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. 2021 Jul;304(1):81-90.
doi: 10.1007/s00404-020-05929-0. Epub 2021 Feb 14.

Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome

Aline Wolter  1 , Natalia Markert  2 , Jan Sebastian Wolter  3 , Andrii Kurkevych  4 , Jan Degenhardt  5 , Jochen Ritgen  5 , Rüdiger Stressig  5 , Christian Enzensberger  2 , Ivonne Bedei  2 , Carina Vorisek  2 , Johanna Schenk  2 , Oliver Graupner  6 , Markus Khalil  7 , Josef Thul  7 , Christian Jux  7 , Roland Axt-Fliedner  2
Affiliations

Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome

Aline Wolter et al. Arch Gynecol Obstet. 2021 Jul.

Abstract

Objectives: To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS).

Methods: We evaluated 82 foetuses from 01/08 to 10/18 in 3 centres in intervals 1 (< 24 weeks), 2 (24-30 weeks) and 3 (> 30 weeks).

Results: 61/82 (74.4%) were livebirths, 5 (8.2%) lost for follow-up, 3 (4.9%) had compassionate care leaving 53 (64.6% of the whole cohort and 86.9% of livebirths) with intention to treat. 9 died, 44/53 (83.0%) survived. 24/38 (63.2%) with information on postnatal outcome had BV outcome, 14 (36.8%) non-BV outcome (2 × 1.5 circulation). One with BV outcome had prenatal valvuloplasty. Best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). Ventriculo-coronary-arterial communications (VCAC) were present in 11 (78.6%) in non-BV outcome group vs. 2 (8.3%) in BV outcome group (p < 0.001). Tricuspid insufficiency (TI)-Vmax > 2.5 m/s was present in BV outcome group in75.0% (18/24) vs. 14.3% (2/14) in non-BV outcome group. Including the most predictive markers (VCAC presence, TI- Vmax < 2.5 m/s, TV/MV ratio < cutoff) to a score, non-BV outcome was correctly predicted when > 1 criterion was fulfilled in all cases. After recently published criteria for foetal intervention, only 4/9 (44.4%) and 5/14 (35.7%) in our interval 2 + 3 with predicted non-BV outcome would have been candidates for intervention. Two (1 × intrauterine intervention) in interval 2, two in interval 3 reached BV outcome and one 1.5 circulation without intervention.

Conclusion: TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases. Criteria for foetal intervention must further be evaluated.

Keywords: Biventricular outcome; Critical pulmonary stenosis; Fetal echocardiography; Pulmonary atresia with intact ventricular septum.

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Conflict of interest statement

No conflict of interest.

Figures

Fig. 1
Fig. 1
Example of fetus with PAIVS
Fig. 2
Fig. 2
Example of measurement of MV
Fig. 3
Fig. 3
Example of measurement of TV
Fig. 4
Fig. 4
Overview of our cohort
Fig. 5
Fig. 5
ROC curve for TV/MV ratio interval 2
Fig. 6
Fig. 6
ROC curve for TV/MV ratio interval 3
Fig. 7
Fig. 7
ROC curve for score parameters interval 2
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References

    1. Daubeney PEF, Sharland GK, Cook AC, Keeton BR, Anderson RH, Webber SA. Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. Circulation. 1998;98(6):562–566. doi: 10.1161/01.CIR.98.6.562. - DOI - PubMed
    1. Daubeney PEF, Wang D, Delany DJ, Keeton BR, Anderson RH, Slavik Z, et al. Pulmonary atresia with intact ventricular septum: Predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg. 2005;130(4):1–9. doi: 10.1016/j.jtcvs.2005.05.044. - DOI - PubMed
    1. Dyamenahalli U, McCrindle BW, McDonald C, Trivedi KR, Smallhorn JF, Benson LN, et al. Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients. Cardiol Young. 2004;14(3):299–308. doi: 10.1017/S1047951104003087. - DOI - PubMed
    1. Schwedler G, Lindinger A, Lange PE, Sax U, Olchvary J, Peters B, et al. Frequency and spectrum of congenital heart defects among live births in Germany : a study of the competence network for congenital heart defects. Clin Res Cardiol. 2011;100(12):1111–1117. doi: 10.1007/s00392-011-0355-7. - DOI - PubMed
    1. Gardiner HM, Belmar C, Tulzer G, Barlow A, Pasquini L, Carvalho JS, et al. Morphologic and functional predictors of eventual circulation in the fetus with pulmonary atresia or critical pulmonary stenosis with intact septum. J Am Coll Cardiol. 2008;51(13):1299–1308. doi: 10.1016/j.jacc.2007.08.073. - DOI - PubMed

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