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

Heart Disease (ACHD)

© American College of Cardiology Foundation and American Heart Association, Inc.

Publication Information

This slide set is adapted from the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease

Published on August 16, 2018, available at: Circulation andJournal of the American College of Cardiology.

The full-text guidelines are also available on the following websites: AHA (professional.heart.org) and ACC (www.acc.org)

http://www.cardiosource.org/

2018 Adults With Congenital Heart Disease Guideline Writing Committee

Karen K. Stout, MD, FACC, Chair†Curt J. Daniels, MD, Vice Chair*†‡

Jamil A. Aboulhosn, MD, FACC, FSCAI*§ Stephanie Fuller, MD, MS, FACC#Biykem Bozkurt, MD, PhD, FACC, FAHA║ Michelle Gurvitz, MD, FACC**Craig S. Broberg, MD, FACC*† Paul Khairy, MD, PhD*†Jack M. Colman, MD, FACC† Michael J. Landzberg, MD, FACC*†Stephen R. Crumb, DNP, AACC† Arwa Saidi, MB, BCH, FACC*†Joseph A. Dearani, MD, FACC¶ Anne Marie Valente, MD, FACC, FAHA, FASE††George F. Van Hare, MD‡‡

†ACC/AHA Representative. ‡International Society for Adult Congenital Heart Disease Representative. §Society for Cardiovascular Angiography and Interventions. ║ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. ††American Society of Echocardiography Representative. ‡‡Heart Rhythm Society Representative.

Applying Class of Recommendation and Level of Evidence to Clinical Strategies,

Interventions, Treatments, or

Diagnostic Testing in Patient Care*

(Updated August 2015)

Systematic Reviews on ACHD

• “Medical Therapy for Systemic Right Ventricles: A Systematic Review (Part 1)

• “Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2)

ACHD AP CLASSIFICATION(CHD Anatomy + Physiological Stage = ACHD AP Classification)

• CHD Anatomy (This list is not meant to be comprehensive; other conditions may be important in individual patients. ASD, atrial septal defect; AVSD, atrioventricular septal defect; CCTGA, congenitally corrected transposition of the great arteries; CHD, congenital heart disease; d-TGA, dextro-transposition of the great arteries; FC, functional class; HCM, hypertrophic cardiomyopathy; l-TGA, levo-transposition of the great arteries; NYHA, New York Heart Association; TGA, transposition of the great arteries; and VSD, ventricular septal defect. )

I: SimpleNative disease• Isolated small ASD• Isolated small VSD• Mild isolated pulmonic stenosisRepaired conditions• Previously ligated or occluded ductus arteriosus• Repaired secundum ASD or sinus venosus defect without significant residual shunt or chamber

enlargement• Repaired VSD without significant residual shunt or chamber enlargement

(Con’t.)

ACHD AP CLASSIFICATION• CHD Anatomy

II: Moderate ComplexityRepaired or unrepaired conditions• Aorto-left ventricular fistula• Anomalous pulmonary venous connection, partial or total• Anomalous coronary artery arising from the pulmonary artery• Anomalous aortic origin of a coronary artery from the opposite sinus• AVSD (partial or complete, including primum ASD)• Congenital aortic valve disease• Congenital mitral valve disease• Coarctation of the aorta• Ebstein anomaly (disease spectrum includes mild, moderate, and severe variations)• Infundibular right ventricular outflow obstruction• Ostium primum ASD• Moderate and large unrepaired secundum ASD• Moderate and large persistently patent ductus arteriosus• Pulmonary valve regurgitation (moderate or greater)• Pulmonary valve stenosis (moderate or greater)• Peripheral pulmonary stenosis• Sinus of Valsalva fistula/aneurysm• Sinus venosus defect• Subvalvar aortic stenosis (excluding HCM; HCM not addressed in these guidelines)• Supravalvar aortic stenosis• Straddling atrioventricular valve• Repaired tetralogy of Fallot• VSD with associated abnormality and/or moderate or greater shunt

(Con’t.)

ACHD AP CLASSIFICATION

• CHD Anatomy III: Great Complexity (or Complex)

• Cyanotic congenital heart defect (unrepaired or palliated, all forms)• Double-outlet ventricle• Fontan procedure• Interrupted aortic arch• Mitral atresia• Single ventricle (including double inlet left ventricle, tricuspid atresia, hypoplastic left

heart, any other anatomic abnormality with a functionally single ventricle)• Pulmonary atresia (all forms)• TGA (classic or d-TGA; CCTGA or l-TGA)• Truncus arteriosus• Other abnormalities of atrioventricular and ventriculoarterial connection (i.e.,

crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion)

(Con’t.)

ACHD AP CLASSIFICATION

• Physiological StateA

• NYHA FC I symptoms• No hemodynamic or anatomic sequelae• No arrhythmias• Normal exercise capacity• Normal renal/hepatic/pulmonary function

B• NYHA FC II symptoms• Mild hemodynamic sequelae (mild aortic enlargement, mild ventricular

enlargement, mild ventricular dysfunction)• Mild valvular disease • Trivial or small shunt (not hemodynamically significant)• Arrhythmia not requiring treatment• Abnormal objective cardiac limitation to exercise

(Con’t.)

ACHD AP CLASSIFICATION• Physiological Stage

C• NYHA FC III symptoms• Significant (moderate or greater) valvular disease; moderate or greater ventricular

dysfunction (systemic, pulmonic, or both)• Moderate aortic enlargement• Venous or arterial stenosis• Mild or moderate hypoxemia/cyanosis• Hemodynamically significant shunt• Arrhythmias controlled with treatment• Pulmonary hypertension (less than severe)• End-organ dysfunction responsive to therapy

D• NYHA FC IV symptoms• Severe aortic enlargement• Arrhythmias refractory to treatment• Severe hypoxemia (almost always associated with cyanosis)• Severe pulmonary hypertension• Eisenmenger syndrome• Refractory end-organ dysfunction

2018 ACHD Clinical Practice Guidelines

Access to Care

Access to Care

Recommendation for Access to CareCOR LOE Recommendation

I

B-NRPhysicians caring for patients with ACHD should support access tocare by 1) assuring smooth transitions for adolescents and youngadults from pediatric to adult providers (Level of Evidence: B-NR);and 2) promoting awareness of the need for lifelong specializedcare through outreach and educational programs (Level ofEvidence: C-EO).

C-EO

2018 ACHD Guideline

Delivery of Care

Delivery of CareRecommendations for Delivery of Care

COR LOE Recommendations

I B-NRPatients with ACHD AP classification IB-D, IIA-D, and IIIA-D*should be managed in collaboration with an ACHD cardiologist .

I C-LD

Cardiac surgery, catheter-based interventional cardiacprocedures, and electrophysiological procedures involvingcongenital heart lesions in patients with ACHD should beperformed by operators with expertise in CHD procedures and incollaboration with an ACHD cardiologist.

*See details on the ACHD Anatomic and Physiological classification system.

2018 ACHD Guideline

Evaluation of Suspected and Known CHD

Electrocardiogram

Recommendations for ElectrocardiogramCOR LOE Recommendations

I C-EO

A standard 12-lead electrocardiogram (ECG) is recommended in adultswith CHD with serial assessment depending on the specific ACHD APclassification or when symptoms develop or worsen.

I C-EO

Ambulatory electrocardiographic monitoring should be performed inpatients with CHD who are at risk of tachyarrhythmia, bradyarrhythmia orheart block, or when symptoms possibly of arrhythmic origin develop.

(Con’t.)

Ionizing Radiation principlesRecommendation for Ionizing Radiation Principles

COR LOE Recommendation

I B-NR

Strategies to limit and monitor radiation exposure are recommendedduring imaging of patients with ACHD, with studies not involvingionizing radiation chosen whenever appropriate.

(Con’t.)

EchocardiographyRecommendations for Echocardiography


I B-NRIntraoperative TEE is recommended to guide surgical repair of CHD inadults.

I C-EO

Patients with ACHD should undergo transthoracic echocardiography(TTE) for initial assessment, with timing of serial assessment based onanatomic and physiological severity and clinical status.

(Con’t.)

CMR ImagingRecommendations for CMR Imaging


I B-NRIn patients with ACHD who have or who are at risk of developing RVenlargement and dysfunction, serial CMR is recommended for quantitativeassessment of RV size and function.

IIa C-LDCMR can be useful in the initial evaluation and serial assessment ofselected patients with CHD based on anatomic complexity and clinicalstatus.

Cardiac Computed Tomography

Recommendation for Cardiac Computed Tomography


IIa C-LD

CCT imaging can be useful in patients with ACHD when informationthat cannot be obtained by other diagnostic modalities is importantenough to justify the exposure to ionizing radiation.

Cardiac Catheterization

Recommendations for Cardiac CatheterizationCOR LOE Recommendations

I C-LD

Cardiac catheterization (hemodynamic and/or angiographic) in patientswith ACHD AP classification II and III, or interventional cardiaccatheterization in patients with ACHD AP classification I to III should beperformed by, or in collaboration with, cardiologists with expertise inACHD.

IIa B-NR

In patients with a low or intermediate pretest probability of coronaryartery disease (CAD), use of CT coronary angiography is reasonable toexclude significant obstructive CAD when cardiac catheterization hassignificant risk or because of patient preference.

Exercise Testing

Recommendations for Exercise TestingCOR LOE Recommendations

IIa B-NRIn patients with ACHD, cardiopulmonary exercise testing (CPET) can beuseful for baseline functional assessment and serial testing.

IIa C-LDIn symptomatic patients with ACHD, a 6-minute walk test can be usefulto objectively assess symptom severity, functional capacity, andresponse to therapy.

Transition Education

2018 ACHD Guideline

Transition Education

Recommendation for Transition EducationCOR LOE Recommendation

I B-NR

Clinicians caring for patients with CHD should deliver developmentallyappropriate transition education to adolescent and young patients with CHD,and to their families/support network.

2018 ACHD Guideline

Exercise and Sports

Exercise and Sports

Recommendations for Exercise and Sports


I C-LDClinicians should assess activity levels at regular intervals andcounsel patients with ACHD about the types and intensity ofexercise appropriate to their clinical status.

IIa C-LDCPET can be useful to guide activity recommendations for patientswith ACHD.

IIa B-NRCardiac rehabilitation can be useful to increase exercise capacityin patients with ACHD.

2018 ACHD Guideline

Mental Health and Neurodevelopmental Issues

Mental Health and Neurodevelopmental IssuesRecommendations for Mental Health and Neurodevelopmental Issues


I B-NR Patients with ACHD should be evaluated for depression and anxiety.

IIa B-NRReferral for mental health evaluation and treatment is reasonable inpatients with ACHD.

IIb B-NR

Neurodevelopmental or neuropsychological testing may be consideredin some patients with ACHD to guide therapies that enhance academic,behavioral, psychosocial, and adaptive functioning.

2018 ACHD Guideline

Concomitant Syndromes

Concomitant SyndromesRecommendation for Concomitant Syndromes


IIa B-NRGenetic testing for 22q11 deletions is reasonable for patients withconotruncal cardiac defects.

2018 ACHD Guideline

Noncardiac Medical Issues

Noncardiac Medical IssuesRecommendation for Noncardiac Medical Issues


I C-LDPatients with ACHD at risk for hepatitis C should be screened andvaccinated for viral hepatitis and treated as appropriate.

2018 ACHD Guideline

Noncardiac Surgery

Noncardiac SurgeryRecommendations for Noncardiac Surgery


I C-LDOptimization before and close surveillance after invasive procedures,regardless of the complexity of the anatomic defect or type of procedureis beneficial for patients with ACHD.

I B-NRIn patients with ACHD AP classification IB-D, IIA-D, and IIIA-D*noncardiac surgical and interventional procedures should be performedin a hospital with or in consultation with experts in ACHD when possible.

*See Table on the ACHD AP classification system

2018 ACHD Guideline

Pregnancy, Reproduction, and Sexual Health

Pregnancy


Recommendations for PregnancyCOR LOE Recommendations

I C-LDWomen with CHD should receive prepregnancy counseling with input from an ACHDcardiologist to determine maternal cardiac, obstetrical and fetal risks, and potentiallong-term risks to the mother.

I C-LDAn individualized plan of care that addresses expectations and contingencies shouldbe developed for and with women with CHD who are pregnant or who may becomepregnant and shared with the patient and all caregivers.

I B-NRWomen with CHD receiving chronic anticoagulation should be counseled, ideallybefore conception, on the risks and benefits of specific anticoagulants duringpregnancy.

I B-NRWomen with ACHD AP classification IB-D, IIA-D, and IIIA-D* should be managedcollaboratively during pregnancy by ACHD cardiologists, obstetricians, andanesthesiologists experienced in ACHD.

(Con’t.)

Pregnancy


I C-EO

In collaboration with an ACHD cardiologist to ensure accurate assessment ofpregnancy risk, patients at high risk of maternal morbidity or mortality, includingwomen with pulmonary arterial hypertension (PAH), Eisenmenger syndrome, severesystemic ventricular dysfunction, severe left-sided obstructive lesions, and/or ACHDAP classification ID, IID, and IIID* should be counseled against becoming pregnant orbe given the option of terminating pregnancy.

I B-NR Men and women of childbearing age with CHD should be counseled on the risk of CHDrecurrence in offspring.

IIa B-NRExercise testing can be useful for risk assessment in women with ACHD APclassification IC-D, IIA-D, and IIIA-D* who are considering pregnancy.

IIa B-NR When either parent has CHD, it is reasonable to perform fetal echocardiography.

(Con’t.)

Contraception

(Con’t.)

Recommendations for Contraception


I C-LDWomen of childbearing potential with CHD should be counseled about therisks associated with pregnancy and appropriate contraceptive options .

III: Harm B-NR

Estrogen-containing contraceptives are potentially harmful for women withCHD at high risk of thromboembolic events (e.g., cyanosis, Fontanphysiology, mechanical valves, prior thrombotic events, PAH) .

Heart Failure and Transplant

2018 ACHD Guideline

Heart FailureRecommendation for Heart Failure


I C-LDConsultation with ACHD and HF specialists is recommended forpatients with ACHD and HF or severe ventricular dysfunction.

Palliative Care

2018 ACHD Guideline

Palliative CareRecommendation for Palliative Care


IIa B-NRDiscussion of end-of-life issues and advance directives can bebeneficial for patients with ACHD or their surrogates.

Shunt Lesions

2018 ACHD Guideline

Atrial Septal Defect

(Con’t.)

Recommendations for Atrial Septal Defect

COR LOE RecommendationsDiagnostic

I C-EOPulse oximetry at rest and during exercise is recommended forevaluation of adults with unrepaired or repaired ASD with residual shuntto determine the direction and magnitude of the shunt.

I B-NRCMR, CCT, and/or TEE are useful to evaluate pulmonary venousconnections in adults with ASD .

I B-NREchocardiographic imaging is recommended to guide percutaneous ASDclosure .


(Con’t.)

Therapeutic

I B-NRSR

In adults with isolated secundum ASD causing impaired functional capacity,right atrial and/or RV enlargement, and net left-to-right shunt sufficiently largeto cause physiological sequelae (e.g., pulmonary–systemic blood flow ratio[Qp:Qs] ≥1.5:1) without cyanosis at rest or during exercise, transcatheter orsurgical closure to reduce RV volume and improve exercise tolerance isrecommended, provided that systolic PA pressure is less than 50% of systolicsystemic pressure and pulmonary vascular resistance is less than one third ofthe systemic vascular resistance.

I B-NR

Adults with primum ASD, sinus venosus defect or coronary sinus defectcausing impaired functional capacity, right atrial and/or RV enlargement andnet left-to-right shunt sufficiently large to cause physiological sequelae (e.g.,Qp:Qs ≥1.5:1) without cyanosis at rest or during exercise, should be surgicallyrepaired unless precluded by comorbidities, provided that systolic PApressure is less than 50% of systemic pressure and pulmonary vascularresistance is less than one third of the systemic vascular resistance.

IIa C-LDSR

In asymptomatic adults with isolated secundum ASD, right atrial and RVenlargement, and net left-to-right shunt sufficiently large to causephysiological sequelae (e.g., Qp:Qs 1.5:1 or greater), without cyanosis at restor during exercise, transcatheter or surgical closure is reasonable to reduceRV volume and/or improve functional capacity, provided that systolic PApressure is less than 50% of systemic pressure and pulmonary vascularresistance is less than one third systemic resistance.


(Con’t.)

IIa C-LD

Surgical closure of a secundum ASD in adults is reasonable when aconcomitant surgical procedure is being performed and there is a net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs1.5:1 or greater) and right atrial and RV enlargement without cyanosis atrest or during exercise.

IIb B-NR

Percutaneous or surgical closure may be considered for adults with ASDwhen net left-to-right shunt (Qp:Qs) is 1.5:1 or greater, PA systolic pressureis 50% or more of systemic arterial systolic pressure, and/or pulmonaryvascular resistance is greater than one third of the systemic resistance.

III: Harm C-LD

ASD closure should not be performed in adults with PA systolic pressuregreater than two thirds systemic, pulmonary vascular resistance greaterthan two thirds systemic, and/or a net right-to-left shunt.

Secundum ASDSecundum ASD

Shunt direction

Left-to-right

Pulmonary vascular resistance <1/3 systemic vascular

resistance, PASP <50% systemic, right heart enlargement, AND shunt large

enough to cause physiologic sequelae (e.g.,Qp:Qs ≥1.5:1)

Surgical or device closure

(Class I)

Consultation with ACHD and PH experts


(Class IIb)

Yes

Pulmonary vascular resistance >1/3 systemic

vascular resistance, AND/OR PASP ≥50%

systemic


(Class IIa)

Functional impairment

No

Confirm PAH diagnosis (often requiring invasive

hemodynamic assessment)

(Class I)

PDE-5 inhibitors(Class IIa)

Bosentan(Class I)

Combination therapy*

(Class IIa)

Hemodynamic assessment

No closure (Class III: Harm)

Yes

Right-to-left (e.g., Eisenmenger

syndrome)

Anomalous Pulmonary Venous Connections

(Con’t.)

Recommendations for Anomalous Pulmonary Venous ConnectionsCOR LOE Recommendations

Diagnostic

I B-NR CMR or CTA is recommended for evaluation of partial anomalouspulmonary venous connection.

IIa B-NR Cardiac catheterization can be useful in adults with partial anomalouspulmonary venous connection to further define hemodynamics.

Anomalous Pulmonary Venous ConnectionsTherapeutic

I B-NR

Surgical repair is recommended for patients with partial anomalous pulmonaryvenous connection when functional capacity is impaired and RV enlargement ispresent, there is a net left-to-right shunt sufficiently large to cause physiologicalsequelae (e.g., Qp:Qs ≥1.5:1), PA systolic pressure is less than 50% systemicpressure and pulmonary vascular resistance is less than one third of systemicresistance.

I B-NR Repair of partial anomalous pulmonary venous connection is recommended atthe time of closure of a sinus venosus defect or ASD.

I B-NR

Repair of a scimitar vein is recommended in adults when functional capacity isimpaired, evidence of RV volume overload is present, there is a net left-to-rightshunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs ≥1.5:1), PAsystolic pressure is less than 50% systemic pressure and pulmonary vascularresistance is less than one third systemic.

IIa B-NR

Surgery can be useful for right- or left-sided partial anomalous pulmonaryvenous connection in asymptomatic adults with RV volume overload, net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs≥1.5:1), pulmonary pressures less than 50% systemic and pulmonary vascularresistance less than one third systemic.

IIa B-NR Surgery can be useful for repair of a scimitar vein in adults with evidence of RVvolume overload, with Qp:Qs 1.5:1 or greater.

Ventricular Septal DefectRecommendations for Ventricular Septal Defect

COR LOE RecommendationsTherapeutic

I B-NR

Adults with a VSD and evidence of left ventricular volume overload andhemodynamically significant shunts (Qp:Qs ≥1.5:1) should undergo VSDclosure, if PA systolic pressure is less than 50% systemic and pulmonaryvascular resistance is less than one third systemic.

IIa C-LDSurgical closure of perimembranous or supracristal VSD is reasonable inadults when there is worsening aortic regurgitation (AR) caused by VSD.

IIb C-LDSurgical closure of a VSD may be reasonable in adults with a history of IEcaused by VSD if not otherwise contraindicated.

IIb C-LD

Closure of a VSD may be considered in the presence of a net left-to-rightshunt (Qp:Qs ≥1.5:1) when PA systolic pressure is 50% or more thansystemic and/or pulmonary vascular resistance is greater than one thirdsystemic.

III: Harm C-LD

VSD closure should not be performed in adults with severe PAH with PAsystolic pressure greater than two thirds systemic, pulmonary vascularresistance greater than two thirds systemic and/or a net right-to-left shunt.

(Con’t.)

Hemodynamically Significant Ventricular Level ShuntHemodynamically significant

ventricular level shunt

Left-to-right

LV enlargement Qp:Qs ≥1.5:1,

PASP <50% systemic, AND pulmonary vascular resistance <1/3 systemic

Yes No

Progressive AR due to perimembranous or supracristal VSD

Yes No

Consult with ACHD and PH experts

(Class I)


(Class IIb)

Right-to-left (e.g., Eisenmenger

syndrome)

No closure (Class III: Harm)

Confirm PAH diagnosis (often requiring invasive

hemodynamic assessment)(Class I)

PDE-5 inhibitors(Class IIa)

Bosentan(Class I)

Combination therapy*

(Class IIa)

Shunt direction

Hemodynamicassessment

Pulmonary vascular resistance >1/3

systemic, AND/OR PASP ≥50%

systemic


(Class I)


(Class IIa)History of IE

Yes

Continued follow-up

No


(Class IIb)

Yes

Atrioventricular Septal DefectRecommendations for Atrioventricular Septal Defect


IIa C-EO Cardiac catheterization can be useful in adults with atrioventricular septal defect whenpulmonary hypertension is suspected.

Therapeutic

I C-LD Surgery for severe left atrioventricular valve regurgitation is recommended per GDMTindications for mitral regurgitation.

I C-EO

Surgery for primary repair of atrioventricular septal defect or closure of residual shuntsin adults with repaired atrioventricular septal defect is recommended when there is anet left-to-right shunt (Qp:Qs ≥1.5:1), PA systolic pressure less than 50% systemic andpulmonary vascular resistance less than one third systemic.

IIa C-EO

Operation for discrete LVOT obstruction in adults with atrioventricular septal defect isreasonable with a maximum gradient of 50 mm Hg or greater, a lesser gradient if HFsymptoms are present, or if concomitant moderate-to-severe mitral or AR are present.

IIb C-EO

Surgery for primary repair of atrioventricular septal defect or closure of residual shuntsin adults with repaired atrioventricular septal defect may be considered in the presenceof a net left-to-right shunt (Qp:Qs ≥1.5:1), if PA systolic pressure is 50% or moresystemic, and/or pulmonary vascular resistance is greater than one third systemic.

III: Harm C-LD

Surgery for primary repair of atrioventricular septal defect or closure of residual shuntsin adults with repaired atrioventricular septal defect should not be performed with PAsystolic pressure greater than two thirds systemic, pulmonary vascular resistancegreater than two thirds systemic, or a net right-to-left shunt.

Patent Ductus Arteriosus

(Con’t.)

Recommendations for Patent Ductus ArteriosusCOR LOE Recommendations

Diagnostic

I C-EOMeasurement of oxygen saturation should be performed in feet and bothhands in adults with a PDA to assess for the presence of right-to-left shunting.

IIa C-EOIn addition to the standard diagnostic tools, cardiac catheterization can beuseful in patients with PDA and suspected pulmonary hypertension.

Therapeutic

I C-LD

PDA closure in adults is recommended if left atrial or LV enlargement ispresent and attributable to PDA with net left-to-right shunt, PA systolicpressure less than 50% systemic and pulmonary vascular resistance less thanone third systemic.

IIb B-NRPDA closure in adults may be considered in the presence of a net left-to-rightshunt if PA systolic pressure is 50% or greater systemic, and/or pulmonaryvascular resistance is greater than one third systemic.

III: Harm C-LDPDA closure should not be performed in adults with a net right-to-left shuntand PA systolic pressure greater than two thirds systemic or pulmonaryvascular resistance greater than two thirds systemic.

Left-Sided Obstructive Lesions

2018 ACHD Guideline

Cor TriatriatumRecommendations for Cor Triatriatum


I B-NRAdults presenting with cor triatriatum sinister should be evaluated for othercongenital abnormalities, particularly ASD, VSD, and anomalous pulmonaryvenous connection.

IIa B-NRIn adults with prior repair of cor triatriatum sinister and recurrent symptoms,it is reasonable to evaluate for pulmonary vein stenosis.

Therapeutic

I B-NRSurgical repair is indicated for adults with cor triatriatum sinister forsymptoms attributable to the obstruction or a substantial gradient acrossthe membrane.

Congenital Mitral StenosisRecommendation for Congenital Mitral Stenosis


I B-NRAdults with congenital mitral stenosis or a parachute mitral valveshould be evaluated for other left-sided obstructive lesions.

Subaortic StenosisRecommendations for Subaortic Stenosis


IIb C-LD

Stress testing for adults with LVOT obstruction to determine exercisecapacity, symptoms, electrocardiographic changes, or arrhythmias may bereasonable in the presence of otherwise equivocal indications forintervention.

Therapeutic

I C-EOSurgical intervention is recommended for adults with subAS, a maximumgradient 50 mm Hg or more and symptoms attributable to the subAS.

I C-LDSurgical intervention is recommended for adults with subAS and less than 50mm Hg maximum gradient and HF or ischemic symptoms, and/or LV systolicdysfunction attributable to subAS.

IIb C-LDTo prevent the progression of AR, surgical intervention may be consideredfor asymptomatic adults with subAS and at least mild AR and a maximumgradient of 50 mm Hg or more.

Congenital Valvular Aortic StenosisRecommendations for Congenital Valvular Aortic Stenosis


I B-NR Adults with bicuspid aortic valve should be evaluated for coarctation of theaorta by clinical examination and imaging studies.

IIa B-NRIt is reasonable to screen first-degree relatives of patients with bicuspidaortic valve or unicuspid aortic valve with echocardiography for valvedisease and aortopathy.

Therapeutic

IIb B-NRIn adults with bicuspid aortic valve stenosis and a noncalcified valve withno more than mild AR meeting indications for intervention per GDMT, itmay be reasonable to treat with balloon valvuloplasty.

(Con’t.)

Turner SyndromeRecommendations for Turner Syndrome


I B-NRWomen with Turner syndrome should be evaluated for bicuspid aorticvalve, coarctation of the aorta, and enlargement of the ascending aorta.

Therapeutic

IIa B-NRProphylactic replacement of the aortic root or ascending aorta in adultswith Turner syndrome is reasonable when the aortic diameter is 2.5cm/m2 or greater.

Coarctation of the AortaRecommendations for Coarctation of the Aorta


I B-NRInitial and follow-up aortic imaging using CMR or CTA is recommended inadults with coarctation of the aorta, including those who have had surgical orcatheter intervention.

I C-EO Resting blood pressure should be measured in upper and lower extremities inall adults with coarctation of the aorta.

IIa C-LD Ambulatory blood pressure monitoring in adults with coarctation of the aortacan be useful for diagnosis and management of hypertension.

IIb B-NRScreening for intracranial aneurysms by magnetic resonance angiography orCTA may be reasonable in adults with coarctation of the aorta.

IIb C-LD Exercise testing to evaluate for exercise-induced hypertension may bereasonable in adults with coarctation of the aorta who exercise.

Therapeutic

I B-NRSurgical repair or catheter-based stenting is recommended for adults withhypertension and significant native or recurrent coarctation of the aorta.

I C-EO GDMT is recommended for treatment of hypertension in patients withcoarctation of the aorta.

IIb B-NRBalloon angioplasty for adults with native and recurrent coarctation of theaorta may be considered if stent placement is not feasible and surgicalintervention is not an option.

Right-Sided Lesions

2018 ACHD Guideline

Valvular Pulmonary StenosisRecommendations for Valvular Pulmonary Stenosis


I B-NR

In adults with moderate or severe valvular pulmonary stenosis andotherwise unexplained symptoms of HF, cyanosis from interatrial right-to-left communication, and/or exercise intolerance, balloon valvuloplasty isrecommended.

I B-NR

In adults with moderate or severe valvular pulmonary stenosis andotherwise unexplained symptoms of HF, cyanosis, and/or exerciseintolerance who are ineligible for or who failed balloon valvuloplasty,surgical repair is recommended.

IIa C-EOIn asymptomatic adults with severe valvular pulmonary stenosis,intervention is reasonable.

(Con’t.)

Isolated PR After Repair of PSRecommendations for Isolated PR After Repair of Pulmonary Stenosis


I C-EO

In symptomatic patients with moderate or greater PR resulting from treatedisolated pulmonary stenosis, with RV dilation or RV dysfunction,pulmonary valve replacement is recommended.

I C-EO

For asymptomatic patients with residual PR resulting from treatment ofisolated pulmonary stenosis with a dilated right ventricle, serial follow-upis recommended.

IIb C-EO

In asymptomatic patients with moderate or greater PR resulting fromtreatment of isolated pulmonary stenosis with progressive RV dilationand/or RV dysfunction, pulmonary valve replacement may be reasonable.

(Con’t.)

Isolated PR After Repair of PSIsolated PR after repair of PS

Assessment of PR severity and RV size/function

Symptoms†

Pulmonary valve replacement

(Class I)

Yes

Imaging and CPET

No

Progressive RV dilation and/or RV dysfunction

and/or progressive decrease in exercise capacity

Pulmonary valve replacement(Class IIb)

Yes No

Interval follow-up with ACHD cardiologist

Interval follow-up(Class I)

Moderate or greater PR and

RV enlargement*

Mild PR and RV enlargement*

Branch and Peripheral Pulmonary StenosisRecommendations for Branch and Peripheral PS


I B-NR For adults with peripheral or branch PS, ongoing surveillance isrecommended.

Therapeutic

IIa B-NR In adults with peripheral or branch PA stenosis, PA dilation and stentingcan be useful .

Double-Chambered Right VentricleRecommendations for Double-Chambered Right Ventricle


I C-LD

Surgical repair for adults with double-chambered right ventricle andmoderate or greater outflow obstruction is recommended in patientswith otherwise unexplained symptoms of HF, cyanosis, or exerciselimitation.

IIb C-LD Surgical repair for adults with double-chambered right ventricle with asevere gradient may be considered in asymptomatic patients.

Ebstein AnomalyRecommendations for Ebstein Anomaly


IIa B-NRIn adults with Ebstein anomaly, CMR can be useful to determine anatomy, RVdimensions, and systolic function .

IIa B-NRIn adults with Ebstein anomaly, TEE can be useful for surgical planning if TTEimages are inadequate to evaluate tricuspid valve morphology and function.

IIa B-NRElectrophysiological study with or without catheter ablation can be useful inthe diagnostic evaluation of adults with Ebstein anomaly and ventricularpreexcitation but without supraventricular tachycardia.

IIa B-NRIn adults with Ebstein anomaly, electrophysiological study (and catheterablation, if needed) is reasonable before surgical intervention on the tricuspidvalve even in the absence of preexcitation or supraventricular tachycardia.

(Con’t.)

Ebstein Anomaly

(Con’t.)

Therapeutic

I B-NR

Surgical repair or reoperation for adults with Ebstein anomaly andsignificant TR is recommended when one or more of the following arepresent: HF symptoms, objective evidence of worsening exercisecapacity, progressive RV systolic dysfunction by echocardiography orCMR.

I C-LDCatheter ablation is recommended for adults with Ebstein anomaly andhigh-risk pathway conduction or multiple accessory pathways.

IIa B-NR

Surgical repair or reoperation for adults with Ebstein anomaly andsignificant TR can be beneficial in the presence of progressive RVenlargement, systemic desaturation from right-to-left atrial shunt,paradoxical embolism, and/or atrial tachyarrhythmias.

IIb B-NR

Bidirectional superior cavopulmonary (Glenn) anastomosis at time ofEbstein anomaly repair may be considered for adults when severe RVdilation or severe RV systolic dysfunction is present, LV function ispreserved, and left atrial pressure and LV end diastolic pressure are notelevated.

Tetralogy of Fallot

(Con’t.)

Recommendations for TOF


I B-NRCMR is useful to quantify ventricular size and function, pulmonary valvefunction, pulmonary artery anatomy and left heart abnormalities in patientswith repaired TOF.

I B-NR Coronary artery compression testing is indicated before right ventricle–to-PAconduit stenting or transcatheter valve placement in repaired TOF.

IIa B-NR Programmed ventricular stimulation can be useful to risk stratify adults withTOF and additional risk factors for SCD.

IIa C-EO

In patients with repaired TOF, cardiac catheterization with angiography, ifindicated, is reasonable to assess hemodynamics when adequate data cannotbe obtained noninvasively in the setting of an arrhythmia, HF, unexplainedventricular dysfunction, suspected pulmonary hypertension or cyanosis.

Tetralogy of Fallot

Therapeutic

I B-NR

Pulmonary valve replacement (surgical or percutaneous) for relief ofsymptoms is recommended for patients with repaired TOF andmoderate or greater PR with cardiovascular symptoms not otherwiseexplained.

IIa B-NR

Pulmonary valve replacement (surgical or percutaneous) is reasonablefor preservation of ventricular size and function in asymptomaticpatients with repaired TOF and ventricular enlargement or dysfunctionand moderate or greater PR .

IIa B-NRPrimary prevention ICD therapy is reasonable in adults with TOF andmultiple risk factors for SCD.

IIb C-EOSurgical pulmonary valve replacement may be reasonable for adultswith repaired TOF and moderate or greater PR with other lesionsrequiring surgical interventions.

IIb C-EOPulmonary valve replacement, in addition to arrhythmia management,may be considered for adults with repaired TOF and moderate orgreater PR and ventricular tachyarrhythmia.

(Con’t.)

TOF repair with PR

Symptoms*

Any 2 of the following:

• Mild or moderate RV or LV systolic dysfunction• Severe RV dilation

(RVEDVI ≥160 mL/m2, orRVESVI ≥80 mL/m2, or RVEDV ≥2x LVEDV)

• RVSP due to RVOT obstruction ≥2/3 systemic pressure

• Progressive reduction in objective exercise

tolerance

Pulmonary valve replacement

(Class I)

Yes

Moderate or more PR

Follow-up with ACHD cardiologist

(Class I)

PR severity

Mild PR

No

Severely decreased LV or RV

systolic function

No

Evaluation by an ACHD cardiologist & advanced HF

team(Class I)

Yes

Pulmonary Valve Replacement in Patients With TOF Repair and PR

(Con’t.)

Yes

Pulmonary valve replacement(Class IIa)

No

Sustained tachyarrhythmias

Yes


Residual lesions requiring surgical

interventions


Follow-up with ACHD cardiologist

(Class I)

Yes No

No

Pulmonary Valve Replacement in Patients With TOF Repair and PR

Right Ventricle–to-Pulmonary Artery ConduitRecommendations for Right Ventricle–to-PA Conduit


I B-NR

Coronary artery compression testing with simultaneous coronary angiographyand high-pressure balloon dilation in the conduit is indicated before rightventricle–to-PA conduit stenting or transcatheter valve placement.

I B-NR

In patients with stented right ventricle–to-PA conduits and worsening PS or PR,evaluation for conduit complications should be performed, includingfluoroscopy to evaluate for stent fracture and blood cultures to assess for IE.

IIa C-LDIn adults with right ventricle–to-PA conduit and arrhythmia, congestive HF,unexplained ventricular dysfunction or cyanosis cardiac catheterization isreasonable to assess the hemodynamics.

Therapeutic

IIa B-NR

Right ventricle–to-PA conduit intervention is reasonable for adults with rightventricle–to-PA conduit and moderate or greater PR or moderate or greaterstenosis with reduced functional capacity or arrhythmia.

IIb B-NR

Right ventricle–to-PA conduit intervention may be reasonable for asymptomaticadults with right ventricle–to-PA conduit and severe stenosis or severeregurgitation with reduced RV ejection fraction or RV dilation.

Right Ventricle–to-Pulmonary Artery ConduitRecommendations for Right Ventricle–to-PA Conduit


I B-NR

Coronary artery compression testing with simultaneous coronary angiographyand high-pressure balloon dilation in the conduit is indicated before rightventricle–to-PA conduit stenting or transcatheter valve placement.

I B-NR

In patients with stented right ventricle–to-PA conduits and worsening PS or PR,evaluation for conduit complications should be performed, includingfluoroscopy to evaluate for stent fracture and blood cultures to assess for IE.

IIa C-LDIn adults with right ventricle–to-PA conduit and arrhythmia, congestive HF,unexplained ventricular dysfunction or cyanosis cardiac catheterization isreasonable to assess the hemodynamics.

Therapeutic

IIa B-NR

Right ventricle–to-PA conduit intervention is reasonable for adults with rightventricle–to-PA conduit and moderate or greater PR or moderate or greaterstenosis with reduced functional capacity or arrhythmia.

IIb B-NR

Right ventricle–to-PA conduit intervention may be reasonable for asymptomaticadults with right ventricle–to-PA conduit and severe stenosis or severeregurgitation with reduced RV ejection fraction or RV dilation.

Complex Lesions (Transposition of the Great Arteries)

2018 ACHD Guideline

Transposition of the Great Arteries With Atrial Switch

(Con’t.)

Recommendations for d-TGA With Atrial SwitchCOR LOE Recommendations

Diagnostic

I C-EOAmbulatory monitoring for bradycardia or sinus node dysfunction isrecommended for adults with d-TGA with atrial switch, especially if treatedwith beta blockers or other rate-slowing agents.

I C-EOAdults with d-TGA with atrial switch repair should undergo annual imagingwith either echocardiography or CMR to evaluate for common long-termcomplications of the atrial switch.

IIa C-LD

Assessment for a communication through the interatrial baffle or venousstenosis is reasonable for adults with d-TGA with atrial switch, particularly iftransvenous pacemaker/ICD implantation is considered or leads are alreadypresent.

Therapeutic

I B-NRGDMT with appropriate attention to the need for anticoagulation isrecommended to promptly restore sinus rhythm for adults with d-TGA withatrial switch repair presenting with atrial arrhythmia.

Transposition of the Great Arteries With Arterial Switch

(Con’t.)

Recommendations for d-TGA With Arterial SwitchCOR LOE Recommendations

Diagnostic

I C-LD

1. Baseline and serial imaging with either echocardiography or CMR shouldbe performed in adults with d-TGA with arterial switch who haveneoaortic dilation, valve dysfunction or PA or branch PA stenosis orventricular dysfunction.

I C-EO

1. Coronary revascularization for adults with d-TGA with arterial switchshould be planned by surgeons or interventional cardiologists withexpertise in revascularization in collaboration with ACHD providers toensure coronary and pulmonary artery anatomy are understood

IIa B-NR1. It is reasonable to perform anatomic evaluation of coronary artery

patency (catheter angiography, or CT or MR angiography) inasymptomatic adults with d-TGA with arterial switch.

IIa C-EO1. Physiological tests of myocardial perfusion for adults with d-TGA after

arterial switch can be beneficial for assessing symptoms suggestive ofmyocardial ischemia.

IIa C-EO 1. GDMT is reasonable to determine the need for coronary revascularizationfor adults with d-TGA after arterial switch.

Transposition of the Great Arteries With Arterial SwitchTherapeutic

IIa C-EO

GDMT is reasonable to determine indications for aortic valve replacementin adults with d-TGA after arterial switch with severe neoaortic valveregurgitation.

IIa C-EO

Catheter or surgical intervention for PS is reasonable in adults with d-TGA after arterial switch with symptoms of HF or decreased exercisecapacity attributable to PS.

(Con’t.)

Congenitally Corrected Transposition of the Great Arteries

Recommendations for Congenitally Corrected Transposition of the Great Arteries


IIa C-LDCMR is reasonable in adults with CCTGA to determine systemic RV dimensionsand systolic function.

Therapeutic

I B-NRTricuspid valve replacement is recommended for symptomatic adults withCCTGA and severe TR, and preserved or mildly depressed systemic ventricularfunction.

IIa C-LDTricuspid valve replacement is reasonable for asymptomatic adults with CCTGAand severe TR with dilation or mild dysfunction of the systemic ventricle.

IIb B-NR

Conduit intervention/replacement may be considered for adults with CCTGA andsymptomatic subpulmonary left ventricle–to-PA conduit dysfunction,recognizing that unloading the subpulmonary ventricle may have a detrimentalimpact on systemic atrioventricular valve function.

Complex Lesions {Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet Left Ventricle)}

2018 ACHD Guideline

Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet Left

Ventricle)Recommendations for Fontan Palliation of Single Ventricle Physiology


I C-LDNew presentation of an atrial tachyarrhythmia in adults with Fontan palliationshould be managed promptly and include prevention of thromboembolic eventsand consultation with an electrophysiologist with CHD expertise.

I C-EO Adults after Fontan palliation should be evaluated annually with eitherechocardiography or CMR.

I C-EO

Cardiac catheterization should be performed in adults before initial Fontansurgery or revision of a prior Fontan connection to assess suitability ofpreintervention hemodynamics for Fontan physiology or revision of a priorFontan connection.

I C-EONew onset or worsening atrial tachyarrhythmias in adults with single ventricleafter Fontan palliation should prompt a search for potential hemodynamicabnormalities, which may necessitate imaging and/or cardiac catheterization.

IIa B-R In adults with Fontan palliation, it is reasonable to encourage a regular exerciseprogram appropriate to their abilities.

(Con’t.)


Ventricle)

(Con’t.)

IIa B-RIn adults with Fontan palliation, it is reasonable to encourage a regularexercise program appropriate to their abilities.

IIa C-LDImaging of the liver (ultrasonography, CMR, CT) and laboratory evaluation ofliver function for fibrosis, cirrhosis, and/or hepatocellular carcinoma arereasonable in adults after Fontan palliation.

IIa C-EOIn adults after Fontan palliation, it is reasonable to perform biochemical andhematological testing on an annual basis especially for liver and renalfunction.

IIa C-LDCardiac catheterization can be useful to evaluate a symptomatic adult afterFontan palliation when noninvasive testing is insufficient to guide therapy.

IIa C-LDEvaluation for cardiac transplantation is reasonable in adults with Fontanpalliation and signs and symptoms of protein-losing enteropathy.

IIb C-EO

It may be reasonable to perform catheterization in asymptomatic adults afterFontan palliation to evaluate hemodynamics, oxygenation and cardiac functionto guide optimal medical, interventional and/or surgical therapy.


Ventricle)Therapeutic

I C-EOAnticoagulation with a vitamin K antagonist is recommended for adults withFontan palliation with known or suspected thrombus, thromboembolicevents, or prior atrial arrhythmia, and no contraindications to anticoagulation.

IIa C-LDCatheter ablation can be useful in adults after Fontan palliation with intra-atrial reentrant tachycardia or focal atrial tachycardia.

IIa C-LD

Fontan revision surgery, including arrhythmia surgery as indicated, isreasonable for adults with atriopulmonary Fontan connections with recurrentatrial tachyarrhythmias refractory to pharmacological therapy and catheterablation who have preserved systolic ventricular function and severe atrialdilation.

IIa B-RPulmonary vasoactive medications can be beneficial to improve exercisecapacity in adults with Fontan repair.

IIb B-NRAntiplatelet therapy or anticoagulation with a vitamin K antagonist may beconsidered in adults after Fontan palliation without known or suspectedthrombus, thromboembolic events, or prior arrhythmia.

IIb C-LDReoperation or intervention for structural/anatomic abnormalities in a Fontanpalliated patient with symptoms or with failure of the Fontan circulation maybe considered.

Severe PAH and Eisenmenger Syndrome

2018 ACHD Guideline

Severe PAHRecommendations for Severe PAH


I B-NR

Patients with ACHD with pulmonary vascular resistance 2.5 Wood units orgreater (≥4 Wood units/m2) should be assessed collaboratively by an ACHDcardiologist and an expert in pulmonary hypertension to develop amanagement plan.

I B-NRAdults with septal or great artery shunts should undergo periodic screeningfor pulmonary hypertension with TTE.

I B-NR

Cardiac catheterization to assess pulmonary vascular hemodynamics isrecommended for adults with septal or great artery shunts and clinicalsymptoms, signs, or echocardiographic findings suggestive of pulmonaryhypertension.

I B-NRIn adults with septal or great artery shunts, cardiac catheterization withhemodynamics (performed before or at time of closure) is beneficial to assesssuitability for closure.

I C-EOBNP, chest x-ray, 6-minute walk test, and cardiac catheterization are useful forinitial and follow-up evaluation of patients with ACHD with PAH.

(Con’t.)

Eisenmenger SyndromeRecommendations for Eisenmenger Syndrome


I C-EO

When evaluating adults with presumed Eisenmenger syndrome, cliniciansshould confirm diagnostic imaging and cardiac catheterization dataaccuracy and exclude other potential contributors to right-to-left shunting orpulmonary hypertension.

Therapeutic

I A Bosentan is beneficial in symptomatic adults with Eisenmenger syndromewith ASD or VSD.

IIa B-RIn symptomatic adults with Eisenmenger syndrome, bosentan and PDE-5inhibitors are reasonable in combination if symptomatic improvement doesnot occur with either medication alone.

IIa

C-EO Bosentan is a reasonable therapy to treat symptomatic adults withEisenmenger syndrome with 1 of the following: shunts other than ASD/VSD(e.g., PDA, aortopulmonary window) (Level of Evidence C-EO), or complexcongenital heart lesions or Down syndrome (Level of Evidence B-NR).

B-NR

IIa B-NRIt is reasonable to use PDE-5 inhibitors (e.g., sildenafil, tadalafil) to treatsymptomatic adults with Eisenmenger syndrome with ASD, VSD, or greatartery shunt.

Coronary Anomalies

2018 ACHD Guideline

Anomalous Coronary Artery Evaluation

Recommendations for Anomalous Coronary Artery EvaluationCOR LOE Recommendations

Diagnostic

I C-LDCoronary angiography, using catheterization, CT, or CMR, is recommendedfor evaluation of anomalous coronary artery.

I C-LDAnatomic and physiological evaluation should be performed in patientswith anomalous aortic origin of the left coronary from the right sinusand/or right coronary from the left sinus.

(Con’t.)

Anomalous Aortic Origin of Coronary Artery

(Con’t.)

Recommendations for Anomalous Aortic Origin of Coronary Artery


I B-NR

Surgery is recommended for AAOCA from the left sinus or AAOCA from the rightsinus for symptoms or diagnostic evidence consistent with coronary ischemiaattributable to the anomalous coronary artery.

IIa C-LDSurgery is reasonable for anomalous aortic origin of the left coronary artery fromthe right sinus in the absence of symptoms or ischemia.

IIa C-EO Surgery for AAOCA is reasonable in the setting of ventricular arrhythmias.

IIb B-NR

Surgery or continued observation may be reasonable for asymptomatic patientswith an anomalous left coronary artery arising from the right sinus or rightcoronary artery arising from the left sinus without ischemia or anatomic orphysiological evaluation suggesting potential for compromise of coronaryperfusion (e.g., intramural course, fish-mouth-shaped orifice, acute angle).

Anomalous Aortic origin of the Coronary Artery

(Con’t.)

*Surgical intervention to involve unroofing or coronary revascularization for patients with concomitant fixed obstruction.

Anomalous Coronary Artery Arising From the PA

Recommendations for Anomalous Coronary Artery Arising From the PA


I B-NRSurgery is recommended for anomalous left coronary artery from the PA.

I C-EOIn a symptomatic adult with anomalous right coronary artery from the PAwith symptoms attributed to the anomalous coronary, surgery isrecommended.

IIa C-EOSurgery for anomalous right coronary artery from the PA is reasonable in anasymptomatic adult with ventricular dysfunction or with myocardial ischemiaattributed to anomalous right coronary artery from the PA.

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