Guidelines for echocardiography

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


ACC/AHA/ASE 2003 Guideline Update for the Clinical Application of Echocardiography

Terminology

  • Class I: Conditions for which there is evidence and/or general agreement that a given procedure or treatment is useful and effective.
  • Class II: Conditions for which there is conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of a procedure or treatment.
  • Class IIa: Weight of evidence/opinion is in favor of usefulness/efficacy.
  • Class IIb: Usefulness/efficacy is less well established by evidence/opinion.
  • Class III: Conditions for which there is evidence and/or general agreement that the procedure/treatment is not useful/effective and in some cases may be harmful.

Evaluation of Patients With a Heart Murmur

  • Class I
    • A patient with a murmur and cardiorespiratory symptoms.
    • An asymptomatic patient with a murmur in whom clinical features indicate at least a moderate probability that the murmur is reflective of structural heart disease.
  • Class IIa
    • A murmur in an asymptomatic patient in whom there is a low probability of heart disease but in whom the diagnosis of heart disease cannot be reasonably excluded by the standard cardiovascular clinical evaluation.
  • Class III
    • In an asymptomatic adult, a heart murmur that has been identified by an experienced observer as functional or innocent.

Valvular Stenosis

  • Class I
    • Diagnosis; assessment of hemodynamic severity.
    • Assessment of LV and right ventricular (RV) size, function, and/or hemodynamics.
    • Re-evaluation of patients with known valvular stenosis with changing symptoms or signs.
    • Assessment of changes in hemodynamic severity and ventricular compensation in patients with known valvular stenosis during pregnancy.
    • Re-evaluation of asymptomatic patients with severe stenosis.
  • Class IIa
    • Assessment of the hemodynamic significance of mild to moderate valvular stenosis by stress Doppler echocardiography.
    • Re-evaluation of patients with mild to moderate aortic stenosis with LV dysfunction or hypertrophy even without clinical symptoms.
  • Class IIb
    • Re-evaluation of patients with mild to moderate aortic valvular stenosis with stable signs and symptoms.
    • Dobutamine echocardiography for the evaluation of patients with low-gradient aortic stenosis and ventricular dysfunction.
  • Class III
    • Routine re-evaluation of asymptomatic adult patients with mild aortic stenosis having stable physical signs and normal LV size and function.
    • Routine re-evaluation of asymptomatic patients with mild to moderate mitral stenosis and stable physical signs.

Native Valvular Regurgitation

  • Class I
    • Diagnosis; assessment of hemodynamic severity.
    • Initial assessment and re-evaluation (when indicated) of LV and RV size, function, and/or hemodynamics.
    • Re-evaluation of patients with mild to moderate valvular regurgitation with changing symptoms.
    • Re-evaluation of asymptomatic patients with severe regurgitation.
    • Assessment of changes in hemodynamic severity and ventricular compensation in patients with known valvular regurgitation during pregnancy.
    • Re-evaluation of patients with mild to moderate regurgitation with ventricular dilation without clinical symptoms.
    • Assessment of the effects of medical therapy on the severity of regurgitation and ventricular compensation and function when it might change medical management.
    • Assessment of valvular morphology and regurgitation in patients with a history of anorectic drug use, or the use of any drug or agent known to be associated with valvular heart disease, who are symptomatic, have cardiac murmurs, or have a technically inadequate auscultatory examination.
  • Class IIb
    • Re-evaluation of patients with mild to moderate mitral regurgitation without chamber dilation and without clinical symptoms.
    • Re-evaluation of patients with moderate aortic regurgitation without chamber dilation and without clinical symptoms.
  • Class III
    • Routine re-evaluation in asymptomatic patients with mild valvular regurgitation having stable physical signs and normal LV size and function.
    • Routine repetition of echocardiography in past users of anorectic drugs with normal studies or known trivial valvular abnormalities.

Mitral Valve Prolapse

  • Class I
    • Diagnosis; assessment of hemodynamic severity, leaflet morphology, and/or ventricular compensation in patients with physical signs of MVP.
  • Class IIa
    • To exclude MVP in patients who have been diagnosed but without clinical evidence to support the diagnosis.
    • To exclude MVP in patients with first-degree relatives with known myxomatous valve disease. **Risk stratification in patients with physical signs of MVP or known MVP.
  • Class III
    • Exclusion of MVP in patients with ill-defined symptoms in the absence of a constellation of clinical symptoms or physical findings suggestive of MVP or a positive family history.
    • Routine repetition of echocardiography in patients with MVP with no or mild regurgitation and no changes in clinical signs or symptoms.

Recommendations for Echocardiography in Infective

Endocarditis: Native Valves

  • Class I
    • Detection and characterization of valvular lesions, their hemodynamic severity, and/or ventricular compensation.
    • Detection of vegetations and characterizations of lesions in patients with congenital heart disease suspected of having infective endocarditis.
    • Detection of associated abnormalities (eg, abscesses, shunts).
    • Re-evaluation studies in complex endocarditis (eg, virulent organism, severe hemodynamic lesion, aortic valve involvement, persistent fever or bacteremia, clinical change, or symptomatic deterioration).
    • Evaluation of patients with high clinical suspicion of culture-negative endocarditis.
    • If TTE is equivocal, TEE evaluation of bacteremia, especially staphylococcus bacteremia and fungemia without a known source.
  • Class IIa
    • Evaluation of persistent nonstaphylococcus bacteremia without a known source.
    • Risk stratification in established endocarditis.
  • Class IIb
    • Routine re-evaluation in uncomplicated endocarditis during antibiotic therapy.
  • Class III
    • Evaluation of transient fever without evidence of bacteremia or new murmur.

Interventions for Valvular Heart Disease and Prosthetic Valves

  • Class I
    • Assessment of the timing of valvular intervention based on ventricular compensation, function, and/or severity of primary and secondary lesions.
    • Selection of alternative therapies for mitral valve disease (such as balloon valvuloplasty, operative valve repair, valve replacement).*
    • Use of echocardiography (especially TEE) in guiding the performance of interventional techniques and surgery (eg, balloon valvotomy and valve repair) for valvular disease.
    • Postintervention baseline studies for valve function (early) and ventricular remodeling (late).
    • Re-evaluation of patients with valve replacement with changing clinical signs and symptoms; suspected prosthetic dysfunction (stenosis, regurgitation) or thrombosis.*
  • Class IIa
    • Routine re-evaluation study after baseline studies of patients with valve replacements with mild to moderate ventricular dysfunction without changing clinical signs or symptoms.
  • Class IIb
    • Routine re-evaluation at the time of increased failure rate of a bioprosthesis without clinical evidence of prosthetic dysfunction.
  • Class III
    • Routine re-evaluation of patients with valve replacements without suspicion of valvular dysfunction and with unchanged clinical signs and symptoms.
    • Patients whose clinical status precludes therapeutic interventions.

Infective Endocarditis: Prosthetic Valves

  • Class I
    • Detection and characterization of valvular lesions, their hemodynamic severity, and/or ventricular compensation.*
    • Detection of associated abnormalities (eg, abscesses, shunts).*
    • Re-evaluation in complex endocarditis (eg, virulent organism, severe hemodynamic lesion, aortic valve involvement, persistent fever or bacteremia, clinical change, or symptomatic deterioration).*
    • Evaluation of suspected endocarditis and negative cultures.*
    • Evaluation of bacteremia without known source.*
  • Class IIa
    • Evaluation of persistent fever without evidence of bacteremia or new murmur.*
  • Class IIb
    • Routine re-evaluation in uncomplicated endocarditis during antibiotic therapy.*
  • Class III
    • Evaluation of transient fever without evidence of bacteremia or new murmur.

Echocardiography in Patients With Chest Pain

  • Class I
    • Diagnosis of underlying cardiac disease in patients with chest pain and clinical evidence of valvular, pericardial, or primary myocardial disease (see sections II, IV through VI, VIII, and IX).
    • Evaluation of chest pain in patients with suspected acute myocardial ischemia, when baseline ECG and other laboratory markers are nondiagnostic and when study can be obtained during pain or within minutes after its abatement (see section IV).
    • Evaluation of chest pain in patients with suspected aortic dissection (see section VIII).
    • Evaluation of patients with chest pain and hemodynamic instability unresponsive to simple therapeutic measures (see section XIII).
  • Class III
    • Evaluation of chest pain for which a noncardiac etiology is apparent.
    • Diagnosis of chest pain in a patient with electrocardiographic changes diagnostic of myocardial ischemia/infarction (see section IV).

Diagnosis of Acute Myocardial Ischemic Syndromes

  • Class I
    • Diagnosis of suspected acute ischemia or infarction not evident by standard means.
    • Measurement of baseline LV function.
    • Evaluation of patients with inferior myocardial infarction and clinical evidence suggesting possible RV infarction.
    • Assessment of mechanical complications and mural thrombus.*
  • Class IIa
    • Identification of location/severity of disease in patients with ongoing ischemia.
  • Class III
    • Diagnosis of acute myocardial infarction already evident by standard means.

Risk Assessment, Prognosis, and Assessment of Therapy in Acute Myocardial Ischemic Syndromes

  • Class I
    • Assessment of infarct size and/or extent of jeopardized myocardium.
    • In-hospital assessment of ventricular function when the results are used to guide therapy.
    • In-hospital or early postdischarge assessment of the presence/extent of inducible ischemia whenever baseline abnormalities are expected to compromise electrocardiographic interpretation.*
    • Assessment of myocardial viability when required to define potential efficacy of revascularization.†
  • Class IIa
    • In-hospital or early postdischarge assessment of the presence/extent of inducible ischemia in the absence of baseline abnormalities expected to compromise ECG interpretation.*
    • Re-evaluation of ventricular function during recovery when results are used to guide therapy.
    • Assessment of ventricular function after revascularization.
  • Class IIb
    • Assessment of late prognosis (greater than or equal to 2 years after acute myocardial infarction).
  • Class III
    • Routine re-evaluation in the absence of any change in clinical status.

Diagnosis and Prognosis of Chronic Ischemic Heart Disease

  • Class I
    • Diagnosis of myocardial ischemia in symptomatic individuals.*
    • Exercise echocardiography for diagnosis of myocardial ischemia in selected patients (those where ECG assessment is less reliable because of digoxin use, LVH or with more than 1 mm ST depression at rest on the baseline ECG, those with pre-excitation [Wolff-Parkinson-White] syndrome, complete left bundlebranchblock) with an intermediate pretest likelihood of CAD.
    • Assessment of global ventricular function at rest.
    • Assessment of myocardial viability (hibernating myocardium) for planning revascularization.†
    • Assessment of functional significance of coronary lesions (if not already known) in planning percutaneous transluminal coronary angioplasty.*
  • Class IIa
    • Prognosis of myocardial ischemia in selected patients(those in whom ECG assessment is less reliable) with the following ECG abnormalities: pre-excitation (Wolff-Parkinson-White) syndrome, electronically paced ventricular rhythm, more than 1 mm of ST depression at rest, complete left bundle-branch block.*
    • Detection of coronary arteriopathy in patients who have undergone cardiac transplantation.†
    • Detection of myocardial ischemia in women with an intermediate pretest likelihood of CAD.*
  • Class IIb
    • Assessment of an asymptomatic patient with positive results from a screening treadmill test.*
    • Assessment of global ventricular function with exercise.*
  • Class III
    • Screening of asymptomatic persons with a low likelihood of CAD.
    • Routine periodic reassessment of stable patients for whom no change in therapy is contemplated.
    • Routine substitution for treadmill exercise testing in patients for whom ECG analysis is expected to suffice.*
  • Exercise or pharmacological stress echocardiogram.

†Dobutamine stress echocardiogram.

Assessment of Interventions in Chronic Ischemic Heart Disease

  • Class I
    • Assessment of LV function when needed to guide institution and modification of drug therapy in patients with known or suspected LV dysfunction.
    • Assessment for restenosis after revascularization in patients with atypical recurrent symptoms.*
  • Class IIa
    • Assessment for restenosis after revascularization in patients with typical recurrent symptoms.*
    • Assessment of LV function in patients with previous myocardial infarction when needed to guide possible implantation of implantable cardioverter-defibrillator(ICD) in patients with known or suspected LV dysfunction.
  • Class III
    • Routine assessment of asymptomatic patients after revascularization.
  • Exercise or pharmacological stress echocardiography.

Patients With Dyspnea, Edema, or Cardiomyopathy

  • Class I
    • Assessment of LV size and function in patients with suspected cardiomyopathy or clinical diagnosis of heart failure.*
    • Edema with clinical signs of elevated central venous pressure when a potential cardiac etiology is suspected or when central venous pressure cannot be estimated with confidence and clinical suspicion of heart disease is high.*
    • Dyspnea with clinical signs of heart disease.
    • Patients with unexplained hypotension, especially in the intensive care unit.*
    • Patients exposed to cardiotoxic agents, to determine the advisability of additional or increased dosages.
    • Re-evaluation of LV function in patients with established cardiomyopathy when there has been a documented change in clinical status or to guide medical therapy.
    • Suspicion of hypertrophic cardiomyopathy based on abnormal physical examination, ECG, or family history.
    • Contrast echocardiographic assessment of myocardial infarct zone during interventional septal alcohol ablation studies.
  • Class IIb
    • Re-evaluation of patients with established cardiomyopathy when there is no change in clinical status but where the results might change management.
    • Re-evaluation of patients with edema when a potential cardiac cause has already been demonstrated.
  • Class III
    • Evaluation of LV ejection fraction in patients with recent (contrast or radionuclide) angiographic determination of ejection fraction.
    • Routine re-evaluation in clinically stable patients in whom no change in management is contemplated and for whom the results would not change management.
    • In patients with edema, normal venous pressure, and no evidence of heart disease.

Pericardial Disease

  • Class I
    • Patients with suspected pericardial disease, including effusion, constriction, or effusive-constrictive process.
    • Patients with suspected bleeding in the pericardial space (eg, trauma, perforation).
    • Follow-up study to evaluate recurrence of effusion or to diagnose early constriction. Repeat studies may be goal directed to answer a specific clinical question.
    • Pericardial friction rub developing in acute myocardial infarction accompanied by symptoms such as persistent pain, hypotension, and nausea.
  • Class IIa
    • Follow-up studies to detect early signs of tamponade in the presence of large or rapidly **Echocardiographic guidance and monitoring of pericardiocentesis.
  • Class IIb
    • Postsurgical pericardial disease, including postpericardiotomy syndrome, with potential for hemodynamic impairment.
    • In the presence of a strong clinical suspicion and nondiagnostic TTE, TEE assessment of pericardial thickness to support a diagnosis of constrictive pericarditis.
  • Class III
    • Routine follow-up of small pericardial effusion in clinically stable patients.
    • Follow-up studies in patients with cancer or other terminal illness for whom management would not be influenced by echocardiographic findings.
    • Assessment of pericardial thickness in patients without clinical evidence of constrictive pericarditis.
    • Pericardial friction rub in early uncomplicated myocardial infarction or in the early postoperative period after cardiac surgery.

Patients with Cardiac Masses and Tumors

  • Class I
    • Evaluation of patients with clinical syndromes and events that suggest an underlying cardiac mass.
    • Evaluation of patients with underlying cardiac disease known to predispose to mass formation for whom a therapeutic decision regarding surgery or anticoagulation

will depend on the results of echocardiography.

    • Follow-up or surveillance studies after surgical removal of masses known to have a high likelihood of recurrence (ie, myxoma).
    • Patients with known primary malignancies when echocardiographic surveillance for cardiac involvement is part of the disease staging process.
  • Class IIb
    • Screening persons with disease states likely to result in mass formation but for whom no clinical evidence for the mass exists.
  • Class III
    • Patients for whom the results of echocardiography will have no impact on diagnosis or clinical decision making.

Suspected Thoracic Aortic Disease

  • Class I
    • Aortic dissection, diagnosis, location, and extent.
    • Aortic aneurysm.*
    • Aortic intramural hematoma.
    • Aortic rupture.
    • Aortic root dilation in Marfan syndrome or other connective tissue syndromes.*
    • Degenerative or traumatic aortic disease with clinical atheroembolism.
    • Follow-up of aortic dissection, especially when complication or progression is suspected.
    • First-degree relative of a patient with Marfan syndrome or other connective tissue disorder for whichTTE is recommended* (see section XIIa).
  • Class IIa
    • Follow-up of a patient with surgically repaired aortic dissection.*

Pulmonary and Pulmonary Vascular Disease

  • Class I
    • Suspected pulmonary hypertension.
    • For distinguishing cardiac versus noncardiac etiology of dyspnea in patients in whom all clinical and laboratory clues are ambiguous.*
    • Follow-up of pulmonary artery pressures in patients with pulmonary hypertension to evaluate response to treatment.
    • Lung disease with clinical suspicion of cardiac involvement (suspected cor pulmonale).
  • Class IIa
    • Pulmonary emboli and suspected clots in the right atrium or ventricle or main pulmonary artery

branches.*

    • Measurement of exercise pulmonary artery pressure.
    • Patients being considered for lung transplantation or other surgical procedure for advanced lung disease.*
  • Class III
    • Lung disease without any clinical suspicion of cardiac involvement.
    • Re-evaluation studies of RV function in patients with chronic obstructive lung disease without a change in clinical status.

-TEE is indicated when TTE studies are not diagnostic.

Hypertension

  • Class I
    • When assessment of resting LV function, hypertrophy, or concentric remodeling is important in clinical decision making (see LV function).
    • Detection and assessment of functional significance of concomitant CAD by stress echocardiography (see coronary disease).
    • Follow-up assessment of LV size and function in patients with LV dysfunction when there has been a documented change in clinical status or to guide medical therapy.
  • Class IIa
    • Identification of LV diastolic filling abnormalities with or without systolic abnormalities.
    • Assessment of LV hypertrophy in a patient with borderline hypertension without LV hypertrophy on ECG to guide decision making regarding initiation of therapy. A limited goal-directed echocardiogram may be indicated for this purpose.
  • Class IIb
    • Risk stratification for prognosis by determination of LV performance.
  • Class III
    • Re-evaluation to guide antihypertensive therapy based on LV mass regression.
    • Re-evaluation in asymptomatic patients to assess LV function.

Patients With Neurological Events or Other Vascular Occlusive Events

  • Class I
    • Patients of any age with abrupt occlusion of a major peripheral or visceral artery.
    • Younger patients (typically less than 45 years) with cerebrovascular events.
    • Older patients (typically more than 45 years) with neurological events without evidence of cerebrovascular disease or other obvious cause.
    • Patients for whom a clinical therapeutic decision (eg, anticoagulation) will depend on the results of echocardiography.
  • Class IIa
    • Patients with suspicion of embolic disease and with cerebrovascular disease of questionable significance.
  • Class IIb
    • Patients with a neurological event and intrinsic cerebrovascular disease of a nature sufficient to cause the clinical event.
  • Class III
    • Patients for whom the results of echocardiography will not impact a decision to institute anticoagulant therapy or otherwise alter the approach to diagnosis or treatment.

Patients With Arrhythmias and Palpitations

  • Class I
    • Arrhythmias with clinical suspicion of structural heart disease.
    • Arrhythmia in a patient with a family history of a genetically transmitted cardiac lesion associated with arrhythmia such as tuberous sclerosis, rhabdomyoma, or hypertrophic cardiomyopathy.
    • Evaluation of patients as a component of the workup before electrophysiological ablative procedures.
  • Class IIa
    • Arrhythmia requiring treatment.
    • TEE or intracardiac ultrasound guidance of radiofrequency ablative procedures.
  • Class IIb
    • Arrhythmias commonly associated with, but without clinical evidence of, heart disease.
    • Evaluation of patients who have undergone radiofrequency ablation in the absence of complications. (In centers with established ablation programs, a postprocedural echocardiogram may not be necessary.)
    • Postoperative evaluation of patients undergoing the Maze procedure to monitor atrial function.
  • Class III
    • Palpitation without corresponding arrhythmia or other cardiac signs or symptoms.
    • Isolated premature ventricular contractions for which there is no clinical suspicion of heart disease.

Echocardiography Before Cardioversion

  • Class I
    • Patients requiring urgent (not emergent) cardioversion for whom extended precardioversion anticoagulation is not desirable.*
    • Patients who have had prior cardioembolic events thought to be related to intra-atrial thrombus.*
    • Patients for whom anticoagulation is contraindicated and for whom a decision about cardioversion will be influenced by TEE results.*
    • Patients for whom intra-atrial thrombus has beendemonstrated in previous TEE.*
    • Evaluation of patients for whom a decision concerning cardioversion will be impacted by knowledge of prognostic factors (such as LV function or coexistent mitral valve disease).
  • Class IIa
    • Patients with atrial fibrillation of less than 48 hours’ duration and other heart disease.*
  • Class IIb
    • Patients with atrial fibrillation of less than 48 hours’ duration and no other heart disease.*
    • Patients with mitral valve disease or hypertrophic cardiomyopathy who have been on long-term anticoagulation at therapeutic levels before cardioversion unless there are other reasons for anticoagulation (eg, prior embolus or known thrombus on previous TEE).*
    • Patients undergoing cardioversion from atrial flutter.*
  • Class III
    • Patients requiring emergent cardioversion.
    • Patients who have been on long-term anticoagulation at therapeutic levels and who do not have mitral valve disease or hypertrophic cardiomyopathy before cardioversion unless there are other reasons for anticoagulation (eg, prior embolus or known thrombus on previous TEE).*
    • Precardioversion evaluation of patients who have undergone previous TEE and with no clinical suspicion of a significant interval change.

Patient With Syncope

  • Class I
    • Syncope in a patient with clinically suspected heart disease.
    • Periexertional syncope.
  • Class IIa
    • Syncope in a patient in a high-risk occupation (eg,pilot).
  • Class IIb
    • Syncope of occult etiology with no findings of heart disease on history or physical examination.
  • Class III
    • Recurrent syncope in a patient in whom previous echocardiographic or other testing demonstrated a cause of syncope.
    • Syncope in a patient for whom there is no clinical suspicion of heart disease.
    • Classic neurogenic syncope.

Screen for the Presence of Cardiovascular Disease

  • Class I
    • Patients with a family history of genetically transmitted cardiovascular disease.
    • Potential donors for cardiac transplantation.
    • Patients with phenotypic features of Marfan syndrome or related connective tissue diseases.
    • Baseline and re-evaluations of patients undergoing chemotherapy with cardiotoxic agents.
    • First-degree relatives (parents, siblings, children) of patients with unexplained dilated cardiomyopathy in whom no etiology has been identified.
  • Class IIb
    • Patients with systemic disease that may affect the heart.
  • Class III
    • The general population.
    • Routine screening echocardiogram for participation in competitive sports in patients with normal cardiovascular history, ECG, and examination.

Critically Ill

  • Class I
    • The hemodynamically unstable patient.
    • Suspected aortic dissection (TEE).
  • Class III
    • The hemodynamically stable patient not expected to have cardiac disease.
    • Re-evaluation follow-up studies on hemodynamically stable patients.

Critically Injured

  • Class I
    • Serious blunt or penetrating chest trauma (suspected pericardial effusion or tamponade).
    • Mechanically ventilated multiple-trauma or chest trauma patient.
    • Suspected pre-existing valvular or myocardial disease in the trauma patient.
    • The hemodynamically unstable multiple-injury patient without obvious chest trauma but with a

mechanism of injury suggesting potential cardiac or aortic injury (deceleration or crush).

    • Widening of the mediastinum, postinjury suspected aortic injury (TEE).
    • Potential catheter, guidewire, pacer electrode, or pericardiocentesis needle injury with or without signs of tamponade.
  • Class IIa
    • Evaluation of hemodynamics in multiple-trauma or chest trauma patients with pulmonary artery catheter monitoring and data disparate with clinical situation.
    • Follow-up study on victims of serious blunt or penetrating trauma.
  • Class III
    • Suspected myocardial contusion in the hemodynamically stable patient with a normal ECG who has no abnormal cardiac/thoracic physical findings and/or lacks a mechanism of injury suggesting cardiovascular contusion.

Adult Patient With Congenital Heart Disease

  • Class I
    • Patients with clinically suspected congenital heart disease, as evidenced by signs and symptoms such as a murmur, cyanosis, or unexplained arterial desaturation,

and an abnormal ECG or radiograph suggesting congenital heart disease.

    • Patients with known congenital heart disease on follow-up when there is a change in clinical findings.
    • Patients with known congenital heart disease for whom there is uncertainty as to the original diagnosis or when the precise nature of the structural abnormalities

or hemodynamics is unclear.

    • Periodic echocardiograms in patients with known congenital heart lesions and for whom ventricular function and atrioventricular valve regurgitation must be followed (eg, patients with a functional single ventricle after Fontan procedure, transposition of the

great vessels after Mustard procedure, L-transposition and ventricular inversion, and palliative shunts).

    • Patients with known congenital heart disease for whom following pulmonary artery pressure is important(eg, patients with hemodynamically important, moderate, or large ventricular septal defects, atrial septal defects, single ventricle, or any of the above with an additional risk factor for pulmonary hypertension).
    • Periodic echocardiography in patients with repaired(or palliated) congenital heart disease with the following: change in clinical condition or clinical suspicion of residual defects, obstruction of conduits and baffles, LV or RV function that must be followed, or when

there is a possibility of hemodynamic progression or a history of pulmonary hypertension.

    • To direct interventional catheter valvotomy, radiofrequency ablation, and interventions in the presence of complex cardiac anatomy.
    • Identification of site of origin and initial course of coronary arteries (TEE may be indicated in some patients).*
  • Class IIb
    • A follow-up echocardiographic study, annually or once every 2 years, in patients with known hemodynamically significant congenital heart disease without evident change in clinical condition.
  • Class III
    • Multiple repeat echocardiography in patients with repaired patent ductus arteriosus, atrial septal defect, ventricular septal defect, coarctation of the aorta, or bicuspid aortic valve without change in clinical condition.
    • Repeat echocardiography in patients with known hemodynamically insignificant congenital heart

lesions (eg, small atrial septal defect, small ventricular septal defect) without a change in clinical condition.

Neonatal Echocardiography

  • Class I
    • Cyanosis, respiratory distress, congestive heart failure,or abnormal arterial pulses.
    • Chromosomal abnormality or major extracardiac abnormality associated with a high incidence of

coexisting cardiac abnormality.

    • Lack of expected improvement in cardiopulmonary status in a premature infant with a clinical diagnosis of pulmonary disease.
    • Systemic maternal disease associated with neonatal comorbidity
    • Loud or abnormal murmur or other abnormal cardiac finding in an infant.
    • Presence of a syndrome associated with cardiovascular disease and dominant inheritance or multiple affected family members.
    • Presence of a syndrome associated with heart disease,with or without abnormal cardiac findings, for which an urgent management decision is needed.
    • Cardiomegaly on chest radiograph.
    • Dextrocardia, abnormal pulmonary or visceral situs by clinical, electrocardiographic, or radiographic examination.
    • Arrhythmias or other abnormalities on standard ECG suggesting structural heart disease or peripartum myocardial injury.
    • Clinical suspicion of residual or recurrent abnormality,poor ventricular function, pulmonary

artery hypertension, thrombus, sepsis, or pericardial effusion after cardiovascular surgical therapy for congenital heart disease.

    • Re-evaluation after initiation or termination of medical therapy for pulmonary artery hypertension.
    • Re-evaluation during initiation or withdrawal of extracorporeal cardiopulmonary support.
    • Nonimmunologic fetal hydrops.
    • Follow-up assessment of a neonate with patent ductus arteriosus who has undergone medical or surgical intervention.
  • Class IIa
    • Short, soft murmur at the lower left sternal border in the neonate.
    • Failure to thrive in the absence of definite abnormal clinical findings.
    • Presence of a syndrome associated with a high incidence of congenital heart disease for which there are no abnormal cardiac findings and no urgency of management decisions.
  • Class III
    • History of nonsustained fetal ectopy in the absence of postpartum arrhythmias.
    • Acrocyanosis with normal upper-and-lower extremity pulsed oximetry oxygen saturations.

Echocardiography in the Infant, Child, and Adolescent

  • Class I
    • Atypical or pathological murmur or other abnormal cardiac finding in an infant or older child.
    • Cardiomegaly on chest radiograph.
    • Dextrocardia, abnormal pulmonary or visceral situs on clinical, electrocardiographic, or radiographic examination.
    • Patients with a known cardiac defect, to assess timing of medical or surgical therapy.
    • Selection, placement, patency, and monitoring of endovascular devices, as well as identification of intracardiac or intravascular shunting before, during,and after interventional cardiac catheterization.
    • Immediate assessment after percutaneous interventional cardiac catheterization procedure.
    • Immediate preoperative evaluation for cardiac surgery of a patient with a known cardiac defect to guide cardiac surgical management and inform the patient and family of risks of surgery.
    • Patient with known cardiac lesion and change in physical finding.
    • Postoperative congenital or acquired heart disease with clinical suspicion of residual or recurrent abnormality, poor ventricular function, pulmonary artery hypertension, thrombus, sepsis, or pericardial effusion.
    • Presence of a syndrome associated with cardiovascular disease and dominant inheritance or multiple affected family members (eg, Marfan syndrome or Ehlers-Danlos syndrome).
    • Patients with a family history of genetically transmitted myocardial disease, with or without abnormal cardiac finding.
    • Baseline and follow-up examinations of patients with neuromuscular disorders having known myocardial involvement.
    • Exercise-induced precordial chest pain or syncope.
  • Class IIb
    • Failure to thrive in the absence of definite abnormal clinical findings.
  • Class III
    • In a child or adolescent, an asymptomatic heart murmur identified by an experienced observer as functional or an insignificant cardiovascular abnormality.
    • In an otherwise asymptomatic child or adolescent,chest pain identified by an experienced observer as musculoskeletal in origin.

Recommendations for Echocardiography in Pediatric Patients With Arrhythmias/Conduction Disturbances

  • Class I
    • Arrhythmia in the presence of an abnormal cardiac finding.
    • Arrhythmia in a patient with a family history of a genetically transmitted cardiac lesion associated with arrhythmia, such as tuberous sclerosis or hypertrophic cardiomyopathy.
    • Complete atrioventricular block or advanced second degree atrioventricular block.
    • Complete or high-degree secondary atrioventricular block.
    • Arrhythmia requiring treatment.
  • Class IIa
    • Ventricular arrhythmia in a patient referred for evaluation for competitive sports.
    • Evidence of pre-excitation on ECG with symptoms.
  • Class IIb
    • Pre-excitation on ECG in the absence of abnormal cardiac findings.
    • Recurring arrhythmia not requiring treatment in the presence of normal findings on examination.
    • Examination immediately after radiofrequency ablation.
  • Class III
    • Sinus arrhythmia or isolated extrasystoles in a child with otherwise normal cardiac findings and no family history of a genetically transmitted abnormality associated with arrhythmia.

Pediatric Acquired Cardiovascular Disease

  • Class I
    • Baseline studies and re-evaluation as clinically indicated on all pediatric patients with suspected or documented Kawasaki disease, myopericarditis, HIV, or rheumatic fever.
    • After cardiac or cardiopulmonary transplant to monitor for signs of acute or chronic rejection, thrombus, and cardiac growth.
    • Baseline and re-evaluation examinations of patients receiving cardiotoxic chemotherapeutic agents.
    • Patients with clinical evidence of myocardial disease.
    • Patients with severe renal disease and/or systemic hypertension.
    • Donors undergoing evaluation for cardiac transplantation.
  • Class IIa
    • An acutely ill child with suspected bacterial sepsis or rickettsial disease.
  • Class IIb
    • Follow-up examinations after acute rheumatic fever in patients with normal cardiac findings.
    • A single late follow-up study after acute pericarditis with no evidence of recurrence or chronic pericardial disease.
  • Class III
    • Routine screening echocardiogram for participation in competitive sports in patients with normal cardiovascular examination.
    • Long-term follow-up studies in patients with Kawasaki disease who have no coronary abnormalities during the acute phase of the disease process.

Recommendations for Echocardiography in Pediatric Acquired Cardiopulmonary Disease

  • Class I
    • Any patient with clinical findings of pulmonary artery hypertension.
    • Re-evaluation after surgical intervention or initiation of oral and/or parenteral vasodilator therapy for pulmonary artery hypertension.
    • Re-evaluation during withdrawal of extracorporeal cardiopulmonary support.
  • Class IIa
    • Baseline study of patients with cystic fibrosis and no findings of cor pulmonale.

Pediatric Thromboembolic Disease States

  • Class I
    • Thromboembolic event in an infant, child, or adolescent.
    • Finding or family history of tuberous sclerosis.
    • Appearance of sepsis, cyanosis, or right-heart failure in a patient with a long-standing indwelling catheter.
    • Systemic embolization or acute-onset hypertension in a patient with right-to-left-shunting and an indwelling catheter.
    • Superior vena caval syndrome in the presence of central venous catheter.
  • Class IIb
    • Patient with indwelling catheter and fever but without evidence of pulmonary or systemic embolization.
  • Class III
    • Routine surveillance of asymptomatic patients with indwelling catheter.

Recommendations for TEE in Pediatric Patients

  • Class I
    • Any patient with congenital or acquired heart disease needing echocardiography when significant diagnostic information cannot be obtained by TTE.
    • Monitoring and guidance during cardiothoracic surgicalprocedures.
    • Guidance of catheter/device placement during interventional catheterization/radiofrequency ablation in patients with congenital heart disease.
    • Study of patients with intra-atrial baffle in whom the potential for thrombus is of concern because of elevated central venous pressures, atrial chamber dilation,increasing cyanosis, or the appearance of arrhythmia.
    • Patients with long-term placement of intravascular devices in whom thrombus or vegetation is suspected.
    • Patients with a prosthetic valve in whom thrombus or vegetation is suspected.
    • Any patient with suspected endocarditis and inadequate transthoracic acoustical window.
    • Patients with right atrial to pulmonary artery Fontan connection for identification of atrial thrombus.
  • Class IIa
    • Patients with lateral tunnel Fontan palliation.
  • Class III
    • Performing TEE in a patient who has not previously had careful study by TTE.
    • Patients with structural esophageal abnormality.

Fetal Echocardiography

  • Class I
    • Abnormal-appearing heart on general fetal ultrasound examination.
    • Fetal tachycardia, bradycardia, or persistent irregular rhythm on clinical or screening ultrasound examination.
    • Maternal/family risk factors for cardiovascular disease, such as a parent, sibling, or first-degree relative with congenital heart disease.
    • Maternal diabetes.
    • Maternal systemic lupus erythematosus.
    • Teratogen exposure during a vulnerable period.
    • Other fetal system abnormalities (including chromosomal).
    • Performance of transplacental therapy or presence of a history of significant but intermittent arrhythmia. Re-evaluation examinations are required in these conditions.
  • Class IIa
    • Fetal distress or dysfunction of unclear etiology.
  • Class IIb
    • Previous history of multiple fetal losses.
    • Multiple gestation.
  • Class III
    • Low-risk pregnancies with normal anatomic findings on ultrasound examination.
    • Occasional premature contractions without sustained tachycardia or signs of dysfunction or distress.
    • Presence of a noncardiovascular system abnormality when evaluation of the cardiovascular system will not alter either management decisions or fetal outcome.

Intraoperative Echocardiography

  • Class I
    • Evaluation of acute, persistent, and life-threatening hemodynamic disturbances in which ventricular function and its determinants are uncertain and have not responded to treatment.
    • Surgical repair of valvular lesions, hypertrophic obstructive cardiomyopathy, and aortic dissection with possible aortic valve involvement.
    • Evaluation of complex valve replacements requiring homografts or coronary reimplantation, such as the Ross procedure.
    • Surgical repair of most congenital heart lesions that require CPB.
    • Surgical intervention for endocarditis when preoperative testing was inadequate or extension to perivalvular tissue is suspected.
    • Placement of intracardiac devices and monitoring of their position during port-access and other cardiac surgical interventions.
    • Evaluation of pericardial window procedures in patients with posterior or loculated pericardial effusions.
  • Class IIa
    • Surgical procedures in patients at increased risk of myocardial ischemia, myocardial infarction, or hemodynamic disturbances.
    • Evaluation of valve replacement, aortic atheromatous disease, the Maze procedure, cardiac aneurysm repair, removal of cardiac tumors, intracardiac thrombectomy, and pulmonary embolectomy.
    • Detection of air emboli during cardiotomy, heart transplant operations, and upright neurosurgical procedures.
  • Class IIb
    • Evaluation of suspected cardiac trauma, repair of acute thoracic aortic dissection without valvular involvement, and anastomotic sites during heart and/or lung transplantation.
    • Evaluation of regional myocardial function during and after off-pump CABG procedures.
    • Evaluation of pericardiectomy, pericardial effusions, and pericardial surgery.
    • Evaluation of myocardial perfusion, coronary anatomy, or graft patency.
    • Dobutamine stress testing to detect inducible demand ischemia or to predict functional changes after myocardial revascularization.
    • Assessment of residual duct flow after interruption of patent ductus arteriosus (831).
  • Class III
    • Surgical repair of uncomplicated secundum atrial septal defect.



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