Which of the following conditions is worsened by prostaglandin E infusion?
Correct Answer: Obstructive TAPVC
Description: Ans. c.'Obstructive TAPVC { Rc/: Ll lllohttn Retlth,. Cunliut. Srr ('unliut surgen''4,,uar oJ rht' se,tinurs in Trnrucic and Cardiovascular Surgery 2003;4:271-276)Obstructive TAPVC is worsened by prostaglandin E infusionln infants with or who have a clinical suspicion for a ductal dependent congenital hea defect, prostaglandin E, should be administered until a definitive diagnosis or treatment is established.TAPVC Total anomalous pulmonary venous connection (TAPVC) is characterized by abnormal drainage of pulmonary veins into the right hea either by direct connection into the right atrium or into its tributaries.According to the site or level of connection of the pulmonary veins to the systemic venous system TAPVC has been classified into four types :-Type I (most common: 45%) : Anomalous connection at supracardiac level (PV drains into left innominate vein or SVC)Type II (25%) : Anomalous connection at cardiac level (PV joins the coronary sinus or enter right atrium directly).Type III (25%) : Anomalous connection at infracardiac level (PV drain into poal vein).Type IV (5%) : Anomalous connection at multiple levels.Infracardiac type of TAPVC is always obstructive whereas cardiac and supracardiac type may be obstructive or nonobstructive.X-ray findings of TAPVCCardiomegalyPlethoric lung fieldsSnowman or figure of '8' configuration - In supracardiac TAPVC.Ground glass appearance of lung - In obstructive TAPVC.Clinical manifestations of TAPVCNonobstructive TAPVC - Patients presents with mild cyanosis and CHF at 6-8 weeks.Obstructive TAPVC - Paients presents with severe cyanosis and CHF within first week.In supracardiac TAPVC the pulmonary veins join to form a single trunk (common pulmonary vein) which than drain through anomalous connection
Category:
Pediatrics
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