Article, Cardiology

A special shape of the heart in an old woman

Case Report

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American Journal of Emergency Medicine

journal homepage: www. elsevier. com/ locate/ajem

American Journal of Emergency Medicine 33 (2015) 737.e3-737.e5

A special shape of the heart in an old woman?,??,?,??

Abstract

Yamaguchi syndrome is a rare form of inherited cardiomyopathy, which may mimic acute coronary syndrome. A 73-year-old woman was admitted for exertional angina. Electrocardiogram showed flat- tened T waves in the limb leads, V1 and V2 and inverted T waves in V3 to V6. Echocardiography showed a hypokinetic apex and increased left ventricular wall thickness. Cardiac multislice computed tomography confirmed a rare disease: apical Hypertrophic cardiomyopathy. Despite its rare incidence, it must be considered in the differential diagnosis of chest pain.

Hypertrophic cardiomyopathy (HCM) is the most common cause of cardiac death in children and adolescents [1]. Typically, the hypertrophy is asymmetric and involves the septum. Apical cardiac hypertrophy or Yamaguchi syndrome is a rare form of inherited HCM, which may mimic acute coronary syndrome.

A 73-year-old woman presented with symptoms of exertional angi- na and palpitations. She had a history of documented Paroxysmal atrial fibrillation and sequelae of anterior myocardial infarction, arterial hy- pertension, and multiple allergies, including iodine.

Clinical examination showed a blood pressure of 150/60 mm Hg, a regular heart rate of 65 beats per minute, a systolic murmur on the aortic valve auscultation, no jugular venous distension, and no carotid bruits. The electrocardiogram (ECG) showed flattened T waves in the limb leads, V1 and V2 and inverted T waves in leads V3 to V6 (Fig. 1). The car- diac isoenzymes were normal. The chest X-ray indicated mild cardiomegaly. On transthoracic echocardiography, a hypokinetic apex of the Left ventricle and an LV wall thickness of 18.5 mm were diag- nosed, with a preserved LV ejection fraction of 55%. There was no evi- dence of middle or basal obstructive hypertrophy. An effort testing could not be performed due to severe arthrosis. Because of multiple aller-

gies, a coronary angiography could not be performed.

The cardiac multislice computed tomography (MSCT) (before which a desensitization therapy was applied) did not reveal significant coro- nary artery stenosis; the calcium score was 10 (with an equivalent mass of 2.18 mg/cm3). Instead, a markedly increased thickness of the anterior papillary muscle and of the apical segment of the LV (up to 18.3 mm in diastole) was noticed, determining LV cavity apical collapse

? No conflicts of interest.

?? Acknowledgments: None.

? No financial disclosure.

?? All the authors agreed with the content of the manuscript.

during systole–the ace of spades-like cavity–confirming the apical hy- pertrophy diagnosis (Fig. 2 A-D).

The apical nonobstructive type of HCM is an uncommon type of HCM in the European population. Yamaguchi et al [2] in 1979 first described the syndrome and its ventriculographic features. Although patients with apical HCM may develop symptoms and signs that appear in other forms of HCM, its course is usually benign (with a 74% probabil- ity of survival at 15 years without morbid events) [3] in the absence of certain risk factors and of negative prognostic factors (family history of death by apical HCM, young age at diagnosis, males, the appearance of syncope, or effort-induced hypotension).

There are studies on a long period that tried to determine the outcome of these patients. One study determined the overall survival at 15 years being 95% and identified 3 predictors of cardiovascular morbidity: age at presentation younger than 41 years, left atrial enlargement, and New York Heart Association class II or more at baseline. The annual cardiovas- cular mortality in these patients was of 0.1% [3]. Another study found that left ventricular outflow obstruction, atrial fibrillation, and female sex were predictors of mortality [4]. The patients with apical HCM are at risk for developing arrythmias such as atrial fibrillation (12%), paroxysmal atrial tachycardia, ventricular arrythmias, or myocardial infarction with a secondary apical LV aneurysm (10%) that might determine the disappear- ance of the giant T waves [3,5].

The diagnostic criteria for apical HCM were asymmetric LV hypertrophy–predominantly at the apex of the ventricle, LV wall thick- ness of 15 mm or more during diastole (18.5 mm in our patient), apical to posterior wall thickness ratio of 1.5 or more determined by 2- dimensional echocardiography, or cardiac magnetic resonance imag- ing/MSCT (calculated by cardiac MSCT in our patient, 1.66).

There are studies that sustain the importance of cardiac magnetic resonance imaging as being the most important in differentiating between hypertensive LV hypertrophy and those determined by a sarcomeric mutation (as in apical HCM) [6,7]. However, a more recent method of evaluating cardiomyopathy is cardiac MSCT; when trying to rule out coronary artery disease, this method has the benefit of evaluat- ing both morphology of the LV wall, the shape of the LV cavity, and the morphology of the coronary arteries [8]. Ruling out coronary artery dis- ease is very important in these patients because the symptoms usually occur in middle aged or elderly patients who might as well have this associated pathology. Apical HCM has a good long-term prognosis, with only a small percentage of patients being affected by the complica- tions that usually appear in other forms of HCM.

Even if it appears to show a predilection for middle-aged men and has a good prognosis, apical HCM must be considered in elderly patients presenting with angina pectoris and a modified ECG that suggests HCM, especially when giant Negative T waves appear.

0735-6757/(C) 2014

737.e4 C.C. Diaconu et al. / American Journal of Emergency Medicine 33 (2015) 737.e3737.e5

Fig. 1. The ECG showing left ventricular hypertrophy with strain pattern in precordial limbs.

Fig. 2. Cardiac MSCT. A, Aspect of the base of the LV–normal wall thickness. B, Apex of the LV in diastole (notice the small LV cavity due to hypertrophy). C, Left ventricle cavity spade-like shape. D, Aspect of the apex of the LV in diastole–wall thickness of 18.3 mm.

C.C. Diaconu et al. / American Journal of Emergency Medicine 33 (2015) 737.e3737.e5 737.e5

Camelia C. Diaconu, PhD Internal Medicine Clinic, University of Medicine and Pharmacy Carol Davila, clinical emergency Hospital of Bucharest, Romania Corresponding author. UMF Carol Davila, Internal Medicine Clinic Clinical Emergency Hospital of Bucharest Romania

8 Calea Floreasca, 1st district, Bucharest, 014461

Tel.: +0040 726 377 300

E-mail-address: [email protected]

Nicoleta Dumitru, MD

Internal Medicine Clinic, Clinical Emergency Hospital of Bucharest, Romania

Ana Fruntelata, PhD

Cardiology Clinic, Monza Hospital, Bucharest, Romania

http://dx.doi.org/10.1016/j.ajem.2014.10.049

References

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  2. Yamaguchi H, Ishimura T, Nishiyama S, Nagasaki F, Nakanishi S, Takatsu F, et al. Hy- pertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hy- pertrophy), ventriculographic and echocardiographic features in 30 patients. Am J Cardiol 1979;44:401-12.
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  8. Eun JC, Sang IIC, Kwang NJ, Hyon JK, Young JK, Byoung WC, et al. Hypertrophic cardio- myopathy: assessment with MR imaging and multidetector CT. RadioGraphics 2010; 30:1309-28.

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