Anomalous Aortic Origin of a Coronary Artery (AAOCA) Fall Work Weekend Nov. 2013

Transcription

1 Anomalous Aortic Origin of a Coronary Artery (AAOCA) Fall Work Weekend Nov. 2013

2 Work Weekend Objectives Update cohort status Present Analysis Develop 12 mo. analysis plan - maximize cohort potential Discuss/finalize best practice paper

3 Registry Establishment Includes patients 30 years of age with admission to a CHSS institution after = retrospective enrollment Excludes patients with other hemodynamiclly significant cardiac lesions

4 Registry Objectives Describe spectrum of morphology Identify morphologic risk factors associated with increased risk of SCD Define natural & unnatural history during long term follow up Develop evidence-based management strategies

5 Demographics N=243 at analysis (current enrollment ~300) Mean age at diagnosis= 9.6 +/- 5.9 y (0-29.3) Mean age at operation=11.7+/-4.3 y ( ) Median age at f/u /- 5.9y ( ) 8 Deaths (3%) N=243 Surgery N=139 (57%) Medical N=104 (43%)

6 Causes of Death study Anomaly Cause of Death AA001 M ARCA Genetic Arthrogryphosis AA002 M ARCA 23 preterm, broncopulmonary dysplasia AA007 M ARCA Complications 2/2 to neuroblastoma AA015 F ALCA 6y/o, sudden collapse-taken to OR AA028 F ARCA AA048 M ALCA Complications 2/2 to cardiomyopathy AA084 F ALCA Post op complication-bleeding AA208 M ALCA 14 y/o with SCD-playing basketball 8 total - 2 surgical - 6 medical

7 % Survival KM Survival: Surgical vs. Medical N=243 Surgical Died = 2 (1%) Surgical N=139 Medical N=104 Medical Died = 6 (2%) Time From Initial Diagnosis

8 Frequently Observed Surgical ARCA IA+IM Course Anomalies N=87 (63%) ALCA IA+IM Course N=29 (21%) ARCA IA Course N=4 (3%) ALCA IA Course N=3 (2%)

9 Frequently observed Expert ARCA IA+IM Course Review Anomalies N=95 (65%) ALCA IA+IM Course N=25 (17%) ARCA IA Course N=9 (6%) ALCA IA Course N=2 (1%)

10 Spectrum of Morphology: Surgical Reports SURGICAL N=139 AAOCA+origin N ARCA=98 Left sinus 94 Suprasinsu 4 ALCA=37 R.sinus 35 Suprasinus 1 NCS 1 LAD 1 Both=3 3 TOTAL 139 Both= single AAOCA from above Sinus of Valsavla IA=Interarterial IM=Intramural IC=Intraconal N/A=not recorded or missing

11 Spectrum of Morphology: Surgical Reports SURGICAL N=139 Course AAOCA+origin N IA+IM IM IA IC IA+IM+IC IA+IC ARCA=98 Left sinus Suprasinsu ALCA=37 R.sinus Suprasinus NCS LAD Both= TOTAL

12 Spectrum of Morphology: Surgical Reports SURGICAL N=139 Course Acute angle High osteal takeoff AAOCA+origin N IA+IM IM IA IC IA+IM+IC IA+IC yes no n/a Yes no n/a ARCA=98 Left sinus Suprasinsu ALCA=37 R.sinus Suprasinus NCS LAD Both= TOTAL

13 Spectrum of Morphology: Expert Review ECHO REVIEW N=159 Course Acute angle High osteal takeoff AAOCA+origin N IA IM IC IA+IM IA+IM+IC IA+IC n/a yes no n/a Yes no n/a ARCA=113 Left sinus Suprasinus ALCA=31 Right Sinus NCS LAD=2* Right Sinus RCA LCx=1* TOTAL(diagnostic) Non diagnostic 12 *LAD and LCx Likely intraconal LAD with retroaortic LCx, may have single or split origin.

14 1. Comparing Agreement Expert Echo Review with Surgery Institution Echo Reports with Surgery

15 Comparison Preoperative echo assessment to surgical findings Institution Report N=135 Expert Review N=82 Surgery (gold standard) N=139

16 Comparison Objective Determine the agreement between preoperative expert reviews, institution reports, and surgical reports - the gold standard?

17 Agreement Variables for analysis Anomalous Coronary (Right vs Left) Sinus/location of origin Inter-arterial Course Intramural Course Intraconal Course Dual orifice Acute angle at origin High ostial takeoff

18 5 non diagnostic Frequency of Variables: Expert Review Expert reviews N=82 yes no Denominator % missing % missing AAOCA 77 n/a 82 94% 5 6% Origin 76 n/a 82 93% 6 7% Interaterial(IA) % 7 9% Intramural(IM) % 9 11% Intraconal(IC) % 8 10% Dual ostia % 9 11% Acute Angle % 7 9% High Ostial Takeoff % 56 68%

19 Frequency of Variables: Institution Report Institution report N=135 yes no Denominator % missing % missing AAOCA 132 n/a % 3 2% Origin 130 n/a % 5 4% Interaterial(IA) % 47 35% Intramural(IM) % 62 46% Intraconal(IC) % 37 27% Dual ostia % 16 12% Acute Angle % 64 47% High Ostial Takeoff % % 3 non diagnostic for AAOCA

20 Agreement Results: Expert Review w/ Surgery Agreement Expert Review vs surgery: 77/82 diagnostic Agreement variable Kappa P-value n Missing Anomalous coronary artery 0.94 < Origin coronary artery 0.94 < Interarterial course* 0.49 < Intramural course Intraconal course 0.79 < Acute Angle * 0.66 < Dual ostia right 0.87 < Dual ostia left 0.74 < High ostial takeoff X

21 Agreement Results: Institution Reports w/ Surgery Agreement Echo Report vs surgery:132/135 diagnostic Agreement variable: Kappa P-value n missing Anomalous coronary artery 0.9 < Origin coronary artery 0.81 < Interarterial course Intramural course Intraconal Course * 0.49 < Acute Angle Dual ostia right 0.89 < Dual ostia left 0.73 < High ostial take off X X X

22 Results Expert review Institution Report Agreement variable Anomalous coronary artery Origin coronary artery Interarterial course Intramural course Intraconal course Acute Angle Dual ostia right Dual ostia left High ostial takeoff X Kappa Kappa X X X Interpretation <.20=Poor =Fair =moderate good =Excellent

23 Significant Findings Overall agreement was stronger for expert echo reviews than institution reports Some details of morphology appear more difficult to discern on echo acute angle at origin high ostial takeoff

24 Questions Regarding Analysis How to treat missing variables and non-diagnostic imaging? What to do in the case of multiple echos? Some patients have multiple institution reports Should we account for reviewer bias?

25 Implication Identifies potential limitations in current imaging strategies Justification for standardized imaging approach Best practice paper Need for further imaging analysis for predicting morphology

26 2. Preliminary Comparison Expert Echo Review to Institution Echo Reports

27 Expert Review compared to Institution Report Agreement Expert Review vs Echo Institution Reports:147 Echos Agreement variable: Kappa P-value n missing Anomalous coronary artery 0.88 < Origin coronary artery 0.74 < X X Interarterial course Intramural course Intraconal Course 0.99 < Acute Angle Dual ostia right 0.82 < Dual ostia left 0.8 < X

28 Moving Forward Develop 12 month analysis plan to focus efforts on answering key objectives Aggressive data collection for surgical patients, Describe Unnatural History Identify subsets of patients in whom risk of intervention exceeds risk of observation, or vice versa Address follow up issues

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