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Published on: August 30, 2024
Reverse septal curvature in hypertrophic cardiomyopathy: Implications for function, structure and arrhythmic risk
Roberto Polizzi1, Grazia Canciello1, Leopoldo Ordine1
1Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy.
Insights
Reverse septal curvature (RSC) in hypertrophic cardiomyopathy (HCM) patients is linked to a distinct phenotype. This includes apical aneurysms, mid-cavity obliteration, and a worse arrhythmic profile, independent of LV aneurysm.
Area of Science:
- Cardiology
- Medical Imaging
- Biomedical Engineering
Background:
- Reverse septal curvature (RSC) is a recognized marker of increased clinical burden in hypertrophic cardiomyopathy (HCM).
- The precise mechanical and structural implications of RSC in HCM patients are not fully understood.
Purpose of the Study:
- To investigate the mechanical and structural implications of RSC in patients with HCM.
- To determine if RSC is associated with specific cardiac structural changes and functional impairments.
Main Methods:
- 107 HCM patients underwent 2D echocardiography, 3D echocardiography, and cardiac magnetic resonance (CMR).
- RSC was defined as left ventricular (LV) mid-septal convexity toward the LV cavity.
- LV systolic behavior, including cavity and mid-cavity obliteration, was assessed.
Main Results:
- 49% of patients had RSC, exclusively associated with LV apical aneurysms (20%).
- RSC patients showed higher rates of sarcomeric mutations, non-sustained ventricular tachycardia (NSVT), greater wall thickness, impaired global longitudinal strain (GLS), and more frequent late gadolinium enhancement.
- RSC was an independent predictor of NSVT, and mid-cavity obliteration was observed in all RSC patients with reduced apical ejection fraction.
Conclusions:
- RSC identifies a distinct HCM phenotype, not just an anatomical variant.
- This phenotype is characterized by apical aneurysms, mid-cavity obliteration, impaired apical function, and a worse arrhythmic profile.
- These findings are independent of LV aneurysm presence.
Background:
In patients with hypertrophic cardiomyopathy (HCM) reverse septal curvature (RSC) is a marker of higher clinical burden; however, its mechanical and structural implications remain underexplored.
Methods And Results:
A total of 107 patients with HCM underwent comprehensive 2D echocardiography, 3D echocardiography, and cardiac magnetic resonance (CMR). In both imaging modalities, reverse septal curvature (RSC) was defined as left ventricular (LV) mid-septal convexity toward the LV cavity extending over more than one-third of the distance from the base to the apex. LV systolic behavior, including cavity and mid-cavity obliteration, was assessed. Forty-nine patients (46 %) were classified as having RSC. LV apical aneurysms were observed exclusively in patients with RSC (20 %). Subsequent analyses excluded patients with LV aneurysms. Compared with those without RSC, patients with RSC had higher rates of sarcomeric mutations (57 % vs. 33 %), non-sustained ventricular tachycardia (NSVT) (61 % vs. 21 %), greater wall thickness (23 ± 5 vs. 19 ± 4 mm), impaired GLS (-10 ± 3 % vs. -13 ± 3 %), and more frequent late gadolinium enhancement (100 % vs. 69 %) (all p < 0.05). In this subgroup, multivariate analysis identified RSC as an independent predictor of NSVT (OR = 5.571, 95 % CI 1.446-21.566, p = 0.013). Mid-cavity obliteration was observed in nine patients, all with RSC. Apical ejection fraction (EF) was significantly reduced in this group, with a marked apical-basal EF gradient (β = -30, p < 0.001).
Conclusions:
Our data demonstrated that RSC is not merely an anatomical variant, but it identifies a distinct HCM phenotype characterized by exclusive association with apical aneurysms, mid-cavity obliteration, impaired apical function, and worse arrhythmic profile independently of LV aneurysm.
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