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Background: Congenital mitral valve disease (CMVD) presents major challenges in its medical and surgical management. Objectives: The purpose of this study was to investigate the value of 3-dimensional echocardiography (3DE) and identify associations with MV reoperation in this setting. Methods: All children <18 years of age who underwent MV reconstruction for CMVD in 2002 to 2018 were included. Preoperative and postoperative 2-dimensional echocardiography (2DE) and 3DE data were collected. Competing risks and Cox regression analysis were used to identify independent associations with MV reoperation. Receiver operating characteristic and decision-tree analysis were implemented for comparison of 3DE vs 2DE. Results: A total of 206 children underwent MV reconstruction for CMVD (mitral stenosis, n = 105, mitral regurgitation [MR], n = 75; mixed disease, n = 26); 64 (31%) required MV reoperation. Variables independently associated with MV reoperation were age <1 year (HR: 2.65; 95% CI: 1.13-6.21), tethered leaflets (HR: 2.00; 95% CI: 1.05-3.82), ≥ moderate 2DE postoperative MR (HR: 4.26; 95% CI: 2.45-7.40), changes in 3D-effective orifice area (3D-EOA) and in 3D-vena contracta regurgitant area (3D-VCRA). Changes in 3D-EOA and 3D-VCRA were more strongly associated with MV reoperation than changes in mean gradients (area under the curve [AUC]: 0.847 vs AUC: 0.676, P = 0.006) and 2D-VCRA (AUC: 0.969 vs AUC: 0.720, P = 0.012), respectively. Decision-tree analysis found that a <30% increase in 3D-EOA had 80% accuracy (HR = 8.50; 95% CI: 2.9-25.1) and a <40% decrease in 3D-VCRA had 93% accuracy (HR: 22.50; 95% CI: 2.9-175) in discriminating MV reoperation for stenotic and regurgitant MV, respectively. Conclusions: Age <1 year, tethered leaflets, 2DE postoperative MR, changes in 3D-EOA and 3D-VCRA were all independently associated with MV reoperation. 3DE parameters showed a stronger association than 2DE. 3DE-based decision-tree algorithms may help prognostication and serve as a support tool for clinical decision-making.
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Background: Single ventricle (SV) patients with interrupted inferior vena cava (iIVC) and azygos continuation are at high risk for unbalanced hepatic venous flow (HVF) distribution to the lungs after Fontan completion and subsequent pulmonary arteriovenous malformations (AVMs) formation. Objectives: The aim of the study was to utilize computational fluid dynamics (CFD) analysis to avoid maldistribution of HVF to the lungs after Fontan surgery. Methods: Four SV subjects with iIVC were prospectively studied with a 3-dimensional (3D) modeling workflow with digital 3D models created from segmented magnetic resonance images or computer tomography scans, virtual surgery, and CFD analysis over multiple physiologic states for the evaluation of operative plans to achieve balanced HVF to both lungs. Three of the patients were Fontan revision candidates with existing AVMs. All patients underwent Fontan completion or revision surgery. Results: CFD predicted that existing or proposed Fontan completion in all patients would result in 100% of HVF to one lung. Improved HVF balance was achieved with CFD analysis of alternative surgical approaches resulting in the average distribution of HVF to the right/left pulmonary arteries of 37%/63% ± 10.4%. A hepatoazygos shunt was required in all patients and additional creation of an innominate vein in one. CFD analysis was validated by the comparison of pre-operative predicted and postoperative MRI-measured total right/left pulmonary flow (51%/49% ± 5.4% vs 49%/51% ± 8.5%). Conclusions: A 3D modeling workflow with CFD simulation for SV patients with iIVC may avoid HVF maldistribution and development of AVMs after Fontan completion.
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Accurate knowledge of right ventricular (RV) volumes and ejection fraction is fundamental to providing optimal care for pediatric patients with congenital and acquired heart disease, as well as pulmonary hypertension. Traditionally, these volumes have been measured using cardiac magnetic resonance because of its accuracy, reproducibility, and freedom from geometric assumptions. More recently, an increasing number of studies have described the measurement of RV volumes using three-dimensional (3D) echocardiography. In addition, volumes by 3D echocardiography have also been used for outcome research studies in congenital heart surgery. Importantly, 3D echocardiographic acquisitions can be obtained over a small number of cardiac cycles, do not require general anesthesia, and are less costly than CMR. The ease and safety of the 3D echocardiographic acquisitions allow serial studies in the same patient. Moreover, the studies can be performed in various locations, including the intensive care unit, catheterization laboratory, and general clinic. Because of these advantages, 3D echocardiography is ideal for serial evaluation of the same patient. Despite these potential advantages, 3D echocardiography has not become a standard practice in children with congenital and acquired heart conditions. In this report, the authors review the literature on the feasibility, reproducibility, and accuracy of 3D echocardiography in pediatric patients. In addition, the authors investigate the advantages and limitations of 3D echocardiography in RV quantification and offer a pathway for its potential to become a standard practice in the assessment, planning, and follow-up of congenital and acquired heart disease.
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Ecocardiografia Tridimensional , Humanos , Ecocardiografia Tridimensional/métodos , Criança , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Reprodutibilidade dos Testes , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/fisiopatologia , Volume Sistólico/fisiologia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Pré-Escolar , Lactente , Sensibilidade e Especificidade , Cardiopatias/diagnóstico por imagem , Cardiopatias/fisiopatologia , Cardiopatias/diagnósticoRESUMO
Background: Accurate measurement of ventricular volumes is an important clinical imaging goal. Three-dimensional echocardiography (3DEcho) is used increasingly as it is more available and less costly than cardiac magnetic resonance (CMR). For the right ventricle (RV), the current practice is to acquire 3DEcho volumes from the apical view. However, in some patients the RV may be better seen from the subcostal view. Therefore, this study compared RV volume measurements from the apical vs. the subcostal view, using CMR as a reference standard. Methods: Patients <18 years old undergoing a clinical CMR examination were prospectively enrolled. 3DEcho was performed on the day of the CMR. 3DEcho images were acquired with Philips Epic 7 ultrasound system from apical and subcostal views. Offline analysis was performed with TomTec 4DRV Function for 3DEcho images and cvi42 for CMR ones. RV end-diastolic volume and end-systolic volume were collected. Agreement between 3DEcho and CMR was assessed with Bland-Altman analysis and the intraclass correlation coefficient (ICC). Percentage (%) error was calculated using CMR as the reference standard. Results: Forty-seven patients were included in the analysis (age range 10 months to 16 years). The ICC was moderate to excellent for all volume comparisons to CMR (subcostal vs. CMR: end-diastolic volume 0.93, end-systolic volume 0.81; apical vs. CMR: end-diastolic volume 0.94, end-systolic volume 0.74).The 3DEcho mean % error vs. CMR for end-systolic volume was 25% for subcostal and 31% for apical; for end-diastolic volume it was 15% for subcostal and 16% for apical. The % error was not significantly different between apical vs. subcostal views for end-systolic and end-diastolic volume measurements. Conclusions: For apical and subcostal views, 3DEcho-derived ventricular volumes agree well with CMR. Neither echo view has a consistently smaller error when compared to CMR volumes. Accordingly, the subcostal view can be used as an alternative to the apical view when acquiring 3DEcho volumes in pediatric patients, particularly when the image quality from this window is superior.
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Objective: Restoration of biventricular circulation is an alternative management strategy in unbalanced atrioventricular canal defects (uAVCDs), especially in patients with risk factors for single-ventricle palliation (SVP) failure. When ventricular volume is inadequate for biventricular circulation, recruitment procedures may accommodate its growth. In this study, we review our uAVCD experience with biventricular conversion (BIVC) after prior SVP. Methods: This is a single-institution, retrospective cohort study of uAVCD patients who underwent BIVC after SVP, with staged recruitment (staged) or primary BIVC (direct) between 2003 to 2018. Mortality, unplanned reinterventions, imaging, and catheterization data were analyzed. Results: Sixty-five patients underwent BIVC from SVP (17 stage 1, 42 bidirectional Glenn, and 6 Fontan). Decision for conversion was based on poor SVP candidacy (n = 43) or 2 adequately sized ventricles (n = 22). Of the 65 patients, 20 patients underwent recruitment before conversion. The staged group had more severe ventricular hypoplasia than the direct group, reflected in prestaging end-diastolic volume z scores (-4.0 vs -2.6; P < .01), which significantly improved after recruitment (-4.0 to -1.8; P < .01). Median follow-up time was 1.0 years. Survival and recatheterizations were similar between both groups (hazard ratio, 0.9; 95% CI, 0.2-3.7; P = .95 and hazard ratio, 1.9; 95% CI, 0.9-4.1; P = .09), but more reoperations occurred with staged approach (hazard ratio, 3.1; 95% CI, 1.3-7.1; P = .01). Conclusions: Biventricular conversion from SVP is an alternative strategy to manage uAVCD, particularly when risk factors for SVP failure are present. Severe forms of uAVCDs can be converted with staged BIVC with acceptable mortality, albeit increased reinterventions, when primary BIVC is not possible.
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Acute rheumatic fever and its chronic sequela, rheumatic heart disease (RHD), pose major health problems globally, and remain the most common cardiovascular disease in children and young people worldwide. Echocardiography is the most important diagnostic tool in recognizing this preventable and treatable disease and plays an invaluable role in detecting the presence of subclinical disease needing prompt therapy or follow-up assessment. This document provides recommendations for the comprehensive use of echocardiography in the diagnosis and therapeutic intervention of RHD. Echocardiographic diagnosis of RHD is made when typical findings of valvular and subvalvular abnormalities are seen, including commissural fusion, leaflet thickening, and restricted leaflet mobility, with varying degrees of calcification. The mitral valve is predominantly affected, most often leading to mitral stenosis. Mixed valve disease and associated cardiopulmonary pathology are common. The severity of valvular lesions and hemodynamic effects on the cardiac chambers and pulmonary artery pressures should be rigorously examined. It is essential to take advantage of all available modalities of echocardiography to obtain accurate anatomic and hemodynamic details of the affected valve lesion(s) for diagnostic and strategic pre-treatment planning. Intraprocedural echocardiographic guidance is critical during catheter-based or surgical treatment of RHD, as is echocardiographic surveillance for post-intervention complications or disease progression. The role of echocardiography is indispensable in the entire spectrum of RHD management.
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Estenose da Valva Mitral , Febre Reumática , Cardiopatia Reumática , Criança , Humanos , Adolescente , Cardiopatia Reumática/diagnóstico por imagem , Ecocardiografia , Febre Reumática/complicações , Estenose da Valva Mitral/diagnóstico por imagem , Valva Mitral , Progressão da DoençaRESUMO
BACKGROUND: We sought to validate the technical performance score (TPS) as a predictor of midterm outcomes after congenital aortic valve repair. METHODS: This was a single-center, retrospective review of consecutive patients who underwent aortic valve repair between January 1, 2011, and December 31, 2019. Predischarge echocardiograms were used to assign a TPS for each index operation as class 1, no aortic valve residua; class 2, minor aortic valve residua; or class 3, major aortic valve residua or predischarge reintervention for major residua. The primary outcome was postdischarge (late) unplanned aortic valve reintervention. Secondary outcomes included late mortality and at least moderate aortic regurgitation or stenosis at the latest follow-up or before the earliest reintervention. Associations between TPS and outcomes were assessed using competing risk, Cox proportional hazards, or logistic regression models, adjusting for preoperative patient- and procedure-related covariates. RESULTS: Of 507 patients, there were 110 (21.7%) reinterventions, 22 (4.3%) deaths, and 67 (13.2%) cases of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention. On multivariable analysis, class 3 patients had a greater risk of reintervention (subdistribution hazard ratio, 2.6; 95% CI, 1.3-5.1; P = .005) and mortality (hazard ratio, 5.3; 95% CI. 1.1-25.2; P = .038) compared with class 1 patients. Adjusting for duration of follow-up, class 3 patients also had a greater risk of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention (odds ratio, 7.7; 95% CI, 2.5-24.2; P < .001) vs class 1 patients. CONCLUSIONS: Patients with major residua after congenital aortic valve repair have significantly worse midterm outcomes compared with those with no residua, warranting closer follow-up.
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Insuficiência da Valva Aórtica , Humanos , Insuficiência da Valva Aórtica/cirurgia , Constrição Patológica , Assistência ao Convalescente , Resultado do Tratamento , Alta do Paciente , Estudos Retrospectivos , Fatores de RiscoRESUMO
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.
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Insuficiência da Valva Aórtica , Doenças das Valvas Cardíacas , Adulto , Criança , Humanos , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/congênito , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/cirurgia , Aorta/cirurgia , Valva Mitral , Estudos Retrospectivos , Reoperação , Resultado do TratamentoRESUMO
Objective: To assess the safety and feasibility of low-dose, novel, allogenic mesenchymal precursor cell (MPC) therapy as an adjunct to left ventricular (LV) recruitment for patients with hypoplastic left heart syndrome (HLHS) and borderline left ventricles. MPC injections into the hypoplastic left ventricle may stimulate neovascularization and beneficial LV remodeling and may improve the likelihood of achieving biventricular (BiV) or 1.5 ventricle (1.5V) circulation. Methods: Children <5 years with prior single ventricle palliation undergoing LV recruitment surgery at a single center were randomized to MPC injections into the LV endocardium/papillary muscles (MPCs) or standard-of-care (controls) and followed for 24 months. The primary endpoint was safety, including (serious) adverse events (S/AEs), and panel reactive antibodies (PRAs). Secondary endpoints included BiV/1.5V conversion and LV size and function. Results: Nineteen subjects were enrolled, including 9 MPC recipients and 10 controls. Fourteen patients (74%) had >1 AE, and 2 patients had SAEs, both deemed unrelated to the trial product. AE severity and frequency were similar in the 2 groups. Baseline PRA levels were high, with no difference between the groups at 12 months. The overall probability of BiV/1.5V conversion was 0.16 (95% confidence interval [CI], 0.05 to 0.41) at 12 months and 0.52 (95% CI, 0.31 to 0.77) at 24 months. For patients with imaging data at both time points, increases in LV volumes from baseline to 12 months were larger in the MPC group by 3-dimensional echocardiography and cardiac magnetic resonance imaging. For children who successfully underwent BiV conversion (n = 12), full BiV conversion was achieved at 24 months in 5 of 5 (100%) MPC-treated children compared with 4 of 7 (57%) controls. Conclusions: MPC injections were considered safe and feasible in HLHS patients. More than 50% of subjects underwent BiV/1.5V conversion within 2 years. Larger trials are needed to investigate the therapeutic potential of MPCs in this population.
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This is the first description of active clinical manifestation of endocardial fibroelastosis (EFE) and remodeling of the endocardium via endothelial-to-mesenchymal transformation (EndMT) in an adolescent with Shone's variant hypoplastic left heart complex (HLHC) and a genetic heterozygous ABL1 variant. While EFE has not been typically associated HLHC or Shone's syndrome, in this patient flow alterations in the left ventricle (LV), combined with genetic alterations of intrinsic EndMT pathways led to active clinical manifestation of EFE in adolescence. This case emphasizes that new therapies for EFE might need to focus on molecular factors influenced by intrinsic and extrinsic stimuli of EndMT.
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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.
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Transposição das Grandes Artérias , Comunicação Interventricular , Transposição dos Grandes Vasos , Humanos , Lactente , Pré-Escolar , Transposição das Grandes Artérias/efeitos adversos , Transposição das Grandes Artérias Corrigida Congenitamente , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Resultado do Tratamento , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , SeguimentosRESUMO
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.
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Valvopatia Aórtica , Insuficiência da Valva Aórtica , Humanos , Bovinos , Animais , Criança , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Insuficiência da Valva Aórtica/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Pericárdio/transplanteRESUMO
OBJECTIVE: To assess the feasibility and outcomes of biventricular conversion following takedown of Fontan circulation. METHODS: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to biventricular circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mm Hg and/or the presence of associated complications. RESULTS: Biventricular conversion was performed in 23 patients at a median age of 10.0 (7.5-13.0) years. Indications included failing Fontan physiology in 15 (65%) and elective takedown in 8 (35%) patients. A subset of patients (n = 6) underwent procedures for staged recruitment of the nondominant ventricle before conversion. Median z score of end-diastolic volume of borderline ventricle before takedown was -2.3 (-3.3, -1.3). Hypoplastic left heart syndrome (P < .01) and sub-/aortic stenosis (P < .01) were more common in these patients. Biventricular conversion with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (P < .01), indexed end-systolic volume (P < .01), and ventricular mass (P < .01) of the nondominant ventricle (14 right, 9 left ventricle). There were 5 (22%) deaths (1 [4%] early death). All who underwent elective biventricular conversion survived, whereas 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% confidence interval, 37%-90%). The overall, 3-year reoperation-free survival was 86.7% (95% confidence interval, 56%-96%). Left dominant atrioventricular canal defect (P < .01) and early era of biventricular conversion (P = .02) were significant predictors for mortality. CONCLUSIONS: A primary as well as a staged biventricular conversion is feasible in patients who have had previous Fontan procedure. Although this provides an alternative to transplantation in patients with failing Fontan, outcomes are worse in those with failing Fontan compared with elective takedown of Fontan circulation. Optimal timing needs further evaluation.
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Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Reoperação , Função Ventricular Esquerda , Função Ventricular Direita , Adolescente , Criança , Pré-Escolar , Estudos de Viabilidade , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Masculino , Reoperação/efeitos adversos , Reoperação/mortalidade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Falha de TratamentoRESUMO
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.
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Anomalia de Ebstein , Insuficiência da Valva Tricúspide , Adolescente , Adulto , Criança , Pré-Escolar , Anomalia de Ebstein/complicações , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/cirurgia , Humanos , Lactente , Recém-Nascido , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/complicações , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/cirurgia , Adulto JovemRESUMO
[Figure: see text].
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Transposição dos Grandes Vasos , Septo Interventricular , Transposição das Grandes Artérias Corrigida Congenitamente , Humanos , Cuidados Paliativos , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Resultado do Tratamento , Septo Interventricular/diagnóstico por imagem , Septo Interventricular/cirurgiaRESUMO
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.
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Valvopatia Aórtica , Valva Aórtica/cirurgia , Procedimentos Cirúrgicos Cardíacos , Adolescente , Adulto , Valvopatia Aórtica/mortalidade , Valvopatia Aórtica/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Criança , Pré-Escolar , Ecocardiografia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Reoperação , Estudos Retrospectivos , Adulto JovemRESUMO
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.
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Manuseio das Vias Aéreas/métodos , Artéria Pulmonar/cirurgia , Valva Pulmonar/anormalidades , Tetralogia de Fallot/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses. METHODS: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated. RESULTS: Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%]). CONCLUSIONS: Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk.
Assuntos
Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Valva Mitral , Feminino , Doenças das Valvas Cardíacas/etiologia , Doenças das Valvas Cardíacas/mortalidade , Humanos , Lactente , Masculino , Países Baixos , Desenho de Prótese , Reoperação , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento , Estados UnidosRESUMO
BACKGROUND: Strain and synchrony can be calculated from a variety of software packages, but there is a paucity of data with inter-vendor comparisons in children. To test the hypothesis that different packages may affect results, independent of acquisition, we compared values obtained using two commercially available analysis tool (QLAB and TomTec), with several different settings. METHODS: The study population included 108 children; patients were divided into three groups: (1) normal cardiac structure and conduction; (2) ventricular paced rhythm; and (3) flattened ventricular septum (reflecting right ventricular pressure or volume load lesions). We analyzed the same image acquired from the apical 4-chamber (AP4) and short-axis at the mid-papillary level (SAXM) views in both QLAB (versions 10.5 and 10.8) and TomTec (version 1.2). In QLAB version 10.8, low, medium, and high quantification smoothness settings were employed. In TomTec, images were analyzed with both low and high frame rates. Tracking quality for each package was graded. AP4 and SAXM strain and synchrony values were recorded. A mixed-effects linear regression model was used, with main effect considered significant if the p-value was < 0.05. RESULTS: Tracking scores were high for all packages except QLAB 10.5 in the SAXM view. AP4 and SAXM strain values varied significantly between QLAB 10.5 and the other packages. Synchrony values varied widely for all strain values (p < 0.001 for both) in all packages. Quantification smoothness changes in QLAB 10.8 did not impact strain significantly in any patient group; temporal resolution changes in TomTec resulted in strain differences in children with flat ventricular septums, but not those with normal or ventricular paced hearts. CONCLUSION: Synchrony values varied substantially among all packages in children. Strain values varied widely between QLAB 10.5 and all other software packages, recommending avoidance of QLAB 10.5 for future studies. Quantification smoothness settings in QLAB 10.8 resulted in minimal strain differences. In TomTec, low and high frame rate strain values differed only in a subset of patients (flattened septum). These data suggest that reliable comparisons between strain values derived from QLAB and TomTec is possible in certain cases, but that caution should be used especially in different hemodynamics conditions.