Acquired left ventricular noncompaction as a cardiac manifestation of neuromuscular disorders

Abstract

Objectives: In single cases left-ventricular-hypertrabeculation/noncompaction (LVHT) develops after birth (acquired-LVHT). This study aimed to determine the prevalence of acquired-LVHT, to look for echocardiographic similarities between acquired-LVHT cases, to determine the interval between the last normal echocardiography and detection of acquired-LVHT, and which neuromuscular-disorders are associated with acquired-LVHT.

Design: We retrospectively looked for acquired-LVHT among a cohort of 22 LVHT-patients, diagnosed during 3 years. We compared cardiac and extra-cardiac findings between patients with acquired-LVHT.

Results: Among the 22 patients with LVHT, acquired-LVHT was found in four of them. One of the four was echocardiographically normal when investigated before the detection of LVHT. At diagnosis of LVHT echocardiography showed cardiac abnormalities in addition to LVHT in three of the four patients. The last echocardiography without LVHT was carried out on the average 3.8 y before LVHT was detected. Two patients had a dystrophinopathy and two patients a metabolic myopathy.

Conclusion: Acquired-LVHT is more frequent than previously thought, requires review of previous echocardiographies, is largely associated with other cardiac abnormalities, and occurs most frequently together with neuromuscular-disorders.