Glenn and Fontan Caths:

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Glenn and Fontan Caths: Pre-operative evaluation and Trouble-shooting Cavo-Pulmonary Shunts Daniel H. Gruenstein, M.D. Director, Pediatric Interventional Cardiology University of Minnesota Children s Hospital, Fairview Asst. Professor, Pediatrics University of Minnesota Medical School

Objectives Pre-operative catheterization What information do we need to obtain? What can catheterization provide that non-invasive imaging cannot? (Do we need pre-operative caths?) Case studies/discussion: PA problems Residual/new shunts causing desaturation Collaterals (AP, veno-venous/atrial) These are secondary effects of another problem!!!

Objectives Pre-operative catheterization still needed? What catheterization can provide that noninvasive imaging cannot

Many Anatomical Variations One General Principle General Principle systemic venous return circumventing the heart requires passive flow through the pulmonary circulation Low resistance no pulmonary arterial obstruction Low pulmonary arteriolar resistance Low atrial pressure (AV valve stenosis/regurgitation) Low systemic ventricular diastolic pressures

Theoretical, or actual risk? Pre-operative factors associated with worse outcomes: PA obstruction/distortion Elevated arteriolar resistance (>2 Woods x m 2 ) Elevated mean PA pressure (> 15 mmhg) AV valve abnormalities (L AV valve atresia, common AV valve) <3 years old Factors associated with improved survival after modified Fontan operations. Mayer, JE, et al J Am Coll Cardiol 17:33A, 1991

Theoretical, or actual risk? Pre-operative factors associated with worse outcomes: PA obstruction/distortion better with angio? Elevated arteriolar resistance (>2 Woods x m 2 ) Elevated mean PA pressure (> 15 mmhg) AV valve abnormalities (L AV valve atresia, common AV valve) <3 years old Factors associated with improved survival after modified Fontan operations. Mayer, JE, et al J Am Coll Cardiol 17:33A, 1991

Hemodynamics PAp and Rp Need direct access to PA Across AP shunt risk of acute occlusion, thrombosis, inaccurate measurements Into Glenn via IJ or subclavian risk of pneumothorax/hemothorax, thrombosis Can we avoid these risks? What if there is no direct access? Diagnostic cases have risks!!!

Pressure Wire

Problem - Cannot Directly Access PAs Pulmonary Venous Wedge Pressure Provides an Accurate Assessment of Pulmonary Artery Pressure in Children with a Bidirectional Glenn Shunt Gruenstein, DH, Beekman RH - Journal of Interventional Cardiology Volume 16, Issue 5, pages 367-370, 20 OCT 2003 DOI: 10.1046/j.1540-8183.2003.01010.x http://onlinelibrary.wiley.com/doi/10.1046/j.1540-8183.2003.01010.x/full#f1

Can non-invasive studies replace diagnostic caths for single-v palliation?

Cardiac Magnetic Resonance Versus Routine Cardiac Catheterization Before Bidirectional Glenn Anastomosis in Infants With Functional Single Ventricle A Prospective Randomized Trial Brown, DW, et al DOI: 10.1161/CIRCULATIONAHA.107.723213 prospective, randomized, single-center clinical trial comparing catheterization with CMR in patients considered for bidirectional Glenn End points - adverse events of the preoperative evaluation and a composite score of clinically successful surgery. 82 enrolled Catheterization resulted in more minor adverse events (78% versus 5%; p<0.001), longer preoperative hospital stays (median, 2 versus 1 day; p<0.001), and higher hospital charges ($34 477 versus $14 921; p<0.001). There was 1 major adverse event in the CMR group. The operative course and frequency of postoperative complications were similar between the 2 groups. The proportion of patients who had a successful bidirectional Glenn operation was similar (71% versus 83%; p=0.3). At the 3-month follow-up - no differences in clinical status, oxygen saturation, or frequency of reinterventions.

Role for Pre-cavopulmonary anastomosis catheterization Perhaps a decreasing role for pre-operative hemodynamics, as anatomy (PA and venous) is well assessed by CT/MR CT/MR may give indication of need for catheterization PA obstruction, collaterals Increased role for pre-operative interventions

Role for Catheterization Beyond pre-operative assessment Intraoperative/Exit Angiography

Exit Angiography

Exit Angiography

Exit Angiography

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

AC s/p Glenn with desaturations

PA stenoses Be Appropriately Aggressive! Correct them EARLY!!!

Typical Paradigm Diagnostic Cath Interventional Cath

Single-V Paradigm Diagnostic Cath Interventional Cath

Connor HLHS variant (DORV/mitral atresia/coa) s/p CoA repair/pa band (DOL #5) s/p DKS/BDG (6 months)

Connor Presented to ED 3 months after BDG Tachypnea Cough Facial Swelling

A pleural effusion is a sign of another problem

LPA stenosis

Presenting issues that should prompt a search for an underlying problem Pleural effusions contralateral PA obstruction Aorto-pulmonary collaterals ipsilateral PA obstruction Veno-venous/atrial collaterals cavo-pulmonary anastomosis and/or PAs

AJ Heterotaxy, TAPVR, bilateral SVCs

AJ Heterotaxy, TAPVR, bilateral SVCs

AJ Heterotaxy, TAPVR, bilateral SVCs

AJ 10-7-2009

AJ 10-27-2009 After anticoagulation

AJ 10-27-2009 after anticoagulation

AJ 3-2010 s/p Fontan revision

AJ 3-2010 s/p Fontan revision

AJ 3-2010 s/p Fontan revision

AJ 3-2010 s/p Fontan revision

AJ 3-2010 s/p Fontan revision

AJ 3-2010 s/p Fontan revision

AJ desaturated 9-2011

AJ desaturated 9-2011

AJ hepatic ligation exit angio 12-2011

Take-Home Messages Cavo-pulmonary shunts require low-resistance, unobstructed pulmonary circulations At the earliest evidence of PA obstruction ACT!!! Be appropriately aggressive

Don t just stand there Do something!!! Where there s smoke there s fire!

Take-Home Messages Collaterals and effusions are the smoke it means there is a fire (PA obstruction, high PVR, AV valve regurgitation, etc)

Take-Home Messages Therefore, you have to find the fire (underlying cause) and put it out, or the smoke just keeps coming back Don t get distracted find the cause and treat it!

Take-Home Messages In the end, smoke kills just as well as fire treat the cause and the collaterals, before a feedback loop starts Interventionalists need to take a cue from the tobacco industry go after them early!!!