Aortic perfusion score for pulmonary atresia with intact ventricular septum: An antegrade coronary perfusion scoring system that is predictive of need for transplant and mortality

2017 ◽  
Vol 13 (1) ◽  
pp. 92-97
Author(s):  
Rohit S. Loomba ◽  
Andrew N. Pelech
Keyword(s):  
Scoring System ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Coronary Perfusion ◽  
Intact Ventricular Septum ◽  
Aortic Perfusion

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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