The success of treatment for pulmonary hypertension (PH), including pulmonary arterial hypertension (PAH), is dependent on early detection and an accurate diagnosis. “PH treatments are typically directed toward the underlying cause, but there can be overlap between various patient groups,” says Mardi Gomberg-Maitland, MD, MSc. “This overlap can complicate diagnostic approaches, therapeutic choices, and anticipated outcomes.”

Patients are often referred to PH centers with incomplete evaluations. “PH centers offer important benefits to patients because they have clinicians and nurse teams who are dedicated and trained in caring for these patients,” says Dr. Gomberg-Maitland. “Unfortunately, patients are often referred late in the course of illness. Some are given PAH-specific medications without appropriate testing or indications.” Furthermore, PAH medications are expensive and difficult to manage.

Data on Pulmonary Hypertension Diagnosis & Referral

In JAMA Internal Medicine, Dr. Gomberg-Maitland, Cherylanne Glassner-Kolmin, BS, and colleagues had a study published that assessed PH diagnosis accuracy in patients referred to PH centers. “We also wanted to look at the appropriateness of use of PAH-specific medications based on current guidelines,” says Glassner-Kolmin. According to findings, 68% of patients were referred by cardiologists or pulmonologists to PH centers and 61% had late-stage disease. One-third of patients who received a definitive PAH diagnosis before their referral received a misdiagnosis. “We also found that many patients were treated contradictorily to published recommendations, either having not undergone a heart catheterization, having been inappropriately prescribed medications, or both,” says Glassner-Kolmin.

Pulmonary-Hypertension-Callout

Dr. Gomberg-Maitland says major efforts have been made to educate medical professionals about PH, but misdiagnoses and inappropriate treatment are still occurring. “Inexperienced clinicians should be cautious of giving patients a definitive diagnosis that implies a poor prognosis.”

Early Referrals Matter in Pulmonary Hypertension

Guidelines recommend that physicians refer patients early to PH centers and before medications are initiated, even if the pre-referral diagnosis is incomplete. “Physicians with experience in the technical and interpretive aspects of PH should perform testing before any drug is administered,” says Glassner-Kolmin. “Referrals to PH centers are also warranted for confirmation and joint management so patients can receive advanced therapies early and perhaps be enrolled in studies involving novel or approved therapies.”

Dr. Gomberg-Maitland adds that successful care requires that PH referral centers respond to and communicate with referring physicians and their patients. “Current educational efforts haven’t succeeded in promoting early diagnosis or early referral,” she says. “PAH necessitates a team approach to treatment strategies because of the potential need for multiple therapies and repeated objective testing to monitor progression.”

References

Deano 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;173:887-893. Available at: http://archinte.jamanetwork.com/article.aspx?articleid=1675880.

Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54 (suppl):S55-S66.

Gomberg-Maitland M, Dufton C, Oudiz RJ, Benza RL. Compelling evidence of long-term outcomes in pulmonary arterial hypertension? a clinical perspective. J Am Coll Cardiol. 2011;57:1053-1061.

McLaughlin VV, Archer SL, Badesch DB, et al; American College of Cardiology Foundation/American Heart Association. 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. Circulation. 2009;119:2250-2294.

Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long- Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010;122:164-172.

Thenappan T, Shah SJ, Rich S, Tian L, Archer SL, Gomberg-Maitland M. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J. 2010;35:1079-1087.