Does pulmonary hypertension require consultation with a pulmonologist?

Management of Pulmonary Hypertension: The Role of Pulmonologist Consultation

Patients with pulmonary hypertension should be referred to specialized pulmonary hypertension centers with multidisciplinary teams that include pulmonologists and cardiologists, rather than to a pulmonologist alone. 1

Specialized Care Requirements for Pulmonary Hypertension

• Pulmonary hypertension (PH) is a complex condition requiring management at specialized referral centers with expertise in PH diagnosis and treatment 1
• PH is defined as a mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg with a pulmonary capillary wedge pressure ≤ 15 mm Hg measured by cardiac catheterization 1
• The 2015 ESC/ERS guidelines recommend referral to centers that provide care through a multidisciplinary team including both cardiology and respiratory medicine physicians 1

Multidisciplinary Team Approach

• Referral centers should provide care through an interprofessional team that includes, at minimum 1:
  • Two consultant physicians (typically from cardiology and respiratory medicine) with special interest in PH
  • Clinical nurse specialist
  • Radiologist with expertise in PH imaging
  • Cardiologist or PH physician with expertise in echocardiography
  • Cardiologist or PH physician with expertise in right heart catheterization and vasoreactivity testing
  • Access to psychological and social work support

When to Refer to a PH Center

• Patients with suspected or confirmed PAH should be referred to a specialized PH center rather than to a pulmonologist alone 1
• Referral is particularly urgent for patients with clinical high-risk features or those at risk for pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) 2
• The American College of Chest Physicians (ACCP) strongly recommends referral to specialized centers due to the complexity of diagnostic evaluation and treatment options available 1

Characteristics of PH Referral Centers

• Referral centers should follow at least 50 patients with PAH or CTEPH and receive at least two new referrals per month 1
• Centers should perform at least 20 vasoreactivity tests in PAH patients per year 1
• Facilities should include specialized wards, intensive care units, outpatient services, and comprehensive diagnostic capabilities 1
• Centers should have established networks with other services including genetics, connective tissue disease specialists, family planning, pulmonary endarterectomy, lung transplantation, and adult congenital heart disease specialists 1

Pitfalls of Inadequate Specialized Care

• Delays in diagnosis and appropriate treatment can lead to disease progression, right ventricular failure, and increased mortality 3
• Inappropriate use of pulmonary vasodilators in certain types of PH (such as PH due to left heart disease or lung diseases) may worsen gas exchange and clinical outcomes 4
• Abrupt interruption of prostanoid therapy can lead to rebound pulmonary hypertension and clinical deterioration 5
• Patients with severe PH may require interventions not readily available outside specialized centers, such as pulmonary artery catheterization, inhaled pulmonary vasodilators, or mechanical support 3

Follow-up and Monitoring

• Regular clinical assessment every 3-6 months is recommended to evaluate functional class, exercise capacity, and signs of disease progression 5
• Management should include risk assessment and appropriate adjustment of therapy based on treatment goals 6
• Participation in clinical research and trials is encouraged at specialized centers to advance treatment options 1

In conclusion, while pulmonologists play a crucial role in the management of pulmonary hypertension, patients should be referred to specialized PH centers with multidisciplinary teams rather than to a pulmonologist alone, to ensure comprehensive care and optimal outcomes.