Congenital heart disease ACC/AHA guidelines for management of adults


 * Associate Editors-In-Chief: Priyamvada Singh, MBBS [mailto:psingh@perfuse.org]; ; Keri Shafer, M.D. [mailto:kshafer@bidmc.harvard.edu]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [mailto:kfeeney@perfuse.org]

Overview
ACC/AHA 2008 guidelines for the management of adults with congenital heart disease.(DO NOT EDIT)

The American College of Cardiology/American Heart Association (ACC/AHA) classification of the recommendations for patient evaluation and treatment (classes I-III) and the levels of evidence (A-C) are defined at the end of the "Major Recommendations" field.

Recommendations for Delivery of Care and Ensuring Access

Class I


 * The focus of current healthcare access goals for adult congenital heart disease (ACHD) patients should include the following:
 * Strengthening organization of and access to transition clinics for adolescents and young adults with congenital heart disease (CHD), including funding of allied healthcare providers to provide infrastructure comparable to that provided for children with CHD. (Level of Evidence: C)
 * Organization of outreach and education programs for patients, their families, and caregivers to recapture patients leaving pediatric supervisory care or who are lost to follow-up. Such programs can determine when and where further intervention is required. (Level of Evidence: C)
 * Enhanced education of adult cardiovascular specialists and pediatric cardiologists in the pathophysiology and management of ACHD patients. (Level of Evidence: C)
 * A liaison with regulatory agencies at the local, regional, state, and federal levels to create programs commensurate with the needs of this large cardiovascular population. (Level of Evidence: C)
 * Health care for ACHD patients should be coordinated by regional ACHD centers of excellence that would serve as a resource for the surrounding medical community, affected individuals, and their families (See "Table 2: Personnel and Services Recommended for Regional ACHD Centers" in the original guideline document).
 * Every academic adult cardiology/cardiac surgery center should have access to a regional ACHD center for consultation and referral. (Level of Evidence: C)
 * Each pediatric cardiology program should identify the ACHD center to which the transfer of patients can be made. (Level of Evidence: C)
 * All emergency care facilities should have an affiliation with a regional ACHD center. (Level of Evidence: C)
 * ACHD patients should carry a complete medical "passport" that outlines specifics of their past and current medical history, as well as contact information for immediate access to data and counsel from local and regional centers of excellence. (Level of Evidence: C)
 * Care of some ACHD patients is complicated by additional special needs, including but not restricted to intellectual incapacities or psychosocial limitations that necessitate the inclusion of designated healthcare guardians in all medical decision making. (Level of Evidence: C)
 * Every ACHD patient should have a primary care physician. To ensure and improve communication, current clinical records should be on file with the primary care physician and a local cardiovascular specialist, as well as at a regional ACHD center; patients should also have copies of relevant records. (Level of Evidence: C)
 * Every cardiovascular family caregiver should have a referral relationship with a regional ACHD center so that all patients have geographically accessible care. (Level of Evidence: C)

Recommendations for Access to Care

Class I


 * An individual primary caregiver or cardiologist without specific training and expertise in ACHD should manage the care of adults with complex and moderate CHD (See "Table 3: Types of Adult Congenital Heart Disease of Great Complexity" and "Table 4: Diagnoses in Adult Patients With Congenital Heart Disease of Moderate Complexity" in the original guideline document) only in collaboration with level 2 or level 3 ACHD specialists. (Child et al., 2001) (Level of Evidence: C)
 * For ACHD patients in the lowest-risk group (simple CHD; See "Table 5: Diagnoses in Adult Patients With Simple Congenital Heart Disease" in the original guideline document), cardiac follow-up at a regional ACHD center is recommended at least once to formulate future needs for follow-up. (Level of Evidence: C)
 * Frequent follow-up (generally every 12 to 24 months) at a regional ACHD center is recommended for the larger group of adults with complex and moderate CHD. A smaller group of adults with very complex CHD will require follow-up at a regional ACHD center at a minimum of every 6 to 12 months. (Level of Evidence: C)
 * Stabilized adult patients with CHD who require admission for urgent or acute care should be transferred to a regional ACHD center, except in some circumstances after consultation with the patient's primary level 2 or level 3 ACHD specialist. (Child et al., 2001) (Level of Evidence: C)
 * Diagnostic and interventional procedures, including imaging (i.e., echocardiography, magnetic resonance imaging [MRI], or computed tomography [CT]), advanced cardiac catheterization, and electrophysiology procedures for adults with complex and moderate CHD should be performed in a regional ACHD center with appropriate experience in CHD and in a laboratory with appropriate personnel and equipment. Personnel performing such procedures should work as part of a team with expertise in the surgical and transcatheter management of patients with CHD. (Level of Evidence: C)
 * Surgical procedures that require general anesthesia or conscious sedation in adults with moderate or complex CHD should be performed in a regional ACHD center with an anesthesiologist familiar with ACHD patients. (Level of Evidence: C)
 * ACHD patients should be transferred to an ACHD center for urgent or acute care of cardiac problems. (Level of Evidence: C)
 * Adult patients with complex or high-risk CHD should be transferred to an ACHD center for urgent or acute noncardiac problems. (Level of Evidence: C)
 * An ACHD specialist should be notified or consulted when a patient with simple or low-risk CHD is admitted to a non-ACHD center. (Level of Evidence: C)

Recommendations for Psychosocial Issues

Class I


 * Individual and family psychosocial screening (including knowledge assessment of cardiac disease and management; perceptions about health and the impact of CHD; social functioning with family, friends, and significant others; employment and insurability status; and screening for cognitive, mood, and psychiatric disorders) should be part of the care of ACHD patients. Advanced practice nurses, physician assistants, psychologists, and social workers should play an integral role in assessing and providing for the psychosocial needs of ACHD patients. (Level of Evidence: C)
 * Informational tools should be developed before transfer from adolescent to adult care and used for patient/family education regarding CHD, including the following elements, to be provided in electronic format:
 * Demographic data, including physician contact. (Level of Evidence: C)
 * Description of CHD, surgeries, interventional procedures, and most recent diagnostic studies. (Level of Evidence: C)
 * Medications. (Level of Evidence: C)
 * Additional health maintenance screening and information should be offered to ACHD patients as indicated during each visit to their ACHD healthcare provider, including the following:
 * Endocarditis prophylaxis measures (refer to "Recommendations for Infective Endocarditis" below). (Level of Evidence: C)
 * Exercise prescription, guidelines for exercise, and athletic participation for patients with CHD should reflect the published recommendations of the 36th Bethesda Conference report. (Webb & Williams, 2001) (Level of Evidence: C)
 * Contraception and pregnancy information, including education regarding risk of CHD in offspring (for men and women). (Level of Evidence: C)
 * General medical/dental preventive care (e.g., smoking cessation, weight loss/maintenance, hypertension/lipid screening, oral care, and substance abuse counseling). (Level of Evidence: C)
 * Recommended follow-up with cardiology. (Level of Evidence: C)
 * Vocational referral and health insurance information should be offered to ACHD patients during the transition period and refreshed at the time of their initial consultation in a tertiary referral center and intermittently as indicated by their social situation. (Level of Evidence: C)
 * A formal transition process should be used to provide optimal transfer of patients into ACHD care. This process should begin by 12 years of age and should be individualized on the basis of the patient's maturity level, with the goal being to transition and ultimately transfer the patient into adult care settings depending on the stability of the disease and psychosocial status. (Level of Evidence: C)
 * A psychological evaluation should be obtained if an adult's mental competency is in question and no appointed adult surrogate is available. (Level of Evidence: C)
 * All ACHD patients should be encouraged to complete an advance directive, ideally at a time during which they are not extremely ill or hospitalized, so that they can express their wishes thoughtfully in a less stressful setting and communicate these wishes to their families and caregivers. (Level of Evidence: C)

Recommendations for Infective Endocarditis

Class I


 * ACHD patients must be informed of their potential risk for infective endocarditis (IE) and should be provided with the American Heart Association (AHA) information card with instructions for prophylaxis. (Level of Evidence: B)
 * When patients with ACHD present with an unexplained febrile illness and potential IE, blood cultures should be drawn before antibiotic treatment is initiated to avoid delay in diagnosis due to "culture-negative" IE. (Level of Evidence: B)
 * Transthoracic echocardiography (TTE) should be performed when the diagnosis of native-valve IE is suspected. (Level of Evidence: B)
 * Transesophageal echocardiography (TEE) is indicated if TTE windows are inadequate or equivocal, in the presence of a prosthetic valve or material or surgically constructed shunt, in the presence of complex congenital cardiovascular anatomy, or to define possible complications of endocarditis (e.g., sepsis, abscess, valvular destruction or dehiscence, embolism, or hemodynamic instability). (Wilson et al., 2007) (Level of Evidence: B)
 * ACHD patients with evidence of IE should have early consultation with a surgeon with experience in ACHD because of the potential for rapid deterioration and concern about possible infection of prosthetic material. (Level of Evidence: C)

Class IIa


 * Antibiotic prophylaxis before dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa is reasonable in patients with CHD with the highest risk for adverse outcome from IE including those with the following indications:
 * Prosthetic cardiac valve or prosthetic material used for cardiac valve repair. (Level of Evidence: B)
 * Previous IE. (Level of Evidence: B)
 * Unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits. (Level of Evidence: B)
 * Completely repaired CHD with prosthetic materials, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure. (Level of Evidence: B)
 * Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device that inhibits endothelialization. (Level of Evidence: B)
 * It is reasonable to consider antibiotic prophylaxis against IE before vaginal delivery at the time of membrane rupture in select patients with the highest risk of adverse outcomes. This includes patients with the following indications:
 * Prosthetic cardiac valve or prosthetic material used for cardiac valve repair. (Level of Evidence: C)
 * Unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits. (Level of Evidence: C)

Class III


 * Prophylaxis against IE is not recommended for nondental procedures (such as esophagogastroduodenoscopy or colonoscopy) in the absence of active infection. (Level of Evidence: C)

Recommendations for Noncardiac Surgery

Class I


 * Basic preoperative assessment for ACHD patients should include systemic arterial oximetry, an ECG, chest x-ray, TTE, and blood tests for full blood count and coagulation screen. (Level of Evidence: C)
 * It is recommended that when possible, the preoperative evaluation and surgery for ACHD patients be performed in a regional center specializing in congenital cardiology, with experienced surgeons and cardiac anesthesiologists. (Level of Evidence: C)
 * Certain high-risk patient populations should be managed at centers for the care of ACHD patients under all circumstances, unless the operative intervention is an absolute emergency. High-risk categories include patients with the following:
 * Prior Fontan procedure. (Level of Evidence: C)
 * Severe pulmonary arterial hypertension (PAH). (Level of Evidence: C)
 * Cyanotic CHD. (Level of Evidence: C)
 * Complex CHD with residua such as heart failure, valve disease, or the need for anticoagulation. (Level of Evidence: C)
 * Patients with CHD and malignant arrhythmias. (Level of Evidence: C)
 * Consultation with ACHD experts regarding the assessment of risk is recommended for patients with CHD who will undergo noncardiac surgery. (Level of Evidence: C)
 * Consultation with a cardiac anesthesiologist is recommended for moderate- and high-risk patients. (Level of Evidence: C)

Recommendations for Pregnancy and Contraception

Class I


 * Patients with CHD should have consultation with an ACHD expert before they plan to become pregnant to develop a plan for management of labor and the postpartum period that includes consideration of the appropriate response to potential complications. This care plan should be made available to all providers. (Level of Evidence: C)
 * Patients with intracardiac right-to-left shunting should have fastidious care of intravenous lines to avoid paradoxical air embolus. (Level of Evidence: C)
 * Prepregnancy counseling is recommended for women receiving chronic anticoagulation with warfarin to enable them to make an informed decision about maternal and fetal risks. (Vitale et al., 1999; Sareli et al., 1989; van Driel et al., 2001) (Level of Evidence: B)

Class IIa


 * Meticulous prophylaxis for deep venous thrombosis, including early ambulation and compression stockings, can be useful for all patients with intracardiac right-to-left shunt. Subcutaneous heparin or low-molecular-weight heparin is reasonable for prolonged bed rest. Full anticoagulation can be useful for the high-risk patient. (Level of Evidence: C)

Class III


 * The estrogen-containing oral contraceptive pill is not recommended for ACHD patients at risk of thromboembolism, such as those with cyanosis related to an intracardiac shunt, severe PAH, or Fontan repair. (Level of Evidence: C)

Recommendations for Arrhythmia Diagnosis and Management

Class I


 * Complete and appropriate noninvasive testing, as well as clear knowledge of the specific anatomy and review of all surgical and procedural records, is recommended before electrophysiological testing or device placement is attempted in ACHD patients. (Level of Evidence: C)
 * Decisions regarding tachycardia management in ACHD patients should take into account the broad cardiovascular picture, particularly repairable hemodynamic issues that might favor a surgical or catheter-based approach to treatment. (Level of Evidence: B)
 * Catheter ablation procedures for ACHD patients should be performed at centers where the staff is experienced with the complex anatomy and distinctive arrhythmia substrates encountered in congenital heart defects. (Level of Evidence: B)
 * Pacemaker and device lead placement (or replacement) in ACHD patients should be performed at centers where the staff is familiar with the unusual anatomy of congenital heart defects and their surgical repair. (Level of Evidence: B)
 * Epicardial pacemaker and device lead placement should be performed in all cyanotic patients with intracardiac shunts who require devices. (Level of Evidence: B)

Class IIa


 * It is reasonable to recommend the use of an implantable cardioverter defibrillator for any patient who has had a cardiac arrest or experienced an episode of hemodynamically significant or sustained ventricular tachycardia (VT). (Level of Evidence: C)
 * Pacemaker implantation can be beneficial in ACHD patients with bradyarrhythmias and may be helpful in overdrive pacing in patients with difficult-to-control tachyarrhythmias (see ACC/AHA/HRS 2008 Guidelines for Device-Based therapy of Cardiac Rhythm Abnormalities). (Epstein et al., 2008) (Level of Evidence: B)

Class IIb


 * Pacemaker implantation may be beneficial for asymptomatic adult patients with resting heart rates of less than 40 beats per minute or abrupt pauses in excess of 3 seconds. (Level of Evidence: C)

Recommendations for Hematologic Problems

Class I


 * Indications for therapeutic phlebotomy are hemoglobin greater than 20 g per dL and hematocrit greater than 65%, associated with headache, increasing fatigue, or other symptoms of hyperviscosity in the absence of dehydration or anemia. (Level of Evidence: C)

Class III


 * Repeated routine phlebotomies are not recommended because of the risk of iron depletion, decreased oxygen-carrying capacity, and stroke. (Level of Evidence: C)

Recommendations for General Health Issues for Cyanotic Patients

Class I


 * Cyanotic patients should drink nonalcoholic and noncaffeinated fluids frequently on long-distance flights to avoid dehydration. (Level of Evidence: C)

Class IIb


 * Supplemental oxygenation may be considered for cyanotic patients during long-distance flights. (Level of Evidence: C)

Recommendations for Heart and Heart/Lung Transplantation

Class I


 * Patients with CHD and heart failure who may require heart transplantation should be evaluated and managed in tertiary care centers with medical and surgical personnel with experience and expertise in the management of both CHD and heart transplantation. (Level of Evidence: C)
 * Patients with CHD and heart or respiratory failure who may require lung or heart/lung transplantation should be evaluated and managed in tertiary care centers with medical and surgical personnel with experience and expertise in the management of CHD and lung or heart/lung transplantation. (Level of Evidence: C)''

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