Atrial Isomerism Research Articles

Cardiac rhythm and symptomatic arrhythmia in right atrial isomerism

Background The conduction system in right atrial isomerism may be complicated by the presence of paired sinus nodes and twin atrioventricular nodes. With a large cohort of infants and children with right atrial isomerism, we sought to investigate the nature of atrial rhythm, its prevalence, factors predisposing patients to symptomatic cardiac arrhythmia, and the relation of arrhythmia to long-term morbidity and mortality. Methods Standard 12-lead baseline electrocardiograms were performed in 110 infants and children with right atrial isomerism at a median age of 1 day, and their clinical records were reviewed. The type, timing, and precipitating factors of symptomatic cardiac arrhythmia that occurred in 15 patients, among a cohort of 85 patients who had or were awaiting surgical interventions, were noted. Results All patients except 1 had a sinus rhythm with intact atrioventricular conduction. Of these, 87% (95/109) had single P-wave morphology, whereas 13% (14/109) had multiple P-wave morphologies. For patients with a single P-wave morphology, the frontal P-wave axis was between 0 degrees and 90 degrees in 62% (59/95), 90 degrees and 180 degrees in 23% (22/95), and superior in 15% (14/95). There was no relation between either P-wave axis or morphology and cardiac anatomy. Symptomatic cardiac arrhythmia occurred in 15 of 85 patients (18%); 11 of the 15 patients had supraventricular tachycardia, and 1 patient each had atrial tachycardia, atrial flutter, ventricular tachycardia, and congenital complete heart block. The arrhythmias occurred before surgery in 4 patients, early after surgery in 5 patients, and late after surgery in 6 patients. Although 3 of the 7 patients who died suddenly had a history of symptomatic arrhythmia, arrhythmia was the documented cause of mortality in only 1 of the 32 fatalities (3.1%). Freedom from arrhythmia at 1, 5, 10, 15, and 20 years was 93% ± 3%, 86% ± 4%, 80% ± 6%, 73% ± 9%, and 48% ± 15% (mean ± SE), respectively. No risk factors for symptomatic arrhythmia were identified by means of logistic regression. Conclusions The atrial pacemaker varies in location within and between patients with right atrial isomerism. Although symptomatic cardiac arrhythmias are not uncommon, they do not seem to relate to the overall high mortality rate and occurrence of sudden death in this patient group. Nonetheless, detailed assessment and aggressive management of cardiac arrhythmias once they occur are warranted because of the precarious single ventricular hemodynamics. (Am Heart J 2002;144:159-64.)

Gene‐dosage‐sensitive genetic interactions between inversus viscerum (iv), nodal, and activin type IIB receptor (ActRIIB) genes in asymmetrical patterning of the visceral organs along the left‐right axis

We have shown previously that mice deficient in the activin type IIB receptor (ActRIIB) exhibit right isomerism, which is characterized by mirror-image symmetrical right lungs, complex cardiac malformations, and hypoplasia of the spleen. These observations led us to hypothesize that the signaling of a TGF-beta family member by means of ActRIIB is necessary for the determination of the left-sidedness of the visceral organs. To test this hypothesis, we examined laterality defects in mice carrying mutations in both ActRIIB and inversus viscerum (iv) genes, because iv(-/-) mice display a spectrum of laterality defects, including situs inversus, right isomerism, and left isomerism. We found that all mice homozygous for both iv and ActRIIB mutations displayed the right isomerism. The phenotype of right isomerism in the double mutants was also more severe than that in ActRIIB(-/-) mice as shown by persistent left inferior vena cava, right atrial isomerism, and hypoplasia of spleen. Interestingly, the incidence of right isomerism also increased significantly in iv(-/-);ActRIIB(+/-) and iv(+/-);ActRIIB(-/-) mice compared with homozygous mice carrying either of single gene mutations. A mechanism of the genetic modulation between ActRIIB and iv genes may be that iv modulates the asymmetric expression of a TGF-beta family member that signals through activin type II receptors, ActRIIA and ActRIIB, to specify the "left-sidedness." Nodal is the most likely candidate. We show here that the penetrance and severity of the right isomerism is significantly elevated in nodal(+/-); ActRIIB(-/-) mice, compared with ActRIIB(-/-) mice. Furthermore, the chimeric mice derived from nodal(-/-) ES cells displayed right isomerism, indistinguishable from that in (iv(-/-);ActRIIB(-/-)) mice. We propose that iv functions to establish asymmetric expression of nodal in a gene-dosage-sensitive manner and that nodal signals through the activin type II receptors to specify the left-sidedness by means of a threshold mechanism.

Cardinal vein isomerism: An embryological hypothesis to explain a persistent left superior vena cava draining into the roof of the left atrium in the absence of coronary sinus and atrial septal defect

Background: A persistent left superior vena cava (PLSVC) is a relatively frequent systemic venous anomaly associated with congenital heart defects. This anomaly has been explained with the persistence of the left superior cardinal vein. PLSVC usually drains into the right atrium, via coronary sinus, but it joins the left atrium in approximately 8% of the cases either directly in the setting of atrial isomerism, or via an unroofed coronary sinus, or through a coronary sinus type atrial septal defect. Case report: We describe a case of an adult patient with atria in the situs solitus, PLSVC draining into the left atrium, atresia of coronary sinus without atrial septal defect, and with additional cardiac anomalies (ventricular septal defect and discrete subaortic stenosis). Conclusion: A possible embryological explanation to this case rises from a right partial isomerism of the superior cardinal veins, which gives reason for both the coexistence of the PLSVC draining into the left atrium and the absence of coronary sinus, atrial septal defect, or coronary sinus ostium.

Invited commentary

Surgical palliation for patients with right atrial isomerism requiring a total cavopulmonary connection presents a formidable technical challenge due to the anatomic variability described in the present report of Aeba and colleagues. Separate orifices for the hepatic veins and inferior vena cava, a single orifice for entry of the pulmonary veins into the left atrium after surgical repair of anomalous venous drainage, and the relative location of the atrioventricular valve comprise the obstacles which must be circumvented to create unobstructed flow from the hepatic veins and inferior vena cava to the pulmonary artery while maintaining unobstructed flow between the pulmonary veins and the atrioventricular valve.

Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes

Background. The historic outcome of the Fontan procedure in children with single ventricle and heterotaxy syndrome has been poor, and in the current era it has been incompletely described. Methods. From January 1993 through April 2000, 30 patients (age range, 5.3 ± 3.6 years) had total cavopulmonary connection for single ventricle and isomerism and heterotaxy syndrome. Right atrial isomerism and heterotaxy (n = 19) or left atrial isomerism and heterotaxy (n = 11) were associated with a morphologic right ventricle (n = 26), a common atrioventricular valve (n = 25), pulmonary atresia (n = 9) or stenosis (n = 17), anomalous pulmonary venous drainage (partial, n = 7; total, n = 11; obstructed, n = 4), and interrupted inferior vena cava (n = 13). A previous systemic to pulmonary artery shunt had been performed in 21 patients, and 3 patients had previous pulmonary artery banding. The Fontan procedure was staged in all but 3 patients at a median age of 12 months (range, 3 to 114 months). Before the Fontan procedure, atrial arrhythmia was present in 7 patients (23%). Results. An extracardiac conduit was constructed in 22 patients (median diameter, 20 mm; range, 16 to 27 mm), and a lateral tunnel was constructed in 4 patients. Associated procedures included pulmonary artery augmentation (n = 17), repair of anomalous pulmonary venous drainage (n = 7), and repair of a regurgitant atrioventricular valve (n = 2). The mean cardiopulmonary bypass time was 134 ± 57 minutes. Cardioplegic cardiac arrest was used in 12 patients for a mean duration of 62 ± 28 minutes. There were four hospital deaths (13%), three that occurred in children having concomitant repair of anomalous pulmonary venous drainage (previously undetected in one). Thirteen children (41%) developed early postoperative atrial arrhythmias, 11 of the 13 children required temporary pacing. There was one late death. Follow-up (3.4 ± 2.7 years) was available on 24 of 25 survivors. All but 2 patients were in sinus rhythm. Conclusions. Early and midterm outcomes after the Fontan procedure in this patient group improved and may be further ameliorated by detecting and repairing associated obstruction to pulmonary venous flow and performing atrioventricular valvuloplasty before the Fontan procedure.

Cardiac rhythm disturbances in patients with left atrial isomerism.

This long-term study sought to determine the clinical implication of defective sinus node and AV conduction tissue in patients with left atrial isomerism (LAI). From 1984 to 1998, a total of 22 patients were identified as LAI. Patient age at the last follow-up ranged from 2 to 276 months (90+/-70 months). Associated cardiac anomalies were interruption of the inferior vena cava (n = 18, 82%), common atrium (n = 9, 41%), AV canal (n = 14, 64%), double-outlet right ventricle (n = 8, 36%), and pulmonary stenosis (n = 15, 68%). Palliative interventions were performed in 16 patients (Fontan-type operation in 4 patients, shunt followed by Fontan-type operation in 2, repair of septal defect in 4, and extracardiac intervention in 6). During the follow-up, over half of the patients (n = 14, 64%) developed bradyarrhythmia (onset age: from 1 to 264 months; median 78 months): junctional rhythm (n = 11), sinus bradycardia (n = 8) (5 patients also had junctional rhythm), and AV block (n = 2, both also had junctional rhythm). The probability free from bradyarrhythmia was 80% and 46% at the age of 2 and 6 years, respectively. None of the bradyarrhythmias were directly related to open-heart surgery. Besides, junctional ectopic tachycardia occurred after Fontan-type operation in three of six patients. In two patients, a Mahaim-like pathway was identified during the electrophysiological study. The patients with LAI had a high probability of developing bradyarrhythmias due to abnormal sinus node function. Varied AV conduction abnormalities may include compromised AV conduction, junctional ectopic tachycardia after Fontan-type operation, and an association of Mahaim-like pathway.

Prenatal diagnosis of cardiosplenic syndromes by fetal echocardiography: a 10‐year experience

Purpose: To assess the accuracy of fetal echocardiography in the diagnosis of cardiosplenic syndromes.Methods: Retrospective survey of 27 fetuses with the pre‐ or postnatal diagnosis of cardiosplenic syndromes in our database over a period of 10 years.Results: In 24 out of 27 fetuses, the diagnosis of cardiosplenic syndrome was confirmed postpartum. In the remainder, two cases of situs inversus totalis with heart block and multiple cardiac malformations, but no evidence of heterotaxy were diagnosed at autopsy or after birth. In one fetus with situs ambiguus, dextrocardia, bradyarrhythmia and transposition of great arteries (TGA) no autopsy was performed after termination of pregnancy. In 18 fetuses, left atrial isomerism were prenatally diagnosed and confirmed at autopsy or postpartum. Main echocardiographic features were azygos continuation of interrupted inferior caval vein (15), complete atrioventricular septal defect (CAVSD) (11), persistent bradyarrhythmia (11), pulmonary stenosis (PS) (9), ventricular septal defect (VSD) (8), fetal hydrops (8) and dextrocardia (5). Other associated cardiac malformations were l‐TGA (2), double outlet right ventricle (DORV) (3), hypoplastic left ventricle (3), and partial/total anomalous venous connection (1/1). Ten pregnancies were terminated, two fetuses demised in utero, six children survived. In six fetuses, right atrial isomerism was prenatally diagnosed and confirmed postpartum. Echocardiographic features were CAVSD (4), dextrocardia (4), PS (2), l‐TGA (1), azygos continuation of interrupted inferior caval vein (1), fetal hydrops (1), DORV (1) and bradyarrhythmia with cardiomegaly (1). One pregnancy was terminated, two fetuses demised within 10 days postpartum, three children survived.Conclusion: A combination of CAVSD, first and early second trimester persistent bradyarrhythmia and/or azygos continuation of interrupted inferior caval vein, and/or visceral situs ambiguus should suggest specific diagnosis of cardiosplenic syndrome. The correct diagnosis made in utero allows for maternal counseling and transfer to an appropriate facility prior to delivery for corrective surgery.

Congenital Heart

Most publications on atrial isomerism are autopsy or case reports. The authors review 41 consecutive children operated on from 1980 through to 1996 with emphasis on associated cardiac anomalies, surgical procedures and outcome. Left atrial isomerism was present in 23 patients. Interruption of the inferior vena cava (56%), atrio-ventricular septal defect (47%), common atrium (38%) and cor triatriatum sinistrum (30%) were the most common diagnoses. Biventricular repair was achieved in 17 children and total cavo-pulmonary connection in two. Three underwent staged palliation: modified Blalock–Taussig shunt for two and bidirectional Glenn anastomosis for one. The remainder received a cardiac pacemaker. One patient died early after repair. Two underwent reoperation to correct a regurgitant left atrio-ventricular valve: one of these, in another hospital, had peroperative death. Three died later. Actuarial survival rate after repair and total cavo-pulmonary connection that was stabilized after 2 years was 84%. In the 18 children with right atrial isomerism, pulmonary atresia or stenosis predominated (89%) with discordant ventriculo-arterial connection (72%), atrio-ventricular septal defect (72%), 'single' ventricle (55%) and extracardiac total anomalous pulmonary venous drainage (50%). Biventricular repair was achieved in two patients and complete Fontan circulation in eight. The other eight underwent various staged palliative procedures and correction of extracardiac total anomalous pulmonary venous drainage. Five patients died postoperatively: two in our unit after modified Blalock–Taussig shunt and total cavo-pulmonary connection, three in other hospitals after repair (n=1) and Fontan (n=2). Five died later. One was lost for review. Survival after repair and Fontan stabilized after 6 months at 49%. In conclusion, the cardiovascular malformations associated with left atrial isomerism can often be successfully corrected. Those accompanying right atrial isomerism usually preclude a biventricular repair, require staged palliation and carry a poor prognosis.

Anatomically corrective repair of complete atrioventricular septal defects and major cardiac anomalies

Background. Although satisfactory results of corrective surgery for atrioventricular septal defects (AVSDs) with complex lesions such as double-outlet right ventricle (DORV) or atrial isomerism have been reported in recent years, the optimal surgical options for isomerism hearts is still a controversial issue. Methods. We performed anatomically corrective repair on 13 children with balanced forms of complete AVSDs and associated major cardiac anomalies. Eight of the 13 patients had atrial isomerism (right in 1, left in 7), 5 of whom had DORV. Four others had DORV with trisomy 21, and 1 had tetralogy of Fallot. Atrial septation for isomerism with the placement of an additional prosthesis was performed on 4 patients. Results. Two hospital deaths and one late death occurred only in the isomerism group. Three reoperations were required solely in the left isomerism group: one replacement of the valved external conduit concomitantly with reconstruction of the left ventricular outflow obstruction, one mitral valve replacement for severe regurgitation of left atrioventricular valve, and one relief of progressing left ventricular outflow obstruction. Conclusions. The results of anatomically corrective surgery for AVSDs with major associated cardiac anomalies in the nonisomerism group were excellent. The optimal surgical options for isomerism heart, however, remain a controversial issue.

MLC3F transgene expression in iv mutant mice reveals the importance of left-right signalling pathways for the acquisition of left and right atrial but not ventricular compartment identity.

Abstract Transcriptional differences between left and right cardiac chambers are revealed by an nlacZ reporter transgene controlled by regulatory sequences of the MLC3F gene, which is expressed in the left ventricle (LV), atrioventricular canal (AVC), and right atrium (RA). To examine the role of left-right signalling in the acquisition of left and right chamber identity, we have investigated MLC3F transgene expression in iv mutant mice. iv/iv mice exhibit randomised direction of heart looping and an elevated frequency of associated laterality defects, including atrial isomerism. At fetal stages, 3F-nlacZ-2E transgene expression remains confined to the morphological LV, AVC, and RA in L-loop hearts, although these appear on the opposite side of the body. In cases of morphologically distinguishable right atrial appendage isomerism, both atrial appendages show strong transgene expression. Conversely, specimens with morphological left atrial appendage isomerism show only weak expression in both atrial appendages. The earliest left-right atrial differences in the expression of the 3F-nlacZ-2E transgene are observed at E8.5. DiI labelling experiments confirmed that transcriptional regionalisation of the 3F-nlacZ-2E transgene at this stage reflects future atrial chamber identity. In some iv/iv embryos at E8.5, the asymmetry of 3F-nlacZ-2E expression was lost, suggesting atrial isomerism at the transcriptional level prior to chamber formation. These data suggest that molecular specification of left and right atrial but not ventricular chambers is dependent on left-right axial cues.

Pitx2 Expression Defines a Left Cardiac Lineage of Cells: Evidence for Atrial and Ventricular Molecular Isomerism in the iv/iv Mice

The homeobox gene Pitx2 has been characterized as a mediator of left-right signaling in heart, gut, and lung morphogenesis. However, the relationship between the developmental role of Pitx2 and its expression pattern at the organ level has not been explored. In this study we focus on the role of Pitx2 in heart morphogenesis. Chicken Pitx2 transcripts are present in the left portion of the cardiac crescent and in the left side of the heart tube. Through looping Pitx2 is present in the left atrium, in the ventral portion of the ventricles and in the left-ventral part of the outflow tract. Mouse Pitx2 shows a similar developmental profile of expression. To test whether Pitx2 represents a lineage marker we have tagged the left portion of the chicken cardiac tube with fluorescent DiD. Labeled cells were found at HH16 in the left atrium and in the ventral region of the ventricles and the outflow tract. In the iv/iv mouse model of cardiac heterotaxia Pitx2 was abnormally expressed in the atrial and in the ventricular chambers. Furthermore, altered Pitx2 expression correlated with the occurrence of DORV. Our data reveal the existence of molecular isomerism not only in the atrial, but also in the ventricular compartment of the heart.

Outcomes of left atrial isomerism over a 28-year period at a single institution

Outcomes of left atrial isomerism over a 28-year period at a single institution

Infradiaphragmatic totally anomalous pulmonary venous return with two separate descending veins in association with right atrial isomerism

We present the case of a 1-month-old infant with right atrial isomerism and complex functionally univentricular heart disease, in whom totally anomalous pulmonary venous return drained below the diaphragm via two separate vertical veins. One of the descending vertical veins drained the entire right lung and the left upper lobe, while the other drained the remainder of the left lung. Only one similar case has been described previously.

F58Anatomical variation of the fetal venous system

ObjectiveThis report presents our experience in the prenatal diagnosis of anomalies of fetal veins using high‐resolution and color Doppler ultrasound.DesignIn an observatorial study 15 cases of abnormalities of the umbilical, portal, hepatic and caval venous system have been diagnosed at our division over the past 5 years. The abnormality, the underlying embryologic disorder and the outcome of the pregnancy are presented.ResultsIn group A, 8 fetuses had an abnormal course of the umbilical vein with a patent (n = 3) or absent (n = 5) ductus venosus. No portal veins and absent or abnormal hepatic veins were visualized. Six fetuses (75%) did not have an associated malformation and have survived. Two pregnancies were terminated or ended in fetal demise. In group B, 7 fetuses with an abnormal caval system had a left (n = 5) and right (n = 2) atrial isomerism with a cardiac defect in 6 cases (86%). The pregnancies ended in 4 terminations of pregnancy and two infant deaths. Only an infant with a left atrial isomerism and a normal heart has survived.ConclusionIn a targeted fetal scan the course of the venous system should be carefully examined using color Doppler. Any suspicious finding should be followed by a detailed assessment of the specifity of this abnormality considering the embryological development as well as the associated malformations.

Outcomes of left atrial isomerism over a 28-year period at a single institution

OBJECTIVESWe determined long-term outcomes in a large cohort with left atrial isomerism (LAI).BACKGROUNDLeft atrial isomerism is associated with a complex spectrum of cardiac and noncardiac anomalies that may impact on outcomes.METHODSThe records of all patients with LAI, born between 1970 and 1998, and treated at one center were reviewed. Kaplan-Meier survival was estimated, and independent factors associated with time-related death were identified.RESULTSThere were 163 patients (63% women), and extracardiac anomalies were noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, atrioventricular septal defect in 49%, pulmonary atresia or stenosis in 28% and aortic coarctation in 16%, with congenital atrioventricular block in 7%. Of 22 patients with a normal heart, 18% died of extracardiac anomalies. Of 71 patients with hearts suitable for biventricular repair, 62 (87%) had surgery, with survival of 80% at one year, 71% at five years, 66% at 10 years and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, 47 (67%) had surgery, with survival of 73% at one year, 61% at five years, 53% at 10 years and 48% at 15 years (p < 0.001). Independent factors associated with time-related death included congenital atrioventricular block, aortic coarctation, single ventricle, biliary atresia and other gastrointestinal malformations.CONCLUSIONSBoth cardiac and noncardiac anomalies contribute to a high mortality with LAI. Cardiac transplantation may need to be a considered a primary option for selected high-risk patients.

Heterotaxy: Associated conditions and hospital-based prevalence in newborns

PurposeTo provide insight into the possible etiology and prevalence of heterotaxy, we studied conditions associated with heterotaxy in a consecutive hospital population of newborns. MethodsFrom 1972 to March, 1999 (except February 16, 1972 to December 31, 1978), 58 cases of heterotaxy were ascertained from a cohort of 201,084 births in the ongoing Active Malformation Surveillance Program at the Brigham and Women’s Hospital. This registry includes livebirths, stillbirths, and elective abortions. Prevalence among nontransfers (i.e., patients whose mothers had planned delivery at this hospital) was calculated as approximately 1 per 10,000 total births (20 of 201,084). ResultsWe analyzed a total of 58 patients consisting of 20 (34%) nontransfers and 38 (66%) transfers. Patients were categorized by spleen status as having asplenia (7 nontransfers, 25 total), polysplenia (8, 20), right spleen (4, 11), normal left (0,1), and unknown (1, 0). Among the 20 nontransfer and 59 total heterotaxy patients, the following associated medical conditions were present: chromosome abnormality (1 nontransfer, 2 total), suspected Mendelian or chromosome microdeletion disorder (1 nontransfer, 6 total), and maternal insulin-dependent diabetes mellitus (1 nontransfer, 2 total). There were 6 twins (1 member each from 6 twin pairs including 1 dizygous, 4 monozygous, 1 conjoined; 2 were nontransfers). An associated condition occurred in 5 (25%) nontransfer and 16 (28%) total patients, or among 10 of 53 singleton births (19%). ConclusionsAlthough most cases of heterotaxy in this series were sporadic events, an associated condition was present in about one-fourth of the cases. Not all of these conditions would be considered causative etiologies. Based on this small series alone, maternal insulin-dependent diabetes cannot be viewed as a risk factor for heterotaxy. However, the specific association of diabetes with polysplenia with/without left atrial isomerism is noteworthy, and adds weight to animal and epidemiologic case-control data.

Prenatal diagnosis of abnormalities of the fetal venous system.

To present our experience in the prenatal diagnosis of anomalies of fetal veins using high-resolution color Doppler ultrasound. An observational study of 16 fetuses with abnormalities of the umbilical, portal, hepatic and caval venous system being diagnosed at the Division of Prenatal Diagnosis and Therapy (Bonn, Germany) over the past 5 years. The abnormality of the venous system, the underlying embryologic disorder and the outcome of the pregnancy are presented and compared with the literature. In group A, eight fetuses had an abnormal course of the umbilical vein with a patent (n = 3) or absent (n = 5) ductus venosus. No portal veins and absent or abnormal hepatic veins were visualized by color Doppler sonography. Six fetuses (75%) did not have an associated malformation and have survived. Two pregnancies with fetal hydrops due to a small heart and to Turner's syndrome were terminated or ended in fetal demise. In group B, seven of eight fetuses with an abnormal caval system had a situs ambiguus or an atrial isomerism. A cardiac defect was detected in six cases (86%). These six pregnancies ended in four terminations of pregnancy and two infant deaths due to the severity of the congenital cardiac defect. One child with a normal heart and a child with an isolated abnormal course of the lower inferior vena cava are developing well. In a targeted fetal scan the course of the umbilical vein, ductus venosus, the portal and hepatic veins and inferior vena cava should be carefully examined using color Doppler. Any suspicious finding should be followed by a detailed assessment of the specificity of this abnormality taking into consideration the embryologic development of the fetal venous system together with the associated malformations.

Radiofrequency catheter ablation of tachycardia in children with and without congenital heart disease: indications and limitations

From 1993 to 1998, a total of 100 consecutive pediatric patients with tachycardia (45 male and 55 female, aged 1 year 10 months to 17 years, 11±4 year) who underwent electrophysiological study were reviewed. Eleven of them were younger than 5 years. Two had tachycardia-related cerebrovascular accident. Congenital heart disease was found in 12 patients. After propofol anesthesia, the clinical tachycardia could not be induced in three (two atrial tachycardia and one AV nodal re-entrant tachycardia) and became nonsustained in five (atrial tachycardia). Mechanical ablation occurred in three and two had subsequent recurrences. Among the 85 cases who received radiofrequency ablation, the overall final success rate of RF ablation for all diagnoses was 94% with a diagnosis-specific success rate ranging from 100 to 57%. Tachycardia cardiomyopathy was noted in four (three atrial tachycardia and one junctional ectopic tachycardia) and all regressed after successful ablation. Success in two patients with left posterioseptal accessory pathway could only be achieved by delivering the energy at the middle cardiac vein. Two patients with right atrial isomerism had an ‘AV nodal-to-AV nodal tachycardia’ which was eliminated by ablation. Total recurrence rate was 13% but final success was achieved in all during re-study except the three patients who refused re-intervention. The atrial tachycardia developed in postoperative congenital heart disease was associated with the lowest success rate (57%) and highest recurrence rate (25%). Procedure-related complications occurred in four; two with transient brachial palsy, one with first-degree AV block and one with blood loss requiring blood transfusion. In conclusion, the experience of this single center confirmed the efficacy and safety of radiofrequency catheter ablation in treating pediatric arrhythmias, but the limitations in postoperative arrhythmias and the effects of propofol on tachycardia induction (especially the atrial tachycardia) need to be improved.

New indicator for the Fontan operation: diameters of the pulmonary veins in patients with univentricular heart.

Operative survival after the Fontan procedure is good; however, there are some patients with disappointing results, especially those with atrial isomerism. We tested whether the diameter of the pulmonary veins, which is reported as a useful indicator of pulmonary blood flow, predicts operative results after the Fontan operation. We evaluated 30 consecutive patients undergoing either the bidirectional Glenn anastomosis (BDG) or the Fontan operation. Age at operation ranged from 3 to 81 months (mean 30). Diagnosis was right or left isomeric heart in 15 patients, double-outlet right ventricle in 4 and various other malformations in 11. BDG was performed in 16 patients and the Fontan operation in 14 patients. The diameters of the pulmonary veins were measured proximal to the entrance into the atrium in the late phase of a pulmonary arteriogram. The pulmonary vein (PV) index (in mm2/m2) was calculated from the sum of the cross-sectional areas of these veins divided by the body surface area. Of the patients undergoing BDG (+/- ancillary procedures), 12 had successful results and 4 had unsuccessful results. The PV index for hemodynamically successful patients was 361 +/- 153 and 275 +/- 60 mm2/m2 (mean +/- SD) for unsuccessful patients (p = 0.30). Of the patients who underwent the Fontan operation, 13 had successful and 1 had unsuccessful results. The PV index for successful patients was > 285 mm2/m2 and 137 mm2/m2 for the nonsuccessful patients. The new pulmonary vascular resistance (PVR) calculated by using the PV index (mean pressure difference between the pulmonary artery and the atrium/PV index) for BDG patients with successful or unsuccessful results was 2.0 +/- 0.5 or 3.5 +/- 0.2 mmHg/mm2 per m2, respectively (p < 0.01). The new PVR for Fontan patients with successful results was < 2.0 mmHg/mm2 per m2, while that for the patient with an unsuccessful result was 4.4. The new PVR completely separated patients into successful and unsuccessful groups, while conventionally calculated PVR did not (p = 0.63). PV index appears to be a useful morphological indicator of pulmonary blood flow and "new" PVR may improve the decision-making strategy for patients presenting with univentricular heart, especially those associated with isomeric heart.

Atrial isomerism: a surgical experience

Most publications on atrial isomerism are autopsy or case reports. The authors review 41 consecutive children operated on from 1980 through to 1996 with emphasis on associated cardiac anomalies, surgical procedures and outcome. Left atrial isomerism was present in 23 patients. Interruption of the inferior vena cava (56%), atrio-ventricular septal defect (47%), common atrium (38%) and cor triatriatum sinistrum (30%) were the most common diagnoses. Biventricular repair was achieved in 17 children and total cavo-pulmonary connection in two. Three underwent staged palliation: modified Blalock–Taussig shunt for two and bidirectional Glenn anastomosis for one. The remainder received a cardiac pacemaker. One patient died early after repair. Two underwent reoperation to correct a regurgitant left atrio-ventricular valve: one of these, in another hospital, had peroperative death. Three died later. Actuarial survival rate after repair and total cavo-pulmonary connection that was stabilized after 2 years was 84%. In the 18 children with right atrial isomerism, pulmonary atresia or stenosis predominated (89%) with discordant ventriculo-arterial connection (72%), atrio-ventricular septal defect (72%), 'single' ventricle (55%) and extracardiac total anomalous pulmonary venous drainage (50%). Biventricular repair was achieved in two patients and complete Fontan circulation in eight. The other eight underwent various staged palliative procedures and correction of extracardiac total anomalous pulmonary venous drainage. Five patients died postoperatively: two in our unit after modified Blalock–Taussig shunt and total cavo-pulmonary connection, three in other hospitals after repair ( n=1) and Fontan ( n=2). Five died later. One was lost for review. Survival after repair and Fontan stabilized after 6 months at 49%. In conclusion, the cardiovascular malformations associated with left atrial isomerism can often be successfully corrected. Those accompanying right atrial isomerism usually preclude a biventricular repair, require staged palliation and carry a poor prognosis.