Congenital Heart Disease - AHF 2 PDF

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