Dataset Information

Imaging cardiac innervation in hereditary transthyretin (ATTRm) amyloidosis: A marker for neuropathy or cardiomyopathy in case of heart failure?

ABSTRACT:

Background

Nuclear imaging modalities using ¹²³Iodine-metaiodobenzylguanidine (¹²³I-MIBG) and bone seeking tracers identify early cardiac involvement in ATTRm amyloidosis patients. However, little is known whether results from ¹²³I-MIBG scintigraphy actually correlate to markers for either cardiac autonomic neuropathy or cardiomyopathy.

Methods

All TTR mutation carriers and ATTRm patients who underwent both ¹²³I-MIBG and ^99mTechnetium-hydroxymethylene diphosphonate (^99mTc-HDP) scintigraphy were included. Cardiomyopathy was defined as NT-proBNP > 365 ng/L, and cardiac autonomic neuropathy as abnormal cardiovascular reflexes at autonomic function tests. Late ¹²³I-MIBG heart-to-mediastinum ratio (HMR) < 2.0 or wash-out > 20%, and any cardiac ^99mTc-HDP uptake were considered as abnormal.

Results

39 patients (13 carriers and 26 ATTRm patients) were included in this study. Patients with cardiomyopathy, with or without cardiac autonomic neuropathy, had lower late HMR than similar patients without cardiomyopathy [median 1.1 (range 1.0-1.5) and 1.5(1.2-2.6) vs 2.4 (1.4-3.8) and 2.5 (1.5-3.7), respectively, P < 0.001]. Late HMR and wash-out (inversely) correlated with NT-proBNP r = - 0.652 (P < 0.001) and r = 0.756 (P < 0.001), respectively. Furthermore, late HMR and wash-out (inversely) correlated with cardiac ^99mTc-HDP uptake r = - 0.663 (P < 0.001) and r = 0.617 (P < 0.001), respectively.

Conclusion

In case of heart failure, ¹²³I-MIBG scintigraphy reflects cardiomyopathy rather than cardiac autonomic neuropathy in ATTRm patients and TTR mutation carriers. ¹²³I-MIBG scintigraphy may already be abnormal before any cardiac bone tracer uptake is visible.

SUBMITTER: Jonker DL

PROVIDER: S-EPMC7599160 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature

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Publications

Imaging cardiac innervation in hereditary transthyretin (ATTRm) amyloidosis: A marker for neuropathy or cardiomyopathy in case of heart failure?

Jonker Daphne L DL Hazenberg Bouke P C BPC Nienhuis Hans L A HLA Slart Riemer H J A RHJA Glaudemans Andor W J M AWJM Noordzij Walter W

Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology 20181029 5

<h4>Background</h4>Nuclear imaging modalities using <sup>123</sup>Iodine-metaiodobenzylguanidine (<sup>123</sup>I-MIBG) and bone seeking tracers identify early cardiac involvement in ATTRm amyloidosis patients. However, little is known whether results from <sup>123</sup>I-MIBG scintigraphy actually correlate to markers for either cardiac autonomic neuropathy or cardiomyopathy.<h4>Methods</h4>All TTR mutation carriers and ATTRm patients who underwent both <sup>123</sup>I-MIBG and <sup>99m</sup>Te ...[more]

PMID: 30374850

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Project description:BackgroundTransthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly recognized as a treatable cause of heart failure (HF). Advances in diagnosis and therapy have increased the number of patients diagnosed at early stages, but prognostic data on patients without HF symptoms are lacking. Moreover, it is unknown whether asymptomatic patients benefit from early initiation of transthyretin (TTR) stabilizers.ObjectivesThe aim of this study was to describe the natural history and prognosis of ATTR-CM in patients without HF symptoms.MethodsClinical characteristics and outcomes of patients with ATTR-CM without HF symptoms were retrospectively collected at 6 international amyloidosis centers.ResultsA total of 118 patients (78.8% men, median age 66 years [IQR: 53.8-75 years], 68 [57.6%] with variant transthyretin amyloidosis, mean left ventricular ejection fraction 60.5% ± 9.9%, mean left ventricular wall thickness 15.4 ± 3.1 mm, and 53 [45%] treated with TTR stabilizers at baseline or during follow-up) were included. During a median follow-up period of 3.7 years (IQR: 1-6 years), 38 patients developed HF symptoms (23 New York Heart Association functional class II and 14 functional class III or IV), 32 died, and 2 required cardiac transplantation. Additionally, 20 patients received pacemakers, 13 developed AF, and 1 had a stroke. Overall survival was 96.5% (95% CI: 91%-99%), 90.4% (95% CI: 82%-95%), and 82% (95% CI: 71%-89%) at 1, 3, and 5 years, respectively. Treatment with TTR stabilizers was associated with improved survival (HR: 0.31; 95% CI: 0.12-0.82; P = 0.019) and remained significant after adjusting for sex, age, ATTR-CM type, and estimated glomerular filtration rate (HR: 0.18; 95% CI: 0.06-0.55; P = 0.002).ConclusionsAfter a median follow-up period of 3.7 years, 1 in 3 patients with asymptomatic ATTR-CM developed HF symptoms, and nearly as many died or required cardiac transplantation. Treatment with TTR stabilizers was associated with improved prognosis.

Dataset Information

Imaging cardiac innervation in hereditary transthyretin (ATTRm) amyloidosis: A marker for neuropathy or cardiomyopathy in case of heart failure?

Background

Methods

Results

Conclusion

Publications

Imaging cardiac innervation in hereditary transthyretin (ATTRm) amyloidosis: A marker for neuropathy or cardiomyopathy in case of heart failure?

Similar Datasets

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