Jiaquan Zhu, Atsuko Kato, Arezou Saedi, Devin Chetan, Rachel Parker, Christopher A. Caldarone, Glen S. Van Arsdell, Osami Honjo

Transcription

1 Pulmonary Flow Study Predicts Medium-term Survival in Patients Undergoing Repair of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries Jiaquan Zhu, Atsuko Kato, Arezou Saedi, Devin Chetan, Rachel Parker, Christopher A. Caldarone, Glen S. Van Arsdell, Osami Honjo Division of Cardiovascular Surgery Labatt Family Heart Centre, The Hospital for Sick Children

2 No disclosure 2

3 Introduction Decision for VSD closure in patients with borderline pulmonary artery (PA) and MAPCAs anatomy poses significant challenges VSD closure in high PVR patients High post-operative RV pressure VSD left open in low PVR patients Pulmonary over circulation Disadvantages of using an anatomic index, eg. Total neo PA index (TNPAI) Stenosis or kinking of the unifocalized MAPCAs Segmental pulmonary vascular resistance 3

4 Physiologic index-- pulmonary flow study Reddy et al. JTCVS, 1997; 113:

5 Pulmonary flow study predicts VSD closure and intraoperative RV pressure Predictability of VSD closure Area under ROC curve PAP by the flow study 0.83 TNPAI 0.42 Total pulmonary segments Pulmonary segment artery ratio However, the relationship among the mean PAP on the pulmonary flow study, status of VSD, and medium-term clinical outcomes are largely unknown. Honjo et al. Circulation, 2009; 120:S46-S52 5

6 Hypothesis Intraoperative pulmonary flow study may predict medium-term clinical outcome after the unifocalization operation 6

7 Patients and Methods 50 consecutive patients (2000 to 2013 ) Surgical strategy Prefer 1-stage unifocalization Staged operation was applied Diminutive native branch pulmonary artery (PA) Distal MAPCA stenosis is president Intraoperative pulmonary flow study (n=40 since 2003) Incrementally increased from 1 to 2.5 L/min/m 2 Cut off mean PA pressure (PAP) to close the VSD was 30 mmhg 7

8 Demographics Characters Number Median age at complete unifocalization (years) 0.88 (rang 0.05~16.2) Median weight at complete unifocalization (kg) 7.7 (range 2.7~64.9) Native pulmonary artery (NPA) anatomy Confluent 39 (78%) Non-confluent 3 (6%) Absent 8 (16%) Total number of MAPCAs 182 Unifocalized 143 (78.6%) Ligated 33 (18.1%) Waiting for unifocalization 2 (1.1%) Not unifocalized 4 (2.2%) 8

9 Flowchart of surgical strategy One Stage (60%) Stage repair (40%) (94%) 9 The number in the bracket represents number of death

10 Distribution of the mean PAP and TNPAI stratified by VSD status (N=38) 10

11 Both mean PAP and TNPAI predicted the VSD closure ROC curve analysis 11

12 Survival stratified by VSD status Overall survival Stratified by VSD status 12

13 Survival stratified by mean PAP Mean PAP, cut off=25 Mean PAP, cut off=25 and 30 13

14 Survival in patients with VSD closed (n=30) stratified by mean PAP mpap < 25 mmhg mpap >= 25 mmhg Log-Rank test: P=

15 Causes of death (n=9) No. Unifocalization strategy Age at complete unifocalization (Years) VSD status Mean PAP of flow study (mmhg) RVSP/LVSP during operation Interval after complete Unifocalization Cause of death 1 Staged 0.6 Closed m Airway 2 1-stage 0.5 Open (fen) 25 >1 1 m Airway 3 1-stage 0.6 Open (fen) n/a >1 0.9 yr Cath-related 4 1-stage 0.4 Open 31 n/a 6.8 yr Cath-related 5 1-stage 0.6 Closed yr Cath-related 6 1-stage 0.9 Closed yr Heart 7 Staged 1 Closed yr Heart 8 1-stage 0.7 Open(fen) n/a >1 2 m Sudden death 9 Staged 1.5 Open n/a n/a 6 m Other disease 15

16 Death plotted by mean PAP and TNPAI 16

17 Reoperation and reintervention However, mean PAP from the pulmonary flow study is not associated with the reoperation and reintervention 17

18 Higher follow-up RVSP/LVSP associated with late mortality (n=17) 18

19 Post-operative RV function Moderate 5% Severe 5% Moderate/severe RV dysfunction mpap < 25 mmhg Mild 45% Normal 45% mpap >= 25 mmhg Log-Rank test: P=0.546 Follow-up RV dysfunction (n=40)

20 Univariate Cox regression Risk factors of survival P value Hazard ratio (95% CI) Mean PAP of pulmonary flow study ( ) Mean PAP > 25 mmhg ( ) TNPAI (cut off =150 mm 2 /m 2 ) Unifocalization strategy Native PA anatomy Age Body weight

21 Conclusions The pulmonary flow study predicted not only VSD closure but also medium-term survival Mean PAP 25 mmhg is considered high risk. Sensible judgment and low threshold for VSD fenestration are required 21