Ebstein's malformation is usually treated by replacing the tricuspid valve, albeit with controversial results. The “Carpentier technique,” introduced in 1980, offers both a new classification and new surgical methods for treatment of this lesion. The classification is based on the severity of the displacement of the septal and posteroinferior leaflets, with four grades of increasing severity. The surgical techniques involve mobilization of the anterosuperior leaflet, reduction of the diameter of the annulus, and longitudinal plication of the atrialized part of the right ventricle. Following this approach, 64 patients underwent consecutive surgical evaluation, with only one valvar replacement. The mean age was 26±15 years. The hospital mortality was nine percent. The mean follow-up is now 5.18±3.25 years. The actuarial survival rate was 87% at one year and 82% at five and 10 years. Of the patients, 90% were in postoperative functional class I or II. Control echocardiography revealed either no insufficiency, or minimal regurgitation in 80%. A second operation was needed in six patients because of right ventricular failure (two patients) or recurrence of valvar insufficiency (four patients). Sinus rhythm was present in 44 (88%) of the surviving patients. We conclude that repair of the malformed tricuspid valve, associated with longitudinal plication of the right ventricle, is safe and efficient. The prognosis depends on right ventricular contractility, which should be evaluated carefully before and after the operative procedure.

Autonomic denervation has been assumed to persist after orthotopic heart transplantation. Analyzing spontaneous and induced variations of heart rate, the status of autonomic cardiac innervation was investigated in six children 19–37 months after cardiac transplantation. The age at the time of transplantation varied from three weeks to 15.4 years. Heart rate variability was assessed on 24-hour Holter recordings by calculating time-domain indices (standard deviation of all RR intervals; standard deviation of the mean RR intervals from successive five-minute periods; mean of the standard deviations of RR intervals from successive five-minute periods; proportion of adjacent RR intervals > 50 msec different; square root of mean square successive differences in RR intervals) and frequency-domain variables (low frequency power, high frequency power and total spectrum power). Sinus node recovery time, sinoatrial conduction time and post-pacing cycle lengths were examined at different rates of endocardial atrial pacing. After the first year subsequent to transplantation, standard deviation of the mean RR intervals reached the normal range in four patients, whereas the other time-domain variables became normal in two patients. Both patients displayed normal or near-normal power spectrums of heart rate with normal day-to-night variations. For the remaining patients, spectrums of heart rate failed to show the main frequency peaks. Only the patient with normal variability in heart rate exhibited a decrease in corrected sinus node recovery time at higher stimulation rates, shortening of the first recovery cycle below the pre-pacing level, and a rapid decline of the accelerated post-pacing heart rate as described in innervated hearts. These results suggest the evolution of time-dependent efferent autonomic reinnervation after cardiac transplantation in children.

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.

Magnetic resonance velocity mapping of intraaortic flow was performed prospectively in adolescents and adults after coarctation repair. The aims were to assess the feasibility and clinical usefulness of the technique in this patient group, and to study flow velocity distributions in repaired regions. Twenty consecutive patients attending for follow-up after repair of aortic coarctation, aged 15–39, mean 25 years, were studied using a 0.5 tesla Picker magnetic resonance machine. Spin echo and cine imaging with phase velocity mapping, echo time 3.6 ms, were used to study anatomy and flow in the repaired region. Transcutaneous ultrasonic examination, with continuous wave Doppler velocity measurement was performed independently on the same day. Velocity maps, acquired successfully in all patients, showed asymmetry and nonhomogeneity of flow in relation to anatomical distortions of repaired regions. Magnetic resonance and Doppler measurements of peak velocity compared as follows: n=20, range 1.2–3.9 m/sec, mean 2.33 m/sec, mean of differences (Doppler-MR) 0.22 m/sec, standard deviation of differences ±0.27 m/sec. Localized velocity peaks adjacent to wall deformations were identified by magnetic resonance in five patients without significant restenosis. Magnetic resonance imaging with velocity mapping proved reliable and informative in follow-up assessment in adolescents and adults after surgical repair of aortic coarctation. There was satisfactory agreement between magnetic resonance and Doppler measurements of peak velocity. Velocity maps showed that localized velocity peaks may occur in limited parts of the stream adjacent to distorted aortic boundaries without stenosis. This could be a cause of overestimation of pressure gradients from peak velocity data, a possibility which requires further investigation.

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.