This study aims to report our preliminary experience and anatomic findings in the surgical treatment and postoperative management of hypertrophic obstructive cardiomyopathy. This study included 277 patients with hypertrophic obstructive cardiomyopathy (168 [60.65%] were male), with a median age of 47 years (interquartile range, 35-54 years), who underwent surgical myectomy performed by 1 surgeon in Fuwai Hospital between May 2010 and April 2015. The median follow-up was 14 months (interquartile range, 7-24 months). A total of 127 patients (45.85%) underwent concomitant procedures, and 2 patients (0.72%) died in the early perioperative days. The left ventricular outflow gradient decreased from 78 mm Hg (interquartile range, 61-100 mm Hg) to 11 mm Hg (interquartile range, 8-15 mm Hg) when discharged (P < .001). Of the 228 patients with well-documented anatomic description, more than 80% had various intraventricular anomalies. The cumulative survival was 99.28% (95% confidence interval, 97.15-99.82) at 1 year and 96.98% (95% confidence interval, 92.56-98.79) at 5 years. Of the surviving 272 patients, 268 (98.53%) were categorized with functional class I and II of the New York Heart Association classification at the latest evaluation. Anomalous muscle bundles are common in hypertrophic obstructive cardiomyopathy, and they may lead to middle-apical obstruction. Surgical myectomy provides excellent clinical outcomes with low risk for sufficient relief of obstruction and radical correction of intraventricular anomalies in patients with hypertrophic obstructive cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) represents one of the most common heritable heart diseases. However, the signalling pathways and regulatory networks underlying the pathogenesis of HCM remain largely unknown. Here, we present a strand-specific RNA-seq dataset for both coding and lncRNA profiling in myocardial tissues from 28 HCM patients and 9 healthy donors. This dataset constitutes a valuable resource for the community to examine the dysregulated coding and lncRNA genes in HCM versus normal conditions.

Background: Filamin C (FLNC) mutation was reported as a cause of HCM, with a high probability of sudden cardiac death. However, the mutation profile of FLNC, and its relationship with phenotypic expression in HCM, remains to be elucidated.Methods: In this study, FLNC gene was sequenced in 540 HCM patients and 307 healthy controls.Results: We found that 39 (7.2%) patients carried FLNC mutations, with a similar frequency to that of controls (4.2%, p = 0.101). Pedigree analysis showed that mutations were not well segregated with HCM. The baseline characteristics between HCM patients, with and without mutations, were comparable. FLNC mutations did not increase the risk for either all‐cause mortality (HR 0.746, 95% CI 0.222–2.295, p = 0.575) or cardiac mortality (HR 0.615, 95% CI 0.153–1.947, p = 0.354) in HCM patients during a follow‐up of 4.7 ± 3.2 years. Moreover, there was no significant difference in survival free from sudden cardiac arrest (HR 0.721, 95% CI 0.128–3.667, p = 0.660) and heart failure (HR 0.757, 95% CI 0.318–1.642, p = 0.447).Conclusions: FLNC mutations were common in both HCM patients and healthy population. The pathogenicity of FLNC mutations detected in HCM patients and its association with the clinical outcomes should be cautiously interpreted.