Article
Critical Care Medicine
Nicholas A. Kolaitis
Summary: Lung transplantation is a unique and effective treatment option for patients with end-stage pulmonary arterial hypertension. However, these patients face various challenges and inequities in the organ allocation process, resulting in less favorable long-term outcomes.
Review
Cardiac & Cardiovascular Systems
Beate Stubbe, Christian F. Opitz, Michael Halank, Dirk Habedank, Ralf Ewert
Summary: Intravenous prostacyclin analogues therapy for PAH patients has a long history and is now a crucial part of current guidelines. The development of fully implantable pump systems has improved convenience and reduced infection rates.
RESPIRATORY MEDICINE
(2021)
Article
Medicine, General & Internal
Li Zhang, Yao Wang, Ruifeng Zhang
Summary: The article reported two cases of PVOD initially misdiagnosed as idiopathic pulmonary arterial hypertension due to lack of typical findings, with subsequent correct diagnosis through EIF2AK4 mutation screening. This suggests that next-generation sequencing for EIF2AK4 mutation screening may be useful in differentiating PVOD from other PAH subtypes. PVOD is a heterogeneous population with different characteristics, including response to PAH-targeted therapy, highlighting the importance of timely identification of such patients and the need for further study.
Article
Cardiac & Cardiovascular Systems
Akihiro Ohsumi, Akihiro Aoyama, Hideyuki Kinoshita, Tomoya Yoneda, Kazuhiro Yamazaki, Satona Tanaka, Daisuke Nakajima, Tadashi Ikeda, Kenji Minatoya, Hiroshi Date
Summary: Tapering pretransplant use of epoprostenol may improve perioperative outcomes for patients with severe pulmonary hypertension undergoing lung transplantation, reducing the occurrence of primary graft dysfunction and shortening mechanical ventilation duration.
GENERAL THORACIC AND CARDIOVASCULAR SURGERY
(2022)
Review
Respiratory System
Sabina Solinas, Athenais Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Melanie Eyries, Peter Dorfmueller, Olivier Sitbon, Marc Humbert, David Montani
Summary: Pulmonary veno-occlusive disease (PVOD), characterized by substantial involvement of small pulmonary veins and capillaries, is an uncommon etiology of pulmonary arterial hypertension (PAH). Recent advancements include the discovery of biallelic mutations in the EIF2AK4 gene causing heritable PVOD. Differentiating PVOD from PAH is vital due to the worse prognosis and poor response of PVOD to PAH-approved drugs.
EXPERT REVIEW OF RESPIRATORY MEDICINE
(2023)
Review
Medicine, General & Internal
Asma Mohammadi, Wanessa F. Matos, Cesar Intriago, Keval Thakkar, Nasrin Jahan, Heeya Shah, Rifath I. Nishu, Sima Marzban
Summary: Pulmonary Arterial Hypertension (PAH) is a chronic progressive disease that can lead to right heart failure and premature death. Epoprostenol, a synthetic analog of prostacyclin, is an effective treatment for PAH, reducing mortality rates and improving survival rates. It has been successful in treating different subtypes of PAH and is a focus of ongoing clinical trials for potential therapeutic options.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Article
Biochemistry & Molecular Biology
Spiro Khoury, Antoine Beauvais, Jenny Colas, Anais Saint-Martin Willer, Frederic Perros, Marc Humbert, Clarisse Vandebrouck, David Montani, Thierry Ferreira, Fabrice Antigny
Summary: Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension (PAH) characterized by remodeling of the pulmonary venous system and venous obstruction. There is currently no effective medical therapy for PVOD and lung transplantation is the only available treatment option. Recent research suggests that PVOD patients exhibit lung lipointoxication, potentially playing a role in the development of the disease.
Article
Cardiac & Cardiovascular Systems
Kaori Takeuchi, Ayumi Goda, Junnosuke Ito, Hanako Kikuchi, Takumi Inami, Takashi Kohno, Kyoko Soejima, Toru Satoh
Summary: This study retrospectively reviewed the experience of safely withdrawing EPO from patients with PAH. Results indicated that patients with PAH who recovered normal haemodynamic function were able to withdraw EPO safely with the aid of an exercise stress test.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2022)
Article
Rheumatology
Haruka Moriya, Masaru Kato, Ryo Hisada, Keita Ninagawa, Maria Tada, Kodai Sakiyama, Mitsutaka Yasuda, Michihito Kono, Yuichiro Fujieda, Olga Amengual, Yasuka Kikuchi, Ichizo Tsujino, Takahiro Sato, Tatsuya Atsumi
Summary: This study investigated the relationship between clinical signs of pulmonary veno-occlusive disease (PVOD) and pulmonary vasculopathy severity in systemic sclerosis (SSc) patients. Results showed that the CT signs for PVOD correlated positively with mean pulmonary arterial pressure (mPAP) but negatively with diffusing capacity of carbon monoxide (DLCO) in SSc patients, suggesting a spectrum of pulmonary vascular disease from the artery to the vein.
Article
Pediatrics
Wataru Takemori, Kenichiro Yamamura, Yoshitaka Tomita, Naoki Egami, Katsuhide Eguchi, Hazumu Nagata, Hiromitsu Shirouzu, Yuichi Ishikawa, Daisuke Nakajima, Akihiko Yoshizawa, Hiroshi Date, Shouichi Ohga
Summary: This study reported a case of a 29-month-old boy with PVOD and a novel BMPR2 gene variant associated with I/HPAH. This finding supports the concept that I/HPAH and PVOD are part of a spectrum of pulmonary vascular disease.
PEDIATRIC PULMONOLOGY
(2022)
Article
Respiratory System
Xiaofang Zeng, Qiong Liu, Anandharajan Rathinasabapathy, Lihuang Zha, Dongliang Liu, Yiyang Tang, Jing Sun, Hui Luo, Zaixin Yu
Summary: This is a case report of a patient with Sjogren's syndrome associated with pulmonary veno-occlusive disease (PVOD). The patient's condition was well controlled with PAH-targeted therapies and did not show signs of pulmonary edema.
BMC PULMONARY MEDICINE
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Briac Lefebvre, Maeva Kyheng, Jessica Giordano, Nicolas Lamblin, Pascal de Groote, Marie Fertin, Marie Delobelle, Thierry Perez, Jean-Baptiste Faivre, Jacques Remy, Alain Duhamel, Martine Remy-Jardin
Summary: This study compared the dual-energy CT perfusion characteristics between PAH and PVOD/PCH patients, and found a higher percentage of PE-type defects in PVOD/PCH patients. Additionally, the iodine concentration in abnormal perfusion segments was significantly lower than in the normal group.
EUROPEAN RADIOLOGY
(2022)
Article
Respiratory System
Julia Degering, Benjamin Egenlauf, Satenik Harutyunova, Nicola Benjamin, Amina Salkic, Panagiota Xanthouli, Christina A. Eichstaedt, Rebekka Seeger, Olivier Sitbon, Ekkehard Gruenig
Summary: This study aims to expand clinical experience in the use of Veletri((R)), especially regarding tolerability, safety and survival. The results showed that epoprostenol AS (Veletri((R))) was comparable to previous prostacyclin formulations in terms of safety and tolerability, and was feasible for most patients.
RESPIRATORY RESEARCH
(2023)
Article
Cardiac & Cardiovascular Systems
Noah C. Schoenberg, Nicole F. Ruopp, Raj D. Parikh, Harrison W. Farber
Summary: The development of ascites in pulmonary arterial hypertension (PAH), without pre-existing hepatic dysfunction, is usually linked to decompensated right heart failure or cardiac cirrhosis. Ascites in PAH rarely occurs as a result of intravenous epoprostenol administration, a synthetic prostaglandin PGI(2).
PULMONARY CIRCULATION
(2022)
Article
Health Care Sciences & Services
Stormi Gale
Summary: This article reviews the goals of therapy, prognosis determinants, patient risk levels, and factors guiding treatment decisions in PAH. Recent research on combination therapies has shifted the paradigm in PAH treatment.
AMERICAN JOURNAL OF MANAGED CARE
(2021)
Editorial Material
Respiratory System
Jason Weatherald, Marc Humbert
Article
Critical Care Medicine
Mithum Kularatne, Melanie Eyries, Laurent Savale, Marc Humbert, David Montani
Summary: Heritable pulmonary arterial hypertension (PAH) is often associated with pathogenic variants of BMPR2. This study describes two patients with pulmonary arteriovenous malformations (AVMs) who were found to carry variants of BMPR2 through gene sequencing. Through these cases and understanding of the BMP9 pathway, it is suggested that BMPR2 variants may increase the risk of pulmonary AVMs.
Article
Cardiac & Cardiovascular Systems
Christophe Guignabert, Laurent Savale, Athenais Boucly, Raphael Thuillet, Ly Tu, Mina Ottaviani, Christopher J. Rhodes, Pascal De Groote, Gregoire Prevot, Emmanuel Bergot, Arnaud Bourdin, Luke S. Howard, Elie Fadel, Antoine Beurnier, Anne Roche, Mitja Jevnikar, Xavier Jais, David Montani, Martin R. Wilkins, Olivier Sitbon, Marc Humbert
Summary: Activin A and FSTL3 serum levels are associated with transplant-free survival in PAH. They can serve as prognostic biomarkers for PAH, and their diagnostic values have been confirmed in an independent validation cohort. The activin signaling system in PAH involves phosphorylated Smad2/3 nuclear accumulation and differential immunoreactivities of various proteins in vascular endothelial and smooth muscle layers.
Article
Cardiac & Cardiovascular Systems
Jeremie Pichon, Anne Roche, Charles Fauvel, Athenais Boucly, Olaf Mercier, Nathan Ebstein, Antoine Beurnier, Jonathan Cortese, Mitja Jevnikar, Xavier Jais, Muriel Fartoukh, Elie Fadel, Olivier Sitbon, David Montani, Guillaume Voiriot, Marc Humbert, Laurent Savale
Summary: This study aimed to evaluate the clinical relevance and prognostic value of arterial and venous renal Doppler in acute decompensated precapillary pulmonary hypertension (PH). The results showed that the renal Doppler provides additional information to assess the severity and prognosis of patients with acute decompensated precapillary PH admitted to the intensive care unit.
EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING
(2023)
Article
Cardiac & Cardiovascular Systems
Julien Grynblat, Sophie-Guiti Malekzadeh-Milani, Mathilde Meot, Frederic Perros, Isabelle Szezepanski, Stephane Morisset, Caroline Ovaert, Caroline Bonnet, Pascale Maragnes, Julien Ranchoup, Marc Humbert, I. David Montani, Marilyne Levy, Damien Bonnet
Summary: This study aims to define the goals of repeated right heart catheterization (RHC) and evaluate the correlation between noninvasive criteria and hemodynamic parameters. The results showed that pulmonary vascular resistance index, pulmonary artery compliance index, and right atrial pressure were associated with adverse outcomes in pediatric pulmonary arterial hypertension. Noninvasive criteria accurately predicted the evolution of hemodynamic parameters, but some patients still had at least one at-risk hemodynamic parameter at second RHC.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2023)
Editorial Material
Respiratory System
Vincent Cottin, Claudia Valenzuela, Marc Humbert
EUROPEAN RESPIRATORY JOURNAL
(2023)
Review
Critical Care Medicine
Jason Weatherald, Rhea A. Varughese, Jonathan Liu, Marc Humbert
Summary: Pulmonary arterial hypertension (PAH) is a rare disease characterized by progressive pulmonary arterial remodeling, increased resistance, right ventricular dysfunction, and reduced survival. Current approved therapies for PAH target nitric oxide, endothelin-1, and prostacyclin pathways. Treatment includes oral medications for low- and intermediate-risk patients, with consideration of triple therapy for high-risk patients. Combination therapy may not be suitable for PAH patients with cardiopulmonary comorbidities. Supportive and adjunctive treatments are also important, and lung transplantation may be considered for patients who do not respond to medical care. Additional therapies are needed to improve outcomes in PAH.
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Respiratory System
Sabina Solinas, Athenais Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Melanie Eyries, Peter Dorfmueller, Olivier Sitbon, Marc Humbert, David Montani
Summary: Pulmonary veno-occlusive disease (PVOD), characterized by substantial involvement of small pulmonary veins and capillaries, is an uncommon etiology of pulmonary arterial hypertension (PAH). Recent advancements include the discovery of biallelic mutations in the EIF2AK4 gene causing heritable PVOD. Differentiating PVOD from PAH is vital due to the worse prognosis and poor response of PVOD to PAH-approved drugs.
EXPERT REVIEW OF RESPIRATORY MEDICINE
(2023)
Review
Respiratory System
Fida Charif, Fatima Dakroub, Imad Bou Akl, Mithum Kularatne, David Montani
Summary: COVID-19 remains a healthcare concern even after the end of the pandemic. Patients with cardiovascular disease (CVD) are at higher risk for severe COVID-19 complications. Studies on the clinical characteristics of COVID-19 in patients with pulmonary arterial hypertension (PAH) have produced conflicting results. In this review, we summarize the literature on the clinical presentation of COVID-19 in PAH patients and discuss common pathological aspects and disease mechanisms between PAH and COVID-19. We also provide an overview of PAH-approved therapies and their potential use in COVID-19 treatment, as well as summarize clinical trials on the safety and efficacy of PAH-approved drugs in COVID-19 patients. Finally, we propose future research studies.
RESPIRATORY MEDICINE AND RESEARCH
(2023)
Review
Respiratory System
David Montani, Fabrice Antigny, Etienne-Marie Jutant, Marie-Camille Chaumais, Helene Le Ribeuz, Julien Grynblat, Charles Khouri, Marc Humbert
Summary: The ATP-sensitive potassium channels and their regulatory subunits, SUR1 and SUR2, have a significant role in the pathophysiology of pulmonary hypertension. Loss-of-function variants in ABCC8 gene, which encodes for SUR1, are associated with heritable pulmonary arterial hypertension, while activation of SUR1 and SUR2 leads to relaxation of pulmonary arteries and reduces cell proliferation and migration. Diazoxide, an activator of SUR1, has shown potential as a therapeutic option for pulmonary hypertension. However, there are contradictory reports of diazoxide-induced pulmonary hypertension in infants.
Correction
Cardiac & Cardiovascular Systems
Hossein-Ardeschir Ghofrani, Ekkehard Grunig, Pavel Jansa, David Langleben, Stephan Rosenkranz, Ioana R. Preston, Franck Rahaghi, Namita Sood, Dennis Busse, Christian Meier, Marc Humbert
PULMONARY CIRCULATION
(2023)
Article
Respiratory System
Sharon Mumby, Frederic Perros, Julien Grynblat, Gregoire Manaud, Alberto Papi, Paolo Casolari, Gaetano Caramori, Marc Humbert, S. John Wort, Ian M. Adcock
Summary: In this study, elevated levels of BET proteins were found in pulmonary vascular cells of PAH patients, and JQ1+ was able to suppress TNF-α-induced inflammatory responses. These findings suggest that BET proteins could be a potential target for treating PAH.
RESPIRATORY RESEARCH
(2023)
Letter
Medicine, General & Internal
Jason Weatherald, Marc Humbert
Meeting Abstract
Respiratory System
Chin Kook Rhee, Jonathan Corren, Mario Castro, Jorge F. Maspero, Marc Humbert, David M. G. Halpin, Arman Altincatal, Nami Pandit-Abid, Xavier Soler, Shahid Siddiqui, Juby A. Jacob-Nara, Yamo Deniz, Paul J. Rowe
Letter
Critical Care Medicine
David Montani, Jason Weatherald, Marc Humbert, Olivier Sitbon
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)