Pulmonary Vascular Program

Pulmonary Vascular Program

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. 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.

Patients Entered in the Pulmonary Hypertension Registry (N = 3023)
CategoryNumber of Patients
Pulmonary Arterial Hypertension
  • Idiopathic
415
  • Pulmonary veno-occlusive disease
26
  • Heritable
44
Associated with:
  • Connective tissue disease
331
  • Portal hypertension
128
  • Congenital heart disease
169
Left Heart Disease
  • Systolic dysfunction
106
  • Diastolic dysfunction
557
  • Valvular disease
164
Lung Disease and/or Hypoxia
  • Chronic obstructive pulmonary disease
138
  • Interstitial lung disease
153
  • Mixed restrictive/obstructive
63
  • Sleep-disordered breathing
37
  • Alveolar hypoventilation
13
Chronic Thromboembolic Pulmonary Hypertension501
Unclear or Multifactorial178
Total3023
Pulmonary Artery Catheterization Volume

2015 – 2021

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.

The COVID-19 pandemic had an impact on the operation of the Pulmonary Vascular Program, particularly in 2020. The providing of usual care returned for most of 2021. While SARS-CoV-2 infection might have direct implications for the pulmonary vasculature,¹ so far a clear link has not been seen between COVID-19 and new cases of pulmonary hypertension.

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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.