title: Left ventricular noncompaction-A rare form of cardiomyopathy: Revelation modes and predictors of mortality in adults through 23 cases.
abstract: OBJECTIVE
  To describe modes of clinical presentation and echocardiographic, angiographic, and rhythmic features, and prognostic characteristics of left ventricular noncompaction cardiomyopathy (LVNC) in North African adults, through one of the first series in Morocco.
  
  
  BACKGROUND
  LVNC is a rare congenital disorder, described for the first time by Engberding in 1984. The suspected diagnosis in thromboembolic, hemodynamic, or rhythm events requires both echocardiography and cardiovascular magnetic resonance (CMR). Its therapeutic management is not yet well codified but akin to that proposed for dilated cardiomyopathy.
  
  
  METHODS
  This study included a retrospective, descriptive, and analytical cohort of 23 cases of cardiomyopathy with LVNC diagnosed in the Noninvasive Explorations Laboratory at the Military Hospital of Rabat, Morocco, between January 2009 and October 2014. The echocardiographic criteria for LVNC include the absence of any coexisting cardiac anomalies. The characteristic appearance of numerous excessively prominent trabeculations and deep intertrabecular recesses and intertrabecular spaces filled by direct blood flow from the ventricular cavity, as visualized on color Doppler imaging with noncompacted/compacted ratio > 2 according to Jenni criteria. Twenty-three adults fulfilled the diagnostic criteria and were followed prospectively.
  
  
  RESULTS
  At diagnosis, the mean age was 47 ± 13 years with a male predominance at 65.2%. Of them, 56.5% had a left bundle branch block and 21.7% were in atrial fibrillation. Left ventricular end-diastolic diameter was 67.7 ± 6.6 mm and ejection fraction was at 27 ± 8%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in more than 80% of patients with an average of 4.8 noncompacted segments. CMR was performed in 12 patients and was decisive for the diagnosis. Major complications were heart failure in 31% of patients, ventricular tachycardia in three patients, and thromboembolic events in one patient. Twenty eight point six percent of patients started a long-term anticoagulant therapy. One patient underwent implantation of a double-room pacemaker. Automated defibrillators were implanted in two patients. There were three deaths: one sudden death and two end-stage heart failure.
  
  
  CONCLUSIONS
  LVNC should be looked for at any dilated cardiomyopathy particularly in young patients. It requires a careful echocardiographic examination and sometimes CMR to confirm the diagnosis. It is characterized by severe systolic and diastolic dysfunction that would provide poor prognosis.
authors.affiliation: Department of Cardiac Non-Invasive Explorations, Mohamed V Military Hospital, IBN SINA Hospital Center, Mohamed V University, Rabat, aMorocco.
authors.forename: Iliyasse
authors.initials: I
authors.lastname: Asfalou
authors.affiliation: Department of Cardiac Non-Invasive Explorations, Mohamed V Military Hospital, IBN SINA Hospital Center, Mohamed V University, Rabat, aMorocco.
authors.forename: Sanae
authors.initials: S
authors.lastname: Boulaamayl
authors.affiliation: Department of Cardiac Non-Invasive Explorations, Mohamed V Military Hospital, IBN SINA Hospital Center, Mohamed V University, Rabat, aMorocco.
authors.forename: Maha
authors.initials: M
authors.lastname: Raissouni
authors.affiliation: Department of Cardiac Non-Invasive Explorations, Mohamed V Military Hospital, IBN SINA Hospital Center, Mohamed V University, Rabat, aMorocco.
authors.forename: Najat
authors.initials: N
authors.lastname: Mouine
authors.affiliation: Department of Cardiac Non-Invasive Explorations, Mohamed V Military Hospital, IBN SINA Hospital Center, Mohamed V University, Rabat, aMorocco.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Sabry
authors.affiliation: Department of Cardiac Non-Invasive Explorations, Mohamed V Military Hospital, IBN SINA Hospital Center, Mohamed V University, Rabat, aMorocco.
authors.forename: Jamal
authors.initials: J
authors.lastname: Kheyi
authors.affiliation: Department of Cardiology B, IBN SINA Hospital Center, Mohamed V University, Rabat, bMorocco.
authors.forename: Nawal
authors.initials: N
authors.lastname: Doghmi
authors.affiliation: Department of Cardiac Non-Invasive Explorations, Mohamed V Military Hospital, IBN SINA Hospital Center, Mohamed V University, Rabat, aMorocco.
authors.forename: Aatif
authors.initials: A
authors.lastname: Benyass
keywords: CMR; Echocardiography; Left ventricular noncompaction; Predictors of mortality