(2), p = 0.034) and the number of segments with LGE (5.8 ± 3.3 vs. 2.7 ± 2.7, p < 0.0001) was increased, and the LV ejection fraction (54.8 ± 10.9 % vs. 65.1 ± 10.6 %, p = 0.0007) was decreased, compared to the NSVT-negative group. On multivariate analysis, the presence of atrial fibrillation (OR 29.49, p = 0.0025) and DM (OR 7.36, p = 0.0455) and T2-high signal (OR 14.96, p = 0.0014) and reduced LV ejection fraction (OR 0.93, p = 0.0222) were significantly associated with NSVT. The presence of myocardial T2-high signal is a significant independent predictor of NSVT in HCM patients.

Silent pulmonary artery dissection in a patient with Eisenmenger syndrome due to ventricular septal defect: a case report.

A 62-year-old woman was admitted to our hospital because of fever in August 2002. She had been treated under a diagnosis of Eisenmenger syndrome with ventricular septal defect since 1988. On admission, echocardiography and color Doppler echocardiography revealed a markedly enlarged pulmonary artery with a mobile flap, and dissection of the pulmonary artery. The origin of the fever could not be identified, and the fever subsided spontaneously without specific treatment. She had no chest pain, but fever might have been a sign of dissection in this patient. Longstanding pulmonary hypertension may cause dissection, which may lead to sudden death or pulmonary hemorrhage often seen in patients with Eisenmenger syndrome. Our patient was a rare survivor without serious bleeding complication.

[Complex myxoma detected by syncope: a case report].

A 23-year-old man was admitted to our hospital for evaluation of syncope and intracardiac masses. Echocardiography revealed three masses in the right ventricle and one in the left ventricle. The largest mass, 4 by 5 cm, occupied the right ventricular outflow tract and prolapsed through the pulmonary valve orifice. Right ventricular systolic pressure was 65 mmHg. A soft mass, 4 by 5 cm, was found on the left subcostal abdominal wall and multiple pigmented spots on the face and trunk. Histological examination of the resected tumors, including the abdominal soft mass, were consistent with myxoma. The combination of multiple cardiac and abdominal wall myxomas and pigmented skin lesions in this young patient is considered to be a diagnostic feature of Carney complex.