The survey was opened online 177 times with 111 complete responses leading to a completion rate of 62.7%. There was no way of calculating the response rate because of the overlap between SOCCS and Pediheart, but clearly, we received responses from a minority of the two groups.
Table 1
Variable
|
Number
|
|
Practice Location
|
US
|
107
|
US Region
|
South
|
28
|
|
Northeast
|
27
|
|
Midwest
|
26
|
|
West coast
|
16
|
|
Southwest
|
5
|
|
Central Plains/ Rocky Mountains
|
4
|
Field of practice
|
Peds Cardiology
|
106
|
|
Cardiac surgery
|
3
|
|
Peds ICU
|
2
|
Role
|
Physician
|
94
|
|
Trainee
|
12
|
|
APP
|
3
|
|
Other
|
2
|
Years in practice
|
< 10 years
|
36
|
|
10–24 years
|
36
|
|
25 years or more
|
27
|
Yearly Surgical Volume
|
Nonsurgical
|
19
|
|
less than 100
|
3
|
|
101–200
|
22
|
|
201–300
|
18
|
|
more than 300
|
49
|
Do You Read Echocardiograms?
|
yes
|
98
|
|
no
|
13
|
The demographic features of the respondents are given in Table 1 and their responses in Tables 2–7. A recommendation for repeat echocardiography in the context of poor coronary visualization was the only area in which a significant difference was found between respondents based on region and years in practice. Respondents’ surgical volume had no statistically significant influence on their responses in any of the questions posed.
Table 2
Visualization of the Coronary Artery Origins on First Transthoracic Echocardiogram
< 1 year
|
1–4 years
|
5–9 years
|
1–20 years
|
|
Percent seen on echo
|
85.35%
|
80.54%
|
82.38%
|
75.68%
|
Recommendations if not seen
|
|
|
|
|
No recommendation
|
13
|
10
|
13
|
13
|
Specific mention in report
|
69
|
76
|
76
|
76
|
Repeat echo recommended
|
27
|
23
|
20
|
20
|
The frequency in which the coronaries were adequately defined on “first time” complete echocardiograms and the response to the inadequate definition of the coronary arteries is seen in Table 2. For patients ages 1–4 years of age, respondents with > 25 years’ experience (P = 0.037) or from the northeastern US (P = 0.023) were more likely to recommend repeat echocardiography if the coronary arteries were not clear on initial echocardiography. Respondents from the northeast were also more likely to recommend repeat echocardiography in the 5-9-year age group (P = 0.023).
Although respondents reported the coronary artery origins to be reliably visualized in between 76 and 85% of patients on their first echocardiogram (Table 2), a minority (20–27%) recommended repeat echocardiography to define the coronary anatomy if it was not demonstrated. Most respondents (69–76%) made a specific comment when the coronaries were not visualized, deferring the decision on repeat imaging to the ordering provider. This suggests a certain ambivalence regarding the need to define the coronaries in patients undergoing echocardiography.
Table 3: When are the Following Recommended After Incidental Diagnosis of AAOCA at One Day of Age? (Number of Responses in Each Age Interval)
Anomalous Aortic Origin of the Right Coronary Artery
|
|
|
|
<1 year
|
1-6 years
|
7-12 years
|
>12 years
|
Not Planned
|
First Clinic Visit
|
11
|
92
|
4
|
0
|
4
|
Next Echocardiogram
|
8
|
91
|
6
|
0
|
6
|
ECG Exercise Test
|
1
|
4
|
76
|
3
|
27
|
Coronary CT
|
9
|
28
|
43
|
1
|
30
|
Stress Imaging
|
1
|
2
|
50
|
1
|
57
|
AED Recommended
|
2
|
3
|
19
|
1
|
86
|
Cardiac MRI
|
2
|
3
|
14
|
0
|
92
|
Exercise Restriction
|
2
|
4
|
10
|
1
|
94
|
Surgery
|
2
|
3
|
8
|
0
|
98
|
Cardiac Catheterization
|
3
|
1
|
0
|
0
|
107
|
|
|
|
|
|
|
Anomalous Aortic Origin of the Left Coronary Artery
|
|
|
|
<1 year
|
1-6 years
|
7-12 years
|
>12 years
|
Not Planned
|
First Clinic Visit
|
20
|
85
|
3
|
0
|
3
|
Next Echocardiogram
|
15
|
86
|
4
|
0
|
6
|
Coronary CT
|
12
|
34
|
47
|
2
|
16
|
ECG Exercise Test
|
0
|
7
|
71
|
2
|
31
|
Surgery
|
3
|
19
|
48
|
5
|
36
|
Exercise Restriction
|
2
|
15
|
47
|
3
|
44
|
Stress Imaging
|
1
|
2
|
48
|
1
|
59
|
AED Recommended
|
3
|
10
|
30
|
2
|
66
|
Cardiac MRI
|
5
|
6
|
16
|
1
|
83
|
Cardiac Catheterization
|
3
|
2
|
1
|
0
|
105
|
Table 4
Actions Planned Based on Coronary Anatomy When Diagnosed Incidentally at One Day of Age
AAORCA
|
AAOLCA
|
P value
|
|
AED for practice or competition
|
25
|
45
|
< 0.001
|
Exercise restriction
|
17
|
67
|
< 0.001
|
Surgery
|
13
|
75
|
< 0.001
|
Coronary CT
|
81
|
95
|
0.001
|
Cardiac MRI
|
19
|
28
|
0.023
|
ECG Exercise Testing
|
84
|
80
|
0.371
|
Stress Imaging (echo, MRI or nuclear)
|
54
|
52
|
0.593
|
Cardiac Catheterization
|
4
|
6
|
0.625
|
Repeat cardiology clinic visit
|
107
|
108
|
1
|
Repeat Echocardiography
|
105
|
105
|
1
|
Respondents were given the scenario of an AAOCA diagnosed incidentally in a one-day-old baby and asked when they would recommend when a number of actions should next be undertaken. Their recommendations for the timing in both AAORCA and AAOLCA are given in Table 3. This vignette was used to assess the relative value placed on various actions in the diagnosis and treatment of AAOCA. Whether or not an action was anticipated at any time in either AAORCA or AAOLCA are given in Table 4.
After an incidental neonatal diagnosis, 75 respondents (67.6%) anticipated surgery for AAOLCA (at an average anticipated age of 7.84 years) and 13 (11.7%) anticipated surgery for AAORCA (at an average anticipated age of 6.54 years). The 2017 AATS guidelines discuss age for operation as “In those with an interarterial AAOLCA, most children ages 10 and older are referred for surgical intervention.” (21)
Survey participants relied on basic exercise tests more often than stress imaging despite the increased sensitivity afforded by the latter and the recommendation for its use in risk stratification in the 2017 AATS recommendations.
Among actions taken for both AAORCA and AAOLCA by an individual respondent, clinic visits and diagnostic testing was performed somewhat later in those patients with AAORCA (Supplemental Table 1).
Table 5: Appropriateness of Surgical Intervention in a 16-year-old with AAOCA
Respondents were asked to rate the appropriateness of surgical intervention on a five-point Likert score for a 16-year-old with six combinations of coronary anatomy, symptoms and signs of ischemia on stress imaging, as demonstrated in Table 5. Most respondents’ recommendations for care mirrored the 2017 AATS guidelines. However, in the case of an asymptomatic 16-year-old with the AAOCLA with no evidence of ischemia on stress imaging, only 69.4% of survey participants considered surgical intervention appropriate or somewhat appropriate despite the 2017 AATS recommendations for surgery in all patients with AAOCLA.
Table 6: Change in Recommendation for Surgery in An Asymptomatic 16-year-old with AAORCA and no Evidence of Ischemia on Stress Imaging
Given an asymptomatic 16-year-old with an incidental finding of an AAORCA on an echocardiogram performed for other indications who had no evidence of ischemia on stress imaging, respondents were asked if membership in one of nine screening populations would alter their recommendations for surgery. Their responses are provided in Table 6. The 2017 AATS expert guideline doesn’t address this aspect of patient management. In the most notable case, 42% of respondents were more likely to recommend surgery for a competitive athlete with asymptomatic AAORCA showing no signs of ischemia on stress imaging. Unlike the other populations, the origins of the coronary arteries are a specific focus of the screening echocardiograms in athletes.
Table 7
Postoperative Antiplatelet Therapy
Responses
|
|
Aspirin indefinitely
|
26
|
Other antiplatelet indefinitely
|
1
|
Aspirin for a limited time
|
64
|
Other antiplatelet for a limited time
|
5
|
Not Recommended
|
15
|
Weeks of limited antiplatelet Rx
|
Mean 20.56
|
|
Range 4–52
|
Table 7 demonstrates the respondent’s recommendation for the use and duration of antiplatelet therapy after surgery for AAOCA. Only 24% of respondents recommended indefinite use of aspirin or another antiplatelet agent despite 2017 AATS recommendations for lifelong aspirin therapy.