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Cardiac Adrenergic Activity Is Associated with Left Ventricular Hypertrophy in Genetically Homogeneous Subjects with Hypertrophic Cardiomyopathy

¹ Department of Clinical Radiology, University of Kuopio and Kuopio University Hospital, Kuopio, Finland
² Department of Clinical Physiology and Nuclear Medicine, University of Kuopio and Kuopio University Hospital, Kuopio, Finland
³ Department of Radiology, Helsinki University Central Hospital, Helsinki, Finland
⁴ Department of Medicine, University of Kuopio and Kuopio University Hospital, Kuopio, Finland
⁵ Niuvanniemi Hospital, Kuopio, Finland

Hypertrophic cardiomyopathy (HCM) is a genetic disease caused by mutations in genes encoding sarcomeric proteins. However, other genetic and possibly also environmental factors modify the phenotypic expression of left ventricular (LV) hypertrophy. The present study investigated whether cardiac adrenergic activity affects the severity of LV hypertrophy in genetically identical patients with HCM. Methods: The study population consisted of 21 patients with HCM caused by the Asp175Asn substitution of the -tropomyosin gene (TPM1-Asp175Asn) and 9 healthy volunteers. LV mass and segmental wall thickness were measured with MRI. Presynaptic cardiac adrenergic activity was measured with ¹²³I-metaiodobenzylguanidine (MIBG) SPECT. Global and segmental washouts of ¹²³I-MIBG were calculated. Results: Global myocardial ¹²³I-MIBG washout was faster in patients with TPM1-Asp175Asn than in healthy volunteers (50% ± 9% vs. 37% ± 8%, P = 0.001). In linear regression analysis, global ¹²³I-MIBG washout was associated with the LV mass index and LV maximal wall thickness index in HCM patients (r = 0.512, P = 0.018, and r = 0.478, P = 0.028, respectively). The mean ¹²³I-MIBG washout was higher in LV segments 15 mm thick than in LV segments < 15 mm thick (56 ± 10 vs. 49% ± 10%, P = 0.002). Conclusion: In patients with HCM sharing the same causal gene defect, the degree of LV hypertrophy is related to ¹²³I-MIBG washout, suggesting that cardiac adrenergic activity modifies phenotypic expression in HCM.

Key Words: cardiomyopathy • genetics • hypertrophy • MRI • norepinephrine • scintigraphy

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