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Management of Adults With Congenital Heart Disease

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19 3.6.2. Heart Transplantation and MCS COR LOE Recommendations 1 B-NR 1. In patients with ACHD and heart failure, evaluation by specialists with medical and surgical experience in ACHD, as well as by specialists with expertise in advanced heart failure, is recommended to assess appropriateness for advanced heart failure therapies, such as heart transplantation or MCS. 2a B-NR 2. In patients with ACHD who are considered for heart transplantation, multiorgan transplantation (heart-lung, heart-kidney, or heart-liver) is reasonable based on center- specific assessment of the degree of respective lung, kidney, or liver dysfunction. 2a B-NR 3. In patients with ACHD and advanced heart failure that is refractory to optimal medical and device-based therapy, the use of durable MCS devices is reasonable to improve quality of life or as a bridge to transplantation. 3.7. Genetic Syndromes Screening COR LOE Recommendations 2a B-NR 1. For adults with conotruncal defects, screening for 22q11.2 deletion syndrome is reasonable to assess the risk of transmission to offspring and to screen for associated comorbidities. 2a C-EO 2. In adults with congenital heart disease and a family history of congenital heart disease or clinical features associated with a genetic syndrome, referral for genetic evaluation is reasonable to guide management.

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