Abstract
Absent pulmonary valve syndrome is rare. We report the case of a child with absent pulmonary valve syndrome noted after birth. Progressive dilatation of the main pulmonary artery and ascending aorta were noted by echocardiography and confirmed by heart computed tomography. He underwent ascending aorta graft replacement and pulmonary artery reduction angioplasty at the age of 6. Good outcome was achieved at 18-month follow-up where subsequent computed tomography revealed a normal size ascending aorta and main pulmonary artery.
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Norgaard MA, Alphonso N, Newcomb AE, Brizard CP, Cochrane AD (2006) Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up. Eur J Cardio-thoracic Surg 29:682–687
Godart F, Houyel L, Lacour-Gayet F, Serraf A, Sousa-Uva M, Bruniaux J, Petit J, Piot JD, Binet JP, Conte S, Planche C (1996) Absent pulmonary valve syndrome: surgical treatment and considerations. Ann Thorac Surg 62:136–142
Kececioglu D, Galal O, Abbag F (1994) Absent pulmonary valve syndrome associated with muscular ventricular septal defect and aneurysmal dilation of the ascending aorta. Cardiol Young 4:184–186
Stafford EG, Mair DD, McGoon DC, Danielson GK (1973) Tetralogy of Fallot with absent pulmonary valve. Surgical considerations and results. Circulation 47:24–30
Pinsky WW, Nihill MR, Mullins CE, Harrison GH, McNamara DG (1978) The absent pulmonary valve syndrome: considerations of management. Circulation 57:159–162
Freedom RM, Mawson JB, Yoo SJ, Benson LN (1997) Congenital heart disease: textbook of angiography. Futura Publishing Co, New York, pp 597–615
Niwa K, Perloff JK, Bhuta SM, Laks H, Drinkwater DC, Child JS, Miner PD (2001) Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Circulation 103:393–400
Lee ST, Kim JA, Jang SY, Kim DK, Kim JW, Ki CS (2008) A novel COL3A1 gene mutation in patient with aortic dissected aneurysm and cervical artery dissections. Heart Vessels 23:144–148
Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY (2005) Intrinsic histological abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy. Circulation 112:961–968
Cattaneo SM, Bethea BT, Alejo DE, Spevak PJ, Clauss SB, Dietz HC, Gott VL, Cameron DE (2004) Surgery for aortic root aneurysm in children: a 21-year experience in 50 patients. Ann Thorac Surg 77:168–176
Yamaguchi R, Ujiie H, Haida S, Nakazawa N, Tomokatsu H (2008) Velocity profile and wall shear stress of saccular aneurysms at the anterior communicating artery. Heart Vessels 23:60–66
Hong YM, Jin HS, Park IS, Hong SJ (2008) Association of the matrix metalloproteinase-3 (-439C/G) promoter polymorphism with Kawasaki disease in Korean children. Heart Vessels 23:341–347
Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparrelli DJ, Dietz HC (2002) Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg 73:438–443
Bachet J, Larrazet F, Goudot B, Dreyfus G, Folliguet T, Laborde F, Guilmet D (2007) When should the aortic arch be replaced in Marfan patients? Ann Thorac Surg 83:S774–S779
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Liang, CD., Ko, SF., Chang, JP. et al. Absent pulmonary valve syndrome with ascending aortic aneurysm. Heart Vessels 25, 569–572 (2010). https://doi.org/10.1007/s00380-010-0014-3
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DOI: https://doi.org/10.1007/s00380-010-0014-3