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Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy.

ABSTRACT: Bleeding with severe aortic stenosis is linked to acquired von Willebrand syndrome and loss of high-molecular-weight multimers of von Willebrand factor. Valve replacement resolves bleeding tendency and loss of high-molecular-weight multimers. We report outcomes in 5 patients with symptomatic obstructive hypertrophic cardiomyopathy and spontaneous gastrointestinal, mucosal, or excessive postsurgical bleeding in whom acquired von Willebrand syndrome was documented. All 5 patients underwent surgical septal myectomy with resolution of acquired von Willebrand syndrome.

SUBMITTER: Blackshear JL

PROVIDER: S-EPMC3046942 | biostudies-literature | 2011 Mar

REPOSITORIES: biostudies-literature

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Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy.

Blackshear Joseph L JL Schaff Hartzell V HV Ommen Steve R SR Chen Dong D Nichols William L WL

Mayo Clinic proceedings 20110301 3

Bleeding with severe aortic stenosis is linked to acquired von Willebrand syndrome and loss of high-molecular-weight multimers of von Willebrand factor. Valve replacement resolves bleeding tendency and loss of high-molecular-weight multimers. We report outcomes in 5 patients with symptomatic obstructive hypertrophic cardiomyopathy and spontaneous gastrointestinal, mucosal, or excessive postsurgical bleeding in whom acquired von Willebrand syndrome was documented. All 5 patients underwent surgica ...[more]

PMID: 21364113

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Project description:BackgroundThe thoracoscopic trans-mitral approach can not only facilitate exposure of the ventricular septum, mitral valve, and subvalvular apparatus, it also enables the surgeons to perform concomitant mitral valve intervention. This study aimed to determine the safety and efficacy of thoracoscopic trans-mitral septal myectomy in elderly patients with hypertrophic obstructive cardiomyopathy (HOCM).MethodsWe reviewed the demographic to clinical characteristics and outcomes of patients who underwent thoracoscopic trans-mitral septal myectomy in our center between April 2019 and April 2021. The population was divided into a younger group (<60 years) and an elderly group (≥60 years).ResultsThere were 46 and 20 patients in the younger and elderly groups, respectively. The majority of patients in the elderly group were female (39.1 vs. 80.0%, P < 0.01). Patients in the elderly group were more likely to be in New York Heart Association Class IV (2.2 vs. 80.0%, P < 0.01). The European System for Cardiac Operation Risk Evaluation II predicted mortality rates were significantly higher (3.97 ± 1.81 vs. 1.62 ± 0.86%, P < 0.01) in the elderly group. In the elderly group, a patient converted to median sternotomy due to left ventricular posterior free wall rupture following septal myectomy and mitral bioprosthetic valve replacement. The patient then underwent double-patch sandwich repair for rupture and mitral mechanical valve replacement and was eventually discharged. All patients in the elderly group were discharged, while one in the younger group died. No patient in the elderly group required permanent pacemaker implantation vs. one in the younger group. Patients in the elderly group were more likely to spend more time in the intensive care unit than those in the younger group (5.44 ± 5.80 days vs. 3.07 ± 2.72, P < 0.05). However, there was no significant intergroup difference in in-hospital mortality or complications. Importantly, the left ventricular outflow tract pressure gradient was significantly decreased from 96.15 ± 32.89 mmHg to 8.2 ± 3.42 mmHg with no residual obstruction in the elderly group. The interventricular septal thickness was significantly decreased from 19.73 ± 3.14 mm to 11.30 ± 2.23 mm. Postoperative mitral regurgitation severity was significantly improved in the elderly group.ConclusionThis study demonstrated that thoracoscopic trans-mitral septal myectomy is a feasible option for selected elderly patients with satisfactory outcomes similar to those of young patients.

Project description:Importance:Recent data indicate that women with hypertrophic cardiomyopathy (HCM) are older and more symptomatic at presentation and have worse clinical outcomes than men. However, to our knowledge, there are no large studies of the association of patient sex with outcomes after surgical myectomy. Objective:To analyze preoperative characteristics and overall survival of women and men undergoing septal myectomy for obstructive HCM. Design, Setting, and Participants:This retrospective, single-center study included the clinical data of adult patients who underwent septal myectomy from January 1961 through April 2016. Data analysis occurred from December 2017 to December 2018. Exposures:Septal myectomy. Main Outcomes and Measures:Survival. Results:A total of 2506 adults were included; 1379 patients (55.0%) were men. At the time of surgery, women were older, with median (IQR) age of 59.5 (46.6-68.2) years vs 52.9 (42.9-62.7) years in men (P < .001). Women were more likely to have New York Heart Association class III or IV status at presentation (women, 1023 [90.8%]; men, 1169 [84.8%]; P < .001) and more severe obstructive physiology, as reflected in higher resting left ventricular outflow tract gradients (women, 67.0 [36.0-97.0] mm Hg; men, 50.0 [23.0-81.0] mm Hg; P < .001). Women also had a greater likelihood of having moderate or severe mitral regurgitation (606 [55.2%]) than men (581 [43.1%]; P < .001) and higher right ventricular systolic pressure (women, 36.0 [30.0-46.0] mm Hg; men, 33.0 [28.0-39.0] mm Hg; P < .001). The unadjusted overall survival was lower in women, corresponding to a median 3.9-year shorter survival than men (median [IQR] survival time: women, 18.2 [12.1-27.2] years; men, 22.1 [15.1-32.5] years; P < .001). In a multivariable Cox regression analysis, however, the association between sex and mortality was attenuated and not significant after controlling for other baseline variables (hazard ratio, 0.98 [95% CI, 0.76-1.26]; P = .86). Among the covariates in the model, older age at surgery (adjusted hazard ratio [aHR], 3.09 [95% CI, 2.12-4.52]; P < .001), higher body mass index (aHR, 1.22 [95% CI, 0.90-1.66]; P < .001), greater NYHA class (aHR, 2.31 [95% CI, 1.03-5.15]; P = .04), and presence of diabetes prior to surgery (aHR, 1.57 [95% CI, 1.10-2.24]; P = .01) were each independently associated with increased mortality. Operations performed later in the study period (2013 vs 2004) were associated with decreased mortality (aHR, 0.82 [95% CI, 0.55-1.22]; P = .001). Conclusions and Relevance:In this large cohort of surgical patients with obstructive HCM, we observed significant differences at clinical presentation between women and men, in that women were older and more symptomatic. However, after adjustment for important baseline prognostic factors, there was no survival difference after septal myectomy by sex. Improved care of women with obstructive HCM should focus on early identification of disease and prompt surgical referral of appropriate patients who do not respond to medical treatment.

Dataset Information

Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy.

Publications

Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy.

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