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Tytuł:
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Interventricular Septal Rupture in a 62-Year-Old Man With Familial Amyloid Polyneuropathy.
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Autorzy:
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Pidello S; Division of Cardiology, Città della Salute e della Scienza, University Hospital of Torino, 10126 Torino, Italy.
Simonato E; Division of Cardiac Surgery, Città della Salute e della Scienza, University Hospital of Torino, 10126 Torino, Italy.
Orzan F; Division of Cardiology, Città della Salute e della Scienza, University Hospital of Torino, 10126 Torino, Italy.
Frea S; Division of Cardiology, Città della Salute e della Scienza, University Hospital of Torino, 10126 Torino, Italy.
Barreca A; Division of Pathology, Città della Salute e della Scienza, University Hospital of Torino, 10126 Torino, Italy.
Rinaldi M; Division of Cardiac Surgery, Città della Salute e della Scienza, University Hospital of Torino, 10126 Torino, Italy.
Boffini M; Division of Cardiac Surgery, Città della Salute e della Scienza, University Hospital of Torino, 10126 Torino, Italy.
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Źródło:
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Texas Heart Institute journal [Tex Heart Inst J] 2020 Aug 01; Vol. 47 (4), pp. 302-305.
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Typ publikacji:
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Case Reports; Journal Article
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Język:
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English
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Imprint Name(s):
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Original Publication: Houston, TX : published in the Cardiovascular Surgical Research Laboratories, Texas Heart Institute, c1982-
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MeSH Terms:
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Amyloid Neuropathies, Familial/*complications
Ventricular Septal Rupture/*etiology
Amyloid Neuropathies, Familial/diagnosis ; Cardiac Surgical Procedures/methods ; Echocardiography ; Electrocardiography ; Humans ; Male ; Middle Aged ; Ventricular Septal Rupture/diagnosis ; Ventricular Septal Rupture/surgery
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References:
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Ann Thorac Surg. 2001 Dec;72(6):2132-4. (PMID: 11789816)
Eur J Echocardiogr. 2009 Jun;10(4):585-7. (PMID: 19339261)
Rev Port Cardiol. 1999 Jul-Aug;18(7-8):689-97. (PMID: 10466370)
Amyloid. 2011 Sep;18(3):160-4. (PMID: 21504342)
JAMA. 1983 Mar 11;249(10):1322-4. (PMID: 6600795)
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Amyloid. 2009 Dec;16(4):221-5. (PMID: 19922334)
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Contributed Indexing:
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Keywords: Familial amyloid polyneuropathy; heart rupture; heart septal defects; hypotension; ventricular septal rupture
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Entry Date(s):
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Date Created: 20210120 Date Completed: 20210906 Latest Revision: 20221011
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Update Code:
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20240105
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PubMed Central ID:
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PMC7819432
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DOI:
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10.14503/THIJ-18-6799
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PMID:
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33472226
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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.
(© 2020 by the Texas Heart® Institute, Houston.)