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A case study of likely wild-type cardiac transthyretin amyloidosis causing rapid deterioration
Author(s) -
Thomas Davies,
Aarash Saleh,
Gerry Coghlan,
Carol Whelan,
Banwari Agarwal
Publication year - 2016
Publication title -
journal of the intensive care society
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.551
H-Index - 14
eISSN - 2057-360X
pISSN - 1751-1437
DOI - 10.1177/1751143716682263
Subject(s) - medicine , cardiac amyloidosis , amyloidosis , heart failure , inotrope , cardiology , atrial fibrillation , transthyretin , restrictive cardiomyopathy , cardiomyopathy , carpal tunnel syndrome , diastole , surgery , blood pressure
We present the case of an 88-year-old gentleman who presented to hospital septic with bilateral leg cellulitis, pulmonary oedema and hypotension. He had no history of heart disease but had had bilateral carpal tunnel releases. His condition deteriorated with refractory hypotension in spite of fluid filling, inotropic and vasopressor support. His echocardiogram showed an infiltrative cardiomyopathy with a speckled myocardium, severe concentric left and right ventricular increased wall thickness, diastolic dysfunction, biatrial dilatation and restrictive physiology in keeping with cardiac amyloidosis. He developed atrial fibrillation and worsening respiratory failure due to fluid overload and was intubated and ventilated but continued to decline and passed away. The degree of heart failure in the absence of ischaemia, the patient's advanced age, echocardiographic findings and past history of carpal tunnel syndrome in a male are strongly indicative of a diagnosis of wild-type cardiac transthyretin amyloidosis. We discuss the key features and intensive care management of this disease.

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