Pulmonary veno-occlusive disease: advances in clinical management and treatments

Figures & data

Figure 1. Pulmonary vascular lesions in a patient suffering from pulmonary veno-occlusive disease.

(A) Vascular remodeling in a patient with pulmonary veno-occlusive disease (PVOD): septal vein displaying loose fibrosis with cushion-like narrowing of the native lumen. (B) Vascular remodeling in a patient with PVOD: preseptal venules with occlusive intimal fibrosis; note the partial thickening of alveolar septa due to focal multiplication of alveolar capillaries. (C) Vascular remodeling in a patient with PVOD: associated precapillary remodeling in the same patient with typical hyperplasia of small arterioles; note numerous intra-alveolar macrophages and capillary filling/multiplication as indirect signs of congestion due to post-capillary obstruction. (A–C) Hematoxylin-eosin stained, magnification ×100.

Figure 2. Measurement of pulmonary capillary wedge pressure in pulmonary veno-occlusive disease

This diagram explains why pulmonary capillary wedge pressure (PCWP) is usually normal in pulmonary veno-occlusive disease (PVOD). PVOD mostly affects small pulmonary veins, leading to an elevation of pressure in this region (Pv), as well as to an elevation in true pulmonary capillary pressure (Pc) and precapillary pulmonary arterial pressure (Pa). Larger pulmonary veins are usually not affected by PVOD, and it is in fact the pressure here that is reflected by PCWP: the static column of blood (hatched) occluded by pulmonary arterial catheter wedging or balloon inflation of a pulmonary arterial branch (balloon 1) reflects the pressure in a vein of similar diameter (balloon 2), usually of a larger size than those vessels affected by PVOD. Therefore, this measurement technique does not reflect the important elevation of pressure in the smaller diameter vessels associated with PVOD.

Reprinted with permission from [5].

Figure 3. High-resolution computed tomography of the chest in pulmonary veno-occlusive disease.

(A) High-resolution computed tomography of the chest showing marked ground-glass opacities with centrilobular pattern, ground-glass opacities with poorly defined nodular opacities, septal lines. (B) High-resolution computed tomography of the chest showing mediastinal lymph node enlargement.

Table 1. Selected characteristics of idiopathic pulmonary arterial hypertension and idiopathic pulmonary veno-occlusive disease.

	Idiopathic PAH	PVOD
Genetic mutations	BMPR2 mutations in 10–40% of cases	Cases of BMPR2 mutation
Epidemiology/risk factors
Female predominance	Yes (female:male = 2:1)	No (female:male = 1:1)
Tobacco exposure	Unrelated	More frequent and higher exposure than in iPAH
Chemotherapy	Case reports	Case reports
Clinical exam
Auscultatory crackles	No	Possible
Clubbing	Possible	Possible
Hemoptysis	Possible	Possible
Pulmonary function tests
FEV1, FVC, TLC	Normal (possible mild restrictive pattern)	Normal (possible mild restrictive pattern)
DLCO and DLCO/VA	Often reduced	Lower than iPAH
PaO2 at rest	Often reduced	Lower than iPAH
High-resolution CT scan of the chest	Normal (important to exclude other causes of PH)	Frequent association of abnormalities including centrilobular ground-glass opacities, septal lines and lymph node enlargement
Bronchoalveolar lavage	Normal	Possible occult alveolar hemorrhage
Management
Acute NO testing	When positive, it is predictive of long-term CCB response and better prognosis	Not a predictor of CCB response (risk of pulmonary edema after initiation of CCB)
Response to PAH therapy	Improve hemodynamics, functional status and outcome	May deteriorate PVOD with a risk of pulmonary edema. Cases of transient stabilization (bridge therapy to LTx)

CCB: Calcium channel blockers; DL_CO: Diffusing capacity of the lung for carbon monoxide; FEV₁: Forced expiratory volume in 1 s; FVC: Forced vital capacity; LTx: Lung transplantation; NO: Nitric oxide; PAH: Pulmonary arterial hypertension; PaO₂: Partial pressure of arterial oxygen; PVOD: Pulmonary veno-occlusive disease; SpO₂: Pulsoximetry saturation of O₂; TLC: Total lung capacity; VA: Alveolar volume.

Table reproduced with permission from [93].

Activity Evaluation: Where 1 is strongly disagree and 5 is strongly agree

	1	2	3	4	5
1. The activity supported the learning objectives.
2. The material was organized clearly for learning to occur.
3. The content learned from this activity will impact my practice.
4. The activity was presented objectively and free of commercial bias.

Montani D, Price LC, Dorfmuller P et al. Pulmonary veno-occlusive disease. Eur. Respir. J.33(1), 189–200 (2009).

 PubMed Web of Science ®Google Scholar

Montani D, Kemp K, Dorfmuller P, Sitbon O, Simonneau G, Humbert M. Idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease. Semin. Respir. Crit. Care Med.30, 411–420 (2009).

 Google Scholar