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Purpose of Review <p>Non-dilated left ventricular cardiomyopathy (NDLVC) is a newly recognized cardiomyopathy phenotype, characterized by systolic dysfunction and/or non-ischemic myocardial scar, in the absence of ventricular dilatation. This review summarizes current evidence on its epidemiology, imaging features, genetic background, arrhythmic substrate, differential diagnosis, risk stratification, and management.</p> Recent Findings <p>Advanced cardiac magnetic resonance has demonstrated that late gadolinium enhancement, particularly septal and ring-like pattern, is common in NDLVC and predicts malignant ventricular arrhythmias (MVA) and evolution to dilated phenotype. High-risk genotypes (e.g., <i>DSP</i>, <i>FLNC</i>, <i>LMNA</i>, <i>PLN</i>) and myocardial inflammation further increase arrhythmic and heart failure risk. Novel tools, including the NDLVC-5-year risk score, allow improved individualized prediction, especially when combined with genotype-specific score.</p> Summary <p>NDLVC resulting from the complex interplay between genetic background and environmental factors may lead to arrhythmias and heart failure. A multimodal, phenotype-first approach integrating imaging, genetics, and electrophysiology is essential for accurate diagnosis, risk stratification, and personalized management.</p>

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Non-Dilated Left Ventricular Cardiomyopathy (NDLVC): A Name, Multiple Souls

  • 期刊论文

  • 发表日期：

    2026年3月31日

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  • Andrea Villatore,
  • Emanuela Sergianni,
  • Alessia F. Trezza,
  • Matteo A. di Padova,
  • Chiara Amman,
  • Chiara Fiorentino,
  • Emanuela Favalli,
  • Francesca Rumore,
  • Paolo Della Bella,
  • Giovanni Peretto

摘要

Purpose of Review

Non-dilated left ventricular cardiomyopathy (NDLVC) is a newly recognized cardiomyopathy phenotype, characterized by systolic dysfunction and/or non-ischemic myocardial scar, in the absence of ventricular dilatation. This review summarizes current evidence on its epidemiology, imaging features, genetic background, arrhythmic substrate, differential diagnosis, risk stratification, and management.

Recent Findings

Advanced cardiac magnetic resonance has demonstrated that late gadolinium enhancement, particularly septal and ring-like pattern, is common in NDLVC and predicts malignant ventricular arrhythmias (MVA) and evolution to dilated phenotype. High-risk genotypes (e.g., DSP, FLNC, LMNA, PLN) and myocardial inflammation further increase arrhythmic and heart failure risk. Novel tools, including the NDLVC-5-year risk score, allow improved individualized prediction, especially when combined with genotype-specific score.

Summary

NDLVC resulting from the complex interplay between genetic background and environmental factors may lead to arrhythmias and heart failure. A multimodal, phenotype-first approach integrating imaging, genetics, and electrophysiology is essential for accurate diagnosis, risk stratification, and personalized management.