Tricuspid Atresia Work Weekend Nov. 0
Work Weekend Objectives Define Current Cohort Present/Evaluate Analysis Finalize Analysis Topic
Original Goals Describe the impact of patient characteristics and management strategies on outcomes of Fontan track
Enrollment Criteria Diagnosis of TA with normally related great arteries Age < months at diagnosis Admitted to CHSS institution after //99 Excludes AV or VA Discordance, first procedure at non CHSS institution
Previous CHSS Analysis Competing risks analysis (005, N=50) Factors for death before : MR, BTS not originating from innominate Factors for reduced transition to : younger age at admission, non-cardiac anomalies, larger BTS diameter Survival by initial operation among patients with unrestricted blood flow Objective: define late outcomes
Demographics N=0 0 with index procedure 7 deaths overall (%) death prior to intervention
Index Procedure Total N=0 SP Shunt N=89 Alive= 85% PAB N=50 Alive= 9% N=6 Alive= 95%
Initial Physiology Total N=0 Alive=66 (88%) PV Atresia N=5 Alive=44 (8%) Restricted PBF N=54 Alive=7 (89%) Unrestricted PBF N=96 Alive=85 (89%) (Type-a) (Type-b) (Type-c)
All Patients 0 86 4 8 4 DIAPH 7 PDA 4 DIAPH 4 98 5 5 PACE 5 5 6 4 6 PBF 5 0 5 5 PBF PAB 8 48 PAB 4 PACE PACE x x6 x 6 PAB 50 4 PAB 0 8 PBF 4 PACE PACE PACE PACE PAB HTX = Systemic-pulmonary shunt = Alive, no further surgery = sternal exploration/bleeding proc PBF= other procedure to adjust PBF DIAPH= diaphragm plication PBF
% Survival Overall Survival From Birth N=0 Dead = 7 Non-parametric Parametric Years after Birth
Hazard Hazard For Death: From Birth N=0 N at risk y= 40 4y= 06 6y= 79 Years after Birth
Mortality by Stage 7 total deaths - denominator=0(%) Prior to surgery following - stage 5 following - stage 8 following Fontan- stage Definitions for analysis: -Stage = initial PAB or procedure -Stage = initial -Stage = initial Fontan
PV Atresia N=5 5 4 5 6 PBF PDA 9 4 5 PACE = Systemic-pulmonary shunt = Alive, no further surgery = sternal exploration/bleeding proc PBF= other procedure to adjust PBF DIAPH= diaphragm plication
Restricted PBF N=54 PBF 9 4 9 6 PBF PAB 5 5 DIAPH 7 4 PACE 8 64 PACE PACE = Systemic-pulmonary shunt = Alive, no further surgery = sternal exploration/bleeding proc PBF= other procedure to adjust PBF DIAPH= diaphragm plication
Unrestricted PBF N=96 DIAPH 4 4 7 5 PACE PAB x x6 x 6 9 PACE PBF PAB 50 4 0 8 4 PACE PACE PAB PACE PACE PAB HTX PBF = Systemic-pulmonary shunt = Alive, no further surgery = sternal exploration/bleeding proc PBF= other procedure to adjust PBF DIAPH= diaphragm plication
. Competing Risk Analysis Transition to Fontan vs. Death
Research Question How does timing of affect the successful transition to Fontan or death before Fontan? What procedural, morphologic, and demographic factors are associated with successful transition to Fontan?
% In each Group Competing Risks: Transition to Fontan from Birth N=0 Alive w/o Fontan (N=5) Died before Fontan (N=9) Survived to Fontan (N=5) yr: 44% alive w/out Fontan 9% died w/out Fontan 46% survived to Fontan 5 yr: % alive w/out Fontan 0% died w/out Fontan 79% survived to Fontan Years after Birth
% Free from Fontan Achievement of Fontan: From Birth N=0 Achievement of Fontan: N= Late phase: 0 events >6 years Years after Birth
% Free from Death Death Before Fontan: From Birth N=0 Death before Fontan=9 At risk: y N=69 y N = 9 5 y N=4 Years after Birth
Age at Operation Median Age At (N=77).54 +/-.5y (. - 6.7) Median Age At Fontan (N=).8 +/-.y (.9-8.5) 6 Children alive > 5yrs w/out Fontan (47, 7, 77, 98, 0, 0)
% In each Group Competing Risks: Completion of from Birth N=0 Alive w/o (N=) Died before ( N=4) Survived to (N=77) At 6 mo: 56% alive w/out intervention 4% survived to % died w/out At year: 8% alive w/out intervention 90% survived to 4% died before Years after Birth
% In each Group Competing Risks: Transition to Fontan from N=77 Alive w/o Fontan (N=40) Died before Fontan(N=5) Survived to Fontan(4) At yr: 6% alive w/out Fontan after 5% died w/out Fontan after % survived to Fontan after At 4 yr: 0% alive w/out Fontan after 5% died w/out Fontan after 85% survived to Fontan after Years after
Time Related Competing Risks Time Zero DOB Model Outcomes Achievement of Fontan Death Before Fontan Time varying co-variables Age at operation Surgical procedures and associated variables Morphology based on baseline echo and echo prior to
Univariate Analysis Results Increased Age at resulted in increased transition to Fontan when unadjusted P=.046 Increased shunt size resulted in increased risk for death when unadjusted P=.06
Multivariate Analysis Results Transition to Fontan BCPA as procedure increased transition to Fontan P<.00 Larger RPA diameter on Echo prior to decreased transition P=.0 Survival Having a BTS as stage procedure increased risk of death P=.0
Summary Timing of does not appear to have a significant affect on mortality before Fontan, or transition to Fontan As previously shown, patients who have a BTS as a first procedure have an increased risk of death.
. Functional result of Fontan Repeated measures of LV function and AV-valve function
Grade of LV dysfunction post-fontan 559 echos on 70 patients Severe Moderate Mild Normal 0 4 6 8 0 Years post-fontan
Grade of LV dysfunction post-fontan 559 echos on 70 patients Severe Moderate 0 patients Mild Normal 0 4 6 8 0 Years post-fontan
Grade of AV-valve regurgitation post-fontan 505 echos on 60 patients Moderate Mild Trivial Normal 0 4 6 8 0 Years post-fontan
Grade of AV-valve regurgitation post-fontan 505 echos on 60 patients Moderate 5 patients Mild Trivial Normal 0 4 6 8 0 Years post-fontan
. Practical question When doing a mbts for tricuspid atresia, is it better to leave the native mpa open?
Sub-analysis: All patients with type Ib or Ic (patent RVOT) who required a mbts N = 5 Univariate analysis of mpa ligation: Overall survival: P = 0.45 Transition to Fontan: P =.85