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2.
Zhonghua Wai Ke Za Zhi ; 57(12): 939-943, 2019 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-31826600

RESUMO

Objective: To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow. Methods: Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University. There were 29 males and 20 females. The age at the time of surgery was 5.6 (11.5) months (M(Q(R))), and the weight was 5.2 (3.9) kg. The medical records and results after pulmonary artery banding (death/reoperation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death, transition to the Fontan procedure) were reviewed retrospectively. Actuarial survivals were estimated by the Kaplan-Meier curve. Relative factors for affecting outcomes were analyzed using the Cox regression hazard model. Results: There were 8 early deaths, with a mortality of 16.3%, including 4 cases who received simultaneous arch repair. There were 5 late deaths. During the follow-up of 47(62) (M(Q(R))) months, 11 patients (22.4%) underwent pulmonary artery banding adjustment, 29 patients (59.2%) underwent the Glenn procedure, 21 patients (42.8%) underwent the Fontan procedure. The survival of patients after the initial pulmonary artery banding were 77.4% (95%CI: 65.6% to 89.2%) and 72.6% (95%CI: 59.9% to 85.3%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that systemic ventricular outflow tract obstruction (HR=4.25, 95%CI: 1.50 to 12.03, P=0.006) and total anomalous pulmonary venous connection (HR=6.49, 95%CI: 3.24 to 12.98, P=0.000) were relative factors for death. Conclusions: The early and midterm outcomes of pulmonary artery banding as an initial palliative strategy is not satisfactory. Systemic ventricular outflow tract obstruction and total anomalous pulmonary venous connection are associated with high mortality.


Assuntos
Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Cuidados Paliativos/métodos , Artéria Pulmonar/cirurgia , Procedimentos Cirúrgicos Vasculares/mortalidade , Feminino , Seguimentos , Ventrículos do Coração/anormalidades , Humanos , Lactente , Recém-Nascido , Masculino , Circulação Pulmonar , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/métodos
3.
J Med Case Rep ; 13(1): 307, 2019 Oct 10.
Artigo em Inglês | MEDLINE | ID: mdl-31601276

RESUMO

BACKGROUND: A coronary cameral fistula is a rare connection between a coronary artery and a cardiac chamber or vein bypassing the cardiac capillary bed system. Most of these fistulae are congenital and solitary, although they can be acquired and multiple. CASES PRESENTATION: Case 1: A 10-year-old black South African boy presented with a long-standing history of fatigue; he had a heart murmur, and a bounding pulse and wide pulse pressure. An echocardiogram demonstrated a large coronary cameral fistula involving his left coronary artery and his left ventricle. This was also confirmed on ascending aortogram. Surgical ligation was done and his symptoms improved afterward, but a small residual fistula remained. Case 2: A 7-year-old black South African boy had decreased effort tolerance and a heart murmur on the mid-sternal border. He had cardiomegaly on chest roentgenogram and a dilated left coronary artery origin on echocardiogram. An ascending aortogram confirmed a large left coronary cameral fistula draining to the left ventricle. Case 3: A 28-year-old black South African woman with decreased effort tolerance and chest pain on exertion had a continuous murmur over the lower sternal border. Echocardiography demonstrated a dilated right coronary artery with a fistulous connection to her right ventricle. An ascending aortogram demonstrated a tortuous coronary cameral fistula arising from her right coronary artery to her right ventricle. All three patients were successfully treated percutaneously using the Amplatzer vascular plug type II device. CONCLUSION: The availability of numerous vascular closure devices has made transcatheter occlusion the treatment of choice for the majority of coronary cameral fistulae, rather than the traditional surgical ligation.


Assuntos
Cateterismo Cardíaco , Ventrículos do Coração/anormalidades , Ventrículos do Coração/cirurgia , Dispositivo para Oclusão Septal , Fístula Vascular/congênito , Fístula Vascular/cirurgia , Adulto , Criança , Angiografia Coronária , Ecocardiografia , Fadiga/etiologia , Feminino , Sopros Cardíacos/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Fístula Vascular/diagnóstico por imagem
4.
Cardiol Young ; 29(10): 1248-1256, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31477187

RESUMO

BACKGROUND: While echocardiographic parameters are used to quantify ventricular function in infants with single ventricle physiology, there are few data comparing these to invasive measurements. This study correlates echocardiographic measures of diastolic function with ventricular end-diastolic pressure in infants with single ventricle physiology prior to superior cavopulmonary anastomosis. METHODS: Data from 173 patients enrolled in the Pediatric Heart Network Infant Single Ventricle enalapril trial were analysed. Those with mixed ventricular types (n = 17) and one outlier (end-diastolic pressure = 32 mmHg) were excluded from the analysis, leaving a total sample size of 155 patients. Echocardiographic measurements were correlated to end-diastolic pressure using Spearman's test. RESULTS: Median age at echocardiogram was 4.6 (range 2.5-7.4) months. Median ventricular end-diastolic pressure was 7 (range 3-19) mmHg. Median time difference between the echocardiogram and catheterisation was 0 days (range -35 to 59 days). Examining the entire cohort of 155 patients, no echocardiographic diastolic function variable correlated with ventricular end-diastolic pressure. When the analysis was limited to the 86 patients who had similar sedation for both studies, the systolic:diastolic duration ratio had a significant but weak negative correlation with end-diastolic pressure (r = -0.3, p = 0.004). The remaining echocardiographic variables did not correlate with ventricular end-diastolic pressure. CONCLUSION: In this cohort of infants with single ventricle physiology prior to superior cavopulmonary anastomosis, most conventional echocardiographic measures of diastolic function did not correlate with ventricular end-diastolic pressure at cardiac catheterisation. These limitations should be factored into the interpretation of quantitative echo data in this patient population.


Assuntos
Cateterismo Cardíaco/métodos , Ecocardiografia Doppler/métodos , Enalapril/uso terapêutico , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/anormalidades , Função Ventricular Esquerda/fisiologia , Pressão Ventricular/fisiologia , Anti-Hipertensivos/uso terapêutico , Diástole , Método Duplo-Cego , Feminino , Seguimentos , Cardiopatias Congênitas/tratamento farmacológico , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
5.
Rev Port Cir Cardiotorac Vasc ; 26(2): 147-149, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31476817

RESUMO

We report the case of a 44 year-old patient with complex ACHD, admitted with acute decompensated heart failure (ADHF) in hemodynamic profile B. He had a single ventricle with pulmonary atresia, previously submitted to three modified Blalock-Taussig shunts (mBTs) at the age of 2, 12 and 19 years old. Despite conventional treatment with diuretics, ß-blockers (BB) and isosorbide dinitrate the patient progressed to profile C and the transthoracic echocardiogram disclosed a reduced systolic function. Likewise, levosimendan was commenced and an appropriate decongestion and a marked reduction in the NT-proBNP were seen. Treatment with angiotensin-converting-enzyme inhibitor, BB, ivabradine and mineralocorticoid receptor was optimized. The patient was discharged home after 26 days in NYHA class III and referred for heart transplant after right heart catheterization. To our knowledge, this is the first report of successful levosimendan's use in ADHF in a mBTs long-term survivor.


Assuntos
Fármacos Cardiovasculares/uso terapêutico , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/tratamento farmacológico , Ventrículos do Coração/anormalidades , Simendana/uso terapêutico , Adulto , Procedimento de Blalock-Taussig , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/cirurgia , Humanos , Masculino , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Resultado do Tratamento
6.
J Artif Organs ; 22(4): 338-340, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31392523

RESUMO

We present a case of successful ventricular assist device support in a 13-year-old female diagnosed with right single ventricle, asplenia, dextrocardia, who had undergone a Fontan operation at 4 years old in an associated children hospital. She underwent placement of Jarvik 2000 axial flow ventricular assist device to the morphologic right ventricle which worked as systemic ventricle. The postoperative course was not eventful. She was waiting for heart transplantation attending high school 3 years after implantation.


Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/cirurgia , Ventrículos do Coração/anormalidades , Coração Auxiliar , Adolescente , Feminino , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Humanos , Desenho de Prótese , Reoperação , Tomografia Computadorizada por Raios X
7.
Congenit Heart Dis ; 14(5): 765-771, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31282062

RESUMO

SETTING: Fontan-associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra-cardiac dysfunction as measured by MELD and MELD-XI. OBJECTIVE: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD-XI scores. DESIGN: Single-center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function were tested for association with serum creatinine, MELD, and MELD-XI. Linear regression was used to perform multivariable analysis in the echocardiogram cohort. RESULTS: Fifty-seven patients had congruent lab and catheterization data for analysis. Sixty-three and sixty-nine patients had congruent lab and echocardiogram data for MELD and MELD-XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD and MELD-XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD-XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 and P < .001 for MELD and MELD-XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02). CONCLUSIONS: In adults following Fontan palliation, systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction as assessed by elevated serum creatinine, MELD, and MELD-XI scores.


Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Nefropatias/etiologia , Hepatopatias/etiologia , Complicações Pós-Operatórias , Disfunção Ventricular Esquerda/complicações , Adolescente , Adulto , Cateterismo Cardíaco , Criança , Pré-Escolar , Ecocardiografia , Feminino , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/anormalidades , Ventrículos do Coração/diagnóstico por imagem , Humanos , Incidência , Nefropatias/epidemiologia , Hepatopatias/epidemiologia , Masculino , Estudos Retrospectivos , Estados Unidos/epidemiologia , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda/fisiologia , Adulto Jovem
8.
World J Pediatr Congenit Heart Surg ; 10(4): 426-432, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31307296

RESUMO

BACKGROUND: Single-stage biventricular repair remains a challenging and difficult decision in high-risk newborns and early infants with the presence of left ventricular outflow tract obstruction (LVOTO) or borderline hypoplasia of the left ventricle (LV). METHODS: Six high-risk patients underwent the initial hybrid procedure (bilateral pulmonary banding + ductal stenting) for staged biventricular repair. Their median age was 17 days (range: 7-55 days). The diagnosis was interrupted aortic arch (IAA), ventricular septal defect (VSD), and LVOTO (n = 3); IAA and VSD (n = 1); and aortic annular hypoplasia, aortic arch hypoplasia, VSD, and LVOTO (n = 1). The last patient had borderline LV with large atrial septal defect (ASD) and aortic arch hypoplasia. The patient with borderline LV had also ASD closure with small fenestration. RESULTS: One patient died of sepsis after the hybrid procedure. Other patients underwent biventricular repair 8 to 13 months later. Three patients had conventional repair with conal septum resection. The other patient with IAA, in whom LVOTO was considered nonresectable, underwent Yasui operation. The last patient with borderline LV had enough development of left heart structures during follow-up and underwent aortic arch repair. One patient who had conal septum resection died after biventricular repair. One patient needed a tracheostomy; four patients were discharged uneventfully and their clinical conditions were good on postoperative year 1. CONCLUSION: Staged biventricular repair with the initial hybrid procedure may be a feasible and safe alternative in high-risk neonates and early infants. Hybrid intervention may provide the development of cardiac structures in time and a better evaluation for the possibility of biventricular repair in borderline patients.


Assuntos
Coartação Aórtica/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Ventrículos do Coração/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico , Ecocardiografia , Feminino , Comunicação Interventricular/complicações , Comunicação Interventricular/diagnóstico , Ventrículos do Coração/anormalidades , Humanos , Lactente , Recém-Nascido , Masculino , Obstrução do Fluxo Ventricular Externo/diagnóstico , Obstrução do Fluxo Ventricular Externo/etiologia
9.
Congenit Heart Dis ; 14(5): 854-863, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31332952

RESUMO

OBJECTIVE: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long-term outcomes in patients with T21 and SV palliation. DESIGN: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anomaly following Fontan palliation for similar diagnoses. Kaplan-Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers. RESULTS: We identified 118 children with T21 who underwent initial surgical SV palliation. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in-hospital survival of 80.3% and 76.0%, respectively. Fifty-three patients had sufficient identifiers for PCCC-NDI-OPTN linkage. Ten-year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34-7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD). CONCLUSIONS: Children with T21 and SV are at high risk for procedural and long-term mortality related to their genetic condition and underlying CHD. Nevertheless, a select group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long-term survival.


Assuntos
Anormalidades Múltiplas , Síndrome de Down/diagnóstico , Previsões , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Adolescente , Causas de Morte/tendências , Criança , Pré-Escolar , Síndrome de Down/mortalidade , Seguimentos , Testes Genéticos , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Lactente , Recém-Nascido , Período Pós-Operatório , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologia , Adulto Jovem
10.
Pediatr Cardiol ; 40(7): 1331-1338, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31342111

RESUMO

Left ventricular noncompaction (LVNC) is a genetically heterogeneous disorder the etiology of which is still debated. During fetal development, trabecular cardiomyocytes contribute extensively to the working myocardium and the ventricular conduction system. The impact of developmental defects in trabecular myocardium in the etiology of LVNC has been debated. Recently we generated new mouse models of LVNC by the conditional deletion of the key cardiac transcription factor encoding gene Nkx2-5 in trabecular myocardium at critical steps of trabecular development. These conditional mutant mice recapitulate pathological features similar to those observed in LVNC patients, including a hypertrabeculated left ventricle with deep endocardial recesses, subendocardial fibrosis, conduction defects, strain defects, and progressive heart failure. After discussing recent findings describing the respective contribution of trabecular and compact myocardium during ventricular morphogenesis, this review will focus on new data reflecting the link between trabecular development and LVNC.


Assuntos
Ventrículos do Coração/anormalidades , Miocárdio Ventricular não Compactado Isolado/genética , Animais , Modelos Animais de Doenças , Ventrículos do Coração/embriologia , Humanos , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Masculino , Camundongos , Miocárdio/patologia , Miócitos Cardíacos/patologia , Deleção de Sequência
11.
Pediatr Cardiol ; 40(7): 1476-1487, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31342112

RESUMO

The optimal timing of stage-2-palliation (S2P) in single left ventricle is not clear. The aim of this study was to identify S2P related factors associated with outcomes after total cavopulmonary connection (TCPC), particularly relative to the dominant systemic ventricle. A total of 405 patients who underwent both S2P and TCPC at our institute between 1997 and 2017 was included. Patients were divided into two groups, dominant right ventricle (RV type, n = 235) and dominant left ventricle (LV type, n = 170). S2P related factors associated with mortality, postoperative ventricular function, and late exercise capacity following TCPC, were analyzed. The median age at S2P was 4 [3-7] and 6 [3-11] months in RV and LV type patients, respectively (p = 0.092). Survival after TCPC was similar in RV and LV type patients (p = 0.280). In those with RV type, risk factors for mortality following TCPC were older age (p < 0.001), heavier weight (p = 0.001), higher PAP (p < 0.001), higher TPG (p = 0.010), and lower SO2 (p = 0.008) at S2P. In those with LV type, no risk factor was identified. Risk factors for postoperative impaired ventricular function were older age and higher weight at S2P in both RV and LV type patients. Older age at S2P was also identified as a risk for inferior peak oxygen uptake (VO2) years after TCPC both in RV and LV type patients. Older age at S2P was associated with higher mortality after Fontan completion only in RV type patients. However, it was associated with postoperative ventricular dysfunction and lower exercise capacity after TCPC in both RV and LV type patients.


Assuntos
Técnica de Fontan/mortalidade , Cuidados Paliativos/métodos , Disfunção Ventricular/fisiopatologia , Fatores Etários , Pré-Escolar , Feminino , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Humanos , Lactente , Masculino , Fatores de Risco , Resultado do Tratamento
13.
Semin Thorac Cardiovasc Surg ; 31(3): 526-534, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31150828

RESUMO

To review the outcome after atrioventricular valve replacement in single ventricle patients. The medical records of 37 consecutive patients who underwent initial valve replacement between 2001 and 2016 were reviewed. Actuarial survival rates were 73%, 65%, and 59% at 1, 5, and 10 years, respectively. Multivariate analysis indicated body surface area <0.28 m2 (P = 0.007; hazard ratio, 31.1), preoperative inotropic support (P < 0.001; hazard ratio, 24.5), primary valve replacement (P = 0.044; hazard ratio, 6.1), oversized prosthesis (P = 0.001; hazard ratio, 14.5), and intra- or postoperative extracorporeal membrane oxygenation support (P < 0.001; hazard ratio, 53.2) were the risk factors for mortality. Cumulative incidences of redo replacement were 11%, 17%, and 17% at 1, 5, and 10 years, respectively. There was no risk factor for redo replacement. Among 11 patients undergoing valve replacement before or at the time of bidirectional cavopulmonary shunt, all 4 patients who reached Fontan completion survived. Among 13 patients undergoing valve replacement after bidirectional cavopulmonary shunt, only 3 patients reached Fontan completion. Among 11 patients undergoing valve replacement after Fontan completion, there were 3 operative mortalities and 2 late mortalities. For 7 of 8 hospital survivors, cardiac index improved from 2.2 L/min/m2 (interquartile range, 2.0-2.4) to 3.4 (2.8-3.7) (P = 0.002). Atrioventricular valve replacement was a reasonable choice of treatment for single ventricle patients. For small patients with impaired ventricular function who had no choice other than valve replacement, commercially available valves were oversized and outcomes remained poor.


Assuntos
Cardiopatias Congênitas/fisiopatologia , Implante de Prótese de Valva Cardíaca , Ventrículos do Coração/fisiopatologia , Insuficiência da Valva Mitral/cirurgia , Valva Mitral/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Função Ventricular , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/mortalidade , Ventrículos do Coração/anormalidades , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Masculino , Valva Mitral/diagnóstico por imagem , Valva Mitral/fisiopatologia , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/mortalidade , Insuficiência da Valva Mitral/fisiopatologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Recuperação de Função Fisiológica , Reoperação , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/mortalidade , Insuficiência da Valva Tricúspide/fisiopatologia
14.
Congenit Heart Dis ; 14(5): 720-725, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31231979

RESUMO

BACKGROUND/HYPOTHESIS/OBJECTIVES: Postoperative complications after the Fontan operation for single ventricle heart disease are common and include persistent pleural drainage and prolonged length of hospital stay (LOS). Diastolic ventricular dysfunction may increase risk for postoperative complications by raising central venous pressures. We sought to determine the relationship between preoperative echocardiographic measurements of diastolic function, including myocardial deformation imaging, on (a) preoperative invasive catheterization measurements and (b) postoperative outcomes after the Fontan procedure. DESIGN/METHODS: All patients that underwent Fontan procedure from 2011 to 2017 were included. Echocardiograms performed within 6 months prior to Fontan operation were evaluated. Measurements of ventricular global and diastolic strain and strain rate were performed offline with TomTec speckle tracking software. Other diastolic function measurements included atrioventricular valve inflow and annular tissue Doppler imaging. Diastolic function measurements were correlated with pre-Fontan catheterization measurements and postoperative Fontan outcomes using Spearman's rho. Multivariable logistic regression for a prolonged LOS (>75%ile for postoperative LOS) was performed to adjust for preoperative risk factors. RESULTS: A total of 141 patients were included in the study. Majority had single morphologic right ventricle (58.9%). Median age at time of Fontan was 3.4 years (IQR 2.9-4). Median hospital LOS was 9 days (IQR 7-11). Circumferential diastolic strain rate weakly correlated with LOS (rho = -0.21, P = .01). There was no correlation between any other diastolic strain measurements and pre-Fontan end-diastolic pressure or postoperative LOS. In multivariable analysis, E/E' was the only echo measurement that predicted prolonged hospital LOS (OR 1.4, 95%CI: 1.1-1.8, P = .003). CONCLUSION: Preoperative diastolic strain measurements did not have a strong association with postoperative Fontan outcomes. Increased E/E' ratio, however, did predict greater LOS after Fontan procedure, and may be useful in preoperative risk stratification. Future studies are needed to further assess the utility of diastolic strain imaging in the single-ventricle population.


Assuntos
Ecocardiografia Doppler/métodos , Técnica de Fontan/métodos , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Função Ventricular/fisiologia , Pré-Escolar , Diástole , Feminino , Seguimentos , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Período Pós-Operatório , Estudos Retrospectivos
15.
Pediatr Cardiol ; 40(6): 1224-1230, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31240371

RESUMO

Heterotaxy syndrome (HS) is a significant determinant of outcome in single ventricle (SV) physiology. Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined. We sought to evaluate hospital and interstage outcomes in relation with operative intervention for IM (IMO). Twelve SV/HS patients, who underwent IMO, from January 2004 to December 2016, were studied. Early shunt failure, time to superior cavopulmonary connection (SCPC) and interstage attrition were assessed. Since September 2014, based on a comprehensive standardized protocol, IMO was performed at the time of hospitalization for stage-I palliation (S1P) irrespective of clinical manifestations. Patients were assigned to Group A (n = 8): expectant /symptoms-driven versus Group B (n = 4): protocol-driven. At S1P 7 had systemic-to-pulmonary shunt (SPS), 1 SPS with anomalous pulmonary venous return (APVR) repair (Group A) compared to 2 SPS, 1 SPS with APVR repair and 1 Norwood operation (Group B). Median duration from S1P to IMO was 82 days (range 57-336; Group A) compared to 14 days (range 11-31; Group B); p < 0.05. Median age at IMO was 87 days (range 8-345) [Group A: 99 days (range 68-345) vs Group B: 25 days (range 8-39)] (p < 0.05). Early SPS failure occurred in 25% (2 of 8) for Group A compared to none in Group B (p < 0.05). Hospital mortality following IMO was 25% [Group A: 37.5% (3 of 8) vs Group B: 0; p < 0.05]. Interstage survival was 67% [Group A: 50% (4 of 8) vs Group B: 100%; p < 0.05]. Time to SCPC following S1P was 186 days (range 169-218) for Group A compared to 118 days (range 97-161) (Group B); p < 0.05. Operative intervention for IM in SV/HS is associated with significant interstage attrition and might impact the time to SCPC. SPS is at risk for early failure after IMO. A comprehensive standardized concept can mitigate detrimental implications.


Assuntos
Anormalidades do Sistema Digestório/mortalidade , Anormalidades do Sistema Digestório/cirurgia , Ventrículos do Coração/anormalidades , Síndrome de Heterotaxia/mortalidade , Volvo Intestinal/mortalidade , Volvo Intestinal/cirurgia , Estudos de Casos e Controles , Feminino , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Masculino , Cuidados Paliativos/métodos , Assistência Perioperatória/métodos , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
16.
Tex Heart Inst J ; 46(2): 136-138, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31236081

RESUMO

Congenital complete heart block with concomitant biventricular noncompaction cardiomyopathy has been reported once previously. Although not universal, when restrictive physiology is present, impaired diastolic filling may pose a distinct challenge to pacing during the neonatal period. We present the case of a neonate with congenital complete heart block and biventricular noncompaction that resulted in severe diastolic dysfunction and atrioventricular dyssynchrony. We intentionally used 2:1 ventricular pacing to provide atrioventricular synchrony with every paced beat, and this resulted in hemodynamic and clinical improvement. This unconventional pacing technique may be beneficial in other neonates who have complete heart block and diastolic dysfunction.


Assuntos
Anormalidades Múltiplas , Estimulação Cardíaca Artificial/métodos , Bloqueio Cardíaco/congênito , Cardiopatias Congênitas/terapia , Ventrículos do Coração/anormalidades , Eletrocardiografia , Seguimentos , Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/terapia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Recém-Nascido , Masculino
17.
World J Pediatr Congenit Heart Surg ; 10(3): 338-342, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31084309

RESUMO

Surgical palliation has remarkably improved survival of functionally single ventricle (FSV) patients born in developed nations but such outcomes have not occurred in Africa. The poor care coverage for FSV patients in Africa exists within the larger sphere of deficient health care for children born with congenital heart defects (CHDs) in Africa generally. This review takes the position that to improve health-care coverage for CHD patients on the continent, political priority is paramount. This can be attained with cohesive leadership for the CHD agenda, a guiding institution, and the mobilization of civil society to drive advocacy at national and international levels.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Assistência à Saúde/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Cuidados Paliativos/organização & administração , África , Criança , Ventrículos do Coração/cirurgia , Humanos
18.
Cardiol Young ; 29(5): 583-588, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31046854

RESUMO

OBJECTIVES: To analyse the feasibility and effectiveness in humanitarian practice of surgical management of children with single-ventricle heart condition. METHODS: Retrospective study of children with a single ventricle, managed by the association Mécénat-Chirurgie Cardiaque since 1996, with long-term follow-up after their return home. RESULTS: Of the 138 children in our cohort, 119 had one or more surgeries (180 procedures): palliative surgery alone (systemic-pulmonary anastomosis or banding), 41; partial cavo-pulmonary connection, 47; total cavo-pulmonary connection (mean age 8.5 years), 31. Operative mortality is 5.5%. After a mean follow-up of 5.6 years, 18 children (13%) were lost to follow-up. Survival at 10 years is 79% in children receiving surgery (palliative only, 72%; partial cavo-pulmonary connection, 77%; total cavo-pulmonary connection, 97%) versus 29% in children with no surgical intervention. The prognosis is better for tricuspid atresia and double-inlet left ventricle (86 and 83% survival at 10 years) than for double-outlet right ventricle or complete atrio-ventricular canal defect (64 and 68% at 5 years). CONCLUSION: The surgery of the single ventricle in humanitarian medicine allows a very satisfactory survival after one or more surgeries tending towards a total cavo-pulmonary connection as soon as possible.


Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/cirurgia , Criança , Pré-Escolar , Países em Desenvolvimento , Feminino , Seguimentos , Técnica de Fontan/estatística & dados numéricos , França , Cardiopatias Congênitas/mortalidade , Humanos , Masculino , Cuidados Paliativos/métodos , Socorro em Desastres , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Resultado do Tratamento
19.
Cardiol Young ; 29(5): 660-666, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31142394

RESUMO

INTRODUCTION: Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes. METHODS: Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves. RESULTS: There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64. CONCLUSION: Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.


Assuntos
Cateterismo/efeitos adversos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood , Alta do Paciente , Ecocardiografia , Feminino , Ventrículos do Coração/anormalidades , Ventrículos do Coração/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , Monitorização Ambulatorial/métodos , Cuidados Paliativos/métodos , Philadelphia , Complicações Pós-Operatórias/etiologia , Curva ROC , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
20.
Congenit Heart Dis ; 14(2): 138-139, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30945806

RESUMO

The purpose of this study was to determine the optimal age for performing Fontan operation using data from the National Inpatient Sample. Our results showed that although the Fontan operation was most commonly performed at age 2 in the United States, age 3 is the optimum age for this procedure as evident by lower rate of in-hospital mortality, procedure-related complications, and rate of nonroutine home discharge when procedure is performed at age 3 years.


Assuntos
Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Distribuição por Idade , Fatores Etários , Pré-Escolar , Seguimentos , Técnica de Fontan , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/mortalidade , Ventrículos do Coração/cirurgia , Mortalidade Hospitalar/tendências , Humanos , Lactente , Masculino , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Resultado do Tratamento , Estados Unidos/epidemiologia
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