13/06/ Year Old Man BAV Valvotomy Age 7 Years. 47-Year Old Man. Patent Ductus Arteriosus

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1 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-6 47-Year Old Man BAV Valvotomy Age 7 Years Congenital Heart Disease Workshop The aorta will be 1. Normal 2. Mildly enlarged 3. Moderately enlarged 4. Markedly enlarged 5. No clue 47-Year Old Man BAV Valvotomy Age 7 Years 65-Year-Old Female Dyspnea Aorta = 10.8 cm What is the Diagnosis? 65-Year-Old Female with PDA PDA Device Closure Pre-procedure EDD = 70 mm EF 21% 16 months post EDD = 54 mm EF 52% Patent Ductus Arteriosus Associated with maternal rubella F:M = 3:1 Continuous machinery murmur, envelops S2 Differential diagnosis Pulmonary or CA A-V fistula AP window Ruptured sinus of Valsalva aneurysm 1

2 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-12 Indications for intervention in PDA 31-Year-Old Female with Dyspnea EDD 76 mm What is the Diagnosis? PA pressure 68/34 mmhg Mean PA pressure 46 mmhg Rpa = 5 units*m 2 TR Velocity = 3.5 m/sec 14 mm Amplatzer Vascular Plug Post PA pressure 42/17, mean 27 mmhg Pre PDA Closure EDD = 76 mm RVSP = 54 mmhg Post PDA Closure EDD = 47 mm RVSP = 34 mmhg 2

3 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-18 Echo assessment of PDA High left parasternal or suprasternal Color and CW Doppler across PDA LA and LV enlargement Secundum ASD Atrial Septal Defects Secundum and Primum Secundum ASD Most common ASD ~75% of defects Central portion of atrial septum Only ASD amenable to deviceclosure Holt-Oram syndrome Specimen (Four-Chamber View) Courtesy of WD Edwards Indications for intervention in ASD Causes of RHE Baumgartner H et al; GUCH ESC

4 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-24 Shunt ASD What else? Shunt ASD, APVC TV or PV Regurgitation RV Myopathy Complex CHD Right Heart Enlargement in CHD Primum ASD Atrial level (or above) shunt ASD, APVC Right-sided valve regurgitation TR, PR Right heart myopathy Ebstein, ARVC Systemic right ventricle cctga (l-tga) End-stage RV hypertension PH Acquired disorders OSA etc What else should we look for? 4

5 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-30 Atrioventricular Septal Defects Primum ASD Partial AV Defect Deficient AV septum; abnormal AV valves Partial Primum ASD, cleft MV Complete AVSD (ASD + VSD) Common AV valve Intermediate or transitional Partial plus inlet VSD RA LA Courtesy of Dr. WD Edwards ECG - left axis deviation, first degree AV block Associations - MV and TV cleft, VSD, LVOT obstruction Partial AV Septal Defect Cleft Mitral Valve 3-D Imaging in CHD Cleft Mitral Valve Partial AV Septal Defect Double Orifice Mitral Valve 44% Down patients have CHD 5

6 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide Year-Old Man Asymptomatic What is the diagnosis? What is the diagnosis? Transitional AV septal defect MV regurgitation, VSD 6

7 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide Year-Old Woman Dyspnea and Palpitations Question Which one of the following is the least likely to cause these echo findings? Anomalous Left-sided Pulmonary Veins 1. Pulmonary stenosis 2. Scimitar syndrome 3. Sinus venosus ASD 4. Anomalous pulmonary veins Anomalous Left-sided Pulmonary Veins Complete AVSD 7

8 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-48 What is this? Membranous VSD Membranous VSD Membranous VSD Most common type in adults Adjacent to aortic and tricuspid valves TV leaflet may become aneurysmal and close defect AV cusp prolapse can lead to AR PSSAX and 5 chamber view Associations - IE, AV and TV regurgitation Management depends on size and associations Perimembranous VSD on Echo Parasternal Short Axis Perimembranous What is this? 8

9 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-54 Subarterial VSD (Outlet, Supracristal) Outlet septum Adjacent to both semilunar valves AR due to prolapse of the AV cusps PSSAX and PSLAX Outlet VSD Infundibular/Supracristal/Doubly Committed Associations AV regurgitation often progressive Management surgery to prevent worsening AV regurg Trabecular Outlet VSD on Echo Outlet or perimembranous Outlet Indications for intervention in VSD Baumgartner H et al; GUCH ESC Year-Old Man Murmur early in life Diagnosis VSD no prior intervention Recent change in murmur Paroxysmal atrial fibrillation, PPM Recent dyspnea EDD = 67 mm 9

10 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-60 LVOT Vel = 1.7 m/s TVI = 39 cm AV Vel = 2.7 m/s MG = 16 TVI = 56 cm 10

11 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-66 What is causing the continuous murmur? 1. VSD and AR 2. Ruptured sinus of Valsalva 3. PDA 4. No clue What do you recommend? 1. Surgical intervention 2. Device intervention 3. Observation 4. No clue What type of VSD did this patient most likely have? 1. Membranous 2. Muscular 3. Inlet 4. Outlet 5. No clue 11

12 2018 MFMER slide MFMER slide MFMER slide MFMER slide-72 Ventricular Septal Defect Locations Outlet 6% (33% Asia) Membranous 80% Muscular 10% (20% infants) Inlet 4% Ruptured sinus of Valsalva (SoV) aneurysm from R cusp into the RVOT Patch repair of ruptured SoV aneurysm RA I M O T RV LV T O M I LA Outlet VSD - Supracristal, Subarterial Doubly Committed Outlet VSD Infundibular/Supracristal/Doubly Committed/Subarterial Located in outlet septum Adjacent to semilunar valves Prone to AR due to cusp prolapse Right sinus dilates Rarely sinus rupture Associations AV regurgitation often progressive Right sinus of Valsalva aneurysm and rupture Outlet Ventricular Septal Defect Dilation and Distortion of the Right Sinus Aneurysm of Sinus of Valsalva 0.1% autopsy series, males 65-80% Associated with outlet VSD Mean age 39 years Location R>NC>L Rupture related to sinus 60% R, 30% NC, 10% L Rupture location RV 60%, RA 29%, LA 6%, LV 4%, Pericard 1% Surgery rupture, unruptured large, complicated 12

13 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-78 Ruptured Sinus of Valsalva Aneurysm Often associated with outlet VSD Presents with dyspnea, heart failure and continuous murmur Look for continuous flow on TTE/TOE AV fistula low DBP and reversal desc ao Surgical repair treatment of choice Reversible cause of heart failure Other Examples Muscular VSD (trabecular) Common in children Single or multiple Remote from valves Can be closed with device 13

14 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-84 Inlet VSD Inlet VSD VSD Classification Scheme STS and European Association CT Surgery 1 2 Ao 3 4 LA 1) Outlet 6% (33% Asia) 2) Membranous 80% 3) Muscular 10% (20% infants) 4) Inlet 4% 1. Inlet ventricular septum 2. Trabecular ventricular septum 3. Outlet ventricular septum Jacobs JP et al: Ann Thorac Surg 2000 What is this? What else do we need to look for? 1. Ebstein 2. Uhl s anomaly 3. cctga 1. Cleft MV 2. Anomalous PV 3. ASD 4. Coarctation 14

15 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-90 What else do we need to look for? 1. Cleft MV 2. Anomalous PV 3. ASD 4. Coarctation Ebstein Anomaly Apical displacement septal TV leaflet Atrialized RV, small RV 50% ASD or PFO 25% accessory pathway Variable spectrum RV always abnormal Ebstein Anomaly Spiral Displacement of TV Anterior/Apical Rotation of Functional Orifice 28-Year-Old Woman Heart Murmur Modified from Schrieber C Anderson: J Thorac Cardiovasc Surg, 1999 Ebstein Anomaly Indications for repair Symptoms or exercise capacity Cyanosis - risk of stroke Severe TR - esp when repairable Right heart enlargement/cardiomegaly 28-Year-Old Female TV repair, RA and RV plication, PFO closed ACHD Guidelines 2008 Preop 6 months postop 15

16 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide Year-Old Female TV repair, RA and RV plication, PFO closed Ebstein Anomaly Pearls Repair feasible in most pt at experienced center Recognize indications for repair ASD/PFO - risk of paradoxical embolism* Rare isolated PFO closure Preop 6 months postop *Attenhofer Jost et al Congenit Heart Dis 2014 Cone Repair for Ebstein Anomaly Cone Technique Repair at True Anatomic Annulus by Leaflet Delamination Preop Postop Indications for intervention in Ebstein Question Baumgartner H et al; GUCH ESC

17 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-102 Mean Gradient = 7 mmhg (HR 57 BPM) What is the diagnosis? 1. Cor triatriatum 2. Supravalvular mitral ring 3. Anomalous PV 4. Left atrial appendage anomaly CorTriatriatum LA divided into 2 sections Proximal portion receives pulmonary veins Distal portion communicates with MV and contains LAA Membrane contains one or more fenestrations ranging from small, restrictive to large May be associated with secundum ASD, dilated coronary sinus due to persistent LSVC, and BAV What is this? Question 17

18 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-108 What is this? The echo-doppler images are most consistent with which of the following? 1. Severe TV regurgitation 2. Coarctation of aorta 3. Scimitar syndrome 4. Anomalous right upper pulmonary vein Remember sub-costal imaging! Scimitar Syndrome Scimitar Syndrome Partial anomalous pulmonary venous return from the right lung to systemic venous drainage, rather than to LA Named after the curvilinear pattern created on CXR by PV that drain to IVC Hypoplastic right lung Displacement of cardiac structures into the right hemithorax Question 18

19 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide Year-Old Male Exertional Dyspnea 71-Year-Old Male Exertional Dyspnea TR Velocity 5.2 m/sec Estimated RV systolic pressure = 120 mmhg 71-Year-Old Male Echo diagnosis? 71-Year-Old Male Exertional Dyspnea Peak Vel 5.2 m/sec Peak Grad 108 mmhg Mean gradient 64 mmhg 19

20 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide Year-Old Male Echo diagnosis? 1. Pulmonary hypertension 2. Severe tricuspid valve regurgitation 3. Pulmonary valve stenosis 4. Membranous VSD 71-Year-Old Male What would you recommend? 1. Percutaneous intervention 2. Medical therapy 3. Observation 4. Operative intervention Natural History of PS Survival into adulthood is the rule Better tolerated than AS Most adults are NYHA I-II Balloon valvotomy recommended: Asymptomatic pt, domed PV Pk Doppler grad >60 or MG >40 mmhg Symptomatic pt with domed PV Pk grad >50 or MG >30 mmhg with <mod PR ACHD Guidelines JACC 2008 Indications for intervention in RVOT Obstruction Baumgartner H et al; GUCH ESC 2010 Diagnosis? Question 20

21 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-126 Coronary Sinus ASD Left-sided SVC Coronary Sinus ASD Left-sided SVC Coronary Sinus ASD Left-sided SVC Coronary Sinus ASD Often difficult to diagnose May be an isolated abnormality May be associated with LSVC or complex CHD What is this? Question 21

22 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-132 What is the most likely cause of this echo finding? 1. Aortic coarctation 2. Severe mitral regurgitation 3. Anomalous pulmonary vein 4. Left-sided superior vena cava Persistent Left SVC Failure of left cardinal vein to regress, allowing drainage of rtsided deoxygenated blood into CS LSVC - common asymptomatic cong anomaly Presents as dilated CS seen on TTE Dx - injection of agitated saline into left UE, contrast appears in CS before entering RA Differential CS increased right heart pressure, anomalous PV, unroofed CS, TR Question 54-Year-Old Female CV disease diagnosed age 19, prognosis guarded 22

23 2018 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide Year-Old Female 54-Year-Old Female PR EDV = 3.6 m/sec 54-Year-Old Female What is the diagnosis? 1. Idiopathic PHT 2. Secondary PHT 3. Pulmonary thromboembolic disease 4. Other Eisenmenger Syndrome Large L R shunt produces PHT and irreversible PVD Shunt reverses R L causing cyanosis Dyspnea, syncope, hemoptysis, stroke 23

24 2018 MFMER slide MFMER slide-140 Eisenmenger Syndrome Hematologic Issues New equilibrium Tissue hypoxia O 2 sat erythropoietin Recommendations for targeted PAH therapy in CHD Improved tissue oxygenation number RBCs RBC mass Negative feedback Bone marrow Erythroid cell proliferation Baumgartner H et al; GUCH ESC 2010 Complex Congenital Heart Disease Cases Distribution of Age at Death in Patients With Congenital Heart Disease in and Year Age at death (years) <1 Year Proportion of all deaths (%) Khairy et al: JACC 56:1149, MFMER MFMER What is the most likely problem? What is the most likely problem? 1. Residual pulmonary stenosis 2. Residual pulmonary regurgitation 3. Residual VSD 4. Residual cleft mitral valve 2018 MFMER MFMER

25 33-Year-Old Female Tetralogy of Fallot Right BT shunt age 18 months TOF repair age 3 years 33-Year-Old Female CHD 2 Operations Early in Life Asymptomatic wants to have a pregnancy 2018 MFMER MFMER Year-Old Female CHD prior repair CHD Prior Repair 2018 MFMER MFMER CHD Prior Repair TOF with PR TR = 2.7 m/sec 2018 MFMER MFMER

26 What would you suggest? 1. OK to have a pregnancy 2. More imaging 3. Refer to a specialist 4. No idea Cardiac MR RV EF = 46% Severely dilated RV, end-diastolic volume of 161 ml/m MFMER MFMER CHD Prior Repair Severe Pulmonary Regurgitation Tetralogy of Fallot 2018 MFMER NEJM: Vol. 342 No. 5, MFMER Tetralogy of Fallot Sub-aortic VSD Large subaortic VSD Overriding aorta RVOT obstruction RV hypertrophy 2018 MFMER MFMER

27 Tetralogy of Fallot Associated Defects 2-10% anomalous CA LAD from RCA LAD from right sinus 15% secundum ASD 25% right aortic arch Right Aortic Arch 25% tetralogy of Fallot 40% pulmonary atresia 40% truncus arteriosus 2018 MFMER MFMER Tetralogy of Fallot Problems after Repair Pulmonary regurgitation (PR) Pulmonary stenosis Residual VSD Aortic regurgitation Most Common Postop Problem in TOF is PR RA PR Most Common Residua in TOF R V RVOT patch Obtain operative report 2018 MFMER MFMER Tetralogy of Fallot Clues to Pulmonary Regurgitation Arrhythmias Dyspnea and fatigue Cardiomegaly RV enlargement Tricuspid regurgitation Postop TOF Pearls Severe PR most common late problem Dyspnea, arrhythmias Right heart enlargement, cardiomegaly, TR PVR indications 2018 MFMER MFMER

28 19-Year-Old Man with Tetralogy of Fallot 19-Year-Old Male TR Velocity = 5.5 m/sec 2018 MFMER MFMER Year-Old Male Outflow Tract Velocity = 4.5 m/sec 2018 MFMER MFMER What would you recommend? 1. Observation 2. Operation 3. Clinical FU 4. Other Melody Valve Bovine jugular venous valve segment Platinum-Iridium stent 2018 MFMER MFMER

29 Transcatheter Pulmonary Valve First Human Use Indications Dysfunctional RVOT conduit or prosthesis TOF Pulmonary Atresia Truncus TGA S/P Ross Operation Bonhoeffer P: Lancet MFMER MFMER Valve Design Rationale Natural venous valve and vein wall segment Deep coaptation provides competence across range of diameters Thin leaflets open and close under minimal pressure Tetralogy of Fallot Anomalous CA Anomalous CA in 3-7% LAD from RCA in TOF 2018 MFMER MFMER Indications for intervention after Repair of TOF Moving On Baumgartner H et al; GUCH ES 2018 MFMER MFMER

30 Abdominal Aorta PW Doppler This abdominal aorta PW Doppler profile is seen with which of the following? 1. Aortic valve stenosis 2. Arteriovenous fistula 3. Aortic coarctation 4. Aortic dissection 2018 MFMER MFMER Year-Old Male with Hypertension 21-Year-Old Male Mid Ascending Aorta = 54 mm Peak AV 1.4 m/sec Trivial aortic valve regurgitation 2018 MFMER MFMER What would you recommend? Abdominal Aorta PW Doppler 1. Medical therapy and observation 2. Percutaneous intervention 3. Operative intervention 4. Additional imaging 2018 MFMER MFMER

31 Abdominal Aorta PW Doppler 21-Year-Old Male New Diagnosis of Coarctation Normal Abnormal Coarctation Femoral pulses very difficult to palpate RLE BP 70 systolic 2018 MFMER MFMER Echo in Aortic Coarctation Clues to the Diagnosis Echo in Aortic Coarctation Clues to the Diagnosis 2018 MFMER MFMER Coarctation Postop Residua Hypertension Bicuspid AV Ventricular dysfunction, CAD Recoarctation Aneurysm, dissection Mitral valve disease Endocarditis, endarteritis Intracranial aneurysm, stroke 2018 MFMER MFMER

32 Indications for intervention in Coarctation of the Aorta Multimodality Imaging Baumgartner H et al; GUCH ES 2018 MFMER MFMER Fold Increase in ICA Risk in Patients With Coarctation Aneurysm - Postop Coarctation Anastomotic aneurysms have been found >30 years after operation Mayo Clinic Proc MFMER MFMER Abdominal Aorta PW Doppler What is the differential diagnosis? Moving Along 2018 MFMER MFMER

33 18-Year-Old Female with Palpitations 18-Year-Old Female with Palpitations 2018 MFMER MFMER MFMER Vel = 2 m/s 2018 MFMER MFMER

34 What is the diagnosis? 1. Dilated cardiomyopathy 2. LV non-compaction 3. D-transposition of the great arteries Moving Along 4. L-transposition of the great arteries 2018 MFMER MFMER Year-Old Man Progressive Dyspnea and Murmur 43-Year-Old Man Progressive Dyspnea and Murmur 2018 MFMER MFMER What valve lesion do you expect with this type of CHD? 1. Aortic regurgitation 2. Mitral regurgitation 3. Tricuspid regurgitation 4. Pulmonic regurgitation What is the diagnosis? 1. Non-compaction 2. Eisenmenger syndrome 3. Mitral regurgitation 4. Congenitally corrected TGA 2018 MFMER MFMER

35 Cardiac Looping d-loop Morphologic RV (with TV) to right of LV l-loop Morphologic RV (with TV) to left of LV Aorta follows loop d-tga aorta ant and right l-tga aorta ant and left RV Identification Coarse trabeculation Septoparietal muscle bundle TV chordal insertions into RV 2018 MFMER MFMER LV Identification Cardiac Crux Less trabeculated Smooth septal surface Mitral valve Papillary muscles Meeting point between Atrial and ventricular septa Septal portion of MV and TV Inflow portion of LV and RV 2018 MFMER MFMER Congenitally Corrected TGA Congenitally Corrected TGA 2018 MFMER MFMER

36 Congenitally Corrected TGA Congenitally Corrected TGA PT Ao MRA MLA MLV MRV 2018 MFMER MFMER Congenitally Corrected TGA cctga Presentation Early in Life Associated Lesions Percent VSD Pulmonary stenosis Tricuspid valve anomaly >90 Complete heart block 2/year Aortic regurgitation 25 None <10* Associated defects Murmur VSD Heart failure PS Cyanosis Early intervention * 2018 MFMER MFMER cctga Presentation Later in Life Associated TV Abnormalities Symptoms and signs Murmur, arrhythmia, CHF Abnormal ECG, CXR, Echo Isolated <1% Observation, medical therapy, operation MRA PT MLV Ao MLA MRV TR occurs in 50-90% Age Ebstein-like deformity Deficient or thickened TV Straddling TV with VSD Volume overload (VSD, shunts) 2018 MFMER MFMER

37 Proportion Without CHF Proportion Without RV Dysfunction Congenitally Corrected TGA 182 patients from 19 institutions Associated Lesions (n = 132) % Large VSD 68 Pulmonary stenosis 47 RV dysfunction Any 70 Mod-severe 39 PPM 45 No (Mild) Lesions (n = 50) % Small VSD 18 Mild PS 14 RV dysfunction Any 55 Mod-severe 32 PPM 27 Graham TP: J Am Coll Cardiol MFMER Graham TP: J Am Coll Cardiol MFMER Congenitally Corrected TGA Symptoms/Findings Associated Lesions (%) n = 132 CHF 51 TR Any 82 Mod/Severe 57 Arrhythmia 47 No Assoc Lesions (%) n = 50 34* By age 45 yr, 67% with assoc lesions had CHF vs 25% without assoc lesions * Corrected TGA Freedom from CHF 1.0 Group II 0.8 (no assoc lesions n=50) 0.6 Group I (assoc lesions 0.4 (n=125) 0.2 p= Age (yr) Freedom from Moderate/Severe RV Dysfunction Group I (assoc lesions (n=121) 0.2 p= Age (yr) Group II (no assoc lesions n=47) Graham TP: J Am Coll Cardiol MFMER Graham TP: J Am Coll Cardiol MFMER cctga and srv Dysfunction Predisposing Factors Age Associated cardiac lesions TR Arrhythmia Pacemaker implantation Prior surgery especially stv CTA Coronaries in cctga RCA Supplies the SRV Graham TP: J Am Coll Cardiol MFMER Sing: Circulation MFMER

38 Congenitally Corrected TGA Tale of Three Adults LA AV Valve Regurgitation srv 40 yo asymptomatic 41 yo severe subps 42 yo recent CHF 2018 MFMER Congenitally Corrected TGA Tale of Three Adults cctga Vent Dysfunction and AV Valve Regurgitation 40 yo asymptomatic 41 yo severe subps 42 yo recent CHF 2018 MFMER cctga and AV Valve Regurgitation Predisposing Factors Age Ebstein-like valve Intracardiac surgical procedure/ppm Volume overload VSD, surgical shunts Systemic RV dysfunction Myocardial ischemia Non-systemic ventricular pressure 2018 MFMER Congenitally Corrected TGA Tale of Three Adults Observation 20 yr Observation 17 yr SAVVR + CRT-D 60 yo asymptomatic 58 yo severe subps 42 yo recent CHF 38

39 Natural History of cctga Congenitally Corrected TGA Associated Lesions No associated Lesions Uncertainties Think l-tga with abnormal ECG, CHB, ventricular dysfunction, AV valve regurgitation AV valve regurgitation and ventricular dysfunction Intervention Early srv and str determine timing/type intervention Late Role of medical therapy/crt How to prevent srv/stv dysfunction are major late concerns l-tga pt may present in adulthood 2018 MFMER MFMER Indications for intervention in cctga Postoperative CHD population Surgical history operative report Important to know the residua associated with various lesions Baumgartner H et al; GUCH ES 2018 MFMER MFMER This CW Doppler profile is most likely from which of the following shunts? Question 1. Subclavian artery to pulmonary artery 2. Superior vena cava to pulmonary artery 2018 MFMER MFMER

40 Surgically Created Shunts Cyanotic pt with low pulmonary blood flow (eg TOF) Improves oxygenation Make PA s grow Usually through lateral thoracotomy All have potential for complications Which of the following best describes the Blalock-Taussig shunt? 1. Subclavian artery and pulmonary artery 2. Ascending aorta to right pulmonary artery 3. Superior vena cava to pulmonary artery 4. Descending aorta and left pulmonary artery 2018 MFMER MFMER Blalock-Taussig Shunt Subclavian Artery to Pulmonary Artery Echo in Right Blalock-Taussig Shunt Classic Modified 2018 MFMER MFMER Palliated Single Ventricle Echo in Left Blalock-Taussig Shunt Subclavian Artery to Pulmonary Artery 2018 MFMER MFMER

41 Which of the following best describes the Glenn anastomosis? 1. Subclavian artery and pulmonary artery 2. Ascending aorta to right pulmonary artery 3. Superior vena cava to pulmonary artery 4. Descending aorta and left pulmonary artery Classic Glenn SVC to PA Connection Rarely used due to propensity to develop pulmonary AV fistulae 2018 MFMER MFMER Echo in Classic Glenn Shunt SVC to PA Connection Bidirectional Glenn 2018 MFMER MFMER Glenn Anastomosis Isolated or TCP Connection Suprasternal short axis view Use low Nyquist limit and filter Low velocity biphasic forward flow Laminar color flow Waterston Pott s 2018 MFMER MFMER

42 Problems With Surgical Shunts PA distortion Kinking, thrombosis, occlusion Problems with large shunts Pulmonary HTN Pulmonary vascular disease LV volume overload Moving Along 2018 MFMER MFMER Year Old Man Operation for Cyanotic CHD What is the diagnosis? 1. d-transposition 2. Eisenmenger syndrome 3. Congenitally corrected TGA 4. Pulmonary regurgitation 2018 MFMER MFMER Transposition Great Arteries TGA or d-tga Baffles 2018 MFMER NEJM: Vol. 342 No. 5, MFMER

43 d-transposition of Great Arteries (d-tga) Surgery early in life Atrial switch Mustard, Senning Rastelli when VSD present LV aorta, RV PA conduit Arterial switch d-tga Post Atrial Switch NEJM: Vol. 342 No. 5, MFMER MFMER d-tga Status Post Mustard Operation d-tga Following Atrial Switch Complications Atrial arrhythmia Baffle obstruction Baffle leak - ASD RV failure Tricuspid regurg 2018 MFMER MFMER Atrial Switch Operations Mustard or Senning Indications for intervention in TGA after Atrial Switch PV RV IVC LV 20 year survival 80% Successful pregnancies Late attrition from RV failure Arrhythmia Reduced exercise capacity 61% late gadolinium enhancement (fibrosis) Wilson et al: JACC, MFMER Baumgartner H et al; GUCH ES 2018 MFMER

44 d-tga Following Rastelli Complications LVOT obstruction Conduit obstruction Recurrent VSD Aortic enlargement AV regurgitation d-tga Status Post Rastelli Operation 2018 MFMER MFMER Indications for intervention in Patients with RV-PA Conduits Arterial Switch Procedure Cut in arterial trunks * Ao * Pulm trunk Coronary orifices sewn over Pulm trunk Ao CA reimplanted to neoaortic root Morph RV Morph LV Morph RV Morph LV Baumgartner H et al; GUCH ES 2018 MFMER MFMER d-tga Status Post Arterial Switch Operation Indications for intervention in TGA after ASO 2018 MFMER Baumgartner H et al; GUCH ES 2018 MFMER

45 Recent patient with complex CHD No surgical records On exam sternotomy and right thoracotomy Recent patient with complex CHD What operation has this patient had? 2018 MFMER MFMER Recent patient with complex CHD What operation has this patient had? Modified Fontan RPA IVC RA LA Exclusion Right AV Valve 2018 MFMER MFMER Fontan Operation Complex Cyanotic CHD Single ventricle Vena cava blood from RA to PA Normal PA size Normal PA pressure - tricuspid atresia Many modifications RA Appendage to PA Connection 2018 MFMER MFMER

46 25-Year-Old With PA/IVS, Status-Post Fontan Intracardiac Conduit 25-Year-Old With PA/IVS, Status-Post Fontan Intracardiac Conduit 2018 MFMER MFMER Lateral Tunnel Fontan Lateral Right Atrial Tunnel LA 21-Year-Old With TA, Status-Post Fontan Lateral Tunnel Lateral Right Atrial Tunnel LA LV LV 2018 MFMER MFMER Tricuspid Atresia, Normally Related GA Extracardiac Fontan Fontan Complications Arrhythmias atrial >> ventricular Bradycardia pacemaker Residual shunts cyanosis Obstruction JVP, edema, hepatomegaly Ventricular dysfunction systolic/diastolic Protein-losing enteropathy Liver disease cirrhosis, hepatocellular carcinoma Reoperations 2018 MFMER MFMER

47 Fontan Complications Atrial arrhythmias Fontan obstruction RA thrombus TEE for DCCV Bonus Question 2018 MFMER MFMER TEE Asymptomatic Patient 40-Year-Old Male What is this? 1. Unicuspid aortic valve 2. Bicuspid aortic valve 3. Tricuspid aortic valve 4. Quadricuspid aortic valve 5. Can t tell 2018 MFMER MFMER Quadricuspid AV Abnormal AV development Rare congenital disorder ~0.033% in autopsy series Male predominance 1.6:1 7 QAV types (A G) AR most common presentation Type B 2018 MFMER MFMER * 47

48 Quadricuspid AV Thank you! Good Luck! 2018 MFMER MFMER