0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:19
Pulmonary Hypertension 1245➣Idiopathic- seen in women> men (up to 3:1), mean age of
onset=35 years
➣Familial- rare (6% of cases)
■Secondary Pulmonary HTN
➣Pre-capillary (pulmonary arteriolar vasoconstriction)
Hypoxia due to chronic lung disease from COPD, restriction,
insterstial lung dz, connective tissue disorders, granulomatous
disease, sleep apnea
Chronic pulmonary emboli (risk factors include trauma, HTN,
malignancy, recent surgery/immobilization, oral contracep-
tives, smoking, obesity, and hypercoagulable states)
High pulmonary blood flow from left to right intra-cardiac
shunts
Anorectic agents
Cirrhosis with portal hypertension and subsequent porto-
pulmonary HTN
HIV infection
➣Post-capillary (pulmonary venous hypertension)
Left ventricular dysfunction
Valvular heart disease (classically mitral stenosis)
Signs & Symptoms
■Earliest symptoms (fatigue) are nonspecific leading to a delay in
diagnosis
■Dyspnea- most common
■Chest pain from right ventricular ischemia
■Syncope or near syncope
■Lower extremity edema
■Raynaud’s phenomenon (10% of cases)
■Ortner’s syndrome (hoarseness due to laryngeal nerve compression
by dilated pulmonary artery)
■Exam may show
➣prominent “a” waves in jugular venous contour
➣prominent P2
➣right-sided S4 or S3 gallop
➣palpable pulm artery in 2nd left ICS
➣right ventricular heave.
■If advanced, the Graham-Steel murmur of pulmonary insufficiency
may be audible.
■With tricuspid insufficiency, prominent CV wave and systolic mur-
mur that increases with inspiration at lower left sternal border are
common.