Case Presentation:
A 55‐year‐old woman presented with 2 months of progressively worsening dyspnea on exertion, cough productive of yellow sputum, and occasional hemoptysis. She had noted edema of the lower extremities. Her lungs were clear to auscultation bilaterally; there was no wheezing or crackles. The jugular venous pressure was not elevated. There was mild lower‐extremity edema. The patient had a normal ejection fraction and mild pulmonary hypertension by transthoracic echocardiogram. CT angio‐gram revealed a hypoplastic right pulmonary artery. The right lung on ventilation perfusion scan had no perfusion, but normal ventilation in the right middle and lower lobes and decreased activity in the right upper lobe. A pulmonary arteriogram revealed complete occlusion of the right main pulmonary artery. These findings were diagnostic of Swyer‐James‐Macleod syndrome.
Discussion:
Dyspnea on exertion is a commonly encountered problem. An approach to determining the cause of dyspnea is to investigate each organ system, while allowing the history and physical to direct us to the appropriate diagnosis. In our patient with dyspnea on exertion, productive cough, and occasional hemoptysis, this led us to further investigate the pulmonary system. Through imaging, we found a ventilation–perfusion mismatch and hypoplasia of the right pulmonary artery tree, thus identifying the lungs as the etiology of the patient's symptoms. Dyspnea on exertion is a common presentation of Swyer‐James‐Macleod syndrome in adults. Swyer‐James‐Macleod syndrome is a postinfectious state secondary to bronchiolitis obliterans. It is usually diagnosed in childhood, but asymptomatic individuals may go undiagnosed until adulthood. Inflammation and fibrosis cause narrowing in the bronchioles. The pulmonary capillary bed is secondarily affected, leading to decreased blood flow to the pulmonary arteries, thereby causing decreased arterial development.
Conclusions:
Given that dyspnea is a commonly encountered problem, internists must broaden their differential diagnosis to include diseases of childhood that may go undiagnosed but that present in symptomatic adults.
Disclosures:
H. Echols ‐ none