Pulmonary atresia natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2], Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [4]
Prognosis
- The outcome varies for every child. Studies has shown that the survival rate mainly is dependant on the degree of the right ventricular outflow tract obstruction and extent of the right ventricular dilatation/hypertrophy.[1]
- Right Ventricular hypertrophy can be a key predictor of fetal mortality and neonatal outcome. The retrospective studies have shown right ventricular hypertrophy in 50 % of fetuses who died during the neonatal period compared to those who survived it.[1]
- If the condition is left uncorrected it is fatal, but the prognosis has greatly improved over the years for babies with pulmonary atresia due to advancement in medicine. Most cases of pulmonary atresia can be helped with surgery. If the patient's right ventricle is exceptionally small, many surgeries will be needed in order to help stimulate normal circulation of blood to the heart.
- If uncorrected, babies with this type of congenital heart disease will only survive for the first few days of life while the fetal shunts between left and right circulations remain patent. It is recommended to screen the fetuses at the second trimester via Echocardiographic ultrasound and those with positive findings should only be delivered at the tertiary care setup to minimize the poor outcome.[2]
- The long term prognosis of corrected defect is excellent and the children will go on to lead relatively normal, uncomplicated healthy. Children with pulmonary atresia will need to be seen regularly throughout their lifetime by a cardiologist to be sure that their heart is functioning properly. Due to their increased risk of endocarditis, children diagnosed with pulmonary atresia should have amoxicillin before any type of dental work or other types of surgeries to help prevent this infection from occurring.[3]
References
- ↑ 1.0 1.1 Todros T, Paladini D, Chiappa E, Russo MG, Gaglioti P, Pacileo G; et al. (2003). "Pulmonary stenosis and atresia with intact ventricular septum during prenatal life". Ultrasound Obstet Gynecol. 21 (3): 228–33. doi:10.1002/uog.63. PMID 12666215.
- ↑ Shinebourne EA, Rigby ML, Carvalho JS (2008). "Pulmonary atresia with intact ventricular septum: from fetus to adult: congenital heart disease". Heart. 94 (10): 1350–7. doi:10.1136/hrt.2006.108936. PMID 18801793.
- ↑ Day MD, Gauvreau K, Shulman S, Newburger JW (2009). "Characteristics of children hospitalized with infective endocarditis". Circulation. 119 (6): 865–70. doi:10.1161/CIRCULATIONAHA.108.798751. PMID 19188508.
- ↑ Garrocho-Rangel A, Echavarría-García AC, Rosales-Bérber MA, Flores-Velázquez J, Pozos-Guillén A (2017). "Dental management of pediatric patients affected by pulmonary atresia with ventricular septal defect: A scoping review". Med Oral Patol Oral Cir Bucal. 22 (4): e458–e466. doi:10.4317/medoral.21864. PMC 5549532. PMID 28624838.