EMCrit.org

Pulmonary Hypertension

Review Article

best: (Crit Care Med 2007;35:2037)

 

 

(Clinical Pulmonary Medicine 2004;11(1):40)

 

Cor pulmonale is pulmonary hypertension in the setting of respiratory disease either acute or chronic, it does not require right heart failure thought that is sometimes a sequelae

 

Divided into primary, which is idiopathic (PPH or IPH) and secondary, from a cause outside of the lung vasculature.

 

While acute disease may be reversible, vascular remodeling in chronic disease may be permanent

 

PPH

unknown etiology, there have been associations with amphetamine diet aids, contaminated rapeseed oil, and contaminated l-tryptophan.

 

Patients present with substernal chest pain or pressure, exertional dyspnea, fatigue, and possibly syncope.

Diagnostics

Chest x-ray will show prominent pulmonary arterial vasculature

ECG will may show right ventricular hypertrophy

 

High resolution CT, Perfusion nuclear scanning, MRI, and angiography may all be useful.  Cardiac cath may be needed as well.  Lung biopsy may be a final, necessary step to make a diagnosis of cause for PH.

 

Therapy

Correct hypoxemia

PaO2<55 or PaO2<59 with Crit>55, p pulmonale, or edema should be treated with chronic supplemental O2

CCBs, Iv nitroglycerin, IV Prostacyclin, epoprostenol (Flolan), eventual lung transplantation

vasodilator therapy is not without risks as it can cause systemic hypotension or worsen PH b/c if the pulmonary diameter is fixed, it will just cause decreased RH pressures without affecting pulmonary.

 

at risk for venous thromboembolic disease

chronic VTE can also be the cause of pulmonary hypertension and often the patient will have dramatic improvement if the clot is removed

 

HIV

can cause pulmonary hypertension

 

PAH associated with syncope, vasovagal, and sudden death

 

hypoxemia and hypercarbia are both vasoconstrictors

 

sleep apnea to pulm htn to apnea to worsening pulm htn

 

coumadin o2 diuretics

 

 

Hanging Flolan using normal IV pump

1.        Get Intervia (empty IV bag) and light proof plastic bag.

2.        Mix Flolan with sterile diluent

3.        Inject Flolan into intervia

4.        Place Ice on both sides in light-proof bag

5.        Use normal IV tubing, no filter required.

6.        Convert pt’s 24 hour rate to hourly rate