Article
Cardiac & Cardiovascular Systems
Rohit Gupta, Matthew Zheng, Andrew J. Gangemi, Huaqing Zhao, Francis C. Cordova, Gerard J. Criner, Albert J. Mamary, Sameep Sehgal
Summary: In patients with sarcoidosis on the lung transplant waitlist, female gender and severe pulmonary hypertension are associated with a higher likelihood of death, while taller minimum donor height and blood type B are protective factors against death on the waitlist. Earlier referral for transplantation should be considered for patients with these characteristics.
RESPIRATORY MEDICINE
(2022)
Article
Critical Care Medicine
Nicholas A. Kolaitis, Hubert Chen, Daniel R. Calabrese, Kerry Kumar, Jill Obata, Carrie Bach, Jeffrey A. Golden, Marc A. Simon, Jasleen Kukreja, Steven R. Hays, Lorriana E. Leard, Jonathan P. Singer, Teresa De Marco
Summary: The 2015 revision of the lung allocation score (LAS) aimed to improve waiting list mortality and transplant rates for patients with pulmonary arterial hypertension (PAH). A study comparing the pre- and post-revision eras found that the revision did achieve its intended goals, providing better transplantation opportunities and reducing the risk of death on the waiting list for patients with PAH.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Samantha Greissman, Michaela Anderson, Angela Dimango, Harpreet Grewal, Gabriela Magda, Hilary Robbins, Lori Shah, Joseph Costa, Bryan Stanifer, Frank D'-Ovidio, Miguel Leiva Juarez, Philippe Lemaitre, Joshua Sonett, Selim Arcasoy, Luke Benvenuto
Summary: Candidates with blood group O have lower lung transplantation rates despite being the most common blood type. The study reveals that waitlist outcomes for these candidates and those with other blood types vary based on disease severity and lung allocation score (LAS).
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
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.
Article
Cardiac & Cardiovascular Systems
Oliver K. Jawitz, Vignesh Raman, David Becerra, Jacob Klapper, Matthew G. Hartwig
Summary: This study aimed to identify factors associated with survival beyond a decade after lung transplant. The results showed that approximately one-quarter of lung transplant recipients survived 10 years post-transplant. There were different factors associated with 1-year and 10-year survival, and the Lung Allocation Score did not impact long-term survival. Further research is needed to improve patient selection and management strategies to increase long-term survival.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2022)
Article
Respiratory System
Derlis C. Fleitas Sosa, Shameek Gayen, Matthew Zheng, Andrew J. Gangemi, Huaqing Zhao, Victor Kim, Sameep Sehgal, Gerard J. Criner, Rohit Gupta, A. James Mamary
Summary: The study shows that patients with sarcoidosis have a higher risk of mortality while waiting for lung transplantation compared to other candidates. They suggest a revision of the grouping of sarcoidosis candidates in allocation systems.
Article
Cardiac & Cardiovascular Systems
Rachel L. Deitz, Leonid Emerel, Ernest G. Chan, John P. Ryan, Eric Hyzny, Masashi Furukawa, Pablo G. Sanchez
Summary: This study investigated variables associated with mortality on the waiting list for lung transplant in patients supported on extracorporeal membrane oxygenation (ECMO). The results showed that patients at high-risk centers were more likely to survive to transplantation. This suggests that ECMO is an appropriate strategy to bridge high-risk patients to lung transplant.
ANNALS OF THORACIC SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Travis D. Hull, Gregory A. Leya, Andrea L. Axtell, Philicia Moonsamy, Asishana Osho, David C. Chang, Thoralf M. Sundt, Mauricio A. Villavicencio
Summary: Restricted listing preference is associated with increased waitlist mortality, but DLT recipients have superior posttransplant survival.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2022)
Article
Gastroenterology & Hepatology
Sarah Bernards, Ryutaro Hirose, Francis Y. Yao, Chengshi Jin, Jennifer L. Dodge, Chiung-Yu Huang, Neil Mehta
Summary: The UNOS policy to limit exception points for liver cancer patients on the waitlist has successfully reduced the dropout probability and equalized waitlist dropout rates between patients with and without HCC. However, it has also disadvantaged certain HCC patients.
LIVER TRANSPLANTATION
(2022)
Article
Cardiac & Cardiovascular Systems
Carli J. Lehr, Jarrod E. Dalton, Paul R. Gunsalus, Douglas D. Gunzler, Maryam Valapour
Summary: A clinically intuitive indexing system was developed to simplify mortality risk assessment of lung transplant candidates, offering the opportunity to identify more nuanced and interpretable clinical phenotypes by reducing the dimensionality of waitlist mortality risk.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Review
Cardiac & Cardiovascular Systems
Xiaokun Hu, Ningying Ding, Wanqiu Songchen, Ruifeng Wang, Jing Chen, Ailing Zhong, Jinzhu Nan, Yujie Zuo, Heng Huang, Dong Tian
Summary: Pulmonary hypertension (PH) was a devastating and fatal disease, but medical treatment algorithms have significantly improved its outlook. Lung transplantation (LTx) has been the only effective therapeutic option for patients with PH, but they have high mortality rates on the transplant waiting list. Balancing optimized medical treatment with the appropriate timing of referral and listing for LTx has been controversial. The 2021 consensus document by the International Society for Heart and Lung Transplantation (ISHLT) provides updated recommendations for the referral and listing time based on risk stratification.
JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE
(2023)
Article
Respiratory System
Marius M. Hoeper, Christine Pausch, Karen M. Olsson, Doerte Huscher, David Pittrow, Ekkehard Gruenig, Gerd Staehler, Carmine Dario Vizza, Henning Gall, Oliver Distler, Christian Opitz, J. Simon R. Gibbs, Marion Delcroix, H. Ardeschir Ghofrani, Da-Hee Park, Ralf Ewert, Harald Kaemmerer, Hans-Joachim Kabitz, Dirk Skowasch, Juergen Behr, Katrin Milger, Michael Halank, Heinrike Wilkens, Hans-Juergen Seyfarth, Matthias Held, Daniel Dumitrescu, Iraklis Tsangaris, Anton Vonk-Noordegraaf, Silvia Ulrich, Hans Klose, Martin Claussen, Tobias J. Lange, Stephan Rosenkranz
Summary: The study investigated a modified risk stratification approach based on four risk categories, using refined cutoff levels for functional class, 6MWD, and BNP/NT-proBNP. The four-stratum model was found to be more sensitive in predicting long-term survival and prognostic changes compared to the original three-stratum model.
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Cardiac & Cardiovascular Systems
Sandeep Sahay, Jiken Bhatt, Sarah Beshay, Ashrith Guha, Duc T. Nguyen, Edward A. Graviss, Sherif F. Nagueh
Summary: Combining echocardiographic parameters with REVEAL Lite 2.0 provides more accurate risk predictions for PAH.
PULMONARY CIRCULATION
(2022)
Article
Critical Care Medicine
Athenais Boucly, Laurent Savale, Xavier Jais, Fabrice Bauer, Emmanuel Bergot, Laurent Bertoletti, Antoine Beurnier, Arnaud Bourdin, Helene Bouvaist, Sophie Bulifon, Celine Chabanne, Ari Chaouat, Vincent Cottin, Claire Dauphin, Bruno Degano, Pascal De Groote, Nicolas Favrolt, Yuanchao Feng, Delphine Horeau-Langlard, Mitja Jevnikar, Etienne-Marie Jutant, Zhiying Liang, Pascal Magro, Pierre Mauran, Pamela Moceri, Jean-Francois Mornex, Sylvain Palat, Florence Parent, Francois Picard, Jeremie Pichon, Patrice Poubeau, Gregoire Prevot, Sebastien Renard, Martine Reynaud-Gaubert, Marianne Riou, Pascal Roblot, Olivier Sanchez, Andrei Seferian, Cecile Tromeur, Jason Weatherald, Gerald Simonneau, David Montani, Marc Humbert, Olivier Sitbon
Summary: The study findings suggest that initial triple combination therapy containing parenteral prostacyclin is associated with a higher survival rate in patients with PAH, particularly in younger high-risk patients.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Article
Cardiac & Cardiovascular Systems
Tatsuaki Watanabe, Satoshi Matsuo, Yui Watanabe, Takashi Hirama, Yasushi Matsuda, Masafumi Noda, Hiromichi Niikawa, Hisashi Oishi, Yamato Suzuki, Yutaka Ejima, Hiroaki Toyama, Yoshikatsu Saiki, Yoshinori Okada
Summary: This study examined the outcomes of bilateral lung transplantation (BLT) with pulmonary artery reconstruction (PAR) using donor aorta for pulmonary arterial hypertension (PAH) patients with a giant pulmonary arterial aneurysm (PAA). The study found that the operation time was longer in the PAR group, but the 90-day mortality and 5-year survival rates were comparable to the non-PAR group. This suggests that lung transplantation with PAR using donor aorta is a valid surgical option for PAH patients with a giant PAA.
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY
(2023)
Review
Cardiac & Cardiovascular Systems
Jane A. Leopold, Steven M. Kawut, Micheala A. Aldred, Stephen L. Archer, Ray L. Benza, Michael R. Bristow, Evan L. Brittain, Naomi Chesler, Frances S. DeMan, Serpil C. Erzurum, Mark T. Gladwin, Paul M. Hassoun, Anna R. Hemnes, Tim Lahm, Joao A. C. Lima, Joseph Loscalzo, Bradley A. Maron, Laura Mercer Rosa, John H. Newman, Susan Redline, Stuart Rich, Franz Rischard, Lissa Sugeng, W. H. Wilson Tang, Ryan J. Tedford, Emily J. Tsai, Corey E. Ventetuolo, YouYang Zhou, Neil R. Aggarwal, Lei Xiao
Summary: Understanding of the right ventricle in health and disease is incomplete, with challenges including defining and standardizing normal RV structure and function, accessing RV tissue for research, and advancing imaging and invasive methodologies. Recommendations were made to incorporate precision medicine and innovative therapeutics for patients with pulmonary vascular disease.
CIRCULATION-HEART FAILURE
(2021)
Article
Health Care Sciences & Services
Kim M. Kerr, C. Greg Elliott, Raymond L. Benza, Richard N. Channick, Kelly M. Chin, R. Duane Davis, Sonia Jain, Andrea Z. LaCroix, Michael M. Madani, Vallerie V. McLaughlin, Myung H. Park, Victor F. Tapson, William R. Auger
Summary: The US CTEPH Registry aims to provide essential information to better understand the demographics, risk factors, evaluation, and treatment of CTEPH in the United States, as well as the short- and long-term outcomes of surgical and nonsurgical therapies in the modern treatment era. Data from this registry will improve understanding of the demographics, risk factors, and treatment patterns of patients with CTEPH, and the longitudinal impact of therapies on quality of life, health care utilization, and survival.
JMIR RESEARCH PROTOCOLS
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Jason H. Anderson, Allison K. Cabalka, Robert P. Frantz, Hector R. Cajigas, Nathaniel W. Taggart
CIRCULATION-CARDIOVASCULAR INTERVENTIONS
(2022)
Article
Cardiac & Cardiovascular Systems
Raymond L. Benza, Athenais Boucly, Harrison W. Farber, Adaani E. Frost, Hossein-Ardeschir Ghofrani, Marius M. Hoeper, Marc Lambelet, Claudia Rahner, Sameer Bansilal, Sylvia Nikkho, Christian Meier, Olivier Sitbon
Summary: This analysis confirms the risk-reduction benefits of riociguat in patients with pulmonary arterial hypertension (PAH) and further contributes to the validation of REVEAL Lite 2 in facilitating PAH risk assessment.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2022)
Article
Cardiac & Cardiovascular Systems
Raymond L. Benza, Harrison W. Farber, Adaani E. Frost, Hossein-Ardeschir Ghofrani, Paul A. Corris, Marc Lambelet, Sylvia Nikkho, Christian Meier, Marius M. Hoeper
Summary: This post hoc analysis evaluated the utility of RRS 2.0 in predicting outcomes and monitoring treatment response in patients with CTEPH. The results showed significant improvement in RRS 2.0 with riociguat treatment and its significant association with survival and clinical worsening-free survival.
RESPIRATORY MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Kevin Y. Chang, Sue Duval, David B. Badesch, Todd M. Bull, Murali M. Chakinala, Teresa De Marco, Robert P. Frantz, Anna Hemnes, Stephen C. Mathai, Erika Berman Rosenzweig, John J. Ryan, Thenappan Thenappan
Summary: Using the PHAR registry, we evaluated the mortality rate of patients with PAH in the modern era and found that the mortality rate remains unacceptably high, especially for intermediate and high-risk patients. This emphasizes the importance of early diagnosis, aggressive treatment, and the development of better therapeutics.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2022)
Editorial Material
Critical Care Medicine
Nicholas J. Shelburne, Robert P. Frantz
Article
Cardiac & Cardiovascular Systems
Adaani E. Frost, Carol Zhao, Harrison W. Farber, Raymond Benza, Joseph Yen, Mona Selej, C. Gregory Elliott
Summary: The prevalence of smoking and its association with pulmonary arterial hypertension (PAH) outcomes in the United States have not been described. This study used the US-based Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) to determine the prevalence, demographics, and outcomes in ever-smokers and never-smokers with PAH.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Article
Respiratory System
Raymond L. Benza, David Langleben, Anna R. Hemnes, Anton Vonk Noordegraaf, Stephan Rosenkranz, Thenappan Thenappan, Paul M. Hassoun, Ioana R. Preston, Stefano Ghio, Roberto Badagliacca, Carmine D. Vizza, Irene M. Lang, Christian Meier, Ekkehard Gruenig
Summary: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) can lead to right heart failure, with right ventricular changes as long-term indicators. Riociguat, an approved treatment for these diseases, is examined for its effects on right ventricular remodeling, function, and structural parameters in this review.
EUROPEAN RESPIRATORY REVIEW
(2022)
Article
Cardiac & Cardiovascular Systems
Anne B. Rohlfing, Kara E. Bischoff, Nicholas A. Kolaitis, Richard A. Kronmal, Noah A. Kime, Michael P. Gray, Sonja Bartolome, Murali M. Chakinala, Robert P. Frantz, Corey E. Ventetuolo, Stephen C. Mathai, Teresa De Marco
Summary: Patients with pulmonary arterial hypertension (PAH) are rarely referred to palliative care, typically close to the end of life. Predictors of referral include increasing age, lower body mass index, supplemental oxygen use, parenteral prostanoid use, and lower quality of life scores.
RESPIRATORY MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Amber McCormick, Amita Krishnan, David Badesch, Raymond L. Benza, Todd M. Bull, Teresa De Marco, Jeremy Feldman, Anna R. Hemnes, Russel Hirsch, Evelyn Horn, Jaime Kennedy, Stephen C. Mathai, Wesley McConnell, Steven C. Pugliese, Jeffrey S. Sager, Oksana A. Shlobin, Marc A. Simon, Matthew R. Lammi
Summary: This study investigated the pulmonary artery compliance and resistance-compliance time in pulmonary arterial hypertension (PAH) patients, and compared the differences between different PAH subgroups. The results showed that there was no difference in pulmonary artery compliance among PAH subgroups, and it was not an independent predictor of transplant-free survival in PAH. However, resistance-compliance time differed among PH subgroups, challenging the constancy of resistance-compliance time.
Article
Cardiac & Cardiovascular Systems
Harrison W. Farber, Murali M. Chakinala, Michelle Cho, Robert P. Frantz, Andrew Frick, Lisa Lancaster, Scott Milligan, Ronald Oudiz, Sumeet Panjabi, Yuen Tsang, Steven D. Nathan
Summary: "TRIO CIPDR" is a US repository that captures clinical data, drug prescription and dispensing information of 946 adult patients with pulmonary arterial hypertension (PAH) from nine US specialist tertiary care centers. The data reveals that most patients were female, white, and diagnosed with PAH at the median age of 53 years. The majority of patients received PAH-specific therapy, with 40% on monotherapy, 43% on dual therapy, and 17% on triple therapy. This repository will provide longitudinal data to track the treatment journey and outcomes of PAH patients.
PULMONARY CIRCULATION
(2023)
Article
Cardiac & Cardiovascular Systems
Robert P. Frantz, Jane A. Leopold, Paul M. Hassoun, Anna R. Hemnes, Evelyn M. Horn, Stephen C. Mathai, Franz P. Rischard, A. Brett Larive, W. H. Wilson Tang, Margaret M. Park, Nicholas S. Hill, Erika B. Rosenzweig
Summary: Chronic thromboembolic pulmonary hypertension (CTEPH) involves both vascular obstruction and vasoconstriction. Vasodilators like riociguat may benefit CTEPH patients. However, acute vasoreactivity testing (AVT) is rarely performed in CTEPH patients, leading to limited understanding of its significance. A systematic vasodilator testing with oxygen (O-2) and oxygen plus inhaled nitric oxide (O-2 + iNO) was conducted, revealing significant variability in vascular tone in CTEPH patients.
PULMONARY CIRCULATION
(2023)
Article
Cardiac & Cardiovascular Systems
Paul Forfia, Raymond Benza, Michele D'Alto, Teresa De Marco, Jean M. Elwing, Robert Frantz, Francois Haddad, Ronald Oudiz, Ioana R. Preston, Stephan Rosenkranz, John Ryan, Robert Schilz, Oksana A. Shlobin, Jean-Luc Vachiery, Carmine Dario Vizza, Anton Vonk Noordegraaf, Margaret R. Sketch, Meredith Broderick, Vallerie McLaughlin
Summary: Right heart imaging is important for treatment decisions in pulmonary arterial hypertension (PAH), but there is limited evidence and guidelines on its use. A Delphi study was conducted to gather expert opinions on the role of right heart imaging in treatment escalation for PAH. The panel of experts agreed that right heart imaging can improve risk stratification and help differentiate risk levels in intermediate-risk patients. Specific measures such as tricuspid annular plane systolic excursion and right ventricular fractional area change should be included in routine echocardiography for PAH. Cardiac magnetic resonance imaging is valuable but limited by cost and access. Abnormal right heart imaging should prompt further evaluation and possible treatment escalation.
PULMONARY CIRCULATION
(2023)