Appendix A. Group B Cardiac Diagnoses

Appendix A. Group B cardiac diagnoses

Of 155 patients, there were 101 with known congenital heart disease (CHD) and 54 without known CHD. In the following list, the number of patients is indicated first, the number of studies is indicated in parentheses (if different than number of patients), and the patient number is indicated in brackets (if applicable).

·  101 patients with known CHD (121 studies)

o  42 had surgery (58)

§  15 transposition of great arteries (20)

§  6 Tetralogy of Fallot (7)

§  5 anomalous left coronary artery from pulmonary artery (6)

§  4 anomalous right coronary artery from left coronary artery (6)

§  4 Fontan operations (6)

§  2 s/p heart transplant (5)

§  1 aortic valve replacement , LV to Ao conduit (2)

§  1 double outlet right ventricle, LV to Ao conduit (1)

§  1 s/p Ross procedure (1)

§  1 s/p complete AV canal repair (1)

§  1 s/p VSD repair (2)

§  1 s/p subaortic stenosis resection (1)

o  59 had CHD without surgery (63)

§  13 decreased function (15)

§  12 Kawasaki syndrome (13)

§  6 idiopathic hypertrophic subaortic stenosis

§  5 coronary artery fistulas

§  2 pulmonary stenosis (3)

§  1 pulmonary stenosis s/p ballooning

§  3 aortic stenosis/regurgitation

§  3 aortic regurgitation

§  1 s/p aortic valve ballooning

§  2 bicuspid aortic valve

§  1 coil in right coronary artery to right ventricle

§  1 Takayasu’s arteritis

§  2 dilated aorta

§  1 thick intraventricular septum

§  1 mitral regurgitation

§  1 noncompaction left ventricle

§  1 single left coronary artery

§  1 partial anomalous pulmonary venous return

§  1 leftward takeoff of right coronary artery

§  1 prominent right coronary artery

·  54 patients without known CHD

§  26 ECG changes

§  14 dizziness or syncope

§  5 dyspnea on exertion

§  1 family history of idiopathic hypertrophic subaortic stenosis

§  1 family history of sudden unexpected death

§  3 chest pain with elevated cardiac enzymes

§  1 pectus excavatum surgery

§  1 supraventricular tachycardia s/p radiofrequency ablation

§  1 muscle cramps

§  1 positive perfusion test at outside hospital

Appendix B. (Other Heart Disease)

Group B patients with decreased cardiac function on echocardiogram:

·  B5 (2005) - false positive

·  2 myocarditis

o  B3 (2004) - had 2 studies in 2004

o  B9 (2005)

·  5 cardiomyopathy

o  B7 (2005) - had 2 studies in 2005

o  B20 (2008)

o  B23 (2009)

o  B25 (2009)

o  B77 (2005)

·  5 negative tests

o  B52 (2004)

o  B64 (2005)

o  B122 (2008)

o  B140 (2009)

B154 (2010)

Positive tests in patients with idiopathic hypertrophic subaortic stenosis and action taken:

·  B13 (2006) - was on beta blockers previously

·  B21 (2008) - ICD placed

·  B32 (2010) - was on beta blockers previously

Negative tests in patients with idiopathic hypertrophic subaortic stenosis:

·  B42 (2004)

·  B68 (2005)

·  B99 (2006)

Patients with coronary artery anomalies:

Group B patients with small coronary artery fistulas with chest pain, dyspnea, and/or family history of sudden unexplained death (all nuclear):

·  B44 (2004) - chest pain

·  B57 (2004) - chest pain/dyspnea

·  B59 (2004) - chest pain/dyspnea

·  B66 (2005) - family history of sudden unexplained death

·  B128 (2008) - chest pain with exercise

Group B patients s/p anomalous left coronary artery from the pulmonary artery repair:

·  B47 (2004) test - not seen after 2004

·  B80 (2005) test - no clinical action taken on patient

·  B80 (2009) test - no clinical action taken on patient

·  B121 (2008) test - no clinical action taken on patient

·  B148 (2010) test - no clinical action taken on patient

·  B149 (2010) test - no clinical action taken on patient

Group B patients with right coronary artery abnormalities:

·  Patient B18 false positive (see text in paper) anomalous RCA from LCA

·  One patient, B125 (2008), presented with a heart murmur. Echo was suspicious for an anomalous RCA from the left and was confirmed by CT angiogram. At surgery, the RCA had an intramural course from the LCA and was unroofed. The patient did not have ischemia on the postoperative exercise test (myocardial perfusion test was not done postoperatively)

·  Patient B143 (2009) anomalous RCA from LCA suspected by echo and confirmed by CT. Both preoperative and postoperative ETT with MPI were negative.

·  Patient B150 (2010) anomalous RCA from the LCA suspected by echo and confirmed by MRI. He underwent preoperative and post operative MPI were negative.

·  Patient, B10 (2005), had a coil in the right coronary artery that drained to the right ventricle. There were no ECG changes of ischemia during the ETT. The LVEF was 65%, but there was dyssynergy of the septum and inferior walls which led to a positive test.

·  One patient (B137) presented at 8 years old with a family history of dilated cardiomyopathy in the father. Echocardiogram suggested the RCA originating from the LCA. MRI confirmed finding with RCA passing posterior to aorta (and not in between the great arteries). At age 8 y/o due to chest pain during exercise, the patient had a MPI which was normal.

Patients with electrocardiogram changes (n = 26):

·  8 with previous ETT abnormalities

o  B38 (2004) - ECG changes prev ETT, neg echo, neg perfusion test

o  B45 (2004) - ECG changes prev ETT, neg echo, neg perfusion test

o  B53 (2004) - ECG changes prev ETT, neg echo, neg perfusion test

o  B56 (2004) - ECG changes prev ETT, neg echo, neg perfusion test

o  B75 (2005) – chest pain, prev ETT abnl, neg echo, neg perfusion test

o  B98 (2006) - prev abnl ETT, neg echo, neg perfusion test

o  B104 (2007) - prev ETT abnl, neg echo, neg perfusion test

o  B155 (2010) - prev ETT abnl, neg echo, neg perfusion test

·  9 with ST-T wave changes

o  B35 (2004) – chest pain, T wave infersion/inferior/lateral leads, persisted during test neg echo, neg perfusion test

o  B36 (2004) - chest pain, palpitations, T wave inversion inf/lateral leads, corrected with exercise, neg echo, neg perfusion test

o  B71 (2005) - left ventricular hypertrophy with strain, changes persisted with exercise, neg echo, neg perfusion test

o  B73 (2005) - drowning, (initial ECG showed repolarization changes vs. j point depression with ST-T elevation), LVH at time of stress test perfusion test shows dyschronous intraventricular septum motion

o  B79 (2005) - palpitations, ECG changes (LVH, T wave inversion in lead III due to postion), persisted during exercise, neg echo, neg perfusion test

o  B89 (2006) - chest pain, ECG changes (LVH with T wave inversion in inferior/lateral leads), did not correct with exercise neg echo, neg perfusion test

o  B112 (2007) - chest pain, ECG changes (ST-T wave elevation at baseline, inverted T waves), resolved with exercise neg echo, neg perfusion test

o  B133 (09) - abnormal ECG at rest (T), ETT showed ST-T wave depression at peak exercise, neg echo, neg perfusion test

o  B145 (10) – chest pain, LVH with strain (T wave inversion inferior/lateral leads, persisted during exercise, LVH on echo, neg perfusion test, nl function

·  6 with other ECG changes

o  B65 (2005) - chest pain, left axis deviation on ECG, neg echo, neg perfusion test

o  B85 (2005) - delta wave, negative echo, neg perfusion test

o  B93 (2006) - chest pain, dyspnea, LVH, neg echo, neg perfusion test

o  B94 (2006) - chest pain, dyspnea, dizzy, premature ventricular contractions, neg echo, neg perfusion test

o  B102 (2006) - chest pain, LVH, neg echo, neg perfusion test

o  B111 (2007) - ectopy, shortness of breath, palpitations, neg echo, neg perfusion test

·  2 with postive test

o  B11 (2006) - Chest pain, premature ventricular contractions, obese, echo low shortening fraction, decreased left ventricular ejection fraction (LVEF) post test

o  B19 (2007) - Sinus bradycardia, superior axis, RBBB, neg echo, pos perfusion test, LVEF = 44% - He was restricted from isometric exercises. Diagnosis was cardiomegaly and decreased ejection fraction.

·  1 False positive test

o  B1 (2004) ischemia of lateral wall with normal cardiac catheterization

14 Patients with syncope/near syncope (all nuclear portions of test were negative except B30 in 2010); 12/14 had echocardiograms; 8/12 coronary origins normal; 4/12 had either had abnormalities of the coronary arteries or did not see both coronary arteries well:

·  B40 (2004) - normal coronary artery origins

·  B41 (2004) - normal coronary origins

·  B43 (2004) – no echo done

·  B48 (2004) - normal coronary origins

·  B50 (2004) - normal coronary origins

·  B51 (2004) – normal coronary origins

·  B54 (2004) – left coronary artery seen but not RCA

·  B58 (2004) – no echo done

·  B61 (2005) – left coronary artery sell seen but not RCA

·  B62 (2005) - normal coronary artery origins

·  B67 (2005) – normal coronary artery origins

·  B82 (2005) - mildly dilated coronary artery origins , normal coronary artery origins

·  B118 (2008) – right coronary artery origin seen but not LCA by echo

·  B30 (2010) – normal coronary artery origins, reversible defect at septum - patient to have an MRI