A patient with haemoptysis and a smaller right lung

Abstract

A A p pa at ti ie en nt t w wi it th h h ha ae em mo op pt ty ys si is s a an nd d a a s sm ma al ll le er r r ri ig gh ht t l lu un ng g Case historyA 41-year-old businessman was admitted to National Taiwan University Hospital for evaluation of a 2-week history of haemoptysis productive of 50-100 mL of fresh blood daily. The patient stated that haemoptysis had first occurred 10 yrs earlier, had lasted for about 1 month and had spontaneously subsided. Both episodes of haemoptysis were preceded by flu-like symptoms. There was no past history of epistaxis, exertional dyspnoea, or loss of body weight.Physical examination revealed a well-developed man in no distress. His blood pressure was 112/70 mmHg, pulse 96 beats . min -l and respiratory rate 22 breaths . min -1 . There was a slightly decreased chest wall excursion, and breath sounds in the right hemithorax. No audible bruits were heard over the chest. Heart sounds were regular, without murmurs. No clubbing, cyanosis, telangiectasia or oedema were noted. Arterial blood gases in room air showed: a pH of 7.41; arterial carbon dioxide tension (Pa,CO 2 ) of 4.9 kPa (36.5 mmHg); and arterial oxygen tension (Pa,O 2 ) of 13.1 kPa (98.3 mmHg). The sputum was negative for acid-fast bacilli, fungi and malignant cells. An electrocardiogram was normal. Echocardiography showed normal cardiac size and good left ventricular contractility, with no evidence of congenital cardiac lesions or pulmonary hypertension. On bronchoscopy, no endobronchial lesion or definite site of bleeding was found.Imaging studies, including a posteroanterior (PA) chest radiograph ( fig. l), and computed tomography (CT) scan at the level of the aorticopulmonary window ( fig. 2) are shown.

An Adult with Haemoptysis: A Rare Case of Congenital Anomaly

“…A case of right lung hypoplasia with UAPA manifesting as haemoptysis was described in a middle aged male. 1 Another case of pulmonary hypoplasia with RAA along with UAPA was reported in a 21-month-old child. 2 A 5-month child with RAA, right descending aorta and agenesis of the left pulmonary artery has been previously described in literature, 10 presenting with lung infection and progressive respiratory failure.…”

“…4 Warburton et al 5 described an interaction between blood vessels and airways during the development of lung and also highlighted that development of the former controlled airway growth, particularly the formation of alveoli. The possible mechanism for recurrent pulmonary infections in pulmonary hypoplasia may be: (1) reduced blood supply and defective ventilation of the lung parenchyma due to pulmonary artery defect; 6 (2) altered venous pulmonary circulation and (3) extrinsic compression of the large airways by the great vessels. 7 Haemoptysis may occur due to infection in the presence of above predisposing factors or may be due to increased blood flow and pulmonary hypertension in the opposite lung due to UAPA and consequent hypoplasia of the ipsilateral lung.…”

“…Unilateral absence of pulmonary artery (UAPA) has been described previously in association with pulmonary hypoplasia. 1,2 More recently it has been described as 'ductal origin of distal pulmonary artery'. 3 Here the distal pulmonary artery is supplied by ductus arteriosus in utero and the proximal pulmonary artery segment from the pulmonary trunk upto the hilum is absent.…”

“…Pulmonary hypoplasia in association with UAPA and manifesting in adults has been reported only in one case previously, to the best of our knowledge. 1 We describe an adult with RAA and left lung hypoplasia in association with probable autosomal dominant polycystic kidney disease (ADPKD). This case is being reported due to its rarity of occurrence.…”

Recurrent bronchopulmonary infections in the left lung of a 21-month-old female

“…Dysphagia may be present when congenital anomalies of the great vessels also lead to extrinsic compression of the oesophagus, such as in the double aortic arch [7]. Haemoptysis may occur due to increased blood flow and pulmonary hypertension in the opposite lung due to unilateral absence of a pulmonary artery with hypoplasia of the ipsilateral lung [10], such as in the case reported here.…”