A 4-yr-old presenting with chronic cough and asymmetrical chest.

A 4-yr-old female was referred by her general practitioner with history of recurrent cough of 18 months duration. The cough was predominantly nocturnal with no associated wheeze, fever or systemic symptoms and no symptoms of gastrooesophageal reflux. There were no exercise-induced symptoms. Asthma was suspected and she had been treated with inhaled salbutamol with no significant improvement in her symptoms. There was no history of recurrent chest infections, foreign body aspiration or previous hospital admissions. On examination her weight was on the 3rd centile and height on the 50th centile. There were no cyanosis, clubbing or ear, nose and throat abnormalities. She had a markedly asymmetrical chest with less prominent right hemithorax compared with the left and mediastinal shift to the right. Breath sounds were diminished on the right side with no wheeze or crepitations. The rest of the cardiovascular examination was normal. There were no other abnormalities on clinical examination. In view of her chronic cough and failure to thrive she had a mantoux test, which was strongly positive. She had bacille Calmette-Guerin vaccination at birth and there was no recent contact with tuberculosis (TB). The sputum was negative for Mycobacterium tuberculosis. She was given TB chemoprophylaxis with Isoniazid and Rifampicin for 6 months, during which she had a symptomatic improvement in her cough. A chest radiograph (fig. 1) and computed tomography (CT) scan of the thorax (fig. 2) revealed characteristic findings. Bronchoscopy revealed abnormalities of the bronchial tree. She had a transthoracic echocardiogram, which revealed characteristic findings. The rest of the cardiac anatomy was normal. She underwent diagnostic cardiac catheterisation, which showed normal right-sided pressures (right atrial mean pressure 4 mmHg, right ventricle (RV) pressure 26 over 3 mmHg, main pulmonary artery (MPA) 24 over 8 mmHg, the right pulmonary artery (RPA) 20 over 8 mmHg and left pulmonary artery (LPA) 20 over 8 mmHg). Oximetry showed pulmonary blood flow/systemic blood flow ratio of 1.3:1. The superior vena cava saturation was 83%, right atrial saturation 88%, lower inferior vena cava (IVC) saturation 83%, right atrium (RA)-IVC junction 99%, RV 91%, MPA 90%, RPA 90% and LPA 86%. The follow through of the RPA angiogram (fig. 3) revealed the characteristic abnormality. There were no aortic collaterals supplying the lungs. There was no significant intracardiac leftto-right shunt. Currently she is being managed conservatively with regular clinical and echocardiograph assessment.