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We present a rare case of descending aorta pulmonary venous fistula (DAPVF) in a 4-month-old child with mitral valve prolapse who was being evaluated for failure to thrive. Echocardiogram revealed abnormal turbulence in the left atrial appendage and mitral valve prolapse with mitral regurgitation (to a mild degree). Low dose non-ECG-gated cardiac CT (two postcontrast phases) and CT of the thorax (80 kVp, 50 mAs with automated tube current modulation) were performed using a Philips Brilliance 64-slice MDCT machine. An abnormal dilated artery arising from the descending thoracic aorta at the D7-D8 level on the left, giving rise to a nidus of tortuous vessels in the superior segment of the left lower lobe (figures 11–4) and draining into the left inferior pulmonary vein resulting in a left to left shunt was noted (figure 5). Left ventricular enlargement and mild plethora in the left lower lobe were seen, with no pulmonary vein dilation. Pulmonary arterial circulation and bronchial tree were normal with no sequestration. Surgical management was planned for a later age. To the best of our knowledge, no other similar case with a concomitant valvular heart disease has been reported in the English literature. Cardiac CT is helpful when echocardiography fails to identify the cause of abnormal pressure variation in cardiac chambers. Cardiac CT can also diagnose the presence of lung sequestration,1 which decides subsequent disease management.2 Systemic branches from the aorta supply the primitive lung and are replaced by the pulmonary arteries (from the 6th aortic arch) around the fifth week, failure of which (due to pulmonary oligaemia) is proposed as the theory for pulmonary arterialisation (pseudosequestration).3
Competing interests: None declared.
Patient consent: Obtained.
Provenance and peer review: Not commissioned; externally peer reviewed.