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The Ross Classification for Heart Failure in

Children After 25 Years: A Review and an AgeStratified Revision
ARTICLE in PEDIATRIC CARDIOLOGY APRIL 2012
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Pediatr Cardiol
DOI 10.1007/s00246-012-0306-8

ORIGINAL ARTICLE

The Ross Classification for Heart Failure in Children After 25

Years: A Review and an Age-Stratified Revision
Robert D. Ross

Received: 10 January 2012 / Accepted: 14 March 2012

Springer Science+Business Media, LLC 2012

Abstract Accurate grading of the presence and severity

of heart failure (HF) signs and symptoms in infants and
children remains challenging. It has been 25 years since the
Ross classification was first used for this purpose. Since
then, several modifications of the system have been used
and others proposed. New evidence has shown that in
addition to signs and symptoms, data from echocardiography, exercise testing, and biomarkers such as N-terminal
pro-brain natriuretic peptide (NT-proBNP) all are useful in
stratifying outcomes for children with HF. It also is
apparent that grading of signs and symptoms in children is
dependent on age because infants manifest HF differently
than toddlers and older children. This review culminates in
a proposed new age-based Ross classification for HF in
children that incorporates the most useful data from the last
two decades. Testing of this new system will be important
to determine whether an age-stratified scoring system can
unify the way communication of HF severity and research
on HF in children is performed in the future.
Keywords Age-based Ross classification
Heart failure

Ross classification

The grading of heart failure (HF) signs and symptoms in

infants and children remains challenging. Ideally, a system
for doing this would be accurate, reproducible, correlated
closely with disease severity and outcome, and fluid to

R. D. Ross (&)
Division of Pediatric Cardiology, Carmen and Ann Adams
Department of Pediatrics, Childrens Hospital of Michigan,
Wayne State University School of Medicine,
3901 Beaubien Blvd., Detroit, MI 48201, USA
e-mail: rross@dmc.org

reflect changes in symptoms over time and with therapy.

The classification also should predict risk from disease so
that management can be tailored to HF class. Children
classified as no risk would not require treatment. Mild
risk might be managed by closer observation, early
intervention, or even prophylaxis. Moderate risk would
engender more intensive treatment, and severe risk
would require maximal therapy and perhaps transplantation
referral.
Until 1987, the only system available for grading HF in
children was the New York Heart Association (NYHA)
classification. However, this system was based on limitations to physical activity for adults, which did not translate
well for use with children, particularly infants. Therefore,
we developed a symptom-based classification using more
age-appropriate variables (Table 1) and demonstrated that
plasma norepinephrine correlated in a stepwise fashion
with this new Ross HF classification from grades I to IV
[16]. This was significant in that it mirrored norepinephrine
changes in adults with HF that correlated closely with
mortality [3]. Subsequently, Wu et al. [25] confirmed the
catecholamine changes with Ross class and further showed
that progressive beta receptor downregulation occurred
with each progressive Ross class.
More recently, Fernandes et al. [7] evaluated children
with idiopathic dilated cardiomyopathy. These authors
found that both the presence and severity of mitral regurgitation (MR) increased with the Ross class and that the
presence of MR stratified children to a significantly worse
outcome of either death or transplantation, with a hazard
ratio of 1.9 (p \ 0.01). Progression of MR severity
increased these risks significantly.
Over time, use of the Ross classification system has
increased, but the system is associated with problems.
Assignment of classes I and IV tends to be straightforward,

123

Pediatr Cardiol
Table 1 Original Ross classification [16]
I: No limitations or symptoms
II: Mild tachypnea or diaphoresis with feedings in infants, dyspnea
at exertion in older children; no growth failure
III: Marked tachypnea or diaphoresis with feedings or exertion and
prolonged feeding times with growth failure from CHF
IV: Symptomatic at rest with tachypnea, retractions, grunting, or
diaphoresis

with either no symptoms (class I) or symptoms with the

patient at rest (class IV). However, classes II and III are
more subjective and can overlap. Also, few class IV
patients are found in children who may have a greater
ability to compensate for HF early on. This leads to many
studies combining classes III and IV to have adequate
numbers for data analysis.
In addition, growth failure may be the only manifestation of HF in young children, which makes it difficult to
determine whether that puts them in Ross class III or a
lower class in the absence of respiratory or other symptoms
listed. Also confusing is the definition of growth failure
which has differed over time with various authors, and
growth failure may be related to noncardiac conditions.
To evaluate the variables that have most accurately
defined HF, we studied 41 infants and used the blinded
average grade from four pediatric cardiologists to compare
signs and symptoms. Based on the most sensitive and
specific variables, a scoring system for grading HF in
infants was derived (Table 2) [17]. Interestingly, neither
diaphoresis nor growth failure proved to be significant.
This is likely due to the frequent sweating that normal
infants exhibit and the young median age of 2.5 months in

the study, which may have been too early for growth
failure from HF to have become manifest.
Several authors have modified the aforementioned
scoring system to expand its use to older children. The first
such attempt was by Reithmann et al. [14] in their study on
adenylyl cyclase in severe HF. They added basal heart and
respiratory rates for children by age, namely, for infants
and children 16 years old, children 710 years old, and
children 1114 years old. This was a useful modification,
but these authors also reinstituted diaphoresis and added
cyanosis and a precordial thrill, neither of which are typically associated with HF. In 2002, Laer et al. [10] modified
this further, limiting their new version to six variables
(Table 3). A potential downside of this simplified version
is that half of the six categories relate to the work of
breathing, which may undervalue the other manifestations
of congestive heart failure (CHF) in children. Nevertheless,
many subsequent studies have used this Laer-modified
Ross scoring system to study HF.
At about the same time, a new system called the New
York University Pediatric Heart Failure Index (PHFI) was
proposed by Connelly et al. [4]. This 30-point scale uses
many of the signs and symptoms in the Ross classification
but adds points for medications used to treat HF and also
for a single-ventricle physiology. Although sicker patients
generally do require more medications, scoring in this way
may be problematic. If a child with severe symptoms of HF
is treated and the symptoms improve, the change in score

Table 3 Modified Ross score [10]

0

?1

?2

Diaphoresis

Head only

Head and body

at exertion

Head and
body at rest

Tachypnea

Rare

Several times

Frequent

Retractions

Dyspnea

History
Table 2 Ross scoring system for heart failure in infants [17]
Score
0

Breathing

Feeding history
Volume consumed per feeding (oz)

[3.5

2.53.5

\2.5

Time taken per feeding (min)

\40

[40

Physical exam
Respiratory rate (n/min)

\50

5060

[60

Heart rate (n/min)

\160

160170

[170

Respiratory pattern

Normal

Abnormal

Peripheral perfusion

Normal

Decreased

S3 or diastolic rumble

Absent

Present

Liver edge from right costal margin \2

(cm)

23

[3

Total score: 02 (no CHF), 36 (mild CHF), 79 (moderate CHF),

1012 (severe CHF)
CHF congestive heart failure

123

Physical examination
Normal

Age (years)
Respiratory rate (breaths/min) (years)
01
\50
5060

[60

16

\35

3545

[45

710

\25

2535

[35

1114

\18

1828

[28

Heart rate (beats/min) (years)

01

\160

160170

[170

16

\105

105115

[l15

710

\90

90100

[100

1114

\80

8090

[90

\2

23

[3

Hepatomegaly
size (cm)

Pediatr Cardiol

will be blunted by the points received for these medications

and thus may not reflect the improvement [15]. Regarding
single-ventricle patients, while most have reduced exercise
capacity, some children with Fontan palliation have no
symptoms, experience normal aerobic capacity for age and
body size, and thus do not deserve higher HF scores.
In 2006, Tissieres et al. [23] compared the NYHA
classification, the Laer-modified Ross classification, and
the PHFI using 20 children with HF from rheumatic heart
disease. Although all three systems correlated with the
cardiothoracic index on chest X-ray, the PHFI faired better
on left ventricle (LV) mass, end-systolic wall stress, left
atrium/aortic ratio, and N-terminal pro-brain natriuretic
peptide (NT-proBNP).
Recently, a great deal of interest in both the adult and
pediatric HF literature has focused on the natriuretic peptides in HF. From myocardial cells, BNP is released into
the bloodstream in response to various stressors on the
heart including LV volume and pressure overload. This
correlates well with symptoms of HF in adults and children
and can differentiate cardiac from pulmonary causes of
respiratory distress [6]. As the N-terminal fragment of the
prohormone BNP, NT-proBNP is a good marker of clinical
severity and worsening systolic function in children with
HF [13] and has a longer half-life than BNP. Sugimoto
et al. [22] found very sensitive and specific cutoff points of
NT-proBNP for Ross classes I to IV that had area-underthe receiver operating curves of 0.96 to 0.99. There was a
dichotomy of values, with lower numbers for children older
than 3 years than for children younger than 3 years. For
distinguishing each class independently, NT-proBNP was
better than BNP itself.
Multiple other studies have confirmed the usefulness of
NT-proBNP and BNP as correlates of HF symptoms in
children, as markers of systolic dysfunction, and importantly, as predictors of the need for mechanical circulatory
support, heart transplantation, and death [1, 18, 24]. This
has held true for HF from cardiomyopathy and from congenital heart disease such as single ventricle with failing
Fontan palliation [11, 20]. The trend of NT-proBNP over
time in individual patients is most useful for predicting
outcomes [24].
Another factor found to be useful for predicting outcomes in HF is exercise capacity. In adults, a peak exercise
oxygen consumption of less than 14 ml/kg/min is an
independent predictor of mortality and a criterion for listing a patient to receive a heart transplant. However, this
cutoff may be less sensitive in the current era of improved
medical management for HF [2]. In addition, children have
different oxygen consumptions as they grow such that the
absolute number of 14 ml/kg/min is not a sensitive marker
of the need for transplantation [5]. Giardini et al. [8] performed exercise stress tests on 82 children with dilated

cardiomyopathy and found that in a 4-year follow-up period, the percentage of peak oxygen consumption (based on
age and sex) stratified outcomes accurately. Using 62 % of
predicted normal as a cutoff, survival curves indicated a
significantly higher rate of death or urgent listing for
transplantation of 50.6 (for those B62 %) versus 4.4 %
(for those [62 %) at 24 months, with a hazard ratio of
10.8.
Growing evidence also indicates that poor systolic
function bodes ill in terms of long-term outcomes for
children as it does for adults. In both dilated cardiomyopathy and HF from congenital heart disease, low ejection
fractions predict death or the need for transplantation
[9, 12].
It is clear that with all this recent data on factors
predictive of outcomes in children with HF that a revision
in how we grade symptom severity is required. It also is
apparent that an age stratification is required to encompass the changes in signs and symptoms that children
manifest from infancy to late childhood. A classification
system should include the biomarkers, echo parameters of
systolic function and mitral or systemic atrioventricular
valve (AV) insufficiency, and reflect exercise limitations
reflected by feeding and growth in infants and exercise
capacity indicated by percentage of predicted maximal oxygen uptake (VO2) in older children. Therefore, I
propose an age-based Ross classification using the original variables that proved to be sensitive and specific and
adding the new evidence-based data. Table 4 depicts this
revised system.
The age ranges of 03 months, 412 months, 13 years,
48 years, and 918 years were chosen because the variables in the classification are generally stable during these
periods but vary between them. Each age range has 10
variables with scores of 0, 1, or 2 possible for a range of 0
to 20. The scoring system can be used as a continuous data
set for comparison with outcomes, or it can be categorized
by points assessed as Ross classes I (05), II (610), III
(1115) and IV (1620).
For all children, hepatomegaly is measured as the distance below the right costal margin with abdominal situs
solitus or from the left costal margin for situs inversus. The
ejection fraction generally is obtained from echocardiography but can be derived from MRI or other imaging
methods for single ventricles or systemic right ventricles.
Systemic AV insufficiency refers to the mitral valve for
systemic left ventricles and to the systemic AV valve for
single ventricles or systemic right ventricles.
Each age range has unique aspects that require comment. All heart rate and respiratory rates should be recorded with the infant or child in the basal state without crying
or undo agitation, and the cutoff points have been selected
based on normals for these age ranges [19].

123

Pediatr Cardiol
Table 4 Age-based Ross classification for heart failure in children
0

Oz/feeding

[3.5

2.53.5

\2.5

Time for feeding

(min)

\20

2040

[40

Breathing

Nl

Tachypnea

Retractions

RR/min

\50

5060

HR/min

\160

Perfusion
Hepatomegaly (cm)

Table 4 continued
0

N/V

Nl

Intermittent

Frequent

Breathing

Nl

Tachypnea

Retractions

RR/min

\20

2030

[30

HR/min

\90

90100

[100

[60

Perfusion

Nl

Reduced

Shocky

160170

[170

Hepatomegaly (cm)

\2

23

[3

Nl
\2

Reduced
23

Shocky
[3

NT-proBNP (pg/ml)

\300

3001,500

[1,500

EF%

[50

3050

\30

NT-proBNP (pg/ml)

\450
([4 days)

4501,700

[1,700

Max %VO2

[80

6080

\60

EF%

[50

3050

\30

AV insufficiency

None

Mild

Moderate/
severe

AV insufficiency

None

Mild

Moderate/
severe

Feeding

Nl

Decreased

Gavaged

Wt%
Breathing

Nl
Nl

C1 Curve
Tachypnea

C2 Curve
Retractions

RR/min

\40

4050

[50

HR/min

\12

120130

[130

Perfusion

Nl

Reduced

Shocky

Hepatomegaly (cm)

\2

23

[3

NT-proBNP (pg/ml)

\450

4501,700

[1,700

EF%

[50

3050

\30

AV insufficiency

None

Mild

Moderate/
severe

Feeding

Nl

Decreased

Gavaged

Growth
Breathing

Nl
Nl

Weight loss
Tachypnea

Cachexia
Retractions

RR/min

\30

3040

[40

HR/min

\110

110120

[120

Perfusion

Nl

Reduced

Shocky

Hepatomegaly (cm)

\2

23

[3

03 Months

918 Years

412 Months

Oz ounce, Nl normal, Wt% fall-off on weight curve %, RR respiratory

rate, HR heart rate, NT-proBNP N-terminal pro-brain natriuretic
peptide, EF ejection fraction, AV systemic atrioventricular valve, N/
V nausea/vomiting; Max %VO2 % of predicted maximal oxygen
uptake for age and sex

Age 03 Months
The volume of formula per feeding is for bottle-fed babies.
For breastfed infants, the volume taken has to be rated
subjectively as normal, decreased, or gavage supplemented. Normally, NT-proBNP is elevated in newborns, so
this measurement should be obtained after 4 days of life.

13 Years

NT-proBNP (pg/ml)

\450

4501,700

[1,700

EF%

[50

3050

\30

AV insufficiency

None

Mild

Moderate/
severe

48 Years
N/V

None

Intermittent

Frequent

Growth

Nl

Weight loss

Cachexia

Breathing
RR/min

Nl
\25

Tachypnea
2535

Retractions
[35

HR/min

\100

90100

[100

Perfusion

Nl

Reduced

Shocky

Hepatomegaly (cm)

\2

23

[3

NT-proBNP (pg/ml)

\300

3001,500

[1,500

EF%

[50

3050

\30

AV insufficiency

None

Mild

Moderate/
severe

123

Age 412 Months

Feeding is qualitatively graded because diets vary in this
age range and specific volumes of formula are not applicable. The time of feeding is replaced by growth, as
depicted on the growth curve. A fall-off in growth, defined
as a decrease of C1 weight curve percentile (i.e., from the
50 to the 25 %) earns 1 point, whereas a fall-off of C2
percentile curves (i.e., from 50 to 10 %) earns 2 points. If
no previous weights are available, then 1 point is awarded
for a weight percentage of C1 curve below the current
height percentile and 2 points for C2 curve percentiles for
weight below that for height because increases in body
length typically are preserved in HF, whereas weight gain
is not.

Age 13 Years
Because the time of early rapid growth has passed by these
ages, the pattern on the growth curves has been changed to
recent weight loss and cachexia for respectively 1 and 2
points.

Pediatr Cardiol

Age 48 Years
As children age, their gastrointestinal symptoms from HF
change to reports of nausea or vomiting, so scores are 1 for
these symptoms intermittently and 2 for frequent nausea or
vomiting. The cutoff values for NT-proBNP have been
adjusted down for the lower values found after the age of
3 years.

differences in symptoms. Standardizing our approach for

future research and communication using one system that
incorporates all the significant features of HF culled from
the literature is a big step toward an evidence-based
approach to studying and treating childhood HF in the
future.

References
Age 918 Years
At this age, most children can perform a maximal stress
test, so this has replaced growth failure as a more objective
measure of heart failure decompensation.

BNP
Although NT-proBNP has a longer half-life than BNP and
correlates better with symptom class, retrospective studies
may have access only to BNP levels. If so, then BNP may
replace NT-proBNP in the grid for all ages beyond 4 days,
with points of 0 for less than 30 pg/ml, 1 for 30140 pg/ml,
and 2 for more than 140 pg/ml. Because these peptides also
may be elevated from renal dysfunction, children with
advanced renal insufficiency may have a somewhat inflated
score for this component [21].

Class IV
Finally, some children will be so sick from HF that they
will require intravenous medications, circulatory support
such as extracorporeal membrane oxygenation (ECMO) or
a ventricular assist device, and mechanical ventilation.
These interventions make the children unfit for the use of
this grading system and should put them by definition into
class IV.

Future Challenges
The next challenge is to test this age-based Ross classification using large numbers of infants and children with and
without overt HF to determine whether it accurately predicts outcomes for each age range. Although it does entail
more information gathering than the original Ross system,
most patients with HF get tested for these variables in
addition to the readily obtained historical and physical
examination findings. Use of this age-based stratification
should allow for more accurate grading, eliminating
the variance previously encountered based on age-related

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