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
Presenter Disclosure Information Matthew J. Gillespie, MD No Relevant Disclosures
Tools for Embolization (Particles) Coils Vascular plugs
Tools for Embolization: Coils
Tools for Embolization: AVP1
Tools for Embolization: AVP2
Tools for Embolization: AVP4
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
Pulmonary AVMs: primary case examples
Newborn Profound hypoxia Pulmonary AVMs: case 1
Newborn Profound hypoxia Pulmonary AVMs: case 1
Pulmonary AVMs: case 1 Newborn Profound hypoxia AVP1 pre release
Pulmonary AVMs: case 1 Newborn Profound hypoxia AVP1 POST release
Pulmonary AVMs: primary case 2
Newborn Profound hypoxia Pulmonary AVMs: case 2
Newborn Profound hypoxia Pulmonary AVMs: case 2
Pulmonary AVMs: case 2 Newborn Profound hypoxia AVP2 pre release
Pulmonary AVMs: case 2 Newborn Profound hypoxia AVP2 POST release
PAVMs in Palliated Single Ventricle Patients
PAVMs in Palliated Single Ventricle Patients spongiform ground glass nodular Rapid transit just plain ol ugly
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
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
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
Y Graft Fontan to promote hepatic flow to the LPA Prevention of PAVMs
Y Graft Fontan to promote hepatic flow to the LPA Prevention of PAVMs
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
Embolization Therapy
Embolization of Pulmonary AVMs Not A Definitive Therapy
Jack Rome MD Re-routing Hepatic venous flow
Jack Rome MD Re-routing Hepatic venous flow
Veno-venous decompression
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
Elevated CVP in great veins leads to venous decompression and reverse flow in existing systemic venous channels Veno-venous decompression
Veno-venous collaterals in palliated Single Ventricle Patients
Veno-venous collaterals in palliated Single Ventricle Patients
Veno-venous collaterals in palliated Single Ventricle Patients
Veno-venous collaterals in palliated Single Ventricle Patients
Veno-venous collaterals in palliated Single Ventricle Patients
Veno-venous collaterals in palliated Single Ventricle Patients
Veno-venous collaterals in palliated Single Ventricle Patients
Veno-venous collaterals in palliated Single Ventricle Patients: Case 2 systemic-to-systemic connection Reverse flow in accessory hemiazygos
Veno-venous collaterals in palliated Single Ventricle Patients: Case 2 AVP2 pre-release
Veno-venous collaterals in palliated Single Ventricle Patients: Case 2 Post AVP2 placement
Veno-venous collaterals in palliated Single Ventricle Patients: Case 3
Veno-venous collaterals in palliated Single Ventricle Patients: Case 3
Veno-venous collaterals in palliated Single Ventricle Patients: Case 3
Veno-venous collaterals in palliated Single Ventricle Patients: Case 3 = Systemic-to-pulmonary vein
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)
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