How to proceed with treatment of pulmonary hypertension in a 70-year-old patient with an RVSP (right ventricular systolic pressure) of 78 mmHg on echocardiogram?

Management of Pulmonary Hypertension in a 70-Year-Old Patient with RVSP of 78 mmHg

The patient with an RVSP of 78 mmHg should undergo immediate right heart catheterization to confirm pulmonary hypertension diagnosis, determine its classification, and initiate appropriate targeted therapy based on the underlying cause. 1

Diagnostic Evaluation Algorithm

1. Confirm PH diagnosis with right heart catheterization

   • Essential to verify the elevated RVSP of 78 mmHg seen on echocardiography
   • Obtain complete hemodynamic assessment including:
     • Mean pulmonary artery pressure (mPAP)
     • Pulmonary capillary wedge pressure (PCWP)
     • Pulmonary vascular resistance (PVR)
     • Cardiac output

2. Determine PH classification

   • Group 1: Pulmonary arterial hypertension (PAH)
   • Group 2: PH due to left heart disease
   • Group 3: PH due to lung disease/hypoxia
   • Group 4: Chronic thromboembolic PH (CTEPH)
   • Group 5: PH with unclear/multifactorial mechanisms

3. Additional diagnostic testing

   • Complete pulmonary function tests
   • Chest imaging (CT scan or high-resolution CT)
   • V/Q scan to rule out CTEPH 1
   • Laboratory studies including:
     • Complete blood count
     • Comprehensive metabolic panel
     • Thyroid function
     • NT-proBNP
     • HIV testing
     • Hepatitis serologies
     • Autoimmune antibody panel if ANA positive 1

Treatment Approach Based on PH Classification

For Group 1 (PAH)

1. Initial therapy:

   • For high-risk patients (which this patient likely is with RVSP 78 mmHg):
     • Consider intravenous epoprostenol at 2 ng/kg/min, titrated upward in increments of 2 ng/kg/min every 15 minutes until dose-limiting effects occur 2
   • For intermediate-risk patients:
     • Oral PAH-specific therapy with phosphodiesterase-5 inhibitors, endothelin receptor antagonists, or soluble guanylate cyclase stimulators

2. Combination therapy:

   • Consider upfront combination therapy for high-risk patients
   • Selexipag can be added to existing therapy, starting at 200 mcg twice daily and titrating up to maximum tolerated dose (up to 1600 mcg twice daily) 3

For Group 2 (PH due to left heart disease)

• Optimize treatment of underlying heart failure, valvular disease, or other left heart conditions
• Diuretics to reduce volume overload
• PAH-specific medications are generally not recommended

For Group 3 (PH due to lung disease)

• Optimize treatment of underlying lung disease
• Long-term oxygen therapy if hypoxemic
• Consider referral for lung transplantation evaluation

For Group 4 (CTEPH)

• Lifelong anticoagulation
• Refer to expert center for pulmonary endarterectomy evaluation
• If not a surgical candidate, consider balloon pulmonary angioplasty or medical therapy with riociguat 1

Monitoring and Follow-up

1. Regular clinical assessment:

   • Every 3-6 months based on severity and stability
   • Monitor for signs of right heart failure

2. Serial echocardiography:

   • To assess RV function and estimate pulmonary pressures
   • Every 3-6 months depending on clinical status 1

3. Functional capacity assessment:

   • 6-minute walk test
   • WHO functional class evaluation

Important Considerations and Pitfalls

• Age consideration: At 70 years, carefully assess for left heart disease as the most common cause of PH in this age group
• RVSP reliability: While an RVSP of 78 mmHg strongly suggests significant PH, echocardiographic estimates can sometimes be inaccurate, making right heart catheterization essential 1
• Avoid delay: The high RVSP (78 mmHg) indicates severe PH with increased mortality risk, requiring prompt evaluation and treatment 4
• Multidisciplinary approach: Involve a PH specialist team for optimal management, particularly if considering advanced therapies
• Beware of misclassification: Treating the wrong type of PH with PAH-specific medications can worsen outcomes, especially in left heart disease

Treatment Response Assessment

• Improvement in symptoms and functional capacity
• Reduction in RVSP on follow-up echocardiography
• Improvement in right ventricular function
• Decrease in NT-proBNP levels

This patient's elevated RVSP of 78 mmHg represents severe pulmonary hypertension requiring urgent evaluation and treatment to reduce morbidity and mortality.