Massive Pulmonary Edema and Death After Prostacyclin Infusion in a Patient With Pulmonary Veno-occlusive Disease

Palmer, Scott M.; Robinson, Lisa J.; Wang, Andrew; Gossage, James R.; Bashore, Thomas M.; Tapson, Victor F.

doi:10.1378/chest.113.1.237

Cited by 223 publications

(127 citation statements)

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“…One of the main concerns in PVOD is the risk of pulmonary oedema with continuous intravenous epoprostenol and other specific PAH therapies [4,7,60]. Pulmonary oedema has been described both following vasodilator testing [7,60] and at varying intervals after initiation of specific PAH therapies [4].…”

Section: Acute Vasodilator Testingmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2008

Eur Respir J

272

250

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Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.KEYWORDS: Alveolar haemorrhage, BMPR2, computed tomography, diffusing capacity of the lung for carbon monoxide, pulmonary arterial hypertension, pulmonary veno-occlusive disease P ulmonary arterial hypertension (PAH) is a severe condition characterised by elevated pulmonary artery pressure leading to right heart failure and death [1,2]. Pulmonary veno-occlusive disease (PVOD) is classified as a subgroup of PAH and accounts for 5-10% of histological forms of cases initially considered to be idiopathic PAH. Even though the first welldocumented case of PVOD was described .70 yrs ago, the characteristics and pathophysiology of this disease remain poorly understood [3,4]. While pulmonary vascular pathology of idiopathic or familial PAH is characterised by a major remodelling of small pre-capillary pulmonary arteries with typical plexiform and/or thrombotic lesions, PVOD preferentially affects the post-capillary venous pulmonary vessels [5,6]. Despite this anatomical histological difference, PVOD has a very similar clinical presentation to PAH but is characterised by a worse prognosis and the possibility that severe pulmonary oedema can develop with specific PAH therapy, justifying the importance of diagnosing this disease. A definitive diagnosis of PVOD requires histological analysis of a lung sample [7,8]; however, surgical lung biopsy is a high-risk procedure in these patients and the development of a less invasive diagnostic approach would be preferable [9][10][11]. The present manuscript will summa...

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Section: Acute Vasodilator Testingmentioning

confidence: 99%

“…Pulmonary oedema has been described both following vasodilator testing [7,60] and at varying intervals after initiation of specific PAH therapies [4]. The development of pulmonary oedema is extremely suggestive of PVOD in a context of PAH.…”

Section: Acute Vasodilator Testingmentioning

confidence: 99%

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2008

Eur Respir J

272

250

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“…epoprostenol or adenosine in PVOD may lead to the development of sometimes fatal pulmonary oedema [5,14]. The mechanism of oedema is probably related to pulmonary arterial vasodilation without concomitant pulmonary venodilation, resulting in increased transcapillary hydrostatic forces and transudation of fluid into the pulmonary interstitium.…”

Section: Discussionmentioning

confidence: 99%

Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture

Dwyer¹,

Smith²,

Challis³

et al. 2009

Eur Respir J

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Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension characterised by extensive fibrotic occlusion of pulmonary veins. PVOD has a similar insidious presentation to idiopathic pulmonary arterial hypertension but responds poorly to conventional therapies and has a worse prognosis.The current study reports the case of a Caucasian female with a long history of progressive dyspnoea ultimately diagnosed as focal granulomatous venulitis leading to a pulmonary venoocclusive disease-like pathology. The present study highlights the challenges in diagnosing and treating this condition.KEYWORDS: Acute pulmonary oedema, bosentan, granulomatous venulitis, infliximab, pulmonary veno-occlusive disease, sildenafil CASE REPORT A 43-yr-old female was extensively investigated because of progressive exercise induced fatigue and breathlessness without wheeze or cough. The patient was otherwise well. She ceased smoking at the time of symptom onset, having accumulated a 25 pack-yr smoking history. There was no relevant family history or occupational and drug exposures. There were no abnormal examination findings early in the natural history of the disease. Arterial oxygen saturation (Sa,O 2 ) at rest was 97% on room air.A chest radiograph (CXR), electrocardiogram, echocardiogram and myocardial thallium scan were all unremarkable. Lung function tests demonstrated a mild degree of airflow obstruction with no reversibility: forced expiratory volume in one second (FEV1) 2.15 L (85% predicted); forced vital capacity (FVC) 2.84 L (94% pred); FEV1/FVC ratio 76%; mean forced expiratory flow between 25 and 75% FVC 61% pred; diffusing capacity of the lung for carbon monoxide (DL,CO) 94% pred. A histamine challenge showed a provocation concentration causing a fall in FEV1 of 20% of 0.825 mg?mL -1 , which is consistent with severe airway hyperresponsiveness. A provisional diagnosis of asthma prompted a 3-week trial of oral prednisolone and introduction of a corticosteroid/long-acting b 2 -agonist combination inhaler device. This intervention was ineffective and did not improve lung function tests. A cardiopulmonary exercise test was aborted due to severe fatigue at 87% of predicted workload. The maximal oxygen uptake (V9O 2, max) was 93% of predicted with no exercise induced fall in SaO 2 . The patient was then lost to follow-up.The patient re-presented with worsening breathlessness and tender, erythematous nodules on her legs consistent with a diagnosis of erythema nodosum 3 yrs later. The cutaneous lesions resolved spontaneously without any treatment. A ventilation/perfusion (V9/Q9) scan was normal. High-resolution computed tomography (HRCT) of the lungs showed mild expiratory gas trapping with patchy peripheral bronchocentric opacities and minor septal thickening, but no mediastinal lymphadenopathy. Her serum biochemistry and full blood count were normal, erythrocyte sedimentation rate (ESR) was elevated at 41 mm?h -1 (normal rate ,15 mm?h -1 ) and angiotensin-converting enzyme level was 21.5 IU?L -1 (norm...

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“…Administration of this medication requires a central line and a pump. Treatment with epoprostenol causes several side effects, with central line infection being a serious side effect (Palmer et al, 1998. Other stable prostacyclins such as Treprostinil and Iloprost are also used in the treatment of PAH.…”

Section: Current Treatments and New Therapeutic Strategies Using Omegmentioning

confidence: 99%

ω3 and ω6 CYP450 Eicosanoid Derivatives: Key Lipid Mediators in the Regulation of Pulmonary Hypertension

Morin¹,

Fortin²,

Guibert³

et al. 2011

Pulmonary Hypertension - From Bench Research to Clinical Challenges

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Massive Pulmonary Edema and Death After Prostacyclin Infusion in a Patient With Pulmonary Veno-occlusive Disease

Cited by 223 publications

References 10 publications

Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease

Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture

ω3 and ω6 CYP450 Eicosanoid Derivatives: Key Lipid Mediators in the Regulation of Pulmonary Hypertension

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Massive Pulmonary Edema and Death After Prostacyclin Infusion in a Patient With Pulmonary Veno-occlusive Disease

Cited by 223 publications

References 10 publications

Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease

Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture

&#969;3 and &#969;6 CYP450 Eicosanoid Derivatives: Key Lipid Mediators in the Regulation of Pulmonary Hypertension

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ω3 and ω6 CYP450 Eicosanoid Derivatives: Key Lipid Mediators in the Regulation of Pulmonary Hypertension