APPROACH TO PULMONARY HYPERTENSION
Allen Repp, M.D.
September 18, 2002
Description
 Pulmonary hypertension represents abnormally increased pulmonary artery (PA) pressures. This
is minimally defined as PA systolic pressure > 30 and mean PA pressure > 20.
 This may occur in the absence of identifiable causes (primary pulmonary hypertension)
 Or may result from intrinsic lung disease or diseases extrinsic to the lung (secondary
pulmonary hypertension)
Pathophysiology and Etiologies
 Pulmonary artery pressure (Ppa) is directly proportional to pulmonary vascular resistance (PVR),
cardiac output (CO), and pulmonary venous pressure (Ppv): Ppa = CO x PVR + Ppv
 A variety of underlying disease states can produce pulmonary hypertension by affecting one of
these pathophysiologic parameters
 Increased pulmonary blood flow
 Congenital heart disease with left to right shunt
 Marked increase in cardiac output (e.g., in severe anemia)
 Pulmonary artery abnormalities leading to increased resistance to flow or decreased
cross-sectional area
 Pulmonary embolism
 Pulmonary fibrosis, sarcoidosis, scleroderma
 Severe COPD
 Schistosomiasis
 Pulmonary resection
 Extensive neoplastic or inflammatory infiltration (as in vasculitis)
 Thoracic deformities such as severe kyphoscoliosis or pectus excavatum
 Pulmonary arteriolar abnormalities secondary to vasoconstriction and/or obliteration
 Hypoxia (altitude, COPD, other parenchymal diseases, hypoventilation syndromes)
 Toxic substances such as anorectic drugs, amphetamines, and cocaine
 Acidosis
 Primary pulmonary hypertension
 Elevated pulmonary venous pressure – translating to elevated pulmonary capillary
pressures with compensatory increase in PA systolic pressures
 Left atrial hypertension (mitral stenosis, LV failure, atrial myxoma, constrictive
pericarditis)
 Mediastinitis (e.g., methysergide-induced sclerosing mediastinitis)
 Increased blood viscosity
 Polycythemia vera
 Profoundly elevated leukocyte counts
 Increased intrathoracic pressure
 COPD, mechanical ventilation
Clinical Presentation
 Historical Features
 Mild to moderate pulmonary HTN is often asymptomatic
 Moderate to severe pulmonary HTN usually presents with complaints of dyspnea on exertion,
reflecting exercise-induced decreases in cardiac output secondary to increased pulmonary
vascular resistance
 Other common complaints include easy fatigability, exertional chest discomfort resembling
angina, syncope, cough, hemoptysis
 Rarely, patients present with hoarseness due to compression of the left recurrent laryngeal
nerve by a dilated left pulmonary artery (Ortner’s syndrome)
 Physical Examination
 Increased intensity of pulmonic component of S2
 Left parasternal lift or heave consistent with RV hypertrophy / dilatation
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Prominent A wave in jugular venous pulse representing RVH
Signs of RV failure: elevated JVP, right sided S3 gallop, peripheral edema, ascites, and
tricuspid regurgitant murmur
Graham Steell murmur = diastolic murmur of pulmonary regurgitation in severe disease 
high pitched diastolic murmur heard best at the left upper sternal border and increasing with
inspiration
Signs of concomitant disease (COPD, RA, scleroderma, etc.)
Diagnostic Studies
 Echocardiogram
 May demonstrate RV dilatation and/or hypertrophy, RA dilatation, paradoxical septal wall
motion (bulging of the septum into the LV during systole)
 Doppler evaluation of tricuspid regurgitant flow is the most reliable non-invasive measure of
PA pressure
 May identify associated conditions such as mitral stenosis, LV systolic failure, congenital
heart disease
 ECG – characteristic findings include RVH, marked rightward axis, tall R wave in V1, delayed
precordial transition with prominent S waves in V5 and V6, inverted T waves and ST depressions
in V1-V3 suggestive of RV strain, peaked P waves in II consistent with RA enlargement, and new
RBBB
 Chest Radiography – may reveal enlarged central pulmonary arteries with relative attenuation of
the peripheral vessels, enlarged RV and RA, or evidence of other pathologic processes such as
emphysema or pulmonary fibrosis. Spiral CT angiography may be useful in diagnosing
pulmonary embolism
 Pulmonary Function Tests with ABG – may aid in distinguishing primary pulmonary
hypertension from secondary causes (but note that a mild restrictive defect can be seen with
isolated pulmonary hypertension)
 Polysomnography
 Ventilation-Perfusion Scan or Pulmonary Angiography – to evaluate chronic thromboembolic
disease
 Auto-antibody Tests (ANA, ANCA, RF) – if significant clinical suspicion for a vasculitic process
or other rheumatologic disease
 HIV-1 Testing
 Cardiac Catheterization – Aids in distinguishing “precapillary” from “passive” causes of
pulmonary hypertension by evaluating the mean PA pressure to pulmonary capillary pressure
gradient (>12 mm Hg suggests precapillary cause, whereas <12 suggests a passive cause).
Useful in excluding acquired or congenital shunt. Allows quantitation of severity of RV failure and
assessment of pulmonary vascular response to rapid acting vasodilators.
 Biopsy – rarely indicated and often not diagnostic
Treatment
 General principles include supplementary O2 and correction of acid-base abnormalities
 Treatment of primary pulmonary HTN is based on assessment of response to one of several
vasodilators: IV epoprostenol, IV adenosine, IV nitroprusside, or inhaled nitric oxide
 Marked reduction in pulmonary vascular resistance with increased or unchanged cardiac
output and stable systemic blood pressure suggests responsiveness to vasodilators and
warrants treatment with escalating doses of oral calcium channel blocker (diltiazem or
nifedipine) + warfarin
 Minimal reduction in pulmonary vascular resistance in setting of advanced symptoms
warrants trial of continuous infusion of epoprostenol + transplant evaluation
 New modalities include oral endothelin receptor antagonists (bosentan), phosphodiesterase
inhibitors (sildenafil), and inhaled vasodilators such as epoprostenol and nitric oxide
 Treatment of secondary pulmonary HTN relies primarily on treatment of underlying disease;
however use of vasodilators (calcium channel blockers and epoprostenol) may result in clinical
improvements in a subset of these patients
Prognosis
 Depends on underlying condition
 Mean survival in patients with primary pulmonary HTN is 3 years; however patents with right
heart failure have mean survival closer to 1 year
 Increased risk of sudden death, intrapulmonary thrombosis, and thromboembolism
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