What is the diagnosis for a patient with severe pulmonary hypertension, as indicated by a Pulmonary Artery (PA) pressure of 63/32/41 mmHg, Pulmonary Capillary Wedge (PCW) pressure of 7 mmHg, Right Ventricular (RV) pressure of 5/2/11 mmHg, and Right Atrial (RA) mean pressure of 8 mmHg, with a Cardiac Output (CO) of 2.7 liters per minute and Pulmonary Vascular Resistance (PVR) of 12 Woods units?

From the Guidelines

The patient should be started on pulmonary vasodilators, such as phosphodiesterase-5 inhibitors (sildenafil 20 mg three times daily or tadalafil 40 mg once daily) or endothelin receptor antagonists (ambrisentan 5-10 mg daily or bosentan 62.5 mg twice daily for 4 weeks, then 125 mg twice daily), to manage severe pulmonary hypertension, as evidenced by the elevated pulmonary artery pressures (63/32/41 mmHg) and pulmonary vascular resistance (12 Wood units) 1. The right heart catheterization results indicate severe pulmonary hypertension with significantly elevated pulmonary artery pressures and pulmonary vascular resistance. The patient has a low cardiac output (2.7 L/min) and cardiac index (1.7 L/min/m²), suggesting compromised right heart function.

• Key findings from the right heart catheterization include:
  • Pulmonary artery pressures: 63/32/41 mmHg
  • Pulmonary vascular resistance: 12 Wood units
  • Cardiac output: 2.7 L/min
  • Cardiac index: 1.7 L/min/m²
• The patient's low cardiac output and cardiac index suggest right heart failure, which can be managed with diuretics and oxygen therapy to maintain oxygen saturation above 90% 1.
• Referral to a pulmonary hypertension specialist center is recommended for comprehensive management, as severe pulmonary hypertension can lead to right heart failure if not properly treated 1.
• The use of exercise training, such as pulmonary rehabilitation, may be beneficial in improving mobility, social interaction, exercise tolerance, and quality of life, but should be done under the guidance of a pulmonary hypertension specialist and with careful monitoring of the patient's condition 1.

From the FDA Drug Label

Baseline Mean Change from Baseline at End ofTreatment Period* Hemodynamic Parameter Epoprostenol(N = 52) StandardTherapy(N = 54) Epoprostenol(N = 48) StandardTherapy(N = 41) CI (L/min/m2) 2 2 0.3†† -0. 1 PAPm (mmHg) 60 60 -5† 1 PVR (Wood U) 16 17 -4† 1

Table 12: Hemodynamics during Chronic Administration of Epoprostenol in Patients with PAH/SSD CI = cardiac index, PAPm = mean pulmonary arterial pressure, RAPm = mean right atrial pressure, PVR = pulmonary vascular resistance, SAPm = mean systemic arterial pressure * Denotes statistically significant difference between Epoprostenol and conventional therapy groups (N is the number of patients with hemodynamic data). Baseline Mean Change from Baseline at 12 Weeks HemodynamicParameter Epoprostenol(N = 56) ConventionalTherapy(N = 55) Epoprostenol(N = 50) ConventionalTherapy(N = 48) CI (L/min/m2) 1.9 2.2 0.5* -0. 1 PAPm (mmHg) 51 49 -5* 1 RAPm (mmHg) 13 11 -1* 1 PVR (Wood U) 14 11 -5* 1

The patient has Severe Pulmonary Hypertension with a PVR of 12 Woods units. The drug label for epoprostenol shows that it can decrease PVR and increase CI in patients with pulmonary hypertension.

• Key benefits of epoprostenol include:
  • Decrease in PVR
  • Increase in CI
  • Improvement in exercise capacity
  • Reduction in dyspnea and fatigue Based on the information provided, epoprostenol may be beneficial for this patient with severe pulmonary hypertension, as it has been shown to improve hemodynamic parameters and exercise capacity in similar patients 2.

From the Research

Patient Data

• Right Heart Catheterization results:
  • PCW: 7 mmHg
  • PA: 63/32/41 mmHg
  • RV: 5/2/11
  • RA mean: 8 mmHg
  • PA sat: 61%
  • mRA sat: 58%
  • No significant shunt noted
  • CO: 2.7 Liters per minute
  • CI: 1.7 Liters per minute per meter squared
  • PVR: 12 Woods units
  • Severe Pulmonary Hypertension

Diagnosis and Treatment

• The patient has been diagnosed with severe Pulmonary Hypertension, which is a debilitating chronic disorder of the pulmonary vasculature characterized by elevated mean pulmonary arterial pressure, right-sided heart failure, and early mortality 3.
• Treatment options for Pulmonary Arterial Hypertension (PAH) include targeted therapies such as phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists 4.
• The use of intravenous epoprostenol has been shown to improve exercise capacity and survival in patients with PAH, but it can have intolerable side effects, and switching to treprostinil may be an option 5.
• Subcutaneous treprostinil has been shown to have favorable hemodynamic effects and can be given safely to ambulatory patients with a novel subcutaneous delivery pump system 6.

Management and Care

• Patients with PAH require anticipatory interventions to prevent severe exacerbations of the pulmonary hypertensive condition, promote pulmonary vasodilation, and optimize ventricular function 7.
• Nurses play a critical role in providing anticipatory care to prevent the development of pulmonary hypertensive events and in monitoring ongoing responses to adjustments in therapeutic interventions 7.
• Right-sided heart catheterization is essential for accurate diagnosis and classification of PAH, and all patients with PAH or CTEPH must be referred to a specialist center 4.