April 03, 2021
The Mayo Clinic pulmonary vascular disease program fulfills Mayo Clinic's commitment to clinical practice excellence, education enhancement and research advancement. Mayo Clinic's campuses in Phoenix/Scottsdale, Arizona; Jacksonville, Florida; and Rochester, Minnesota, are all Pulmonary Hypertension Association-Accredited Centers of Comprehensive Care, providing the expertise and resources necessary for the evaluation and integral care of patients with pulmonary vascular disease.
Pulmonary hypertension fellowship
"Patients with pulmonary hypertension often are diagnosed late in their course of disease, delaying intervention and ultimately leading to poorer outcomes," says Hector R. Cajigas, M.D., Pulmonology and Critical Care Medicine, at Mayo Clinic in Rochester, Minnesota. "We believe education is a cornerstone that allows practitioners — particularly specialists in cardiology, rheumatology and pulmonary medicine — to recognize, diagnose and treat patients with pulmonary hypertension."
In 2020, Pulmonary and Critical Care Medicine collaborated with Cardiovascular Medicine to enhance education and expand the number of specialists capable of fulfilling practice needs. Mayo Clinic in Rochester, Minnesota, became one of few medical centers in the United States offering a specialized fellowship in pulmonary hypertension. "This fellowship not only will create pulmonary hypertension specialists. It also will expand and motivate individuals to improve clinical practice and start research projects," notes Dr. Cajigas.
Chronic thromboembolic pulmonary hypertension
An important differentiator of Mayo Clinic's pulmonary medicine practice, notes Dr. Cajigas, is the successful performance of balloon pulmonary angioplasties for patients with nonoperable chronic thromboembolic pulmonary hypertension. Mayo Clinic is one of the few centers in the United States that have developed the experience and confidence to engage this practice.
Chronic thromboembolic pulmonary hypertension is a debilitating form of pulmonary hypertension that ultimately leads to right heart failure if uncorrected. When possible, pulmonary thrombendarterectomy, an open surgery that entails the dissection and extraction of the organized clots, provides the best outcomes. At Mayo Clinic in Rochester, Minnesota, cardiovascular surgery specialists provide this open surgical option through collaboration with pulmonary hypertension specialists.
In a portion of patients with this condition, either because of anatomic distribution and surgical inaccessibility of the clots or because the comorbid and surgical risks prohibit a surgical approach, medical therapy provides beneficial outcomes.
"In addition to medical therapy, current world experience on the use of balloon angioplasty of the affected nonoperable vessels has defined that when the appropriate program is combined with the appropriate experience, it is a safe procedure that provides improvement in quality of life and better performance in those affected individuals," says Dr. Cajigas.
At Mayo Clinic in Rochester, Minnesota, Gurpreet S. Sandhu, M.D., Ph.D., and Robert P. Frantz, M.D., Cardiovascular Medicine, are key players in the performance of these procedures. "Our initial experience, from 2014 to 2018, was published in Mayo Clinic Proceedings: Innovations, Quality & Outcomes in 2019. It describes 75 procedures in 31 patients. We found a significant improvement in hemodynamic values, six-minute walk distance, functional class and ventilatory efficiency in those patients. An acceptable rate of complication was only 4%," says Dr. Cajigas.
Under the leadership of Michael J. Krowka, M.D., Mayo Clinic's integrated practice of pulmonary and liver consultative services has led to current practices that foster shared knowledge and integral treatment of patients with portopulmonary hypertension and hepatopulmonary syndrome. The integrated service is part of a multidisciplinary approach to these complex cases, often facilitating the pursuit of appropriate treatments or comprehensive evaluation to facilitate liver transplantation or appropriate medical therapies.
Portopulmonary hypertension and liver transplant
In 2020, a group from the three Mayo Clinic sites, led by Rodrigo Cartin-Ceba, M.D., Pulmonary Medicine, at Mayo Clinic in Phoenix/Scottsdale, Arizona, documented clinical outcomes of patients with portopulmonary hypertension undergoing liver transplantation from 1996 to 2019. This research, published in Transplantation, highlighted the need to continue a comprehensive program of treatment and evaluation in this very high-risk group of patients.
On a larger scale, Hilary M. DuBrock, M.D., a pulmonologist at Mayo Clinic in Rochester, Minnesota, in collaboration with several other centers in the United States, explored the Organ Procurement and Transplantation Network database for patients with portopulmonary hypertension who were also liver transplant candidates. The research, published in Chest in 2021, demonstrated that in this rare condition, female patients had more compromised survival at a younger age than male counterparts. Females also had worse hemodynamics and lower model for end-stage liver disease scores as well as more likelihood to have autoimmune liver disease.
"This new information increases the wealth of knowledge into this rare disease with poor outcomes," says Dr. Cajigas. "Mayo Clinic specialists have developed years of experience and understanding of this condition, helping to make Mayo Clinic a destination center for the evaluation and treatment of patients with pulmonary-liver disease."
Hereditary Hemorrhagic Telangiectasia (HHT) Center of Excellence
Mayo Clinic in Rochester, Minnesota, is designated a center of excellence by Cure HHT, the national foundation dedicated to supporting efforts to diagnose, treat and organize research for patients with hereditary hemorrhagic telangiectasia. This disease affects the pulmonary vasculature, among other complications, requiring a comprehensive multidisciplinary approach to diagnosis and treatment. Vivek N. Iyer, M.D., M.P.H., director of the Mayo Clinic Hereditary Hemorrhagic Telangiectasia (HHT) Center of Excellence, co-authored updated guidelines for diagnosis and management of HHT, published in Annals of Internal Medicine in 2020. "Mayo's HHT center is a comprehensive referral center in the United States," notes Dr. Cajigas.
For more information
Pulmonary Hypertension Fellowship (Minnesota). Mayo Clinic.
Anand V, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: Initial single-center experience. Mayo Clinic Proceedings: Innovations, Quality & Outcomes. 2019;3:311.
Cartin-Ceba R, et al. Clinical outcomes after liver transplantation in patients with portopulmonary hypertension. Transplantation. In press.
Organ Procurement and Transplantation Network.
DuBrock HM, et al. Sex differences in portopulmonary hypertension. Chest. 2021;159:328.
Faughnan ME, et al. Second international guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia. Annals of Internal Medicine. 2020;173:989.