Axel Van Der Gucht1,2, Anne-Ségolène Cottereau3,4, Mukedaisi Abulizi3,4, Aziz Guellich3,5,6,7, Paul Blanc-Durand3,4, Jean-Marc Israel3,4, Arnault Galat3,5,6,7, Violaine Plante-Bordeneuve3,5,7,8, Jean-Luc Dubois-Randé3,5,6,7, Diane Bodez3,5,6,7, Jean Rosso3,4,5, Thibaud Damy3,5,6,7, Emmanuel Itti3,4,5,7. 1. Mondor Amyloidosis Network, Créteil, France. axel.vandergucht@gmail.com. 2. Department of Nuclear Medicine, AP-HP, Henri Mondor Teaching Hospital, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France. axel.vandergucht@gmail.com. 3. Mondor Amyloidosis Network, Créteil, France. 4. Department of Nuclear Medicine, AP-HP, Henri Mondor Teaching Hospital, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France. 5. DHU ATVB, Paris Est University, Créteil, France. 6. Department of Cardiology, AP-HP, Henri Mondor Teaching Hospital, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France. 7. INSERM U955, GRC Amyloid Research Institute, Créteil, France. 8. Department of Neurology, AP-HP, Henri Mondor Teaching Hospital, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France.
Abstract
BACKGROUND: A decreased longitudinal strain in basal segments with a base-to-apex gradient has been described in patients with cardiac amyloidosis (CA). OBJECTIVES: Aim was to investigate the left ventricular (LV) regional distribution of early-phase 99mTc-Hydroxymethylene diphosphonate (99mTc-HMDP) uptake in patients with transthyretin-related cardiac amyloidosis (TTR-CA). METHODS: All patients underwent a whole-body planar 99mTc-HMDP scintigraphy acquired at 10-min post-injection (early-phase) followed by a thorax SPECT/CT. The segmental uptake (expressed as % of maximal myocardial HMDP uptake) was investigated on the AHA 17-segment model and 3-segment model (basal, mid-cavity, apical). RESULTS: Sixty-one TTR-CA patients were included of whom 29 were wild-type (wt-TTR-CA) and 32 had hereditary TTR-CA (m-TTR-CA). Early myocardial 99mTc-HMDP uptake occurred in all TTR-CA. In all patients, segmental analysis of the LV myocardial distribution of 99mTc-HMDP uptake showed an increased median uptake (interquartile range) in basal/mid-cavity segments compared to the lowest median uptake of apical segments (respectively, 79% [72%-86%] vs. 72% [64%-81%]; P < 10-6). This pattern was similar in wt-TTR-CA group (78% [70%-84%] vs. 70% [61%-81%]; P < 10-6), in m-TTR-CA group (80% [74%-86%] vs. 73 [66%-82%]; P < 10-7) and remained constant independently of the TTR mutation subtype with P ranging 10-5 to 0.03. CONCLUSIONS: Early-phase myocardial scintigraphy identified regional distribution of 99mTc-HMDP uptake characterized by a base-to-apex gradient, corroborating echocardiographic, and cardiac magnetic resonance findings. This apical sparing pattern was similar across TTR-CA and TTR mutation subtypes.
BACKGROUND: A decreased longitudinal strain in basal segments with a base-to-apex gradient has been described in patients with cardiac amyloidosis (CA). OBJECTIVES: Aim was to investigate the left ventricular (LV) regional distribution of early-phase 99mTc-Hydroxymethylene diphosphonate (99mTc-HMDP) uptake in patients with transthyretin-related cardiac amyloidosis (TTR-CA). METHODS: All patients underwent a whole-body planar 99mTc-HMDP scintigraphy acquired at 10-min post-injection (early-phase) followed by a thorax SPECT/CT. The segmental uptake (expressed as % of maximal myocardial HMDP uptake) was investigated on the AHA 17-segment model and 3-segment model (basal, mid-cavity, apical). RESULTS: Sixty-one TTR-CA patients were included of whom 29 were wild-type (wt-TTR-CA) and 32 had hereditary TTR-CA (m-TTR-CA). Early myocardial 99mTc-HMDP uptake occurred in all TTR-CA. In all patients, segmental analysis of the LV myocardial distribution of 99mTc-HMDP uptake showed an increased median uptake (interquartile range) in basal/mid-cavity segments compared to the lowest median uptake of apical segments (respectively, 79% [72%-86%] vs. 72% [64%-81%]; P < 10-6). This pattern was similar in wt-TTR-CA group (78% [70%-84%] vs. 70% [61%-81%]; P < 10-6), in m-TTR-CA group (80% [74%-86%] vs. 73 [66%-82%]; P < 10-7) and remained constant independently of the TTR mutation subtype with P ranging 10-5 to 0.03. CONCLUSIONS: Early-phase myocardial scintigraphy identified regional distribution of 99mTc-HMDP uptake characterized by a base-to-apex gradient, corroborating echocardiographic, and cardiac magnetic resonance findings. This apical sparing pattern was similar across TTR-CA and TTR mutation subtypes.
Entities:
Keywords:
HMDP; SPECT/CT; apical sparing; cardiac amyloidosis; protein deposition; transthyretin
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