Publication Date
8-1-2020
Journal
The Texas Heart Institute Journal
DOI
10.14503/THIJ-18-6799
PMID
33472226
Publication Date(s)
August 2020
Language
English
PMCID
PMC7819432
PubMedCentral® Posted Date
8-20-2020
PubMedCentral® Full Text Version
Post-Print
Published Open-Access
yes
Keywords
Familial amyloid polyneuropathy, heart rupture, heart septal defects, hypotension, ventricular septal rupture
Copyright
This work is licensed under a Creative Commons Attribution-NonCommercial-No Derivative Works 4.0 International License.
Abstract
Cardiac involvement in familial amyloid polyneuropathy consists of arrhythmias, conduction disturbances, and heart failure. To our knowledge, heart rupture has never been described in association with this condition. We report the case of a 62-year-old man with a 6-year history of refractory familial amyloid polyneuropathy who underwent liver transplantation. The operation was complicated by severe hypotension because the neuropathy involved the autonomic system. Perioperatively, the patient had a myocardial infarction, and during the next 10 days, a complete interventricular septal rupture developed, resulting in a systemic-to-pulmonary shunt. Coronary angiographic findings were normal. However, the shunt caused unstable hemodynamics, resulting in cardiogenic shock. An attempt to close the rupture percutaneously failed. The patient underwent successful heart transplantation 50 days later. Macroscopic examination of the explanted heart showed thickening of both ventricles, septal rupture, and a gray scar in the interventricular septum around the cavity. Histopathologic examination revealed intramural amyloid angiopathy. Our case shows that heart rupture can occur in patients with familial amyloid polyneuropathy who have no history of obstructive coronary artery disease, perhaps as a result of tissue fragility caused by amyloid angiopathy. Therefore, autonomic disturbances should be regarded with concern and promptly treated in the perioperative period.