Pulmonary hypertension in patients with amyloidosis

Research output: Contribution to journalArticle

47 Citations (Scopus)

Abstract

Pulmonary hypertension (PH) with right-sided cardiac failure is a rare complication of amyloidosis, and its natural history is not well-defined. The aim of our study was to evaluate patients who were seen at our institution who had PH and amyloidosis and to describe the natural history of this complication. The study was a retrospective chart review of patients seen at the Mayo Clinic with both PH and amyloidosis listed as major diagnoses between January 1, 1980, and December 31, 1999. Patients with known causes of PH were excluded. Five patients met our criteria (four women and one man). Four patients had light-chain amyloidosis and one had amyloid A deposition secondary to familial Mediterranean fever. All patients had symptoms related to PH without echocardiographic evidence of left ventricular dysfunction. The median survival time after the diagnosis of amyloidosis was 2.8 years, and PH was found a median of 73 days before death. Five patients died of cardiac complications, including one with sudden cardiac death. PH is an unusual complication of amyloidosis. Patients develop PH late in the disease process and do not have a worse prognosis compared to other patients with cardiac amyloidosis. PH is a marker of advanced amyloidosis.

Original languageEnglish (US)
Pages (from-to)1735-1738
Number of pages4
JournalChest
Volume120
Issue number5
DOIs
StatePublished - 2001

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Amyloidosis
Pulmonary Hypertension
Natural History
Familial Mediterranean Fever
Sudden Cardiac Death
Left Ventricular Dysfunction
Amyloid
Heart Failure
Light
Survival

Keywords

  • Amyloidosis
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Pulmonary hypertension in patients with amyloidosis. / Dingli, David M; Utz, James P; Gertz, Morie.

In: Chest, Vol. 120, No. 5, 2001, p. 1735-1738.

Research output: Contribution to journalArticle

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