Pulmonary Vascular Program

Pulmonary Vascular Program

The Pulmonary Vascular Program at Cleveland Clinic consists of 9 pulmonologists, 2 cardiologists, 2 advanced practice nurses, a nurse, a pharmacist, 2 program coordinators, and 3 research coordinators. Additionally, as part of Cleveland Clinic, the program draws on expertise in cardiology, cardiothoracic surgery, lung transplantation, hepatology, liver transplantation, sleep medicine, rheumatology, hematology, and interventional radiology to provide a comprehensive and multidisciplinary approach to patient care. The program has dedicated multidisciplinary teams to care for patients with chronic thromboembolic pulmonary hypertension, hereditary hemorrhagic telangiectasia, congenital heart disease, pulmonary hypertension associated with the scleroderma spectrum of diseases and other connective tissue diseases, pulmonary hypertension associated with interstitial lung disease and chronic obstructive lung disease, portopulmonary hypertension, and sickle cell disease. The program offers advanced hemodynamic evaluation including invasive cardiopulmonary exercise testing (ie, simultaneous right heart catheterization and cardiopulmonary exercise testing) and maintains an Institutional Review Board-approved comprehensive registry.

The Pulmonary Vascular Program is involved in a wide range of research activities, from NIH-funded basic research and translational research to clinical trials of the latest therapies. Many patients referred to the program benefit from enrollment in 1 or more of these ongoing clinical trials and research studies.

Pulmonary Artery Catheterization Volume

2015 – 2022

CPET = cardiopulmonary exercise test, esoph = esophageal

The number of pulmonary artery catheterizations (standard, with vaso-reactivity testing using nitric oxide, with exercise, with concomitant CPET, and with esophageal probe performed at the Respiratory Institute has increased each year and has more than doubled since 2015. In addition, an increasing number of procedures include advanced techniques such as invasive CPET.

Actual and Predicted Survival of Group 1 Pulmonary Arterial Hypertension Patients (N = 446)

2022

The graph shows the survival curve for patients with category 1 pulmonary arterial hypertension enrolled in Cleveland Clinic’s Pulmonary Hypertension Registry, compared with their predicted survival based on the NIH registry equation¹ and with survival in a large French registry.² For both comparisons, Cleveland Clinic patients had better than expected survival.

Survival of Group 1 Pulmonary Arterial Hypertension Patients According to Current Risk Stratification (N = 446)

2022

COMPERA = Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension

The graph shows the survival curve for patients with group 1 pulmonary arterial hypertension enrolled in the Cleveland Clinic’s Pulmonary Hypertension Registry according to the most recent risk assessment criteria³ ⁴. In this cohort 3% of patients were classified as low risk, 28.5% intermediate-low risk, 41% intermediate-high risk, and 27.6% were high risk. The proportion of high-risk patients at the Cleveland Clinic is higher than in the COMPERA and French registries (15%)³ ⁴. Survival was better for the Cleveland Clinic patients. For example, 5-year survival for intermediate-high and high-risk patients was 74.4% and 40.1%, respectively, compared to 50.3% and 28.2% in COMPERA³, and 44% and 31% in the French registry⁴, respectively.

References

  1. D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT. Survival in patients with primary pulmonary hypertension; results from a national prospective registry. Ann Intern Med. 1991 Sep 1;115(5):343-349.
  2. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006 May 1;173(9):1023-1030.
  3. Hoeper MM, Pausch C, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Park DH, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Halank M, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Lange TJ, Rosenkranz S. COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension. Eur Respir J. 2022 Jul 7;60(1):2102311.
  4. Boucly A, Weatherald J, Savale L, de Groote P, Cottin V, Prévot G, Chaouat A, Picard F, Horeau-Langlard D, Bourdin A, Jutant EM, Beurnier A, Jevnikar M, Jaïs X, Simonneau G, Montani D, Sitbon O, Humbert M. External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry. Eur Respir J. 2022 Jun 30;59(6):2102419.