The Fontan procedure provides a means of separating the systemic and pulmonary circulations in patients with congenital heart defects characterized by a functionally single ventricle. The procedure has undergone numerous changes since its introduction 25 years ago. Technical modifications have now increased its applicability to virtually all forms of univentricular atrioventricular connection, as well as many hearts with biventricular connections. More recently, philosophical changes have been introduced to address increased rates of morbidity and mortality observed in certain subsets of patients. These changes appear to have improved short- and midterm results. The purpose of this review is to offer our strategy for management of such patients. We will discuss the interrelationship between the bidirectional Glenn and Fontan procedures. We will also focus on a number of observations concerning the possible role of accessory pulmonary blood flow, aortopulmonary collateral arteries, and endocrinologic changes.

In western Sweden, less than one-third of children born with complete transposition between 1964 and 1983 are long-time survivors. During bicycle ergometry, using a steady-state protocol with analyses of the expiratory gases and lactate, we investigated the exercise capacity in survivors of atrial redirection. There were 16 patients who had a Mustard procedure, now aged 16.9±3.6 years (12.0–22.0) and 15 patients who underwent a Senning correction aged 9.4±1.6 years (7.2–12.1). Nineteen percent of Mustard cases and 53% of Senning cases had minor or no sequels. Moderate sequels were found in 44% of post-Mustard cases, and 40% of post-Senning cases, including baffle complications, moderate systemic ventricular dysfunction, sinus node dysfunction and tracheal stenosis. Severe problems were encountered in 37% of post-Mustard cases and 7% of post-Senning cases, including pulmonary hypertension, pulmonary venous obstruction, atrioventricular block and hemiparesis. The maximal exercise capacity was low (p<0.0001); 71% had an oxygen uptake below −2 SD of predicted values, with severely impaired oxygen uptake in all except one of the cases with severe sequels, but no significant difference was found between patients having mild and moderate sequels. The maximally achieved heart rate was low (p<0.0001); 71% had values below −2 SD of predicted, including all cases with sinus nodal dysfunction, as well as 40% of cases without it. The maximal oxygen pulse was low (p<0.01), below −2 SD in 36%, but it was less affected than other exercise variables in 68% of the patients. Our study, therefore, presents exercise data in an epidemiologically-based population, relates the data to postoperative sequels and discusses some possible adaptive mechanisms.

Valvar regurgitation has been commonly found by echocardiography in adults and older children with normal hearts, but there is limited knowledge in neonates. To evaluate the prevalence of valvar regurgitation and analyze risk factors, echocardiography was performed on 975 consecutive neonates (1–7 days of age) with normal hearts. Regurgitation was detected and graded with pulsed, continuous-wave and color Doppler imaging techniques. Thirty-five cases with valvar regurgitation were followed for one month. The results showed that 369 (37.8%) neonates had evidence of regurgitation in one or more valves. Tricuspid regurgitation was most commonly found and was detected in 345 (35.4%) neonates, mitral regurgitation in 51 (5.2%), pulmonic regurgitation in 25 (2.6%), and aortic regurgitation in eight (0.8%). Most of them were trivial or mild and involved just one valve. Moderate or severe regurgitation was detected only in the tricuspid valve (3%) and the mitral valve (0.1 %). Five (0.5%) neonates with severe tricuspid regurgitation had heart murmurs, and two (0.2%) of them presented with cardiorespiratory distress. The risk factors for tricuspid and mitral regurgitation included lower chronologic age, gestational age and whether the neonates had a patent arterial duct; whereas those for tricuspid regurgitation also related to lower Apgar scores, birth weight and whether the neonates had a patent oval foramen. About 90% of the tricuspid regurgitation disappeared and the remaining 10% decreased in one month. We concluded that trivial or mild valvar regurgitation is commonly found in neonates with normal hearts. Moderate or severe regurgitation is relatively rare and occurs only in the tricuspid and mitral valves with a few cases causing clinical attention.

Most patients with DiGeorge syndrome require surgery to their heart defect during infancy and are at risk of developing transfusion-associated graft-versus-host disease if non-irradiated blood is used perioperatively. Because of its wide clinical variability, DiGeorge syndrome may not be recognized before surgery and thus a high level of vigilance is required. An infant is described who developed fatal graft-versus-host disease after receiving non-irradiated blood components during surgery for classical tetralogy of Fallot; the postoperative clinical course and autopsy findings were consistent with underlying DiGeorge syndrome. An informal survey of the 18 pediatric cardiology units in the United Kingdom and the Republic of Ireland showed no consensus as to which patients should be screened for DiGeorge syndrome, which screening test to use and to whom irradiated blood components should or should not be given to prevent transfusion associated graft-versus-host disease. Assessment of T-cell numbers appears to be the preferred screening method to determine those who might benefit from receiving such therapy.

We report the progressive development of pulmonary hypertension despite the presence of severe pulmonary stenosis in three patients with cyanotic congenital heart disease. The associated intracardiac lesions were complete transposition with a ventricular septal defect, double outlet right ventricle, and a heart with univentricular atrioventricular connection, respectively. Serial measurements on hemodynamics and histological findings documented the progression of pulmonary hypertension and pulmonary vascular diseases. The underlying etiology remains speculative. In view of the possibility of development of pulmonary hypertension despite a ‘protected’ pulmonary vasculature, corrective surgery should be contemplated as soon as technically feasible.

Valproic acid is a known teratogen, giving rise to a typical craniofacial appearance, the fetal valproate syndrome. Congenital heart malformations in this syndrome have most commonly been ventricular septal defects. We report a child with familial craniosynostosis, and tetralogy of Fallot, following intrauterine exposure to valproic acid.

We describe two fetuses presenting with hydrops and tachycardia who were treated by prolonged dosage with procainamide. Initial therapy with digoxin in both had been unsuccessful in controlling the rapid heart rate. The first child was delivered after five weeks of transplacental therapy, and demonstrated Wolff-Parkinson-White syndrome on the second day which was controlled with flecainide. The second fetus required three and a half weeks of intrauterine treatment. Wolff-Parkinson-White syndrome became manifest four days after premature labor at 33 weeks gestation. No tachycardia occurred postnatally and the infant has been well during follow-up. The implications of treatment are discussed.

We report a case of right aortic arch and isolation of the left brachiocephalic artery. Stenosis of the left pulmonary artery, ventricular septal defect and left superior caval vein draining to the right atrium via the coronary sinus were also present in a 13-month-old asymptomatic girl with Down's syndrome. To our knowledge, this association of lesions has not previously been described.

Despite early arterial switch repair of complete transposition of the great vessels, significant physiologic aberrations may still occur. Two cases of vascular disease in the left pulmonary arterial system are described and potential mechanisms are discussed. We conclude a small number of children with simple transpostion may develop asymmetric patterns of pulmonary blood flow without evidence of anatomic narrowing of either arterial vessel.