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 |
REPOSITORIES: biostudies-literature
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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: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:The burgeoning evidence of patients diagnosed with sigmoidal hypertrophic cardiomyopathy (HCM) later in life has revived the quest for distinctive features that may help discriminate it from more benign forms of isolated septal hypertrophy often labelled ventricular septal bulge (VSB). HCM is diagnosed less frequently than VSB at older ages, with a reversed female predominance. Most patients diagnosed with HCM at older ages suffer from hypertension, similar to those with VSB. A positive family history of HCM and/or sudden cardiac death and the presence of exertional symptoms usually support HCM, though they are less likely in older patients with HCM, and poorly investigated in individuals with VSB. A more severe hypertrophy and the presence of left ventricular outflow obstruction are considered diagnostic of HCM, though stress echocardiography has not been consistently used in VSB. Mitral annulus calcification is very prevalent in both conditions, whereas a restrictive filling pattern is found in a minority of older patients with HCM. Genetic testing has low applicability in this differential diagnosis at the current time, given that a causative mutation is found in less than 10% of elderly patients with suspected HCM. Emerging imaging modalities that allow non-invasive detection of myocardial fibrosis and disarray may help, but have not been fully investigated. Nonetheless, there remains a considerable morphological overlap between the two conditions. Comprehensive studies, particularly imaging based, are warranted to offer a more evidence-based approach to elderly patients with focal septal thickening.
| S-EPMC6127866 | biostudies-literature
Project description: Not available
| S-EPMC8298854 | 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
Project description:Takotsubo cardiomyopathy (TCM) is a condition characterized by transient left ventricular dysfunction and apical ballooning, best seen on an echocardiogram or left ventriculogram. It mimics acute myocardial infarction but without evidence of coronary artery disease on an angiogram. Hypertrophic cardiomyopathy (HCM) is an autosomal dominant heart muscle disease that is significant with hypertrophy of the left ventricle with various morphologies. We hereby report a case of TCM in a male patient with a known history of HCM. The patient's hemodynamic findings were challenging because the TCM produced an increased left ventricular outflow tract (LVOT) gradient that was previously not seen on his prior echocardiogram or cardiac catheterizations. Assessment and continuous monitoring are warranted in such a rare case. Supportive care afterward with beta blockers, along with echocardiogram surveillance, are the mainstay of management of such a patient.
| S-EPMC6333265 | biostudies-other
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:AimsObstructive hypertrophic cardiomyopathy (oHCM) is characterized by dynamic obstruction of the left ventricular (LV) outflow tract (LVOT). Although this may be mediated by interplay between the hypertrophied septal wall, systolic anterior motion of the mitral valve, and papillary muscle abnormalities, the mechanistic role of LV shape is still not fully understood. This study sought to identify the LV end-diastolic morphology underpinning oHCM.Methods and resultsCardiovascular magnetic resonance images from 2398 HCM individuals were obtained as part of the NHLBI HCM Registry. Three-dimensional LV models were constructed and used, together with a principal component analysis, to build a statistical shape model capturing shape variations. A set of linear discriminant axes were built to define and quantify (Z-scores) the characteristic LV morphology associated with LVOT obstruction (LVOTO) under different physiological conditions and the relationship between LV phenotype and genotype. The LV remodelling pattern in oHCM consisted not only of basal septal hypertrophy but a combination with LV lengthening, apical dilatation, and LVOT inward remodelling. Salient differences were observed between obstructive cases at rest and stress. Genotype negative cases showed a tendency towards more obstructive phenotypes both at rest and stress.ConclusionsLV anatomy underpinning oHCM consists of basal septal hypertrophy, apical dilatation, LV lengthening, and LVOT inward remodelling. Differences between oHCM cases at rest and stress, as well as the relationship between LV phenotype and genotype, suggest different mechanisms for LVOTO. Proposed Z-scores render an opportunity of redefining management strategies based on the relationship between LV anatomy and LVOTO.
| S-EPMC10229266 | biostudies-literature
Project description:BackgroundPatients with hypertrophic cardiomyopathy (HCM) are at a fourfold to sixfold higher risk of developing atrial fibrillation (AF) compared to the general population, though incidence rates among patients undergoing alcohol septal ablation (ASA) are not well characterized. The purpose of this study was to evaluate atrial fibrillation incidence following ASA.MethodsWe studied 132 consecutive HCM patients without comorbid AF that underwent 154 ASA procedures. The incidence of AF in follow-up was assessed through chart abstraction including electrocardiography. Survival free of AF was estimated using Kaplan-Meier methodology.ResultsOver a mean follow-up of 3.6 ± 2.7 years (maximum 11.3 years), 10 (7.6%) patients developed new-onset AF. Of those who developed AF, both resting and provoked left ventricular outflow tract (LVOT) gradients had improved significantly (difference -79.78 mm Hg, P ≤ 0.005). Severity of mitral regurgitation improved in 7 (70%) patients. Survival free of AF was estimated to be 99.1%, 93.7%, and 91.7% at 1, 3, and 5 years.ConclusionsDespite relieving LVOT obstruction and improving mitral regurgitation severity via ASA, new-onset AF remained a common complication of hypertrophic cardiomyopathy.
| S-EPMC6931872 | biostudies-literature