Management of Pulmonary Hypertension: Specialist Referral
Patients with pulmonary hypertension should be managed by a multidisciplinary team at a specialized pulmonary hypertension center that includes both cardiologists and pulmonologists, not by a single pulmonologist or cardiologist alone. 1, 2
Why Specialized Centers Are Essential
Pulmonary hypertension is a rare, complex disease with high mortality that requires expertise beyond what a single specialist can provide. 1 The management involves:
Multidisciplinary team composition that must include at minimum two consultant physicians (from cardiology and/or respiratory medicine) with dedicated PH expertise, clinical nurse specialists, radiologists with PH imaging expertise, and physicians skilled in echocardiography and right heart catheterization. 1, 2
Access to comprehensive diagnostic capabilities including right heart catheterization laboratories, advanced imaging (CT, MRI, nuclear scanning), exercise testing, and vasoreactivity testing facilities. 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
Characteristics of Qualified PH Centers
A legitimate pulmonary hypertension referral center should meet these criteria:
Volume requirements: Follow at least 50 patients with PAH or CTEPH and receive at least 2 new referrals per month with documented disease. 1, 2
Technical expertise: Perform at least 20 vasoreactivity tests annually in appropriate PAH patients. 1, 2
Access to all approved therapies available in the country, including intravenous prostacyclin therapy and other advanced treatments. 1
Participation in clinical research including phase II and III clinical trials to advance treatment options. 1
When to Refer
All patients with suspected or confirmed pulmonary arterial hypertension (Group 1 PH) or chronic thromboembolic pulmonary hypertension (Group 4 CTEPH) must be referred to a specialized center. 2, 3, 4
Immediate referral is critical because treatment approaches differ fundamentally based on PH classification, and inappropriate therapy can cause harm. 5
Early telephone consultation between local physicians and PH specialists should occur promptly, with plans for in-person evaluation as soon as possible. 5
Common Pitfalls to Avoid
Do not attempt solo management: Even experienced cardiologists or pulmonologists should not manage PH patients alone outside of a specialized center, as the complexity requires multidisciplinary expertise. 1, 2, 6
Do not delay referral: Attempting to complete the entire workup locally without specialist input often results in incomplete evaluation and inappropriate therapy initiation. 5
Recognize the risk of therapy interruption: Abrupt discontinuation of prostanoid therapy can lead to rebound pulmonary hypertension and clinical deterioration, emphasizing the need for centers with 24/7 expertise. 2
Collaborative Care Model
While specialized centers provide definitive management, family physicians and general cardiologists maintain an important role in:
Managing comorbidities that contribute to PH, particularly in Group 2 (left heart disease) and Group 3 (lung disease) PH where no directed therapies exist. 7
Providing supportive care including immunizations, contraception counseling, preoperative assessment, and mental health support in coordination with the PH center. 7
Regular monitoring with follow-up assessments every 3-6 months in stable patients, coordinated with the specialized center. 2, 8