Reoperation for nonobstructive hypertrophic cardiomyopathy in a patient with extreme septal thickness.
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ABSTRACT:
SUBMITTER: Sun D
PROVIDER: S-EPMC8350794 | biostudies-literature | 2021 Aug
REPOSITORIES: biostudies-literature
Publications
Reoperation for nonobstructive hypertrophic cardiomyopathy in a patient with extreme septal thickness.
Sun Daokun D Schaff Hartzell V HV Nishimura Rick A RA
JTCVS techniques 20210504
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Project description:BackgroundHypertrophic cardiomyopathy (HCM) with extreme interventricular septal thickness (IVST) is associated with a higher incidence of adverse cardiovascular events. However, the results of these patients who underwent septal myectomy are unclear.MethodsWe studied 47 HCM patients with IVST ≥30 mm who underwent septal myectomy between 2011 and 2018 in Anzhen Hospital. After a 2:1 propensity score matching, the study cohort included 141 patients and 94 patients with IVST <30 mm.ResultsPatients with IVST ≥30 mm had a longer clinical course of disease, high incidence of syncope, palpitation, and moderate or severe mitral regurgitation. After a mean follow-up of 34.0±21.3 months, 6 patients died, including 5 with IVST ≥30 mm and 1 with IVST <30 mm. The 5-year survival free of all-cause mortality was significantly higher in patients with IVST ≥30 mm than in those with IVST <30 mm (98.9% vs. 85.7%, P=0.03). Multivariable Cox analysis revealed that IVST ≥30 mm (HR: 1.12, 95% CI: 1.01-1.25, P=0.028) was an independent risk factor for all-cause mortality. Meanwhile, left ventricular end diastole diameter (HR: 0.72, 95% CI: 0.54-0.97, P=0.031) and age (HR: 0.91, 95% CI: 0.83-0.99, P=0.025) were also independent risk factors for all-cause mortality in this special cohort. Furthermore, the incidence of NYHA class III or IV was significantly higher in patients with IVST ≥30 mm.ConclusionsThe surgical outcome was poor in a matched cohort of HCM patients with IVST ≥30 mm, which was mainly reflected by mortality and the incidence of NYHA class III or IV.
| S-EPMC7656406 | biostudies-literature
Project description:Septal reduction therapy is an effective treatment for hypertrophic obstructive cardiomyopathy (HOCM). Alcohol septal ablation (ASA) is indicated for HOCM patients who are ineligible for surgical myectomy, but several tips exist for the management of high-risk patients with ASA. Here, we present a case of successful ASA in a HOCM patient with multiple comorbidities, including severe obesity, drug-refractory bronchial asthma, poorly controlled diabetes, and steroid-induced immunosuppression. Pre-procedural strict glycemic control, pre-treatment with corticosteroids for bronchospasm prevention, minimal puncture sites for device insertion, and myocardial contrast echocardiography-guided procedure contributed to the achievement of successful ASA. With careful periprocedural management, ASA is a safe and effective treatment option for drug-refractory HOCM, even in high-risk patients with multiple comorbidities.Learning objectiveAlcohol septal ablation can be a beneficial and safe treatment option for hypertrophic obstructive cardiomyopathy patients with multiple comorbidities, including severe obesity, drug-refractory bronchial asthma, poorly controlled diabetes, and steroid-induced immunosuppression. Detailed periprocedural management, including myocardial contrast echocardiography-guided procedure, is the key for achievement.
| S-EPMC11745786 | biostudies-literature
Project description: Not available
| S-EPMC1861204 | biostudies-literature
Project description:BackgroundThe clinical presentation and prognosis of hypertrophic cardiomyopathy (HCM) are heterogeneous between nonobstructive HCM (HNCM) and obstructive HCM (HOCM). Electrocardiography (ECG) has been used as a screening tool for HCM. However, it is still unclear whether the features presented on ECG could be used for the initial classification of HOCM and HNCM.ObjectiveWe aimed to develop a pragmatic model based on common 12-lead ECG features for the initial identification of HOCM/HNCM.MethodsBetween April 1st and September 30th, 2020, 172 consecutive HCM patients from the International Cooperation Center for Hypertrophic Cardiomyopathy of Xijing Hospital were prospectively included in the training cohort. Between January 4th and February 30th, 2021, an additional 62 HCM patients were prospectively included in the temporal internal validation cohort. External validation was performed using retrospectively collected ECG data with definite classification (390 HOCM and 499 HNCM ECG samples) from January 1st, 2010 to March 31st, 2020. Multivariable backward logistic regression (LR) was used to develop the prediction model. The discrimination performance, calibration and clinical utility of the model were evaluated.ResultsOf all 30 acquired ECG parameters, 10 variables were significantly different between HOCM and HNCM (all P < 0.05). The P wave interval and SV1 were selected to construct the model, which had a clearly useful C-statistic of 0.805 (0.697, 0.914) in the temporal validation cohort and 0.776 (0.746, 0.806) in the external validation cohort for differentiating HOCM from HNCM. The calibration plot, decision curve analysis, and clinical impact curve indicated that the model had good fitness and clinical utility.ConclusionThe pragmatic model constructed by the P wave interval and SV1 had a clearly useful ability to discriminate HOCM from HNCM. The model might potentially serve as an initial classification of HCM before referring patients to dedicated centers and specialists.HighlightsWhat are the novel findings of this work? Evident differences exist in the ECG presentations between HOCM and HNCM.To the best of our knowledge, this study is the first piece of evidence to quantify the difference in the ECG presentations between HOCM and HNCM.Based on routine 12-lead ECG data, a probabilistic model was generated that might assist in the initial classification of HCM patients.
| S-EPMC10402817 | biostudies-literature
Project description:A 63-year-old symptomatic female with apical hypertrophic cardiomyopathy and diastolic disfunction was admitted to the hospital. What is the best way to manage this patient? This study is a literature review that was performed to answer this question. The following PubMed search strategy was used: 'Hypertrophic obstructive cardiomyopathy' [All Fields] OR 'apical myectomy' [All Fields], NOT 'animal [mh]' NOT 'human [mh]' NOT 'comment [All Fields]' OR 'editorial [All Fields]' OR 'meta-analysis [All Fields]' OR 'practice-guideline [All Fields]' OR 'review [All Fields]' OR 'pediatrics [mh]'. The natural history of the disease has a benign prognosis; however, a watchful strategy was associated with the risk of adverse cardiovacular events. Contrastingly, transapical myectomy was associated with low surgical risk and acceptable outcomes. In our case, the patient underwent transapical myectomy with an unconventional post-operative period. Control echocardiography showed marked left ventricular (LV) cavity enlargement: LV end-diastolic volume, 74 mL; LV ejection fraction, 65%; and LV stroke volume index increased to 27 mL/m2. The patient was discharged 7 days after myectomy. At 6 months post-operation, the patient was NYHA Class I, with a 6 min walk test score of 420 m. Therefore, transapical myectomy may be considered as a feasible procedure in patients with apical hypertrophic cardiomyopathy and progressive heart failure.
| S-EPMC10817431 | biostudies-literature
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:BackgroundThe aim of this study was to assess the impact of septal thickness on long-term outcomes of surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM) and correction of mitral subvalvular anomalies.MethodsSixty-six consecutive patients (58 ± 12 years, 56% female) undergoing extended septal myectomy and subvalvular mitral apparatus remodeling from 2007 to 2021 were retrospectively reviewed. Patients were divided into 2 groups according to septal thickness: moderate [< 18 mm, 29 patients (44%)] and severe [≥ 18 mm, 37 patients (56%)]. End points included survival, symptom improvement, reduction of left ventricle outflow tract (LVOT) gradient, resolution of mitral regurgitation (MR), and reoperation.ResultsThe mean interventricular septal thickness was 19 ± 3 mm, 15.8 ± 0.8 mm in patients with moderate and 21.4 ± 3.2 mm in those with severe hypertrophy. Preoperative data, intraoperative variables, postoperative complication rates, pre-discharge echocardiographic and clinical parameters did not differ between the two study groups [except for procedures involving the posterior mitral leaflet (p = 0.033) and septal thickness after myectomy (p = 0.0001)]. Subvalvular apparatus remodeling (secondary chordae of mitral valve resection and papillary muscle and muscularis trabecula procedures including resection, splitting, and elongation) was invariably added to septal myectomy (100%). Four (6%) procedures involved the posterior mitral leaflets. Mitral valve replacement was carried out in two patients (3%, p = 0.4). Reoperation for persistent MR was necessary in one patient (1%, p = 0.4). Neither iatrogenic ventricular septal defect nor in-hospital mortality occurred. During follow-up (mean 4.8 ± 3.8 years), two deaths occurred. NYHA class was reduced from 2.9 ± 0.7 to 1.6 ± 0.6 (p < 0.0001), the LVOT gradient from 89.7 ± 34.5 to 16.3 ± 8.8 mmHg (p < 0.0001), mitral valve regurgitation grade from 2.5 ± 1 to 1.2 ± 0.5 (p < 0.0001), and septal thickness from 18.9 ± 3.7 to 13.9 ± 2.7 mm (p < 0.0001).ConclusionsRegardless of septal thickness, subvalvular apparatus remodeling with concomitant septal myectomy can provide satisfactory long-term outcomes in terms of symptom improvement, LVOT obstruction relief, and MR resolution (without mitral valve replacement in most cases) in patients with HOCM.
| S-EPMC9242021 | biostudies-literature
Project description:BackgroundAlthough surgical treatment of residual obstruction after alcohol septal ablation (ASA) is often challenging in patients with obstructive hypertrophic cardiomyopathy (OHCM) there are very few relevant clinical reports. Thus, outcomes of surgical septal myectomy (SSM) in this subgroup of patients remain to be determined. Therefore, this study aimed to determine the surgical and follow-up outcomes in patients with OHCM exhibiting residual obstruction after ASA.MethodsWe collected case data for 62 patients with OHCM and residual obstruction after ASA who underwent SSM at Fuwai Hospital between January 2002 and June 2019. Propensity score matching with patients having had a myectomy as the only invasive procedure-was conducted in a 1:2 ratio. Echocardiography parameters, surgery results, and follow-up outcomes were compared between the groups.ResultsThe prior ASA group had a higher incidence of complete atrioventricular block (AVB) and subsequently postoperative permanent pacemaker (PPM) implantation than the primary myectomy group (9.7% vs. 1.6%, P=0.01). Two patients died within 30 days after surgery in the prior ASA group, and one patient died in the primary myectomy group, with an operative mortality rate of 3.2% and 0.8%, respectively (P=0.2). The 5-year event-free survival rate was 86.0% in the prior ASA group (median follow-up period: 3.2 years; mean: 3.9±2.6 years; maximum, 10.6 years) and 88.5% in the primary myectomy group (median follow-up period: 2.4 years; mean 2.8±1.7 years; maximum, 9.1 years) (P=0.2). During follow-up, four of 62 (6.5%) patients in the prior ASA group and one of 124 (0.8%) patients in the primary myectomy group progressed to advanced heart failure (P=0.025).ConclusionsPatients with OHCM following ASA are at an increased risk of developing AVB after SSM. Their surgical outcomes, and long-term survival rate were satisfactory and, osimilar to those for patients having had a myectomy as the only invasive procedure. In addition, they had an increased risk of advanced heart failure after SSM in the present study.
| S-EPMC7947546 | biostudies-literature
Project description:BackgroundHypertrophic cardiomyopathy is estimated to affect 1 out of every 500 adults in the USA. One of its main complications is left ventricular outflow obstruction, which may require surgical septal myectomy in severe cases. We report a rare complication of postoperative septal akinesis leading to thrombus formation presenting as an acute ischaemic stroke.Case summaryA 48-year-old woman presented with acute stroke 2 years after surgical septal myectomy for hypertrophic obstructive cardiomyopathy. Diagnostic workup identified an intraventricular thrombus arising in the left ventricular outflow tract (LVOT). After comprehensive evaluation, it was determined that the thrombus development was a complication of the prior septal myectomy causing focal septal akinesis. Treatment with anticoagulation resulted in improvement of neurological symptoms and resolution of the intraventricular thrombus.DiscussionThis case illustrates the rarity and unusual presentation of an intracardiac thrombus that arises from septal myectomy site. A thrombus arising in the LVOT, which is characterized by high gradient laminar flow, is highly unusual. This suggests microscopic and macroscopic alteration in the ventricular septal wall structure, as evident by the septal wall akinesis seen on echocardiography. Recognition of this complication is critical to the selection of appropriate anticoagulation as secondary stroke prevention in these patients.
| S-EPMC8526918 | biostudies-literature
Project description:Hypertrophic Cardiomyopathy (HCM) is a common inherited disorder characterized by unexplained left ventricular hypertrophy with or without left ventricular outflow tract (LVOT) obstruction. Single-nuclei RNA-sequencing (snRNA-seq) of both obstructive and nonobstructive HCM patient samples has revealed alterations in communication between various cell types, but no direct and integrated comparison between the two HCM phenotypes has been reported. We performed a bioinformatic analysis of HCM snRNA-seq datasets from obstructive and nonobstructive patient samples to identify differentially expressed genes and distinctive patterns of intercellular communication. Differential gene expression analysis revealed 37 differentially expressed genes, predominantly in cardiomyocytes but also in other cell types, relevant to aging, muscle contraction, cell motility, and the extracellular matrix. Intercellular communication was generally reduced in HCM, affecting the extracellular matrix, growth factor binding, integrin binding, PDGF binding, and SMAD binding, but with increases in adenylate cyclase binding, calcium channel inhibitor activity, and serine-threonine kinase activity in nonobstructive HCM. Increases in neuron to leukocyte and dendritic cell communication, in fibroblast to leukocyte and dendritic cell communication, and in endothelial cell communication to other cell types, largely through changes in the expression of integrin-β1 and its cognate ligands, were also noted. These findings indicate both common and distinct physiological mechanisms affecting the pathogenesis of obstructive and nonobstructive HCM and provide opportunities for the personalized management of different HCM phenotypes.
| S-EPMC8780670 | biostudies-literature