scholarly journals Prenatal Tricuspid Valve Size as a Predictor of Postnatal Outcome in Patients with Severe Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum

2014 ◽  
Vol 35 (2) ◽  
pp. 101-107 ◽  
Author(s):  
Alexander Lowenthal ◽  
Breniel Lemley ◽  
Alaina K. Kipps ◽  
Michael M. Brook ◽  
Anita J. Moon-Grady
Keyword(s):  
Tricuspid Valve ◽  
Pulmonary Stenosis ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Valve Size ◽  
Postnatal Outcome

Fetal Tricuspid Valve Size and Growth as Predictors of Outcome in Pulmonary Atresia With Intact Ventricular Septum

PEDIATRICS ◽  
2006 ◽  
Vol 118 (2) ◽  
pp. e415-e420 ◽  
Author(s):  
J. W. Salvin ◽  
D. B. McElhinney ◽  
S. D. Colan ◽  
K. Gauvreau ◽  
P. J. del Nido ◽  
...  
Keyword(s):  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Predictors Of Outcome ◽  
Valve Size

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

2021 ◽  
Author(s):  
Aline Wolter ◽  
Natalia Markert ◽  
Jan Sebastian Wolter ◽  
Andrii Kurkevych ◽  
Jan Degenhardt ◽  
...  
Keyword(s):  
Pulmonary Stenosis ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Intention To Treat ◽  
Outcome Group ◽  
History Of ◽  
Postnatal Outcome ◽  
Critical Pulmonary Stenosis

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.

Chromosome 22q11 deletion in a patient with pulmonary atresia, intact ventricular septum, and confluent branch pulmonary arteries

2017 ◽  
Vol 28 (3) ◽  
pp. 467-470 ◽  
Author(s):  
Varun Aggarwal ◽  
Michaki Imamura ◽  
Carlos Acuna ◽  
Antonio G. Cabrera
Keyword(s):  
Pulmonary Stenosis ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
22Q11.2 Deletion Syndrome ◽  
Ventricular Septum ◽  
Deletion Syndrome ◽  
Intact Ventricular Septum ◽  
Chromosome 22Q11 ◽  
Chromosome 22Q11.2 ◽  
22Q11.2 Microdeletion

AbstractIn this study, we report a patient with pulmonary atresia with intact ventricular septum (PA/IVS), confluent pulmonary arteries supplied by an arterial duct, and chromosome 22q11.2 microdeletion. The 22q11.2 deletion syndrome has been associated with anomalies of the outflow tracts, such as tetralogy of Fallot with either pulmonary stenosis or atresia, but we are aware of a solitary case described with pulmonary atresia when the ventricular septum is intact. The presence of genetic malformations can have long-term co-morbidities. By describing our patient, we aim to create awareness of this rare association.

scholarly journals Pulmonary atresia, intact ventricular septum, and Ebstein anomaly of the tricuspid valve

1993 ◽  
Vol 106 (2) ◽  
pp. 255-261 ◽  
Author(s):  
Giovanni Stellin ◽  
Francesco Santini ◽  
Gaetano Thiene ◽  
Uberto Bortolotti ◽  
Luciano Daliento ◽  
...  
Keyword(s):  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Ebstein Anomaly
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