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J Am Coll Cardiol, 2000; 36:493-500
© 2000 by the American College of Cardiology Foundation
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CLINICAL STUDIES

Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis

Erwin N. Oechslin, MDa, Christine H. Attenhofer Jost, MDa, Jerry R. Rojas, MDa, Philipp A. Kaufmann, MDa and Rolf Jenni, MD, MSEEa

a Division of Echocardiography, University Hospital Zurich, Zurich, Switzerland

Manuscript received October 28, 1999; revised manuscript received February 11, 2000, accepted March 30, 2000.

Reprint requests and correspondence: Dr. Rolf Jenni, Division of Cardiology, University Hospital Zurich, Raemistrasse 100, CH-8091 Zurich, Switzerland
karjer{at}usz.unizh.ch

OBJECTIVES

We sought to describe characteristics and outcome in adults with isolated ventricular noncompaction (IVNC).

BACKGROUND

Isolated ventricular noncompaction is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Knowledge regarding diagnosis, morbidity and prognosis is limited.

METHODS

Echocardiographic criteria for IVNC include—in the absence of significant heart lesions—segmental thickening of the left ventricular myocardial wall consisting of two layers: a thin, compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep recesses. Thirty-four adults (age ≥16 years, 25 men) fulfilled the diagnostic criteria and were followed prospectively.

RESULTS

At diagnosis, mean age was 42 ± 17 years, and 12 patients (35%) were in New York Heart Association class III/IV. Left ventricular end-diastolic diameter was 65 ± 12 mm and ejection fraction 33 ± 13%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in >80% of patients. Follow-up was 44 ± 40 months. Major complications were heart failure in 18 patients (53%), thromboembolic events in 8 patients (24%) and ventricular tachycardias in 14 patients (41%). There were 12 deaths: sudden in six, end-stage heart failure in four and other causes in two patients. Four patients underwent heart transplantation. Automated cardioverter/defibrillators were implanted in four patients.

CONCLUSIONS

Diagnosis of IVNC by echocardiography using strict criteria is feasible. Its mortality and morbidity are high, including heart failure, thrombo-embolic events and ventricular arrhythmias. Risk stratification includes heart failure therapy, oral anticoagulation, heart transplantation and implantation of an automated defibrillator/cardioverter. As IVNC is a distinct entity, its classification as a specific cardiomyopathy seems to be more appropriate.

Abbreviations and Acronyms
  ECG = electrocardiogram
  IVNC = isolated ventricular noncompaction
  LV = left ventricle or left ventricular
  NYHA = New York Heart Association
  N/C = noncompacted/compacted




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