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Original Article
Creating a lesion-specific“roadmap”for ambulatory care
following surgery for complex congenital cardiac disease
Gil Wernovsky,
1,2
Stacey L. Lihn,
3
Melissa M. Olen
1
1
The Heart Program at Nicklaus Children’s Hospital, Miami Children’s Health System;
2
Herbert Wertheim College of
Medicine, Florida International University, Miami, Florida;
3
Sisters By Heart, Phoenix, Arizona; National Pediatric
Cardiology Quality Improvement Collaborative, United States of America
Abstract Over the past 20 years, the successes of neonatal and infant surgery have resulted in dramatically changed
demographics in ambulatory cardiology. These school-aged children and young adults have complex and, in some
cases, previously unexpected cardiac and non-cardiac consequences of their surgical and/or transcatheter procedures.
There is a growing need for additional cardiac and non-cardiac subspecialists, and coordination of care may be quite
challenging. In contrast to hospital-based care, where inpatient care protocols are common, and perioperative
expectations are more or less predictable for most children, ambulatory cardiologists have evolved strategies of care
more or less independently, based on their education, training, experience, and individual styles, resulting in highly
variable follow-up strategies. We have proposed a combination proactive–reactive collaborative model with a
patient’s primary cardiologist, primary-care provider, and subspecialists, along with the patient and their family.
The goal is to help standardise data collection in the ambulatory setting, reduce patient and family anxiety, increase
health literacy, measure and address the non-cardiac consequences of complex cardiac disease, and aid in the transition
to self-care as an adult.
Keywords: Ambulatory cardiology; cardiac surgery; guidelines; CHD
Received: 17 May 2016; Accepted: 26 May 2016
Background
In 1980, Fyler et al
1
from the New England Regional
Infant Cardiac Program coined the term “critical
CHD”,defined as CHD severe enough to require
surgery or cardiac catheterisation before 1 year of age.
At that time, the prognosis for many neonates with
critical CHD was grim, because of the following
reasons:
∙stabilisation with prostaglandin E
1
had just been
approved by the Food and Drug Administration in
the United States of America,
∙two-dimensional echocardiography was in its
infancy, and
∙the fields of cardiac intensive care, perfusion,
anaesthesia, and neonatal surgery were less
developed.
Mortality was high following surgery in the
1st month of age, and the longer-term outcomes
were purely speculative. Those with critical CHD
surviving the neonatal period without surgery to reach
infancy frequently received palliative procedures before
definitive correction at a later date; typical examples of
such patients include the following:
∙transposition of the great arteries with a ventricular
septal defect,
∙common arterial trunk,
∙tetralogy of Fallot, and
∙large ventricular septal defects or atrioventricular
septal defects
Over the past 20 years, the successes of neonatal
and infant surgery, including “corrective”procedures
Correspondence to: G. Wernovsky, Nicklaus Children’s Hospital, Miami
Children’s Health System, 3100 SW 62nd Street, Miami, FL 33155, United
States of America. Tel: +1 786 624 3278; Fax: +1 305 666 3078;
E-mail: gwernovsky@gmail.com
Cardiology in the Young 2016; Page 1 of 15 © Cambridge University Press, 2016
doi:10.1017/S1047951116000974
for those with two ventricles and “palliative”
procedures for babies born with a functionally
univentricular heart, have resulted in dramatically
changed demographics in ambulatory cardiology.
These school-aged children and young adults have
complex, and in some cases, previously unexpected
cardiac consequences of their surgical and/or
transcatheter procedures
2–29
(Table 1). Some of these
consequences seem to affect many of these patients to
some degree, such as the following:
∙diminished exercise performance,
∙chronotropic impairment, and
∙a risk of obesity and sedentary lifestyle.
Meanwhile, some of these consequences are lesion
specific, such as the following:
∙arrhythmia,
∙valvar regurgitation, and
∙asymmetric pulmonary blood flow.
In addition, there is a growing recognition of
additional non-cardiac consequences, including the
following:
∙challenges with school performance,
∙social disintegration,
∙anxiety and depression,
∙restrictive lung disease,
∙reactive airways disease,
∙sensorineural hearing loss,
∙reduced health-related quality of life,
∙significant medication burden,
∙post-traumatic stress, for the patient and the
family,
∙anti-social behaviour, and
∙challenges with employment and healthcare
insurance.
For those with a functionally univentricular heart,
additional burdens may include the following:
∙delayed puberty and short stature,
∙thrombosis,
∙renal dysfunction,
∙protein-losing enteropathy,
∙cirrhosis, and
∙plastic bronchitis.
As these interdisciplinary challenges have become
more apparent, ambulatory cardiology has become
increasingly complicated for these patients, and now
consists of much more than simply the following
aspects:
∙physical examination including auscultation,
∙an electrocardiogram,
∙the evaluation of an echocardiogram, and
∙discussion of recommendations.
Table 1. Important longer-term consequences of selected cardiac
surgical procedures in neonates and infants.
All
Unplanned interventions
Chronotropic impairment
Decreased exercise performance
Obesity
Risk of bacterial endocarditis
Restrictive lung disease
Recurrent laryngeal nerve injury
Neurodevelopmental delay
Psychosocial maladjustment
Genetic co-morbidities, if present
Neonatal surgery
Arterial switch operation
Supravalvular pulmonary stenosis and branch pulmonary
artery narrowing
Neo-aortic valve regurgitation
Neo-aortic root dilation
Coronary obstruction or occlusion
Pulmonary hypertension
Residual septal defects
Repair of arch obstruction/interruption with ventricular
septal defect
Residual septal defects
Left ventricular outflow tract obstruction
Residual arch obstruction
Repair of common arterial trunk
Neo-aortic valve stenosis or regurgitation
Neo-aortic root dilation
Conduit obstruction
Branch pulmonary artery narrowing
Pulmonary hypertension
Residual septal defects
Repair of totally anomalous pulmonary venous return
Pulmonary venous obstruction
Pulmonary hypertension
Infant surgery
Repair of atrioventricular septal defect
Residual septal defects
Residual atrioventricular valve regurgitation or stenosis
Left ventricular outflow tract obstruction
Pulmonary hypertension
Repair of tetralogy of Fallot
Residual septal defects
Right ventricular outflow tract obstruction
Pulmonary regurgitation
Branch pulmonary artery narrowing
Aortic root dilation
Aortic regurgitation
Atrial and ventricular arrhythmia
Staged reconstruction for functionally univentricular heart
(Fontan procedure)
Pulmonary artery narrowing
Ventricular dysfunction
Atrioventricular valve regurgitation or stenosis
Veno-venous collaterals
Aortopulmonary collaterals
Ventricular outflow obstruction
Residual arch obstruction
Pulmonary arteriovenous malformations
Atrial arrhythmias
Neo-aortic root dilation and regurgitation
Conduit or venous pathway obstruction
Hypercoagulability
2Cardiology in the Young 2016
There is a growing need for additional cardiac
and non-cardiac subspecialists, and coordination
of care may be quite challenging. Unfortunately,
routine office visits are frequently time constrained,
particularly for those with the most complex
disease and/or multiple cardiac and non-cardiac
consequences. In fact, time may not allow for all
information and counselling to be given. Some
questions of a patient or family may go unaddressed.
Traditionally, ambulatory care has focussed on
health maintenance and management of acute
changes in health, but there is now a growing need
for chronic interdisciplinary care, sometimes termed
the “medical home”. In 2004, Palfrey et al introduced
the concept of paediatric alliance for coordinated care.
The study consisted of a paediatric nurse practitioner
who was assigned as the case manager of children
with special healthcare needs, most with severe
chronic illness and with more than five conditions. In
one study,
30
after 2 years of utilisation of the “medical
home”, evaluations by the family (n =117) of the
“medical home”were obtained and revealed marked
improvement in patient satisfaction scores in the
following areas:
∙a decrease in emergency room visits throughout
the year,
∙a decrease in sick leaves taken from work,
∙improved accessibility to resources,
∙earlier intervention when the child was acutely
ill, and
∙enhanced communication with the medical team.
Current approach to outpatient follow-up
As the focus of outcome studies shifts to the long
term, outcomes in the ambulatory setting play an
increasingly important role. The outcomes measured
in childhood, adolescence, and young adulthood not
only describe the patient cohort but also serve to
inform and modify earlier care strategies; however, in
contrast to hospital-based care, where inpatient care
protocols are common and perioperative expectations
are more or less predictable for most children,
ambulatory cardiologists have evolved strategies
of care more or less independently, based on their
education, training, experience, and individual styles.
Systematic collection of data may occur for specific,
targeted research proposals, typically utilising a
chart review or a cross-sectional study design, with
limitations and biases inherent in those approaches.
The care local cardiologists provide is likely to
vary because there have been a few standards for
these children as there have been little systematically
collected data to inform their care. Instead, clinicians
may be influenced by multiple factors leading to
variability and potential inefficiencies in follow-up
strategies, including the potential for over-testing;
these factors, including recall bias, the struggles
of “that last difficult case”, concern of “missing
something”, or worse the concept of “I had a
patient once who …”. In some developed countries,
there may be financial incentives to perform more
diagnostic testing, whereas in other countries there
may be financial incentives to do just the opposite.
To illustrate the variability in outpatient practices
following surgery for critical CHD, between
10 October, 2006 and 4 November, 2006, the internet
was used to conduct a survey of ambulatory paediatric
cardiologists to determine strategies used for follow-up
after the Fontan operation, arterial switch operation,
and repair of tetralogy of Fallot (previously unpub-
lished data, presented at the Scientific Sessions of the
American Heart Association in 2006). A previously
piloted questionnaire consisting of 43 items was
distributed via an internet listserv (PediHeart) and
known email addresses. Demographics of the survey
respondents (n =434) are shown in Table 2 and results
in Figure 1a–g. Although this survey was conducted
~10 years ago, the results are likely to be similar if
conducted today and show significant practitioner-
based variability in diagnostic testing. Preliminary
analysis of the data suggested that more “senior”
cardiologists performed diagnostic testing less
frequently, but because of the subjective nature of the
responses, and lack of subsequent validation, formal
statistical analysis was not undertaken.
This “case-by-case”approach in the ambulatory
setting, however, has important drawbacks:
∙Unless the cohort is well defined and within one
healthcare network or academic setting, the results
of ambulatory testing may not provide feedback to
the surgical and inpatient team, limiting or
delaying modifications of technique to address
problems.
∙There are often too few patients at any one centre
or practice to inform local care guidelines.
Frequently, follow-up of a surgical cohort is spread
among many different clinicians, frequently in
multiple locations.
∙The cost–benefit analyses of follow-up testing are
difficult if not impossible to undertake.
Table 1. Continued
Plastic bronchitis
Altered bone density
Short stature and delayed puberty
Cirrhosis
Protein-losing enteropathy
Oesophageal varicies
Peripheral venous stasis and varicies
Wernovsky et al: Ambulatory paediatric cardiac care 3
Indeed, in their landmark publication “Crossing
the Quality Chasm”, the Institute of Medicine stated
that “patients should receive care based on the best
available scientific knowledge. Care should not vary
illogically from clinician to clinician or from place to
place”.
31
The report further states that clinicians
and institutions should actively collaborate and
communicate to ensure an appropriate exchange of
information and coordination of care.
Recently, landmark work by the team at Boston
Children’s Hospital has resulted in the development of
standardized clinical assessment and management
plans –“SCAMPs”.
32–37
This initiative was produced
as a means to reduce variation in practice and promote
standardisation, with the ability to individualise the
care provided, and thereby provide a means for
ongoing modification of the plan. This breakthrough
approach to care was first initiated for the care
of children with critical CHD due to variation in
cardiology practices. Similar to this approach, the goal
of a “roadmap”approach is to decrease the variability
in medical practice, while allowing for individual
variation in practice and collection of data. In most
non-academic settings, collection of data and evalua-
tion of strategies of surgical care may face challenges
when multiple cardiologists throughout the commu-
nity provide postoperative care, a care model that
centres on individual providers rather than centres.
Developing a roadmap for children born with critical
CHD is meant to be complimentary to a “SCAMP”
approach, and it is community-based as well as
patient-based approach, such a roadmap will provide
the following:
∙pre-set parental expectations for the type and
timing of routine surveillance,
∙an understanding of the expected consequences of
surgery for critical CHD, and
∙a framework for screening that will be necessary
over the lifetime of the patient.
In 2006, following meetings to determine consensus
at the Children’s Hospital of Philadelphia, establishing
follow-up guidelines for patients with critical CHD
was attempted,
38
but the proposal did not meet with
sustained success. As has been previously described
by Cabana et al, the inertia of the individual practice
was, and is, hard to overcome.
36,37,39,40
Anumberof
additional barriers became apparent, as in many
guidelines, including the following:
∙provider lack of agreement,
∙resistance or lack of knowledge of the guidelines,
∙an inadequate database for tracking outcomes, and
∙at the time, an electronic health record with only
rudimentary capabilities to identify patients who
would best be served by these guidelines and the
appropriate intervals for follow-up and testing.
In addition, the plan may have been too ambitious,
with yearly or biannual recommendations of diag-
nostic testing. Many of these factors have changed in
the last decade, as bioinformatics and the electronic
health record have matured.
Lost in all of this variability of management have
been the patient and family. Many go from year to
year, hoping nothing will “come up”during the
annual or biannual visit, reassured by the “see you
next year”and “everything looks great”sometimes
paternalistic style of medicine. Results of tests may not
be communicated in a manner that is understood.
Barriers of language may exist. Health literacy, a key
component to improved health-related quality of life
and improved medical compliance, is quite variable
across patients, and may be related to socio-economic
status as well as the interest and experience of the
provider. The anxiety of the annual check-up can be
severe at times, leading to incomplete “hearing”and
processing of the information presented. Casually
stating that a new test “might be helpful”can be
received in a variety of ways, but in our experience may
lead to anxiety, uncertainty, and worry, and some
families re-live the trauma of the initial diagnosis and
uncertainty of the future. Transition programmes to
self-care are sparse or non-existent in some areas,
18
although online resources are recently available.
41
Consultants for the non-cardiac consequences are
Table 2. Survey respondents (n =434).
Location
United States of America
North-east 104 (24.4%)
Mid-Atlantic 44 (10.1%)
South-east 85 (19.8%)
Mid-west 54 (12.4%)
South 16 (3.7%)
North-west 16 (3.7%)
West 41 (9.4%)
Outside United States of America 74 (17.1%)
Practice type
Hospital faculty 266 (58.6%)
Private practice 135 (31.1%)
Fellowship 33 (7.3%)
Years in practice
Fellow 31 (7.3%)
<5 years 72 (15.9%)
5–10 years 78 (17.2%)
10–15 years 82 (18.1%)
15–20 years 70 (15.4%)
>20 years 101 (23.3%)
Average number of outpatients per week
<10 58 (13.4%)
10–20 180 (41.5%)
21–30 105 (24.2%)
>30 91 (21.0%)
4Cardiology in the Young 2016
0
100
200
300
400
Fallot
Electrocardiogram
Echocardiogram
Holter Monitor
Magnetic Resonance Imaging
Pulse Oximetry
Exercise Testing
Chest Radiograph
Arterial Switch Fontan Fallot Arterial Switch Fontan
Rarely Occasionally Frequently Almost Always
Fallot Arterial Switch Fontan
Fallot Arterial Switch Fontan
Rarely Occasionally Frequently Almost Always
Fallot Arterial Switch Fontan
Rarely Occasionally Frequently Almost Always
Rarely Occasionally Frequently Almost Always
0
100
200
300
400
0
100
200
300
400
0
100
200
300
400
0
100
200
300
400
Other Yearly Every 2-4 Years Every 5-10 Years
Case by Case Basis
Other Yearly Every 2-4 Years Every 5-10 Years
Case by Case Basis
No Access Yearly Every 2-4 Years Every 5-10 Years
Case by Case Basis
Fallot Arterial Switch Fontan
Fallot Arterial Switch Fontan
0
100
200
300
400
0
100
200
300
400
(a)(b)
(c)(d)
(e)
(g)
(f)
Figure 1.
Results from an internet survey conducted in 2006 regarding frequency of diagnostic testing following surgery for tetralogy of Fallot, the
arterial switch operation, and Fontan palliation for functionally univentricular heart. See text for details. Number of respondents are noted on
the y-axis.
Wernovsky et al: Ambulatory paediatric cardiac care 5
frequently at a disadvantage. Often, these consultants
may never have seen a child or young adult with
the particular critical CHD and may not see the
relationship of the problem in “their organ system”to
the underlying critical CHD. This is particularly
problematic in patients with neurodevelopmental
challenges and in those with a functionally
univentricular heart and multi-system consequences.
Strategy
We have proposed a combination proactive–reactive
collaborative model with a patient’s primary cardi-
ologist, primary-care provider, and family in order to
address the following aspects:
∙the complexities of critical CHD consequences, or
“complications”as they are sometimes referred to,
∙the variability of the timing and severity of
presentation, and
∙the need for non-cardiac specialists.
To illustrate the combination proactive–reactive
model, we frequently use the analogy of planned
maintenance in the automotive industry. When we
purchase an automobile, we are given a suggested
maintenance schedule, a plan of surveillance for
potential mechanical issues that are suggested at
predictable intervals. This represents a “proactive”
strategy, usually based on time or miles or kilometres
driven, and not because the owner identifies a problem.
Not each “check-up”is comprehensive, but at
important epochs –for example, at 100,000 km –a
very comprehensive evaluation is performed, in
contrast to a less-complete evaluation performed every
10,000 km. If the owner senses something unusual in
the drive of the automobile, or the wipers need
changing, a “reactive”strategy is always available, and
is analogous to an unplanned visit to the cardiologist.
Many families have embraced this approach, with
“major”check-ups occurring infrequently but
coordinated by the surgical centres –“dealership”–
and less comprehensive and/or unplanned visits
occurring locally –“the local expert mechanic”.This
strategy results in a number of major benefits:
∙from the time of the surgical procedure, the family
knows what to expect moving forward, when
certain tests will be ordered, what the purpose of
the test is, and what will be done with the results,
∙the primary-care provider and primary cardiologist
direct these evaluations locally whenever possible,
communicating the results with the surgical
centre, and
∙over time consistency in follow-up creates a
database of “expected”findings and consequences
for each particular critical CHD.
The roadmap: standardised testing and routine
surveillance –“STARS”–for critical CHD
A good deal of thought was put into the frequency of
surveillance testing. These recommendations are
likely to be different in different scenarios, including
the following:
∙different models of care
∙different socio-economic situations,
∙rural versus urban settings,
∙free-market health care versus single-payer national
health coverage, and
∙many more.
Nevertheless, it seems reasonable from a develop-
mental perspective, fiscal perspective, as well as exten-
sive review of the literature and discussion with families
that five planned visits between surgery and transition
to self-care as an adult is a good starting point (see Fig
2). Importantly, these recommended comprehensive
evaluations include non-cardiac evaluations and
recommendations. We advocate comprehensive eva-
luations at the following five time points:
∙at the first birthday,
∙upon entering elementary education (~5–6 years
of age),
∙between elementary and middle school (~10–11 years
of age),
∙during transition between middle school and high
school (~14–15 years of age), and
∙upon transfer to adult care (~18–21 years of age).
These times are just starting points for discussion,
but setting up a lifelong plan for patients and their
families has multiple advantages –such a “roadmap”
has the following advantages:
∙manages expectations and anxiety,
∙leads to in-depth conversations about the critical
CHD and current status of the repair, improving
health literacy,
∙emphasises the need for lifelong care, particularly if
introduced early in follow-up,
∙emphasises and evaluates the safety and importance
of exercise
42,43
and primary prevention of athero-
sclerotic cardiovascular disease,
44,45
∙leads to simultaneous investigations of other organ
systems, particularly neurodevelopment, as recently
recommended by the American Heart Association
and American Academy of Pediatrics,
46
and
∙may aid in transition and self-reliance.
18,47
Following the comprehensive “100,000 km check-
up”, a full summary of the results, including
interpretation, is reviewed with the patient’s primary
cardiologist, primary-care provider, and family, with
all questions addressed as best as possible.
6Cardiology in the Young 2016
Examples of a potential roadmap, including non-
cardiac and cardiac evaluations, are included in the
Appendix. Note that for each lesion there are specific
tests for known consequences specific to the repair, as
well as more general screens for consequences such as
the following:
∙exercise performance,
∙level of activity,
∙arrhythmia,
∙obesity,
∙neurodevelopment, and
∙general health status.
It is also important to recognise and emphasise the
importance of the 1st year of life following surgery for
critical CHD. Some of these patients remain physiolo-
gically fragile following palliative or corrective proce-
dures. Many, particularly those requiring intervention
in the 1st weeks of life, have a higher incidence
of challenges with feeding, nutrition, heart failure,
developmental delay, medication burden, and a
heightened level of family stress and anxiety. For
neonates, with a functionally univentricular heart,
many programmes have instituted an intensified
level of ambulatory surveillance and support with
“interstage monitoring programmes”, but we would
argue that the same level of support and inter-
disciplinary involvement should be extended to infants
with biventricular repairs, particularly if there are
multi-system challenges as mentioned above. Setting
up the framework and expectations for interdisciplinary
follow-up and family support should start immediately
after the surgical repair and continue throughout
childhood. Critical in this process is a structured “hand-
off”from the surgical centre to an interdisciplinary
team, led by the child’s primary cardiologist and
primary-care provider.
One may argue that it is over ambitious, or perhaps
not indicated, for the cardiology programme where
surgery was performed to provide the medical home
for these patients. Some may feel that it is not the
responsibility of the primary cardiologist to coordi-
nate the evaluation of non-cardiac domains, includ-
ing the following:
∙neurodevelopment,
∙growth failure,
∙sedentary behaviours, and
∙lack of separation from parents.
We disagree. Although it is certainly not the
responsibility of the cardiologist at the surgical centre
to provide all of these services, we feel it is the
responsibility of the child’s cardiologist to coordinate
the care team, and know what colleagues need to
collaborate in order to provide the medical home,
along with the child’s primary-care physician,
primary cardiologist, and family. The cardiology
team best knows the mid-term and longer-term
consequences of heart surgery, and we feel they are
responsible for educating the rest of the stakeholders
in the care of the patient, including psychosocial and
mental health support as indicated. This strategy is
precisely the model used for inpatient care, where one
individual is not responsible for all aspects of
Figure 2.
Schematic representation of “the roadmap”.
Wernovsky et al: Ambulatory paediatric cardiac care 7
optimal care, such as surgery, imaging, nutrition,
nursing, anaesthesia, etc.; rather, a team is assembled
to provide the best possible outcome utilising
the expertise of multiple different individuals.
This strategy of care is the model we propose to
bring to the ambulatory setting. In addition to
educating the rest of the care team, members
of the medical home team should provide the
patient and family education in the ambulatory
setting to assist in real-time understanding of mul-
tiple topics:
∙the name and anatomy of the critical CHD,
∙the interventions performed,
∙the current cardiovascular assessment,
∙the potential long-term consequences of the
procedures, and
∙the importance of transition to self-care.
Models implementing advanced practice nursing
after discharge from critical CHD surgery have been
shown to reduce maternal worry as well as improve
outcomes of infants, and it is our expectation that
similar benefits would be seen over time in the
ambulatory setting.
48
Finally, it is our hope that this model will provide
the following advantages:
∙will stimulate discussion and information sharing
between centres, practitioners, patients, and
families,
∙be modified constantly over time, and
∙potentially adopted by other invested parties such
as the Society of Thoracic Surgeons and European
Association for Cardio-Thoracic Surgery.
These groups have collaborated for a number of
years to create very large databases to examine
short-term outcomes. Their results have been
impressive.
49–52
Adding a longer-term care module
will allow improved feedback on surgical and in-
hospital strategies of care and speed up the process
of collaborative learning across centres. Reliance
solely on perioperative studies to improve outcomes
is helpful but short-sighted. Similarly, reliance on
outpatient cardiology appointments with highly
variable follow-up strategies to determine the
“success”of cardiac surgery for critical CHD is
inconsistent and incomplete. Ambulatory strategies
must evolve for continued improvement in
outcomes; tens of thousands of data points are being
wasted.
We anticipate the following aspects:
∙some will disagree with this strategy,
∙additional technology and/or later findings will
change these recommendations,
∙many, if not most, will disagree with the exact type
and timing of testing, and
∙implementation will necessarily be variable across
centres and geography depending upon interest
and resources.
To our knowledge, routine, standardised follow-
up protocols do not exist for most critical CHD,
although there is increasing interest in standardisa-
tion for patients with a univentricular heart, includ-
ing at institutions such as Children’s Healthcare of
Atlanta (Mahle W, personal communication), Lucille
Packard Children’s Hospital (Wright G, personal
communication), and undoubtedly others. Much
of what we have learnt in this regard is based on
the recognition of multi-system consequences of the
Fontan procedure from the seminal work from
the Single Ventricle Survivorship Program at the
Children’s Hospital of Philadelphia.
53–58
We spec-
ulate that other forms of critical CHD, particularly
with residual physiological perturbations such as in
tetralogy of Fallot, may also have as-yet-to-be-defined
multi-system consequences as well; only systematic
follow-up of large cohorts of patients will identify
these potential morbidities. Our roadmap put forth
here for patients with a univentricular heart,
although similar, is somewhat different than that
recently proposed by Rychik,
7
both in terms of fre-
quency of testing and by non-inclusion of standar-
dised invasive testing. Which protocol is “right”,
most cost-effective, or likely to become standardised
remains to be seen; however, we must start
somewhere.
The authors, as well as the editors of Cardiology
in the Young, welcome contrary and conflicting
opinions and look forward to the dialogue.
Acknowledgements
The authors thank Chitra Ravishankar, MBBS,
Girish Shirali, MBBS, Carole M. Lannon, MD, MPH,
and Andrea Baer (mendedlittlehearts.org) for their
critical review of the manuscript and thoughtful
comments during the preparation. The authors also
thank their colleagues in Cardiology and Cardiac
Surgery at the Nicklaus Children’s Hospital of the
Miami Children’s Health System for their enthusiasm
of the concept and their support.
Financial Support
The research received no specific grant from any
funding agency, commercial, or not-for-profit sectors.
Conflicts of Interest
None.
8Cardiology in the Young 2016
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10 Cardiology in the Young 2016
Appendix
Roadmap following arterial switch operation
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous arrhythmia
or other concerns
Holter Holter Holter Holter
MRI or perfusion scan if echo
suggests asymmetric
pulmonary blood flow
MRI or perfusion scan if echo
suggests asymmetric
pulmonary blood flow
MRI MRI MRI
6-minute walk test or exercise
testing without metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Lipid panel Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness evaluation School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional evaluation Nutritional evaluation Nutritional evaluation;
diet and healthy
lifestyle consultation
Nutritional evaluation;
diet and healthy lifestyle
consultation
Nutritional evaluation;
diet and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health
literacy counselling
Self-care and health
literacy counselling
Self-care and health
literacy counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Roadmap following repair of total anomalous pulmonary venous return
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter Holter Holter Holter Holter
MRI MRI
6-minute walk test or
exercise testing without
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness
evaluation
School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional
evaluation
Nutritional evaluation Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health literacy
counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Wernovsky et al: Ambulatory paediatric cardiac care 11
Roadmap following repair of ventricular septal defect with arch obstruction or interruption
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous
arrhythmia or other
concerns
Holter if previous
arrhythmia or other
concerns
Holter Holter Holter
MRI or CT if arch or
LVOT obstruction is
suspected
MRI or CT if arch or
LVOT obstruction is
suspected
MRI MRI MRI
6-minute walk test or
exercise testing without
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness
evaluation
School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional evaluation Nutritional evaluation Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health literacy
counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram; LVOT =left ventricular outflow tract
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Roadmap following repair of common arterial trunk
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous arrhythmia
or other concerns
Holter if previous
arrhythmia or other
concerns
Holter Holter Holter
MRI or perfusion scan if echo
suggests asymmetric
pulmonary blood flow
MRI or CT as indicated
based on
echocardiogram
MRI MRI MRI
6-minute walk test or
exercise testing
without metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness
evaluation
School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional evaluation Nutritional evaluation Nutritional evaluation;
diet and healthy lifestyle
consultation
Nutritional evaluation;
diet and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health
literacy counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
12 Cardiology in the Young 2016
Roadmap following repair of tetralogy of Fallot
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous arrhythmia
or other concerns
Holter if prior arrhythmia or
other concerns
Holter Holter Holter
MRI or perfusion scan if echo
suggests asymmetric
pulmonary blood flow
MRI or perfusion scan if echo
suggests asymmetric
pulmonary blood flow
MRI MRI MRI
6-minute walk test or exercise
testing without metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness evaluation School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional evaluation Nutritional evaluation Nutritional evaluation;
diet and healthy
lifestyle consultation
Nutritional evaluation;
diet and healthy lifestyle
consultation
Nutritional evaluation;
diet and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health
literacy counselling
Self-care and health
literacy counselling
Self-care and health
literacy counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Roadmap following repair of ventricular septal defect
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous
arrhythmia or other
concerns
Holter Holter
Exercise testing with
metabolics
Exercise testing with metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic panel,
lipid panel
Neurodevelopmental
evaluation
School readiness
evaluation
School performance evaluation School performance evaluation;
career and vocational
counselling
Career and vocational counselling
Nutritional evaluation Nutritional
evaluation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet and
healthy lifestyle consultation
Nutritional evaluation; diet and
healthy lifestyle consultation
Exercise
prescription
Exercise prescription Exercise prescription Exercise prescription
Self-care and health literacy
counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Wernovsky et al: Ambulatory paediatric cardiac care 13
Roadmap following repair of atrioventricular septal defect
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous
arrhythmia or other
concerns
Holter Holter
MRI (if >mild
atrioventricular valvar
regurgitation)
MRI (if >mild
atrioventricular valvar
regurgitation)
MRI
6-minute walk test or
exercise testing without
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness
evaluation
School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional
evaluation
Nutritional evaluation Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health literacy
counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Roadmap following repair of neonatal repairs (other)
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous
arrhythmia or other
concerns
Holter if previous
arrhythmia or other
concerns
Holter Holter Holter
MRI (as indicated based on
repair)
MRI (as indicated based on
repair)
MRI (as indicated based on
repair)
6-minute walk test or
exercise testing without
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness
evaluation
School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional
evaluation
Nutritional evaluation Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health literacy
counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
14 Cardiology in the Young 2016
Roadmap following repair of infant repairs (other)
1 year Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter if previous
arrhythmia or other
concerns
Holter if previous
arrhythmia or other
concerns
Holter Holter if previous arrhythmia
or other concerns
Holter
MRI (as indicated based on
repair)
MRI (as indicated based on
repair)
MRI (as indicated based on
repair)
6-minute walk test or
exercise testing without
metabolics
Exercise testing with
metabolics
Exercise testing with
metabolics
Screening cholesterol Complete blood count,
comprehensive metabolic
panel, lipid panel
Neurodevelopmental
evaluation
School readiness
evaluation
School performance
evaluation
School performance
evaluation; career and
vocational counselling
Career and vocational
counselling
Nutritional
evaluation
Nutritional evaluation Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Nutritional evaluation; diet
and healthy lifestyle
consultation
Exercise prescription Exercise prescription Exercise prescription Exercise prescription
Self-care and health literacy
counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Roadmap following staged reconstruction for univentricular heart (Fontan)
1 year Pre-Fontan Pre-school Pre-middle school Pre-high school Pre-transfer to ACHD
ECG ECG ECG ECG ECG ECG
Echocardiogram Echocardiogram Echocardiogram Echocardiogram Echocardiogram
Holter Holter Holter Holter Holter Holter
MRI MRI MRI
6-minute walk test 6-minute walk test Exercise testing with metabolics Exercise testing with metabolics Exercise testing with metabolics
Liver ultrasound Liver ultrasound Liver ultrasound
Bone scan
Complete blood
count, PT/INR,
comprehensive
metabolic panel
Complete blood
count, PT/INR,
comprehensive
metabolic panel
Complete blood count, screening
cholesterol, PT/INR,
comprehensive metabolic panel,
thyroid function tests, growth
hormone, vitamin D
Complete blood count, PT/INR,
comprehensive metabolic panel,
thyroid function tests, growth
hormone, vitamin D
Complete blood count, PT/INR,
comprehensive metabolic and
lipid panel, thyroid function tests,
growth hormone, vitamin D
Neurodevelopmental
evaluation
School readiness
evaluation
School performance evaluation School performance evaluation;
career and vocational
counselling
Career and vocational counselling
Nutritional
evaluation
Nutritional
evaluation
Nutritional evaluation; diet and
healthy lifestyle consultation
Nutritional evaluation; diet and
healthy lifestyle consultation
Nutritional evaluation; diet and
healthy lifestyle consultation
Exercise
prescription
Exercise prescription Exercise prescription Exercise prescription
Self-care and health literacy
counselling
Self-care and health literacy
counselling
Self-care and health literacy
counselling
ACHD =adult congenital heart disease; ECG =electrocardiogram; PT/INR =prothrombin time/international normalized ratio
Testing and routine follow-up at the discretion of primary cardiologists in between roadmap evaluations
Neurodevelopmental evaluations are ongoing following infancy based on current professional guidelines
40
Exercise prescriptions, self-care, and health literacy counselling are ongoing elements of transition to adult care
Wernovsky et al: Ambulatory paediatric cardiac care 15
