A Multi-center Cardiac PYP Scan Registry in Taiwan.

A Multi-center Registry for Clinical Information and 99m Tc-pyrophosphate Scintigraphy in Patients With Suspected Cardiac Amyloidosis in Taiwan.

Amyloid deposition in the heart is called cardiac amyloidosis (CA); 95% is immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Hereditary (ATTRm) or wild-type (ATTRwt) depends on whether the ATTRm gene is mutated or not. The most common mutation in Taiwan is A97S, 80% have left ventricular hypertrophy. The good prognosis depends on early diagnosis and correct treatment strategy.

Bone-avid tracers such as 99mTc-PYP/DPD/HMDP could detect CA. The mechanism is not clear yet, which may be related to the microcalcification. AL amyloidosis is mostly between visual score grade 0-2, and ATTR-CM is usually grade≥2 on PYP scan, or heart to contralateral (H/CL) ratio, and it might replace invasive myocardial biopsy. However, there are no large-scale clinical studies, lack of standardization data, and limited information in comparison between clinical and imaging parameters.

This project will enroll patients with suspected or diagnosed with CA according to CA diagnostic algorithm. Clinical data and image parameters are collected and compared. The project aims to set up prediction models based on the multi-parameters of PYP scan using artificial intelligence technology, including imaging registration and alignment technology, and standardization. We further use the key cardiovascular data elements and imaging-derived database using model training network to extract image features to develop the diagnostic and prognostic prediction models, which are expected to validate the clinical significance and improve patient-centric performance and efficient clinical decision making.

Study Overview

• Status: Not yet recruiting
• Conditions: Heart Failure; Cardiac Amyloidosis
• Intervention / Treatment: Diagnostic test: Tc-99m PYP Cardiac scan

Detailed Description

We will retrospectively and prospectively collect total PYP scans that had been performed on suspected or diagnosed ATTR-CM patients in medical centers in Taiwan and follow-up at least 1 year. All patients are enrolled based on clinical suspicions such as "red-flag" signs using ultrasound, NT-pro BNP and clinical data according to the diagnostic algorithms. In patients with Grade 2 and 3 PYP myocardial uptake, genetic testing is arranged, serum and urine AL tests results will be confirmed. Before reading, the imaging quality will be evaluated (good quality, fair but interpretable, poor but interpretable, poor and insufficient for interpretation). All the images will be visually graded by experienced nuclear medicine physicians with consensus. It helps understand the real-world data.

• Study Type: Observational
• Enrollment (Anticipated): 2000

Contacts and Locations

• Study Contact: Name: Yen-Wen Wu; Phone Number: 1090 886-2-8966-7000; Email: wuyw0502@gmail.com

Study Locations

• Taiwan
  • New Taipei City, Taiwan, 220
    • Far Eastern Memorial Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 20 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patients screened for ATTR-CM

Description

Inclusion Criteria:

1. ≥ 20 years of age
2. Documented diagnosis or r/o of ATTR amyloidosis

Exclusion Criteria:

1. Pregnant or nursing (lactating) women.
2. Important systemic diseases (except heart disease or neurological diseases) such as liver cirrhosis, active malignant tumors, etc., with a life expectancy of less than 6 months.
3. Patient who refused clinical follow up.

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Only
• Time Perspectives: Other

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Death	Death	1 year

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
CV death	CV death	1 year
Hospitalization for heart failure	Hospitalization for heart failure	1 year

Collaborators and Investigators

• Sponsor: Far Eastern Memorial Hospital
• Investigators: Principal Investigator: Yen-Wen Wu, Far Eastern Memorial Hospital

Study record dates

Study Major Dates

• Study Start (Anticipated): November 1, 2022
• Primary Completion (Anticipated): August 29, 2027
• Study Completion (Anticipated): December 31, 2027

Study Registration Dates

• First Submitted: October 20, 2022
• First Submitted That Met QC Criteria: October 20, 2022
• First Posted (Actual): October 25, 2022

Study Record Updates

• Last Update Posted (Actual): October 25, 2022
• Last Update Submitted That Met QC Criteria: October 20, 2022
• Last Verified: October 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Metabolic Diseases; Proteostasis Deficiencies; Amyloidosis
• Other Study ID Numbers: 111229-E

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No