PH is a life-threatening cardiopulmonary disease characterized by progressive right heart failure and premature death. Among the various forms of PH, pulmonary arterial hypertension (PAH) is a devastating condition that can occur on its own or as a consequence of chronic diseases such as liver disease, scleroderma, or congenital heart disease. Even with aggressive medical therapy and specialized medical care, current PAH event-free 5-year survival rates peak at only 50-65%.^1,2 PAH is considered a rare disease among the general population, with an estimated prevalence of 5-52 per million adults.^3-7 However, modern patient registries have shown that the annual incidence and prevalence of all types of PH over time has increased to 28.7/100,000 and 127.3/100,000 respectively, and that a diagnosis of PH is associated with an estimated sevenfold increase in the 1-year standardized mortality ratio.^8,9 These disturbing trends highlight the growing awareness of the potentially catastrophic consequences that PH can have if left undiagnosed.

Consensus statements among PH experts endorse that early, accurate diagnosis is critical to providing patients with PH with optimal outcomes. Patients too often present at advanced stages of disease, which most PH experts believe adversely impacts outcome in spite of confounders like lead-time bias.¹⁰ Indeed, over the past 3 decades, the average time between symptom onset and diagnosis is consistently greater than 2 years¹¹, and about 70% of newly diagnosed patients are symptomatic with less than ordinary activity or at rest.^3,6,12 These patients are classified as New York Heart Association Functional Class III or IV and on average die sooner than their healthier Class I and II counterparts (estimated 3-year survival rates are 30-65% versus 85-90%, respectively).¹³ Lack of recognition of PH "red flag" symptom patterns like progressive dyspnea, chest pain, and syncope and general unfamiliarity with PH as a disease process among health care practitioners are thought to significantly contribute to these sobering statistics.

In addition to missing early diagnosis, clinicians may have difficulty accurately diagnosing the specific forms of PH afflicting their patients. In an effort to standardize the management of PH, the task forces of the 2018 World Symposium on PH recognized 5 major forms of PH and more than 25 different subclasses requiring different treatment approaches.¹⁴ Using this clinical classification, subspecialists have to decide whether a patient would benefit from or be harmed by the use of complex pulmonary vasodilators, open-heart surgery, aggressive treatment of the underlying disease process, or lung transplantation. PH quality initiatives have shed light on the fact that the misclassification of patients with PH is all too common. In one study, 33% of patients initially diagnosed with some form of PH had to be reclassified by a tertiary care center team once transferred there for higher level of care.¹⁵ Furthermore, in another investigation, incomplete guideline-driven diagnostic work-ups plagued 94% of cases, and 93% of patients were inappropriately receiving calcium-channel blockers,³ a treatment reserved for vasoreactive disease that is proven effective in only 6.8% of patients with PAH.¹⁶ These studies highlight the urgent need for educational resources to assist practitioners in choosing the best practices for PH diagnosis and management in a timely manner.

In 2011, the Scientific Leadership Council of the Pulmonary Hypertension Association (PHA), comprising 28 global leaders in the field of PH, spearheaded the PHA-accredited PH Care Centers initiative. The PH Care Centers initiative is a program that accredits PH care centers capable of demonstrating excellence in the quality of care delivered to patients with PH as well as a commitment to improve PH clinical outcomes nationwide. Criteria were designed for the declaration of Centers of Comprehensive Care and Regional Clinical Programs across the country. These criteria for adult patients with PH covered important areas like access to multidisciplinary care teams, cardiac catheterization laboratories, and cutting-edge research. They also mandated that center directors be trained and board-certified in pulmonary and critical care or cardiovascular medicine, have at least 2 years of experience treating PAH beyond fellowship training, and complete 30 hours of continuing medical education related to PH over the 3 years preceding directorship.¹⁷

Outside of PHA-accredited PH Care Centers, it is important to recognize that there are a large number of pulmonologists and cardiologists in the United States who treat patients without formal subspecialty training or direct supervision from a PH expert. As of July 1, 2020, the Accreditation Council for Graduate Medical Education program requirements for graduate medical education in adult pulmonary and critical care medicine, cardiology, and advanced heart failure and transplant cardiology fellowships mention pulmonary hypertension only once as part of a list of conditions in which fellows should demonstrate competence, although no further details are provided.¹⁸ Because not all academic training programs treat PH, fellows may graduate with minimal to no experience in the management of these complex patients.

Perhaps the most commonly applied PH training model employed to date is the apprenticeship model; in which practitioners learn by shadowing other more senior PH-focused practitioners. Some of these practitioners are bright, motivated pulmonologists or cardiologists who carefully review guidelines on the diagnosis and treatment of PH and try to uphold standards of care. But some are not. And other practitioners do not subscribe to any training model; they apply antiquated diagnostics and therapeutics and, albeit with good intentions, often misdiagnose and mistreat patients with PH. In light of these concerns and our steadfast commitment to offering the best level of care to our patients with PH, we contend that formal, subspecialty training in PH is imperative.

At Stanford University's Vera Moulton Wall Center for Pulmonary Vascular Disease, home to 1 of the first 6 accredited adult PHA-accredited PH Care Centers, we have trained 1-2 fellows per year since 2000 in a 1-year fellowship program dedicated to PH. This fellowship program harbors a detailed curriculum, goals and objectives platform, and milestone-based 360° evaluation system. Fellows devote 12 months to the inpatient and outpatient care of patients with PH. Importantly, they also become certified in the execution and interpretation of right heart catheterizations, the gold standard diagnostic test for PH. Moreover, among other requirements, they are expected to attend or present at a bimonthly Stanford PH grand rounds series that is accredited for continuing medical education and complete a scholarly pursuit project in PH prior to graduation and certification. We are proud to state that we have certified 22 fellows thus far in pulmonary vascular disease. When they leave their year of PH fellowship training here, we are confident that they possess the knowledge and tools to expertly navigate and teach the complexities in PH diagnostics, therapeutics, and research.

This confidence is grounded in some tangible measures of programmatic success: 13 fellows who graduated the PH fellowship are in academic medicine, 8 are PH center directors, and 1 is a renowned clinical proceduralist performing balloon pulmonary angioplasty at a major chronic thromboembolic PH center.¹⁹ Additionally, our fellows together have produced more than 200 publications in the field of PH. We believe their success is due in large part to the focused, necessary training they received as PH fellows.

Notably, we are not alone in our desire to enhance PH education. In 2018, we conducted a survey among 55 PHA-accredited PH Care Center directors and found that 94% of respondents agreed that 1) subspecialty training in PH beyond current cardiology and pulmonary fellowship training is warranted, 2) were themselves interested in sponsoring a PH fellowship at their institution, and 3) wanted to participate in standardized curriculum development for a PH subspecialty fellowship. The main barrier to implementation of a PH fellowship for 70% of these respondents was lack of funding. Additionally, 87% claimed that fellows in training have been and continue to be interested in pursuing PH subspecialty training.²⁰

In summary, PH persists as a progressive, devastating disease. Even today, patients are still diagnosed too late and, at times, inaccurately. The majority of accredited PH providers acknowledge that further specialized training in PH is essential. We at Stanford have trained PH fellows for two decades now with great success, thanks in large part to a comprehensive curriculum that balances the clinical aspects of PH care with educational and research practices. Implementation at the national level of an accredited PH fellowship is the next step, one which we hope would ultimately break down funding barriers and arm the next generation of PH practitioners with the subspecialty knowledge and experience this disease warrants in an effort to enhance outcomes.

Given the lack of curative options, successful management of PH will remain dependent on well-trained practitioners who do not flinch when faced with challenging clinical scenarios. It is our duty to serve our patients using the best of our abilities.

References

1. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation 2009;119:2250-94.
2. Zamanian RT, Hedlin H, Greuenwald P, et al. Features and Outcomes of Methamphetamine-associated Pulmonary Arterial Hypertension. Am J Respir Crit Care Med 2018;197:788-800.
3. 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;173:1023-30.
4. Ling Y, Johnson MK, Kiely DG, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med 2012;186:790-6.
5. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013;62:D51-9.
6. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010;137:376-87.
7. Peacock AJ, Murphy NF, McMurray JJV, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007;30:104-9.
8. Wijeratne DT, Lajkosz K, Brogly SB, et al. Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension: A Population-Based Cohort Study in Ontario, Canada. Circ Cardiovasc Qual Outcomes 2018;11:e003973.
9. Ryan JJ, Rich JD, Maron BA. Building the case for novel clinical trials in pulmonary arterial hypertension. Circ Cardiovasc Qual Outcomes 2015;8:114-23.
10. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J 2019;53:1801904.
11. Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest 2011;140:19-26.
12. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national perspective study. Ann Intern Med 1987;197:216-23.
13. McLaughlin VV, Langer A, Tan M, et al. Contemporary trends in the diagnosis and management of pulmonary arterial hypertension: an initiative to close the care gap. Chest 2013;143:324-32.
14. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019;53:1801913.
15. Deaño RC, Glassner-Kolmin C, Rubenfire M, et al. Referral of patients with pulmonary hypertension diagnoses to tertiary pulmonary hypertension centers: the multicenter RePHerral study. JAMA Intern Med 2013;173:887-93.
16. Sitbon O, Humbert M, Jaїs X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005;111:3105-11.
17. Accreditation Criteria (Pulmonary Hypertension Association website). 2020. Available at: https://phassociation.org/phcarecenters/center-criteria/. Accessed August 20, 2020.
18. Specialties (Accreditation Council for Graduate Medical Education website). 2020. Available at: https://acgme.org/Specialties. Accessed August 20, 2020.
19. Clinical Fellowships (Standford Medicine website). 2020. Available at: http://med.stanford.edu/wallcenter/education/clinical-fellowship.html. Accessed August 20, 2020.
20. Kudelko K, Sung Y, Zamanian R. The current state of pulmonary hypertension subspecialty education: results of a national survey. Adult Pulmonary Hypertension Program, Division of Pulmonary and Critical Care Medicine, Stanford University. 2018; unpublished.

Clinical Topics: Cardiovascular Care Team, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Pulmonary Hypertension, Hypertension

Keywords: Hypertension, Pulmonary, Calcium Channel Blockers, Education, Medical, Continuing, Survival Rate, Incidence, Vasodilator Agents, Mortality, Premature, Rare Diseases, Consensus, Inpatients

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