Pulmonary Disorders II
Unloading the RV with pulmonary vasodilators is essential to controlling decompensated PH and RV
failure.
In select scenarios, temporary inhaled epoprostenol or inhaled nitric oxide may be considered
in those who are too unstable to tolerate the systemic blood pressure effects of PAH therapies.
These inhaled options have been described in the literature administered by invasive MV, high-
epoprostenol, given the complexity of the administration method. Initial doses described in the
literature for inhaled epoprostenol include 0.01 to 0.05 ΞΌg/kg/min, and for nitric oxide, the range
Patients admitted to the ICU for PAH compensation are generally candidates for escalation in
disease-targeted medications. These patients typically meet the criteria for intermediate-high or
high-risk classification. When clinically appropriate (eg, hemodynamically stable, able to tolerate
medications by mouth), escalation of therapy is warranted in conjunction with a PAH specialist.
All PAH medications, except for PDE5i, are specialty medications. The process to assess for patient
access to therapy once stable for discharge should be initiated once the patient has been stabilized.
If a patient is not responsive to therapy and may be a transplant candidate, extracorporeal life support
can be considered as a bridge to lung transplant. Candidates must be chosen prudently because RV
failure must be reversible for this strategy to be successful.
Patient Case
Questions 3 and 4 pertain to the following case.
A 44-year-old man is transferred to the medical ICU for treatment of his worsening PAH. He currently receives
no PAH treatment. The patient has had increased dyspnea on exertion for the past 6 months with exercise. For the
past 2 months, he has had difficulty going for walks with his dog without getting fatigued, and occasionally, he
has chest pain. In the past few days, he has had severe shortness of breath at rest, which prompted him to come
to the hospital. His physical examination is remarkable for blood pressure 105/64 mm Hg and heart rate 85 beats/
minute. Lung examination is clear, and extremities are notable for trace edema. An echocardiogram reveals an
elevated pulmonary systolic pressure and a normal ejection fraction. The patient has an unfavorable response
to vasodilator challenge. Pertinent laboratory data are serum urea nitrogen 10 mg/dL, SCr 0.6 mg/dL, AST 160
U/L, and ALT 100 U/L.
3. Which best describes his WHO FC and risk of mortality in the next year?
4. Which medication therapy would be best to recommend initiating at this time?