Two Cases of Hypertrophic Cardiomyopathy With Coronary Vasospasm

Abstract

atients with hypertrophic cardiomyopathy sometimes complain of chest pain even at rest. Electrocardiogram during chest pain may show deeper ST depression than that before chest pain. Beta blockers are the most common basic treatment for hypertrophic obstructive cardiomyopathy. Although they are also commonly administered for angina pectoris on effort, they aggravate anginal attack in cases of variant angina pectoris. 1 Thus, beta-blockers must be administered carefully with nitrates and calcium antagonists in cases of hypertrophic cardiomyopathy associated with coronary vasospasm.We report here 2 cases of hypertrophic cardiomyopathy associated with coronary vasospasm. Case Reports Case 1A 72-year-old man had complained of a precordial squeezing sensation with dyspnea at rest and on effort for 6 months. The patient's risk factors for ischemic heart disease were smoking, age and gender.Sublingual nitroglycerin was somewhat effective in relieving the squeezing sensation. The fourth heart sound and a grade 3/6 mid-systolic murmur at the third left sternal border were audible. The murmur was accentuated by Valsalva's maneuver. Electrocardiogram showed marked left ventricular hypertrophy (Fig 1). Asymmetric hypertrophy of the left ventricular wall and incomplete systolic anterior movement were found by echocardiogram. The pressure gradient between the mid-portion of the left ventricle and outflow was 20 mmHg and increased after extrasystolic contraction. Left ventriculogram showed myocardial hypertrophy of the base and the apex (Fig 2). Left ventricular myocardial biopsy showed hypertrophic myocytes. A beta-blocker (metoprolol) was administered for hypertrophic obstructive cardiomyopathy, but the precordial squeezing sensation did not decrease. On the coronary arteriogram, ergonovine induced obstruction of the right coronary artery and its right ventricular branch (Fig 3) with ST segment depression in the inferior limb leads and ST segment elevation in V1-3 leads (Fig 1). The patient complained of dyspnea during right coronary artery spasm. He was diagnosed as having hypertrophic obstructive cardiomyopathy associated with coronary vasospasm. Although metoprolol decreased the left ventricular outflow pressure gradient by the Doppler method (49 mmHg to 13 mmHg), it did not decrease the chest discomfort. The addition of isosorbide dinitrate and diltiazem completely prevented the precordial squeezing sensation in the patient. Thus, the precordial sensation seemed to be due to coronary spasm, not to myocardial ischemia associated with hypertrophic cardiomyopathy. The additional use of isosorbide dinitrate and diltiazem with a beta-blocker did not increase the left ventricular outflow pressure gradient by the Doppler method (13 mmHg to 9 mmHg). Case 2A 59-year-old man had been diagnosed as having hypertyrophic cardiomyopathy (apical type) 4 years earlier.Because he was asymptomatic, he did not consult Tenri Hospital thereafter. The patient's risk factors were diabetes, hyperlipidemia and gender.Then he started to feel a squ...

Coexistence of Familial Hypertrophic Cardiomyopathy and Vasospastic Angina Pectoris in Two Brothers

“…The administration of these two drugs was effective at reducing the chest pain without causing any cardiovascular events. Comparable effects of a calcium antagonist and nitrate on coronary vasospasm in HCM patients were observed by Konishi, et al 10) They suggested that, in addition to the coronary vasodilation, an improvement of the relaxation of hypertrophied ventricle induced by calcium antagonists may be related to this chest pain ameliorating effect.…”

“…3) Vasospastic angina pectoris (VSA) is usually isolated but occasionally shows a familial occurrence. [4][5][6][7][8] Whereas the coexistence of HCM and VSA has been demonstrated in some patients, [9][10] familial occurrence of both HCM and VSA has not been reported. We report here the cases of two brothers with familial HCM who are suffering from VSA concurrently.…”

Cardiac Magnetic Resonance Imaging Demonstrates the Coexistence of Hypertrophic Cardiomyopathy and Ischemic Cardiomyopathy due to Coronary Vasospasm

“…These reports suggested that coronary vasospasm in patients with HCM might play a potential role for the conversion of HCM to dilated cardiomyopathy, thus affecting their prognosis. Also in the clinical setting, calcium channel blockers should be the first-line therapy recommended for coronary spastic angina in HCM patients in order to induce coronary vasodilatation and to prevent the aggravation of coronary spasm by beta-blockers which are commonly administered to HCM patients to relieve chest pain [5].…”

Coronary Endothelium-Dependent and Independent Vasomotor Responses in Patients With Hypertrophic Cardiomyopathy

“…Previous studies have reported that coronary vasospasm often occurs in these patients, [1][2][3] and endothelial dysfunction has been regarded as a key factor in the pathogenesis of coronary vasospasm in patients with coronary spastic angina because intracoronary injection of acetylcholine (ACh) induces coronary vasospasm. 4,5 However, ACh is not only an endothelium-dependent vasodilator, but can also serve as a potent vasoconstrictor in human coronary arteries.…”