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Peer-reviewedCC BY 4.0JACC: Case Reports

Cardiopulmonary Exercise Testing Identifies Treatable ATTR Cardiac Amyloidosis

  • Sara Rioja-Abad, MD1
  • Ramon Andion-Ogando, MD1
  • Maria Perez-Diaz, MD1
  • Cristina Barinaga-Martin, MD1
  • Jordi Candela-Ferre, MD1
  • Maria Jesus Rollan-Gomez, MD, PhD1
  • Ignacio Hernandez-Gonzalez, MD, PhD1
  • Maria del Mar de la Torre-Carpente, MD, PhD1
  • Hector Garcia-Pardo, MD1
  • Juan Carlos Muñoz-San Jose, MD, PhD1
  1. 1Department of Cardiology, Rio Hortega University Hospital, Valladolid, Spain
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Abstract

Background: Transthyretin cardiac amyloidosis (ATTR-CM) is a progressive infiltrative cardiomyopathy where early disease-modifying therapy improves outcomes. However, treatment access may be restricted by biomarker thresholds not reflecting true functional impairment. Case Summary: A 73-year-old man with exertional dyspnea (NYHA functional class II) and confirmed wild-type ATTR-CM did not meet reimbursement criteria for tafamidis because N-terminal pro–B-type natriuretic peptide was below the required threshold (≥600 pg/mL), despite impaired functional capacity on cardiopulmonary exercise testing (CPET). Acoramidis was obtained through expanded access. At 6-month follow-up, the patient showed clinical stability and improved exercise capacity. Discussion: Fixed N-terminal pro–B-type natriuretic peptide cutoffs may exclude patients with early-stage heart failure who would benefit from treatment. CPET helps identify therapy candidates below biomarker thresholds. Take-Home Messages: CPET detects functional impairment in ATTR-CM even when natriuretic peptides remain low. Early transthyretin-stabilizer initiation in NYHA functional class I-II yields better outcomes; treatment eligibility should integrate functional assessment beyond biomarkers alone.

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Cite

BibTeXRIS
BibTeX
@article{sarariojaabad2026cardiopulmonary,
  title = {{Cardiopulmonary Exercise Testing Identifies Treatable ATTR Cardiac Amyloidosis}},
  author = {Sara Rioja-Abad, MD and Ramon Andion-Ogando, MD and Maria Perez-Diaz, MD and Cristina Barinaga-Martin, MD and Jordi Candela-Ferre, MD and Maria Jesus Rollan-Gomez, MD, PhD and Ignacio Hernandez-Gonzalez, MD, PhD and Maria del Mar de la Torre-Carpente, MD, PhD and Hector Garcia-Pardo, MD and Juan Carlos Muñoz-San Jose, MD, PhD},
  journal = {JACC: Case Reports},
  year = {2026},
  month = aug,
  doi = {10.1016/j.jaccas.2026.110006},
  url = {https://doi.org/10.1016/j.jaccas.2026.110006},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Cardiopulmonary Exercise Testing Identifies Treatable ATTR Cardiac Amyloidosis
AU  - Sara Rioja-Abad, MD
AU  - Ramon Andion-Ogando, MD
AU  - Maria Perez-Diaz, MD
AU  - Cristina Barinaga-Martin, MD
AU  - Jordi Candela-Ferre, MD
AU  - Maria Jesus Rollan-Gomez, MD, PhD
AU  - Ignacio Hernandez-Gonzalez, MD, PhD
AU  - Maria del Mar de la Torre-Carpente, MD, PhD
AU  - Hector Garcia-Pardo, MD
AU  - Juan Carlos Muñoz-San Jose, MD, PhD
PY  - 2026
DA  - 2026/08/26
JO  - JACC: Case Reports
DO  - 10.1016/j.jaccas.2026.110006
UR  - https://doi.org/10.1016/j.jaccas.2026.110006
AB  - Background: Transthyretin cardiac amyloidosis (ATTR-CM) is a progressive infiltrative cardiomyopathy where early disease-modifying therapy improves outcomes. However, treatment access may be restricted by biomarker thresholds not reflecting true functional impairment. Case Summary: A 73-year-old man with exertional dyspnea (NYHA functional class II) and confirmed wild-type ATTR-CM did not meet reimbursement criteria for tafamidis because N-terminal pro–B-type natriuretic peptide was below the required threshold (≥600 pg/mL), despite impaired functional capacity on cardiopulmonary exercise testing (CPET). Acoramidis was obtained through expanded access. At 6-month follow-up, the patient showed clinical stability and improved exercise capacity. Discussion: Fixed N-terminal pro–B-type natriuretic peptide cutoffs may exclude patients with early-stage heart failure who would benefit from treatment. CPET helps identify therapy candidates below biomarker thresholds. Take-Home Messages: CPET detects functional impairment in ATTR-CM even when natriuretic peptides remain low. Early transthyretin-stabilizer initiation in NYHA functional class I-II yields better outcomes; treatment eligibility should integrate functional assessment beyond biomarkers alone.
SN  - 2666-0849
ER  -