Eisenmenger’s syndrome ACC/AHA guidelines

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

Associate Editor-In-Chief: Priyamavada Singh, MBBS [[2]]

Assistant Editor-In-Chief: Kristin Feeney, B.S. [[3]]

Eisenmenger's syndrome ACC/AHA guidelines

Major Recommendations (DONOT EDIT)

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The American College of Cardiology/American Heart Association (ACC/AHA) classification of the recommendations for patient evaluation and treatment (classes I-III)

Major Recommendations

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 Evaluation of the Patient With Congenital Heart Disease–Pulmonary Arterial Hypertension

Class I

• Care of adult patients with congenital heart disease (CHD)-related pulmonary arterial hypertension (PAH) should be performed in centers that have shared expertise and training in both adult congenital heart disease (ACHD) and PAH. (Level of Evidence: C)
• The evaluation of all ACHD patients with suspected PAH should include noninvasive assessment of cardiovascular anatomy and potential shunting, as detailed below:
  • Pulse oximetry, with and without administration of supplemental oxygen, as appropriate. (Level of Evidence: C)
  • Chest x-ray. (Level of Evidence: C)
  • Electrocardiogram (ECG). (Level of Evidence: C)
  • Diagnostic cardiovascular imaging via transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), magnetic resonance imaging (MRI), or computed tomography (CT) as appropriate. (Level of Evidence: C)
  • Complete blood count and nuclear lung scintigraphy. (Level of Evidence: C)
• If PAH is identified but its causes are not fully recognized, additional testing should include the following:
  • Pulmonary function tests with volumes and diffusion capacity (diffusing capacity of the lung for carbon monoxide). (Level of Evidence: C)
  • Pulmonary embolism–protocol CT with parenchymal lung windows. (Level of Evidence: C)
  • Additional testing as appropriate to rule out contributing causes of PAH. (Level of Evidence: C)
  • Cardiac catheterization at least once, with potential for vasodilator testing or anatomic intervention, at a center with expertise in catheterization, PAH, and management of CHD-PAH. (Level of Evidence: C)

Class IIa

• It is reasonable to include a 6-minute walk test or similar nonmaximal cardiopulmonary exercise test as part of the functional assessment of patients with CHD-PAH. (Level of Evidence: C)

Management Strategies

Recommendations for Medical Therapy of Eisenmenger Physiology

Class I

• It is recommended that patients with Eisenmenger syndrome avoid the following activities or exposures, which carry increased risks:
  • Pregnancy. (Level of Evidence: B)
  • Dehydration. (Level of Evidence: C)
  • Moderate and severe strenuous exercise, particularly isometric exercise. (Level of Evidence: C)
  • Acute exposure to excessive heat (e.g., hot tub or sauna). (Level of Evidence: C)
  • Chronic high-altitude exposure, because this causes further reduction in oxygen saturation and increased risk of altitude-related cardiopulmonary complications (particularly at an elevation greater than 5000 feet above sea level). (Level of Evidence: C)
  • Iron deficiency. (Level of Evidence: B)
  • Patients with Eisenmenger syndrome should seek prompt therapy for arrhythmias and infections. (Level of Evidence: C)
  • Patients with Eisenmenger syndrome should have hemoglobin, platelet count, iron stores, creatinine, and uric acid assessed at least yearly. (Level of Evidence: C)
  • Patients with Eisenmenger syndrome should have assessment of digital oximetry, both with and without supplemental oxygen therapy, at least yearly. The presence of oxygen-responsive hypoxemia should be investigated further. (Level of Evidence: C)
  • Exclusion of air bubbles in intravenous tubing is recommended as essential during treatment of adults with Eisenmenger syndrome. (Level of Evidence: C)
  • Patients with Eisenmenger syndrome should undergo noncardiac surgery and cardiac catheterization only in centers with expertise in the care of such patients. In emergent or urgent situations in which transportation is not feasible, consultation with designated caregivers in centers with expertise in the care of patients with Eisenmenger syndrome should be performed and sustained throughout care. (Level of Evidence: C)

Class IIa

• All medications given to patients with Eisenmenger physiology should undergo rigorous review for the potential to change systemic blood pressure, loading conditions, intravascular shunting, and renal or hepatic flow or function. (Level of Evidence: C)
• Pulmonary vasodilator therapy can be beneficial for patients with Eisenmenger physiology because of the potential for improved quality of life. (Level of Evidence: C)

Key Issues to Evaluate and Follow-Up

Recommendations for Reproduction

Class I

• Women with severe CHD-PAH, especially those with Eisenmenger physiology, and their partners should be counseled about the absolute avoidance of pregnancy in view of the high risk of maternal death, and they should be educated regarding safe and appropriate methods of contraception. (Level of Evidence: B)
• Women with CHD-PAH who become pregnant should:
  • Receive individualized counseling from cardiovascular and obstetric caregivers collaborating in care and with expertise in management of CHD-PAH. (Level of Evidence: C)
  • Undergo the earliest possible pregnancy termination after such counseling. (Level of Evidence: C)
  • Surgical sterilization carries some operative risk for women with CHD-PAH but is a safer option than pregnancy. In view of advances in minimally invasive techniques, the risks and benefits of sterilization modalities should be discussed with an obstetrician experienced in management of high-risk patients, as well as with a cardiac anesthesiologist. (Level of Evidence: C)

Class IIb

• Pregnancy termination in the last 2 trimesters of pregnancy poses a high risk to the mother. It may be reasonable, however, after the risks of termination are balanced against the risks of continuation of the pregnancy. (Level of Evidence: C)

Class III

• Pregnancy in women with CHD-PAH, especially those with Eisenmenger physiology, is not recommended and should be absolutely avoided in view of the high risk of maternal mortality. (Level of Evidence: B)
• The use of single-barrier contraception alone in women with CHD-PAH is not recommended owing to the frequency of failure. (Level of Evidence: C)
• Estrogen-containing contraceptives should be avoided. (Level of Evidence: C)

Recommendations for Follow-Up

Class I

• Patients with CHD-related PAH should:
  • Have coordinated care under the supervision of a trained CHD and PAH provider and be seen by such individuals at least yearly. (Level of Evidence: C)
  • Have yearly comprehensive evaluation of functional capacity and assessment of secondary complications. (Level of Evidence: C)
  • Discuss all medication changes or planned interventions with their CHD-related PAH caregiver. (Level of Evidence: C)

Class III

• Endocardial pacing is not recommended in patients with CHD-PAH with persistent intravascular shunting, and alternative access for pacing leads should be sought (the risks should be individualized). (Khairy et al., 2006) (Level of Evidence: B)