title: Exercise capacity and cardiac reserve in children and adolescents with corrected pulmonary atresia with intact ventricular septum after univentricular palliation and biventricular repair.
abstract: 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.
authors: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
authors: Soha
authors: S
authors: Romeih
authors: Maarten
authors: M
authors: Groenink
authors: Arno A W
authors: AA
authors: Roest
authors: Mart N
authors: MN
authors: van der Plas
authors: Mark G
authors: MG
authors: Hazekamp
authors: Barbara J M
authors: BJ
authors: Mulder
authors: Nico A
authors: NA
authors: Blom
mesh_terms: D000293:Adolescent; D058665:Adrenergic beta-1 Receptor Agonists; D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D002648:Child; D003287:Contrast Media; D004280:Dobutamine; D005080:Exercise Test; D017079:Exercise Tolerance; D005355:Fibrosis; D006330:Heart Defects, Congenital / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D006339:Heart Rate; D006352:Heart Ventricles / Q000002:abnormalities / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D009426:Netherlands; D010101:Oxygen Consumption; D010166:Palliative Care; D011237:Predictive Value of Tests; D018633:Pulmonary Atresia / Q000503:physiopathology / Q000601:surgery*; D013318:Stroke Volume; D013997:Time Factors; D016896:Treatment Outcome; D016277:Ventricular Function, Left; D054088:Ventricular Septum / Q000473:pathology*; D055815:Young Adult
chemical_list: D058665:Adrenergic beta-1 Receptor Agonists; D003287:Contrast Media; D004280:Dobutamine