Acquired noncompaction?
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Cardiomyopathies↗
Biomedical subjects
Erwin N Oechslin
Publications and source records attributed to Erwin N Oechslin.
Validation of noncompaction criteria in dilated cardiomyopathy, and valvular and hypertensive heart disease.
BACKGROUND: Echocardiographic characteristics typical of isolated left ventricular noncompaction (IVNC) have been well defined. The aim of this study was to validate diagnostic criteria of IVNC in valvular or hypertensive heart disease (HHD) or dilated cardiomyopathy. METHODS: We conducted a retrospective analysis of records and blind review of videotapes of all 19 patients with IVNC seen within 7 years in comparison with randomly selected patients from the same study period with dilated cardiomyopathy (31 patients), HHD (22 patients), and chronic severe valvular heart disease: mitral regurgitation (22 patients); aortic regurgitation (20); and aortic stenosis with bicuspid (22) or tricuspid (22) valves. RESULTS: Clinical characteristics and electrocardiographic findings did not differ between IVNC and other diseases. In IVNC, all patients had noncompacted (NC) segments with a 2-layered structure and wall thickening, and in most patients perfused recesses (95%) or hypokinetic segments (89%) were present. Both hypertrabeculation or presence of a meshwork were specific for IVNC, but the sensitivity for IVNC was only 11% for hypertrabeculation, respectively, 68% for meshwork. In dilated cardiomyopathy, perfused recesses (48%) and a 2-layered structure (26%) were seen but without wall thickening of these segments; all NC criteria including wall thickening were fulfilled in one patient (3%) only. In valvular heart disease or HHD, perfused recesses and a 2-layered myocardium were rare: two patients (5%) with aortic stenosis and one patient with HHD (5%) had NC. Although in IVNC wall thickening was confined to the 2-layered myocardial segments, it was diffuse in other diseases. CONCLUSIONS: Although some NC criteria are occasionally found in other heart disease, the combination of all criteria is very specific. All criteria of NC are rarely met in other disease than IVNC (< or = 5%).
Adult↗
Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction.
OBJECTIVES: We sought to analyze whether a microcirculatory dysfunction might be associated with isolated ventricular noncompaction (IVNC). BACKGROUND: In IVNC, which is a cardiomyopathy thus far "unclassified" by the World Health Organization, heart failure and sudden cardiac death are common findings, but the pathophysiologic mechanisms are unknown. METHODS: In 12 patients with IVNC and 14 control subjects, quantitative evaluation of regional myocardial perfusion (myocardial blood flow [MBF]) and coronary flow reserve (CFR, hyperemic/baseline MBF) was performed using positron emission tomography and (13)N-ammonia. The left ventricular myocardium was divided into nine segments, and the two-dimensional echocardiogram in each patient with IVNC was compared with CFR in each segment. Noncompaction was defined as a two-layered structure with excessive trabeculation. RESULTS: The CFR in control subjects averaged 4.2+/-0.9, providing a cut-off value > or =2.5, but it was 2.1+/-0.8 in patients with IVNC. A perfusion scan defect was found in 14 of 24 segments with noncompaction, although no defect was found in 76 of 84 normal segments (overall agreement 83%, p < 0.0001 by the chi-square test). In 16 of 21 segments with noncompaction, a decreased CFR was found; but a decreased CFR was also found in 36 of 60 segments without noncompaction (p = NS). In 45 of the 57 segments with wall motion abnormalities, CFR was decreased, but it was preserved in 17 of the 24 segments with normal wall motion (agreement 77%, p < 0.0001). CONCLUSIONS: In patients with IVNC, a decreased CFR is not confined to noncompacted segments, but extends to most segments with wall motion abnormalities. Thus, coronary microcirculatory dysfunction is associated with IVNC.
Adult↗