Establishment of Cardiac Laterality.

Formation of the vertebrate heart with its complex arterial and venous connections is critically dependent on patterning of the left-right axis during early embryonic development. Abnormalities in left-right patterning can lead to a variety of complex life-threatening congenital heart defects. A highly conserved pathway responsible for left-right axis specification has been uncovered. This pathway involves initial asymmetric activation of a nodal signaling cascade at the embryonic node, followed by its propagation to the left lateral plate mesoderm and activation of left-sided expression of the Pitx2 transcription factor specifying visceral organ asymmetry. Intriguingly, recent work suggests that cardiac laterality is encoded by intrinsic cell and tissue chirality independent of Nodal signaling. Thus, Nodal signaling may be superimposed on this intrinsic chirality, providing additional instructive cues to pattern cardiac situs. The impact of intrinsic chirality and the perturbation of left-right patterning on myofiber organization and cardiac function warrants further investigation. We summarize recent insights gained from studies in animal models and also some human clinical studies in a brief overview of the complex processes regulating cardiac asymmetry and their impact on cardiac function and the pathogenesis of congenital heart defects.

Modified Senning procedure in a patient with dextrocardia with left atrial isomerism and anomalous systemic venous drainage: a rare case report.

The atrial switch procedure by Senning or Mustard technique primarily aims in correcting parallel systemic and pulmonary circulations at atrial level. This procedure may be used in late presenting D-transposition of great arteries with a deconditioned left ventricle, congenitally corrected transposition of great arteries and isolated ventricular inversion. We describe the case of a child with dextrocardia, left atrial isomerism with complex pulmonary and systemic venous drainage resulting in mixing at atrial level. She was successfully operated by modified Senning procedure performed through the left-sided atrium.

Contemporary Outcomes of Heart Transplantation in Children with Heterotaxy Syndrome: Sub-Optimal Pre-Transplant Optimization Translates into Early Post-Transplant Mortality.

Patients with heterotaxy syndrome and congenital heart disease (CHD) experience inferior cardiac surgical outcomes. Heart transplantation outcomes are understudied, however, particularly compared to non-CHD patients. Data from UNOS and PHIS were used to identify 4803 children (< 18 years) undergoing first-time heart transplant between 2003 and 2022 with diagnoses of heterotaxy (n = 278), other-CHD (n = 2236), and non-CHD cardiomyopathy (n = 2289). Heterotaxy patients were older (median 5 yr) and heavier (median 17 kg) at transplant than other-CHD (median 2 yr and 12 kg), and younger and lighter than cardiomyopathy (median 7 yr and 24 kg) (all p < 0.001). UNOS status 1A/1 at listing was not different between groups (65-67%; p = 0.683). At transplant, heterotaxy and other-CHD patients had similar rates of renal dysfunction (12 and 17%), inotropes (10% and 11%), and ventilator-dependence (19 and 18%). Compared to cardiomyopathy, heterotaxy patients had comparable renal dysfunction (9%, p = 0.058) and inotropes (46%, p = 0.097) but more hepatic dysfunction (17%, p < 0.001) and ventilator-dependence (12%, p = 0.003). Rates of ventricular assist device (VAD) were: heterotaxy-10%, other-CHD-11% (p = 0.839 vs. heterotaxy), cardiomyopathy-37% (p < 0.001 vs. heterotaxy). The 1-year incidence of acute rejection post-transplant was comparable between heterotaxy and others (p > 0.05). While overall post-transplant survival was significantly worse for heterotaxy than others (p < 0.05 vs. both), conditional 1-year survival was comparable (p > 0.3 vs. both). Children with heterotaxy syndrome experience inferior post-heart transplant survival, although early mortality appears to influence this trend, with 1-year survivors having equivalent outcomes. Given similar pre-transplant clinical status to others, heterotaxy patients are potentially under risk-stratified. Increased VAD utilization and pre-transplant end-organ function optimization may portend improved outcomes.

Post-operative Morbidity and Mortality After Fontan Procedure in Patients with Heterotaxy and Other Situs Anomalies.

Heterotaxy is a complex, multisystem disorder associated with single ventricle heart disease and decreased survival. Ciliary dysfunction is common in heterotaxy and other situs abnormalities (H/SA) and may increase post-operative complications. We hypothesized that patients with H/SA have increased respiratory and renal morbidities and increased in-hospital mortality after Fontan procedure. We queried the Pediatric Health Information System database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 through 11 years from 2004 to 2019. H/SA was identified by codes for dextrocardia, situs inversus, asplenia/polysplenia, or atrial isomerism and compared to non-H/SA controls. Outcomes were in-hospital mortality or heart transplantation, ECMO, hemodialysis, length of stay (LOS), and mechanical ventilation or vasoactive medication use ≥ 4 days. We adjusted estimates with multivariable logistic regression. Of 7897 patients at 50 centers, 1366 (17%) met criteria for H/SA. H/SA had worse outcomes for all study measures: death/transplantation (1.9 vs 1.1%, OR 1.74 (95% CI 1.01-3.03); p = 0.047), ECMO (3.7 vs 2.3%, OR 1.74 (1.28-2.35); p < 0.001), hemodialysis (2.1 vs 1.2%, OR 1.66 (1.06-2.59); p = 0.026), prolonged mechanical ventilation (13.2% vs 7.6%, OR 1.85 (1.53-2.25); p < 0.001) and vasoactive medication use (29.4 vs 19.7%, OR 1.65 (1.43-1.90), and longer LOS (11 (8-17) vs 9 (7-14) days; p < 0.001). H/SA is associated with increased cardiovascular, renal, and respiratory morbidity, as well as in-hospital mortality after Fontan procedure. Attention to renal and respiratory needs may improve outcomes in this difficult population. The relationship between ciliary dysfunction and lung and renal morbidity should be explored further.

Balloon atrial septostomy through azygos vein in two cases of D-TGA with left atrial isomerism.

Balloon atrial septostomy is a palliative procedure that is performed in D-transposition of great arteries when surgery is not immediately available. Although D-TGA and left isomerism association are rare, it is an important condition as the BAS procedure approach is unique. In this case report, we present two cases of D-TGA with left isomerism where BAS was performed due to restrictive atrial septal defect and lack of immediate availability of the paediatric cardiac surgeon.

Prenatal diagnosis of hepatic interruption of the inferior vena cava with azygos/hemiazygos continuation without structural heart defects: A case series.

OBJECTIVES: To describe fetal spectrum and echocardiographic characteristics of interrupted inferior vena cava (IIVC) with azygos/hemiazygous continuation without other structural heart defects and to evaluate its association with visceral heterotaxy and isomerism, extracardiac and genetic anomalies, and to review neonatal outcome. METHODS: This was a retrospective study of 14 fetuses with a confirmed diagnosis of IIVC with normal intracardiac anatomy. The following variables were collected; indication for referral, gestational age at diagnosis; associated isomerism and visceral heterotaxy, heart rhythm, genetic and extracardiac abnormalities, and fetal/neonatal outcome. RESULTS: Among 36 fetuses with IIVC, 14 cases (38.8%) had normal intracardiac anatomy. These IIVC cases correspond to 0.19% (14/7250) of all fetal cardiac examinations, and to 1.5% (14/922) of all cardiac abnormalities. Six patients had visceral abnormalities. Atrial appendage morphology was clearly depicted in three fetuses, both appendages were left. One fetus had bradyarrhythmia revealing atrial ectopic rhythm. Six fetuses did not have any concomitant cardiac or visceral abnormalities, therefore regarded as isolated. All babies were delivered at term with a good prognosis. CONCLUSION: Our study has shown that almost half of the IIVC cases without intracardiac structural anomalies displayed other findings of isomerism while the other half was isolated benign vascular variant. Therefore, prenatal diagnosis of IIVC should prompt a comprehensive evaluation for cardiac, situs, and visceral anomalies. The outcome is favorable.

Cardiac surgery in patients with atrial isomerism: Long-term results and outcomes.

PURPOSE: The aim of the study was to identify, determine, and analyze the clinical and surgical outcomes of patients with atrial isomerism (AI) undergoing cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of AI undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2010 and March 31, 2020 were included; demographic characteristics and perioperative conditions of the patients were considered. RESULTS: Sixty-five patients were included, with an average age of 6.4 ± 4.9 years, 50.8% males. Thirty-eight (58.5%) had right atrial isomerism (RAI) and 27 (41.5%) had left atrial isomerism (LAI); univentricular physiology (78.5%) predominated. Atrioventricular septal defect (AVSD) in RAI and septal defects in LAI were identified as the main associated defects. The most common surgical procedures performed were modified Blalock-Taussig shunt (MBTS) (27.6%), MBTS with total anomalous pulmonary venous connection (TAPVC) repair (15.3%) and total cavopulmonary connection (TCPC) with an extracardiac conduit fenestrated (10.8%); 100% RAI required a univentricular approach, while in LAI it was 48.1%. Overall survival was 92.3%, with 100% survival in LAI with biventricular physiology and 86.8% in RAI with univentricular physiology. CONCLUSIONS: The survival of our institution is similar to that of other referral centers, where patients with LAI had a better evolution than RAI; in addition, the univentricular approach was required in all with RAI. Patients with AI must undergo a rigorous evaluation to determine an adequate repair strategy, considering univentricular RAI with a high possibility of morbidity and mortality.

The multisystem nature of isomerism: left isomerism complicated by Abernethy malformation and portopulmonary hypertension.

Isomerism, also referred to as "heterotaxy" is a complex set of anatomic and functional perturbations. One of the most obvious manifestations of isomerism is the disturbance of organ arrangement, such that the thoracic organs are no longer asymmetric on the left and right. We report the case of a 14-year-old female in whom exercise-induced dyspnea led to a late diagnosis of left isomerism complicated by Abernethy malformation and portopulmonary hypertension. A comprehensive evaluation revealed two anatomic left lungs and hyparterial bronchi, bilateral left atria, an interrupted inferior caval vein with azygos continuation, multiple spleens, sinus node dysfunction, hepatic hypertrophy with focal nodular hyperplasia, and absence of the portal vein. Pulmonary vasodilator therapy was initiated resulting in clinical improvement. This case exhibits unique features including a late diagnosis of isomerism with Abernethy malformation and portopulmonary hypertension. The patient's presentation, medical workup, and future treatment emphasise the importance of multidisciplinary care in children with complex multisystem disease. We review the multiple cardiac and extracardiac manifestations of isomerism.

Retrograde sharp potential resembling the his-bundle electrogram: is this a case of atrioventricular re-entrant tachycardia involving twin atrioventricular nodes with the anterior node exhibiting only retrograde conduction?

A 2-year-old male with right isomerism was referred for supraventricular tachycardias. Atrial pacing study revealed that anterograde conduction was only through the posterior atrioventricular node. During the mapping of ventriculoatrial conduction, we identified a sharp potential resembling a His-bundle electrogram with a decremental property at the anterior wall of the common atrium. Catheter ablation for the potential eliminated the anterior ventriculoatrial conduction, thereby indicating retrograde activation of the possible anterior atrioventricular node.

Hepatic vein redirection to improve pulmonary arteriovenous malformations after Fontan completion in patients with absence of inferior vena cava and hemiazygos continuation.

Pulmonary arteriovenous malformation is an important complication after Fontan completion in patients with univentricular circulation. Lack of hepatic venous flow in a pulmonary artery has been identified as a cause of pulmonary arteriovenous malformation. We report our experience with a case of redirection of the hepatic vein to the hemiazygos vein using a conduit via left thoracotomy and median sternotomy for the correction of unequal distribution of hepatic venous flow in the pulmonary arteries. The pulmonary arteriovenous malformation improved, leading to increased arterial saturation levels.

Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis.

OBJECTIVES: The main aim of this systematic review was to evaluate the prevalence and type of associated anomalies in fetuses with heterotaxy diagnosed prenatally on ultrasound; the perinatal outcome of these fetuses was also studied. METHODS: An electronic search of MEDLINE, EMBASE and CINAHL databases was performed. Only studies reporting the prenatal diagnosis of isomerism were included. Outcomes observed included associated cardiac and extracardiac anomalies, fetal arrhythmia, abnormal karyotype, type of surgical repair and perinatal mortality. The analysis was stratified according to the type of heterotaxy syndrome (left (LAI) or right (RAI) atrial isomerism). Meta-analyses of proportions were used to combine data. Quality assessment of the included studies was performed using the Newcastle-Ottawa Scale for cohort studies. RESULTS: Sixteen studies (647 fetuses) were included in the analysis. Atrioventricular septal defect was the most common associated major cardiac anomaly found both in fetuses with LAI (pooled proportion (PP), 59.3% (95% CI, 44.0-73.7%)), with obstructive lesions of the right outflow tract occurring in 35.5% of these cases, and in fetuses with RAI (PP, 72.9% (95% CI, 60.4-83.7%)). Fetal arrhythmias occurred in 36.7% (95% CI, 26.9-47.2%) of cases with LAI and were mainly represented by complete atrioventricular block, while this finding was uncommon in cases with RAI (PP, 1.3% (95% CI, 0.2-3.2%)). Abnormal stomach and liver position were found, respectively, in 59.4% (95% CI, 38.1-79.0%) and 32.5% (95% CI, 11.9-57.6%) of cases with LAI, and in 54.5% (95% CI, 38.5-70.1%) and 45.9% (95% CI, 11.3-83.0%) of cases with RAI, while intestinal malrotation was detected in 14.2% (95% CI, 2.5-33.1%) of LAI and 27.1% (95% CI, 7.9-52.0%) of RAI cases. Hydrops developed in 11.8% (95% CI, 2.9-25.6%) of fetuses diagnosed prenatally with LAI. Biventricular repair was accomplished in 78.2% (95% CI, 64.3-89.4%) of cases with LAI, while univentricular repair or palliation was needed in 17.0% (95% CI, 9.7-25.9%); death during or after surgery occurred in 26.8% (95% CI, 4.6-58.7%) of LAI cases. Most children with RAI had univentricular repair and 27.8% (95% CI, 15.5-42.1%) died during or after surgery. CONCLUSIONS: Fetal heterotaxy is associated with a high prevalence of cardiac and extracardiac anomalies. Approximately one quarter of fetuses with heterotaxy died during or after surgery. Abnormal heart rhythm, especially heart block, is common in fetuses with LAI, while this finding is uncommon in RAI. Biventricular repair was common in LAI while univentricular repair was required in the majority of children affected by RAI. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Primary Draining Vein Stenting for Obstructive Total Anomalous Pulmonary Venous Connection in Neonates with Right Atrial Isomerism and Functional Single Ventricle Improves Outcome.

For neonates with right atrial isomerism (RAI), functional single ventricle (f-SV), and obstructive total anomalous pulmonary venous connection (TAPVC), primary TAPVC repair (TAPVCR) has a poor outcome. At our hospital, the survival rate at 1 year of such neonates undergoing primary TAPVCR between 1999 and 2010 (TAPVCR group) was 30% (3/10). Most deceased cases suffered from capillary leak syndrome and unstable pulmonary resistance after the surgeries. We sought to determine whether less invasive primary draining vein stenting (DVS) improved the outcome of these neonates. We investigated outcomes in consecutive nine such neonates (median gestational age 38 weeks, birth weight 2.8 kg, females 4) who underwent primary DVS with 6-mm-diameter Palmaz® Genesis® stents at our hospital between 2007 and 2017 (DVS group). Eight patients underwent subsequent surgeries to adjust the pulmonary flow after decreased pulmonary resistance. The survival rate at 1 year after the first interventions in the DVS group improved to 77% (7/9), although there was a difference between the interventional eras of the two groups. Of the seven patients who underwent multiple stent redilations with a larger balloon or additional stenting in other sites until the next stage of surgery at a median age of 8 months, four received a bidirectional Glenn (BDG) shunt and TAPVCR and three underwent TAPVCR, with two of those cases reaching BDG. Less invasive primary DVS improved the outcome of neonates with RAI, f-SV, and obstructive TAPVC, with many reaching BDG. Patient selection to advance toward Fontan is thought to further improve the outcome.

An alternative technique for Fontan completion in patients with widely separated connections of the inferior systemic veins to the atrial mass.

Several surgical approaches for completion of a total cavopulmonary connection have been established for patients with anomalies of systemic venous connection in association with a functionally univentricular heart. We report an alternative technique designed to address the problem of widely separated inferior systemic veins, in which two extracardiac conduits were used. This technique was performed on three patients and yielded excellent short-term results, allowing us to operate on a beating heart with no need for cardioplegic arrest and aortic cross-clamping.

Individual Pulmonary Veins Outgrow Somatic Growth After Primary Sutureless Repair for Total Anomalous Pulmonary Venous Drainage.

Indications of sutureless repair (SR) for pulmonary vein anomalies have evolved from re-operational SR for pulmonary vein stenosis after the repair of total anomalous pulmonary venous drainage (TAPVD) to primary SR for TAPVD associated with right atrial isomerism or isolated TAPVD with small individual pulmonary veins (IPVs) and an unfavorable pulmonary vein anatomy. We sought to determine whether small IPVs outgrow somatic growth after primary SR. Between 2004 and 2013, 21 children underwent primary SR for TAPVD: 13 with a functionally single ventricle, 11 with right atrial isomerism, six with isolated TAPVD, and 13 with a pulmonary venous obstruction. TAPVD types were supracardiac in nine, infracardiac in 10, and mixed in two. Utilizing cardiac computed tomography (CT), the maximal diameter of each IPV was measured, and pulmonary vein index (PVI, summation of cross-sectional areas of all four IPVs divided by body surface area) was calculated. There were five early deaths after SR. Among survivors, 10 had both preoperative and postoperative cardiac CT at a 3.6-month median interval. On postoperative cardiac CT, IPVs were patent in all patients except one who developed a left lower pulmonary vein obstruction. There was a 71 ± 48 % postoperative increase in the actual diameter of all four IPVs, and PVI increased significantly from 215 ± 55 to 402 ± 117 mm(2)/m(2) (P value = 0.005). IPVs outgrew somatic growth after primary SR of TAPVD. Primary SR may be a useful measure in TAPVD patients whose IPVs are small.

Left Atrial Isomerism in Afro-Caribbean Adolescents and Adults with no Intervention: Echocardiographic Findings.

Left atrial isomerism (LAI) is a rare congenital anomaly usually associated with major or minor congenital cardiac lesions. Presentation beyond childhood is unusual as there is usually high morbidity of 63% associated with the co-morbid major congenital cardiac lesions in the first year of life. The four index cases highlight the myriad cardiac lesions facilitating survival into adolescence and adulthood of this rare cardiac condition. Presented are markedly different echocardiographic findings of four cases of LAI in Afro-Caribbeans who had no intervention. Their diagnostic transthoracic echocardiographic findings are the focussed features of this article and the first documentation of isolated patent ductus arteriosus with LAI. Fascinating diagnostic chest X-ray findings of the LAI index cases are presented. They are the first cases of LAI documented in Afro-Caribbeans.

Severe bacterial infection in patients with heterotaxy syndrome.

OBJECTIVE: To determine the incidence of sepsis in patients with heterotaxy syndrome. STUDY DESIGN: From our institutional database, we identified patients with heterotaxy syndrome and other complex congenital heart disease (CHD) born between 2001 and 2011. Severe bacterial infection was defined as sepsis with positive culture result or infection with abscess formation. RESULTS: We enrolled 95 patients with heterotaxy syndrome (88 with right atrial isomerism and 7 with left atrial isomerism) and 142 patients with complex CHD. With 1026 person-years follow-up, the 5-year survival was 52% and 65.7% in heterotaxy and complex CHD groups, respectively (P = .239). Community-acquired severe bacterial infection occurred only in heterotaxy syndrome (13 episodes in 10 patients, 3 of whom had spleen noted at imaging study) with 2- and 5 years cumulative severe bacterial infection rate of 9.6% and 14.5%, respectively. The overall mortality rate of those with community-acquired severe bacterial infection was 31%. Pneumococcus and Citrobacter freundii were the most common pathogens. Nosocomial severe bacterial infection occurred in 33.3% of all patients and 12.5% of all procedures. The rates (0.59 and 0.52/100 hospitalization days in heterotaxy and complex CHD group) and the pathogens of nosocomial severe bacterial infection were similar between heterotaxy and complex CHD groups. CONCLUSIONS: Patients with heterotaxy syndrome are at high risk for community-acquired severe bacterial infection and also have high mortality rate whether the spleen is present or not. The risk of nosocomial severe bacterial infection seems similar to that of patients with other complex CHD.

Noncompaction in the fetus and neonate: an autopsy study.

Noncompaction refers to an uncommon structural abnormality of the heart's ventricular myocardium characterized by an abnormally thick layer of left ventricular trabeculations, as well as hypoplastic papillary muscles. The condition is associated with a variable clinical phenotype including heart failure, thromboembolism, and sudden death. In this retrospective study of fetal and neonatal autopsy hearts with noncompaction, clinical profiles were correlated with gross and histologic findings and compared with a set of age-matched controls. Pathologic criteria for noncompaction included hypoplastic left ventricular papillary muscles, abnormal trabecular architecture and greater than 50% penetration of the left ventricular wall thickness by intertrabecular recesses. Among eight fetuses and full-term neonates with pathologic features of noncompaction, all had evidence of severe heart failure, including four with complete heart block and two others with bradycardia. None experienced sudden death. Seven of eight hearts had associated heart malformations-four with left atrial isomerism, two with aortic and/or pulmonary valve dysplasia consistent with stenosis and, one with atrial septal defect. One heart with noncompaction had no associated malformations. With characteristic excessive trabeculation in the left ventricle, noncompaction also included biventricular endocardial fibroelastosis that denoted right ventricular involvement in all hearts. Thus, (1) among autopsied fetuses and neonates with noncompaction, heart failure including heart block is a common cause of death, (2) noncompaction is often associated with various cardiovascular malformations, but even in isolation it can be the basis for severe cardiac failure, and (3) biventricular endocardial fibroelastosis in noncompaction suggests a global pathologic process.

Magnetic navigation in adults with atrial isomerism (heterotaxy syndrome) and supraventricular arrhythmias.

AIMS: We analysed the type and mechanism of supraventricular arrhythmias encountered in a series of symptomatic adults with atrial isomerism undergoing catheter ablation procedures. METHODS AND RESULTS: The study population included consecutive adults with atrial isomerism who had previously undergone surgical repair or palliation of the associated anomalies. Patients underwent electrophysiological study for symptomatic arrhythmia in our institution between 2010 and 2012 using magnetic navigation in conjunction with CARTO RMT and three-dimensional (3D) image integration. Eight patients (five females) with a median age of 33 years [interquartile range (IQR) 24-39] were studied. Access to the cardiac chambers of interest was obtained retrogradely via the aorta using remotely navigated magnetic catheters in six patients. Radiofrequency ablation successfully targeted twin atrioventricular (AV) nodal reentrant tachycardia in two patients, atrial fibrillation (AF) in three, focal atrial tachycardia (AT) mainly originating in the left-sided atrium in four patients, and macro-reentrant AT dependent on a right-sided inferior isthmus in three patients. The median fluoroscopy time was 3.0 min (IQR 2-11). After a median follow-up of 10 months (IQR 6-21), five of the ablated patients are free from arrhythmia; two patients experienced episodes of self-terminated AF and AT, respectively, within one month post-ablation; the remaining patient had only non-sustained AT during the electrophysiological study and was managed medically. CONCLUSION: Various supraventricular tachycardia mechanisms are possible in adults with heterotaxy syndrome, all potentially amenable to radiofrequency ablation. The use of remote magnetic navigation along with 3D mapping facilitated the procedures and resulted in a short radiation time.

Cardiac Laterality: Surgical Results of Right Atrial Isomerism.

Right atrial isomerism (RAI) is a complex entity with varying diagnostic and treatment outcomes due to its rarity. Treatment options range from palliative to corrective surgeries, resulting in heterogeneous outcomes. The aim of this study was to analyze the results obtained after cardiac surgery in patients with RAI. A retrospective study was conducted, including patients diagnosed with RAI who underwent cardiac surgery. Their follow-up was from 1 January 2010 to 31 March 2020. Demographic characteristics and perioperative conditions were described. Thirty-eight patients were included, the median age was 4 years (IQR 2-9.2) and 57.9% were men. The main diagnoses were atrioventricular canal (63.2%) and pulmonary stenosis (55.3%). The most common surgical procedures were modified Blalock-Taussig shunt (65.8%) and total cavopulmonary connection with an extracardiac conduit fenestrated without cardiopulmonary bypass (15.9%). We did not find any factors associated with negative outcomes in these patients. The overall survival was 86.8%, with a better outcome in those who did not require reintubation (log rank, p < 0.01). The survival of RAI was similar to other centers. Individuals with RAI should be evaluated rigorously to determine an adequate repair strategy, considering high morbidity and mortality.