A classic physical examination finding pointed to underlying disease in a 43-year-old Hispanic woman. On presenting to the Emergency Department, she reported progressive shortness of breath, fatigue, and chest pressure. One month earlier, she could exercise without limitation. However, in the ensuing interval, she developed increasing dyspnea and constant, nonexertional, left-sided chest pressure. A review of systems was notable for dysphagia. The patient denied palpitations, orthopnea, paroxysmal nocturnal dyspnea, or edema. She had no significant past medical or family history and denied tobacco, alcohol, or illicit drug use. An undocumented immigrant from Mexico, she had limited access to health care.
Assessment
The patient had stable vital signs: temperature 36°C (96.8°F); heart rate 73 beats per minute; blood pressure 116/89 mm Hg; and respirations 17 breaths per minute. Her oxygen saturation was 95% on room air, and her jugular venous pressure was elevated to 10 cm above the sternal angle. Cardiac examination revealed a nondisplaced point of maximum impulse, left parasternal heave, regular rhythm, normal S1, widely split S2, intensified P2, and no murmurs. Her lungs were clear with no crackles or rales. She had 1+ bilateral pretibial edema and notable sclerodactyly of the fingers (Figure 1). Telangiectasias marked the patient’s cheeks and forehead (Figure 2).
Diagnosis
Despite the patient’s preliminary diagnosis of pulmonary hypertension, the etiology remained uncertain. The World Health Organization (WHO) classifies patients with pulmonary hypertension by etiology as follows: pulmonary arterial hypertension (Group 1), pulmonary hypertension secondary to left heart disease (Group 2), chronic lung disease or hypoxia, or both (Group 3), chronic thromboembolic disease (Group 4), and unclear or multifactorial etiology (Group 5).1
Right heart catheterization confirmed the diagnosis of severe pulmonary hypertension, measuring pulmonary artery pressure at 114/37 mm Hg (mean, 64 mm Hg), pulmonary capillary wedge pressure at 1 mm Hg, and right atrial mean pressure at 6 mm Hg. The cardiac index was 2.58 L/min/m2, and pulmonary vascular resistance was markedly elevated at 17.4 Wood units. Coronary angiography showed no evidence of obstructive coronary artery disease. Cardiac magnetic resonance imaging verified reduced right ventricular systolic function with an estimated ejection fraction of 20% (reference, 40%). A ventilation-perfusion scan was negative for any mismatch.
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-Kavita Khaira, MD, Roblee Allen, MD, Ezra A. Amsterdam, MD
This article originally appeared in the August 2016 issue of The American Journal of Medicine.
