The prognosis and treatment of PH varies by WHO Group. The hospitalist will often undertake initial management of symptomatic patients (see Table 3). Intravenous loop diuretics will successfully treat peripheral edema and hepatic congestion in all PH patients.20 Due to the possibility of decreased cardiac output or worsened hypotension in some PH groups, patients should be monitored closely during initial diuresis.
All patients with PH should be assessed for hypoxia during rest, ambulation, and sleep during their hospitalization. Supplemental oxygen therapy should be initiated in all patients with evidence of persistent hypoxia (arterial oxygen blood pressure <60 mmHg).20 Vaccination against pneumococcus and influenza should also be performed during the initial hospitalization. Pregnant patients diagnosed with PH require urgent maternal-fetal medicine consultation.
Further management should be guided by the underlying etiology of the PH:17,18
- Group 1 PH. These patients should be evaluated by a pulmonology consultant, if one is available, as they require intense outpatient follow-up with a pulmonologist. Specialized treatment regimens include calcium channel blockers, phosphodiesterase inhibitors, prostanoids, endothelin receptor antagonists, or newly approved guanylate cyclase stimulants. In previously diagnosed patients, these medications should be continued during a patient’s admission unless the medication is clearly causing the patient harm (such as worsening hypotension) or preventing improvement. Many of these patients are placed on chronic anticoagulation with warfarin, with a goal international normalized ratio (INR) of 1.5 to 2.5.
- Group 2 PH. Patients with left heart or valvular dysfunction and PH have a worse prognosis than similar patients without PH. Management of these patients should focus on treating the underlying etiology. Use of prostanoids may be harmful in this patient population.18
- Group 3 PH. Patients whose PH is fully explained by pulmonary disease should be started on continuous oxygen therapy to treat persistent hypoxemia, and their underlying disorder should be treated, with pulmonologist consultation and referral if necessary.
- Group 4 PH. Patients with newly diagnosed CTEPH should be initiated on warfarin with a goal INR of 2.0 to 3.0. They should undergo evaluation by a pulmonologist for thromboendarterectomy and possibly advanced medical therapies.
- Group 5 PH. Patients with sarcoidosis as the cause of their PH may benefit from prostanoid or endothelin receptor antagonist therapy and should undergo evaluation by a pulmonologist.21,22
Patients with sickle cell anemia, metabolic disorders, and other causes should undergo further subspecialist evaluation prior to initiating therapy to treat their PH.
Back to the Case
The patient underwent diuresis with intravenous furosemide over several days, with gradual improvement in her lower extremity edema and dyspnea. She was placed on oxygen therapy for persistent hypoxemia. As her highly elevated pulmonary artery pressure appeared to be “out of proportion” to her mild left ventricular diastolic dysfunction, further evaluation was pursued. Ventilation-perfusion scanning was performed and showed no mismatch of perfusion and ventilation, effectively ruling out CTEPH. Liver function, HIV, and connective tissue disease testing yielded unremarkable results.
The patient was euvolemic after one week of diuresis and was discharged home with plans for PH specialist follow-up, polysomnography to evaluate for sleep-disordered breathing, and likely RHC. The etiology of her PH was not clear at discharge.
Evaluation of PH is a step-wise process that starts with history and physical exam and may require extensive evaluation, including right heart catheterization to confirm the diagnosis and define the etiology. A primary goal of evaluation is to define the appropriate therapy for a given patient, which may include advanced therapies in some cases.
Dr. Griffith is a quality improvement fellow and instructor of medicine in the Hospital Medicine Division at the University of Colorado Denver. Drs. McFarland and Smolkin are hospitalists and instructors of medicine at the University of Colorado Denver.