Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.120
Filtrar
1.
Ann Agric Environ Med ; 27(3): 384-387, 2020 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-32955219

RESUMO

INTRODUCTION AND OBJECTIVE: Single ventricle anomaly is one of the complex congenital heart defects. A dependable non-invasive method of evaluation of Fontan circulation haemodynamics for early diagnosing unstable patients is hardly available in routine clinical practice. The aim of the study is non-invasive evaluation of the haemodynamic parameters in patients after Fontan operation. MATERIAL AND METHODS: The study involved 11 participants (age 24.4±4.3 years) with functionally univentricular hearts after Fontan operation. Evaluation of haemodynamic parameters was performed in supine and sitting positions using the impedance cardiography method. RESULTS: In comparative analysis, heart rate (70.1 vs.78.3 1/min; p=0.001), diastolic blood pressure (73.9 vs. 76.7 mm Hg; p=0.026), mean arterial blood pressure (84.5 vs. 88.0 mm Hg; p=0.013), systemic vascular resistance (1284.8 vs. 1334.9 dyn*s*cm-5; p=0.024), systemic vascular resistance index (2178.7 vs. 2272.8 dyn*s*cm-5*m2 ; p=0.018), pre-ejection period (124.2 vs. 136.2 ms; p=0.009), systolic time ratio (0.43 vs. 0.53; p=0.0001), and Zo (26.2 vs. 28.7 Ω; p<0.00001), were significantly higher in the sitting position. Stroke volume (75.4 vs. 68.5 ml; p=0.013), stroke index (42.7 vs. 39.0 ml*m-2; p=0.014), thoracic fluid content (38.5 vs. 35.4 1*kΩ-1; p=<0.00001), thoracic fluid content index (22.8 vs. 21.0 1*kΩ-1*m-2; p=<0.00001), and leftventricular ejection time 291.1 vs. 260.1 ms; p <0.00001, were significantly higher in the supine position. CONCLUSIONS: In patients after Fontan procedure, impedance cardiography can be a useful tool the assessment of shortterm haemodynamic changes provoked by postural changes. Its clinical value in patients with congenital heart defects should be further investigated.


Assuntos
Pressão Sanguínea , Técnica de Fontan/efeitos adversos , Frequência Cardíaca , Resistência Vascular , Adulto , Cardiografia de Impedância , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Humanos , Polônia , Adulto Jovem
2.
Int Heart J ; 61(4): 851-855, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32728004

RESUMO

Protein-losing enteropathy (PLE) is one of the major complications after a Fontan operation. Some PLE patients suffer from concurrent gastrointestinal bleeding. An effective treatment regimen for such patients has not been established yet. Further, it remains unknown whether PLE and gastrointestinal bleeding coexist independently, or protein losing is associated with gastrointestinal bleeding. We report a 7-year-old steroid-refractory post-Fontan PLE case suggesting the latter pathogenesis together with a literature review.


Assuntos
Técnica de Fontan/efeitos adversos , Hemorragia Gastrointestinal/complicações , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Enteropatias Perdedoras de Proteínas/etiologia , Administração Oral , Anemia Refratária/etiologia , Cateterismo Cardíaco/métodos , Criança , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Hipoalbuminemia/etiologia , Masculino , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Remissão Espontânea , Resultado do Tratamento
3.
Int J Cardiovasc Imaging ; 36(9): 1667-1677, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32363447

RESUMO

The principal aim of this study was to evaluate changes in systolic function in the single right ventricle (SRV), during progression of the same patient through the three stages of surgical repair for hypoplastic left heart syndrome and during a 5-year follow-up. We hypothesize that, SRV global longitudinal strain (GLS) will be low during 3 stages of repair even in stable patients. We retrospectively evaluated 140 echocardiograms in 20 patients with HLHS (ages 0-11.3 years), before and after 3 stages of surgical palliation. Five-year follow-up data were available in all 20 patients. Controls with structurally normal hearts and in the same age group were used for comparison. We utilized speckle-tracking imaging for assessment of SRV segmental and global longitudinal and circumferential strains, from previously acquired 4-chamber and mid-cavity short-axis views prior to and within 1-3 months of each surgical stage. Longitudinal strain (LS) remained low through all 3 stages of repair and during follow-up. The pre-Fontan stage demonstrated significant interstage improvement compared to the post-Glenn stage despite similar volume status. Global LS was (- 15.6 ± 4.5% after Fontan surgery and remained similar (- 15.32 ± 3.2%) 5 years later. The SRV also showed increased dominance of circumferential strain compared to the normal RV, where the longitudinal deformation was dominant. In SRV, longitudinal strain may be a useful clinical index for evaluating both segmental and global function in an objective manner. Due to lack of significant clinical deterioration over a 10-year period, we speculate that a "lower-than-normal" longitudinal strain may be used as an objective measure of SRV function in clinically stable patients, particularly after the Fontan operation. Compensatory mechanisms where the longitudinal pattern of contraction switches to a more circumferential pattern, may play a role in asymptomatic patients with HLHS.


Assuntos
Ecocardiografia , Técnica de Fontan , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Contração Miocárdica , Cuidados Paliativos , Função Ventricular Direita , Criança , Pré-Escolar , Feminino , Técnica de Fontan/efeitos adversos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Lactente , Recém-Nascido , Masculino , Variações Dependentes do Observador , Projetos Piloto , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
5.
BMC Surg ; 20(1): 80, 2020 Apr 21.
Artigo em Inglês | MEDLINE | ID: mdl-32316959

RESUMO

BACKGROUND: The Fontan procedure has become the standard operation for patients with single ventricle physiology. Due to cardiac hypokinesis and high central venous pressure, laparoscopic approach, especially in hepatectomy, was considered as controversial after the Fontan procedure. We presented a case of hepatocellular carcinoma (HCC) that was successfully treated by pure laparoscopic hepatectomy with stable pneumoperitoneum after the Fontan procedure. CASE PRESENTATION: An 18-year-old man was referred to our hospital for examination of a hepatic tumor. The patient underwent the Fontan procedure for single ventricle physiology at 6 years of age. Abdominal contrast-enhanced computed tomography (CT) revealed a hypovascular mass in segment 2 and a hypervascular mass in segment 4 of the arterial phase, followed by a delayed washout. CT arteriography revealed that both masses showed hypervascular tumors, and CT during arterial portography showed that both were low-density masses. The patient's general condition was good, and cardiac and respiratory functions were well maintained. Pure laparoscopic hepatectomy was safely performed by keeping the pneumoteritoneum pressure under 6-8 mmHg and monitoring central venous pressure (11-21 mmHg) and end-tidal carbon dioxide. The Pringle maneuver was applied during hepatic resection. The non-anatomical resections were completed without intraoperative complications. The patient was discharged on the 9th postoperative day without postoperative complications. CONCLUSIONS: Our report suggests that treatment of HCC by pure laparoscopic hepatectomy after Fontan circulation can be safely performed in patients under sufficient circulatory management.


Assuntos
Carcinoma Hepatocelular/cirurgia , Técnica de Fontan/efeitos adversos , Neoplasias Hepáticas/cirurgia , Adolescente , Hepatectomia/métodos , Humanos , Laparoscopia/métodos , Masculino , Pneumoperitônio Artificial , Tomografia Computadorizada por Raios X
7.
Artigo em Inglês | MEDLINE | ID: mdl-32167782

RESUMO

The Fontan circulation is characterized as a nonpulsatile flow propagation without a pressure-generating ventricle. However, flow through the Fontan circulation still exhibits oscillatory waves as a result of pressure changes generated by the systemic single ventricle. Identification of discrete flow patterns through the Fontan circuit may be important to understand single ventricle performance. Ninety-seven patients with Fontan circulation underwent phase-contrast MRI of the right pulmonary artery, yielding subject-specific flow waveforms. Principal component (PC) analysis was performed on preprocessed flow waveforms. Principal components were then correlated with standard MRI indices of function, volume, and aortopulmonary collateral flow. The first principal component (PC) described systolic versus diastolic-dominant flow through the Fontan circulation, accounting for 31.3% of the variance in all waveforms. The first PC correlated with end-diastolic volume (R = 0.34, P = 0.001), and end-systolic volume (R = 0.30, P = 0.003), cardiac index (R = 0.51, P < 0.001), and the amount of aortopulmonary collateral flow (R = 0.25, P = 0.027)-lower ventricular volumes and a smaller volume of collateral flow-were associated with diastolic-dominant cavopulmonary flow. The second PC accounted for 19.5% of variance and described late diastolic acceleration versus deceleration and correlated with ejection fraction-diastolic deceleration was associated with higher ejection fraction. Principal components describing the diastolic flow variations in pulmonary arteries are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to preserved ventricular volume and function, respectively.NEW & NOTEWORTHY The exact physiological significance of flow oscillations of phasic and temporal flow variations in Fontan circulation is unknown. With the use of principal component analysis, we discovered that flow variations in the right pulmonary artery of Fontan patients are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to more ideal ventricular volume and systolic function, respectively.


Assuntos
Técnica de Fontan/efeitos adversos , Ventrículos do Coração/fisiopatologia , Hemodinâmica , Modelos Cardiovasculares , Complicações Pós-Operatórias/fisiopatologia , Artéria Pulmonar/fisiopatologia , Adolescente , Criança , Circulação Coronária , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Masculino , Contração Miocárdica , Modelagem Computacional Específica para o Paciente , Complicações Pós-Operatórias/diagnóstico por imagem , Análise de Componente Principal , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia
8.
Pediatr Cardiol ; 41(5): 905-909, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32125444

RESUMO

This investigation analyzed the rate of hepatic fibrosis progression in post-Fontan patients that underwent hepatic biopsy. The study cohort comprised post-Fontan patients that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2019. We identified 126 patients that met inclusion criteria. Of the 126, 27 (21%) had a lateral tunnel Fontan, and 99 (79%) had an extracardiac Fontan. For the 27 lateral tunnel Fontan patients, age at Fontan was 4 ± 2 years, and for the 99 extracardiac Fontan patients age at Fontan was 4 ± 2 years (p = 0.98). For the 27 lateral tunnel Fontan patients, the average total fibrosis score was 3.0 ± 1.5; and for the 99 extracardiac Fontan patients, the average total fibrosis was 2.7 ± 1.7 (p = 0.48). For the lateral tunnel Fontan patients, the average Fontan duration was 20 ± 6 years; and for the 99 extracardiac Fontan patients, the average Fontan duration was 11 ± 5 years (p < 0.001). For the 27 lateral tunnel Fontan patients, the average rate of fibrosis progression was 0.16 ± 0.10 total fibrosis score/year; and for the 99 extracardiac Fontan patients, the average rate of fibrosis progression was 0.30 ± 0.23 total fibrosis score/year (p < 0.001). In conclusion, our findings suggest that those with extracardiac Fontans have a faster rate of hepatic fibrosis progression than those with lateral tunnel Fontans. More extensive or multi-institutional studies will be needed to confirm these findings and define the clinical significance of discrepant rates of hepatic fibrosis in post-Fontan patients.


Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Cirrose Hepática/epidemiologia , Adolescente , Adulto , Biópsia , Cateterismo Cardíaco , Criança , Pré-Escolar , Progressão da Doença , Feminino , Seguimentos , Técnica de Fontan/efeitos adversos , Humanos , Fígado/patologia , Cirrose Hepática/etiologia , Masculino , Adulto Jovem
9.
Pediatr Cardiol ; 41(4): 736-746, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32006084

RESUMO

Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). In this study, we performed a detailed hemodynamic and hepatic assessment to analyze the incidence and spectrum of FALD and its association with patients' hemodynamics. From 2017 to 2019, 145 patients underwent a detailed, age-adjusted hepatic examination including laboratory analysis (FibroTest®, n = 101), liver ultrasound (n = 117) and transient elastography (FibroScan®, n = 61). The median patient age was 16.0 years [IQR 14.2], and the median duration of the Fontan circulation was 10.3 years [IQR 14.7]. Hemodynamic assessment was performed using echocardiography, cardiopulmonary exercise capacity testing and cardiac catheterization. Liver ultrasound revealed hepatic parenchymal changes in 83 patients (70.9%). Severe liver cirrhosis was detectable in 20 patients (17.1%). Median liver stiffness measured by FibroScan® was 27.7 kPa [IQR 14.5], and the median Fibrotest® score was 0.5 [IQR 0.3], corresponding to fibrosis stage ≥ 2. Liver stiffness values and Fibrotest® scores correlated significantly with Fontan duration (P1 = 0.013, P2 = 0.012). Exercise performance was significantly impaired in patients with severe liver cirrhosis (P = 0.003). Pulmonary artery pressure and end-diastolic pressure were highly elevated in cirrhotic patients (P1 = 0.008, P2 = 0.003). Multivariable risk factor analysis revealed Fontan duration to be a major risk factor for the development of FALD (P < 0.001, OR 0.77, CI 0.68-0.87). In the majority of patients, hepatic abnormalities suggestive of FALD were detectable by liver ultrasound, transient elastography and laboratory analysis. The severity of FALD correlated significantly with Fontan duration and impaired Fontan hemodynamics. A detailed hepatic assessment is indispensable for long-term surveillance of Fontan patients.


Assuntos
Técnica de Fontan/efeitos adversos , Cirrose Hepática/etiologia , Adulto , Cateterismo Cardíaco/efeitos adversos , Pré-Escolar , Estudos Transversais , Ecocardiografia , Técnicas de Imagem por Elasticidade , Feminino , Hemodinâmica , Humanos , Cirrose Hepática/diagnóstico por imagem , Cirrose Hepática/patologia , Masculino , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Ultrassonografia
10.
Pediatr Cardiol ; 41(4): 789-794, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32016581

RESUMO

Single ventricle congenital heart defect patients have improved survival with Fontan palliation. However, they remain at risk for nephropathy, as indicated by pathologic microalbuminuria. We sought to investigate whether echocardiographic measures of the inferior vena cava diameter (a surrogate for elevated CVP) indexed to the body surface area (iIVC) or cardiac index (CI) can predict the presence of nephropathy in Fontan patients. We performed a single-center case-control study, including 39 asymptomatic Fontan (age 14.8 ± 7.9 years) and 29 healthy controls (age 12.7 ± 2.7 years). The primary outcome was abnormal microalbumin-creatinine ratio (MCR) from the first-morning void urine in Fontan patients. Measurements of iIVC and CI were derived using transthoracic echocardiography by two investigators with a high intra-class correlation coefficient (ICC = 0.97). Group comparison between Fontan and controls as well as between Fontan with normal and abnormal MCR was performed using Fisher's exact and t tests. Pearson and Spearman's correlations and multivariate regressions were performed to analyze the relations between the MCR, iIVC, and CI. Abnormal MCR was noted in 13/39(33%) of Fontan patients. The mean iIVC was larger in the Fontan compared to controls (p < 0.0001) and in Fontan with abnormal MCR compared to those with normal MCR (p = 0.0006). A positive correlation (r = 0.62; p < 0.001) was noted between MCR and iIVC. All patients with abnormal MCR had the iIVC > 1 cm/m2. There were no significant relations between the CI and MCR. Significant prevalence of nephropathy late after Fontan palliation warrents screening. Echocardiographic measurement of iIVC could serve as one of the screening measures. The finding of the iIVC diameter > 1 cm/m2 should prompt further renal evaluation.


Assuntos
Técnica de Fontan/efeitos adversos , Nefropatias/diagnóstico , Coração Univentricular/cirurgia , Veia Cava Inferior/patologia , Estudos de Casos e Controles , Pré-Escolar , Estudos Transversais , Ecocardiografia , Feminino , Humanos , Nefropatias/etiologia , Masculino , Veia Cava Inferior/diagnóstico por imagem
11.
Semin Thorac Cardiovasc Surg ; 32(3): 522-528, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32087242

RESUMO

Despite improvements in operative and perioperative care, the risk of significant morbidity and mortality for children undergoing Fontan procedures persists. Previous investigations have identified peri-Fontan characteristics that may predict early adverse events. The purpose of this study was to identify characteristics from throughout a patient's lifespan, including all perioperative stages, that might predict early Fontan failure-defined as death, Fontan takedown, or listing for cardiac transplantation before hospital discharge or within 30 postoperative days. A single-center retrospective study of all patients undergoing a Fontan procedure was performed. Patient and intervention-related characteristics were examined from birth through Fontan. Data were described using standard summary statistics. Univariable, logistic regression was used to examine associations with early Fontan failure. In total, 191 patients met inclusion criteria. The incidence of early Fontan failure was 4% (n = 8: 6 deaths, 2 Fontan takedowns). Neonatal balloon atrial septostomy was the only patient characteristic significantly associated with Fontan failure. Patients who underwent balloon septostomy had 8.5 times higher odds of Fontan failure (confidence interval (CI) 2.6-28.1, P < 0.001) than those who did not. Children who require balloon septostomy as neonates remain at higher risk of Fontan takedown, listing for heart transplantation, or death in the early post-Fontan period.


Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Pré-Escolar , Feminino , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Falha de Tratamento
12.
Cardiovasc Eng Technol ; 11(3): 268-282, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32072439

RESUMO

PURPOSE: Additional pulmonary blood flow (APBF) can provide better pulsating blood flow and systemic arterial oxygen saturation, while low blood pulsation and low oxygen saturation are defects of the Fontan and Glenn procedure. Studying the hemodynamic effect of APBF is beneficial for clinical decisions. This study aimed to explore the effect on particle washout, as well as the differences among the sensitivities of both different hemodynamic parameters and different procedures to APBF. METHODS: The patient-specific clinical datasets of a patient who underwent bilateral bidirectional Glenn (BBDG) with APBF were enrolled in this study, and using these datasets, Glenn- and Fontan-type artery models were reconstructed. A series of parameters, including the total caval flow pulsatility index (TCPI), indexed energy loss (iPL), wall shear stress (WSS), systemic arterial oxygen saturation (Satart), particle washout time (WOT), pressure in the right superior vena cava (PRSVC), pulmonary flow distribution (PFD) and hepatic flow distribution (HFD), were computed from computational fluid dynamic (CFD) simulation to evaluate the hemodynamic effect of APBF. RESULTS: The result showed that APBF led to better iPL and Satart but worse PRSVC and heart load accompanied by a great impact on HFD, making hepatic flow easier to perfuse the side without MPA and APBF. The increase in the APBF rate also effectively results in larger flow pulsation, region velocity, and wall shear stress and lower WOT, and this effect may be more effective for patients with persistent left superior vena cava (PLSVC). However, APBF might have little effect on PFD. Furthermore, APBF might affect WOT, iPL and HFD more significantly than PRSVC and has a greater improvement effect in patients with poorer iPL and WOT. CONCLUSIONS: Moderate APBF is not only a measure to promote pulmonary artery growth and systemic arterial oxygen saturation but also an effective method against endothelial dysfunction and thrombosis. However, moderate APBF is patient-specific and should be determined based on hemodynamic preference that leads to desired patient outcomes, and care should be taken to prevent PRSVC and heart load from being too high as well as an imbalance in HFD.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Hemodinâmica , Artéria Pulmonar/cirurgia , Circulação Pulmonar , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Artéria Pulmonar/fisiopatologia , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
13.
Pediatr Cardiol ; 41(3): 642-649, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32006081

RESUMO

Exercise performance declines as patients who have undergone Fontan operation enter adolescence. However, the effect of altitude on functional capacity after Fontan remains inadequately studied. Our aim was to describe exercise performance in a cohort of patients with Fontan physiology living at increased altitude and compare to a normal control group and relate these data to invasively derived hemodynamics. We hypothesized that peak oxygen consumption ([Formula: see text]) would be decreased, in association with elevated mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVRi). Patients were evaluated in a multidisciplinary clinic for patients with Fontan physiology. Evaluation included cardiopulmonary exercise test and cardiac catheterization at predetermined intervals. Descriptive statistics were calculated. Associations of catheterization and exercise testing measures with [Formula: see text] were estimated with Spearman correlation coefficients. One hundred patients with age- and gender-matched controls were included in the analysis. The mean age was 13.3 ± 3.9 years, with mean weight of 47.1 ± 18.4 kg. The mean [Formula: see text] was 29.0 ± 7.8 ml/kg/min, significantly lower than the control group, 40.2 ± 8.4 ml/kg/min (p < 0.0001). There was no statistically significant linear correlation between [Formula: see text] and mPAP or PVRi. We characterized exercise performance in a large cohort with Fontan physiology living at increased altitude and showed a decrease in [Formula: see text] compared to controls. Our data do not support the hypothesis that moderately increased altitude has a detrimental effect on exercise performance, nor is there a substantial link between poor cavopulmonary hemodynamics and exercise in this setting.


Assuntos
Altitude , Exercício Físico/fisiologia , Técnica de Fontan/efeitos adversos , Adolescente , Estudos de Casos e Controles , Criança , Estudos Transversais , Feminino , Cardiopatias Congênitas/cirurgia , Hemodinâmica/fisiologia , Humanos , Masculino , Estudos Retrospectivos
14.
J Thorac Cardiovasc Surg ; 159(4): 1480-1491.e2, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31928823

RESUMO

OBJECTIVES: The study aim was to compare Fontan patients undergoing lateral tunnel (LT) versus extracardiac conduit (ECC) technique. METHODS: Fontan patients (LT vs ECC) from January 2000 to December 2017 were analyzed retrospectively. Baseline characteristics were analyzed as covariates. Primary outcomes (ie, mortality, Fontan failure, thrombosis, and pacemaker implantation) were compared using time-to-event models. Subgroup analysis including only initially fenestrated cases and propensity score matching were performed. RESULTS: Eight hundred one Fontan patients: LT (n = 638) versus ECC (n = 163) were included. Median follow-up time was 4.8 years (range, 1.1-10.8 years). Baseline characteristics were similar except for age: LT versus ECC: 2.6 years (range, 2.2-3.2 years) versus 3.1 years (range, 2.6-4.7 years) (P < .01) and mean pulmonary artery pressure: LT versus ECC: 12 mm Hg (11-15 mm Hg) versus 11 mm Hg (10-13 mm Hg) (P < .05). Early mortality was significantly higher in ECC versus LT group (3.1%; vs 0.5%; P < .05). Freedom from death, heart transplantation and Fontan failure were significantly longer in LT vs ECC (P < .01). After correcting for age, diagnosis, surgical technique, surgeon, mean pulmonary artery pressure, and fenestration, the ECC group showed worse freedom from death (hazard ratio, 2.8; P < .01) and Fontan failure (hazard ratio, 3.0; P < .01). No difference in pacemaker implantation rate was demonstrated (P = .25). Early fenestration closure was associated with higher risk of early (hazard ratio, 30.5) and late mortality (hazard ratio, 3.5). After matching, log-rank tests showed significant differences between the 2 groups for Fontan failure at 5 and 10 years (P < .01) and mortality at 5 years (P = .02). CONCLUSIONS: When compared with ECC, LT Fontan is associated with better short and midterm outcomes. Spontaneous fenestration closure is an independent risk factor for early/late mortality.


Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias/epidemiologia , Criança , Pré-Escolar , Feminino , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/diagnóstico , Humanos , Masculino , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento
15.
Medicine (Baltimore) ; 99(4): e18986, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31977915

RESUMO

RATIONALE: Anesthetic management of pregnant women with Fontan circulation remains challenging. There are few reports that describe the anesthetic management of cesarean section after Fontan surgery. Here, we present a case of successful epidural anesthesia in a woman with Fontan circulation who required emergency cesarean section. PATIENT CONCERNS: A 29-year-old woman at gestational week 28 was scheduled for emergency cesarean section because of fetal distress. Her past medical history was significant for congenital transposition of the great arteries that had been treated by Fontan surgery 26 years earlier. Her postoperative course had been uneventful and she had reached a near normal level of activity with no arrhythmias or thrombotic complications. On presentation, her oxygen saturation was approximately 84% and she had digital clubbing. Arterial blood gas analysis showed a PCO2 of 35 mmHg, PO2 of 55.5 mmHg, and hemoglobin of 16.3 g/dL. Her blood coagulation parameters were within normal limits except for a high fibrinogen concentration (4.55 g/L). DIAGNOSIS: The diagnosis was pregnancy requiring emergency cesarean section because of fetal distress. INTERVENTIONS: Before anesthesia, a radial artery line was established for continuous measurement of blood pressure. An air pressure pump was placed on the patient's lower limbs and a low-dose dobutamine infusion was started. Next, epidural anesthesia was successfully performed at L2-3. Five milliliters of 2% lidocaine followed by 10 mL of 0.75% ropivacaine were injected. Dobutamine was infused to maintain a target blood pressure of 100-120/60-70 mmHg. OUTCOMES: The procedure was uneventful with the patient maintaining a stable heart rate of 80 to 90 beats/min and an oxygen saturation of 90% to 94%. A male infant weighing 840 g was delivered. The Apgar score was 9 at 1 and 5 minutes. The patient was transferred to the intensive care unit for 20 hours of monitoring and discharged 9 days later. The neonate was discharged after 2 months of specialist neonatal treatment. LESSONS: Epidural anesthesia may be used in women with Fontan circulation undergoing emergency cesarean section. Knowledge of the physiology of the heart lesion and that of pregnancy are critical to the outcome.


Assuntos
Anestesia Epidural/métodos , Anestesia Obstétrica/métodos , Técnica de Fontan/efeitos adversos , Adulto , Cesárea/métodos , Feminino , Sofrimento Fetal , Humanos , Lactente Extremamente Prematuro , Recém-Nascido , Gravidez , Complicações Cardiovasculares na Gravidez/terapia
16.
Int Heart J ; 61(1): 174-177, 2020 Jan 31.
Artigo em Inglês | MEDLINE | ID: mdl-31956137

RESUMO

An 18-year-old male who had a past medical history of an intracardiac total cavopulmonary connection (TCPC) operation was referred to our hospital for radiofrequency catheter ablation (RFCA) of supraventricular tachycardia (SVT). Two types of SVTs were induced, and 3-dimensional (3D) maps were created using an ultra-high-density 3-dimensional mapping system (Rhythmia). The earliest atrial activation site (EAAS) of SVT1 was at the superior part of the conduit, and the EAAS of SVT2 was at the inferior part of the single atrium (SA). The SVTs were terminated by energy deliveries to the EAAS from the conduit in SVT1 and from inside the single atrium in SVT2. Detailed maps of the SVTs were important to understand the mechanisms of the SVTs. The Rhythmia system was useful for the detailed mapping of complex arrhythmias. The use of Rhythmia in patients after a TCPC is difficult, because puncturing the TCPC conduit and proceeding and manipulating the Orion catheter via a narrow puncture hole are difficult. We were the first to succeed in ablating two atrial tachycardias (ATs) originating from the inside and outside of the conduit after a TCPC operation by using an ultra-high-density 3-dimensional mapping system.


Assuntos
Técnica de Fontan/efeitos adversos , Taquicardia Atrial Ectópica/cirurgia , Taquicardia Supraventricular/cirurgia , Adolescente , Ablação por Cateter , Técnicas Eletrofisiológicas Cardíacas , Humanos , Masculino , Tomografia Computadorizada por Raios X
17.
Catheter Cardiovasc Interv ; 95(5): 950-953, 2020 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-31912986

RESUMO

Trans-apical approach has been proved successful in failing surgical bio-prosthesis in both mitral and aortic position in adult patients. Recently, valve-in-valve treatments have been applied even in patients with complex congenital heart disease. Here, we report the case of a 32 years old lady with left atrial isomerism, complete AV septal defect, interrupted inferior vena cava with azygos continuation who underwent Kawashima procedure with atrial Fontan. Severe systemic atrioventricular valve regurgitation necessitated a 33 mm Perimount valve implantation and conversion to lateral tunnel Fontan. After only 4 years there was severe valve stenosis and the patient underwent successful trans-apical transcatheter implantation of a 29 mm Sapien valve.


Assuntos
Cateterismo Cardíaco/instrumentação , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca/instrumentação , Próteses Valvulares Cardíacas , Insuficiência da Valva Tricúspide/cirurgia , Estenose da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Adulto , Feminino , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Desenho de Prótese , Recuperação de Função Fisiológica , Resultado do Tratamento , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/fisiopatologia , Estenose da Valva Tricúspide/diagnóstico por imagem , Estenose da Valva Tricúspide/etiologia , Estenose da Valva Tricúspide/fisiopatologia
18.
Pediatr Cardiol ; 41(4): 703-708, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31894396

RESUMO

The objective of this study was to determine medium-term morbidity and mortality of patients who have undergone device closure of an extracardiac Fontan fenestration with an Amplatzer Vascular Plug II (AVPII) or Septal Occluder (ASO). A secondary objective was to compare medium-term morbidity and mortality between these patients and other fenestrated Fontan patients. A retrospective chart review was performed on patients who underwent an extracardiac fenestrated Fontan procedure between 1992 and 2015 at Cardinal Glennon Children's Medical Center. Procedural and follow-up data were obtained and compared between those who underwent fenestration closure and those who did not. Additional outcome measures included whether the fenestration had spontaneously closed, morbidity and mortality, oxygen saturations, and hemodynamics pre- and post-closure. Fifty-nine of 118 patients (50%) with a fenestrated Fontan underwent 60 device closures of the fenestration. Thirty-two (53%) of these were with the AVPII and 28 (47%) with the ASO. There was one device embolization. At a median follow-up of 3.9 years, five patients suffered morbidity, including 2 with arrhythmias, 1 with plastic bronchitis, 1 with protein losing enteropathy, and 1 with stroke. There were no cardiopulmonary deaths in this group. Twenty-three of 118 patients (19%) had spontaneous closure. There was no difference in morbidity and mortality between patients who underwent percutaneous fenestration closure and those who either had spontaneous closure or a persistently patent fenestration. Device closure of Fontan fenestrations is a safe and effective procedure with minimal morbidity and mortality comparable to other patients with fenestrations.


Assuntos
Embolização Terapêutica/instrumentação , Técnica de Fontan/métodos , Adolescente , Cateterismo Cardíaco/métodos , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Técnica de Fontan/efeitos adversos , Humanos , Masculino , Estudos Retrospectivos , Dispositivo para Oclusão Septal
19.
Pediatr Cardiol ; 41(3): 624-631, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31980851

RESUMO

We developed a Fontan surveillance catheterization protocol as part of routine assessment of stable patients 10 years after Fontan completion. The surveillance catherization includes hemodynamic assessment with inhaled nitric oxide, angiography, liver biopsy, and transcatheter intervention if indicated. We aimed to describe hemodynamic and liver biopsy findings, response to pulmonary vasoreactivity testing, rates of transcatheter intervention, and changes in medical therapy following surveillance catheterization in stable Fontan patients. A single-center retrospective review of Fontan patients undergoing surveillance catheterization between November 2014 and May 2019 was performed. Liver biopsies were independently scored by two pathologists. Sixty-three patients underwent surveillance catheterization (mean age 14.6 ± 3.0 years). The mean Fontan pressure was 11.8 ± 2.1 mmHg. The mean cardiac index was 2.9 ± 0.6 L/min/m2. In the 51 patients who underwent pulmonary vasoreactivity testing, there was a significant decrease in median pulmonary vascular resistance (1.8 [range 0.8-4.1] vs 1.4 [range 0.7-3.0] Wood units × m2; p < 0.001). The mean cardiac index increased (3.0 ± 0.6 vs 3.2 ± 0.7 L/min/m2, p = 0.009). The Fontan pressure did not change significantly. Fifty-seven patients underwent liver biopsy, and all but one showed fibrosis. Nineteen patients (33.3%) demonstrated bridging fibrosis or cirrhosis. Twenty-five patients underwent 34 transcatheter interventions. Pulmonary artery or Fontan stent placement was performed in 19 patients. Phosphodiesterase type 5 inhibitors were initiated in nine patients following surveillance catheterization. Routine surveillance catheterization with liver biopsy in adolescent Fontan patients reveals information that can guide interventional and medical management. Further long-term follow-up and assessment are indicated to assess the benefit of these interventions.


Assuntos
Cateterismo Cardíaco/métodos , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Adolescente , Biópsia , Criança , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Hemodinâmica , Humanos , Cirrose Hepática/diagnóstico , Cirrose Hepática/etiologia , Masculino , Estudos Retrospectivos , Fatores de Tempo
20.
J Thorac Cardiovasc Surg ; 159(6): 2459-2466.e5, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31866080

RESUMO

OBJECTIVES: The purpose of this article is to provide thoracic and cardiovascular surgeons with a practical step-by-step strategy to use in collaboration with a biostatistician for implementation of competing risks analysis when analyzing time-to-event data. Patients may have an outside event that precludes the event of interest. Traditional time-to-event analysis incorrectly assumes noninformative censoring in this scenario, which will lead to invalid results and conclusions. METHODS: The steps are (1) to determine whether competing risks analysis is needed, (2) to perform a nonparametric analysis, (3) to perform a model-based analysis, (4) to interpret the results, and (5) to compare to traditional survival analysis methods. We apply our approach to a hypothetical cardiovascular surgery example in determining the hazard of mortality after the stage 3 Fontan operation associated with prematurity among patients with hypoplastic left heart syndrome who had successful completion of Norwood stage 1 while incorporating mortality during the stage 2 bidirectional Glenn procedure as a competing risk. We apply nonparametric, semiparametric, and parametric methods. RESULTS: Although Cox regression establishes prematurity as a significant risk factor of mortality after stage 3 (hazard ratio, 1.26; 95% confidence interval, 1.06-1.50; P = .009), the competing risks analysis with the Fine-Gray model accounting for mortality after stage 2 determines that prematurity is not a significant predictor (hazard ratio, 1.07; 95% confidence interval, 0.90-1.27; P = .467). CONCLUSIONS: This article provides a practical step-by-step approach for making competing risks more accessible for cardiac surgeons collaborating with a biostatistician in analyzing and interpreting time-to-event data.


Assuntos
Bioestatística , Técnica de Fontan/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/mortalidade , Nascimento Prematuro/mortalidade , Cirurgiões , Técnica de Fontan/efeitos adversos , Técnica de Fontan/estatística & dados numéricos , Idade Gestacional , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Procedimentos de Norwood/efeitos adversos , Procedimentos de Norwood/estatística & dados numéricos , Intervalo Livre de Progressão , Medição de Risco , Fatores de Risco , Fatores de Tempo
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA