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Ductus Arteriosus Aneurysm Thrombosis with Mass Effect Causing Pulmonary Hypertension in the First Week of Life
THE JOURNAL OF PEDIATRICS • www.jpeds.com
INSIGHTS AND IMAGES
Ductus Arteriosus Aneurysm Thrombosis with Mass Effect Causing Pulmonary Hypertension in the First Week of Life
A
5-day-old term boy, born via emergency cesarean delivery because of a nonreassuring cardiotocograph, was admitted to the neonatal intensive care unit because of a systolic murmur and cyanosis without any respiratory distress. He was found to have pulmonary hypertension, right ventricular hypertrophy, and a large mass arising from the ductus arteriosus protruding into the pulmonary artery on echocardiography. Computed tomography of the chest (Figures 1 and 2; Figure 2 available at www.jpeds.com) revealed an extrinsic low-attenuation lesion compressing the main and left pulmonary arteries, suggestive of a ductus arteriosus aneurysm thrombosis or a bronchogenic cyst. The diagnosis was confirmed at surgery when the patient had a pulmonary thromboendarterectomy for a large thrombus that originated from an aneurysmal ductus arteriosus and extended to the main pulmonary and proximal left pulmonary artery. Aneurysm of the ductus arteriosus is a rare congenital condition.1 The mechanism is unknown, however, it is thought to be secondary to problems with intimal cushion formation, defective, or decreased elastin fibers and connective tissue during natural involution, and delayed aortic segment closure exposing the ductus to high pressures.2,3 Only ~25% of cases seem to be associated with an underlying genetic disorder (eg, Marfan syndrome, Ehlers-Danlos syndrome, or trisomy disorders).4 Typically, patients are asymptomatic, with incidence estimated as high as 8.8% in term infants.5 Symptoms vary and generally arise following acute complications such as rupture, infection, thromboembolism, or mass effect upon the airways or vasculature.1,2,4 Echocardiogram is the standard imaging modality for diagnosis and surveillance,1 although cardiac catheterization or computed tomography angiogram may clarify the diagnosis.6 Thrombosis of a ductus arteriosus aneurysm is uncommon, with thrombogenesis likely related to turbulent flow or endothelial injury within the narrowed pulmonary ductus segment.7 The major complications are thromboembolisms or mass effect, if the ductus is nonpatent. Thrombus causing obstruction to the pulmonary artery has been previously reported in term infants8 and in utero,9 and is potentially
Figure 1. Computed tomography of the chest revealing a 7.8 × 8.2 mm ductus arteriosus aneurysm thrombosis with mass effect on the thoracic vasculature (arrows). A, Axial slices demonstrated a low-attenuation filling defect within the aortopulmonary window, adjacent to the origin of the left pulmonary artery. B, The sagittal reconstruction showed the lowattenuation lesion indenting the main pulmonary and proximal left pulmonary artery with significant narrowing.
lethal. Definitive management in a symptomatic infant involves thrombectomy, however, conservative management with anticoagulation and antiplatelet therapy can be effective, relieving symptoms and normalizing pulmonary artery pressures.10 ■ David J. T. McArdle, MBBS Department of General Medicine Royal Hobart Hospital Tasmania, Australia Felix L. J. Paterson, MBBS Lloyd L. Morris, MBBS, MRCP, FRANZCR Department of Radiology Women’s and Children’s Hospital Adelaide, Australia References available at www.jpeds.com
J Pediatr 2016;■■:■■-■■ 0022-3476/$ - see front matter. © 2016 Elsevier Inc. All rights reserved. http://dx.doi.org10.1016/j.jpeds.2016.09.004
1 CRP 5.4.0 DTD ■ YMPD8655_proof ■ October 6, 2016
THE JOURNAL OF PEDIATRICS • www.jpeds.com
Volume ■■
References 1. Koneti NR, Kanchi V, Kandraju H, Jaishankar S. Symptomatic aneurysm of ductus arteriosus in neonates. Ann Pediatr Cardiol 2011;4:15963. 2. Shneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114:1873-82. 3. Lund JT, Jensen MB, Hjelms E. Aneurysm of the ductus arteriosus: a review of the literature and the surgical implications. Eur J Cardiothorac Surg 1991;5:566-70. 4. Dyamenahalli U, Smallhorn JF, Geva T, Fouron JC, Cairns P, Jutras L, et al. Isolated ductus arteriosus aneurysm in the fetus and infant: a multi-institutional experience. J Am Coll Cardiol 2000;36:2629. 5. Jan SL, Hwang B, Fu YC, Chai JW, Chi CS. Isolated neonatal ductus arteriosus aneurysm. J Am Coll Cardiol 2002;39:342-7. 6. Nyp MF, Drake W, Kilbride H. Prenatal ductal thrombosis presenting as cyanotic heart lesion. J Perinatol 2011;31:685-6. 7. Downing GJ, Thibeault DW. Pulmonary vasculature changes associated with idiopathic closure of the ductus arteriosus and hydrops fetalis. Pediatr Cardiol 1994;15:71-5. 8. Fripp RR, Whitman V, Waldhausen JA, Boal DK. Ductus arteriosus aneurysm presenting as pulmonary artery obstruction: diagnosis and management. J Am Coll Cardiol 1985;6:234-6. 9. Sheridan RM, Michelfelder EC, Choe KA, Divanovic A, Liu C, Ware S, et al. Ductus arteriosus aneurysm with massive thrombosis of pulmonary artery and fetal hydrops. Pediatr Dev Pathol 2012;15:79-85. 10. Pereira AG, Teixeira A, Martins FM. Spontaneous thrombosis of the ductus arteriosus. Rev Port Cardiol 2011;30:537-40.
Figure 2. Computed tomography of the chest revealing a 7.8 × 8.2 mm ductus arteriosus aneurysm thrombosis with mass effect on the thoracic vasculature (arrow). Coronal slices highlighted the lesion to be extrinsic and have mass effect, compressing the left pulmonary artery.
1.e1
McArdle, Paterson, and Morris CRP 5.4.0 DTD ■ YMPD8655_proof ■ October 6, 2016
INSIGHTS AND IMAGES
Ductus Arteriosus Aneurysm Thrombosis with Mass Effect Causing Pulmonary Hypertension in the First Week of Life
A
5-day-old term boy, born via emergency cesarean delivery because of a nonreassuring cardiotocograph, was admitted to the neonatal intensive care unit because of a systolic murmur and cyanosis without any respiratory distress. He was found to have pulmonary hypertension, right ventricular hypertrophy, and a large mass arising from the ductus arteriosus protruding into the pulmonary artery on echocardiography. Computed tomography of the chest (Figures 1 and 2; Figure 2 available at www.jpeds.com) revealed an extrinsic low-attenuation lesion compressing the main and left pulmonary arteries, suggestive of a ductus arteriosus aneurysm thrombosis or a bronchogenic cyst. The diagnosis was confirmed at surgery when the patient had a pulmonary thromboendarterectomy for a large thrombus that originated from an aneurysmal ductus arteriosus and extended to the main pulmonary and proximal left pulmonary artery. Aneurysm of the ductus arteriosus is a rare congenital condition.1 The mechanism is unknown, however, it is thought to be secondary to problems with intimal cushion formation, defective, or decreased elastin fibers and connective tissue during natural involution, and delayed aortic segment closure exposing the ductus to high pressures.2,3 Only ~25% of cases seem to be associated with an underlying genetic disorder (eg, Marfan syndrome, Ehlers-Danlos syndrome, or trisomy disorders).4 Typically, patients are asymptomatic, with incidence estimated as high as 8.8% in term infants.5 Symptoms vary and generally arise following acute complications such as rupture, infection, thromboembolism, or mass effect upon the airways or vasculature.1,2,4 Echocardiogram is the standard imaging modality for diagnosis and surveillance,1 although cardiac catheterization or computed tomography angiogram may clarify the diagnosis.6 Thrombosis of a ductus arteriosus aneurysm is uncommon, with thrombogenesis likely related to turbulent flow or endothelial injury within the narrowed pulmonary ductus segment.7 The major complications are thromboembolisms or mass effect, if the ductus is nonpatent. Thrombus causing obstruction to the pulmonary artery has been previously reported in term infants8 and in utero,9 and is potentially
Figure 1. Computed tomography of the chest revealing a 7.8 × 8.2 mm ductus arteriosus aneurysm thrombosis with mass effect on the thoracic vasculature (arrows). A, Axial slices demonstrated a low-attenuation filling defect within the aortopulmonary window, adjacent to the origin of the left pulmonary artery. B, The sagittal reconstruction showed the lowattenuation lesion indenting the main pulmonary and proximal left pulmonary artery with significant narrowing.
lethal. Definitive management in a symptomatic infant involves thrombectomy, however, conservative management with anticoagulation and antiplatelet therapy can be effective, relieving symptoms and normalizing pulmonary artery pressures.10 ■ David J. T. McArdle, MBBS Department of General Medicine Royal Hobart Hospital Tasmania, Australia Felix L. J. Paterson, MBBS Lloyd L. Morris, MBBS, MRCP, FRANZCR Department of Radiology Women’s and Children’s Hospital Adelaide, Australia References available at www.jpeds.com
J Pediatr 2016;■■:■■-■■ 0022-3476/$ - see front matter. © 2016 Elsevier Inc. All rights reserved. http://dx.doi.org10.1016/j.jpeds.2016.09.004
1 CRP 5.4.0 DTD ■ YMPD8655_proof ■ October 6, 2016
THE JOURNAL OF PEDIATRICS • www.jpeds.com
Volume ■■
References 1. Koneti NR, Kanchi V, Kandraju H, Jaishankar S. Symptomatic aneurysm of ductus arteriosus in neonates. Ann Pediatr Cardiol 2011;4:15963. 2. Shneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114:1873-82. 3. Lund JT, Jensen MB, Hjelms E. Aneurysm of the ductus arteriosus: a review of the literature and the surgical implications. Eur J Cardiothorac Surg 1991;5:566-70. 4. Dyamenahalli U, Smallhorn JF, Geva T, Fouron JC, Cairns P, Jutras L, et al. Isolated ductus arteriosus aneurysm in the fetus and infant: a multi-institutional experience. J Am Coll Cardiol 2000;36:2629. 5. Jan SL, Hwang B, Fu YC, Chai JW, Chi CS. Isolated neonatal ductus arteriosus aneurysm. J Am Coll Cardiol 2002;39:342-7. 6. Nyp MF, Drake W, Kilbride H. Prenatal ductal thrombosis presenting as cyanotic heart lesion. J Perinatol 2011;31:685-6. 7. Downing GJ, Thibeault DW. Pulmonary vasculature changes associated with idiopathic closure of the ductus arteriosus and hydrops fetalis. Pediatr Cardiol 1994;15:71-5. 8. Fripp RR, Whitman V, Waldhausen JA, Boal DK. Ductus arteriosus aneurysm presenting as pulmonary artery obstruction: diagnosis and management. J Am Coll Cardiol 1985;6:234-6. 9. Sheridan RM, Michelfelder EC, Choe KA, Divanovic A, Liu C, Ware S, et al. Ductus arteriosus aneurysm with massive thrombosis of pulmonary artery and fetal hydrops. Pediatr Dev Pathol 2012;15:79-85. 10. Pereira AG, Teixeira A, Martins FM. Spontaneous thrombosis of the ductus arteriosus. Rev Port Cardiol 2011;30:537-40.
Figure 2. Computed tomography of the chest revealing a 7.8 × 8.2 mm ductus arteriosus aneurysm thrombosis with mass effect on the thoracic vasculature (arrow). Coronal slices highlighted the lesion to be extrinsic and have mass effect, compressing the left pulmonary artery.
1.e1
McArdle, Paterson, and Morris CRP 5.4.0 DTD ■ YMPD8655_proof ■ October 6, 2016