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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Morphologic and Functional Predictors of Eventual Circulation in the Fetus With Pulmonary Atresia or Critical Pulmonary Stenosis With Intact Septum

Helena M. Gardiner, PhD, MD, FRCP^_*,,_*, Cristian Belmar, MD^_*, Gerald Tulzer, MD, PhD, Anna Barlow, BS^_*,, Lucia Pasquini, MD^_*, Julene S. Carvalho, PhD, FRCPCH^,, Piers E.F. Daubeney, MRCP, Michael L. Rigby, FRCP, Fabiana Gordon, PhD^||, Elena Kulinskaya, PhD^|| and Rodney C. Franklin, FRCP

^_* Institute of Reproductive and Developmental Biology, Faculty of Medicine, Imperial College at Queen Charlotte’s and Chelsea Hospital, London, United Kingdom
Brompton Fetal Cardiology, Royal Brompton Hospital and the National Heart and Lung Institute, London, United Kingdom
Childrens’ Heart Centre, Linz, Austria
Fetal Medicine Unit, St. George’s Hospital, London, United Kingdom
^|| Statistical Advisory Service, Imperial College, London, United Kingdom.

Manuscript received March 8, 2007; revised manuscript received August 13, 2007, accepted August 20, 2007.

^_* Reprint requests and correspondence: Dr. Helena M. Gardiner, Institute of Reproductive and Developmental Biology, Faculty of Medicine, Imperial College, Queen Charlotte’s and Chelsea Hospital, Du Cane Road, London, W12 0HS, United Kingdom. (Email: helena.gardiner{at}imperial.ac.uk).

Objectives: The purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or critical pulmonary stenosis with reversal of ductal flow (CPS) using statistical modeling.

Background: Pulmonary atresia with intact ventricular septum is rarely associated with chromosomal or extra cardiac malformations, so decisions about continuing a pregnancy are strongly influenced by the prediction of univentricular (UV) or biventricular (BV) circulation.

Methods: Predictive scores were derived, using a combination of z-scores of fetal cardiac measurements (for femoral length) and tricuspid/mitral valve (TV/MV) ratios, to facilitate early prediction of UV or BV circulation in 21 fetuses with PAIVS (18 fetuses) or CPS (3 fetuses) between 1998 and 2004. We also assessed the predictive value of coronary fistulae and right atrial pressure (RAP) score (comprising the tricuspid valve, foramen ovale, and ductus venosus Doppler).

Results: One-half of the cohort was first assessed before 23 gestational weeks (range 15.7 to 33.7 weeks). The TV z-score was a good predictor at all gestations, but the best predictive scores for specific gestations were pulmonary valve (PV) z-score (<23 weeks), median TV z-score (<26 weeks), the combination of median PV z-score and the median TV/MV ratio (26 to 31 weeks), and the combination of median TV z-score and median TV/MV ratio (>31 weeks). The RAP score and coronary fistulae were good independent predictors: RAP score >3 predicted BV with area under the curve of 0.833, and detection of fistulae usually predicted a UV route.

Conclusions: The best predictive scores for post-natal outcome in fetal PAIVS/CPS are a combination of morphologic and physiological variables, which predict a BV circulation with a sensitivity of 92% and specificity of 100% before 26 weeks.

Abbreviations and Acronyms   AUC = area under the receiver-operating characteristic curve   BV = biventricular   CPS = critical pulmonary stenosis with reversal of ductal flow   dP/dt = first derivative of pressure measured over time   DV = ductus venosus   LV = left ventricle/ventricular   MV = mitral valve   PAIVS = pulmonary atresia with intact ventricular septum   PV = pulmonary valve   RA = right atrium   RAP = right atrial pressure   ROC = receiver-operating characteristic   RV = right ventricle/ventricular   RVDCC = right ventricular-dependent coronary circulation   TR = tricuspid regurgitation   TV = tricuspid valve   UV = univentricular

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