Congenital heart disease medical therapy

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Congenital heart disease Microchapters


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Differentiating Congenital heart disease from other Disorders

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis


Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings


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Prenatal Ultrasound

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Medical Therapy





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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Kalsang Dolma, M.B.B.S.[2]

Medical Therapy

Sometimes CHD improves with no treatment necessary. At other times the defect is so small and does not require any treatment. Most of the time CHD is serious and requires surgery and/or medications. Medications include diuretics, which aid the baby in eliminating water, salts, and digoxin and in strengthening the contraction of the heart. This slows the heartbeat and removes some fluid from tissues.

2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1]

Recommendations for Delivery of Care and Ensuring Access (DO NOT EDIT)[1]

Class I
"1. The focus of current healthcare access goals for adult congenital heart disease (ACHD) patients should include the following:"
"a. 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)"
"b. 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)"
"c. Enhanced education of adult cardiovascular specialists and pediatric cardiologists in the pathophysiology and management of ACHD patients. (Level of Evidence: C)"
"d. 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)"
"2. 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."
"a. Every academic adult cardiology/cardiac surgery center should have access to a regional ACHD center for consultation and referral. (Level of Evidence: C)"
"b. Each pediatric cardiology program should identify the ACHD center to which the transfer of patients can be made. (Level of Evidence: C)"
"c. All emergency care facilities should have an affiliation with a regional ACHD center. (Level of Evidence: C)"
"3. 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)"
"4. 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)"
"5. 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)"
"6. 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)"

Access to Care (DO NOT EDIT)[1]

Class I
"1. An individual primary caregiver or cardiologist without specific training and expertise in ACHD should manage the care of adults with complex and moderate CHD only in collaboration with level 2 or level 3 ACHD specialists.[2] (Level of Evidence: C) "
"2. For ACHD patients in the lowest-risk group, cardiac follow-up at a regional ACHD center is recommended at least once to formulate future needs for follow-up. (Level of Evidence: C)"
"3. 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)"
"4. 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.[2](Level of Evidence: C)"
"5. 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)"
"6. 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) "
"7. ACHD patients should be transferred to an ACHD center for urgent or acute care of cardiac problems. (Level of Evidence: C)"
"8. 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)"
"9. 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)"

Psychosocial Issues (DO NOT EDIT)[1]

Class I
"1. 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) "
"2. 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:"
"a. Demographic data, including physician contact. (Level of Evidence: C)"
"b. Description of CHD, surgeries, interventional procedures, and most recent diagnostic studies. (Level of Evidence: C)"
"c. Medications. (Level of Evidence: C)"
"3. 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: "
"a. Endocarditis prophylaxis measures (refer to "Recommendations for Infective Endocarditis" below). (Level of Evidence: C)"
"b. Exercise prescription, guidelines for exercise, and athletic participation for patients with CHD should reflect the published recommendations of the 36th Bethesda Conference report. [3](Level of Evidence: C)"
"c. Contraception and pregnancy information, including education regarding risk of CHD in offspring (for men and women). (Level of Evidence: C)"
"d. 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)"
"e. Recommended follow-up with cardiology. (Level of Evidence: C)"
"4. 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)"
"5. 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)"
"6. 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)"
"7. 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)"

Arrhythmia Diagnosis and Management (DO NOT EDIT)[1]

Class I
"1. 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) "
"2. 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)"
"3. 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)"
"4. 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)"
"5. 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
"1. 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) "
"2. 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).[4](Level of Evidence: B) "
Class IIb
"1. 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) "

Hematologic Problems (DO NOT EDIT)[1]

Class I
"1. 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
"1. Repeated routine phlebotomies are not recommended because of the risk of iron depletion, decreased oxygen-carrying capacity, and stroke. (Level of Evidence: C) "

General Health Issues for Cyanotic Patients (DO NOT EDIT)[1]

Class I
"1. Cyanotic patients should drink nonalcoholic and noncaffeinated fluids frequently on long-distance flights to avoid dehydration. (Level of Evidence: C) "
Class IIb
"1. Supplemental oxygenation may be considered for cyanotic patients during long-distance flights. (Level of Evidence: C) "


  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
  2. 2.0 2.1 Child JS, Collins-Nakai RL, Alpert JS, Deanfield JE, Harris L, McLaughlin P, Miner PD, Webb GD, Williams RG (2001). "Task force 3: workforce description and educational requirements for the care of adults with congenital heart disease". Journal of the American College of Cardiology. 37 (5): 1183–7. PMID 11300420. Retrieved 2012-11-06. Unknown parameter |month= ignored (help)
  3. Webb GD, Williams RG (2001). "Care of the adult with congenital heart disease: introduction". Journal of the American College of Cardiology. 37 (5): 1166. PMID 11300416. Retrieved 2012-11-06. Unknown parameter |month= ignored (help)
  4. Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO, Smith SC, Jacobs AK, Adams CD, Anderson JL, Buller CE, Creager MA, Ettinger SM, Faxon DP, Halperin JL, Hiratzka LF, Hunt SA, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Ornato JP, Page RL, Riegel B, Tarkington LG, Yancy CW (2008). "ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons". Journal of the American College of Cardiology. 51 (21): e1–62. doi:10.1016/j.jacc.2008.02.032. PMID 18498951. Retrieved 2012-11-06. Unknown parameter |month= ignored (help)

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