A retrospective study: Long term prognosis in adults with PA-VSD-MAPCAs.

BACKGROUND: Pulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment. METHODS: A prospectively established database of 169 PA-VSD-MAPCAs patients treated and followed up at University Hospitals Leuven was accessed. Patients were divided into three groups according to the treatment strategy. Kaplan-Meier survival curves were plotted, and Log Rank tests were used for comparison. RESULTS: The overall mean survival for patients with PA-VSD-MAPCAs was 38.5 years (95%-CI: 33.1-43.9). Patients with complete intracardiac repair had the longest mean survival of 43.8 years (95%-CI: 38.1-49.6) versus the other groups (p < 0.001). A longer mean event-free survival time was found in patients with normal, well-developed NPAs (p = 0.047). Finally, patients with poorly developed or absent NPAs had worse survival rates when a surgical approach was followed. Systemic-pulmonary shunt placement or unifocalisation had limited effect on prognosis in the absence of total repair (p = 0.167). CONCLUSIONS: Patients with PA-VSD-MAPCAs who underwent complete intracardiac repair and/or with well-developed native pulmonary arteries had the best prognosis. Our analyzed data suggest that incomplete surgical repair resulted in survival rates comparable to those seen with a non-surgical approach.

Multiple Major Aortopulmonary Collateral Arteries With Tetralogy of Fallot and Other Congenital Cardiac Disorders Detected in the Fourth Decade: A Report of a Rare Case.

The occurrence of MAPCAs (major aortopulmonary collateral arteries) with TOF (tetralogy of Fallot) and bilateral hypoplastic pulmonary arteries together is a rare condition. Patients are typically middle-aged men who usually present with acute signs of cardiac manifestations. The anomalies have survival up to the fourth decade of life and are fraught with clinical challenges. Additionally, various congenital syndromic associations, such as DiGeorge syndrome, are associated with these anomalies. We report an extremely rare case of a 41-year-old male who came with complaints of chest pain, dyspnea on exertion, and headaches. The patient had a previous history of tuberculosis and a rare combination of MAPCAs with TOF and bilateral hypoplastic pulmonary arteries, with a right-sided aortic arch with an aplastic left subclavian artery. The importance of the case comes from the need to perform surgery on a middle-aged male who was completely asymptomatic prior to this.

Pulmonary atresia and ventricular septal defect: How accurate is the fetal echocardiography, and do the major aortopulmonary collateral arteries matter?

OBJECTIVE: To assess the accuracy of prenatal echocardiography in defining pulmonary vasculature in pulmonary atresia with VSD (PAVSD). The second aim is to compare the perinatal and postnatal outcomes of different pulmonary blood supply types. STUDY DESIGN: The cases prenatally diagnosed with PAVSD between 2017 and 2022 in a single tertiary fetal medicine center were identified on the electronic database. Fetal echocardiography reports and images were reviewed retrospectively. Postnatal outcomes were acquired from the hospital records of relevant pediatric cardiology and cardiovascular surgery clinics. Fetal echocardiography results were compared with postnatal results. Perinatal and postnatal outcomes were compared between the different pulmonary vascular supply types. RESULTS: Among the 24 PAVSD cases, six were diagnosed with major aortopulmonary collateral arteries (MAPCA) dependent, eleven were diagnosed with ductus arteriosus (DA) dependent pulmonary supply, and seven were diagnosed with double pulmonary supply (MAPCA + DA) on prenatal echocardiography. Seventeen cases were live-born and have undergone postnatal investigations. Fetal echocardiography was 88.2% accurate about the type of pulmonary supply. The accuracy of fetal echocardiography regarding pulmonary vascular anatomy was 82.3%. Postoperative survival was 69.2%. Mortality before surgery and postoperative survival did not differ between pulmonary supply groups. Survival was impaired by the extracardiac anomalies. The need for early interventions was significantly higher in the DA group. CONCLUSION: Pulmonary vascularization in PAVSD can be defined precisely on fetal echocardiography. The source of pulmonary blood supply does not impact postnatal short-term outcomes significantly but it impacts the management. The associated anomalies highly contribute to postnatal mortality.

A rare case of pulmonary atresia with ventricular septal defect with right-sided aortic arch.

Pulmonary atresia with ventricular septal defect is a congenital heart malformation in which a lack of continuity between ventricles and the pulmonary artery is accompanied by ventricular septal defect. We report a 3-year-old female patient, who presented with a history of shortness of breath and bluish lips. Computed tomography pulmonary angiography showed an absent main pulmonary artery, right pulmonary artery, left pulmonary artery and showed a defect that connects the right ventricle and left ventricle, which concluded a VSD (ventricular septal defect).

Clinical deterioration following arterial switch surgery due to extensive aortopulmonary collaterals, unusual condition but worth considering: case report.

BACKGROUND: The occurrence of major aortopulmonary collateral arteries (MAPCAs) is infrequent in patients with D-transposition of great arteries (D-TGA) with intact ventricular septum (IVS). Hemodynamically significant MAPCAs may complicate the postoperative course of these patients after arterial switch operation (ASO). CASE PRESENTATION: We present a rare case of neonatal D-TGA-IVS associated with extensive MAPCAs. After the ASO, the patient developed pulmonary hemorrhage, chest wall edema, and deterioration of lung compliance with the need for high-frequency ventilation (HFV). The patient also had a significant capillary leak with skin edema, high chest tube drainage, and high peritoneal drainage. Cardiac catheterization revealed extensive MAPCAs supplying the whole lung segments. After the catheter closure of most of these MAPCAs, the patient had clinical improvement. CONCLUSIONS: Although the occurrence of MAPCAs with D-TGA-IVS is infrequent, clinicians should suspect their presence in cases with unexplained heart failure, pulmonary hemorrhage, or cardiovascular compromise after ASO. Catheter closure of MAPCAs is feasible with an acceptable short-term outcome.

Alternative Hybrid Approach to Promote Native Pulmonary Artery Growth in Pulmonary Atresia VSD MAPCAs.

We present a case of right ventricle to pulmonary artery hybrid perforation and stenting in a patient with pulmonary atresia with ventricular septal defect major aortopulmonary collaterals and diminutive native pulmonary arteries, then discuss how it compares with established approaches. (Level of Difficulty: Advanced.).

A Rare Cause of Hemoptysis in West Syndrome-Isolated Aortopulmonary Collaterals in Structurally Normal Heart.

Major aortopulmonary collateral arteries (MAPCAs) are abnormal systemic to pulmonary collateral vessels originating from the persistent segmental arteries. The common conditions concomitant with MAPCA are congenital heart diseases with reduced pulmonary blood flow. Isolated MAPCAs represent occurrence of collaterals in the absence of underlying heart disease, which commonly present as heart failure, recurrent respiratory tract infection, and pulmonary artery hypertension. Here, we describe a case of West syndrome presenting with hemoptysis due to isolated MAPCAs and its causal relation and management.

Pregnancy in a Patient With Tetralogy of Fallot and Borderline Pulmonary Arterial Hypertension.

Management of pregnancy in patients with complex congenital heart disease and pulmonary arterial hypertension has always been a challenge. We are presenting a patient with complex congenital heart disease and borderline pulmonary arterial hypertension who complicated with pulmonary embolism during pregnancy. (Level of Difficulty: Beginner.).

Patient-Specific 3-Dimensional Printing of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

A 3-dimensional printed model derived from a computed tomography dataset was obtained in a patient with tetralogy of Fallot and major aortopulmonary collaterals. The virtual and solid 3-dimensional heart models provided intuitive representation of such complex and highly individual pathologies and allowed comprehensive spatial conceptualization of the cardiac structures. (Level of Difficulty: Advanced.).

Aortopulmonary collaterals in neonates with d-transposition of the great arteries - Clinical significance early after arterial switch operation.

OBJECTIVES: Purpose of this study is to evaluate the clinical significance of major aortopulmonary collateral arteries (MAPCAs) during the early postoperative course after arterial switch operation (ASO) in d-transposition of the great arteries (dTGA). METHODS: Clinical data of 98 patients with simple dTGA between January 2007 and December 2016 at the University Children's Hospital Zurich, Switzerland were analyzed retrospectively. RESULTS: 37 from 98 patients (38%) required cardiac catheterization (CC) due to an early complicated postoperative course or difficult coronary transfer due to special coronary anatomy. In 15 (15%) patients, hemodynamically relevant MAPCAs were found during CC and coil embolization was performed. Patients with relevant MAPCAs had a significantly longer PICU stay (7 versus 6 days, p = 0.021), longer hospital stay (41 versus 27 days, p = 0.005), longer mechanical ventilation time (5 versus 3 days, p = 0.005), longer need for inotropic support (5 versus 4 days, p = 0.001) and delayed chest closure time (3 versus 2 days, p = 0.030) in those in whom it was left open in comparison to all other patients. In patients having CC, pre-surgery oxygen saturation was significantly lower in patients with relevant MAPCAs (58% vs 70%, p 0.019). Echocardiography had a sensitivity of 53% and a specificity of 100% in detecting relevant MAPCAs, accurately. CONCLUSIONS: MAPCAs are frequently found in dTGA patients and can be associated with lower baseline oxygen saturation and a prolonged postoperative course after ASO. Transthoracic echocardiography cannot replace CC as diagnostic tool. If significant MAPCAs are suspected, early CC should be performed for diagnostic and therapeutic reasons.

Dynamic four-dimensional CT angiography for the assessment of pulmonary perfusion in an adult patient with pulmonary artery occlusion and major aortopulmonary collateral after multistage repair of Fallot's Pentalogy.

Dynamic CT angiography provides haemodynamic assessment combined with detailed information on complex cardiac anatomy in patients with congenital malformations such as multistage correction of Fallot's Pentalogy.

Bronchoscopy in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals.

OBJECTIVE: Children with Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for developing more postoperative respiratory complications than children undergoing other types of congenital heart surgery. Bronchoscopy is used in the perioperative period for diagnostic and therapeutic purposes. In this study, we describe bronchoscopic findings and identify factors associated with selection for bronchoscopy. DESIGN: Retrospective case-control. PATIENTS AND METHODS: All patients with TOF/PA/MAPCAs who underwent unifocalization surgery from September 2005 through March 2016 were included. Patients who underwent bronchoscopy in the perioperative period were compared to a randomly selected cohort of 172 control patients who underwent unifocalization without bronchoscopy during the study period. RESULTS: Forty-three children underwent perioperative bronchoscopy at a median of 9 days postoperatively. Baseline demographics were similar in bronchoscopy patients and controls. Patients who underwent bronchoscopy were more likely to have a chromosome 22q11 deletion and were more likely have undergone unifocalization surgery without intracardiac repair. These patients had a longer duration of mechanical ventilation, ICU duration, and length of hospitalization. Abnormalities were detected on bronchoscopy in 35 patients (81%), and 20 (35%) of bronchoscopy patients underwent a postoperative intervention related to abnormalities identified on bronchoscopy. CONCLUSION: Bronchoscopy is a useful therapeutic and diagnostic instrument for children undergoing unifocalization surgery, capable of identifying abnormalities leading to an additional intervention in over one third of patients. Special attention should be given to children with a 22q11 deletion to expedite diagnosis and intervention for possible airway complications.

Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries: A 15-Year Experience With 458 Patients.

BACKGROUND: Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction. METHODS AND RESULTS: We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure. CONCLUSIONS: Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.

Ruptured aneurysm of major aortopulmonary collateral artery: management using amplatzer vascular plug.

Aneurysm of a major aortopulmonary collateral artery (MAPCA) is quite rare. Aneurysmally dilated MAPCA may be complicated with rupture and massive hemoptysis leading to sudden death. Possible pathophysiology for aneurysm formation is persistent high pressure state in collateral circulation. High index of suspicion is necessary to avoid catastrophic complications as the amount of hemoptysis does not correlate with disease severity and etiology. We present a case of large ruptured aneurysm of a MAPCA presenting with massive haemoptysis in a patient of cyanotic congenital heart disease which was salvaged by endovascular deployment of vascular plug.

Implantation of an Absorb bioresorbable vascular scaffold in the stenotic aortopulmonary collateral artery of a young child with Alagille syndrome.

Stent implantation in children can be problematic due to the possibility of growth incapacity and difficulties during later surgery. For these reasons, bioresorbable stents may be a good alternative to conventional stents. We report our experience with implantation of an Absorb bioresorbable stent in the stenotic major aortopulmonary collateral artery (MAPCA) of a 1-year-old girl born with pulmonary atresia with ventricular septal defect.

Myocardial ischemia following arterial switch operation: An uncommon etiology.

Myocardial ischemia following arterial switch operation (ASO) usually occurs due to coronary ostial narrowing that might result from technical failure in translocation of the coronary arteries to the neoaorta. We present an unusual case report of neonatal myocardial ischemia caused by coronary steal secondary to aortopulmonary collaterals, following ASO in transposition of great arteries.