Role of imaging for diagnosis and management of aortic valve papillary fibroelastoma and cardiac amyloid light chain amyloidosis: a case report.
Cardiac amyloidosis
Case report
Global longitudinal strain
Heart surgery
Papillary fibroelastoma
Speckle-tracking echocardiography
Journal
European heart journal. Case reports
ISSN: 2514-2119
Titre abrégé: Eur Heart J Case Rep
Pays: England
ID NLM: 101730741
Informations de publication
Date de publication:
Aug 2021
Aug 2021
Historique:
received:
07
05
2021
revised:
22
06
2021
accepted:
26
07
2021
entrez:
13
9
2021
pubmed:
14
9
2021
medline:
14
9
2021
Statut:
epublish
Résumé
We report the case of a patient who presented with concomitant aortic valve papillary fibroelastoma (PFE) and cardiac amyloidosis. Although histologically benign, PFE confers an increased thromboembolic risk, and surgical excision is often indicated. However, outcomes of cardiac surgery are poor in patients with cardiac amyloidosis. A 61-year-old man with complaints of dyspnoea and weight loss of 10 kg developing over the past 5 months was evaluated in the cardiology clinic. Echocardiography revealed sessile aortic valve PFE and was also highly suggestive of cardiac amyloidosis. The diagnosis of amyloid light chain amyloidosis secondary to indolent multiple myeloma was eventually confirmed. Therapy with daratumumab, bortezomib, cyclophosphamide, and dexamethasone allowed full remission over a 6-month period and resulted in marked improvement in symptoms and cardiac function as evaluated by global longitudinal strain. Further workup with cerebral magnetic resonance revealed multiple vascular sequelae. Surgical removal of the aortic fibroelastoma with bioprosthetic aortic valve replacement was performed successfully and the patient had an uneventful recovery. Papillary fibroelastoma and cardiac amyloidosis are rare and most likely unrelated entities. Concomitant presentation of both conditions in the same patient presents a unique therapeutic challenge. By allowing cardiac function to be monitored during chemotherapy, speckle-tracking echocardiography can prove instrumental in determining the optimal timing of surgical intervention.
Sections du résumé
BACKGROUND
BACKGROUND
We report the case of a patient who presented with concomitant aortic valve papillary fibroelastoma (PFE) and cardiac amyloidosis. Although histologically benign, PFE confers an increased thromboembolic risk, and surgical excision is often indicated. However, outcomes of cardiac surgery are poor in patients with cardiac amyloidosis.
CASE SUMMARY
METHODS
A 61-year-old man with complaints of dyspnoea and weight loss of 10 kg developing over the past 5 months was evaluated in the cardiology clinic. Echocardiography revealed sessile aortic valve PFE and was also highly suggestive of cardiac amyloidosis. The diagnosis of amyloid light chain amyloidosis secondary to indolent multiple myeloma was eventually confirmed. Therapy with daratumumab, bortezomib, cyclophosphamide, and dexamethasone allowed full remission over a 6-month period and resulted in marked improvement in symptoms and cardiac function as evaluated by global longitudinal strain. Further workup with cerebral magnetic resonance revealed multiple vascular sequelae. Surgical removal of the aortic fibroelastoma with bioprosthetic aortic valve replacement was performed successfully and the patient had an uneventful recovery.
DISCUSSION
CONCLUSIONS
Papillary fibroelastoma and cardiac amyloidosis are rare and most likely unrelated entities. Concomitant presentation of both conditions in the same patient presents a unique therapeutic challenge. By allowing cardiac function to be monitored during chemotherapy, speckle-tracking echocardiography can prove instrumental in determining the optimal timing of surgical intervention.
Identifiants
pubmed: 34514307
doi: 10.1093/ehjcr/ytab322
pii: ytab322
pmc: PMC8422351
doi:
Types de publication
Case Reports
Langues
eng
Pagination
ytab322Informations de copyright
© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.
Références
Herz. 2003 Aug;28(5):453-6
pubmed: 12928745
Amyloid. 2021 Mar;28(1):12-18
pubmed: 32981389
Circ Cardiovasc Imaging. 2016 Aug;9(8):
pubmed: 27511979
Nat Rev Cardiol. 2017 Sep;14(9):536-549
pubmed: 28436488
Eur Heart J Case Rep. 2019 Dec;3(4):1-6
pubmed: 31911987
Heart. 2012 Oct;98(19):1442-8
pubmed: 22865865
Am Heart J. 2003 Sep;146(3):404-10
pubmed: 12947356
Thorac Cardiovasc Surg. 2007 Mar;55(2):65-7
pubmed: 17377855
Gen Thorac Cardiovasc Surg. 2013 Jul;61(7):417-21
pubmed: 23086612
Circulation. 2001 Jun 5;103(22):2687-93
pubmed: 11390338
J Am Coll Cardiol. 2015 Jun 9;65(22):2420-9
pubmed: 26046736
Eur Heart J. 2021 Apr 21;42(16):1554-1568
pubmed: 33825853
Curr Probl Cardiol. 1999 Feb;24(2):57-116
pubmed: 10028128
JACC Cardiovasc Imaging. 2020 Jun;13(6):1384-1391
pubmed: 32061555
Eur J Heart Fail. 2021 Feb;23(2):250-258
pubmed: 32729170