Individuals with Fontan circulation are at risk of late mortality from both cardiac and noncardiac causes. Despite the known risk of mortality, referral indications for advanced heart failure care vary between centers, and many individuals die from Fontan circulation-related complications either after late consideration for advanced heart failure therapies or having never seen a heart failure specialist. There is a critical need for guidelines to direct appropriately timed referral for advanced heart failure consultation. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Fontan Committee has developed recommended thresholds for advanced heart failure referral to guide primary cardiologists. These recommendations are divided into 4 categories of clinical Fontan circulatory dysfunction including (1) cardiac/systemic ventricular dysfunction, (2) Fontan pathway dysfunction, (3) lymphatic dysfunction, and (4) extracardiac dysfunction.

Adults with congenital heart disease (ACHD) experience worse waitlist outcomes and higher early post-transplant mortality compared to non-ACHD patients. On October 18, 2018; the UNOS donor heart allocation system was redesigned giving unique listing status to ACHD patients. The impact of this change on outcomes in transplant-listed patients is unstudied. Using the Scientific Registry of Transplant Recipients (SRTR) we compared ACHD patients listed for the first-time for heart transplantation from 2 eras of equal duration. We analyzed waitlist outcomes, post-transplant mortality and length of stay among ACHD patients in both eras and between ACHD and non-ACHD patients in the new era. Of 12723 listed patients, 535 had ACHD (293 in the new era) and 12188 did not (6258 in the new era). A total of 163 (56%) ACHD patients in the new era versus 150 (62%) in the prior era were transplanted; 11 (3.8%) versus 15 (6.2%) died on the waitlist; 32 (11%) versus 35 (14%) were delisted and 15 (9.2%) versus 19 (12.7%) died within 30 days of transplant respectively. The new UNOS donor heart allocation system improved waitlist outcomes among ACHD patients without altering early post-transplant outcomes or significantly changing the gap in outcomes compared to non-ACHD patients.

Adults with congenital heart disease are hospitalized at increasing rates in the Western world. Identification of rates of and risk factors for hospitalization is essential for research and improving patient outcomes. We conducted a single-center retrospective analysis of patients with a primary diagnosis of tetralogy of Fallot (TOF), transposition of the great arteries (both d- and l-transposition of the great arteries [TGAs]), or single ventricle (SV). We investigated the rates of mortality, annual hospitalization, and patient-specific risk factors for unplanned cardiac hospitalization. Adult patients with complex congenital heart disease are hospitalized at a rate four to eight times greater than the general US population (P < .001). In addition, there are significant differences between the rates of hospitalization in TOF and TGA (0.39 and 0.41 hospitalizations per patient-year, respectively) and SV (0.72 hospitalizations per patient-year). The majority of excess hospitalizations in the study group were due to cardiac disease (P < .001 for all three groups). Risk factors for unplanned cardiac hospitalization in TOF included pulmonary atresia, depressed left ventricular and right ventricular ejection fraction, and smoking; in TGA, they included Ebstein malformation, surgeries other than primary repair, noncardiac diagnoses, atrial arrhythmias, atrioventricular nodal block, left ventricular ejection fraction, and smoking; and in SV, they included atrial arrhythmias and cyanosis. Patients born with complex congenital heart disease are hospitalized far more frequently than the general US population, primarily for cardiac-related illness. Future research should focus on confirming the present findings and on identification of strategies to improve outcomes in this growing group of patients.