Pulmonary veno-occlusive disease presenting with recurrent pulmonary oedema and the use of nitric oxide to predict response to sildenafil.
Pulmonary veno-occlusive disease (PVOD) is a disorder which causes progressive pulmonary hypertension, usually presenting with worsening dyspnoea and right heart failure. Pulmonary oedema induced by pulmonary vasodilator therapy to reduce pulmonary arterial pressure has been well described in PVOD, but here we describe a case of PVOD presenting with recurrent episodes of acute non-cardiogenic pulmonary oedema, in the absence of significant pulmonary hypertension. Concern over the risk of precipitating pulmonary oedema led us to use inhaled nitric oxide to predict the safety and efficacy of sildenafil.
Creagh-Brown
B C
BC
Adult Intensive Care Unit [corrected] Royal Brompton Hospital, London, UK. drbencb@gmail.com
Nicholson
A G
AG
Showkathali
R
R
Gibbs
J S R
JS
Howard
L S G E
LS
D000328:Adult; D001993:Bronchodilator Agents / Q000627:therapeutic use*; D004417:Dyspnea / Q000209:etiology; D006801:Humans; D008297:Male; D009569:Nitric Oxide / Q000627:therapeutic use*; D010879:Piperazines / Q000627:therapeutic use*; D011654:Pulmonary Edema / Q000209:etiology*; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000188:drug therapy*; D011687:Purines / Q000627:therapeutic use; D012008:Recurrence; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D014665:Vasodilator Agents / Q000627:therapeutic use*
D001993:Bronchodilator Agents; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D009569:Nitric Oxide; D000068677:Sildenafil Citrate