Echocardiographic Manifestations in Patients with Cardiac Sarcoidosis

BackgroundCardiac sarcoidosis is a life-threatening disease with protean clinical manifestations, including congestive heart failure (CHF), conduction disturbance, ventricular arrhythmia and sudden death. Nonetheless, it is difficult to diagnose cardiac sarcoidosis in the clinical setting. Some echocardiographic findings of cardiac sarcoidosis associated with other diagnostic tools (201thallium scintigraphy, 67gallium citrate scan, serum markers and others) may be helpful upon early suspicion and diagnosis of cardiac sarcoidosis.Materials and MethodsFifty-two patients (36 female) with cardiac sarcoidosis, with a mean age of 48 ± 14 years (range, 21–70 yr), underwent a series of echocardiographic follow-up (mean, 88 ± 48 mo) examinations, and important echocardiographic parameters and findings were recorded.ResultsThere were left ventricular (LV) regional wall motion abnormalities (RWMAs) noted in 40 (localized in 16, multiple in 24), dilatation of the LV with impaired LV contractility in 28, thinning of the basal interventricular septum (IVS) in 27, thinning of LV free wall in 18, apical aneurysm in 12, apical thrombus in two, mimicking hypertrophic cardiomyopathy (HCM) in two, pericardial effusion (PE) in two (with cardiac tamponade in one), and LV wall thinning and aneurysm formation after steroid therapy for cardiac sarcoidosis in two of 43 patients.ConclusionThinning of the basal IVS or LV free wall is a specific echocardiographic finding of cardiac sarcoidosis. Other echocardiographic findings of cardiac sarcoidosis may mimic coronary artery disease (LV RWMA or apical aneurysm), CHF, or HCM. PE and thinning of the LV wall after steroid therapy were also noted in rare situations

Characteristic cardiovascular manifestation in homozygous and heterozygous familial hypercholesterolemia

Background The aortic valve dysfunction of patients with homozygous familial hypercholesterolemia (FH) suggests that hypercholesterolemia affects not only coronary arteries, but also the aortic valve. We studied the aortic root of patients with homozygous FH and those of heterozygous FH to characterize the premature atherosclerotic lesions, using histopathological specimens. Methods and results The aortic roots of 10 homozygous FH patients, aged 9 to 58 years, were studied by cardiac catheterization with several angiographies. The aortic root of 39 heterozygous FH patients under age 60 were also examined for aortic and mitral valvular functions by color Doppler echocardiography, and 30 normocholesterolemic patients with coronary artery disease were examined as controls. In addition, in 22 FH and 20 control subjects, the internal diameter of the aortic annulus and the aortic ridge in cardiac cycles were measured. Of the 10 FH homozygotes, 8 patients had aortic regurgitation demonstrated by aortography; three of them showed significant transvalvular pressure gradients. Stenotic changes of coronary ostia were observed in 8 of the 10 homozygotes with moderate coronary atherosclerosis. Of the 39 FH heterozygotes, ten patients had aortic regurgitation shown by Doppler echocardiography, as did only one of the 30 control subjects (P<0.05). The average diameter and distensibility of the ascending aorta were significantly reduced in the heterozygotes compared to the control subjects. The surgically resected cusp specimens of aortic valves obtained from one homozygous and one heterozygous patient showed significant thickening of the cusp with foam cell infiltration. Conclusions Premature atherosclerosis in FH had a characteristic distribution, affecting the aortic root dominantly. The involvement of the aortic valve indicating “hypercholesterolemic valvulopathy” was a peculiar feature of FH, especially its homozygous form, but was reminiscent of ubiquitous processes due to hypercholesterolemia

Long-term follow-up of atrial contraction after the maze procedure in patients with mitral valve disease

OBJECTIVES: We sought to determine the effectiveness of the maze procedure for maintaining sinus rhythm and atrial contraction for a long period in patients with mitral valve disease. BACKGROUND: Although the maze procedure for atrial fibrillation (AF) has been effective in restoring sinus rhythm in patients with mitral valve disease, the long-term results of this procedure have not been determined. METHODS: We echocardiographically studied 94 consecutive patients with mitral valve disease before, as well as early (3.1 ± 3.3 months) and late (2.2 ± 0.9 years) after, the maze procedure. Peak velocity and the time-velocity integral of the left ventricular (LV) diastolic filling wave during atrial contraction (A wave), as well as the atrial filling fraction (calculated as the ratio of the time-velocity integral of the A wave to total diastolic filling), were obtained from transmitral flow recordings. Peak A wave velocity ≥ 10 cm/s was considered to indicate echocardiographic evidence of effective atrial contraction. RESULTS: Regular rhythm with P waves was restored in 70 patients (74%) in the early stage and in 59 patients (63%, p = 0.09) in the late stage after the maze procedure. Forty-seven patients (50%) in the early stage and 36 patients (38%, p = 0.14) in the late stage showed effective atrial contraction by Doppler echocardiography. Left atrial (LA) and LV end-diastolic diameters significantly decreased after the procedure (from 59 ± 13 to 48 ± 7 mm, p < 0.01; and from 54 ± 9 to 47 ± 5 mm, p < 0.01, respectively) and did not show significant changes during the follow-up period. Once atrial contraction was resumed, its degree did not change between the early and late stages after the maze procedure (17 ± 6% vs. 17 ± 6% for atrial filling fraction). CONCLUSIONS: Sinus rhythm and atrial contraction recovered early after the maze procedure in most patients and were maintained for more than two years. Once active atrial contraction was resumed, the degree of contraction did not change thereafter. These results demonstrate that the maze procedure is effective for a long period in patients with mitral valve disease