Manu Varma DO Pediatric Cardiology Fellow University of Texas Health Science Center at Houston Houston TX Disclosures None Ebstein Anomaly Congenital anomaly of the tricuspid valve and right ventricle due to incomplete delamination of tricuspid valve leaflets ID: 910932
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Slide1
Surgical Shunts Causing Circular Shunt: An Approach to Management
Manu Varma, DO
Pediatric Cardiology Fellow
University of Texas Health Science Center at Houston; Houston, TX
Slide2Disclosures
None
Slide3Ebstein Anomaly
Congenital anomaly of the tricuspid valve and right ventricle due to incomplete delamination of tricuspid valve leaflets
Apical displacement of tricuspid valve, leads to decreased size of functional right ventricle
Right ventricular outflow and degree of pulmonary blood flow is variable and unpredictable
O’Leary
,
PW.
E
bstein’s
malformation and tricuspid valve diseases. in:
E
idem
BW,
C
etta
F,
O’Leary PW,
eds.
Echocardiography in Pediatric and Adult
Congenital
Heart Disease.
Philadelphia,
PA:
L
ippincott
W
illiams
& W
ilkins
; 2009:116-130
Slide4Patient History
Infant with
Ebstein
anomaly and pulmonary atresia
Initial surgical palliation with 8 mm
valved femoral vein RV-PA conduit and 3 mm central shunt (added due to hypoxia coming off bypass)Admitted at 3 months of age with increased work of breathing and cyanosis. Central shunt upsized to 3.5 mm
Condition worsened postoperatively with hypoxia and increasing inotrope requirementsEchocardiogram showed free pulmonary insufficiency through conduit and tricuspid regurgitation
Slide5Initial Hemodynamics
Obtained on 100% FiO2 and
iNO
20 ppm due to instability
Venous saturations: LINNV 12.5%, SVC 15%, IVC 38%
Arterial saturations: LV 67%, aAO 63%, dAO 75.2%PVR 6.05 WU/m2
with mPAP 23 mmHg
Slide6Initial Angiogram
Injection into central shunt
Significant reflux of contrast from pulmonary arteries to right ventricle
Ongoing contrast movement in the heart for several beats with no forward flow
Slide7Test Occlusion
Amplatzer
Vascular Plug (AVP) II 10 mm x 7 mm placed in RV-PA conduit, providing complete occlusion of 8 mm conduit.
Test injection with device in place demonstrated stable position.
Slide8Device Release
Device deployed in stable position.
dAO
saturation 92% (increased from 75.2% pre-intervention)
Slide9Patient Outcome
Patient’s condition improved in ICU with inotropes stopped and supplemental oxygen weaned.
Post-
cath
day 11: Repeat catheterization for pre-Glenn assessment showed PVR 0.62 WU/m
2 with mPAP 13 mmHg on room air. Post-cath
day 13: Bidirectional Glenn with RV-PA conduit replacement, central shunt takedown, and tricuspid valve replacement.
Slide10Discussion and Lessons
Pulmonary blood flow in
Ebstein’s
anomaly is unpredictable, both before and after surgical palliation
“Circular shunt” physiology can be a cause of hypoxia as a late complication after multiple surgical shunts are placed
Device occlusion of RV-PA conduit allowed resolution of critical illness from circular shunt and stabilization for surgical repair
Slide11Contact Information
Manu.R.Varma@uth.tmc.edu