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Published on: November 2, 2020
Helical distribution of hypertrophy in patients with hypertrophic cardiomyopathy: prevalence and clinical
Dafne Viliani1, Eduardo Pozo1, Norma Aguirre1
1Cardiology Department, Hospital Universitario de la Princesa, IIS-IP, Universidad Autónoma de Madrid, Calle Diego de León, 62, 28006, Madrid, Spain.
Insights
A helical pattern of hypertrophy is common in hypertrophic cardiomyopathy (HCM) and linked to worse outcomes. This pattern, assessed via cardiac magnetic resonance (CMR), indicates a higher risk of sudden cardiac death (SCD).
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Medical Diagnostics
Background:
- A novel helical distribution pattern of hypertrophy has been recently described in hypertrophic cardiomyopathy (HCM) patients.
- The prevalence and clinical significance of this helical pattern in an unselected HCM cohort require further investigation.
Purpose of the Study:
- To determine the prevalence of the helical hypertrophy pattern in a cohort of hypertrophic cardiomyopathy patients.
- To assess the potential clinical implications and associations of this helical pattern with adverse cardiovascular events.
Main Methods:
- One hundred and eight consecutive patients diagnosed with HCM via cardiac magnetic resonance (CMR) were included.
- Helical pattern presence was assessed by measuring maximal left ventricle (LV) wall thickness in 17 LV segments.
- Clinical data and follow-up information were prospectively collected for analysis.
Main Results:
- A helical distribution pattern was detected in 58% of HCM patients.
- This pattern was significantly associated with increased left ventricular outflow tract obstruction (LVOT), non-sustained ventricular tachycardia (NSVT), and a higher estimated risk of sudden cardiac death (SCD).
- Patients with more extensive helical patterns showed higher incidences of heart failure and all-cause death.
Conclusions:
- A helical pattern of hypertrophy is a frequent finding in hypertrophic cardiomyopathy and can be readily assessed using standard CMR cine sequences.
- The presence of a helical pattern is associated with significant negative clinical implications, including an elevated risk of sudden cardiac death.
- This finding underscores the importance of recognizing and evaluating helical hypertrophy patterns in HCM management.
Abstract:
Recently a novel pattern of helical distribution of hypertrophy has been described in patients with hypertrophic cardiomyopathy (HCM). Our aim was to determine its prevalence and potential implications in an unselected cohort. One-hundred- and eight consecutive patients diagnosed with HCM by cardiac magnetic resonance (CMR) were included (median clinical follow up of 1718 days). All clinical and complementary test information was prospectively collected. The presence of a helical pattern was assessed by a simple measurement of the maximal left ventricle (LV) wall thickness (LVWT) for each of the 17 classical LV segments and it was classified in one of three types according to its extension. A helical distribution was detected in 58% of patients, and was associated to a higher incidence of left ventricular outflow tract obstruction (LVOT; 35% vs. 10%; p = 0.005) and systolic anterior motion of the mitral valve (SAM; 30% vs. 13%, p = 0.053). No significant difference in the maximal LVWT was observed. However, the presence of a helical pattern showed a significant association with non sustained ventricular tachycardia (NSVT; 22% vs. 7%; p = 0.029) and was associated to a higher risk of sudden cardiac death (SCD) calculated with the European society of cardiology (ESC) calculator (p = 0.006). Notably, patients with a more extense spiral had a higher incidence of heart failure (75% vs. 34%, p = 0.012) and all-cause death (21 vs. 3%, p = 0.049). A helical pattern is frequent in HCM and can be readily assessed on CMR standard cine sequences. In conclusion, a helical pattern carries negative clinical implications and is associated to a higher estimated risk of SCD.
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