Sameer Thakur

Choice of valve prosthesis in a rare clinical condition: aortic stenosis due to alkaptonuria.

Alkaptonuria is a rare inherited disorder of tyrosine metabolism, which results in deposition of homogentisic acid in the connective tissues. The accumulation of homogentisic acid in connective tissue causes the syndrome known as ochronosis, which is typically manifested by skin pigmentation, degenerative arthropathy and discolouration of urine. Cardiovascular involvement is a much less common complication of alkaptonuria but poses a greater risk to the patients health. We present the case of a 65 year-old man with aortic stenosis and a previous diagnosis of alkaptonuria who underwent successful aortic valve replacement with a mechanical prosthesis.

Anomalous systemic arterial supply to the left lower lobe without evidence of pulmonary sequestration: Images for Surgeons

An 18‐year‐old male was referred to our department after developing episodes of haemoptysis following heavy contact during sport. He had no significant past medical history and clinical examination was unremarkable. A chest X‐ray revealed no abnormal shadow. Subsequent computed tomography (CT) of the chest with contrast showed an anomalous systemic artery measuring 14 mm in diameter arising from the anterior surface of the descending thoracic aorta passing into the left lung on the inferior aspect of the left hilum inferior to the left inferior pulmonary vein (Fig. 1). There was a small amount of blood seen within the left lower lobe peripherally. Radiologically guided percutaneous intervention with balloon occlusion or embolization was considered; however, the size of the anomalous artery was considered too big to achieve a satisfactory result. A transthoracic echocardiogram was performed, which showed normal left atrial size, normal biventricular size and function, and both inferior pulmonary veins were seen to drain in to the left atrium as expected. Without intervention, the patient would be at risk of ongoing haemoptysis and bleeding, as well as left heart volume overload and pulmonary hypertension secondary to significant aorto‐pulmonary shunt. At surgery, the patient initially underwent bronchoscopy which confirmed normal bronchial anatomy, and no evidence of bleeding to suggest a communication between the anomalous artery and the airways. A mini‐thoracotomy was then performed and the anomalous artery was found to feed the left lower lobe. The fissures were dissected and the normal pulmonary arterial branches were seen to enter the left lower lobe also. These vessels were not atresic or hypoplastic. On direct palpation, there was no thrill evident and so the presence of an arterial‐venous malformation was unlikely. The lung parenchyma appeared normal. The anomalous vessel was clamped and the left lower lobe remained well perfused. The findings were consistent with systemic arterialization of the lung without sequestration, and so the anomalous artery was divided with a stapler device and we did not perform lobectomy. The patient was discharged without any complication. At 6 weeks follow‐up, the patient reports no further symptoms. Systemic arterial supply from the descending thoracic aorta to the basal segment of the left lower lobe is a rare congenital anomaly within the spectrum of pulmonary sequestration. CT of the chest is the most useful diagnostic test as it demonstrates both the bronchial and vascular anatomy of the lung, and CT angiography can clearly demonstrate the origin of the aberrant systemic artery. Although angiographic interventions such as embolization have been described, surgery remains the gold standard of treatment and is always indicated in order to prevent the long‐term potential adverse effects associated with this anomaly.