Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy.
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ABSTRACT:
SUBMITTER: Blackshear JL
PROVIDER: S-EPMC3046942 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Surgical septal myectomy is the treatment of choice for patients with symptomatic hypertrophic obstructive cardiomyopathy refractory to medications. This report describes our minimally invasive approach for performing a septal myectomy via a ministernotomy that has been used at our institution for more than a decade. In particular, patient preparation, surgical technique, and clinical considerations are highlighted. Performed properly, this minimally invasive technique is a feasible and effective approach in our experience.
| S-EPMC5774662 | biostudies-literature
Project description:Hypertrophic cardiomyopathy (HCM) is a complex, underestimated, multifaceted disease frequently associated with left ventricular outflow tract (LVOT) obstruction. It is clearly demonstrated that this is due not only to septal hypertrophy but also to systolic anterior motion (SAM) of mitral valve leaflets secondary to mitral valve/subvalvular apparatus abnormalities. Surgical treatment involves performing an extended septal myectomy, eventually followed by ancillary procedures to those structures responsible for maintaining LVOT obstruction, if necessary. In this review, we describe the spectrum of possible surgical techniques beyond septal myectomy and their pathophysiologic rationale.
| S-EPMC8509570 | biostudies-literature
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.
| S-EPMC8965461 | biostudies-literature
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.
| S-EPMC6439557 | biostudies-literature
Project description:Surgical myectomy (SM) and alcohol septal ablation (ASA) are two invasive therapies for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM), despite medical therapy. This meta-analysis aims to compare the efficacy of the two procedures. We searched all electronic databases until February 2020 for clinical trials and cohorts comparing clinical outcomes of ASA and SM treatment of patients with HOCM. The primary endpoint was all-cause mortality, cardiovascular (CV) mortality, sudden cardiac death (SCD), re-intervention, and complications. Secondary endpoints included relief of clinical symptoms and drop of left ventricular outflow tract (LVOT) gradient. Twenty studies (4547 patients; 2 CTs and 18 cohorts) comparing ASA vs. SM with a mean follow-up of 47 ± 28.7 months were included. Long term (8.72 vs. 7.84%, p = 0.42) and short term (1.12 vs. 1.27%, p = 0.93) all-cause mortality, CV mortality (2.48 vs. 3.66%, p = 0.26), SCD (1.78 vs. 0.76%, p = 0.20) and stroke (0.36 vs. 1.01%, p = 0.64) were not different between procedures. ASA was associated with lower peri-procedural complications (5.57 vs. 10.5%, p = 0.04) but higher rate of re-interventions (10.1 vs. 0.27%; p < 0.001) and pacemaker dependency (12.4 vs. 4.31%, p = 0.0004) compared to SM. ASA resulted in less reduction in LVOT gradient (-47.8 vs. -58.4 mmHg, p = 0.01) and less improvement of clinical symptoms compared to SM (New York Heart Association (NYHA) class III/IV, 82.4 vs. 94.5%, p < 0.001, angina 53.2 vs. 84.2%, p = 0.02). Thus, ASA and SM treatment of HOCM carry a similar risk of mortality. Peri-procedural complications are less in alcohol ablation but re-intervention and pacemaker implantations are more common. These results might impact the procedure choice in individual patients, for the best clinical outcome.
| S-EPMC7598206 | biostudies-literature
Project description:BackgroundSurgical myectomy (SM) and Alcohol septal ablation (ASA) are effective therapies for patients with hypertrophic cardiomyopathy who remain symptomatic despite medical therapy. A plethora of data has recently emerged on the long-term outcomes of these procedures. We hence sought to perform an updated meta-analysis comparing both procedures.MethodsStudies reporting long-term (>3-years) outcomes of SM and/or ASA were included. The primary endpoint was all-cause mortality. Secondary endpoints included cardiovascular mortality, sudden cardiac death (SCD), reintervention, and complications including death, pacemaker implantation, and stroke.ResultsTwenty-two ASA cohorts (n = 4213; follow-up = 6.6-years) and 23 SM cohorts (n = 4240; follow-up = 6.8-years) were included. Septal myectomy was associated with higher periprocedural mortality and stroke (2% vs 1.2%, P = 0.009 and 1.5% vs 0.8% P = 0.013, respectively), but ASA was associated with more need of pacemaker (10% vs 5%, P < 0.001). During long-term follow-up, all-cause mortality, cardiovascular mortality, and sudden cardiac death rates were 1.5%, 0.4%, and 0.3% per person-year in the ASA group and 1.1%, 0.5%, and 0.3% per person-year in the SM group (P = 0.21, P = 0.53, P = 0.43), respectively. Repeat septal reduction intervention(s) were more common after ASA (11% vs 1.5%, P < 0.001).ConclusionCompared with SM, ASA is associated with lower periprocedural mortality and stroke but higher rates of pacemaker implantations and reintervention. However, there was no difference between ASA and SM with regards to long-term all-cause mortality, cardiovascular mortality, or SCD.
| S-EPMC6436521 | biostudies-literature
Project description:BackgroundGuidelines on the diagnosis and management of hypertrophic cardiomyopathy (HCM) recommend that septal myectomy be performed by experienced operators. However, the impact of operator volume on surgical treatment outcomes for isolated HCM has been poorly investigated.MethodsFrom 2002 to 2014, 435 consecutive patients with isolated HCM undergoing myectomy at the Fuwai Hospital were retrospectively enrolled. All 29 surgeons were divided into beginner surgeons (operator volume ≤20) and experienced surgeons (operator volume >20) according to the guidelines for the diagnosis and treatment of HCM. Propensity score matching of patients in the two groups was performed.ResultsBaseline differences included advanced New York Heart Association classification and older age in the experienced surgeon group. After matching, in the beginner surgeon group (107 cases), residual obstruction (18.7% vs. 0.9%, P<0.001) was more common, and the postoperative left ventricular outflow tract pressure gradient (20.7±15.1 vs. 14.3±7.4 mmHg, P<0.001) was higher than that of the experienced surgeon group. In the experienced surgeon group (107 cases), the incidence of mitral valve replacement (1.9% vs. 11.2%, P<0.001) and permanent pacemaker implantation (1.9% vs. 3.7%, P<0.001) was significantly lower than that in the beginner surgeon group. However, there was no difference in procedural mortality (1.9% vs. 1.9%) between the two groups.ConclusionsOperator volume is an important factor in achieving better obstruction obliteration after septal myectomy in patients with isolated HCM.
| S-EPMC7947483 | biostudies-literature
Project description: Not available
| S-EPMC10750430 | biostudies-literature
Project description:Background Sleep-disordered breathing (SDB) is a risk factor for heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, the relationship between preoperative SDB and postoperative functional status after septal myectomy (SM) is unknown. In this study, we investigated the association of preoperative SDB with late self-reported health status among patients undergoing SM for obstructive HCM. Methods Prior to SM, an overnight pulse oximetry test was used to measure oxygen desaturation index (ODI), which indicates the average number of desaturation episodes with at least 4% of drops in oxygen level from baseline per hour of sleep. Patients reported postoperative function through a questionnaire-based survey completed 3-5 years following SM. Results We analyzed 264 patients who underwent transaortic SM from November 2005 through April 2016. On predischarge transthoracic echocardiography post-procedure, no significant difference was present in the extent of mitral valve regurgitation (P = 0.524), left ventricular outflow tract gradient (P = 0.405), or septal thickness (P = 0.744) related to ODI. At 3 to 5 years after their operation, 236 patients (89%) reported good or excellent health. Approximately 18% (n = 48) and 8% (n = 22) of the cohort continued to experience exertional dyspnea and chest tightness, respectively, on walking 2 blocks or less. A greater ODI was not an independent predictor of worse health status or residual symptoms. Conclusions Relief of left ventricular outflow tract obstruction in patients with obstructive HCM improves symptoms, with 89% of patients reporting good or excellent health. Preoperative SDB is not significantly associated with late functional status after septal myectomy.
| S-EPMC9568685 | biostudies-literature
Project description: Not available
| S-EPMC8298854 | biostudies-literature