Project description:BackgroundA series of studies showed that endocardial radiofrequency ablation (ERFA) could reduce the left ventricular outflow tract (LVOT) gradient in patients with septal hypertrophy. This study aimed to determine the safety and efficacy of a modified ERFA approach guided by transthoracic echocardiography (TTE) as an alternative to ablation performed under a three-dimensional (3D) electroanatomical system or intracardiac echocardiography (ICE).MethodsTwenty-five patients with hypertrophic obstructive cardiomyopathy (HOCM) underwent ERFA of septal hypertrophy, guided by echocardiography. The LVOT gradient, left ventricular ejection fraction (LVEF), LV thickness, New York Heart Association (NYHA) class, and biochemical laboratory values were recorded before ablation and during follow-up.ResultsThe patients' peak and stress-induced LVOT gradients were significantly reduced after 12 months of follow-up (resting gradient: from 123.2±17.7 to 15.7±7.8 mmHg, P<0.05; provocative gradient: from 140.2±20.8 to 18.4±8.0 mmHg, P<0.05). Compared with baseline, the septal diameter was reduced slightly after 12 months, but the difference was not significant (24.8±3.5 vs. 24.2±3.4 mm, P>0.05). The reduction in LVOT gradient was associated with an improvement in NYHA functional classification (from 3.0±0.0 to 1.6±0.7, P<0.05), the 6-minute walking distance (413±129 m at baseline; 458±108 m immediately after ERFA; 471±139 m after 12 months, P<0.05), and pro B-type natriuretic peptide levels (from 924.00±139 to 137.45 ±75.73 pg/mL, P<0.05). After the procedure, the patients showed no worsening of LVEF compared with baseline (64%±5.3%), and no cases of bundle branch block nor complete heart block occurred.ConclusionsERFA guided by TTE provides a new treatment option for HOCM which can achieve symptomatic improvement as well as a significant and sustained reduction of the LVOT gradient. Moreover, by avoiding the use of the 3D electroanatomical system or ICE, this treatment has an acceptable cost.

Project description:BackgroundAnomalous papillary muscle (APM) insertion into the mitral valve leaflet is rare but clinically important in hypertrophic obstructive cardiomyopathy (HOCM). In this study, we report the detection rate of APM insertion into the mitral valve using preoperative imaging modalities and the surgical outcomes of the patients.MethodsBy retrospectively reviewing the clinical records of patients with HOCM who underwent surgical treatment by a single operation group at our center from January 2020 to June 2023, patients with APM insertion into the mitral valve leaﬂet were identified. Baseline data, image characteristics, and surgical outcomes were analyzed.ResultsThe incidence of APM insertion into the mitral valve leaﬂet was 5.1% (8/157). The insertion site was located at A3 in six cases, which was more common than at A2 (n = 2). Preoperative echocardiography was used to identify two patients (25%) with APM insertion. We observed a particular echocardiographic feature for APM in HOCM patients, which was noted as a "lip nevus sign", with a higher detection rate (62.5%). All patients successfully underwent septal myectomy with concomitant APM excision or mitral valve replacement via the transaortic (n = 5) or transmitral (n = 3) approach. The mean age was 49.0 ± 17.4 years and seven patients (87.5%) were female. Interventricular septum thickness (17.0 mm vs. 13.3 mm, P = 0.012) and left ventricular outflow gradient (117.5 mmHg vs. 7.5 mmHg, P = 0.012) were significantly decreased after surgery. Residual outflow obstruction, systolic anterior motion, and ≥3+ mitral regurgitation were negative. During the follow-up of 26.2 ± 12.2 months, there were no reported operations, adverse events, mitral regurgitation aggravations, recurrences of outflow obstruction, or instances of SAM.ConclusionsPapillary muscles inserted into the mitral valve leaﬂet are a subtype of subvalvular malformation in HOCM that requires surgical correction. The lip nevus sign on echocardiography is a characteristic of APM insertion in HOCM and may improve the preoperative detection rate. Adequate myectomy with anomalous papillary muscle excision has achieved good results in reducing the outflow gradient and eliminating mitral regurgitation, with good outcomes at short-to-intermediate follow-up.

Project description:BackgroundPerfusion defects during stress can occur in hypertrophic cardiomyopathy (HCM) from either structural or functional abnormalities of the coronary microcirculation. In this study, vasodilator stress myocardial contrast echocardiography (MCE) was used to quantify and spatially characterize hyperemic myocardial blood flow (MBF) deficits in HCM.MethodsRegadenoson stress MCE was performed in patients with septal-variant HCM (n = 17) and healthy control subjects (n = 15). The presence and spatial distribution (transmural diffuse, patchy, subendocardial) of perfusion defects was determined by semiquantitative analysis. Kinetic analysis of time-intensity data was used to quantify MBF, microvascular flux rate (β), and microvascular blood volume. In patients undergoing septal myectomy (n = 3), MCE was repeated > 1 years after surgery. RESULTS: In HCM subjects, perfusion defects during stress occurred in the septum in 80%, and in non-hypertrophied regions in 40%. The majority of septal defects (83%) were patchy or subendocardial, while 67% of non-hypertrophied defects were transmural and diffuse. On quantitative analysis, hyperemic MBF was approximately 50% lower (p < 0.001) in the hypertrophied and non-hypertrophied regions of those with HCM compared to controls, largely based on an inability to augment β, although hypertrophic regions also had blood volume deficits. There was no correlation between hyperemic MBF and either percent fibrosis on magnetic resonance imaging or outflow gradient, yet those with higher degrees of fibrosis (≥ 5%) or severe gradients all had low septal MBF during regadenoson. Substantial improvement in hyperemic MBF was observed in two of the three subjects undergoing myectomy, both of whom had severe pre-surgical outflow gradients at rest.ConclusionPerfusion defects on vasodilator MCE are common in HCM, particularly in those with extensive fibrosis, but have a different spatial pattern for the hypertrophied and non-hypertrophied segments, likely reflecting different contributions of functional and structural abnormalities. Improvement in hyperemic perfusion is possible in those undergoing septal myectomy to relieve obstruction. TRIAL REGISTRATION: ClinicalTrials.gov NCT02560467.

Project description:BackgroundHypertrophic cardiomyopathy (HCM) can be genetic and occurs as obstructive and non-obstructive 21 subtypes. Little is known about the clinical burden of the obstructive subtype of HCM at a national 22 level, and how it may differ by New York Heart Association (NYHA) class. Therefore, this study 23 aimed to describe the clinical burden of patients hospitalized with obstructive HCM from a 24 nationwide study in France.MethodsThis retrospective, longitudinal, observational study was performed using data from the French National Health Data System. All adult patients (≥ 18 years old) with a hospitalization related to obstructive HCM [International Classification of Diseases, Tenth Revision (ICD-10) code I42.1], or at least one hospitalization with ICD-10 code I42.2 or I42.9 and at least one code for septal reduction therapy between 2012 and 2018 were included. Patients were followed up for a minimum of 1 year. NYHA class was assigned using an algorithm based on treatment and symptom codes. Treatment patterns and clinical outcomes by NYHA class over time were examined.ResultsOverall, 6,823 patients with obstructive HCM were included (54.7% male, mean [standard deviation (SD)] age 66.2 [16.7] years). At inclusion, the proportion of patients in NYHA classes I, II, III, and IV were 4%, 32%, 60%, and 4%, respectively. Over the follow-up [mean (SD) follow-up: 4.4 (2.5) years; cumulative patient follow-up: 30,021 patient-years], 73% of patients remained in the same NYHA class, 14% of patients worsened, and 13% improved. At inclusion, 22% of patients had no HCM-related treatment, 56% were receiving β-blockers, 12% calcium-channel blockers, and 11% a combination of both. The incidence of cardiovascular-related hospitalization was high (35,436 hospitalizations; 117,229 per 100,000 patient-years) and this risk increased with NYHA class (from 81,247 per 100,000 patient-years for NYHA class I/II patients to 140,790 per 100,000 patient-years for NYHA class III/IV patients, p < 0.0001).ConclusionsPatients with obstructive HCM are at high risk of death and cardiovascular outcomes, especially those in higher NYHA classes. Despite current therapeutics, the clinical burden of symptomatic obstructive HCM remains high, supporting the need for additional therapies.

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Echocardiography for hypertrophic obstructive cardiomyopathy.

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Echocardiography for hypertrophic obstructive cardiomyopathy.

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