Echocardiographic assessment of pulmonary hypertension in Gaucher's disease

Deborah Elstein*, Marc W. Klutstein, Amnon Lahad, Ayala Abrahamov, Irith Hadas-Halpern, Ari Zimran

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

92 Scopus citations

Abstract

Background. Enzyme therapy has been shown to decrease the signs and symptoms of Gaucher's disease. A few patients, however, develop pulmonary hypertension on such treatment. We investigated the frequency of pulmonary hypertension in Gaucher's disease. Methods. We studied 134 adults with type 1 Gaucher's disease, including 73 patients on enzyme replacement, with echocardiography. We measured tricuspid incompetence (TI) with continuous-wave doppler. Pulmonary hypertension was indicated by a TI gradient of more than 30 mmHg. Findings. Nine (7%) patients had pulmonary hypertension: all were treated and six had undergone splenectomy. Chest radiographs confirmed the presence of pulmonary hypertension in these patients as well as in most patients with TI gradients of 25-29 mmHg. Interpretation. The confounding effects of disease severity and splenectomy in many treated patients precluded definitive conclusion of cause and effect. Nonetheless, we found an unexpectedly high rate of pulmonary hypertension and recommended routine echocardiographic monitoring of all treated and untreated patients with type 1 Gaucher's disease. We also suggest consideration of treatment withdrawal if the TI gradient progresses to more than 30 mmHg.

Original languageAmerican English
Pages (from-to)1544-1546
Number of pages3
JournalLancet
Volume351
Issue number9115
DOIs
StatePublished - 23 May 1998
Externally publishedYes

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