A Review of Heart Transplantation for Adults With Congenital Heart Disease.

As techniques for corrective and palliative surgery in congenital heart disease improve, the number of patients surviving to become adults with congenital heart disease (ACHD) has increased. A significant proportion of these patients will progress to develop advanced heart failure, the symptoms of which vary, complicating prediction of life expectancy. Unlike acquired heart failure, there is a lack of evidence-based treatments with which to relieve symptoms and prolong survival. As a result, a number of ACHD patients will proceed to heart transplantation. Referral for transplantation should be considered early, given the difficulties with prognostication, and should take place in a center with surgical and medical expertise in the management of ACHD patients and transplantation. In addition to assessing contraindications to heart transplantation for the general population, factors specific to ACHD should be considered. These include pulmonary hypertension, cyanosis, liver disease, previous surgeries, and the degree of allosensitization. Once listed for transplantation, ACHD patients spend longer on the waitlist, and are more likely to die or be delisted than their non-ACHD counterparts. Mechanical circulatory support is used less commonly as a bridge to transplantation given the difficulties with implantation and unfamiliarity of use. Recent evidence suggests that with increased experience and early consideration, mechanical circulatory support can be used successfully as a bridging therapy. Despite a higher early mortality, long-term survival is greater for ACHD patients after transplant due to younger age and relative lack of comorbidities. With early referral, careful assessment of each individual's unique anatomy and physiology, and care in a center experienced with ACHD patients and transplantation, outcomes will continue to improve.