Pulmonary Vascular Program

Pulmonary Vascular Program

The Pulmonary Vascular Program at Cleveland Clinic consists of a team of 6 pulmonologists, 1 cardiologist, 2 advanced practice nurses, a nurse, a patient care coordinator, 2 research coordinators, and clinical and research fellows. Additionally, as part of Cleveland Clinic, the program draws on expertise in cardiothoracic surgery, lung transplantation, hepatology, liver transplantation, sleep medicine, and rheumatology to provide a comprehensive and multidisciplinary approach to patient care. Active clinical and research programs are offered for all types of pulmonary hypertension, including idiopathic pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, hereditary hemorrhagic telangiectasia, portopulmonary hypertension, congenital heart disease, and pulmonary hypertension associated with the scleroderma spectrum of diseases and other connective tissue diseases. The program offers advanced hemodynamic evaluation including invasive cardiopulmonary exercise testing (i.e., simultaneous right heart catheterization and cardiopulmonary exercise testing).

The 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 Pulmonary Vascular Program benefit from enrollment in 1 or more of these ongoing clinical trials and research studies.

Patients Entered in the Pulmonary Hypertension Registry (N = 2,417)
Category Number of Patients
**Pulmonary Arterial Hypertension**
- Idiopathic 369
- Pulmonary veno-occlusive disease 22
- Heritable 35
_Associated with:_
- Connective tissue disease 291
- Portal hypertension 117
- Congenital heart disease 160
**Left Heart Disease**
- Systolic dysfunction 92
- Diastolic dysfunction 443
- Valvular disease 158
**Lung Disease and/or Hypoxia**
- Chronic obstructive pulmonary disease 106
- Interstitial lung disease 110
- Mixed restrictive/obstructive 52
- Sleep-disordered breathing 32
- Alveolar hypoventilation 8
**Chronic Thromboembolic Pulmonary Hypertension** 263
**Unclear or Multifactorial** 159
**Total** **2417**
Pulmonary Artery Catheterization

The number of pulmonary artery catheterizations (standard and with exercise) performed at the Respiratory Institute has increased each year and has nearly doubled since 2015.

Actual and Predicted Survival of Patients With Category 1 Pulmonary Arterial Hypertension (N = 994)

2018

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. For example, actual 3-year survival for Cleveland Clinic patients was 67.6%, compared with 51.4% predicted by the NIH equation and 56.1% in the French registry.

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.