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A portrait of non-dilated left ventricular cardiomyopathy - clinical, electrical and structural features in a real-world population
Session:
Sessão de Posters 48 - Risco arrítmico, tomada de decisão e desfechos nas miocardiopatias
Speaker:
David Vicente Marques
Congress:
CPC 2026
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.6 Myocardial Disease – Clinical
Session Type:
Posters Eletrónicos
FP Number:
---
Authors:
David Vicente Marques; Leonor Magalhães; Isabel Cardoso; José Viegas; Pedro Brás; Inês Grácio de Almeida; Vera Ferreira; Diana Antunes; Sílvia Aguiar Rosa; Rui Cruz Ferreira
Abstract
<p><strong>Abstract</strong></p> <p>Non-dilated left ventricular cardiomyopathy (NDLVC) is a recently recognized form of cardiomyopathy which can be characterized by the presence or absence of left ventricular (LV) disfunction without dilation and the presence of non-ischaemic LV scarring or fatty replacement. LV global hypokinesis without scarring may also be considered.</p> <p>This study aims to characterize the population of patients (P) with NDLVC diagnosis with follow up in our center.</p> <p><strong>Methods</strong></p> <p>Our analysis enrolled 48 patients (P) with NDLVC diagnosis, followed up at cardiomyopathy outpatient clinic at a tertiary center. A descriptive analysis of clinical, electrocardiographic and imaging data were analyzed. Major arrhythmic events (MAE) were defined as ventricular fibrillation, ventricular tachycardia or implantable cardioverter-defibrillator (ICD) therapies.</p> <p><strong>Results</strong></p> <p>The mean age was 51 ±15.1years, 64.6% of P were male, 51.1% had family history of sudden cardiac death (SCD) and 53.2% had family history of known cardiomyopathy. Genetic testing available for 42 P, in 24 (57.1%) had pathogenic or likely pathogenic variants, 13 (31%) VUS and 5 (11.9%) were negative. Pathogenic or likely pathogenic (P/LP) variants were detected in the following genes: FLNC (23.8%), LMNA (16.6%), TTN (9.5%) and DSP (9.5%).</p> <p>Analytically creatinine was 0.89 mg/dL (± 0.29 mg/dL), NT-proBNP 84 ± 487 pg/mL (normal range < 50pg/ml)and high sensitivity troponin T was 7 ng/L ± 2 ng/L (Normal range <14).</p> <p>On the ECG, 41,7% of P presented low QRS voltage and 47.9% had at least 1 T wave inversion. Atrial fibrillation (at diagnosis) was present in 6.3% of cases, but the prevalence at the end of follow up was 35.4%.</p> <p>Cardiac magnetic resonance evaluation showed a median LV ejection faction (LVEF) of 53% ± 9.5%, presence of late gadolinium enhancement (LGE) in 89.4% of P with a median number of segments affected of 2.00 ± 1.77. The pattern of fibrosis was septal LGE in 60.4% and ring like pattern in 10.4%, with mean native T1 of 1065 ± 115ms and mean T2 of 47.8 ± 4.6ms. RVEF was 60%± 8.2%. Indexed LV telediastolic volume (iLVTV) was 80 ± 15mL/m2 and iRVTV was 73.5 (± 14 mL/m2). Echocardiographic GLS was -15.4± 3.1% and E/e’ ratio was 7 ± 2.5. This means that globally systolic and diastolic function is preserved. Inflammation by updated Lake Louise criteria was present in 16.7% of P.</p> <p>About 41.7% of P had an ICD, 6.25% in secondary prevention. We recorded 6 MAE and non-sustained ventricular tachycardia was observed in 40.4% of P, most of them more than 1 time during follow up. Only 8.3% of P had an hospitalization or emergency occurrence due to heart failure decompensation.</p> <p><strong>Conclusions</strong></p> <p>NDLVC frequently associates with preserved systolic and diastolic function. P/LP variants were found in 57.1% of P and myocardial fibrosis on CMR is frequent. Inflammation is part of the fisiopathologic process and was identified in 16.7% of P.</p>
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