Prolonged Pleural Effusion Research Articles

Early morbidities following paediatric cardiac surgery: a mixed-methods study

BackgroundOver 5000 paediatric cardiac surgeries are performed in the UK each year and early survival has improved to > 98%.ObjectivesWe aimed to identify the surgical morbidities that present the greatest burden for patients and health services and to develop and pilot routine monitoring and feedback.Design and settingOur multidisciplinary mixed-methods study took place over 52 months across five UK paediatric cardiac surgery centres.ParticipantsThe participants were children aged < 17 years.MethodsWe reviewed existing literature, ran three focus groups and undertook a family online discussion forum moderated by the Children’s Heart Federation. A multidisciplinary group, with patient and carer involvement, then ranked and selected nine key morbidities informed by clinical views on definitions and feasibility of routine monitoring. We validated a new, nurse-administered early warning tool for assessing preoperative and postoperative child development, called the brief developmental assessment, by testing this among 1200 children. We measured morbidity incidence in 3090 consecutive surgical admissions over 21 months and explored risk factors for morbidity. We measured the impact of morbidities on quality of life, clinical burden and costs to the NHS and families over 6 months in 666 children, 340 (51%) of whom had at least one morbidity. We developed and piloted methods suitable for routine monitoring of morbidity by centres and co-developed new patient information about morbidities with parents and user groups.ResultsFamilies and clinicians prioritised overlapping but also different morbidities, leading to a final list of acute neurological event, unplanned reoperation, feeding problems, renal replacement therapy, major adverse events, extracorporeal life support, necrotising enterocolitis, surgical infection and prolonged pleural effusion. The brief developmental assessment was valid in children aged between 4 months and 5 years, but not in the youngest babies or 5- to 17-year-olds. A total of 2415 (78.2%) procedures had no measured morbidity. There was a higher risk of morbidity in neonates, complex congenital heart disease, increased preoperative severity of illness and with prolonged bypass. Patients with any morbidity had a 6-month survival of 81.5% compared with 99.1% with no morbidity. Patients with any morbidity scored 5.2 points lower on their total quality of life score at 6 weeks, but this difference had narrowed by 6 months. Morbidity led to fewer days at home by 6 months and higher costs. Extracorporeal life support patients had the lowest days at home (median: 43 days out of 183 days) and highest costs (£71,051 higher than no morbidity).LimitationsMonitoring of morbidity is more complex than mortality, and hence this requires resources and clinician buy-in.ConclusionsEvaluation of postoperative morbidity provides important information over and above 30-day survival and should become the focus of audit and quality improvement.Future workNational audit of morbidities has been initiated. Further research is needed to understand the implications of feeding problems and renal failure and to evaluate the brief developmental assessment.FundingThis project was funded by the NIHR Health Services and Delivery Research programme and will be published in full inHealth Services and Delivery Research; Vol. 8, No. 30. See the NIHR Journals Library website for further project information.

Ruptured sinus of valsalva presenting late following repair of tetralogy of fallot

Rupture of sinus of valsalvafollowing repair of tetralogy of fallot (TOF) is very rare. It should be suspected as a cause of recurrent or prolonged pleural effusion and congestive cardiac failure in patients who have undergone repair of TOF. We report one such patient.

A Simple Predictive Factor for Mortality in Fontan Surgery: Serum Hypo-Osmolality.

ObjectiveClose follow-up is important after the Fontan procedure, which is a palliative surgical method for a single ventricle. In this period, serum osmolality is an important parameter with the advantages of easy to obtain and poor outcome prediction.MethodsPatients who had undergone Fontan operation between May 2011 and February 2017 were retrospectively evaluated. Patients were divided into three groups based on their serum osmolality values: hypoosmolar (Group 1), isosmolar (Group 2), and hyperosmolar (Group 3). Demographics, clinical information and postoperative data of the groups were compared.ResultsForty-three patients had undergone extracardiac Fontan operation in the study period. There were 8, 19 and 16 patients in Groups 1, 2 and 3, respectively. Among the three groups, postoperative intubation and length of hospital stay, prolonged pleural effusion, need for inotropic support and mortality were statistically significantly higher in Group 1.ConclusionAfter the Fontan procedure, one of the determinants of cardiac output might be affected by serum osmolality. Decreased serum osmolality might be associated with poor prognosis after Fontan procedure. Serum osmolality monitoring may be beneficial to improve postoperative outcomes in these patients.

Variation in care for children undergoing the Fontan operation for hypoplastic left heart syndrome.

The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion. Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7days). The median Fontan age (14 centres, 320 patients) was 3.1years (range from 1.7 to 3.9), and the weight-for-age z-score was -0.56 (-1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4-100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3-100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7-33%). The length of stay was 9.5 days (9-12); 15% (6-33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98). Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.

Risk Factors for Prolonged Pleural Effusion Following Total Cavopulmonary Connection Surgery: 9 Years' Experience at Fuwai Hospital.

Background: Prolonged pleural effusion (PPE) contributes to adverse outcomes after total cavopulmonary connection (TCPC) completion. We aimed to identify risk factors for PPE following TCPC surgery.Methods: We studied a retrospective cohort of 525 who undergoing TCPC surgery from 2010 to 2019. We defined PPE as the duration of pleural effusion exceeding 14 days. Logistic regression was applied to identify risk factors for PPE and Cox regression was used to identify risk factors for predicting the duration of pleural effusion. The impacts of PPE on the short-term outcomes were evaluated.Results: The rate of PPE was 27.4% in our study and independent risk factors for PPE included: young age, no fenestration, low postoperative total protein, prolonged mechanical ventilation and chylothorax. These predictors were also achieved in the Cox regression for predicting the duration of pleural effusion. The applicability of the model was acceptable in different subgroups, which derived from the total cohort. Patients with PPE were associated with more renal replacement treatment, longer length of ICU and hospital stay, more hospitalization costs and a higher rate of in-hospital mortality.Conclusions: PPE in our study occurs at a relatively lower rate than that reported in previous studies and patients with PPE was associated with higher rate of in-hospital mortality when compared to patients without PPE. Young age, no fenestration, low postoperative total protein, prolonged mechanical ventilation, and chylothorax were identified as independent risk factors to predict PPE. A preventive strategy that targets the identified risk factors to reduce the incidence of PPE following TCPC surgery could be beneficial for in-hospital outcomes, and the model needs further validation before its application.

What are the important morbidities associated with paediatric cardiac surgery? A mixed methods study

ObjectivesGiven the current excellent early mortality rates for paediatric cardiac surgery, stakeholders believe that this important safety outcome should be supplemented by a wider range of measures. Our objectives were...

Prophylactic Opening of the Pleural Cavity for Postoperative Drainage is a Risk Factor for Prolonged Pleural Effusion After a Fontan Operation.

Postoperative prolonged pleural effusion (PPE) remains a confounding problem after a Fontan operation. We aimed to describe the risk factors for PPE after a Fontan operation and to clarify the impact of prophylactic opening of the pleural cavity (POPC) for drainage tube insertion on PPE. We retrospectively reviewed the medical charts of 50 consecutive patients who underwent a Fontan operation at our institution. POPC for postoperative drainage was performed based on each surgeon's preference. Patients were divided into three groups for analysis: group A (n = 12), no opening; group B (n = 14), unilateral opening; and group C (n = 24), bilateral opening. At the time of surgery, the median age of our patient group was 26months, with a median body weight of 10.5kg. The volume of pleural effusion tended to be lower in group A than in groups B and C (p = 0.08). The median duration of drainage was significantly shorter (p = 0.03) in group A (3days) than in group B (4days) or C (5days). Overall, 12 patients required chest tube drainage for ≥ 7days. Multivariate analysis revealed POPC (p = 0.01) and postoperative water balance (p = 0.03) as independent predictors of PPE. POPC and postoperative water balance are risk factors for PPE after a Fontan operation. Therefore, avoiding POPC for postoperative drainage may reduce the risk of postoperative pleural effusion and morbidities associated with PPE after a Fontan operation.

Risk Factors for Prolonged Pleural Effusion After Extracardiac Fontan Operation.

Prolonged pleural effusion after Fontan operation is a significant morbidity that leads to long hospital stays. We investigated the association of multiple risk factors, including clinical characteristics, hemodynamic parameters, and preoperative, operative, and postoperative factors, with prolonged pleural effusion after Fontan operation. Eighty-five patients who underwent a Fontan operation between January 2005 and June 2018 in our center were included in this retrospective study. Patients were divided into two groups: group 1 (n = 36, 42.4%) included those with prolonged pleural effusion, defined as lasting > 14days after the Fontan operation, and group 2 included patients without prolonged pleural effusion. Patients with hypoplastic left heart syndrome (HLHS) were more prevalent in group 1 (n = 15, P = 0.006). No differences in age at Fontan operation, central venous pressure at Fontan operation, or hemodynamic parameters during the pre-Fontan evaluation were found between the two groups. In multivariable analysis, HLHS (P = 0.002), non-fenestration (P = 0.018), and high central venous pressure at bidirectional cavopulmonary shunt (BCPS) operation (P = 0.043) were independent risk factors for prolonged pleural effusion after Fontan operation. Adverse outcomes such as death, need for heart transplantation, and Fontan failure were not associated with prolonged pleural effusion. In conclusion, patients with HLHS and higher central venous pressure at BCPS were more likely to have a prolonged pleural effusion after Fontan operation, but fenestration was more likely to decrease prolonged effusion. We should consider closer management of fluid status before, during, and after surgery in patients with these risk factors after Fontan operation.

Transcatheter occlusion of antegrade pulmonary blood flow in children with univentricular heart.

This study aims to evaluate the results of transcatheter occlusion of antegrade pulmonary blood flow in children with univentricular heart. Medical data of a total of seven patients (4 females, 3 males; median age 11.7 years; range, 1 to 24 years) who underwent transcatheter occlusion of the antegrade pulmonary blood flow following Glenn shunt or Fontan operation between September 2014 and January 2017 were retrospectively analyzed. Data including demographic and clinical characteristics of the patients, type of surgery, echocardiographic and cardiac catheterization findings were recorded. Four patients had a previous pulmonary artery banding operation, while three had pulmonary stenosis. Two patients had facial and upper extremity edema after Glenn operation, one had prolonged pleural effusion, one had prolonged pleural effusion after Fontan operation, and one developed dyspnea and effort intolerance several years after Fontan operation. In two patients, antegrade pulmonary blood flow was occluded to decrease systemic ventricular load before surgery. The Amplatzer Septal Occluder was used in five patients and the Amplatzer Vascular Plug-2 was used in two patients. Two patients developed transient, complete atrioventricular block during the procedure and the procedure was terminated early in one of these patients. Transient hemolysis was observed in one patient following the operation. Transcatheter occlusion of antegrade pulmonary blood flow is an effective alternative to surgery in patients with hemodynamic compromise after Glenn shunt or Fontan operation.

Incidence and risk factors for important early morbidities associated with pediatric cardiac surgery in a UK population

ObjectiveGiven excellent 30-day survival for pediatric cardiac surgery, other outcome measures are important. We aimed to study important early postoperative morbidities selected by stakeholders following a rigorous and evidenced-based process, with a view to identifying potential risk factors. MethodsThe incidence of selected morbidities was prospectively measured for 3090 consecutive pediatric cardiac surgical admissions in 5 UK centers between October 2015 and June 2017. The relationship between the candidate risk factors and the incidence of morbidities was explored using multiple regressions. Patient survival, a secondary outcome, was checked at 6 months. ResultsA total of 675 (21.8%) procedure episodes led to at least 1 of the following: acute neurologic event, unplanned reoperation, feeding problems, renal replacement therapy, major adverse events, extracorporeal life support, necrotizing enterocolitis, surgical infection, or prolonged pleural effusion. The highest adjusted odds ratio of morbidity was in neonates compared with children, 5.26 (95% confidence interval, 3.90-7.06), and complex heart diseases (eg, hypoplastic left heart), 2.14 (95% confidence interval, 1.41-3.24) compared with low complexity (eg, atrial septal defect, P < .001 for all). Patients with any selected morbidity had a 6-month survival of 88.2% (95% confidence interval, 85.4-90.6) compared with 99.3% (95% confidence interval, 98.9-99.6) with no defined morbidity (P < .001). ConclusionsEvaluation of postoperative morbidity provides important information over and above 30-day survival and should become a focus for audit and quality improvement. Our results have been used to initiate UK-based audit for 5 of these 9 morbidities, co-develop software for local monitoring of these morbidities, and parent information about these morbidities.

279Neck lymphatic collaterals are associated with prolonged pleural effusion after total cavopulmonary connection (TCPC) palliation

279Neck lymphatic collaterals are associated with prolonged pleural effusion after total cavopulmonary connection (TCPC) palliation

A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome

This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18-44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63-96%) at 20 years for lateral tunnel and 99% (95% CI 96-100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65% (95% CI 52-76%) for atriopulmonary connection/Björk, 90% (95% CI 73-97%) for lateral tunnel and 90% (95% CI 82-94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0-11.1; P < 0.001). With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.

Mycoplasma Pneumoniae Pericarditis

Mycoplasma pneumoniae is an atypical bacterium that is frequently implicated in respiratory infections, but uncommonly identified as a cause of pericarditis. We report 2 cases of pericarditis attributed to M. pneumoniae that were characterized by prolonged respiratory prodromes, pericardial, and pleural effusions, elevated inflammatory markers, and relapsing clinical courses. In conclusion, our experience suggests that M. pneumoniae should be considered as a potential cause in cases of pericarditis associated with upper respiratory symptoms, pneumonia, pleural effusions, arthralgia, and/or a recurrent/refractory clinical course. The availability of effective antibiotic treatment makes this an important diagnosis to make.

Predictors of unfavourable early outcome following Fontan completion

BackgroundIn spite of the magnificent improvement in Fontan operation results in the last two decades, there are still some concerns regarding the unfavourable early outcomes that may follow Fontan completion. MethodsFrom 2003 to 2016, 79 patients underwent Fontan operation at IRCCS Policlinico San Donato. Unfavourable early outcome was defined by the presence of one or more of these occurrences: prolonged hospital stay >25 days, prolonged pleural effusion ≥14 days and prolonged inotropic support ≥72 h. Univariable and multivariable analyses were performed to detect the risk factors associated with early unfavourable outcome after Fontan completion. ResultsProlonged hospital stay >25 days was found in 24.05% of patients and its associated significant risk factors were low preoperative O2 saturation (p 0.007), Fontan fenestration (p 0.009) and plasma transfusion (p 0.030). Prolonged pleural effusion ≥14 days was found in 24.05% and no significant risk factors were detected. Prolonged inotropic support ≥72 h was found in 35.44% and significant risk factors were prolonged cardiopulmonary bypass (CPB) time (P 0.003), fenestration (P 0.023), plasma transfusion (P 0.028) and non staged Fontan (P 0.039). In multivariable analysis of combined unfavourable outcome, significant risk factors were fenestration (P 0.030) with some trends towards low preoperative O2 saturation (P 0.056). ConclusionsUnfavourable early outcome can occur following Fontan completion with associated prolonged hospital stay. Risk factors include low preoperative O2 saturation, prolonged CPB time, Fontan fenestration, plasma transfusion and non staged Fontan.

A prospective study of risk factors associated with persistent pleural effusion after total cavopulmonary connection with special reference to serum cortisol level.

The Fontan operation is usually followed by significant pleural effusion. We aimed to study the factors associated with persistent pleural effusion with special reference to serum cortisol levels. Thirty-eight patients undergoing the Fontan operation between September 2015 and November 2016 were prospectively studied. Parameters studied included age, weight, symptoms, atrio- ventricular valve regurgitation/stenosis/atresia, ventricular function, pulmonary artery pressures, oxygen saturation, aorto-pulmonary, and veno-venous collaterals, type of Fontan, duration of cardiopulmonary bypass, need for inotropes, duration of mechanical ventilation, conduit size, presence or absence of fenestration, and serum cortisol levels. The latter were measured before and after the Fontan operation and the co-relation between pleural effusion and change in serum cortisol levels was studied. Mean age at operation was 13.1 ± 5.6years (median 13years). Mean duration and amount of pleural drainage was 15.76 ± 13.2days (median 11.5days) and 9.15 ± 4.6mL/kg/day (median 9mL/kg/day) respectively. Statistically significant risk factors for prolonged pleural effusion were higher pulmonary artery (PA) pressures (r = 0.328, p = 0.003, odds ratio 1.30), higher inotropic score (r = 0.4, p = 0.01), lower rise in serum cortisol (p = 0.03),elevated superior caval venous pressure (CVP) at 6h (r = 0.44, p = 0.005) and 12h (r = 0.4, p = 0.01) and higher duration of mechanical ventilation (r = 0.45, p = 0.005). PA pressures > 15mmHg, higher inotropic score, higher CVP and lower rise in serum cortisol levels following the Fontan operation were associated with persistent pleural effusion.

Chylothorax and pleural effusion in contemporary extracardiac fenestrated fontan completion

BackgroundWe hypothesized that chylothorax could be a sign of intolerance to the Fontan physiology, and thus patients who develop chylothorax or pleural effusion have worse medium-term to long-term survival. MethodsA total of 324 patients who underwent the Fontan operation between 2000 and 2013 were included. Chylothorax was defined as ≥5 mL/kg/day of chylomicron-positive chest drainage fluid no earlier than postoperative day 5 or drainage with >80% lymphocytes. Outcomes were compared between the chylothorax and non-chylothorax groups by the Kaplan–Meier method and log-rank test. Independent predictors of chylothorax and number of days of any chest drainage were analyzed with multivariable logistic regression and multivariable generalized negative binomial regression for count data, respectively. ResultsChylothorax occurred in 78 patients (24%). Compared with the non-chylothorax group, the chylothorax group had a longer duration of chest tube requirement (median, 18 days vs 9 days; P < .000) and a longer length of hospital stay (median, 19 days vs 10 days; P < .000). Eight patients (10.3%) required thoracic duct ligation. The chylothorax group had lower freedom from death (P = .013) and from composite adverse events (P = .021). No predictor was found for chylothorax. Pulmonary atresia (P = .031) and pre-Fontan pulmonary artery pressure (P = .01) were predictive of prolonged pleural effusion (>14 days). ConclusionsOccurrence of chylothorax following the Fontan operation can be a marker of poorer medium-term clinical outcomes. It is difficult to predict occurrence of chylothorax owing to its multifactorial nature and involvement of lymphatic compensatory capacity that is unmasked only after the Fontan operation.

Coil Localization-Guided Video-Assisted Thoracoscopic Surgery for Lung Nodules.

To determine the clinical efficacy of preoperative coil localization-guided video-assisted thoracoscopic surgery (VATS) for lung nodules. Between November 2015 and July 2017, 56 patients with lung nodules underwent coil localization-guided VATS procedure. The coil implantation was performed under the guidance of computed tomography (CT). The end tail of the coil remained above the visceral pleura. The target lung nodules were removed by VATS wedge resection. Data on the technical success of coil localization and wedge resection, procedure-related complications, and pathological results were collected and analyzed. Sixty-seven lung nodules in 56 patients (1.2 nodules/case) were localized. The technical success rate of coil localization was 89.6% (60/67). Sixty-three nodules were localized with one coil and four nodules with two coils. The mean time taken to perform CT-guided coil implantation was 15.7 ± 5.3 (range: 8-40) minutes. Six patients (9.0%) experienced pneumothorax after coil implantation. The technical success rate of wedge resection was 97.0% (65/67). Two nodules were removed directly by video-assisted lobectomy. Nine patients with multiple target lung nodules underwent single-stage resection. The mean total operating time was 147.2 ± 79.1 (range: 50-360) minutes. The mean volume of blood loss was 113.2 ± 113.0 (range: 10-700) mL. Postoperative complications included prolonged air leak (n = 2) and pleural effusion (n = 5). Preoperative coil localization is a safe and effective method to facilitate a high successful rate of VATS wedge-resection for lung nodules.

Outcomes of Patients Undergoing Primary Fontan Operation Beyond First Decade of Life.

Studies on older patients undergoing primary Fontan operation (FO) are limited, with conflicting results. We review our experience with these patients beyond the first decade of life. Between January 2000 and December 2014, a total of 105 patients ≥10 years of age (mean 15.6 ± 4.9, range 10-31, median 15 years) underwent primary FO without a prior bidirectional superior cavopulmonary anastomosis (Bidirectional Glenn [BDG]). Mean preoperative New York Heart Association (NYHA) class was 2.2 ± 0.57. Operative procedure was extra-cardiac FO in 62 patients (8 were fenestrated). Forty-three had a lateral tunnel FO (26 were fenestrated). There were 11 (10.5%) early deaths. Fourteen of the 94 early survivors experienced prolonged pleural effusions, 7 had arrhythmias, and 2 had thromboembolic events. Two patients underwent Fontan takedown. On univariate analysis, NYHA functional class III, mean pulmonary artery (PA) pressure ≥15 mm Hg, hematocrit ≥60%, preoperative ventricular dysfunction, and atrioventricular valve regurgitation (AVVR) were associated with early mortality. Median follow-up was 78 (mean 88.9 ± 6.3) months. In 94 survivors, 6 (6.4%) late deaths were encountered. At last follow-up, 81 (86.2%) survivors were in NYHA class I. Actuarial survival was 84.7% ± 3.7% at 5, 10, and 15 years. Carefully selected adolescents and young adults can safely undergo the primary FO. However, persistent pleural effusions, arrhythmias, thromboembolic events, and the need for reoperation mandate regular follow-up in such patients. Preoperative NYHA functional class III, mean PA pressure ≧ 15 mm Hg, hematocrit ≥ 60%, ventricular dysfunction, and AVVR were associated with early mortality, suggesting that primary FO should be avoided in such patients.

The hemi-Mustard, bidirectional Glenn and Rastelli procedures for anatomical repair of congenitally corrected transposition of the great arteries/left ventricular outflow tract obstruction with positional heart anomalies†.

The hemi-Mustard and bidirectional Glenn (BDG) procedures combined with the Rastelli procedure have been applied to selected cases of congenitally corrected transposition of the great arteries (ccTGA) for potential benefit over the classic atrial switch procedure. The aim of this study was to analyse our experience with the hemi-Mustard, BDG and Rastelli procedures as an anatomical correction for patients with ccTGA/left ventricular outflow tract obstruction (LVOTO) with positional heart anomalies. In this retrospective study, 31 consecutive patients with corrected transposition underwent the hemi-Mustard/BDG procedures with the Rastelli operation between 2011 and 2015. The median age was 5.4 (range: 0.75-12) years. Positional anomalies were present in all patients. Eleven patients underwent BDG initially; they then had the second-stage hemi-Mustard and Rastelli procedures; 'one-stage repair' (hemi-Mustard/BDG and the Rastelli procedures) was performed in 20 cases. There were no in-hospital deaths, and 3 patients received a pacemaker. One patient had an atrial baffle obstruction that was observed in the early postoperative period; 7 patients had prolonged pleural effusions that developed more frequently in the one-stage repair group (7/20 vs 0/11, P = 0.033). During the mean follow-up of 3.3 years, 1 late death was noted, and no conduit replacements were required. Twenty-five (83.3%) patients are in New York Heart Association classes I and II at the latest follow-up. Hemi-Mustard, BDG and the Rastelli procedures are technically feasible for correction of ccTGA/left ventricular outflow tract obstruction (LVOTO) and cardiac malposition. Nevertheless, postoperative pleural effusion is the most prevalent complication in the one-stage repair. Two-stage repair may reduce the risk of pleural effusion-related complications.

Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure

The objective of this study was to characterize late outcomes of the lateral tunnel (LT) Fontan procedure. The outcomes of all patients who underwent an LT Fontan procedure in Australia and in New Zealand were analyzed. Original files were reviewed and outcomes data were obtained through a binational registry. Between 1980 and 2014, a total of 301 patients underwent an LT Fontan procedure across 6 major centers. There were 13 hospital mortalities, 21 late deaths, 8 Fontan conversions and revisions, 8 Fontan takedowns, and 4 heart transplantations. Overall survival at 15 and 25 years was 90% (95% confidence interval [CI]: 86%-93%) and 80% (95% CI: 69%-91%), respectively. Protein-losing enteropathy or plastic bronchitis was observed in 14 patients (5%). Freedom from late failure at 15 and 25 years was 88% (95% CI: 84%-92%) and 82% (95% CI: 76%-87%), respectively. Independent predictors of late Fontan failure were prolonged pleural effusions post Fontan operations (hazard ratio [HR] 3.06, 1.05-8.95, P = 0.041), age >7 years at Fontan (vs 3-5 years, HR 9.7, 2.46-38.21, P = 0.001) and development of supraventricular tachycardia (HR 4.67, 2.07-10.58, P < 0.001). Freedom from tachy- or bradyarrhythmias at 10 and 20 years was 87% (95% CI: 83%-91%) and 72% (95% CI: 66%-79%), respectively. Thromboembolic events occurred in 45 patients (16%, 26 strokes), and freedom from symptomatic thromboembolism at 10 and 20 years was 93% (95% CI: 89%-96%) and 80% (95% CI: 74%-86%), respectively. Over a 25-year period, the LT technique has achieved excellent late survival. As this population ages, it is at an increasing risk of failure and adverse events. We are likely to see an increasing proportion requiring heart transplantation and late reintervention.