Is a pulmonologist consultation useful to evaluate pulmonary hypertension?

Pulmonary Hypertension Evaluation: Specialist Referral Recommendation

Patients with suspected or confirmed pulmonary hypertension should be referred to a specialized multidisciplinary pulmonary hypertension center—not to a pulmonologist alone—as this approach is associated with superior clinical outcomes and is strongly recommended by international guidelines. 1, 2

Why Specialized PH Centers, Not Just Pulmonology Consultation

The Multidisciplinary Requirement

Pulmonary hypertension management requires an interprofessional team approach that extends beyond pulmonology expertise alone. 1 The 2015 ESC/ERS guidelines explicitly recommend that referral centers provide care through a team that includes:

• At minimum two consultant physicians from either or both cardiology and respiratory medicine with dedicated PH expertise 1
• Clinical nurse specialists with PH training 1
• Radiologists with PH imaging expertise 1
• Cardiologists or PH physicians skilled in echocardiography and right heart catheterization with vasoreactivity testing 1
• Access to psychological and social work support 1, 2

This multidisciplinary structure is a Class I recommendation (highest level), reflecting that medical centers with high patient volumes and comprehensive expertise achieve the best outcomes. 1

Why Pulmonology Alone Is Insufficient

A pulmonologist consultation in isolation lacks the critical diagnostic and therapeutic infrastructure required for optimal PH management. 2 Specialized PH centers must provide:

• Comprehensive diagnostic capabilities including right heart catheterization laboratories, advanced imaging (CT, MRI, nuclear scanning), and exercise testing facilities 1
• Access to the full range of PAH and CTEPH-specific drug therapies available in the country 1
• Established networks with other essential services including genetics, connective tissue disease specialists, pulmonary endarterectomy surgeons, lung transplantation programs, and adult congenital heart disease specialists 1, 2
• Intensive care capabilities with relevant PH expertise for managing acute decompensation 1, 3

Volume and Expertise Standards

Referral centers should follow at least 50 patients with PAH or CTEPH and receive at least two new referrals per month with documented disease. 1, 2 Centers should perform at least 20 vasoreactivity tests annually in appropriate PAH patients. 1, 2 These volume requirements ensure sufficient expertise to manage this rare and complex condition effectively. 1

Clinical Implications and Pitfalls

Critical Diagnostic Considerations

Right heart catheterization is essential to confirm PAH diagnosis and guide treatment decisions—this specialized procedure requires expertise typically found only at PH centers. 1, 4 Vasoreactivity testing during catheterization identifies the subset of patients who may respond to calcium channel blockers, a determination that cannot be made through standard pulmonology evaluation alone. 1, 4

Treatment Complexity Requires Specialized Care

Patients on pulmonary vasodilator therapy, particularly prostacyclin analogues, can rapidly deteriorate and die if these medications are interrupted. 3 This underscores why PH patients need access to centers with 24/7 expertise and appropriate on-call coverage. 1 Emergency physicians should consult PH specialists early and consider transfer to tertiary centers with invasive monitoring and mechanical support capabilities. 3

Risk of Inappropriate Management

Without specialized PH center evaluation, patients risk:

• Misclassification of PH type, leading to inappropriate therapy (e.g., PAH-specific drugs are not recommended for PH due to left heart disease or lung disease) 1
• Missed surgical opportunities in CTEPH, where pulmonary endarterectomy can be curative but requires multidisciplinary team assessment 1
• Suboptimal risk stratification and treatment selection, as therapy should be guided by comprehensive risk assessment using clinical parameters, exercise capacity, biomarkers, and imaging 1, 4

Practical Referral Approach

When PH is suspected based on unexplained dyspnea on exertion, syncope, or signs of right ventricular dysfunction 3, 5:

1. Initiate basic evaluation with echocardiography, but recognize this is screening only 5
2. Refer promptly to a specialized PH center rather than to general pulmonology 2, 5
3. Use fast-track referral pathways for clinically high-risk patients or those at risk for PAH or CTEPH 5
4. Ensure continuity of existing PH medications if patient is already on therapy, as abrupt discontinuation can cause rebound pulmonary hypertension and clinical deterioration 2, 3

The complexity of PH diagnosis, classification into five distinct groups with different treatment approaches, and the need for specialized therapeutic interventions make referral to comprehensive PH centers—not isolated pulmonology consultation—the standard of care. 2, 6