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April 18, 1977

Displaced Mediastinum and Heart Murmur

JAMA. 1977;237(16):1721-1722. doi:10.1001/jama.1977.03270430063024

History  A 6-year-old boy had been referred at age 22 months for evaluation of a grade 2/6 systolic ejection murmur at the upper right sternal border. Cardiac catheterization showed mild pulmonic valvular stenosis, with main pulmonary artery pressure of 35/12 mm Hg and right ventricular pressure of 32/5 mm Hg. Cardiovascular history was negative for tachypnea, cyanosis, or squatting.

Diagnosis  Congenital absence of the left pulmonary artery.

Comment  The chest roentgenogram (Fig 1) shows the heart and mediastinal structures displaced into the smaller left hemithorax. The left hilum appears small, and pulmonary vascularity is slightly diminished on the affected side. Cardiac size is within normal limits, but a right-sided aortic arch is noted.Pulmonary angiography (Fig 2) illustrates absence of the left pulmonary artery. Aortic angiography (Fig 3) demonstrates a right-sided aortic arch with bronchial arterial collateral supply to the left lung. No additional cardiovascular anomalies were shown. Pulmonary vascular