Major aortopulmonary collateral arteries: clinical, echocardiographic, and radiologic characteristics.

Purpose: Major aortopulmonary collateral arteries (MAPCAs) are rare congenital anomalies with significant clinical implications, often associated with congenital heart diseases like tetralogy of Fallot (TOF) and pulmonary atresia (PA). This study aimed to investigate the clinical, echocardiographic, and radiologic characteristics of MAPCAs in patients with congenital heart diseases admitted to our clinic between 2016 and 2023. Material and methods: A retrospective analysis of 46 cases was conducted using chest computed tomography exams performed on a dual-source 128-slice CT scanner. Clinical data and radiologic characteristics were collected and analysed. Results: The study revealed a strong correlation between congenital heart diseases and the presence of MAPCAs, with TOF, PA, and ventricular septal defect (VSD) being the most common, and it indicated that these collaterals may exist with non-diagnosed congenital heart disease. Tricuspid regurgitation and aortic insufficiency were the predominant echocardiographic findings. Radiologically, MAPCAs primarily originated from the descending aorta (type II) in 85% of cases, and their sizes ranged from ≤ 3 mm to > 10 mm, with an average of 5 mm. Conclusions: This study provides comprehensive insights into the clinical and radiologic aspects of MAPCAs in patients with congenital heart diseases. The findings emphasise the importance of early detection and intervention for better managing of these complex conditions and the need for further research.

Abernethy malformation with unusual cardiac malformation: Case report and literature review.

Abernethy malformation, also known as congenital extrahepatic shunt, is a rare anomaly, which is characterized by partial or complete diversion of the portal blood into the systemic venous circulation. The clinical manifestations of Abernethy malformation during childhood include neonatal cholestasis, failure to thrive, mental retardation, and other congenital defects. We report a case of Abernethy malformation Type II in a 9-year-old boy, whose left ventricle was slightly enlarged because of several major aortopulmonary collateral arteries (MAPCAs) but laboratory examinations were normal 5 years earlier. The characteristics of congenital heart disease in patients with Abernethy malformation are discussed. We propose that physicians should be aware of the possibility of Abernethy malformation in children with enlargement of the left ventricular due to systemic-pulmonary collateral circulation.

Follow the MAPCA: an unusual source of right pulmonary artery flow.

Major aortopulmonary collateral arteries in the setting of pulmonary atresia with intact ventricular septum are very rarely encountered, having been documented in only a handful of case reports. We present the right ventriculogram of a patient found to have this rare combination of findings along with right ventricular-dependent coronary artery circulation and unusual supply of the right pulmonary artery.

Computed tomography in tetralogy of Fallot: pre- and postoperative imaging evaluation.

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although children with TOF share four characteristic features (subaortic ventricular septal defect, overriding aorta, right ventricular hypertrophy, pulmonary stenosis), the clinical spectrum and course are in fact greatly heterogeneous. Echocardiography remains the mainstay for diagnosis, presurgical planning and postoperative follow-up. However, with continued technological advances, CT now plays an increasing role in TOF evaluation and management, helping to minimize routine invasive catheter angiography. Preoperatively, CT is uniquely suited to assess associated pulmonary arterial, aortic and coronary anomalies as well as extra-cardiovascular structures and is particularly helpful for delineating complex anatomy in the TOF subtypes of absent pulmonary valve and pulmonary atresia with major aortopulmonary collaterals. Postoperatively, CT is useful for identifying surgical complications and for long-term monitoring including volumetry quantification, especially in children for whom MRI is contraindicated or limited by implanted devices such as pacemakers and stents. In this article, we review key clinical features and considerations in the pre- and postoperative TOF patient and the burgeoning role of CT for facilitating accurate diagnosis and personalized intervention.

Unifocalization with pericardial roll technique in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10). RESULTS: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group. CONCLUSION: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization.

Overview of Cardiopulmonary Bypass Techniques and the Incidence of Postoperative Complications in Pediatric Patients Undergoing Complex Pulmonary Artery Reconstruction.

Cardiopulmonary bypass (CPB) is routinely used for performing congenital heart operations. While most congenital heart operations can be performed with bypass times under 2 hours, complex pulmonary artery reconstructions require longer periods of CPB to facilitate the surgical repair. This article is intended to summarize the surgical and perfusion techniques utilized in patients undergoing complex pulmonary artery reconstructions at our institution. The initial portion of this manuscript provides an in-depth description of the surgical techniques employed for pulmonary artery reconstructions. This information is important in order to understand why prolonged CPB is a necessary requirement. The manuscript then provides a detailed description of the perfusion techniques and the modifications to the CPB circuit. Finally, the manuscript provides a summary of data from a clinical study evaluating the application of these techniques in 100 consecutive children undergoing complex pulmonary artery reconstruction. The data from this study demonstrated that there was a poor correlation between duration of CPB and both the number of postoperative complications and hospital length of stay. Major adverse cardiac events occurred in 11 (11%) patients with one hospital mortality. These results suggest that prolonged CPB does not predispose to adverse outcomes in this select population of patients.

The Case of a 44-Year-Old Survivor of Unrepaired Tetralogy of Fallot, Right Aortic Arch and Abdominal Aortopulmonary Collateral Vessels.

The most common congenital cyanotic heart disease is described in the literature as the Tetralogy of Fallot. This abnormality is characterized by the presence of ventricular septal defect (VSD), obstruction of the right ventricular (RV) outflow tract, right ventricular hypertrophy, and overriding aorta. In patients with pulmonary atresia with ventricular septal defect (PA/VSD), major aortopulmonary collateral arteries (MAPCA) are common; however, although some of them do not have PA/VSD, they do have other particular anatomical variants. The case we are presenting in this article is a rare mild symptomatic adult noncorrected TOF, with preserved RV function, right aortic arch, and MAPCAs ("classic" thoracic MAPCAs but also abdominal MAPCAs). The anatomy of a complex congenital defect is well illustrated by cardiac magnetic resonance (CMR) and computer tomography angiography (CTA), and these imaging techniques are mostly used to understand the relative clinical "silence" TOF. Imaging scans thus play a key role in the evaluation of these patients, being very important to know the indications and limitations of each method, but also to learn to combine them with each other depending on the clinical picture of the patient's presentation. Additionally, the close collaboration between clinicians and imagers is essential for a correct, complete and detailed preoperative evaluation, being subsequently essential for cardiovascular surgeons, the whole team thus deciding the best therapeutic management.

Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease.

OBJECTIVE: To assess outcomes in a large cohort of patients with Alagille syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex pulmonary artery disease. STUDY DESIGN: Patients with ALGS who underwent pulmonary artery reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch pulmonary artery stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs. RESULTS: Fifty-one patients with ALGS underwent pulmonary artery surgery at our center, including 22 with severe branch pulmonary artery stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) underwent complete repair at the first surgery, compared with 8 (89%) with TOF without MAPCAs and 19 (86%) with isolated branch pulmonary artery stenosis. At a median follow-up of 1.7 years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median pulmonary artery:aortic systolic pressure of 0.38. Nine patients (18%), including 8 with isolated branch pulmonary artery stenosis, underwent liver transplantation. CONCLUSIONS: Most patients with ALGS and complex pulmonary artery disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcomes, with higher mortality and more frequent pulmonary artery interventions compared with patients with TOF without MAPCAs or isolated branch pulmonary artery stenosis. Complex pulmonary artery disease is not a contraindication to liver transplantation in patients with ALGS.

Successful surgical management of cyanotic congenital heart disease complicated by pulmonary aspergillosis.

Pulmonary aspergillosis associated with cyanotic congenital heart disease is a rare condition, which is known to have a poor prognosis. We report a case of a 21-year-old woman with truncus arteriosus and major aortopulmonary collateral arteries who underwent primary Rastelli procedure after thoracoscopic lobectomy for the management of progressive pulmonary aspergillosis.

Rare cause of dynamic aortic obstruction in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries: a case report.

A 2-month-old infant with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries developed an aneurysmatic elongation of the tricuspid valve tissue that partially closed and dynamically protruded through the ventricular septal defect, beneath the aortic valve. This rare finding caused dynamic left ventricle outflow tract obstruction and recurrent cardiac arrests and ultimately required surgical intervention.

Systemic-to-pulmonary shunt vs right ventricle to pulmonary artery connection in the treatment of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

OBJECTIVE: The systemic-to-pulmonary shunt (SPS) and right ventricle to pulmonary artery (RV-PA) connection were evaluated to pursue the goal of rehabilitating dysplastic native PAs via establishment of antegrade blood flow. However, the application of these two palliative operations was still confusing. We compared the two operations to determine their different effects on patients who have pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries (MAPCAs). METHODS: From January 2011 to January 2016, 44 patients received the SPS procedure, and 54 patients received the RV-PA connection procedure; these procedures were compared based on perioperative data and follow-up data. There was no significant difference between the two groups for follow-up time (15.5 ± 11.8 vs 11.4 ± 10 months; P = .073). RESULTS: The SPS patients had a smaller preoperative pulmonary artery index (68.57 ± 38.25 vs 112.62 ± 61.63 mm2 /m2 ; P < .01), more MAPCAs (2.4 ± 1.1 vs 1.8 ± 1.5; P = .045) and had a shorter intubation time (26.73 ± 27.20 vs 40.88 ± 36.93 hours; P = .045), intensive care unit stay (3.6 ± 3.9 vs 5.7 ± 5.5 days; P = .033), and hospital stay (9.9 ± 3.9 vs 14.7 ± 11.9 days; P = .014) than the RV-PA connection patients. The cumulative complete repair rate and cumulative survival rate did not differ significantly between the two groups. CONCLUSIONS: Both the SPS and the RV-PA connection could rehabilitate the PA and produce complete repair, while the SPS could achieve better early postoperative outcomes and be suitable for patients with severe dysplastic PAs and large MAPCAs.

Approaches to Pulmonary Atresia With Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs) is one of the most challenging surgical conditions to manage-not only because of the technical complexity of the surgery but also in terms of defining the anatomy of the pulmonary vasculature, the timing of surgery, and decision making on staged vs complete repair. The importance of early definition of pulmonary blood supply is paramount, establishing which areas of the lung are supplied by MAPCAs alone and which have dual supply with the native system (noting that 20% of patients have absent intrapericardial native vessels). Early unifocalization (3-6 months) is ideal, with closure of the ventricular septal defect (VSD) performed if 15 or more out of 20 lung segments can be recruited. Leaving the ventricular septal defect open with a limiting right ventricle-pulmonary artery conduit can be a useful interim or even definitive circulation in patients with borderline vasculature. Rehabilitation of small native vessels with central shunts can be very effective, but best outcomes are achieved by a combination of unifocalization of MAPCAs together with the native vessels (if present). A variety of reconstructive techniques are necessary to be able to effect these complex repairs with careful choice of materials. Ideally, surgery can be completed through sternotomy alone, but separate thoracotomies may be necessary to control and access some MAPCAs.

Comprehensive Management of Major Aortopulmonary Collaterals in the Repair of Tetralogy of Fallot.

The heterogenous anatomy of Tetralogy of Fallot with major aortopulmonary collateral arteries has engendered a similar degree of diversity in its management and, ultimately, outcome. We summarize our comprehensive treatment paradigm for this lesion evolved over 15 years of experience through 458 patients and the results obtained. An updated analysis of 307 patients treated primarily at our institution is included. A review of recent literature, comparison of management strategies, and discussion of ongoing controversies are provided.

Pulmonary flow-study can predict in-hospital prognosis of unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries.

Objectives: With the development of perioperative treatment, the results of the unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries have been significantly improved. However, the in-hospital recovery is quite different individually. Therefore, it is essential to find prognostic indicators to avoid unsatisfactory recovery. Design: This was a case-control study. Setting: The study was conducted in the national center for cardiovascular diseases in China. Participants: Pediatric patients between 2014 and 2022. Interventions: None. Measurements & main results: A total of 19 patients were included. The possible prognostic indicators included were commonly used clinical data. Unsatisfactory postoperative recovery was defined as mechanical ventilation≥ 7 days and/or in-hospital death. Satisfactory postoperative recovery was defined as mechanical ventilation<7 days and survival at discharge. We separated patients into two groups and compared the peri-operative data through univariable analysis. There were 8 patients in unsatisfactory recovery group (including 1 death) and 11 patients in satisfactory recovery group. Among all the possible prognostic indicators, through univariable analysis, pulmonary arterial pressure in pulmonary flow study was statistically different (p = 0.027 < 0.05). The ROC curve showed that the area under curve and cut-off values in predicting satisfactory recovery were 0.841 and 22 mmHg; the corresponding sensitivity and specificity were 100% and 72.7%. There was no statistical difference between the two groups in ventricular septal fenestration and pulmonary hypertension targeting drugs. Conclusion: A pulmonary arterial pressure <22 mmHg in pulmonary flow study may avoid unsatisfactory in-hospital recovery after unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries.

ARCAPA in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

We report a case of anomalous origin of the right coronary artery from the pulmonary artery in a patient with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. The diagnosis was made during a proposed hybrid approach to stent the native right ventricular outflow tract, and an alternative surgical strategy was created.

"One-stop" Hybrid approach for tetralogy of fallot with aortopulmonary collateral arteries in adults.

We report a 45 year-old Chinese woman with tetralogy of fallot that had two aortopulmonary collateral arteries and tricuspid regurgitation. Collateral circulation was blocked and total correction was successfully performed in our "one-stop" hybrid operation room. The patient was weaned from cardiopulmonary bypass after 97 minutes and was transferred to the intensive care unit for about 36 hours. Without any complications, the patient was discharged home in the following eight days.

Basic Concepts and Insights into Aortopulmonary Collateral Arteries in Congenital Heart Diseases.

Aortopulmonary collateral arteries are persistent embryological vessels supplying lung parenchyma in various cardiopulmonary diseases with underlying pulmonary hypoperfusion. Their identification and mapping are important because of associated clinical implications and tendency to affect the surgical outcome. This article describes the embryological development and clinical relevance of aortopulmonary collaterals in various congenital cardiopulmonary conditions, along with the significance for treatment planning. Roles, strength, and shortcomings of the various imaging options and image-guided interventions are discussed, with a focus on presurgical planning and preparation, as well as postsurgical management.

Modified central shunt in pulmonary atresia with diminutive pulmonary arteries.

The commonly used central shunt (i.e. direct connection of the divided main pulmonary artery to the ascending aorta) to facilitate the growth of the diminutive branch pulmonary artery in patients with pulmonary atresia with major aortopulmonary collateral arteries can cause main pulmonary artery distortion, and subsequent disproportional branch pulmonary artery flow and growth. This report describes a novel technique of shunt construction to minimize pulmonary artery torque and tension by using a modified central shunt constructed from autologous pericardium that is anastomosed in an end-to-side fashion to the lateral aspect of the ascending aorta. This shunt will serve as an intermediary conduit to facilitate the main pulmonary artery anastomosis while minimizing torque and tension on the main pulmonary artery. The tension-free connection between the main pulmonary artery and the aorta enabled by the modified central shunt supports proportional pulmonary artery flow and growth in patients with pulmonary atresia, ventricular septal defect, or multiple aortopulmonary collateral arteries and concomitant diminutive native pulmonary arteries.

Impact of Anatomical Sub-types and Shunt Types on Aortopulmonary Collaterals in Hypoplastic Left Heart Syndrome.

This study aims to clarify the relation of development of aortopulmonary collateral arteries (APCs) with anatomical sub-types and the shunt types at Norwood procedure in patients with hypoplastic left heart syndrome (HLHS). A total of 140 patients with HLHS who completed 3 staged palliation between 2003 and 2019 were included. Incidence of APCs and corresponding interventions were examined using angiogram by cardiac catheterization, with respect to the anatomical sub-types and shunt types. Totally, APCs were observed in 87 (62%) of the patients; pre-stage II in 32 (23%), pre-stage III in 64 (46%), and after stage III in 40 (29%). The incidence of APCs before stage II was significantly higher in patients with aortic atresia/mitral atresia (AA/MA) compared with other sub-types (P = 0.022). Patients with right ventricle to pulmonary artery conduit (RVPAC) had a higher incidence of APCs originating from the descending aorta, compared with those with modified Blalock-Taussig shunt (20% vs 2%, P= 0.002). Interventions for APCs were performed in 58 (41%) patients; before stage II in 10 (7%), after stage II in 7 (5%), before stage III in 22 (16%), and after stage III in 32 (23%). Patients with AA/MA had more interventions before stage II (P= 0.019), and patients with aortic stenosis/mitral stenosis (AS/MS) had a lower incidence of interventions after stage III (P= 0.047). More than half of the patients with HLHS developed APCs. Before stage II, patients with AA/MA sub-type had a higher incidence of APCs, and those with RVPAC had significantly more APCs from the descending aorta.

Challenges and outcomes of pregnancy in an uncorrected Tetralogy of Fallot with pulmonary atresia and major aorta-pulmonary collateral arteries (MAPCA): a case report.

BACKGROUND: Tetralogy of Fallot is a severe type of congenital heart disease (CHD) and one of the leading indirect causes of mortality & morbidity among women with CHD. We came across a rare case of an uncorrected Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries in pregnancy. CASE PRESENTATION: We are reporting the challenges in managing a pregnancy of 25-years-old G3 P0110, previous one stillbirth and who was diagnosed to have congenital heart disease during pregnancy following spontaneous abortion. CONCLUSIONS: This case report highlights the role of multidisciplinary care in managing such a high risk case. It also emphasizes the role of cardiac examination of every woman before pregnancy so that definitive treatment or optimization can be done in time for a better outcome.