Source: Annual Congress 2001 - Disease of the pulmonary circulation
Disease area: Interstitial lung diseases, Pulmonary vascular diseases

Abstract

An echocardiographic finding of an elevated pulmonary artery pressure leads to most referrals for assessment at our pulmonary hypertension clinic. We report three cases referred primarily because of lung biopsies consistent with pulmonary hypertension, who were subsequently shown to have normal pulmonary artery pressures.
CASE 1:
A 50 year old female presented with a two year history of breathlessness and chest pain. Coronary angiography was normal. Echocardiographic pulmonary artery pressure was 28 mm Hg. Slight impairment of gas transfer led to a lung biopsy, which showed changes of pulmonary hypertension.
CASE 2:
A 61 year old male presented with a two year history of dyspnoea. Echocardiographic pulmonary artery pressure was 35 mm Hg. Although a CT scan showed minimal fibrosis, lung function tests showed marked reduction of gas transfer. A lung biopsy suggested pulmonary hypertension.
CASE 3:
A 51 year old female with lupus and positive anticardio-lipin antibodies presented with a two years of breathlessness. A thoracic CT showed mild nodularity. Her gas transfer was slightly decreased. A lung biopsy suggested pulmonary hypertension.
Each patient's right heart cathether was normal. In cases 1 and 3, despite extensive investigation, the patients's symptoms remain unexplained. In case 2, the unexplained low gas transfer accounts for the symptoms, but this in itself remains unaccounted for. We speculate the histological changes may result from previous small emboli or possibly even previous vaculitis, but investigations have been unable to support these suggestions. Without a specific diagnosis, treatment remains symptomatic. The patients are undergoing close review.


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Citations should be made in the following way:
K. Amshar, I. Armstrong, W. Wallice, T. W. Higenbottam, R. A. Lawson (Sheffield, United Kingdom). When is pulmonary hypertension not pulmonary hypertension?. Eur Respir J 2001; 16: Suppl. 31, 3515

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