Systemic Ventricular Dysfunction Between Stage One and Stage Two Palliation
Infants with a single ventricle can develop systemic ventricular dysfunction (SVD) after stage 1 operation, but available information is sparse. We reviewed our patients having Norwood, Sano, or hybrid procedures to better understand this problem. We conducted a retrospective, case-controlled cohort study of 267 patients having stage1 operation, examining outcomes between stages 1 and 2 (survival and subsequent cardiac surgeries), predictor variables, and histology of hearts explanted at transplantation. SVD developed in 32 (12%) patients and resolved in 13 (41%); mean age of onset was 3.0 ± 1.63 months; median = 2.79. SVD was not associated with cardiac anatomy, type of stage 1 procedure, weight, coronary abnormality, or atrioventricular valve regurgitation. The mean age of resolution = 12.1 ± 9.6 months; median = 6.3, and resolution may have been more likely with a systemic LV than RV (p = 0.067). Outcomes for the entire SVD group were less favorable than for those without, but patients with resolution of SVD had outcomes at least as good those without SVD. Myocardial histology (n = 4) suggested chronic ischemia. The risk of SVD after stage 1, while low, may be a fundamental feature of this patient population. SVD occurs with either a systemic RV or LV, although patients with a systemic LV may be more likely to have resolution than those with an RV. We identified no predictor variables, but histologic findings suggest chronic ischemia may be involved. Given the low incidence of SVD, multi-center studies will be required to better define predictors of onset and resolution.
KeywordsHypoplastic left heart syndrome Norwood Sano Cardiomyopathy Ventricular dysfunction
We acknowledge the expert assistance of Lynne Patkin, MBA, in aiding with preparation of this manuscript.
Compliance with Ethical Standards
Conflict of interest
T. Kulik, L. Sleeper, C. VanderPluym and S. Sanders declares that they have no conflict of interest.
All procedures performed in studies involving human participants were is accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This study was approved by the Institutional Review Board of Boston Children’s Hospital.
- 2.Hsu DT, Zak V, Mahony L, Sleeper LA, Atz AM, Levine JC, Barker PC, Ravishankar C, McCrindle BW, Williams RV, Altmann K, Ghanayem NS, Margossian R, Chung WK, Border WL, Pearson GD, Stylianou MP, Mital S, Pediatric Heart Network Investigators (2010) Enalapril in infants with single ventricle: results of a multicenter randomized trial. Circulation 122(4):333–340. https://doi.org/10.1161/CIRCULATIONAHA.109.927988 CrossRefPubMedPubMedCentralGoogle Scholar
- 3.Khoo NS, Smallhorn JF, Kaneko S, Myers K, Kutty S, Tham EB (2011) Novel insights into RV adaptation and function in hypoplastic left heart syndrome between the first 2 stages of surgical palliation. JACC Cardiovasc Imaging 4(2):128–137. https://doi.org/10.1016/j.jcmg.2010.09.022 CrossRefPubMedGoogle Scholar
- 4.Jean-St-Michel E, Chetan D, Schwartz SM, Van Arsdell GS, Floh AA, Honjo O, Conway J (2016) Outcomes in patients with persistent ventricular dysfunction after stage i palliation for hypoplastic left heart syndrome. Pediatr Cardiol 37(2):239–247. https://doi.org/10.1007/s00246-015-1268-4 CrossRefPubMedGoogle Scholar
- 6.Sugiyama H, Yutani C, Iida K, Arakaki Y, Yamada O, Kamiya T (1999) The relation between right ventricular function and left ventricular morphology in hypoplastic left heart syndrome: angiographic and pathological studies. Pediatr Cardiol 20(6):422–427. https://doi.org/10.1007/s002469900504 CrossRefPubMedGoogle Scholar
- 10.Aiyagari R, Rhodes JF, Shrader P, Radtke WA, Bandisode VM, Bergersen L, Gillespie MJ, Gray RG, Guey LT, Hill KD, Hirsch R, Kim DW, Lee KJ, Pelech AN, Ringewald J, Takao C, Vincent JA, Ohye RG, Pediatric Heart Network Investigators (2014) Impact of pre-stage II hemodynamics and pulmonary artery anatomy on 12-month outcomes in the Pediatric Heart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 148(4):1467–1474. https://doi.org/10.1016/j.jtcvs.2013.10.057 CrossRefPubMedGoogle Scholar
- 13.Sathanandam S, Cui W, Nguyen NV, Husayni TS, Van Bergen AH, Sajan I, El-Zein C, Polimenakos A, Ilbawi MN, Roberson DA (2010) Ventriculocoronary artery connections with the hypoplastic left heart: a 4-year prospective study: incidence, echocardiographic and clinical features. Pediatr Cardiol 31(8):1176–1185. https://doi.org/10.1007/s00246-010-9783-9 CrossRefPubMedGoogle Scholar
- 17.Donnelly JP, Raffel DM, Shulkin BL, Corbett JR, Bove EL, Mosca RS, Kulik TJ (1998) Resting coronary flow and coronary flow reserve in human infants after repair or palliation of congenital heart defects as measured by positron emission tomography. J Thorac Cardiovasc Surg 115(1):103–110CrossRefGoogle Scholar
- 19.Topol EJ, Weiss JL, Guzman PA, Dorsey-Lima S, Blanck TJ, Humphrey LS, Baumgartner WA, Flaherty JT, Reitz BA (1984) Immediate improvement of dysfunctional myocardial segments after coronary revascularization: detection by intraoperative transesophageal echocardiography. J Am Coll Cardiol 4(6):1123–1134CrossRefGoogle Scholar
- 21.Margossian R, Schwartz ML, Prakash A, Wruck L, Colan SD, Atz AM, Bradley TJ, Fogel MA, Hurwitz LM, Marcus E, Powell AJ, Printz BF, Puchalski MD, Rychik J, Shirali G, Williams R, Yoo SJ, Geva T, Pediatric Heart Network Investigators (2009) Comparison of echocardiographic and cardiac magnetic resonance imaging measurements of functional single ventricular volumes, mass, and ejection fraction (from the Pediatric Heart Network Fontan Cross-Sectional Study). Am J Cardiol 104(3):419–428. https://doi.org/10.1016/j.amjcard.2009.03.058 CrossRefPubMedPubMedCentralGoogle Scholar