scholarly journals Pulmonary artery atresia with ventricular septal defect, segmental pulmonary hypertension, major aortopulmonary collaterals (MAPCAs) and giant MAPCA aneurysm

2016 ◽  
Vol 3 ◽  
pp. 280-281
Author(s):  
Marek Grabka ◽  
Jacek Kusa ◽  
Błażej Kusz ◽  
Katarzyna Mizia-Stec
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Aortopulmonary Collaterals ◽  
Major Aortopulmonary Collaterals

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.

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.

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