Pulmonary Veno-Occlusive Disease Associated with Severe Reduction of Single-Breath Carbon Monoxide Diffusing Capacity

Cg, Elliott; Tv, Colby; Hill, Terrence D.

doi:10.1159/000195438

Cited by 32 publications

(15 citation statements)

References 9 publications

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“…As compared with IPAH, the present data suggest that PVOD is associated with a more pronounced hypoxaemia and a severe reduction of DL,CO, while spirometry and lung volume measurements are within normal limits [36]. These abnormalities may be explained by interstitial oedema and/or pulmonary capillary angiogenesis secondary to chronic pulmonary venous obstruction [37].…”

Section: Discussionmentioning

confidence: 49%

Occult alveolar haemorrhage in pulmonary veno-occlusive disease

Rabiller¹

2006

Eur Respir J

131

111

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Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension that affects predominantly post-capillary pulmonary vessels. A major concern with PVOD is the poor response to available therapies and the risk of pulmonary oedema with continuous intravenous epoprostenol.The present authors hypothesised that alveolar haemorrhage may be a characteristic feature of pulmonary veno-occlusive disease, as compared with other forms of pulmonary arterial hypertension that predominantly involve pre-capillary pulmonary arteries.This paper reports a series of 19 patients with either PVOD (n58) or idiopathic pulmonary arterial hypertension (IPAH; n511) who underwent bronchoalveolar lavage. Cytological analyses were performed and differential counts were made on Perls-stained preparations. The Golde score was used to assess alveolar haemorrhage. As compared with IPAH, PVOD was characterised by a higher percentage of haemosiderin-laden macrophages (40¡37 versus 3¡6%), resulting in elevated Golde scores (81¡88 versus 4¡10).It was concluded that occult alveolar haemorrhage is a common feature of pulmonary venoocclusive disease. Detecting occult alveolar haemorrhage may be of interest in the diagnostic approach of pulmonary veno-occlusive disease.

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Section: Discussionmentioning

confidence: 49%

Occult alveolar haemorrhage in pulmonary veno-occlusive disease

Rabiller¹

2006

Eur Respir J

131

111

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“…Reports of low diffusing capacity of the lung for carbon monoxide (DL,CO) in patients with PAH have previously been published. A low DL,CO has also been described in PVOD patients, with possible severe reductions (,50%) in some patients [7,61]. It could be hypothesised that DL,CO may be normal or increased in PVOD because of the frequent occult alveolar haemorrhage [9].…”

Section: Pulmonary Function Testsmentioning

confidence: 99%

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2008

Eur Respir J

270

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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“…Their clinical presentation is non-specific, mainly characterised by dyspnoea, and they cannot be distinguished neither hemodynamically [9] or by perfusion lung scan [9,10]. However, PVOD has lower arterial oxygen tension, lower nadir oxygen saturation during exercise, and lower diffusing capacity for carbon monoxide and occult pulmonary haemorrhage with haemosiderin-laden macrophages at bronchoalveolar lavage [8,[11][12][13]. A definitive diagnosis of PVOD necessitates surgical lung biopsy, but patients with PVOD often present with advanced disease and surgical lung biopsy is contra-indicated, as it is invasive and at high risk for bleeding, pneumothorax, worsening of respiratory distress, and death [8].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension nosography: are all patients classifiable?

et al. 2017

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A 73-year-old never smoking woman was admitted for exertional dyspnoea to the Respiratory Medicine Section of our hospital in June 2014. The patient's past clinical history revealed systemic arterial hypertension, persistent atrial fibrillation, non-insulin dependent diabetes, hypothyroidism, and hypercholesterolemia. She was taking in treatment with acenocoumarol and metformin. The patient had progressive exercise dyspnoea (NYHA class II) and ankle swelling since 2011. She had been diagnosed with interstitial lung disease with pulmonary hypertension (PAPs of 60 mmHg by transthoracic echocardiography) and hypoxemic respiratory insufficiency. She was referred to our hospital following further worsening of dyspnoea and reduction in exercise tolerance despite long-term oxygen therapy (1 L/min). At admission, she had dyspnoea on minor activities (modified Medical Research CouncilmMRC = 3, NYHA class III), cyanosis, increased respiratory rate (22 breaths/min), and ankle swelling. She had systemic pressure at lower limits of normality (105/ 65 mmHg) and a BMI of 28 (158 cm, 71 kg). At physical examination, fine crackles were audible at both lung bases. She had low (54 mmHg) arterial oxygen tension in oxygen therapy with 1 L/min with CO 2 tension of 50 mmHg, in a framework of compensated respiratory acidosis (HCO 3 -33 mmHg). No alterations of serum electrolytes were found. We got sufficient correction of hypoxia (PaO 2 63 mmHg) by increasing flow oxygen support at 3 L/min with a modest increase of hypercapnia (PaCO 2 54 mmHg). The chest radiograph showed increased cardiac size, bulging of the second arch on the left mediastinal border compatible with enlargement of the pulmonary artery common trunk (Fig. 1). No signs of pulmonary congestion were observed. The electrocardiogram showed atrial fibrillation with average ventricular rate of 77 beats per minute, intraventricular conduction delay, and right axial deviation. Transthoracic echocardiography showed severe tricuspidal regurgitation with TAPSE (tricuspid annular plane systolic excursion) of 15 mm, PAPs of 70 mmHg, right and left atrial enlargement, interventricular septum paradoxical movement, inferior vena cava dilatation with no change on inspiration, mild pericardial effusion, and normal size and wall thickness of the left ventricle, without alteration on the segmental kinesis. Cardiac ejection fraction was preserved (61%). No diastolic relaxation abnormalities were detected. E/A ratio [early transmitral flow (E) and the late flow with atrial contraction (A)] was 0.8 and the ratio E/e 0 [early filling velocity on transmitral Doppler (E) with the early relaxation velocity on tissue Doppler (e 0 )] was 7. Only mild mitral regurgitation was described. Calcification of the cusps of the aortic valve with normal opening was described. Aortic valve peak gradient was 22 mmHg.We decided to treat the patient with furosemide 20 mg iv TID, oxygen therapy (3 L/min) with a partial clinical

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Pulmonary Veno-Occlusive Disease Associated with Severe Reduction of Single-Breath Carbon Monoxide Diffusing Capacity

Cited by 32 publications

References 9 publications

Occult alveolar haemorrhage in pulmonary veno-occlusive disease

Occult alveolar haemorrhage in pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease

Pulmonary hypertension nosography: are all patients classifiable?

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