Received: February 16, 2011;
Initial review: March 10, 2011;
Accepted: April 8, 2011.

Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy of emerging importance. We report a case of a 3- months- old boy who presented with congestive heart failure due to LVNC.

Key words: Cardiomyopathy, Noncompaction, Congestive heart failure.

A 3-months-old boy presented with feeding difficulty and sweating over forehead since 15 days and breathlessness since 2 days. His parents were unrelated and he was their first child. On examination, he had congestive heart failure (heart rate -100 /min, weak pulses, capillary refill time of 5 seconds, S3 gallop, grade 3/6 pansystolic murmur at apex, hepatomegaly, tachypnea and bilateral basal crepitations). Other systemic examination was normal. He had no dysmorphic features.

The X-ray chest showed cardiomegaly. The ECG revealed left ventricular hypertrophy. The echocardiography demonstrated grossly enlarged left ventricle. A two layer structure was seen, with a compacted thin epicardial band and a thick noncompacted endocardial layer with coarse trabeculations having deep intertrabecular recesses at the apical and lateral wall segments of the left ventricle (Fig. 1). The end systolic ratio of noncompacted to compacted layers was 3.2. The color Doppler imaging showed blood filling the recesses from the ventricular cavity. The origin of both the coronary arteries was normal. The M-mode showed left ventricular end- diastolic diameter (LVEDD) – 3.72 cm (Z score = 7.52), left ventricular end- systolic diameter (LVESD) – 3.39 cm (Z score = 12.12), the left ventricle ejection fraction (LVEF) -30.2% and shortening fraction (SF) -9%. There was mitral regurgitation with velocity of 279 cm/s. The right ventricle was normal. These findings were consistent with isolated left ventricular noncompaction.

Fig.1 (A) Parasternal short axis view. The end- systolic still frame shows the two layered structure with thin epicardial layer and an extremely thickened endocardial layer with prominent trabeculations and deep recesses (arrows). (B) To quantify the thickness of the noncompacted layer at the site of maximum thickness, the end- systolic ratio of the noncompacted (NC) to compacted (C) layer is determined. (C) Apical four- chamber view still frame. (D) M-Mode study demonstrating LVEF- 30%. (E) mitral valve Doppler demonstrating regurgitation.

He was treated with decongestive therapy and inotropes for 3 days, and subsequently discharged on oral enalapril, frusemide and ecosprin. The echocardiography of parents was normal.

Left ventricular non-compaction represents an arrest in endomyocardial morphogenesis, which normally occurs between 5- 8 weeks of fetal life and is characterised by gradual compaction of myocardium, transformation of large intertrabecular spaces into capillaries, and evolution of the coronary circulation [1]. Clinically, patients may be asymptomatic or present with CHF, arrhythmia and embolic events [4-6]. The presenting symptoms include tachypnea, cyanosis, syncope, or failure to thrive, Chin, et al. [7] have described facial dysmorphism, including a prominent forehead, low set ears, strabismus, high- arched palate and micrognathia.

LVNC can be diagnosed by two-dimensional echocardiography and color Doppler [7,8]. The criterion for diagnosis is ratio of noncompacted to compacted layer of >2 measured at end systole.

There is no specific therapy. The mainstay of treatment is diuretics, ACE inhibitors and beta- blockers (Carvedilol) to improve the left ventricular systolic function. Anticoagulation (target INR – 2.0-3.0) is recommended when LVEF <40% [3]. Pignatelli, et al.[4] recommend oral aspirin and a metabolic cocktail including thiamine, coenzyme Q10, riboflavin and carnitine. Poor prognostic markers predicting death or heart transplant are age at onset, NYHA functional class III–IV, sustained ventricular arrhythmias, ratio of noncompacted to compacted layers, number of affected segments, LVEDD and abnormal lateral mitral tissue Doppler Ea velocity [3,9].

Funding: None; Competing interests: None declared.

1. Elshershari H, Okutan V, Celiker A. Isolated noncompaction of the ventricular myocardium. Cardiol Young. 2001;11:472-5.

2. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. 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.

3. Oechslin E, Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. [online]. Available from: URL: http://www.eurheartj.oxfordjournals.org/content/early/2011/01/31/eurheartj.ehq508.full. Accessed February 9, 2011

4. Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, et al. Clinical characterization of left ventricular noncompaction in children: A relatively common form of cardiomyopathy. Circulation. 2003;108:2672-78.

5. Suvarna JC, Deshmukh CT, Hajela SA. Left ventricular noncompaction: a cardiomyopathy often mistaken. Indian J Med Sci. 2009;63:303-7.

6. Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi 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; 34:233-40.

7. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium: A study of eight cases. Circulation. 1990;82:507-13.

8. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann P. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001;86:666-71.