A 36-year-old woman with a history of dextrocardia and dyspnea. Diagnosis: unilateral absence of a pulmonary artery (UAPA) with right hypoplastic lung.

A 36-year-old woman with a history of dextrocardia presented to the clinic for evaluation of several months of exertional dyspnea. She denied any cough, wheezing, fever, chills or rigors, as well as any orthopnea, leg edema or hemoptysis. Past medical history was significant for tachycardia, orthostatic hypotension, dextrocardia diagnosed 15 years ago, hyperlipidemia and chronic headaches. She had a history of endometrial ablation in May 2007. She had had an uneventful pregnancy 10 years ago. Current medications included nadolol 20 mg twice a day. Family history was not significant for any cardiac or pulmonary disease. She had worked at a childcare center in the past but was now a student. She denied any smoking history and drank 1–2 glasses of wine per week. Review of systems was positive for postnasal drip, nasal congestion and headaches. On examination, vitals were normal. Chest exam showed decreased breath sounds on the right side. There was limited movement of the diaphragm on the right side with respiration. Heart sounds were shifted to the right side. There was no clubbing, cyanosis or edema. A 2D echocardiogram done 15 years ago was interpreted as dextrocardia with normal valves and normal cardiac function. A chest radiograph (fig. 1) done at an outside hospital was read as volume loss on the right side with dextrocardia. Pulmonary function tests including spirometry, lung volumes, and diffusing capacity were within normal limits. What is your diagnosis? Received: January 29, 2009 Accepted after revision: May 27, 2009 Published online: July 31, 2009