Two-Stage Hepatectomy for Bilateral Colorectal Liver Metastases: A Multi-institutional Analysis.

BACKGROUND: Two-stage hepatectomy (TSH) is an important tool in the management of bilateral colorectal liver metastases (CRLM). This study sought to examine the presentation, management, and outcomes of patients completing TSH in major hepatobiliary centers in the United States (US). METHODS: A retrospective review from five liver centers in the US identified patients who completed a TSH procedure for bilateral CRLM. RESULTS: From December 2000 to March 2016, a total of 196 patients were identified. The majority of procedures were performed using an open technique (n = 194, 99.5%). The median number of tumors was 7 (range 2-33). One-hundred and twenty-eight (65.3%) patients underwent portal vein embolization. More patients received chemotherapy prior to the first stage than chemotherapy administration preceding the second stage (92% vs. 60%, p = 0.308). Median overall survival (OS) was 50 months, with a median follow-up of 28 months (range 2-143). Hepatic artery infusion chemotherapy was administered to 64 (32.7%) patients with similar OS as those managed without an infusion pump (p = 0.848). Postoperative morbidity following the second-stage resection was 47.4%. Chemotherapy prior to the second stage did not demonstrate an increased complication rate (p = 0.202). Readmission following the second stage was 10.3% and was associated with a decrease in disease-free survival (p = 0.003). OS was significantly decreased by positive resection margins and increased estimated blood loss (EBL; p = 0.036 and p = 0.05, respectively). CONCLUSION: This is the largest TSH series in the US and demonstrates evidence of safety and feasibility in the management of bilateral CRLM. Outcomes are influenced by margin status and operative EBL.

[Programa de detección del alelo APOE-E4 en adultos mayores mexicanos con deterioro cognitivo].

INTRODUCTION: In Mexico, the prevalence of neurocognitive disorders (NCDs) has increased in parallel with the increase in life expectancy. The E4 allele of the gene that encodes apolipoprotein E (APOE) is the main genetic risk factor for cognitive impairment. OBJECTIVE: To replicate the association of APOE-E4 allele with neurocognitive impairment in a Mexican population, as well as to implement a genetic risk-detection program with the APOE-E4 allele. METHOD: A program was structured for the detection of APOE-E4 allele risk in different recruiting centers from the central zone of the Mexican Republic, with three stages: recruitment and selection of candidates for the detection of the risk-allele, genetic risk analysis and delivery of results. RESULTS: In the genetic-association study to replicate the association with neurocognitive disorders by means of multivariate logistic models, the APOE-E4 allele increased the risk for cognitive impairment in the Mexican populations by approximately 6 % (OR: 5.83, p = 0.0025). In addition, 367 genetic risk results were delivered. CONCLUSIONS: The present program is the first one to be implemented in Mexico with the purpose to inform on a genetic risk factor for neurocognitive disorders in several centers of the country.

INTRODUCCIÓN: En México, la prevalencia de los trastornos neurocognitivos (TNC) han aumentado a la par del incremento en la esperanza de vida. El alelo E4 del gen que codifica la apolipoproteína E (APOE) es el principal factor de riesgo genético para deterioro neurocognitivo. OBJETIVO: Reproducir la asociación en población mexicana entre APOE-E4 y el deterioro neurocognitivo, así como implementar un programa de detección de riesgo genético con el alelo APOE-E4. MÉTODO: Se estructuró un programa de detección de riesgo basado en APO-EA en diferentes centros de reclutamiento en la zona centro de la República Mexicana, con tres etapas: reclutamiento y selección de los candidatos para la detección del alelo de riesgo, análisis del riesgo genético y entrega del resultado. RESULTADOS: El análisis de asociación genética para replicar la asociación con trastornos neurocognitivos mediante modelos logísticos multivariados mostró que el alelo E4 de APOE incrementó aproximadamente 6 % el riesgo en población mexicana (RM = 5.83, p = 0.0025). Se entregaron 367 resultados de riesgo genético. CONCLUSIONES: El presente programa es el primero en México implementado para dar a conocer un factor de riesgo genético para trastornos neurocognitivos en varios centros del país.

Oral Health Assessment of a Group of Children with Autism Disorder.

OBJECTIVE: The aim of this research was to determine the oral status of a group of children with autism. STUDY DESIGN: An observational transversal study was carried out in 96 pediatric patients between the ages of 2 and 16 years old with a diagnosis of autism. The patients were assessed to determine the presence of caries with Index Caries (WHO criteria) and debris and calculus with the Simplified Oral Hygiene Index, as well as the type of diet they followed. RESULTS: It was established that 41.7% of the patients had caries, with the result of an index of DMFT= 0.96 and dmft =2.41. In terms of the periodontal health 59.4% suffered from calculus. The OHI-S was 3.4. CONCLUSIONS: Children with autism exhibited a higher caries prevalence in primary teeth than in permanents. They also presented poor hygiene and an extensive presence of calculus.

Proteomic and morphometric study of the in vitro interaction between Oncidium sphacelatum Lindl. (Orchidaceae) and Thanatephorus sp. RG26 (Ceratobasidiaceae).

Orchidaceae establish symbiotic relationships with fungi in the Rhizoctonia group, resulting in interactions beneficial to both organisms or in cell destruction in one of them (pathogenicity). Previous studies have focused mostly on terrestrial species with a few, preliminary studies, on epiphytes. To further our understanding of the molecular mechanisms involved in these symbioses, we evaluated the interaction between Oncidium sphacelatum Lindl. and the mycorrhizal fungus Thanatephorus sp. strain RG26 (isolated from a different orchid species) in vitro using morphometric and proteomic analyses. Evidence from the morphometric and microscopic analysis showed that the fungus promoted linear growth and differentiation of orchid protocorms during 98 days interaction. On day 63, protocorm development was evident, so we analyzed the physiological response of both organisms at that moment. Proteome results suggest that orchid development stimulated by the fungus apparently involves cell cycle proteins, purine recycling, ribosome biogenesis, energy metabolism, and secretion that were up-regulated in the orchid; whereas in the fungus, a high expression of proteins implicated in stress response, protein-protein interaction, and saccharides and protein biosynthesis were found in the symbiotic interaction. This is the first work reporting proteins differentially expressed in the epiphytic orchid-fungus interaction and will contribute to the search for molecular markers that will facilitate the study of this symbiosis in both wild orchids and those in danger of extinction.

Association of tetralogy of Fallot and complete atrioventricular canal: a single-centre 40-year experience.

OBJECTIVES: Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population. METHODS: Single-centre, retrospective analysis of patients who underwent surgical repair with the diagnosis of ToF-CAVC from 1979 to 2022, divided into 2 different periods and compared. RESULTS: A total of 116 patients were included: 1979-2007 (n = 61) and 2008-2021 (n = 55). Balanced CAVC (80%) and Rastelli type C CAVC (81%) were most common. Patients in the later era were younger (4 vs 14 months, P < 0.001), fewer had trisomy 21 (60% vs 80%, P = 0.019) and fewer had prior palliative prior procedures (31% vs 43%, P < 0.001). In the earlier era, single-patch technique was more common (62% vs 16%, P < 0.001), and in recent era, double-patch technique was more common (84% vs 33%, P < 0.001). In the earlier era, right ventricular outflow tract was most commonly reconstructed with transannular patch (51%), while in more recent era, valve-sparing repairs were more common (69%) (P < 0.001). In-hospital mortality was 4.3%. The median follow-up was 217 and 74 months for the first and second eras. Survival for earlier and later eras at 2-, 5- and 10-year follow-up was (85.1%, 81.5%, 79.6% vs 94.2%, 94.2%, 94.2% respectively, log-rank test P = 0.03). CONCLUSIONS: The surgical approach to ToF-CAVC has evolved over time. More recently, patients tended to receive primary repair at younger ages and had fewer palliative procedures. Improved surgical techniques allowing for earlier and complete repair have shown a decrease in mortality, more valve-sparing procedures without an increase in total reoperations. Presented at the 37th EACTS Annual Meeting, Vienna, Austria.

Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.

OBJECTIVE: Symmetric bicuspidizing repair has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported. METHODS: We performed a retrospective review of patients who underwent the symmetric bicuspidizing repair at Boston Children's Hospital between December 2019 and June 2022 with a contemporary comparator group of patients who underwent other forms of bicuspidization. Survival, valve-related reoperation, and the development of moderate or greater aortic or truncal valve regurgitation were assessed. RESULTS: There were 23 patients who underwent symmetric bicuspidizing repair and 18 who underwent another form of bicuspidization. Preoperative aortic regurgitation was present in 87.0%. Patients who underwent symmetric bicuspidizing repair more commonly underwent suture annuloplasty (100% vs 55.6%; P = .002) and ascending aortoplasty (78.3% vs 27.8%; P = .004). There was 1 operative mortality (2.4%) in the entire cohort and 1 late mortality. Freedom from moderate aortic regurgitation was 87.5% at 21 months after symmetric bicuspidizing repair compared with 43.5% for patients who underwent other types of bicuspidization; P = .03. Freedom from valve-related reoperation was 100% in the symmetric bicuspidizing repair group compared with 64.4%; P = .02. CONCLUSIONS: The symmetric bicuspidizing repair may be safely extended to patients with various forms of congenital aortic and truncal valve disease. Longer term follow-up will be necessary to determine the comparative effectiveness of this technique compared with neocuspidization and the Ross procedure.

The Yasui operation: A single institutional experience over 30 years.

Objective: The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation. Methods: We retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR). Results: Twenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention. Conclusions: The Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

Preliminary Results With a Novel Expanded Polytetrafluoroethylene-based Pulmonary Valved Conduit.

BACKGROUND: A novel polymeric pulmonary valved conduit, resistant to calcification and structural valve deterioration, may provide a more durable therapy option for the pediatric population by preventing loss of right ventricular function and increasing freedom from valve-related reintervention and mortality. METHODS: This was a prospective, multicenter, single-arm study evaluating safety and performance of an investigational novel expanded polytetrafluoroethylene-based valve. Patients met study inclusion/exclusion criteria, had a signed informed consent, had pre- and postoperative evaluation via transthoracic echocardiography, and 6-month cardiac magnetic resonance imaging. RESULTS: Seventeen patients were enrolled from 3 sites. Median age was 12 years (range, 6-17 years) with 52.9% male. Body surface area ranged from 0.82 to 1.57 m2. There has been no mortality and 100% freedom from device related reinterventions. Baseline compared with 6-month cardiac magnetic resonance imaging (in 11 of 16 patients with available data) suggests favorable right ventricular remodeling (right ventricular end-diastolic volume, 123 ± 37 to 94 ± 25 mL/m2) with no significant change in ejection fraction. Through current follow-up, no patient has a right ventricular outflow tract gradient >20 mm Hg (mean, 11.2 ± 4.3 mm Hg). No evidence of worsening valvular insufficiency was observed throughout postoperative serial transthoracic echocardiogram evaluations. No pulmonary regurgitation above baseline (≤ mild) was observed. No patient developed endocarditis. No thrombus or calcification was identified. CONCLUSIONS: This preliminary evaluation of a novel expanded polytetrafluoroethylene-based valved conduit suggests promising valve function with no thromboembolic or infectious complications, no valve related reinterventions, no valve-related adverse events or unexpected findings, improved right ventricular volumes, and encouraging hemodynamic performance through current follow-up.

Contemporary outcomes of the double switch operation for congenitally corrected transposition of the great arteries.

OBJECTIVE: To determine the contemporary outcomes of the double switch operation (DSO) (ie, Mustard or Senning + arterial switch). METHODS: A single-institution, retrospective review of all patients with congenitally corrected transposition of the great arteries undergoing a DSO. RESULTS: Between 1999 and 2019, 103 patients underwent DSO with a Mustard (n = 93) or Senning (n = 10) procedure. Segmental anatomy was (S, L, L) in 93 patients and (I, D, D) in 6 patients. Eight patients had heterotaxy and 71 patients had a ventricular septal defect. Median age was 2.1 years (range, 1.8 months-40 years), including 34 patients younger than age 1 year (33%). Median weight was 10.9 kg (range, 3.4-64 kg). Sixty-one patients had prior pulmonary artery bands for a median of 1.1 years (range, 14 days-12.9 years; interquartile range, 0.7-3.1 years). Median intensive care unit and hospital lengths of stay were 5 and 10 days, respectively. Median follow-up was 3.4 years (interquartile range, 1-9.8 years) and 5.2 years (interquartile range, 2.3-10.7 years) in 79 patients with >1 year follow-up. At latest follow-up, aortic, mitral, tricuspid valve regurgitation, and left ventricle dysfunction was less than moderate in 96%, 98%, 96%, and 93%, respectively. Seventeen patients underwent reoperation: neoaortic valve intervention (n = 10), baffle revision (n = 5), and ventricular septal defect closure (n = 4). At latest follow-up, 17 patients (17%) had a pacemaker and 27 (26%) had cardiac resynchronization therapy devices. There were 2 deaths and 2 transplants. Transplant-free survival was 94.6% at 5 years. Risk factors for death or transplant included longer cardiopulmonary bypass time and older age at DSO. CONCLUSIONS: The outcomes of the DSO are promising. Earlier age at operation might favor better outcomes. Progressive neoaortic regurgitation and reinterventions on the neo-aortic valve are anticipated problems.

The Association of Age and Repair Modification with Outcome after Cone Repair for Ebstein's Malformation.

Cone repair (CR) uses native tissue for tricuspid valve (TV) repair and provides potential for growth. Results after CR were investigated in different age groups including several surgical modifications. Single institution retrospective analysis of all CR excluding neonatal procedures. Endpoints included TV reoperation, late tricuspid regurgitation (TR) and death. Between April 2006 and August 2019, 157 patients underwent CR at a median age of 11.7 years (range, 0.3-57.2). 20% (n=32) of patients had previous surgery. Repair modifications included atrial reduction (n=111,71%), right ventricular plication (n=85,55%), leaflet augmentation (n=36,23%), papillary muscle repositioning (n=50,32%), ring annuloplasty (n=70,45%). Early re-operation for recurrent TR occurred in 11 patients. Median follow-up time was 4.3 years (range, 9d-12.3y). There was no significant association between age at repair and time to TV reoperation (p=0.25). However, age <4 years at CR was identified as the most discriminating binary age threshold for the patients with TV reoperation (25.0% in <4y group vs 9.3% in the ≥4y group). Placement of an annuloplasty ring was protective against ≥moderate TR (OR=0.39, 95% CI 0.16-0.95, p=0.039). Freedom from late TV re-operation was 94.1% at 7 years. Survival was 97.9% at 6 years. Repair after age 18 years was associated with mortality in early follow-up (p=0.037). Mid-term results for CR are favorable in children and adults. Time to TV reoperation may be shorter when CR is performed before age four years, but this result requires confirmation in a larger sample. An annuloplasty ring should be considered when appropriate.

Pulmonary vein stenosis: Anatomic considerations, surgical management, and outcomes.

OBJECTIVE: The study objective was to evaluate outcomes of pulmonary vein stenosis repair in a large single-center cohort. METHODS: Clinical data from a pulmonary vein stenosis registry were retrospectively reviewed identifying patients who underwent pulmonary vein stenosis repair. The primary/index operation was defined as the patient's first pulmonary vein stenosis operation during the study period. RESULTS: Between January 2007 and August 2019, 174 patients underwent pulmonary vein stenosis repair. Bilateral pulmonary vein stenosis occurred in 111 patients (64%); 71 patients (41%) had 4-vessel disease. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein-left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention (balloon dilation ± stenting) was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate (hazard ratio, 2.38, P = .25) or transcatheter intervention (hazard ratio, 0.97, P = .95). The anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P = .042) and multivariable analyses (hazard ratio, 0.19, P = .014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P = .026). CONCLUSIONS: This large single-center surgical pulmonary vein stenosis experience demonstrates encouraging midterm results. A new anatomically focused repair strategy aims to alleviate pulmonary vein angulation to minimize turbulence and shows promising early outcomes. Continued follow-up is required to understand longer-term outcomes for this surgical approach.

Single-Leaflet Aortic Valve Reconstruction Utilizing the Ozaki Technique in Patients With Congenital Aortic Valve Disease.

Evaluate outcomes of single leaflet aortic valve reconstruction using Ozaki sizer and template. Single institute retrospective analysis between August 2015 and August 2019. Thirty-three patients, median age 9.3 years and weight 29.2 kg underwent single leaflet Ozaki repair. Preoperative indications were: AR (n = 17), AS (n = 3) or AS/AR (n = 13). Baseline anatomy was unicuspid (n = 15), bicuspid (n = 9) or tricuspid (n = 9). Two patients had endocarditis. Prior interventions included balloon valvuloplasty (n = 22) and aortic valve repair (n = 9). Pre-op average native annulus diameter was 19.6 mm and peak echo gradient was 36 mm Hg. Autologous pericardium, Photofix and CardioCel bovine pericardium were used in 26, 5, and 2 patients. Non-coronary sinus enlargement was required in 3 and aortic root reduction in 9 patients. Single leaflet reconstruction was done for the right coronary cusp (n = 25), non-coronary cusp in (n = 6) and left coronary cusp (n = 2). Additional procedures were done in 30 patients. Median ICU and hospital LOS were 2.1 and 6.3 days. There were no early re-interventions or conversions to valve replacement and one unrelated mortality.en At discharge, all patients had < moderate AR and/or AS with average peak gradients of 15 mm Hg. The median follow-up was 1.1 year, (IQR 0.7-1.8 years). Freedom from ≥ moderate AR and AS at 2 years was 76% and 86%. One patient required surgical re-intervention for severe AR 1.5 years after surgery for inflammatory infiltrate with calcification and fibrosis. Single-leaflet aortic valve leaflet reconstruction utilizing the Ozaki technique has promising early results and can be considered in patients when there are acceptable native leaflets.

Pulmonary Valve Reconstruction Using the Ozaki Leaflet Reconstructive Techniques.

The aortic valve neocuspidization, or Ozaki procedure, has been shown to be technically reproducible and have acceptable results in the aortic position in adult and pediatric patients. We report a case in which innovative techniques were utilized to create a three-leaflet pulmonary valve using a modified aortic valve neocuspidization technique.

Congenital aortic and truncal valve reconstruction using the Ozaki technique: Short-term clinical results.

OBJECTIVES: Aortic valve reconstruction (AVRec) with neocuspidization or the Ozaki procedure with complete cusp replacement for aortic valve disease has excellent mid-term results in adults. Limited results of AVRec in pediatric patients have been reported. We report our early outcomes of the Ozaki procedure for congenital aortic and truncal valve disease. METHODS: A retrospective analysis was performed on all 57 patients with congenital aortic and truncal valve disease who had a 3-leaflet Ozaki procedure at a single institution from August 2015 to February 2019. Outcome measures included mortality, surgical or catheter-based reinterventions, and echocardiographic measurements. RESULTS: Twenty-four patients had aortic regurgitation (AR), 6 had aortic stenosis (AS), and 27 patients had AS/AR. Two patients had quadricuspid valves, 26 had tricuspid, 20 had bicuspid, and 9 had unicusp aortic valves. Four patients had truncus arteriosus. Thirty-four patients had previous aortic valve repairs and 5 had replacements. Preoperative echocardiography mean annular diameter was 20.90 ± 4.98 cm and peak gradient for patients with AS/AR was 53.62 ± 22.20 mm Hg. Autologous, Photofix, and CardioCel bovine pericardia were used in 20, 35, and 2 patients. Eight patients required aortic root enlargement and 20 had sinus enlargement. Fifty-one patients had concomitant procedures. Median intensive care unit and hospital length of stay were 1.87 and 6.38 days. There were no hospital mortalities or early conversions to valve replacement. At discharge, 98% of patients had mild or less regurgitation and peak aortic gradient was 16.9 ± 9.5 mm Hg. Two patients underwent aortic valve replacement. At median follow-up of 8.1 months, 96% and 91% of patients had less than moderate regurgitation and stenosis, respectively. CONCLUSIONS: The AVRec procedure has acceptable short-term results and should be considered for valve reconstruction in pediatric patients with congenital aortic and truncal valve disease. Longer-term follow-up is necessary to determine the optimal patch material and late valve function and continued annular growth.

Ascending Aortoplasty in Pediatric Patients Undergoing Aortic Valve Procedures.

BACKGROUND: The risks of ascending aortic dilation and indications for intervention in pediatric patients are unclear. Given the concern for aortic size mismatch with growth, larger ascending aortic diameters have been accepted in pediatric patients. The purpose of this study was to evaluate the effectiveness of ascending aortic reduction at the time of aortic valve surgery and its effect on recurrent aortic regurgitation. METHODS: A retrospective 8-year observational review (2010-2018) was conducted in pediatric patients with dilated ascending aorta undergoing aortic valve surgery at a single institution. RESULTS: Forty-seven patients underwent ascending aortoplasty during aortic valve surgery, of which 39 patients with complete data points had preoperative mean ascending aortic Z-scores of 5.35 ± 1.52 reduced to 1.22 ± 1.63 (P < .001) postoperatively. Ascending aortic Z-scores remained similar at 1.37 ± 1.72 at latest follow-up (P = .306). In contrast our matched control group (n = 39) without ascending aortoplasty had preoperative mean ascending aortic Z-scores of 4.15 ± 1.65 reduced to 3.26 ± 2.0 at the midterm follow-up. This reduction was significantly larger in the aortoplasty group, with an average difference in these changes of 3.12 (95% confidence interval, 2.43-3.8; P < .001). Multivariable logistic regression (controlling for aortic valve intervention type and body surface area) showed a 6.84 odds of moderate to severe aortic regurgitation at follow-up in the control group compared with the aortoplasty group (95% confidence interval, 1.43-32.8; P = .016). There were no complications associated with ascending aortoplasty. CONCLUSIONS: Ascending aortoplasty is effective in reducing ascending aortic dimensions and recurrent aortic regurgitation in pediatric patients. Longer-term follow-up is necessary to determine continued rate of growth.

Experience and Outcomes of Surgically Implanted Melody Valve in the Pulmonary Position.

BACKGROUND: Multiple congenital cardiac malformations require pulmonary valve replacement and/or right ventricular outflow (RVOT) reconstruction. Pulmonary valve replacement remains challenging in children owing to the limited growth potential of prosthetic valves. We evaluated outcomes in patients undergoing surgical implantation of a Melody valve in the RVOT. METHODS: Data were retrospectively collected for 23 patients undergoing surgical Melody valve implantation at Boston Children's Hospital between 2009 and 2019. We assessed postoperative valve function, reintervention rates, and mortality. RESULTS: Median age was 1.7 years (range, 2 months to 6 years); 12 patients were aged greater than 2 years (52%). Diagnosis was tetralogy of Fallot in 15 patients (65%); 15 had a prior RVOT operation (65%). The Melody valve was dilated before surgery to a median diameter of 14 mm (range, 10-20 mm). No patients had acute pulmonary regurgitation. One required transcatheter RVOT reintervention before discharge. Median follow-up was 3.7 years (range, 0.02-8.7 years) with moderate or greater pulmonary regurgitation in 2 patients. Catheter-based interventions (mean, 0.83 ± 1.07/patient) occurred at a median of 1 year (range, 16 days to 5.4 years) and included valve expansion for somatic growth (n = 10) and subsequent valve-in-valve replacement (n = 3). Three patients (13%) required surgical valve explant or replacement at a median of 1.0 year (range, 0.6-3.7 years) for Melody-specific indications. One-, 3-, and 5-year freedom from Melody-driven reoperation was 90%, 90%, and 83%, respectively. CONCLUSIONS: The Melody valve can be surgically implanted in the RVOT of young patients with acceptable early results. These valves can be successfully dilated through transcatheter reintervention to accommodate growth.

Reintervention rates after bioprosthetic pulmonary valve replacement in patients younger than 30 years of age: A multicenter analysis.

OBJECTIVES: To assess the difference in time to and predictors of reintervention according to valve type in surgical bioprosthetic pulmonary valve replacement (PVR) in patients younger than 30 years of age from multiple centers. METHODS: Data were retrospectively collected for 1278 patients <30 years of age undergoing PVR at 8 centers between 1996 and 2015. RESULTS: Mean age at PVR was 19.3 ± 12.8 years, with 719 (56.3%) patients ≤18 years of age. Diagnosis was tetralogy of Fallot in 626 patients (50.5%) and 165 (12.9%) had previous PVR. Median follow-up was 3.9 years (interquartile range, 1.2, 6.4). Multiple valve types were used, most commonly CE PERIMOUNT, 488 (38.2%), CE Magna/Magna Ease, 361 (28.2%), and Sorin Mitroflow 322 (25.2). Reintervention occurred in 12.7% and was most commonly due to pulmonary stenosis (68.8%), with most reinterventions occurring in children (85.2%) and with smaller valve sizes (P < .001) Among adults aged 18 to 30 years, younger age was not a significant risk factor for reintervention. Surgical indication of isolated pulmonary regurgitation was associated with a lower risk of reintervention (P < .001). Overall, 1-, 3-, 5-, and 10-year freedom from reintervention rates were 99%, 97%, 92%, and 65%. The only independent risk factors for reintervention after controlling for age and valve size were lack of a concomitant tricuspid valve procedure (P = .02) and valve type (P < .001); Sorin and St Jude valves were associated with similar time to reintervention, and deteriorated more rapidly than other valve types. CONCLUSIONS: In this large multicenter study, 8% of patients have undergone reintervention by 5 years. Importantly, independent of age and valve size, reintervention rates vary by valve type.

Dental treatment of Marfan syndrome. With regard to a case.

Marfan syndrome (MS) is the most common dominant autosomic genetic disorder of the connective tissue. It has a reported incidence of 1 per each 5000 individuals without any distinction of gender or ethnicity. This pathology's diagnosis is mainly based on physical characteristics, presenting three main different symptomatic charts: neonatal Marfan, infant Marfan and classical Marfan. The mayor characteristic of these patients consists of an exaggerated length of the upper and lower limbs, hyperlaxity, scoliosis, alterations in the cardiovascular and pulmonary systems and atypical bone overgrowth. The individual implied in the present investigation concerned to a 14 year old male patient presenting multiple mouth lesions and dental alterations, attended in the Department of Pediatric Dentistry degree at the Dentistry School in the Santa Maria University. The patient has been treated following the necessary considerations required according to his systemic compromise under oral premedication for decrease the anxiety and make easier the behavior management. The patient with MS has multiple oral decrease that may be diagnosed as treated on time to increase the life quality of the patient.

Modified Ozaki Procedure Including Annular Enlargement for Small Aortic Annuli in Young Patients.

BACKGROUND: Aortic valve neocuspidization (AVNeo) (Ozaki procedure) has excellent midterm results in adults. Outcomes in patients with a small native aortic annulus are unknown. We report early outcomes in young patients with small native aortic valve annuli. METHODS: Retrospective data of patients undergoing AVNeo between 2015 and 2019 were reviewed. Patients with native aortic annulus less than 21 mm undergoing 3-leaflet AVNeo were included. RESULTS: A total of 51 patients were identified (median age 7.9 years; median weight 21 kg), and 80% patients were less than or equal to 12 years age. Preoperative indication was aortic regurgitation (AR) (n = 23), aortic stenosis (AS) (n = 22), or mixed AS and AR (n = 6). Baseline anatomy was quadricuspid (n = 1), tricuspid (n = 23), bicuspid (n = 15), or unicuspid (n = 12) valve. Preoperative peak gradient for AS and mixed AS and AR patients was 55.36 mm Hg. Median native annulus diameter was 17 mm; sinus and annular enlargements were required in 22 patients and 9 patients, respectively. Median intensive care unit and hospital length of stay were 2.0 days and 7.2 days, respectively. There were no reinterventions, and there was 1 hospital mortality unrelated to aortic valve. At discharge, 94% of patients had less than or equal to mild AR, and the median peak gradient was 18 mm Hg. At mean follow-up of 11.9 months, 80% and 82% of patients had less than moderate AR and AS, respectively. Three patients required surgical reintervention. In annular enlargement patients, mean annulus Z score remained greater than 0 at follow-up. CONCLUSIONS: The Ozaki procedure has acceptable short-term results in young patients with small aortic annuli. A larger aortic annulus can be achieved with surgical annular enlargement. Long-term follow-up is necessary to determine late valve function and potential continued annular growth.

Early Infant Symptomatic Patients With Tetralogy of Fallot With Absent Pulmonary Valve: Pulmonary Artery Management and Airway Stabilization.

BACKGROUND: Tracheobronchomalacia and airway obstruction from severely dilated pulmonary arteries in tetralogy of Fallot with absent pulmonary valve (TOF-APV) has been associated with high rates of respiratory failure and mortality (15% to 25%). It is not known whether aggressive pulmonary artery (PA) or direct airway intervention during early definitive cardiac repair improves outcomes. METHODS: A retrospective observational study was made of all patients undergoing surgical repair for TOF-APV at our center between 2006 and 2018. RESULTS: Twenty patients underwent repair at a median age of 51 days and PA Z-scores of 8.1. Twelve patients had a valve implanted, 6 of whom required reoperation for valve replacement at a median of 9 months (range, 3 to 28) compared with 8 who had initial transannular patch, and only 1 patient required subsequent valve replacement (P < .05). Seven patients had central PAs replaced with thin-walled Gore-Tex (WL Gore, Flagstaff, AZ) grafts; none of these required PA reoperation during a median follow-up of 26.5 months, whereas 3 of 13 patients who did not have PA replacement with Gore-Tex required subsequent PA reoperation (P < .05). Concomitant airway interventions (eg, tracheobronchopexy/plasty) were performed in 4 patients and none required subsequent airway interventions, whereas 2 patients not having initial airway intervention required subsequent tracheopexy (P < .05). Three patients in the cohort eventually required tracheostomy (15%), and 2 patients died (10%; on postoperative days 30 and 326); none had received initial airway intervention. CONCLUSIONS: Pulmonary artery replacement and aggressive direct airway management at initial definitive repair of cardiac TOF-APV can be performed safely with acceptable survival outcomes and low rates of airway and PA reintervention.