Original Article
Repair of congenital heart defects associated with single pulmonary artery
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(2) 157–163 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314536568 aan.sagepub.com
Leo A Bockeria, Osman A Makhachev, Titalav Kh Khiriev, Vladimir P Podzolkov, Mikhail A Zelenikin, Aleksey I Kim and Sergey B Zaets
Abstract Background: Experience with complete repair of congenital heart defects associated with unilateral absence of a pulmonary artery is limited. The aim of this retrospective study was to present our surgical experience of this complex category of patients, to analyze immediate results of surgical interventions, and to suggest a rational surgical strategy. Methods: Of 37 patients with a single pulmonary artery who underwent complete repair of associated heart defects, the left or right pulmonary artery was absent in 32 and 5, respectively. The most frequent heart defects were tetralogy of Fallot (n ¼ 25) and ventricular septal defect (n ¼ 8). The median age of these patients was 7.1 years. Preoperative examinations included echocardiography, cardiac catheterization and angiocardiography, with quantitative assessment of the single pulmonary artery. In-hospital parameters of surgical outcome were analyzed. Results: Recorded hospital mortality was 2.7% (1/37). The single death was in a patient with tetralogy of Fallot, agenesis of the left pulmonary artery, and a small diameter of the contralateral pulmonary artery (Nakata index 174 mm2m2). The right-to-left ventricular systolic pressure ratio after complete tetralogy of Fallot repair in patients who survived the operation was 0.58 0.11. Conclusions: Complete repair of congenital heart defects in patients with unilateral absence of a pulmonary artery is associated with a relatively low risk. If the hilar artery is of adequate size, surgical intervention should attempt restoration of the communication between the disconnected hilar artery and the pulmonary trunk, in addition to repairing the heart defects.
Keywords Pulmonary artery, Pulmonary circulation, Heart defects, congenital, Tetralogy of Fallot
Introduction Agenesis or unilateral absence of a brunch pulmonary artery (UAPA) is complete absence of the intrapericardial segment of one of the main pulmonary arteries. This lesion can be isolated or associated with other congenital heart defects (CHD). Of 395 patients with UAPA described in the available literature, 178 (45%) had associated CHD. CHD with decreased or normal/ increased pulmonary blood flow were reported in 129 (72.5%) and 49 (27.5%) cases, respectively. If patients with UAPA and associated CHD do not have a pulmonary artery at the hilum of the ipsilateral lung, surgical repair is limited to the intracardiac anomalies. This type of surgical intervention can be termed uni-pulmonary correction because the ipsilateral lung
remains excluded from the physiological pulmonary circulation. World experience in uni-pulmonary correction of UAPA associated with CHD consists of 89 cases. In 76 (85%) of these, pulmonary blood flow in the contralateral lung was decreased. Hospital mortality in this group of patients reached 7.9% (6/76). In 13 (15%) patients, pulmonary blood flow in the contralateral lung was normal or increased. There was no fatal outcome after complete repair of CHD in this Bakoulev Center for Cardiovascular Surgery, Moscow, Russia Corresponding author: Sergey B Zaets, 1 Wall Street, Apt. 5B, Fort Lee, NJ 07024, USA. Email: [email protected]
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group of patients. The current optimal surgical strategy in patients with UAPA associated with CHD includes not only repair of the CHD but also restoration of the communication between the pulmonary trunk and the hilar artery of the ipsilateral lung (bi-pulmonary correction).1 The available literature describes 15 successful operations for restoration of antegrade blood flow in the ipsilateral lung. The aim of this retrospective study was to present our surgical experience of complete repair of CHD in patients with associated UAPA and analyze the immediate results of surgical interventions as well as to suggest a rational surgical strategy.
Patients and methods The protocol of this retrospective study was approved by the institutional review board of the Bakoulev Center for Cardiovascular Surgery, Moscow, Russia. All patients signed an informed consent form that allowed the use of their data for scientific analysis and publication. The charts of these patients as well as data of their examinations (echocardiograms, angiocardiograms) are being kept in our institutional archive. Between 1971–2011, 37 patients with UAPA underwent complete repair of concomitant CHD. There were 21 females and 16 males. Their median age was 7.1 years (range 3 months to 26 years). Complete repair was performed during the first year of life in 8 (22%) cases, at 1–18 years of age in 25 (67%), and > 18 years of age in 4 (11%). Thirty-one (84%) patients had decreased pulmonary blood flow in the contralateral lung, whereas in 6 (16%) cases, pulmonary blood flow was normal or increased. CHD with decreased pulmonary blood flow included tetralogy of Fallot (n ¼ 25), double-outlet right ventricle with pulmonary stenosis (n ¼ 1), ventricular septal defect (VSD) with pulmonary stenosis
(n ¼ 1), and VSD with absent pulmonary valve (n ¼ 4). CHD with normal or increased pulmonary blood flow included VSD (n ¼ 3), atrial septal defect (ASD; n ¼ 1), patent ductus arteriosus (n ¼ 1), and aortic stenosis with patent ductus arteriosus (n ¼ 1). The left pulmonary artery was absent in 32 (86%) patients, and the right pulmonary artery was absent in 5 (14%) patients (Table 1). Right-sided aortic arch was diagnosed in 20 patients; 19 of them had agenesis of the left pulmonary artery, and one had agenesis of the right pulmonary artery. Thus in the majority of cases (23/37), the aortic arch was located at the opposite side to the absent pulmonary artery. Sixteen patients (all with tetralogy of Fallot) underwent palliative surgical interventions prior to complete repair. These interventions included right-sided subclavian-to-pulmonary artery Gore-Tex shunt (n ¼ 8), reconstruction of the right ventricular outflow tract without VSD closure (n ¼ 6), and transluminal balloon pulmonary valvuloplasty (n ¼ 2). Five of these patients required a repeat palliative procedure because of inadequate enlargement of the single pulmonary artery. The median interval between the last palliative intervention and complete repair was 3.4 years (range, from 4 months to 17 years). All patients underwent echocardiography, cardiac catheterization and angiography prior to complete repair. We used several parameters to quantitatively assess the single pulmonary artery. First, we took into consideration the pulmonary arterial index according to Nakata and colleagues,2 which reflects the ratio of the pulmonary artery cross-sectional area to body surface area. A Nakata index of 330 30 mm2m2 was considered to be normal. The Nakata index Z-score was used to assess the deviation from normal values. One unit of Z-score corresponds to one standard deviation from the mean of the normal parameter.3 We also
Table 1. Type of agenesis of the pulmonary artery in 37 patients with concomitant congenital heart defects. Patient category Decreased pulmonary blood flow (n ¼ 31)
Agenesis of LPA
Increased pulmonary blood flow (n ¼ 6)
Agenesis of RPA Agenesis of LPA Agenesis of RPA
Concomitant CHD
No. of patients
Tetralogy of Fallot DORV þ pulmonary stenosis VSD þ pulmonary stenosis VSD þ absent pulmonary valve Tetralogy of Fallot VSD ASD PDA Aortic stenosis þ PDA
23* 1 1 4 2 3y 1 1 1
Total 29
2 3 3
*One patient also had PDA. yOne patient also had subaortic stenosis. ASD: atrial septal defect; CHD: congenital heart defects; DORV: double-outlet right ventricle; LPA: left pulmonary artery; PDA: patent ductus arteriosus; RPA: right pulmonary artery; VSD: ventricular septal defect.
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calculated the ratio of the diameter of the single pulmonary artery to the diameter of the normal right or left pulmonary artery as well as the Z-score of this parameter. Data on normal pulmonary arterial diameters were taken from the report of Van Meurs-Van Woezik and colleagues.4 Finally, we determined the ratio of the diameter of the single pulmonary artery to the sum of diameters of the normal pulmonary arteries as well as the Z-score of this parameter. Data on the sum of normal pulmonary arterial diameters were taken from the report of Sairanen and Louhimo.5 Quantitative parameters of the single pulmonary artery are presented separately for the following 3 groups of patients: tetralogy of Fallot/double-outlet right ventricle with pulmonary stenosis (n ¼ 26), VSD with absent pulmonary valve (n ¼ 5), and CHD with increased pulmonary blood flow (n ¼ 6). All surgical interventions, except embolization of a patent ductus arteriosus in one patient, were performed with cardiopulmonary bypass, aortic crossclamping, and hypothermia. Cardiopulmonary bypass in patients with decreased pulmonary blood flow was longer than in those with normal or increased pulmonary blood flow (124.9 38.1 min vs. 72.8 21.1 min, p < 0.05). Aortic crossclamp time and rectal temperature in these two groups of patients did not differ significantly (72.0 28.7 vs. 44.0 23.2 min and 24.7 C 2.6 C, vs. 27.0 C 1.9 C, respectively, p > 0.05). Twenty-one of the 25 patients with tetralogy of Fallot underwent reconstruction of the right ventricular outflow tract using an infundibular patch (n ¼ 3) or transannular patch (n ¼ 18). In 11 cases, a monocusp patch ranging from 12 to 18 mm was used for transannular reconstruction. Subaortic (n ¼ 22) or subarterial (n ¼ 3) VSD were closed with a patch. In the patient with double-outlet right ventricle, a tunnel leading from the left ventricle to the aorta was created using a xenograft pericardial patch, followed by transannular reconstruction of the right ventricular outflow tract with a monocusp patch. The patient with VSD and pulmonary valvular stenosis as well as patients with VSD and absent pulmonary valve underwent VSD patch closure and reconstruction of the right ventricular outflow tract with a transannular patch. A monocusp patch ranging from 12 to 16 mm was used in those with absent pulmonary valve. Three patients with subaortic VSD as well as the patient with ASD underwent patch closure of the defects. The patient with aortic stenosis and patent ductus arteriosus underwent valve replacement with a mechanical ATS prosthesis, enlargement of the aortic annulus using the Manouguian-SeyboldEpting technique, and suturing of the patent ductus arteriosus. All patients were followed up during the hospital period, and subjected to echocardiography upon discharge.
Data are presented as mean standard deviation and/or median and range or 95% confidence interval, as appropriate, or as absolute numbers and percentages. The Student’s t test or analysis of variance were used, as appropriate, to compare continuous variables (clinical, hemodynamic, and anatomical parameters) between groups or before and after surgical intervention. Statistical significance was set at the level of p < 0.05.
Results Patients with decreased pulmonary blood flow, except those with VSD and absent pulmonary valve, demonstrated decreased arterial blood oxygen saturation that ranged from 68% to 94% (mean 82.5% 7.2%). It did not differ significantly in patients with tetralogy of Fallot or double-outlet right ventricle, compared to those with VSD and absent pulmonary valve. Relatively high saturation (above 90%) was recorded in patients with tetralogy of Fallot and well-functioning systemic-to-pulmonary shunts and in patients with VSD and absent pulmonary valve who had moderate pulmonary stenosis caused by the hypoplasia of the fibrous annulus. The patient with VSD, absent pulmonary valve, and tracheobronchial compression by an enlarged aneurysmal single pulmonary artery (Nakata index 1739 mm2m2) had the lowest arterial blood oxygen saturation (68%). The systolic pressure gradient between the right ventricle and the single pulmonary artery in patients with decreased pulmonary blood flow reached 87.5 14.3 mm Hg. Four of the 6 patients with normal or increased pulmonary blood flow had pulmonary hypertension with a ratio of systolic pulmonary arterial to aortic pressure equal to 82.0% 2.4%. Three of them had VSD and one had an ASD. The systolic pressure gradient between the left ventricle and the aorta in a patient with aortic stenosis and patent ductus arteriosus reached 105 mm Hg. Preoperative angiometric parameters of the single pulmonary artery in patients with various types of concomitant CHD are presented in Table 2. Five patients had a Nakata index less than 200 mm2m2. In none of these cases had the hilar artery been identified preoperatively or during the surgical intervention. One patient with tetralogy of Fallot and agenesis of the left pulmonary artery died after surgery. Thus the hospital mortality was 2.7% (1/37). This intraoperative fatal outcome was caused by heart failure; the autopsy revealed iatrogenic trauma of the conus branch of the right coronary artery as well as excessive dimensions of the patch used for right ventricular outflow tract reconstruction (one half of its length). This patient also had a low preoperative arterial blood oxygen saturation (73%) and a small diameter of the contralateral
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Table 2. Preoperative clinical, hemodynamic, and angiocardiographic parameters of 37 patients with congenital heart defects and single pulmonary artery.
Parameters PA index
Ratio of single PA diameter and normal diameter of corresponding LPA or RPAz Ratio of diameter of single PA and sum of normal diameters of LPA and RPA§
Median Nakata index (mm2m2) Median Nakata index Z-score [95%CI] Median Z-score [95%CI] % of normal value
Tetralogy of Fallot/DORV/VSD þ pulmonary stenosis (n ¼ 27)
VSD þ absent pulmonary valve (n ¼ 4)
Other CHD þ normal or increased pulmonary blood flow (n ¼ 6)
242.0 47.0*
967.0 688.0
392.0 43.0
2.8* [3.8 to 1.8] þ9.9 [þ2.3 to þ 46] þ0.7* [0 to þ 1.6] 112.0% 16.0%*
þ1.9 [þ0.9; þ 3.2]
þ6.8 [þ4.6 to þ 14.3] þ4.1 [þ3.2 to þ 6.1] 246.0% 84.0%y 149.0% 23.0%
Median Z-score [95%CI] 1.2* [1.8 to 1.0] þ2.6 [þ0.3 to þ 8.7] % of normal value 83.0% 8.0%* 161.0% 64.0%
þ0.3 [þ0.2 to þ 0.8] 108.0% 5.0%
*p < 0.01 vs. other groups. yp < 0.05 vs. CHD with normal or increased blood pulmonary flow. zAccording to Van Meurs-van Woezik et al.4 §According to Sairanen and Louhimo.5 CHD: congenital heart defects; CI: confidence interval; DORV: double-outlet right ventricle; LPA: left pulmonary artery; PA: pulmonary artery; RPA: right pulmonary artery; VSD: ventricular septal defect.
pulmonary artery (Nakata index 174 mm2m2; Z-score 5.2). Nonfatal postoperative complications were recorded in 38% (14/37) of patients. The most common complication that occurred in 6 patients was cardiac and/or respiratory failure. These patients were subjected to complete repair of tetralogy of Fallot (n ¼ 4), VSD with pulmonary stenosis (n ¼ 1), or VSD with absent pulmonary valve (n ¼ 1). All patients required inotropic support and prolonged mechanical ventilation (median 12 days; range 5–21 days); one of them underwent a tracheostomy. Three patients (all with tetralogy of Fallot) developed chylothorax that required multiple punctures or drainage of the pleural cavity. Other complications that occurred in one patient each included hemorrhage that required resternotomy, hydropericardium that required pericardiocentesis, wound infection, complete atrioventricular blockade that required a permanent pacemaker, and brain edema. All of these complications, except hydropericardium after ASD patch closure, occurred after tetralogy of Fallot repair. Thus, in majority of cases (13/14 or 93%), nonfatal postoperative complications were recorded in patients with decreased pulmonary blood flow. It should also be mentioned that none of the patients with nonfatal postoperative complications had a preoperative Nakata index lower than 200 mm2m2. The postoperative ratio of systolic pulmonary arterial to aortic pressure in patients with preoperative pulmonary hypertension decreased to 47.0% 1.6% (p < 0.05). Arterial blood oxygen saturation at discharge in patients with decreased pulmonary blood
flow increased to 95.8% 1.7% (p < 0.05 vs. preoperative levels).
Discussion We identified 104 cases of complete repair of CHD in patients with UAPA in the available literature. The operations included both uni-pulmonary and bi-pulmonary repair, with an overall hospital mortality of 5.8% (6/104). However, only a few institutions described their experience of several surgical interventions (Table 3).6–12 Eighty-six (83%) of these patients had CHD with decreased pulmonary blood flow, and 18 (17%) had normal or increased pulmonary blood flow. These numbers fully agree with our series (84% vs. 16%), and also show that the most frequent concomitant CHD is tetralogy of Fallot. Successful uni-pulmonary repair of concomitant CHD with decreased pulmonary blood flow (tetralogy of Fallot in particular) in patients with the agenesis of the left or right pulmonary artery was first described by Sherrick and colleagues9 in 1962, and by Turinetto and colleagues13 in 1975. Literature data as well as our findings show that uni-pulmonary repair of UAPA associated with CHD and decreased pulmonary blood flow can be successfully performed as the primary surgical intervention. The hospital mortality in our series of uni-pulmonary repairs in this category of patients (1/31 or 3.2%) is comparable with the cumulative literature data (6/76 or 7.9%). It should be remembered that this surgery directs venous blood supply to a single
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Table 3. Published series of complete repair of congenital heart defects with concomitant unilateral absence of a brunch pulmonary artery. Concomitant CHD Author (year)
No. of cases
Agenesis of LPA
Agenesis of RPA
Zhang6 (1997) Gamba7 (1984) Kim8 (2011)
24 6 6
Tetralogy of Fallot (n ¼ 20) Tetralogy of Fallot (n ¼ 5) Tetralogy of Fallot (n ¼ 4)
Tetralogy of Fallot (n ¼ 4) Tetralogy of Fallot (n ¼ 1) VSD (n ¼ 1) Coarctation of aorta (n ¼ 1)
Sherrick9 (1962)
4
Laborde10 (1983) Mistrot11 (1977) Murphy12 (2004)
4 3 3
Tetralogy of Fallot VSD þ PS (n ¼ 2) Tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot
(n ¼ 2) (n ¼ 4) (n ¼ 3) (n ¼ 3)
CHD: congenital heart defects; LPA: left pulmonary artery; PS: pulmonary stenosis; RPA: right pulmonary artery; VSD: ventricular septal defect.
lung and may predispose to the development of cardiac and/or respiratory failure, which dominated in the spectrum of postoperative complications in our series. This is why preoperative assessment of the pulmonary tree at all levels, as well as left ventricular function, is of major importance. Zhang and colleagues6 suggested the following criteria for complete repair of tetralogy of Fallot in patients with UAPA: left ventricular end-diastolic index above 30 mLm2 and normal development of the single pulmonary artery (ratio of diameters of the proximal pulmonary artery segment and the descending aorta at diaphragm level > 1.5). In our series, the diameter of the contralateral pulmonary artery in patients with decreased pulmonary blood flow reached 100% of the normal diameter of the corresponding left or right pulmonary artery. Moreover, the diameter of the single pulmonary artery was around 80% of the sum of the normal diameters of the left and right pulmonary arteries. We share the opinion regarding the importance of a normal diameter of the contralateral pulmonary artery for a favorable outcome of CHD repair in this category of patients. The single mortality recorded in our institution was in a patient with a low Nakata index of the single pulmonary artery (< 200 mm2m2). At the same time, no patient with a low Nakata index artery developed a nonfatal postoperative complication. Pulmonary hypertension in patients with UAPA and concomitant CHD with increased pulmonary blood flow frequently develops at a young age and leads to right ventricular failure. Regression of pulmonary hypertension may be achieved by early repair of CHD. The first successful uni-pulmonary repairs of concomitant CHD with increased pulmonary blood flow (patent ductus arteriosus in particular) in patients
with agenesis of the left or right pulmonary artery were reported by Swan and colleagues14 in 1963 and by Kucera and colleagues15 in 1982. Similar to the literature data, we had no mortality in this category of patients. In all cases, we observed a significant decrease in the pulmonary arterial pressure. Worldwide experience of the surgical treatment of CHD associated with UAPA demonstrates that the ideal surgical intervention for this category of patients is an organ-saving procedure that restores communication between the pulmonary trunk and the hilar artery, aimed at including the ipsilateral lung into the circulation. Timely restoration of physiological circulation in the ipsilateral lung helps to develop its vascular bed. The necessary prerequisite for this operation is identification of a pulmonary artery in the hilum of the lung. Kim and colleagues8 succeeded in identifying the hilar artery in 80% (12/15) patients with UAPA. The ages of their patients ranged from 10 days to 33 months (median, 5 months). The median diameter reached 2.7 0.7 mm (range 1.6–3.7 mm). Current imaging techniques identify the hilar artery in 66% of cases, so still missing in a third compared to surgical recognition.16 There were no patients with a preoperatively identified hilar artery in our series of 37 cases of CHD repair in patients with UAPA, partly because we were not equipped with each and every method of diagnosis during a substantial part of the data collection. However, we do have experience of hilar artery identification in a 2 month-old patient with agenesis of the right pulmonary artery and concomitant VSD.17 At the age of 7 months, this patient underwent direct anastomosis between the left hilar artery that had a diameter of 4 mm and the pulmonary trunk. We did not perform simultaneous VSD closure because it was
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Figure 1. Algorithm of the preferred surgical treatment in patients with unilateral absence of a brunch pulmonary artery (UAPA) and concomitant congenital heart defects (CHD). *Stages of pulmonary hypertension-induced morphological changes in the pulmonary vessel. **Primary repair of concomitant CHD is indicated in patients with normal dimensions of the contralateral pulmonary artery; palliative reconstruction of the right ventricular outflow tract is indicated in patients with severe hypoplasia of the contralateral pulmonary artery; systemic-to-pulmonary anastomosis with the contralateral pulmonary artery or transluminal balloon valvuloplasty is indicated in patients with hypoplasia of the contralateral pulmonary artery who are in a critical condition. PA: pulmonary artery.
small (2 mm in diameter), and the pulmonary arterial pressure was relatively low (42% of systemic). This case was not included in this series because the concomitant CHD was not repaired. Based on a literature data analysis, we suggest the following classification of bi-pulmonary repair of UAPA associated with CHD. Group I: Single-stage operations with simultaneous repair of intracardiac defects and restoration of the antegrade blood flow in the ipsilateral lung:8,18–20 (a) Single-access technique (b) Double-access technique.
(b) Restoration of the antegrade blood flow in the ipsilateral lung in combination with a palliative operation: (i) In combination with systemic-to-pulmonary shunt with the hilar artery (ii) In combination with systemic-to-pulmonary shunt with the contralateral pulmonary artery (iii) In combination with the reconstruction of the right ventricular inflow tract (iv) In combination with an endovascular procedure. Second stage: complete repair of intracardiac defects.
Group II: Multi-stage operations with primary restoration of communication between the pulmonary trunk and the hilar artery:8,12,17 First stage: (a) Restoration of the antegrade blood flow in the ipsilateral lung
Group III: Multi-stage operations with secondary restoration of communication between the pulmonary trunk and hilar artery:8,12 First stage: palliative operation: (a) Systemic-to-pulmonary anastomosis with the hilar artery
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(b) Systemic-to-pulmonary anastomosis with the contralateral pulmonary artery (c) Reconstruction of the right ventricular inflow tract (d) Endovascular procedure. Second stage: restoration of the antegrade blood flow in the ipsilateral lung with simultaneous or postponed complete repair of intracardiac defects.
6.
7.
8.
Based on literature data as well as on our own experience, we believe that the algorithm in Figure 1 for surgical treatment in patients with UAPA and concomitant CHD is appropriate. Our work has several limitations. First, this was a retrospective analysis of data obtained over 4 decades, which is why there was no standard protocol for the study. In addition, many diagnostic methods being used now were not available for the whole study period. Finally, we were unable to provide long-term results as many patients were lost to follow-up because they are currently residing in different countries. However, we concluded that our data show that unipulmonary repair of concomitant CHD in patients with UAPA can be performed with low hospital mortality. Long-term follow-up is required to see whether surgical intervention can provide a substantial improvement in quality of life. Bi-pulmonary repair is the preferred method of surgical repair. Different strategies for this approach need to have well-defined indications.
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10.
11.
12.
13.
14.
Funding Budget of Bakoulev Center for Cardiovascular Surgery, Moscow, Russia.
15.
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Conflict of interest statement None declared. 17.
References 1. Bockeria LA, Makhachev O, Arakelyan V and Khiriev T. eComment: Congenital absence of the left pulmonary artery: the feasibility of an ‘ideal’ correction. Interact Cardiovasc Thorac Surg 2009; 8: 279. 2. Nakata Y, Imai Y, Takanashi Y, et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984; 88: 610–619. 3. Kirklin JW. Anatomy, dimensions and terminology. In: Kirklin JW, Baratt-Boyes BG (eds) Cardiac surgery. New York: Churchill Livingstone, 1993, pp. 3–60. 4. Van Meurs-van Woezik H, Debets T and Klein HW. Growth of the internal diameters in the pulmonary arterial tree in infants and children. J Anat 1987; 151: 107–115. 5. Sairanen H and Louhimo I. Dimensions of the heart and great vessels in normal children. A postmortem study of
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cardiac ventricles, valves and great vessels. Scand J Thorac Cardiovasc Surg 1992; 26: 83–92. Zhang GC, Wang ZW, Zhang RF, Zhu HY and Yi DH. Surgical repair of patients with tetralogy of Fallot and unilateral absence of pulmonary artery. Ann Thorac Surg 1997; 64: 1150–1153. Gamba A, Villani M, Tiraboschi R, et al. Surgical treatment of the tetralogy of Fallot with a single pulmonary artery. G Ital Cardiol 1984; 14: 499–504. Kim GB, Ban JE, Bae EJ, et al. Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. J Thorac Cardiovasc Surg 2011; 141: 171–178. Sherrick DW, Kincaid OW and Dushane JW. Agenesis of a main branch of the pulmonary artery. Am J Roentgenol Radium Ther Nucl Med 1962; 87: 917–928. Laborde F, de Riberolles C, Escande G, Lino R, Hazan E and Neveux JY. Tetralogy of Fallot with congenital absence of a pulmonary artery. Apropos of 4 cases. Sem Hop 1983; 59: 3043–3046. Mistrot JJ, Bernhard WF, Rosenthal A and Castaneda AR. Tetralogy of Fallot with a single pulmonary artery: operative repair. Ann Thorac Surg 1977; 23: 249–253. Murphy DN, Winlaw DS, Cooper SG and Nunn GR. Successful early surgical recruitment of the congenitally disconnected pulmonary artery. Ann Thorac Surg 2004; 77: 29–35. Turinetto B, Colı` G, Donati A, Galli R, Mikus P and Pierangeli A. Absent right pulmonary artery complicating Tetralogy of Fallot. J Cardiovasc Surg (Torino) 1975; 16: 322–326. Swan H, Owens JC, Pool PE, Vogel JH and Blount SG. Absent left pulmonary artery and right-sided patent ductus arteriosus. Arch Surg 1963; 87: 196–205. Kucera V, Fiser B, Tu˚ma S and Hucin B. Unilateral absence of pulmonary artery: a report on 19 selected clinical cases. Thorac Cardiovasc Surg 1982; 30: 152–158. Bockeria LA, Makhachev OA and Khiriev TK. Unilateral absence of the pulmonary artery. Moscow: Bakoulev Center for Cardiovascular Surgery at Russian Academy of Medical Sciences, 2012, p. 141. Bockeria LA, Makhachev OA, Berishvili OD, et al. Congenital unilateral absence of the pulmonary artery: surgical restoration of antegrade blood flow in the ipsilateral lung. Pediatr Dis Heart Vessels 2011; 3: 47–53. (Available at: http://elibrary.ru/item.asp?id=17112383. Accessed April 29, 2014). Apostolopoulou SC, Kelekis NL, Brountzos EN, Rammos S and Kelekis DA. ‘‘Absent’’ pulmonary artery in one adult and five pediatric patients: imaging, embryology, and therapeutic implications. AJR Am J Roentgenol 2002; 179: 1253–1260. Hamdan MA, Al Meshham Y and Najm HK. Successful one-stage repair of unilateral agenesis of pulmonary artery. Pediatr Cardiol 2005; 26: 724–726. Welch K, Hanley F, Johnston T, Cailes C and Shah MJ. Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann Thorac Surg 2005; 79: 1399–1402.
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Repair of congenital heart defects associated with single pulmonary artery
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(2) 157–163 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314536568 aan.sagepub.com
Leo A Bockeria, Osman A Makhachev, Titalav Kh Khiriev, Vladimir P Podzolkov, Mikhail A Zelenikin, Aleksey I Kim and Sergey B Zaets
Abstract Background: Experience with complete repair of congenital heart defects associated with unilateral absence of a pulmonary artery is limited. The aim of this retrospective study was to present our surgical experience of this complex category of patients, to analyze immediate results of surgical interventions, and to suggest a rational surgical strategy. Methods: Of 37 patients with a single pulmonary artery who underwent complete repair of associated heart defects, the left or right pulmonary artery was absent in 32 and 5, respectively. The most frequent heart defects were tetralogy of Fallot (n ¼ 25) and ventricular septal defect (n ¼ 8). The median age of these patients was 7.1 years. Preoperative examinations included echocardiography, cardiac catheterization and angiocardiography, with quantitative assessment of the single pulmonary artery. In-hospital parameters of surgical outcome were analyzed. Results: Recorded hospital mortality was 2.7% (1/37). The single death was in a patient with tetralogy of Fallot, agenesis of the left pulmonary artery, and a small diameter of the contralateral pulmonary artery (Nakata index 174 mm2m2). The right-to-left ventricular systolic pressure ratio after complete tetralogy of Fallot repair in patients who survived the operation was 0.58 0.11. Conclusions: Complete repair of congenital heart defects in patients with unilateral absence of a pulmonary artery is associated with a relatively low risk. If the hilar artery is of adequate size, surgical intervention should attempt restoration of the communication between the disconnected hilar artery and the pulmonary trunk, in addition to repairing the heart defects.
Keywords Pulmonary artery, Pulmonary circulation, Heart defects, congenital, Tetralogy of Fallot
Introduction Agenesis or unilateral absence of a brunch pulmonary artery (UAPA) is complete absence of the intrapericardial segment of one of the main pulmonary arteries. This lesion can be isolated or associated with other congenital heart defects (CHD). Of 395 patients with UAPA described in the available literature, 178 (45%) had associated CHD. CHD with decreased or normal/ increased pulmonary blood flow were reported in 129 (72.5%) and 49 (27.5%) cases, respectively. If patients with UAPA and associated CHD do not have a pulmonary artery at the hilum of the ipsilateral lung, surgical repair is limited to the intracardiac anomalies. This type of surgical intervention can be termed uni-pulmonary correction because the ipsilateral lung
remains excluded from the physiological pulmonary circulation. World experience in uni-pulmonary correction of UAPA associated with CHD consists of 89 cases. In 76 (85%) of these, pulmonary blood flow in the contralateral lung was decreased. Hospital mortality in this group of patients reached 7.9% (6/76). In 13 (15%) patients, pulmonary blood flow in the contralateral lung was normal or increased. There was no fatal outcome after complete repair of CHD in this Bakoulev Center for Cardiovascular Surgery, Moscow, Russia Corresponding author: Sergey B Zaets, 1 Wall Street, Apt. 5B, Fort Lee, NJ 07024, USA. Email: [email protected]
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group of patients. The current optimal surgical strategy in patients with UAPA associated with CHD includes not only repair of the CHD but also restoration of the communication between the pulmonary trunk and the hilar artery of the ipsilateral lung (bi-pulmonary correction).1 The available literature describes 15 successful operations for restoration of antegrade blood flow in the ipsilateral lung. The aim of this retrospective study was to present our surgical experience of complete repair of CHD in patients with associated UAPA and analyze the immediate results of surgical interventions as well as to suggest a rational surgical strategy.
Patients and methods The protocol of this retrospective study was approved by the institutional review board of the Bakoulev Center for Cardiovascular Surgery, Moscow, Russia. All patients signed an informed consent form that allowed the use of their data for scientific analysis and publication. The charts of these patients as well as data of their examinations (echocardiograms, angiocardiograms) are being kept in our institutional archive. Between 1971–2011, 37 patients with UAPA underwent complete repair of concomitant CHD. There were 21 females and 16 males. Their median age was 7.1 years (range 3 months to 26 years). Complete repair was performed during the first year of life in 8 (22%) cases, at 1–18 years of age in 25 (67%), and > 18 years of age in 4 (11%). Thirty-one (84%) patients had decreased pulmonary blood flow in the contralateral lung, whereas in 6 (16%) cases, pulmonary blood flow was normal or increased. CHD with decreased pulmonary blood flow included tetralogy of Fallot (n ¼ 25), double-outlet right ventricle with pulmonary stenosis (n ¼ 1), ventricular septal defect (VSD) with pulmonary stenosis
(n ¼ 1), and VSD with absent pulmonary valve (n ¼ 4). CHD with normal or increased pulmonary blood flow included VSD (n ¼ 3), atrial septal defect (ASD; n ¼ 1), patent ductus arteriosus (n ¼ 1), and aortic stenosis with patent ductus arteriosus (n ¼ 1). The left pulmonary artery was absent in 32 (86%) patients, and the right pulmonary artery was absent in 5 (14%) patients (Table 1). Right-sided aortic arch was diagnosed in 20 patients; 19 of them had agenesis of the left pulmonary artery, and one had agenesis of the right pulmonary artery. Thus in the majority of cases (23/37), the aortic arch was located at the opposite side to the absent pulmonary artery. Sixteen patients (all with tetralogy of Fallot) underwent palliative surgical interventions prior to complete repair. These interventions included right-sided subclavian-to-pulmonary artery Gore-Tex shunt (n ¼ 8), reconstruction of the right ventricular outflow tract without VSD closure (n ¼ 6), and transluminal balloon pulmonary valvuloplasty (n ¼ 2). Five of these patients required a repeat palliative procedure because of inadequate enlargement of the single pulmonary artery. The median interval between the last palliative intervention and complete repair was 3.4 years (range, from 4 months to 17 years). All patients underwent echocardiography, cardiac catheterization and angiography prior to complete repair. We used several parameters to quantitatively assess the single pulmonary artery. First, we took into consideration the pulmonary arterial index according to Nakata and colleagues,2 which reflects the ratio of the pulmonary artery cross-sectional area to body surface area. A Nakata index of 330 30 mm2m2 was considered to be normal. The Nakata index Z-score was used to assess the deviation from normal values. One unit of Z-score corresponds to one standard deviation from the mean of the normal parameter.3 We also
Table 1. Type of agenesis of the pulmonary artery in 37 patients with concomitant congenital heart defects. Patient category Decreased pulmonary blood flow (n ¼ 31)
Agenesis of LPA
Increased pulmonary blood flow (n ¼ 6)
Agenesis of RPA Agenesis of LPA Agenesis of RPA
Concomitant CHD
No. of patients
Tetralogy of Fallot DORV þ pulmonary stenosis VSD þ pulmonary stenosis VSD þ absent pulmonary valve Tetralogy of Fallot VSD ASD PDA Aortic stenosis þ PDA
23* 1 1 4 2 3y 1 1 1
Total 29
2 3 3
*One patient also had PDA. yOne patient also had subaortic stenosis. ASD: atrial septal defect; CHD: congenital heart defects; DORV: double-outlet right ventricle; LPA: left pulmonary artery; PDA: patent ductus arteriosus; RPA: right pulmonary artery; VSD: ventricular septal defect.
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calculated the ratio of the diameter of the single pulmonary artery to the diameter of the normal right or left pulmonary artery as well as the Z-score of this parameter. Data on normal pulmonary arterial diameters were taken from the report of Van Meurs-Van Woezik and colleagues.4 Finally, we determined the ratio of the diameter of the single pulmonary artery to the sum of diameters of the normal pulmonary arteries as well as the Z-score of this parameter. Data on the sum of normal pulmonary arterial diameters were taken from the report of Sairanen and Louhimo.5 Quantitative parameters of the single pulmonary artery are presented separately for the following 3 groups of patients: tetralogy of Fallot/double-outlet right ventricle with pulmonary stenosis (n ¼ 26), VSD with absent pulmonary valve (n ¼ 5), and CHD with increased pulmonary blood flow (n ¼ 6). All surgical interventions, except embolization of a patent ductus arteriosus in one patient, were performed with cardiopulmonary bypass, aortic crossclamping, and hypothermia. Cardiopulmonary bypass in patients with decreased pulmonary blood flow was longer than in those with normal or increased pulmonary blood flow (124.9 38.1 min vs. 72.8 21.1 min, p < 0.05). Aortic crossclamp time and rectal temperature in these two groups of patients did not differ significantly (72.0 28.7 vs. 44.0 23.2 min and 24.7 C 2.6 C, vs. 27.0 C 1.9 C, respectively, p > 0.05). Twenty-one of the 25 patients with tetralogy of Fallot underwent reconstruction of the right ventricular outflow tract using an infundibular patch (n ¼ 3) or transannular patch (n ¼ 18). In 11 cases, a monocusp patch ranging from 12 to 18 mm was used for transannular reconstruction. Subaortic (n ¼ 22) or subarterial (n ¼ 3) VSD were closed with a patch. In the patient with double-outlet right ventricle, a tunnel leading from the left ventricle to the aorta was created using a xenograft pericardial patch, followed by transannular reconstruction of the right ventricular outflow tract with a monocusp patch. The patient with VSD and pulmonary valvular stenosis as well as patients with VSD and absent pulmonary valve underwent VSD patch closure and reconstruction of the right ventricular outflow tract with a transannular patch. A monocusp patch ranging from 12 to 16 mm was used in those with absent pulmonary valve. Three patients with subaortic VSD as well as the patient with ASD underwent patch closure of the defects. The patient with aortic stenosis and patent ductus arteriosus underwent valve replacement with a mechanical ATS prosthesis, enlargement of the aortic annulus using the Manouguian-SeyboldEpting technique, and suturing of the patent ductus arteriosus. All patients were followed up during the hospital period, and subjected to echocardiography upon discharge.
Data are presented as mean standard deviation and/or median and range or 95% confidence interval, as appropriate, or as absolute numbers and percentages. The Student’s t test or analysis of variance were used, as appropriate, to compare continuous variables (clinical, hemodynamic, and anatomical parameters) between groups or before and after surgical intervention. Statistical significance was set at the level of p < 0.05.
Results Patients with decreased pulmonary blood flow, except those with VSD and absent pulmonary valve, demonstrated decreased arterial blood oxygen saturation that ranged from 68% to 94% (mean 82.5% 7.2%). It did not differ significantly in patients with tetralogy of Fallot or double-outlet right ventricle, compared to those with VSD and absent pulmonary valve. Relatively high saturation (above 90%) was recorded in patients with tetralogy of Fallot and well-functioning systemic-to-pulmonary shunts and in patients with VSD and absent pulmonary valve who had moderate pulmonary stenosis caused by the hypoplasia of the fibrous annulus. The patient with VSD, absent pulmonary valve, and tracheobronchial compression by an enlarged aneurysmal single pulmonary artery (Nakata index 1739 mm2m2) had the lowest arterial blood oxygen saturation (68%). The systolic pressure gradient between the right ventricle and the single pulmonary artery in patients with decreased pulmonary blood flow reached 87.5 14.3 mm Hg. Four of the 6 patients with normal or increased pulmonary blood flow had pulmonary hypertension with a ratio of systolic pulmonary arterial to aortic pressure equal to 82.0% 2.4%. Three of them had VSD and one had an ASD. The systolic pressure gradient between the left ventricle and the aorta in a patient with aortic stenosis and patent ductus arteriosus reached 105 mm Hg. Preoperative angiometric parameters of the single pulmonary artery in patients with various types of concomitant CHD are presented in Table 2. Five patients had a Nakata index less than 200 mm2m2. In none of these cases had the hilar artery been identified preoperatively or during the surgical intervention. One patient with tetralogy of Fallot and agenesis of the left pulmonary artery died after surgery. Thus the hospital mortality was 2.7% (1/37). This intraoperative fatal outcome was caused by heart failure; the autopsy revealed iatrogenic trauma of the conus branch of the right coronary artery as well as excessive dimensions of the patch used for right ventricular outflow tract reconstruction (one half of its length). This patient also had a low preoperative arterial blood oxygen saturation (73%) and a small diameter of the contralateral
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Table 2. Preoperative clinical, hemodynamic, and angiocardiographic parameters of 37 patients with congenital heart defects and single pulmonary artery.
Parameters PA index
Ratio of single PA diameter and normal diameter of corresponding LPA or RPAz Ratio of diameter of single PA and sum of normal diameters of LPA and RPA§
Median Nakata index (mm2m2) Median Nakata index Z-score [95%CI] Median Z-score [95%CI] % of normal value
Tetralogy of Fallot/DORV/VSD þ pulmonary stenosis (n ¼ 27)
VSD þ absent pulmonary valve (n ¼ 4)
Other CHD þ normal or increased pulmonary blood flow (n ¼ 6)
242.0 47.0*
967.0 688.0
392.0 43.0
2.8* [3.8 to 1.8] þ9.9 [þ2.3 to þ 46] þ0.7* [0 to þ 1.6] 112.0% 16.0%*
þ1.9 [þ0.9; þ 3.2]
þ6.8 [þ4.6 to þ 14.3] þ4.1 [þ3.2 to þ 6.1] 246.0% 84.0%y 149.0% 23.0%
Median Z-score [95%CI] 1.2* [1.8 to 1.0] þ2.6 [þ0.3 to þ 8.7] % of normal value 83.0% 8.0%* 161.0% 64.0%
þ0.3 [þ0.2 to þ 0.8] 108.0% 5.0%
*p < 0.01 vs. other groups. yp < 0.05 vs. CHD with normal or increased blood pulmonary flow. zAccording to Van Meurs-van Woezik et al.4 §According to Sairanen and Louhimo.5 CHD: congenital heart defects; CI: confidence interval; DORV: double-outlet right ventricle; LPA: left pulmonary artery; PA: pulmonary artery; RPA: right pulmonary artery; VSD: ventricular septal defect.
pulmonary artery (Nakata index 174 mm2m2; Z-score 5.2). Nonfatal postoperative complications were recorded in 38% (14/37) of patients. The most common complication that occurred in 6 patients was cardiac and/or respiratory failure. These patients were subjected to complete repair of tetralogy of Fallot (n ¼ 4), VSD with pulmonary stenosis (n ¼ 1), or VSD with absent pulmonary valve (n ¼ 1). All patients required inotropic support and prolonged mechanical ventilation (median 12 days; range 5–21 days); one of them underwent a tracheostomy. Three patients (all with tetralogy of Fallot) developed chylothorax that required multiple punctures or drainage of the pleural cavity. Other complications that occurred in one patient each included hemorrhage that required resternotomy, hydropericardium that required pericardiocentesis, wound infection, complete atrioventricular blockade that required a permanent pacemaker, and brain edema. All of these complications, except hydropericardium after ASD patch closure, occurred after tetralogy of Fallot repair. Thus, in majority of cases (13/14 or 93%), nonfatal postoperative complications were recorded in patients with decreased pulmonary blood flow. It should also be mentioned that none of the patients with nonfatal postoperative complications had a preoperative Nakata index lower than 200 mm2m2. The postoperative ratio of systolic pulmonary arterial to aortic pressure in patients with preoperative pulmonary hypertension decreased to 47.0% 1.6% (p < 0.05). Arterial blood oxygen saturation at discharge in patients with decreased pulmonary blood
flow increased to 95.8% 1.7% (p < 0.05 vs. preoperative levels).
Discussion We identified 104 cases of complete repair of CHD in patients with UAPA in the available literature. The operations included both uni-pulmonary and bi-pulmonary repair, with an overall hospital mortality of 5.8% (6/104). However, only a few institutions described their experience of several surgical interventions (Table 3).6–12 Eighty-six (83%) of these patients had CHD with decreased pulmonary blood flow, and 18 (17%) had normal or increased pulmonary blood flow. These numbers fully agree with our series (84% vs. 16%), and also show that the most frequent concomitant CHD is tetralogy of Fallot. Successful uni-pulmonary repair of concomitant CHD with decreased pulmonary blood flow (tetralogy of Fallot in particular) in patients with the agenesis of the left or right pulmonary artery was first described by Sherrick and colleagues9 in 1962, and by Turinetto and colleagues13 in 1975. Literature data as well as our findings show that uni-pulmonary repair of UAPA associated with CHD and decreased pulmonary blood flow can be successfully performed as the primary surgical intervention. The hospital mortality in our series of uni-pulmonary repairs in this category of patients (1/31 or 3.2%) is comparable with the cumulative literature data (6/76 or 7.9%). It should be remembered that this surgery directs venous blood supply to a single
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Table 3. Published series of complete repair of congenital heart defects with concomitant unilateral absence of a brunch pulmonary artery. Concomitant CHD Author (year)
No. of cases
Agenesis of LPA
Agenesis of RPA
Zhang6 (1997) Gamba7 (1984) Kim8 (2011)
24 6 6
Tetralogy of Fallot (n ¼ 20) Tetralogy of Fallot (n ¼ 5) Tetralogy of Fallot (n ¼ 4)
Tetralogy of Fallot (n ¼ 4) Tetralogy of Fallot (n ¼ 1) VSD (n ¼ 1) Coarctation of aorta (n ¼ 1)
Sherrick9 (1962)
4
Laborde10 (1983) Mistrot11 (1977) Murphy12 (2004)
4 3 3
Tetralogy of Fallot VSD þ PS (n ¼ 2) Tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot
(n ¼ 2) (n ¼ 4) (n ¼ 3) (n ¼ 3)
CHD: congenital heart defects; LPA: left pulmonary artery; PS: pulmonary stenosis; RPA: right pulmonary artery; VSD: ventricular septal defect.
lung and may predispose to the development of cardiac and/or respiratory failure, which dominated in the spectrum of postoperative complications in our series. This is why preoperative assessment of the pulmonary tree at all levels, as well as left ventricular function, is of major importance. Zhang and colleagues6 suggested the following criteria for complete repair of tetralogy of Fallot in patients with UAPA: left ventricular end-diastolic index above 30 mLm2 and normal development of the single pulmonary artery (ratio of diameters of the proximal pulmonary artery segment and the descending aorta at diaphragm level > 1.5). In our series, the diameter of the contralateral pulmonary artery in patients with decreased pulmonary blood flow reached 100% of the normal diameter of the corresponding left or right pulmonary artery. Moreover, the diameter of the single pulmonary artery was around 80% of the sum of the normal diameters of the left and right pulmonary arteries. We share the opinion regarding the importance of a normal diameter of the contralateral pulmonary artery for a favorable outcome of CHD repair in this category of patients. The single mortality recorded in our institution was in a patient with a low Nakata index of the single pulmonary artery (< 200 mm2m2). At the same time, no patient with a low Nakata index artery developed a nonfatal postoperative complication. Pulmonary hypertension in patients with UAPA and concomitant CHD with increased pulmonary blood flow frequently develops at a young age and leads to right ventricular failure. Regression of pulmonary hypertension may be achieved by early repair of CHD. The first successful uni-pulmonary repairs of concomitant CHD with increased pulmonary blood flow (patent ductus arteriosus in particular) in patients
with agenesis of the left or right pulmonary artery were reported by Swan and colleagues14 in 1963 and by Kucera and colleagues15 in 1982. Similar to the literature data, we had no mortality in this category of patients. In all cases, we observed a significant decrease in the pulmonary arterial pressure. Worldwide experience of the surgical treatment of CHD associated with UAPA demonstrates that the ideal surgical intervention for this category of patients is an organ-saving procedure that restores communication between the pulmonary trunk and the hilar artery, aimed at including the ipsilateral lung into the circulation. Timely restoration of physiological circulation in the ipsilateral lung helps to develop its vascular bed. The necessary prerequisite for this operation is identification of a pulmonary artery in the hilum of the lung. Kim and colleagues8 succeeded in identifying the hilar artery in 80% (12/15) patients with UAPA. The ages of their patients ranged from 10 days to 33 months (median, 5 months). The median diameter reached 2.7 0.7 mm (range 1.6–3.7 mm). Current imaging techniques identify the hilar artery in 66% of cases, so still missing in a third compared to surgical recognition.16 There were no patients with a preoperatively identified hilar artery in our series of 37 cases of CHD repair in patients with UAPA, partly because we were not equipped with each and every method of diagnosis during a substantial part of the data collection. However, we do have experience of hilar artery identification in a 2 month-old patient with agenesis of the right pulmonary artery and concomitant VSD.17 At the age of 7 months, this patient underwent direct anastomosis between the left hilar artery that had a diameter of 4 mm and the pulmonary trunk. We did not perform simultaneous VSD closure because it was
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Figure 1. Algorithm of the preferred surgical treatment in patients with unilateral absence of a brunch pulmonary artery (UAPA) and concomitant congenital heart defects (CHD). *Stages of pulmonary hypertension-induced morphological changes in the pulmonary vessel. **Primary repair of concomitant CHD is indicated in patients with normal dimensions of the contralateral pulmonary artery; palliative reconstruction of the right ventricular outflow tract is indicated in patients with severe hypoplasia of the contralateral pulmonary artery; systemic-to-pulmonary anastomosis with the contralateral pulmonary artery or transluminal balloon valvuloplasty is indicated in patients with hypoplasia of the contralateral pulmonary artery who are in a critical condition. PA: pulmonary artery.
small (2 mm in diameter), and the pulmonary arterial pressure was relatively low (42% of systemic). This case was not included in this series because the concomitant CHD was not repaired. Based on a literature data analysis, we suggest the following classification of bi-pulmonary repair of UAPA associated with CHD. Group I: Single-stage operations with simultaneous repair of intracardiac defects and restoration of the antegrade blood flow in the ipsilateral lung:8,18–20 (a) Single-access technique (b) Double-access technique.
(b) Restoration of the antegrade blood flow in the ipsilateral lung in combination with a palliative operation: (i) In combination with systemic-to-pulmonary shunt with the hilar artery (ii) In combination with systemic-to-pulmonary shunt with the contralateral pulmonary artery (iii) In combination with the reconstruction of the right ventricular inflow tract (iv) In combination with an endovascular procedure. Second stage: complete repair of intracardiac defects.
Group II: Multi-stage operations with primary restoration of communication between the pulmonary trunk and the hilar artery:8,12,17 First stage: (a) Restoration of the antegrade blood flow in the ipsilateral lung
Group III: Multi-stage operations with secondary restoration of communication between the pulmonary trunk and hilar artery:8,12 First stage: palliative operation: (a) Systemic-to-pulmonary anastomosis with the hilar artery
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(b) Systemic-to-pulmonary anastomosis with the contralateral pulmonary artery (c) Reconstruction of the right ventricular inflow tract (d) Endovascular procedure. Second stage: restoration of the antegrade blood flow in the ipsilateral lung with simultaneous or postponed complete repair of intracardiac defects.
6.
7.
8.
Based on literature data as well as on our own experience, we believe that the algorithm in Figure 1 for surgical treatment in patients with UAPA and concomitant CHD is appropriate. Our work has several limitations. First, this was a retrospective analysis of data obtained over 4 decades, which is why there was no standard protocol for the study. In addition, many diagnostic methods being used now were not available for the whole study period. Finally, we were unable to provide long-term results as many patients were lost to follow-up because they are currently residing in different countries. However, we concluded that our data show that unipulmonary repair of concomitant CHD in patients with UAPA can be performed with low hospital mortality. Long-term follow-up is required to see whether surgical intervention can provide a substantial improvement in quality of life. Bi-pulmonary repair is the preferred method of surgical repair. Different strategies for this approach need to have well-defined indications.
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14.
Funding Budget of Bakoulev Center for Cardiovascular Surgery, Moscow, Russia.
15.
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Conflict of interest statement None declared. 17.
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