Evidence map›Paper›PMID 42584373›Full record

ArticleJACC. Case reports2026

p.(V142I)-Associated Variant Transthyretin Amyloid Cardiomyopathy Complicating Complex Congenital Heart Disease: A Lethal Combination.

Zafraan Zathar, Chern Hsiang Choy, Nada Al-Sakini, Jennifer Pinney, Prithwish Banerjee, Dawn L Adamson, Julian D Gillmore, Marianna Fontana, Richard P Steeds, William E Moody

Abstract readCase Reports
In one paragraph

Article in JACC. Case reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors.

Zafraan ZatharMidlands Amyloidosis Service, Centre for Rare Diseases, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.
Chern Hsiang ChoyMidlands Amyloidosis Service, Centre for Rare Diseases, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.
Nada Al-SakiniInstitute of Cardiovascular Sciences, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom.
Jennifer PinneyMidlands Amyloidosis Service, Centre for Rare Diseases, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.
Prithwish BanerjeeDepartment of Cardiology, University Hospitals Coventry and Warwickshire, Coventry, United Kingdom.
Dawn L AdamsonDepartment of Cardiology, University Hospitals Coventry and Warwickshire, Coventry, United Kingdom.
Julian D GillmoreNational Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom.
Marianna FontanaNational Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom.
Richard P SteedsMidlands Amyloidosis Service, Centre for Rare Diseases, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; Institute of Cardiovascular Sciences, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom.
William E MoodyMidlands Amyloidosis Service, Centre for Rare Diseases, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; Institute of Cardiovascular Sciences, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom. Electronic address: william.moody@nhs.net.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundp.(V142I)-associated variant transthyretin amyloid cardiomyopathy (ATTRv-CM) is biologically aggressive and predominantly affects individuals of African ancestry. CASE SUMMARY: A 51-year-old Nigerian man was diagnosed with double outlet right ventricle by the adult congenital heart disease service, although challenging social circumstances hindered adherence to clinic appointments. He re-established regular contact after 3 years and underwent workup for corrective surgery. Cardiac amyloidosis was suspected on repeat echocardiogram and confirmed on cardiac magnetic resonance and bone scintigraphy. Genetic sequencing later identified homozygosity for the p.(V142I) transthyretin variant. He was referred for emergency transplant assessment after developing cardiogenic shock. Unfortunately, he died despite receiving supportive therapies. DISCUSSION: Homozygous p.(V142I)-ATTRv-CM presents earlier and advances more aggressively than its heterozygous counterpart. This unusual case highlights the diagnostic pitfalls when genetics, adult congenital heart disease, and advanced heart failure intersect. TAKE-HOME MESSAGE: In patients of African ancestry, a high index of suspicion for p.(V142I)-ATTRv-CM is needed to prompt early diagnosis and initiation of disease-modifying therapies.

Indexed as

adult congenital heart diseasep.(V142I) varianttransthyretin amyloid cardiomyopathy

Identifiers

PMID42584373
PMCPMC13616560

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