Case Of Hypertrophic Obstructive Cardiomyopathy Research Articles

Macroscopic and microscopic features in a fatal case of hypertrophic obstructive cardiomyopathy after myectomy

Hypertrophic Obstructive Cardiomyopathy (HOCM) is an autosomal dominant genetic disorder that results in interstitial fibrosis and ventricular hypertrophy. There is severe and asymmetric enlargement of the wall of the left ventricle and especially the septum, which may be localized or diffuse [1].

Therapeutic Strategy for Patients with Concomitant Pulmonary Artery Hypertension and Hypertrophic Obstructive Cardiomyopathy: A Rare Case Report

Combined cases of hypertrophic obstructive cardiomyopathy (HOCM) and pulmonary arterial hypertension (PAH) are rare and have a management dilemma. Although preload is crucial in the management of HOCM, anti-PAH agents dramatically change the preload, leading to improving or worsening heart failure in patients with HOCM. We had a 74-year-old woman with Sjogren-syndrome-associated PAH. Her heart failure worsened following the initiation of anti-PAH agents due to an incremental preload on the left ventricle, whereas HOCM clinically developed following the termination of anti-PAH agents and progressing anorexia due to the progression of the left ventricular outflow obstruction. Careful monitoring of the left ventricular outflow obstruction during initiation/termination of anti-PAH agents and medical intervention to the HOCM are highly recommended.

A Case of Hypertrophic Obstructive Cardiomyopathy in Which Intra-Aortic Balloon Pumping Contributed to Cardiogenic Shock

A Case of Hypertrophic Obstructive Cardiomyopathy in Which Intra-Aortic Balloon Pumping Contributed to Cardiogenic Shock

Efficacy of cibenzoline for hypertrophic obstructive cardiomyopathy in paediatric patients with RAS/MAPK pathway syndromes.

RASopathies - caused by mutations in the RAS/MAPK signaling pathway - are frequently associated with cardiac diseases, such as hypertrophic obstructive cardiomyopathy. Although cibenzoline is useful for adult hypertrophic obstructive cardiomyopathy patients, little is known about its effect in children. Here, we report two paediatric cases of hypertrophic obstructive cardiomyopathy associated with RASopathies where the condition was improved by cibenzoline.

Case of hypertrophic obstructive cardiomyopathy with rhino-orbito cerebral mucormycosis - Difficult airway as anaesthestic challenge

Case of hypertrophic obstructive cardiomyopathy with rhino-orbito cerebral mucormycosis - Difficult airway as anaesthestic challenge - IJCA- Print ISSN No: - 2394-4781 Online ISSN No:- 2394-4994 Article DOI No:- 10.18231/j.ijca.2022.083, Indian Journal of Clinical Anaesthesia-Indian J Clin Anaesth

Anaesthetic Management of Patient with Hypertrophy Obstructive Cardiomyopathy Posted for Elective Caesarean

Introduction: Dynamic valvular insufficiency and LVOT blockage are also possible outcomes of hypertrophic obstructive cardiomyopathy (HOCM). Furthermore, attempting to replace an inadequate mitral valve surgically can result in iatrogenic LVOT blockage. In the general population, it affects one out of every 500 adults, with a male to female ratio of 2:1. Among the general population, the prevalence is around 0.2 percent, and the incidence in pregnant women is around 0.1–0.5 percent. Case Report: 21 year old female patient presented with 9 months of amenorrhea posted for elective lower segment caesarean section. Her previous documents revealed that she was a diagnosed case of hypertrophic obstructive cardiomyopathy during her last pregnancy 1 year ago. History of previous surgery that is induced abortion at two and half months of gestation age a year back operated under spinal anaesthesia. ECG showed ST elevation present in V2 , V3, V4 , V5 leads and features of left ventricular hypertrophy (LVH) , Her echocardiography report revealed features of hypertrophic obstructive cardiomyopathy with severe LVOT obstruction. Left ventricular ejection fraction (LVEF) was 20-25%. Conclusion: HOCM is normally well tolerated during pregnancy, although those who have had previous symptoms or arrhythmias may experience an exacerbation of symptoms. The majority of these people, on the other hand, can be effectively controlled medically. This case report showed that patients with HOCM can be safely managed under general anaesthesia with muscle relaxants and inhalational volatile anaesthetics, as well as an erector spinae block for analgesia

Alcohol Septal Ablation in Hypertrophic Obstructive Cardiomyopathy- Is Myocardial Contrast Echocardiography Mandatory?

Alcohol septal ablation (ASA) is an effective treatment for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). Although routine use of myocardial contrast echocardiography (MCE) in guiding ASA has been recommended by guidelines, it has not come into common clinical practice. Here, we present three cases of HOCM where MCE helped in decision-making.

Mega Coronary Arteries with Obstructive Coronary Artery Disease in Hypertrophic Obstructive Cardiomyopathy: A Case Report and Literature Review

We report a rare case of hypertrophic obstructive cardiomyopathy (HOCM) presenting with mega coronary arteries with obstructive coronary artery disease. Interestingly, the patient had large septals, large diagonals, large obtuse marginal with a large left atrial branch, and a large conus almost mimicking a duplicate right coronary artery. Because of massive left ventricular hypertrophy, to overcome the supply-demand mismatch, coronary arteries may become large or with more arborization in hypertrophic cardiomyopathy but the same is rarely encountered in routine clinical practice. Our case is a rare illustration of the simultaneous presence of mega coronary arteries and critical obstructive coronary artery disease in HOCM in a young male of 38 years of age.

A Case of Hypertrophic Obstructive Cardiomyopathy Required Repeat Percutaneous Transluminal Septal Myocardial Ablation for Mid-Ventricular Obstruction After Release of Left Ventricular Outflow Tract in the First Percutaneous Transluminal Septal Myocardial Ablation

A Case of Hypertrophic Obstructive Cardiomyopathy Required Repeat Percutaneous Transluminal Septal Myocardial Ablation for Mid-Ventricular Obstruction After Release of Left Ventricular Outflow Tract in the First Percutaneous Transluminal Septal Myocardial Ablation

Dynamic thromboembolic left ventricular outflow tract obstruction after aggressive procoagulant treatment in hemorrhagic shock: a case report

BackgroundIn cases of hypertrophic obstructive cardiomyopathy (HOCM), the systolic anterior motion of the mitral valve apparatus results in an obstruction of the left ventricular outflow tract (LVOT), which is known as the SAM [systolic anterior motion] phenomenon. Hypothetically, a pathological obstruction of the LVOT of a different etiology would result in a comparable hemodynamic instability, which would be refractory to inotrope therapy, and may be detectable through echocardiography.Case presentationWe observed a severely impaired left ventricular function due to a combination of a thrombotic LVOT obstruction and distinctive mitral regurgitation in a 56-year-old Caucasian, female patient after massive transfusion with aggressive procoagulant therapy. Initially, the patient had to be resuscitated due to cardiac arrest after a long-distance flight. The resuscitation attempts in combination with lysis therapy due to suspected pulmonary artery embolism were initially successful but resulted in traumatic liver injury, hemorrhagic shock and subsequent acute respiratory distress syndrome (ARDS). Oxygenation was stabilized with veno-venous extracorporeal membrane oxygenation (ECMO), but the hemodynamic situation deteriorated further. Transesophageal echocardiography (TEE) showed a massive, dynamic LVOT obstruction. Two thrombi were attached to the anterior leaflet of the mitral valve, resulting in a predominantly systolic obstruction. Unfortunately, the patient died of multiple-organ failure despite another round of lysis therapy and escalation of the ECMO circuit to a veno-venoarterial cannulation for hemodynamic support.ConclusionMassive transfusion with aggressive procoagulant therapy resulted in mitral valve leaflet thrombosis with dynamic, predominantly systolic LVOT obstruction, comparable to the SAM phenomenon. The pathology was only detectable with a TEE investigation.

Automated heart segmentation using a convolutional neural network accelerates 3D model creation for cardiac surgery

Abstract Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Japan Organization of Occupational Health and Safety onbehalf J3D Introduction Improvements in cardiovascular imaging have contributed to the accurate diagnosis and treatment of cardiovascular diseases. In recent years, we can find a new demand from surgical departments, i.e., 3D heart models of patients for preoperative simulations. However, a 3D surgical model needs a detailed segmentation of heart structures from clinical images because these details are essential for achieving accurate polygon data of an exact 3D model. Thus, in most cases, manual segmentation is required for complicated heart shapes such as the chamber wall, valves, and papillary muscles, which causes a prolonged duration time. Purpose We aim to achieve an automated heart segmentation using a convolutional neural network (CNN) trained using deep learning techniques for the rapid creation of 3D surgical heart models of individual patients. Methods We constructed our original CNN program based on the latest artificial intelligence techniques and trained it to extract shapes of the heart from cardiac computed tomography (CT) images. The training data was 361 slices selected from CT scans of 10 patients. We used data augmentation to increase the amount and diversity of the data into 24,052 slices. The training result with the best Intersection over Union (IoU), one of the evaluation metrics used in deep learning, was saved. Finally, we used the best-trained CNN to construct 3D polygon data of two surgical cases of hypertrophic obstructive cardiomyopathy (HOCM) for preoperative assessments. Results The IoU attained 0.85 after deep learning. The time required to complete the 3D polygon data for the first HOCM case was 5 minutes for segmentation by the trained CNN and 3 hours for data correction by a human operator. Similarly, the time required for the second case was 5 minutes for segmentation without manual correction. We had to correct the segmentation for the first case because we needed an exact 3D model for the preoperative assessment (Fig. 1). According to our records of the other eight 3D heart models in the lab, the work for a 3D polygon shape from CT images needs a median 30 hours (quartiles 23-50 hours) when the procedure is fully manual and non-continuous with breaks in between. Conclusion The CNN-based segmentation aided the constructing heart shapes from cardiac CT images of preoperative patients. Although the performance, reaching IoU of 0.85, was insufficient for fully automatic segmentation, the methodology can shorten the process duration from several hours to several minutes for detailed segmentation of heart structures. We previously applied the CNN-based segmentation to the aorta, aortic stenosis valves, and atheromatous plaque in clinical images, demonstrating adequate segmentation performance. The proposed methodology can be applied as a fundamental technology of cardiovascular imaging for obtaining the actual structures of a target object as 3D coordinate values or a 3D model within a reasonable duration time. Abstract Figure 1

Hypertrophic obstructive cardiomyopathy and its outcome following surgical myectomy: a retrospective study

Background: Hypertrophic cardiomyopathy is highly heterogeneous with a diverse anatomy, pathophysiology, and clinical course. It is obstruction to left ventricular outflow that has become the major hallmark of the disease. Septal myectomy has been the gold standard treatment for the relief of left ventricular outflow tract obstruction and cardiac symptoms in both adults and children with obstructive hypertrophic cardiomyopathy. Objective of the study was to evaluate effect of Myomectomy and its impact on survival for a period of one year.Methods: The study design is a retrospective record based observational study. Data was retrieved from previous records both electronic as well as manual records of all the patients who underwent myectomy with or without concomitant procedures such as mitral valve replacement or aortic valve replacement or coronary artery bypass surgery during 2014 to 2018.Results: Majority of the patients 11 (52.4%) in fourth decade i.e. 40-59 years age group. majority were males i.e. 16 (76.2%) and remaining 5 i.e. 23.8% were females. Male to female ratio was 3.2:1. Dyspnoea was present 81% and chest pain in 76.2%. Preoperative LVOT gradient was 86.86±20.33 and post-operative gradient was 23.47±20.49.Conclusions: Operative techniques have evolved from simple myotomy to the present method of extended septal myectomy which can be done in all adult cases of hypertrophic obstructive cardiomyopathy.

MicroRNA-221 is a potential biomarker of myocardial hypertrophy and fibrosis in hypertrophic obstructive cardiomyopathy.

Aim: Circulating microRNA expression has become a biomarker of cardiovascular disease; however, the association of microRNA expression between circulation and myocardium in hypertrophic cardiomyopathy remains unclear. The present study aimed to find a circulating biomarker correlated not only to myocardial expression, but also to cardiac hypertrophy and fibrosis. Method: Forty-two cases of hypertrophic obstructive cardiomyopathy (HOCM) diagnosed by echocardiography and magnetic resonance were analysed for microRNA expression in plasma and myocardial tissue. Results: The results showed that myocardial miR-221 was significantly increased (z = −2.249, P = 0.024) and significantly correlated with collagen volume fraction (CVF) (r = 0.516, P < 0.001), late gadolinium enhancement (LGE) (r = 0.307, P = 0.048), and peripheral circulation (r = 0.434, P = 0.004). Moreover, circulating miR-221 expression was significantly correlated with CVF (r = 0.454, P = 0.002), LGE (r = 0.630, P = 0.004), maximum interventricular septal thickness (MIVST) of echocardiography (r = 0.318, P = 0.042), and MIVST of magnetic resonance (r = 0.342, P = 0.027). The area under the receiver operating characteristic curve of miR-221 was 0.764. Conclusions: Circulating miR-221 is consistent with that in myocardial tissue, and correlated with myocardial fibrosis and hypertrophy. It can be used as a biomarker for evaluating myocardial hypertrophy and fibrosis in HOCM.

The change of left ventricular function and mechanical dispersion in patients with hypertrophic cardiomyopathy: a study with exercise stress echocardiography

Objective To investigate the association between the change of left ventricular (LV) function and mechanical dispersion (MD) and exercise capacity in patients with hypertrophic cardiomyopathy (HCM) by exercise stress echocardiography. Methods Sixty-five HCM patients [40 cases of hypertrophic non-obstructive cardiomyopathy (HNCM), 25 cases of hypertrophic obstructive cardiomyopathy (HOCM)] and 25 control subjects were recruited.LV function, MD and exercise capacity were evaluated by two-dimensional speckle-tracking imaging and echocardiography at rest and during exercise, and the following parameters of LV function were recorded: LV global longitudinal strain (LVGLS), MD, early diastolic strain rate (SRe), the ratio of peak early diastolic mitral inflow and annulus velocity (E/e′), LV outflow tract gradient (LVOTG); LV functional reserve was assessed by ΔLVGLS and ΔSRe; exercise capacity was evaluated by metabolic equivalents (METs). The association between the change of LV function and MD and exercise capacity was investigated. Results ①Compared with normal controls, LVOTG, E/e′ and MD increased, and LVGLS, SRe, ΔLVGLS, ΔSRe and METs decreased in HNCM patients at rest and during exercise (all P<0.05). ②LVOTG, E/e′ and MD were further increased, LVLGS, SRe, ΔSRe and METs were further reduced in HOCM patients compared with HNCM patients (all P<0.05). ③LVGLS and MD measured at peak exercise were associated with METs(r=-0.68, P<0.001; r=-0.43, P<0.001). ④ROC curve analysis showed LVGLS had a better predictive value for exercise intolerance in HCM patients, followed by E/e′ and MD. Conclusions LV function and mechanic reserve are reduced but MD is increased in HCM patients, especially in HOCM patients. Exercise capacity is associated with LV function and MD, which can predict the reduced exercise capacity in HCM patients. Key words: Exercise stress echocardiography; Two-dimensional speckle-tracking imaging; Cardiomyopathy, hypertrophic; Ventricular function, left; Mechanical dispersion

A case of myocarditis assessed by multi-parametric cardiovascular magnetic resonance

该患者临床表现为胸闷和胸痛，起病前有发热病史，辅助检查提示炎症指标和心肌坏死标志物升高，考虑心肌炎可能性大。通过应用磁共振定量组织标记技术结合传统路易斯湖诊断标准，诊断该患者为心肌炎。予大剂量激素及支持治疗后，患者病情好转。该病例表明，心脏磁共振多序列成像在心肌炎的诊断和指导治疗方面具有重要意义。.

Anaesthetic management of a patient with hypertrophic obstructive cardiomyopathy posted for emergency lower segment caesarean section

Hypertrophic cardiomyopathy is characterised by asymmetric hypertrophy of the interventricular septum causing intermittent obstruction of the left ventricular outflow tract. This disease may present to the anaesthesiologist in an undiagnosed form during routine pre-operative visit. Surgery and anaesthesia often complicate the peri-operative outcome in this group of patients. A 22-year-old patient was brought in for emergency lower segment caesarean section. She was a diagnosed case of hypertrophic obstructive cardiomyopathy (HOCM) with severe left ventricular outflow tract obstruction and moderate subaortic stenosis. Factors such as tachycardia, hypovolemia, vasodilatation, and increased cardiac contractility leads to exacerbation of this obstruction. We managed this case successfully considering the pathological issues caused by the disease and pregnancy.

Anterior mitral leaflet repair in hypertrophic obstructive cardiomyopathy: A tailored approach under transesophageal echocardiographic guidance

Hypertrophic obstructive cardiomyopathy (HOCM) is the most common inherited cardiovascular disease, which causes significant involvement of mitral valve in the pathogenesis of left ventricular outflow tract (LVOT) obstruction. A 37-year-old man, a diagnosed case of HOCM, reported to us with complaints of angina and difficulty in breathing. His preoperative echocardiography revealed a predominant LVOT obstruction which caused a resting gradient of 50 mmHg. It was associated with the presence of systolic anterior motion (SAM) and posteriorly directed moderately severe regurgitation jet. Plication of anterior mitral leaflet (AML) was done through transaortic approach while performing septal myectomy. Plicating AML through transaorta was tailored under transesophageal echocardiographic guidance where the height of AML was reduced by 1 cm. As a result, postoperative SAM was significantly reduced. The approach of plicating AML through transaorta became technically simpler with the help of transesophageal echocardiography.

A Case of Hypertrophic Obstructive Cardiomyopathy and Vasospastic Angina, in Which Controlling Medical Treatment was in Great Distress

Vasospastic angina (VSA) is accompanied with hypertrophic obstructive cardiomyopathy (HOCM). In such patients, controlling medical treatment may be sometimes difficult, because some drugs for VSA may deteriorate the status of HOCM. We experience such case. A woman in her seventies, was admitted to our institution, because of detail examination of chest symptoms at rest. Coronary angiography showed the presence of coronary spasm. In addition, cardiac catheterization showed that the positive Brochenbrough phenomenon. This patient was diagnosed as having HOCM and VSA. Diltiazem was prescribed and she had been followed by her family doctor. Two years later, she could not walk for a short distance and was admitted to our institution. At admission, she took some medications, such as diltiazem, telmisartan and diuretics. Blood chemical data showed the elevation of BNP (769 pg/mL). Echocardiography (UCG) showed the significant pressure gradient through left ventricle outflow tract (LVOT, 159 mmHg) at rest. We judged the worsening status of HOCM and prescribed cibenzoline with 150 mg, but pressure gradient did not changed. We stopped the diuretics and changed from diltiazem to verapamil. Thereafter, her symptoms improved and the pressure gradient though LVOT reduced and she was discharged. Several cardiovascular medications may affect the status of HOCM and the cardiologists should be careful in selection of such drugs in patients with HOCM.

A rare case of hypertrophic obstructive cardiomyopathy with mitral valve stenosis

A rare case of hypertrophic obstructive cardiomyopathy with mitral valve stenosis

A case of hypertrophic obstructive cardiomyopathy (HOCM) complicated with Takotsubo cardiomyopathy

A case of hypertrophic obstructive cardiomyopathy (HOCM) complicated with Takotsubo cardiomyopathy