REVIEW

CURRENT
OPINION Single-ventricle infant home monitoring programs:
outcomes and impact
David A. Hehir a,b and Nancy S. Ghanayem b

Purpose of review
Patients with single-ventricle, shunt-dependent physiology are at increased risk for interstage death due to
the inherent instability of parallel circulation. Enhanced surveillance and early identification of deteriorating
physiology via interstage home monitoring result in significant reduction in mortality. These programs are
an important focus of improving outcomes for patients with single-ventricle heart disease.
Recent findings
In the multi-institutional Pediatric Heart Network Single-Ventricle Reconstruction Trial, interstage mortality
was 12%, highlighting the continued opportunity to improve on this metric. A number of single-center series
have demonstrated significant benefit of interstage monitoring on survival and growth. The focus on
interstage monitoring by the National Pediatric Cardiology Quality Improvement Collaborative of the Joint
Council on Congenital Heart Disease should improve our understanding of patients at greatest risk and
help establish best practices for interstage care. In addition, a number of pilot projects utilizing newer
communication technologies seek to improve the connection between program and patient.
Summary
Interstage home monitoring programs are a model of collaborative care that improves outcomes. Continued
research in this area will refine the elements of home monitoring programs and continue to guide improved
results. In addition, this model may serve as a template for the care of other populations of medically
complex infants.
Keywords
congenital heart disease, hypoplastic left heart syndrome, interstage, single ventricle

INTRODUCTION monitoring, and summarize recommendations for

Since their initial description in 2003, the success of
home monitoring programs (HMPs) in reducing
interstage mortality for infants with single-ventricle
heart disease has been reproduced widely [1]. As a
result, the development of HMPs at institutions
caring for children with single-ventricle heart dis-
ease has become a critical component of patient
care. The interstage period between discharge fol-
lowing stage 1 Norwood palliation (S1P) and admis-
sion for stage 2 palliation (S2P) represents a time of
high risk due to circulatory instability inherent to
shunt-dependent physiology. Causes of interstage
mortality are multifactorial and unlikely to be
improved by implementing a therapeutic strategy
that targets a single cause [2,3]. The goal of inter-
stage monitoring is therefore to provide heightened
surveillance of physiologic instability that may
precede important hemodynamic deterioration,
allowing early intervention and prevention of
potentially fatal interstage events. We review the
recently published literature related to interstage
the structure of an effective HMP.
RISK FACTORS FOR INTERSTAGE
MORTALITY
Previous studies have identified a wide range of risk
factors for interstage mortality, including anatomic,
demographic, and physiologic features [2–5]. In the
only multicenter study of risk factors for interstage
mortality, Ghanayem et al. analyzed the Pediatric
Heart Network Single Ventricle Reconstruction trial
(SVR) database. The authors found gestational age
a
Division of Pediatric Cardiology and bDivision of Pediatric Critical Care
Medicine, Children’s Hospital of Wisconsin and Medical College of
Wisconsin, Milwaukee, Wisconsin, USA
Correspondence to David A. Hehir, MD, 9000 West Wisconsin Avenue,
Children’s Hospital of Wisconsin, Milwaukee, WI 53226, USA. Tel: +1
414 266 5711; e-mail: dhehir@mcw.edu
Curr Opin Cardiol 2013, 28:97–102
DOI:10.1097/HCO.0b013e32835dceaf

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 Pediatrics
KEY POINTS

Individual centers have reduced interstage mortality to
0% after initiating a home monitoring program (HMP);
the average change following institution of a HMP in
published series is a reduction from 15.7 to 5.7%.

Effective HMPs consist of daily parental recording of
oxygen saturation, weight, and enteral intake
combined with regular communication and clinic visits
with HMP staff.

HMP use is associated with benefits beyond survival,
including improved growth and reduced family stress.

The HMP concept may be generalized to other high-risk
CHD populations, and is a model of successful family-
centered, multidisciplinary care.
less than 37 weeks [odds ratio (OR) 3.9, P ¼ 0.03],
Hispanic ethnicity (OR 2.6, P ¼ 0.04), aortic atresia/
mitral atresia (OR 2.3, P ¼ 0.03), greater number of
post-Norwood complications (OR 1.2, P ¼ 0.006),
census block poverty level (P ¼ 0.03), and those with
a Blalock-Taussig shunt (BTS) in the setting of less
than moderate atrio-ventricular valve regurgitation
(OR 9.7, P < 0.001) to be at higher risk of interstage
&&
death [6 ,7]. In univariate analysis, feeding issues
were significant risk factors for mortality, with those
who did not eat by mouth at the time of discharge
and those using a nasogastric vs. a gastrostomy tube
found to be at higher risk.
There is no clear agreement between studies of
which anatomic subtypes present the greatest risk
for interstage mortality. Patients with aortic atresia
have been considered at higher risk at every stage
when compared with aortic stenosis, both due to
technical considerations of the repair and from an
increased risk of coronary insufficiency. While the
aortic atresia/mitral stenosis subtype has been found
to be a higher-risk group in some studies, Polime-
nakos et al. found that, in 100 consecutive patients,
there was no increased hospital or interstage
mortality in this group, despite the identification
of ventriculo-coronary connections in 56% [8,9].
The authors found interstage mortality more com-
monly associated with intact atrial septum and
significant renal dysfunction following S1P. The
presence of intact or highly restrictive atrial septum
was also found to be a significant risk factor for
interstage death (OR 7.6, P ¼ 0.003) in the largest
single-center analysis of interstage risk factors, high-
lighting the ongoing impact of restriction at the
level of pulmonary venous egress throughout staged
palliation [2].
A number of single-center studies have shown a
survival advantage during the interstage period with
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the right ventricle to pulmonary artery (RV-PA)
conduit, a result borne out by the SVR trial with
higher interstage mortality in the modified BTS
&& &
group (6 vs. 18%) [6 ,10 ,11]. In a single-center
comparison of survival by shunt type, Ruffer et al.
&
[10 ] found survival at 120 days to be significantly
higher in the RV-PA conduit group (92 vs. 63%). The
authors also noted that, although there was no differ-
ence in interstage death between groups (5 vs. 4),
most deaths in the RV-PA conduit group occurred
after more than 120 days, therefore concluding that
the survival advantage of the RV-PA conduit may be
enhanced through earlier S2P timing.
The relationship of arrhythmia to interstage
mortality is unclear. Hehir et al. [2] found those
with a clinically relevant arrhythmia following
S1P to be at higher risk for interstage death. Trivedi
et al. [12] recently looked at this question in more
detail, finding a surprisingly high incidence of
arrhythmias between S1P and S2P (57%). Although
mortality was not found to be higher in the group
with arrhythmia of any kind, those with persistent
bradycardia including sinus node dysfunction and
high-grade heart block had the worst outcome, with
73% mortality.
It can be seen from the above discussion that
variables which influence the risk of interstage
mortality are many and varied, and, with the
possible exception of shunt type, are not readily
modifiable. While the above factors contribute to
the ultimate cause of interstage mortality, in most
cases they are not the proximate cause; rather it is
often a simple intercurrent illness or other physio-
logic stressor which destabilizes the at-risk patient,
leading to an interstage event. Therefore, an effec-
tive HMP focuses on early assessment and interven-
tion in patients with common physiologic signs
of early deterioration, rather than attempting to
reverse or prevent the myriad nonmodifiable risk
factors identified in the literature.
STUDIES REPORTING RESULTS OF HOME
MONITORING PROGRAMS
Structured interstage home monitoring was first
reported by Ghanayem et al. [1] nearly a decade
ago, with a reduction in the incidence of interstage
mortality from 15.8 to 0%. Interstage home
monitoring has since been accepted by many as
standard of care after staged palliation of single-
&&
ventricle lesions [13 ]. Recently, the National
Pediatric Cardiology Quality Improvement Collab-
orative (NPC-QIC) of the Joint Council on Congen-
ital Heart Disease has made the reduction of
interstage mortality its primary mission, and has
involved 47 centers in a collaborative approach to
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March 2013

Table 1. Studies reporting interstage mortality and impact of HMP
First author and study Period
Mahle 2000 (CHOP) 1984–1999
Ghanayem 2003 (CHW) 1996–2001
Fenton 2003 (PITT) 1991–2000
Hehir 2009 (CHOP) 1998–2005
Furck 2010 (Kiel) 1996–2007
Hansen 2011 (Kiel) 1996–2009
Debrolet 2011 (Miami) 2006–2010
Petit 2011 (TCH) 2007–2010
Husain 2012 (NCH) 2006–2011
Ghanayem 2012 (SVR) 2005–2008
CHOP, Children’s Hospital of Philadelphia; CHW, Children’s Hospital of Wisconsin; HMP, home monitoring program; Kiel, University Hospital Schleswig-
Holstein, Kiel, Germany; Miami, Miami Children’s Hospital and Arnold Palmer Children’s Hospital, Miami; NCH, Nationwide Children’s Hospital, Columbus,
Ohio; PITT, Children’s Hospital of Pittsburgh; SVR, Single Ventricle Reconstruction Trial; TCH, Texas Children’s Hospital [1,2,4,6 ,15,16,17 ,18 ,19 ,20].
gathering data on interstage practices with the goal
of reducing interstage mortality nationally [14 ].
Centers demonstrating improved results with
initiation of home monitoring are for the most part
modeled on the original program consisting of daily
parental recording of oxygen saturation, weight and
intake, paired with frequent contact with HMP staff
and visits to a centralized HMP clinic. Table 1 lists
published rates of interstage mortality and the
&& &&
impact of HMPs [1,2,4,6 ,15,16,17 ,18 ,19 ,20].
On average, interstage mortality in the absence of
a HMP is 13.8% across these studies. In those studies
which evaluated the impact of instituting a HMP,
interstage mortality was reduced on average from
15.7 to 5.7%, with two programs reporting no
mortality after HMP.
A number of studies have expanded the popu-
lation enrolled in HMPs beyond those undergoing
the Norwood procedure. Dobrolet et al. [18 ]
reported the success of a HMP in an expanded
cohort of ‘high-risk’ infants with shunt-dependent
lesions. In the study group, there was only one
interstage death in a shunted patient with unbal-
anced atrio-ventricular canal and no deaths in the
Norwood group, compared with 6% interstage
mortality in the historical controls. In this study,
11 of 12 patients found to have significant residual
cardiac lesions during the interstage period were
identified as a direct result of a breach of HMP
criteria, including violation of prescribed oxygen
saturation and/or weight gain parameters. This
allowed careful interventional planning and avoid-
ance of the potential for sudden hemodynamic
collapse at home. The authors noted that, prior to
the development of their HMP, care was scattered
across multiple referring physicians covering a large
geographic area, and lacked consistency.
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Single-ventricle infant home monitoring programs Hehir and Ghanayem
Population (n) Results/Comments
840 Interstage death ¼ 13.9% (no HMP)
87 Pre-HMP ¼ 15.8%; HMP ¼ 0%
146 Interstage death ¼ 14% (no HMP)
368 Interstage death ¼ 10.5% (no HMP)
157 Pre-HMP ¼ 15.8%; HMP ¼ 0%
187 Pre-HMP ¼ 12.4%; HMP ¼ 2.2%
59 Control ¼ 6%; HMP group ¼ 3%
230 Pre-HMP ¼ 12%; HMP ¼ 8%
51 Non-HMP ¼ 26%; HMP ¼ 21%
426, multicenter Interstage death ¼ 12%
&& && & &
One of the purported benefits of a centralized
&&
HMP clinic is the reduction of practice pattern vari-
ation between multiple providers. Many authors
point to variability in medication prescription, feed-
ing practices, and surgical timing as a potential
factor in the high rates of interstage mortality.
&&
Schidlow et al. [14 ] found little consistency in
interstage care in an analysis of interstage practices
of 21 centers across the US. Home interstage surveil-
& &
lance was used in 81/100 patients enrolled, with 77
using both oxygen saturation and weight monitor-
ing and four using only oxygen saturation. The
greatest variability was seen in the frequency and
manner of monitoring, ranging from twice daily to
weekly recording of saturation and weight. This
study also highlighted the high degree of variability
in care provider communication, with only 45% of
referring cardiologists and 26% of primary care
&
physicians receiving complete communication
(written medication list, nutrition plan and ‘red flag’
checklist) upon hospital discharge of interstage
patients.
As the role of the HMP has expanded, some
studies have identified continued unacceptable inter-
stage mortality, particularly in high-risk groups. In an
analysis of the impact of a HMP in patients under-
going an initial hybrid S1P, Husain et al. [20] found
high mortality in both the HMP and control groups.
However, the authors note the potential for selection
bias due to the HMP group containing a greater
proportion of high-risk patients. Of concern, the
authors found those who experienced a ‘breach’ in
monitoring criteria were no more likely to have an
adverse event, calling into question the predictive
value of the standard HMP criteria in this high-risk
&
population. Petit et al. [19 ] was not able to show a
statistically significant improvement in interstage
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 Pediatrics
mortality following institution of a HMP. However,
1-year survival did improve during the study period,
and interstage growth velocity significantly
improved as a result of the HMP allowing earlier
S2P at a similar weight to historical controls [19 ].
Although these studies failed to demonstrate statisti-
cally significant improvement in interstage mortality
with use of a HMP, they have an important role in
highlighting the challenges in further decreasing
interstage mortality, and identifying high-risk groups
who will most benefit from continued innovation
and improvement in interstage management.
HOME MONITORING PROGRAM AND
TIMING OF STAGE 2 PALLIATION, LENGTH
OF STAY, AND READMISSIONS
A potential role of the HMP is in informing the ideal
timing of S2P for an individual patient. A number of
authors have reported a trend towards earlier timing
& &&
of S2P after the initiation of a HMP [1,19 ,21–23].
The optimal timing of S2P remains a healthy debate,
with opponents of early S2P (at 3–4 months of age)
pointing to the potential for greater complications
related to increased length of stay as well as longer
duration of intubation and chest tube days [24,25].
In fact early S2P has not been shown to increase S2P
mortality, and has been associated with improved
&
interstage and intermediate outcomes [10 ,23]. In
addition, earlier S2P is associated with accelerated
catch-up growth as a result of the elimination of the
at-risk shunt-dependent circulation and ventricular
&
volume-unloading [26 ,27,28]. Due to strict dis-
charge criteria following S1P and low threshold
for readmission in many programs using a HMP,
length of stay and readmissions may potentially
increase following initiation of a HMP. However,
the cost associated with these issues may be out-
weighed by the avoidance of the readmission of a
patient in extremis requiring a high level of care
&
[18 ].
Table 2. Growth outcomes of HMP [19 ,29 ,30 ] & &&
First author and year Outcome
Growth without HMP
Kelleher 2006 Interstage weight gain
Anderson 2010 Interstage weight gain
Growth with HMP
Petit 2011 Interstage weight gain
Hehir 2012 Interstage weight gain
Anderson 2012 Multicenter interstage weight gain
HMP, home monitoring program; WAZ, weight for age z-score.
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BEYOND SURVIVAL: GROWTH, FEEDING,
AND FAMILY IMPACT
In addition to improved survival, infants enrolled in
a multidisciplinary HMP generally demonstrate
&
greater growth velocity during the interstage period
& && &
(Table 2) [19 ,29 ,30 ,31,32]. Experience with
heightened interstage surveillance has identified
failure to thrive as a modifiable risk factor that
has been linked to adverse outcomes at subsequent
palliative surgeries in this population. Carlo et al.
[33] found patients who died or required transplant
between S2P and Fontan completion were more
likely to have a low weight for age z-score (WAZ)
at S2P. Francois et al. [27] demonstrated a correlation
between long-term WAZ and heart failure symp-
toms following Fontan completion, noting that ear-
lier S2P was associated with improved long-term
growth. Ensuring normal infant growth has become
a priority of HMP management, and is the primary
aim of the initial quality improvement goal of the
NPC-QIC [14 ]. Whereas past studies have demon-
strated a consistent pattern of worsening WAZ
during the Norwood hospitalization and the inter-
stage period, recent experience with programs
utilizing a HMP has demonstrated the ability to
maintain normal infant growth patterns and even
catch up growth during the interstage period
& & && & &
[19 ,26 ,29 ,30 ,34 ].
Family involvement is pivotal in the develop-
ment and success of a HMP, and may have beneficial
effects on family stress and quality of life. Various
researchers have demonstrated a high level of
parental stress in households of single-ventricle chil-
dren, especially at the time of initial hospital dis-
charge [35,36]. In a study of the impact of a HMP on
parental stress, Stewart et al. [37] found that parental
anxiety significantly decreased after 1 month in the
program. The HMP creates an instant support net-
work for families of children with single-ventricle
heart disease and can provide resources beyond
medical interventions. The involvement of parents
&
Results
Median WAZ decreased from 1.4 to 2 during interstage
Median interstage weight gain 18.1 g/day
Pre-HMP: 17.9 g/day; HMP: 22.5 g/day
Mean interstage weight gain 26 g/day with HMP
Improved growth in programs with HMP
Volume 28
Number 2
March 2013
 Single-ventricle infant home monitoring programs Hehir and Ghanayem
in local support groups and national organizations
advocating for children with congenital heart
disease and their families may help alleviate
family stress and provide a much needed support
structure.
RECOMMENDATIONS FOR STRUCTURE OF
HOME MONITORING PROGRAM
While the structure of an individual HMP varies
based on local practice and resources, the basic
philosophy is a low-cost, family-centered interven-
tion to identify at-risk patients before physiologic
deterioration occurs. In the initial phases of a HMP,
many programs have struggled with securing per-
sonnel and funding, as well as establishing buy-in
from referring cardiologists. However, improve-
ments in survival as well as infant growth, family
satisfaction, and caregiver communication associ-
ated with the use of a HMP outweigh the challenges.
A centralized HMP clinic with a cohesive prac-
titioner group has been shown to play a significant
role in improving continuity and communication
& &
for interstage patients [18 ,19 ]. In a survey of par-
&&
ticipating NPC-QIC centers, Anderson et al. [29 ]
identified HMP practices which correlated with best
growth outcomes. Centers experiencing better inter-
stage growth were more likely to use standardized
postoperative feeding evaluations, distribute home
scales, make regular phone contact with families,
and use specific weight loss ‘red flags’ to trigger a
HMP evaluation.
At the Children’s Hospital of Wisconsin, the
HMP continues to function as a multidisciplinary,
&
adjunctive clinical service [30 ,38]. Since its incep-
tion, the program has undergone frequent cycles of
review and modification, with the goal of ongoing
quality improvement. In addition to the support
provided by their pediatricians and primary cardiol-
ogists, families receive weekly phone calls from HMP
nurse practitioners and return for HMP clinic visits
every 2–4 weeks. Each clinic visit includes input
from a HMP nurse practitioner, cardiologist, and
dietician, and may include consultation with speech
and feeding experts, social services, and other
specialists. Prior to discharge, parents are trained
to obtain and record daily oxygen saturation,
weight, and enteral volume intake. Once dis-
charged, parents are instructed to notify the HMP
team of breaches of the following criteria:
(1) enteral intake less than 100 ml/kg per day,
(2) any weight loss greater than 30 g,
(3) failure to gain at least 20 g over a 3-day
period, and
(4) oxygen saturation below 75% or above 90%.
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The ongoing evaluation and improvement of
an individual center’s HMP is paramount to its
success. This should be done using both internal
and external benchmarks. While interstage survival
is the ultimate measure of success, other important
areas of evaluation include S2P outcomes, measures
of growth and nutrition, family satisfaction, and
indicators of family stress and quality of life.
FUTURE RESEARCH AND INNOVATION IN
HOME MONITORING PROGRAM
DEVELOPMENT
Research in interstage monitoring continues to
focus on refining the components of a successful
HMP, expanding the HMP concept to other high-
risk populations, and exploring innovative concepts
and technologies which will augment current prac-
tice. Further multi-institutional collaboration will
help in identifying best practices in this area, and
facilitate data-sharing and early adoption of new
technologies and concepts as they become available.
Moving forward, the original pen-and-paper system
of HMP data recording is giving way to digital
options of data recording and communication, with
real-time tele-medicine and smart phone appli-
cations already in use in some programs [39,40].
CONCLUSION
The early interstage continues to represent a period
of high risk of mortality for infants with single-
ventricle heart disease. Home monitoring is a simple
and inexpensive strategy which improves interstage
survival through heightened surveillance and early
identification of subtle physiologic signs which may
precede patient deterioration.
Acknowledgements
None.
Conflicts of interest
There are no conflicts of interest.
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READING
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& of special interest
&& of outstanding interest
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Volume 28
Number 2
March 2013