49 Primary Pulmonary Hypertension

Clinical Presentation

A 15-year-old girl presents with exertional dyspnea and exercise intolerance.

Figure 49A

Radiologic Findings

A frontal chest radiograph (Fig. 49A1) at presentation shows mild cardiomegaly, convex bulging of pulmonary arterial segment of the left heart border. The central pulmonary arteries are dilated, whereas peripheral pulmonary vessels are attenuated. The lateral chest radiograph (Fig. 49A2) also shows central-peripheral discrepancy in pulmonary artery size. The retrosternal space is obliterated because of right ventricular hypertrophy.

Diagnosis

Primary pulmonary hypertension

Differential Diagnosis

Congenital heart disease with left-to-right shunt
Chronic thromboembolism
Pulmonary venous obstructive lesions including veno-occlusive disease
Chronic lung disease
Miscellaneous conditions (Chatterjee et al, 2002)

Discussion

Clinical Findings

The clinical features of primary pulmonary hypertension vary with the severity of the lesion. Patients frequently complain of unusual fatigue, shortness of breath, and chest pain on minor exertion. The chest pain is attributable to increased myocardial oxygen demand from increased right ventricular workload. Some patients present with episodes of fainting or loss of consciousness. On physical examination, the most consistent finding is an increased pulmonic component of the second heart sound. Elevation of jugular venous pulsation and ankle swelling are secondary findings of right heart failure.