Algorithm of Diagnosis Pulmonary Hypertension

Is There a reason to suspect PAH? Clinical

history (symptoms, risk factors, family
history), Do examination, radiography, ECG

No further
evaluation for
PAH

Is PAH likely?
Do Echocardiography

TRV to measure RVSP; RVE; RAE; RV dysfunction

Is PAH due to LeftHeart Disease? Do

echocardiography

Diagnosis of LV systolic, diastolic dysfunction and

valvular disease: Appropriate tretment and
further evaluation if necessary,
Including right-and left-heart catheterization

Is PAH due to CHD?

Do
echocardiography with
contrast
Is PAH due to CTD,
HIV? Do serologic test

Diagnosis of abnormal morphologic characteristics,

shunt: Surgery, Medical treatment of PAH or
evaluation for further defiition or other contribution,
including right-and left-heart catheterization
Diagnosis of scleroderma, SLE, other CTD, HIV
infection: Medical treatment of PAH and further
evaluation for other contributing causes, including
right-and left heart catheterization

Is Chronic PE
Suspected?
Do V/Q scan

Is Chronic PE Confirmed and

operable? Do pulmonary
angiography

Anatomic definition (CT and MRI may provide

additional useful but not definitive information):Do
thromboendarterectomy if appropriate or medical
treatment; clotting evaluation
Is PAH due to lung disease or
Hypoxemia? Do PFTs, arterial
saturation

Diagnosis of parenchymal lung desease, hypoxemia, or

sleep disorder: Medical treatment, oxygen, positivepressure breathing as appropriate, and further
evaluation for other contributing causes, including rightheart catheterization if necessary

What limitations are caused

by PAH? Functional class; 6minute walk test

Document exercise capacity regardless of cause of PH:

Establish baseline and prognosis and document
progression/response to treatment with serial
reassessments

What are the precise

pulmonary Hemodynamic
Characteristics? Right-heart
catheterization

Document PA and RA pressures, PCWP (LV or LA

Pressure if PCWP unobtainable or uncertain)
transpulmonary gradient, CO, PVR, SvO2, response to
vasodilators; Confirm PAH or IPAH if no other cause
identified; discuss genetic testing and counselling of
IPAH family members; refer to section on therapy

Symptoms and Signs of Pulmonary Hypertension

Symptoms
Dyspnea on exertion
Fatigue
Syncope
Anginal chest pain
Hemoptysis
Raynaud's
phenomenon

Signs

Jugular vein distention

Prominent right
ventricular impulse
Accentuated pulmonic
valve component (P2)
Right-sided third heart
sound (S3)
Tricuspid insufficiency
murmur
Hepatomegaly
Peripheral edema

Electrocardiography of PH
In pulmonary hypertension, the electrocardiogram (ECG)
may demonstrate signs of right ventricular hypertrophy,
such as tall right precordial R waves, right axis deviation
and right ventricular strain . The higher the pulmonary
artery pressure, the more sensitive is the ECG
The chest radiograph is inferior to the ECG in detecting
pulmonary hypertension, but it may show evidence of
underlying lung disease
Not infrequently, recognition of pulmonary hypertension
begins with the discovery of right ventricular hypertrophy
on the ECG or prominent pulmonary arteries on the chest
radiograph.

Anatomy of the thorax, along with indication of the

upper limits of normal pulmonary vascular dimensions.
A right interlobar pulmonary diameter of greater than 16
mm or a hilar-to-thoracic ratio of greater than 0.44 is
specific but not sensitive for the diagnosis of
pulmonary hypertension.
Widimsky J. Prog Respir Res 1985;20:69-75.

Algorithm for the evaluation of suspected pulmonary hypertension

American Family Physician Journal, vol 63/N0.9;2001

Functional Assessment of Patients

with Pulmonary Hypertension
Class I:

Patients with pulmonary hypertension but without resulting

limitation of physical activity. Ordinary physical activity does
not cause undue dyspnea or fatigue, chest pain or near syncope.
Class II: Patients with pulmonary hypertension resulting in slight
limitation of physical activity. These patients are comfortable at
rest, but ordinary physical activity causes undue dyspnea or
fatigue, chest pain or near syncope.
Class III: Patients with pulmonary hypertension resulting in marked
limitation of physical activity. These patients are comfortable at
rest, but less than ordinary physical activity causes undue
dyspnea or fatigue, chest pain or near syncope.
Class IV: Patients with pulmonary hypertension resulting in inability to
perform any physical activity without symptoms. These patients
manifest signs of right heart failure. Dyspnea and/or fatigue may
be present at rest, and discomfort is increased by any physical
activity. .
Modified from the New York Heart Association classification of patients with cardiac disease. Executive summary from the
World Symposium on Primary Pulmonary Hypertension 1998, Evian, France, September 6-10, 1998.

Clinical algorithm for the diagnosis of pulmonary hypertension.

CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 70 SUPPLEMENT 1 APRIL 2003