Congenital Heart Disease - AHF 2 PDF

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Arizona Heart Foundation

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congenital heart disease atrial septal defects cardiology heart anatomy

Summary

This document presents an overview of congenital heart disease, focusing on atrial septal defects (ASDs). It details different types of ASDs, associated lesions, and the pathophysiology behind them. Diagrams illustrate the anatomical aspects. This is a learning resource rather than a quiz.

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Congenital Heart Disease Atrial Septal Defects & Associated Lesions Atrial Septal Defects: Abnormal opening in the interatrial septum Ostium secundum - 70% ○ Located in the mid portion of the interatrial septum Associated with hemodynamic mitral valve...

Congenital Heart Disease Atrial Septal Defects & Associated Lesions Atrial Septal Defects: Abnormal opening in the interatrial septum Ostium secundum - 70% ○ Located in the mid portion of the interatrial septum Associated with hemodynamic mitral valve prolapse More prevalent in women Ostium primum - 20% ○ Located at the inferior portion of the interatrial septum Associated with cleft mitral valve Sinus venosus - Superior and inferior vena caval - 10% ○ Located posterior and superior portion of the interatrial septum near SVC or inferior and posterior near the IVC Associated with a (partial) total anomalous pulmonary venous return Usually a single vein is anomalous - returning blood to RA instead of LA Ostium secundum ASD vs PFO Ostium primum plus inlet VSD - Common AV Primum ASD without VSD canal Types continued Coronary Sinus (rare) ○ Roof of coronary sinus is partially or completely absent Creates shunt between both atria and the coronary sinus Dilated coronary sinus can affect mitral valve Common atrium ○ Absence of interatrial septum ○ Associated with situs abnormalities Shunts ○ Predominantly left to right with brief reversal during atrial relaxation Sinus venosus ASD Coronary sinus ASD with dilated coronary sinus Coronary sinus ASD Etiology: Defects occur in 1:1000 PFO found in 25% of population Primum ASD ○ Failure of septum primum to fuse with ○ Located in fossa ovalis region endocardial septation of the AV canal above Primum and secundum make up 67% of all the level of the AV valves ASDs ○ Located between inferior septum primum and Secundum ASD AV valves ○ Mostly sporadic causes Coronary sinus ○ MOST COMMON ○ Rare ○ Complete or partial “unroofing” of coronary sinus ○ Associated with persistent left superior vena cava drainage to the coronary sinus Common atrium Pathophysiology Pathophysiology Interatrial communication caused by atrial septal defect Volume/pressure overload ○ Initially left to right shunting - RV compliance is less than LV compliance ○ Prolonged large volume shunting can result in right to left shunting with Clinical presentation ○ May be asymptomatic for many years - mid to late adult life elevated right sided pressures Eisenmenger’s physiology - rare with ASDs (when prolonged large volume shunting results ○ Dyspnea upon exertion in right to left flow of the shunt with elevated right sided pressures) ○ Recurrent respiratory infections Conditions that increase left to right shunting through ASD ○ Atrial arrhythmias - especially afib ○ LVH ○ Systolic murmur secondary to increased flow across PV ○ Cardiomyopathy Relative PV stenosis ○ Large shunts may have diastolic murmur through tricuspid valve ○ Mitral stenosis/regurgitation ○ Mitral valve atresia (malformation of mitral valve) ○ Cor triatriatum sinister (LA) ○ Aortic stenosis ○ Coarctation of the aorta Associated Lesions Secundum ASD and PFO Sinus venosus defect ○ Mitral valve prolapse ○ Anomalous pulmonary vein return ○ Tricuspid atresia Total/partial Malformation or missing ○ Right upper and lower pulmonary veins to SVC/RA/LA Primum ASD ○ Cleft mitral valve Slit-like hole in a leaflet ○ Atrioventricular valvular regurgitation TV atresia Hypoplastic left heart syndrome - HPLS Cleft MV Echocardiographic features of ASD 2D/M-mode ○ Location and size of defect >10 mm is considered larger, < 20 mm ideal for closure, > 25 mm is difficult ○ RV function Dilatation RVOT diameter - normal 1.7-2.3 cm distal Volume/pressure overload - paradoxical septal motion/flattening ○ Right/Left atrial dilatation LAE associated with left heart failure, cleft MV or MVP ○ Pulmonary artery/branch dilatation ○ Tricuspid/pulmonic valve annulus dilated over time Tricuspid annulus - RV basal measurement > 42 mm ○ Partial anomalous venous return Total anomalous pulmonary vein return Doppler Color/PW ○ Demonstrate direction/velocity of shunt ○ Turbulent flow in pulmonary artery due to increased volume ○ Presence and severity of valvular regurgitation/stenosis ○ Presence of partial/ total anomalous venous return ○ RVOT VTI - increased with large ASD, LVOT VTI - decreased with large ASD Higher blood volume = higher VTI Lower blood volume = lower VTI ○ Qp/Qs - hemodynamically significant ≥ 1.5:1 Ratio of pulmonary to systemic flow ○ Determine SPAP/MPAP/PAEDP/PVR Review from Doppler ○ Width of ASD with color doppler ○ Left atrial pressure Partial anomalous pulmonary vein return ○ Right/left ventricular function - diastolic/systolic Qp/Qs - Pulmonary-Systemic flow ratio - ASD/VSD Refer to formula for Qp/Qs on the VSD powerpoint. QpQs Left Atrial Pressures The Qp/Qs ratio can be assessed by echo. It With the presence of PFO/ASD ○ LAP mmHg = 4(peak PFO/ASD velocity2) + RAP is the principal way of quantifying the degree of a shunt across a defect. It also provides precise indications as to whether and when the defect should be closed. Imaging techniques Bubble study Subcostal imaging is usually Detect PFO and ASD optimal for evaluation ○ Temporarily increases pressures on right Pediatric high right side through saline injection to force a R to L shunt, the bubbles should appear on parasternal/supraclavicular the left side of the heart within the first view may be attempted 3 to 4 cardiac cycles. ○ Presences of partial Evaluation for persistent SVC anomalous pulmonary venous return to SVC Late appearance may indicate Crab view intrapulmonary shunting ○ Short axis of SSN for pulmonary vein connection Bubble Study Diagnose Echo https://www.youtube.com/watch?v=sYM1HYFpyXA https://www.youtube.com/watch?v=mbxqvfZRh3Q Transesophageal echo Evaluate type of defect ○ Helpful for PFO, sinus venosus, partial anomalous pulmonary venous return (PAPVR), cleft mitral valve Evaluate for associated lesions/malformations Monitor intraprocedural progress/guidance for device closure ○ Confirm lack of residual shunting ○ Follow up post procedure Device impingement on surrounding structures Residual shunting If sinus venosus ASD repair evaluate for SVC or pulmonary venous channel obstruction Subcostal/RPS views Persistent SVC Treatment Cleft MV Catheterization Antiarrhythmics Device closure ○ PFO/Secundum ASD Mitral valve repair/replacement (cleft MV) Surgical ○ Primary (suture) closure for small defects ○ Patch repair Video Amplatzer Primum ASD Secundum ASD Sinus venosus

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