Mid-ventricular Hypertrophic Obstructive Cardiomyopathy Research Articles

Resection of hypertrophic papillary muscles and mitral valve replacement in a patient with midventricular hypertrophic obstructive cardiomyopathy – a new approach

Midventricular hypertrophic obstructive cardiomyopathy (HOCM) is characterized by hypertrophy of the interventricular septum (IVS) and - in rare cases - of the papillary muscles (PM), which subsequently can cause dynamic left ventricular outflow tract obstruction (LVOTO) and severe heart failure symptoms. We report on a rare case of a 44-year-old patient suffering from midventricular HOCM with hypertrophic anterolateral PM and an additional chorda between the PM and the IVS.We describe a new surgical approach via right anterolateral thoracotomy in 3-dimensional (3D) video-assisted minimal-invasive technique with resection of hypertrophic PMs as well as the entire mitral valve-apparatus and valve replacement avoiding septal myectomy and potentially associated complications. After an uneventful procedure clinical symptoms improved from NYHA III-IV at baseline to NYHA 0-I postoperatively and remained stable over a follow-up period of 24 months. Therefore, the presented technique may be considered as a new and alternative approach in patients with hypertrophic PMs and hypertrophic IVS as subtype of midventricular HOCM.

Abstract 15675: Mid-Ventricular Hypertrophic Cardiomyopathy Presenting With Minoca

Background: Mid-ventricular hypertrophic obstructive cardiomyopathy (HOCM) is an uncommon form of cardiomyopathy that is linked to an apical aneurysm, increasing the risk of sudden death. Cases: We described four patients who presented with chest pain or SOB. Troponin elevation, acute myocardial infarction on cardiac MRI and absence of obstructive coronary disease confirmed the diagnosis of MINOCA. Echo revealed a pressure gradient between the apical and basal chambers of the left ventricle. Cardiac MRI showed mid-ventricular HOCM and mid-cavity obliteration with akinetic apical chamber LGE revealed areas of sub-endocardial or transmural myocardial infarction at the level of obstruction and at the apical segments distal to the obstruction. The presence of myocardial edema on tissue mapping in the same area suggested acute myocardial injury (Figure). Discussion: The pathogenesis of development of apical myocardial scarring and aneurysm formation in mid-ventricular HOCM remains unknown. Other variant of hypertrophic cardiomyopathy shows mid-myocardial foci of scar inside the hypertrophied segments, indicating fibrosis. Whereas in our patients, the LGE revealed a sub-endocardial or transmural infarct (myocardial infarction pattern) - an uncommon pattern in other hypertrophic cardiomyopathy variants. This pattern and elevation of troponin during the acute phase of illness supports the hypothesis that apical aneurysm is due to apical myocardial ischemia in mid-ventricular HOCM. Tissue characterization and pattern of scar by cardiac MRI in these patients could be useful in understanding the pathophysiology of this type of HOCM.

Midventricular Hypertrophic Obstructive Cardiomyopathy with Left Ventricular Aneurysm and Clot: The Role of Transesophageal Echocardiogram in Assessment and Management of Myomectomy

ABSTRACT Hypertrophic obstructive cardiomyopathy (HOCM) with mid ventricular obstruction leading to apical aneurysm and clot is very rare. Intraoperative transesophageal echocardiogram (TEE) can be used to know the maximum thickness of the septum, its distance from the aortic annulus and the apical extent of septal bulge. Postresection TEE can provide information about residual obstruction or any complications. We present a case of 65-year-old male patient who underwent CABG, septal myectomy, Dor's procedure for aneurysm and clot removal. Septal resection was done under TEE guidance. How to cite this article Dutta V, Raj R, Kumar B, Bahl A, Thingam SKS, Negi SL, Puri GD. Midventricular Hypertrophic Obstructive Cardiomyopathy with Left Ventricular Aneurysm and Clot: The Role of Transesophageal Echocardiogram in Assessment and Management of Myomectomy. J Perioper Echocardiogr 2014;2(2):58-60.

Comparison of the Prevalence, Clinical Features, and Long-term Outcomes of Midventricular Hypertrophy vs Apical Phenotype in Patients With Hypertrophic Cardiomyopathy

BackgroundPrevious studies on the association between the distribution of left ventricle hypertrophy and the clinical features of hypertrophic cardiomyopathy (HCM) have yielded unclear results. The aim of this study was to investigate the differences in the prevalence, clinical features, management strategies, and long-term outcomes between patients with midventricular hypertrophic obstructive cardiomyopathy (MVHOCM) and patients with apical HCM (ApHCM). MethodsA retrospective study of 60 patients with MVHOCM and 263 patients with ApHCM identified in a consecutive single-centre cohort consisting of 2068 patients with HCM was performed. The prevalence, clinical features, and natural history of the patients in these 2 groups were compared. ResultsCompared with ApHCM patients, patients with MVHOCM tended to be much younger and more symptomatic during their initial evaluation. Over a mean follow-up of 7 years, the probability of cardiovascular mortality and that of morbidity was significantly greater in MVHOCM patients compared with ApHCM patients (log-rank, P < 0.001). ConclusionsOur results suggest that, compared with ApHCM, MVHOCM represents an uncommon presentation of the clinical spectrum of HCM that is characterized by progressive clinical deterioration leading to increased cardiovascular mortality and morbidity. Our results also underscore the importance of the timely recognition of MVHOCM for the prediction of prognosis and the early consideration of appropriate management strategies.

Multimodality imaging of the ace of spades

A 53-year-old man was admitted to our institute with chest pain and palpitation. Using contrast echocardiography, left ventriculography and cardiac MRI, a subsequent diagnosis of mid-ventricular hypertrophic obstructive cardiomyopathy with an apical aneurysm was made. The current case highlights the benefits of multimodality imaging in not only achieving a diagnosis but also in comprehending the pathophysiological origins of this unusual phenomenon.

Effective Disopyramide Treatment in a Boy With Mid-Ventricular Hypertrophic Obstructive Cardiomyopathy.

A 14-year-old boy with mid-ventricular hypertrophic obstructive cardiomyopathy (MVHOCM) first presented at the age of 10 years with severe chest pain. Two-dimensional echocardiography disclosed marked hypertrophy at the mid-portion of the ventricular septum, and left ventriculography showed an hourglass appearance at systole. He was initially treated with propranolol, but the chest pain and dyspnea on exertion worsened at the age of 12 years. After disopyramide was started, the chest pain disappeared and the degree of the pressure gradient at the mid-ventricular level was reduced. There was also significant improvement on a 123I beta-methyliodophenyl pentadecaonic acid (BMIPP) myocardial scintigram.

Surgical treatment of hypertrophic obstructive cardiomyopathy with special reference to complications and to atypical hypertrophic obstructive cardiomyopathy.

The Düsseldorf surgical series of patients with hypertrophic obstructive cardiomyopathy (HOCM) (1963-82) comprises 137 patients, of whom 123 had typical HOCM and 14 atypical midventricular stenosis. Indications for surgery were unsatisfactory response to drug therapy and clinical symptoms according to at least functional class III (NYHA). The predominant approach for the relief of both types of HOCM was transaortic (112 cases; two deaths = 1.8%). The hospital mortality of the total series was 6.6% (nine of 137 patients). The most severe problems were caused by additional acquired mitral valve lesions. There is no special risk for patients with atypical midventricular HOCM. The systolic gradient between left ventricle and aorta could be removed or diminished in all cases.

Surgical aspects of typical subaortic and atypical midventricular hypertrophic obstructive cardiomyopathy (HOCM).

Hypertrophic obstructive cardiomyopathy (HOCM) is a special form of left ventricular outflow tract obstruction. Clinical experience demonstrates 2 different types of HOCM of the left ventricle: the typical subaortic or subvalvular HOCM and the atypical midventricular form of myocardial obstruction. In a series of 108 patients operated upon, 96 had typical and 12 had atypical HOCM. Indications for surgical treatment were increase of clinical symptoms to class III or IV (NYHA) and unresponsiveness to drug therapy (beta-blocking agents, calcium-antagonists). The male: female ratio was 21:1. The mean age in the group with typical HOCM was 38.8 years, in the group with atypical HOCM 46.5 years. Transaortic myectomy (n = 85) was the predominant surgical approach for relief of isolated subaortic and midventricular obstruction. Patients with severe mitral insufficiency and mitral valve replacement were identified as a problematic group. The total hospital mortality was 8.3% (9 of 108 patients), 7.9% (7 of 96 patients) in the typical group, and 16.7% (2 of 12 patients) in the atypical group. Postoperative cardiological follow-up indicates the superior effect of surgery as compared to drug therapy. The late mortality for the medical group was 10% and for the postoperative group 2.5%. These facts support our recommendation for the surgical treatment of HOCM in symptomatic patients in clinical stages III and IV (NYHA).