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.

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.

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.

A Deep Dive Into Retroesophageal Major Aortopulmonary Collateral Arteries.

Background: The anatomy of major aortopulmonary collateral arteries (MAPCAs) can be highly variable with regard to number, anatomic origin, course, and relationship to the native pulmonary arteries. Some MAPCAs travel behind the esophagus (retroesophageal) and bronchus before entering the lung parenchyma. The purpose of this paper was to review the anatomy, physiology, and surgical characteristics of retroesophageal MAPCAs. Methods: This manuscript summarizes the data from a series of three papers that have focused on the subject of retroesophageal MAPCAs from our institution over the past ten years. Results: Two-thirds of patients evaluated had a retroesophageal MAPCA identified at surgery. Retroesophageal major aortopulmonary collateral arteries (REMs) were more common with a left arch (77%) compared with a right arch (53%). Of all REMs evaluated, 83% were single supply, 13% were dual supply with an inadequate connection, and 4% were dual supply with an adequate connection. Based on these findings, 96% of retroesophageal MAPCAs were unifocalized. Follow-up catheterization was performed at a median of 17 months after surgery; 75% of unifocalized MAPCAs were widely patent, 20% were patent but stenotic, and 5% were occluded. Conclusions: The data demonstrate that retroesophageal MAPCAs are relatively common and almost always require unifocalization. At mid-term follow-up, 95% of unifocalized MAPCAs were found to be patent.

Aortic valve replacement in a 41-year-old woman with uncorrected tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries: a case report.

BACKGROUND: Tetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare. CASE PRESENTATION: A 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE). As vegetation gradually increased despite intravenous antibiotic administration, she was scheduled for urgent aortic valve replacement under CPB. Pulmonary blood flow was primarily provided by the MAPCAs originating from the descending aorta. Intra-aortic balloon occlusion for MAPCAs was performed to ensure a bloodless surgical field. Aortic valve replacement was successful. CONCLUSION: An adult with uncorrected TOF developed acute IE and subsequently had successful cardiac surgery under CPB. Understanding TOF physiology with PA and MAPCAs, particularly pulmonary blood flow through MAPCAs, is crucial.

Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects.

Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associated with complex congenital heart disease (CHD), pulmonary atresia with ventricular septal defect, and aortopulmonary collateral arteries. Some cases of complex CHD, associated with a single ventricle after Fontan operation, show pathological changes in the small peripheral pulmonary arteries and pulmonary vascular resistance similar to those observed in pulmonary arterial hypertension (PAH). This condition is termed as the pediatric pulmonary hypertensive vascular disease (PPHVD). Recent advances in genetics have identified the genes responsible for PAH associated with developmental defects of the heart and lungs, such as TBX4 and SOX17. Targeted therapies for PAH have been developed; however, their effects on PH associated with developmental heart and lung defects remain to be established. Real-world data analyses on the anatomy, pathophysiology, genetics, and molecular biology of unique PPHVD cases associated with developmental defects of the heart and lungs, using nationwide and/or international registries, should be conducted in order to improve the treatments and prognosis of patients with these types of pediatric PH.

Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect.

The existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease's rarity. We reviewed 69 cases from 42 studies to reveal the clinical overview of patients with CPF and PAVSD. Among the included patients, the male-to-female ratio was exactly 1:1, and only two patients (3%) exhibited the 22q11.2 microdeletion syndrome. Regarding anatomical features, CPFs originated from the left coronary artery in 65% of patients, and 62% had other major aortopulmonary collateral arteries. Thirty-nine percent of patients had a definitive CPF diagnosis at 0 years of age, whereas 10% were diagnosed in adulthood. Seventy percent underwent catheter angiography to obtain a definitive CPF diagnosis. Ninety-five percent of patients underwent cardiac surgery, and among them, 43% underwent palliative surgery, whereas 52% underwent one-stage repair. Four patients including three adult patients developed cardiac dysfunction due to myocardial ischemia, and three of them exhibited improved cardiac function after the intervention for CPF. Of all the patients, 88% survived and 12% died. The surgical strategy and prognosis were similar to those in PAVSD patients without CPF. This review provides detailed clinical phenotypes that are potentially useful in enhancing the management of patients with this rare disease.

Single-center outcomes after surgical creation of aortopulmonary window in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Background: The aim of this study is to present our experience regarding the creation of an aortopulmonary window as the initial palliative procedure. Methods: Between February 2016 and February 2021, a total of eight patients (3 males, 5 females; median age: 2 months; range, 0.7 to 6 months) who underwent aortopulmonary window creation were retrospectively analyzed. Data collection was performed by review from our institution. Results: There was no occurrence of early or late mortality in any patient. The median postoperative duration of mechanical ventilation and length of hospital stay were five and eight days, respectively. No postoperative reperfusion injury or severe pulmonary overcirculation was observed in any of the patients. Four patients achieved complete repair with unifocalization of the major aortopulmonary collateral arteries, one patient had a second procedure, and the remaining three patients waited for complete repair. The median right ventricle-toaortic pressure ratio after complete repair was 0.6 (range, 0.4 to 0.7). The median follow-up after complete repair was 1.4 (range, 0.9 to 2.8) years, and the median follow-up period for all survivors was 2.7 (range, 0.9-5.8) years. Conclusion: Our study results suggest that aortopulmonary window operation can be safely performed in selected patients with good early and mid-term outcomes. Although the central pulmonary arteries are very small, half of the patients underwent complete repair and achieved acceptable right ventricle-to-aortic pressure ratios. Patient selection criteria and early postoperative interventions are of utmost importance to prevent postoperative pulmonary overcirculation.