Abstract
Fetal aortic valvuloplasty (FAV) has shown promise in averting the progression of fetal aortic stenosis to hypoplastic left-heart syndrome. Altered loading conditions due to valvar disease, intrinsic endomyocardial abnormalities, and procedures that alter endomyocardial mechanics may place patients with biventricular circulation (BiV) after FAV at risk of abnormal LV remodeling and function. Using the most recent echo data on BiV patients after technically successful FAV (n = 34), we evaluated LV remodeling pattern, risk factors for pathologic LV remodeling, and the association between LV remodeling pattern and LV function. Median age at follow-up was 4.7 years (range 1.0–12.5). Cardiac interventions were common. At latest follow-up, no patient had hypoplastic LV. Nineteen patients (55 %) had dilated LV, and five (16 %) patients had severely dilated LV. LV remodeling patterns were as follows: 12 (35 %) normal ventricle, 11 (32 %) mixed hypertrophy, 8 (24 %) eccentric hypertrophy or remodeling, and 3 (9 %) concentric hypertrophy. Univariate factors associated with pathologic LV remodeling were long-standing AR, ≥2 cardiac interventions, EFE resection, and aortic or mitral regurgitation ≥ moderate at most recent follow-up. In multivariate analysis, only long-standing AR fraction remained associated with pathologic remodeling. Pathologic LV remodeling was associated with depressed ejection fraction, lower septal E´, and higher E/E´. Pathologic LV remodeling, primarily eccentric or mixed hypertrophy, is common in BiV patients after FAV and is related to LV loading conditions imposed by valvar disease. Pathologic remodeling is associated with both systolic and diastolic dysfunction in this population.
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Beroukhim RS, Graham DA, Margossian R, Brown DW, Geva T, Colan SD (2010) An echocardiographic model predicting severity of aortic regurgitation in congenital heart disease. Circ Cardiovasc Imaging 3:542–549
Brown DW, Dipilato AE, Chong EC, Lock JE, McElhinney DB (2010) Aortic valve reinterventions after balloon aortic valvuloplasty for congenital aortic stenosis intermediate and late follow-up. J Am Coll Cardiol 56:1740–1749
Chin CH, Chen CH, Chen CC, Chen TH, Chang ML, Chiou HC (2005) Prediction of severity of isolated aortic regurgitation by echocardiography: an aortic regurgitation index study. J Am Soc Echocardiogr 18:1007–1013
Emani SM, McElhinney DB, Tworetzky W, Myers PO, Schroeder B, Zurakowski D, Pigula FA, Marx GR, Lock JE, del Nido PJ (2012) Staged left ventricular recruitment after single-ventricle palliation in patients with borderline left heart hypoplasia. J Am Coll Cardiol 60:1966–74
Emani SM, Bacha EA, McElhinney DB, Marx GR, Tworetzky W, Pigula FA, del Nido PJ (2009) J Thorac Cardiovasc Surg 136:1276–1282
Fifer MA, Borow KM, Colan SD, Lorell BH (1985) Early diastolic left ventricular function in children and adults with aortic stenosis. J Am Coll Cardiol 5:1147–1154
Friedman KG, Margossian R, Graham DA, Harrild DM, Emani SM, Wilkins-Haug LE, McElhinney DB, Tworetzky W (2011) Postnatal left ventricular diastolic function after fetal aortic valvuloplasty. Am J Cardiol 108:556–560
Friedman KG, Schidlow DN, Freud L, Escobar-Diaz MC, Tworetzky W (2014) Left ventricular diastolic function and characteristics in fetal aortic stenosis. Am J Cardiol 114:122–127
Gaasch WH, Zile MR (2011) Left ventricular structural remodeling in health and disease: with special emphasis on volume, mass, and geometry. J Am Coll Cardiol 58:1733–1740
Hasan BS, Keane JF, Tworetzky W, Lock JE, Marshall AC (2009) Postnatal angiographic appearance of left ventricular myocardium in fetal patients with aortic stenosis having in utero aortic valvuloplasty. Am J Cardiol 104:1271–1275
Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, Younoszai AK, Lai WW, Geva T (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23:465–495
Marshall AC, Tworetzky W, Bergersen L, McElhinney DB, Benson CB, Jennings RW, Wilkins-Haug LE, Marx GR (2005) Lock JE Aortic valvuloplasty in the fetus: technical characteristics of successful balloon dilation. J Pediatr 147:535–539
McElhinney DB, Marshall AC, Wilkins-Haug LE, Brown DW, Benson CB, Silva V, Marx GR, Mizrahi-Arnaud A, Lock JE, Tworetzky W (2009) Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome. Circulation 120:1482–1490
McElhinney DB, Tworetzky W, Lock JE (2010) Current status of fetal cardiac intervention. Circulation 121:1256–1263
McElhinney DB, Vogel M, Benson CB, Marshall AC, Wilkins-Haug LE, Silva V, Tworetzky W (2010) Assessment of left ventricular endocardial fibroelastosis in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome. Am J Cardiol 106:1792–1797
Mikikallio K, McElhinney DB, Levine JC, Marx GR, Colan SD, Marshall AC, Lock JE, Marcus EN, Tworetzky W (2006) Circulation 113:1401–1405
Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, O’Gara PT, Ruiz CE, Skubas NJ, Sorajja P, Sundt TM 3rd, Thomas JD (2014) J Am Coll Cardiol 63:2438–2488
Robinson JD, del Nido PJ, Geggel RL, Perez-Atayde AR, Lock JE, Powell AJ (2010) Left ventricular diastolic heart failure in teenagers who underwent balloon aortic valvuloplasty in early infancy. Am J Cardiol 106:426–429
Sharland GK, Chita SK, Fagg NL, Anderson RH, Tynan M, Cook AC, Allan LD (1991) Left ventricular dysfunction in the fetus: relation to aortic valve anomalies and endocardial fibroelastosis. Br Heart J 66(6):419–424
Tworetzky W, Wilkins-Haug L, Jennings RW et al (2004) Balloon dilation of severe aortic stenosis in the fetus: potential for prevention of hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention. Circulation 110:2125–2131
Tworetzky W, del Nido PJ, Powell AJ, Marshall AC, Lock JE, Geva T (2005) Usefulness of magnetic resonance imaging of left ventricular endocardial fibroelastosis in infants after fetal intervention for aortic valve stenosis. Am J Cardiol 96:1568–1570
Vlahos AP, Marx GR, McElhinney D, Oneill S, Goudevenos I, Colan SD (2008) Clinical utility of Doppler echocardiography in assessing aortic stenosis severity and predicting need for intervention in children. Pediatr Cardiol 29:507–514
Freud LR, McElhinney DB, Marshall AC, Marx GR, Friedman KG, del Nido PJ, Emani SM, Lafranchi T, Silva V, Wilkins-Haug LE, Benson CB, Lock JE, Tworetzky W Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: postnatal outcomes of the first 100 patients. Circulation 130(8):638–45
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Friedman, K.G., Freud, L., Escobar-Diaz, M. et al. Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty. Pediatr Cardiol 36, 1502–1509 (2015). https://doi.org/10.1007/s00246-015-1193-6
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DOI: https://doi.org/10.1007/s00246-015-1193-6