Wide spectrum of presentation and variable outcomes of isolated left ventricular noncompaction

Carla Lofiego ¹, Elena Biagini ¹, Ferdinando Pasquale ¹, Marinella Ferlito ¹, Guido Rocchi ¹, Enrica Perugini ¹, Letizia Bacchi-Reggiani ¹, Giuseppe Boriani ¹, Ornella Leone ², Kadir Caliskan ³, Folkert J ten Cate, ³, Fernando M Picchio ⁴, Angelo Branzi ⁵ and Claudio Rapezzi ^1*

¹ Institute of Cardiology, University of Bologna and S.Orsola-Malpighi Hospital , Bologna, Italy
² Department of Pathology, S.Orsola-Malpighi Hospital, Bologna, Italy, Italy
³ Thoraxcenter, Erasmus Medical Center, Rotterdam, the Netherlands, Netherlands
⁴ Pediatric Cardiology and Adult Congenital Unit, University of Bologna and S.Orsola-Malpighi Hospital, Italy
⁵ Institute of Cardiology, University of Bologna and S.Orsola-Malpighi Hospital , Bologna, Netherlands

^* To whom correspondence should be addressed. E-mail: crapezzi{at}aosp.bo.it.

Accepted 21 April 2006

Abstract

Objectives: To investigate diagnostic routes, echocardiographic substrates, outcomes and prognostic factors in IVNC patients identified by echocardiographic laboratories with referral from specialists and primary care physicians.

Background: Most studies suggested that prognosis of isolated ventricular noncompaction (IVNC) is ominous.

Methods: Since 1991, all patients with suspected IVNC were flagged and followed on dedicated databases. Patients were divided into 'symptom-based' and 'nonsymptom-based' diagnostic subgroups.

Results: Sixty-five eligible patients were followed for 6-193 months (mean 43±40). In 53 (82%) patients, IVNC was associated with variable degrees of left ventricular (LV) dilatation and hypokinesia, while in the remaining 12 (18%) LV volumes were normal. Diagnosis was symptom-based in 48 (74%) and nonsymptom-based in 17 (26%) (familial referral in 10). The nonsymptom-based subgroup was characterized by younger age, lower prevalence of ECG abnormalities, better systolic function, and lower left atrial size, whereas no difference was observed in extension of noncompaction. No major cardiovascular events occurred in the nonsymptom-based group, whereas 15/48 (31%) symptomatically diagnosed patients experienced cardiovascular death or heart transplantation (HT) (P=0.01, Kaplan Meier analysis). Independent predictors of cardiovascular death/HT were NYHA III-IV, sustained ventricular arrhythmias and left atrial size.

Conclusions: IVNC is associated with a broad spectrum of clinical and pathophysiological findings, and the overall natural history and prognosis may be better than previously thought. Adult patients with incidental/familial discovery of IVNC have an encouraging outlook, whereas those diagnosed with symptoms of heart failure, history of sustained ventricular tachycardia, or enlarged left atrium have an unstable course and more severe prognosis.

Keywords: cardiomyopathies, myocardium, prognosis, ventricular noncompaction

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