A 37-year-old man was reviewed in the respiratory clinic for 20-year history of chronic productive cough. He reported daily cupful sputum production and a susceptibility to respiratory tract infection since he was a teenager. He experienced dyspnoea and dizziness on exertion. There was no history of chest pain, haemoptysis or syncope. He was born prematurely and had a stillborn twin sibling. His medical history included Crohn's colitis well-controlled with infliximab infusions for the past 5 years and latent tuberculosis for which he had completed treatment. On examination, his right chest size was smaller than the left, with reduced respiratory excursion. Chest auscultation demonstrated reduced breath sounds and course crackles on the right side. The left side examined normally. He had dual heart sounds with no murmurs.
Plain film chest radiograph showed contracted right hemithorax, raised right hemidiaphragm, and right lower lobe bronchial wall thickening. Left lung was hypertrophied and had normal lung fields. A contrast CT chest identified unilateral absence of pulmonary artery (UAPA), hypoplastic right lung, compensatory left lung hypertrophy and right lung bronchiectasis. The pulmonary trunk appeared to be of normal diameter. Review of imaging identified a non-contrast CT chest performed 4 years which suggested right pulmonary agenesis, but no referral was initiated. A transthoracic echocardiogram showed normal cardiac function, no congenital anomalies and no pulmonary hypertension. Lung function test was of mild restrictive pattern, with TLC of 5.6 L (76%), normal FEV1/FVC ratio, without bronchodilator response. He also had normal gas exchange.
He was provided with counselling, and chest physiotherapy for education on sputum clearance strategies. Lung protection strategies including annual flu vaccinations, early antibiotic therapy for exacerbations and avoidance of sick contacts were advised. CT surveillance was planned in 24 months.