La maladie veino-occlusive pulmonaire

Boucly, Athénaïs; Girerd, Barbara; Bourlier, Delphine; Nemlaghi, S.; Caliez, Julien; Savale, Laurent; Jaïs, Xavier; Dorfmüller, Peter; Simonneau, Gérald; Sitbon, Olivier; Humbert, Marc; Montani, David

doi:10.1016/j.rmr.2017.11.005

Cited by 6 publications

(12 citation statements)

References 49 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…PVOD is a rare form of pulmonary hypertension characterized by preferential remodeling of the pulmonary venules and angioproliferation 3,6 . While PPTHN is a form of precapillary pulmonary hypertension, PVOD is due to a post‐capillary pathology.…”

Section: Discussionmentioning

confidence: 99%

“…While the gold standard to diagnose PVOD would be a surgical lung biopsy, these patients are often too ill to tolerate such a biopsy with a risk of life‐threatening hemorrhage 3 . Diagnosis is made by combining advanced imaging with high‐resolution chest CT (combined picture of pulmonary edema with pulmonary hypertension) and analyzing pulmonary function tests 3,6 . LT remains the only viable option for advanced PVOD compared to PTHN, where initial palliation can be obtained with pulmonary vasodilatory therapy 1,3,7 .…”

Section: Discussionmentioning

confidence: 99%

“…Diagnosis is made by combining advanced imaging with high‐resolution chest CT (combined picture of pulmonary edema with pulmonary hypertension) and analyzing pulmonary function tests 3,6 . LT remains the only viable option for advanced PVOD compared to PTHN, where initial palliation can be obtained with pulmonary vasodilatory therapy 1,3,7 . PVOD can occur either in a sporadic form or as a heritable one with a bi‐allelic EIF2AK4 mutation 6,7 .…”

Section: Discussionmentioning

confidence: 99%

“…End‐stage lung disease due to advanced primary pulmonary hypertension (PPHTN) and pulmonary hypertension due to veno‐occlusive disease (PVOD) may require lung transplantation (LT). While various medical therapies exist for the palliation of PPHTN, no such therapies exist for PVOD, and hence it carries a poorer prognosis 1–3 . Given the lack of alternative therapies for PVOD and the rarity of its diagnosis compared to the more common PPHTN, in this study, we compared the clinical outcomes of LT in the two conditions.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Lung transplantation in primary pulmonary arterial hypertension and pulmonary venous hypertension

Sainathan,

Ryan,

Mullinari

et al. 2023

Clinical Transplantation

View full text Add to dashboard Cite

ObjectivesEnd‐stage lung disease from primary pulmonary hypertension (PPHTN) and pulmonary venous‐occlusive disease (PVOD) may require lung transplantation (LT). While medical therapies exist for the palliation of PPHTN, no therapies exist for PVOD. The study's objective is to compare outcomes of LT in these patients.MethodsPatients with PPHTN and PVOD who had undergone LT were identified in the UNOS database (2005–2022). Univariable analyses compared differences between groups in demographic, clinical, and post‐transplant outcomes. Multivariable logistic regression examined the association between the diagnosis group and survival. Overall survival time between groups was compared using the Kaplan–Meier method.ResultsSix hundred and ninety‐six PPHTN and 78 PVOD patients underwent LT during the study period. Patients with PVOD had lower pulmonary artery mean pressure (47 vs. 53 mmHg, p < .001), but higher cardiac output (4.51 vs. 4.31 L/min, p = .04). PVOD patients were more likely to receive lungs from donation after cardiac death donors (7.7 vs. 2.9%, p = .04). There were no differences in postoperative complications or length of stay. PVOD was associated with superior survival at 30‐day (100 vs. 93%, p = .02) and 90‐day post‐transplant (93 vs. 83%, p = .03), but not at later time points. In multivariable analyses, PVOD and brain death donor use were associated with better survival up to 90‐day mark.ConclusionsPatients undergoing LT for PVOD had better initial survival, which disappeared after 1 year of transplantation. Donation after circulatory death donor use had a short‐term survival disadvantage.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Lung transplantation in primary pulmonary arterial hypertension and pulmonary venous hypertension

Sainathan,

Ryan,

Mullinari

et al. 2023

Clinical Transplantation

View full text Add to dashboard Cite

show abstract

“…It is characterized by progressive occlusion of pulmonary venules, resulting in increased pulmonary vascular resistance and right heart failure. 45 The incidence of PVOD ranges from approximately 0.1 to 0.2 cases per million individuals. The onset of PVOD can occur at any age, with the majority of cases occurring under 50 years old.…”

Section: Overview Of Respiratory Diseasesmentioning

confidence: 99%