Abstract 4567: Functional Intraoperative Pulmonary Blood Flow Study is a Sensitive Predictor of Right Ventricular Pressure and Successful VSD Closure for Following Complete Uniforcalization in Patients with Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Osami Honjo ◽  
Osman O Al-Radi ◽  
Cathy MacDonald ◽  
Lisa Davey ◽  
Christopher A Caldarone ◽  
...  

OBJECTIVE: We hypothesized mean pulmonary artery (PA) pressure obtained from an intraoperative pulmonary flow study would better predict the ability to close the ventricular septal defect (VSD) and better predict postoperative right ventricular systolic pressure (RSVP) than classic anatomical parameters after complete uniforcalization in patients with pulmonary atresia, VSD, and major aortopulmonary collaterals. METHODS: Sixteen consecutive patients (median, 11 mo, range 2 mo – 16 yrs) underwent one-stage (75%) or staged (25%) uniforcalization between 1/03 and 8/07. Intraoperative functional pulmonary blood flow study was achieved by inserting an arterial cannula in a reconstructed central PA. Flow was increased to 2.5 L/min/m2 while measuring PA pressure. RVSP and systemic systolic pressure (SBP) were recorded after VSD closure. Total neopulmonary artery index (TNPAI) (MAPCA + native PA index), total incorporated pulmonary vascular segments, and pulmonary segment artery ratio (PSAR) (ratio of incorporated segment to 18) were analyzed. Spearman rank correlation and area under the receiver operator characteristics curve (ROC-AUC) were used. RESULTS: The mean PA pressure on flow study was 21.8+/−6.2 mmHg (range, 11–31). Three patients had a pressure of > 30 mmHg. The VSD was closed in 14 (87%). One patient with flow study mean PA pressure of 25 mmHg had suprasystemic RVSP and underwent intraoperative VSD fenestration. One with a flow study mean PA pressure of 30 mmHg had a prospective fenestrated VSD patch placed. There is a weak negative correlation between TNPAI and the flow study PA pressure (rho=−0.4, p=0.12). The flow study mean PA pressure was correlated with post-repair RVSP (rho=0.72, p=0.0027), and with RVSP/SBP ratio (rho=0.67, p=0.0063). TNPAI, total incorporated segments, and PSAR were not correlated with the postoperative RVSP or RVSP/SBP ratio. Flow study mean PA pressure had the highest sensitivity in predicting VSD closure: ROC-AUC (0.82) vs. TNPAI (0.46), pulmonary segment (0.64), and PSAR (0.64). CONCLUSIONS: Intraoperative pulmonary flow study predicted the ability of VSD closure better than total incorporated segments, TNPAI, and PSAR. Flow study mean PA pressure highly correlated with postoperative RVSP and RVSP/SBP ratio.

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Katia Bravo-Jaimes ◽  
Brian Walton ◽  
Poyee Tung ◽  
Richard W. Smalling

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.


2018 ◽  
Vol 28 (9) ◽  
pp. 1091-1098 ◽  
Author(s):  
Sophie C. Hofferberth ◽  
Jesse J. Esch ◽  
David Zurakowski ◽  
Christopher W. Baird ◽  
John E. Mayer ◽  
...  

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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