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J Am Coll Cardiol, 2002; 39:450-454
© 2002 by the American College of Cardiology Foundation
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CLINICAL STUDY: CARDIOMYOPATHY

Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction

Rolf Jenni, MD, MSEE*, Christophe A. Wyss, MD{dagger}, Erwin N. Oechslin, MD* and Philipp A. Kaufmann, MD{dagger},*

* Department of Echocardiography, Cardiovascular Center, University Hospital, Zurich, Switzerland
{dagger} 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.

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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