Rationale and Strategies for Early Diagnosis of Pulmonary Arterial Hypertension

Course Director

Roham T. Zamanian, MD, FCCP

Roham T. Zamanian, MD, FCCP
Assistant Professor of Medicine
Director, Adult Pulmonary Hypertension Clinical Service
Vera Moulton Wall Center for Pulmonary Vascular Disease
Division of Pulmonary & Critical Care Medicine
Stanford University School of Medicine
Stanford, California

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Part 1 of a 2-part series

Dr. Zamanian provides expert feedback to the questions submitted by your peers during a recent survey on this topic.


In the first activity in this series, Dr. Zamanian answers questions related to screening and diagnosis of pulmonary arterial hypertension (PAH) from an electronic survey of US-based pulmonologists and cardiologists. The importance and value of earlier diagnosis and treatment, which may improve the prognosis for patients with PAH, is unequivocal. However, diagnosing PAH can be a challenge, as the early symptoms of the disease—such as dizziness, dyspnea, and fatigue—are common to other conditions, and patients may be asymptomatic at rest. Practice guidelines from the American College of Cardiology Foundation Task Force/American Heart Association in collaboration with the American College of Chest Physicians, American Thoracic Society, and the Pulmonary Hypertension Association state that diagnosis should include patient history, chest x‐ray, and electrocardiogram, confirmed by a complete right heart catheterization.

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How can we catch pulmonary arterial hypertension [PAH] early?

Dr. Zamanian: As PAH is a rare disease, it is important to have a consideration of the diagnosis in any patients with dyspnea of unexplained etiology. However, the most important aspect to early diagnosis of PAH is recognizing the population at risk. Certainly, patients who are idiopathic in etiology will have a presentation at typically a young age, and generally are female. But it's important to recognize that patients with connective tissue diseases, especially scleroderma, patients with congenital heart diseases, and patients who have had exposures to stimulants and diet pills are at exceptional risk for PAH.1 

Therefore, the guidelines recommend having a screening echocardiogram performed in patients with shortness of breath, fatigue, or exertional symptoms that are not explained by other disease processes. And typically those patients will have physical findings of right heart failure, including lower extremity edema, a loud pulmonic second heart sound, and a distended jugular vein.1 

So recognizing and diagnosing the disease early is crucial, because we believe that the sooner the patients are treated appropriately, the ultimately better outcome in the long term they will have. As this is an uncurable cardiopulmonary disease, it is likely best for patients to have an amount of time to educate themselves on the disease, and the psychosocial impact of the diagnosis will, in my opinion, be better tolerated in these patients.

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At what point should right heart catheterization be considered in a patient with PAH?

Dr. Zamanian: Pulmonary arterial hypertension requires a right heart catheterization as a gold-standard diagnostic test. Physicians base the diagnosis on echocardiographic results, which is appropriate as a screening tool but not a diagnostic tool. Therefore, the guidelines recommend that all patients with possible pulmonary arterial hypertension diagnosis receive a diagnostic right heart catheterization.1

Other noninvasive tests currently that could be used to confirm the diagnosis include pulmonary function tests, with specific attention to a reduced diffusion capacity; serologic testing, such as NT-proBNP [N-terminal pro-brain natriuretic peptide], to evaluate signs and serologic signs of right heart failure. And other tests include nuclear scanning to rule out pulmonary embolism, CT scanning to rule out interstitial lung disease, as well as evaluation of the patient's exertional performance using a 6-minute walk test.1

As the scientific technologies are improving, specifically in relation to pulmonary arterial hypertension, there are multiple novel technologies and methods being used that will eventually get incorporated into clinical guidelines. These include cardiac MRI imaging and all the specific modalities that come with the cardiac MRI. It includes novel echocardiographic parameters, such as the tricuspid annular plane systolic excursion, known as TAPSE, and three-dimensional echocardiographic testing as well.2,3 Some of them have higher sensitivities for evaluation of right ventricular performance and are currently being evaluated for their use in routine evaluation and treatment of patients. But those are currently considered novel and remain still to some extent experimental.

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What are the benefits and risks of early treatment for PAH?

Dr. Zamanian: Numerous studies and registries have found that all too often patients get referred for the diagnosis of PAH late in their disease in terms of symptomatology, and especially when patients are referred in New York Heart Association [NYHA] Class IV symptom setting, those patients don't do as well as patients who are NYHA Class II and III, when they receive even prostacyclin therapy.4,5 So essentially, at least in my opinion, there is no increased risk of early treatment when patients are diagnosed with PAH. However, the impact of side effects on quality of life for these patients should be recognized. So a patient with early disease, as defined by early symptoms, such as NYHA Class II symptoms, those patients can expect a rather substantial improvement in their symptoms with the use of PAH-specific therapies.

The studies have continued to show that despite its subjectivity, patient symptoms, as evaluated by the WHO or NYHA classification schemes, continue to predict long-term prognosis.5 So the goal of early diagnosis and early treatment is stabilization of disease, with the hope that that results in a longer and more meaningful life prognosis.

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  1. McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573‐1619.
  2. Iwasawa T. Magn Reson Med Sci. 2013;12:1-9.
  3. Sato T et al. J Am Soc Echocardiogr. 2012;25:280-286.
  4. Brown LM et al. Chest. 2011;140:19-26.
  5. Benza R et al. J Heart Lung Transplant. 2011;30:982-989.

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