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(Circulation. 2008;118:S182-S190.)
© 2008 American Heart Association, Inc.

Surgery for Congenital Heart Disease

Biventricular Response After Pulmonary Valve Replacement for Right Ventricular Outflow Tract Dysfunction

Is Age a Predictor of Outcome?

Alessandra Frigiola, MD; Victor Tsang, MD, FRCS; Catherine Bull, MRCP; Louise Coats, MRCP; Sachin Khambadkone, MD, MRCP; Graham Derrick, MD, MRCP; Bryan Mist, PhD; Fiona Walker, FRCP; Carin van Doorn, MD, FRCSC(/Th); Philipp Bonhoeffer, MD; Andrew M. Taylor, MD, MRCP, FRCR

From the Cardiothoracic Unit (A.F., V.T., C.B., L.C., S.K., G.D., C.v.D., P.B., A.M.T.), Great Ormond Street Hospital for Children, London, UK; the Centre for Cardiovascular MR (A.F., A.M.T.), UCL Institute of Child Health, London, UK; Policlinico San Donato Milanese (A.F.), IRCCS, Milano, Italy; and The Heart Hospital (V.T., B.M., F.W., C.v.D., P.B.), UCLH, London, UK.

Correspondence to Alessandra Frigiola, MD, Cardiothoracic Unit, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK. E-mail alessandra.frigiola{at}gmail.com

Background— The timing of pulmonary valve replacement (PVR) for free pulmonary incompetence in patients with congenital heart disease remains a dilemma for clinicians. We wanted to assess the determinants of improvement after PVR for pulmonary regurgitation over a wide range of patient ages and to use any identified predictors to compare clinical outcomes between patient groups.

Methods and Results— Seventy-one patients (mean age 22±11 years; range, 8.5 to 64.9; 72% tetralogy of Fallot) underwent PVR for severe pulmonary regurgitation. New York Heart Association class improved after PVR (median of 2 to 1, P<0.0001). MRI and cardiopulmonary exercise testing were performed before and 1 year after intervention. After PVR, there was a significant reduction in right ventricular volumes (end diastolic volume 142±43 to 91±18, end systolic volume 73±33 to 43±14 mL/m², P<0.0001), whereas left ventricular end diastolic volume increased (66±12 to 73±13 mL/m², P<0.0001). Effective cardiac output significantly increased (right ventricular: 3.0±0.8 to 3.3±0.8 L/min, P=0.013 and left ventricular: 3.0±0.6 to 3.4±0.7 L/min, P<0.0001). On cardiopulmonary exercise testing, ventilatory response to carbon dioxide production at anaerobic threshold improved from 35.9±5.8 to 34.1±6.2 (P=0.008). Normalization of ventilatory response to carbon dioxide production was most likely to occur when PVR was performed at an age younger than 17.5 years (P=0.013).

Conclusions— A relatively aggressive PVR policy (end diastolic volume <150 mL/m²) leads to normalization of right ventricular volumes, improvement in biventricular function, and submaximal exercise capacity. Normalization of ventilatory response to carbon dioxide production is most likely to occur when surgery is performed at an age 17.5 years. This is also associated with a better left ventricular filling and systolic function after surgery.

Key Words: echocardiography • exercise • MRI • pulmonary regurgitation • surgical repair