TY - JOUR
T1 - Exercise capacity and cardiac reserve in children and adolescents with corrected pulmonary atresia with intact ventricular septum after univentricular palliation and biventricular repair
AU - Romeih, Soha
AU - Groenink, Maarten
AU - Roest, Arno A. W.
AU - van der Plas, Mart N.
AU - Hazekamp, Mark G.
AU - Mulder, Barbara J. M.
AU - Blom, Nico A.
PY - 2012
Y1 - 2012
N2 - Objective: Management of pulmonary atresia with intact ventricular septum is challenging and depends on the severity of right ventricular hypoplasia. Clinical outcomes of biventricular repair seem favorable to univentricular palliation, but data on superiority of biventricular repair regarding exercise capacity are conflicting. We investigated the response to physical and pharmacologic stress in patients with surgically corrected pulmonary atresia with intact ventricular septum. Methods: Sixteen patients (7 patients after univentricular palliation, age 11.8 +/- 2.6 years; 7 patients after biventricular repair, age 12.9 +/- 2.7 years; and 2 patients after 1.5 ventricular repair, age 12 and 19 years) underwent cardiopulmonary exercise test, dobutamine stress magnetic resonance imaging, and delayed contrast-enhanced magnetic resonance imaging. Results: The univentricular group showed impaired exercise capacity in contrast with normal exercise capacity in the biventricular group. Left ventricular ejection fraction increased in both groups. With dobutamine, left ventricular stroke volume increased only in the biventricular group (+11.3 +/- 5.0 mL, P <.001) and not in the univentricular group (+0.04 +/- 3.6 mL, P = .9). Heart rate increase was inadequate in the univentricular group. Maximum oxygen consumption and oxygen pulse were strongly correlated with left ventricular stroke volume during stress but not at rest. The results of the 2 patients after 1.5 ventricular repair were comparable to those of the univentricular group. No myocardial fibrosis was detected. Conclusions: Impaired exercise capacity in children and adolescents with pulmonary atresia with intact ventricular septum after univentricular palliation is related to decreased cardiac reserve and inadequate chronotropic response. Young patients with pulmonary atresia with intact ventricular septum after biventricular repair show normal exercise capacity and cardiac reserve. (J Thorac Cardiovasc Surg 2012;143:569-75)
AB - Objective: Management of pulmonary atresia with intact ventricular septum is challenging and depends on the severity of right ventricular hypoplasia. Clinical outcomes of biventricular repair seem favorable to univentricular palliation, but data on superiority of biventricular repair regarding exercise capacity are conflicting. We investigated the response to physical and pharmacologic stress in patients with surgically corrected pulmonary atresia with intact ventricular septum. Methods: Sixteen patients (7 patients after univentricular palliation, age 11.8 +/- 2.6 years; 7 patients after biventricular repair, age 12.9 +/- 2.7 years; and 2 patients after 1.5 ventricular repair, age 12 and 19 years) underwent cardiopulmonary exercise test, dobutamine stress magnetic resonance imaging, and delayed contrast-enhanced magnetic resonance imaging. Results: The univentricular group showed impaired exercise capacity in contrast with normal exercise capacity in the biventricular group. Left ventricular ejection fraction increased in both groups. With dobutamine, left ventricular stroke volume increased only in the biventricular group (+11.3 +/- 5.0 mL, P <.001) and not in the univentricular group (+0.04 +/- 3.6 mL, P = .9). Heart rate increase was inadequate in the univentricular group. Maximum oxygen consumption and oxygen pulse were strongly correlated with left ventricular stroke volume during stress but not at rest. The results of the 2 patients after 1.5 ventricular repair were comparable to those of the univentricular group. No myocardial fibrosis was detected. Conclusions: Impaired exercise capacity in children and adolescents with pulmonary atresia with intact ventricular septum after univentricular palliation is related to decreased cardiac reserve and inadequate chronotropic response. Young patients with pulmonary atresia with intact ventricular septum after biventricular repair show normal exercise capacity and cardiac reserve. (J Thorac Cardiovasc Surg 2012;143:569-75)
U2 - https://doi.org/10.1016/j.jtcvs.2011.08.012
DO - https://doi.org/10.1016/j.jtcvs.2011.08.012
M3 - Article
C2 - 21908009
SN - 0022-5223
VL - 143
SP - 569
EP - 575
JO - Journal of Thoracic and Cardiovascular Surgery
JF - Journal of Thoracic and Cardiovascular Surgery
IS - 3
ER -