Abstract
BackgroundUltrasonography is commonly used to diagnose left ventricular noncompaction (LVNC). A ratio of noncompacted to compacted myocardium (NC/C ratio) > >2 is often used to diagnose LVNC. However, a large proportion of patients with noncompact myocardium have NC/C < 2, and the prognosis of these patients have not been studied.MethodsWe included children diagnosed with LVNC between 0 and 15 years of age from January 2007 to December 2018. LVNC was diagnosed based on Stöllberger standard when over three trabeculae were found to be associated with the interventricular recesses. A maximal end systolic ratio of noncompacted to compacted layers was NC/C ratio. Outcomes for LVNC subjects with NC/C < 2 and NC/C > 2 were compared using Kaplan-Meier methods.ResultsThere were 124 newly diagnosed LVNC cases, classified as isolated (i-LVNC, n = 47) or non-isolated (ni-LVNC, n = 77) LVNC and NC/C > 2 (n = 43) or < 2 (n = 81). The median (interquartile range) follow-up duration was 12 (3–30) months for all patients and 16 (6–36) months for survivors. Sixteen patients with i-LVNC died during follow-up. Patients with i-LVNC and NC/C > 2 had worse survival than those with NC/C < 2 (p = 0.022).ConclusionsIn conclusion, during a 12-month follow-up, patients with i-LVNC with NC/C < 2 had a benign prognosis and better outcomes than those with NC/C > 2, suggesting that the former could have a more active and routine lifestyle.
Highlights
Ultrasonography is commonly used to diagnose left ventricular noncompaction (LVNC)
Left ventricular noncompaction (LVNC) is increasing in prevalence and is a type of cardiomyopathy classified by an extensive trabeculated myocardium, which was separated into two distinct layers composed of compacted and noncompacted myocardium [1, 2]
Ultrasound is used for diagnosis, and the three most commonly cited echocardiographic criteria include the depths of intertrabecular recesses [11], ratio of noncompacted to compacted myocardium (NC/C ratio) [12], and the number of trabeculations
Summary
We included children diagnosed with LVNC between 0 and 15 years of age from January 2007 to December 2018. Outcomes for LVNC subjects with NC/C < 2 and NC/C > 2 were compared using Kaplan-Meier methods. This observational, single-center, and retrospective study included all children, between 0 and 15 years of age, from the Children’s Hospital of Chongqing Medical University diagnosed with LVNC from January 2007 to December 2018. Medical records were reviewed to document clinical presentations, including symptoms, primary diagnosis, New York Heart Association (NYHA)/Ross classification, associated dysmorphic features, presence of arrhythmia, and a positive family history. For some patients, cine cardiac magnetic resonance images (MRIs) were analyzed to assist diagnosis. Ethical approval was obtained from the ethics committee of Children’s Hospital of Chongqing Medical University (Approval number: no. 24 of 2015), and informed consents were obtained from parents or guardians on the behalf of participants
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