Transposition of the great arteries
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1 EuroEcho Teaching course on CHD Transposition of the great arteries - Follow-up after the arterial switch Gertjan Tj. Sieswerda, MD PhD Nothing to disclose Interuniversitary Institute for Congenital Heart Disease Nijmegen / Utrecht
2 Transposition of the Great Arteries Definition Atrio-ventricular concordance + Ventriculo-arterial discordance Ao arises from the RV and PA arises from the LV Ao PA TV MV RV LV Ao PA
3 Transposition of the Great Arteries AV concordance, VA discordance; aka D-TGA, complete transposition, physiologically uncorrected transposition First described in by Matthew Baillie in 1797 Term transposition applied in 1884 by John Richard Farré: APC and aorta placed (positio) across (trans) the IVS
4 Transposition of the Great Arteries Transposition: the extended family TGA Family DORV Discordant VA Connection Taussig Bing (sub-pa VSD) Univentricular AV Connection Concordant AV Connection Discordant AV Connection Karl Tex Heart Inst J 1997 Ao from "RV" +/- SAS, AAO cctga TGA.IVS TGA.VSD cctga.ivs cctga.vsd (+/- LVOTO)
5 Transposition of the Great Arteries Incidence ~1:3,500-5,000 live births, M:F = ~2.5:1, 5-7% of all CHD Isolated in 50% of cases ( simple TGA), main associated cardiac lesions: VSD (~35%) and LVOTO (~10%) Pulmonary and systemic circulation run in parrallel; survival depends on septal or arterial level Natural history: <5% survival >2 months
6 Transposition of the Great Arteries Shunt size and degree of LVOTO determine symptoms: CHF and cyanosis Palliative treatment usually required soon after birth (PG-E1 and/or atrial septostomy) At a later stage physiological ie atrial switch or (preferable) anatomical correction ie arterial switch necessary ASO: long-term survival 15 years; but exercise tolerance, cognitive function and quality of life is impaired
7 TGA: the atrial switch Pulmonary venous return: posterior from superior baffle, anterior from inferior baffle Teaching slides Mayo Clinic
8 TGA: the arterial switch Adib D. Jatene (1975) Physiologic and anatomic correction Concordant AV and VA connections RV and TV at pulmonary pressure Low early & late risk in current era Late problems are uncommon anatomic correction
9 TGA: the arterial switch anatomic correction 1. Transection of pulmonary and aortic trunks 2. Translocation of coronaries to neoaorta 3. De-transposition + anastomosing of distal ends
10 TGA: the arterial switch Lecompte: pulmonary bifurcation anterior from aorta
11 TGA: atrial vs. arterial switch Long-term outcome Arterial Switch: 2000 s Atrial Switch: 1980 s Natural History: 1950 s Years Williams Circulation 2008
12 TGA after ASO: late outcome Survival n = 132 adult ASO survivors Single center Median FU 21 yrs (18-33) 17% significant residua (EF, valve problem, arrrhytmia) No intervention for root or AoR 82% reduced X-tolerance Tobler JACC 2010
13 TGA after ASO: late outcome Freedom of reintervention n = 195 (151) ASO pts Single center FU 25 yrs 1.5% late mortality Reintervention in 23% (mainly PS) 1 pt grade III-IV AoR 3% reduced LVEF 3% coronary sequelae Hutter JACC 2002
14 TGA after ASO: late outcome AoR, coronary events, arrythmia AoR > II: 15 yrs Coronary obstruction: 2.1% (23/278pts by CAG) Ventricular dysfunction: 1.9% Aortic regurgitation Sinus rhythm : 98% Losay Circulation 2001
15 TGA after ASO: late outcome Coronary events Event-free survival 93%, 91% and 1, 10 and 15 yrs 89% events <3mo after ASO CAG: 4% significant obstructions n = 1198 post-aso pts Mean FU 5 yrs (0.5-17) Single institution (among largest studies available) Legendre Circulation 2003
16 TGA after ASO: late outcome Coronary physiology n = 21 post-aso pts Mean 11.2 yrs after OR Single institution All w/o symptoms CAG w/o stenoses Hauser Circulation 2001
17
18 ASO-specific questions to be answered LV function (global, regional) arterial anastomic sites; mainly supravalvar PS / RVOTO ( evaluate trunk, bifurcation, branches) RV function (global) and RVSP Neo-AoV regurgitation Diameter Ao ascendens, acute angle aortic arch Stress-echo to unmask coronary insufficiency? ESC guidelines management GUCH 2010
19 LV systolic function PSLAX, AP 4-2-3CV M-mode dimensions, global function / EF, regional WMA
20 LV systolic function PSLAX, AP 4-2-3CV M-mode dimensions, global function / EF, regional WMA
21 LV systolic function PSLAX, AP 4-2-3CV M-mode dimensions, global function / EF, regional WMA
22 LV diastolic function E/A = 1.5 DT 180ms E med = 10.2 cm/s E lat = 14.3 cm/s AP4CV - MV transvalvular flow (PW), VP flow (PW) - TDI septal and lateral basal segments - LA size
23 Long term myocardial function Hövels Ann Thorac Surg 2003
24 Stress echo: ischemia detection Hui Heart 2005
25
26 RVOTO Neo-PV stenosis most frequent complication after ASO: 5yr till 10 yrs Prone to increase in severity and location over time Intervention rate ~5% / 10 yrs Surgical techniques may influence RVOTO occurrence
27 RVOTO High PSSAX - origin LPA and RPA - stenosis/gradient (PW/CW) at origin and more distally - peak gradients <16mmHg ~normal!
28 RVOTO PSSAX - origin LPA and RPA - stenosis / gradient (PW/CW) at origin and more distally
29 RVOTO High PSSAX - origin LPA and RPA - stenosis/gradient (PW/CW) at origin and more distally
30 RVOTO Vmax 3.2 m/s gradient ~40mmHg Bernouilli may not apply: needs confirmation thru RVSP, RV anatomy and function Echo Doppler often allows identification of increased RV pressures, but not always its mechanism High PSSAX - origin LPA and RPA - stenosis/gradient (PW/CW) at origin and more distally
31 Confirm RVOTO: RV evaluation TAPSE 15mm PSLAX, PSSAX, AP4CV eyeballing, TAPSE, TDI S wave TDI S-wave 8.2cm/s
32 Confirm RVOTO: RVSP by TR RVSP 60-65mmHg PSRVinflow, AP4CV - presence and grading of TR; RVSP
33 RVOTO Suprasternal - distal RPA - indicative rather than proof - prone to underestimation (angle)
34 RVOT on TEE
35 Echo Doppler often allows identification of increased RV pressures, but not always its mechanism CT / MRA may be necessary to image APC and its branches
36
37 Neo-aorta: dilatation, AoR LVOTO hardly ever a problem Mild regurgitation common, +/- root dilatation AoR increases slowly with age; HD significant ~8%/15yr Mild AoR associated with root > annular dilatation Moderate (early) root dilatation common; little progression at later age; low chance of dissection?
38 Neo-Aorta: root dilatation LVOT 22mm H-H 26mm Root 40mm STJ 36mm PSLAX, SAX, PS3/5CV - diameter hinge-hinge, root, STJ, ascending - flow across neo-aov (color + spectral Doppler)
39 Neo-Aorta: root dilatation PSLAX, SAX, PS3/5CV - diameter hinge-hinge, root, STJ, ascending - flow across neo-aov (color + spectral Doppler)
40 Neo-Aorta: dilatation From: Lai cs Echo in pediatric and CHD 2009
41 Aortic root / AoR PSLAX, SAX, PS3/5CV - diameter hinge-hinge, root, STJ, ascending - flow across neo-aov (color + spectral Doppler)
42 Aortic root / AoR PSLAX, SAX, PS3/5CV - diameter hinge-hinge, root, STJ, ascending - flow across neo-aov (color + spectral Doppler)
43 Aortic root / AoR Grading AoR: VC / backflow desc - abdominal
44 Aorta: arch / abdominal Suprasternal - dimensions, acute angulation arch (2D) - flowcharacteristics:gradient, s awtooth
45 Aorta: arch / abdominal Suprasternal - dimensions, acute angulation arch (2D) - flowcharacteristics:gradient, s awtooth
46 Aorta: arch / abdominal Suprasternal - dimensions, acute angulation arch (2D) - flowcharacteristics: gradient, sawtooth
47 Aorta: arch / abdominal Suprasternal - dimensions, acute angulation arch (2D) - flowcharacteristics: gradient, sawtooth
48 Aorta: arch / abdominal Subcostal - flowcharacteristics: pulsatile flow (color); sawtooth (spectral)
49
50 Conclusion Late outcome of ASO is generally good RVOTO most common complication, AoR less common (due to root dilatation) reason for intervention Resting myocardial function preserved, myocardial reserve less than normal; non-coronary mechanism? Echocardiography uniquely suited to steer clinical decision making; value of stress imaging doubtful
51 Suggested reading Highly recommended Free download thru ESC website (
52 Thank you for your attention
53
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