scholarly journals P04.02: Characteristics and outcomes in fetuses with Ebstein's anomaly or pulmonary atresia with intact ventricular septum

2011 ◽  
Vol 38 (S1) ◽  
pp. 178-178
Author(s):  
H. Lee ◽  
H. Won ◽  
K. Lee ◽  
M. Lee ◽  
J. Shim ◽  
...  
Keyword(s):  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Ebstein’S Anomaly ◽  
Intact Ventricular Septum ◽  
Ebstein's Anomaly

Comparison of the offset distance of the tricuspid septal leaflet in neonates with Ebstein’s anomaly and neonates with pulmonary atresia with intact ventricular septum

2014 ◽  
Vol 25 (1) ◽  
pp. 129-136 ◽  
Author(s):  
Min Jeong Kim ◽  
Jeong Jin Yu ◽  
So Yeon Kang ◽  
Chang Deok Seo ◽  
Jae Suk Baek ◽  
...  
Keyword(s):  
Body Surface ◽  
Pulmonary Atresia ◽  
The Body ◽  
Ventricular Septum ◽  
Ebstein’S Anomaly ◽  
Intact Ventricular Septum ◽  
Quantitative Criterion ◽  
Ebstein's Anomaly ◽  
Offset Distance ◽  
Offline Analysis

AbstractAn indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m2is a quantitative criterion for the diagnosis of Ebstein’s anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebstein’s anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebstein’s anomaly and 66 with pulmonary atresia with intact ventricular septum, were enrolled. Diagnosis of each anomaly was based on typical morphologic features. Echocardiographic variables, including the offset distance of the tricuspid septal leaflet, were measured via an offline analysis of images recorded before 1 month of age. The offset distance of the tricuspid septal leaflet was indexed by the body surface area, and the indexed offset distances in the Ebstein’s anomaly and pulmonary atresia with intact ventricular septum groups were 34.2 mm/m2(7.1–119.1 mm/m2) and 7.2 mm/m2(0.0–25.6 mm/m2), respectively. The indexed offset distance was ⩾8 mm/m2in 29 (43.9%) of the patients with pulmonary atresia with intact ventricular septum; clinical and echocardiographic characteristics were comparable between these 29 patients and the remaining 37 patients with pulmonary atresia with intact ventricular septum. When an indexed offset distance ⩾8 mm/m2was applied as a cut-off for the diagnosis of Ebstein’s anomaly, the sensitivity was 0.963 and the specificity was 0.561. In conclusion, indexed offset distance ⩾8 mm/m2cannot be used as a cut-off for the diagnosis of complicated Ebstein’s anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

Point ablation for macro-reentrant ventricular tachycardia associated with Ebstein’s anomaly and pulmonary atresia: a case report

2020 ◽  
pp. 1-3
Author(s):  
Keiko Toyohara ◽  
Yasuko Tomizawa ◽  
Morio Shoda
Keyword(s):  
Ventricular Tachycardia ◽  
Case Report ◽  
Catheter Ablation ◽  
Right Ventricle ◽  
Low Voltage ◽  
Pulmonary Atresia ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Slow Conduction ◽  
Hypoplastic Right Ventricle

Abstract We report a case with Ebstein’s anomaly and pulmonary atresia with sustained monomorphic ventricular tachycardia in a patient without a ventriculotomy history. In the low voltage area between the atrialised right ventricle and hypoplastic right ventricle, there was a ventricular tachycardia substrate and slow conduction. The tachycardia circuit was eliminated by a point catheter ablation at the area with diastolic fractionated potentials.

Survival With Respect to Morphology in Pulmonary Atresia and Intact Ventricular Septum in Sweden

2021 ◽  
Vol 12 (1) ◽  
pp. 27-34
Author(s):  
Stina Manhem ◽  
Katarina Hanséus ◽  
Håkan Berggren ◽  
Britt-Marie Ekman-Joelsson
Keyword(s):  
Pulmonary Atresia ◽  
Early Time ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Early Group ◽  
Time Period ◽  
Medical Reports ◽  
One Year ◽  
Survival Risk ◽  
Late Group

Background: Patients born with pulmonary atresia and intact ventricular septum represent a challenge to pediatric cardiologists. Our objective was to study changes in survival with respect to morphology in all children born with pulmonary atresia and intact ventricular septum in Sweden during 36 years. Methods: A retrospective, descriptive study based on medical reports and echocardiographic examinations consisting of those born between 1980 and 1998 (early group) and those born between 1999 and 2016 (late group). Results: The cohort consists of 171 patients (early group, n = 86 and late group, n = 85) yielding an incidence of 4.35 and 4.46 per 100,000 live births, respectively. One-year survival in the early group was 76% compared to 92% in the late group ( P = .0004). For patients with membranous atresia, one-year survival increased from 78% to 98%, and for muscular pulmonary atresia, from 68% to 85%. In patients with muscular pulmonary atresia and ventriculocoronary arterial communications, there was no significant increase in survival. Risk factors for death were being born in the early time period hazard ratio (HR), 6; 95% CI (2.33-14.28) P = .0002, low birth weight HR, 1.26; 95% CI (1.14-1.4) P < .0001 and having muscular pulmonary atresia HR, 3.74; 95% CI (1.71-8.19) P = .0010. Conclusion: The incidence of pulmonary atresia and intact ventricular septum remained unchanged during the study period. Survival has improved, especially for patients with membranous pulmonary atresia, while being born with muscular pulmonary atresia is still a risk factor for death. To further improve survival, greater focus on patients with muscular pulmonary atresia and ventriculocoronary arterial communications is required.

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