From the Guidelines
The latest European Society of Cardiology guidelines for managing Adult Congenital Heart Disease emphasize the need for a comprehensive and multidisciplinary approach to care, with a focus on specialized management and follow-up for patients with complex defects. The management of Adult Congenital Heart Disease (ACHD) requires a tailored approach, taking into account the specific type and complexity of the defect, as well as the patient's overall health status and medical history 1.
Key Recommendations
- Patients with simple defects may require less frequent follow-up, typically every 3-5 years, while those with complex conditions should be seen annually.
- Transition programs from pediatric to adult care are crucial, with transfer typically occurring between ages 16-18.
- Specific management strategies include:
- Anticoagulation with vitamin K antagonists for patients with mechanical valves (target INR 2.5-3.5)
- Endocarditis prophylaxis for high-risk patients (including those with prosthetic valves or previous endocarditis)
- Careful pregnancy planning with pre-conception counseling
- Pulmonary arterial hypertension associated with congenital heart disease should be treated with advanced therapies, including endothelin receptor antagonists, phosphodiesterase-5 inhibitors, or prostacyclins.
- Heart failure management should follow standard guidelines, with special consideration of the underlying anatomy.
- Arrhythmia management may involve catheter ablation for symptomatic supraventricular tachycardias. These recommendations highlight the importance of specialized care for ACHD patients, who face unique challenges including residual lesions, sequelae from previous interventions, and age-related comorbidities that require expertise beyond general cardiology care 1.
From the Research
Latest ESC Guidelines on Adult Congenital Heart Disease
The latest European Society of Cardiology (ESC) guidelines for managing Adult Congenital Heart Disease (ACHD) were published in 2020 2. The guidelines reflect the diagnostic and therapeutic progress made in the past decade and are based on an evidence level C, which is a consensus of opinion of experts or knowledge from small studies, retrospective studies, or registries.
Key Recommendations
- The guidelines emphasize the importance of comprehensive longitudinal follow-up for ACHD patients, as the condition is chronic and lifelong 2.
- The guidelines address the management of coronary anomalies for the first time 2.
- Progress in arrhythmia diagnosis and management, percutaneous interventions, and the treatment of pulmonary arterial hypertension have led to revised or new recommendations 2.
- The guidelines also highlight the need for better education in the management of ACHD patients and the existence of multiple knowledge gaps 3.
Specific Considerations
- Pulmonary valve replacement is a common procedure in ACHD patients, particularly those with tetralogy of Fallot 4, 5, 6.
- The timing and techniques of pulmonary valve replacement are crucial, and the guidelines provide recommendations for asymptomatic patients 4.
- Transcatheter pulmonary valve implantation (TPVI) is emerging as a potential gold standard intervention for pulmonary valve replacement 6.
Management of ACHD
- The guidelines emphasize the need for a multidisciplinary approach to managing ACHD patients, including cardiologists, surgeons, and other healthcare professionals 2, 3.
- The management of ACHD patients requires careful consideration of the individual patient's needs and the complexity of their condition 2, 3.