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Hypertrophic cardiomyopathy with massive midventricular hypertrophy, midventricular obstruction and an akinetic apical chamber/Akinetik apikal bosluk, midventrikuler obstruksiyon ve midventrikuler hipertrofi ile seyreden hipertrofik kardiyomiyopati.

Introduction

Hypertrophic Cardiomyopathy (HCM) with midventricular obstruction and an akinetic apical chamber is a rare condition occuring in 1% of HCM patients, that carries significant risk of adverse clinical events. This case presentation provides examples of the echocardiographic findings, prognostic implications and review of recent literature regarding diagnosis, managment, and treatment of this uncommon abnormality.

Case Report

A 65-year-old female with dyspnea on exertion was diagnosed with hypertrophic cardiomyopathy at age 58 by echocardiography, after experiencing palpitations. She now presents with dyspnea after walking up two flights of stairs, and also on level ground after only 2 blocks; she was New York Heart Association Class III. Her medication regimen was alendronate and Ecotrin 325 mg. She has no history of hypertension and, with the exception of the above, her past medical history is significant only for ocular migraines. There is no family history of hypertrophic cardiomyopathy or sudden cardiac death. She smokes one pack of cigarettes a day.

Her blood pressure was 110/70 mmHg and a pulse was 68 beats per minute. Chest was clear. Cardiac examination: regular rhythm and grade I/VI systolic ejection murmur that rises to II/VI with standing or Valsalva. No peripheral edema. Electrocardiogram showed sinus rhythm, premature atrial contraction, intraventricular conduction delay, and left ventricular (LV) hypertrophy. Echocardiogram revealed complete systolic obliteration of the left ventricle seen in the parasternal view. There was obstruction of the mid ventricle and apex due to systolic apposition over a very long segment of the mid and apical left ventricle (Fig 1. See corresponding video movie at www.anakarder.com). There was massive hypertrophy of the mid left ventricle with the mid posterior septum and lateral wall measuring 30 mm and the mid inferior wall measuring 37 mm (Fig. 2).

[FIGURES 1-2 OMITTED]

There was an akinetic apical cavity measuring 2x2 cm containing an apical thrombus (Fig 3. See corresponding video movie at www.anakarder.com). In the apical view on color Doppler there was a diastolic high velocity jet from apex towards the base that appeared in early diastole--paradoxical jet flow (Fig. 4). Spectral Doppler also revealed the paradoxical jet flow in early diastole along with mitral regurgitation in systole (Fig. 5). After injection of Definity, an intravenous contrast agent, visualization of the akinetic apical chamber was greatly improved. Interestingly, intramyocardial perforator vessels were seen coursing from epicardium to endocardium (Fig. 6,7,8, See corresponding video movies at www.anakarder.com).

[FIGURES 3-8 OMITTED]

Impression: The patient presented multiple issues. Her overall risk stratification for sudden death was estimated at 2-3% per year because of the massive left ventricular hypertrophy and greater predisposition for fatal ventricular arrhythmias originating from the apical akinetic chamber.

Akinesis of the apical chamber is attributed to ischemia from increased wall stress and work due to high systolic and diastolic pressures combined with massive hypertrophy. This leads to supply-demand mismatch. She has a higher risk of embolism because she had a thrombus in the apical chamber.

Cardiac catheterization showed large coronary arteries, with no significant coronary stenoses, with systolic milking of the apical and mid septal perforators, milking of the distal left anterior descending artery and milking of the distal right coronary artery. Left ventriculogram showed an ejection fraction of 80%, midventricular systolic obliteration, elevated left ventricular end-diastolic pressure, and an akinetic apical chamber.

To address her risk of sudden cardiac death an implanted defibrillator was placed. Coumadin was prescribed for the apical thrombus. For her symptoms, she had trial of DDD pacing with short atrioventricular (AV) delay with improvement in symptoms. Nicotine patches and smoking cessation were recommended. Screening of her first degree relatives for inheritance of HCM was recommended.

Discussion

Hypertrophic cardiomyopathy with midventricular obstruction with an apical akinetic chamber is uncommon in non-Asian populations, occurring in 1% of cases (1). Echocardiography is characterized by hypertrophy occurring solely in the mid left ventricle and systolic apposition of midventricular walls (2-4). Doppler echocardiography may reveal an unusual intracavitary signal termed diastolic paradoxic jet flow directed from the apex toward the base during LV isovolumetric relaxation and the LV filling period (5,6). There is an abnormal akinetic apical chamber caused by systolic trapping of blood in the apex. This causes high gradients and symptoms (5,6).

The apical akinetic chamber may be difficult to visualize due to massive hypertrophy. Thus, paradoxic jet flow is an important marker of concealed apical asynergy and also risk of adverse clinical events--ventricular tachycardia, myocardial ischemia and necrosis (5,6). The high diastolic apical pressure indicated by the high velocity flow contributes to the development of the apical aneurysm that occurs in the absence of fixed coronary artery disease (5,7). The aneurysm predisposes to thrombus and systemic embolism (7). The apical aneurysm is thought to be the result of supply-demand mismatch and also due to ischemia caused by narrowings in the intramural penetrating coronary arteries (8).

There is ambiguity in these patients with akinetic chambers as to whether they have apical or mid HCM (2). Our patient fits better in the category of midobstruction. Apical hypertrophy in Japan and North America is generally benign (7,9-10) while midventricular obstruction may be more symptomatic and more of a clinical problem (2-4,11). Eriksson et al. (10) reviewed their series of 105 patients with apical HCM with a mean follow-up of 13.6 [+ or -] 8.3 years from presentation; overall cardiovascular mortality was 1.9% (2/105) and annual cardiovascular mortality was 0.1%. Overall survival was 95% at 15 years. Probability of survival without morbid events was 74% at 15 years. Three predictors of cardiovascular morbidity were identified: age at presentation < 41 years, left atrial enlargement, and New York Heart Association (NYHA) class [greater than or equal to] II at baseline (10). However heart failure due to atrial fibrillation and left ventricular aneurysm have prognostic importance (7); aneurysm can be complicated by sustained ventricular tachycardia in some patients (12,13) Implantable defibrillators may be considered in patients with ventricular tachycardia or with massive hypertrophy (14).

For symptoms, negatively inotropic drugs should generally be tried first. For treatment failures in midventricular HCM, surgery is a less feasible option because of the midventricular site of obstruction but has been performed (4,15). DOD pacing with short AV delay may result in hemodynamic and symptomatic improvement (16-19).

References

(1.) Kitaoka H, Doi Y, Casey SA, Hitomi N, Furuno T, Maron BJ. Comparison of prevalence of apical hypertrophic cardiomyopathy in Japan and the United States. Am J Cardiol 2003; 92: 1183-6.

(2.) Wigle E, Rakowski H. Hypertrophic Cardiomyopathy. When do you diagnose midventricular obstruction versus apical cavity obliteration with a small nonobliterated area at the apex of the left ventricle? J Am Coll Cardiol 1992; 19: 525-6.

(3.) Zoghbi W, Haichin R, Quinones M. Midcavity obstruction in apical hypertrophy: Doppler evidence of diastolic intraventricular gradient with higher apical pressure. Am Heart J 1988; 116: 1469-74.

(4.) Bircks W, Schulte HD. Surgical treatment of hypertrophic obstructive cardiomyopathy with special reference to complications and to atypical hypertrophic obstructive cardiomyopathy. Eur Heart J 1983; 4: 187-90.

(5.) Nakamura T, Matsubara K, Furukawa K, Azuma A, Sugihara H, Katsume H, et al. Diastolic paradoxic jet flow in patients with hypertrophic cardiomyopathy: evidence of concealed apical asynergy with cavity obliteration. J Am Coll Cardiol 1992; 19: 516-24.

(6.) Penas Lado M, Freire E, Garcia Castelo A, Vazquez N, Castro-Beiras A. A paradoxical intraventricular gradient in a patient with atypical hypertrophic myocardiopathy. Rev Esp Cardiol 1995; 48: 686-9. 7. Sakamoto T. Apical hypertrophic cardiomyopathy (apical hypertrophy): an overview. J Cardiol 2001;37 Suppl 1:161-78.

(8.) Sherrid MV, Mahenthiran J, Casteneda V, Fincke R, Gasser M, Barac I, et al. Comparison of diastolic septal perforator flow velocities in hypertrophic cardiomyopathy versus hypertensive left ventricular hypertrophy. Am J Cardiol 2006; 97: 106-12.

(9.) Sakamoto T, Amano K, Hada Y, Tei C, Takenaka K, Hasegawa I, et al. Asymmetric apical hypertrophy: ten years experience. Postgrad Med J 1986; 62: 567-70.

(10.) Eriksson MJ, Sonnenberg B, Woo A, Rakowski P, Parker TG, Wigle ED,. et al. Long-term outcome in patients with apical hypertrophic cardiomyopathy. J Am Coll Cardiol 2002; 39: 638-45.

(11.) Falicov RE, Resnekov, L. Mid-ventricular obstruction: Variant of obstructive cardiomyopathy. Am J Cardiol 1976; 37: 432-7.

(12.) Wilson P, Marks A, Rastegar H, Manolis AS, Estes NA, 3rd. Apical hypertrophic cardiomyopathy presenting with sustained monomorphic ventricular tachycardia and electrocardiographic changes simulating coronary artery disease and left ventricular aneurysm. Clin Cardiol 1990;13: 885-7.

(13.) Ito N, Suzuki M, Enjoji Y, Nakamura M, Namiki A, Hase H, et al. Hypertrophic cardiomyopathy with mid-ventricular obstruction complicated with apical left ventricular aneurysm and ventricular tachycardia: a case report. J Cardiol 2002; 39: 213-9.

(14.) Maron BJ, Shen WK, Link MS, Epstein AE, Almquist AK, Daubert JP, et al. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. New Engl J Med 2000; 342: 365-73.

(15.) Schulte HD. Bircks W. Surgical aspects of typical subaortic and atypical midventricular hypertrophic obstructive cardiomyopathy (HOCM). Thoracic Cardiovasc Surg 198; 29: 375-80.

(16.) Ommen SR, Nishimura RA, Squires RW, Schaff HV, Danielson GK, Tajik AJ. Comparison of dual-chamber pacing versus septal myectomy for the treatment of patients with hypertrophic obstructive cardiomyopathy: a comparison of objective hemodynamic and exercise end points. J Am Coll Cardiol 1999; 34: 191-6.

(17.) Maron BJ, Nishimura RA, McKenna WJ, Rakowski H, Josephson ME, Kieval RS. Assessment of permanent dual-chamber pacing as a treatment for drug-refractory symptomatic patients with obstructive hypertrophic cardiomyopathy. A randomized, double-blind, crossover study (M-PATHY). Circulation 1999; 99: 2927-33.

(18.) Begley D, Mohiddin S, Fananapazir L. Dual chamber pacemaker therapy for mid-cavity obstructive hypertrophic cardiomyopathy. Pacing Clin Electrophysiol 2001; 24: 1639-44.

(19.) Pak PH, Maughan WL, Baughman KL, Kieval RS, Kass DA. Mechanism of acute mechanical benefit from VDD Pacing in hypertrophied heart : Similarity of responses in hypertrophic cardiomyopathy and hypertensive heart disease Circulation 1998; 98: 242-8.

Karl Duncan, Ajay Shah, Farooq Chaudhry, Mark V. Sherrid

Department of Medicine, Division of Cardiology, St. Luke's-Roosevelt Hospital Center, Columbia University, College of Physicians and Surgeons, New York, USA

Address for Correspondence: Mark Sherrid, MD 10th Avenue, 3B-30 New York, New York 10019 Tel.: 212 523 73 72 E-mail: MSherrid@chpnet.org
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Title Annotation:Case Report/Olgu Sunumu
Author:Duncan, Karl; Shah, Ajay; Chaudhry, Farooq; Sherrid, Mark V.
Publication:The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)
Geographic Code:1USA
Date:Sep 1, 2006
Words:1698
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