Reconstruction of the pulmonary posterior wall using in situ autologous tissue for the treatment of pulmonary atresia with ventricular septal defect
© The Author(s). 2017
Received: 9 August 2016
Accepted: 21 February 2017
Published: 23 February 2017
To evaluate the early and mid-term results of pulmonary trunk reconstruction using a technique in which autogenous tissue is preserved in situ in pulmonary atresia patients with a ventricular septal defect (PA-VSD).
The pulmonary artery was reconstructed using autogenous tissue that had been preserved in situ and a bovine jugular venous patch in 24 patients who were diagnosed with PA-VSD (the observation group). The traditional operation using a bovine jugular venous conduit was performed in 40 other cases of PA-VSD (the control group).
In the observation group, all patients survived and recovered successfully without complications. Follow-up echocardiography 2–10 years after the procedure showed that the reconstructed right ventricular outflow tract (RVOT) and pulmonary artery were patent, showing no evidence of flow obstruction. Only mild regurgitation of the bovine jugular vein valve was observed. In the control group, early postoperative death occurred in two cases. Another two patients had obstruction of the anastomotic stoma and underwent conduit replacement surgery within 2 weeks of the initial procedure. During the 2–10 years of follow-up care, six patients presented with valvular stenosis of the BJVC, with a pressure gradient of more than 50 mmHg.
The technique for preserving autogenous tissue to reconstruct the pulmonary posterior wall is a satisfactory method for treating PA-VSD.
KeywordsCongenital heart disease Pulmonary atresia Pulmonary reconstruction In situ autogenous tissue
Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare and complex cyanotic congenital heart malformation that has a high incidence of early mortality. Although surgical interventions have been employed successfully for a number of years, the long-term prognosis has remained poor with an increased incidence of sudden death . In 1955, Lillehei et al. successfully corrected an atresia of the main pulmonary artery (MPA) and a ventricular defect in a patient who had been diagnosed with PA-VSD by anastomosing the distal pulmonary artery with the right ventricle . A decade later, Rastelli and colleagues reported a case in which a cardiac conduit from the right ventricle to the pulmonary trunk was used to treat the abnormality . Various extracardiac valved conduits have subsequently been used to correct a right ventricle to pulmonary artery discontinuity since Ross and Somerville successfully employed a homograft aortic valved conduit in 1966 . Weldon et al. reported the same result 2 years later . In 1973, PA-VSD was successfully employed by Bowman and colleagues, who used a Dacron conduit containing a porcine heterograft aortic valve . Despite these improvisations to the Rastelli procedure, most patients with PA-VSD have had to undergo repeated surgeries resulting from first conduit failure because of either calcification or degeneration of the extracardiac graft or a mismatch of biologic prosthesis. Thus, treating a ventricle-pulmonary artery discontinuity remains a major challenge; surgeons have tried to develop an ideal prosthesis while concomitantly developing techniques to reconstruct the right ventricular outflow tract (RVOT) [7, 8]. Herein, we describe a strategy that was performed on 24 patients who were diagnosed with PA-VSD in which autogenous tissue was preserved in situ as the posterior part of a newly reconstructed pulmonary artery. Forty PA-VSD patients underwent control surgeries using the Rastelli procedure of connecting a bovine jugular venous conduit (BJVC) between the right ventricle and the bifurcation of the MPA.
Preoperative data from the two groups
Age (month median)
Body weight (kg median)
162 ± 19
165 ± 13
Oxygen blood pressure (mmHg)
71 ± 9
69 ± 7
Pulmonary artery index (mm2/m2)
175 ± 15
179 ± 11
Mcgoon index (median)
PA-VSD Type A (case)
PA-VSD Type B (case)
Data are expressed as the mean ± SE and median. All statistical calculations were performed using the Statistical Product and Service Solutions 14.0 software (SPSS Institute). X 2 test, Log-rank test and Gehan-Breslow-Wilcoxon test were performed to determine differences between the two groups. The critical alpha level for these analyses was set at p < 0.05.
Postoperative data from the two groups
Cardiopulmonary bypass time (min)
102 ± 18
99 ± 22
Aortic cross clamp time (min)
85 ± 10
82 ± 13
Mechanical ventilation time (h)
68 ± 23
73 ± 18
ICU residence time (d)
5.5 ± 2.2
6.2 ± 2.4
Postoperative residence time (d)
20 ± 3
26 ± 2
PA-VSD accounts for approximately 2% of all congenital heart disease cases and is one of the most common causes of cyanosis and hypoxemia in neonates. The pathological anatomic characteristics of PA-VSD include VSD, intramuscular or valvar atresia of the pulmonary artery, an overriding of the aorta, and PDA or MAPCAs. According to the classification presented by Castaneda et al., there are four types of PA-VSD . In this study, 48 of the patients were type A, and 16 patients were type B (Table 1). Selection of the surgical procedure for treatment of PA-VSD depends mainly on development of the right ventricle and the pulmonary artery . There are two evaluation indices for pulmonary artery development that can be considered: the McGoon index and the pulmonary artery index (PAI) . A patient is considered suitable for radical repair if the McGoon index is greater than 1.2 or the PAI is greater than 150 mm2/m2, suggesting no severe hypoplasia of the pulmonary artery, which indicates that a patient would be a suitable candidate for radical repair.
Currently, the most common method of radical repair for the treatment of PA-VSD is the classical Rastelli procedure, in which a right ventriculotomy is repaired with an artificial patch. Following the ligation of the co-existing PDA or MAPCAs, the remnant pulmonary trunk is excised, the proximal end is closed, and a vascular prosthesis is then used to connect the right ventricle and the pulmonary artery.
Despite the short-term success of employing different conduit modifications of the Rastelli procedure [7, 8, 12–14], many common complications remain. These include conduit dilation, twisting, thrombosis, calcification, endocarditis, arrhythmia and re-stenosis [15–17]. Shebani et al.  found that the early mortality rate resulting from conduit dilation, complete conduit thrombosis, and sudden arrhythmia was 6.4%, as determined from a retrospective analysis of 62 cases of RVOT and pulmonary trunk reconstruction using a bovine Contegra valved conduit. Distal conduit stenosis at the suture line associated with a size-mismatch with the conduit being too small was the main indicator for conduit-related secondary intervention. The high incidence of conduit-related complications, particularly with smaller conduits, included characteristics such as neointimal proliferation, thrombosis, calcification, and chronic inflammation. Reoperation is therefore inevitable for some patients with pulmonary atresia (PA) who receive a heterograft or homograft in a primary Rastelli operation. To treat PA-VSD, Chiu and colleagues  adopted a method used in the repair of Fallot’s tetralogy, a congenital heart defect that is understood to involve four anatomical abnormalities, in which a piece of fresh pericardium is harvested and sutured to cover the anterior part of the RVOT. However, pulmonary regurgitation and tricuspid regurgitation are common complications of this procedure. Schreiber et al.  replaced the traditional conduit with the Shelhigh No-React (NR-4000PA series)-treated porcine pulmonic valve conduit (SPVC). However, they concluded that a small-sized SPVC cannot be considered an ideal conduit in the reconstruction of the RVOT because although the No-React-treated valve largely resisted calcification, pseudointimal peel formation was found in all explanted conduits and led to multilevel conduit stenosis. Moreover, after reviewing the long-term results of RVOT and pulmonary trunk reconstruction with homografts, Kim et al.  determined that although long-term survival was excellent, freedom from reoperation was unsatisfactory, particularly in patients who had small grafts in the initial repair. After analyzing a large patient cohort, Sekarski et al. concluded that smaller pulmonary arteries increase the risk of surgical intervention when using a BJVC to reconstruct the RVOT . Thus, alternative surgical strategies that do not employ small grafts must be considered for young children. In most Chinese areas, especially in rural areas, because of economic considerations, the patient’s custodian tends to refuse to repeat surgical procedures; thus, the BJVC were more commonly used in older children, and BJVP were used more in young children, as shown in Table 1.
To address the problems described, the 24 cases in this study were radically repaired using a procedure in which autogenous tissue was preserved in situ to reconstruct the pulmonary posterior wall. During the follow-up care period, only mild regurgitation of the bovine jugular vein valve was observed in all patients. In the control group, two patients had severe anastomotic stenosis and underwent a further conduit replacement procedure in the early postoperative stage, four patients underwent balloon valvuloplasty and two patients underwent conduit replacement due to stenosis of the BJVC. The most significant differences between the procedures described in the observation group and the control group are as follows: 1. The was a more efficient relief of the right ventricular outflow tract obstruction. 2. The linear bloodstream flow solved the problem of right ventricular overload compared with a traditional conduit that involves the grade climbing of non-linear bloodstream flow. 3. A valved conduit reduced regurgitation, even when there was a gap present between the posterior wall and the bovine valve leaflets. It is important to note, however, that the minimal pulmonary regurgitation did not affect the function of the right ventricle. 4. This technique avoided the severance of remnant pulmonary trunk. Because the preserved autologous vascular bed could stimulate the continual development of the residual pulmonary trunk, young patients, especially children, who receive this procedure will generally not require future conduit replacement. 5. This surgical technique is suitable for PA and does not carry a risk of the major coronary arteries crossing the RVOT.
Reconstructing the pulmonary posterior wall by using in situ autologous tissue is a satisfactory method for treating PA-VSD. Increasing the sample size and continued follow-up care are necessary to fully assess the long-term benefits and advantages of the procedure.
the blind end of inherent pulmonary artery
bovine jugular venous conduit
bovine jugular venous patch
major aortopulmonary collateral arteries
Pulmonary artery index
Pulmonary atresic tissue
Pulmonary atresia with ventricular septal defect
Patent ductus arteriosus
Right ventricular outflow tract
Right ventricular outflow tract obstruction
Shelhigh No-React (NR-4000PA series)-treated porcine pulmonic valve conduit
Visceral layer of pericardium
Ventricular septal defect
This study was supported by the Major Research Plan of the National Natural Science Foundation of China (No. 91539111).
Availability of data and materials
The datasets from the current study are available from the corresponding author upon request.
JY, YY and CF carried out the design of the operative procedure for the technique, performed all the operations and wrote the manuscript. NY carried out the data collection. LX carried out the figure collection. QW and MT carried out the data analysis. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written consent was obtained from the patients’ custodians for publication of the study. All authors have read and approved the content and agree to submit it for consideration for publication in your journal.
Ethics approval and consent to participate
Prior consent from all patients and approval from the local Institutional Research Ethics Committee were obtained.
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