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

Abstract

alicov et al first reported in 1976 a subtype of hypertrophic obstructive cardiomyopathy (HOCM) with mid-ventricular obstruction, 1 which is a rare subtype of HOCM, comprising 1% of hypertrophic cardiomyopathies. 2 We present an unusual, pediatric case of mid-ventricular hypertrophic obstructive cardiomyopathy (MVHOCM). Case ReportAt the age of 10 years, the patient first presented to hospital with severe chest pain, which occurred early in the morning and disappeared spontaneously with bed rest. The family history was not significant. Based on the echocardiographic findings, he was diagnosed as having hypertrophic cardiomyopathy (HCM) and was prescribed 30 mg of propranolol daily. Four months later, he was admitted for further evaluation: blood pressure was 118/78 mmHg, pulse rate 80 beats/min and respiratory rate 20 breaths/min; grade 3/6 harsh systolic murmur at the lower left sternal border. Electrocardiogram showed a mean frontal QRS axis of -60°and a deep S wave in V1; chest X-ray showed neither cardiomegaly nor pulmonary venous congestion. Two-dimensional echocardiography showed asymmetric septal hypertrophy, most marked at the mid-ventricular level, and papillary muscles coming into contact at the mid-ventricular level during systole. The thickness of the interventricular septum and of the left ventricular posterior wall was 15 and 10 mm, respectively. There was no evidence of systolic anterior motion of the mitral valve. Color Doppler echocardiography showed formation of a stenotic jet into the body of the left ventricle, and a peak systolic gradient of 100 mmHg was recorded by continuous-wave Doppler echocardiography. MVHOCM was suspected, and (Fig 1). The coronary angiogram was normal. A right ventricular endomyocardial biopsy specimen showed hypertrophic cardiomyocytes and augmentation of interstitial collagen fiber. Plasma -galactosidase activity was 13.8 nmol·h -1 ·ml -1 (normal, 4.8-17.6). The patient did not have the characteristic skin manifestation of Fabry's disease. An exercise stress 99m Tc tetrofosmin myocardial scintigram disclosed no obvious perfusion defect, but the first 123 I--methyl iodophenyl pentadecanoic acid (BMIPP) myocardial scintigram (Fig 2A, at the age of 11 years and 11 months) showed a reduced uptake by the septal and inferior walls. He was put on restricted exercise with 30 mg/day of propranolol and continued for approximately 2 years without complaint.The chest pain and dyspnea on exertion re-appeared at the age of 12 years and 3 months. He sometimes became pale, which was considered to be indicative of presyncope. The second 123 I-BMIPP myocardial scintigram (Fig 2B, 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 exer...

A pediatric case of hypertrophic cardiomyopathy with mid-ventricular obstruction incidentally detected by electrocardiography

“…In contrast, in children, only the two above-mentioned reports are available. One report noted that disopyramide was effective in a 14-year-old boy who was refractory to propranolol [3], and the other report stated that cibenzoline was effective in a 18-year-old male, who was refractory to propranolol [4]. We are presently discussing the most appropriate therapy for the current patient.…”

“…In contrast, a pediatric case with MVO-HCM was not reported until 2002, by Teraguchi et al [3] To our knowledge, to date, only one further case report, of an 18-year-old male HCM patient with left mid-ventricular and subpulmonic obstructions, has been published [4]. These few reports on pediatric MVO-HCM reveal the following: (1) myocardial hypertrophy in patients with HCM progresses in association with accelerated systemic growth and maturation during adolescence, and is complete by adulthood [5].…”

Effectiveness of Alcohol Septal Ablation Versus Transaortic Extended Myectomy in Hypertrophic Cardiomyopathy with Midventricular Obstruction

“…Second, no patient in this study was treated with disopyramide because this medication is not available in China. Some patients may avoid septal reduction therapy if disopyramide is available . Finally, although this study enrolled the largest series of patients with MVO who underwent ASA till now, the sample size is still limited, which is primarily because MVO is a rare condition.…”

“…Some patients may avoid septal reduction therapy if disopyramide is available. 27,28 Finally, although this study enrolled the largest series of patients with MVO who underwent ASA till now, the sample size is still limited, which is primarily because MVO is a rare condition. Further studies are needed to validate the outcomes of patients with MVO who underwent ASA.…”

Clinical Value of Iodine-123 Beta-Methyliodophenyl Pentadecanoic Acid (BMIPP) Myocardial Single Photon Emission Computed Tomography for Predicting Cardiac Death Among Patients With Chronic Heart Failure

“…8 It is important to note that there may be differential alterations in myocardial metabolism in response to heart failure that are dependent on the particular stage of the disease. 9 Abnormalities of BMIPP SPECT have been shown to be a sensitive marker of severe myocardial damage in ischemic heart disease, idiopathic dilated cardiomyopathy, hypertrophic cardiomyopathy and valvular heart disease, [10][11][12][13][14][15][16][17][18][19][20][21][22][23] and it is possible that BMIPP SPECT may reveal the metabolic switch in the end-stage failing heart and so be useful in predicting cardiac death. On this basis, we investigated whether 123 I BMIPP SPECT could be used clinically for risk stratification of patients with chronic heart failure.…”