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Archives of Medical Science
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1/2018
vol. 14
 
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abstract:
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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references:
Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics 2000; 20: 639-52.
Ginaldi S, Chuang VP, Wallace S. Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr 1980; 4: 112-4.
Kim SJ. Heterotaxy syndrome. Korean Circ J 2011; 41: 227-32.
Pillai JB, Kpodonu J, Yu C, Borger MA Heterotaxy syndrome with azygous continuation-causing pseudo Budd-Chiari syndrome after cardiopulmonary bypass. Ann Thorac Surg 2006; 81: 1890-2.
Ruggeri M, Tosetto A, Castaman G, Rodeghiero F. Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet 2001; 357: 441.
Bass JE, Redwine MD, Kramer LA, Harris JH Jr. Absence of the infrarenal inferior vena cava with preservation of the suprarenal segment as revealed by CT and MR venography. AJR Am J Roentgenol 1999; 172: 1610-2.
Okur A, Intepe YS, Serin HI, Yıldırım U, Mavili E. Recurrent pulmonary embolism in an asthmatic patient who had interrupted inferior vena cava with azygous continuation. Turk Kardiyol Dern Ars 2014; 42: 277-80.
Guardado FJ, Byrd TM, Petersen WG Azygous continuation of the inferior vena cava with anomalous hepatic vein drainage. Am J Med Sci 2012; 343: 259-61.
Mehta AJ, Kate AH, Gupta N, Chhajed PN Interrupted inferior vena cava syndrome. J Assoc Physicians India 2012; 60: 48-50.
Lin AE, Ticho BS, Houde K, Westgate MN, Holmes LB. Heterotaxy: associated conditions and hospital-based prevalence in newborns. Genet Med 2000; 2: 157-72.
Kothari SS. Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol 2014; 7: 187-92.
Yamamura K, Joo K, Ohga S, et al. Thrombocytosis in asplenia syndrome with congenital heart disease: a previously unrecognized risk factor for thromboembolism. Int J Cardiol 2013; 167: 2259-63.
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