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.

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.

One hundred-ninety-four fetuses with irregular heart rates were seen over a five-year period at a tertiary center for fetal cardiology. The median gestation at referral was 31 weeks, with a range from 19 to 41 weeks. Of these fetuses, 157 had extrasystoles of either atrial or ventricular origin. Blocked atrial ectopic beats had led to slow ventricular rates (80–110 beats per minute) in 37 fetuses. The structure of the heart was normal in all except two fetuses. Postnatal outcome was known for 165 of the fetuses. Of these, 157 (95%) had an uneventful antenatal and postnatal course. Tachyarrhythmias developed in eight fetuses (5%) in either the prenatal (n=4) or postnatal (n=4) period. Five of 37 fetuses with blocked atrial ectopic beats (13%) developed a tachyarrhythmia. No fetus developed hydrops, and all infants survived. All cases had required treatment with antiarrhythmic drugs. The occurrence of an irregular heart rhythm in the fetus, therefore, is not always benign. Fetuses with blocked atrial ectopic beats require particularly close monitoring.

The assessment of diastolic function in the developing fetus is difficult because of constantly changing conditions of loading. Previous reports suggesting improved ventricular compliance with fetal development are limited by the Doppler indices utilized. Using load-dependent and independent variables, we examined and compared serially right and left ventricular diastolic function in 11 normal fetuses (44 echocardiographic studies) at 22±1.0 weeks; 30±1.0 weeks; 36±1.0 weeks of gestation and 64±25 hours postnatally. Load-dependent indices included early to late time velocity integral ratios and percentage of early filling to total diastolic filling. The load-independent index was the peak filling rate normalized to the total time velocity integral. Both right and left ventricular time velocity integral ratios were similar initially, and shifted from late to early diastole with increasing gestational age (greater ratios). The shift in left ventricular ratios was greater, and increased earlier in fetal development. The right ventricular normalized peak filling rate decreased with increasing gestational age, while the left ventricular normalized peak filling rate remained constant, but was greater than the right ventricular rate postnatally. Both right and left ventricular filling shifts from late “active” to early “passive” diastole during fetal development. Left ventricular ratios are greater and increase earlier in fetal development, likely influenced by changes in preload. The right and left ventricular normalized peak filling rates were similar prenatally, indicating similar diastolic filling properties, but disparate postnatally, consistent with improved left ventricular relaxation immediately preceding or at the time of onset of transitional circulation.

We described previously that the degree of deficiency of the ventricular septum (scooping) affects electrocardiographic findings in atrioventricular septal defect with separate right and left atrioventricular orifices. The aim of this study was to clarify the clinicomorphological relationships of the degree of scooping, the precise morphology of the atrioventricular valve, and electrocardiographic findings in hearts having a common atrioventricular orifice. In 63 patients with usual atrial arrangement, the outlet, scoop and inlet dimensions of the ventricular septum were measured by biplanar cineangiography and were compared with the body surface area. The scoop/outlet ratio and inlet/outlet ratio were also compared with the body surface area, atrioventricular valvar morphology, electrocardiographic findings, and severity of regurgitation of the atrioventricular valve. The results showed that the three individual dimensions of the septum correlated significantly with the body surface area, even though the scoop/outlet ratio and inlet/outlet ratio did not. A correlation was found between the scoop/outlet ratio and the degree of left axis deviation on the electrocardiogram (r=0.74, p<0.001). In 16 patients with division of the inferior bridging leaflet, there was a lower scoop/outlet ratio than in the remaining 37 with an undivided bridging leaflet (p<0.01). Of 24 patients with counterclockwise rotation in the horizontal loop of the vectorcardiogram, 22 had an undivided inferior bridging leaflet (p<0.01). The morphology of the superior bridging leaflet, in contrast, was not associated with the scoop/outlet ratio, and neither was the severity of valvar regurgitation. We conclude that the degree of scooping of the ventricular septum is related not only to electrocardiographic findings, but also to the morphology of the inferior bridging leaflet in atrioventricular septal defect with common atrioventricular orifice.

Restrictive cardiomyopathy is a rare disease in children and adolescents. We have now encountered eight such cases in infants and children aged between nine months and 14 years. The reasons for admission to hospital were not very specific and included recurrent bronchitis, thoracic deformity, pathologic findings on routine electro-cardiography, or signs of cardiac failure. Despite two cases with atrial flutter, the characteristic electrocardiographic sign was a P-wave in lead II of about 0.6 to 1.5 m V. Echocardiographically, the leading feature was enlargement of the atriums, seen in cross-sectional and M-mode tracings. Diameters and functional parameters for the left ventricle, and the EF-slope of the mitral valve were normal. In four patients examined specifically for diis aspect, a typical E/A-ratio was found. Cardiac catheterization was performed in all patients. Left atrial or pulmonary capillary wedge pressures were all abnormal, and pulmonary arterial pressures ranged from 30 to 60 mm Hg. Calculated pulmonary resistances were 3 to 12 units per meter squared. End-diastolic left ventricular pressures were elevated, while comparable right-sided pressures were normal. Percutaneous endomyocardial biopsy was done in six patients and performed as an open biopsy in two. Only unspecified signs, such as myocytic hypertrophy or interstitial fibrosis, were found. At present, five of the children have died within two years of diagnosis. One boy has undergone cardiac transplantation success-fully. The two youngest children are still alive. As there is such a poor prognosis, and no alternative specific therapy is known, cardiac transplantation must be discussed with the family at an early stage, emphasizing its consequences.

To assess the clinical impact of percutaneously implanted balloon expandable endovascular stents on patient management, we reviewed 20 children aged 14 days to 4.8 years (median 1.3 years) with residual vascular obstructions in the immediate postoperative period. Patients included 11 with pulmonary arterial stenosis, five with stenosis of venous pathways after a modified Fontan procedure or bidirectional cavopulmonary anastomosis, and four with a restrictive modified Blalock-Taussig shunt. Placement of the stent was optimal in 18 of 20 patients (24 of 26 Palmaz or Palmaz-Schatz implants). In two patients, rupture of the balloon resulted in malposition of the stent. Reoperation was avoided and symptomatic improvement was noted in 11 of 20 patients (55%), while procedural complications occurred in eight patients. Seven of 10 patients presenting with a low cardiac output syndrome died despite relief of the obstructions. This early experience supports consideration of the application of these devices in the management of significant vascular obstructive lesions in the immediate postoperative period, thus avoiding early reoperation in this profoundly compromised population.

Interventional catheterization procedures performed via the arterial route are well recognized therapies which are being employed with increasing frequency. This review summarizes our experience with local arterial complications of such procedures performed on 44 patients in our institution since 1985. A total of 51 balloon dilations were performed. All patients were heparinized during the procedure. Twenty-one procedures (41%) were followed by clinical evidence of reduced arterial circulation to the affected leg more than four hours after the procedure. Continuing infusion of heparin was used in 17 patients, with return of the pulse concerned in six, while two further patients developed evidence of arterial obstruction after an interval (having initially been found to have ‘satisfactory’ pulses at the end of the procedure). The final infant, whose femoral artery had been torn and could not be repaired, was observed without continuing heparinization. Streptokinase and/or tissue plasminogen activator was employed in eight patients, with resultant success in four. The remaining three, and three who failed thrombolytic therapy, underwent embolectomy. The incidence of arterial damage was related to the size of the catheter (assessed by the size of sheath required to introduce the collapsed balloon, indexed to body surface area) and to low weight or young age at the time of the procedure. No relationship could be established with other variables, including operator, type of catheter, or year or duration of procedure. At long-term follow-up, 11 (22%) patients still have absent or reduced pulses in the leg concerned. Arterial damage following interventional catheterization procedures remains a frequent occurrence despite increasing experience and technical improvements.

There are limited reports in the literature that address the unique issues related to cardiac catheterization on extracorporeal membrane oxygenation. Since 1990, cardiac catheterization has been performed on nine patients on extracorporeal membrane oxygenation. The median age was 10 months (range four days to 18 years) and the median weight was 4 kg (range 2.3–73.8 kg). The indications for catheterization were to relieve pulmonary edema in five patients with severe left ventricular dysfunction, and in one instance each, evaluation of probable anomalous left coronary artery, tetralogy of Fallot associated with a diaphragmatic hernia, possible pulmonary venous abnormalities, and postoperative evaluation after an arterial switch. Venous access was obtained through a preexisting femoral venous line in six patients, and through a cutdown of the saphenous or femoral vein in the other three. Interventional catheterization procedures were performed in six of the nine patients in this group. These consisted of blade atrial septostomy followed by balloon septostomy or dilation of the atrial septum in four patients, balloon septostomy alone in one, and placement of a catheter from the left atrium to the circuit used for extracorporeal oxygenation in another. A transseptal puncture was performed in five of them. Adequate decompression of the left atrium with relief of pulmonary edema was achieved in five patients. Six of the nine children in this group died, although there were no complications or deaths related to the catheterization. Children in cardiopulmonary failure on extracorporeal support represent a population at high risk. Diagnostic cardiac catheterization and atrial septostomy to decompress the left heart, when indicated, can be performed safely in such patients.

Long-term follow-up studies after intraatrial repair for complete transposition have shown postoperative morphologic and hemodynamic abnormalities in many patients which often require reevaluation and therapeutic intervention. The purpose of this study was to assess, using magnetic resonance imaging, the presence and extent of late postoperative complications in a large cohort of 44 patients undergoing repair with the Mustard procedure. Of these 34 had an intact ventricular septum with or without obstruction of the left ventricular outflow tract, while 10 had an associated ventricular septal defect. Transverse spin-echo and gradient-echo images were acquired of the entire heart from the diaphragm to the bifurcation of the pulmonary trunk. Additional oblique images were acquired for better visualization of venous connections. Abnormal findings were diagnosed by visual inspection of spin-echo and gradient-echo images, and diagnoses were compared to previous findings at cardiac catheterization. On magnetic resonance imaging, a baffle leak was seen in eight patients, two had pulmonary venous obstruction, four had obstruction at the caval veins, 23 had obstruction of the left ventricular outflow tract and 18 had tricuspid regurgitation. Cardiac catheterization showed a leak across the baffle in 16, pulmonary venous obstruction in two, obstruction at the caval veins in four, obstruction of the left ventricular outflow tract in nine, and tricuspid regurgitation in nine. We conclude that combined spin-echo and gradient-echo magnetic resonance imaging provides extensive noninvasive assessment of postoperative sequels and residuals in patients after the Mustard procedure for complete transposition.

Atrial correction for complete transposition involves a potential risk of later development of right ventricular dysfunction. To determine the reasons contributing to such systemic ventricular failure, radioisotope first-pass and SPECT perfusion studies at rest after one dose of Technetium-99m MIBI were performed in 26 asymptomatic patients, 5.9±2.7 years after a Mustard or Senning repair. Mean age at operation was 4.3±4.3 years. All patients were followed with 24-hour Holter monitoring and echocardiography. Normal perfusion was observed in 11 patients with a mean age at operation of 2.25±1.8 years and mean right ventricular ejection fraction of 49.0±8.7%. Perfusion defects were found in 15 patients with a mean age at operation 5.8±4.8 years (p<0.05) and mean right ventricular ejection fraction of 43.2±9.3% (NS). In the group of older patients, 10 had extensive perfusion defects with significantly lower right ventricular ejection fraction (39.7±9.3%, p<0.05) and even older mean age at the surgery (7.1±5.6 years p<0.02). Mild or moderate tricuspid regurgitation was found significantly more frequently in those patients with perfusion defects (73%) than in the group of younger patients (36.3%), p<0.05. There was no difference in the frequency and type of arrhythmias, nor in the extent of follow-up, between the two groups. We conclude that, in patients undergoing surgery at an older age, it is more common to find perfusion defects and, often, lower right ventricular ejection fraction. Perfusion abnormalities found in such asymptomatic patients may be a result of several factors: impaired right ventricular function as a consequence of pressure and volume overload, enhanced myocardial fibrosis, or long-lasting preoperative hypoxemia and tricuspid valvar dysfunction secondary to ventricular dilation. We suggest that the perfusion defects observed in this study may be a better indicator of systemic ventricular impairment.

We performed ten operations in children using a lower mid-line incision and limited minimal sternotomy. The skin incision is made from the level of nipples to the lower end of the xiphoid process, and only the body of the sternum is cut vertically. There was neither operative mortality nor complications. The upper limit of the scar was, at the most, 1 cm beyond the level of the nipples, and was difficult to observe in typical open-necked shirts. We believe our method is suitable in fulfilling the cosmetic needs for eligible children.

We describe the symptoms, the techniques for imaging and the surgical therapy in a patient with divided right atrium (‘cor triatriatum dexter’) combined with atresia of the superior caval vein. In addition to peripheral venous congestion and cyanosis, macrocephaly was the most prominent sign, the high pressure in the superior caval vein disturbing the circulation of cerebrospinal fluid. Transesophageal echocardiography was the optimal technique for imaging the dividing shelf in the right atrium. Cardiac catheterization after surgical excision of the shelf and reconstruction of the junction between the superior caval vein and the right atrium excluded any residual gradient or shunt.

We describe a multicystic tumor of the atrial septum associated with a fatal cardiac malformation. The tumor contained bronchogenic cysts and two types of vascular hamartomas. A complex form of double outlet right ventricle was present. We speculate that this tumor formed at a critical time in cardiac embryogenesis and contributed to the abnormal formation of the heart.

An intravascular stent was successfully implanted in a 15-year-old gid with stenosis of the left pulmonary artery subsequent to a modified Fontan correction. Complaints of tiredness, edema, ascites and enlargement of the left breast disappeared. Unfortunately, she died of a rhythm disturbance unrelated to the stent. Autopsy revealed excellent endothelialization of the stent without any thrombosis and with patency of the orifice of the branch to the upper lobe of the lung.

A combined procedure of a bilateral bidirectional cavopulmonary shunt and a central aortopulmonaiy shunt was performed on an 18-year-old boy with pulmonary atresia. Arterial oxygen saturation was elevated after the combined shunt, whereas the ventricular stroke work was reduced. Such a combined shunt is a useful alternative prior procedure which does not produce ventricular overload in older or higher-risk candidates for the Fontan procedure.

A 37-year-old man with tricuspid atresia, in whom a chronic right apical aspergilloma had stimulated formation of extensive aortopulmonary collateral circulation, suffered reversal of flow within his long-standing fistulous Glenn anastomosis. By reversing the flow through the pulmonary arteriovenous fistula and raising oxygen saturation in the right atrium, the acquired aortopulmonary collateral circulation prevented the increase in cyanosis which usually occurs when fistulous changes develop late after the Glenn operation.