Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.

com

Education in Heart

CONGENITAL HEART DISEASE

Repaired tetralogy of Fallot in the

adult: monitoring and management
Kier V Huehnergarth,1 Michelle Gurvitz,2 Karen K Stout,1 Catherine M Otto1
c Additional references are Tetralogy of Fallot (ToF) is the most common VSD results in equal pressures in the right and left
published online only at http:// cyanotic congenital heart disease (CHD). The ventricles and, typically, a right-to-left or bidirec-
heart.bmj.com/content/vol94/
history of surgical treatment of ToF is closely tional shunt through the VSD. The desaturated
issue12
1 linked with the origins of cardiac surgery. Before blood passing into the aorta results in central
Division of Cardiology,
Department of Medicine, the era of cardiac surgery, most ToF patients died cyanosis. In addition, the pressure loaded RV
University of Washington School in childhood; now well over 85% survive to becomes hypertrophied and may develop diastolic
of Medicine, Seattle, adulthood, resulting in an increased prevalence of dysfunction. Aortic regurgitation may result from
Washington, USA; 2 Division of ToF in adults.1 Patients with ‘‘repaired’’ ToF pose the dilation of the aorta or the abnormal flow
Cardiology, Department of
Pediatrics, University of unique challenges for primary care providers and caused by the overriding aorta and VSD.
Washington School of Medicine, cardiologists. This review will focus on the history
Seattle, Washington, USA and changing epidemiology of ToF in adults, the
HISTORY OF TETRALOGY OF FALLOT
pathophysiology of repaired ToF, and emerging
Correspondence to: ToF was originally defined by Niels Stenson in
Kier V Huehnergarth, MD,
data for monitoring and treatment.
1671.w13 In 1888, Etienne-Louis Fallot described the
Division of Cardiology, Box
356422, University of
anatomic features of this condition which now
Washington, Seattle, WA EMBRYOLOGY/ANATOMY carries his name.w14 Before 1944, there was no
98195, USA; kiervh@gmail.com The aorta and pulmonary artery form from treatment for children with ToF beyond supple-
septation of the distal bulbus cordis and truncus mental oxygen and supportive care. Palliative
arteriosus and rotate to overlie the ventricles. treatment, in the form of a surgically created left-
Faulty rotation and septation with incomplete to-right shunt of blood from the subclavian artery
transfer of the aorta to a position above the left to the pulmonary artery, became available through
ventricle results in a malalignment ventricular the work of Helen Taussig, Alfred Blalock and
septal defect (VSD) and an aorta that ‘‘overrides’’ Vivian Thomas, and the first surgical shunt
the interventricular septum.w1 Right ventricle (RV) procedure was performed in November 1944.
outflow tract obstruction, the other defining ‘‘Complete’’ repair became available in the 1950s
feature of ToF, is the result of one or more of the after the advent of controlled cross circulation, an
following abnormalities: subvalvar obstruction early form of cardiopulmonary bypass.
from septal deviation, hypertrophied muscular There are varying techniques for intracardiac
bands in the RV outflow tract, a hypoplastic repair of ToF, mainly due to anatomical differences
pulmonary valve annulus, pulmonary valve steno- between individual patients. Generally, repair
sis or atresia, and main or branch pulmonary artery includes a patch closure of the VSD and modifica-
stenosis. The RV hypertrophies as a result of the tion of the RV outflow tract to improve pulmon-
outflow tract obstruction. Thus, the characteristic ary flow, which can include opening of the
four features of ToF are an overriding aorta, RV pulmonary valve, resection of muscle bundles,
outflow tract obstruction, malalignment VSD, and and/or placement of a transannular patch if
RV hypertrophy (fig 1). In addition, ToF is necessary to widen the outflow tract. In some
associated with other congenital cardiac abnorm- cases there are anatomic abnormalities precluding a
alities in about 40% of patients. Some of the more transannular patch, such as an anomalous coron-
common anomalies are a right sided aortic arch ary artery crossing the RV outflow tract, so that an
(13–34%), atrial septal defect (,15%), atrioventri- RV to pulmonary artery conduit must be used
cular septal defect (1.7–7%), anomalous coronary instead. In cases where a conduit is required,
arteries (most commonly a prominent conus artery replacement at least once during childhood typi-
or the left anterior descending artery from the right cally is required due to stenosis of the conduit.
coronary artery or right sinus of Valsalva) (2–10%), Placement of a transannular patch results in
fistulas between a coronary artery and the pul- unobstructed flow from the RV to the pulmonary
monary artery or right atrium (12%), hypoplasia of artery, but sacrifices pulmonary valve competency
the coronary tree (0.8%), aortic regurgitation resulting in pulmonary regurgitation. Initially, it
(,1%) and persistent ductus arteriosus (,1%) was thought that the RV would tolerate pulmon-
(these percentages are largely based on small case ary regurgitation without long term sequelae.
series).w2–12 However, it is now known that there are haemo-
The anatomy of ToF explains much of the dynamic and electrophysiologic consequences of
pathophysiology seen. The RV outflow tract this regurgitation, which contribute in large part to
obstruction together with the non-restrictive the long term morbidity and mortality of repaired

Heart 2008;94:1663–1669. doi:10.1136/hrt.2008.147249 1663

 Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.com

Education in Heart

patients require unique management based on

their individual anatomy and physiology.
There has been an increase in severe CHD
prevalence in ages above 13 years due to improved
survival (fig 2).3 From 1985 to 2000, the overall
prevalence of severe CHD in adults increased by
85% compared to an increase of 22% in children.
Thus, the prevalence of severe CHD is now nearly
equal in adults and children. The prevalence of ToF
in adults has also increased over this time period.
Based on these changing demographics, clini-
cians can expect to care for an increasing number
of adults with repaired ToF in both primary care
and cardiology clinics. Now that the long term
sequelae of surgical interventions, including pul-
monary regurgitation, are recognised, data are
accumulating to help clinicians monitor patients
with repaired ToF and to help guide therapeutic
decisions.

Clinical course
The major adverse sequela of ToF repair, specifi-
cally pulmonary regurgitation, is associated with
significant late morbidity and mortality. Chronic
RV volume overload leads to progressive RV
enlargement and eventual systolic dysfunction.
Abnormal RV haemodynamics can be exacerbated
Figure 1 Schematic drawing of tetralogy of Fallot (ToF) by associated conditions including residual pul-
depicting the four characteristic features of ToF: (1) monary stenosis and tricuspid regurgitation. These
overriding aorta; (2) right ventricular outflow tract (RVOT) abnormal haemodynamics lead, in part, to the long
obstruction; (3) malalignment ventricular septal defect
term clinical presentation of exercise intolerance
(VSD); and (4) right ventricular hypertrophy (RVH). Ao,
aorta; LA, left atrium; LV, left ventricle; PA, pulmonary and arrhythmias.w18 Overall long term survival is
artery; RA, right atrium; RV, right ventricle. Reproduced somewhat less than expected survival for healthy
with permission from Otto CM, ed. The practice of clinical individuals (early experience = 86% vs 96% at
echocardiography, 3rd ed. Elsevier, 2007:1070. 30 years), with major adverse clinical outcomes of
recurrent symptoms in up to 23%, arrhythmias in
4.8%, and sudden death in 6%.w19 Abnormalities of
ToF. With these consequences in mind, current the left side of the heart also have been increasingly
surgical practice is to restore the RV to unob- recognised, including left ventricular dysfunction.
structed pulmonary artery flow, while attempting Associated aortic root dilation can lead to aortic
to preserve as much competence of the pulmonary regurgitation, aortic rupture or dissection.4–6 w20 w21
valve as possible. Sometimes significant pulmonary Box 1 summarises potential long term adverse
regurgitation is unavoidable, particularly when the outcomes.
degree of RV outflow tract obstruction necessitates
transannular patch placement. Attempts to mini-
RESIDUAL STRUCTURAL HEART DISEASE
mise regurgitation, such as placement of prosthetic
The most common residual structural defect in
valves during surgery in childhood, have been
repaired ToF is pulmonary regurgitation with
problematic due to poor prosthetic valve longevity
consequent RV dilation and eventual systolic
and inability of the prosthetic valve to grow with
dysfunction. Cardiac imaging is essential in adults
the child. Thus, patients can continue to reach
with repaired ToF because physical examination
adulthood with significant pulmonary regurgita-
findings are often subtle and do not provide needed
tion secondary to repaired ToF.
quantitative information. The diastolic murmur of
pulmonary regurgitation is soft because the dia-
Demographics/epidemiology stolic pressure gradient between the pulmonary
In the USA, congenital heart disease occurs in artery and RV is very low and may even be absent
about 1% of live births.w15 ToF occurs in about 3.9 with the low velocity to-and-fro flow characteristic
per 10 000 live births in the USA and accounts for of severe regurgitation. Other physical findings
up to 10% of cases of CHD.2 w16 Without surgical include a parasternal RV heave due to RV
treatment, the estimated 1 year survival is 66% enlargement, signs of right sided heart failure
and the estimated 30 year survival is 6%. With and, of course, the surgical scar.
surgical treatment over 85% of children survive to Echocardiography in adults with repaired ToF
adulthood.w17 Some patients do survive into adult- demonstrates the VSD patch and the enlarged,
hood without surgical treatment and these overriding aorta (fig 3). Residual VSDs are

1664 Heart 2008;94:1663–1669. doi:10.1136/hrt.2008.147249

 Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.com

Education in Heart

Figure 2 Change in
prevalence of congenital
heart disease (CHD) per
age group from 1985 to
2000. There has been no
change in prevalence of
CHD in the early age group
(1–12). However, in each
of the older age groups,
prevalence has increased
from 1985 to 2000. CI,
confidence interval.
Reproduced with
permission from Marelli et
al.3

uncommon and the degree of aortic dilation particularly useful in patients with repaired ToF.7
typically is stable. However, as mentioned above, Much of the emerging data regarding outcomes
there are case reports of aortic rupture or dissec- and treatment of repaired ToF are based on cardiac
tion. Pulmonary regurgitation with consequent MRI.
RV dilation can be diagnosed by echocardiogra-
phy, although evaluation of RV size and function EXERCISE INTOLERANCE
is mostly qualitative. Exercise intolerance in patients with repaired ToF
Cardiac magnetic resonance imaging (MRI) is is common. The aetiology of exercise intolerance is
also useful in evaluating right and left sided cardiac likely multifactorial and includes abnormal RV
anatomy, ventricular function and valvular pathol- haemodynamics, autonomic dysfunction poten-
ogy (fig 4). In contrast to echocardiography, tially related to damage to the autonomic nervous
cardiac MRI provides quantitative measurements system at the time of childhood surgery, and
of the RV volumes and function, which is restrictive pulmonary function. A study using
cardiopulmonary exercise testing (CPET) demon-
strated that the mean (SD) peak oxygen consump-
Box 1 Potential long term adverse outcomes in tion with exercise (peak VO2) in patients with ToF
patients with repaired tetralogy of Fallot is 25.5 (9.1) ml/kg/min compared to 45.1 (8.6) ml/
kg/min in matched normal subjects.8 Often,
Pulmonary regurgitation leading to: patients with CHD are unaware of any exercise
c Right ventricle (RV) dilation
symptoms as their limitations may have been
c RV dysfunction
lifelong. However, CPET studies of ‘‘asympto-
c Right sided heart failure
matic’’ CHD patients have shown a wide range
c Tricuspid regurgitation
of exercise tolerance, including some with severely
c Exercise intolerance
reduced peak VO2. In contrast, some patients with
repaired ToF have normal to excellent exercise
Other right sided conditions tolerance; some even participate in high level
c RV outflow tract obstruction competitive athletics. Therefore, objective CPET
c Branch pulmonary artery stenosis testing is important to evaluate for exercise
c Endocarditis intolerance, and serial testing is useful to identify
Residual ventricular septal defect decreasing exercise tolerance. The degree of exer-
cise intolerance predicts hospitalisation and death
Left sided conditions in CHD patients, including those with repaired
c Aortic root dilation ToF. In a cohort of CHD patients, peak VO2
c Aortic regurgitation .27 ml/kg/min was associated with a 97% event-
c Left ventricular dysfunction free survival rate at 500 days, versus 63.4% in those
Atrial arrhythmias with a peak VO2 15.5–27 ml/kg/min and 50.5% in
those with a peak VO2 ,15.5 ml/kg/min.
c Atrial fibrillation
c Atrial flutter
CARDIAC ARRHYTHMIAS
Ventricular arrhythmias Atrial and ventricular arrhythmias and an
c Ventricular tachycardia increased risk of sudden death are well described
c Sudden death in patients with repaired ToF, with reported rates
of atrial flutter/fibrillation of 10%, ventricular

Heart 2008;94:1663–1669. doi:10.1136/hrt.2008.147249 1665

 Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.com

Education in Heart

Figure 4 Cardiac magnetic resonance image of a 22-

Figure 3 Echocardiogram of a 22-year-old man with year-old man with repaired tetralogy of Fallot at age
repaired tetralogy of Fallot at age 2 years. In the 2 years. There is dilation of the right ventricle (RV) and the
parasternal long axis view an overriding aorta is seen with RV outflow tract leading to the pulmonary artery (PA).
a dilated right ventricle (RV). Echocardiography provides Quantitation of RV size and ejection fraction is based on
quantitative assessment of left ventricle (LV) size and volumes calculated from traced endocardial borders at
function and aortic root dimensions, in addition to end diastole and end systole in parallel tomographic
evaluation of the presence and severity of pulmonary images of the RV chamber. Pulmonary regurgitant severity
regurgitation, RV size and systolic function, and the is measured based on the forward and retrograde flow
presence of a ventricular septal defect or any other volumes in the PA. LV, left ventricle.
anatomic abnormalities. Ao, aorta; LA, left atrium.
small in each subgroup, no other significant
tachycardia of 12%, and sudden death in 8% at a echocardiographic associations with arrhythmias
mean 21.1 years of follow-up.9 The underlying were identified in this study.
cause of these arrhythmias may be myocardial
fibrosis which has been shown histologically in the
RV both at sites of prior surgical repair and at TREATMENT
distant sites, suggesting another mechanism lead- Since the primary haemodynamic abnormality in
ing to fibrosis in addition to surgical scar.w22 A most patients with repaired ToF is pulmonary
similar distribution of RV fibrosis is seen by cardiac regurgitation, it is plausible that pulmonary valve
MRI with higher degrees of fibrosis associated with replacement would result in improved haemody-
an increased incidence of arrhythmias, decreased namics and outcomes. However, valve replacement
peak V̇O2, decreased RV systolic function, and at the time of ToF repair is not always ideal due to
increased RV end systolic volume index.10 poor longevity of valves and the inability of valves
QRS duration on the resting ECG .180 ms is to grow with the patient. In adults, the limited
associated with ventricular arrhythmias with high durability of prosthetic pulmonary valves remains
risk ratios for ventricular tachycardia and sudden an issue. Repeat valve replacement is an expected
death (41.9 (95%CI 14.7 to 119.4) and 2.29 (95% CI outcome after initial valve replacement. Thus, it is
1.05 to 5.02), respectively).9 11 However, it is ideal to replace the valve as late as possible without
currently unclear how to apply this to patient care compromising clinical outcomes. A major goal in
since no intervention studies have been performed management of adults with recurrent pulmonary
based on QRS duration. Ventricular arrhythmias regurgitation after ToF repair is optimal timing of
are common on Holter monitors (40–50%), but pulmonary valve replacement.
there is no association between asymptomatic Analogous to indicators for intervention with
arrhythmia frequency or complexity on Holter chronic left sided valve regurgitation, attention has
and clinically significant events. focused on the RV response to chronic volume
Early studies suggested an association between overload as a potential predictor of postoperative
elevated RV systolic pressures and sudden death.12 outcome. Several studies using cardiac MRI have
More recently, it has been shown that moderate or demonstrated that RV volumes decrease signifi-
severe pulmonary regurgitation is the main under- cantly following pulmonary valve replacement and
lying haemodynamic lesion for patients with that the degree of improvement correlates with
ventricular tachycardia or sudden death, whereas preoperative RV size.13–16 Improvement of RV
moderate or severe tricuspid regurgitation was systolic function has also been shown following
present in most patients with atrial flutter or pulmonary valve replacement.17 Based on these
fibrillation. Although numbers of events were studies, it has been suggested that surgery should

1666 Heart 2008;94:1663–1669. doi:10.1136/hrt.2008.147249

 Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.com

Education in Heart

functional status following valve replacement.18

Box 2 Suggested monitoring of patients with repaired tetralogy of Fallot Outcome data are not yet available on the long
term prognosis associated with various degrees of
Clinical persistent RV dilation or dysfunction.
c Symptoms of right sided heart failure Pulmonary valve replacement also results in a
c Exercise limitation (although frequently unreliable) decrease in clinical arrhythmias, specifically atrial
c Palpitations fibrillation/flutter and ventricular tachycardia.19
c Presyncope/syncope However, surgical ablation procedures are often
carried out concurrently with valve surgery, so it is
ECG unclear whether the improved haemodynamics or
c QRS duration .180 ms the ablations result in decreased arrhythmias.
Cardiopulmonary exercise test Generally, reoperation for pulmonary regurgita-
tion in ToF patients has a low perioperative
c Significantly impaired exercise tolerance
mortality and a 10 year survival of 92 (6)%.w23
c Decreasing exercise tolerance as documented by serial examinations
However, there is a limited lifespan of biopros-
Echocardiography thetic valves with an estimated nearly half of
c Residual VSD patients requiring reoperation at 10 years. Thus
c LV size and ejection fraction the implantation of a current generation valve
c Pulmonary regurgitation severity ‘‘starts the clock’’ on the next valve replacement
c RV size and systolic function (qualitative) and an individual patient could conceivably require
c Aortic root size multiple subsequent valve replacements during a
lifetime. Percutaneous pulmonary valve replace-
Cardiac MRI (cut-off values above which RV volumes may not return to
ment is an emerging option with experimental
normal):
studies showing high procedural success rates, low
2 13 15 16
c RVESVi 82–100 ml/m complication rates, and improvements in pulmon-
2 14
c RVEDVi 150 ml/m (in children) ary regurgitation, RV volumes, pressures and peak
2 15 16
c RVEDVi 160–170 ml/m (in adults) oxygen uptake.20 With evolution in device design
MRI, magnetic resonance imaging; LV, left ventricle; RV, right ventricle; RVEDVi, and operator experience, along with optimisation
right ventricular end diastolic volume index; RVESVi, right ventricular end systolic of patient selection, hopes are high for the clinical
volume index; VSD, ventricular septal defect. use of percutaneous pulmonary valves.
For the rare patient who presents with unre-
paired ToF in adulthood, management is indivi-
be considered before an RV end systolic volume dualised based on the unique characteristics of each
index .85 ml/m2 or an end diastolic volume index patient. Potential management options include late
.170 ml/m2. It should be noted that these repair, optimisation of medical treatment for
suggested breakpoints are based on small studies, cyanosis, and/or evaluation of the function and
and some patients with an RV end diastolic possible surgical optimisation of palliative shunts.
Some patients with severe disease may require
volume index ,170 ml/m2 did not have complete
consideration for heart or heart/lung transplant.
resolution of RV dilation. In addition, small studies
have shown improvement in exercise capacity and
GENERAL MEDICAL ISSUES
Other important clinical issues that arise in adults
with repaired ToF include safety of pregnancy,
Repaired tetralogy of Fallot in the adult: key points endocarditis prophylaxis, flu shots, and concerns
regarding exercise. In most cases, pregnancy is well
c The prevalence of adults with repaired tetralogy of Fallot is increasing and a tolerated. However, complications can arise in
growing number of these patients are expected to seek care in general patients who have significant residual RV outflow
cardiology clinics. tract obstruction so that evaluation before preg-
c The haemodynamic consequences (primarily pulmonary regurgitation) of nancy is strongly recommended. Children of
repaired tetralogy of Fallot lead to morbidity and mortality. patients with ToF have a risk of CHD about three
c Patients with repaired tetralogy of Fallot can develop exercise intolerance, times higher than the general population (3.1% vs
clinically significant arrhythmias and sudden death. ,1%, respectively).w24 In light of recent changes in
c Patients with repaired tetralogy of Fallot should be monitored clinically and the American College of Cardiology/American
with serial testing to evaluate exercise capacity and right ventricular volume Heart Association (ACC/AHA) valve guidelines,
because physical examination findings are not sufficient for clinical decision endocarditis prophylaxis is only recommended in
making. patients with prosthetic valves, unrepaired cyano-
c Cardiac magnetic resonance imaging and echocardiography are very useful in tic CHD, including palliative shunts, completely
evaluating right ventricular volume and function. repaired CHD with prosthetic material during the
c Pulmonary valve replacement before extreme right ventricular dilation can first 6 months after the procedure, and repaired
result in improvement in right ventricular size and, in conjunction with CHD with residual defects at the site or adjacent to
intraoperative cryoablation, may reduce the incidence of arrhythmias. the site of a prosthetic device (this would include a
c Percutaneous pulmonary valve replacement shows promise as a potential residual VSD).21 Annual flu shots are recommended
future alternative to surgical pulmonary valve replacement. in patients with chronic cardiovascular disease and
are appropriate in adults with repaired ToF.w25

Heart 2008;94:1663–1669. doi:10.1136/hrt.2008.147249 1667

 Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.com

Education in Heart

asymptomatic patients, surgical consultation is

You can get CPD/CME credits for Education in Heart reasonable when RV dilation is severe, nearing
the volumes described in box 2, or when systolic
Education in Heart articles are accredited by both the UK Royal College of function begins to deteriorate. Finally, patient
Physicians (London) and the European Board for Accreditation in Cardiology— education about the anatomy and physiology of
you need to answer the accompanying multiple choice questions (MCQs). To ToF, expected symptoms, long term prognosis,
access the questions, click on BMJ Learning: Take this module on BMJ diagnostic approaches and therapeutic options is
Learning from the content box at the top right and bottom left of the online essential to ensure that patients return to clinic on
article. For more information please go to: http://heart.bmj.com/misc/education. a regular basis and so they (and their families) can
dtl make informed decisions about their medical care.
c RCP credits: Log your activity in your CPD diary online (http://www.
rcplondon.ac.uk/members/CPDdiary/index.asp)—pass mark is 80%. Competing interests: In compliance with EBAC/EACCME guide-
c EBAC credits: Print out and retain the BMJ Learning certificate once you have lines, all authors participating in Education in Heart have disclosed
potential conflicts of interest that might cause a bias in the article.
completed the MCQs—pass mark is 60%. EBAC/ EACCME Credits can now be The authors have no competing interests.
converted to AMA PRA Category 1 CME Credits and are recognised by all
National Accreditation Authorities in Europe (http://www.ebac-cme.org/
newsite/?hit = men02). REFERENCES
1. Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in
Please note: The MCQs are hosted on BMJ Learning—the best available learning patients undergoing surgical repair of tetralogy of Fallot.
website for medical professionals from the BMJ Group. If prompted, subscribers N Engl J Med 1993;329:593–9.
must sign into Heart with their journal’s username and password. All users must 2. Hoffman JH, Kaplan S, Liberthson RR. Prevalence of congenital
heart disease. Am Heart J 2004;147:425–39.
also complete a one-time registration on BMJ Learning and subsequently log in 3. Marelli AJ, Mackie AS, Ionescu-Ittu R, et al. Congenital heart
(with a BMJ Learning username and password) on every visit. disease in the general population: changing prevalence and age
distribution. Circulation 2007;115:163–72.
c This population based study shows that from 1985 to 2000
there has been an increase in the prevalence and age of
Moderate exercise is encouraged for cardiovascular patients with severe congenital heart disease.
benefit and for self monitoring of changes in 4. Niwa K, Siu SC, Webb GD, et al. Progressive aortic root dilatation
exercise tolerance. However, evaluation by an in adults late after repair of tetralogy of Fallot. Circulation
2002;106:1374–8.
adult congenital heart disease centre is needed 5. Kim WH, Seo JW, Kim SJ, et al. Aortic dissection late after repair
before strenuous or competitive athletics to ensure of tetralogy of Fallot. Int J Cardiol 2005;101:515–16.
high risk residual defects are not present. Patients 6. Tan JL, Gatzoulis MA, Ho SY. Aortic root disease in tetralogy of
Fallot. Curr Opin Cardiol 2006;21:569–72.
are encouraged to contact their physician with any c Review of aortic root dilation in patients with repaired ToF.
symptoms of palpitations, light headedness or 7. Davlouros PA, Kilner PJ, Hornung TS, et al. Right ventricular
decrease in exercise tolerance. function in adults with repaired tetralogy of Fallot assessed with
cardiovascular magnetic resonance imaging: detrimental role of
right ventricular outflow aneurysms or akinesia and adverse right-
to-left ventricular interaction. J Am Coll Cardiol 2002;40:2044–52.
SUGGESTED RECOMMENDATIONS FOR CLINICAL c MRI study showing that pulmonary regurgitant fraction and
MONITORING RV aneurysm/akinesia predict RV dilation.
US guidelines for management of adults with CHD 8. Diller GP, Dimopoulos K, Okonko D, et al. Exercise intolerance in
are not yet available, so the approach used in our adult congenital heart disease: comparative severity, correlates,
and prognostic implication. Circulation 2005;112:828–35.
clinical practice is presented here. The suggested c Important study showing that exercise capacity is reduced
recommendations are based on available data and in patients with congenital heart disease including ToF.
clinical experience in the absence of data. Patients Notably, exercise capacity was limited in patients who
were reportedly asymptomatic. Also, exercise limitation
with repaired ToF optimally are cared for in was similar to patients with chronic heart failure.
conjunction with a recognised CHD centre with 9. Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for
expertise in congenital echocardiography, cardiac arrhythmia and sudden cardiac death late after repair of tetralogy
MRI, CPET and congenital heart surgery. of Fallot: a multicentre study. Lancet 2000;356:975–81.
c Large, multicentre cohort study examining surgical,
Suggestions for clinical monitoring of patients are electrocardiographic and late haemodynamic data as risk
listed in box 2. Generally, stable outpatients are factors for sudden cardiac death.
seen at least annually to assess for any symptoms 10. Babu-Narayan SV, Kilner PJ, Li W, et al. Ventricular fibrosis
suggested by cardiovascular magnetic resonance in adults with
of arrhythmia or change in exercise tolerance. The repaired tetralogy of Fallot and its relationship to adverse markers
QRS duration on the resting ECG is useful in risk of clinical outcome. Circulation 2006;113:405–13.
stratification for arrhythmia, and ambulatory c MRI study demonstrating that ventricular fibrosis detected
by late gadolinium enhancement is common in patients with
monitoring is helpful for detection of symptomatic
repaired ToF. Higher degrees of fibrosis were associated
arrhythmias if indicated by history. with multiple adverse markers of outcome.
Annual imaging is performed with echocardio- 11. Gatzoulis MA, Till JA, Somerville J, et al. Mechanoelectrical
graphy and/or cardiac MRI to quantify and serially interaction in tetralogy of Fallot. QRS prolongation relates to right
ventricular size and predicts malignant ventricular arrhythmias and
follow the degree of RV enlargement and other sudden death. Circulation 1995;92:231–7.
anatomic abnormalities. Cardiac MRI is preferred 12. Garson A Jr, Nihill MR, McNamara DG, et al. Status of the adult
when quantitation of RV volumes and function is and adolescent after repair of tetralogy of Fallot. Circulation
1979;59:1232–40.
needed. Periodic CPET testing provides objective 13. Henkens IR, van Straten A, Schalij MJ, et al. Predicting outcome
measurement of changes in exercise capacity over of pulmonary valve replacement in adult tetralogy of Fallot patients.
time. Referral to a surgeon experienced in CHD is Ann Thorac Surg 2007;83:907–11.
appropriate in symptomatic patients with worsen- 14. Buechel ER, Dave HH, Kellenberger CJ, et al. Remodelling of the
right ventricle after early pulmonary valve replacement in children
ing exercise intolerance, heart failure or significant with repaired tetralogy of Fallot: assessment by cardiovascular
arrhythmias in the setting of a dilated RV. In magnetic resonance. Eur Heart J 2005;26:2721–7.

1668 Heart 2008;94:1663–1669. doi:10.1136/hrt.2008.147249

 Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.com

Education in Heart

15. Oosterhof T, van Straten A, Vliegen HW, et al. Preoperative 19. Therrien J, Siu SC, Harris L, et al. Impact of pulmonary valve
thresholds for pulmonary valve replacement in patients with replacement on arrhythmia propensity late after repair of tetralogy
corrected tetralogy of Fallot using cardiovascular magnetic of Fallot. Circulation 2001;103:2489–94.
resonance. Circulation 2007;116:545–51. c This study addresses whether pulmonary valve replacement
c Relatively large retrospective study (71 patients) looking at for pulmonary regurgitation in patients with repaired ToF
the change in RV volume after pulmonary valve reduces arrhythmias. They showed that pulmonary valve
replacement. No threshold existed above which RV replacement results in stabilisation of QRS duration and (with
volumes did not decrease. Thresholds for normalisation of intraoperative cryoablation) a decrease in the incidence of
RV volumes were determined. pre-existing atrial or ventricular tachyarrhythmias.
16. Therrien J, Provost Y, Merchant N, et al. Optimal timing for 20. Khambadkone S, Coats L, Taylor A, et al. Percutaneous
pulmonary valve replacement in adults after tetralogy of Fallot pulmonary valve implantation in humans: results in 59 consecutive
repair. Am J Cardiol 2005;95:779–82. patients. Circulation 2005;112:1189–97.
17. van Straten A, Vliegen HW, Lamb HJ, et al. Time course of c Clinical results of a percutaneously delivered valved stent
diastolic and systolic function improvement after pulmonary valve into the RV outflow tract.
replacement in adult patients with tetralogy of Fallot. J Am Coll 21. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective
Cardiol 2005;46:1559–64. endocarditis: guidelines from the American Heart Association: a
c Prospective MRI study showing improvement in RV ejection guideline from the American Heart Association Rheumatic Fever,
fraction (corrected for pulmonary regurgitation) after Endocarditis, and Kawasaki Disease Committee, Council on
pulmonary valve replacement. Cardiovascular Disease in the Young, and the Council on Clinical
18. Warner KG, O’Brien PK, Rhodes J, et al. Expanding the indications Cardiology, Council on Cardiovascular Surgery and Anesthesia, and
for pulmonary valve replacement after repair of tetralogy of Fallot. the Quality of Care and Outcomes Research Interdisciplinary
Ann Thorac Surg 2003;76:1066–71; discussion 1071. Working Group. Circulation 2007;116:1736–54.

Multiple choice questions

Education in Heart Interactive (heart.bmj.com/misc/education.dtl)

Education in Heart (EiH) articles each have an accompanying series of six multiple choice questions.
These are hosted on BMJ Learning—the best available learning website for medical professionals from
the BMJ Group. Each article is submitted to EBAC (European Board for Accreditation in Cardiology;
ebac-cme.org) for 1 or 2 hours of external CPD credit.

Free access for subscribers: For full details of the resources available to subscribers please see:
heart.bmj.com/misc/education.dtl#access

How to access the questions: Click on BMJ Learning: Take this module on BMJ Learning from the
online article content box, table of contents or EiH collection (heart.bmj.com/cgi/collection/
heart_education).
If prompted, subscribers must sign into Heart with their journal’s username and password.
Please note: All users must also complete a one-time registration on BMJ Learning. Users will then
subsequently log in (with a BMJ Learning username and password) on every visit in order to log
activity and provide appropriate access.

Activating your subscription to Heart: If you have not yet activated your online subscription to Heart,
please visit journals.bmj.com/cgi/activate/basic and enter your six digit (all numeric) customer number
(found above your address label with your print copy). If you have any queries, please contact
subscriptions@bmjgroup.com

Case based learning: You may also be interested in the cardiology interactive case histories published
in association with Heart, for more information please see: heart.bmj.com/misc/education.dtl#ichs

Heart 2008;94:1663–1669. doi:10.1136/hrt.2008.147249 1669

 Downloaded from http://heart.bmj.com/ on December 10, 2014 - Published by group.bmj.com

Repaired tetralogy of Fallot in the adult:

monitoring and management
Kier V Huehnergarth, Michelle Gurvitz, Karen K Stout and Catherine M
Otto

Heart 2008 94: 1663-1669

doi: 10.1136/hrt.2008.147249

Updated information and services can be found at:

http://heart.bmj.com/content/94/12/1663

Supplementary Supplementary material can be found at:

Material http://heart.bmj.com/content/suppl/2008/10/31/94.12.1663.DC1.html
These include:

References This article cites 21 articles, 11 of which you can access for free at:
http://heart.bmj.com/content/94/12/1663#BIBL

Email alerting Receive free email alerts when new articles cite this article. Sign up in the
service box at the top right corner of the online article.

Topic Articles on similar topics can be found in the following collections

Collections Drugs: cardiovascular system (8040)
Congenital heart disease (664)
Clinical diagnostic tests (4448)
Interventional cardiology (2726)
Aortic valve disease (349)
Right sided valvular heart disease (101)
Epidemiology (3301)
Echocardiography (1942)
Hypertension (2727)

Notes

To request permissions go to:

http://group.bmj.com/group/rights-licensing/permissions

To order reprints go to:

http://journals.bmj.com/cgi/reprintform

To subscribe to BMJ go to:

http://group.bmj.com/subscribe/