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Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention.

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Central factors negatively affect the functional capacity of Fontan patients (FP), but "non-cardiac" factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8-17) years, respectively. Dominant ventricle ejection fraction was within normal range. The mean of peak VO expressed in absolute values (L/min), the relative values to body weight (mL/kg/min), and their predicted values were lower in FP compared with HC: 1.69 (±0.56) vs 2.81 (±0.77) L/min; 29.9 (±6.1) vs 41.5 (±9.3) mL/kg/min p < 0.001 and predicted VO Peak [71% (±14) vs 100% (±20) p < 0.001]. The absolute and predicted values of the forced vital capacity (FVC), forced expiratory volume in one second (FEV), inspiratory capacity (IC), total lung capacity (TLC), diffusion capacity of carbon monoxide of the lung (DLCO), maximum inspiratory pressure (MIP), and sniff nasal inspiratory pressure (SNIP) were also significantly lower in the Fontan population compared to HC. An increased risk of restrictive ventilatory pattern was found in patients with postural deviations (OD:10.0, IC:1.02-97.5, p = 0.042). There was a strong correlation between pulmonary function and absolute peak VO [FVC (r = 0.86, p < 0.001); FEV (r = 0.83, p < 0.001); IC (r = 0.84, p < 0.001); TLC (r = 0.79, p < 0.001); and DLCO (r = 0.72, p < 0.001). The strength of the inspiratory muscles in absolute and predicted values was also reduced in FP [-79(±28) vs -109(±44) cmHO (p = 0.004) and 67(±26) vs 89(±36) % (p = 0.016)]. Thus, we concluded that the pulmonary function was impaired in clinically stable Fontan patients and the static and dynamic lung volumes were significantly reduced compared with HC. We also demonstrated a strong correlation between absolute Peak VO with the FVC, FEV, TLC, and DLCO measured by complete pulmonary test.

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From 1980 to 1990, 12 patients (mean age 2.5 years, range 5 months to 9 years) with anomalous origin of the left coronary artery from the pulmonary artery were treated surgically. Five infants were operated upon in the first year of life because of persistent symptoms of congestive heart failure. In all cases, a two-coronary system was constructed by direct aortic reimplantation of the anomalous vessel with no deaths early or late over a follow-up period of up to 10 years. The technique of reimplantation was facilitated by transection of the main pulmonary artery. One patient with severe mitral regurgitation underwent, in addition, a mitral annuloplasty. A pulmonary valvotomy was performed in another patient with associated pulmonary stenosis. Three patients are receiving medication. The reimplanted anomalous left coronary artery was patent in each reevaluated patient (10/12). Left ventricular function improved considerably in all cases. Patients with symptoms should undergo repair soon after diagnosis. Direct aortic reimplantation should be technically feasible in even the smallest infant. Operative mortality is related to preoperative conditions and severity of ischemic damage of the myocardium.

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