Cardiogenic Shock in Obstructive Hypertrophic Cardiomyopathy Plus Apical Ballooning: Management With VA-ECMO and Myectomy.
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ABSTRACT:
SUBMITTER: Caniato F
PROVIDER: S-EPMC8311032 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Background Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic features of mild septal thickening, outflow gradients, and distinctive mitral abnormalities differentiate these patients from others with Takotsubo syndrome, who have normal mitral valves and no outflow obstruction. Methods and Results We analyzed 8 patients from our 4 HCM centers with obstructive HCM and abrupt presentation of cardiogenic shock with LV ballooning, and 6 cases reported in literature. Of 14 patients, 10 (71%) were women, aged 66±9 years, presenting with acute symptoms: LV ballooning; depressed ejection fraction (25±5%); refractory systemic hypotension; marked LV outflow tract obstruction (peak gradient, 94±28 mm Hg); and elevated troponin, but absence of atherosclerotic coronary disease. Shock was managed with intravenous administration of phenylephrine (n=6), norepinephrine (n=6), β-blocker (n=7), and vasopressin (n=1). Mechanical circulatory support was required in 8, including intra-aortic balloon pump (n=4), venoarterial extracorporeal membrane oxygenation (n=3), and Impella and Tandem Heart in 1 each. In refractory shock, urgent relief of obstruction by myectomy was performed in 5, and alcohol ablation in 1. All patients survived their critical illness, with full recovery of systolic function. Conclusions When cardiogenic shock and LV ballooning occur in obstructive HCM, they are marked by distinctive anatomic and physiologic features. Relief of obstruction with targeted pharmacotherapy, mechanical circulatory support, and myectomy, when necessary for refractory shock, may lead to survival and normalization of systolic function.
| S-EPMC8751867 | biostudies-literature
Project description:Apical akinesis and dilation in the absence of obstructive coronary artery disease is a typical feature of stress-induced (takotsubo) cardiomyopathy, whereas apical hypertrophy is seen in apical-variant hypertrophic cardiomyopathy. We report the cases of 2 patients who presented with takotsubo cardiomyopathy and were subsequently found to have apical-variant hypertrophic cardiomyopathy, after the apical ballooning from the takotsubo cardiomyopathy had resolved. The first patient, a 43-year-old woman with a history of alcohol abuse, presented with shortness of breath, electrocardiographic and echocardiographic features consistent with takotsubo cardiomyopathy, and no significant coronary artery disease. An echocardiogram 2 weeks later revealed a normal left ventricular ejection fraction and newly apparent apical hypertrophy. The 2nd patient, a 70-year-old woman with pancreatitis, presented with chest pain, apical akinesis, and a left ventricular ejection fraction of 0.39, consistent with takotsubo cardiomyopathy. One month later, her left ventricular ejection fraction was normal; however, hypertrophy of the left ventricular apex was newly noted. To our knowledge, these are the first reported cases in which apical-variant hypertrophic cardiomyopathy was masked by apical ballooning from stress-induced cardiomyopathy.
| S-EPMC4004496 | biostudies-other
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: Not available
| S-EPMC2000940 | 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: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.
| S-EPMC3046942 | biostudies-literature
Project description: Not available
| S-EPMC6058765 | 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: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