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1.
Autops. Case Rep ; 9(3): e2019118, July-Sept. 2019. ilus
Artigo em Inglês | LILACS | ID: biblio-1017367

RESUMO

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.


Assuntos
Humanos , Feminino , Recém-Nascido , Derivação Cardíaca Direita , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/patologia , Autopsia , Evolução Fatal , Atresia Tricúspide/complicações , Procedimento de Blalock-Taussig/efeitos adversos
2.
Methodist Debakey Cardiovasc J ; 15(2): 105-110, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384373

RESUMO

Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure.


Assuntos
Arritmias Cardíacas/cirurgia , Cardiomegalia/cirurgia , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Adolescente , Adulto , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Cardiomegalia/etiologia , Cardiomegalia/mortalidade , Cardiomegalia/fisiopatologia , Criança , Pré-Escolar , Tomada de Decisão Clínica , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Intervalo Livre de Progressão , Reoperação , Medição de Risco , Fatores de Risco , Fatores de Tempo , Adulto Jovem
3.
Medicine (Baltimore) ; 98(29): e16554, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31335738

RESUMO

BACKGROUND: The benefits of fenestration for patients undergoing Fontan procedure seem controversial at early and late postoperative stages. OBJECTIVE: We aimed to compare the outcomes between the fenestrated and non-fenestrated Fontan procedures. METHODS: Studies comparing the fenestrated and non-fenestrated Fontan procedures were identified by searching the PubMed, EMBASE, and Cochrane Library databases until July 2018. The assessed variables included postoperative oxygen saturation (SaO2), pulmonary artery pressure, mortality, cardiopulmonary bypass (CPB) time, ventilation time, intensive care unit stay, hospital stay, chest tube duration, protein-losing enteropathy, arrhythmia, and other follow-up outcomes including reintervention, stroke/thrombosis, and peak oxygen consumption. A random-effect/fixed-effect model was used to summarize the estimates of the mean difference (MD)/odds ratio (OR) with 95% confidence interval (CI). Subgroup analysis stratified by early and late outcomes was performed. RESULTS: A total of 1929 Fontan patients from 14 studies were included. The early postoperative SaO2 was lower with fenestration than without fenestration (MD -2.52, 95% CI -4.16 to -0.87, P <.05); however, the late postoperative SaO2 showed no difference between the 2 approaches. The CPB time was shorter without fenestration than with fenestration (MD 10.72, 95% CI 2.54-18.9, P <.05); however, the incidence of arrhythmia was lower with fenestration than without fenestration (OR 0.43, 95% CI 0.25-0.75, P <.05). Other variables showed no significant differences between the 2 approaches in Fontan patients. CONCLUSION: Fenestration appears to result in a lower incidence of arrhythmia but with a longer CPB time and lower early SaO2. Other outcomes are comparable between the 2 approaches.


Assuntos
Técnica de Fontan/métodos , Arritmias Cardíacas/etiologia , Ponte Cardiopulmonar , Cuidados Críticos , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Humanos , Tempo de Internação , Oxigênio/sangue , Consumo de Oxigênio , Cuidados Pós-Operatórios , Complicações Pós-Operatórias , Respiração Artificial
4.
World J Pediatr Congenit Heart Surg ; 10(4): 407-413, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31307303

RESUMO

BACKGROUND: Plastic bronchitis is a dreaded complication of single ventricle physiology occurring following palliation via Fontan procedure. Medical management of plastic bronchitis often fails, requiring heart transplantation. Percutaneous lymphatic embolization is an emerging treatment for plastic bronchitis. METHODS: To determine the cost-effectiveness of competing management strategies, a modified Markov model was constructed with patients transiting through treatments-medical management, lymphatic embolization, or heart transplantation from a hospital system perspective. Health state transitions were modeled using an institutional review board-approved retrospective review of the Children's Hospital of Pennsylvania's plastic bronchitis cohort. Medication pricing data were obtained from the National Inpatient Sample. Differences in costs and quality-adjusted life years (QALYs) over a five-year horizon for each group were determined. The incremental cost-effectiveness ratio was then calculated. RESULTS: The mean cost of lymphatic embolization from procedure performance was US$340,941, US$385,841 for heart transplantation, and US$594,520 for medical management. The mean quality-adjusted survival of lymphatic embolization yielded an additional 0.66 QALYs (P < .03) relative to heart transplantation and 1.3 (P < .0001) relative to medical management. Orthotopic heart transplantation yielded an additional 0.66 QALYs (P = .06) when comparing heart transplantation to medical management. Compared to medical management, lymphatic embolization generated an incremental cost-effectiveness ratio of US$192,105. Similarly, compared to heart transplantation, lymphatic embolization yielded an incremental cost-effectiveness ratio of US$68,030. CONCLUSIONS: Of the available plastic bronchitis treatments, with a willingness to pay of US$150,000, lymphatic embolization produces an incremental cost-effectiveness ratio within the bounds considered to be cost-effective, potentially causing financial benefits to the health system.


Assuntos
Bronquite/terapia , Cateterismo Periférico/métodos , Embolização Terapêutica/economia , Técnica de Fontan/efeitos adversos , Vasos Linfáticos/diagnóstico por imagem , Complicações Pós-Operatórias , Bronquite/etiologia , Análise Custo-Benefício , Embolização Terapêutica/métodos , Feminino , Humanos , Linfografia , Estudos Retrospectivos
5.
World J Pediatr Congenit Heart Surg ; 10(4): 416-423, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31307314

RESUMO

BACKGROUND: Investigations of ventricular dominance and outcomes after the Fontan procedure have shown conflicting results. This may be due to the inclusion of multiple modifications of the Fontan or the omission of recently identified complications of the procedure. We examined the association between right ventricular dominance (RVD) and morbidity/mortality in a contemporary cohort following the extracardiac (EC) Fontan. METHODS: We studied all pediatric patients at our center who underwent a predominantly fenestrated EC Fontan from 2004 to 2016. Outcomes assessed were freedom from (1) Fontan failure (death, takedown, listing for transplantation) and (2) complication (arrhythmia requiring medication, postoperative pacemaker, or implantable cardioverter defibrillator requirement, stroke, thrombosis in the Fontan circuit, protein losing enteropathy, plastic bronchitis, New York Heart Association class >2). We defined the perioperative period as occurring before hospital discharge or within 30 days of the Fontan. RESULTS: A total of 137 patients (median age: 34 months, 62% male, 60% RVD) underwent the EC Fontan. Median duration of follow-up was 5.8 years (interquartile range: 2.4-9.0). Freedom from any event was 82.5% (RVD = 77%, LVD = 91%, χ2(1) = 5.03, P = .025) and RVD was associated with reduced event-free survival (hazard ratio: 2.94, P = .02). No confounders were identified. In the perioperative period, RVD was associated with reduced complication-free survival (P = .004). After this period, RVD was associated with reduced failure-free survival (P = .003). CONCLUSIONS: In this contemporary, single-center cohort of EC Fontan patients, RVD was associated with inferior outcomes.


Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Complicações Pós-Operatórias/etiologia , Função Ventricular Direita/fisiologia , Pré-Escolar , Intervalo Livre de Doença , Ecocardiografia , Feminino , Seguimentos , Técnica de Fontan/métodos , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Morbidade/tendências , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/fisiopatologia , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Resultado do Tratamento
6.
Pediatr Cardiol ; 40(6): 1199-1207, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31218373

RESUMO

Right to left (R-L) shunts resulting in cyanosis or systemic embolization occur after the Fontan procedure. The primary modality of diagnosing these is angiography. Successful delineation of these shunts in Fontan patients using selective saline contrast transesophageal echocardiography (SCTEE) may allow for reduced radiation and contrast exposure. We hypothesized that SCTEE could accurately determine the presence, type, and semiquantitative shunt size of R-L shunts in Fontan patients. SCTEE was performed in Fontan patients undergoing angiography for clinical indications. Injections were performed in six sites: mid-Fontan, right and left pulmonary arteries, superior and inferior vena cavae, and innominate vein. R-L shunt size was subjectively graded as 0 = absent, 1 = small, and 2 = medium or large based on echo contrast density in the left atrium. SCTEE was compared to angiography. 33 patients with Fontan were studied with median age 15 years, median weight 50.1 kg, and median O2 saturation of 90% in the R-L shunt group and 95% in the no R-L shunt group. R-L shunt types included intracardiac shunts (ICS), veno-venous collaterals (VVCs), arteriovenous malformations (AVMs), and their combinations. SCTEE versus angiography results were the same for the presence, type, and size of R-L shunts in 79% (26/33). SCTEE identified shunts in 88% (29/33). Angiography identified shunts in 85% (28/33). Neither method missed any medium or large R-L shunts. SCTEE and angiography had similar accuracy. SCTEE accurately detected the presence, type, and size of R-L shunts in most Fontan patients in this study. This can be used to guide targeted angiography, reducing radiation exposure and contrast load.


Assuntos
Angiografia/métodos , Ecocardiografia Transesofagiana/métodos , Técnica de Fontan/efeitos adversos , Átrios do Coração/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Adolescente , Adulto , Cateterismo Cardíaco/métodos , Criança , Pré-Escolar , Cianose/etiologia , Embolização Terapêutica , Feminino , Átrios do Coração/cirurgia , Humanos , Masculino , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia , Adulto Jovem
7.
Pediatr Cardiol ; 40(6): 1208-1216, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31230092

RESUMO

Post-operative length of stay (LOS) is an important metric for both healthcare providers and patients and their families. Predicting LOS is a challenge as it is sensitive to multitudinous patient and system factors. All subjects undergoing a Fontan from 1996-2016 who survived to hospital discharge were included. Details about the pre-operative status, operative conduct, and post-operative course of each patient were obtained. The association between patient characteristics and post-Fontan LOS were determined using stepwise multivariable regression models. Of 320 subjects who underwent a Fontan, 314 (98.1%) survived to hospital discharge. Median age at Fontan was 3.3 years (IQR 2.8, 4.0) and the most common underlying diagnosis was hypoplastic left heart syndrome (106, 33.8%). Median post Fontan LOS was 11 days (IQR 8, 17). Univariable risk factors for longer LOS included number of previous surgeries, post-Glenn LOS, cardiopulmonary bypass time, post-operative chylothorax, and failure to extubate in the operating room (all p < 0.05). In multivariable models, number of previous operations, extubation in the operating room, and postoperative complications predicted LOS (R2 = 0.5185 for full model). The proportion of patients discharged on week days (14.7-18.8% per day) was significantly higher than the proportion discharged on weekend days (5.1-9.9% per weekend day). Pre-operative variables have limited use in predicting post-Fontan length of stay. The most important predictors of post-operative LOS are extubation in the operating room and the occurrence of post-operative complications. However, a significant proportion of variability in LOS was not explained by available measurable variables.


Assuntos
Técnica de Fontan/efeitos adversos , Tempo de Internação/estatística & dados numéricos , Extubação/efeitos adversos , Pré-Escolar , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Masculino , Análise Multivariada , Complicações Pós-Operatórias/epidemiologia , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Risco
8.
Pediatr Cardiol ; 40(5): 1064-1071, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31065760

RESUMO

In 2014, our hospital introduced inhaled nitric oxide (iNO) therapy combined with high-flow nasal cannula (HFNC) oxygen therapy after extubation following the Fontan procedure in patients with unstable hemodynamics. We report the benefits of HFNC-iNO therapy in these patients. This was a single-center, retrospective review of 38 patients who underwent the Fontan procedure between January 2010 and June 2016, and required iNO therapy before extubation. The patients were divided into two groups: patients in Epoch 1 (n = 24) were treated between January 2010 and December 2013, receiving only iNO therapy; patients in Epoch 2 (n = 14) were treated between January 2014 and June 2016, receiving iNO therapy and additional HFNC-iNO therapy after extubation. There were no significant differences between Epoch 1 and 2 regarding preoperative cardiac function, age at surgery, body weight, initial diagnosis (hypoplastic left heart syndrome, 4 vs. 2; total anomalous pulmonary venous return, 5 vs. 4; heterotaxy, 7 vs. 8), intraoperative fluid balance, or central venous pressure upon admission to the intensive care unit. Epoch 2 had a significantly shorter duration of postoperative intubation [7.2 (3.7-49) vs. 3.5 (3.0-4.6) hours, p = 0.033], pleural drainage [23 (13-34) vs. 9.5 (8.3-18) days, p = 0.007], and postoperative hospitalization [36 (29-49) vs. 27 (22-36) days, p = 0.017]. Two patients in Epoch 1 (8.3%), but none in Epoch 2, required re-intubation. Our results suggest that HFNC-iNO therapy reduces the duration of postoperative intubation, pleural drainage, and hospitalization.


Assuntos
Extubação/métodos , Broncodilatadores/administração & dosagem , Técnica de Fontan/efeitos adversos , Óxido Nítrico/administração & dosagem , Cuidados Pós-Operatórios/métodos , Administração por Inalação , Extubação/efeitos adversos , Cânula , Estudos de Casos e Controles , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos
9.
Pediatr Cardiol ; 40(5): 1093-1096, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30982076

RESUMO

We report a case of a 23-year-old male with failing Fontan circulation who was taken to the catheterization lab to better evaluate the Fontan circulation and hemodynamics. Catheterization revealed arteriovenous malformations exclusively present in the right lung leading to the consideration of placing stents to direct the inferior vena cava flow through the Fontan circuit to the right pulmonary artery (RPA), thus increasing the RPA concentration of the hepatic factor. However, comprehensive 4D-Flow MRI analyses indicated sufficient distribution of the hepatic flow between branch pulmonary arteries, and consequently no further invasive intervention to redirect hepatic flow was performed.


Assuntos
Imagem por Ressonância Magnética , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar/fisiologia , Técnica de Fontan/efeitos adversos , Humanos , Masculino , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Adulto Jovem
11.
Pediatr Cardiol ; 40(4): 685-693, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30918992

RESUMO

Sinus node dysfunction (SND) causes significant morbidity in patients after Fontan surgery. Heart rate variability (HRV) reflects the autonomic regulation of the heart, and changes in HRV have been associated with SND in adults. We aimed to study whether changes in HRV could be detected in 24-h electrocardiographic (ECG) recordings in Fontan patients with SND. We compared HRV results from two patient groups; patients with Fontan circulation who later required a pacemaker due to severe SND (n = 12) and patients with Fontan circulation and SND, without indication for pacemaker treatment (n = 11), with two control groups; patients with Fontan circulation without SND (n = 90) and healthy controls (n = 66). The Poincaré plot index SD2 (representing changes in heart rate over 24-h) and the very low-frequency (VLF) HRV component were significantly higher in both SND groups, both compared with healthy controls and patients with Fontan circulation without SND. In SND patients with pacemakers, SD2 and VLF were slightly reduced compared to SND patients without pacemaker (p = 0.06). In conclusion, in Fontan patients with SND the HRV is significantly higher compared to healthy controls and Fontan patients without SND. However, in patients with severe SND requiring pacemaker, SD2 and VLF tended to be lower than in patients with SND without pacemaker, which could indicate a reduced diurnal HRV in addition to the severe bradycardia. This is a small study, but our results indicate that HRV analysis might be a useful method in the follow-up of Fontan patients regarding development of SND.


Assuntos
Arritmias Cardíacas/etiologia , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Frequência Cardíaca/fisiologia , Síndrome do Nó Sinusal/fisiopatologia , Adulto , Arritmias Cardíacas/terapia , Estudos de Casos e Controles , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Marca-Passo Artificial , Complicações Pós-Operatórias
12.
World J Pediatr Congenit Heart Surg ; 10(2): 174-181, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30841839

RESUMO

INTRODUCTION: The Glenn procedure is generally performed as a second-stage palliative procedure toward the completion of Fontan circulation in patients with a functionally univentricular heart. Safe Fontan completion requires normal ventricular function, competent atrioventricular valves, normal pulmonary arteries anatomy, and low pulmonary vascular resistances (PVRs), which is merely an estimation that considers both lungs as a single unit. Clinical observations revealed that patients who previously underwent bilateral bidirectional cavopulmonary anastomosis (b-BCPA) had a stormier postoperative course in respect to other patients with previous unilateral BCPA (u-BCPA) postoperatively after Fontan completion. This retrospective study was designed to compare and analyze the outcomes of patients following Fontan completion with previous u-BCPA versus b-BCPA. PATIENTS AND METHODS: A total of 98 patients were divided into two groups based on the presence or absence of bilateral superior vena cava (SVC). Group 1 included 80 patients with unilateral SVC, and group 2 included 18 patients with bilateral SVC. Univariate and multivariate (SPSS) analyses were performed with regard to mortality and failure. P value of <.05 was considered as significant. RESULTS: Prior to the total cavopulmonary anastomosis operation, the G1 and G2 had similar demographic and physiological findings, ages and weights were 4.3 ± 1.72 years and 15.0 ± 3.71 kg in G1 and 4.8 ± 3.21 years and 17.5 ± 9.76 kg in G2. Mean end-diastolic ventricular pressures (in mm Hg) were 10.50 ± 2.86 in G1 and 10.28 ± 3.07 in G2 and the mean PVRs (in Woods units) were 1.66 ± 1.29 in G1 and 1.49 ± 0.82 in G2. The Stormy postoperative course, Fontan failure, and early mortality were all significantly higher in G2 in respect to G1 ( P value <.05). G1 mortality rate was 3.7%, 3 patients of 80, while in G2, the mortality rate was 22%, 4 patients of 18. Late Fontan failure rates were 6% for G1 patients where two patients developed pulmonary hypertension, two patients developed heart failure, and one patient had protein losing enteropathy (PLE), and 22% for the G2 patients of which two patients developed heart failure, one patient had PLE, and one patient had persistent low systemic venous saturation. CONCLUSIONS: Our results have demonstrated that patients after b-BCPA might have a tendency for worse outcomes upon Fontan completion. The b-BCPA is associated with pulmonary artery bifurcation stenosis and presents multiple sources of pulmonary blood flow where conventional PVR calculation might be imprecise and misleading since it considers the lungs as a single unit. Therefore, b-BCPA engenders correction of the conventional PVR calculation to consider each lung separately. Furthermore, the surgical approach for patients with persistent left SVC and univentricular heart should be modified.


Assuntos
Técnica de Fontan/métodos , Derivação Cardíaca Direita/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Criança , Pré-Escolar , Feminino , Técnica de Fontan/efeitos adversos , Derivação Cardíaca Direita/efeitos adversos , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/cirurgia , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Complicações Pós-Operatórias , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Resistência Vascular , Veia Cava Superior/cirurgia
13.
Pediatr Cardiol ; 40(4): 784-791, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30770935

RESUMO

There are no previous studies on arterial stiffness and its associations with cardiorespiratory fitness in young Fontan patients. Therefore, we examined the arterial stiffness and its relationship to cardiorespiratory fitness in children and young adults with a Fontan circulation. Altogether, 17 Fontan patients and 26 healthy controls (16 females and 27 males aged 8-40 years) participated in this cross-sectional study. The cardiorespiratory fitness was assessed by cardiopulmonary exercise testing on a cycle ergometer and was defined as the standard deviation scores (SDS) of peak oxygen uptake per body mass (VO2peak/kg) based on the national reference values and assessed with cardiopulmonary exercise testing on a cycle ergometer. Aortic pulse wave velocity (PWVao) as a measure of arterial stiffness and aortic Augmentation Index (AIX) as a measure of peripheral arterial tone, were assessed by non-invasive oscillometric device from upper arm. Body adiposity was determined by body mass index SDS and the sport participation by interview. Data were analyzed using linear regression analyses and Pearson's correlations, adjusted for age and sex. Fontan patients had a lower VO2peak/kg-SDS (- 2.69 vs 0.078), higher PWVao-SDS (1.13 vs - 0.24) and higher AIX (19.26% vs 8.49%) in comparison with healthy controls. PWVao and AIX were negatively associated with VO2peak/kg (standard regression coefficient (ß) - 0.525, 95% confidence interval (CI) - 0.722 to - 0.227, p < 0.01 and ß - 0.371, 95% CI - 0.672 to - 0.080, p = 0.014). Young Fontan patients have the arterial stiffness of healthy people who are twice as old. Thereby, children and young adults with a Fontan circulation have a lower cardiorespiratory fitness and less sport participation. Arterial stiffness is inversely associated with cardiorespiratory fitness and exercise training might be an intervention to improve vascular health in this population.


Assuntos
Aorta/fisiopatologia , Aptidão Cardiorrespiratória/fisiologia , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Rigidez Vascular/fisiologia , Adolescente , Adulto , Criança , Estudos Transversais , Teste de Esforço/métodos , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Oscilometria , Análise de Onda de Pulso/métodos , Adulto Jovem
14.
Pediatr Cardiol ; 40(4): 744-752, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30710164

RESUMO

Prolonged pleural drainage is a common complication in patients after Fontan palliation and is associated with short- and long- term morbidities. Among many potential etiologies, prolonged drainage has an inflammatory component, but there are no descriptions of cytokines in Fontan pleural drainage to date. This study aimed to examine the inflammatory make-up of Fontan pleural drainage. This prospective age-range-matched cohort study recruited 25 patients undergoing Fontan procedure and 15 bi-ventricular patients undergoing cardiopulmonary bypass (CPB). Chest tube samples were taken on postoperative day (POD) 1-4, 7, and 10. Cytokines were measured using Bio-Plex Assays. Univariate comparisons were made in patient characteristics and cytokine levels. Median age was 3.7 y (IQR 2.8-3.9) for controls and 2.5 y (IQR 2.1-2.9) in Fontan patients (p = 0.02). Median drainage duration and daily volume was higher in Fontan patients (both p < 0.001). Inflammatory cytokines (IL-17A, IFN-y, MIP-1ß, and TNF-α) were higher in Fontan patients than controls (all p < 0.02). There was an increase in pro-inflammatory cytokines (IL-8, MIP-1ß, and TNF-α) from POD1 to the last chest tube day (LCD) in Fontan patients (all p < 0.0001) and a decrease in the anti-inflammatory cytokine IL-10 (p = 0.001). There was no difference in cytokine concentration from POD1 to LCD among controls. There was a significant association with the cytokine concentration of TNF-α on POD1 and duration of chest tube drainage (p < 0.05). Inflammatory cytokine levels in the pleural fluid of Fontan patients are higher compared to bi-ventricular controls and rise over time where controls do not. This suggests ongoing localized inflammation that is not a result of CPB alone and may be an important contributor to pleural drainage in patients after the Fontan procedure.


Assuntos
Técnica de Fontan/efeitos adversos , Interleucinas/análise , Derrame Pleural/metabolismo , Complicações Pós-Operatórias/metabolismo , Ponte Cardiopulmonar/efeitos adversos , Estudos de Casos e Controles , Quimiocina CCL3/análise , Tubos Torácicos , Pré-Escolar , Citocinas , Drenagem , Feminino , Humanos , Tempo de Internação , Masculino , Proteínas Quimioatraentes de Monócitos/análise , Derrame Pleural/etiologia , Derrame Pleural/fisiopatologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/fisiopatologia , Período Pós-Operatório , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento , Fator de Necrose Tumoral alfa/análise
15.
Asian Cardiovasc Thorac Ann ; 27(3): 172-179, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30789306

RESUMO

BACKGROUND: This study aimed to describe the learning curve of the extracardiac Fontan procedure in a single center and to analyze the changes in clinical applications and outcomes. METHODS: A retrospective chart review of all extracardiac Fontan patients in a single tertiary care center was undertaken. Patients with a diagnosis of hypoplastic left heart syndrome and those who had undergone a lateral tunnel modification, intra/extracardiac Fontan, Kawashima procedure, or inferior vena cava-to-azygous vein connection were excluded from the analysis. RESULTS: Between May 2004 and February 2018, data of 159 extracardiac Fontan patients were analyzed. The median age was 5.5 years (range 4.5-8.2 years). Based on a cumulative sum analysis, a hinge point was determined to divide the cohort into 2 phases. Phase 1 ( n = 70) represented the first learning phase and phase 2 ( n = 89) represented the later phase. Mortality decreased in phase 2 (2/89; 2%) compared to phase 1 (10/70; 14%; p = 0.004). Two (3%) patients had extracorporeal membrane oxygenation in phase 1, and 5 (6%) in phase 2 ( p = 0.47). More patients in phase 2 underwent a prior bidirectional Glenn procedure (83/89 vs. 57/70; p = 0.02), fenestration (80/89 vs. 9/70; p < 0.001), and pulmonary artery reconstruction (37/89 vs. 2/70; p < 001). CONCLUSIONS: This study shows that increased use of extracorporeal membrane oxygenation, strict implementation of the three-stage management plan, routine fenestration, and a low threshold for pulmonary artery reconstruction may be associated with decreased mortality in the extracardiac Fontan procedure.


Assuntos
Competência Clínica , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Curva de Aprendizado , Cirurgiões , Criança , Pré-Escolar , Oxigenação por Membrana Extracorpórea , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Turquia
16.
Pediatr Cardiol ; 40(4): 753-761, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30671646

RESUMO

Isomerism, or heterotaxy, impacts morbidity and mortality after various stages of univentricular palliation. Timing of Fontan completing in these patients based on preoperative factors has not been investigated previously. The aim of this study was to determine the impact of preoperative factors on various outcomes including length of hospital stay and duration of chest tubes. A cross-sectional study was conducted. Patients with isomerism having undergone Fontan at the Children's Hospital of Wisconsin between 1998 and 2014 were identified. Preoperative, operative, and postoperative data were collected on these patients. Linear regression analysis was conducted to determine preoperative characteristics associated with various postoperative outcomes. Receiver operator curve analysis was also performed to determine the sensitivity and specificity of age and pre-Fontan arterial oxygen saturation in predicting increased length of hospitalization and increased duration of chest tubes. Younger age and lower pre-Fontan arterial oxygen saturation were associated with increased length of hospitalization while younger age, lower pre-Fontan arterial oxygen saturation, interrupted inferior caval vein, and worse pre-Fontan atrioventricular valve regurgitation were associated with increased length of chest tubes. Age, arterial oxygen saturation, pulmonary arteriovenous malformations, interruption of the inferior caval vein, and atrioventricular valve regurgitation should all be taken into consideration when timing Fontan completion in patients with isomerism. Arterial oxygen saturations between 82 and 84% with an approximate age of 3 years appear to be a time at which it is reasonable to consider Fontan in patients with isomerism.


Assuntos
Peso Corporal/fisiologia , Técnica de Fontan/efeitos adversos , Síndrome de Heterotaxia/cirurgia , Oxigênio/sangue , Adolescente , Fatores Etários , Tubos Torácicos/estatística & dados numéricos , Criança , Pré-Escolar , Estudos Transversais , Técnica de Fontan/métodos , Técnica de Fontan/mortalidade , Síndrome de Heterotaxia/complicações , Síndrome de Heterotaxia/mortalidade , Humanos , Lactente , Tempo de Internação/estatística & dados numéricos , Oximetria , Curva ROC , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Wisconsin
17.
J Cardiovasc Magn Reson ; 20(1): 85, 2018 12 17.
Artigo em Inglês | MEDLINE | ID: mdl-30558626

RESUMO

BACKGROUND: Maldistribution of pulmonary artery blood flow (MPBF) is a potential complication in patients who have undergone single ventricle palliation culminating in the Fontan procedure. Cardiovascular magnetic resonance (CMR) is the best modality that can evaluate MPBF in this population. The purpose of this study is to identify the prevalence and associations of MPBF and to determine the impact of MPBF on exercise capacity after the Fontan operation. METHODS: This retrospective single-center study included all patients after Fontan operation who had maximal cardiopulmonary exercise test (CPET) and CMR with flow measurements of the branch pulmonary arteries. MPBF was defined as > 20% difference in branch pulmonary artery flow. Exercise capacity was measured as percent of predicted oxygen consumption at peak exercise (% predicted VO2). Linear and logistic regression models were used to determine univariate and multivariable predictors of exercise capacity and correlates of MPBF, respectively. RESULTS: A total of 147 patients who had CMR between 1999 and 2017 were included (median age at CMR 21.8 years [interquartile range (IQR) 16.5-30.6]) and the median time between CMR and CPET was 2.8 months [IQR 0-13.8]. Fifty-three patients (36%) had MPBF (95% CI 29-45%). The mean % predicted VO2 was 63 ± 16%. Patients with MPBF had lower mean % predicted VO2 compared to patients without MPBF (60 ± 14% versus 65 ± 16%, p = 0.04). On multivariable analysis, a lower % predicted VO2 was independently associated with longer time since Fontan, higher ventricular mass-to-volume ratio, and MPBF. On multivariable analysis, only compression of the branch pulmonary arteries by the ascending aorta or aortic root was associated with MPBF (OR 6.5, 95% CI 5.6-7.4, p < 0.001). CONCLUSION: In patients after the Fontan operation, MPBF is common and is independently associated with lower exercise capacity. MPBF was most likely to be caused by pulmonary artery compression by the aortic root or the ascending aorta. This study identifies MPBF as an important risk factor and as a potential target for therapeutic interventions in this fragile patient population.


Assuntos
Tolerância ao Exercício , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar , Estenose de Artéria Pulmonar/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Teste de Esforço , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Imagem por Ressonância Magnética , Masculino , Consumo de Oxigênio , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Risco , Estenose de Artéria Pulmonar/diagnóstico por imagem , Estenose de Artéria Pulmonar/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
18.
World J Pediatr Congenit Heart Surg ; 9(5): 504-508, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30157726

RESUMO

BACKGROUND: Although aspirin has been compared to warfarin for thromboembolic prophylaxis in the general Fontan population, little is known about the optimal preventative strategy for the atriopulmonary right atrium-pulmonary artery [RA-PA]) Fontan particularly. METHODS: A retrospective cohort study was performed including adult patients identified in the Ahmanson/UCLA Adult Congenital Heart Disease Center database with a history of RA-PA Fontan and use of either aspirin or warfarin as most recent primary prophylaxis against thromboembolism. Primary outcome was incident thromboembolism, defined as space-occupying lesion on imaging consistent with thrombus within the Fontan or pulmonary arterial circuit. Secondary outcomes were death, transplantation, Fontan conversion, and bleeding requiring either transfusion or invasive intervention. Follow-up was terminated upon achievement of a primary outcome or achievement of a secondary outcome other than bleeding. Kaplan-Meier analysis of freedom from thrombosis was performed. RESULTS: Twenty-six patients met inclusion criteria. Thirteen (50%) received aspirin as most recent primary prophylaxis and 13 (50%) received warfarin. Tricuspid atresia was the most common underlying diagnosis (42%), followed by double-inlet left ventricle (38%). Median age at Fontan operation was 8.2 years; median age at prophylaxis initiation was 25.9 years. After six years, the aspirin group had 50% ± 35% freedom from thrombosis and the warfarin group 92% ± 8% ( P = .15). Incidences of secondary outcomes were not significantly different between the groups. CONCLUSION: In this cohort of long-term Fontan survivors with RA-PA Fontan, the risk of thromboembolic complications is high, especially in those taking aspirin rather than warfarin. Larger studies are needed to confirm these findings.


Assuntos
Anticoagulantes/uso terapêutico , Técnica de Fontan/efeitos adversos , Átrios do Coração/cirurgia , Cardiopatias Congênitas/cirurgia , Prevenção Primária/métodos , Artéria Pulmonar/cirurgia , Tromboembolia/prevenção & controle , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Técnica de Fontan/métodos , Átrios do Coração/anormalidades , Humanos , Incidência , Lactente , Masculino , Artéria Pulmonar/anormalidades , Estudos Retrospectivos , Tromboembolia/epidemiologia , Tromboembolia/etiologia , Resultado do Tratamento , Adulto Jovem
19.
Int Heart J ; 59(5): 1008-1014, 2018 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-30158375

RESUMO

The aim of this study was to evaluate hepatic dysfunction over 10 years following Fontan surgery. We assessed the clinical usefulness of diagnostic tools for the detection and follow-up of hepatic dysfunction in patients with Fontan circulation.A total of 26 post-Fontan patients (median age 13 years, range 10-35 years; median duration from Fontan procedure 10.5 years, range 4-17 years) were enrolled in this study. Hepatic assessment was performed by ultrasonography, computed tomography (CT), and transient elastography (TE) with biochemical tests, echocardiography, and cardiac catheterization. Related parameters were compared on the basis of different findings in liver sonography, CT, and TE.Liver CT and TE showed abnormal findings in all patients. Liver ultrasonography revealed abnormal results in 24 patients (92.3%). However, liver function test was normal and did not correlate with imaging studies. C-reactive protein was significantly correlated with severity of CT findings. White blood cell, platelet count, and N-terminal pro-brain natriuretic peptide were correlated with severity on TE. Post-Fontan high pulmonary vascular resistance (P = 0.046) and high mean pulmonary artery pressure (P = 0.046) correlated with hepatic changes on liver CT.Changes in the liver post-Fontan surgery are common and occur even after 10 years the procedure. Liver imaging is more sensitive, and CT seems to be more useful for differentiation of severe hepatic changes.


Assuntos
Técnica de Fontan/efeitos adversos , Hepatopatias/diagnóstico por imagem , Fígado/diagnóstico por imagem , Adolescente , Adulto , Fator Natriurético Atrial/sangue , Proteína C-Reativa/metabolismo , Criança , Pré-Escolar , Diagnóstico Precoce , Técnicas de Imagem por Elasticidade/métodos , Humanos , Fígado/química , Fígado/enzimologia , Fígado/patologia , Hepatopatias/sangue , Hepatopatias/etiologia , Hepatopatias/patologia , Testes de Função Hepática/métodos , Precursores de Proteínas/sangue , Artéria Pulmonar/fisiologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X/métodos , Ultrassonografia/métodos , Resistência Vascular/fisiologia , Adulto Jovem
20.
Cardiol Young ; 28(8): 1062-1066, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29972113

RESUMO

This report describes the first clinical experience with the new Occlutech Atrial Flow Regulator (AFR®) device for implementation of a late Fontan fenestration. The AFR® device secured a defined diameter of the fenestration without spontaneous re-occlusion of this extra-anatomic perforation. This ensured a permanent clinical improvement of our failing Fontan patient.


Assuntos
Cateterismo Cardíaco/instrumentação , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Hipertensão Pulmonar/complicações , Adulto , Humanos , Imagem por Ressonância Magnética , Masculino , Desenho de Prótese , Recidiva
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