History

The average time from symptom onset to diagnosis has been reported to be approximately 2 years. Despite recent attempts at increasing the awareness of pulmonary arterial hypertension (PAH), especially associated PAH (APAH), this delay in diagnosis has not changed appreciably in recent years.

Early symptoms are nonspecific. Often, neither the patient nor the physician recognizes the presence of the disease, which leads to delays in diagnosis. Complicating matters, idiopathic PAH (IPAH) requires an extensive workup in an attempt to elucidate an identifiable cause of the elevated pulmonary artery pressure.

The most common symptoms and their frequency, reported in a national prospective study, are as follows:

Dyspnea (60% of patients)
Weakness (19%)
Recurrent syncope (13%)

Additional symptoms include fatigue, lethargy, anorexia, chest pain, and right upper quadrant pain. Cough, hemoptysis, and hoarseness are less common symptoms.

Women are more likely to be symptomatic than men.

Physical Examination

Physical findings in persons with PAH can be quite variable.

Cardiovascular examination often reveals the following findings:

The pulmonic component of the second heart sound is usually increased, which may demonstrate fixed or paradoxic splitting in the presence of severe right ventricular dysfunction; occasionally, the second heart sound may be palpable.
Pulmonic regurgitation (Graham Steell murmur) may be apparent.
A murmur of tricuspid regurgitation can be present, and a right ventricular lift (heave) may be noted.
Jugular venous pulsations may be elevated in the presence of volume overload, right ventricular failure, or both; large V waves are often present because of the commonly present severe tricuspid regurgitation, and large a waves thought to be secondary to poor right ventricular compliance may also be present.^[14]
Right-sided S3 gallop

Other findings may include hepatomegaly with palpable pulsations of the liver and an abnormal abdominal-jugular reflex. In untreated patients and patients with worsening decompensated right heart failure, ascites is not uncommonly present.

Lung examination findings are usually normal.

Extremity examination may reveal pitting edema of varying degrees. Patients who are bedridden may have presacral edema.

Complications

Complications of IPAH include the following:

Advanced right-sided heart failure with hepatic congestion
Pedal edema
Pleural effusions
Ascites
Worsening dyspnea upon exertion

Differential Diagnoses

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