Other causes of pulmonary arterial hypertension: pulmonary veno-occlusive disease, pulmonary capillary hemangiomatosis, porto-pulmonary hypertension, HIV- associated pulmonary arterial hypertension

Pulmonary arterial hypertension and pulmonary hypertension associated with pulmonary venous or capillary abnormalities, i.e. pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis, are discussed.

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Genetic counselling in pre-capillary pulmonary hypertension

ESC CardioMed ◽

10.1093/med/9780198784906.003.0602 ◽

2018 ◽

pp. 2558-2560

Author(s):

Barbara Girerd ◽

David Montani ◽

Marc Humbert

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Genetic Counselling ◽

Autosomal Dominant ◽

Autosomal Dominant Disease ◽

Pulmonary Capillary ◽

Dominant Disease ◽

Pulmonary Arterial ◽

Biallelic Mutations

Pre-capillary pulmonary hypertension can be heritable in the context of pulmonary arterial hypertension (an autosomal dominant disease mainly due to mutations in BMPR2), and pulmonary veno-occlusive disease or pulmonary capillary haemangiomatosis (an autosomal recessive disease due to biallelic mutations in the EIF2AK4 gene). Genetic counselling can be implemented in referral centres for pulmonary hypertension as outlined in this chapter.

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Pulmonary Veno-Occlusive Disease: A Rare Cause of Pulmonary Hypertension

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/2324709619840375 ◽

2019 ◽

Vol 7 ◽

pp. 232470961984037 ◽

Cited By ~ 1

Author(s):

Said Hajouli ◽

Muhamad Alhaj Moustafa ◽

Jessica S. Wang Memoli

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Occlusive Disease ◽

Class I ◽

Rare Entity ◽

Pulmonary Arterial

Pulmonary veno-occlusive disease (PVOD) is a rare entity that is usually mistaken with pulmonary arterial hypertension (PAH) but is considered class I′ of PAH. It is important to subclassify PVOD and distinguish it from PAH as treatment with vasodilators in PVOD patients is controversial and may be fatal. In this article, we describe a case of PVOD and how we diagnosed it.

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Anti-synthetase syndrome-associated pulmonary veno-occlusive disease

Pulmonary Circulation ◽

10.1177/2045894020935289 ◽

2020 ◽

Vol 10 (3) ◽

pp. 204589402093528 ◽

Cited By ~ 1

Author(s):

Dana Kay ◽

Ferdous Kadri ◽

Garrett Fitzpatrick ◽

Hassan Alnuaimat ◽

Raju Reddy ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Right Heart Failure ◽

Right Heart Catheterization ◽

Occlusive Disease ◽

Heart Catheterization ◽

Right Heart ◽

Pulmonary Arterial ◽

Pulmonary Arterial Hypertension Therapy

Pulmonary arterial hypertension has been reported with a prevalence of 7.9% in patients with anti-synthetase syndrome; however, anti-synthetase syndrome associated with pulmonary veno-occlusive disease (PVOD) has never before been described in the literature. We present a novel case of anti-synthetase syndrome-associated PVOD in a patient who presented with hypoxic respiratory failure associated with right heart failure and was diagnosed with anti-synthetase syndrome based on his autoimmune serology and pre-capillary pulmonary hypertension on right heart catheterization. He was initiated on pulmonary arterial hypertension therapy, but with escalating dose of parenteral epoprostenol, experienced acute clinical worsening with chest imaging concerning for PVOD that was confirmed on autopsy. Anti-synthetase syndrome can be associated with PVOD, and it should be suspected in patients who have evidence of pre-capillary pulmonary hypertension and who deteriorate with the initiation of pulmonary hypertension-specific therapy.

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Pulmonary Veno-occlusive Disease

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-14.3.155 ◽

2015 ◽

Vol 14 (3) ◽

pp. 155-160

Author(s):

David Montani ◽

Barbara Girerd ◽

Marc Humbert

Keyword(s):

Risk Factors ◽

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Early Diagnosis ◽

Arterial Hypertension ◽

Clinical Presentation ◽

Occlusive Disease ◽

Rare Form ◽

Pulmonary Arterial ◽

Specific Management

As a rare form of pulmonary hypertension, the pathophysiology and clinical presentation of pulmonary veno-occlusive disease (PVOD) has long remained poorly understood. In this review, we will discuss the distinctions between presentation of PVOD and pulmonary arterial hypertension, and address the importance of early diagnosis in proposing specific management. We will explore the risk factors and conditions associated with PVOD, and describe the challenges surrounding its diagnosis and management.

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Genetic counselling in pre-capillary pulmonary hypertension

ESC CardioMed ◽

10.1093/med/9780198784906.003.0602_update_001 ◽

2018 ◽

pp. 2558-2560

Author(s):

Barbara Girerd ◽

David Montani ◽

Marc Humbert

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Genetic Counselling ◽

Autosomal Dominant ◽

Autosomal Dominant Disease ◽

Pulmonary Capillary ◽

Dominant Disease ◽

Pulmonary Arterial ◽

Biallelic Mutations

Pre-capillary pulmonary hypertension can be heritable in the context of pulmonary arterial hypertension (an autosomal dominant disease mainly due to mutations in BMPR2), and pulmonary veno-occlusive disease or pulmonary capillary haemangiomatosis (an autosomal recessive disease due to biallelic mutations in the EIF2AK4 gene). Genetic counselling can be implemented in referral centres for pulmonary hypertension as outlined in this chapter.

Download Full-text