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Archives of Medical Science
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vol. 14
Letter to the Editor

Massive pulmonary embolism in a patient with polysplenia syndrome and interrupted inferior vena cava with azygous continuation

Carmen K. M. Cheung, Man Fai Law, Ka Tak Wong, Mark T. K. Tam, Kai Ming Chow

Arch Med Sci 2018; 14, 1: 251–253
Online publish date: 2017/12/19
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Interruption of the inferior vena cava (IVC) is a congenital anomaly. In individuals with this anomaly, venous drainage of the lower limbs is achieved through a compensatory dilated vena azygos system. These anomalies are rarely associated with venous thrombosis, especially in young patients. Patients with polysplenia typically have IVC interruption with azygous or hemiazygous continuation. We herein report a young patient who presented with massive pulmonary embolism and recurrent syncope and was later found to have interrupted IVC with azygous continuation and polysplenia syndrome, a condition that has rarely been reported in the literature previously. It highlights the importance of considering rare causes of pulmonary embolism in patients, particularly younger individuals in whom the classical risk factors are absent.
A 19-year-old Chinese secondary school student was admitted for recurrent episodes of syncope, preceded by shortness of breath. The syncope was not associated with limb twitching, or urinary or bowel incontinence. His syncope episodes are notably preceded by exercise including climbing upstairs and basketball game. He had a history of right inguinal hernia with surgical repair done in childhood. He had 10 siblings and they all enjoyed good health; there was no family history of venous thrombosis.
Physical examination of the cardiovascular and respiratory systems was unremarkable. There was no focal neurological deficit, but he had one episode of tonic convulsion after admission. Electroencephalography ruled out epileptiform discharges. Blood tests showed normal complete blood counts with a platelet count 173 × 109/l. However, he had an elevated troponin T level of 59.4 ng/l (normal range < 14.0 ng/l) and electrocardiography revealed marked right axis deviation with predominant S waves in limb lead I, and T wave inversion in limb lead III. Echocardiogram showed right ventricular dilatation with a D-shaped left ventricle, suggestive of right heart pressure overload. He then underwent computed tomography pulmonary angiography (CTPA), which revealed a massive occlusive pulmonary embolism in bilateral lower lobe pulmonary arteries (Figure 1 A), and near occlusive pulmonary embolism in the rest of the segmental pulmonary arteries. The thrombus extended to the most distal part of the right and left main pulmonary arteries, but the main pulmonary trunk remained clear. He was treated with intravenous thrombolytic therapy (alteplase...

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