CLINICAL STUDY: CARDIOMYOPATHY
Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction
Rolf Jenni, MD, MSEE*,
Christophe A. Wyss, MD ,
Erwin N. Oechslin, MD* and
Philipp A. Kaufmann, MD,*
* Department of Echocardiography, Cardiovascular Center, University Hospital, Zurich, Switzerland
Department of Nuclear Cardiology, Cardiovascular Center, University Hospital, Zurich, Switzerland
Manuscript received July 18, 2001;
revised manuscript received October 15, 2001,
accepted November 1, 2001.
* Reprint requests and correspondence: Dr. Philipp Kaufmann, Head of Nuclear Cardiology, Cardiovascular Center C NUK 32, University Hospital, Ramistr. 100, CH-8091 Zurich, Switzerland.
Philipp.Kaufmann{at}dmr.usz.ch
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 13N-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  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.
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Abbreviations and Acronyms
| | NYHA | | CFR | | coronary flow reserve | | IVNC | | isolated ventricular noncompaction | | LV | | left ventricular or ventricle | | MBF | | myocardial blood flow | | PET | | positron emission tomography | | ROI | | region of interest | | WHO | | World Health Organization |
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