P1467 Natural history and outcome of a patient with untreated congenital pulmonary stenosis added value of 3d echo in decision making
Authors: De Alcantara M L , Felix A S , Siciliano A P R V , Veronesi H E , Vedovelli C E F , Nunes E M 
Published: January 2020

Abstract

INTRODUCTION
congenital pulmonary valve stenosis (PVS) accounts for approximately 7% of all congenital heart defects. In less severe and untreated asymptomatic forms, it might have variable outcomes with progressive stenosis or development and worsening of pulmonary regurgitation . Atrial septal defect (ASD) might be associated in up to 20% of the cases rendering an even more unpredictable outcome and efect on right ventricular (RV) volumes and function. Cardiac magnetic ressonance is considered the gold standard method to measure RV function and volumes. Three dimensional echocardiography (3D echo) emerges as a valuable, redproducible and portable alternative not only for RV volume and function analysis but also for structural evaluation.

PURPOSE
To describe the added value of 3D echo for the diagnostic work-up, decision making and post surgical outcome in a patient with longstanding untreated PVS associated with ASD.

CASE PRESENTATION
a 56 year old asymptomatic , non syndromic man , was referred by the cardiac surgeon for a comprehensive echocardiographic evaluation (CEE) due to a previous diagnosis of PVS .The patient was aware of this diagnosis since the age of 17 and no intervention was undertaken at that time. More recently a conventional echocardiogram described a moderate PVS with RV enlargement (RVE) and no futher details regarding RV function or surgical feasibility. CEE showed RVE due to volume overload (VO) with indexed end diastolic volume (EDV) of 144ml/m2 (upper limit 76ml/m2), end sistolic volume (ESV) of 57ml/m2 (upper limit 37ml/m2) and an RV ejection fraction (RVEF) of 60% (lower limit 45%) on 3D echo. RV free wall strain (RVFWS) was -25,3% , TAPSE 32mm and fractional area change 46%, all paramenters within the normal range except for RV volumes. Anatomical analysis showed PVS with three leaflets, partial comissural fusion and retraction of the edges yelding a closure gap of at least 8mm and consequent severe regurgitation. Peak transvalvular gradient was 38mmHg and pulmonary artery was dilated measuring 6.4cm. An ostium secundum atrial septal defect (ASD) with left to right shunt measuring 15x9mm by 3D echo contributed for futher RVVO. With these information , surgical intervention was undertaken and consisted of PV replacement, excision and reconstruction of the pulmonary trunk dilation and correction of the ASD. The patient recovered well and returned three months after, for a new evaluation. At that time RV volumes reduced dramatically with EDV and ESV respectively of 44ml/m2 and 19ml/m2 RVEF of 55,7% and RWFWS of -25,6%.

CONCLUSION
untreated congenital PVS associated with ASD may have a variable outcome . Follow-up is mandatory. In the present case a CEE including more recent technologies like 3D echo and strain imaging helped in defining anatomical findings and RV function analysis thus stating the appropriatness for surgical intervention.
Abstract P1467 Figure. 3Decho pre and post surgical analysis