Pediatric Cardiology

https://doi.org/10.1007/s00246-024-03566-x

RESEARCH

Pulmonary Atresia with Intact Ventricular Septum, a National

Comparison Between Interventional and Surgical Approach,
in Combination with a Systemic Literature Review
Stina Manhem1,4 · Michal Odermarsky2,3 · Håkan Wåhlander1,4 · Britt‑Marie Ekman‑Joelsson1,4

Received: 1 March 2024 / Accepted: 22 June 2024

© The Author(s) 2024

Abstract
This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS)
treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention.
Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS
between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for
initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocar-
diogram. Comparisons between groups were performed with Mann–Whitney U-test and Fisher´s exact test. A systematic
literature review of original studies regarding treatment of PA-IVS (2002 and onward) was conducted following the Preferred
Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to assess the outcomes of patients with PA-IVS. 34
(11 females) patients were included, 18 in group A and 16 in group B. There was no mortality in either group. Follow-up
time ranged from 2 to 15 years (median 9). All attempted perforations in group A were successful, and 16 out of 18 patients
reached biventricular circulation. In the surgical group 15 out of 16 patients reached biventricular circulation. The literature
review presented heterogeneity in standards for treatment. This retrospective population-based multicenter study demonstrates
that both catheter-based intervention and heart surgery are safe procedures. Our results are comparable to, or exceed, those
in the systematic literature review. The systematic literature review displays a great heterogeneity in study design, with no
definitive golden standard treatment.

Keywords Pulmonary atresia with intact ventricular septum · Valve lesions · Catheter-based valve perforation · Surgical
valvulotomy

Introduction

Pulmonary atresia with intact ventricular septum (PA-IVS)

is a rare and heterogeneous congenital heart defect. The
morphology ranges from a hypoplastic right ventricle with a
thick muscular atresia of the pulmonary valve, which renders
* Stina Manhem the circulation to be univentricular with only a left ventricle,
[email protected] to a well-developed right ventricle with a thin membranous
1 atresia capable of sustaining a biventricular circulation. In
Department of Pediatrics, Institution for Clinical Sciences,
Sahlgrenska Academy, University of Gothenburg, the latter type, initial decompression of the right ventricle
Gothenburg, Sweden with biventricular circulation is most often the aim, regard-
2
Department of Pediatric Cardiology, Skåne University less of the presence of ventriculo-coronary artery connec-
Hospital, Lund, Sweden tions (VCACs), given that the coronary circulation is not
3
Department of Clinical Sciences, Lund University, Lund, right ventricle dependent (RVDCC) [1, 2]. Surgical valvu-
Sweden lotomy and cardiopulmonary bypass has been associated
4
Department of Cardiology, Queen Silvia Children’s Hospital, with morbidity and mortality [3, 4]. Catheter-based approach
Behandlingsvägen 7, 416 50 Gothenburg, Sweden instead of surgery in the first days of life is less invasive

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 Pediatric Cardiology

and has become the standard practice in many centers over coronary catheter was placed in the right ventricular outflow
the past three decades [5–9]. The pulmonary valve can be for optimal positioning of the RF-wire. A Nykanen RF per-
perforated through laser, radiofrequency (RF) treatment or foration wire (Baylis Medical, Mississauga, ON) with a Bay-
through mechanical perforation. In Sweden, catheter-based lis RF generator was used. Serial hand injections were used
treatment of the pulmonary valve in PA-IVS was introduced to approach the center of the pulmonary valve plate using
in 2007 and has since been routinely performed at one of the both A–P and lateral projections. We have used a snare cath-
two pediatric cardiac centers in the country. eter placed in the main pulmonary artery via a retrograde
The aim of this retrospective study was to compare the approach through the ductus arteriosus during RF perfora-
long-term morbidity of patients treated with catheter-based tion to assist in aligning the RF catheter with the pulmonary
therapy with those who underwent heart surgery as the valve. An RF pulse was applied at 7 Watts for 2 s to perform
initial intervention for right ventricular decompression. the perforation. Successful perforation was confirmed by
Additionally, we conducted a systematic literature review capture of the perforation catheter with the snare or with
to assess the concurrence of our findings with current inter- injection of contrast. A 0.038-inch ProTrack microcatheter
national studies. was advanced on the RF-wire, which then was exchanged for
a 0.014-inch exchange wire. The exchange wire was secured
in either the distal pulmonary tree or, most often, across the
Materials and Methods patent ductus arteriosus (PDA) into the descending aorta. In
the aorta, the guidewire was often secured with the snare to
Retrospective Population‑Based Multicenter Study enhance stability of the wire. Pulmonary valvuloplasty was
then performed, often with a Tyshak Mini (NuMED Inc,
Patient Population Cornwall, ON) balloon. Balloon diameter was chosen to be
120–140% of measured valvular diameter. Pre-dilation was
This is a retrospective population-based multicenter study only preformed in a few patients early during the experience.
that screened all neonates born in Sweden with PA-IVS Post-dilation pressure waveforms were recorded simultane-
between 2007 and 2019 for inclusion. Patients were iden- ously in the right ventricle and the aorta. A repeat right ven-
tified through the Swedish National Register of Congeni- tricular angiogram was performed prior to completion of the
tal Deformities (which has a 100% national coverage for procedure. Prostaglandin E1 infusion was regularly stopped
PA-IVS), registers at pediatric cardiac centers and SWED- at the end of the procedure. Stenting of the arterial duct
CON—the Swedish Registry of Congenital Heart Disease. or surgical systemic-to-pulmonary shunt was reserved for
There are two pediatric cardiothoracic centers in Sweden, patients that failed repeated attempts of weaning from pros-
with one center, since 2007, primarily using catheter-based taglandin E1 infusion and was consequently performed as
intervention for patients with favorable morphology, while a later and separate procedure. The choice of this treatment
the other center prefers heart surgery. The allocation of strategy is motivated by the objective to minimize radia-
patients to each center is determined by a national agree- tion exposure, reduce the dose of contrast agent, and achieve
ment, where Sweden's 21 regions independently decide optimal hemodynamic stability.
which center to refer patients to for pediatric cardiac surgery.
The severity or morphology of the heart defect does not Surgery
influence the treatment center assignment, and the catchment
areas have equal population sizes. The decision of where a Surgical valvotomy was performed and additional source of
patient is treated is solely based on the place of birth, mak- pulmonary blood flow in form of modified Blalock-Taussig
ing this study pseudorandomized. shunt was added when deemed necessary. Concomitant
atrial septectomy was performed in selected cases.
Interventional Description
Reintervention
Catheterizations were performed under general anesthe-
sia. Venous and arterial access was obtained from femoral A reintervention has been defined as a subsequent interven-
vessels. Right heart and aortic pressures were documented tion (catheter-based or surgery) performed after a successful
prior to angiography. Right ventricular angiograms were opening of the pulmonary valve.
performed with cranial angulation of the anterio-poste-
rior plane to delineate the right ventricular outflow and to Methods
exclude right ventricular-dependent coronary circulation.
Arterial duct and main pulmonary artery locations were All patients underwent an initial echocardiogram and those
determined on aortic angiography. A 5F Judkins 2.5 right who were deemed unfit for biventricular repair, i.e., the right
Pediatric Cardiology

ventricle was not decompressed, were excluded from the atresia”, “pa ivs”, “paivs”, “pa-ivs”, “intact ventricular sep-
study. tum”. We searched the English and Swedish literature with
The inclusion criterion was decompression of the right Medline and Embase databases in a combined search and the
ventricle for initial intervention. Patients were eligible for Cochrane database for all studies published between 2002
initial decompression if they had membranous pulmonary and 2022. Reviews were not included but their reference
atresia and a bi- or tripartite right ventricle. VCACs were lists were examined to identify relevant original studies that
not an exclusion criterion, but RVDCC was. The presence met the inclusion criteria but were not found through the
of tricuspid valve dysplasia, of various degree, was noted but initial search. Inclusion criteria were original studies that
not an exclusion criterium. The initial preoperative angio- focused on initial treatment of PA-IVS performed from
gram, if performed, and the diagnostic echocardiogram were 2002 and onwards. Studies published before year 2002 were
reviewed. Medical records were reviewed regarding weight, excluded, to avoid results that were obsolete. Exclusion cri-
length, reinterventions, complications, time admitted to hos- teria were case reports, duplicates of previously published
pital and time in the pediatric intensive care unit (PICU) data and studies that included patients without prerequisites
in connection with the initial intervention. The follow-up for biventricular circulation or where the morphology of
period for reinterventions and complications ended in March the right ventricle was not clear. A few studies comprised
2022. patients with both severe hypoplastic right ventricles and
The echocardiographic examinations were reviewed by well-developed right ventricles suitable for biventricular
pediatric cardiologist MO, pediatric resident SM, pediatric repair. If the subgroups were clearly separated and the data
consultant BE and pediatric consultant HW. Spot-checks presented separately, the group of patients with biventricular
were conducted to minimize the risk for bias. Dr HW morphology was included in the review. If the subgroups
reviewed the angiograms performed at Queen Silvia Chil- had not been analyzed separately the study was excluded.
dren's Hospital in Gothenburg, there were no angiograms In a similar manner, studies that included both critical pul-
available for review at Skåne University Hospital. The pul- monary stenosis (CPS) and PA-IVS were included only if
monary regurgitation (PR) and tricuspid regurgitation (TR) the subgroups were analyzed separately, the group with PA-
were estimated by eyeballing on echocardiogram with color IVS was then included. This approach was chosen to ensure
Doppler. comparability of results, as the success rate of perforation
The two pediatric cardiothoracic centra have different pri- may differ in studies that include patients with CPS.
mary treatment method. We compared the patients treated
with catheter-based intervention and balloon dilation of the
pulmonary valve to those who underwent heart surgery for Results
initial intervention. The diagnostic echocardiograms were
compared between the two groups along with outcomes Retrospective Population‑Based Multicenter Study
related to final circulatory status, complications, number
and type of reinterventions, and mortality. Patient Characteristics

Statistics Sixty seven patients with PA-IVS were born in Sweden dur-
ing the study period and were reviewed for inclusion. For a
Continuous variables were analyzed using the Mann–Whit- comprehensive understanding of the assessment of prerequi-
ney U-test and are presented as mean and standard devia- sites for biventricular circulation or univentricular palliation
tion. Categorical variables were analyzed using the Fisher’s in the two different pediatric thoracic centers, see Tables 1
exact test and are expressed as numbers and percentages. and 2. 34 patients (11 females) were included in the study.
Statistical significance was set at a p-value < 0.05. Statistics 18 patients (4 females) were initially treated with catheter-
software R version 4.2.1 was used for the analyzes. based treatment (group A) and 16 (7 females) were initially
treated with surgical decompression of the right ventricle
Systematic Review of Current Literature (group B). Three patients were intended for catheter-based
treatment by mechanical perforation but had a failed valve
A systematic literature review was conducted following the perforation. In the analyses they were included in group B.
PRISMA (Preferred Reporting Items for Systematic Reviews All included patients had a membranous atresia and a
and Meta-Analyses) guidelines to assess the outcomes of bi- or tripartite right ventricle. In group A and B the pre-
patients with PA-IVS who underwent initial intervention for operative tricuspid valve (TV) z-score median was − 0.22
right ventricle decompression. The search was performed (− 0.66, 1.18) and 0.61 (− 0.03, 1.17) respectively. Prepro-
in November 2022 using the following keywords, separate cedural echocardiographic measurements are presented in
and in combination: “pulmonary atresia”, “pulmonary valve Table 2. Three patients in group B had VCACs, no patient
Table 1  Patient characteristics and echocardiogram measurements preoperative for patients whose right ventricle was not decompressed
Variable Treatment hospital p-value
Queen Silvia Children's Hospital in Gothenburg Skåne University Hospital, Lund
(N = 12) (N = 21)
Count Median Valid N Count Median Valid N

Female gender 5 (42%) 12 10 (48%) 21

Weight (kg) 3.26 (2.79, 3.44) 12 3.31 (2.89, 3.65) 21 0.42
Body surface area 0.21 (0.19, 0.22) 12 0.21 (0.19, 0.23) 19 0.63
Type of atresia 0.024*
Membranous 2 (17%) 12 12 (57%) 21
Muscular 10 (83%) 12 9 (43%) 21
VCACs 7 (70%) 10 9 (60%) 15 0.69
TV diameter 7.5 (5.8, 9.2) 12 6.7 (5.6, 9.6) 17 0.84
Z-score − 2.08 (− 3.21, − 1.37) 12 − 2.43 (− 2.75, 14 0.58
− 0.31)
TV diameter/ MV 0.50 (0.41, 0.68) 12 0.57 (0.47, 0.82) 16 0.32
diameter ratio
PV diameter 5.00 (4.00, 6.00) 10 6.40 (5.75, 7.15) 15 0.017*
Z-score − 2.33 (− 2.72, − 1.57) 10 − 1.52 (− 2.01, 14 0.035*
− 0.85)
Main pulmonary 6.00 (5.50, 7.00) 10 7.20 (6.20, 8.07) 18 0.067
artery
Circulation type
Univentricular 11 (92%) 12 17 (81%) 21 0.63
1,5-Ventricular 0 12 1 (4.8%) 21 > 0.99
Biventricular 1 (8.3%) 12 3 (14%) 21 > 0.99

All valve and vessel diameters are specified in millimetres. Body Surface Area is measured in square meters, calculated using the Boston Children's Hospital Z-Score Calculator. Data are
reported as count (percentage) or median (IQR). *p-value < 0.05
VCACs ventriculocoronary artery connections, TV tricuspid valve, MV mitral valve, PV pulmonary valve, (1,5-ventricle) open pulmonary valve and bidirectional Glenn anastomosis
Pediatric Cardiology
Pediatric Cardiology

Table 2  Patient characteristics and echocardiogram measurements preoperative for patients whose right ventricle was decompressed
Variable Patients treated with catheter-based intervention Patients treated with heart surgery p-value
(N = 18) (N = 16)
Count Median Valid N Count Median Valid N

Female gender 4 (22%) 18 7 (44%) 16

Weight 3.87 (3.40, 4.15) 18 3.30 (2.89, 3.58) 0.018*
Body surface area 0.24 (0.22, 0.25) 18 0.22 (0.19, 0.23) 0.038*
Type of atresia
Membranous 18 (100%) 16 (100%)
Muscular 0 0
VCACs 0 3 (23%) 0.064
TV diameter 11.50 (10.25, 14.00) 18 12.00 (10.75, 13.00) 16 0.79
Z-score − 0.04 (− 0.66, 1.28) 18 0.52 (− 0.50, 1.05) 16 0.66
TV diameter/ MV diameter ratio 0.93 (0.85, 1.09) 18 0.95 (0.78, 1.04) 16 0.67
PV diameter 6.95 (6.00, 7.00) 18 7.00 (6.88, 7.85) 16 0.11
Z-score − 1.67 (− 2.02, − 1.39) 18 − 0.96 (− 1.10, − 0.47) 16 0.002*
Main pulmonary artery 9.45 (8.25, 10.00) 18 8.20 (7.45, 9.25) 16 0.021*
Dysplasia of TV 3 (17%) 2 (12%) > 0.99
Circulation type
Univentricular 0 18 1 (6.3%) 16 0.47
1,5-Ventricular 2 (11%) 18 0 16 0.49
Biventricular 16 (89%) 18 15 (94%) 16 > 0.99

All valve and vessel diameters are specified in millimetres. Body Surface Area is measured in square meters, calculated using the Boston Chil-
dren's Hospital Z-Score Calculator. Data are reported as count (percentage) or median (IQR). *p-value < 0.05
VCACs ventriculocoronary artery connections, TV tricuspid valve, MV mitral valve, PV pulmonary valve, (1,5-ventricle) open pulmonary valve
and bidirectional Glenn anastomosis

had RVDCC. Five patients had tricuspid valve dysplasia, Outcomes

three in group A and two in group B.
There were few associated extracardiac malformations There was no mortality in either group during the entire
or genetic disorders. Two patients had trisomy 21. One study period. One patient was lost to follow-up after dis-
patient had undescended testicle, one patient had hip joint charge. Excluding this patient, follow-up time ranged from
luxation, one patient had cortisol deficiency. 2 to 15 years (median 9 years, 6, 13).
In group A, all attempted perforations with radiofre-
quency of the pulmonary valve were successful. Two out
Interventional Technique of five of the attempted mechanical perforations were suc-
cessful. The 3 patients that had a failed mechanical perfora-
In group A the initial intervention consisted of a catheter- tion were included in, and their outcome was analyzed with,
based valvotomy. Followed by a balloon dilation of the group B. 16 out of 18 patients reached biventricular circula-
valve. No patient received additional source of pulmonary tion as the end result while 2 patients had a one-and-a half
blood flow placed during the initial intervention. ventricle (1,5-ventrice) repair (open pulmonary valve and
In group B all patients underwent surgical valvulotomy. bidirectional Glenn anastomosis) at the end of the study. In
Additionally, during the initial intervention five patients group B, 15 out of 16 patients reached biventricular circu-
underwent atrioseptostomy, one patient had a fenestrated lation while one patient required univentricular palliation.
patch placed in the ASD. Nine patients had a modified In group A, 30 days after their initial intervention eight
Blalock-Taussig shunt (mBTS), three patients had a patients had no additional pulmonary blood flow and ten
transannular patch repair, two patients had a homograft patients had additional pulmonary blood flow through either
to replace the pulmonary valve, one patient had a dilation a mBTS or a PDA-stent, see Table 3. In group B 30 days
of the pulmonary bifurcation, one patient had the PDA after their initial intervention four patients had no additional
ligated. pulmonary blood flow and twelve patients had additional
 Pediatric Cardiology

Table 3  Reinterventions, outcomes, and complications 30 days after initial intervention

Variable Patients treated with catheter- Patients treated with heart p-value
based intervention surgery
(N = 18) (N = 16)
Count Valid N Count Valid N

Additional interventions performed during initial intervention 0 18 13 (81%) 16 < 0.001

Reintervention within 30 days after initial intervention 10 (56%) 18 4 (25%) 16 0.34
Outcome 30 days after initial intervention
Valvulotomy alone 8 (44%) 18 3 (19%) 16 0.31
Valvulotomy + patch in the pulmonary valve annulus 0 18 1 (6.3%) 16 0.49
Valvulotomy + additional source of pulmonary blood ­flowa 10 (56%) 18 12 (75%) 16 0.78
Complication within 30 days after initial intervention 7 (39%) 18 3 (19%) 16 0.47
Of which occurred periprocedural 1 (5.6%) 18 0 16 1
a
Additional source of pulmonary blood flow; Blalock-Taussig shunt, Patent Ductus Arteriosus-stent
Data are reported as count (percent)

pulmonary blood flow through a mBTS. Four patients with respectively (p-value 0.11). Seven patients in group A were
tricuspid valve dysplasia achieved biventricular circulation not admitted to the intensive care unit at all. The patients
(3 in group A and one in group B), one patient in group B in group B exhibited a tendency towards a shorter overall
ended up with univentricular circulation. hospital stay compared to group A, median 18 (10, 20) and
One patient in group B was lost to follow-up after hospital 21 (15, 36) days, respectively (p-value 0.078).
discharge due to living abroad.
Reinterventions
Length of Hospital Stay
Group A 16 out of 18 patients in group A had at least one
Patients in group A had a tendency towards fewer days in reintervention, see Fig. 1. Ten patients had a reinterven-
the intensive care unit during their initial procedural admis- tion within 30 days after their initial intervention, five had
sion, i.e., when they had their initial intervention, compared a re-catheterization where they received a PDA-stent and
to patients in group B, median 3 (0, 11) and 7 (3, 17) days, five patients underwent heart surgery to facilitate additional

Fig. 1  Overview of reinterventions

Pediatric Cardiology

pulmonary blood flow, see Table 3. 14 patients underwent was discharged after 14 days. Furthermore, within 30 days
at least one re-catheterization. Eleven patients underwent after their initial intervention, six patients experienced com-
heart surgery after the initial catheterization. Nine patients plications, including three shunt occlusions in arterial pul-
had moderate-severe tricuspid regurgitation (TR) preopera- monary shunts (AP-shunts) (one of which led to circulatory
tively, three of these patients needed additional pulmonary collapse and brain hemorrhage) one case of cerebral infarc-
blood flow after initial catheterization, none of the patients tion, one case of necrotizing enterocolitis and acute kidney
that needed a reintervention within 30 days after initial inter- failure, and one case of infection. The patients who received
vention had tricuspid valve dysplasia. During long-term fol- a PDA-stent had no complications related to the stents.
low-up (after the initial procedural admission), there were In group B, three patients had a failed balloon valvul-
in total 24 catheter-based reinterventions and 16 surgical. otomy before undergoing heart surgery. Within 30 days
after the initial intervention, three patients in group B expe-
Group B Eleven out of 16 patients in group B had at least rienced complications: one patient had diaphragm paresis,
one reintervention, of whom four patients had a reinter- one patient had anuria and needed dialysis, one patient had
vention within 30 days after the initial intervention, all of a stroke, suffered arrythmia, had a deep vein thrombosis,
which were surgical, see Table 3. Nine patients received a needed dialysis and ECMO and also suffered necrosis of
mBTS in their first intervention in addition to the surgical fingers and toes.
valvulotomy. Six patients had at least one catheter-based
intervention. Ten patients had at least one reoperation. Six
patients had moderate-severe TR preoperatively, one of Systematic Review of Current Literature
whom required reoperation within 30 days after their first
surgery, this patient had tricuspid valve dysplasia. During 422 articles were identified, after duplicates were removed
long-term follow-up, there were in total nine catheter-based 414 articles remained and their abstracts were screened for
reinterventions and 14 surgical. relevance, see Fig. 2. All studies were assessed in regard
to inclusion criteria and after that 25 studies were included
Complications in the review. Partially the same patient population were
studied in two studies [10, 11]. In order to avoid analyzing
In group A, one patient experienced a periprocedural com- duplicate results, data have been collected from all studies
plication, a right ventricle perforation and atrial flutter dur- but each variable from each patient population was only ana-
ing catheterization, this resolved spontaneously, the patient lyzed once, see Table 4. The objectives, settings, inclusion

Fig. 2  Systematic literature

search according to Preferred
Reporting Items for Systematic
Reviews and Meta-Analyses
(PRISMA)—guidelines
 Pediatric Cardiology

Table 4  Summary of systematic literature review

Study Number of Primary way of treat- Successful opening Reintervention Complication peri- or Early
participants ment of the pulmonary post procedure post procedure ­mortalitya
valve

Alwi (2013) [6] 8 CatheterM 7 2 1 Infection, not known 0

what kind
Bakhru (2017) [12] 20 CatheterM 16 10 2 Perforation of the 3
pericardium, of whom
1 tamponade
Brown (2017) [13] 5 CatheterRF 5 2 1 Perforation of the 0
pericardium
Cho (2013) [2] 9 CatheterM 7 10 3 Artery thrombosis 0
El Saiedi (2018, 2022)b 50 CatheterM, RF 39 9 7 Perforation of the 19
[10, 11] pericardium
El Shedoudy (2018) [14] 13 CatheterM 11 1 1 Sepsis, 1 Heart failure, 2
2 Artery thrombosis
Haddad (2021) [15] 18 CatheterM 14 7 5 Perforation of the 2
pericardium, of whom
4 tamponades
Hascoët (2019) [8] 29 CatheterRF 23 16 2 Perforation of the 7
pericardium with tam-
ponade, 1 Stroke,
4 Septic shock, 1 NEC,
1 AKI,
1 ­Hypothyroidismc
Hu (2015) [16] 22 Hybrid 19 2 1 Low cardiac output 2
syndrome,
1 Ventricular tachy-
cardia
Kamali (2021) [17] 26 CatheterM, RF 22 5 2 Vascular complica- 3
tions,
1 Cardiac arrest
Kim (2015) [18] 7 CatheterM 7 4 1 Atrial fibrillation 0
Lamers (2012) [9] 11 CatheterRF 11 3 4 Thrombosis 0
Lawley (2021) [19] 15 12 ­CatheterU 10 Catheter 6 Catheter: 4 Hemoperi- 2
3 Surgery 3 Surgery cardium, of whom 3
tamponades
Lefort (2019) [20] 5 CatheterM 5 2 0 0
Guanhua (2020) [21] 15 Surgery 15 NA NA 0
Li (2013) [22] 10 Hybrid 10 1 1 Arrythmia, 2 Bleeding 0
Li (2011) [23] 30 Hybrid 30 1 0 0
Lin (2017) [24] 38 Hybrid 38 0 8 Pneumonia, 3 Right 3
heart failure, 2 AKI, 3
Arrythmia
Odemis (2013) [7] 8 CatheterRF 8 NA NA NA
Patil (2016) [25] 9 CatheterM 8 1 1 Perforation of the 1
pericardium with
tamponade
Prakoso (2022) [26] 12 CatheterM 12 NA 1 Supra ventricular tach- 0
ycardia, 1 Tamponade
and cardiac arrest
Shaath (2012) [27] 20 CatheterRF NA 11 NA NA
Song (2022) [28] 100 Surgery/ Hybrid 100 0 2 Hypoxemia, 2 Right 6
heart failure, 1 Pneu-
monia, 1 Sepsis
Yoldas (2020) [29] 31 CatheterRF 31 16 5 Arrythmia, 3 Arterial 6
thrombosis, 2 Perfora-
tion of the pericardium
Pediatric Cardiology

Table 4  (continued)
Study Number of Primary way of treat- Successful opening Reintervention Complication peri- or Early
participants ment of the pulmonary post procedure post procedure ­mortalitya
valve
Zampi (2014) [30] 24 17 Surgery 24 2 Surgery: 3 Arrythmia, 2 0
7 Hybrid Cardiac arrest, 1 AKI,
3 infections, 4 NEC,
1 ­otherd
Hybrid: 4 Arrythmia, 2
Cardiac arrest, 3 AKI,
5 Infections, 5 NEC,
1 ­otherd

Only first author is given for each study

M mechanical perforation of the pulmonary valve, RF radiofrequency perforation of the pulmonary valve, U unknown type of catheter used for
perforation of the pulmonary valve, NEC Necrotizing enterocolitis, AKI Acute kidney injury
a
Death during the first hospitalization, b2 articles, cSeveral smaller complications not specified further, dNot defined further

criteria and z-score models of the included studies differed, Outcomes

and a meta-analysis could therefore not be performed.
Success Rate and Mortality
Patient Characteristics
The success rate of catheter-based intervention in perforat-
The inclusion criterion in several studies was based on TV ing the pulmonary valve ranged between 74 and 100% [2,
z-score, it ranged from > − 5 to > − 1 [9, 11, 12, 14, 17, 29]. 6–10, 12–15, 17, 18, 20, 25, 26, 29] and for hybrid approach
All patients were considered to have a favorable morphol- 86–100% [16, 22–24, 28, 30, 31], see Table 4. Failed inter-
ogy of the right ventricle, suitable for biventricular repair. vention was correlated to lower age, weight and BSA [10].
A few patients had a muscular atresia or a hypoplastic right The early mortality (death within 30 days post procedure or
ventricle [11, 23]. tricuspid valve dysplasia was an exclusion death during the first hospitalization) varied from 0 to 44.1%
criterion in several studies [14, 16]. across studies (hybrid procedure 0–9%, catheter-based pro-
cedure 0–44.1%, surgical procedure 0–12%) [10, 12, 14–17,
Intervention Approaches 19, 21, 24, 25, 29]. The leading cause of death in several
studies was infection or sepsis [11, 15, 24, 25, 29]. Pro-
The included studies evaluated different intervention longed stays in the PICU were found to be associated with
approaches including surgical decompression of the right higher mortality rates, even if the patients initially survived
ventricle, hybrid interventions, radiofrequency perforation the intervention.
with subsequent balloon dilation of the pulmonary valve, The length of follow-up varied among the included stud-
and various mechanical perforation techniques using chronic ies, ranging from no follow-up after discharge from hospital
total occlusion (CTO)-wires and coronary guidewires. Dif- to 5.4 years [6, 7, 9, 11–25, 29, 30].
ferent studies have investigated the possibility to use the soft
or the stiff end of different coronary wires and if a retrograde Predictors and Likelihood for Biventricular
approach instead of the antegrade, which is standard in most Circulation or Reintervention
centers, can have benefits [2, 6, 11, 12, 14, 15, 17, 18, 20,
25, 26]. The hybrid approach has emerged as an alternative Several studies have focused on identifying predictors of
to percutaneous treatment, aiming to reduce the perforation biventricular circulation and the likelihood of reinterven-
risk and enabling the placement of a mBTS if necessary tion or need for additional pulmonary blood flow [7, 9, 19,
without requiring additional interventions. The technique 22, 24, 27, 29]. Reintervention has been defined in various
involves a midline sternotomy and under transesophageal ways across the included studies, the most common type of
echocardiographic guidance the pericardium and pulmonary early reintervention involves additional pulmonary blood
valve is perforated with a needle and balloon pulmonary flow, either through a mBTS or a PDA-stent [6, 9, 11–17,
valvuloplasty is then performed [16, 22–24, 30]. Cardiopul- 19, 20, 22, 23, 25, 29]. Lawley et al. found that a small
monary bypass (CPB) is avoided and the ductus arteriosus TR preoperative was negatively correlated with biven-
can be snared, if the patient then desaturates a stent in the tricular circulation [19]. Prakoso et al. deemed patients
ductus or a mBTS can be placed. with a severe TR unfit for biventricular circulation [26].
 Pediatric Cardiology

Shaath et al. showed that a peak gradient above 43 mm Complications

Hg of the TR was a risk factor for reintervention [27]. A
larger tricuspid valve (TV) z-score was associated with The complication rate differed between the included studies
greater right ventricle growth and served as a good predic- in frequency and in severity, see Table 4.
tor for biventricular circulation [29]. El Shedoudy et al.
showed that there can be significant growth of the TV- Cost
diameter z-score after intervention [14]. Shaath et al. did,
however, see no significant change of the tricuspid valve Radiofrequency perforation of the pulmonary valve is a
z-score, indicating that the right ventricular growth was more expensive intervention compared to mechanical per-
not affected by the intervention [27]. A small pulmonary foration but is less costly than heart surgery. Mechanical
valve (PV) and TV showed to be a risk factor for reinter- perforation with various types of wires has been developed
vention [29]. Hu et al. state that a small PV annulus is as a cost-efficient alternative, utilizing multipurpose equip-
not possible to enlarge through repeated balloon dilation, ment that is more readily available in cardiac centers with
but the intervention will only result in a large pulmonary budgetary limitations [6, 11, 12, 14, 15, 17, 18, 22, 25].
regurgitation (PR) [16]. El Saiedi et al. argue that a larger
balloon/valve ratio and repeated balloon dilations with
the aim to lower post-procedural right ventricle pressure Discussion
(RVP) might benefit right ventricle growth and reduce
the risk for reintervention [10]. The size of the pulmo- Retrospective Population‑Based Multicenter Study
nary regurgitation has been suggested to be smaller after
a hybrid procedure or surgical valvulotomy compared to In our study we found that decompression of the right ven-
after a catheter-based balloon dilation of the pulmonary tricle could be performed safe, with no associated mortality,
valve [23, 28]. Authors to studies evaluating the hybrid using either catheter-based intervention or heart surgery.
approach mention that the balloon dilation can render a It was a tendency towards shorter time in the PICU for the
severe pulmonary regurgitation and they therefore argue to patients who were treated with catheter-based intervention
use a smaller maximal diameter of the balloon [16, 23, 24]. initially (p-value 0.11). A reduced PICU stay can have a pos-
Song and Chen found that when perforating the pulmonary itive effect on the parent–child bonding parents [32]. Heart
valve and balloon dilating the valve, in the setting of a surgery may hinder practices like kangaroo care, commonly
hybrid intervention, the perforation needle can be deviated performed in PICUs. Among the patients in group A, only
from the intended perforation point which could add to the five required heart surgery within 30 days after the initial
risk for pulmonary regurgitation [28]. intervention. Avoiding PICU-care and open chest procedure
Patil et al. noticed that the oxygenation of the patients can also lower the risk for nosocomial infections. CPB is
did not improve significantly until 2–3 days after the associated with a known risk for acute kidney injury, which
intervention [25]. Li et al. demonstrated that saturation three patients in our material suffered from all of whom had
does not normalize immediately after decompression due gone through surgery and CPB [33, 34].
to residual outflow tract obstruction [22]. Postoperative The approach regarding supplementary procedures dur-
hypoxemia could result from muscle edema in the right ing the initial intervention differed in the two cohorts. In
ventricle and the limited capacity of a hypoplastic right group B an additional procedure, such as but not limited to,
ventricle to support sufficient pulmonary blood flow [28]. an extra source for pulmonary blood flow was added during
Post-procedure RVP was directly proportionate to the need the initial intervention based on preoperative morphologic
for prostaglandin [10]. In several centers, if patients can- traits while in group A an additional procedure was planned
not be weaned off prostaglandin after a set number of days for in a second session or decided on when the patient was
and their saturation remains below 70–80%, the ductus not able to be weaned of prostaglandin. There was a ten-
arteriosus is stented or an mBTS is placed [9, 12, 15]. In dency towards fewer reinterventions within 30 days after the
other institutions a PDA-stent or a mBTS is placed in the initial intervention among patients who underwent surgical
same session if the saturation is unsatisfactory when the intervention initially (p-value 0.34), consistent with previous
ductus is closed, or certain morphologic features are pre- studies [35, 36]. Regardless of the initial intervention tech-
sent [6, 12, 13]. If a PDA-stent is placed during the initial nique, the most common reintervention aimed to augment
intervention there are fewer early reinterventions [11]. Li pulmonary blood flow. Among patients initially undergoing
et al. showed that hybrid therapy carries a reduced risk of heart surgery, nine out of 16 (56%) received an additional
reintervention compared to catheter-based treatment [23]. source of pulmonary blood flow during their first interven-
In their study all infants aged under a month old had their tion. This treatment strategy may, at least partially, account
ductus arteriosus ligated and a mBTS placed. for the lower rate of reinterventions in this group. The
Pediatric Cardiology

patients that were treated with catheter-based intervention contribute to the possibility of biventricular circulation and
did, however, have a smaller PV z-score preoperatively (see reduced need for reintervention.
Table 2, p-value 0.01), which in previous studies has shown The studies included in this review have different inclu-
to be a risk factor for reintervention [29]. Half of the patients sion criteria, follow-up durations and varying definitions of
(5 patients) that underwent a catheter-based intervention ini- reintervention. Therefore, meaningful comparisons can only
tially and needed a reintervention within 30 days could be be made in terms of short-term outcomes. Moreover, the age
managed with a new catheter-based intervention, insertion range of patients varies across studies, from newborns to
of a PDA-stent, and thereby avoiding CPB in the neona- 1.5 years old [12, 24]. The use of different methods to cal-
tal period. Comparisons between mBTS to PDA-shunts for culate z-scores sometimes makes it challenging to replicate
patients with duct-dependent pulmonary circulation have results [41]. Notably, all the studies included in this review
shown that patients with PDA-stents compared to patients considered the included patients to have favorable anatomy,
with a mBTS had a lower risk of mortality, shorter hospi- with the smallest tricuspid valve z-score ranging from − 5
tal stay, and more reinterventions [37–40]. Thus, placing a to − 1. Several unaccounted factors, such as prenatal diag-
PDA-stent instead of an AP-shunt, with interventional tech- nostic prerequisites, birthing settings, access to advanced
nique, might be an increasingly attractive option for patients neonatal care and pediatric intensive care units (PICUs),
initially treated with a transcatheter approach. It is worth and the prevalence of resistant bacteria, may also influence
noting that patients treated with heart surgery often undergo the outcomes and need to be considered. The often small
additional procedures, in addition to valvulotomy, during the study populations where individual patient outcomes heav-
initial intervention. Therefore, counting reinterventions may ily affect the overall results for the group might also have
not provide a conclusive overview of treatment outcomes. impacted the sometimes conflicting results observed in the
Looking specifically at the need for extra pulmonary blood reviewed studies.
flow within 30 days can offer a more accurate assessment Only four of the included studies evaluated initial heart
of the overall situation. This study cannot, due to the retro- surgery [19, 21, 28, 30]. It is possible that the variations in
spective nature of the study design, draw any conclusions publication frequency could be influenced by centers utiliz-
regarding if patients treated with surgery have an increased ing different intervention techniques, with a greater inclina-
need for supplementary procedures or if the difference in tion towards publishing. Given that surgery is already an
additional interventions between the two groups is based on established treatment option, this factor may also impact
institutional preference. the likelihood of publication. Alternatively, it could be that
We did not identify any preoperative echocardiographic there are fewer centers that perform heart surgery compared
variables that could predict the need for reintervention to percutaneous treatments. Surgery offers the advantage
within 30 days after the initial intervention. However, the of adaptability during the procedure, allowing surgeons to
compliance of the right ventricle, which is challenging to address potential issues in a single session and potentially
evaluate through echocardiographic examination, can affect reduce the need for reinterventions [35]. It is, however, asso-
the need for additional pulmonary blood flow. Reduced com- ciated with a risk for right ventricle failure and low cardiac
pliance may result from working against high resistance. output syndrome [3, 42]. CPB in the neonatal period is cor-
There was one perforation of the pericardium periproce- related with several risks [4, 43].
dural during catheter-based intervention (5.6%) which is a Percutaneous intervention offers the advantage of avoid-
low periprocedural complication rate comparing to the stud- ing heart surgery and CPB during the neonatal period. It
ies included in the review [2, 6–15, 17–20, 25–27, 29]. Nota- also offers the possibility to place a PDA-stent if needed.
bly, we observed no mortality in the entire study population However, studies have shown higher rates of reinterventions
during long-term follow-up. compared to surgery and hybrid interventions, indicating
The cost of the intervention has been the focus of sev- that CPB might not be entirely avoided [23, 35]. The most
eral studies [11, 13, 14, 25]. In Sweden, with its publicly serious complication associated with catheter-based treat-
financed welfare system, the cost of the intervention is not a ment is a perforation of the right ventricle or of the pul-
determining factor when choosing the type of intervention. monary artery. Various intervention techniques have been
proposed and evaluated to minimize the risk for perforating
Systematic Review of Current Literature the surrounding tissue [7, 9, 11, 13–15, 17, 18, 20].
The hybrid approach avoids CPB while still offering the
There is currently no consensus on specific morphologic opportunity to place a mBTS if needed and it decreases
features that universally determine outcome. Different the risk of perforating the right ventricle or the pulmonary
morphologic traits, such as a large TR (when not associ- artery.
ated with tricuspid valve dysplasia), a well-developed right Previous studies have suggested that decompressing
ventricle, and a large tricuspid valve annulus, are believed to the right ventricle and establishing blood flow over the
 Pediatric Cardiology

pulmonary valve may improve the chances of achieving However, a catheter-based approach followed by conversion
biventricular circulation by promoting right ventricle growth to a hybrid intervention, if necessary, offers an appealing
[23, 44]. The acceptable oxygen level varies and influences alternative. This approach is less invasive than heart surgery
the decision regarding the need for additional pulmonary and provides the flexibility to incorporate a PDA-shunt dur-
blood flow. There has been a proposed correlation between ing the same session or put in a mBTS without using CBP.
both prostaglandin (PGE1) treatment and duct-dependent
Acknowledgements The study received Grants from The Gothenburg
pulmonary circulation and necrotizing enterocolitis, this Society of Medicine, Number GLS-973750.
could speak in favor for shorter PGE1 treatment and ear-
lier establishment of additional pulmonary blood flow via a Author Contributions All authors contributed to the study conception
mBTS or a PDA-shunt [8, 45, 46]. and design. Material preparation, data collection and analysis were per-
formed by Stina Manhem, Michal Odermarsky, Håkan Wåhlander and
Britt-Marie Ekman-Joelsson. The first draft of the manuscript was writ-
Strengths and limitations ten by Stina Manhem and all authors commented on previous versions
of the manuscript. All authors read and approved the final manuscript.
Retrospective Population‑Based Multicenter Study
Funding Open access funding provided by University of Gothenburg.

The major strength of the study is the inclusion of all Data Availability No datasets were generated or analysed during the
patients with PA-IVS born in Sweden for 13 years and the current study.
pseudo randomization design of the study. The heart defect
is, however, rare, so the number of patients remains small, Declarations
which may have influenced the lack of statistical significance Conflict of interest The authors declared no potential conflicts of inter-
observed for several variables, especially when further sub- est with respect to the research, authorship, and/or publication of this
grouping would be desirable. The small study population article. The authors have no relevant financial or non-financial interests
motivates the combination with the systematic literature to disclose.
review and despite the limited study population safety for the Ethical Approval The study was approved by the Central Ethical
procedures could still be demonstrated with statistical sig- Review Board at the University of Gothenburg, case number 960–15.
nificance. The angiograms and echocardiograms have been
performed and interpreted by different personnel and with Open Access This article is licensed under a Creative Commons Attri-
different equipment which could make the results harder to bution 4.0 International License, which permits use, sharing, adapta-
compare but it also limits the risk of bias. Our results also tion, distribution and reproduction in any medium or format, as long
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the article’s Creative Commons licence and your intended use is not
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and review of the subject.

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