Scientific Resources & Research Materials
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PVDOMICS Study Group. Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease
This is the first report from the PVDOMICs study, an NIH funded multicenter project designed to better understand the clinical, biological and genetic features and differences between all forms of pulmonary hypertension. This initial paper describes the clinical features of over 1000 subjects in Groups 1-5 pulmonary hypertension. The notable findings include 1) there is often a mixture of etiologies in each of the groups; 2) DLCO is depressed in Groups 1-3; 3) Right atrial volume is enlarged in Groups 1-4 even when the right ventricle is not; 4) Group 1 patients frequently have ground glass findings on Chest CT scan and nocturnal desaturation; 5) Survival is poorest in Group 3.
The finding that there is right atrial enlargement before it is right ventricular enlargement is particularly relevant to the numerous reports that increased right atrial pressure is a powerful predictor of survival in PH, and that the mechanism appears to be associated with increased stressed blood volume. These data provide support for the potential of levosimendan as a fundamental treatment of pulmonary hypertension.
CitationHemnes AR, Leopold JA, Radeva MK, Beck GJ, Abidov A, Aldred MA, Barnard J, Rosenzweig EB, Borlaug BA, Chung WK, Comhair SAA, Desai AA, Dubrock HM, Erzurum SC, Finet JE, Frantz RP, Garcia JGN, Geraci MW, Gray MP, Grunig G, Hassoun PM, Highland KB, Hill NS, Hu B, Kwon DH, Jacob MS, Jellis CL, Larive AB, Lempel JK, Maron BA, Mathai SC, McCarthy K, Mehra R, Nawabit R, Newman JH, Olman MA, Park MM, Ramos JA, Renapurkar RD, Rischard FP, Sherer SG, Tang WHW, Thomas JD, Vanderpool RR, Waxman AB, Wilcox JD, Yuan JX, Horn EM; PVDOMICS Study Group. Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease. J Am Coll Cardiol. 2022 Aug 16;80(7):697-718.
Elucidating the Clinical Implications and Pathophysiology of Pulmonary Hypertension in Heart Failure With Preserved Ejection Fraction: A Call to Action: A Science Advisory From the American Heart Association
This is a comprehensive review of the published clinical studies related to pulmonary hypertension in heart failure with preserved ejection fraction (PH-HFpEF). It includes clinical and hemodynamic definitions of the disease, epidemiology, pathophysiology, animal models and treatment approaches, and clinical trials. They importantly stress the urgent unmet need to make progress in this disease so as to reduce the high burden of the disease. This is a wonderful source document for finding the latest research and publications.
CitationBrittain EL, Thenappan T, Huston JH, Agrawal V, Lai YC, Dixon D, Ryan JJ, Lewis EF, Redfield MM, Shah SJ, Maron BA; American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Lifestyle and Cardiometabolic Health; and Stroke Council. Elucidating the Clinical Implications and Pathophysiology of Pulmonary Hypertension in Heart Failure With Preserved Ejection Fraction: A Call to Action: A Science Advisory From the American Heart Association. Circulation. 2022 Jul 11:101161CIR0000000000001079
Venous Tone and Stressed Blood Volume in Heart Failure
This soon-to-be classic review offers a very understandable explanation about the importance of venous return in normal physiology and heart failure. While preload is an old concept, it has never been appreciated to have a major impact in managing heart failure patients until now. The studies referenced in this paper predict novel treatments of heart failure based on the concepts of stressed and unstressed blood volume. Levosimendan is the first drug to ever result in clinical improvement based on these concepts.
CitationFudim M, Kaye DM, Borlaug BA, Shah SJ, Rich S, Kapur NK, Costanzo MR, Brener MI, Sunagawa K, Burkhoff D. Venous Tone and Stressed Blood Volume in Heart Failure: JACC Review Topic of the Week. J Am Coll Cardiol. 2022 May 10;79(18):1858-1869.
Association Of Borderline Pulmonary Hypertension With Mortality And Hospitalization In A Large Patient Cohort: Insights From The Veterans Affairs Clinical Assessment, Reporting, And Tracking Program
This is a retrospective study of over 20,000 patients with pulmonary hypertension confirmed by cardiac catheterization, from any cause who were followed for clinical outcomes. Like other studies it confirmed the increasing mortality beginning at the mildest levels of increased pulmonary artery pressure. The case can be made for screening programs to detect early disease to initiate treatments.
CitationMaron BA, Hess E, Maddox TM, Opotowsky AR, Tedford RJ, Lahm T, Joynt KE, Kass DJ, Stephens T, Stanislawski MA, Swenson ER, Goldstein RH, Leopold JA, Zamanian RT, Elwing JM, Plomondon ME, Grunwald GK, Barón AE, Rumsfeld JS, Choudhary G. Association of Borderline Pulmonary Hypertension With Mortality and Hospitalization in a Large Patient Cohort: Insights From the Veterans Affairs Clinical Assessment, Reporting, and Tracking Program. Circulation. 2016 Mar 29;133(13):1240-8
A Global View of Pulmonary Hypertension
This is one of the more in-depth reviews of the global burden of pulmonary hypertension. They estimate a prevalence of 1% of the world population having pulmonary hypertension from any cause. They also estimate that the large majority of these patients (80%) live in developing countries, which emphasizes the challenge for both preventive and treatment strategies that will be affordable and accessible.
CitationHoeper MM, Humbert M, Souza R, Idrees M, Kawut SM, Sliwa-Hahnle K, Jing ZC, Gibbs JS. A global view of pulmonary hypertension. Lancet Respir Med. 2016 Apr;4(4):306-22.
Understanding the Pathobiology of Pulmonary Hypertension Due to Left Heart Disease
This is an in depth authoritative review of the field of pulmonary hypertension due to left heart disease. Written by one of the most renown thought leaders, it covers all facets of basic and clinical science, with insights into potential effective therapies. A must read for anyone who is involved with translational of clinical investigations in this area.