Pediatr Cardiol (2017) 38:1627–1632
DOI 10.1007/s00246-017-1706-6
ORIGINAL ARTICLE
Predictors of Mortality in Children with Pulmonary Atresia
with Intact Ventricular Septum
Stephanie Grant1 • David Faraoni2 • James DiNardo1 • Kirsten Odegard1
Received: 17 April 2017 / Accepted: 7 August 2017 / Published online: 4 September 2017
Ó Springer Science+Business Media, LLC 2017
Abstract Pulmonary atresia with intact ventricular septum
(PA/IVS) is a rare cardiac congenital lesion characterized
by imperforate pulmonary valve, intact ventricular septum,
and atrial level shunt. Although different management
strategies to establish a source of non-ductal dependent
pulmonary blood flow have been described, studies have
not assessed the relationship between the therapeutic
approach, patient characteristics, and outcomes. The pur-
pose of this study was to identify predictors of mortality for
patients with PA/IVS. Neonates and children with PA/IVS
were identified through analysis of the 2012 Kids’ Inpatient
Database of the Healthcare Cost and Utilization Project.
Hospital admissions that included a cardiac catheterization
and/or surgical procedure were analyzed to identify
demographics, co-morbidities, and outcomes. We identi-
fied 508 patients with PA/IVS with hospital admissions that
included cardiac catheterization (n = 165), surgical pro-
cedures (n = 273), or both (n = 70). The incidence of
mortality in this cohort was 6.69% (34/508). Univariable
analysis demonstrated that age less than 12 months
(p \ 0.001), non-elective admission (p \ 0.001), AKI
(p = 0.001), sepsis (p = 0.002), and the use of ECMO
(p \ 0.001) were associated with an increased risk of
mortality, while no difference was observed for the type of
therapeutic approach (p = 0.498). These variables were
used in a multivariable logistic regression analysis to
& Stephanie Grant
stephanie.grant@emory.edu
1 morphology at initial presentation [5–9].
Department of Anesthesiology, Peri-operative and Pain
Medicine, Boston Children’s Hospital, Harvard Medical
School, 300 Longwood Avenue, Boston, MA 02115, USA
2
Department of Anesthesia and Pain Medicine, The Hospital
for Sick Children, University of Toronto, Toronto, Canada
develop the predictive model for mortality. Age less than
12 months, non-elective admission, and the use of ECMO
in children with PA/IVS were predictors for mortality.
Interestingly, the type of therapeutic approach did not
influence mortality, which suggests that patient character-
istics other than the method chosen to provide pulmonary
blood flow determine mortality.
Keywords Pulmonary atresia with intact ventricular
septum
Outcomes
Risk factor
Mortality
Introduction
Pulmonary atresia with intact ventricular septum (PA/IVS)
is a rare cardiac congenital lesion characterized by
imperforate pulmonary valve, intact ventricular septum,
and atrial level shunt associated with both highly variable
right ventricular (RV) development and ventriculocoronary
connections [1–4]. Patients with PA/IVS are dependent on
a patent ductus arteriosus for pulmonary blood flow at
birth. Neonates require cardiac catheterization in order to
characterize the extent and clinical relevance of any ven-
triculocoronary connections. Data obtained determine if the
patient is a candidate for RV decompression as a part of the
initial surgical or catheter-based interventions to establish
non-ductal dependent pulmonary blood flow. The presence
of RV dependent coronary circulation (RV-DCC) is a
contraindication to RV decompression. Management
strategies for definitive repair are guided by the patient’s
PA/IVS is associated with significant morbidity and
mortality. In previous studies, the 1-year and 5-year sur-
vival rates for patients born with PA/IVS range from 68 to
88.3% and 50 to 86.5%, respectively [5, 8, 10, 11]. Risk
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factor analyses of mortality have focused on patient
demographic and anatomical factors. Small tricuspid valve
annulus Z-score, ventriculocoronary artery connections,
unipartite right ventricle morphology, and low birth weight
have been identified as risk factors for mortality in patients
with PA/IVS [11–19].
Although different management strategies have been
described, studies have not assessed the relationship among
therapeutic approach, patient characteristics, co-morbidi-
ties, and outcomes. The purpose of this study was to
identify predictors of mortality in patients with PA/IVS
through analysis of the KID HCUP database.
Materials and Methods
A retrospective analysis of the 2012 Healthcare Cost and
Utilization Project (HCUP) Kids’ Inpatient Database (KID)
[20] was performed to identify patients with PA/IVS. KID
is an administrative data set composed of pediatric inpa-
tient discharge abstracts from participating hospitals in 44
US states. KID is sponsored by the Agency for Healthcare
Research and Quality (AHRQ) and is the largest publicly
available all-payer pediatric inpatient care database in the
USA with approximately 3 million pediatric hospital dis-
charges in 2012. The data set was generated by a system-
atic random sampling to select 80% of pediatric cases, 10%
of uncomplicated in-hospital births, and 80% of compli-
cated in-hospital births in each participating state. Hospi-
tals in the database include specialty hospitals, public
hospitals, and academic medical centers. Patient informa-
tion is de-identified and includes information about hos-
pital stays for patients B20 years of age. This database
contains more than 100 clinical and non-clinical data ele-
ments for each hospital stay. Information for each admis-
sion includes a maximum of 25 diagnoses and 15
procedures; as well as, information regarding demograph-
ics, discharge status, length of stay, and hospital charac-
teristics. Diagnostic and procedure information are
recorded using codes from the International Classification
of Diseases, Ninth Revision, Clinical Modification (ICD-9
CM).
Neonates and children with PA/IVS were identified
using ICD-9 CM code. There is not a specific ICD-9 CM
code for PA/IVS; therefore, patients were identified using
the diagnostic code for pulmonary atresia (746.01). We
excluded patients with ICD-9 CM diagnostic and proce-
dural codes for anomalies incompatible with PA/IVS (e.g.,
ventricular septal defects, Tetralogy of Fallot). Patients
with hospital admissions that included a cardiac catheter-
ization and/or a cardiac surgical procedure were identified
using appropriate procedural ICD-9 CM codes. Each hos-
pital admission for a patient identified with PA/IVS that
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Pediatr Cardiol (2017) 38:1627–1632
included a cardiac catheterization and/or a cardiac surgical
procedure was analyzed to identify demographics, co-
morbidities, and outcomes. The patient’s age at admission
was divided into five age categories that included neonates
(B1 month old), [1 month to 1 year, [1–6 years,
[6–12 years, and [12 years. Using ICD-9 CM codes, we
recorded the incidence of co-morbidities, such as acute
kidney injury (AKI), neurologic complications, and extra-
corporeal membrane oxygenation (ECMO) cannulation.
Each demographic, co-morbid, and procedural variable
was analyzed for survival versus mortality.
Statistical Analysis
Univariable logistic regression analysis was used to iden-
tify demographic, co-morbid, and procedural variables that
were associated with an increased risk of mortality. Vari-
ables were evaluated with a Pearson’s Chi-square test.
Multivariable logistic regression analysis was used to
develop a predictive model for mortality. Variables in the
univariable analysis with a p value \0.05 were included in
the multivariable model. Area under the receiver operating
characteristic (ROC) curve was calculated to assess the
strength of the association between multivariable predic-
tors and mortality.
A p value \0.05 was considered statistically significant
for all tests. All reported values in this study are absolute
values measured from the data set. Statistical analysis was
performed with STATA version 14.0 (StataCorp, College
Station, Texas).
Results
Through analysis of the 2012 KID HCUP database, we
identified 2228 patients with pulmonary atresia. Through
exclusion, we identified 508 patients with PA/IVS with
hospitalizations that included a cardiac catheterization
(n = 165), a cardiac surgical procedure (n = 273), or both
(n = 70). The incidence of mortality in this cohort was
6.7% (34/508).
Demographic, co-morbid, and procedural variables were
evaluated for mortality, as displayed in Table 1. Univari-
able analysis revealed that age less than 12 months
(p \ 0.001), non-elective admission (p \ 0.001), AKI
(p = 0.001), sepsis (p = 0.002), and the use of ECMO
(p \ 0.001) were associated with an increased risk of
mortality. The type of procedure during the hospitaliza-
tion—cardiac catheterization, cardiac surgery, or both—
was not associated with an increased risk of mortality
(p = 0.498). Therefore, the type of therapeutic approach
did not increase the risk of mortality.
Pediatr Cardiol (2017) 38:1627–1632 1629

Table 1 Survival vs. non-survival of PA/IVS patients during hospital Table 2 Multivariable predictive model for mortality
admission
Variables B (SE) OR 95% CI p value
Survival Non-survival p value
Age \12 months 1.35 (0.66) 3.86 1.05–14.16 0.042
Total number of patients 474 (93%) 34 (7%) Non-elective admission 1.99 (0.58) 7.35 2.36–22.89 \0.001
Age \0.001* ECMO 2.53 (0.65) 12.58 3.55–44.60 \0.001
\1 month 61 (13%) 9 (26%)
1–12 months 179 (38%) 22 (65%)
1–6 years old 133 (28%) 2 (6%) The variables age less than 12 months, non-elective
6–12 years old 38 (8%) 1 (3%) admission, and the use of ECMO were used in multivari-
[12 years old 63 (13%) 0 (0%) able logistic regression analysis to develop the predictive
Admission \0.001* model for mortality (Table 2). Increased risk of mortality
Non-elective 170 (36%) 30 (88%) was observed in neonates and children with PA/IVS who
Elective 304 (64%) 4 (12%)
were less than 12 months of age (OR 3.86, 95% CI 1.05-
Gender 0.537
14.16, p = 0.042), who had non-elective admissions (OR
Male 249 (53%) 16 (47%)
7.35, 95% CI 2.36–22.89, p \ 0.001), and who had ECMO
cannulation during the hospitalization (OR 12.58, 95% CI
Female 225 (47%) 18 (53%)
3.55–44.60, p \ 0.001). The area under the ROC curve
Procedure 0.498
was 0.843 (95% CI 0.799–0.887) which indicates a strong
Catheterization 152 (32%) 13 (38%)
association between the multivariable predictors and mor-
Surgery 258 (54%) 15 (44%)
tality. As the number of risk factors increases in a patient,
Catheterization ? surgery 64 (14%) 6 (18%)
the probability of mortality also increases (Fig. 1).
ECMO \0.001*
No 467 (99%) 27 (79%)
Yes 7 (1%) 7 (21%)
Discussion
VAD 0.789
No 473 (100%) 34 (100%)
This retrospective study evaluated the 2012 KID HCUP
Yes 1 (0%) 0 (0%)
Database for predictors of mortality in children with PA/
Thrombosis 0.271 IVS. To the authors’ knowledge, this study is the first to
No 442 (93%) 30 (88%) evaluate the relationship among therapeutic approach,
Yes 32 (7%) 4 (12%) patient characteristics, co-morbidities, and outcomes of
Acute kidney injury 0.001* children with PA/IVS during hospitalizations that included
No 459 (97%) 29 (85%) a cardiac catheterization or surgical intervention. Analysis
Yes 15 (3%) 5 (15%) identified age less than 12 months, non-elective admission,
Cardiac failure 0.176 and the use of ECMO as predictors of mortality for chil-
No 436 (92%) 29 (85%) dren with PA/IVS. Although not included in the multi-
Yes 38 (8%) 5 (15%) variable regression analysis, AKI and sepsis were also
Heart transplant 0.476 associated with an increased risk of mortality.
No 467 (99%) 34 (100%) The survival rates of PA/IVS have improved over the
Yes 7 (1%) 0 (0%) years with advances in catheterization and surgical tech-
Neurologic complication 0.748 niques; as well as, improved selection of procedures based
No 464 (98%) 33 (97%) on morphology [5, 6, 10]. Neonates with PA/IVS require a
Yes 10 (2%) 1 (3%) cardiac catheterization for delineation of anatomy in order to
Sepsis 0.002* guide decisions for the management strategy and to deter-
No 463 (98%) 30 (88%) mine if the patient is a candidate for RV decompression [21].
Yes 11 (2%) 4 (12%) Initial angiography of the right ventricle and coronary
arteries is important to identify ventriculocoronary con-
Neurologic complication includes stroke, seizure, and intracranial
hemorrhage; Data reported as frequency (%); p values calculated nections associated with PA/IVS that contribute to
using a Pearson Chi-square test; p value \0.05 statistically significant myocardial perfusion. The presence of RV-DCC is a con-
ECMO extracorporeal membrane oxygenation, VAD ventricular assist traindication to RV decompression because adequate
device myocardial perfusion relies on a high right ventricular
* Statistically significant pressure (RVP). RV decompression causes a sudden
decrease in RVP and a subsequent decrease in myocardial

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Fig. 1 Predicted probability of
mortality based on the number
Predicted Probability of Mortality
of risk factors present. As the
number of risk factors present
increases, the probability of
mortality in PA/IVS patients
also increases
perfusion of ventriculocoronary connections resulting in
myocardial ischemia in patients with RV-DCC. Bull et al.
and Alwi et al. have developed management strategies based
on RV morphology [22–24]. Normal right ventricle mor-
phology is tripartite and consists of three parts including the
inlet, trabecular, and infundibular regions [25]. Patients with
PA/IVS have a spectrum of RV morphology that consists of
either a tripartite RV, a bipartite RV with only inlet and
infundibular regions, or a unipartite RV with only the inlet
portion. In patients with an adequate infundibulum, non-
ductal dependent pulmonary blood flow can be achieved
with RV decompression either through a transcatheter
radiofrequency-assisted perforation of the pulmonary valve
followed by balloon valvuloplasty or through surgical pul-
monary valvotomy and transannular patch placement. In a
small series of selected patients with mild to moderate RV
hypoplasia and a patent infundibulum, Alwi et al. found
percutaneous radiofrequency-assisted valvotomy and bal-
loon dilation to be an acceptable alternative to closed sur-
gical valvotomy and placement of a modified Blalock-
Taussig shunt (mBTS) [26]. Subsequent experience has
demonstrated that a subset of patients with borderline RV
size and/or dynamic RVOTO may need a PDA stent or
mBTS in conjunction with a balloon atrial septostomy or
surgical valvotomy and transannular patch placement to
provide supplemental pulmonary blood flow following the
initial catheter-based pulmonary valve perforation [24]. In a
series from an institution that aggressively undertakes
catheter-based valve perforation for most patients with non-
RV-DCC, 62% of patients required an additional surgical
procedure to augment pulmonary blood flow prior to hos-
pital discharge [27]. Our data indicate that a subset of
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Pediatr Cardiol (2017) 38:1627–1632
100
90
80
67.5 ± 0.01%
70
60
50
40
30
20 14.1 ± 1.8%
10
0.6 ± 0.01% 2.2 ± 0.9%
0
None One Two Three
Number of Risk Factors Present
patients also required combined catheter-based and surgical
therapy prior to hospital discharge. Biventricular repair can
be achieved in most PA/IVS patients with tripartite or
bipartite RV morphology. For patients with inadequate right
ventricular growth after RV decompression, a one and one-
half ventricle repair can be achieved with a bidirectional
Glenn Shunt in order to support the pulmonary circulation.
Patients with unipartite RV morphology do not have an
adequate RV size to support the pulmonary circulation and,
therefore, initial procedures include a mBTS or PDA stent to
provide a non-ductal source of pulmonary blood flow. These
patients are subsequently staged to a one ventricle repair.
Patients with RV-DCC are initially managed with a mBTS
or PDA stent and are also staged to a one ventricle repair.
Heart transplantation might be an option for patients with
severe forms of PA/IVS. After the initial development of a
non-ductal dependent pulmonary blood flow source, the RV
may grow and must be assessed to determine adequacy to
support the pulmonary circulation. Definitive repair can be
achieved with a biventricular, one and one-half ventricle, or
one ventricle repair based on morphology at initial
presentation.
In this study, the therapeutic approach did not influence
mortality (Table 1). This result is consistent with studies by
Daubeney et al. and Hannan et al. that also demonstrated
that the type of procedure did not influence mortality
[7, 11]. Of the 508 PA/IVS patients included in this study,
the in-hospital mortality rate was 6.7%, which is lower than
the 17% reported by Cleuziou et al. [14].
The use of the 2012 KID HCUP Database for this PA/
IVS study was beneficial in order to study a large number
of patients with a rare disease. However, despite the
Pediatr Cardiol (2017) 38:1627–1632
strength of our methods, there were limitations. This
database was created for administrative purposes and
includes ICD-9 CM codes for patient descriptors. Missing
or miscoded data may exist within this large database,
which would decrease the validity of our results that rely
on ICD-9 CM codes for identification of patient charac-
teristics, co-morbidities, and procedures. Since an ICD-9
CM code does not exist for PA/IVS, identification of PA/
IVS patients may be under- or over-estimated. Another
limitation is the inability to establish details of patient
severity, morphology, biometric data, and timing of
development of co-morbidities based on ICD-9 CM codes.
ICD-9 CM codes do not exist for specific anatomic
variations that are associated with PA/IVS, such as RV-
DCC, ventriculocoronary connections, and variable RV
size and morphology. Therefore, the morphology and
severity of the patients identified with PA/IVS are
unknown in this cohort of patients. This is a limitation to
this study because the relationship of morphology and co-
morbid variables cannot be analyzed in patients identified
as non-survivors. For example, this study identified the use
of ECMO as a predictor of mortality. Patients with PA/IVS
and RV-DCC do poorly with the use of ECMO because
venous drainage of the right atrium from the ECMO can-
nula may decompress the right ventricle. This reduction in
RVP reduces coronary perfusion pressure and leads to
myocardial ischemia [28]. However, this study is unable to
evaluate the relationship of ECMO and other co-morbid
variables with morphologic factors to determine a possible
contribution to mortality.
The time-course of events during hospitalization and the
progression of events preceding death are unknown in this
group of patients due to the nature of using database entries
consisting of ICD-9 CM codes. For this same reason, the
cause of death cannot be determined for the 6.7% of
patients with in-hospital mortality in this study. Possible
etiologies of death in PA/IVS patients include thrombosis
of a shunt, myocardial dysfunction, and myocardial
infarction. The presence of multiple risk factors in PA/IVS
patients increases the risk of mortality. Based on this study,
if a patient is less than 12 months, has a non-elective
admission, and requires ECMO support, the probability of
mortality during a hospitalization with a cardiac catheter-
ization or surgical procedure is 67.5%. This is a significant
risk for mortality.
Despite the limitations identified, the KID HCUP data-
base was chosen to analyze PA/IVS based on elimination of
incompatible diagnoses with pulmonary atresia in order to
evaluate a large cohort with hospital admissions that include
a cardiac surgical or catheterization procedure. However,
the lack of distinct ICD-9 CM codes for PA/IVS and for the
morphologic variations of PA/IVS further limits the use of
this database on the basis of the inability to distinctly
1631
identify patients with PA/IVS and to assess their severity.
The KID HCUP database may not be the ideal tool to
evaluate heterogeneous lesions, such as PA/IVS, because
ICD-9 CM codes cannot elucidate the details and severity of
the lesion. Databases using ICD-9 CM code for patient
descriptors can be of value when analyzing outcomes of
cardiac lesions with specific ICD-9 CM code, such as atrial
septal defects, ventricular septal defects, and a patent ductus
arteriosus. These lesions have minimal anatomic variation
and when identified through a database query can be eval-
uated for patient characteristics, co-morbidities, and proce-
dures using ICD-9 CM code. Databases using ICD-9 CM
code limit the ability to analyze rare or heterogeneous con-
genital cardiac defects and, thus, limit our ability to under-
stand outcomes in congenital heart disease.
Despite the limitations of the 2012 KID HCUP Data-
base, our analysis identifies that the predictors of mortality
in children with PA/IVS are age less than 12 months, non-
elective admission, and the use of ECMO. The type of
therapeutic approach did not influence mortality in this
cohort, which suggests that patient characteristics other
than the method chosen to provide pulmonary blood flow
determine mortality.
Funding This work was solely supported by the Department of
Anesthesiology, Perioperative and Pain Medicine, Boston Children’s
Hospital, Boston, MA.
Compliance with Ethical Standards
Conflict of interest The authors declare that they have no conflict of
interest.
Ethical approval For this type of study, formal consent is not
required.
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