Major Aortopulmonary Collateral Arteries Research Articles

Unifocalization cannot rely exclusively on native pulmonary arteries: the importance of recruitment of major aortopulmonary collaterals in 249 cases†.

We sought to define the early and late outcomes of unifocalization based on a classification of the native pulmonary artery (nPA) system and major aortopulmonary collateral arteries (MAPCAs) with a policy of combined recruitment and rehabilitation and to analyse the role of unifocalization by leaving the ventricular septal defect (VSD) open with a limiting right ventricle-pulmonary artery (RV-PA) conduit in borderline cases. An analysis of 271 consecutive patients assessed for unifocalization at a single institution between 1988 and 2016 was performed. Patients were classified according to the pulmonary blood supply: group A, unifocalization based on nPA only; group B, based on nPA and MAPCAs; group C, MAPCAs only (absent nPAs). Unifocalization was achieved in 249 (91.9%) cases with an early mortality of 2.8%. Group A included 72 (28.9%) patients, group B 119 (47.8%) patients and group C 58 (23.3%) patients with no difference in early survival between groups. Survival at 5, 10 and 15 years was 90.0% (85.9-94.3), 87.2% (83.5-91.2) and 82.3% (75.2-89.9), respectively. Late survival in groups A and B was similar but 10- and 15-year survival in group C decreased to 79.2% (68.2-92.1) and 74.3% (61.1-90.4) (P = 0.02), respectively. A mean of 1.9 (±0.6) MAPCAs were recruited per patient (range 0-6). The VSD was left open with a limiting RV-PA conduit in 97 (39.0%) cases, but subsequently closed in 48 patients, giving a total of 200 (80.3%) patients achieving VSD closure (full repair). Delaying VSD closure was not associated with increased risk for early or late survival. A central shunt to rehabilitate the nPAs was used in 56 (22.5%) cases. This was associated with a reduction in the number of MAPCAs recruited, but still required a mean of 1.8 (±0.5) MAPCAs recruited per patient to achieve unifocalization. In multivariate risk analysis, those suitable for single-stage full repair had the best long-term outcomes. Group C anatomy was associated with poor late survival compared to groups A and B (hazard ratio 2.7). Survival is maximized by a combined approach of rehabilitation and recruitment. MAPCAs should always be recruited if they supply areas with absent nPA supply. A strategy of leaving the VSD open with a limiting RV-PA conduit is a safe and effective way of managing borderline cases.

Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot With MAPCAs

Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair. Patients with TOF/MAPCAs who underwent complete unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size. From November 2001 to December 2017, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35%) had undergone prior surgery elsewhere (n= 16) and/or at our center (n= 7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Five years after unifocalization, survival was 74% ± 6%. At follow-up, the median PA to aortic systolic pressure ratio was 0.36 and was greater than 0.50 in 2 patients. In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.

Prenatal Diagnosis and Postnatal Outcome of Fetuses with Pulmonary Atresia and Ventricular Septal Defect.

To assess the intrauterine course, associated conditions and postnatal outcome of fetuses with pulmonary atresia with ventricular septal defect (PAVSD). All cases of PAVSD diagnosed prenatally over a period of 10 years with a minimum follow-up of 6.5 years were retrospectively collected in 3 tertiary referral centers. 50 cases of PAVSD were diagnosed prenatally. 44.0 % of fetuses had isolated PAVSD, 4.0 % had associated cardiac anomalies, 10.0 % had extra-cardiac anomalies, 38.0 % had chromosomal anomalies, 4.0 % had non-chromosomal syndromes. Among the 32 liveborn children, 56.3 % had reverse flow in the patent arterial duct, 25.0 % had major aortopulmonary collateral arteries (MAPCAs) with ductal agenesis and 18.7 % had a double supply. 17 pregnancies were terminated (34.0 %), there was 1 intrauterine fetal death (2.0 %), 1neonatal death (2.0 %), and 6 deaths (12.0 %) in infancy. 25of 30 (83.3 %) liveborn children with an intention to treat were alive at the latest follow-up. The mean follow-up among survivors was 10.0 years (range 6.5-15.1). 56.0 % of infants underwent staged repair, 44.0 % had one-stage complete repair. After exclusion of infants with additional chromosomal or syndromal anomalies, 88.9 % were healthy, and 11.1 % had mild limitations. The presence of MAPCAs did not differ significantly between survivors and non-survivors (p = 0.360), between one-stage or staged repair (p = 0.656) and healthy and impaired infants (p = 0.319). The prognosis in cases without chromosomal or syndromal anomalies is good. MAPCAs did not influence prognosis or postoperative health. The incidence of repeat interventions due to recurrent stenoses is significantly higher after staged compared with single-stage repair.

Which Pulmonary Homograft to Use in Patients With Major Aortopulmonary Collateral Arteries and Pulmonary Hypertension?

The patient was a 19-year-old male who had complex congenital heart disease with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (MAPCAS). He underwent serial unifocalisation of unilateral MAPCAS to pulmonary homograft tubes with arterial pulmonary shunt supply. Anatomical correction was achieved by removal of arterial shunts, application of a Dacron tube graft to the homograft tubes, which was connected to a 20-mm Hancock bioprosthesis pulmonary valve associated with ventricular septal defect closure at age 7 years. Later in life, he developed right pulmonary artery stenosis and pulmonary valve stenosis. Right pulmonary artery stenosis was treated with a tissue patch and balloon expandable stent. Pulmonary valve stenosis was treated with balloon dilatation. A subsequent attempt at right ventricular outflow tract (RVOT) reconstruction with a pulmonary homograft failed, due to suture line rupture secondary to high RV pressures (90/12 mmHg). This required emergent surgery for a RV-pulmonary artery conduit change with a 22-mm Contegra conduit. Major aortopulmonary collateral arteries is one of the most challenging conditions to manage. Reconstruction of the RVOT may require pulmonary valve replacement with a cryopreserved homograft or bovine jugular vein graft (Contegra) during primary repair or re-operation. One study showed that although survival and freedom from distal stenosis were similar between the two conduits, freedom from failure, dysfunction, and explantation were better with Contegra conduits in patients needing RVOT reconstruction. Furthermore, choice of RV-pulmonary artery conduit must take into account the pulmonary artery pressure, with a pulmonary homograft contraindicated with PAP >50 mmHg.

Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: Part II—Midline Unifocalization and Unifocalization Revision

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare and heterogeneous form of congenital heart disease. Despite improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. Part II of this paper will focus on the surgical results of unifocalization and the need and techniques used for unifocalization revision at Stanford University. We have an experience with more than 300 patients undergoing primary surgical treatment of PA/VSD/MAPCAs. Out of this entire cohort, 80% were selected for a midline unifocalization approach. For patients undergoing a primary unifocalization, 85% underwent a single-stage complete repair, while 15% had a unifocalization and shunt. Ninety-five percent of these patients ultimately achieve complete repair status with a mean right ventricle to pulmonary artery pressure ratio of 0.36 ± 0.09. Of all the patients who undergo a primary unifocalization, 19% will subsequently require unifocalization revision. This was unequally divided into patients who had an initial single-stage complete repair (where 15% required revision) compared with those who had a unifocalization/shunt (46% required revision). The majority of patients (74%) who underwent unifocalization revision had a satisfactory outcome. The surgical treatment of PA/VSD/MAPCAs is complicated due to heterogeneity of MAPCA and pulmonary artery anatomy. The preponderance (80%) of patients treated at Stanford is selected for midline unifocalization. The results of midline unifocalization demonstrate that a high percentage of patients achieve full repair with satisfactory right ventricular pressure ratios.

Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: Part I—Anatomy, Physiology, and Palliative Procedures

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. Despite vast improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. The purpose of this paper is to summarize the surgical algorithm and experience at Stanford University. We have an experience with more than 300 patients undergoing primary surgical treatment of PA/VSD/MAPCAs. Part I will focus on the anatomy, physiology, and the circumstances in which we utilize palliative procedures for the treatment of PA/VSD/MAPCAs. An aortopulmonary window was utilized in 15% of the patients. Indications for an aortopulmonary window at our center are patients with normally arborizing central pulmonary arteries with dual supply MAPCAs in the presence of cyanosis. Five percent of patients had palliative procedures related to the treatment of a ductus or hemitruncus to one lung and unilateral MAPCAs to the contralateral lung. The combined results achieved for these 2 groups demonstrate an eventual complete repair of 78% and midterm survival of 85%. The surgical treatment of PA/VSD/MAPCAs is made more complicated due to heterogeneity of MAPCA and pulmonary artery anatomy. Using the Stanford algorithm, 20% of patients undergo an initial palliative procedure. The results demonstrate that more than 3-quarters of the patients eventually achieve complete repair.

Rehabilitation of Pulmonary Arteries in Pulmonary Atresia, VSD and Mapcas

Patients with pulmonary atresia, Ventricular septal defect (VSD), and Major Aorto Pulmonary Collateral Arteries have a lung circulation depending primarily from perfusion by collateral arteries. A large proportion of them will have central pulmonary vessels connected to a variable proportion of segmental pulmonary vessels. A strategy of rehabilitation of central pulmonary vessels consist in the realization of shunts and conduits between the aorta or the right ventricle and these central vessels in order to obtain a dilatation of these vessels to assist the reconstruction of a pulmonary vascularization. Repetitive patch reconstruction of the central pulmonary vessels or even their replacement are often necessary. Additional procedural steps may be needed at the time of the repair to reconnect to the central pulmonary circulation large intrapulmonary vessels dependent of collateral flow.

Complex Pulmonary Artery Reconstruction

Complex Pulmonary Artery Reconstruction evolved through our long-term clinical experience in the surgical management of patients with major aortopulmonary collateral arteries. A midline sternotomy approach, utilizing novel techniques for exposure and mobilization, enables access to the distal pulmonary artery tree to the segmental and subsegmental levels. This allows reconstructive procedures to address both discrete stenoses and areas of long-segment hypoplasia, to reduce pulmonary hypertension and improve and/or preserve right ventricular function. We have utilized this technique in over 70 patients, with our most recent analysis demonstrating one operative mortality, no operative reintervention, and improvement in right to left ventricular pressure ratio from 0.94 (0.72-1.30) to 0.36 (0.19-0.54) with durable 4 ± 3 year follow-up.

Midline one-stage complete unifocalization early outcomes from a single center.

Objective:This study aims to present our experience with single-stage complete unifocalization and intraoperative flow study for the repair of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries.Methods:This study was conducted through retrospective chart review of all the patients who underwent complete single-stage midline unifocalization in a single tertiary-care institution.Results:Twenty-two patients underwent midline single-stage unifocalization. The median age was 11 months (IQR: 5–21 months). The number of collateral arteries unifocalized was between one and three (median two). In-hospital mortality was 5%. Follow-up was complete; and the median follow-up regarding survival was 20 months (IQR: 10–28 months). There were three late deaths, and the estimated survival rate was 80% at 10 months and on. Out of 22 patients, ventricular septal defect was closed in the first surgery in three patients (14%) and the second surgery in four patients (19%). Total seven patients underwent surgical total repair (32%). Additionally, one out of four patients whose ventricular septal defects were closed with a fenestrated patch is under follow-up with a small ventricular septal defect, while two are waiting for ventricular septal defect closure. Therefore, total eight patients (36%) have reached total correction.Conclusion:Single-stage unifocalization is a feasible treatment option in ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. This cohort had unfavorable results regarding the rate of complete repair. The pitfalls encountered were related to problems with meticulous surgical technique, complete unifocalization, and correct implementation of the flow study.

Outcomes of different rehabilitative procedures in patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries.

The objective of this study was to compare our clinical outcomes of the central shunt and the right ventricle-pulmonary artery (RV-PA) connection in patients with pulmonary atresia, ventricular septal defect and the major aortopulmonary collateral arteries. From November 2009 to October 2017, a total of 157 consecutive patients with pulmonary atresia, ventricular septal defect, the major aortopulmonary collateral arteries and the hypoplastic PAs who underwent palliative surgery were included. Seventy patients underwent the central shunt (the central shunt group) and 87 patients underwent the RV-PA connection (the RV-PA group). Propensity score matching was used to create 2 cohorts with similar baseline characteristics: 56 central shunt patients were one-to-one-matched with 56 RV-PA connection patients. The early and late outcomes were compared. The median duration of follow-up was 18 months in the central shunt group and 22 months in the RV-PA group (P = 0.10). The probability of complete repair was significantly lower in the central shunt group as compared with the RV-PA group (P = 0.048). The Kaplan-Meier estimates of complete repair rates were 47.2 ± 10.0% after 3 years and 56.0 ± 11.6% after 5 years in the central shunt group, which were lower as compared with 62.3 ± 7.6% after 3 years and 74.5 ± 7.2% after 5 years in the RV-PA group. The increase in the mean McGoon ratio and the mean Nakata index were significantly lower in the central shunt group than those in the RV-PA group (0.57 ± 0.52 vs 1.02 ± 0.44, P = 0.036; 98.2 ± 35.1 mm2/m2 vs 176.9 ± 85.4 mm2/m2, P = 0.025, respectively). The in-hospital morbidity and mortality after complete repair were similar between 2 groups. Compared with the central shunt, the RV-PA connection appears to be a more effective palliative procedure to improve the probability of complete repair and PA growth in patients with pulmonary atresia, ventricular septal defect and the major aortopulmonary collateral arteries, in whom primary repair is not feasible.

Surgical results of unifocalization revision

ObjectiveMidline unifocalization has been developed for the surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. All patients will eventually require reoperation due to the presence of a conduit, and some may also require revision of the distal unifocalized bed. The purpose of this study was to evaluate the surgical results of unifocalization revision. MethodsThis was a retrospective review of 254 patients who underwent midline unifocalization for treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Forty-eight of 254 patients (18%) have subsequently undergone unifocalization revision. Thirty-two of these patients had previously undergone a single-stage complete repair, whereas 16 had a unifocalization and placement of a central shunt. ResultsFor the 32 patients who previously underwent a complete repair, there have been no early or late deaths. The peak systolic pulmonary artery to aortic pressure ratio was 0.44 ± 0.11 after the initial repair and increased to 0.82 ± 0.18 before revision. The pressure ratio decreased to 0.41 ± 0.09 after revision. Three of 32 patients (9%) have subsequently undergone a second unifocalization revision. The 16 patients who previously had a unifocalization/shunt underwent unifocalization revision and complete repair (n = 14) and revision and repeat shunt (n = 2). There was 1 operative mortality and 3 late deaths (25% total) in this cohort. Three (25%) of 12 survivors have subsequently undergone a second unifocalization revision. ConclusionsThe data demonstrate that unifocalization revision can be performed with a successful outcome in a majority of patients. Patients who underwent an initial unifocalization/shunt had a higher failure rate than patients who were initially repaired.

Outcome and Associated Findings in Individuals with Pre- and Postnatal Diagnosis of Tetralogy of Fallot (TOF) and Prediction ofEarly Postnatal Intervention.

The aim of our retrospective evaluation was to compare the outcome of patients with prenatal and postnatal diagnosis of Tetralogy of Fallot (TOF) and to analyze prenatal echocardiographic parameters predicting intervention within 30 days postnatal. We evaluated 142 patients in our pediatric heart center and prenatal diagnosis center and prenatal practice Praenatal plus in Cologne between 01/08-06/16. Within the prenatal diagnosis group, 6/74 fetuses (8.1 %) had TOF with pulmonary atresia (TOF-PA), and 6 (8.1 %) had absent pulmonary valve syndrome (TOF-APVS). 14 (18.9 %) had an abnormal karyotype including 9/14 (64.3 %) with microdeletion 22q11.2. 25 (33.8 %) had extracardiac malformation. 4 (5.4 %) had agenesis of ductus arteriosus (DA), 22 (29.7 %) had right aortic arch (RAA) and 9 (12.2 %) had major aortopulmonary collateral arteries (MAPCAs). Within the postnatal diagnosis group, no patient had TOF-PA, 4/68 (5.9 %) had TOF-APVS. 12 (17.6 %) had extracardiac malformations, 9 (13.2 %) had an abnormal karyotype including 2/9 with microdeletion 22q11.2. 10 (14.7 %) had RAA, 9 (13.2 %) had MAPCAs. There were no cases with agenesis of DA. Increasing z-score values of the left/right pulmonary artery (LPA/RPA) prenatally were associated with a lower probability for early postnatal intervention (RPA: p = 0.017; LPA: p = 0.013). Within the prenatal diagnosis group, 12 of 41 (29.3 %) live-born patients with follow-up and intention to treat needed early intervention versus 7 (10.3 %) in the postnatal diagnosis group (p = 0.02). Within the postnatal diagnosis group, there were no deaths, while 2 (4.9 %) post-intervention deaths occurred in the prenatal diagnosis group. There are no significant differences concerning post-intervention survival in the prenatal diagnosis group versus the postnatal diagnosis group. Complex cases may be underrepresented in the postnatal diagnosis group. Smaller RPA/LPA values prenatally seem to be associated with early postnatal intervention.

Analysis of achieving an "ideal" outcome following midline unifocalization.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex form of congenital heart disease. Midline unifocalization has been developed for the surgical treatment of this condition. There are 3 outcome measures that determine long-term success: patients are alive, patients have achieved complete repair (i.e. ventricular septal defect closure), and patients have a relatively low right ventricle-to-aortic pressure ratio (<0.45). However, to date, no studies have combined these 3 outcome measures to analyze the likelihood of achieving an ideal outcome. This was a retrospective review of 255 patients who underwent midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The median age at unifocalization was 4.5 months. Two hundred thirty-five (92%) patients were alive at a mean follow-up of 5.3 years. Two hundred and seventeen (85%) patients underwent single-stage complete repair, and 38 (15%) had an initial unifocalization and shunt. Twenty-four of the 38 palliated patients have subsequently undergone repair. Thus 241 (94%) patients ultimately achieved complete repair. Of the 241 patients who were repaired, 219 (86%) had a right ventricle-to-aortic peak systolic pressure ratio <0.45. Combining these outcome measures, 77% of patients achieved an ideal outcome at one month, 73% at 6 months, 76% at one year, and 80% at 4 years. Most (80%) patients can achieve all 3 measures of favorable outcome at 4 years following midline unifocalization. We speculate that this will be a favorable portend for the future of these patients.

Airway Compression After Unifocalization in Pulmonary Atresia and Aortopulmonary Collateral Arteries

We hypothesized that reconstructed pulmonary artery (PA) size and postrepair PA pressure are associated with airway compression (AC) after complete unifocalization for pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries. Complete unifocalization was performed in 48 consecutive patients between 2000 and 2016. Clinical course and outcome were reviewed, predictors for AC were identified by logistic regression, and the freedom from death was analyzed using Kaplan-Meier method. Postoperative respiratory distress occurred in 23 patients (48%), and AC occurred in 14 (29%). The median duration of follow-up was 3.7 years. AC was caused by central PA and aorta in 7, conduit in 3, and branch PA in 4. Surgical treatment was required in 5 patients (conduit downsizing, suspension of branch PA, conduit+ aorta, branch PA+ aorta, and aorta+ trachea in 1 patient each). Three patients (21%) subsequently required airway stenting. Most (85.7%) of the AC occurred in patients with high right ventricular systolic pressure/left ventricular systolic pressure (>65%), large Nakata index (>200 mm2/m2), and large conduit index (>35 mm/m2). Patients with AC had significantly worse 3-year survival (no AC, 91.2%; AC, 64.2%; p= 0.01). Multivariate analysis identified higher right ventricular systolic pressure/left ventricular systolic pressure (p= 0.04), larger conduit index (p= 0.03), and Nakata index (p= 0.004) as predictors for AC. AC is a common cause of postoperative respiratory distress and tends to be associated with higher postrepair PA pressure, more frequent right ventricular dysfunction, and worse medium-term survival. The study underscores the importance of incorporating all available lung segments to achieve a low PA pressure, potentially preventing pathologic dilatation of the reconstructed PA. Management of patients with poor major aortopulmonary collateral arteries anatomy and physiology remains a challenge.

Utility of a super-flexible three-dimensional printed heart model in congenital heart surgery.

The objective of this study was to assess the utility of 3D printed heart models of congenital heart disease for preoperative surgical simulation. Twenty patient-specific 3D models were created between March 2015 and August 2017. All operations were performed by a young consultant surgeon who had no prior experience with complex biventricular repair. All 15 patients with balanced ventricles had outflow tract malformations (double-outlet right ventricle in 7 patients, congenitally corrected transposition of great arteries in 5, transposition of great arteries in 1, interrupted aortic arch Type B in 1, tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries in 1). One patient had hypoplastic left heart complex, and the remaining 4 patients had a functional single ventricle. The median age at operation was 1.4 (range 0.1-5.9) years. Based on a multislice computed tomography data set, the 3D models were made of polyurethane resins using stereolithography as the printing technology and vacuum casting as the manufacturing method. All but 4 patients with a functional single ventricle underwent complete biventricular repair. The median cardiopulmonary bypass time and aortic cross-clamp time were 345 (110-570) min and 114 (35-293) min, respectively. During the median follow-up period of 1.3 (0.1-2.5) years, no mortality was observed. None of the patients experienced surgical heart block or systemic ventricular outflow tract obstruction. Three-dimensional printed heart models showed potential utility, especially in understanding the relationship between intraventricular communications and great vessels, as well as in simulation for creating intracardiac pathways.

Surgical strategies for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries.

Treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is challenging and controversial. Basically, the collateral arteries are treated surgically by unifocalization to integrate them and unify the blood flow in the pulmonary circulation. These major collateral arteries are anatomically similar to the bronchial arteries; however, they develop into different vessels because of different environmental exposures. Currently, treatment plans involving surgical intervention in early infancy to address the multiple variations of major aortopulmonary collateral arteries are being established to achieve definitive intracardiac repair. Historically, several surgical approaches have been proposed, including multi-stage unifocalization performed by lateral thoracotomy, rehabilitation of the central pulmonary artery followed by intracardiac repair, and single-stage unifocalization by a midline incision. Recently, single-stage unifocalization performed during infancy has become the preferred method for achieving low right ventricular pressure after closing the ventricular septal defect. Furthermore, for the maintenance of the lowest possible right ventricular pressure after definitive repair, combination therapy or hybrid therapy with catheter and surgical intervention is essential. The surgical treatment strategy for a single ventricle with major aortopulmonary collateral arteries is more difficult than that involving two ventricles.

Correction to: Anatomy of the retro-oesophageal major aortopulmonary collateral arteries in patients with pulmonary atresia with ventricular septal defect: results from preoperative CTA.

The original version of this article, originally published online ahead of printing on January 05, 2018, unfortunately contained mistakes.

Association of dead space ventilation and prolonged ventilation after repair of tetralogy of Fallot with pulmonary atresia

BackgroundWe set out to determine whether patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCA) are at risk for elevated dead space ventilation fraction (VD/VT), and whether this is associated with prolonged mechanical ventilation. We hypothesized that elevated VD/VT (>20%) in the first 24 hours after unifocalization surgery is associated with increased risk for prolonged mechanical ventilation (>7 days). MethodsAll patients with TOF/PA/MAPCA undergoing unifocalization surgery between January 2003 and December 2015 were included in this study. Average VD/VT was calculated over the first 24 hours after surgery. Demographic and surgical data were collected. Outcome data included duration of mechanical ventilation. Patients were separated into 2 groups: elevated VD/VT and normal DVSF. Groups were compared using the Student t test, Wilcoxon rank-sum test, and χ2 test. Univariable and multivariable regression analyses were performed with VD/VT as a continuous variable to test for association. ResultsOf the 265 included patients, 127 (48%) had an elevated VD/VT. The 2 groups did not differ significantly in any demographic characteristic. Patients with an elevated VD/VT had longer cardiopulmonary bypass times (P = .03), were more likely to have delayed sternal closure, and more likely to have prolonged respiratory failure (odds ratio, 2.2; 95% confidence interval, 1.2-4.0; P = .007). The percent VD/VT was associated with duration of mechanical ventilation in univariable (P < .001) and multivariable (P < .001) regression analyses when controlled for age, weight and bypass time. ConclusionsElevated postoperative VD/VT is associated with prolonged mechanical ventilation in patients with TOF/PA/MAPCA following unifocalization. Elevated postoperative VD/VT may be an early indicator of patients who will require prolonged duration of mechanical ventilation, allowing optimization of medical management to promote better outcomes.

Repair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung

There are patients born with pulmonary atresia and a ductus arteriosus or hemi-truncus to one lung and major aortopulmonary collateral arteries (MAPCAs) to the contralateral lung. The purpose of this study was to review our surgical results for this relatively rare subset of patients. This was a retrospective review of 35 patients with ductus/hemi-truncus in association with pulmonary atresia with ventricular septal defect and MAPCAs. Our surgical algorithm is bifurcated into two strategies: (1)patients with "favorable" MAPCAs are candidates for single stage complete repair, and (2) patients with "unfavorable"MAPCAs undergo a staged approach. The median age at surgery was 3 months (range, 1 to 7 months), and the mean number of MAPCAs was 3.1 ± 1.3. Twenty patients underwent a single stage complete repair. All 20 of these patients are alive with a right ventricle to aortic pressure ratio of 0.34 ± 0.07. The distribution of pulmonary blood flow by lung perfusion scan was 44% to the right and 56% to the left lung. Fifteen patients underwent a staged approach, including unifocalization of the MAPCAs to a central shunt. There were 2 interim deaths. Twelve patients have undergone complete repair with a right ventricle to aortic pressure ratio of 0.38 ± 0.07. There was 1 late death after complete repair. The distribution of pulmonary blood flow was 39% to the right and 61% to the left lung. The data demonstrate that 32 of 35 patients with ductus/hemi-truncus and MAPCAs ultimately achieved complete repair with relatively low pulmonary artery pressures.

Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals

ObjectivePulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex and heterogeneous form of congenital heart disease. There is a controversy regarding the optimal treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The purpose of this study was to summarize our algorithm and surgical results for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. MethodsThis was a retrospective review of 307 patients undergoing primary surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and major aortopulmonary collateral arteries. There were 3 surgical pathways, including midline unifocalization (n = 241), creation of an aortopulmonary window (n = 46), and other (n = 20). ResultsFor the 241 patients who underwent midline unifocalization, 204 (85.4%) had a single-stage complete repair. There were 37 patients who underwent a midline unifocalization and central shunt, and 24 have subsequently undergone complete repair. Forty-six patients underwent an aortopulmonary window, of whom 36 have subsequently had a complete repair. There were 20 patients who had complex anatomy and underwent procedures other than described, and14 have subsequently undergone complete repair. Thus, for the patients currently eligible, 280 (93.0%) have achieved complete repair. For the 204 patients who had a single-stage complete repair, the mean right ventricle to aortic pressure ratio was 0.36 ± 0.09. Seventy-six patients underwent a staged repair, and the mean right ventricle to aortic pressure ratio was 0.40 ± 0.09 (P < .05 compared with single-stage repair). There were 3 (1.5%) early and 8 (4.0%) late deaths for the single-stage complete repair cohort versus 4 (4.0%) early and 15 (14.9%) late deaths for all other procedures (P < .01). ConclusionsThe data demonstrate that more than 90% of patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries achieved complete repair. The overall mortality was significantly lower in the subgroup of patients who underwent single-stage complete repair.