SPC365

Cardiovascular magnetic resonance in neuromuscular disorders – looking ahead

Session: Comunicações Orais - Sessão 26 - Doenças do Miocárdio
Speaker: Ana Amador

Congress: CPC 2023
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 17. Myocardial Disease
Subtheme: 17.7 Myocardial Disease - Other
Session Type: Comunicações Orais
FP Number: ---
Authors: Ana Amador; Catarina Martins da Costa; João Calvão; Catarina Marques; André Cabrita; Ana Pinho; Luís Santos; Cátia Oliveira; António José Madureira; Elisabete Martins; Teresa Pinho; Filipe Macedo

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="font-size:11.0pt"><span style="color:black">Background:</span></span></strong><span style="font-size:11.0pt"><span style="color:black"> Neuromuscular disorders (NMD) have a wide range of different cardiac presentations. Cardiac magnetic resonance (CMR) has an established role in diagnosis and risk stratification. We sought to access how CMR performs in predicting events in a real cohort of NMD patients (pts).</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="font-size:11.0pt"><span style="color:black">Methods:</span></span></strong><span style="font-size:11.0pt"><span style="color:black"> We included consecutive patients followed in a tertiary clinical center with neuromuscular disorders (NMD) from January 2012 to December 2018. Clinical and CMR data were collected. During follow-up (FUP), we considered major adverse cardiovascular events (MACE) as a composite of device implantation, ventricular tachycardia/appropriate shock therapy and death.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="font-size:11.0pt"><span style="color:black">Results:</span></span></strong><span style="font-size:11.0pt"><span style="color:black"> A total of 65 patients (pts) were included, 33 (51%) women, with mean age of 32 &plusmn; 16 years. Most patients&nbsp;had myotonic dystrophy (34, 52%), followed by limb&ndash;girdle muscular dystrophy (22; 34%); the remained 9 (13%) had other NMD. About half had inferior limbs predominantly affected and 74% had none, mild or moderate functional impairment. Regarding cardiac manifestations, 18% had cardiac symptoms, 97% were in sinus rhythm, median PR and QRS duration were 169 (IQR 47) and 101 (IQR 11), respectively; median BNP was 26 (IQR 25) mg/dl. </span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="color:black">Regarding CMR, 43,3% of pts had &ge; one abnormality. Six pts had left ventricle dilation and 7 had left ventricle&nbsp;ejection fraction (LVEF) 55%. Three pts had significant hypertrophy (&gt;12 mm) and there were isolated cases of hypertrabeculation, segmental alterations or right ventricle dilation. Regarding tissue characterization, 2 pts had T2 hyperintensity, 8 had early gadolinium enhancement&nbsp;(EGE) and 22 had late gadolinium enhancement&nbsp;(LGE). LGE was located mainly intramyocardium (45%) or subepicardial (36%) and the most affected segments were basal and medium inferolateral (40%).</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="color:black">During a median FUP of 77 (IQR 33) months</span></span> <span style="font-size:11.0pt"><span style="color:black">there were 7 deaths, 8 implanted devices (4 pacemakers and 4 CRT-D, 3 in primary prevention) and one sustained ventricular tachycardia in holter; there were no shock therapies. </span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="color:black">Table 1 describes some CMR parameters according to the occurrence of events. </span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="color:black">Using Kaplan Meier curves, there were associations between LVEF&lt;55% and presence of LGE with occurrence of all events (log rank test, p=0,002 and p=0,045, respectively), but no association were found with age, LGE pattern nor number/distribution of affected segments. &nbsp;</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="color:black">Using Cox Regression, we found that the LVEF&lt;55% was associated with 6 fold higher risk of events (HR crude 6,15; 95% CI 1,65-22,93), that remained significant after adjusting for LGE (HR adjusted 4,81, 95% CI 1,07-15,9).</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="font-size:11.0pt"><span style="color:black">Conclusion:</span></span></strong><span style="font-size:11.0pt"><span style="color:black"> In our cohort, CMR LVEF&lt;55% and the presence of LGE were significantly associated with events during FUP, reinforcing the role of this technique on risk stratification of NMD populations.</span></span></span></span></p>