Abstract
Combination of right and left ventricular outflow tracts obstruction is extremely rare. Neonates with combined aortic stenosis (AS) and pulmonary stenosis (PS) present in critical condition and required urgent treatment. The management approach is not well defined. We report five female neonates with combined AS and PS presented to our institute in the last 5 years, age (1–18 days), weight (2.2–3.4 kg). Two had associated muscular ventricular septal defects. The mean Doppler gradient across the aortic valve (AV) was 73 mmHg (53–105 mmHg) and across the pulmonary valve was 62 mmHg (44–76 mmHg). Three had balloon dilatation and one surgical repair. The fifth patient was managed conservatively, but had sudden cardiac death at age of 3 months. One patient arrived in shock and sepsis, underwent emergency balloon dilation of the AV in the ICU. Despite decreasing the gradient, she died next day after the procedure. The surviving children were well at median follow-up age of 3.4 years. This is an extremely rare combination which needs early intervention. The management approach is not well defined. Interventional catheterization is possibly the better option.
This is a preview of subscription content, log in via an institution to check access.
References
Atik FA, Souto FA, Furlanetto BH, Furlanetto G, Soraqqi AM, da Costa AJ (1996) Supravalvar and valvar aortic stenosis associated with valvar and subvalvar pulmonary stenosis. Coexistence of two clinical syndromes. Arq Bras Cardiol 66(5):281–284
Bu Lock FA, Joe HS, Jordan SC, Martin RP (1993) Balloon dilatation (valvoplasty) as first line treatment for severe stenosis of the aortic valve in early infancy: medium term results and determinants of survival. Br Heart J 70:546–553
Chandrashekhar Y, Sen S, Bidwai PS, Khattri HN (1994) Syndrome of combined aortic and pulmonary valve stenosis successful treatment with balloon dilatation. Am Heart J 127(6):1631–1633
da Cruz E, Billieux MH, Beghetti M (2007) A neonate with isolated combined aortic and pulmonary valve stenosis. Int J Cardiol 116(1):e13-4 (Epub 2006 Nov 7)
Elhoury ME, Faqeih E, Almoukirish AS, Galal MO (2013) Cardiac involvement in geleophysic dysplasia in three siblings of a Saudi family. Cardiol Young. doi:10.1017/S1047951113001753
Galal O, Kalloghlian A, Pittapilly BM, Dzimiri N (1999) Phentolamine improves clinical outcome after balloon valvuloplasty in patients with critical pulmonary stenosis. Cardiol Young 9:127–128
Galal MO, Alzahrani AM, Elhoury ME (2012) Angiotensin converting enzyme inhibitor as an additive treatment after successful balloon dilation of a critical pulmonary valve stenosis. J Saudi Heart Assoc 24:47–50
Herberg U, Goltz D, Weiss H, Gembruch U, Breuer J (2009) Combined pulmonary and aortic valve stenosis—prenatal diagnosis and postnatal interventional therapy. Neonatology 96:244–247
Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39(12):1890–1900
Horlick L, Merriman JE (1957) Congenital valvular stenosis of pulmonary and aortic valves with atrial septal defect. Am Heart J 54(4):615–620
Kobayashi T, Momoi N, Fukuda Y, Suzuki H (2005) Percutaneous balloon valvuloplasty of both pulmonary and aortic valves in a neonate with pulmonary atresia and critical aortic stenosis. Pediatr Cardiol 26(6):839–842
Layangool T, Promphan W, Kirawittaya T, Sangtawesin C, Makarapong M (2011) Combined aortic and pulmonic valvular stenosis: report of 2 cases. J Med Assoc Thai 94(Suppl. 3):S217–S221
Martin PR, Radley-Smith R, Yacoub MH (1987) Pulmonary atresia and aortic valve stenosis. Int J Cardiol 16(1):103–105
Milazzo AS, Golioto A, Camitta MG, O’Laughlin MP (2003) An infant with subvalvar and valvar aortic stenosis, subvalvar and valvar pulmonary stenosis, severe biventricular hypertrophy and pulmonary hemorrhage. Pediatr Cardiol 24(2):169–171
Nadas AS, Hauwaert LV, Hauck AJ, Gross RE (1962) Combined aortic and pulmonic stenosis. Circulation 25:346–355
Niinami H, Imai Y, Kurosawa H, Fukuchi K, Harada Y (1990) Konno procedure for congenital aortic valve stenosis with pulmonary valve stenosis. Nihon Kyobu Geka Gakkai Zasshi 38(4):654–659
Shemin RJ, Kent KM, Roberts WC (1979) Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect. Br Heart J 42(4):442–446
Author information
Authors and Affiliations
Corresponding author
Additional information
Motea E. Elhoury and Milad El-Segaier had same contribution in this work.
Rights and permissions
About this article
Cite this article
Elhoury, M.E., El-Segaier, M., Momenah, T. et al. Combined Semilunar Valve Stenoses in Neonates: Management Approaches and Literature Review. Pediatr Cardiol 35, 1469–1473 (2014). https://doi.org/10.1007/s00246-014-1000-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-014-1000-9