Embolization Therapy: Pulmonary AVMs; Veno-Venous Collaterals. Matthew J. Gillespie MD, FSCAI The Children s Hospital of Philadelphia
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1 Embolization Therapy: Pulmonary AVMs; Veno-Venous Collaterals Matthew J. Gillespie MD, FSCAI The Children s Hospital of Philadelphia SCAI Fellows Course 2014 December 8, 2014
2 Presenter Disclosure Information Matthew J. Gillespie, MD No Relevant Disclosures
3 Tools for Embolization (Particles) Coils Vascular plugs
4 Tools for Embolization: Coils
5 Tools for Embolization: AVP1
6 Tools for Embolization: AVP2
7 Tools for Embolization: AVP4
8 Pulmonary AVMs: Defined Primary: fistulous connection between PA and Pulmonary vein Osler-Weber-Rendu (HHT) Secondary: PAVMs in palliated single ventricle pts Especially Heterotaxy patients
9 Pulmonary AVMs: primary case examples
10 Newborn Profound hypoxia Pulmonary AVMs: case 1
11 Newborn Profound hypoxia Pulmonary AVMs: case 1
12 Pulmonary AVMs: case 1 Newborn Profound hypoxia AVP1 pre release
13 Pulmonary AVMs: case 1 Newborn Profound hypoxia AVP1 POST release
14 Pulmonary AVMs: primary case 2
15 Newborn Profound hypoxia Pulmonary AVMs: case 2
16 Newborn Profound hypoxia Pulmonary AVMs: case 2
17 Pulmonary AVMs: case 2 Newborn Profound hypoxia AVP2 pre release
18 Pulmonary AVMs: case 2 Newborn Profound hypoxia AVP2 POST release
19 PAVMs in Palliated Single Ventricle Patients
20 PAVMs in Palliated Single Ventricle Patients spongiform ground glass nodular Rapid transit just plain ol ugly
21 Pulmonary AVMs in Palliated Single Ventricle CHD Pulmonary AVMs are common in pts with heterotaxy and interrupted IVC after cavopulmonary connection Thought to be related in part to absence of unidentified hepatic factor in pulmonary blood supply After Bidirectional Glenn (Kawashima) Slide courtesy of Doff McElhinney After Fontan with Hepatic Venous Flow Streaming
22 Diagnosis of Pulmonary AVMs in Patients with Heterotaxy and Single Ventricle CHD Clinical Suspicion Contrast echo CT or MRI (large AVMs) Catheterization Pulmonary venous desaturation Rapid transpulmonary transit Spongiform appearance May be subtle or significant
23 Prevention of PAVMs At/after BDG/Kawashima Skip BDG and go straight to Fontan completion Leave additional source of pulmonary blood flow at the time of BDG (antegrade, BT shunt) Rapid staging from BDG to Fontan At/after total cavopulmonary connection (Fontan) Ensure bilateral distribution of hepatic venous blood Understand systemic venous anatomy - 1 or 2 SVCs - Lateral relationship of hepatic veins and SVC Various surgical strategies Slide courtesy of Doff McElhinney
24 Y Graft Fontan to promote hepatic flow to the LPA Prevention of PAVMs
25 Y Graft Fontan to promote hepatic flow to the LPA Prevention of PAVMs
26 Treatment of PAVMs Once They Are Present (Embolize) Provide hepatic venous flow to the affected lung (Surgical OR Catheter based) - Hepatic vein inclusion - Revise the Fontan connection - Brachial AV fistula Lobectomy/pneumonectomy Slide courtesy of Doff McElhinney
27 Embolization Therapy
28 Embolization of Pulmonary AVMs Not A Definitive Therapy
29 Jack Rome MD Re-routing Hepatic venous flow
30 Jack Rome MD Re-routing Hepatic venous flow
31
32 Veno-venous decompression
33 Veno-venous collaterals in palliated Single Ventricle Patients Two Basic Varieties 1. Systemic-to-systemic connections BDG patients decompressing to IVC 2. Systemic-to-Pulmonary vein connections BDG and Fontan Pts
34 Elevated CVP in great veins leads to venous decompression and reverse flow in existing systemic venous channels Veno-venous decompression
35 Veno-venous collaterals in palliated Single Ventricle Patients
36 Veno-venous collaterals in palliated Single Ventricle Patients
37 Veno-venous collaterals in palliated Single Ventricle Patients
38 Veno-venous collaterals in palliated Single Ventricle Patients
39 Veno-venous collaterals in palliated Single Ventricle Patients
40 Veno-venous collaterals in palliated Single Ventricle Patients
41 Veno-venous collaterals in palliated Single Ventricle Patients
42 Veno-venous collaterals in palliated Single Ventricle Patients: Case 2 systemic-to-systemic connection Reverse flow in accessory hemiazygos
43 Veno-venous collaterals in palliated Single Ventricle Patients: Case 2 AVP2 pre-release
44 Veno-venous collaterals in palliated Single Ventricle Patients: Case 2 Post AVP2 placement
45 Veno-venous collaterals in palliated Single Ventricle Patients: Case 3
46 Veno-venous collaterals in palliated Single Ventricle Patients: Case 3
47 Veno-venous collaterals in palliated Single Ventricle Patients: Case 3
48 Veno-venous collaterals in palliated Single Ventricle Patients: Case 3 = Systemic-to-pulmonary vein
49 Summary Pulmonary AVMs May present as large fistulous connection in newborn period (rare) Associated with Osler-Weber-Rendu (HHT) In CHD: Most often seen in palliated single ventricle patients ESPECIALLY HETEROTAXY pts Prevention would be ideal Treatment is difficult Embolization is not a permanent fix Catheter-based rerouting shows hope Veno-venous collaterals in palliated single Ventricle patients Systemic-to-systemic (BDG pts) Systemic-to-pulmonary (BDG and Fontan)
50 Thank You
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