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Published on: May 24, 2021
Geometric assessment of asymmetric septal hypertrophic cardiomyopathy by CMR
Anca Florian1, Pier Giorgio Masci, Stijn De Buck
1Department of Radiology, UZ Leuven, Leuven, Belgium.
Insights
This study reveals that most hypertrophic cardiomyopathy (HCM) patients exhibit a spiral pattern of hypertrophy (HT). This counterclockwise spiral, visualized with cardiac magnetic resonance (CMR), offers new insights into HCM
Area of Science:
- Cardiology
- Medical Imaging
- 3D Visualization
Background:
- Hypertrophic cardiomyopathy (HCM) presents with diverse phenotypes.
- Cardiac magnetic resonance (CMR) is a powerful tool for 3D imaging of HCM.
- Understanding the geometric patterns of hypertrophy is crucial for characterizing HCM.
Purpose of the Study:
- To analyze the geometric pattern of hypertrophy (HT) in asymmetrical septal HCM using CMR.
- To investigate if HT follows a longitudinal spiral pattern in HCM patients.
- To correlate spiral HT patterns with clinical characteristics.
Main Methods:
- Utilized short-axis cine CMR to assess 3D hypertrophy extent (radial, circumferential, longitudinal).
- Included patients with septal wall thickness ≥15 mm and septal to free wall ratio >1.3.
- Analyzed the relationship between circumferential and longitudinal hypertrophy extent.
Main Results:
- 86% of 132 patients showed a consistent longitudinal course of HT, often a counterclockwise spiral.
- The spiral pattern originated at the basal anteroseptum with variable rotation (mean 116°).
- Greater rotation was associated with older age, LV outflow tract obstruction, and hypertension.
Conclusions:
- The majority of asymmetrical septal HCM patients display a counterclockwise (left-handed) spiral hypertrophy pattern.
- Significant variability exists in the magnitude of this spiral pattern.
- Further research is needed to link spiral patterns to genetics, hemodynamics, and patient outcomes.
Objectives:
The aim of this study was to analyze the geometric pattern of hypertrophy (HT) in patients with asymmetrical septal hypertrophic cardiomyopathy (HCM) using cardiac magnetic resonance (CMR) and to test the hypothesis that at least in some patients, the HT follows a longitudinal spiral pattern.
Background:
The highly heterogeneous phenotypic expression of HCM is a well-known phenomenon. CMR has emerged as a robust 3-dimensional (3D) tomographic imaging technique that is increasingly used to explore phenotypic expression.
Methods:
Short-axis cine CMR was used to study the 3D extent of HT (i.e., radial, circumferential, and longitudinal extent, as well as the relation between circumferential and longitudinal extent). Inclusion criteria were septal wall thickness (WT) ≥15 mm and septal to free wall WT ratio >1.3.
Results:
CMR was performed in 132 patients. Maximal WT was 22 ± 5 mm, with a circumferential extent of 131 ± 51°, and a longitudinal extent of 64 ± 19%, resulting in a hypertrophied left ventricular (LV) surface of 26 ± 15%. Linear regression analysis showed in 86% of patients a consistent course of HT along the longitudinal direction. The HT invariably started at the basal anteroseptum and rotated, except in 2 patients, in a counterclockwise direction (CC-spiral patients) with a mean global rotation of 116 ± 68° (range 5° to 350°). After the CC-spiral patients were divided according to magnitude of rotation quartiles (Q1: 5° to 70°, Q2: 75° to 105°, Q3: 110° to 150°, and Q4: 155° to 350°), Q4 patients were significantly older and had more LV outflow tract obstruction and hypertension than patients without the spiraling pattern. In 11 patients, continuation of HT into an apical form of HCM was found.
Conclusions:
Using 3D analysis, we found that the majority of patients with asymmetrical septal HCM in fact showed a spiral pattern of HT following a counterclockwise (or "left-handed") spiral trajectory. The variation in magnitude of rotation among patients, however, was highly variable. Further research is warranted to better understand the significance of the current findings, in particular to relate them to the genetic and morphological substrate, hemodynamic consequences, and patient outcome.
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