Impact of Valve Type (Ross vs. Mechanical) on Health-Related Quality of Life in Children and Young Adults with Surgical Aortic Valve Replacement.

BACKGROUND: The impact of aortic valve replacement (AVR) type on health-related quality of life (HRQOL) in adolescents and young adults is unclear, but may vary depending on need for anticoagulation or re-intervention. We sought to determine the differences in HRQOL following AVR with either the Ross procedure or mechanical AVR in this young population. METHODS: Patients 14-35 years old and at least 1 year post-AVR were included. HRQOL was assessed using the Short Form-36 (SF-36). Valve-specific concerns regarding anticoagulation and reoperation were also assessed. Clinical outcome data were obtained by chart review. RESULTS: A total of 51 patients were enrolled: 24 (47%) Ross and 27 (53%) mechanical AVR. Ross patients were younger at time of AVR (16 vs. 22 years, p < 0.01) and study enrollment (23.7 vs 29.5 years, p < 0.01). Median follow-up from AVR to study enrollment was similar (5.4 years for Ross vs. 5.6 years for mechanical, p = 0.62). At last follow-up, clinical outcomes including cardiac function, functional class, and aortic valve re-intervention rates were similar between groups, although mechanical valve patients had more bleeding events (p = 0.012). SF-36 scores were generally high for the entire cohort, with no significant difference between groups in any domain. Mechanical AVR patients reported more concern about frequency of blood draws (p < 0.01). Concern for reoperation was similar between both groups. CONCLUSION: Despite more bleeding events and concern about the frequency of blood draws, adolescents and young adults with mechanical AVR reported similarly high levels of HRQOL compared to those following Ross AVR.

Innominate Vein Turn-down Procedure for Failing Fontan Circulation.

After the Fontan, systemic venous hypertension induces pathophysiologic changes in the lymphatic system that can result in complications of pleural effusion, ascites, plastic bronchitis, and protein losing enteropathy. Advances in medical therapy and novel interventional approaches have not substantially improved the poor prognosis of these complications. A more physiological approach has been developed by decompression of the thoracic duct to the lower pressure common atrium with a concomitant increase of preload. Diverting the innominate vein to the common atrium increases the transport capacity of the thoracic duct, which in most patients enters the circulation at the left subclavian-jugular vein junction. Contrary to the fenestrated Fontan circulation, in which the thoracic duct is drained into the high pressure Fontan circulation, turn down of the innominate vein to the common atrium effectively decompresses the thoracic duct to the lower pressure system with "diastolic suctioning" of lymph. Innominate vein turn-down may be considered for medical-refractory post-Fontan lymphatic complications of persistent chylothorax, plastic bronchitis, and protein losing enteropathy. Prophylactic innominate vein turn-down may also be considered at time of the Fontan operation for patients that are higher risk for lymphatic complications.

Anatomic Repair of Corrected Transposition of the Great Arteries: The Double Switch.

The long-term outcome of patients with corrected transposition of the great arteries and associated lesions after physiologic repair is uncertain. Anatomic correction, utilizing the morphologic left ventricle as a systemic pumping chamber and the mitral valve as the systemic atrioventricular valve, is considered the preferred method, especially for patients with either tricuspid valve regurgitation, with Ebstein's malformation of the tricuspid valve, or with right ventricular dysfunction. The double switch employs both an atrial switch and arterial switch to "correct" the atrioventricular and ventriculoarterial discordance. Associated lesions are also repaired. The best outcomes with double switch are achieved with patients in the first few years of life even if reconditioning of morphologic left ventricle is required. However, the long-term function of the conduction system, the aortic valve, and the ventricles is variable and requires close surveillance.

Neurodevelopmental Outcome in Children With Single Ventricle After Total Cavopulmonary Connection.

BACKGROUND: The purpose of the present study was to assess a broad range of neuropsychological outcome variables in children with functionally single ventricle hearts after a total cavopulmonary connection and to examine potential risk factors for impaired neurodevelopment. PATIENTS & METHOD: A total of 104 patients aged 2 to 20 years underwent follow-up standardized psychological testing, including measures of intelligence, motor function, visuospatial abilities, behavioural outcome and health-related quality of life. RESULTS: With a mean fluid intelligence score of 93.0 and a mean crystallized intelligence score of 92.3, patients scored significantly lower on tests of intelligence compared to the general population (p<0.001). Reduced motor function was found in 34% of the patients (p<0.001), impaired visuospatial abilities in 51%; parents reported significantly more behavioural problems (p<0.001). There was no difference in the self-reported quality of life compared to the general population. Risk factors for reduced fluid intelligence were deep hypothermic circulatory arrest times (p=0.03) and complications causing suboptimal brain perfusion prior to the total cavopulmonary connection (e. g. seizures; p=0.04). CONCLUSIONS: Patients with functionally single ventricle hearts palliated with a total cavopulmonary connection are at an increased risk of neurodevelopmental delays and behavioural disorders. Nevertheless, they adapt well in terms of quality of life. Early diagnostics and interventions are necessary when developmental delays are suspected.

Neonatal Aortic Stenosis Is a Surgical Disease.

Neonates with critical aortic stenosis represent a challenging group of patients with severe obstruction at a valvar level and with symptoms of heart failure. If biventricular repair is chosen, open valvotomy (OV) has been firmly established as the most effective initial treatment. In comparison with blind ballooning, OV, with exact splitting of fused commissures and shaving of obstructing nodules, can produce a better valve with a maximum valve orifice, without causing regurgitation. Thus, predictable and consistent early and longer-lasting results in any type of valve morphology are provided. Clearly superior results can be achieved in a tricuspid valve arrangement. OV not only offers a high survival benefit in the long run, but also a high quality of life, by minimizing re-interventions and preserving the native aortic valve in the majority of patients.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia.

BACKGROUND: Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. PATIENTS AND METHODS: A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. RESULTS: Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). CONCLUSION: Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

Transvascular closure of single and multiple muscular ventricular septal defects in neonates and infants < 20 kg.

PURPOSE: While surgical closure of ventricular septal defects (VSD) is still the gold standard, we review our experience with the interventional closure of single and multiple muscular VSD in newborns and infants under 20 kg. METHOD: Between 2004 and 2012 transcatheter closure of ten Swiss cheese VSD, six single muscular and one apical VSD was attempted in 17 patients between 10 days and 7.3 years of age and with a bodyweight ranging from 2.2 to 19 kg. Seven patients had had a significant shunt after cardiac surgery, five patients a shunt induced congestive heart failure and in five patients postponement of surgery was intended. RESULTS: A total of 20 devices was successfully implanted in 15 of 17 (88%) patients, reducing the interventricular shunt and improving the haemodynamic situation in 14 patients. An acute AV-block led to immediate removal of the device in a patient of 2.2 kg. One Amplatzer muscular VSD occluder could not be delivered due to the sharp bending of the delivery sheath in 2004. Three patients died during follow-up not related to the intervention. Re-intervention was necessary in one patient with Swiss cheese VSD. CONCLUSION: Interventional closure of muscular VSD is possible in newborns and infants and presents an eligible treatment option. New occlusion systems with miniaturized introducer sheaths of 4-6 French have extended the spectrum of treatable lesions. An individual and interdisciplinary risk-benefit stratification is required to choose from surgical, interventional, or combined strategies.

Reimbursement by current German diagnosis-related groups system penalises complex congenital heart surgery.

A total of 458 hospital stays during the year 2011 were analysed to determine whether reimbursement by the current German Diagnosis-Related Groups system covers the costs incurred during hospital stay for congenital heart surgery. The costs of every hospital stay were estimated according to the guidelines of the Institute for the Hospital Remuneration System, an institute responsible for encoding hospital reimbursement in Germany. Cost-weight values of the year 2012 were applied for reimbursement. Related additional compensations were also included. Hospital costs ranged from 8896.26 to 193,671.94 euros per case, with a mean of 30,597 and standard deviation of 25,032 euros. Reimbursement varied from 8630.35 to 173,710.65 euros, with a mean of 25,514 and standard deviation of 18,497 euros: an underfunding of 17%. Fifty-nine per cent (271/458) of cases were classified, according to Aristotle complexity score, in higher comprehensive complexity: Levels 4-6. Costs highly correlated with complexity levels (Spearman's r coefficient = 0.89) and the regression was linear. Underfunding increased, linearly, from 6% for procedures with Level 1, lowest comprehensive complexity, to 23% for those with Level 6, highest complexity. In conclusion, this study demonstrates that reimbursement by the current German Diagnosis-Related Groups system increasingly penalises complex congenital heart surgery. Aristotle complexity score could help to correct this prejudicial situation.

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study.

BACKGROUND: To evaluate indications and results of surgery for primary cardiac tumors in children. METHODS AND RESULTS: Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). CONCLUSIONS: Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

Quantification of morbidity associated with congenital heart surgery.

OBJECTIVE: The objective of this study was to estimate the morbidity according to observed complications after congenital heart surgery over 1-year period. METHODS: The previously established list of conditions prone to affect patients' well-being or increase cost of in-hospital stays was used systematically to score the severity of postoperative complications from 1 to 4 points. The morbidity score was calculated by adding the scores of observed complications. When the sum amounted to more than 5 points, a morbidity score of only 5 points was attributed. If no complication was detected, a score of 0.5 points was assigned. The resulting morbidity scores were correlated with the length of stay in the intensive care unit (ICU) and in the hospital, the duration of mechanical ventilation, and Aristotle complexity scores. RESULTS: A total of 542 primary procedures performed in the year 2011 were studied. Aristotle basic and comprehensive scores amounted to 7.78 ± 2.65 and 10.15 ± 3.83, respectively. Mortality was 1.85% (10/542). The standardized ratio of surgical performance reached 103.10%. Total cavopulmonary connection with extracardiac fenestrated conduit constituted the most frequent operation (n = 34). No complication was observed following 183 (33.8%) procedures. More than two complications were observed in 114 cases (21%). The three most frequent unfavorable conditions were "mechanical ventilation 25 to 95 hours" (n = 150), low cardiac output syndrome (n = 56), and cardiac arrhythmia requiring medication (n = 50). The estimated mean morbidity score amounted to 2.26 ± 1.80 points. Scores ranged from 0.68 ± 0.50 for primary closure of atrial septal defect to 4.50 ± 0.79 for the Norwood procedure. They were perfectly related to the length of ICU stay and to the duration of mechanical ventilation (Spearman coefficient r = 1). Correlation was high with the length of hospital stay (r = 0.83), Aristotle basic score (r = 0.89) (r = 0.96), and comprehensive score (r = 0.94) (C-index = 0.97). The observed mean morbidity score was statistically not different from the expected mean morbidity score according to the basic Aristotle complexity: p = 0.73. CONCLUSION: Quantification of morbidity indicates the length of ICU stay and the duration of mechanical ventilation as the best surrogates for morbidity. Such benchmarking and scoring of observed postoperative complications paves the way for an accurate assessment and improvement of quality care in congenital heart surgery.

Interventions after Norwood procedure: comparison of Sano and modified Blalock-Taussig shunt.

Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock-Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. Forty-six (43.0 %) patients underwent interventions, mostly for dilatation of the aortic arch ([DAA] n = 26 [24.3 %]; Sano n = 10, BT n = 16, p = 0.6), dilatation of the shunt ([DS] n = 15 [14.0 %]; Sano n = 11, BT n = 4; p = 0.002), or closure of aortopulmonary collaterals ([APC] n = 15 [14.0 %]; Sano n = 3, BT n = 12; p = 0.08). Mean interval after NP and body weight at DAA, DS, and APC were 72.4 ± 18.9, 108.5 ± 15.8, and 110.7 ± 17.8 days and 4.5 ± 1.3, 4.9 ± 1.9, 5.3 ± 1.2 kg, respectively. The interventions were not associated with mortality but with a greater rate of complications (9 of 46 [21.4 %]) compared with the rate after diagnostic catheterization (0 of 45, p = 0.03). Complications included closure of the femoral or subclavian artery (n = 5), cerebral embolic or bleeding events (n = 4), cardiopulmonary resuscitation (n = 3), and temporary heart block (n = 2). Actuarial survival was similar from the postoperative month 8 onward at 78.6 ± 4.9 % (95 % confidence interval [CI] 67.0-86.5 %) for Sano and 78.4 ± 6.8 % (95 % CI 61.4-88.6 %) for BT (p = 0.95). Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.

Comparison of Prothrombin Complex Concentrate with Activated Factor VII Use for Bleeding Following Cardiopulmonary Bypass in Children.

OBJECTIVE: This study aims to compare the efficacy and safety of activated recombinant factor VII (rFVIIa) and prothrombin complex concentrate (PCC) in the treatment of bleeding complications following surgery requiring cardiopulmonary bypass (CPB) in children. DESIGN/METHODS: This is a retrospective chart review of a single institution comprising patients aged 0 to 18 years old with congenital heart disease. Patients must have received either PCC or rFVIIa after coming off CPB. Our primary efficacy endpoint is time in the operating room from off-CPB to pediatric intensive care unit admission. Our primary safety endpoint is thrombosis through 30 days. RESULTS: Our primary efficacy outcome was significantly shorter in the PCC group compared with the rFVIIa group (P < .0001). Similarly, secondary efficacy outcomes of packed red blood cell administration, chest tube output, and transfusion exposures all significantly favored PCC administration. However, CPB time was significantly longer, and body temperatures were significantly lower, in the rFVIIa group. Safety outcomes, including our primary safety outcome of thrombosis through 30 days, were similar between the two groups. CONCLUSION: This study questions whether PCC could be favored over rFVIIa for hemostasis in children with congenital heart disease following CPB surgery. In addition, this study has found no difference when comparing PCC and rFVIIa in terms of safety outcomes, particularly thrombosis events. There are several limitations to this study due to the retrospective nature of the design and the differences between the two study groups. Despite the limitations, this study suggests that relatively early administration of PCC could be favored over delayed administration of rFVIIa to control recalcitrant post-CPB bleeding in the operating room.

The Supported vs Unsupported Ross in Pediatric Patients: Neoaortic Root and Ventricular Function.

BACKGROUND: The supported Ross is used to mitigate the neoaortic root dilation that has been described with the unsupported Ross. There is limited literature assessing the efficacy of the supported Ross in young patients. In this study, the fate of the neoaortic root was compared in the supported and unsupported Ross procedure in adolescent patients. METHODS: A retrospective review was performed of patients who underwent the Ross procedure between 1996 and 2019. An analysis was conducted of patients aged 10 to 18 years who underwent the supported and unsupported Ross operation, without a Konno enlargement, to assess for longitudinal echocardiographic changes. Given differences in follow-up time, both regression analysis and Mann-Whitney nonparametric tests were used to correct for time from discharge to most recent follow-up. RESULTS: The median follow-up time for supported and unsupported Ross patients without a Konno enlargement was 2.90 years (0.21-13.03 years) and 12.13 years (2.63-19.47 years), respectively. Unsupported Ross patients experienced a higher rate of change per year in the aortic annulus (P = .003 and P = .014) and aortic sinus (P = .002 and P = .002) diameters, respectively. There was no significant difference in the rate of change of end-diastolic left ventricular internal diameter (P = .703 and P = .92) and aortic insufficiency (P = .687 and P = .215) between the supported and unsupported Ross patients. CONCLUSIONS: Progressive dilation of the neoaortic root in unsupported Ross patients is significantly mitigated with the supported Ross with excellent stability. The supported Ross is safe and effective and may play an increasing role in the management of children with aortic disease.

Nuclear Cell-Free DNA Predicts Adverse Events After Pediatric Cardiothoracic Surgery.

BACKGROUND: Preoperative risk stratification in cardiac surgery includes patient and procedure factors that are used in clinical decision-making. Despite these tools, unidentified factors contribute to variation in outcomes. Identification of latent physiologic risk factors may strengthen predictive models. Nuclear cell-free DNA (ncfDNA) increases with tissue injury and drops to baseline levels rapidly. The goal of this investigation is to measure and to observe ncfDNA kinetics in children undergoing heart operations with cardiopulmonary bypass (CPB), linking biomarkers, organ dysfunction, and outcomes. METHODS: This is a prospective observational study of 116 children <18 years and >3 kg undergoing operations with CPB. Plasma ncfDNA samples were collected and processed in a stepwise manner at predefined perioperative time points. The primary outcome measure was occurrence of postoperative cardiac arrest or extracorporeal membrane oxygenation. RESULTS: Data were available in 116 patients (median age, 0.9 years [range, 0-17.4 years]; median weight, 7.8 kg [range, 3.2-98 kg]). The primary outcome was met in 6 of 116 (5.2%). Risk of primary outcome was 2% with ncfDNA <20 ng/mL and 33% with ncfDNA >20 ng/mL (odds ratio, 25; CI, 3.96-158; P = .001). Elevated ncfDNA was associated with fewer hospital-free days (P < .01). CONCLUSIONS: This study analyzes ncfDNA kinetics in children undergoing operations with CPB for congenital heart disease. Elevated preoperative ncfDNA is strongly associated with postoperative arrest and extracorporeal membrane oxygenation. Further studies are needed to validate this technology as a tool to predict morbidity in children after cardiac surgical procedures.

Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature.

Best evidence protocol was applied and the question addressed, whether there is an outcome advantage of a hybrid approach including bilateral pulmonary banding (BPB) and ductal stenting over Norwood procedure (NP) to maintain systemic circulation and to restrict pulmonary blood flow in neonates with hypoplastic left heart syndrome (HLHS). Out of 80 articles published since the original description of BPB in 2002, eight were selected to answer the clinical question. All studies were retrospective case-series after BLB, only three compared results with NP controls, but not in a randomized fashion. Only three chose high-risk patients. Mean follow-up interval was mentioned only in one study. Reported mean hospital, interstage, and stage 2 mortalities after BPB was 17.3, 12.4, and 25.3%, respectively. Estimated transplant-free survival beyond stage 2 palliation ranged from 7 to 80%, with no significant differences to NP controls in three studies. Reintervention rate was high, ranging from 20 to 42% at different sites. Due to study design or inhomogeneity of patient groups, current literature does not show evidence that bilateral pulmonary banding improves outcome with respect to survival beyond second-stage HLHS palliation.

Aortopulmonary window associated with interrupted aortic arch: report of surgical repair of eight cases and review of literature.

BACKGROUND: Patients with combined aortopulmonary window (APW) and interrupted aortic arch (IAA) malformations are rarely seen. We reviewed cases with such association with emphasis on surgical management and long-term outcome. METHODS: 109 patients with IAA and 25 patients with APW were operated upon between 1981 and 2011. The clinical records, operation and follow-up data were analyzed. Long-term outcome was completed with the help of either outpatient data or inquiring. Related literature was investigated. RESULTS: Combined APW/IAA was found in 8 cases: 7.3% (8/109) of those with IAA diagnosis and 32% (8/25) with APW. All APWs were proximal. 7 patients had interruption type A. A ventricular septal defect (VSD) was associated in one case only. Median age at surgery was 10 days. The two first patients were operated upon without cardio-pulmonary bypass (CPB) and one died during operation. The other six underwent single stage approach under CPB with no death: overall early mortality of 12.5% (⅛). APW was closed with one or two patches; aortic arch was reconstructed either directly (extended end-to-side anastomosis) or by patch augmentation. There was no late death and no reoperation during mean follow-up of 118 months (range 1-360 months). Six patients were in functional NYHA class I, the remainder in class II. The cumulative APW/IAA incidence from literature varies between 4.9% (56/1105) and 22.2% (42/189). Early mortality reaches 15.1% (8/53) (95% CI: 6.8% - 27.6%). CONCLUSION: Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation.

Surgical management of congenital heart disease: contribution of the Aristotle complexity score to planning and budgeting in the German diagnosis-related groups system.

Planning and budgeting for congenital heart surgery depend primarily on how closely reimbursement matches costs and on the number and complexity of the surgical procedures. Aristotle complexity scores for the year 2010 were correlated with hospital costs and with reimbursement according to the German diagnosis-related groups (DRG) system. Unit surgical performance was estimated as surgical performance (complexity score × hospital survival) times the number of primary procedures. This study investigated how this performance evolved during years 2006 to 2010. Hospital costs and reimbursements correlated highly with Aristotle comprehensive complexity levels (Spearman r = 1). Mean costs and reimbursement reached 35,050 ± 32,665 and 31,283 ± 34,732, respectively, for an underfunding of 10.7%. Basic and comprehensive unit surgical performances were respectively 3036 ± 1009 and 3891 ± 1591 points in 2006. Both performances increased in sigmoid fashion to reach 3883 ± 1344 and 5335 ± 1314 points, respectively, in 2010. Top performances would be achieved in year 2011, and extrapolated costs would comprise about 19,434,094.92 (95% confidence interval, 11,961,491.22-22,495,764.42). The current underfunding of congenital heart surgery needs correction. The Aristotle score can help to adjust reimbursement according to complexity of procedures. Unit surgical performance allows accurate budgeting in the current German DRG system.

2640 Meters Closer to the Stars: Does High Altitude Affect Fontan Results?

BACKGROUND: Risk factors for and postoperative results of the Fontan operation in patients living at high altitude (>2500 meters above sea level) in the Andean region remain unknown. This study was conducted to evaluate immediate postoperative outcomes and to assess short- and long-term functional class after the Fontan operation. METHODS: From June 2003 to February 2019, 104 patients receiving the Fontan procedure at 2640 meters (8661 feet) above sea level were retrospectively studied. Preoperative catheterization, intraoperative variables, and postoperative outcomes were described. Functional class was evaluated in patients living permanently below (group I) and at or higher than 2500 meters (8202 feet) above sea level (group II). Risk factors for mortality were analyzed. RESULTS: Median age at operation was 8.5 ± 4.4 years; pulmonary artery pressure, 16.2 ± 3.6 mm Hg; end-diastolic systemic ventricular pressure, 13.3 ± 3.8 mm Hg; and pulmonary vascular resistance index, 2.1 (interquartile range, 07-3.7) Wood units. Chest tube duration was 8.5 (6-12) days. Mortality was 4.8%, with 0 in the last 5 years. Higher preoperative pulmonary pressure (16.2 ± 3.6 mm Hg vs 21.2 ± 3.40 mm Hg; P = .01), aortic cross-clamp time (P < .001), and renal failure (P < .01) were associated with mortality. Functional class improved to class I in 86.4%. Overall survival was 90.7% at 10 years of follow-up. CONCLUSIONS: Increased pulmonary pressure and pulmonary vascular resistance index are directly related to high altitude. The Fontan-Kreutzer operation performed at high altitude in the Andean region is feasible with good results. We routinely fenestrate all cases to avoid dysfunction in the early postoperative period. Functional status is adequate after the operation.

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.

Outcomes Following Surgery to Address Shone Syndrome in Children.

BACKGROUND: Shone syndrome is characterized by coincident mitral valve stenosis and left ventricular outflow tract obstruction. Although first described in 1963, little research has expounded surgical outcomes. We sought to evaluate our experience with this cohort, emphasizing outcomes including mortality, morbidity, and cardiac function. METHODS: A retrospective chart review of 46 patients who underwent operation for Shone syndrome between 1990 and May 2018 was conducted. Index operations included 32 repairs of the left ventricular outflow tract, four mitral valve repair/replacements, nine combined repairs, and one non-Shone's repair. Median age at index procedure was 22 days (2 days-10 years). Mean follow-up was 9.1 years (2 months-21 years), and 70 additional operations (51 reoperations) were required. Three patients were lost to follow-up. RESULTS: Overall survival was 95.7% with two late deaths. Freedom from death or transplant was 93.5%. Thirteen (28.3%) patients remained free from reoperation. Thirty-three patients required 51 reoperations of the left ventricle outflow tract (n = 12), mitral valve (n = 16), combined repairs (n = 21), and transplant (n = 1). At most recent follow-up, patients exhibited mitral stenosis (n = 21), aortic stenosis (n = 7), and diminished LV function (n = 2). CONCLUSION: Surgical correction of Shone's offers excellent survival benefit, but reoperation burden is high, with >70% of patients requiring reintervention in the follow-up period. A total of 65% of patients developed recurrent obstruction of left ventricular inflow or outflow, however, ventricular function is preserved in the majority of patients. All but one patient had no functional deficits, classified as New York Heart Association I with > 60% requiring no medication.