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Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease

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Abstract

Left ventricular noncompaction is a rare form of cardiomyopathy, which results from multiple trabeculations in the left ventricular myocardium. The clinical presentation is highly variable, and spectrum includes asymptomatic patients diagnosed during family screening on one end to patients with depressed systolic function, heart failure, thromboembolic complications, and cardiac arrhythmias on the other (Kim et al in J Am Coll Cardiol 53: 2009, 2009). Further, the progression of the condition is highly variable. Hence, these patients require close follow-up, and management for each patient needs to be individualized and periodically reevaluated. Here, we present a series of five cases that have been followed in our practice and present our experience. A literature review of this rare form of congenital cardiomyopathy is also presented.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, University at Buffalo, Women and Children’s Hospital of Buffalo, 239 Bryant Street, Buffalo, NY, 14203, USA

    Umang Gupta & Pooja Makhija

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  1. Umang Gupta
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  2. Pooja Makhija
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Correspondence to Umang Gupta.

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None of the authors have any potential conflict of interest to declare.

Human and Animal Rights

Since this is a retrospective case review, consent was not obtained and no research performed on human or animal subjects. Also, no data that would help identify the patients have been used in the case series and the data related to patient identity have been stored on a separate unlinked file on a password protected computer kept securely by the investigator.

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Gupta, U., Makhija, P. Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease. Pediatr Cardiol 38, 681–690 (2017). https://doi.org/10.1007/s00246-016-1566-5

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  • DOI: https://doi.org/10.1007/s00246-016-1566-5

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