Long-Term Outcome Survivors are now reaching adulthood, but their numbers are small due to the rarity of the disease and high mortality in previous eras Few data are available about long-term survival and functional state Mortality tends to occur in the first 6 months of life and the survival curves flatten (see Fig 43.17).55,114,120 The Toronto Hospital for Sick Children reported 10-year survival of 43%, the Congenital Heart Surgeons study reported 15-year survival at 58%, the Swedish Collaborative study noted 10-year survival of 68%, but more reassuringly, a series from the University of California, Los Angeles, reported a 10-year survival of 86%.6,116,118,121 Of the patients, 20% had late arrhythmias, and right atrial dilation was found in all patients At the current time, prediction of longerterm outcome for a biventricular repair can only be made by drawing parallels with patients who have other diseases, such as those who have undergone definitive repair of tetralogy of Fallot and pulmonary stenosis (see Chapter 35) Surprisingly, there is limited evidence that biventricular repair is better than univentricular repair Sanghavi and associates122 found no statistical difference in exercise capacity between those with biventricular versus univentricular repair Most patients in both groups had subnormal peak oxygen consumption and a trend toward impaired performance with increasing age Similarly, EkmanJoelsson and colleagues, in the Swedish Collaborative Study,123 found no difference in exercise capacity in patients after biventricular versus univentricular repair Decreased lung function was noted in all groups Karamlou et al.124 found a trend toward higher VO2 in patients with biventricular and 1.5ventricle repairs compared to univentricular patients However, increased performance was strongly associated with the initial tricuspid valve z-score, rather than conferred by repair type Peak VO2 and maximum heart rate were lower in survivors of pulmonary atresia than controls regardless of their type of repair The study also demonstrated an interesting dichotomy whereby patients with pulmonary atresia believe they are doing well despite important physical limitations Numata and coworkers explored whether there was any functional benefit in having a 1.5-ventricle repair compared with a univentricular repair At 5 and 10 years there was no difference in exercise capacity Importantly, atrial arrhythmias were common in the 1.5-ventricle repair group.125 For those embarking on a total cavopulmonary circulation, mortality tends to occur early in childhood, often within a few months of the initial procedure.114,126 There is an ongoing mortality hazard but the early data available indicate that the influence of coronary arterial abnormalities may be less than predicted.126,127 A study from the Mayo Clinic of 40 patients who underwent the Fontan procedure for pulmonary atresia found three operative deaths and also three later deaths at 2.5, 8, and 8 years postoperatively.126 Cause of death was presumed dysrhythmia in two patients and protein-losing enteropathy in the third The median age of survivors was 13 years (range, 4 to 30 years); all but one were in New York Heart Association functional class I or II This was a highly preselected group with a low incidence of coronary fistulas (10%) and RV-dependent coronary blood flow (2.5%) A study from Toronto reported survival after the Fontan procedure of 80% at 10 years with only one late death, 1 year after the procedure.127 This was in spite of a relatively high occurrence of coronary fistulas (68%) and RV-dependent coronary blood flow (22%) Persisting RV hypertension and RV-to-coronary connections can lead to progression of coronary abnormalities such as stenoses, ectasia, and interruptions that can themselves lead to sudden death It is pertinent, in the Toronto study, that patients with fistulae underwent thromboexclusion (patch closure) of the RV, which was believed to be indicated to prevent ongoing coronary artery damage Further follow-up will be required to ascertain whether this strategy leads to improved late outcome A study from Boston examined the outcome of 32 patients with RV-dependent coronary circulation and following the univentricular route.128 There was a surprisingly good outcome with actuarial survival of 81% at 5, 10, and 15 years All mortality occurred within 3 months of the initial systemic-to-pulmonary shunt All patients with aortocoronary atresia died The researchers’ conclusion was that “single ventricle palliation yields excellent long-term survival and should be the preferred management strategy for these patients.”128 A recent series from Colombia University examined the outcome of 17 patients undergoing univentricular palliation They compared those with RVdependent coronary circulations to those without In this cohort, 60% of patients with an RV-dependent coronary circulation died, compared to none in the normal coronary group Of note, 2 of the 3 surviving patients who underwent Fontan completion with RV-dependent coronary circulations had evidence of ischemia during follow-up.129 Recommendations for Long-Term Follow-Up For those with a biventricular repair and minimal residual hemodynamic lesions, patients should be seen every 1 to 3 years by a cardiologist Where there are significant residual lesions, follow-up should be yearly by an adult congenital cardiologist Similarly, patients with mixed or functionally univentricular circulations warrant follow-up in a tertiary center For patients with venous shunts or the Fontan circulation, strong consideration should be given to full anticoagulation, particularly if there is suspicion of coronary arterial abnormalities Exercise limitations need to be reviewed on an individual basis, depending on type of surgical route followed, the underlying hemodynamics, and the overall state of the patient Endocarditis prophylaxis is not required but high levels of dental hygiene are recommended At each visit, it is essential to assess residual morphologic lesions Following a biventricular repair, the patient should be acyanotic, with normal volume pulses, although the jugular venous pulse may be elevated and the right ventricular impulse increased There will usually be a normal first heart sound with single second, which may be split if a homograft has been inserted Murmurs of residual pulmonary stenosis, regurgitation, and tricuspid regurgitation may be evident Hepatomegaly may be present Indeed, if tricuspid regurgitation is severe, the liver may be pulsatile Patients are prone to atrial arrhythmia Following a Fontan procedure, the patient should be pink, with saturations in the 90s Brachial pulses may be absent following previous arterial shunt procedures, and the jugular venous pulse will be greatly elevated and may only be visible on sitting up There will be a single heart sound There may be a murmur from tricuspid regurgitation or a systolic murmur caused by blood flow from a high-pressure RV into a coronary arterial fistula Hepatic congestion may be evident For patients with a mixed circulation, the patient will be cyanosed with clubbing, erythrocytosis, possible continuous murmurs due to patent systemic shunts, and may have features of either circulation described earlier Chest radiography will often show an increased cardiothoracic ratio with, in particular, a dilated right atrial contour In patients with severe tricuspid regurgitation, the RV may also be dilated In those with a mixed circulation, there may be pulmonary oligemia