Profil évolutif de la dénervation cardiaque sympathique dans l’amylose héréditaire à transthyrétine

Abstract

IntroductionCardiac amyloidosis is a rare disease with poor prognosis, requiring an early diagnosis. I123-MIBG plays an important role in early evaluation of sympathetic cardiac innervation, which is decreased due to amyloid infiltration. The aim of this study was to assess the temporal evolution of cardiac denervation in patients with hereditary amyloidosis, particularly the rate of progression and regional abnormalities; and to identify progression markers. MethodsForty-six patients were included. All of them were carriers of a TTR mutation or received domino liver transplantation, and underwent at least two evaluations of cardiac innervation with MIBG, between February 2011 and February 2018. Progression of cardiac denervation was determined by comparing the H/M ratio at first and final assessment. Regional abnormalities were evaluated using the perfusion/innervation mismatch on a 17 segments model. Logistic regression analysis was used to identify predictors of progression. ResultsTwenty-two patients were stable and 24 were progressive. The mean delay between two MIBG scans was 3.2±1.3 years. Late H/M decreased by 3.9%/year in progressive group, and tended to accelerate after symptoms onset (−1.4±5.4 vs−0.49±2.9, P=0.03). Regional abnormalities were initally located in the infero-latéral segments and extended to the inferior and lateral walls at final assessment. LVEF (OR: 0.90, P=0.003) and initiation of amyloidosis targeted treatment (OR=5,35, P=0.003) were independent predictors of sympathetic denervation progression. ConclusionProgression of myocardial sympathetic denervation in hereditary amyloidosis is slow, but accelerates after symptoms onset. LVEF and treatment are potential progression markers. Regional abnormalities are mainly located in the inferolateral segments and tend to extend at the borders of the denervated area.