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1.
Int J Cardiol ; 282: 33-37, 2019 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-30755335

RESUMO

BACKGROUND: The need for permanent pacing has been identified as a predictor of poor outcomes in the late survivors of Fontan surgery. However, it is not clear if the need for a pacemaker is a surrogate marker of a declining Fontan state, or if pacing is deleterious to the Fontan circulation. OBJECTIVES: We sought to compare the long-term outcomes of propensity-matched Fontan patients with and without a permanent pacemaker. METHODS: Patients who have survived Fontan completion with a documented history of cardiac arrhythmia were identified from the Australia and New Zealand Fontan Registry. Pacemaker insertion details, cardiac function and electrophysiological data were obtained for the patients with a permanent pacemaker. Survival analysis was performed with propensity score matching to compare late survival and outcomes in patients with versus without a pacemaker. RESULTS: There was a total of 310 patients with a history of cardiac arrhythmia, of which 126 (41%) had a permanent pacemaker. After propensity-score matching, 99 pairs were generated (n = 198). Patients with a permanent pacemaker had a higher risk of death (HR 3.32 95% CI 1.60-6.90, p = 0.001) and death or transplantation (HR 3.55 95% CI 1.87-6.73, p < 0.001). Patients who were only paced atrially were not at a significantly increased risk of death or transplantation. However, patients who were ventricular paced >50% of the time were much more likely to encounter late death or transplantation (HR 3.82 95% CI 1.64-8.95, p = 0.002). CONCLUSIONS: Having a permanent pacemaker and needing ventricular pacing is likely associated with an increased risk of death and transplantation in patients with a Fontan circulation.


Assuntos
Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/cirurgia , Técnica de Fontan/mortalidade , Transplante de Coração/mortalidade , Marca-Passo Artificial/efeitos adversos , Adolescente , Austrália/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Técnica de Fontan/tendências , Transplante de Coração/tendências , Humanos , Masculino , Mortalidade/tendências , Nova Zelândia/epidemiologia , Marca-Passo Artificial/tendências , Sistema de Registros , Fatores de Risco
2.
Int J Cardiol ; 276: 74-80, 2019 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-30201381

RESUMO

BACKGROUND: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear. PURPOSE: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants. METHOD AND RESULTS: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ±â€¯7 years old, follow-up: 18 ±â€¯6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ±â€¯1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years. CONCLUSIONS: Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.


Assuntos
Causas de Morte/tendências , Técnica de Fontan/mortalidade , Técnica de Fontan/tendências , Cardiopatias/mortalidade , Neoplasias/mortalidade , Adolescente , Adulto , Feminino , Seguimentos , Cardiopatias/diagnóstico , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasias/diagnóstico , Valor Preditivo dos Testes , Estudos Prospectivos , Taxa de Sobrevida/tendências , Adulto Jovem
4.
Int J Cardiol ; 271: 312-316, 2018 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-30223363

RESUMO

BACKGROUND: Elevation in central venous pressure (CVP) plays a fundamental pathophysiologic role in Fontan circulation. Because there is no sub-pulmonary ventricle in this system, CVP also provides the driving force for pulmonary blood flow. We hypothesized that this would make Fontan patients more susceptible to even low-level elevation in pulmonary vascular resistance index (PVRI), resulting in greater systemic venous congestion and adverse outcomes. METHODS: Adult Fontan patients and controls without congenital heart disease undergoing clinical evaluation that included cardiac catheterization and echocardiography were examined retrospectively. Outcomes including all-cause mortality and the development of Fontan associated diseases (FAD, defined as protein losing enteropathy, cirrhosis, heart failure hospitalization, arrhythmia, or thromboembolism) were assessed from longitudinal assessment. RESULTS: As compared to controls (n = 82), Fontan patients (n = 164) were younger (36 vs 45 years, p < 0.001), more likely to be on anticoagulation or antiplatelet therapy, and more likely to have atrial arrhythmia or cirrhosis. There was a strong correlation between CVP and PVRI in the Fontan group (r = 0.79, p < 0.001), but there was no such relationship in controls. Elevated PVRI identified patients at increased risk for FAD (HR 1.92, 95% CI 1.39-2.41, p = 0.01), and composite endpoint of FAD and/or death (HR 1.89, 95% CI 1.32-2.53, p = 0.01) per 1 WU∗m2 increment. CONCLUSIONS: Systemic venous congestion, which is the primary factor in the pathogenesis of FAD and death, is related to even low-level abnormalities in pulmonary vascular function. Multicenter studies are needed to determine whether interventions targeting pulmonary vascular structure and function can improve outcomes in the Fontan population.


Assuntos
Pressão Venosa Central/fisiologia , Técnica de Fontan/tendências , Hiperemia/diagnóstico por imagem , Hiperemia/terapia , Circulação Pulmonar/fisiologia , Adulto , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Hiperemia/etiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento
5.
Int J Cardiol ; 273: 100-107, 2018 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-30060970

RESUMO

BACKGROUND: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. METHODS: A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). RESULTS: Mean age and time since Fontan were 19.8 ±â€¯9.3 and 14.1 ±â€¯7.6 years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (r = 0.24, p = 0.015) and ACR (r = 0.29, p = 0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, p = 0.002). Renal impairment was mild (mGFR 60-89 ml/min/1.73 m2) in 46/131 (35%) and moderate (mGFR 30-59 ml/min/1.73 m2) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (ß = 0.89, 95% CI 0.54-1.25, p = 0.002) and decreased mGFR (ß = -0.77, 95% CI -1.29-0.24, p = 0.005). CONCLUSIONS: In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion.


Assuntos
Técnica de Fontan/tendências , Rim/diagnóstico por imagem , Fígado/diagnóstico por imagem , Sistema de Registros , Relatório de Pesquisa , Adolescente , Adulto , Austrália/epidemiologia , Estudos Transversais , Ecocardiografia/tendências , Feminino , Técnica de Fontan/efeitos adversos , Taxa de Filtração Glomerular/fisiologia , Humanos , Rim/fisiologia , Fígado/fisiologia , Masculino , Nova Zelândia/epidemiologia , Adulto Jovem
6.
Int J Cardiol ; 271: 54-59, 2018 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-29861103

RESUMO

BACKGROUND: Changes in circulatory physiology are common in Fontan patients due to suboptimal cardiac output, which may reduce the peripheral blood flow and impair the skeletal muscle. The objective of this study was to investigate the forearm blood flow (FBF), cross-sectional area (CSA) of the thigh and functional capacity in asymptomatic clinically stable patients undergoing Fontan surgery. METHODS: Thirty Fontan patients and 27 healthy subjects underwent venous occlusion plethysmography, magnetic resonance imaging of the thigh musculature and maximal cardiopulmonary exercise testing. Muscle sympathetic nerve activity (MSNA), norepinephrine measures, cardiovascular magnetic resonance, handgrip strength and 6-minute walk test were also performed. RESULTS: Fontan patients have blunted FBF (1.59 ±â€¯0.33 vs 2.17 ±â€¯0.52 mL/min/100 mL p < 0.001) and forearm vascular conductance (FVC) (1.69 ±â€¯0.04 vs 2.34 ±â€¯0.62 units p < 0.001), reduced CSA of the thigh (81.2 ±â€¯18.6 vs 116.3 ±â€¯26.4 cm2p < 0.001), lower peak VO2 (29.3 ±â€¯6 vs 41.5 ±â€¯9 mL/kg/min p < 0.001), walked distance (607 ±â€¯60 vs 701 ±â€¯58 m p < 0.001) and handgrip strength (21 ±â€¯9 vs 30 ±â€¯8 kgf p < 0.001). The MSNA (30 ±â€¯4 vs 22 ±â€¯3 bursts/min p < 0.001) and norepinephrine concentration [265 (236-344) vs 222 (147-262) pg/mL p = 0.006] were also higher in Fontan patients. Multivariate linear regression showed FVC (ß = 0.653; CI = 0.102-1.205; p = 0.022) and stroke volume (ß = 0.018; CI = 0.007-0.029; p = 0.002) to be independently associated with reduced CSA of the thigh adjusted for body mass index. The CSA of the thigh adjusted for body mass index (ß = 5.283; CI = 2.254-8.312; p = 0.001) was independently associated with reduced peak VO2. CONCLUSION: Patients with Fontan operation have underdeveloped skeletal muscle with reduced strength that is associated with suboptimal peripheral blood supply and diminished exercise capacity.


Assuntos
Tolerância ao Exercício/fisiologia , Técnica de Fontan/tendências , Força da Mão/fisiologia , Músculo Esquelético/diagnóstico por imagem , Músculo Esquelético/fisiologia , Adolescente , Adulto , Estudos Transversais , Teste de Esforço/métodos , Teste de Esforço/tendências , Feminino , Seguimentos , Técnica de Fontan/efeitos adversos , Antebraço/irrigação sanguínea , Antebraço/diagnóstico por imagem , Antebraço/fisiologia , Capacidade Residual Funcional/fisiologia , Humanos , Masculino , Músculo Esquelético/irrigação sanguínea , Adulto Jovem
7.
Int J Cardiol ; 260: 54-59, 2018 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-29622455

RESUMO

BACKGROUND: Little is known about adults living with a Fontan circulation's concerns outside the scope of their clinical outcomes. We examined adults with a Fontan circulations' greatest concerns, as well as their concerns around anti-coagulation, pregnancy and finances. METHODS: Adults with a Fontan circulation in the Australian and New Zealand Fontan Registry were invited to complete an anonymous online survey, of which 57 participated. A qualitative method approach using thematic analyses was used. RESULTS: The greatest concerns for adults living with a Fontan circulation were fear of death/uncertainty around life expectancy which for many individuals colored their concerns around physical health, pregnancy and having children, quality of life and finances. Improving information about outcomes to patients with a Fontan circulation might alleviate uncertainties about their future. CONCLUSIONS: Fear of death is the primary concern of adults with a Fontan circulation. It may require improved communication and targeted psychological interventions. Physical exercise incorporated as part of their lifestyle should be encouraged to alleviate physical concerns and also improve psychological well-being.


Assuntos
Medo/psicologia , Técnica de Fontan/psicologia , Técnica de Fontan/tendências , Autorrelato , Adolescente , Adulto , Austrália/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Nova Zelândia/epidemiologia , Sistema de Registros , Fatores de Tempo , Adulto Jovem
8.
Int J Cardiol ; 257: 62-66, 2018 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-29506739

RESUMO

OBJECTIVES: 1) To determine the accuracy of estimated GFR (eGFR) as compared to directly measured GFR (mGFR) in the adult Fontan population; 2) to determine the true prevalence of chronic kidney damage (CKD) as determined by uACR AND eGFR. METHODS: Prospective study of 81 patients Fontan patients (≥18years) followed at St. Paul's Hospital, University of British Columbia. CKD-EPI and MDRD equations used to calculate eGFR, mGFR determined by 99mTc-DTPA renal dynamic imaging and urine albumin to creatinine ratios were calculated. RESULTS: The mGFR was 93±27ml/min/1.73m2: 28 (53%) had an mGFR<90ml/min/1.73m2 and 1 (2%) had an mGFR <60ml/min/1.73m2. There was a modest correlation between mGFR and eGFR (EPI/MDRD) (r=0.50, p<0.0001 and r=0.54, p<0.0001 respectively). Both eGFR (EPI) (bias 27.0; 95% CI 18.0-27.7ml/min/m2, p<0.0001) and eGFR (MDRD) (bias 15.5; 95% CI 7.6-17.4ml/min/m2, p<0.0001) overestimated GFR as compared to mGFR. Among patients with an eGFR (EPI)/(MDRD) >90ml/min/1.73m2, 50% and 46% respectively had an mGFR <90ml/min/1.73m2. Significant albuminuria (>3mg/mmol) was present in 33% and upwards of 32% of patients with a normal eGFR (MDRD/EPI) had evidence of CKD with uACR >3mg/mmol. Using combined criteria of eGFR <90ml/min/1.73m2 and/or uACR >3mg/mmol, 46% of patients had evidence of CKD. CONCLUSIONS: This study draws attention to the need for stringent CKD screening as an important proportion of CKD is currently not being detected. Mild undetected CKD, an early marker of end organ damage, may also be an early sign of Fontan failure that requires warrants further research.


Assuntos
Técnica de Fontan/tendências , Vigilância da População , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/epidemiologia , Adulto , Feminino , Taxa de Filtração Glomerular/fisiologia , Humanos , Testes de Função Renal/métodos , Testes de Função Renal/tendências , Masculino , Estudos Prospectivos , Insuficiência Renal Crônica/fisiopatologia , Falha de Tratamento , Adulto Jovem
9.
J Am Coll Cardiol ; 71(9): 1009-1017, 2018 03 06.
Artigo em Inglês | MEDLINE | ID: mdl-29495980

RESUMO

BACKGROUND: Long-term outcomes of Fontan patients who survive to age ≥16 years have not been well characterized. The Australian and New Zealand Fontan Registry (ANZFR) provides a unique opportunity to understand survival and complication rates in Fontan patients who transition to adult congenital heart disease centers. OBJECTIVES: This study sought to describe the survival and complications of adult patients who have had a Fontan procedure. METHODS: The study analyzed outcomes in patients ≥16 years of age who were prospectively enrolled in the ANZFR. RESULTS: Data from all 683 adult survivors from the ANZFR were analyzed. Mortality status was confirmed from the National Death Index. There were 201 atriopulmonary (AP) connections and 482 total cavopulmonary connections (249 lateral tunnels and 233 extracardiac conduits). For these subjects, the survival rate at age 30 years was 90% (95% CI: 87% to 93%), and it was 80% (95% CI: 75% to 87%) at 40 years of age. Survival at age 30 years was significantly worse for the patients with AP connections (p = 0.03). At latest follow-up, only 53% of patients were in New York Heart Association functional class I. After the age of 16 years, 136 (20%) had experienced at least 1 new arrhythmia, 42 (6%) required a permanent pacemaker, 45 (7%) had a thromboembolic event, and 135 (21%) required a surgical reintervention. Only 41% (95% CI: 33% to 51%) of Fontan patients were free of serious adverse events at 40 years of age. CONCLUSIONS: This comprehensively followed cohort showed that a variety of morbid complications is common in Fontan adults, and that there is a substantial incidence of premature death, particularly in patients with AP connections.


Assuntos
Técnica de Fontan/tendências , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias/mortalidade , Adolescente , Adulto , Idoso , Austrália/epidemiologia , Estudos de Coortes , Feminino , Seguimentos , Técnica de Fontan/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Nova Zelândia/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Sistema de Registros , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Resultado do Tratamento , Adulto Jovem
10.
Int J Cardiol ; 258: 299-304, 2018 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-29433966

RESUMO

BACKGROUND: The relationship between temporal progression of magnetic resonance elastography derived liver stiffness (MRE-LS) and progression of Fontan associated liver disease (FALD) is unknown. To assess this relationship, we hypothesized that progression of MRE-LS correlated with progression FALD severity and clinical outcomes. METHODS: Retrospective review of Fontan patients who had >1 liver MRE, 2010-2016. Annual change in MRE-LS was the quotient of the difference between baseline and subsequent MRE-LS, and the interval between scans. RESULTS: 22 patients were enrolled; median age 29(19-38) years, 14 (64%) males and 10 (46%) with atriopulmonary Fontan. Baseline and subsequent MRE-LS values were 5.4 ±â€¯1.1 kPa and 5.8 ±â€¯0.9 kPa" for clarity, interval between scans was 25 ±â€¯5 months, and annual change in MRE-LS was 0.3 ±â€¯0.2 kPa. Temporal change in MRE-LS correlated with temporal changes in model for end-stage liver disease (MELD) score (r = 0.84, p < 0.001) and model for end-stage liver disease excluding international normalized ratio (MELD-XI) score (r = 0.75, p = 0.001). The study cohort was divided into 2 groups using the mean annual change in MRE-LS as the cut point. Groups A and B comprised of patients with annual increase in MRE-LS ≥0.3 kPa (n = 6) and <0.3 kPa (n = 16) respectively. Composite adverse event endpoint (death, heart-liver transplant listing, palliative care, hospitalization, paracentesis) was more common in Group A (4 of 6, 67%) compared to Group B (3 of 16, 19%), p = 0.13 although this did not reach statistical significance due to small sample size. CONCLUSIONS: Progression of MRE-LS correlated with clinical deterioration as measured by worsening liver disease severity scores and the occurrence of adverse events.


Assuntos
Progressão da Doença , Técnicas de Imagem por Elasticidade/tendências , Doença Hepática Terminal/diagnóstico por imagem , Técnica de Fontan/tendências , Adulto , Criança , Pré-Escolar , Doença Hepática Terminal/epidemiologia , Doença Hepática Terminal/fisiopatologia , Feminino , Humanos , Cirrose Hepática/diagnóstico por imagem , Cirrose Hepática/fisiopatologia , Estudos Longitudinais , Masculino , Estudos Retrospectivos , Fatores de Tempo , Adulto Jovem
11.
Int J Cardiol ; 259: 71-75, 2018 05 15.
Artigo em Inglês | MEDLINE | ID: mdl-29472024

RESUMO

BACKGROUND: Echocardiographic assessment of diastolic function in the setting of Fontan physiology is not well validated. We recently demonstrated that atrioventricular systolic to diastolic duration ratio (AVV S/D ratio) independently predicts mortality in Fontan-adults and that a value >1.1 was associated with poor prognosis. PURPOSE: To correlate echocardiographic measures of diastolic function with direct measurement of ventricular end-diastolic pressure (VEDP). METHODS: A retrospective analysis was undertaken of Fontan-adults who had transthoracic echocardiography (TTE) within 12 months of direct measurement of VEDP during cardiac catheterisation. RESULTS: Fifteen Fontan adults (3 males, mean age 29 ±â€¯9 years) were evaluated. Thirteen patients had dominant morphologic left ventricle and 2 had morphologic right ventricle. Four had atriopulmonary connection and 11 had total cavopulmonary connection. Twelve patients were NYHA Class I/II and 3 were Class III. Time between TTE and cardiac catheter was 46 ±â€¯113 days; VEDP was 8 ±â€¯5 mmHg. Ten patients had preserved ventricular function, 3 had mild and 2 had moderate systolic impairment by subjective TTE assessment. AVV S/D ratio had the strongest correlation with VEDP (r = 0.8, p = 0.001). AVV S/D ratio ≥ 1.1 had 100% positive predictive value and 92% negative predictive value for detecting VEDP >10 mmHg. The only conventional echocardiographic measure of diastolic function that correlated with VEDP was pulmonary vein A wave - atrioventricular A wave duration difference (r = 0.8, p = 0.02). CONCLUSIONS: TTE measures reflect VEDP in adults with a Fontan circulation. AVV S/D ratio is a simple parameter yet to enter standard practice that can be used to identify elevated VEDP.


Assuntos
Ecocardiografia/normas , Técnica de Fontan/tendências , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Volume Sistólico/fisiologia , Pressão Ventricular/fisiologia , Adulto , Ecocardiografia/métodos , Teste de Esforço/métodos , Teste de Esforço/normas , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Estudos Retrospectivos , Adulto Jovem
12.
Int J Cardiol ; 255: 32-36, 2018 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-29338918

RESUMO

BACKGROUND: Pulmonary artery wedge pressure (PAWP) has been shown to correlate better with left atrial pressure (LAP) than ventricular end-diastolic pressure (VEDP) in acquired heart disease. The correlation between VEDP and PAWP and their performance as surrogates for LAP in Fontan patients is unknown. METHODS: Offline single-beat simultaneous measurement of PAWP and VEDP was performed in 50 adult Fontan patients and non-simultaneous hemodynamic data abstracted for calculation of pulmonary vascular resistance (PVR). For the evaluation of PAWP and VEDP as surrogates for LAP, 14 fenestrated adult Fontan patients were included. RESULTS: Mean age was 34.2±10years and 54% of patients were female. Tricuspid atresia and double inlet left ventricle were the most common congenital defects (44% and 20%, respectively). Simultaneous mean VEDP was 10.8±4.6mmHg and mean PAWP was 11±4.6mmHg; the PAWP-VEDP correlation was 0.91 (p<0.001). Using non-simultaneous data, right-sided (mean difference 0.6WU·m2, 95% CI 0.2-1.0; p=0.005) and left-sided (mean difference 0.5WU·m2, 95% CI 0.1-0.9; p=0.02) PVRs were significantly higher when PAWP rather than VEDP was used. In fenestrated patients, LAP-right PAWP and LAP-left PAWP correlations were 0.97 and 0.95 (p<0.0001 for both), respectively, whereas the correlation between LAP-VEDP was 0.76 (p=0.007). CONCLUSIONS: PAWP and VEDP correlate reasonably well in adult Fontan patients but PAWP is a better surrogate for LAP. The use of VEDP instead of PAWP appears to significantly underestimate PVR in these patients.


Assuntos
Função do Átrio Esquerdo/fisiologia , Técnica de Fontan/tendências , Pressão Propulsora Pulmonar/fisiologia , Volume Sistólico/fisiologia , Adulto , Cateterismo Cardíaco/métodos , Feminino , Humanos , Masculino , Estudos Retrospectivos
13.
Int J Cardiol ; 254: 96-100, 2018 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-29229372

RESUMO

BACKGROUND/OBJECTIVES: Although echocardiographic-Doppler cardiac index (CI) assessment is widely used to guide heart failure management in patients with biventricular circulation, this application has not been studied in the Fontan population. The objective of this study was to: (1) determine the correlation between Doppler and cardiac catheterization CI calculation; (2) determine the association between Doppler CI and the occurrence of Fontan failure. METHODS: Retrospective review of adult Fontan patients followed at Mayo Clinic Adult Congenital Heart Disease program, 1994-2015. Inclusion criteria were: systemic left ventricle and echocardiogram and cardiac catheterization performed within the same week. Fontan failure was defined as a composite of all-cause mortality, heart transplantation listing, and palliative care. RESULTS: 59 patients (age 29±6years; men 32[54%]) underwent 97 studies. Of the 59, 41[69%] had atriopulmonary Fontan and 12 (20%) had cirrhosis. Compared to patients without cirrhosis, patients with cirrhosis had higher Doppler CI (3.6±0.6 vs 2.8±0.4L/min∗m2, p=0.039); Fick CI (3.3 [2.5-3.7] vs 2.4 [1.6-3.1] L/min/m2, p=0.028); lower systemic vascular resistance (20±3 vs 25±4 WU∗m2, p=0.04). There was a positive correlation between Doppler and Fick CI (r=0.52; p<0.0001). Fontan failure occurred in 13 patients (22%) within 7.5±2.1years. In patients without cirrhosis, Fick CI and Doppler CI <2.5L/min/m2 were associated with Fontan failure (odds ratio [OR] 1.58, p=0.046) and (OR 1.43, p=0.051) respectively. CONCLUSIONS: Doppler CI assessment in feasible in a selected group of Fontan patients and it is predictive of clinical outcomes. The application of this concept in systemic right ventricles deserves further research.


Assuntos
Cateterismo Cardíaco/tendências , Ecocardiografia Doppler/tendências , Técnica de Fontan/tendências , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Hemodinâmica/fisiologia , Adulto , Cateterismo Cardíaco/mortalidade , Estudos de Coortes , Ecocardiografia Doppler/mortalidade , Feminino , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/mortalidade , Humanos , Masculino , Mortalidade/tendências , Estudos Retrospectivos , Volume Sistólico/fisiologia , Falha de Tratamento
14.
Int J Cardiol ; 249: 166-168, 2017 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-28886927

RESUMO

BACKGROUND: Cardiac resynchronisation therapy (CRT) is a well-recognised treatment in systolic heart failure. There is limited evidence in congenital patients with univentricular hearts or systemic right ventricles. In 2014 PACES/HRS published a consensus statement recommending CRT if ventricular ejection fraction (EF)≤35%, QRS duration≥150ms (with RBBB in systemic RV), NYHA II-IV and ventricular dilatation. The incidence of patients meeting these criteria in whom CRT is possible is not known. METHODS: Retrospective analysis of 203 patients with a univentricular Fontan circulation and 55 patients with ccTGA under specialist ACHD care. RESULTS: Univentricular functional data was available for 194 (96%), 10 (5%) having EF≤35%. QRS duration was available for 190 (94%) and was ≥150ms in five (3%). EF data was available for 54 (98%) ccTGA patients, and was ≤35% in 6 (11%). QRS duration was ≥150ms in 13 (26%). Only four patients fulfilled recommendations and two received CRT. CONCLUSIONS: Only a small proportion of patients with single ventricles or ccTGA meet the criteria for CRT. In many of these patients there are significant anatomical barriers to CRT which limit its use in this population. The decision to implant CRT in complex ACHD requires discussion in a combined ACHD electrophysiology surgical multidisciplinary meeting and close collaboration with patients.


Assuntos
Terapia de Ressincronização Cardíaca/métodos , Técnica de Fontan , Ventrículos do Coração/anormalidades , Transposição dos Grandes Vasos/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia de Ressincronização Cardíaca/tendências , Feminino , Técnica de Fontan/tendências , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/fisiopatologia , Adulto Jovem
15.
Int J Cardiol ; 240: 178-182, 2017 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-28456482

RESUMO

BACKGROUND: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed. METHODS: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions. Inpatient costing records from the birth year through to Fontan completion were available for 156 patients. The most frequent primary diagnoses were hypoplastic left heart syndrome (33%), double inlet left ventricle (13%), and tricuspid atresia (12%). RESULTS: During the staged surgical treatment period, children had a mean of 10±6 inpatient admissions and spent 85±64days in hospital. Among these admissions, 3±5 were for non-cardiac conditions, totaling 21±41 inpatient days. Whilst cardiac surgeries were the major reason for resource use (77% of the total cost), other cardiac care that is not surgical contributed 5% and non-cardiac admissions 18% of the total cost. The three most prevalent non-cardiac diagnostic admission categories were 'Respiratory system', 'Digestive system', and 'Ear, nose, mouth and throat', affecting 28%, 21% and 34% of the patients respectively. Multivariate regression estimated that admissions for each of these categories resulted in an increased cost of $34,563 (P=0.08), $52,438 (P=0.05) and $10,525 (P=0.53) per patient respectively for the staged surgical treatment period. CONCLUSIONS: Non-cardiac admissions for single-ventricle patients are common and have substantial resource implications. Further research assessing the causes of admission and extent to which admissions are preventable is warranted.


Assuntos
Técnica de Fontan/economia , Custos Hospitalares , Hospitalização/economia , Síndrome do Coração Esquerdo Hipoplásico/economia , Atresia Tricúspide/economia , Austrália/epidemiologia , Criança , Pré-Escolar , Comorbidade , Bases de Dados Factuais/tendências , Feminino , Técnica de Fontan/tendências , Custos Hospitalares/tendências , Hospitalização/tendências , Humanos , Síndrome do Coração Esquerdo Hipoplásico/epidemiologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Masculino , Nova Zelândia/epidemiologia , Sistema de Registros , Atresia Tricúspide/epidemiologia , Atresia Tricúspide/cirurgia
16.
Int J Cardiol ; 240: 172-177, 2017 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-28461021

RESUMO

BACKGROUND: Patients with single ventricle (SV) circulations are at risk for ventricular dysfunction. This study investigates whether there is evidence of increased myocardial fibrosis and myocardial dysfunction in children after the Fontan operation. METHODS: Consecutive children after the Fontan operation who underwent cardiac magnetic resonance (CMR) T1 relaxometry with a modified look-locker inversion recovery approach were included in this retrospective study. Native T1 times (T1) and extracellular volume fractions (ECV) in the free wall of the dominant ventricle (left, SLV; right, SRV) were compared with controls and correlated with hemodynamic and clinical parameters. RESULTS: Twenty-one SV patients (9.7±4.6years; 13 SLV; 8 SRV) and 24 healthy control children (13.9±2.6years, p=0.002) were included. T1 and ECV were higher in SRV patients than in controls (1036±46ms vs 974±27ms, p<0.001; 28±4% vs 22±3%, p=0.002) and SLV patients (978±39ms, p=0.002; 23±5%, p=0.012) while there was no difference between SLV patients and controls. Age at bidirectional cavopulmonary connection was correlated with T1 (R=0.55, p=0.015), while systolic blood pressure (R=-0.68, p<0.001) and body weight (R=-0.54, p=0.012) inversely correlated with ECV. T1 negatively correlated with radial and circumferential strain by CMR feature tracking. CONCLUSIONS: Fontan patients with a SRV show increased CMR markers of diffuse myocardial fibrosis, which are associated with decreased myocardial contractility. Whether their increased fibrosis burden conveys a greater risk for long-term complications in this population remains to be investigated.


Assuntos
Técnica de Fontan/tendências , Atresia Tricúspide/diagnóstico por imagem , Atresia Tricúspide/cirurgia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/cirurgia , Remodelação Ventricular/fisiologia , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Contração Miocárdica/fisiologia , Estudos Retrospectivos , Fatores de Risco , Atresia Tricúspide/fisiopatologia
17.
Int J Cardiol ; 245: 131-134, 2017 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-28532983

RESUMO

A growing population of patients are now surviving the Fontan procedure and face an uncertain future and a clear burden of disease. The best medical therapy and follow-up modalities for these patients have not yet been identified, indications for transplantation are unclear and we have not been successful in mechanically supporting their circulation. There has only been a limited number of trials to address these issues. An international group has now been created to launch the large scale multi-centric research needed to provide the best future to this growing population: the International Fontan Interest Group (I-FIG). Its mission is to "better understand the Fontan circulation, and improve treatment and long-term outcomes of the patients who have undergone the Fontan procedure". We intend to build a large platform which will attract researchers of other specialties such as gastro-enterology, neurology and psychology and reach in the field of genomics, molecular biology, pharmacology and bio-informatics.


Assuntos
Pesquisa Biomédica/tendências , Técnica de Fontan/tendências , Metas , Internacionalidade , Colaboração Intersetorial , Qualidade de Vida , Congressos como Assunto/tendências , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/cirurgia , Humanos , Resultado do Tratamento
18.
Int J Cardiol ; 236: 132-137, 2017 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-28132780

RESUMO

BACKGROUND: The Fontan palliation is associated with numerous complications during long-term. The Fontan conversion operation has been advocated as an option to avoid some of these problems by converting classical Fontan types to modern forms of the circulation. Early mortality of Fontan conversion, however, remains unclear as available reports include limited numbers of patients and the results are heterogeneous. METHODS: We reviewed all original articles from 1994 to 2016 reporting Fontan conversion operations. Reports were analysed with specific reference to patient demographics, patient number, concomitant arrhythmia surgery, pacemaker implantation and early mortality. RESULTS: Overall, 37 Fontan conversion studies with a total of 1182 patients were analysed, including 35 single-centre studies and 2 registers. In the 35 single-centre studies the average age at the time of conversion was 21.6years (range 10.2-30.9years). Concomitant arrhythmia operation was performed in 71.6% of patients and concomitant pacemaker implantation procedure was performed in 59.3% of patients. Early mortality varied greatly between publications ranging from 0 to 21%. Based on a random and a fixed effect model mean mortality was 5.3% and 6.2%, respectively. Lower mortality was observed in series including younger patients at the time of conversion (average age<20years, 4.6%) and in the highest volume centre (1.4%). CONCLUSION: Fontan conversion carries a substantial mortality risk. However, results vary between centres. Overall, the combination with arrhythmia surgery seems to be associated with lower early mortality especially when patients are referred at an earlier age and are treated at highly experienced centres.


Assuntos
Técnica de Fontan/mortalidade , Técnica de Fontan/tendências , Hospitais com Alto Volume de Atendimentos/tendências , Hospitais com Baixo Volume de Atendimentos/tendências , Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Mortalidade/tendências , Resultado do Tratamento , Adulto Jovem
19.
Int J Cardiol ; 227: 691-697, 2017 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-27816303

RESUMO

BACKGROUND: Conceptually the right ventricle (RV) is less suitable to support the Fontan circulation than the left (LV). After palliation of hypoplastic left heart syndrome (HLHS) involving aortic reconstruction during the Norwood procedure the RV is exposed to abnormal afterload. We studied ventricular function and ventriculo-arterial coupling in HLHS patients (RV) and Fontan patients with single LV morphology that did (LV+N) and did not (LV-N) undergo Norwood-type aortic reconstruction. METHODS: Eighty patients (55 RV, 8 LV+N, 17 LV- N) were simultaneously studied with the conductance-catheter and echocardiography 4.8 (0.9-22.9)years after Fontan completion. RESULTS: Ejection fraction (EF) was lowest in the HLHS group (RV 60.9±11.0 vs. LV+N 68.4±10.5 vs. LV-N 69.7±8.0, P=0.003) whereas end systolic elastance (Ees), i.e. ventricular contractility, and end diastolic stiffness (Eed) were highest (Ees: RV 3.38±2.2 vs. LV+N 2.3.±13.8 vs. LV-N 1.92±1.37mmHg/ml, P=0.02; Eed: RV 0.59±0.36 vs. LV+N 0.48±0.29 vs. LV-N 0.32±0.17mmHg/ml, P<0.02). Arterial elastance, a measure of afterload, was highest in HLHS patients and correlated positively with Ees and Eed and inversely with EF in the study cohort. Only long axis function analysis suggested superior ventricular function in HLHS patients whereas all other echocardiographic measures did not reveal any group differences. CONCLUSION: Ventricular contractility of the RV of HLHS patients is higher than that of the ventricle of Fontan patients with LV morphology. This likely reflects a physiological response to higher arterial elastance resulting from aortic arch reconstruction. Increased arterial elastance negatively impacts diastolic stiffness, which is higher in the systemic RV than LV.


Assuntos
Técnica de Fontan/tendências , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Função Ventricular Esquerda/fisiologia , Adolescente , Adulto , Cateterismo Cardíaco/tendências , Criança , Pré-Escolar , Feminino , Seguimentos , Ventrículos do Coração/cirurgia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Masculino , Procedimentos de Norwood/tendências , Estudos Prospectivos , Adulto Jovem
20.
Int J Cardiol ; 227: 625-630, 2017 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-27810293

RESUMO

BACKGROUND: Aerobic capacity (VO2 max) of patients with Fontan circulation (FC) is lowest within patients with congenital heart disease. The reasons have not been completely elucidated. METHODS: Twenty five young patients with non-failing FC underwent a cardiopulmonary test during an upright ramp cycling. By using a signal morphology impedance cardiography device (physioflow®), stroke volume (SV) was evaluated along with effort. The results were compared with paired healthy controls. RESULTS: FC patients had lower VO2 max (24 vs 32ml/Kg/min) and maximal cardiac index (CI) (6.4 vs 9.9l/min/m2) than controls, due to impaired maximal SV (42 vs 54ml/m2) and maximal Heart Rate (HR) (154 vs 184/min) (p<0.001). No correlation between SV and HR at peak was found. At ventilatory threshold, SV continued to rise in a part of FC patients. Other FC patients showed an almost a "plateau" as in controls. The more maximal CI was impaired, the more was maximal arterio-venous difference (r=-0.6, p=0.001). Compared to controls, stroke work was lower in FC patients (p<0.01) even though maximal vascular resistance was higher in them (p<0.001). CONCLUSION: Impaired SV and chronotropic incompetence are both independently responsible for impaired CI at peak. The increase in arteriovenous difference appeared to be an adaptive response. As the stroke work was low among FC patients, high systemic vascular resistance does not appear to be the cause of SV impairment but rather a consequence. SV monitoring at effort evidences heterogeneous SV profiles among FC patients that could be considered for the management of patients.


Assuntos
Teste de Esforço/métodos , Técnica de Fontan/tendências , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Volume Sistólico/fisiologia , Adolescente , Criança , Teste de Esforço/estatística & dados numéricos , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Masculino , Consumo de Oxigênio/fisiologia , Estudos Retrospectivos
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