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2010-09-14-12 Non-compacted cardiomyopathy © Sosa Olavarria www.TheFetus.net


Non-compacted cardiomyopathy


Alberto Sosa Olavarria
, MD.*, Eva Leinart, MD. PhD.**

*  CEUSP. Valencia. Venezuela.
** Inner Vision Women's Ultrasound, Nashville, Tennessee, USA.


Introduction
 
 
Non-compacted cardiomyopathy is a form of unclassified cardiomyopathy, formerly known as a spongiform cardiomyopathy. It can be associated with systolic dysfunction but the most important marker is a restrictive diastolic dysfunction with ellipsoid ventricles. Ventricles are not enlarged, atria can be prominent.[1] Right ventricle is more likely to be affected in prenatally diagnosed cases.

Ventricles are characterized by high and prominent thick trabeculations on the ventricular wall. Apical ventricular myocardium is usually thickened. Deep recesses between trabeculations are filled with blood directly from the ventricles and this can be demonstrated by Doppler imaging during cardiac cycle.
Recesses do not connect with coronary arteries.

The prenatal findings of non-compacted myocardium may be accompanied by bradycardia and fetal hydrops. The prognosis of such affected fetuses is very poor. Most of the cases with hydrops do not survive until late pregnancy. In case of congestive heart failure, myocardial contractility unfortunately does not improve even after delivery.[2] An isolated ventricular noncompaction may be associated with other cardiac or extracardiac findings, often accompanied by neuromuscular anomalies. [3,4] 

Pathogenesis of this condition is unclear. One of the theory suggests that this condition is due to an arrest of myocardial compaction process. Other hypothesis suggest that pathology is caused by the growth of already impaired myocardium; cardiac neuropathy; impaired adhesion of cardiac myocytes or adaptation to special hemodynamic conditions. There are some familial forms of this condition which suggests the genetic basis for this pathology. [3


Case report

The following images show a case of non-compacted cardiomyopathy.

Images 1- 8: Images showing 4-chamber-view. Note thickened wall of the right ventricle with multiple trabeculations.









Images 9,10: Doppler imaging as a diagnostic toll of this condition. Note blood flow into the sinuses among the trabeculations. 



References:

1. Guntheroth W, Komarniski C, Atkinson W, Fligner CL. Criterion for fetal primary spongiform cardiomyopathy: restrictive pathophysiology. Obstet Gynecol. 2002 May;99(5 Pt 2):882-5.
2. Zielinski p., Piccoli A.Jr., Manica JL., Nicoloso LH. Diseases of the myocardium, endocardium, and pericardium during fetal life. In: Fetal Cardiology. New York, MY: Informa Healthcare USA, Inc; 2009:366-367.
3. Sleurs e, De Catte L, Benatar A. Prenatal Diagnosis of Isolated Ventricular Noncompaction of the Myocardium. J Ultrasound Med 2005; 24:1325–1329.
4. Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr 2004; 17:91–100.

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