Index
Module 19 • Pulmonology
Pulmonary Disorders II
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Pulmonary Disorders II
Zachary R. Smith ~3 min read Module 19 of 20
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Pulmonary Disorders II

II.PULMONARY ARTERY HYPERTENSION
A.Pathophysiology and Definitions
1

Pulmonary artery hypertension (PAH) is a chronic, life-threatening disease characterized by vascular

changes, including vasoconstriction, cellular proliferation, and thrombosis. These changes result in

elevations in pulmonary vascular resistance (PVR) with the end result being right ventricular failure.

Thromboxane A2 (potent vasoconstrictor) concentrations are increased.

Prostacyclin (potent vasodilator, inhibitor of platelet aggregation, and antiproliferative properties)

concentrations are decreased.

Endothelin-1 (ET-1; potent vasoconstrictor, mitogenic properties on pulmonary artery smooth

muscle cells) concentrations are increased.

d.Nitric oxide concentrations (vasodilator, inhibitor of platelet activation, and inhibitor of vascular

smooth muscle cell proliferation) are decreased.

Cellular proliferation occurs because of downregulation of antiproliferative growth factors, which

results in upregulation of the activin pathway, leading to increased vascular proliferation (J Heart

Lung Transplant. 2024:S1053-2498(24)01874-6).

Patient Case

Questions 1 and 2 pertain to the following case.

A 20-year-old woman is admitted to the ICU for an acute CF exacerbation. Before admission, her condition had

been stable. She had maintained her ideal body weight and had just completed a home regimen of suppressive

antibiotics. The patient requires MV for the management of hypoxic respiratory failure. She is initiated on AC/

VC mode with a lung-protective strategy (4–8 mL/kg tidal volume).

1

Which of the following would be best to manage her acute CF exacerbation?

A.Tobramycin nebulization; hypertonic saline 7% nebulization; and tube feedings to target a hypocaloric

goal during her acute illness (15 kcal/kg/day).

B.Ceftriaxone 2 g intravenously every 24 hours; tobramycin nebulization; normal saline 0.9% nebuliza-

tion; and tube feedings to target her goal caloric intake (25 kcal/kg/day).

C.Piperacillin/tazobactam 4.5 g intravenously every 6 hours; tobramycin 10 mg/kg intravenously once

daily; hypertonic saline 7% nebulization; and tube feedings to target her goal caloric intake (25 kcal/

kg/day).

D.Cefepime 2 g intravenously every 8 hours extended infusion; tobramycin 10 mg/kg intravenously every

8 hours; hypertonic saline 7% nebulization; and tube feedings to target her goal caloric intake (25 kcal/

kg/day).

2After treatment with intravenous antibiotics, aggressive chest physical therapy, and her maintenance CF

medications for 5 days, this patient improved and was extubated. She was moved out of the ICU on day 6 of

her hospitalization. Which duration of antibiotics would be best to recommend for this patient?

A.7 days.
B.10 days.
C.14 days.
D.Until cultures are negative.
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