• Soha Romeih, Maarten Groenink, Mart N van der Plas, Anje M Spijkerboer, Mark G Hazekamp, Saskia Luijnenburg, Barbara J M Mulder, and Nico A Blom.
• Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands.
• Eur J Cardiothorac Surg. 2012 Jul 1;42(1):50-5.

ObjectivesIn patients with pulmonary atresia with intact ventricular septum (PAIVS), biventricular repair is considered to be the optimal treatment option in the absence of significant right ventricular (RV) hypoplasia. However, long-term clinical outcome studies are limited. We evaluated exercise capacity and cardiac function during pharmacological stress in children and young adults with PAIVS after biventricular repair.MethodsTen PAIVS patients after biventricular repair, with a median age of 12 years (range 9-42 years), underwent a cardiopulmonary exercise test, dobutamine stress magnetic resonance imaging (DS-MRI) and delayed contrast enhancement (DCE) MRI.ResultsThe patients' ages negatively correlated with exercise capacity (r=-0.72, P=0.01) as well as left (LV) and RV stroke volume (SV) response to pharmacological stress (r=-0.72, P=0.02; and r=-0.64, P=0.04; respectively), Furthermore, older age was associated with decreased RV E/A volume ratio and increased pulmonary late diastolic forward flow percentage (r=0-0.65, P=0.04, r=0.66, P=0.03, respectively). RV E/A volume ratio positively correlated with RV-SV response to DS-MRI (r=0.77, P=0.009). and O2-pulse during physical stress correlated with biventricular SV response to DS-MRI. No RV or LV ventricular myocardial fibrosis was detected.ConclusionsIn PAIVS patients after biventricular repair exercise capacity and cardiac reserve decrease with age. These findings appear to be related to impaired diastolic RV function and decreased RV filling, indicating that the function of the relatively small RV deteriorates with time.

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