Response to isoproterenol as a prognostic indicator of evolution from hypertrophic cardiomyopathy to a phase resembling dilated cardiomyopathy.

OBJECTIVES: We sought to assess whether isoproterenol stress echocardiography could detect in advance in which patients hypertrophic cardiomyopathy would progress to a phase resembling dilated cardiomyopathy. BACKGROUND: In a few patients, hypertrophic cardiomyopathy has been reported to progress to a phase characterized by systolic dysfunction and left ventricular dilation, resembling dilated cardiomyopathy. METHODS: Echocardiograms were recorded before and immediately after intravenous infusion of isoproterenol (0.02 microgram/kg body weight per min) for 5 min in 18 patients with typical hypertrophic cardiomyopathy (i.e., hypertrophied, hyperdynamic and nondilated) to determine the difference in fractional shortening. The patients were categorized into those with a good response (difference in fractional shortening > 7%, 14 patients) and those with a poor response (difference < or = 7%, 4 patients). Changes in left ventricular end-diastolic diameter and fractional shortening were evaluated by using serial echocardiography over an average follow-up period of 5.4 years. RESULTS: In the good response group, neither end-diastolic diameter nor fractional shortening changed significantly during the follow-up period. In the poor response group, end-diastolic diameter significantly increased from a mean +/- SD of 41 +/- 5 to 53 +/- 5 mm (p < 0.05), and fractional shortening significantly decreased from 40 +/- 12% to 29 +/- 10% (p < 0.05). All patients in the poor response group showed a substantial decrease (> or = 5%) in fractional shortening and an increase (> or = 5 mm) in end-diastolic diameter. One patient developed congestive heart failure due to systolic dysfunction during the observation period. CONCLUSIONS: The present study confirmed that impaired responses to isoproterenol infusion are related to future deterioration of left ventricular performance in patients with typical hypertrophic cardiomyopathy.

Pathophysiologic significance of left ventricular hypertrophy in dilated cardiomyopathy.

BACKGROUND AND HYPOTHESIS: Patients with dilated cardiomyopathy (DCM) with left ventricular hypertrophy (LVH) have been found to have a better prognosis than patients without LVH. However, the pathophysiologic mechanism for that has not been investigated. We sought to clarify the pathophysiologic significance of LVH in DCM. METHODS: We performed isoproterenol infusion echocardiography (0.02 micrograms/kg/min) in 17 patients with DCM, and measured plasma epinephrine and norepinephrine levels at rest and at the end of ergometer exercise in 14 of the 17 patients. Patients were classified into groups according to the presence (9 patients) (LVH+) or absence (8 patients) (LVH-) of LVH. Left ventricular hypertrophy was defined as an inter-ventricular thickness or posterior wall thickness > or = 13 mm. RESULTS: Although there was no significant difference between groups in fractional shortening at rest during isoproterenol infusion, fractional shortening was significantly higher in the LVH(+) group than in the LVH(-) group (29 +/- 9 vs. 17 +/- 8%; p < 0.025). Although there was no significant difference in plasma norepinephrine level, it was significantly lower in the LVH(+) group than in the LVH(-) group (233 +/- 169 vs. 519 +/- 258 pg/ml; p < 0.05) at the end point of the exercise. CONCLUSION: Systolic reserve, represented by the response to isoproterenol, is greater in patients with DCM with LVH than in those without LVH, and a lower plasma level of norepinephrine is needed to activate the myocardium during exercise in patients with DCM with LVH. This pathophysiologic characteristic could be one of the mechanisms which explain a better prognosis in patients with DCM with LVH.

Hypertrophic cranial pachymeningitis with spinal epidural granulomatous lesion.

A 67-year-old woman with a one-year history of tinnitus and headache had multiple cranial nerve palsies of V, VII, VIII, IX, X, XI and spastic paraparesis. She also had a secretory otitis media. Gd-DTPA-enhanced magnetic resonance imaging (MRI) revealed hypertrophy of the dura of the posterior fossa and spinal epidural mass which extended from C7 to T10. A biopsy of the epidural mass showed chronic granulomatous change. These lesions were completely cured with administration of antibiotics. We believe this case of double-lesion of hypertrophic cranial pachymeningitis and spinal epidural granulomatous lesion originated from a bacterial infection secondary to the secretory otitis media.

Right ventricular cardiomyopathy showing right bundle branch block and right precordial ST segment elevation.

A 73-year-old man who had a family history of sudden death, experienced syncope. His electrocardiogram (ECG) presented right bundle branch block and right precordial ST segment elevation which are findings identical with those in Brugada syndrome. The cardiac MRI showed right ventricular mild dilatation, and endomyocardial biopsy revealed fatty replacement of myocardial fibers. Though no ventricular tachyarrhythmias were induced during an electrophysiologic test, the effects on ECG of antiarrhythmic agents and autonomic modulations were similar to those in Brugada syndrome. This case may suggest the relationship between Brugada syndrome and right ventricular cardiomyopathy.