A Case of Biventricular Hypertrophic Obstructive Cardiomyopathy: Echocardiographic Diagnosis.
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SUBMITTER: Yamano M
PROVIDER: S-EPMC9969789 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
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A Case of Biventricular Hypertrophic Obstructive Cardiomyopathy: Echocardiographic Diagnosis.
Yamano Michiyo M Nakamura Takeshi T Yamano Tetsuhiro T Matoba Satoaki S
Texas Heart Institute journal 20230101 1
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Project description:Hypertrophic Cardiomyopathy (HCM) is an inherited cardiovascular disorder of great genetic heterogeneity and has a prevalence of 0.1 - 0.2 % in the general population. Several hundred mutations in more than 27 genes, most of which encode sarcomeric structures, are associated with the HCM phenotype. Then, HCM is an extremely heterogeneous disease and several phenotypes have been described over the years. Originally only two phenotypes were considered, a more common, obstructive type (HOCM, 70 %) and a less common, non-obstructive type (HNCM, 30 %) (Maron BJ, et al. Am J Cardiol 48:418 -28, 1981). Wigle et al. (Circ 92:1680-92, 1995) considered three types of functional phenotypes: subaortic obstruction, midventricular obstruction and cavity obliteration. A leader american working group suggested that HCM should be defined genetically and not morphologically (Maron BJ, et al. Circ 113:1807-16, 2006). The European Society of Cardiology Working Group on Myocardial and Pericardial Diseases recommended otherwise a morphological classification (Elliott P, et al. Eur Heart J 29:270-6, 2008). Echocardiography is still the principal tool for the diagnosis, prognosis and clinical management of HCM. It is well known that the echocardiographic picture may have a clinical and prognostic impact. For this reason, in this article, we summarize the state of the art regarding the echocardiographic pattern of the HCM phenotypes and its impact on clinical course and prognosis.
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| S-EPMC10057823 | biostudies-literature
Project description:To understand the interplay between cardiomyocyte and nonmyocyte cell types in human obstructive and non-obstructive hypertrophic cardiomyopathy, single nuclei RNA-sequencing was performed on 2 unused donor hearts, 1 obstructive HCM specimen, and 6 non-obstructive HCM specimens.
2022-01-01 | GSE181764 | GEO
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| S-EPMC5408548 | biostudies-literature
Project description:There has been limited data addressing outcomes of extensive septal myectomy in Chinese patients with hypertrophic obstructive cardiomyopathy (HOCM). In this study, the objective was to evaluate the clinical and echocardiographic outcomes of extensive septal myectomy in a relative large number of Chinese HOCM patients over long-term follow-up.We retrospectively studied 139 consecutive HOCM patients (age 43 ± 15 years, 37 % male) who underwent extensive left ventricular septal myectomy. During the perioperative period, all patients were examined by echocardiography. All-cause death and cardiac death were considered as primary endpoints during follow-up. Perioperative data was obtained by retrospective review of institutional surgical databases. Follow-up data of echocardiography and clinical status was recorded through outpatient interview.Perioperative events consisted of arrhythmia, retraction injury to aortic valve leaflets, pleural effusion, and hemodialysis and the use of intra-aortic balloon pump. There was no in-hospital mortality. The follow-up period averaged 5.6 ± 0.9 years and overall survivals were 100.0, 99.3, 99.3, 98.5 and 97.8 % at 1, 2, 3, 4 and 5 years, respectively. Left ventricular outflow tract (LVOT) gradient decreased form preoperative 84 ± 17 mmHg to 12 ± 3 mmHg at 2.5 years after surgery and it further reduced to 6 ± 3 mmHg at 5 years after surgery (P < 0.05). Compared with the preoperative levels, interventricualr septal thickness decreased by 32 % while diastole left ventricular inner diameter approximately increased by 10 % and ejection fraction (EF) was significantly elevated during follow-up (P < 0.05). By echocardiography detection, mitral regurgitation was ameliorated for HOCM patients after surgery. There was significant improvement in New York Heart Association (NYHA) class. The proportion of NYHA III and IV decreased from preoperative 58 to 19 % at 2.5 years after surgery and it reduced to 11 % at 5 years after operation.Extensive septal myectomy offers minimal operative risk and provides long-term relief for LVOT obstruction in Chinese HOCM patients.
| S-EPMC4869192 | biostudies-other
Project description:A diastolic murmur is informative in the diagnosis of valvular heart disease, such as mitral stenosis and aortic regurgitation. Patients with hypertrophic cardiomyopathy (HCM) could have diastolic murmurs, although this is not widely recognized. We describe an illustrative case of HCM which was found to have a diastolic murmur associated with mid-left ventricular obstruction. An asymptomatic 65-year-old male was referred because of an abnormal electrocardiogram. Cardiac auscultation showed a soft fourth heart sound, a systolic ejection murmur, and a third heart sound followed by a diastolic murmur at the apex. On phonocardiography, the mid-diastolic murmur was predominantly low-pitched with an onset of about 200 ms after the second heart sound and a duration of approximately 150 ms. The timing of the diastolic murmur was consistent with an increased blood inflow during diastole in the mid-ventricular obstruction (2.0 m/s), which was produced by narrowing between the hypertrophied ventricular septum and the protrusion of the anterior papillary muscle. <Learning objective: A diastolic murmur is common in valvular heart diseases, such as mitral stenosis and aortic regurgitation, and can also be heard in hypertrophic cardiomyopathy although not widely recognized. We report an illustrative case of hypertrophic cardiomyopathy with a diastolic murmur related to mid-left ventricular obstruction, findings that would highlight the importance of auscultation regarding not only the differential diagnosis but also risk stratification, given the possible association of diastolic murmurs with adverse outcomes, even in the era of advanced imaging techniques.>.
| S-EPMC6283745 | biostudies-literature
Project description:AimsTreatment options for patients with non-obstructive hypertrophic cardiomyopathy (HCM) are limited. We sought to determine whether biventricular (BiV) pacing improves exercise capacity in HCM patients, and whether this is via augmented diastolic filling.Methods and resultsThirty-one patients with symptomatic non-obstructive HCM were enrolled. Following device implantation, patients underwent detailed assessment of exercise diastolic filling using radionuclide ventriculography in BiV and sham pacing modes. Patients then entered an 8-month crossover study of BiV and sham pacing in random order, to assess the effect on exercise capacity [peak oxygen consumption (VO2 )]. Patients were grouped on pre-specified analysis according to whether left ventricular end-diastolic volume increased (+LVEDV) or was unchanged/decreased (-LVEDV) with exercise at baseline. Twenty-nine patients (20 male, mean age 55 years) completed the study. There were 14 +LVEDV patients and 15 -LVEDV patients. Baseline peak VO2 was lower in -LVEDV patients vs. +LVEDV patients (16.2 ± 0.9 vs. 19.9 ± 1.1 mL/kg/min, P = 0.04). BiV pacing significantly increased exercise ΔLVEDV (P = 0.004) and Δstroke volume (P = 0.008) in -LVEDV patients, but not in +LVEDV patients. Left ventricular ejection fraction and end-systolic elastance did not increase with BiV pacing in either group. This translated into significantly greater improvements in exercise capacity (peak VO2 + 1.4 mL/kg/min, P = 0.03) and quality of life scores (P = 0.02) in -LVEDV patients during the crossover study. There was no effect on left ventricular mechanical dyssynchrony in either group.ConclusionSymptomatic patients with non-obstructive HCM may benefit from BiV pacing via augmentation of diastolic filling on exercise rather than contractile improvement. This may be due to relief of diastolic ventricular interaction.Clinical trial registrationClinicalTrials.gov NCT00504647.
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Project description:Hypertrophic cardiomyopathy (HCM) is known to be the most prevalent genetic cardiac condition. However, there have been limited reports on the diagnosis of HCM accompanied by secondary hypertension and the subsequent systematic therapy. In this case report, we present the case of a 65-year-old male patient who presented with recurring chest discomfort during physical activity, along with refractory hypertension. Cardiac magnetic resonance imaging (MRI) and transthoracic echocardiogram(TTE) revealed the presence of HCM in this individual. Further investigation revealed hypokalemia, elevated aldosterone levels, decreased plasma renin activity, and an aldosterone-to-renin ratio above 30. These findings strongly indicated primary aldosteronism (PA) as an additional condition affecting this patient. Through the utilization of whole exome sequencing, we successfully identified a suspected pathogenic gene TTN as the underlying cause of the patient's condition. The presence of HCM accompanied by secondary hypertension due to PA resulted in significant enlargement of the left ventricle, particularly the ventricular septum. While certain genetic mutations may suggest a potential link to cardiomyopathy development, they cannot definitively establish a direct association between HCM and PA.
| S-EPMC10661324 | biostudies-literature
Project description:A newborn presenting with cyanosis at day 9 of life was admitted to the local hospital. Initial local echocardiography confirmed a cardiac issue and the patient was transferred to a tertiary cardiac hospital. Further imaging confirmed a rare presentation of cardiomyopathy with severe right ventricular outflow tract obstruction. Surgery was performed but with postoperative haemodynamic instability complicated by incessant ventricular tachycardia. Following discussion with the family, care was withdrawn. Postmortem demonstrated a rare form of hypertrophic cardiomyopathy with right ventricular outflow tract obstruction not previously described in a neonate.
| S-EPMC4208136 | biostudies-literature
Project description:The clinical spectrum of hypertrophic cardiomyopathy (HCM) is complex and includes a variety of phenotypes, which leads to different types of manifestations. Although most of the patients are asymptomatic, a significant proportion of them will develop symptoms or risk of arrhythmias and sudden cardiac death (SCD). Therefore, the objectives of HCM diagnosis and management are to relieve the patients' symptoms (chest pain, heart failure, syncope, palpitations, etc.), prevent disease progression and major cardiovascular complications and SCD. The heterogeneity of HCM patterns, their symptoms and assessment is a challenge for the cardiologist.
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