Abstract
Children born with a functional single ventricle who undergo Fontan palliation are prone to early pump failure. Whether they develop early arterial stiffness with resultant increase in afterload is not well known. We hypothesized that the aortic stiffness is higher in pediatric Fontan patients when compared to healthy controls. A prospective study was conducted at the Children’s Hospital of Michigan. Twenty-two Fontan patients (aged 6–21 years) were compared with 22 healthy controls (aged 9–17 years) selected from children referred to our clinic who had normal cardiac anatomy and function on the echocardiogram. Aortic stiffness was assessed noninvasively by measuring the aortic augmentation index (AAI) using applanation tonometry (Sphygmocor, Atcor, IL). AAI was calculated as AP/PP where augmentation pressure (AP) is the increase in aortic systolic blood pressure (BP) and pulse pressure (PP) is the difference between aortic systolic and diastolic BP. Ten patients (45 %) had hypoplastic left ventricle, and 11 (50 %) had undergone aortic arch surgery. The median AAI was significantly higher in Fontan patients when compared to controls (12.5, IQR 4.8, 17.3 vs 0, IQR −6.3, 5.8; p = 0.0003). History of aortic arch surgery and single ventricle morphology did not have a significant impact on AAI. Pediatric patients who undergo Fontan palliation have significantly higher AAI, a marker of aortic stiffness and increased afterload, compared to healthy controls. Larger longitudinal studies are warranted to elucidate the possible contribution of elevated AAI on pump failure in these patients.
Similar content being viewed by others
References
Agnoletti G, Ou P, Celermajer DS, Boudjemline Y et al (2008) Acute angulation of the aortic arch predisposes a patient to ascending aortic dilatation and aortic regurgitation late after the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 135(3):568–572
Belz GG (1995) Elastic properties and Windkessel function of the human aorta. Cardiovasc Drugs Ther 9(1):73–83
Boutouyrie P, Tropeano AI, Asmar R et al (2002) Aortic stiffness is an independent predictor of primary coronary events in hypertensive patients: a longitudinal study. Hypertension 39:10–15
Cardis BM, Fyfe DA, Mahle WT (2006) Elastic properties of the reconstructed aorta in hypoplastic left heart syndrome. Ann Thorac Surg 81(3):988–991
Demir S, Akpınar O, Akkus O, Nas K, Unal I, Molnar F, Demir A, Illyés M, Acartürk E (2013) The prognostic value of arterial stiffness in systolic heart failure. Cardiol J 20(6):665–671
d’Udekem Y, Xu MY, Galati JC et al (2012) Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance. J Am Coll Cardiol 59(13):1178–1185
Feinstein JA, Benson DW, Dubin AM et al (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59(1 Suppl):S1–S42
Goldstein BH, Golbus JR, Sandelin AM, Warnke N, Gooding L, King KK, Donohue JE, Gurney JG, Goldberg CS, Rocchini AP, Charpie JR (2011) Usefulness of peripheral vascular function to predict functional health status in patients with Fontan circulation. Am J Cardiol 108(3):428–434
Griffiths ER, Kaza AK, Wyler von Ballmoos MC et al (2009) Evaluating failing Fontans for heart transplantation: predictors of death. Ann Thorac Surg 88(2):558–563 discussion 563–4
Henaine R, Vergnat M, Bacha EA, Baudet B, Lambert V, Belli E, Serraf A (2013) Effects of lack of pulsatility on pulmonary endothelial function in the Fontan circulation. J Thorac Cardiovasc Surg 146(3):522–529
Jacobs JP, O’Brien SM, Pasquali SK et al (2011) Variation in outcomes for benchmark operations: an analysis of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg 92(6):2184–2191
Jin SM, Noh CI, Bae EJ, Choi JY, Yun YS (2007) Impaired vascular function in patients with Fontan circulation. Int J Cardiol 120(2):221–226
Khattar (2001) Pulse pressure and prognosis. Heart 85:484–486
Komajda M, Lam CS (2014) Heart failure with preserved ejection fraction: a clinical dilemma. Eur Heart J 35(16):1022–1032
Lambert E, d’Udekem Y, Cheung M et al (2012) Sympathetic and vascular dysfunction in adult patients with Fontan circulation. Int J Cardiol. doi:10.1016/j.ijcard.2012.04.015
Lurbe E, Torro MI, Carvajal E, Alvarez V, Redón J (2003) Birth weight impacts on wave reflections in children and adolescents. Hypertension 41:646–650
Mahle WT, Todd K, Fyfe DA (2003) Endothelial function following the Fontan operation. Am J Cardiol 91(10):1286–1288
Mortensen K, Baulmann J, Rybczynski M et al (2010) Augmentation index and the evolution of aortic disease in marfan-like syndromes. Am J Hypertens 23(7):716–724
Natarajan S, Heiss C, Yeghiazarians Y, Fineman JR, Teitel DF, Tacy TA (2009) Peripheral arterial function in infants and young children with one-ventricle physiology and hypoxemia. Am J Cardiol 103(6):862–866
Nichols WW, O’Rourke MF (2009) Aortic pulse wave velocity, reflection site distance, and augmentation index. Hypertension 53(1):e9
Patange AR, Valentini RP, Du W, Pettersen MD (2012) Vitamin D deficiency and arterial wall stiffness in children with chronic kidney disease. Pediatr Cardiol 33(1):122–128
Pauca Alfredo L, O’Rourke Michael F, Kon Neal D (2001) Prospective evaluation of a method for estimating ascending aortic pressure from the radial artery pressure waveform. Hypertension 38:932–937
Safar ME, Levy BI, Struijker-Boudier H (2003) Current perspectives on arterial stiffness and pulse pressure in hypertension and cardiovascular diseases. Circulation 107:2864–2869
Senzaki H, Chen CH, Ishido H, Masutani S et al (2005) Arterial hemodynamics in patients after Kawasaki disease. Circulation 111(16):2119–2125
Szczepaniak-Chicheł L, Trojnarska O, Mizia-Stec K et al (2011) Augmentation of central arterial pressure in adult patients after coarctation repair. Blood Press Monit 16(1):22–28
Walsh MA, McCrindle BW, Dipchand A et al (2009) Left ventricular morphology influences mortality after the Norwood operation. Heart 95(15):1238–1244
Weber T, Auer J, O’Rourke MF et al (2004) Arterial stiffness, wave reflections, and the risk of coronary artery disease. Circulation 109:184–189
Wilkinson IB et al (2010) ARTERY Society guidelines for validation of non-invasive haemodynamic measurement devices: Part 1, arterial pulse wave velocity. Artery Res 4:34–40
Acknowledgments
The authors gratefully acknowledge pediatric cardiology faculty and fellows at Children’s Hospital of Michigan for their support in data collection.
Conflict of interest
None of the authors have any financial disclosures.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Bhat, D.P., Gupta, P. & Aggarwal, S. Elevated Aortic Augmentation Index in Children Following Fontan Palliation: Evidence of Stiffer Arteries?. Pediatr Cardiol 36, 1232–1238 (2015). https://doi.org/10.1007/s00246-015-1151-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-015-1151-3