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Health Dictionary

Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Noncompaction cardiomyopathy echocardiography or ultrasound

Introduction

Overview

Overview

On echocardiography there is ventricular hypertrophy with deep recesses which tend to be located apically [1]. Unless there is an awareness of the syndrome, the diagnosis is often missed in up to 89% of children [2]. Likewise, the time from onset of symptoms to the diagnosis was more than 3 years in adult patients[3].

Conditions that Noncompaction Cardiomyopathy should be Distinguished from on Echocardiography

Conditions that Noncompaction Cardiomyopathy should be Distinguished from on Echocardiography

Transthoracic Echocardiographic Findings[4]

Transthoracic Echocardiographic Findings[4]

  • There are deep trabeculations in the ventricular wall
  • There is systolic dysfunction with an average LVEF of 33%
  • It is notable that there is hypokinesis in both the noncompacted as well as the compacted segments. It has been speculated that this is due to the microcirculatory defect observed in these patients.
  • There was diastolic dysfunction in all 17 patients in one series, with 36% of these patients having a restrictive filling pattern.
  • The left ventricular wall is thick with a two layered appearance
  • The epicardial layer is compacted and thin and the endocardial layer is non-compacted and thick
  • The ratio of the non-compacted endocardial layer to the epicardial layer is > 2 in adults and >1.4 in children
  • 41% of patients will have involvement of the RV apex
Role of Transesophageal and Contrast Echocardiography in Improving Sensitivity and Specificity of the Diagnosis of Noncompaction Cardiomyopathy

Role of Transesophageal and Contrast Echocardiography in Improving Sensitivity and Specificity of the Diagnosis of Noncompaction Cardiomyopathy

Transesophageal echocardiography [5] and contrast echocardiography [6]have been advocated as methods for improving the sensitivity and specificity of detecting noncompaction cardiomyopathy.

References

References

  1. ↑ Weiford BC, Subbarao VD, Mulhern KM, Noncompaction of the ventricular myocardium. Circulation 109 (24): 2965-71 2004
  2. ↑ Ichida F, Hanamichi Y, Miyawaki T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999; 34: 233–240.
  3. ↑ .Ritter M, Oechslin E, Sutsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997; 72: 26–31.
  4. ↑ Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 2000; 36: 493–500.
  5. ↑ Maltagliati A, Pepi M. Isolated noncompaction of the myocardium: multiplane transesophageal echocardiography diagnosis in an adult. J Am Soc Echocardiogr. 2000; 13: 1047–1049.
  6. ↑ Koo BK, Choi D, Ha J, et al. Isolated noncompaction of the ventricular myocardium: contrast echocardiographic findings and review of the literature. Echocardiography. 2002; 19: 153–156.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]