Pulm-Pulmonary Hypertension Flashcards
What are the 5 classification groups of pulmonary hypertension?
- PAH: idiopathic, heritable, drug/toxin-induced,associated with CTD/HIV/portal HTN/Congenital/Schistosomiasis/Chronic hemolytic
1’. PULMONARY VENO-OCCLUSIVE DISEASE AND/OR PULMONARY CAPILLARY HEMANGIOMATOSIS
- PULMONARY HYPERTENSION DUE TO LEFT SIDED HEART DISEASE (systolic, diastolic dysfunction, valvular disease)
- PULMONARY HTN DUE TO LUNG DISEASE OR HYPOXIA: COPD, ILD, sleep disordered breathing, alveolar hypoventilation disorders, chronic exposure to high altitude, developmental abnormalities
- CHRONIC THROMBOEMBOLIC PULM HTN
- PULM HTN with unclear or multifactorial causes
How is pulmonary hypertension defined?
It is defined by a resting mean pulmonary artery pressure of 25 mmHg or greater
What are most cases of pulmonary hypertension caused by?
left-sided heart failure and hypoxic respiratory disorders
How is PH diagnosed?
- Echo is a useful initial tool in the evaluation
- Because echo may underestimate true pulmonary artery pressures, right heart Cath should be considered after a normal echo if the index of suspicion for pulmonary HTN is high
Treatment of pulmonary HTN for groups 2-5
directed at the underlying condition
Treatment for CTEPH
Anticoagulation and possible surgery
Group 1 PAH treatment
Usually advanced therapy with vasodilators, no role for them in groups 2-5
How is CTEPH diagnosed? What is the diagnostic criteria?
(1) documentation of PH >25 by right heart. Cath in the absence of LH failure and (2) compatible imaging consistent with thromboembolism
VQ scan is a sensitive indicator of CTEPH and is the preferred first imaging modality
How is CTEPH managed?
Anticoagulation that is lifelong and CTEPH is managed by pulmonary thromboendarterectmy
What is group 1 PAH most commonly associated with?
Other conditions, including CTDs, portal hypertension, HIV infection, illicit drug use, toxin exposure, and congenital heart disease
How is PAH diagnosed?
It is diagnosed with a right heart Cath to confirm pulmonary hemodynamics and to see whether there is a specific response to vasodilator infusion
How do CCB work with PAH?
only for patients with acute vasodilator response at catheterization; acute response does not assure chronic response
Prostanoids (epoprostenol, treprostinil, ilosprost)
supplements endogenous levels of prostacyclin (PGI2) a vasodilator with anti-smooth muscle proliferative properties
Endothelin-1 receptor antagonists (bosentan, ambrisentan)
blocks action of endogenous vasoconstrictor and smooth muscle mitogen endothelia; class-wide risk of liver injury and teratogenicity; liver chemistry testing and pregnancy testing is required
Phosphodiesterase-5 inhibitors
Prolongs effect of intrinsic vasodilator cyclic GMP by inhibiting hydrolysis by PPD-5
