A Wide Comparison of Techniques for Repair of PAPVCs: One Institution's 20-Year Experience.

BACKGROUND: Different methods for surgical correction of partial anomalous pulmonary venous connection (PAPVC) exist. We evaluated the outcomes of four techniques regarding morbidity and mortality. METHODS: A total of 116 patients underwent PAPVC repair in our institution over a period of 20 years. Single-patch technique (n = 82 [71%], mean age: 18.59 ± 20.49 years), double-patch technique (n = 13 [11%], mean age: 43.18 ± 25.14 years), Warden's technique (n = 7 [6%], mean age: 10.04 ± 10.47 years), and direct implantation of anomalous pulmonary veins (n = 14 [12%], mean age: 14.42 ± 18.58 years) were examined. RESULTS: Out of the 116 patients, one patient (0.9%) developed pulmonary hypertension after discharge and three patients (2.6%) with normal right cardiac function showed right ventricular failure. In total, a pacemaker was inserted in seven cases (6%). Three patients (2.6%) presented with persistent nonsinus rhythm during follow-up. This complication was most frequently seen in the double-patch group being significantly increased compared with the other groups (p = 0.035). One patient presented with a mild stenosis of the superior vena cava. There were two early, nonsurgery-related deaths and no late mortality. CONCLUSION: Operative therapy of PAPVC has low postoperative morbidity and mortality. Therefore, surgical repair of this cardiac anomaly is a safe and reproducible treatment independent of the applied method. The surgical technique must be selected based on the anatomy and possible accompanying congenital heart defects. Special care should be taken when using the double-patch technique because of significant more frequent nonsinus rhythm events postoperatively.

Trends in Infant Mortality After TAPVR Repair over 18 Years in Texas and Impact of Hospital Surgical Volume.

For some congenital heart lesions, higher institutional surgical volume has been associated with better survival than in lower volume centers. The relationship between institutional surgical volume and mortality in infants after total anomalous pulmonary vein return (TAPVR) repair has not been well explored. The Texas Inpatient Public Use Data File was queried for hospitalizations including TAPVR repair in infants between January 1, 1999 and December 31, 2016. We first evaluated the change in mortality over the study period. We then evaluated associations between institutional TAPVR surgical volume and mortality using univariable analysis and multivariable analysis accounting for center effects. For secondary analyses, we evaluated the association between volume and mortality among non-mutually exclusive TAPVR subsets, including isolated TAPVR, TAPVR with other congenital heart disease (CHD), TAPVR with heterotaxy, and TAPVR with single ventricle anatomy. Of 971 surgical hospitalizations that met inclusion criteria, 62% were male. Mortality after TAPVR repair decreased over the study period from 15.1% (1999-2004) to 7.6% (2012-2016) with an odds ratio per increasing year of 0.96 (95% CI 0.92-0.99, p = 0.030). By univariable analysis, earlier era, preterm birth, lower institutional surgical volume, heterotaxy, and additional CHD were associated with increased mortality. Institutional surgical volume remained significant in multivariate analysis with an odds ratio per increase in surgical volume of every 10 patients of 0.93 (95% CI 0.90-0.96, p < 0.001). When examining by subgroup, isolated TAPVR had the lowest mortality (n = 606, mortality = 6%), compared to TAPVR with other CHD (n = 359, mortality = 20%), TAPVR with heterotaxy (n = 135, mortality = 21%), and TAPVR with single ventricle (n = 128, mortality = 23%). In all groups except those with single ventricle, higher surgical volume was associated with lower mortality in multivariate analyses (isolated TAPVR p = 0.001, TAPVR with other CHD p = 0.009, TAPVR with heterotaxy p < 0.001, TAPVR with single ventricle p = 0.161). This is the first study to demonstrate an association between institutional surgical volume and mortality after TAPVR repair. Higher volume centers are associated with lower hospital mortality after TAPVR repair, including TAPVR with other CHD.

Surgical Outcomes for Obstructive Total Anomalous Pulmonary Venous Return of the Non-Infracardiac Type Compared with the Infracardiac Type.

BACKGROUND: Infracardiac obstructive total anomalous pulmonary venous return (TAPVR) has a poor outcome following surgical correction. We compared the surgical outcomes of obstructive TAPVR between non-infracardiac and infracardiac types. METHODS: Among 51 patients who underwent surgical repair for obstructive TAPVR, 23 with infracardiac type and 28 with non-infracardiac type were included in this investigation. The study compared the immediate postoperative courses in the intensive care unit and long-term mortality and pulmonary vein stenosis. The risk factors for long-term survival in obstructive TAPVR also were investigated. RESULTS: The postoperative follow-up period was 79.8 ± 81.5 months. Immediate major operative complications were observed in 22 patients (43.1%); 10 patients (19.6%) died, and eight patients (15.7%) experienced pulmonary vein stenosis during the follow-up period. The Kaplan-Meier curve showed better cumulative survival in patients with infracardiac TAPVR (P = 0.308). The significant factors for survival after surgical repair of obstructive TAPVR did not include anatomical type but instead were postoperative course of ventilator care and lengths of intensive care unit and hospital stays. CONCLUSION: Patients with non-infracardiac TAPVR with obstruction had a longer postoperative course and experienced more complications. Their survival rate was poorer, and postoperative pulmonary vein stenosis was more frequent in those patients compared with infracardiac TAPVR patients. However, a large-scale study is mandatory to gather more data and confirm our findings.

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single-secondary center experience.

BACKGROUND AND AIM OF THE STUDY: Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect. This study aimed to evaluate the outcome of isolated TAPVC repairs and the prognoses of affected patients in the last 12 years at a single center. METHODS: We retrospectively analyzed the medical records of 51 patients who underwent isolated TAPVC repair from 2007 to 2018. RESULTS: The median age at operation was 19 days, and the median body weight was 3.3 kg. Thirteen (25.5%) patients had emergency operations, and the median follow-up period was 29.54 ± 36.77 months. Early mortality was noted in five patients and late mortality was noted in one patient. Pulmonary vein stenosis was observed in 22 patients within 3 to 6 months after the operation, and six patients required reoperation or transcatheter interventions. Low birth weight, small left atrial volume, long operation time, and preoperative heart failure were identified as risk factors for mortality. CONCLUSIONS: Isolated TAPVC can rapidly lead to hemodynamic instability during the neonatal period and is associated with high mortality rates. Increasing the prenatal diagnosis rate and stabilizing the patients' condition before the operation are considered important for improving the surgical outcome.

Predictors of In-Hospital Mortality in Isolated Total Anomalous Pulmonary Venous Connection.

BACKGROUND: We assessed risk factors for early mortality in isolated total anomalous pulmonary venous connection over a modern era excluding emergent cases to eliminate the influence of associated factors on surgical outcome.  Methods: 70 patients with isolated total anomalous pulmonary venous connection who were repaired electively between January 2013 and February 2018 were included. RESULTS: In-hospital mortality was encountered in 4 patients (5.7%). Upon univariate analysis, low age (P = .003) and weight (P = .001) at surgery, preoperative pulmonary venous obstruction (P = .010), preoperative low oxygen saturation (P = .031), long cardiopulmonary bypass (P = .001) and aortic cross clamp (P = .003) times, long duration of mechanical ventilation (P = .001), chest infection (P = 0.041), postoperative low CO syndrome (P < .001) and long postoperative inotropic support (P = .015) were significant predictors of in-hospital mortality. In multivariate analysis postoperative low cardiac output syndrome (OR: 1.060; 95% CI: 1.008-1.116) and prolonged postoperative mechanical ventilation (OR: 1.772; 95% CI: 1.141-2.751) were independent factors of in-hospital mortality. CONCLUSION: Surgical repair of TAPVC is now performed with acceptable results. According to our study, postoperative low cardiac output syndrome and prolonged postoperative mechanical ventilation were the most significant predictors for early mortality.

Clinical outcomes after the endovascular treatments of pulmonary vein stenosis in patients with congenital heart disease.

BACKGROUND: Pulmonary vein stenosis (PVS) is a condition with challenging treatment and leads to severe cardiac failure and pulmonary hypertension. Despite aggressive surgical or catheter-based intervention, the prognosis of PVS is unsatisfactory. This study aimed to assess the prognosis and to establish appropriate treatment strategies. METHODS: We retrospectively reviewed endovascular treatments for PVS (2001-2017) from the clinical database at the Okayama University Hospital. RESULTS: A total of 24 patients underwent PVS associated with total anomalous pulmonary venous connection and 7 patients underwent isolated congenital PVS. In total, 53 stenotic pulmonary veins were subjected to endovascular treatments; 40 of them were stented by hybrid (29) and percutaneous procedures (11) (bare-metal stent, n = 34; drug-eluting stent, n = 9). Stent size of hybrid stenting was larger than percutaneous stenting. Median follow-up duration from the onset of PVS was 24 months (4-134 months). Survival rate was 71 and 49% at 1 and 5 years, respectively. There was no statistically significant difference between stent placement and survival; however, patients who underwent bare-metal stent implantation had statistically better survival than those who underwent drug-eluting stent implantation or balloon angioplasty. Early onset of stenosis, timing of stenting, and small vessel diameter of pulmonary vein before stenting were considered as risk factors for in-stent restenosis. Freedom from re-intervention was 50 and 26% at 1 and 2 years. CONCLUSIONS: To improve survival and stent patency, implantation of large stent is important. However, re-intervention after stenting is also significant to obtain good outcome.

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.

Total Anomalous Pulmonary Venous Connection: A 40 years' Experience Analysis.

Background Total anomalous pulmonary venous connection is a rare cardiac malformation associated with significant morbidity and mortality rates. We report a large surgical series study to evaluate mid-term and long-term results of conventional surgical techniques. Methods and Results We performed a retrospective analytic study of all patients operated on for simple total anomalous pulmonary venous connection in the University Hospital of Lyon, France, between January 1973 and June 2014. A total of 180 patients were included (43% supracardiac, 27% intracardiac, 19% infracardiac, and 11% mixed types). Mean cardiopulmonary bypass and aortic cross clamp times were respectively 66 and 39 minutes. Overall mortality was 27.1%, including 38 early deaths (21.1%) and 12 late deaths (6.1%). The percentage of early death greatly decreased over the eras, from 42.1% in the seventies to 7.4% after 2010. Besides the earlier era of intervention (p < 0.0001), significant risk factors for death in multivariate analysis were preoperative pulmonary hypertension, acidosis, and cardiopulmonary bypass time. There were 24 reoperations, including 7 for pulmonary venous obstruction; 6 died. Factors directly and independently associated with late complications were the anatomic type (mixed forms, p = 0.0023), and length of aortic cross clamp time (p = 0.01). Long-term results for survivals are excellent. We report 84.7% of asymptomatic patients with a mean follow-up of 10.8 years. Conclusions The overall prognosis of total anomalous pulmonary venous connection repair with conventional procedures has greatly improved over the years with excellent long-term results. A thorough evaluation of all preoperative characteristics is imperative to achieve the best outcome.

A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution.

BACKGROUND: Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome. METHODS: We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015. RESULTS: A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04). CONCLUSION: The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

Surgical outcomes of total anomalous pulmonary venous connection repair: a 22-year experience.

BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes. METHODS: Data from medical records of patients who underwent surgical repair for TAPVC from 1989 to 2012 were included. The patients were divided in two groups, according to absence or presence of associated major CHDs. RESULTS: Forty-six patients were included (M/F: 26/20, median age 26 days, interquartile range 15 to 59, median weight 3.350 kg, interquartile range 1800 to 4470). Anatomic types of TAPVC were: supracardiac in 48%, intracardiac in 20%, infracardiac in 20%, and mixed in 12%; TAPVC was obstructive in 33%; TAPVC was isolated in 63%, complex in 37%. Single ventricle physiology was present in 11 patients, heterotaxy in eight patients. Overall operative mortality was 19.6% (9/46): 6.9% in isolated TAPVC, 41.2% in complex type (p-value: 0.002). It was associated with low weight at intervention (<3 kg, p = 0.027), single ventricle physiology (p = 0.047), and aortic cross-clamp time >60 minutes (p = 0.097). At a median follow-up of 2.97 years (range 43 days to 22 years, 91% complete), there were nine late deaths (24.3%); 15 patients (40.5%) had major events (including late death). Multivariate analysis of event-free survival showed worse outcome in the complex group (p = 0.001). CONCLUSION: Surgical mortality and morbidity remain consistent in patients with TAPVC and associated major CHD, while the isolated type shows excellent outcomes. Pulmonary vein obstruction is a rare but highly lethal complication. doi: 10.1111/jocs.12399 (J Card Surg 2014;29:678-685).

Factors associated with early postoperative mortality after total anomalous pulmonary venous connection repair: A retrospective cohort study.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect where pulmonary venous plexus fails to connect with the left atrium (LA). Surgical repair is the primary treatment for TAPVC, but factors influencing outcomes are not fully understood. This study investigates the early outcomes of surgical repair for TAPVC and associated factors. A retrospective cohort analysis was conducted on TAPVC patients who underwent surgical repair between 2012 and 2022. Data were collected from medical records and supplemented with phone call validation. Demographic characteristics, surgical data, diagnostic tests, and outcomes were analyzed. Statistical analysis included chi-square, t-tests, and multivariate logistic regression using SPSS. A total of 88 patients underwent surgical repair for TAPVC, resulting in a mortality rate of 21.6%. Weight and bypass time were significantly associated with patient survival. Female patients had a higher likelihood of death. The anatomic type did not significantly influence mortality. Patients with pulmonary venous obstruction (PVO) experienced a higher mortality rate. Notably, ligation of the vertical vein in supracardiac and infracardiac types was associated with lower mortality. In conclusion, our study identifies several key factors contributing to higher mortality rates following TAPVC surgery, including low weight, female gender, prolonged bypass time, and preoperative vein obstruction. Highlighting the significance of surgical technique, particularly the sutureless approach, we advocate for its meticulous consideration to achieve improved outcomes. Furthermore, our findings indicate a potential decrease in mortality associated with vertical vein ligation, which may mitigate the risk of post-repair heart failure. We suggest further rigorous studies to gain comprehensive insights into TAPVC surgical interventions.

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia.

Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.

A 15-Year Experience With Total Anomalous Pulmonary Venous Connection in Vietnam.

BACKGROUND: This article aims to demonstrate the morphology of 261 total anomalous pulmonary venous connection (TAPVC) cases operated at Children's Hospital 1 with in-hospital mortality of 19.5% (51/261). METHODS: All the surgical protocols of TAPVC cases repaired between 2008 and June 2023 were reviewed. The descriptions of TAPVC were based on operative findings by surgeons. RESULTS: A total of 261 TAPVC patients were operated, including 124 (47.5%) supra, 83 (31.8%) intra, 41 (15.7%) infra, and 13 (5%) mixed cases. The in-hospital mortality was 19.5% (51/261). Fifteen cases are associated with other anomalies of the heart. Four subtypes of 124 supra TAPVC were found, with 42 (33.9%) obstructed cases. The standard was all pulmonary veins (PVs) forming a common vein (CV) and draining into the innominate veins, then going to the superior vena cava (SVC) (100/124, 80.6%). Eleven supra TAPVC cases were vascular vise type. Ten cases had the vertical vein running from the right of the CV and draining directly into the SVC. Of 83 intracardiac TAPVCs with 9 (10.8%) obstructed cases, the most common was all PVs draining directly into the coronary sinus (60/83, 72.3%). The second was all PVs draining directly into the right atrium (RA) via separated ostia or forming a CV before entering the RA (17/83, 20.5%). Also, there were three cases with rare variants and 100% obstruction when the diagnosis was explored. The in-hospital mortality of intracardiac type was 13.3% (11/83) 41 infra TAPVC with obstructed rate of 61% (25/41) and in-hospital mortality of 29.3% (12/41). Thirteen mixed TAPVCs were repaired, with most cases having three PVs forming a CV. CONCLUSION: This article provides valuable information about the morphology of TAPVC types in Asian patients.

Outcome predictors and implications for management of scimitar syndrome.

BACKGROUND: Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome. METHODS: The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. RESULTS: Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention. CONCLUSIONS: Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

Early and intermediate term results for surgical correction of total anomalous pulmonary venous connection.

BACKGROUND: This retrospective study evaluated factors associated with mortality and morbidity in patients having functionally biventricular heart (FBH) and functionally univentricular heart (FUH) undergoing total anomalous pulmonary venous connection (TAPVC) repair. METHODS: We reviewed medical records retrospectively and identified 72 patients undergoing TAPVC repair between July 2002 and December 2010; and 62 patients had FBH and 10 patients had FUH. The median age and weight at repair was 9.1 months (range, 8 days-16 years) and 6.7 kg (range, 2.5-57 kg). The TAPVC anatomy was supracardiac in 41 (56.9%), cardiac in 18 (25%), infracardiac in eight (11.1%), and mixed in five patients (6.9%). RESULTS: There were seven early deaths (2 FBH [25%] and 5 FUH [62.5%]) and one late death (FUH). Presence of FUH (p < 0.01), heterotaxy syndrome (p = 0.03), young age (p < 0.01), low weight (p < 0.01) and long CPB time (p = 0.04) at the time of surgery were statistically significant risk factors for mortality. Morbidity was related to long duration of postoperative mechanical ventilation, intensive care unit and hospital stay. Presence of FUH was the only significant risk factor for morbidity (p < 0.01). After a mean follow-up of 52.1 months (range, 3-74 months), 59 (92%) of 64 survivors were asymptomatic without any medication, and five have mild symptoms with regular physical activity. CONCLUSION: Operative results of TAPVC have dramatically improved in recent years. However, patients having FUH (especially with concomitant palliative surgery), heterotaxy syndrome, low weight, early age, and long CPB time still have an increased operative mortality.

Total anomalous pulmonary venous connection: Influence of heterotaxy and venous obstruction on outcomes.

BACKGROUND: Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients. METHODS: A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates. RESULTS: A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P < .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P < .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029). CONCLUSIONS: Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.

Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study.

BACKGROUND: Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. METHODS AND RESULTS: From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. CONCLUSIONS: The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

Younger Age at Operation Is Associated With Reinterventions After the Warden Procedure.

BACKGROUND: Multiple techniques are available for repair of supracardiac partial anomalous pulmonary venous return (PAPVR); however, most series fail to compare the techniques in contemporary cohorts. This study aimed to describe outcomes of the Warden procedure with a single-patch repair cohort to serve as a control. METHODS: A retrospective cohort analysis of all patients at a single institution (Texas Children's Hospital, Houston, TX) included patients undergoing either the Warden procedure or single-patch repair from 1996 to 2019 for PAPVR. Reintervention was defined as any catheter or surgical procedure on the superior vena cava (SVC) or pulmonary veins. Subgroup analysis was performed within the Warden cohort to evaluate for association between an SVC patch and reintervention-free survival. RESULTS: In total, 158 patients (122 in the Warden group and 36 in the single-patch group) were identified. The median age at operation was younger for patients in the Warden cohort (5.4 years; interquartile range, 3.3 to 10.2 years) compared with patients in the single-patch cohort (13.3 years; interquartile range, 6.5 to 18.7 years; P < .001). One patient in each cohort died. One patient required reoperation after the Warden procedure for dehiscence of the intracardiac patch. Ten patients required transcatheter reinterventions. Reintervention-free survival was not different between patients in the Warden cohort and patients in the single-patch cohort (P = .54) or within the Warden cohort in patients with an SVC patch (P = .27). When controlling for repair type, older age at repair was associated with longer reintervention-free survival (hazard ratio, 0.81; 95% confidence interval, 0.71 to 0.93; P = .002). CONCLUSIONS: The Warden procedure is a viable option for younger patients requiring supracardiac PAPVR repair, although these younger patients are likely at greatest risk for reintervention regardless of surgical technique.

Single-institution outcomes of surgical repair of infracardiac total anomalous pulmonary venous connection.

OBJECTIVE: This contemporary study sought to describe the outcomes of patients undergoing biventricular repair of infracardiac total anomalous pulmonary venous connection. METHODS: A retrospective study was performed on patients with infracardiac total anomalous pulmonary venous connection who underwent sutureless technique or conventional repair between 2006 and 2018. Risk factors for survival and post-repair pulmonary vein stenosis (PVS) were assessed with Cox regression model. Time-to-event analysis was conducted using Kaplan-Meier estimates. RESULTS: This study included 82 consecutive patients with the median age of 21 days (interquartile range, 9-40 days). The median follow-up was 29 months (interquartile range, 12.5-59 months) and was available in 95% of the survivors at the end of the study period in 2019. Overall, 8 deaths (8.5%) occurred in the conventional repair group. There was a trend of higher mortality in the conventional repair group, although it did not reach a statistical difference (P = .2). Postrepair PVS occurred at a median of 2 months (interquartile range, 1.2-3.6 months) postoperatively and all occurred in the conventional repair group. Time-to-event analysis with the event of postrepair PVS showed significantly higher freedom from restenosis in the sutureless technique group (P = .0004). Adjusted hazard ratios from time-dependent Cox model described the association between postrepair PVS and pulmonary venous confluence of antler configuration (hazard ratio, 2.14; 95% confidence interval, 1.03-5.47; P = .002) and the use of sutureless technique (hazard ratio, 0.72; 95% confidence interval, 0.39-0.97; P = .003). CONCLUSIONS: Sutureless technique is associated with a lower risk of postrepair PVS in patients with infracardiac total anomalous pulmonary venous connection. pulmonary venous confluence configuration of antler appearance appears to be associated with restenosis and mortality.

Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities.

INTRODUCTION: Heterotaxy syndrome presents a unique challenge in surgical management, even in the current era. We hypothesized that certain anatomic subsets merit novel strategies. METHODS: We analyzed morphologic details, surgeries, comorbidities, subsequent admissions, and survival using Kaplan-Meier methods and multivariable risk models from a single-institution experience of 103 consecutive patients with heterotaxy who underwent cardiac surgery between January 1, 1990, and May 31, 2016. RESULTS: Of the 103 patients (50 males and 53 females), 31 had left atrial isomerism, 64 had right atrial isomerism (RAI), and 8 patients' isomerism was indeterminate (IND), with first cardiac operation at a mean 1.0 year (standard deviation ±3.0 years) of age. Kaplan-Meier overall survival estimate was 83.1% at six months, 77.8% at one year, 65.9% at five years, and 52.1% at ten years. Survival was particularly low among RAI following repair of total anomalous pulmonary venous connection (TAPVC) at first operation, with one- and five-year survival of 57% and 46%, respectively. By multivariable analysis, the only risk factor for death during the early phase (hazard model) was repair of TAPVC at the first cardiac operation (hazard ratio [HR]: 4.4, P = .01), and risk factors during the longer term constant phase were atrioventricular valve (AVV) regurgitation (HR: 4.2, P < .01), male gender (HR: 3.7, P < .01), and two-ventricle repair (HR: 3.0, P = .02). Patients with heterotaxy undergoing the Fontan procedure had excellent subsequent survival (85% at ten years). CONCLUSIONS: This analysis of over 100 patients with heterotaxy identified TAPVC requiring initial repair as the major risk factor for early death and important AVV regurgitation as the major risk factor in the longer term. Survival with RAI and early repair of TAPVC were poor, with one-year mortality exceeding 40%. Patients with single ventricle completing the Fontan operation enjoyed outstanding ten-year survival (85%). Initial management of RAI requiring early repair of TAPVC remains challenging. For this high-risk subset, alternative strategies such as early referral for cardiac transplantation evaluation warrant consideration.