Outcome of Infants With Ductal-Dependent Pulmonary Atresia and Ventricular Septal Defect in a Developing Nation: A Single-Center Experience

Abstract

Objectives: The number of publications on the outcomes of treatment of ductal-dependent pulmonary atresia-ventricular septal defect in developing countries is limited. This study examines the results of surgical repair of this complex congenital heart disease at a high-volume center in Vietnam. Methods: This retrospective study included 144 patients with primary biventricular repair (group p-BVR, n = 70, 48.6%) or staged repair (group SR, n = 74, 51.4%) from April 2014 to March 2023. Concomitant augmentation of individual pulmonary arteries with autologous pericardial patches was performed in most patients who underwent the Rastelli operation (115/120 patients). Results: The overall rates of freedom from right ventricular outflow tract (RVOT) reintervention after the Rastelli operation at 1 year and 6 years were 94% (95% confidence interval [CI], 90%-99%) and 79% (95% CI, 69%-91%), respectively, with a median of 1.9 years (IQR, 0.9-5.0 years) from the Rastelli operation to the first reintervention. Fine and Gray's model for cumulative incidence across groups revealed that being in the p-BVR group was a high-risk factor for RVOT reintervention but not significant relative to being in the SR group (P = .12, SHR = 2.22, 95% CI, 0.82-6.03). The overall survival rate at 6 years was 89% (95% CI, 82%-97%) in the p-BVR group and 76% (95% CI, 66%-87%) in the SR group (P = .048). Conclusions: Concomitant pulmonary arterioplasty may contribute to or be associated with favorable reintervention rates and should be considered in patients with ductal-dependent pulmonary atresia with ventricular septal defect who undergo the primary Rastelli operation.

Keywords

pulmonary atresia ventricular septal defect Rastelli operation right ventricular outflow tract obstruction pulmonary branches stenosis

Introduction

Pulmonary atresia and ventricular septal defect (PA-VSD) is a complex and heterogeneous congenital cardiopulmonary malformation characterized by a deficiency of luminal continuity from the right ventricle (RV) to the pulmonary arteries (PAs), combined with a large malaligned VSD resulting from anterior deviation of the conal septum.¹

There are two concurrent treatment strategies, primary one-stage biventricular repair (p-BVR) and staged surgical repair (SR), with a greater survival rate in p-BVR patients than in those treated with SR but also a significantly greater incidence of right ventricular outflow tract (RVOT) obstruction.^2–5 In contrast, patients treated with SR have a decreased probability of requiring reoperation or reintervention during follow-up compared with patients treated with p-BVR but also have a higher mortality after shunt palliation and during the interim period before the Rastelli-type procedure.^3,4 Furthermore, many studies have shown that a substantial proportion of patients have early distal conduit or PA branch stenosis after RVOT reconstruction, especially infants and young children.^6–8

In Vietnam, in the conditions of a developing country, we have no homograft material, as well as limited fetal diagnosis, and late detection is rather common. Before 2016, almost all patients in our center received Blalock-Taussig-Thomas (BTT) shunt surgery followed by Rastelli surgery at 1.5 to 2 years of age due to limited access to the conduit and concerns that the PA was too small to undergo total repair. The interruption of Gore product distribution in Vietnam poses significant challenges for patients with congenital heart defects with ductal dependencies, particularly those who need an aortopulmonary shunt or Fontan operation. Since 2016, the availability of the Contegra conduit has shifted our treatment strategy for this complex group of disorders, resulting in early complete Rastelli SR for young infants or neonates with lower body weight. However, we are faced with the question of whether early p-BVR or SR will increase the burden of postoperative intervention as in previous studies.

The primary objective of this study was to describe our experience with operative repair of ductal-dependent PA-VSD patients via both p-BVR and SR approaches and to determine the impact of branch PA arterioplasty on clinical outcomes, especially related to RVOT reintervention.

Materials and Methods

Patients and Definitions

This study was approved by the Vietnam National Children's Hospital Research Ethics Council (Ref No: 1076/BVNTU-HDDD in June 2022). A retrospective review was performed for all data collected, and informed consent was waived according to our National Legislation and Institutional Requirements.

Between April 2014 and March 2023, a total of 205 patients diagnosed with PA-VSD underwent SR at Vietnam National Children's Hospital. Patients who were diagnosed with PA-VSD-MAPCA (61 patients) were excluded, and 144 patients who were PA-VSD and ductal-dependent were analyzed—70 patients who underwent p-BVR and 74 patients who underwent SR.

In accordance with our national legislation and institutional requirements, informed consent was waived because of the observational nature of the study. Preoperative, intraoperative, postoperative, and follow-up data were acquired via retrospective review of medical records.

Patient follow-up was completed via regular checkups at 1 month, 3 months, 6 months, and every year after operation at the outpatient clinic department. Contact was maintained with the patient's family via telephone and postal or through direct contact with the referring hospital to obtain external medical records when the patient lived far from Hanoi.

The criteria for p-BVR were a Nakata index greater than 130 mm²/m² and echocardiogram demonstrating balanced ventricles. The BTT shunt was indicated as the first palliation if the patients had a Nakata Index smaller than 130 mm²/m² or patients admitted to our hospital with severe cyanosis combined with multiple organ failure due to late presentation. A central shunt was indicated as the primary operation if severe hypoplasia of both PA branches occurred. Ductal stenting and right ventricle-to-pulmonary artery (RV-PA) shunts were rarely performed in selected patients.

Early mortality was defined as death occurring in the hospital before discharge or within 30 days after the initial operation. Interim mortality was defined as death occurring after discharge from the hospital after shunt palliation and before the Rastelli-type procedure in the SR group. Patients who survived after the shunt operation but completed the Rastelli operation at other institutions (four patients) were excluded from the statistical analysis of the Rastelli procedure. All other deaths were considered late deaths. RVOT reintervention was defined as reoperation or cardiac catheterization related to RVOTO, distal conduit or PA branch stenosis, or severe conduit regurgitation.

Pulmonary Artery Diameter Measurement

A pediatric cardiologist reviewed all echocardiograms, multislice computed tomography (CT), and cardiac catheterization data. The right PA (RPA) and left PA (LPA) diameters measured immediately at the hilum during systole were used to determine the Z scores, and the Nakata index was calculated as the sum of the left and right pulmonary cross-sectional areas indexed to the body surface area. The diameter of the branch PAs, each branch PA Z score, and the Nakata index were compared before the first operation.

Surgical Techniques

The Rastelli-type procedure was performed via median sternotomy with cardiopulmonary bypass and moderate hypothermia. The PA branches were dissected up to the hilum to perform pulmonary arterioplasty with a beating heart to reduce the ischemic time of the aortic cross-clamp. If the patient had a small native PA, irrespective of the diameter, the proximal PA was ligated and divided, with the PA stump preserved as a PA bifurcation. Pulmonary arterioplasty was performed on individual PA branches with separate triangular untreated autologous pericardial patches. If the native PA did not exist, a single rectangular pericardial patch was used to perform pulmonary arterioplasty from the hilum to the hilum, and the central part of the patch was perforated to create the distal RV-PA connection. The distal RV-PA conduit was then sutured to the PA bifurcation before cardioplegia arrest (Figure 1). In neonates or small infants with a body weight less than 5 kg, we always consider reducing the RV-PA conduit size by performing resection of one cusp of the Contegra conduit 12 mm in diameter to create a non-oversized bicuspidized conduit 8 to 9 mm (Supplemental Video 1).

Our institutional protocol for the BTT shunt was as follows: (1) The size of the shunt was calculated according to the body weight (<3 kg: 3 mm; 3-4 kg: 3.5 mm and >4 kg: 4 mm polytetrafluoroethylene graft). (2) The shunt direction was contralateral to the direction of the aortic arch, which was the left side shunt in a patient with a right aortic arch. A central shunt was placed from the ascending aorta to the main PA in patients with severe PA hypoplasia. Patent ductus arteriosus (PDA) stenting was performed in patients whose left PDA and right aortic arch were affected. The median time from the first palliation to the Rastelli-type procedure was 1.02 years (IQR, 0.78-1.34 years).

As per our protocol, all patients received anticoagulant/antiplatelet medications following the BTT shunt and the Rastelli operation. Heparin was administered at 10 IU/kg/h for the first three to five days following the procedure, and aspirin was administered at a dose of 3 to 5 mg/kg/day for the rest of the patient's life.

Data Analysis

Categorical variables are presented as absolute frequencies and percentages. Continuous variables are presented as medians (interquartile ranges) for skewed data. Categorical variables were compared via Pearson χ² test. Continuous variables were compared via Wilcoxon rank-sum test. Fisher exact test was used for cases in which the expected frequency was <5. Predictors for death and RVOT reintervention were determined via a multivariable Cox proportional hazards regression model with a baseline model of multivariable models that included all variables that had a P value <.15. A multivariate variable with a P value <.05 with a hazard ratio and 95% confidence interval (CI) was defined as a multivariate risk factor. The Fine-Gray competing risk regression model was used to plot the cumulative incidence function curve, which compared the two groups’ reoperation hazards. Aalen Johansen methods were used to estimate the survival functions while accounting for competitive risk. Time-dependent endpoints for survival and freedom from reintervention were analyzed via the Kaplan-Meier method, while the log-rank test was used to compare Kaplan-Meier curves, with a P value <.05 indicating statistical significance.

Results

Patient Demographic Characteristics

Among 144 patients who underwent surgical intervention for PA-VSD, 84 (58.3%) were males. These 144 patients were classified into two groups according to the primary operation of each group: the p-BVR group (n = 70, 48.6%) and the SR group (n = 74, 51.4%). There were 10 premature babies (14.3%, 10/70) in the p-BVR group and 11 (14.9%, 11/74) in the SR group.

The median age and median weight at the Rastelli operation in the p-BVR group were 3.4 months (IQR, 1.67-7.47 months) and 4.65 kg (IQR, 3.70-6.85 kg), respectively. Eleven of 70 patients (15.7%) underwent surgery during the neonatal period, and a total of 31 of 70 patients (44.3%) were less than 3 months of age in the p-BVR group. In the SR group, the median age and weight at palliation were 1.4 months (IQR, 0.85-3.1 months) and 3.5 kg (IQR, 3-4.5 kg), respectively, whereas 24 of 74 patients (32.4%) underwent shunt palliation in the neonatal period, and 51 patients (68.9%) were less than three months of age. The Nakata index in the p-BVR group was 174 mm²/m² (IQR, 139-226 mm²/m²), whereas it was 109 mm²/m² (IQR, 88-138 mm²/m²) in the SR group (P < .001). Figure 2 illustrates the patients’ progression diagram for the study's completion, and Table 1 demonstrates the patients characteristics before the first operation.

Table 1.

Patient Characteristics Between Groups Before the First Operation.

Variables	SR group, n (%), median (IQR)(n = 74)	p-BVR group, n (%), median (IQR)(n = 70)	P value
Age (months)	1.5 (0.9, 3.1)	3.4 (1.7, 7.5)	<.001
Weight (kg)	3.80 (3.00, 4.50)	4.65 (3.70, 6.85)	<.001
Dysmorphic			.002
0	59 (80)	39 (56)
1	15 (20)	31 (44)
Nakata index	109 (88, 138)	174 (139, 226)	<.001
Z score
RPA	−1.30 (−1.93, −0.67)	−0.48 (−1.30, 0.43)	<.001
LPA	−1.51 (−2.08, −0.78)	−0.96 (−1.90, −0.08)	.013
Diameter
RPA	4.00 (3.50, 4.50)	5.10 (4.23, 6.40)	<.001
LPA	3.50 (3.10, 4.30)	4.45 (3.70, 5.15)	<.001

Abbreviations: IQR, interquartile range; LPA, left pulmonary artery; PA, pulmonary artery; p-BVR, primary biventricular repair; RPA, right pulmonary artery; SR, staged repair.

Perioperative Variables and Mortality

In the p-BVR group, RV-PA reconstruction involved a Contegra conduit in 64 of 70 patients (91.4%), a transannular autologous pericardial patch in four of 70 patients (5.7%), a Hancock valve conduit in 1 of 70 patients (1.4%), and a polytetrafluoroethylene conduit in 1 of 70 patients (1.4%). Sixty-four patients (91.4%, 64/70) underwent LPA patch arterioplasty, whereas 58 of 70 (82.9%) underwent RPA patch arterioplasty, all with autologous pericardium. The median size of the RV-PA conduit was 12 mm (IQR, 12-14 mm) in the p-BVR group, whereas the median size of the RV-PA conduit was 16 mm (IQR, 14-16 mm) in the SR group (P < .001).

In the SR group, the main palliative procedure was a BTT shunt (85.1%, 63/74), followed by a central shunt (9.5%, 7/74), ductal stenting (4.1%, 3/74), and an RV-PA shunt (1.3%, 1/74). Fifty patients (67.5%, 50/74) in the SR group completed the Rastelli-type procedure at our institution at a median of 12 months (IQR, 9.5-16.3 months) after shunt palliation, whereas four patients (5.4%, 4/74) underwent definitive repair at other institutions (these four patients had all survived at the last follow-up). In 50 patients who underwent the Rastelli procedure at our institution, the RV-PA connection involved a Contegra conduit in 44 patients (88%, 44/50), a Hancock valve conduit in three of 50 patients (6%), a polytetrafluoroethylene valve conduit in two of 50 patients (4.0%), and a transannular patch using autologous pericardium in one of 50 patients (2%). Forty-six of 50 patients (92%) underwent LPA patch arterioplasty, and 41 of 50 (82%) underwent RPA patch arterioplasty concomitant with the Rastelli procedure using autologous pericardium as its only material. The perioperative characteristics of the patients who underwent the Rastelli operation are described in Table 2.

Table 2.

Patient Characteristics at Rastelli Operation.

Variables	SR group, n (%), median (IQR) (n = 74) Patients with completed Rastelli (n = 50)	p-BVR group, n (%), median (IQR)(n = 70)	P value
Bypass time (min)	159 (140, 178)	138 (130, 160)	<.001
Aortic cross-clamp time (min)	76 (63, 102)	67 (57, 79)	.004
RPA plasty	41/50 (82)	58 (83)	>.9
LPA plasty	46/50 (92)	64 (91)	>.9
Conduit size (mm)			<.001
8-10.5 mm	0 (0)	14 (20)
12 mm	3/50 (6)	24 (34)
14 mm	13/50 (26)	22 (31)
16 mm	32/50 (64)	9 (13)
18 mm	2/50 (4)	1 (1.4)
RV/LV pressure ratio	0.50 (0.40, 0.56)	0.49 (0.40, 0.56)	.7
Postop ventilation time (h)	55 (28, 100)	94 (41, 169)	.01
ECMO	0 (0)	6 (8.6)	.04
Overall RVOT reintervention	5/50 (10)	15 (21.4)	.1
RVOT reoperation	5	10
RVOT cardiac catheterization	2 (4)	6 (8.6%)	>.9
Branch PA reintervention	3 (4.1)	9 (13)	.2
Time to first RVOT reintervention (months)	31 (30, 82)	22 (9, 41)	.10
In-hospital mortality	6 (8.1)	5 (7.1)	.4
Overall mortality	16 (21.6)	9 (12.9)	.057

Abbreviations: ECMO, extracorporeal membrane oxygenation; IQR, interquartile range; LPA, left pulmonary artery; PA, pulmonary artery; p-BVR, primary biventricular repair; RPA, right pulmonary artery; RVOT, right ventricular outflow tract; RV/LV, right ventricle/left ventricle; SR, staged repair.

Overall, 11 in-hospital deaths (11/144, 7.6%) occurred, with five deaths (5/70, 7.1%) in the p-BVR group and six (6/74, 8.1%) in the SR group. There were four late deaths (4/70, 5.7%) in the p-BVR group, but eight interstage deaths and two deaths (10/74, 13.5%) after the Rastelli procedure in the SR group. The causes of death and patient characteristics are described in Supplemental Table 1. The overall survival rates at one year and six years after initial surgery were 86% (95% CI, 81%-92%) and 83% (95% CI, 76%-89%), respectively. The survival rates for each group at one year and six years after initial surgery were 91% (95% CI, 85%-98%) and 89% (95% CI, 82%-97%), respectively, in the p-BVR group and 81% (95% CI, 73%-91%) and 76% (95% CI, 66%-87%), respectively, in the SR group (P = .048) (Figure 3). Multivariate Cox analysis revealed that the risk factor for mortality after the Rastelli procedure was an aortic cross-clamp time of more than 80 min (HR, 5.72; 95% CI, 1.26-25.9; P = .024).

Right Ventricular Outflow Tract Reintervention and Risk Factors for RVOT Reintervention

Twenty patients (13.8%, 20/144) underwent 27 RVOT reinterventions during a follow-up of 2.6 years (IQR, 0.9-5.1 years). In the p-BVR group, 15 patients (21.4%, 15/70) underwent reintervention: cardiac RVOT reoperation in five patients (7.1%, 5/70), cardiac catheterization for branch PA dilatation in six patients (8.6%, 6/70), and both reinterventions in four patients (5.7%, 4/70). Five patients (10%, 5/50) required reintervention in the SR group: RVOT reoperations were performed in two patients (4%, 2/50), and both RVOT percutaneous interventions combined with reoperation were performed in three patients (6%, 3/50). The overall rates of freedom from RVOT reintervention after the Rastelli operation at one year and six years were 94% (95% CI, 90%-99%) and 79% (95% CI, 69%-91%), respectively, at a median of 1.9 years (IQR, 0.9-5.0 years) from the Rastelli operation to the first reintervention. The rate of freedom from reintervention for the overall type of RVOT, including stenosis or severe regurgitation at six years, was 69% (95% CI, 53%-90%) in the p-BVR group compared with 91% (95% CI, 82%-100%) in the SR group (P = .059) (Figure 4).

There were 12 patients with distal conduit and/or PA branch stenosis among the 20 patients who needed RVOT reintervention, resulting in rates of freedom from distal conduit and PA branch reintervention at one year and six years of 94% (95% CI, 90%-99%) and 86% (95% CI, 78%-94%), respectively. The rate of freedom from reintervention for distal conduit and PA branch stenosis at six years was 82% (95% CI, 72%-94%) in the p-BVR group and 91% (95% CI, 82%-100%) in the SR group (P = .2). The risk factors for RVOT reintervention after definitive repair were patients who required PGE1 transfusion before the first operation (HR, 4.4; 95% CI, 1.73-11.2; P = .002) and a Z score of less than −1.4 for the LPA (HR, 4.78; 95% CI, 1.82-12.6; P = .002).

A competing risk analysis was performed, with the events being survival without RVOT reintervention, death without RVOT reintervention, and required RVOT reintervention. The cumulative incidence of survival without RVOT reintervention in the p-BVR group at seven years after initial surgery was 68.6% (95% CI, 55.9%-78.3%), that of death without RVOT reintervention was 8.6% (95% CI, 4.1%-18.9%), and that of RVOT reintervention was 20% (95% CI, 11.4%-30.2%); those in the SR group were 85.2% (95% CI, 71.7%-92.6%), 3.7% (95% CI, 6.7%-11.4%), and 9.3% (95% CI, 3.1%-19.7%), respectively (Figure 5). Fine and Gray's model for cumulative incidence across groups revealed that being in the p-BVR group was a high-risk factor for RVOT reintervention but not significant relative to being in the SR group (P = .12, SHR = 2.22, 95% CI, 0.82-6.03).

Discussion

The adverse effects of the Rastelli operation include a heavy burden of RVOT reintervention in patients with ductal-dependent PA-VSD, especially in neonates or small children who undergo p-BVR.^3–5 Given the circumstances in developing countries, the rise in reoperations and reinterventions shortly after surgery will impose additional pressure on the system's limited resources, particularly for high-volume centers. Our study suggested that concomitant pulmonary arterioplasty, especially with the primary Rastelli procedure, created a PA bifurcation that was as close to normal as possible and that integration with a nonoversize conduit might have the potential to limit distal anastomotic stenosis, allowing the natural growth of PA branches after definitive repair of the PA-VSD and potentially reducing the development of PA branch stenosis. In contrast with previous studies,^2–5 our Fine and Gray's model for cumulative incidence across groups demonstrated that the rate of freedom from RVOT reintervention in the p-BVR group is lower but did not differ substantially (P = .12) from that in the SR group, although the weight and age of patients at the time of the Rastelli operation in the p-BVR group were significantly lower than those in the SR group (Figure 5). Moreover, for significantly smaller conduits in the p-BVR group than in the SR group (P < .001), the freedom for reintervention for distal PA and PA branch stenosis did not differ between the groups (P = .2), at least at the six-year follow-up after the Rastelli operation.

Impact of Concomitant Pulmonary Arterioplasty on Right Ventricular Outflow Tract Reintervention

As shown in previous studies, the management of RVOTO after the Rastelli operation in patients with PA-VSD requires multiple reinterventions but combines multiple approaches, including balloon angioplasty catheterization, stent implantation, and reoperation, especially with primary definitive repair.^2–5 Kim et al⁵ reported that RVOTO occurred much earlier in the p-BVR group, with 42.9% and 28.6% freedom from RVOT reintervention at one year and two years, respectively. Lee et al⁴ also demonstrated a sharp decline in freedom from RVOT reintervention during the first two years of life after the Rastelli operation. Data from previous studies show that after RVOT reconstruction, even with a reasonable preoperative Nakata index and equivalent diameter of the RPA and LPA, recurrent RVOTO and hypoplastic PA branches quickly develop.

The mechanism of recurrent RVOTO is complex, but we hypothesized that the main reason is that direct conduit implantation to small PA branches, without a natural PA bifurcation, will create shear stress flow directly on the PA to induce quick stenosis at the distal conduit and PA branches. Another issue in previous studies is that a significant proportion of patients underwent p-BVR with the transannular patch technique. Given the tiny size of the PA branches and the persistently high PA resistance, one of the potential hazards associated with surgery in neonates is the development of severe pulmonary regurgitation. Therefore, establishing a competent pulmonary valve was essential to the success of postoperative resuscitation in our situation, when iNO was frequently inaccessible. In ductal-dependent PA-VSD patients, our surgical approach of enlarging individual PA branches and forming a natural-like PA bifurcation shows promise in reducing recurrent RVOTO, although the use of a small conduit has the unavoidable consequence of requiring RVOT reoperation with conduit replacement. The median time in our study from Rastelli operation to the first RVOTO reintervention was 1.9 years. This demonstrates the potential for delayed RVOT reintervention using our strategy of concomitant PA arterioplasty.

Additionally, previous studies revealed that distal conduit stenosis and younger age were risk factors for RVOT reintervention after Contegra insertion, with freedom from RVOT reintervention ranging from 24.5% to 52.9% at 5 to 6 years.^6–9 Our results modestly suggest that the potential midterm benefit of concomitant PA arterioplasty by the creation of a PA bifurcation that is as close to normal as possible and PA branch enlargement can optimize the outcomes of the Rastelli operation by not only reducing distal PA and PA branch reintervention but also delaying conduit explantation as much as possible for children aged up to six years to implant the largest RV-PA conduit (Table 3). Furthermore, the presence of neonates and small infants was not a risk factor for RVOT reintervention, and a superior rate of freedom from distal conduit and PA branch stenosis (82% at six years) in our p-BVR group, in contrast with previous studies, potentially provides new insight into improving outcomes after primary Rastelli surgery for neonates and young infants. Although our results were encouraging for performing p-BVR without an increased risk of reintervention, a smaller LPA appeared to be a multivariate risk factor for RVOT reintervention, suggesting that patients with LPA coarctation who requested close follow-up after the Rastelli operation and LPA diameter might not benefit in terms of growth, as described by previous studies,^10,11 irrespective of extensive arterioplasty at the time of the Rastelli operation.

Table 3.

Baseline Characteristics of Outcomes Within Studies of Ductal-Dependent PA-VSD.

Studies		Study period	N	Follow-up time (months)	Survival	Freedom of RVOTO reintervention
Current study	SR	2014-2023	144 (74-70)	44.8	76%	91%
Current study	p-BVR	2014-2023	144 (74-70)	41.5	89%	77%
Kim et al⁵	SR	2004-2017	66 (30-36)	60.8	73.3%	40%
Kim et al⁵	p-BVR	2004-2017	66 (30-36)	90.1	94.4%	25%
Alsoufi et al³	SR	2002-2012	86 (58-28)	61.2	72%	70.2%
Alsoufi et al³	p-BVR	2002-2012	86 (58-28)	69.6	85.5%	52.4%
Lee et al⁴	SR	2004-2017	65 (50-15)	59.3	83.6%	61%
Lee et al⁴	p-BVR	2004-2017	65 (50-15)	33.8	86.7%	39%
Kwak²	SR	1997-2008	47 (29-18)	76.1	78%	83%
Kwak²	p-BVR	1997-2008	47 (29-18)	43.8	89%	42%

Abbreviations: PA-VSD, pulmonary atresia and ventricular septal defect; p-BVR, primary biventricular repair; RVOTO, right ventricular outflow tract obstruction; SR, staged repair.

Risk Factors for Death and Survival Between Groups

As seen in previous studies,^2,5 we have seen the superiority of p-BVR over SR in patients with ductal-dependent PA-VSD. Obviously, the interim deaths after shunt palliation contributed substantially to the overall mortality from SRs, especially in low- to middle-income countries with limited resources to follow-up patients with a vulnerable balance of pulmonary circulation. Consequently, p-BVR for ductal-dependent PA-VSDs has become a vital option for midterm outcomes because it can achieve immediate restoration of normal circulation, resulting in comparable overall survival to that reported in previous studies.^2–4 Additionally, it does not impose a heavy burden on early RVOT catheterization or reoperation in our healthcare system owing to resource constraints. However, our patient characteristics might reveal the potential for natural selection with the most severe group of genetic/extracardiac anomalies, which is not a multivariate predictor of death but rather of the complexity of the Rastelli procedure with a longer ischemic time. Nonetheless, we do not present any patient exclusion criteria in our center except for a limited number of patients whose families refuse medical treatment for their children with multiple extracardiac anomalies.

Limitations

This study is limited by its retrospective nature, nonrandomized design, and selection bias. The primary limitation of our study is the lack of a control group of PA-VSD patients who underwent the Rastelli operation without concomitant PA angioplasty.

Although echocardiography and multislice CT results were carefully examined by one cardiologist, accurate measurements could be obtained via cardiac catheterization. The statistical analyses were limited to a small number of patients and outcomes.

Conclusions

Concomitant pulmonary arterioplasty may be associated with increased freedom from RVOT reintervention and delayed RVOT reintervention, provide good midterm outcomes, and should be considered in PA-VSD patients who undergo the Rastelli operation. Primary BVR combined with pulmonary arterioplasty for PA-VSD patients is correlated with better survival than SR (with high interim-stage deaths) and it does not increase the burden of RVOT catheterization or reoperation.

Supplemental Material

sj-docx-1-pch-10.1177_21501351251363158 - Supplemental material for Outcome of Infants With Ductal-Dependent Pulmonary Atresia and Ventricular Septal Defect in a Developing Nation: A Single-Center Experience

Supplemental material, sj-docx-1-pch-10.1177_21501351251363158 for Outcome of Infants With Ductal-Dependent Pulmonary Atresia and Ventricular Septal Defect in a Developing Nation: A Single-Center Experience by Vinh Quang Tran, Anh Vuong Doan, Van-Anh Thi Nguyen, Thai Quoc Ngo, Mai Tuan Nguyen, Doan Vu Tu Quyen, Duyen Dinh Mai, Nhat Huu Nguyen, Doan Quoc Hung, James St. Louis and Truong Ly Thinh Nguyen in World Journal for Pediatric and Congenital Heart Surgery

Footnotes

Abbreviations

Acknowledgments

The authors would like to thank Dr Chang-Ha Lee for his instructions and valuable advice on how to perform p-BRV for ductal-dependent PA-VSD in our hospital.

Author Contributions

Truong Nguyen Ly Thinh contributed conceptualization, project administration, investigation, visualization, writing—original draft, and writing—review and editing. Vinh Quang Tran contributed data curation, validation, and writing—review and editing. Anh Vuong Doan contributed data curation, formal analysis, validation, and writing—review and editing. Van—Anh Thi Nguyen contributed data curation, formal analysis, validation, and writing—review and editing. Thai Quoc Ngo contributed investigation, visualization, and writing—review and editing. Mai Tuan Nguyen contributed investigation, visualization, and writing—review and editing. Doan Vu Tu Quyen contributed investigation, visualization, and writing—review and editing. Duyen Dinh Mai contributed investigation, visualization, and writing—review and editing. Nhat Huu Nguyen contributed investigation, visualization, writing—review and editing. Doan Quoc Hung contributed investigation, visualization, and writing—review and editing. James St. Louis contributed conceptualization, project administration, investigation, visualization, writing—original draft, and writing—review and editing.

Data Availability Statement

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.

Declaration of Conflicting Interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

ORCID iD

Truong Ly Thinh Nguyen

Supplemental Material

Supplemental material for this article is available online.

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