Pulmonary Veno-occlusive Disease

Holcomb, Barry W.; Loyd, James E.; Ely, E. Wesley; Johnson, Joyce E.; Robbins, Ivan M.

doi:10.1378/chest.67.4.487

Cited by 88 publications

(26 citation statements)

References 24 publications

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“…One possible reason for the better prognosis in recent reports, including ours, may be milder disease severity. In fact, in an early study by Holcomb et al, 3 the MPAP of the patients was 65 mmHg, whereas in two recent studies, it was lower at 58 mmHg and 46 mmHg. 4 , 15 In the present study, MPAP at diagnosis was 42 mmHg, suggesting that the disease may have been milder or in its early stages.…”

Section: Discussionmentioning

confidence: 84%

“…Pulmonary edema is the most critical side effect of vasodilator use in PVOD/PCH. In previous reports, the incidence of pulmonary edema varied widely, ranging from <10% 7 to 20%–60%, 3 , 4 , 15 and up to 100%. 14 In the present study, pulmonary edema occurred in 20% of patients, suggesting that the risk of pulmonary edema associated with oral pulmonary vasodilators is not particularly high.…”

Section: Discussionmentioning

confidence: 96%

“…The prognosis of PVOD/PCH is reportedly very poor. For example, in one early study, the 1‐year survival rate was 28% 3 ; in another, the mean survival time was 12 months. 15 However, the survival has improved according to more recent studies.…”

Section: Discussionmentioning

confidence: 99%

“… 4 Additionally, the difference may have been due to variation in treatments. In previous reports, oral calcium channel blockers or intravenous prostaglandin I 2 were primarily used, 3 , 15 whereas in recent reports, current oral pulmonary vasodilators have been more prevalent. 4 , 9 , 10 Finally, the involvement of reporting/publication bias should be considered.…”

Section: Discussionmentioning

confidence: 99%

“… 1 , 2 The prevalence of PVOD/PCH is extremely low and its outcomes remain dismal in contrast with other types of pulmonary hypertension (PH). 3 , 4 …”

mentioning

confidence: 99%

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Efficacy and safety of oral pulmonary vasodilators in pulmonary veno‐occlusive disease

et al. 2022

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Pulmonary veno‐occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH) is a rare subtype of pulmonary hypertension with dismal prognosis. Limited data are available on the efficacy and safety of orally administered pulmonary vasodilators for PVOD/PCH. Whether and how systemic sclerosis (SSc) affects the clinical outcomes of PVOD/PCH is also unknown. This study aimed to determine the clinical and hemodynamic efficacy and safety of oral pulmonary vasodilators for PVOD/PCH and clarify the possible effects of SSc on the clinical presentation of PVOD/PCH. We retrospectively analyzed the clinical data of 15 patients with PVOD/PCH treated with oral pulmonary vasodilators in our department since 2001. Six of them had SSc. Oral pulmonary vasodilators were administered either as single agents ( n = 10) or in combination ( n = 5). Treatment improved the functional class of five patients, and pulmonary arterial pressure and pulmonary vascular resistance decreased by 10 ± 12 mmHg and 36 ± 19%, respectively ( p < 0.05 for both, n = 13), whereas pulmonary edema developed in three patients. The mean survival was 3.9 years, and the 1‐ and 3‐year survival rates were 93% and 65%, respectively. The clinical presentation, including survival, was similar between patients with and without SSc. In our PVOD/PCH cohort, oral pulmonary vasodilators caused pulmonary edema in 20% of patients, but more than 80% of patients experienced significant pulmonary vasodilatory effects, and the overall prognosis was better than that previously reported. SSc does not adversely affect the clinical outcomes of PVOD/PCH.

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Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“… 1 , 2 The prevalence of PVOD/PCH is extremely low and its outcomes remain dismal in contrast with other types of pulmonary hypertension (PH). 3 , 4 …”

mentioning

confidence: 99%

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Efficacy and safety of oral pulmonary vasodilators in pulmonary veno‐occlusive disease

et al. 2022

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When you hear hoofbeats, think zebras – pulmonary veno‐occlusive disease: A case report

et al. 2022

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Pulmonary veno‐occlusive disease (PVOD) is a rare disease. It may be idiopathic or associated, in particular, with connective tissue disease, or it may develop after radiation exposure; in heritable forms of PVOD, the inheritance is autosomal recessive due to the presence of homozygous or compound heterozygous pathogenic variants in the EIF2AK4 gene. We describe the case of a young man whose PVOD was initially misdiagnosed as chronic thromboembolic pulmonary hypertension despite worsening after riociguat, nonspecific computed tomography pulmonary angiogram findings, and parental consanguinity could suggest an autosomal recessive disease. The correct diagnosis and the correct treatment are crucial given the high mortality rate of this disease.

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CT imaging of peripheral pulmonary vessel disease

2005

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The diseases concerning the small pulmonary vessels are difficult to diagnose. Pathologic findings are rarely limited to the small vessels, and a continuum between the involvement of small and large vessels is frequent. Moreover, small vessels can be affected by various disease entities with overlapping radiologic features and a wide spectrum of clinical manifestations. Nevertheless, these various entities can be easily separated into two different groups by imaging techniques, particularly by computed tomography: obstructive and inflammatory diseases. Radiologic findings of obstructive diseases are relatively constant, dominated by the manifestation of pulmonary hypertension. In contrast, radiologic manifestations of inflammatory diseases are often florid and nonspecific. After a recall of the classification of small vessel diseases and the imaging techniques, we show the computed tomography features of the principal diseases involving the small pulmonary vessels by classifying them in these two principal groups.

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Pulmonary Veno-occlusive Disease

Cited by 88 publications

References 24 publications

Efficacy and safety of oral pulmonary vasodilators in pulmonary veno‐occlusive disease

Efficacy and safety of oral pulmonary vasodilators in pulmonary veno‐occlusive disease

When you hear hoofbeats, think zebras – pulmonary veno‐occlusive disease: A case report

CT imaging of peripheral pulmonary vessel disease

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