Pulmonary Vascular Program

Pulmonary Vascular Program

The COVID-19 pandemic created challenges never seen before by the Pulmonary Vascular Program. The program resorted to virtual platforms to remain connected to our patients, while ensuring that those who needed in person care were appropriately identified and supported. The Pulmonary Vascular Program at Cleveland Clinic consists of a team of 7 pulmonologists, 2 cardiologists, 2 advanced practice nurses, a nurse, 2 program coordinators, and 2 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. 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 (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.

Patients Entered in the Pulmonary Hypertension Registry (N = 2,829)
CategoryNumber of Patients
Pulmonary Arterial Hypertension
  • Idiopathic
402
  • Pulmonary veno-occlusive disease
26
  • Heritable
36
Associated with:
  • Connective tissue disease
326
  • Portal hypertension
125
  • Congenital heart disease
168
Left Heart Disease
  • Systolic dysfunction
94
  • Diastolic dysfunction
501
  • Valvular disease
164
Lung Disease and/or Hypoxia
  • Chronic obstructive pulmonary disease
126
  • Interstitial lung disease
140
  • Mixed restrictive/obstructive
63
  • Sleep-disordered breathing
37
  • Alveolar hypoventilation
11
Chronic Thromboembolic Pulmonary Hypertension432
Unclear or Multifactorial178
Total2829
Pulmonary Artery Catheterization Volume

2015 – 2020

CPET = cardiopulmonary exercise test

The number of pulmonary artery catheterizations (standard, with vaso-reactivity testing using nitric oxide, with exercise and with concomitant CPET) performed at the Respiratory Institute has increased each year and has more than doubled since 2015. The decrease in 2020 volumes is a result of significant changes to the number of patients treated, which can be directly attributed to the COVID-19 pandemic.

Actual and Predicted Survival of Patients with Category 1 Pulmonary Arterial Hypertension (N = 1,071)

2020

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 68.8%, compared with 51.3% predicted by the NIH equation and 55.6% in the French registry.

Pulmonary Hypertension Patients Who Tested Positive for COVID-19 at Cleveland Clinic (N = 41)

March 2020 – February 2021

OutcomeDeath (N = 5)Survival (N = 36)
Age mean (SD)67 (6)58 (14)

Female

Male

3 (60%)

2 (40%)

26 (72%)

10 (28%)

ICU Stay3 (60%)4 (11%)
Hospitalization3 (60%)13 (36%)

The COVID-19 pandemic had an impact on the operation of the Pulmonary Vascular Program. SARS-CoV-2 infection might have direct implications for pulmonary hypertension patients .³ Among 636 patients with all forms of pulmonary hypertension actively followed by the program, 41 cases were observed with COVID-19 (6.4% of the cohort), and 5 of 41 patients died (mortality 12%).

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. Farha S, Heresi GA. COVID-19 and Pulmonary Arterial Hypertension: Early Data and Many Questions. Ann Am Thorac Soc. 2020 Dec;17(12):1528-1530.