Akihiro Hayashida

Background: Female sex has been associated with poor prognosis in hypertrophic cardiomyopathy (HCM), but the factors contributing to this disparity remain insufficiently defined. We aimed to clarify sex differences in clinical characteristics and factors associated with cardiovascular death in Japan. Methods: This multicenter, retrospective observational study of HCM was conducted between January 1, 2006, and December 31, 2018 (REVEAL‐HCM study [Multicenter Registry to Evaluate Risk Factors for Disease Progression, Sudden Cardiac Death and Adverse Clinical Outcomes in Japanese Patients With Hypertrophic Cardiomyopathy]; UMIN000046932). Patients aged ≥16 years with HCM were enrolled. Baseline characteristics and clinical outcomes including cardiovascular death were assessed. Univariable and multivariable Cox proportional hazards models were used to identify factors associated with cardiovascular death. Results: Of 3247 patients (median age, 67 years; 43% women), women were older and more symptomatic at presentation (52% New York Heart Association class II–IV versus 35% in men). Cardiovascular death was more common in women (hazard ratio [HR], 1.37 [95% CI, 1.02–1.82]; P =0.03). In multivariable analysis, older age (per 1‐year increase; HR, 1.04 [95% CI, 1.02–1.06]), advanced New York Heart Association class (HR, 1.99 [95% CI, 1.40–2.82]), history of atrial fibrillation (HR, 1.55 [95% CI, 1.10–2.18]), greater maximal wall thickness indexed to body surface area (per 1‐mm/m ² increase; HR, 1.12 [95% CI, 1.06–1.20]), and apical HCM (HR, 0.53 [95% CI, 0.32–0.88]) were independently associated with cardiovascular death, attenuating the HR for female sex. Conclusions: Sex differences in clinical characteristics and outcomes were observed. The excess cardiovascular death in women with HCM was largely explained by older age, more advanced symptoms, greater indexed wall thickness, and sex‐specific differences in both the prevalence and prognostic impact of apical HCM.

BACKGROUND: Mitral regurgitation (MR) is a frequent comorbidity in patients with hypertrophic cardiomyopathy (HCM), which includes distinct subtypes with various characteristics. However, data on the long-term prognostic impact of MR and its progression or regression over time remain limited, particularly across individual HCM subtypes. METHODS: Patients with HCM were retrospectively included from a Japanese multicenter registry and compared by MR severity (moderate or greater versus mild or less) within each subtype: hypertrophic obstructive cardiomyopathy (HOCM), end-stage HCM (ES-HCM), and other HCM (including nonobstructive, midventricular obstruction, and apical HCM). RESULTS: Among 3602 patients (HOCM: n=837; ES-HCM: n=275; other HCM: n=2490), the prevalence of moderate or greater MR was highest in HOCM (36.3%), followed by ES-HCM (21.5%) and other HCM (8.5%). During a median follow-up of 5.3 (interquartile range, 2.1–9.3) years, the cumulative 5-year incidence of all-cause death or heart failure hospitalization was not significantly different between the moderate or greater MR and mild or less MR groups in HOCM (14.6% versus 12.4%; P =0.35) or ES-HCM (60.7% versus 54.7%; P =0.84). After adjustment for clinical covariates, no significant association was observed in either subtype (HOCM: hazard ratio, 1.13 [95% CI, 0.79–1.60], P =0.51; ES-HCM: hazard ratio, 0.84 [95% CI, 0.55–1.28], P =0.42). In contrast, in other HCM, moderate or greater MR was significantly associated with the higher 5-year cumulative incidence of all-cause death or heart failure hospitalization (34.2% versus 13.9%, P P =0.01; P _interaction =0.02). MR severity improved over time in HOCM, showed no clear change in ES-HCM, and worsened in other HCM ( P P _interaction <0.001). CONCLUSIONS: In patients with HCM, moderate or greater MR was not associated with worse outcomes in HOCM or ES-HCM, but had significant prognostic implications in other HCM subtypes, suggesting the need for HCM subtype-specific MR management.

Akihisa Kimura, Akihiro Hayashida, Misako Toki, Atsushi Hirohata, Kiyoshi Yoshida; Inverted left atrial appendage during Impella management: Non-invasive diagno

左室壁運動を評価するためには，左室を正しい断面で描出しなければならない．心尖部が真の心尖部になっておらず，左室の大きさを過小評価していることは多い．エコーベッドを用いたり，呼気時だけではなく吸気時にも撮像して，なるべく左室の長軸径が大きくなるようにする．短軸は乳頭筋レベルで観察を行い，両方の乳頭筋が均等に描出されているかを確認する．均等ではないと斜め振りの可能性あり，壁運動評価が正当にできない．短軸，及び心尖部からの長軸像で局所壁運動異常と心筋の性状を評価する．陳旧性心筋梗塞では，内膜側の輝度上昇，壁厚減少も重要な所見である．冠動脈支配領域に一致しない場合は，心サルコイドーシス，たこつぼ型心筋症などを考える．その際は，心電図も参考にする．左室収縮能評価は，Method of disks （MOD） 法で行う．これは，左室を20個の楕円柱に分割して体積を求める方法である．心エコーの機械でMODと表示されるため，modifyの略と誤解している人も多いが，これは誤用である．

Currently, myocardial unroofing, coronary artery bypass grafting and percutaneous coronary intervention are chosen as treatments for myocardial bridge. We performed a male patient off-pump unroofing of left anterior descending coronary artery with techniques of minimally invasive surgery through the 4 ^th intercoastal space. Intraoperative coronary angiography was performed to evaluate residual lesions. Since this treatment strategy does not require full heparinization, it can be expected to reduce the amount of bleeding. This procedure not only has small incision, but also has some major advantages.

Background: A myocardial bridge (MB) is a congenital coronary anomaly, wherein the epicardial coronary artery tunnels through the myocardial band. Treatment is indicated when clinical symptoms occur, and β-blockers are the first choice of treatment. Symptomatic patients refractory to medical therapy are considered for other options, including stent placement, coronary artery bypass grafting, or surgical supra-arterial myotomy. Supra-arterial myotomy is effective; however, the symptoms might persist if myocardial resection is inadequately performed. Case summary: We encountered a patient experiencing exertional chest pain. Coronary angiography revealed a MB at the mid-left anterior descending artery with systolic compression. The patient’s fractional flow reserves (FFRs) were 0.93 at rest and 0.72 with intravenous administration of 50 µg/kg/min dobutamine. The symptoms were refractory to drugs, and supra-arterial myotomy was performed with intraoperative coronary artery angiography, which revealed the milking effect of the residual myocardium; therefore, additional myocardial resection was performed. Postoperative coronary artery angiography showed no systolic compression, and the postoperative FFRs were 0.88 at rest and 0.92 with intravenous administration of dobutamine 50 µg/kg/min. Discussion: Although surgical supra-arterial myotomy is safe and effective, inadequate myocardial resection might cause symptom recurrence. Intraoperative coronary artery angiography during the surgery can indicate whether additional resection is required. Objective assessment of ischaemia might be useful in cases with a MB, which can cause asymptomatic myocardial ischaemia and sudden cardiac death. FFRs before surgery can help in evaluating the need for surgery and for confirming the therapeutic effect and subsequent treatment.

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