Process in which elevated pressure in the pulmonary arteries is diagnosed by echocardiography and right heart catheterization, and a group-based treatment plan is established.
Indication
- Unexplained progressive shortness of breath and reduced exercise capacity
- Syncope, chest pain, and palpitation complaints
- Evaluation in known heart failure or congenital heart disease
- Suspicion in the setting of chronic lung disease (COPD, pulmonary fibrosis)
- Suspected chronic thromboembolic pulmonary hypertension (CTEPH) after a previous pulmonary embolism
- Follow-up of connective tissue diseases (scleroderma, lupus)
- Family history of pulmonary arterial hypertension (PAH)
Preparation
- Current medication list and previous illnesses are reviewed
- Fasting for 6 hours before right heart catheterization
- Coagulation tests and renal function are checked
- Use of blood-thinning medications is regulated with physician approval
- Comfortable clothing and a companion are recommended on the day of the procedure
How it's performed
- Detailed cardiopulmonary examination and ECG
- Echocardiography (ECHO) for estimation of systolic pulmonary artery pressure
- Pulmonary function tests and 6-minute walk test
- Thoracic CT, V/Q scan (for CTEPH differential diagnosis)
- Right heart catheterization for definitive pressure measurement
- Classification: PAH (Group 1, including primary/idiopathic), left heart disease (Group 2), lung disease (Group 3), CTEPH (Group 4), multifactorial (Group 5)
Post-procedure
- Group-specific treatment: in PAH, endothelin receptor antagonists, PDE5 inhibitors, prostacyclin analogues
- In Group 2-3, treatment of the underlying disease takes priority
- Evaluation for pulmonary endarterectomy or balloon pulmonary angioplasty in CTEPH
- ECHO, 6-minute walk test, and BNP/NT-proBNP follow-up every 3-6 months
- Exercise prescription and vaccination (influenza, pneumococcus) recommendation
Risks
- Rare bleeding, arrhythmia, or vascular damage during right heart catheterization
- Side effects related to PAH medications (headache, hypotension, elevated liver enzymes)
- Development of right heart failure in untreated cases
- Pregnancy is high-risk in PAH; planning must be done with the physician
- Additional evaluation is needed for high-altitude travel and flights
FAQ
Can pulmonary hypertension be treated?
Effective treatments are available in early and correctly classified cases; CTEPH in particular can be completely resolved with surgery.
Can I exercise?
Low-to-moderate intensity exercise programs are beneficial with physician approval. Excessive strain should be avoided.
Are my family members at risk?
In idiopathic or hereditary PAH cases, evaluation of first-degree relatives is recommended.
What is the follow-up interval?
In stable patients, every 3-6 months; more frequent monitoring is needed when treatment changes.
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