Unilateral Branch Pulmonary Artery Origin From a Solitary Arterial Trunk With Major Aortopulmonary Collaterals to the Contralateral Lung: Anatomic and Developmental Considerations

2020 ◽  
Author(s):  
Gregory T. Adamson ◽  
Lucile Houyel ◽  
Doff B. McElhinney ◽  
Shiraz A. Maskatia ◽  
Michael Ma ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Branch Pulmonary Artery ◽  
Contralateral Lung ◽  
Arterial Trunk ◽  
Aortopulmonary Collaterals ◽  
Major Aortopulmonary Collaterals

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies

2018 ◽  
Vol 28 (9) ◽  
pp. 1091-1098 ◽  
Author(s):  
Sophie C. Hofferberth ◽  
Jesse J. Esch ◽  
David Zurakowski ◽  
Christopher W. Baird ◽  
John E. Mayer ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Branch Pulmonary Artery ◽  
Collateral Vessel ◽  
Aortopulmonary Collaterals ◽  
Group 2 ◽  
Major Aortopulmonary Collaterals ◽  
Group 1

AbstractIntroductionThe optimal approach to unifocalisation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (pulmonary artery/ventricular septal defect/major aortopulmonary collaterals) remains controversial. Moreover, the impact of collateral vessel disease burden on surgical decision-making and late outcomes remains poorly defined. We investigated our centre’s experience in the surgical management of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals.Materials and methodsBetween 1996 and 2015, 84 consecutive patients with pulmonary artery/ventricular septal defect/major aortopulmonary collaterals underwent unifocalisation. In all, 41 patients received single-stage unifocalisation (Group 1) and 43 patients underwent multi-stage repair (Group 2). Preoperative collateral vessel anatomy, branch pulmonary artery reinterventions, ventricular septal defect status, and late right ventricle/left ventricle pressure ratio were evaluated.ResultsMedian follow-up was 4.8 compared with 5.7 years for Groups 1 and 2, respectively, p = 0.65. Median number of major aortopulmonary collaterals/patient was 3, ranging from 1 to 8, in Group 1 compared with 4, ranging from 1 to 8, in Group 2, p = 0.09. Group 2 had a higher number of lobar/segmental stenoses within collateral vessels (p = 0.02). Group 1 had fewer catheter-based branch pulmonary artery reinterventions, with 5 (inter-quartile range from 1 to 7) per patient, compared with 9 (inter-quartile range from 4 to 14) in Group 2, p = 0.009. Among patients who achieved ventricular septal defect closure, median right ventricle/left ventricle pressure was 0.48 in Group 1 compared with 0.78 in Group 2, p = 0.03. Overall mortality was 6 (17%) in Group 1 compared with 9 (21%) in Group 2.DiscussionSingle-stage unifocalisation is a promising repair strategy in select patients, achieving low rates of reintervention for branch pulmonary artery restenosis and excellent mid-term haemodynamic outcomes. However, specific anatomic substrates of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals may be better suited to multi-stage repair. Preoperative evaluation of collateral vessel calibre and function may help inform more patient-specific surgical management.

scholarly journals An Unusual Cause of Hypoxia: Ventricular Septal Defect, Pulmonary Artery Atresia, and Major Aortopulmonary Collaterals Diagnosed in the Adult Cardiac Catheterization Lab

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Katia Bravo-Jaimes ◽  
Brian Walton ◽  
Poyee Tung ◽  
Richard W. Smalling
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Cardiac Catheterization ◽  
Mortality Risk ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
High Mortality Risk ◽  
Catheterization Laboratory ◽  
Aortopulmonary Collaterals ◽  
Major Aortopulmonary Collaterals

The association of pulmonary atresia, ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA) is an extreme form of tetralogy of Fallot (TOF). It carries a high mortality risk if not intervened on during infancy with only 20% of unoperated patients surviving into adulthood. We present the case of a 40-year-old man who presented for evaluation prior to retinal surgery and was found to have hypoxia and a loud murmur. Cardiac catheterization was performed in the general catheterization laboratory, demonstrating a membranous VSD, pulmonary atresia, and MAPCA. We highlight the challenges and limitations that an adult interventional cardiologist may have when encountering these patients.

Unusual Case of Common Arterial Trunk With Atresia of the Right Pulmonary Artery and Aortopulmonary Collaterals

2020 ◽  
Vol 11 (4) ◽  
pp. 534-536
Author(s):  
Deborah Kozik ◽  
Philip Dydynski ◽  
Erle Austin ◽  
Bahaaldin Alsoufi
Keyword(s):  
Pulmonary Artery ◽  
Unusual Case ◽  
Ascending Aorta ◽  
Intracardiac Repair ◽  
Common Arterial Trunk ◽  
Arterial Trunk ◽  
Right Pulmonary Artery ◽  
Aortopulmonary Collaterals ◽  
The Right ◽  
Artery Branch

We describe a two-year-old African girl with late diagnosis of unusual case of common arterial trunk with two separate pulmonary artery branch origins from the ascending aorta, hypoplastic right pulmonary artery that becomes atretic and reconstitutes at hilum, and three aortopulmonary collaterals providing right lung blood supply. She underwent single-stage intracardiac repair and unifocalization of collaterals.

Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals

2019 ◽  
Vol 29 (5) ◽  
pp. 610-614 ◽  
Author(s):  
Lisa Wise-Faberowski ◽  
Matthew Irvin ◽  
Douglas R. Sidell ◽  
Sheila Rajashekara ◽  
Ritu Asija ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Direct Laryngoscopy ◽  
Pulmonary Atresia ◽  
Chest Ct ◽  
Reconstruction Surgery ◽  
Aortopulmonary Collaterals ◽  
Pulmonary Artery Reconstruction ◽  
Artery Reconstruction ◽  
Major Aortopulmonary Collaterals ◽  
Airway Abnormalities

AbstractBackground:Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy.Methods:The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities – difficult anaesthetic airway, bronchoscopy, and/or CT findings – were defined.Results:Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3.Conclusion:This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.

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