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The Adult with

Transposition Do two wrongs make a right?

Forms of Transposition

• L-Transposition of the Great Vessels

• D-Transposition without pulmonary stenosis

− Atrial Repair

− Anatomic repair (arterial switch)

• D-Transposition, VSD, pulmonary stenosis

− Rastelli repair

Figure 3. Relative survival of patients and patient groups.

Nieminen H et al. Circulation 2001;104:570-575

Copyright © American Heart Association

L-transposition of the Great

Vessels

• Rare: Less than 1 % of congenital heart disease

• Atrioventricular discordance and ventriculoarterial

discordance

• If no major associated anomalies, survival to

adulthood without intervention is common

• High frequency of major anomalies that can

dominate early history

L-TGV: Two wrongs Normal vs. L-TGV

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L-transposition: tricuspid valve closer to apex L-transposition: mitral-pulmonary valve continuity

L-transposition

• Associated anomalies

− VSD in 70%

− PS 40%

− Systemic AV valve abnormalities

Ebstein’s Anomaly

Progressive Tricuspid Regurgitation

− Progressive dysfunction of systemic right ventricle

L-transposition

• Conduction system abnormalities

− Dual AV node anatomy and abnormal His

bundle

− Progression to AV Block estimated at 2% per

year

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L-TGV: ECG L-TGV: Echocardiography

• Tricuspid valve closer to cardiac apex

− Importance of tricuspid regurgitation

• Mitral-pulmonary valve continuity

• Aorta usually anterior and to the left of pulmonary

artery

L-transposition: tricuspid regurgitation L-transposition: right ventricular function

RV failure

• In those without associated lesions, some degree

on CHF in about 1/3 by fifth decade

− Role of tricuspid regurgitation

• Frequency greater in patients with previous heart

surgery

D-TGV

• Ventriculoarterial discordance

− D-loop, RV on right side of heart

− Ao tends to be on right and

anterior

• Cyanosis

• Associated lesions

• Role of PS

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Atrial Repair

• Senning 1958, Mustard later

− Baffle atrial blood to contralateral AV valve

Atrial repair

Atrial repair

• Good early results

• Late complications

− Sinus node dysfunction;

sinus rhythm in 77% at 5

years, 40% in 20 years

− Atrial flutter may be

marker of sudden death

Atrial repair

• Late ventricular dysfunction and tricuspid regurgitation

• Actuarial survival 80% at 20 years

• Evaluation of RV function difficult due to different

architecture of RV

• Baffle leaks and obstruction (SVC>IVC>pulmonary

venous)

• Pulmonary hypertension more frequent in those

operated at > 2 years of age

Atrial repair

• Actuarial survival

• Mortality 1-5 %

• Late sudden death

• High frequency of good functional class (80% symptom

free with NYHA Class I)

• Progressive increase in RV dysfunction, TR, arrhythmias

− 2.5% early mortality, 2.5% late mortality

Atrial repair

• Loss of sinus rhythm

• Increase in atrial

arrhythmias

• Late sudden death

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Atrial Repair: Echo

• Evaluate right (systemic) ventricular function

− RV size and contractility (often subjective)

− Tricuspid regurgitation

• Evaluate for baffle obstruction

− Systemic venous (more common)

− Pulmonary venous

Atrial repair of D-TGV

Atrial repair: Pulmonary venous baffle Atrial repair: Pulmonary venous baffle

Atrial repair: Tricuspid regurgitation

Arterial switch

• Introduced by Jatene in 1976

• Transect great arteries,

transpose them and move

coronaries

• Distortion of branch

pulmonary arteries, aortic

insufficiency, coronary

occlusion early

postoperatively

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Arterial switch

• Advantage: Left ventricle is the systemic

ventricle

• Substantial morbidity and mortality in early

experience

• Recent results much improved, but long term

results are still evolving

Surgical mortality

• Only studies are

retrospective

• High early mortality

with arterial switch,

probably better long

term mortality

• Increased mortality

with single or

intramural coronaries

Figure 1. Top, Actuarial survival in 1200 patients who had ASO. Numbers indicate number of patients

observed at beginning of interval.

Losay J et al. Circulation 2001;104:I-121-I-126

Copyright © American Heart Association

ASO: Late complications

• Late death relatively rare

• Branch pulmonary stenosis

• Progressive aortic insufficiency

• Evaluate for coronary occlusion

Aortic insufficiency

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Figure 3. Actuarial survival free from grade II or more AI (Ao insufficiency) for 1095 survivors.

Losay J et al. Circulation 2001;104:I-121-I-126

Copyright © American Heart Association

• 19 year-old S/P arterial switch

• Asymptomatic but with increasing left ventricular size

Arterial Switch: Aortic Insufficiency Arterial Switch: Aortic Insufficiency

Arterial switch

• Probably lower risk of late death, but population is

growing older

• Often asymptomatic in presence of progressive

disease

• Increased risk of coronary findings later in life

which are difficult to visualize echocardigraphically

“Complex” D-TGA

• Most frequent complex

lesion is with ventricular

septal defect and

pulmonary (valvar and

sublalvar) stenosis

• Most common mode of

repair is the Rastelli

procedure

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Rastelli repair

• Mean age (initially) age 4 years

• Current survival 93% at 20

years, but early was 52% at 20

years

− Late sudden death

• Reoperation due to progressive

stenosis of RV-PA conduit

Rastelli

• Surgical issues—recurrent

LVOTO, conduit

obstruction, arrhythmias,

late mortality

− Perioperative heart block

− Straddling tricuspid valve

Rastelli: reintervention

Rastelli: Echo

• Evaluate for both LV and RV outflow obstruction

• Evaluate for ventricular dysfunction

• Investigate for residual VSD

“Complex” D-TGA: Rastelli “Complex” D-TGA: Rastelli

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“Complex” D-TGA: Rastelli “Complex” D-TGA: Rastelli

“Complex” D-TGA: Rastelli, residual VSD

Transcatheter valve

replacement

Transcatheter valve

replacement

“Complex” D-TGA: Rastelli

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“Complex” D-TGA: Rastelli

Suprasternal notch view for branch pulmonary arteries

“Complex” D-TGA: Rastelli

Prognosis in Adults Good Intermediate Uncertain/Poor

Atrial Septal

Defect Aortic Stenosis

D-Transposition

(arterial switch)

Patent Ductus

Arteriosus Tetralogy of Fallot L-Transposition

Pulmonary

Stenosis

D-Transposition

(Senning/Mustard

)

Ebstein’s

Anomaly

Ventricular Septal

Defect Single Ventricle

Coarctation

Mortality-->Insurance Lesion

Late Mortality

(%)

Mortality

Ratio Underwriting

ASD/PDA/PS 5-12 70-200 100

VSD 20 667 100-200

CoA 16 320 100-300

Aortic Sten. 15 375 225-400+

ToF 14 350 200-400

Senning 24 480 Declined

Single Vent. 85 >2800 Declined

Cognitive issues

• Cognitive function probably diminished

• Lower than average scores on

neuropsychological testing as adolescents

• Many receive remedial academic or behavioral

services during school years

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Pregnancy in TGA

• Prognosis dictated by ventricular function and

arrhythmias

• Thorough evaluation of rhythm particularly in

atrial switch and L-TGV patients

• Complications of valve anticoagulation

• Increased risk of congenital heart disease in

offspring

Pregnancy

Lesion Success

rate Complications Reference

L-TGV 83%

CHF with valve

replacement after

delivery

JACC 33:1692 (1999)

Atrial

Switch 82%

Arrhythmias 22%,

other 80% Eur Ht J 26:2588 (2005)

Arterial

Switch

13/17

(76%)

VT, valve

thrombosis AJC 106:417 (2010)

Rastelli 50% Increased sub-

aortic stenosis

Aust NZ Obstet Gyn 45:243

(2005)

There are more adults than children

with congenital heart disease in the

U.S…

…boldly going where no group

of patients has gone before.