For May 2016, there are 41 patients waiting for a heart transplant less than 1 year old: 107 waiting, between 1 and 5 years of age: 74 waiting, between 6 and 10 years of age: and 106 waiting between 11 and 18 years of age. Between 2003 and 2004, the median wait times were 77 days for patients less than 1 year, 81 days for patients between 1 and 5 years of age, 63 days for patients between 6 and 10 years of age, and 62 days between 11 and 18 years of age (Kanter et al. 1999; Davies et al. 2008; Bailey et al. 2009; Saczkowski et al. 2010; Davies et al. 2011; Scully et al. 2011; Davies and Pizarro 2012; Jeewa et al. 2014; Karamlou et al. 2014; Alsoufi et al. 2016).

From UNOS, Fig. 32.2 shows the number of patients per year removed from the list secondary to death. It appears the number is reducing per year.

Congenital heart patients have worse 1-, 3-, and 5-year survival compared to patients with coronary artery disease and patients with cardiomyopathy (Fig. 32.3).

It is difficult to determine why there are differences in survival because congenital heart patients are a very heterogeneous population. The most frequent diagnoses are listed in Table 32.1 (Lamour et al. 2009). Frequently they have had multiple previous operations. There surgeries are much more complicated given the pervious palliations and having to deal with arterial-to-pulmonary shunts, Glenn shunts (superior vena cava to pulmonary artery), or Fontan pathways (inferior vena cava to pulmonary artery). As result of the many operations and having foreign materials used in many of the repairs, patients are frequently sensitized with elevated plasma reactive antigens. Patients can also have other congenital abnormalities affecting other organ systems.

Lamour et al. found that patients with TGA had the best survival, 88 %, and patients with atrioventricular canal had the worst survival, 62 %, p = 0.02. In the same report, the mean ischemic time was 228 min for patients with 1–2 previous sternotomies and 242 min for > 3 sternotomies (Bernstein et al. 2006). Fontan patients faired worse after a transplantation, with an actuarial 1-year survival of 75 % compared to an 83 % for other congenital heart diseases and 89 % for patients without congenital heart disease (Hsu and Lamour 2015). Lamour et al. found that patients with Fontan had an increase in relative risk of death of 8.6, with 71 % 1-year survival for Fontan patients and 83 % for non-Fontan patients (Lamour et al. 2009). There is growing concern that Fontan circulation can lead to liver failure, which may explain the worse prognosis. From Lamour’s paper, Table 32.2 shows the multivariable analysis for the risk factors for early- and constant-phase mortality.

Hsu and Lamour reviewed the causes of chronic heart failure in congenital heart disease patients (Table 32.3) (Almond et al. 2009).