Abnormal sympathetic innervation of the heart in a patient with Emery-Dreifuss muscular dystrophy.

A 33-year-old man was admitted for general malaise and vomiting. An electrocardiogram showed a complete atrioventricular block and an echocardiogram showed right atrial dilatation and normal wall motion of left ventricle (LV). Gene analysis showed nonsense mutation in the STA gene, which codes for emerin, and Emery-Dreifuss muscular dystrophy was diagnosed. An endomyocardial biopsy of right ventricle showed mild hypertrophy of myocytes. Myocardial scintigraphic studies with Tc-99m methoxyisobutylisonitrile (MIBI) and I-123-betamethyl-p-iodophenylpentadecanoic acid (BMIPP) scintigrams showed no abnormalities. In contrast, I-123 metaiodobenzylguanidine (MIBG) scintigrams showed a diffuse and severe decrease in accumulation of MIBG in the heart. Six months later, his LV wall motion on echocardiograms developed diffuse hypokinesis. These results suggest that the abnormality on I-123 MIBG myocardial scintigrams may predict LV dysfunction in Emery-Dreifuss muscular dystrophy.

The incidence of stent-edge spasm after stent implantation in patients with or without vasospastic angina pectoris.

Although several investigations have reported that stent implantation is an option for the treatment of vasospastic angina (VSA) that is resistant to medical treatment, we are concerned about the occurrence of new stent-edge spasms after stenting. The purpose of this study was to determine the incidence of new stent-edge spasms after stenting. Twenty-seven patients with VSA and 23 patients without VSA were enrolled. About 6 months after stent implantaion, a spasm provocation test was performed by intracoronary infusion of acetylcholine or ergonovine in 26 patients with VSA and all patients without VSA, and the induced stent-edge spasms were classified as either moderate (stent-edge spasm > 75% and < 95% reduction in coronary artery diameter) or severe (stent-edge spasm > 95% reduction in coronary artery diameter). In one patient with VSA, stent-edge spasm and acute thrombosis occurred several hours after stent implantation. The remaining 26 patients with VSA had no complications during or after stent implantation. However, during the chronic phase, severe stent-edge spasm was provoked in 5 patients with VSA (19.2%) and in 2 patients without VSA (8.7%). Moderate stent-edge spasm was provoked in 5 patients with VSA (19.2%) and 5 patients without VSA (21.7%). The results suggest new onset stent-edge spasm in patients either with or without VSA should not be neglected.

Risk evaluation of coronary heart disease and cerebrovascular disease by the Japan Atherosclerosis Society Guidelines 2002 using the cohort of the Holicos-PAT study.

Our purpose in this study was to evaluate the new JAS guidelines as a risk assessment tool in Japanese patients with hypercholesterolemia, using the cohort of the Holicos-PAT study. The Holicos-PAT study was designed as a prospective observational study. 2039 patients were followed with or without pravastatin for 5 years. We assessed coronary heart disease (CHD) and cerebrovascular disease (CVD) risks by the patient categories described in the JAS guidelines. In the Holicos-PAT study, the primary endpoints were CHD, and the secondary endpoints were CVD and total mortality. CHD event includes onset and worsening of angina pectoris, performing CABG or PTCA, non-fatal and fatal myocardial infarction, and death from CHD including heart death and sudden death. CVD events are onset or recurrence of cerebral infarction, onset of cerebral hemorrhage, and death from cerebral infarction or hemorrhage. The event rates were calculated by the person-years method, and the differences in event rates between category groups were analyzed by chi-square test. The event rates of CHD in Category A, B1, B2, B3, B4 and C, were 1.1, 4.0, 2.8, 5.7, 18.2 and 38.8 per 1,000 person-years. The rates of CHD events in the higher risk category groups, Category B4 group (p = 0.004 in whole patients) and C group (p < 0.001 in whole patients), were significantly higher than that in the combined category groups A + B1 + B2. The event rates of CVD in Category A, B1, B2, B3, B4 and C, were 2.1, 1.8, 1.8, 0.6, 10.8 and 6.4 per 1,000 person-years. The event rates of CHD in men were significantly higher than those in women, in categories B4 (p < 0.001) and C (p < 0.001). From these results, each category classified by accumulation of risk factors, showed increasing event rates of CHD and CVD. The categories in the JAS guidelines are useful to assess CHD and CVD risk in Japanese patients with hypercholesterolemia. However, the risk evaluation by the JAS guideline categories may underestimate the risk in men and overestimate it in women.

Failure of follow-up gallium single-photon emission computed tomography and fluorine-18-fluorodeoxyglucose positron emission tomography to predict the deterioration of a patient with cardiac sarcoidosis.

Although gallium-67-citrate (67Ga) scanning and single-photon emission computed tomography (SPECT) are useful in the assessment of disease activity in cardiac sarcoidosis, a patient with cardiac sarcoidosis in whom SPECT imaging with 67Ga failed to predict the deterioration in the clinical course is presented. A 53-year-old woman diagnosed with cardiac sarcidosis had 67Ga scanning and 67Ga SPECT, both of which showed abnormal high uptake. After treatment with corticosteroid, there was an apparent improvement in the 67Ga SPECT findings, and the dose of the corticosteroid was reduced. Subsequently, the disease activity of the cardiac sarcoidosis was thought to be well controlled, because abnormal uptake was not found on repeat 67Ga SPECT. However, 4 years after initial diagnosis, thinning at the basal ventricular septal wall and complete atrioventricular block were noted. Despite repeating the evaluation with 67Ga SPECT and additional fluorine-18-fluorodeoxyglucose positron emission tomography (18FDG PET) after discovering this progression, neither of these examinations showed any abnormality. Unfortunately, in this patient, the disease activity of cardiac sarcoidosis was underestimated by the diagnostic imaging modalities.

Reduction of hemolysis without reoperation following mitral valve repair.

Hemolytic anemia following mitral valve repair and annular ring placement is uncommon compared with mitral valve replacement. A 60-year-old man, who had undergone mitral valve repair with a Duran ring, developed hemolytic anemia and needed a blood transfusion. Transesophageal echocardiography revealed a paravalvular mitral regurgitation jet colliding with the Duran ring. Most cases of severe hemolysis after mitral valve repair have undergone reoperation, but in the present case study, the hemolysis after mitral valve repair reduced without the need for reoperation, although the paravalvular mitral regurgitation jet continued to collide with the Duran ring.

A case of hypersensitivity pneumonitis caused by Humicola fuscoatra.

A 51-year-old housewife with hypersensitivity pneumonitis caused by Humicola fuscoatra is reported. The diagnosis was made by an inhalation challenge with H. fuscoatra antigen. She was admitted for diagnosis and treatment of a fever and productive cough. Auscultation of her lungs revealed inspiratory fine crackles. Her chest CT showed diffuse miliary nodules in a centri-lobular distribution with patchy ground glass opacities. Findings of transbronchial lung biopsy and BAL fluid were compatible with a hypersensitivity pneumonitis. Her symptoms worsened on returning home, which suggested the existence of some aetiological agent in the subject's house. H. fuscoatra, Penicillium decumbens and Aspergillus versicolor were isolated from a number of rooms. High titres of serum anti H. fuscoatra, P. decumbens and A. versicolor were detected. Inhalation challenge tests with both P. decumbens and A. versicolor antigen were negative, in contrast to that with H. fuscoatra which was positive. Based on these results, we advised the patient to cleanse her entire house. Since cleaning, her symptoms have not worsened upon returning home. This is the first report of hypersensitivity pneumonitis caused by H. fuscoatra antigen.

Self-vasodilating ability at the spastic site of patients with vasospastic angina: estimation by acetylcholine delayed phase.

Deficient nitric oxide (NO) release is thought to be the principal mechanism of coronary spasm, however, the precise mechanisms are unknown. Although acetylcholine (ACh) is used for provocation of coronary spasm, ACh is also used for the augmentation of blood flow and flow-mediated vasodilation is induced. We estimated the self-vasodilating ability (endothelial function) at the spastic site of coronary arteries in patients with vasospastic angina (VSA) during the provocation test of coronary spasm by ACh. This study included 93 patients with VSA and 77 patients with atypical chest pain (ACP). Intracoronary injection of ACh (20, 50, and 100 microg) was performed over 30 seconds and the coronary artery diameter of the spastic site was measured 3 to 4 minutes after ACh injection (delayed phase). The ability of dilation (AOD) was calculated as: ([diameter of delayed phase-baseline diameter]/[diameter after isosorbide dinitrate-baseline diameter]) x 100 (%). No significant difference was noted between the AOD in patients with ACP and VSA (28 +/- 36 vs 15 +/- 60%, respectively). The AOD values of 49% of patients with VSA were greater than the mean value of AOD of patients with ACP. At least almost half of the patients with VSA may have preserved self-vasodilating ability at the spastic site, and an abnormality other than endothelial dysfunction is involved in the mechanism of coronary spasm in these patients.