NURS 506 Week 7 Cardiovascular-Pulmonary Presentation on Pulmonary Arterial Hypertension Example

Pulmonary Arterial Hypertension in Older Adults: From the Endothelial Cell to the Bedside

Slide 1: Why This Matters for Older Adults

• No longer only a disease of young women
• Older patients are diagnosed later and sicker
• Nurses often notice the first signs

Speaker notes: Pulmonary arterial hypertension was long described as a disease of young adults, especially women. Registries now show more patients diagnosed in their 60s, 70s and 80s. In older adults, breathlessness is easy to blame on age, deconditioning or the heart, so the disease is often found late. Nurses who understand its mechanism can recognize it sooner.

Slide 2: Definition

• Mean pulmonary artery pressure above 20 mm Hg at rest
• Precapillary: wedge pressure 15 mm Hg or less
• Pulmonary vascular resistance above 2 Wood units

Speaker notes: The 2022 European guidelines lowered the threshold for pulmonary hypertension to a mean pulmonary artery pressure above 20 mm Hg, measured by right heart catheterization. Pulmonary arterial hypertension is the precapillary form, with a normal left-sided filling pressure and raised resistance (Humbert et al., 2022). It is group 1 of five groups; most older adults with high pressures have group 2 disease from the left heart, which is why careful diagnosis matters.

Slide 3: Incidence and Prevalence

• About 6 new cases per million adults each year
• About 48 to 55 cases per million adults living with it
• Rising share of older patients

Speaker notes: Rare as it is, this disease now reaches older adults more often than our training suggests. In European registries, pulmonary arterial hypertension has an incidence of about 6 and a prevalence of about 48 to 55 cases per million adults (Humbert et al., 2022). It is rare, but its share among older adults is growing. In the U.S. Pulmonary Hypertension Association Registry, 113 of 3,207 patients were 80 or older (Reddy et al., 2026).

Slide 4: The Endothelium Loses Its Balance

• Nitric oxide and prostacyclin fall
• Endothelin-1 rises
• Vasoconstriction plus growth signals

Speaker notes: The disease begins in the endothelial cells of the small pulmonary arteries. Normally these cells release nitric oxide and prostacyclin, which relax smooth muscle and inhibit its growth, balanced by endothelin-1, a vasoconstrictor. In this disease the balance shifts: nitric oxide and prostacyclin production fall, and endothelin-1 production rises, so the vessels constrict and their smooth muscle receives a constant signal to grow (McCance & Huether, 2019).

Slide 5: Cells That Grow and Will Not Die

• Smooth muscle and endothelial cells proliferate
• Resistance to apoptosis
• BMPR2 and activin signaling out of balance

Speaker notes: Vasoconstriction is only part of the story. Smooth muscle cells, fibroblasts and endothelial cells multiply and resist programmed cell death, thickening the vessel wall, while in situ clots and inflammation add to the narrowing. Many heritable cases involve loss-of-function variants in the gene for bone morphogenetic protein receptor type 2, and its loss tilts signaling in the transforming growth factor beta family toward growth-promoting activin pathways (Humbert et al., 2022).

Slide 6: Remodeled Vessels

• Medial hypertrophy and intimal thickening
• Plexiform lesions in advanced disease
• Lumen narrows, resistance climbs

Speaker notes: Over time, the media thickens, the intima scars, and complex plexiform lesions of disordered endothelial channels form. The narrowed lumens raise pulmonary vascular resistance, and because the remodeling is structural, vasodilators alone cannot fully reverse it.

Slide 7: The Right Ventricle Pays the Price

• Hypertrophy, then dilation
• Tricuspid regurgitation, falling output
• Right ventricular failure causes most deaths

Speaker notes: The thin-walled right ventricle is built for low pressure. Facing rising resistance, it first hypertrophies, then dilates and fails. As it enlarges, the septum shifts toward the left ventricle and limits its filling, so cardiac output falls further. Exertional breathlessness, fatigue, chest pain and syncope follow, and right heart failure is the usual cause of death.

Slide 8: Nursing Assessment

• Exertional dyspnea, fatigue, presyncope
• Loud P2, right ventricular heave, tricuspid murmur
• Raised jugular veins, edema, enlarged liver

Speaker notes: Each finding traces back to the mechanism. When the pulmonic valve closes loudly, accentuating P2, the pressure behind it is high; a left parasternal heave reflects the hypertrophied right ventricle; a holosystolic murmur at the lower left sternal border reflects tricuspid regurgitation; and raised jugular venous pressure, ankle edema and an enlarged liver signal right heart failure. Nurses should also track the six-minute walk distance, oxygen saturation with exertion and daily weights.

Slide 9: An Older Adult Patient

• 81, breathless climbing stairs for a year
• Told it was "just age" and deconditioning
• Echo, then catheterization: pulmonary arterial hypertension

Speaker notes: Consider a composite patient, 81, a retired church organist, who could no longer climb to the choir loft. Two clinicians attributed the breathlessness to age. A nurse noticed ankle swelling and a loud second heart sound, prompting an echocardiogram and referral. Catheterization confirmed the diagnosis, after ruling out left heart disease, which is far more common at this age.

Slide 10: How Age Changes the Picture

• Lower walk distance and worse functional class at diagnosis
• More often treated with one drug
• Higher hospitalization and mortality risk

Speaker notes: Reddy et al. (2026) compared registry patients 80 or older with younger patients. The oldest group had lower pressures on catheterization yet walked shorter distances and had worse functional class at enrollment. They were more often started on a single drug and less often on prostacyclin therapy. After adjustment, they had a 57% higher rate of hospitalization and more than twice the risk of death, though the authors could not say whether this reflects a different disease, weaker response to therapy or comorbidity.

Slide 11: Treatment Matches the Mechanism

• Endothelin receptor antagonists block endothelin-1
• PDE5 inhibitors and riociguat boost the nitric oxide pathway
• Prostacyclin-pathway drugs replace what is missing

Speaker notes: Each established drug class corrects one of the pathways on slide 4. Endothelin receptor antagonists, such as macitentan and ambrisentan, block constriction and growth signals. Phosphodiesterase-5 inhibitors, such as sildenafil and tadalafil, and the soluble guanylate cyclase stimulator riociguat strengthen the nitric oxide pathway. Prostacyclin analogs and receptor agonists, from epoprostenol infusions to oral selexipag, replace prostacyclin. Most patients receive combinations (Humbert et al., 2022).

Slide 12: A New Target: Activin Signaling

• Sotatercept rebalances growth signaling
• STELLAR trial: 40.8 m greater walk distance at 24 weeks
• Watch for nosebleeds, telangiectasia, high hemoglobin, low platelets

Speaker notes: Sotatercept is the first drug aimed at the remodeling itself, trapping activin-class ligands to restore balance to the signaling described on slide 5. In the STELLAR trial, 323 patients on background therapy were randomized; the sotatercept group improved its six-minute walk distance by 40.8 m more than placebo, and eight of nine secondary endpoints improved (Hoeper et al., 2023). Nurses monitor hemoglobin, platelets, blood pressure and bleeding.

Slide 13: Safety in Older Adults

• Never combine PDE5 inhibitors with nitrates
• Watch for low blood pressure, falls and syncope
• Review every new medication

Speaker notes: Older adults often take nitrates for angina, and combining them with a phosphodiesterase-5 inhibitor can cause profound hypotension. Vasodilators also raise fall risk, and diuretics add dehydration and kidney risks. A continuous prostacyclin infusion must never be interrupted, because sudden withdrawal can cause rebound pulmonary hypertension; nurses keep backup pumps and supplies ready.

Slide 14: Patient and Family Education

• Daily weights; call for a gain of 3 pounds in 2 days
• Pace activity; supervised rehabilitation is safe
• Vaccines, salt limits, and when to seek help

Speaker notes: Teaching focuses on noticing right heart failure early through daily weights and swelling, taking medications exactly as prescribed, limiting sodium and fluids as directed, staying active within limits, and keeping influenza, COVID-19 and pneumococcal vaccines current. For older adults, involve a family caregiver, use large-print materials and teach back each point.

Slide 15: Cultural and Spiritual Care

• Ask how the patient and family understand the illness
• Respect decision-making roles and language needs
• Offer chaplaincy and discuss goals of care early

Speaker notes: Our composite patient's faith was central, and losing the ability to play for worship was a real grief. Asking about meaning, offering chaplaincy and connecting the patient with the congregation honored that. Culture shapes who makes decisions and how bad news is shared, so nurses ask rather than assume and use interpreters when needed. Because this disease shortens life, especially in the oldest patients, goals of care and palliative support belong early in the plan, alongside treatment.

References

Hoeper, M. M., Badesch, D. B., Ghofrani, H. A., Gibbs, J. S. R., Gomberg-Maitland, M., McLaughlin, V. V., Preston, I. R., Souza, R., Waxman, A. B., Grünig, E., Kopeć, G., Meyer, G., Olsson, K. M., Rosenkranz, S., Xu, Y., Miller, B., Fowler, M., Butler, J., Koglin, J., . . . Humbert, M. (2023). Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension. New England Journal of Medicine, 388(16), 1478-1490. https://doi.org/10.1056/NEJMoa2213558

Humbert, M., Kovacs, G., Hoeper, M. M., Badagliacca, R., Berger, R. M. F., Brida, M., Carlsen, J., Coats, A. J. S., Escribano-Subias, P., Ferrari, P., Ferreira, D. S., Ghofrani, H. A., Giannakoulas, G., Kiely, D. G., Mayer, E., Meszaros, G., Nagavci, B., Olsson, K. M., Pepke-Zaba, J., . . . Wort, S. J. (2022). 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart Journal, 43(38), 3618-3731. https://doi.org/10.1093/eurheartj/ehac237

McCance, K. L., & Huether, S. E. (2019). Pathophysiology: The biologic basis for disease in adults and children (8th ed.). Elsevier.

Reddy, R., Moutchia, J., Ravichandran, A., Runo, J., Badesch, D. B., Moss, J., Burger, C., De Marco, T., Horn, E., Ataya, A., Bryant, A., Al-Naamani, N., Kawut, S. M., & PHAR Investigators. (2026). Pulmonary arterial hypertension in octogenarians: The Pulmonary Hypertension Association Registry. Pulmonary Circulation, 16(3), Article e70387. https://doi.org/10.1002/pul2.70387