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Case #1 Left Heart Obstruction Congenital Heart Disease for the Adult Echocardiographer From Diagnosis to Repair Cheryl Cammock MD Case #1 Left Heart Obstruction Shone’s Complex with Severe Coarctation Aorta 8 yo with poor lower extremity pulses, hypertension and leg pain. Cardiology consultation obtained because abdominal ultrasound suggestive of coarctation of the aorta Case #1 Left Heart Obstruction Shone’s Complex Parashute Mitral Valve Shone's complexsupra valvar mitral ring parachute mitral valve bicuspid aortic valve coarctation of the aorta and LVH Subaortic obstruction D. Mayer and C. Mullins, Congenital Heart Disease, A Diagrammatic Atlas. New York: Alan R. Liss, Inc, 1988. p. 5. Case #1 Shone's Coarctation Aorta Case #1 Left Heart Obstruction Shone’s Complex- Treatment Aortic arch repair (extended end to end anastomosis) 4m post op aortic arch gradient 56mmHg No associated systemic hypertension, blood pressure gradient, LVH Mitral valve replacement Case # 2 Left Heart Obstruction Shone's Left Atrial Hypertension 3 year old male presented at age 7m with recurrent cough and respiratory difficulty possible pneumonia, poor growth and cardiomegaly on chest x-ray Case #2 Left Heart Obstruction Shone's Left atrial hypertension Mitral stenosis Coarctation Large VSD L—R shunt. Intact atrial septum PDA L – R shunt Elevated LA pressure Subaortic membrane Case #2 Left Heart Obstruction Shone's Left Atrial Hypertension Severe mitral stenosis Case #2 Left Heart Obstruction Shone's Severe Mitral stenosis Initial Intervention: Atrial septectomy sub AS resection Arch repair, end to end anastomosis Pulmonary artery band Case #2 Shone's Severe Mitral stenosis Pre-op TEE Post op TEE Case #2 Left Heart Obstruction Shone's Severe Mitral stenosis Subsequent surgeries: ASD and VSD closure Pulmonary artery reconstruction and takedown of band Mitral valve replacement (age 10m) Case #1&2 Left Heart Obstruction Shone’s Complex Echo Summary Severe coarctation aorta narrow aortic isthmus continuous flow descending aorta LVH parachute valve – (PSS,PSL,4C) all MV chordae attach to single papillary muscle supravalvar mitral ring – (PSS,PSL,4C) membrane and anywhere between LA appendage and MV, may attach to MV Case #2 Shone's Mitral Stenosis, Valve Replacement Manufacturers Guidelines Prosthetic MV pressure gradient TEE Carpentier Edwards St Jude Case #1&2 Left Heart Obstruction Shone’s Complex Echo Summary Coarctation re-stenosis or residual arch obstruction Progressive mitral valve stenosis LA enlargement Pulmonary vein dilation , inc. a wave Progressive aortic valve stenosis or insufficiency bicuspid valve Aortic valve area (continuity equation, direct planimetry) acceleration time, ejection time pressure gradient parallel to flow (high right parasternal apical, notch) Case #3 Newborn Tetralogy of Fallot Case #3 Newborn Tetralogy of Fallot Tetralogy of Fallot with severe valvar and branch pulmonary stenosis Premature baby boy with cyanosis at birth and murmur Initial intervention: Prostaglandin, then aorta to pulmonary shunt (1 modified BT shunt) D. Mayer and C. Mullins, Congenital Heart Disease, A Diagrammatic Atlas. New York: Alan R. Liss, Inc, 1988. p. 5. Case #3 Newborn Tetralogy of Fallot Case #3 Newborn Tetralogy of Fallot Transthoracic Case #3 Newborn Tetralogy of Fallot TEE Case #3 Newborn Tetralogy of Fallot Subsequent intervention age 3m Transannular patch VSD closure Subsequent problems/ intervention Persistent pulmonary hypertension Right pulmonary artery stenosis, RPA stent Severe pulmonary valve insufficiency Right ventricular dysfunction Pulmonary valve placement ,age 2yr Case #4 Adult Repaired Tetralogy of Fallot 34 y.o. with hx Tetralogy of Fallot complete repair at age 4y. Recent pregnancy c/o edema, orthopnea prior to delivery. Frequent PVC on Holter monitor PSHx: Waterston shunt age 6 months Transannular patch and VSD patch Patch repair of a residual VSD at age 5 Pulmonary valve placement at age 34y Tetrology of Fallot Waterston Shunt Case #4 Adult “Repaired”Tetralogy of Fallot Case #4 Adult “Repaired” Tetrology of Fallot Echo findings Pulmonary insufficieny Dilated right atrium Dilated right ventricle Mild depressed right ventricle function Paradoxical ventricular septal wall motion No residual VSD Case #4 Tetrology of Fallot Pulmonary Valve Replacement Case # 5 Double Outlet Right Ventricle with Malposition of Great Vessels Case # 5 Double Outlet Right Ventricle with dmalposition of Great Vessels Sub Pulmonary VSD Both great vessels (GV)arise at least 50%from RV d-malposed GV Sub –pulmonary VSD Bicuspid dysplastic pulmonary valve (unique) Mild Pulmonary stenosis Persistent LSVC 1m old infant boy with failure to thrive respiratory distress cyanosis. PE: holosystolic murmur LLSD radiating to LUSB and posterior Chest x-ray pulmonary edema Taussig – Bing Anomaly D. Mayer and Mullins, C. Congenital Heart Disease, A Diagrammatic Atlas. New York: Alan R. Liss, Inc, 1988 Case #5 Double Outlet Right Ventricle (DORV) Case #5 DORV with d-Malposition Great Vessel Bicuspid pulmonary valve Case #5 DORV with d-Malposition Great Vessel PA overrides VSD Case #5 Repair DORV d-Malposed vessels Arterial Switch VSD Patch 1 native aortic root 2 native pulmonary root (bi-comissural) VSD patch communicates native pulmonary root to LV Persistent left SVC – high parasternal image D. Mayer and Mullins, C. Congenital Heart Disease, A Diagrammatic Atlas. New York: Alan R. Liss, Inc, 1988 DORV d-Malposed Great Vessel Repair VSD Patch to PA and Arterial Switch Case # 5 DORV D-Malposed Vessels s/p Arterial Switch VSD Patch Post operative TEE Mild Pulmonary valve insufficiency Normal aortic valve function Case # 5 DORV D-Malposed Vessels Echo Summary Case # 5 DORV D-Malposed Vessels Echo Summary Important diagnostic features Great vessel relationship (subcostsal parasternal sweeps) VSD location and relationship to great vessel (subcostsal parasternal sweeps,4c) Outflow tract obstruction from conus Important Follow up Residual shunt (color flow doppler, all views) Semilunar valve function Outflow tract obstruction (color flow,PW &CW doppler) (subcostsal parasternal sweeps) Semilunar valve function and anatomy Persistent LSVC (high parasternal) Case # 6 Single Ventricle 26 year old female with singleton pregnancy, no significant prior medical family history referred for fetal cardiac ultrasound at 23 weeks gestation because of disproportionate ventricle chamber size Case 6 Fetal Diagnosis Congenital Heart Defect 26 year old female with singleton pregnancy, no significant prior medical family history referred for fetal cardiac ultrasound at 23 weeks gestation because of suspected congenital defect.VSD found on screening fetal ultrasound Case #6 Fetal Diagnosis Congenital Heart Defect Case #6 Fetal Diagnosis Congenital Heart Defect – Type I Truncus Truncus Arteriosus Anatomical features Truncal Valve Fetal Single semilunar valve and that supplies systemic and pulmonary circulation Truncal valve usually abnormal (unicuspid to quadracuspid) Ventricular septal defect Pulmonary arteries arise from short main stump See live presentation D. Mayer and Mullins, C. Congenital Heart Disease, A Diagrammatic Atlas. New York: Alan R. Liss, Inc, 1988 Truncal valve postnatal Case #6 Fetal Diagnosis Congenital Heart Defect – Type I Truncus Case #6 Fetal Diagnosis Congenital Heart Defect – Type I Truncus Post repair VSD patch and Right Ventricle to pulmonary artery conduit, Truncal valve repair Case #6 Fetal Diagnosis Congenital Heart Defect – Type I Truncus Post operative findings Mild conduit insufficiency Mild Neo-aorta (previous truncal valve) insufficiency Case # 7 Congenital Corrected Transposition & Cor Triatriatum rvRV rvLV R R Left sided morphologic right ventricle Core triatriatum Obstructed pulmonary vein return Bilateral SVC Normal systemic ventricle function Case # 7 Congenital Corrected Transposition & Cor Triatriatum 22 yo female history chronic recurrent syncope, and SOB with exercise PE: O2 sats 99% on RA, normal HR, rhythm Grade I SEM RUSB 12 lead EKG left axis deviation, Q wave in anterior and inferior leads, lack of septal Q posterior Case # 7 Congenital Corrected Transposition & Cor Triatriatum Use Segmental Analysis to diagnose complex congenital heart defects Examine heart by 3 regions and 2 parts according to morphology and location 1. Atria -right versus left Ex: left sided anatomical left atrium 2. Ventricles - right versus left Ex: left sided anatomical right ventricle 3. Great vessels - right versus left Modified from original D. Mayer and Mullins, C. Congenital Heart Disease, A Diagrammatic Atlas. New York: Alan R. Liss, Inc, 1988 Ex: Aorta valve arises left and anterior or from the left sided ventricle Case # 7 Congenital Corrected Transposition Cortriatriatum Ra Cardiac MRI: atrial situs solitus (S), left sided morphologic right ventricle (Lloop), aorta left and anterior (L- transposition) intact atrial septum, left SVC in the medial border of the cortriatriatum membrane, no bridging vein left to right LSVC Case #7 Congenital Corrected Transposition LV La RV Case #7 Congenital Corrected Transposition, Cortriatriatum Echo Features Right Case #7 Congenital Corrected Transposition, Cortriatriatum Pre-Op TEE Cortriatriatum left Pulmonary veins drain to vein confluence Left atrium partitioned into 2 sections, superior inferior Flow acceleration through the core No atrial communcation Case #7 Congenital Corrected Transposition Cor-triatriatum PreOp TEE Bubble Contrast Post Resection of Cor triatriatum membrane LSVC remains intact Case #7 Echo Summary Core triatriatum vs supravalvar mitral ring Cortriatriatum membrane higher in left atrium superior to LAA LA partitioned into 2 chambers (proximal and distal where PV enter) Degree of obstruction may be underestimated if ASD decompresses distal chamber Case # 8 Heterotaxy Syndrome Now 3 year old with history of cyanosis at 10hrs of life Chest x-ray with dextrocardia (apex to right, OG tube right ward, leftward umbilical vein catheter, bilateral pneumothorax Pneumothorax relieved Persistent cyanosis : Prostaglandin started and cardiac consultation obtained Case # 8 Heterotaxy Syndrome Dx: Dextrocardia, Situs Inversus, Pulmonary Atresia, Inlet VSD PMH: Plastic Bronchitis PSH: 5 days - Aorta to pulmonary artery shunt 5 m– Glenn (cavapulmonary anastomosis), Pulmonary artery patch 3 y – Fenestrated Lateral Tunnel Fontan Case #8Case #8 Cardiac Position Case # 8 Heterotaxy Syndrome Dextrocardia Abdominal Situs Case #8 Heterotaxy L-loop ventricle &VSD La LV left Right Ra RV Case #8 Heterotaxy Pulmonary Atresia La LV Case #8 Heterotaxy Pulmonary Atresia Ra RV 3m: Shunt from Innominate artery to pulmonary artery supplies PA flow 3m:Shunt from Innominate artery to pulmonary artery supplies PA flow Case #8 Heterotaxy Pulmonary Atresia 5m : left sided caval pulmonary anatomosis Case #8 Heterotaxy Pulmonary Atresia 3yr Left sided Lateral Tunnel Fenestrated Fontan Case # 8 Echo Summary Imaging situs abnormalities Start subcostal (if window available) Take segmental approach to determine abdominal situs, atrial situs, ventricular looping, great vessel relationship Case # 8 Echo Summary Caval pulmonary anatomosis doppler profile should be continuous low velocity flow Aorta to pulmonary shunts ( PDA, surgical central shunts) have more pulsatile flow
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