Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease

HomeCirculationVol. 123, No. 22Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessResearch ArticlePDF/EPUBIndications for Cardiac Catheterization and Intervention in Pediatric Cardiac DiseaseA Scientific Statement From the American Heart Association Timothy F. Feltes, MD, FAHA, Emile Bacha, MD, Robert H. BeekmanIII, MD, FAHA, John P. Cheatham, MD, Jeffrey A. Feinstein, MD, MPH, Antoinette S. Gomes, MD, FAHA, Ziyad M. Hijazi, MD, MPH, FAHA, Frank F. Ing, MD, Michael de Moor, MBBCh, W. Robert Morrow, MD, Charles E. Mullins, MD, FAHA, Kathryn A. Taubert, PhD, FAHA and Evan M. Zahn, MD Timothy F. FeltesTimothy F. Feltes Search for more papers by this author , Emile BachaEmile Bacha Search for more papers by this author , Robert H. BeekmanIIIRobert H. BeekmanIII Search for more papers by this author , John P. CheathamJohn P. Cheatham Search for more papers by this author , Jeffrey A. FeinsteinJeffrey A. Feinstein Search for more papers by this author , Antoinette S. GomesAntoinette S. Gomes Search for more papers by this author , Ziyad M. HijaziZiyad M. Hijazi Search for more papers by this author , Frank F. IngFrank F. Ing Search for more papers by this author , Michael de MoorMichael de Moor Search for more papers by this author , W. Robert MorrowW. Robert Morrow Search for more papers by this author , Charles E. MullinsCharles E. Mullins Search for more papers by this author , Kathryn A. TaubertKathryn A. Taubert Search for more papers by this author and Evan M. ZahnEvan M. Zahn Search for more papers by this author and on behalf of the American Heart Association Congenital Cardiac Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Clinical Cardiology, and Council on Cardiovascular Radiology and Intervention Originally published2 May 2011https://doi.org/10.1161/CIR.0b013e31821b1f10Circulation. 2011;123:2607–2652Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: January 1, 2011: Previous Version 1 PreambleSince publication of the last American Heart Association (AHA) scientific statement on this topic in 1998,1 device technology, advances in interventional techniques, and an innovative spirit have opened the field of congenital heart therapeutic catheterization. Unfortunately, studies testing the safety and efficacy of catheterization and transcatheter therapy are rare in the field because of the difficulty in identifying a control population, the relatively small number of pediatric patients with congenital heart disease (CHD), and the broad spectrum of clinical expression. This has resulted in the almost exclusive “off-label” use of transcatheter devices, initially developed for management of adult diseases, for the treatment of CHD.The objective of the present writing group, which included representatives of the AHA and endorsements from the Society for Cardiovascular Angiography and Interventions and the American Academy of Pediatrics, was not only to provide the reader with an inventory of diagnostic catheterization and interventional treatment options but also to critically review the literature and formulate relative recommendations that are based on key opinion leader expertise and level of evidence. The writing group was charged with the task of performing an assessment of the evidence and giving a classification of recommendations and a level of evidence to each recommendation. The American College of Cardiology/AHA classification system was used, as follows:Classification of RecommendationsClass I: Conditions for which there is evidence and/or general agreement that a given procedure or treatment is beneficial, 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 a procedure/treatment is not useful/effective and in some cases may be harmful.Level of Evidence:Level of Evidence A: Data derived from multiple randomized clinical trials or meta-analyses.Level of Evidence B: Data derived from a single randomized trial or nonrandomized studies.Level of Evidence C: Only consensus opinion of experts, cases studies, or standard of care.For the practice recommendations provided in this statement, the classification of recommendations and the level of evidence determinations were taken from data available from clinical trials or registries about the usefulness/efficacy in different subpopulations. A recommendation with level of evidence B or C does not imply that the recommendation is weak. Many important clinical questions addressed in the indications do not lend themselves to clinical trials. Although randomized trials may not be available, there may be a very clear clinical consensus that a particular test or therapy is useful or effective. In 2003, the American College of Cardiology/AHA Task Force on Practice Guidelines developed a list of suggested phrases to use when writing recommendations. All recommendations have been written in full sentences that express a complete thought, such that a recommendation, even if separated and presented apart from the rest of the document (including headings above sets of recommendations), would still convey the full intent of the recommendation. It is hoped that this will increase readers' comprehension of the indications and allow queries at the individual recommendation level.In pediatrics, there are not likely to be any randomized controlled trials for devices, which posed a challenge for the writing group in compiling recommendations. Therefore, many of the indications are based on consensus opinion. In cases of strong consensus that an intervention be considered as standard-of-care practice with scientific evidence, interventions were designated as Class I indications. In these cases, the wording often used was “is indicated.” The writing group acknowledges that these recommendations may not exclude surgical management of the cardiac condition. Therefore, we opted to use the term “is indicated” versus “should be performed” in recognition of the fact that individual centers must assess their own capabilities and limitations. A device use may have a Class I indication but not necessarily be preferred at an individual center. Cardiac lesions, for instance, do not always exist in isolation, and patients with solitary or multiple lesions may be deemed better served by surgery. Cardiologists and surgeons must work together to formulate an interventional approach to patients based on individuality of the program. The writing group adhered to conflict of interest rules set by the AHA, and members were obliged to reveal all potential conflicts of interest. The writing group was chaired by a member of the Congenital Cardiac Defects Committee of the AHA who had no conflicts related to the topic of this scientific statement.1. Preparation for Cardiac CatheterizationCardiac catheterization has long served as the “gold standard” for the anatomic and physiological assessment of patients with CHD. Real-time fluoroscopy with contrast injection coupled with rapid digital angiography has provided the high-resolution images of the heart necessary for successful surgical management of these patients. The direct measurement of pressures within cardiac chambers and great vessels helps to stratify patients according to risk, assists in evaluation of medical therapy, and helps to indicate a need for intervention.Although it still serves a valuable role in this respect, cardiac catheterization has its limitations. Angiographic imaging for the most part is limited to 2 simultaneous imaging planes, and 3-dimensional reconstruction from angiographic data is still in development. The invasive nature of cardiac catheterization and common need for anesthesia forces the clinician to at least consider noninvasive alternatives for data collection. Fortunately, advances in noninvasive imaging have allowed cardiac catheterization to become increasingly a catheter-based therapeutic option rather than a diagnostic tool. Two-dimensional echocardiography and 3-dimensional imaging by echocardiography, magnetic resonance imaging (MRI), and computed tomography (CT) in many cases has replaced the need for cardiac catheterization.2–7 These same modes of noninvasive imaging can improve the success of catheter-based therapies and help to minimize the risks of invasive procedures by helping to target the data collection or therapy intended by catheterization. Thus, dialogue between the interventionalist, surgeon, and managing cardiologist is paramount to the provision of quality care for the patient with CHD.General Recommendations for Cardiac Catheterization PreparationClass IComplete cardiac echocardiographic imaging or alternative noninvasive imaging modes such as MRI or CT are indicated before invasive cardiac catheterization to facilitate planning of data collection and performance of an intervention (Level of Evidence: A).2. Indications for Diagnostic CatheterizationCardiac catheterization and angiography have transformed the care of children with CHD and have greatly increased the safety and efficacy of surgery for CHD. As a result of advances in noninvasive imaging as outlined in section 1, “Preparation for Cardiac Catheterization,” diagnostic catheterization is no longer indicated in the routine preoperative evaluation of most congenital defects, such as ventricular septal defects (VSDs), atrial septal defects (ASDs), atrioventricular canal, tetralogy of Fallot, double-outlet right ventricle (RV), coarctation of the aorta, hypoplastic left heart syndrome (HLHS), and other complex CHD. Details of anatomy, including situs, venous, and arterial connections; septal integrity; severity of valvular stenosis or insufficiency; size of pulmonary arteries; coronary artery origins; and aortic arch anatomy are easily established with echocardiography to the degree of certainty required for surgical intervention. Assessment of patient hemodynamics and, when necessary, assessment of anatomy by angiography should be accomplished before interventional catheterization to confirm congenital or acquired heart disease in infants and children.The availability of adequate noninvasive imaging is not uniform at all centers. At centers where MRI and CT imaging for congenital diagnosis are not state-of-the-art and where echocardiography does not provide sufficient detail, diagnostic catheterization is indicated. However, diagnostic catheterization should not be considered routine for diagnosis of congenital defects, and the performance of complete right- and left-sided heart studies may subject patients to unnecessary risk and exposure to radiation. All diagnostic catheterizations may lead to the necessity for an interventional procedure. As a consequence, the capability to proceed with the interventional procedure should be a requirement of the individual and institution that will perform the diagnostic portion of the catheterization. Individual centers must assess their abilities as they relate to this ability when determining which catheterization cases they might perform.It is not the purpose of the present statement to provide a comprehensive list of all indications for diagnostic catheterization; however, there are certain broad categories in which the need for diagnostic catheterization is well established in current practice. In most instances, these indications have been developed empirically, and there are no prospective studies, controlled or otherwise, that prove the superiority of catheterization over other diagnostic modalities. Therefore, most indications are supported by patient series, retrospective case reviews, or opinion of authorities. Diagnostic catheterization is or may be indicated in the circumstances outlined in the following section.Cardiac catheterization should be used in any circumstance in which the anatomy of a child with CHD is inadequately defined by noninvasive means. Although many congenital cardiac lesions are diagnosed and referred directly to surgery on the basis of noninvasive studies, there are some occasions, particularly in very complex lesions, on which more specific details about the anatomy or hemodynamics are necessary. The diagnostic cardiac catheterization remains the “final authority” for this definitive anatomic and hemodynamic information for many of the very complex lesions. Some circumstances that suggest the need for diagnostic catheterization include the following: High-flow or low-flow physiology associated with semilunar valve stenosis can lead to inaccurate gradients determined by noninvasive Doppler assessment. Combined aortic stenosis (AS) and insufficiency and pulmonary stenosis and insufficiency, especially with multiple levels of obstruction of both the systemic and pulmonary circulation, can be confusing when examined on the basis of echocardiographic studies alone. A diagnostic catheterization is necessary to determine true peak-to-peak gradient measurements, which still represent the standard by which the need for both surgical and catheter interventions is determined in some conditions. In many cases, noninvasive determination of pressure gradients is sufficient to proceed with surgical intervention; however, catheterization may be required to resolve conflicting noninvasive data.Low-flow lesions for which calculation of pulmonary arteriolar resistance can be misleading may be best studied by combining angiographic assessment of pulmonary artery anatomy and transit time with pulmonary artery pressures. Details of anatomy alone without corresponding pressure data can be misleading. This is often the case in the assessment of patients after cavopulmonary anastomosis and after Fontan completion. Diagnostic catheterization is useful in these patients in the evaluation for proceeding with completion of Fontan, revision of Fontan, or transplantation. In addition, assessment of a fenestrated Fontan with trial occlusion is useful in patients with excessive cyanosis and in making the decision to proceed with closing the fenestration. The ability to proceed with closure of fenestrations is required. Similar to the above category of patients, the postoperative patient who is not following an anticipated clinical course merits strong consideration for cardiac catheterization (further discussed in section 10, “Unique Catheter/Interventional Care Considerations”).There are many potential problems that can occur after surgery for congenital defects for which diagnostic catheterization is indicated because of the inability of noninvasive tests to provide the diagnosis. As noted above, the diagnostic procedure may become an interventional procedure, and cardiologists performing these studies should be prepared to proceed with intervention. Examples of some but certainly not all of the more common indications in this setting are provided below: In situations in which excessive desaturation occurs after a systemic-to–pulmonary artery shunt has been created, catheterization is useful to exclude branch pulmonary artery stenosis and shunt stenosis or occlusion. Such a situation may arise if the patient is too unstable to tolerate an MRI or MRI/magnetic resonance angiography (MRA), which can readily show pulmonary artery stenosis and shunt occlusion.If excessive systemic desaturation is present after cavopulmonary anastomosis, catheterization is indicated to exclude venovenous, venoatrial, or pulmonary arteriovenous malformations. The ability to proceed with venous collateral occlusion is a requirement in this setting. State-of-the-art MRA and CT angiography may help to detect and define the presence of these lesions.If an excessive residual left-to-right shunt is suspected after closure of a VSD that included atrioventricular canal repair, catheterization is indicated to calculate shunt size and severity.If the presence of excessive aortopulmonary collateral flow is suspected, diagnostic catheterization should be performed. The ability to proceed to occlusion of the offending systemic-to-pulmonary collateral arteries is required.When there is suspected RV outflow tract obstruction after surgery for tetralogy of Fallot or double-outlet RV and MRI is either not available or the patient is unable to tolerate MRI, diagnostic catheterization is indicated.If there is an unexplained need for extracorporeal membrane oxygenator support or failure to wean without obvious reasons, catheterization should be performed to identify problems and direct care.In the past, the utility of diagnostic catheterization for providing a definitive diagnosis in the setting of unexplained heart failure with diminished systolic function was limited. Endomyocardial biopsy with routine light microscopic examination is useful when positive findings of myocarditis are found, but in most cases, light microscopic findings of myocarditis or specific criteria by light and electron microscopy that suggest a specific diagnosis of cardiomyopathy are absent. The Dallas criteria for myocarditis are rarely met.8 MRI with assessment of myocardial edema and delayed enhancement is useful in this situation but does not provide a definitive diagnosis.9 Although there may be situations in which the clinical condition of the patient precludes catheterization, catheterization with biopsy should be considered whenever possible, and if performed, appropriate samples of myocardium should be obtained for histological diagnosis and viral polymerase chain reaction.10 In addition, in some instances, examination of biopsy material for abnormalities in contractile proteins and for other molecular abnormalities may enable a definitive diagnosis of cardiomyopathy. The latter should occur in the setting of a comprehensive evaluation of potential heritable causes of cardiomyopathy.Cardiac catheterization continues to play an important role in the assessment of pulmonary hypertension and its responsiveness to medical therapy. This may be the case in patients with CHD in whom an accurate assessment of pulmonary resistance is needed to make surgical and medical decisions. In addition, catheterization to assess resistance is essential in the management of patients with pulmonary vascular disease to assess response to pharmacological agents. Laboratories that perform catheterization to assess reactivity of the pulmonary vascular bed must have the capability to deliver inhaled nitric oxide or other pulmonary vasodilators.11A preoperative evaluation of pulmonary resistance is commonly performed in patients undergoing a staged Fontan approach to single-ventricle palliation. Some authorities do not recommend catheterization in this setting, but for most, catheterization to assess patients with single-ventricle physiology before stage 2 and 3 palliation remains common practice. However, the presence of elevated pulmonary arteriolar resistance, unsuspected pulmonary venous obstruction, and elevation of ventricular filling pressures all impact the outcome of second- and third-stage Fontan palliation.12 Catheterization in this setting is justified on this basis until prospective studies demonstrate the safety of its omission from preoperative assessment of these patients.In specific cases, such as patients with pulmonary valve atresia, the pulmonary arterial supply of the lungs may be complex. Catheterization in patients with pulmonary atresia–VSD or pulmonary atresia and complex ventricular anatomy is directed toward determining details of the aortopulmonary collateral supply to the lungs and the size of the true pulmonary arteries. Accurate characterization of dual supply by collaterals and true pulmonary arteries versus single supply by collaterals is important in planning the surgical approach to be used.13,14 The ability to perform selective catheterization of collaterals, balloon occlusion angiography, and pulmonary venous wedge angiography is essential. Noninvasive imaging such as MRI/MRA may be helpful in delineating these vessels.Likewise, the coronary artery anatomy in patients with pulmonary valve atresia and an intact ventricular septum can influence the success of the intervention (catheter-based or surgical) and patient survival. Although the presence of RV-to–coronary artery fistulae in patients with pulmonary atresia and severe RV hypoplasia can be recognized with echocardiography, an RV-dependent coronary circulation cannot. The exclusion of RV dependence of the coronary circulation is essential before procedures are performed to decompress the RV and establish RV-to–pulmonary artery continuity either through a transcatheter or surgical approach. Catheterization for this indication requires the ability to perform RV angiography in the hypoplastic RV, aortic angiography, and in some instances selective coronary angiography.15,16Diagnostic catheterization is indicated in the evaluation of patients for listing for cardiac transplantation in most instances, both in patients with congenital heart defects and in those with cardiomyopathy. Catheterization is indicated for endomyocardial biopsy, determination of filling pressures, and assessment of pulmonary resistance.17 Many patients are clinically compromised, and catheterization may need to be delayed or omitted. Catheterization is part of a multimodality evaluation and should be used in combination with echocardiography, MRI, and multislice CT. Likewise, situations may exist in which prior catheterization data suffice for transplantation listing (eg, the postoperative patient being listed who had a preoperative catheterization).Graft vasculopathy in cardiac transplant patients is a reflection of chronic rejection and occurs eventually in most patients. Graft vasculopathy is less common in children, although with time, most will develop some degree of coronary disease.18 Coronary angiography is performed routinely in the assessment of heart transplant patients to monitor for graft vasculopathy. It is well recognized that coronary angiography is an insensitive method for the detection of graft vasculopathy; it is usually only positive in the setting of moderate to severe disease. Intravascular ultrasound, however, is highly sensitive in detecting vasculopathy even in its earliest stages.19 Although intravascular ultrasound is established as the definitive procedure in adults, its use in children has been limited. Several reports have demonstrated that intravascular ultrasound can be performed safely in children >6 years of age.20,21 The addition of intravascular ultrasound to surveillance in pediatric heart transplantation programs has been limited by inexperience and lack of training and, in part, because of the size of the catheters and sheaths that are required. Yet intravascular ultrasound should be used increasingly as part of the routine annual or semiannual evaluation of pediatric heart transplant recipients, especially given the progressive nature of coronary disease, the potential for sudden death or need for retransplantation, and increasing evidence that changing or augmenting immunotherapy may slow the process of intimal proliferation in the early stages of graft vasculopathy.Despite promising new diagnostic methods, such as surveillance that includes biomarkers (brain natriuretic peptide), catheterization with endomyocardial biopsy is the mainstay of rejection identification. Endomyocardial biopsy does not diagnose rejection in all cases of unexplained deterioration in graft function but remains the “gold standard.” Samples obtained at catheterization should be studied for both cellular and antibody-mediated rejection. Criteria for the diagnosis of both are well developed at this time, including the International Society for Heart and Lung Transplantation revised criteria for cellular rejection and newly published criteria for diagnosis of humoral rejection.22,23 Diagnosis of antibody-mediated rejection by biopsy requires both light microscopic findings and fluorescent microscopy for recognition of deposition of complement fragments C4D and C3D.Risks/ComplicationsCardiac catheterizations are not without risk to the patient. The following is a listing of the more common complications. The reader is referred to one of the cited references for more information.24–26Exposure to ionized radiation (decreasing with newer equipment)Risk of general anesthesia (when used)Hypothermia (especially in small infants)Aggravation of hypoxiaArrhythmias (temporary instability or even permanent, as in heart block)Vascular injury/perforations/tearsCardiac perforationCardiac valve injuryBlood loss that requires transfusionAllergic reactions to contrast, drugs, or anestheticsRenal insufficiency caused by contrast materialDiffuse central nervous system injuryStrokeDeathRecommendations for Diagnostic CatheterizationClass IIt is recommended that hemodynamic and anatomic data be obtained (via angiography when necessary) at the time of a planned interventional cardiac catheterization (Level of Evidence: A).It is recommended that cardiac catheterization be used to assess pulmonary resistance and reversibility of pulmonary hypertension in patients with CHD or primary pulmonary hypertension when accurate assessment of pulmonary resistance is needed to make surgical and medical decisions (Level of Evidence: B).Cardiac catheterization is indicated in patients with complex pulmonary atresia for the detailed characterization of lung segmental pulmonary vascular supply, especially when noninvasive imaging methods incompletely define pulmonary artery anatomy (Level of Evidence: B).Cardiac catheterization is indicated in determination of coronary circulation in pulmonary atresia with intact septum (Level of Evidence: B).Cardiac catheterization is indicated in patients being assessed for cardiac transplantation unless the patient's risk for catheterization outweighs the potential benefit (Level of Evidence: C).Cardiac catheterization is recommended for surveillance of graft vasculopathy after cardiac transplantation (Level of Evidence: B).Class IIaIt is reasonable to perform a cardiac catheterization to determine pulmonary pressure/resistance and transpulmonary gradient in palliated single-ventricle patients before a staged Fontan procedure (Level of Evidence: B).Cardiac catheterization is reasonable in any CHD patient in whom complete diagnosis cannot be obtained by noninvasive testing or in whom such testing yields incomplete information (Level of Evidence: C).Cardiac catheterization is reasonable for the assessment of cardiomyopathy or myocarditis (Level of Evidence: B).Cardiac catheterization is reasonable for the assessment of coronary circulation in some cases of Kawasaki disease in which coronary involvement is suspected or requires further delineation or in the assessment of suspected congenital coronary artery anomalies (Level of Evidence: B).Cardiac catheterization is reasonable to perform for the assessment of anatomy and hemodynamics in postoperative cardiac patients when the early postoperative course is unexpectedly complicated and noninvasive imaging techniques (eg, MRA, CT angiography) fail to yield a clear explanation (Level of Evidence: C).3. Opening of Atrial Communications3.1. Transseptal TechniquesAn atrial transseptal approach is indicated whenever access to the left side of the heart, and in particular the left atrium, is desired and a preexisting communication between the right and left heart is not present or, when present, cannot be crossed readily from the right side of the heart. An atrial transseptal puncture/perforation from the right atrium into the left atrium provides dependable, direct access to the left atrium in the presence of a previously intact atrial septum. With the use of an atrial transseptal approach in the investigation of the entire left side of the heart, the potential for further compromise of an artery from a retrograde study is reduced or even eliminated, because most of the “left heart” information can be obtained by way of the transseptal procedure while arterial pressure is monitored simultaneously through a small-caliber, indwelling arterial line.27 In the event a retrograde study is necessary for specific aortic root or arterial information, collection of data from the left side of the heart by the transseptal approach may allow the retrograde study to be performed with a smaller-diameter catheter and requires a much shorter arterial cannulation time. For assessment of mitral valve disease, the transseptal approach may be used. Although the widespread use of pulmonary capillary wedge pressure with simultaneous left ventricular end-diastolic pressure monitoring usually proves sufficient for the assessment of mitral valve disease, atrial pressures obtained by the transseptal approach are generally of higher fidelity and therefore provide optimal waveforms. The final decision about this approach rests with the experienced interventionalist.After the performance of complex congenital heart surgical repairs, such as a Mustard or Senning venous switch procedure for transposition of the great arteries or the lateral tunnel Fontan procedure for single ventricle, the most reasonable access to critical locations in the pulmonary venous circulation of these patients is by a transsept