#JustRelased 2018 AHA/ACC Guideline for the Management of Ad

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease

The American College of Cardiology (ACC) and the American Heart Association (AHA) have released an updated guideline for the management of adult congenital heart disease (ACHD) patients. The guideline has been published recently in the Journal of the American College of Cardiology and replaces the original guideline published in 2008. 

Specifically, the guideline presents a new classification system for ACHD patients that retains the traditional classification based on the structural complexity of the disease while taking into account the patient's functional status and other factors, including the presence of cardiovascular and non-cardiovascular problems. The new system is intended to capture the complexity of ACHD in terms of both anatomy and physiology, rather than anatomy alone. 

The following are the key points from the guideline:-

• The guideline development process prompted the commissioning of two independent evidence review committees (ERCs) to address two significant clinical questions. The first ERC addressed the issue of the benefit of intervention for asymptomatic patients with secundum atrial septal defects (ASDs) and right ventricular (RV) dilatation. The second ERC addressed the issue of the role of medical therapy, including angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, beta-blockers, and aldosterone antagonists in adults with systemic right ventricles.

• The guideline presents a new classification system for ACHD. The adult congenital heart disease anatomic and physiological (ACHD AP) classification system uses both anatomic complexity and as well as physiologic status. Anatomic classification includes Class I (simple), Class II (moderate complexity), and Class III (great complexity). The physiological classification is divided into stages A-D and is overall similar to the AHA heart failure classification. The physiological classification system takes into account a patient’s functional status as well as other factors including presence of valve disease, pulmonary hypertension, arrhythmias, aortic dilatation, end-organ function, and cyanosis.

• The need for such a classification system is based on data that allow better refinement of patient classification that uses physiologic variables in addition to anatomic categorization. For example, a patient with repaired tetralogy of Fallot with no significant pulmonary regurgitation, no symptoms, and no other sequelae would be ACHD AP classification IIA, whereas another patient with tetralogy of Fallot but with severe pulmonary valve regurgitation and exercise intolerance would be ACHD AP classification IIC. This distinction allows patients to be followed prospectively in a fashion more reflective of the interplay between their congenital abnormality and physiologic impact.

• Two recommendations related to closure of secundum ASD have been formed

  1. In adults with isolated secundum ASD causing impaired functional capacity, right atrial and/or RV enlargement, and net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., pulmonary– systemic blood flow ratio [Qp:Qs] ≥1.5:1) without cyanosis at rest or during exercise, transcatheter or surgical closure to reduce RV volume and improve exercise tolerance is recommended, provided that systolic PA pressure is less than 50% of systolic systemic pressure and pulmonary vascular resistance is less than one third of the systemic vascular resistance (Strong recommendation)
  2. In asymptomatic adults the closure is reasonable (Moderate Recommendation)

• Patients with bicuspid aortic valve should be evaluated for coarctation of the aorta with clinical exam and imaging studies (Strong recommendation). It is reasonable to screen first-degree relatives of patients with bicuspid or unicuspid aortic valve for aortic valve disease or aortopathy using echocardiography (Moderate Recommendation)

• Screening for intracranial aneurysms by magnetic resonance (MR) angiography or computed tomography (CT) angiography may be reasonable in adults with coarctation of the aorta (Moderate Recommendation).

• Pulmonary valve replacement (PVR) for relief of symptoms is recommended for patients with repaired tetralogy of Fallot and moderate or greater pulmonary regurgitation with cardiovascular symptoms not otherwise explained (Strong recommendation)

• PVR is reasonable for preservation of ventricular size and function in asymptomatic patients with tetralogy of Fallot and ventricular enlargement or dysfunction and moderate or greater pulmonary regurgitation (Moderate Recommendation). Specifically, PVR is reasonable in patients with ≥2 of the following:-

  1. Mild or greater RV or left ventricular (LV) dysfunction
  2. Severe RV dilatation (RV end-diastolic volume index ≥160 ml/m2
  3. RV end-systolic volume index ≥80 ml/m2)
  4. RV end-diastolic volume ≥2x LV end-diastolic volume
  5. RV systolic pressure ≥2/3 systemic pressure, and/or progressive reduction in objective exercise capacity

• Primary prevention implantable cardioverter-defibrillator therapy is reasonable in adults with tetralogy of Fallot and multiple risk factors for sudden cardiac death (SCD) (Moderate Recommendation). Risk factors for SCD include LV systolic or diastolic dysfunction, nonsustained ventricular tachycardia, QRS duration ≥180 ms, extensive RV scarring, and inducible sustained ventricular tachycardia at electrophysiological study.

• The systemic review conducted regarding impact of medical therapy in patients with systemic right ventricles demonstrated that medical therapy for systolic ventricular dysfunction remains largely uncertain. As a result, no recommendations regarding medical therapy for systolic dysfunction of systemic right ventricles were made.

• It is reasonable to perform anatomic evaluation of coronary artery patency, i.e., catheter angiography or CT or MR angiography in asymptomatic adults with dextro-transposition of the great arteries (d-TGA) with arterial switch (Moderate Recommendation). Physiologic tests of myocardial perfusion for adults with d-TGA after arterial switch can be beneficial for assessing symptoms of myocardial ischemia (Moderate Recommendation).

• Pulmonary vasoactive medications can be beneficial to improve exercise capacity in adults with Fontan repair (Moderate Recommendation).

• At the conclusion of the document, the writing committee describes evidence gaps in the management of ACHD and proposes future directions to address these issues.




The American College of Cardiology is a 52,000-member medical society that is the professional home for the entire cardiovascular care team. The mission of the College is to transform cardiovascular care and to improve heart health. The American Heart Association is a non-profit organization in the United States that fosters appropriate cardiac care in an effort to reduce disability and deaths caused by cardiovascular disease and stroke.

Note: This list is a brief compilation of some of the key recommendations included in the Guideline and is not exhaustive and does not constitute medical advice. Kindly refer to the original publication here: https://pxmd.co/L6N36

About Author
Dr. Prachi Chhimwal
Dr. Prachi Chhimwal is an Editor at PlexusMD and is a part of the Engagment Team. She curates the Technical Content posted daily on the news feed. She graduated from Army College of Dental Sciences (B.D.S) and went on to pursue her post-graduation (M.D.S) in Oral & Maxillofacial Pathology. After a decade in the field of dentistry she took a leap of faith and joined PlexusMD. A badminton enthusiast, when not working you can find her reading, Netflixing or enjoying stand-up comedy shows.
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