Right ventricular outflow tract enlargement prior to pulmonary valve replacement is associated with poorer structural and functional outcomes, in adults with repaired Tetralogy of Fallot

Shamus O'Meagher; Madhusudan Ganigara; Phillip Munoz; David J Tanous; Richard B Chard; David S Celermajer; Rajesh Puranik

doi:10.1016/j.hlc.2013.11.009

Right ventricular outflow tract enlargement prior to pulmonary valve replacement is associated with poorer structural and functional outcomes, in adults with repaired Tetralogy of Fallot

Heart Lung Circ. 2014 May;23(5):482-8. doi: 10.1016/j.hlc.2013.11.009. Epub 2013 Nov 28.

Authors

Shamus O'Meagher¹, Madhusudan Ganigara², Phillip Munoz³, David J Tanous⁴, Richard B Chard⁴, David S Celermajer⁵, Rajesh Puranik⁵

Affiliations

¹ The University of Sydney, Faculty of Medicine, Sydney, Australia; Royal Prince Alfred Hospital, Department of Cardiology, Sydney, Australia. Electronic address: [email protected].
² The University of Sydney, Faculty of Medicine, Sydney, Australia; The Children's Hospital at Westmead, Department of Cardiology, Sydney, Australia.
³ The University of Sydney, Faculty of Medicine, Sydney, Australia; Royal Prince Alfred Hospital, Department of Respiratory and Sleep Medicine, Sydney, Australia.
⁴ The University of Sydney, Faculty of Medicine, Sydney, Australia; Westmead Hospital, Department of Cardiology, Sydney, Australia.
⁵ The University of Sydney, Faculty of Medicine, Sydney, Australia; Royal Prince Alfred Hospital, Department of Cardiology, Sydney, Australia.

PMID: 24345378
DOI: 10.1016/j.hlc.2013.11.009

Abstract

Background: Pulmonary valve replacement (PVR) is commonly performed late after Tetralogy of Fallot (TOF) repair. We examined the effects of PVR on cardiac structure, function and exercise capacity in adults with repaired TOF.

Methods: Eighteen adult patients with repaired TOF and severe pulmonary regurgitation (PR) with right ventricular (RV) dilatation requiring PVR for clinical reasons (age; 25±8 years) were recruited to undergo cardiac MRI (1.5T) and cardiopulmonary exercise testing before and 14±3 months after PVR.

Results: Reduced indexed RV end-diastolic volume (RVEDVi; 186±32mL/m(2) pre-op vs 114±20mL/m(2) post-op, p<0.001) was observed after PVR. "Normalisation" of RVEDVi (≤108mL/m(2)) was achieved in only seven of 18 patients. Pre-PVR RVEDVi correlated with post-operative change in RVEDVi (change=-72.1±20.4mL/m(2), r=-0.815, p<0.001). Exercise capacity remained high-normal post-PVR (% predicted maximal workload: 93±16% vs 91±12%, p=0.5). Regional RV volumes were assessed; RV outflow tract (RVOT) volumes were compared to the RV muscular corpus. Large pre-PVR RVOT volumes correlated negatively with post-surgical RV ejection fraction, peak VO2 and delta VO2 at anaerobic threshold (p<0.05 for all).

Conclusions: Normalisation of RV volume is unlikely to be achieved above a pre-PVR RVEDVi of 165mL/m(2) or more. In particular, an enlarged RVOT prior to PVR predicts suboptimal structural and functional outcomes.

Keywords: Adult congenital heart disease; Cardiac magnetic resonance imaging; Cardiopulmonary exercise test; Pulmonary valve replacement; Right ventricular dilatation; Tetralogy of Fallot.

Publication types

Clinical Trial
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adult
Female
Heart Valve Prosthesis Implantation*
Humans
Magnetic Resonance Imaging
Male
Pulmonary Valve Insufficiency* / diagnostic imaging
Pulmonary Valve Insufficiency* / physiopathology
Pulmonary Valve Insufficiency* / surgery
Pulmonary Valve* / diagnostic imaging
Pulmonary Valve* / physiopathology
Pulmonary Valve* / surgery
Radiography
Tetralogy of Fallot* / diagnostic imaging
Tetralogy of Fallot* / physiopathology
Tetralogy of Fallot* / surgery
Ventricular Dysfunction, Right* / diagnostic imaging
Ventricular Dysfunction, Right* / physiopathology
Ventricular Dysfunction, Right* / surgery