CMR EVALUATION OF AORTO- PULMONARY COLLATERALS PRIOR TO FONTAN AND THEIR IMPACT ON EARLY OUTCOME

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XLIII CONGRESSO NAZIONALE SOCIETÀ ITALIANA DI CARDIOLOGIA PEDIATRICA Padova 16-19 Ottobre 2013 L.Ait-Ali, L. Arcieri, V. Pak, R. Moschetti, P. Festa. Istiituto di fisiologia clinica CNR Massa U.O. Cardiologia Pediatrica Fondazione Gabriele Monastero-CNR, Massa - CMR EVALUATION OF AORTO- PULMONARY COLLATERALS PRIOR TO FONTAN AND THEIR IMPACT ON EARLY OUTCOME

Background Aorto-pulmonary collaterals (APCs) are frequent in patients with uni-ventricular heart being found in nearly two thirds of patients after a bidirectional Glenn operation Triedman J e all. J Am Coll Cardiol 1993

Controversial management Background Banka P, et al. Practice variability and outcomes of coil embolization of aortopulmonary collaterals before Fontan completion: a report from the Pediatric Heart Network Fontan cross-sectional study. Am Heart J 2010;162:125e30

Background ControversiaL clinical significance of APCs Spicer et al graded SPC on pre-fontan x-ray angiography and found that higher angiographic SPC grade was associated with longer duration of pleural drainage after the Fontan operation Bradley et al quantified SPC flow intraoperatively by measuring the flow rate of pulmonary venous effluent while on cardiopulmonary bypass and found no association between this estimate of SPC flow and a number of postoperative outcomes

Background Lack of a reliable technique to quantify flow Spicer et al graded SPC on pre-fontan x-ray angiography and found that higher angiographic SPC grade was associated with longer duration of pleural drainage after the Fontan operation Bradley et al quantified SPC flow intraoperatively by measuring the flow rate of pulmonary venous effluent while on cardiopulmonary bypass and found no association between this estimate of SPC flow and a number of postoperative outcomes

Background Qantitative assessment of APCs blood by CMR

Aims To measure APCs flow volume (QAPCs) by CMR To evalute it s association with anatomical and hemodynamical data To evaluate their impact on early post-fontan outcomes

Popolation study 50 pts Univentricular heart CMR pre-fontan 2004-2013 2013 Inclusion Criteria SPC flow quantified at CMR prior to Fontan Fontan completion Population study: 25 patients age 5.5 ± 2.5 yrs

Protocol Surgical history Pulse oxymetry CMR Ventricular volumes and function Vascular Anatomy and Flow. Hemodynamic data (19/25 pz) Pulmonary pressure Ed ventricular function Mac Goon and Nakata index PVR Mayo clinici index Surgical data Fenestration (Yes/Not) BY pass time Fontan early outcome ICU and hospitalization duration Complication Pleurage drains

Protocol APCs flow volume (QAPCs) QAPCs= (QLPA- QLPVs) + ( QRPA- QRPVs)= 465 ml/min= 714 ml/min/m 2 QAPCs/QAO = 0,34

Population study Demographic data and surgical history N: 25 Age 5,5 ± 2.5 y.o. (3-13) Weight 20 ± 9 Kg (12-50) Ventricular type -Left UVH -Right UVH -Complex 2 ventricle Previous surgery -PAB -Systemic-pulmonary shunt -Atriospectetomy -Norwood -DK Stensel -Glenn 9 (36 %) 6 (24 %) 9 (36 %) 3 9 5 5 1 25 (100%)

Population study Echo and CMR data N: 25 Pulse O 2 sat 84 ± 4.5 % AV Valve regurgitation -Absent/trivial -Mild/moderate -Moderate/severe Ventricular volumes Ventricular EF Pulmonary artery diameters -Right pulmonary artery -Left pulmonary artery 13 (52 %) 11 (44 %) 1 (4%) 109 ± 39 ml/m 2 57 ± 7 % 11.5 ± 2.5 mm (7-17) 9 ± 3 mm (5-17)

Population study Pulmonary pressure Cardiac Cath data N: 19/25 11 ± 3 mmhg Ed ventricular pressure 8 ± 2.5 mmhg Nakata Index 179 (77-481) Mac Goon Index 1.8 ± 0.3 Pulmonary vascular resistances 1.9 ± 0.6 Mayo Clinic index 3.1 ± 0.8

Population study Surgical data and outcome N: 25 Age at Fontan 6 ± 2.5 y.o. Type of Fontan Fenestrated Non Fenestrated 12 (49 %) 13 (51%) By Pass time 106 ± 44 min Extubation 1 (1-7) p.o day ICU duration Hospitalistion duration Chest tube duration Complication Major pleural effusion (> 10 days) Low cardiac output Chilotorax Others 3 (2-9) days 20 ± 7 min 8 (2-14) days 11 (44%) 4(16 %) 5(16%) 4

Results

Results QAPCS: 1.2 ± 0.6 L/min/m2 QAPCS flow / aortic flow: 39% ± 20% (4.5-75%)

Results Distribution of QAPCS 0.62 ± 0.3 (L/min/m2) 0.59 ± 0.35 (L/min/m2) QAPCS to right QAPCS to left

Results Correlation QAPCS/AO with Pulm. Branches Flows Right Polm. Flow (ml/min) Left Polm. Flow (ml/min) R: -0.48, p= 0.01 R: -0.67, p< 0.001

Results QAPCS/AO P Ventricular type -Left UVH -Right UVH -Complex 2 ventricle 28 ± 13% 44 ± 22% 32 ± 16% 0.2

Results Correlation QAPcs/AO R P Age - 0.43 0.03 Pulse O 2 sat -0.45 0.02 Ventricular volumes Ventricular EF -0.38-0.05 0.06 0.8 Pulmonary pressure 0.3 0.2 Ed ventricular pressure 0.2 0.4 Nakata Index -0.03 0.9 Mac Goon Index 0.2 0.5 Mayo clinic -0.06 0.8 Pulmonary vascular resistances -0.1 0.7

Results Correlation QAPcs/Ao R P By Pass duration 0.22 0.2 Extubation 0.24 0.25 ICU duration -0.04 0.8 Hospitalization duration -0.02 0.9 Chest tube duration -0.22 0.3 Complication Prolugated pleural effusion (> 10 days) 0.03 ns 0.02 ns

Results Coralation QAPcs/Ao R P By Pass time 0.08 0.6 Extubation 0.05 0.9 ICU duration -0.04 0.8 Hospitalisation duration -0.1 0.5 Chest tube duration -0.1 0.9 QAPcs/Ao Complication Prolugated pleural effusion (> 10 days) P 0.02 ns

Conclusion QAPCs. inversly Correlate to pulmonary arteries flow Further studies could prospectively valuate the effect of APCs on immediate and late outcome after Fontan palliation and could help to assess the usefulness and effect of APCs embolization New CMR techniques allow reliable quantification of QAPCs.

Ospedale G.Pasquinucci Massa FTGM- IFC Pisa Fondazione Gabriele Monasterio CNR/Regione Toscana IFC CNR

Background Aorto-pulmonary collaterals (APCs) are frequent in patients with uni-ventricular heart (UVH).

Correlation RV EF/ LV EF Results R: 0.5, p= 0.01 LV EF (%) RV EF (%)

Correlation RV EF/ LV EF Results R: 0.4, p= 0.06-0,08 LV EF (%) RV EF (%)

Correlation RV EF/ LV EF Results R: 0.4, p= 0,05 LV EF (%) RV EF (%)

Correlation RV EF/ LV EF Results R: -0.4, p= 0,04 LV EF (%) RV EF (%)

Correlation RV EF/ LV EF Results R: 0.55, p= 0,02 LV EF (%) RV EF (%)

Correlation RV EF/ LV EF Results LV EF (%) RV EF (%)

CMR Protocol LPA Volume/min = 810 ml/min LPVs Volume/min = 960 ml/min SVC; Volume/min = 720ml/min RPA Volume/min = 227 ml/min IVC Volume/min = 909l/min RPVs Volume/min = 510 ml/min Aorta Volume/min = 2043 ml/mim QAPCs= (QLPA- QLPVs) + ( QRPA- QRPVs)= 465 ml/min= 714 ml/min/m 2 QAPCs/QAO = 0,34

Results Right QAPCS R: -0.4, p= 0.06 R: -0.5, p= 0.01

Results Left QAPCS R: -0.4, p= 0.06 R: -0.2, p= 0.1

Results Correlation QAPCS/Pulm. diameters R: -0.45, p= 0.04 R: -0.4, p= 0.04

Results Correlation QAPCS/ AO Pulm. Flows R: -0.3, p= 0.09 R: -0.48, p= 0.01

R: -0.4, p= 0.05 Results

Results Correlation QAPCS/AO with Pulm. Branches diameters Right Polm. Artery Ø (mm) Left Polm. Artery Ø (mm) R: -0.55, p= 0.01 R: -0.57, p= 0.003