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Anomalies of Pulmonary Arteries and Veins NASCI 37th Annual Meeting Pediatric/Congenital Heart Disease Boot Camp Himesh Vyas, MD Assistant Professor of Cardiology University of Arkansas for Medical Sciences Arkansas Children's Hospital, Little Rock, AR • No financial disclosures • Off label use of gadolinium in children Learning Objectives z Recognize common anomalies of: z Pulmonary arteries z Pulmonary veins z Describe utility of cardiac MRI in evaluation Pulmonary Artery Anomalies Pulmonary Artery Anomalies PA anomalies Anomalies of number Anomalies of location Anomalies of size Anomalies of Number Unilaterally Absent PA z Associated with congenital heart disease: z z Normal LPA L R Absent LPA with tetralogy of Fallot Distal pulmonary artery often normally distributed Ao LA Absent RPA Ductal Origin of Branch PA CTA RPA from MPA Ductal origin of LPA PDA LPA Anomalies of Location Origin of LPA from RPA (PA sling) MPA Ao RPA Trachea LPA Fiore A. C. et al.; Ann Thorac Surg 2005;79:38-46 Aberrant LPA posterior to trachea Origin of LPA from RPA (PA sling) z Airway anomalies common z Complete membranous rings z Tracheomalacia z Abnormal lung lobulation z Bronchus suis CTA Anomalies of Location Aortic Origin of Branch PA Branch pulmonary artery origin from the dorsal ascending aorta z Right >> left z Associations: z z z z Tetralogy of Fallot Patent ductus arteriosus “Hemitruncus” Asc. Ao MPA RPA LPA Aortic Origin of RPA CTA MPA LPA Aorta RPA LA Anomalies of Size Branch PA Stenosis/Hypoplasia z z Isolated Associated congenital heart disease z z z Tetralogy of Fallot Congenital rubella Williams syndrome z z RPA Ao LPA Supravalvar Branch PA Bilateral branch PA stenosis – Williams syndrome Pulmonary Atresia with Ventricular Septal Defect (PA-VSD) z Intra-cardiac anatomy similar to tetralogy of Fallot z Pulmonary blood supply variable z PDA z Major aortopulmonary collaterals PA-VSD: Goals of MRI • Sources of pulmonary blood flow • PA size and confluence • Collateral communication with PAs R L R L PDA Confluent PAs Collateral Pulmonary Vein Anomalies Pulmonary Vein Embryology LUNG BUDS RA LA SPLANCHNIC VEINS PULMONARY XXVEINS LA RA RV LV DEVELOPING HEART z z z Lung buds from foregut – splanchnic drainage Left atrium connects to PV Regression of primitive splanchnic connections Classification of PV Anomalies Totally anomalous pulmonary venous connection (TAPVC) z Partially anomalous pulmonary venous connection (PAPVC) z Sinus venosus defect z Cor triatriatum z Pulmonary vein stenosis/atresia z TAPVC z Types: LPA Aorta z Supracardiac z Cardiac RPA z Infracardiac z Mixed Lower PVs Confluence of PV draining into posterior right atrial wall PAPVC Common types: z Left PV to innominate vein z Right PV to SVC z With sinus venosus defect z Without sinus venosus defect z Right PV to IVC (scimitar syndrome) Left PV to Innominate Vein Innominate vein Ascending vertical vein LPA RPA LA Normal left lower PV Scimitar syndrome z z z z Shadow on X-ray resembles Turkish sword (scimitar) Hypoplasia of right lung Pseudo-sequestration of right lower lobe of lung Aortopulmonary collaterals Mesoposed heart, hypoplastic right lung and scimitar sign (arrows) Scimitar Syndrome RPA LPA Dextroposed heart, large LPA, small RPA Descending anomalous vein (arrow) Sinus Venosus Defect Unroofing of right upper PV to SVC z Defect is dilated orifice of right upper pulmonary vein SVC z PAPVC of right PV to SVC (blue arrow) z RA LA Cor Triatriatum Membrane in LA (black arrow) z Proximal PV “chamber” z Stenotic connection to LA z RV RA LV Pulmonary LA venous chamber Unilateral PV Atresia No perfusion to left lung Time resolved Gd-MRA Atretic left PV Post-Operative Problems Limitations of Echocardiography Branch pulmonary arteries by 2D echo Post-operative deterioration of acoustic windows! Post-Operative PA Stenosis RPA RPA LPA conduit LPA conduit Severe stenosis of RPA after repair of truncus arteriosus Post-Operative PV Stenosis/Atresia May follow pulmonary vein surgery z Progressive z Focal or diffuse z LPA RPA Bilateral PV stenosis Warden Procedure for Repair of Sinus Venosus Defect SVC to RA appendage RPV to LA baffle Conclusion z Reviewed important anomalies of z Pulmonary arteries z Pulmonary veins z MRI particularly well suited because: z Accurate anatomical details z Gadolinium MRA z Black/bright blood imaging z Functional information Acknowledgements z ACH cardiac MRI team: z S. Bruce Greenberg z Sadaf Bhutta z Danny Meek z Bruce Stockmeyer z Raj Krishnamurthy – contributing many of the images in this talk