- Academic Editor
†These authors contributed equally.
Background: Both systemic-to-pulmonary shunt and right
ventricle-pulmonary artery (RV-PA) connection are extensively applied to
initially rehabilitate the pulmonary artery in pulmonary atresia with the
ventricle septal defect (PA/VSD). However, which of these options is the most
ideal for promoting pulmonary artery development and improving outcomes remains
controversial. Methods: A total of 109 PA/VSD patients undergoing
initial rehabilitative surgery at Guangdong Provincial People’s Hospital from
2010 to 2020 were enrolled in this study. A series of clinical data were
collected to compare the perioperative and postoperative outcomes between
systemic-to-pulmonary and RV-PA connection. Results: The mean duration
of follow-up was 61.1 months in the systemic-to-pulmonary shunt group and 70.3
months in the RV-PA connection group (p
Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex and unusual form of congenital heart lesion that is estimated to occur in 7 of 100,000 live births [1]. It is characterized by a pulmonary artery size ranging from absent/diminutive to reasonable, a tetralogy-type ventricular septal defect (VSD), and heterogeneous pulmonary blood supply generated from isolation or combination of major aortopulmonary collateral arteries (MAPCAs) and native pulmonary arteries. Prognosis of this condition is poor, with approximately one-fifth of patients only surviving to the age of 30 years [2]. The surgical management of this lesion has evolved for decades but remains challenging including two opposed strategies: MAPCAs unifocalization and pulmonary vessel rehabilitation.
The rehabilitation strategy aims to promote the development of native pulmonary arteries for definitive completer repair. It can be categorized into several subgroups according to the initial operative technique, such as the establishment of systemic-to-pulmonary shunt and right ventricle-pulmonary artery (RV-PA) connection. Both are beneficial for native pulmonary growth and eventual complete repair and are therefore extensively employed in many institutions [3, 4, 5, 6]. However, the ideal rehabilitative surgical option for treating patients with PA/VSD is highly debated.
In this paper, we sought to summarize our 11-year experience and describe our single-center outcomes of the RV-PA connection and systemic-to-pulmonary shunt in PA/VSD patients [7, 8].
The present study was approved by the Guangdong Provincial People’s Hospital
ethics committee following the ethical guidelines of the Declaration of Helsinki
(1975). From January 2010 to December 2020, a total of 109 PA/VSD patients who
underwent the initial rehabilitative surgery at Guangdong Provincial People’s
Hospital were enrolled in this study. Those PA/VSD patients who received a
unifocalization strategy rather than a rehabilitative strategy were excluded.
Those combined with other complex congenital lesions such as transposition of the
great arteries and dextrocardia were also excluded. Fifty-three patients
underwent RV-PA connection, while fifty-six underwent systemic-to-pulmonary
shunt. The choice of rehabilitative strategies was mainly
determined by experienced surgeons in our center. Nonetheless, the following
preferences were taken into consideration. Systemic-to-pulmonary shunting was
performed in patients with poor native pulmonary artery growth within the
pericardium
The included patients adopted rehabilitative strategies in terms of a right ventricle-to-pulmonary artery connection or a systemic-to-pulmonary artery shunt, which have been explicitly described before [10]. Briefly, in the context of the right ventricle to pulmonary artery connection, a median sternotomy was performed, followed by the establishment of cardiopulmonary bypass. The autologous pericardial graft, bovine pericardial grafts, bovine jugular vein, or Gore-Tex conduit would be used to widen or reconstruct the right ventricle to pulmonary artery connection. In patients adopting a systemic-to-pulmonary artery shunt, a central shunt by anastomosis of the aorta and the main pulmonary artery or a modified Blalock-Taussing shunt by anastomosis of the innominate artery and the ipsilateral branch pulmonary artery would be performed in the presence or absence of the cardiopulmonary bypass.
Angiography and more often CT would be performed preoperatively to determine the MAPCAs’ origin, size, supply, and distribution. The MAPCAs that were accompanied by serious stenosis or were the only source of pulmonary blood would be untreated. In contrast, those that were the sole pulmonary blood flow would be ligated during the rehabilitative surgery simultaneously or percutaneous occluded preoperatively. If heart failure or severe pulmonary hypertension occurs during the postoperative follow-up, percutaneous occlusion might also be considered.
If there is a satisfactory growth of the pulmonary artery with the McGoon ratio
In-hospital morbidity was defined as the isolation or combination of the
following postoperative complications at the hospital: extracorporeal membrane
oxygenation support, delayed sternal closure, diaphragm plication, pneumonia, or
cerebrovascular issues. In-hospital mortality referred to postoperative death
before discharge. Late mortality was defined as post-discharge death either
before or after the next stage of surgery.
All patients were requested for outpatient visits at 3, 6, and 12 months after the initial rehabilitative surgery and annually thereafter. The endpoints were follow-up death or complete repair. Therefore, we primarily compared the long-term outcomes in terms of survival and complete repair between two rehabilitative strategies in the overall PA/VSD cohort. Given more heterogenicity in PA/VSD with MAPCAs (PA/VSD/MAPCAs) patients, we also compared the outcomes targeting this sub-cohort.
Continuous variables with normal distribution and abnormal distribution were shown as means with standard deviations and medians with ranges, respectively. Categorical variables were shown as frequencies with percentages. Student’s test or Mann-Whitney test was used to compare the continuous variables between two groups as appropriate. Chi-squared or Fisher’s exact test was adopted in categorical variables. The probability of survival by time was estimated by the Kaplan-Meier curve and compared between the two groups using the Log-rank test. The cumulative complete repair by time was estimated by the cumulative incidence curve and compared between the two groups using Gray’s test. The subdistribution hazards model was used to identify factors associated with complete repair (death as a competing event). Those factors with a p value less than 0.1 in the univariable competing risk regression analysis and those clinically significant factors were included in the multivariable analysis. Given few positive events, cox regression multivariable analysis for mortality was not performed. A two-sided p value less than 0.05 was considered significant. All statistical analysis were performed using IBM SPSS Statistics for Windows, version 26.0 (IBMCorp., Armonk, Westchester County, NY, USA) and R (R Core Team, 2022).
Overall, there were approximately 10–15 rehabilitative surgeries every year but
less than 5 in 2019 and 2020. In this study, there were 17 patients who underwent
complete repair surgery, with 5 deaths and 8 lost to follow-up among the
systemic-to-pulmonary shunt group. In contrast, 29 patients were repaired
completely with 6 deaths and 6 lost to follow-up among 53 patients receiving the
initial RV-PA connection (Fig. 1). The baseline characteristics of the overall
PA/VSD cohort and PA/VSD/MAPCAs sub-cohort are summarized in Table 1. In the
overall PA/VSD cohort, the McGoon ratio in the RV-PA connection was significantly
larger than that in the systemic-to-pulmonary shunt (p = 0.003). The
other preoperative data were distributed evenly between the two groups. In the
PA/VSD/MAPCAs sub-cohort, there was also a significantly larger McGoon ratio and
lower levels of hemoglobin and hematocrit (Hct) in the RV-PA connection group
(p
Flowchart of rehabilitative technique options and outcomes in the overall PA/VSD patients cohort in our center. PA/VSD, pulmonary atresia with the ventricular septal defect; RV-PA connection, right ventricle-pulmonary artery connection.
Overall PA/VSD cohort | PA/VSD/MAPCAs sub-cohort | |||||
Systemic-to-pulmonary shunt | RV-PA connection | p-value | Systemic-to-pulmonary shunt | RV-PA connection | p-value | |
n = 56 | n = 53 | n = 25 | n = 22 | |||
Gender, male | 30 (53.6%) | 32 (60.4%) | 0.473 | 10 (40.0%) | 8 (36.4%) | 0.798 |
Age (month) | 12.0 (1.6–85.7) | 12.8 (4.5–48.2) | 0.739 | 66.0 (10.8–222.9) | 32.4 (16.1–68.1) | 0.316 |
Height (cm) | 71.0 (51.0–112.0) | 72.0 (59.5–95.0) | 0.783 | 115.0 (70.5–151.0) | 86.0 (73.5–101.0) | 0.166 |
Weight (kg) | 8.0 (3.6–16.0) | 8.0 (5.3–12.4) | 0.966 | 18.0 (8.0–44.0) | 11.0 (8.0–13.8) | 0.086 |
BSA (m |
0.39 (0.20–0.74) | 0.40 (0.27–0.59) | 0.804 | 0.78 (0.39–1.35) | 0.49 (0.41–0.65) | 0.138 |
PaO |
73 (66–80) | 73 (66–80) | 0.930 | 73 (71–81) | 77 (72–84) | 0.375 |
RBC | 6.1 |
6.2 |
0.800 | 6.4 |
6.0 |
0.209 |
Hemoglobin (g/L) | 168.0 |
159.4 |
0.130 | 177.6 |
157.2 |
0.025 |
Hct (%) | 0.52 |
0.49 |
0.122 | 0.54 |
0.47 |
0.022 |
ALT (μ/L) | 18.0 (13.0–27.6) | 17.5 (14.0–22.8) | 0.572 | 18.0 (12.5–27.0) | 15.0 (13.8–21.0) | 0.387 |
TBil (μmol/L) | 14.9 (10.4–23.1) | 14.6 (9.0–21.0) | 0.238 | 16.3 (10.1–23.4) | 12.2 (9.1–20.6) | 0.301 |
DBil (μmol/L) | 4.5 (3.0–6.3) | 4.6 (3.2–6.4) | 0.734 | 3.9 (2.8–5.4) | 4.1 (3.2–5.9) | 0.670 |
Scr (μmol/L) | 32.0 (23.0–43.0) | 30.9 (22.0–36.5) | 0.337 | 37.0 (23.9–55.0) | 28.2 (22.7–34.3) | 0.070 |
PT (sec) | 14.1 |
13.8 |
0.119 | 14.3 |
13.7 |
0.143 |
Presence of MAPCAs | 25 (44.6%) | 22 (41.5%) | 0.741 | 25 (100%) | 22 (100%) | / |
Patent ductus arterious | 21 (38.5%) | 22 (41.5%) | 0.669 | 6 (24.0%) | 7 (31.8%) | 0.550 |
McGoon ratio | 0.79 |
0.99 |
0.003 | 0.66 |
0.91 |
0.020 |
Nakata index (mm |
74.1 |
61.3 |
0.114 | 57.7 |
54.5 |
0.789 |
PA/VSD, pulmonary atresia with ventricular septal defect; PA/VSD/MAPCAs, pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries; RV-PA connection, right ventricle-pulmonary connection; BSA, body surface area; MAPCAs, major aortopulmonary collateral arteries; RBC, red blood cells; Hct, hematocrit; ALT, alanine aminotransferase; TBil, total bilirubin; DBil, direct bilirubin; Scr, serum creatine; PT, prothrombin time.
As shown in Table 2, the cardiopulmonary bypass (CPB) time and aortic
cross-clamp time when adopting systemic-to-pulmonary shunt were all 0 min, which
was significantly shorter than when adopting the RV-PA connection (p
Overall PA/VSD cohort | PA/VSD/MAPCAs sub-cohort | |||||
Systemic-to-pulmonary shunt | RV-PA connection | p-value | Systemic-to-pulmonary shunt | RV-PA connection | p-value | |
n = 56 | n = 53 | n = 25 | n = 22 | |||
CPB time (min) | 0 (0–0) | 107 (86.0–138.5) | 0.00 (0.00–0.00) | 103.5 (86.5–145.8) | ||
Aortic cross-clamp time (min) | 0 (0–0) | 62 (44.0–84.0) | 0 (0–0) | 61.0 (44.5–76.3) | ||
Shunt/conduit size (mm) | 5.0 (4.0–6.0) | 10.0 (10.0–12.0) | 6.0 (5.0–7.5) | 10.0 (9.5–10.0) | ||
Postoperative PaO |
85 (80–90) | 90 (85–95) | 85 (82–89) | 90 (85–95) | 0.005 | |
Mechanical ventilation time (h) | 57.7 (26.1–118.1) | 55.8 (27.8–140.2) | 0.132 | 53.9 (23.8–92.1) | 51.2 (25.9–108.6) | 0.848 |
In-hospital morbidity | 10 (17.9%) | 7 (13.2%) | 0.504 | 5 (20.0%) | 2 (9.1%) | 0.524 |
In-hospital mortality | 0 | 0 | / | 0 | 0 | / |
Hospital stay (d) | 28.0 (21.3–40.5) | 33.0 (24.0–47.5) | 0.482 | 28.0 (21.0–39.5) | 33.5 (23.8–41.8) | 0.153 |
PA/VSD, pulmonary atresia with ventricular septal defect; PA/VSD/MAPCAs, pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries; RV-PA connection, right ventricle-pulmonary connection; CPB, cardiopulmonary bypass.
In the overall cohort, the mean duration of follow-up in the
systemic-to-pulmonary shunt and RV-PA connection group was 61.1
Overall PA/VSD cohort | PA/VSD/MAPCAs sub-cohort | ||||||
Systemic-to-pulmonary shunt | RV-PA connection | p-value | Systemic-to-pulmonary shunt | RV-PA connection | p-value | ||
n = 56 | n = 53 | n = 25 | n = 22 | ||||
Duration of follow-up (month) | 61.1 |
70.3 |
0.103 | 62.7 |
70.6 |
0.349 | |
RBC | 5.9 (5.3–6.8) | 5.3 (4.8–5.9) | 5.7 (5.3–6.6) | 5.3 (4.8–6.1) | 0.021 | ||
Hemoglobin (g/L) | 156.0 (145.0–179.5) | 140.0 (130.0–154.6) | 161.0 (150.5–177.5) | 139.0 (128.2–157.0) | |||
Hct (%) | 0.48 (0.44–0.55) | 0.42 (0.39–0.47) | 0.52 (0.46–0.56) | 0.43 (0.37–0.47) | |||
ΔMcGoon ratio | 0.41 (0.24–0.64) | 0.69 (0.43–1.04) | 0.33 (0.24–0.56) | 0.57 (0.35–0.94) | 0.017 | ||
ΔNakata index (mm |
57.8 (26.8–92.7) | 131.3 (94.5–232.4) | 49.7 (27.9–85.0) | 111.4 (56.1–144.8) | 0.006 | ||
Inter-stage intervention | |||||||
Pulmonary angioplasty | 2 (3.6%) | 2 (3.8%) | 0 | 0 | / | ||
Pulmonary stenting | 1 (1.8%) | 1 (1.9%) | 0 | 0 | / | ||
MAPCAs coil occlusion | 0 | 1 (1.9%) | 0.978 | 0 | 1 (4.5%) | 0.948 | |
Shunt/conduit replacement | 20 (35.7%) | 2 (3.8%) | 9 (36.0%) | 1 (4.5%) | 0.023 | ||
Late mortality | 5 (8.9%) | 6 (11.3%) | 0.679 | 1 (4.0%) | 3 (13.6%) | 0.511 | |
Complete repair | 17 (30.4%) | 29 (54.7%) | 0.010 | 2 (8.0%) | 11 (50.0%) | 0.001 |
PA/VSD, pulmonary atresia with ventricular septal defect; PA/VSD/MAPCAs, pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries; RV-PA connection, right ventricle-pulmonary connection; MAPCAs, major aortopulmonary collateral arteries; RBC, red blood cells; Hct, hematocrit.
The late mortality in the two groups was similar with no significant difference
in either the overall PA/VSD and PA/VSD/MAPCAs sub-cohort. In the overall cohort,
there were 5 deaths in the systemic-to-pulmonary shunt group and 6 deaths in the
RV-PA connection group. In the systemic-to-pulmonary shunt group, one patient
died of multiple organ dysfunction syndrome after 35 months and one patient died
of cardiogenic shock after 19 months. Two patients died of pneumonia after 65 and
72 months. In the RV-PA connection group, one death occurred due to myocarditis
after 32 months. The cause of other sudden deaths in the two groups was unknown.
Compared with those undergoing systemic-to-pulmonary shunt, patients with the
RV-PA connection showed a significantly higher incidence of complete repair in
either the overall PA/VSD and PA/VSD/MAPCAs sub-cohort (p
Long-term outcomes between the two rehabilitative surgeries in
the overall PA/VSD cohort (A,B) and the PA/VSD/MAPCAs sub-cohort (C,D). (A) No
significant difference regarding the probability of survival by the time between
the two rehabilitative surgeries in the overall PA/VSD cohort (p
Long-term outcomes between PA/VSD patients without MAPCAs and
PA/VSD/MAPCAs patients. (A) No significant difference regarding the probability
of survival by the time between PA/VSD patients without MAPCAs and PA/VSD/MAPCAs
patients (p
In a univariable competing risk regression analysis, age, presence of MAPCAs,
Patent ductus arteriouos, and RV-PA connection were identified as predictors for
complete repair. These factors combined with clinically significant factors
(PaO
Univariable analysis | Multivariable analysis | |||
HR (95% CI) | p-value | HR (95% CI) | p-value | |
Age | 0.989 (0.981–0.998) | 0.012 | 0.990 (0.980–0.999) | 0.039 |
PaO |
0.981 (0.959–1.004) | 0.108 | 0.994 (0.970–1.018) | 0.627 |
Presence of MAPCAs | 0.425 (0.224–0.808) | 0.009 | 1.269 (0.611–2.638) | 0.523 |
Patent ductus arterious | 2.035 (1.139–3.637) | 0.016 | 0.599 (0.327–1.096) | 0.096 |
McGoon ratio | 1.569 (0.721–3.414) | 0.257 | 0.331 (0.084–1.295) | 0.112 |
Nakata index | 1.003 (0.997–1.010) | 0.309 | 1.009 (0.997–1.019) | 0.154 |
RV-PA connection | 1.914 (1.050–3.487) | 0.034 | 2.348 (1.131–4.873) | 0.022 |
PA/VSD, pulmonary atresia with ventricular septal defect; RV-PA connection, right ventricle-pulmonary connection; MAPCAs, major aortopulmonary collateral arteries; HR, hazard ratio; 95% CI, 95% confidence interval.
This study summarized the 11-year experience of PA/VSD patients’ outcomes
between two different initial rehabilitative strategies at our center over the
past decade. The main findings are shown as follows: (1) Compared with the RV-PA
connection, the systemic-to-pulmonary shunt would result in a lower PaO
The PaO
RV-PA connection resulted in a significantly higher cumulative completer repair rate than the systemic-to-pulmonary shunt despite the similar impact on promoting pulmonary vasculature development. The multivariable analysis in this study showed that RV-PA connection was an independent predictor for complete repair (HR = 2.348, 95% CI = 1.131–4.873). The previous investigation on the postoperative outcome of PA/VSD patients undergoing two different rehabilitative surgeries remains controversial. Fan et al. [7] reviewed and compared 44 patients undergoing systemic-to-shunt and 54 patients undergoing RV-PA connection from 2011 to 2016 at their center, showing that the pulmonary vessel growth, as well as complete repair rate, were similar between these two groups. In their study, the mean age of systemic-to-pulmonary shunt and RV-PA connection was 25.0 months and 27.6 months, respectively, much older than our patients. They underwent the initial surgery late, in part probably due to their relatively stable condition. In this context, the impact of both these two rehabilitative strategies on pulmonary artery growth and complete repair might not be distinct. In contrast, Zhao also compared the outcome of these two rehabilitative strategies, enrolling a total of 157 PA/VSD/MAPCAs patients from 2009 to 2017. They found that the RV-PA connection was more beneficial for improving the cumulative complete repair rate, which is consistent with our findings [8]. Multiple reasons could explain the better potential of the RV-PA connection in promoting pulmonary vasculature development and final complete repair in our study. First of all, the RV-PA connection directs the aforementioned sufficient blood flow into the lungs in an antegrade and pulsatile manner, beneficial for pulmonary vasculature development. Indeed, more frequent replacement of shunt/conduit that usually aimed to provide more adequate blood flow occurred in the systemic-to-pulmonary shunt group than in the RV-PA connection group. It also allows pulmonary artery intervention like balloon angioplasty via antegrade access. Secondly, the systemic-to-pulmonary shunt was associated with pulmonary artery distortion, overcirculation, and thrombosis [12]. Shunt thrombosis and obstruction occurred in several systemic-to-pulmonary shunt patients in our study, which might aggregate the oxygenation desaturation and hinder pulmonary artery growth. Thirdly, the mean McGoon ratio in the RV-PA connection group was higher, indicating an initially better pulmonary vessel size, which probably in part contributed to our findings. The higher McGoon index and pulmonary artery confluence have been demonstrated to be capable of improving the probability of complete repair [13].
The previous major studies on PA/VSD patients who initially underwent rehabilitative surgery were summarized in Table 5 (Ref. [3, 6, 7, 8, 13, 14, 15, 16, 17, 18, 19, 20, 21]). The complete repair rate in either the overall PA/VSD cohort or PA/VSD sub-cohort was slightly lower than the previous investigation [6, 14, 22], which is somewhat attributed to the following reasons. First, patients (median age: 12.8 months) in this study were older than those in most of the previous studies, as evidenced by Table 5. Some patients were referred to hospitals late in our country mainly owing to their family’s poor financial situation, limited pediatric specialists, insufficient bed availability, and mild cyanosis. It has been suggested that the initial rehabilitative surgery performed in PA/VSD patients with age more than approximately half a year old appeared to reduce the probability of complete repair [23]. Secondly, regular outpatient clinic visits after the initial rehabilitative surgery are imperative for the assessment of pulmonary vasculature growth, timely inter-stage intervention, and eventual complete repair. However, proportional patients did not strictly follow the schedule because of misconceptions about PA/VSD disease, the long distance from home to the hospital, and the family’s poor financial situation, which may have compromised the likelihood of complete repair. Thirdly, the mean period of follow-up was 70.3 months in the RV-PA connection group and 61.1 months in the systemic-to-pulmonary shunt group, which is relatively short compared to some previous studies [15, 16]. Of note, the complete repair rate in the PA/VSD/MAPCAs patients was particularly lower than that in the previous studies, compared with PA/VSD patients without MAPCAs. We assumed that PA/VSD/MAPCAs had a worse dysplasia of pulmonary artery than PA/VSD without MAPCAs so that proportional PA/VSD/MAPCAs were unable to reach satisfactory pulmonary vasculature and achieve complete repair in the context of mere rehabilitative strategy. Hence, the adoption of a unifocalization strategy or combined strategy for these patients is also practical and important [11]. The management of PA/VSD/MAPCAs remains highly challenging because of great heterogeneity. More future studies focusing on individual choice of treatment strategies (rehabilitation, unifocalization, or combination) for PA/VSD/MAPCAs to improve long-term outcomes are warranted.
Author | Years of study | Patient number | Age on surgery | Rehabilitation technique | Follow-up period | Survival | Complete repair |
1. Macalister et al. [15] | 1989–2019 | 107 | 7 d (4–26) | SPS | 10.5 y (3.6–18.8) | Estimated 10-year survival rate: 81% | 85% |
2. Zhao et al. [8] | 2009–2017 | 56 | 13.9 m (1.8–211.5) | Central shunt | 18 m (0–66) | 92.9% | Estimated 5-year complete repair rate: 56.0 |
56 | 10.4 m (2.6–216.9) | RV-PA connection | 22 m (0–62) | 91.1% | Estimated 5-year complete repair rate: 74.5 | ||
3. Fan et al. [7] | 2011–2016 | 44 | 25.0 |
SPS | 11.4 |
97.7% | 20.5% |
54 | 27.6 |
RV-PA connection | 15.5 |
88.9% | 37% | ||
4. Zou et al. [17] | 2010–2019 | 29 | 8 m (0.5–144) | SPS | 24 m (6–116) | Estimated 10-year survival rate: 76.1% | 54.2% |
68 | 14 m (2.2–209.6) | RV-PA connection | 47 m (22–222) | 50% | |||
5. Chen et al. [6] | 2009–2014 | 69 | 1.8 |
RV-PA connection | 2.8 |
5-year survival rate: 93.8 |
Estimated 3-year complete repair rate: 60.1 |
6. Alsoufi et al. [18] | 2002–2012 | 58 | 6 d (3–11) | B-T shunt | 5.1 |
Estimated 8-year survival rate: 72.2% | 79.3% |
7. Choi et al. [19] | 2011–2015 | 13 | 1.8 |
RV-PA connection | 26.0 |
84.6% | 76.9% |
8. Bradley et al. [14] | 2004–2007 | 10 | 9 d (4–86) | RV-PA connection | 1.9 |
90% | 80% |
9. Hofbeck et al. [13] | 1976–1988 | 104 | 218.3 d | Rehabilitation (not detailed) | 4.95 y (2 d–13.75 y) | Estimated 10-year survival rate: 69% | 36.5% |
10. Soquet et al. [20] | 2003–2014 | 33 | 3.3 w (0.4–31.9) | Central shunt (most frequent) | 4.5 y | 90.9% | 73% |
11. Lee et al. [21] | 2004–2017 | 50 | 22 d (16.0–36) | Modified B-T shunt (most frequent) | 59.3 m (22–115) | Estimated 5-year survival rate: 83.6%) | 86% |
12. Kim et al. [3] | 1993–2013 | 15 | 1.91 m (0.2–26.36) | Central shunt | 70.7 |
Estimated 5-year survival rate: 82.5% | 92.9% |
13. Kaskinen et al. [16] | 1970–2007 | 109 | 0.96 m | SPS (most frequent) | 11.4 y (0.01–41.77) | Estimated 5-year survival rate: 66% | 50.0% |
PA/VSD, pulmonary atresia with ventricular septal defect; SPS, systemic-to-pulmonary shunt; RV-PA connection, right ventricle-pulmonary artery connection; B-T shunt, Blalock-Taussig shunt; d, day; w, week; m, month; y, year.
There are several limitations in this study. First, the retrospective nature and single-center design may limit its generalization to other centers. The initial pulmonary vasculature between groups was different, which at least, in part, influenced the final comparison of pulmonary growth and complete repair rate. Therefore, these findings should be assessed with caution.
The rehabilitative strategies in terms of RV-PA connection and systemic-to-pulmonary shunt resulted in a similar survival rate in PA/VSD patients. The RV-PA connection is more advantageous as an initial rehabilitative technique than the systemic-to-pulmonary shunt to improve the eventual complete repair rate.
PA/VSD, pulmonary atresia with ventricular septal defect; PA/VSD/MAPCAs, pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries; RV-PA connection, right ventricle-pulmonary artery connection; VSD, ventricular septal defect; MAPCAs, major aortopulmonary collateral arteries; CT, computed tomography; B-T shunt, Blalock-Taussig shunt.
Data are available from the corresponding author upon reasonable request.
JZC and HYY designed the research study. SZ and JRM performed the research. SSW, JMC, and JZ provided help and advice on research. TT, WX, HZL, HMW, and HLQ were responsible for data collection. SZ, JRM, and XL analyzed the data. JRM wrote the manuscript. All authors contributed to editorial changes in the manuscript. All authors read and approved the final manuscript. All authors have participated sufficiently in the work and agreed to be accountable for all aspects of the work.
This study was approved by the Ethics Committee Board of Guangdong Provincial People’s Hospital on 17th September 2019 (No.GDREC2019338H(R2)). The need for individual written informed consent was waived.
We thank each member of Cen’s group for their helpful comments.
This work was supported by the National Key Research and Development Program of China (No. 2022YFB4600604 and No. 2022YFC2407406), Science and Technology Foundation of Guangzhou Health (No. 2023A031004), Guangzhou Science and Technology Planning Project (2023B03J0596, 2023B03J1254), Guangdong peak project (No. DFJH2020029), National Natural Science Foundation of China (No. 62006050, No. 62276071), National Natural Science Foundation of Guangdong Province (No. 2023A1515012501), Guangdong Special Support Program-Science and Technology Innovation Talent Project (No. 0620220211).
The authors declare no conflict of interest.
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