2018 AHA/ACC Guideline for the Management of Adults With PDF

Double-Chambered Right Ventricular – Section 4.3.3 . n=10.4 million women y with. 3.3 million women y on oral contraceptive ... 200 pts; 48 doctors.

8th Asia Pacific Heart Rhythm Society Scientific Sessions

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2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease

Data Supplements

(Section numbers correspond to the full-text guideline.)

Data Supplement 1. Anatomic and Physiologic Terms - Section 2.1 ............................................................................ 5

Data Supplement 2. Severity of ACHD - Section 2.2 ..................................................................................................... 7

Data Supplement 3. Access to Care and Delivery of Care - Sections 3.2 and 3.3 .......................................................

Data Supplement 4. Catheter Interventions ................................................................................................................ 17

Data Supplement 5. Surgery

........................................................................................................................................ 18

Data Supplement 6. Ionizing Radiation Principles

- Section 3.4.2 .............................................................................. 20

Data Supplement 7. Echocardiography

- Section 3.4.3 ............................................................................................... 21

Data Supplement 8. CMR Imaging

- Section 3.4.4 ...................................................................................................... 24

Data Supplement 9. Cardiac Computed Tomography

- Section 3.4.5 ........................................................................ 28

Data Supplement 10. Cardiac Catheterization

- Section 3.4.6 .................................................................................... 30

Data Supplement 11. Exercise Testing

- Section 3.4.7 ................................................................................................ 34

Data Supplement 12. Transition Education

- Section 3.5 ........................................................................................... 35

Data Supplement 13. Exercise and Sports

- Section 3.6 ............................................................................................. 36

Data Supplement 14. Mental Health and Neurodevelopmental Issues - Section 3.7................................................. 39

Data Supplement 15. Endocarditis Prevention

- Section 3.8 ...................................................................................... 41

Data Supplement 16. Concomitant Syndromes - Section 3.9 ..................................................................................... 43

Data Supplement 17. Acquired Cardiovascular Disease - Section 3.10 ...................................................................... 45

Data Supplement 18. Noncardiac Medical Issues and Noncardiac Surgery - Sections 3.11 and 3.12 ........................ 54

Data Supplement 19. Pregnancy

- Section 3.13.1 ....................................................................................................... 58

Data Supplement 20. Contraception

- Section 3.13.2 ................................................................................................ 69

Data Supplement 21. Sexual Function

- Section 3.13.4 .............................................................................................. 70

Data Supplement 22. Pharmacological Therapy for ACHD - Section 3.17 .................................................................. 71

Data Supplement 23. Heart Failure and Transplant

- Section 3.14.2 .......................................................................... 76

Data Supplement 24. Palliative Care - Section 3.15 .................................................................................................... 78

Data Supplement 25. Cyanosis

- Section 3.16............................................................................................................. 78

Data Supplement 26. Atrial Septal Defect - Section 4.1.1 .......................................................................................... 80

Data Supplement 27. Anomalous Pulmonary Venous Connections - Section 4.1.2 ................................................... 92

Data Supplement 28. Ventricular Septal Defect

- Section 4.1.3 ................................................................ 96

Data Supplement 29. Atrioventricular Septal Defect - Section 4.1.4 .......................................................................... 98

Data Supplement 30. Patent Ductus Arteriosus - Section 4.1.5 ................................................................................. 99

Data Supplement 31. Cor Triatriatum

- Section 4.2.1 ............................................................................................... 101

Data Supplement 32. Congenital Mitral Stenosis - Section 4.2.2 ............................................................................. 101

Data Supplement 33. Subaortic Stenosis

- Section 4.2.3 .......................................................................................... 102

Data Supplement 34. Congenital Valvular Aortic Stenosis - Sections 4.2.4 .............................................................. 108

Data Supplement 35. Turner Syndrome

- Section 4.2.4.1 ........................................................................................ 109

Data Supplement 36. Aortopathies

- Section 4.2.4.2 ............................................................................................... 110

Data Supplement 37. Supravalvular Aortic Stenosis - Section 4.2.5 ......................................................................... 112

Data Supplement 38. Coarctation of the Aorta - Section 4.2.6 ................................................................................ 116

Data Supplement 39. Valvular Pulmonary Stenosis - Section 4.3.1 .......................................................................... 123

Data Supplement 40. Branch and Peripheral Pulmonary Stenosis Section 4.3.2 ................................................... 125

Data Supplement 41. Double-Chambered Right Ventricular - Section 4.3.3 ............................................................ 128

Data Supplement 42. Ebstein Anomaly

- Section 4.3.4............................................................................................. 129

Data Supplement 43. Tetralogy of Fallot

- Section 4.3.5 .......................................................................................... 132

Data Supplement 44. Right Ventricle to Pulmonary Artery Conduit - Section 4.3.6 ................................................ 147

Data Supplement 45. Transposition of the Great Arteries with Atrial Switch - Section 4.4.1.1 ............................... 158

Data Supplement 46. Transposition of the Great Arteries with Arterial Switch - Section 4.4.1.2 ............................ 165

Data Supplement 47. Congenitally Corrected Transposition of the Great Arteries - Section 4.4.1.4 ....................... 167

Data Supplement 48. Fontan Palliation of Single Ventricle Physiology Including Tricuspid Atresia and Double Inlet

Right Ventricle

- Section 4.4.2 ................................................................................................................................... 176

Data Supplement 49. Severe Pulmonary Arterial Hypertension - Section 4.4.6.1 .................................................... 186

Data Supplement 50. Eisenmenger Syndrome

- Section 4.4.6.2 .............................................................................. 193

Data Supplement 51. Anomalous Coronary Artery Evaluation - Section 4.4.7.1 ...................................................... 206

References ................................................................................................................................................................. 213

~ indicates approximately; 1°, primary; 2°, secondary; 2D, 2-dimensional; 3D, 3-dimensional; 4D, 4-dimensional; 6MWT, 6-minute walk test; AAOCA, anomalous aortic

origin of the coronary artery; AAOLCA, anomalous aortic origin of the left coronary artery; AAORCA, anomalous aortic origin o

f the right coronary artery; ACC, American

College of Cardiology; ACEI, angiotensin-converting-enzyme inhibitor; ACHD, adult congenital heart disease; AE, adverse events; AF, atrial fibrillation; AHA, American

Heart Association;

ALCAPA, anomalous left coronary artery arising from the pulmonary artery; ANP, atrial natriuretic peptide; ARB, angiotensin-receptor blocker; AS, aortic stenosis;

ASD, atrial septal defect;

ASO, arterial switch operation;

AT, anaerobic threshold; AVC, atrioventricular canal; AVNRT, atrioventricular nodal reentrant tachycardia; AV, atrioventricular;

AVS, aortic valve stenosis;

AVSD, atrioventricular septal defect;

BAV, bicuspid aortic valve; BB, beta blockers; BDCPA, bidirectional cavopulmonary anastomosis; BMI, body mass index; BNP, brain natriuretic peptide;

BP, blood pressure;

BREATHE-5, Bosentan Randomized Trial of Endothelin

Antagonist Therapy

-5; CABG, coronary artery bypass grafting; CAC, coronary artery calcium; CAD, coronary artery disease; CAMPHOR, Cambridge Pulmonary

Hypertension Outcome Review; CACH, Canadian Adult Congenital Heart; CCT, cardiac computed tomography; CCTGA, congenitally corrected transposition of the great

arteries; CHD, congenital heart disease; CI, confidence interval; CKD, chronic kidney disease; CK-MB, creatine kinase-MB; CMR, cardiac magnetic resonance; CoA,

coarctation of the aorta;

COR, class of recommendation;

CPB, cardiopulmonary bypass;

CPET, cardiopulmonary exercise stress test; CRT, cardiac resynchronization

therapy; CT, computed tomography; CTA, computed tomography angiography; CV, cardiovascular; CVA, cerebrovascular accident; CVD, cardiovascular disease; CXR,

chest x-ray; DBP, diastolic blood pressure; DCCV, direct current cardioversion; DCM, dilated cardiomyopathy; DCRV, double-chambered right ventricle; DM, diabetes

mellitus; double outlet right ventricle; dp/dt, measurement of the rate of systolic right ventricular pressure increase; d-TGA, dextro-transposition of the great arteries;

echo, echocardiography;

ECG, electrocardiogram; ED, emergency department; EDA, end diastolic area; EF, ejection fraction; EOL, end-of-life; EP,

electrophysiology/electrophysiologic; EPS, electrophysiological studies; ERA, endothelin receptor antagonist; FC, functional class; FDA, Food and Drug Administration;

FDR, first-degree relative; GI, gastrointestinal; GUCH, grown up congenital heart; HCT, hematocrit; HD, heart disease; HF, heart failure; HGB, hemoglobin; HLHS,

hypoplastic left heart syndrome;

HR, hazard ratio; HTN, hypertension;

IART, intra-atrial reentrant tachycardia; ICD-9-CM, International Classification of Diseases, Ninth

Revision, Clinical Modification;

ICE, intracardiac echocardiography; ICD, implantable cardioverter-defibrillator; ICU, intensive care unit; IE, infective endocarditis; IQ,

intelligence quotient;

IQR, interquartile range; IV, intravenous; LDL, low-density lipoprotein; LIMA, left internal mammary artery; LMCA, left main coronary artery; LOE,

level of evidence; LOS, length of stay;

LV, left ventricular; LVEDD, left ventricular end diastolic diameter; LVEF, left ventricular ejection fraction; LVOT, left ventricular

outflow tract; LVOTO, left ventricular outflow obstruction; MACE, major adverse cardiac events; MCV, mean corpuscular volume; MDCTA, multidetector computed

tomographic angiography;

MI, myocardial infarction;

mPAP, mean pulmonary artery pressure; MR, mitral regurgitation; MRA, magnetic resonance

angiogram/angiography; MRCA, magnetic resonance coronary angiography; MRI, magnetic resonance imaging; MS, mitral stenosis; MSCT, multi-slice computer

tomography; N/A, not applicable; NHLBI, National Heart, Lung, and Blood Institute; NPV, negative predictive value; NS, not significant; NT-BNP, N-terminal-brain

natriuretic peptide; NT-proANP, N-terminal pro-atrial natriuretic peptide; NT-proBNP, N-terminal-pro-brain natriuretic peptide; NYHA, New York Heart Association;

NSVT, nonsustained ventricular tachycardia; NVE, native valve endocarditis; O

2, oxygen; OR, odds ratio; PA, pulmonary artery; PAD, patent ductus arteriosus; PAH,

pulmonary arterial hypertension; PAP, pulmonary artery pressure; PAPVC, partial anomalous pulmonary venous connection;

PAPVR, partial anomalous pulmonary

venous drainage;

PASP, pulmonary artery systolic pressure;

PCI, percutaneous coronary intervention; PDA, patent ductus arteriosus; PDE-5, phosphodiesterase type-5;

PFO, patent foramen ovale;

PH, pulmonary hypertension; PLE, protein losing enteropathy; PPVI, percutaneous pulmonary valve implant; PR, pulmonary regurgitation;

PS, pulmonary stenosis; PVR, pulmonary vascular resistance; pt, patient; PV, pulmonary valve; PVE, prosthetic valve endocarditis; QOL, quality of life; Qpeff, effective

pulmonary blood flow; Qpi, pulmonary blood flow index; Qp:Qs, pulmonary to systemic flow ratio; RACHS-1, Risk Adjusted Classification for Congenital Heart Surgery;

RCT, randomized controlled trial; RT3DE real-time 3-dimensional echocardiography; RV, right ventricular; RVEDV, right ventricular end diastolic volume; RVEF, right

ventricular ejection fraction; RVESV, right ventricular end systolic volume; RVOT, right ventricular outflow tract; RVOTO; right ventricular outflow tract obstruction; RV-

PA, right ventricular to pulmonary artery;

Rx, medical prescription; SBP, systolic blood pressure; SCD, sudden cardiac death; SD, standard deviation; SEM, standard error

of the mean; SF-12, Short Form Health Survey; s/p, status post; SND, sinus node dysfunction; SPAP, systolic pulmonary artery pressure; SpO

2, oxygen saturation; STAT,

Stending in Aneurysm Treatmen

ts Trial; STS, Society of Thoracic Surgeons; SVA, supraventricular arrhythmia; SVC, superior vena cava; SVG, saphenous vein graft; SVT,

supraventricular tachycardia; TA, tricuspid annulus; TEE, transesophageal echocardiography; TGA, transposition of the great arteries; TIA, transient ischemic attack;

TOF, tetralogy of Fallot; TOF/PA, tetralogy of Fallot with pulmonary atresia; TPVR, transcatheter pulmonary valve replacement; TR, tricuspid regurgitation; TTE,

transthoracic echocardiogram; TV, tricuspid valve; TVR, tricuspid valve replacement; UK, United Kingdom; UL, upper limbs; UNOS, United Network for Organ Sharing;

U.S., United States; VA, ventricular arrhythmias; VAD, ventricular assist device; VF, ventricular fibrillation;

VHD, valvular heart disease; VO

2, oxygen consumption; VSD,

ventricular septal defect;

VT, ventricular tachycardia; VTE, venous thromboembolism; WHO, World Health Organization; WPW, Wolf-Parkinson-White; and WU, Wood

units.

Data Supplement 1. Anatomic and Physiologic Terms

- Section 2.1 Study

Name, Author,

Year

Study Type/

Design

Study Size Inclusion/Exclusion

Criteria

Classification

System

1° Endpoint Results/p Values Summary/Conclusions

Afilalo J, et al.

2011 (1)

21939837

Observational,

case control, cohort, retrospective n=3,239 pts;

Quebec CHD

Database

Inclusion criteria: Pts

-2005,

ACHD, Quebec,

healthcare interaction

Bethesda 32

(severe, shunt, valvular) with older adults with CHD. database has limitations.

Tutarel O, et al.

2014 (2)

23882067

Observational,

cohort, retrospective n=375 pts Inclusion criteria: Pts 2000
-March 2012

Bethesda 32 Prevalence,

demographics, interventions, mortality mortality ratio simple 0.6 (p=0.06), moderate 1.89 (p=0.002), complex 2.91 (p=0.01); overall 1.82. multivariate as HF, FC, ventricular function took over). increase in number of -up at

ACHD centers.

in ACHD pts determines outcomes.

Kim YY, et al.

2011 (3)

22010202

Observational,

case control, retrospective database n=3,061 pts Inclusion criteria: 18 -49 y, 2000-2008,

ACHD surgery in

pediatric hospital, total compared to pediatric surgical pts, ACHD high resource utilization vs. nonhigh resource utilization

RACHS-1 Resource

utilization, cost, LOS

RACHS classification (much

tighter RACHS 3 and 4 as compared to 2). database limited. undergoing congenital heart surgery is increasing. utilization admissions associated with higher death rates.

Kim YY, et al.

2011 (4)

21693722

Observational,

case control, retrospective n=3,061 pts Public

Health Information

System database

Inclusion criteria:

18 -49 y, 2000-2008,

ACHD surgery in

pediatric hospital, total compared to pediatric surgical pts, ACHD mortality vs. nonmorality significant for RACHS 3 and 4). congenital heart surgery is lower in pedia tric hospitals with high congenital heart surgery volume.

Opotowsky AR,

et al.

2012 (5)

Observational,

case control, retrospective n=622,000 pts Inclusion criteria: Pts in 1998 -2007

Bethesda 32 Combined:

death, HF, arrhythmia, CVA,

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[PDF] 2018 AHA/ACC Guideline for the Management of Adults With