PA/VSD/MAPCAs What a surgeon needs to know
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1 PA/VSD/MAPCAs What a surgeon needs to know Patrick McConnell, MD Nationwide Children s Hospital....
2 Name MRN Procedure Allergies Absolute Surgical Requirements Things needed to complete a Time out....
3 Overview 1. MAPCAs paradigm? Single stage unifocalization vs. central PA growth Clinical status/intra-cardiac anatomy Age, sats, circulation (over/under), genetics 2. Pulmonary arterial anatomy/hemodynamics Intrapericardial & intrapulmonary characteristics 3. Systemic arterial anatomy/hemodynamics Arch sidedness, MAPCA locations **dual supplied segments 4. Mediastinal/pulmonary anatomy Airway, esophagus, veins, adenopathy, lung status....
4 MAPCAs paradigm... When in Rome (Spain?)....
5 Paradigm Single Stage Unifocalization Incorporate as many segments into a central PA compartment. Unifocalization to the heart/conduit Intra-cardiac repair VSD closure +/- fenestration 3-9 months of age Average is 7-9 months for most series Multi-stage or central PA growth focused Early cath (< 1mos) to delineate central PAs and MAPCAs Surgery (< 3 mos): achieve central PA blood flow RV-PA conduit Central shunt No intracardiac repair vs. fenestrated VSD Later catheterizations to control dual supply MAPCAs and PA growth Surgery (< 2 yo): unifocalize & intra-cardiac repair
6 Surgery & Central PA growth MAPCAs and surgically manipulated central PAs fail to grow Archives of Cardiovascular Diseases, Volume 105, Issue 12, 2012, The Annals of Thoracic Surgery, Volume 101, Issue 3, 2016, The Annals of Thoracic Surgery, Volume 97, Issue 6, 2014,
7 Central PAs Central PA Present Branch PA sizes Nakata index Define dual-supply MAPCAs Not present MAPCA circulation/pulmonary circulation Confirm by MDCT 5 yo male, unrepaired PA/VSD/MAPCAs. Sats 72%; Nakata = 155 mm 2 /m 2
8 Surgery for Cong Heart Defects. 3rd Edition. Tsang V. Ch 30. Figure MAPCAs
9 Retro-Esophageal MAPCAs REMs (Stanford ) 68 patients 45 (67%) with REM 84% stenotic LAA RAA 80% Intra and 32% extra 77% REM 32% Intra 53% REM 72% Intra The Annals of Thoracic Surgery, Volume 102, Issue 3, 2016,
10 REM and Stenosis The Annals of Thoracic Surgery, Volume 102, Issue 3, 2016,
11 Dual Supply/Over-circulation Systemic saturations Lower (< 85%) Plan to keep all nonunifocalized segments Later coil/embolize Higher Ligate non (<50%) stenotic collaterals Number and location of single-source segments If over-circulated pre-op very likely there are adequate central PAs and early repair is indicated.
12 Value of Advanced Imaging? PMC full text: PLoS One. 2016; 11(1): e Complete Preoperative Published online Evaluation 2016 Jan 7. doi: of /journal.pone Pulmonary Atresia with Ventricular Septal Defect with Multi-Detector Computed Tomography. Copyright/License Request permission to reuse PLoS One. 2016; 11(1). Table 1 Findings at MD CT, TTE, and Cardiac Catheterization Compared with Findings at surgery. Parameter No. of Surgical Findings Test Parameters Technique True- False- True- False- Sensitivity Specificity PPV NPV Accuracy Positive Negative Negative Positive Main PA MDCT % 100% 100% 100% 100% Catheterization % 98.8% 96.8% 96.5% 96.6% TTE % 96.4% 89.7% 92.0% 91.4% Right/ Left MDCT % 100% 100% 100% 100% PA Catheterization % 100% 100% 86.0% 94.8% TTE % 94.6% 96.6% 60.3% 78.4% PA MDCT % 100% 100% 100% 100% confluence Catheterization % 100% 100% 92.3% 96.6% TTE % 89.6% 91.4% 74.1% 82.8% PA stenosis MDCT % 99.0% 94.7% 100% 99.1% Catheterization % 99.0% 100% 97.0% 96.6% TTE % 95.9% 73.3% 84.7% 90.5% Overall MDCT % 99.7% 99.6% 100% 99.8% native PA Catheterization % 99.3% 99.2% 93.2% 95.9% TTE % 94.7% 92.7% 79.3% 85.9% MAPCA MDCT % 98.7% 97.0% 99.1% 98.5% stenosis Catheterization % 97.4% 93.4% 94.4% 94.2% TTE NA NA NA NA NA NA NA NA NA PDA MDCT % 100% 100% 100% 100% Catheterization % 100% 100% 100% 100% TTE % 100% 100% 100% 100%
13 Imaging: What is Helpful?
14 Traditional Outcome....
15 Less > More 4 mos - preop 5 mos intra-op
16 Less > More....
17 Less > More 1 year post-op
18 Less > More
19 Summary Clinical status and the status of the central PAs should dictate palliative strategy. MAPCAs need to be defined (+/- stenoses) Dual-supply Single source Mediastinal structures involved Diminutive central PAs have growth potential Data/experience demonstrates that surgically manipulated PAs and MAPCAs do not grow. Avoid surgical and stent based interventions in early PA rehab Focus interventions controlling dual sources overtime. 3-D rotational angiography would seem less informative than selective MAPCA angios to define location, dual-supply and stenoses and advanced imaging may be value added.....
20 Thank you Pre-op sats = 72% Post-op sats = 93%
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