Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 43
Filtrar
1.
Int J Cardiol ; 407: 132027, 2024 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-38583591

RESUMO

BACKGROUND: In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions. OBJECTIVES: We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention. METHODS: TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention. The following outcome parameters were included: predicted (%) peak oxygen uptake (peak VO2), tricuspid annular plane systolic excursion (TAPSE), tricuspid Lateral Annular Systolic Velocity (TV S'), right ventricle (RV)-arterial coupling (defined as TAPSE/RV systolic pressure ratio), and N-terminal proBNP (NT-proBNP). RESULTS: 447 TGA patients with a mean age of 25.0 (interquartile range (IQR) 21-29) years were included. Patients without previous RVOT intervention (n = 338, 76%) had a significantly higher predicted peak VO2 (78.0 ± 17.4%) compared to patients with single approach catheter-based RVOT intervention (73.7 ± 12.7%), single approach surgical RVOT intervention (73.8 ± 28.1%), and patients with multiple approach RVOT intervention (66.2 ± 14.0%, p = 0.021). RV-arterial coupling was found to be significantly lower in patients with prior catheter-based and/or surgical RVOT intervention compared to patients without any RVOT intervention (p = 0.029). CONCLUSIONS: TGA patients after a successful arterial switch repair have a decreased exercise capacity. A considerable amount of TGA patients with either catheter or surgical RVOT intervention perform significantly worse compared to patients without RVOT interventions.


Assuntos
Transposição dos Grandes Vasos , Humanos , Masculino , Feminino , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/fisiopatologia , Adulto , Adulto Jovem , Europa (Continente)/epidemiologia , Obstrução do Fluxo Ventricular Externo/cirurgia , Obstrução do Fluxo Ventricular Externo/fisiopatologia , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Transposição das Grandes Artérias/métodos , Transposição das Grandes Artérias/efeitos adversos , Tolerância ao Exercício/fisiologia , Teste de Esforço/métodos , Resultado do Tratamento , Função Ventricular Direita/fisiologia , Seguimentos
2.
Eur J Heart Fail ; 25(7): 1105-1114, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37264734

RESUMO

AIMS: In adults with congenital heart disease and systemic right ventricles, progressive right ventricular systolic dysfunction is common and is associated with adverse outcomes. Our aim was to assess the impact of the phosphodiesterase-5-inhibitor tadalafil on right ventricular systolic function. METHODS AND RESULTS: This was a double-blind, randomized, placebo-controlled, multicentre superiority trial (NCT03049540) involving 100 adults with systemic right ventricles (33 women, mean age: 40.7 ± 10.7 years), comparing tadalafil 20 mg once daily versus placebo (1:1 ratio). The primary endpoint was the change in right ventricular end-systolic volume after 3 years of therapy. Secondary endpoints were changes in right ventricular ejection fraction, exercise capacity and N-terminal pro-B-type natriuretic peptide concentration. Primary endpoint assessment by intention to treat analysis at 3 years of follow-up was possible in 83 patients (42 patients in the tadalafil group and 41 patients in the placebo group). No significant changes over time in right ventricular end-systolic volumes were observed in the tadalafil and the placebo group, and no significant differences between treatment groups (3.4 ml, 95% confidence interval -4.3 to 11.0, p = 0.39). No significant changes over time were observed for the pre-specified secondary endpoints for the entire study population, without differences between the tadalafil and the placebo group. CONCLUSIONS: In this trial in adults with systemic right ventricles, right ventricular systolic function, exercise capacity and neuro-hormonal activation remained stable over a 3-year follow-up period. No significant treatment effect of tadalafil was observed. Further research is needed to find effective treatment for improvement of ventricular function in adults with systemic right ventricles.


Assuntos
Insuficiência Cardíaca , Transposição dos Grandes Vasos , Adulto , Humanos , Feminino , Pessoa de Meia-Idade , Ventrículos do Coração/diagnóstico por imagem , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/tratamento farmacológico , Tadalafila/uso terapêutico , Tadalafila/farmacologia , Nucleotídeo Cíclico Fosfodiesterase do Tipo 5/farmacologia , Nucleotídeo Cíclico Fosfodiesterase do Tipo 5/uso terapêutico , Volume Sistólico , Função Ventricular Direita/fisiologia , Método Duplo-Cego
3.
Can J Cardiol ; 39(10): 1338-1345, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37116790

RESUMO

BACKGROUND: Fontan-associated liver disease is an increasing concern. Our aim was to assess prevalence and predictors of advanced liver fibrosis with a specific focus on utility of liver stiffness measurement by ultrasound transient elastography. METHODS: A total of 97 adult Fontan patients (55% male, median age: 23.1 years, interquartile range [IQR]: 18.7-30.6); 92 (95%) were evaluated with transient elastography, and 50 (52%) underwent transjugular liver biopsy. Advanced liver fibrosis was defined as congestive hepatic fibrosis score 3 or 4. RESULTS: Only 4 patients (4%) had liver stiffness values < 10 kilopascal (kPa). Liver-stiffness measurements correlated weakly with peak oxygen uptake on exercise testing and Fontan pressure but not with Model for End-Stage Liver Disease excluding INR (MELD-XI) score or spleen size. Serial follow-up liver stiffness measurements in 73 clinically stable patients showed large variability among individual patients. Advanced liver fibrosis was present in 35 of 50 (70%) patients on liver biopsy and was associated to MELD-XI-Score ≥ 11 and splenomegaly but not to liver-stiffness measurements. Advanced liver fibrosis was not associated with patient age or time since Fontan operation but with younger age at completion of Fontan (3.7 years, IQR: 2.3-6.3 vs 6.8 years; IQR: 3.5-12.1; P = 0.037). CONCLUSIONS: In our cohort, advanced liver fibrosis was present in the majority of adult Fontan patients. Liver stiffness as measured by transient elastography was not associated with the degree of liver fibrosis. Because of its high variability on serial measurements, it seems not to be useful for clinical decision making. The unexpected finding that younger age at completion of Fontan was associated with advanced liver fibrosis merits further evaluation.

4.
Artigo em Inglês | MEDLINE | ID: mdl-36802249

RESUMO

Leakage of bone cement is a known complication after percutaneous kyphoplasty. In rare cases, bone cement can reach the venous system and cause life-threatening embolism. We present the case of a 73-year-old male, who was admitted to our hospital with new-onset chest pain and dyspnoea. He had a history of percutaneous kyphoplasty. Multimodal imaging showed intracardiac cement embolism in the right ventricle with penetration of the interventricular septum and perforation of the apex. The bone cement was successfully removed during open cardiac surgery.

5.
J Am Coll Cardiol ; 81(4): 374-389, 2023 01 31.
Artigo em Inglês | MEDLINE | ID: mdl-36697138

RESUMO

BACKGROUND: The optimal strategy for thromboprophylaxis in patients with a Fontan circulation is unknown. OBJECTIVES: The aim of this study was to compare the efficacy and safety of aspirin, warfarin, and nonvitamin K oral anticoagulants (NOACs) in a network meta-analysis. METHODS: Relevant studies published by February 2022 were included. The primary efficacy outcome was thromboembolic events; major bleeding was a secondary safety outcome. Frequentist network meta-analyses were conducted to estimate the incidence rate ratios (IRRs) of both outcomes. Ranking of treatments was performed based on probability (P) score. RESULTS: A total of 21 studies were included (26,546 patient-years). When compared with no thromboprophylaxis, NOAC (IRR: 0.11; 95% CI: 0.03-0.40), warfarin (IRR: 0.23; 95% CI: 0.14-0.37), and aspirin (IRR: 0.24; 95% CI: 0.15-0.39) were all associated with significantly lower rates of thromboembolic events. However, the network meta-analysis revealed no significant differences in the rates of major bleeding (NOAC: IRR: 1.45 [95% CI: 0.28-7.43]; warfarin: IRR: 1.38 [95% CI: 0.41-4.69]; and aspirin: IRR: 0.72 [95% CI: 0.20-2.58]). Rankings, which simultaneously analyze competing interventions, suggested that NOACs have the highest P score to prevent thromboembolic events (P score 0.921), followed by warfarin (P score 0.582), aspirin (P score 0.498), and no thromboprophylaxis (P score 0.001). Aspirin tended to have the most favorable overall profile. CONCLUSIONS: Aspirin, warfarin, and NOAC are associated with lower risk of thromboembolic events. Recognizing the limited number of patients and heterogeneity of studies using NOACs, the results support the safety and efficacy of NOACs in patients with a Fontan circulation.


Assuntos
Fibrilação Atrial , Técnica de Fontan , Acidente Vascular Cerebral , Tromboembolia , Humanos , Varfarina/efeitos adversos , Anticoagulantes/efeitos adversos , Técnica de Fontan/efeitos adversos , Técnica de Fontan/métodos , Administração Oral , Hemorragia/epidemiologia , Tromboembolia/epidemiologia , Tromboembolia/etiologia , Tromboembolia/prevenção & controle , Aspirina/efeitos adversos , Fibrilação Atrial/complicações , Acidente Vascular Cerebral/prevenção & controle , Resultado do Tratamento
6.
Int J Cardiovasc Imaging ; 37(9): 2651-2660, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34052973

RESUMO

Abnormal atrial mechanics in biventricular circulations have been associated with elevated left heart filling pressures. Similar associations in the Fontan circulation are unknown. The aim of this study was to examine the relationship between atrial mechanics and invasively assessed hemodynamic parameters late after the Fontan operation. Thirty-nine Fontan patients with echocardiographic and invasive hemodynamic studies done within 48 h were included and were compared to 40 age-matched healthy controls. Atrial and ventricular strain measurements were measured offline using 2-dimensional speckle-tracking. Mean age was 10.2 ± 6.7 years and 24 (62%) were male. Atrial strain measures were lower in Fontan patients compared to healthy controls. There was no significant association between atrial strain measurements and Fontan systemic ventricular filling pressures (SVFP) as indicated by pulmonary artery occlusion pressures, direct left atrial pressure or systemic ventricular end-diastolic pressure. Global atrial strain was not correlated with segmental atrial strain in the pulmonary venous atrium. Global atrial reservoir strain was positively correlated with pulmonary vascular resistance (r = 0.508, p = 0.045). Global atrial conduit strain was positively correlated with E/A ratio of the AV valve inflow (r = 0.555, p = 0.002). Atrial and ventricular strain measurements were not significantly correlated. In patients with a Fontan, global atrial function is significantly depressed, and is uncoupled from segmental left lateral atrial function. Global as well as segmental atrial mechanics are not significantly associated with SVFPs in Fontan patients. Instead, global atrial reservoir function appears to parallel pulmonary vascular resistance.


Assuntos
Técnica de Fontan , Função do Átrio Esquerdo , Criança , Ecocardiografia , Técnica de Fontan/efeitos adversos , Humanos , Masculino , Valor Preditivo dos Testes , Pressão Ventricular
7.
J Am Heart Assoc ; 10(9): e019942, 2021 05 04.
Artigo em Inglês | MEDLINE | ID: mdl-33880928

RESUMO

Background Hepatic steatosis, caused by nonalcoholic fatty liver disease, is a leading cause of chronic liver disease. The interplay between hepatic steatosis and the development of liver disease following the Fontan procedure is not well understood. This study examined the prevalence and associations of hepatic steatosis in patients with a Fontan circulation. Methods and Results This was a single-center retrospective study of 95 patients with a Fontan circulation with liver magnetic resonance imaging performed between 2012 and 2019. The average age at magnetic resonance imaging was 21.5±8.5 years. The percent liver fat signal was determined using magnetic resonance chemical shift-encoded proton density fat fraction imaging. Hepatic steatosis was defined as liver fat ≥5% and was present in 10.5% of the cohort. The presence of hepatic steatosis was associated with higher body mass index (29±4 versus 24±6 kg/m2, P=0.006), a higher frequency of obesity (50% versus 12%, P=0.015), lower high-density lipoprotein cholesterol (35±9 versus 43±14 mg/dL, P=0.050), and greater subcutaneous fat thickness (2.6±0.7 versus 1.8±1.0 cm, P=0.043). There was no association between hepatic steatosis and cardiovascular imaging or hemodynamic variables from cardiac catheterization. Conclusions Risk factors for hepatic steatosis in patients with Fontan circulation include obesity and dyslipidemia, similar to what is seen in the general population. Fontan hemodynamics were not associated with hepatic steatosis.


Assuntos
Fígado Gorduroso/etiologia , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Fígado/patologia , Complicações Pós-Operatórias/etiologia , Adolescente , Adulto , Criança , Ecocardiografia , Fígado Gorduroso/diagnóstico , Fígado Gorduroso/epidemiologia , Feminino , Seguimentos , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Ohio/epidemiologia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Prevalência , Prognóstico , Estudos Retrospectivos , Adulto Jovem
8.
Cardiol Young ; 31(8): 1283-1289, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33504408

RESUMO

BACKGROUND: Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response. METHODS: In this prospective, double-blind, placebo-controlled, crossover trial, 14 patients with a Fontan circulation were randomised to inhalation of placebo and Treprostinil. Incremental and constant work rate exercise tests were performed to assess the effect of Treprostinil on exercise tolerance. Venous pressures were measured throughout and liver stiffness at rest and immediately after peak exercise. RESULTS: Mean age was 27.8 ± 7.9 years and 66% were females. Exercise acutely increased liver stiffness by 30% (mean shear wave speed: 2.38 ± 0.71 versus 2.89 ± 0.51 ms, p = 0.02). Peripheral venous pressures increased acutely during both incremental (12.1 ± 2.4 versus 22.6 ± 8.0 mmHg, p < 0.001) and constant work rate exercise (12.5 ± 2.5 versus 23.4 ± 5.2 mmHg, p < 0.001). Overall, Treprostinil failed to attenuate exercise-induced increases in liver stiffness. Compared with placebo, Treprostinil did not significantly impact venous pressure responses, VO2peak, nor exercise endurance times. CONCLUSIONS: Peripheral venous pressure increased acutely during exercise by an average of 88% above baseline and was not altered by administration of inhaled Treprostinil. Liver stiffness measured immediately post-exercise increased acutely by an average of 30%, with no attenuation following Treprostinil inhalation.


Assuntos
Epoprostenol , Tolerância ao Exercício , Adulto , Epoprostenol/análogos & derivados , Feminino , Humanos , Fígado , Estudos Prospectivos , Pressão Venosa , Adulto Jovem
9.
Cardiol Rev ; 29(2): 62-67, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-31934899

RESUMO

Early complete repair of tetralogy of Fallot (ToF) prior to 1 year of age has been demonstrated to be safe and has survival benefits over late repair. The age at repair of ToF affects long-term outcomes. This may largely be related to preserved, or comparatively better, myocardial health. Most studies advocate for an age of repair between 3 and 6 months and certainly below the age of 1 year. Patients with severe right ventricular outflow tract obstruction represent an exception to this rule and may require neonatal repair or surgical and catheter-based palliation before surgery. Older age at repair beyond the first birthday leads to unfavorable right ventricular remodeling with increased right ventricular stiffness and hypertrophy and is associated with increased long-term ventricular tachycardia and all-cause mortality. In this article, we review the short- and long-term benefits of early repair, with a focus on long-term morbidity. In conclusion, we emphasize the importance of myocardial health and the relationship to early repair and advocate for the use of magnetic resonance imaging in adult patients with repaired ToF to detect myocardial fibrosis.


Assuntos
Tetralogia de Fallot , Adulto , Idoso , Ventrículos do Coração , Humanos , Lactente , Recém-Nascido , Miocárdio , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Resultado do Tratamento , Remodelação Ventricular
10.
Int J Cardiol ; 322: 142-148, 2021 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-32828959

RESUMO

BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.


Assuntos
Carcinoma Hepatocelular , Técnica de Fontan , Neoplasias Hepáticas , Carcinoma Hepatocelular/diagnóstico por imagem , Carcinoma Hepatocelular/epidemiologia , Feminino , Técnica de Fontan/efeitos adversos , Humanos , Cirrose Hepática , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/epidemiologia , Masculino
11.
Curr Cardiol Rep ; 22(12): 157, 2020 10 09.
Artigo em Inglês | MEDLINE | ID: mdl-33037480

RESUMO

PURPOSE OF REVIEW: Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population. RECENT FINDINGS: Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence. Despite an improvement in understanding and recognition of EA, significant disease heterogeneity and complex treatment options continue to challenge providers, with the best outcomes achieved at expert congenital heart disease centers.


Assuntos
Anomalia de Ebstein , Cardiopatias Congênitas , Insuficiência da Valva Tricúspide , Adulto , Arritmias Cardíacas/etiologia , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/cirurgia , Humanos , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia
12.
J Am Heart Assoc ; 9(11): e015737, 2020 06 02.
Artigo em Inglês | MEDLINE | ID: mdl-32419552

RESUMO

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology. AVVR can be attributable to diverse structural and functional abnormalities at multiple levels of the valvar apparatus, as well as ventricular dysfunction and dilation. Multiple imaging modalities including recent advances in 3-dimensional echocardiography and cross-sectional imaging have been used to further understand AVVR. Surgery to address AVVR must be tailored to the underlying mechanism and the timing of surgical repair should be chosen carefully. In this review, we discuss the etiologies, treatment options, surgical timing, and outcomes of valve repair or replacement for AVVR in patients with single ventricle congenital heart disease, with a focus on those with a Fontan circulation as AVVR is associated with increased risk for Fontan failure and mortality. In-depth understanding of the current literature will help guide clinicians in their approach and management of AVVR in this population.


Assuntos
Técnica de Fontan/efeitos adversos , Doenças das Valvas Cardíacas/etiologia , Valvas Cardíacas/fisiopatologia , Hemodinâmica , Coração Univentricular/cirurgia , Anuloplastia da Valva Cardíaca , Deterioração Clínica , Progressão da Doença , Técnica de Fontan/mortalidade , Doenças das Valvas Cardíacas/mortalidade , Doenças das Valvas Cardíacas/fisiopatologia , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Valvas Cardíacas/diagnóstico por imagem , Valvas Cardíacas/cirurgia , Humanos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Coração Univentricular/mortalidade , Coração Univentricular/fisiopatologia
13.
Can J Cardiol ; 36(11): 1815-1825, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32416063

RESUMO

BACKGROUND: Patients with repaired tetralogy of Fallot (rTOF) have increased risk for mortality, sudden cardiac death, and ventricular tachycardia (VT). The aim of this systematic review and meta-analysis is to offer an updated analysis of risk factors following significant changes in surgical and perioperative management. METHODS: A meta-analysis based on the published literature between 2008 and 2018 was conducted. Endpoints were VT, cardiac mortality/VT, and all-cause mortality/VT. Studies with ≥100 patients and ≥10 events were included. RESULTS: Fifteen studies including 7218 patients (average age 27.5 years) were analyzed. Risk factors for VT included older age (per 1 year, odds ratio [OR]: 1.039; 95% confidence interval [CI]: 1.025-1.053), older age at corrective surgery (per 1 year, OR: 1.034; CI: 1.017-1.051), previous palliative shunt (OR: 3.063; CI: 1.525-6.151), number of thoracotomies (OR: 1.416; CI: 1.249-1.604), longer QRS duration (per 1 ms, OR: 1.031; CI: 1.008-1.055), and at least moderate right-ventricular dysfunction (OR: 2.160; CI_ 1.311-3.560). Additional risk factors for cardiac death/VT were previous ventriculotomy (OR: 2.269; CI: 1.226-4.198), lower left-ventricular ejection fraction (per 1%, OR: 1.049; CI: 1.029-1.071), and higher right-ventricular end diastolic volume (per 1 mL/m2, OR: 1.009; CI: 1.002-1.016). Supraventricular tachycardia/atrial fibrillation was an additional risk factor for all-cause mortality/VT (OR: 1.939; CI: 1.088-3.457). CONCLUSIONS: The study highlights the importance of preservation of biventricular systolic function on late outcomes. Ventricular function appears to have a greater impact on outcomes than the severity of pulmonary regurgitation alone in this patient population.


Assuntos
Volume Sistólico/fisiologia , Taquicardia Ventricular/etiologia , Tetralogia de Fallot/complicações , Função Ventricular Esquerda/fisiologia , Saúde Global , Humanos , Incidência , Fatores de Risco , Taxa de Sobrevida/tendências , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/fisiopatologia , Tetralogia de Fallot/fisiopatologia
14.
Cardiol Young ; 30(5): 641-648, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32248856

RESUMO

Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Using a retrospective cohort study design, we studied 73 adult Fontan patients (age 25.0 ± 8.4 years) who had a complete blood count and abdominal imaging performed. Patients with protein-losing enteropathy were excluded. Clinical data were extracted from hospital records. The mean white blood cell count was 6580 ± 220/ml with a mean lymphocyte count of 1223 ± 508/ml. Lymphopenia, defined as lymphocyte count <1000/ml, was present in 23 (32%) patients. Patients with lymphopenia had a lower total white blood cell count (5556 ± 2517 versus 7136 ± 1924/ml, p = 0.009) and a lower platelet count (162 ± 69 versus 208 ± 69 k/ml, p = 0.008). Lymphopenia was also associated with findings of portal hypertension, including splenomegaly (36 versus 14%, p = 0.04), varices (22 versus 6%, p = 0.04), and ascites (39 versus 14%, p = 0.02). Lymphopenia did not correlate with any cardiac imaging, haemodynamic or exercise testing variables. In conclusion, lymphopenia is common in adult Fontan patients and is associated with markers of portal hypertension. Larger studies are needed to better define the relationship between lymphopenia and clinical outcomes.


Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Linfopenia/etiologia , Adulto , Ascite/etiologia , Feminino , Humanos , Hipertensão Portal/etiologia , Contagem de Linfócitos , Linfopenia/sangue , Linfopenia/diagnóstico , Masculino , Contagem de Plaquetas , Prevalência , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
15.
CJC Open ; 2(2): 55-61, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32190826

RESUMO

BACKGROUND: Fontan palliation results in a chronic multisystem disorder with diminished exercise capacity and increased risk of muscle wasting. The aims of this study were to assess the feasibility of skeletal muscle mass measurements in Fontan patients undergoing magnetic resonance imaging liver surveillance to compare muscle mass with a historic control and to assess its correlation with cardiorespiratory fitness. METHODS: Skeletal muscle area (SMA) and skeletal muscle index (SMI) were measured at T12 and L3. A young, healthy historic cohort was used as a comparison group. RESULTS: Forty patients with a Fontan circulation (mean age, 25.5 ± 7.9 years; 50% were men) were included. Measurements of SMA and SMI were feasible and highly reproducible. Mean SMA and SMI were significantly lower in women compared with men at both T12 (SMA: 25.1 ± 4.9 cm2 vs 33.5 ± 8.4 cm2, P < 0.001; SMI: 9.7 ± 2.1 cm2/m2 vs 11.3 ± 2.7 cm2/m2, P = 0.045) and L3 (SMA: 121 ± 12 cm2 vs 162 ± 24 cm2, P < 0.001; SMI: 46.9 ± 7.0 cm2/m2 vs 54.5 ± 7.4 cm2/m2, P = 0.002). Mean SMI at L3 was significantly lower in the male Fontan population compared with the healthy historic cohort (54.5 ± 7.4 cm2/m2 vs 60.9 ± 7.8 cm2/m2, P < 0.001), but was similar for women (46.9 ± 7.0 cm2/m2 vs 47.5 ± 6.6 cm2/m2, P = 0.692). SMI at L3, but not at T12, was positively correlated with peak oxygen consumption, oxygen pulse, and workload. Four patients (10%) met criteria for muscle wasting in the sarcopenic range based on L3 measurements. CONCLUSIONS: Abdominal skeletal muscle mass can be reproducibly determined on surveillance liver magnetic resonance imaging scans. Muscle wasting appears to occur commonly in Fontan patients. Further research is needed to better define the value of SMI as a biomarker in the Fontan population.


CONTEXTE: L'intervention palliative de Fontan engendre une atteinte multiviscérale chronique, notamment une diminution de la capacité à l'effort et une hausse du risque d'atrophie musculaire. L'étude visait à évaluer la possibilité de mesurer la masse musculaire squelettique des patients ayant subi une intervention de Fontan et faisant l'objet d'une surveillance du foie par imagerie par résonance magnétique, afin de comparer les résultats à ceux d'une cohorte historique témoin et d'en évaluer la corrélation avec la capacité cardiorespiratoire. MÉTHODOLOGIE: La surface musculaire squelettique (SMS) et l'indice musculaire squelettique (IMS) ont été mesurés en T12 et en L3. Une cohorte historique de sujets jeunes et en santé a servi de groupe témoin. RÉSULTATS: Quarante patients ayant subi une intervention de Fontan (âge moyen, 25,5 ± 7,9 ans; 50 % des sujets étaient de sexe masculin) ont été admis dans l'étude. Il a été possible d'obtenir des mesures de la SMS et de l'IMS hautement reproductibles. Les valeurs moyennes de la SMS et de l'IMS étaient significativement plus faibles chez les femmes que chez les hommes, tant en T12 (SMS : 25,1 ± 4,9 cm2 vs 33,5 ± 8,4 cm2, p < 0,001; IMS : 9,7 ± 2,1 cm2/m2 vs 11,3 ± 2,7 cm2/m2, p = 0,045) qu'en L3 (SMS : 121 ± 12 cm2 vs 162 ± 24 cm2, p < 0,001; IMS : 46,9 ± 7,0 cm2/m2 vs 54,5 ± 7,4 cm2/m2, p = 0,002). L'IMS moyen en L3 était significativement plus bas chez les hommes ayant subi une intervention de Fontan que chez ceux de la cohorte historique de témoins en santé (54,5 ± 7,4 cm2/m2 vs 60,9 ± 7,8 cm2/m2, p < 0,001), mais comparable chez les femmes (46,9 ± 7,0 cm2/m2 vs 47,5 ± 6,6 cm2/m2, p = 0,692). L'IMS mesuré en L3 était corrélé positivement avec la consommation maximale d'oxygène, le pouls d'oxygène et la charge d'effort, ce qui n'était pas le cas de l'IMS mesuré en T12. Selon les mesures effectuées en L3, quatre patients (10 %) répondaient aux critères d'atrophie musculaire de la sarcopénie. CONCLUSIONS: La masse musculaire squelettique abdominale peut être déterminée de façon reproductible à partir des clichés d'imagerie par résonance magnétique obtenus aux fins de la surveillance du foie. Il semble que les patients ayant subi une intervention de Fontan présentent fréquemment une atrophie musculaire. Des recherches plus poussées s'imposent afin de mieux définir l'utilité de l'IMS comme biomarqueur au sein de la population des patients ayant subi une intervention de Fontan.

16.
J Clin Med ; 9(2)2020 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-32032996

RESUMO

Background: Thromboembolism (TE) and Fontan-associated liver disease (FALD) are common and lead to significant morbidity in Fontan circulations. Risk factors for TE and the potential link between TE and FALD are not well understood. The objective of this study was to evaluate the association between TE and the severity of FALD based on radiologic liver stiffness. Methods: Using a retrospective cohort study design, 85 Fontan patients (aged 27.7 ± 8.2 years) who had liver stiffness measurement were included. Multivariable logistic regression was used to determine independent associations with TE. Results: Sixteen patients (19%) had a history of TE after the Fontan procedure at a mean age of 21.4 ± 15.0 years. Patients with TE were significantly older at the time of the last evaluation (33.8 ± 11.7 vs. 26.3 ± 6.5 years, p = 0.03). Liver stiffness by MRI and ultrasound was higher in the TE group (5.1 ± 1.4 vs. 4.3 ± 1.2 kPa, p = 0.04 and 2.8 ± 0.4 vs. 2.4 ± 0.5 m/s, p = 0.04, respectively). On multivariable analysis, higher liver stiffness (odds ratio (OR): 2.12, p = 0.03) and older age (OR: 1.11, p = 0.03) were associated with TE. Conclusions: This study found an association between TE, age, and radiologic liver stiffness.

17.
Int J Cardiol ; 299: 110-115, 2020 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-31337551

RESUMO

BACKGROUND: The arterial switch operation (ASO) for repair of transposition of the great arteries (TGA) requires transection of the great arterial trunks and re-implantation of the coronary arteries into the neoaortic root resulting in cardiac sympathetic denervation which may affect myocardial blood flow (MBF) regulation. The aims of the present study were to evaluate sympathetic (re-)innervation in young adults after ASO and its impact on MBF. METHODS: Twelve patients (age 22.5 ±â€¯2.6 years) after ASO for TGA in the neonatal period and ten healthy controls (age 22.0 ±â€¯1.7 years) were included. Positron emission tomography (PET) was used for measuring cardiac sympathetic innervation with [11C]meta-hydroxyephedrine (mHED) and MBF with [15O]H2O PET at rest, during adenosine stimulation, and during sympathetic stimulation with cold pressor test. Cold pressor-induced MBF response capacity was calculated as maximal global MBF over peak rate-pressure product multiplied by 10'000. RESULTS: Global [11C]mHED uptake was significantly lower in patients compared to controls (7.0 ±â€¯2.3 versus 11.8 ±â€¯2.1%/min, p < 0.001). Global MBF was lower in patients compared to controls at rest and during adenosine-induced hyperemia (0.66 ±â€¯0.08 versus 0.82 ±â€¯0.15 ml/min/g, p = 0.005; 2.23 ±â€¯1.19 versus 3.36 ±â€¯1.04 ml/min/g, p = 0.030, respectively). Interestingly, MBF during cold pressor test did not differ between patients and controls (0.99 ±â€¯0.20 versus 1.07 ±â€¯0.16 ml/min/g, p = 0.330). However, cold pressor-induced MBF response capacity was significantly lower for patients as compared to controls (1.09 ±â€¯0.35 versus 1.44 ±â€¯0.39 ml/g/10,000 mmHg, p = 0.040). CONCLUSIONS: With only partial sympathetic re-innervation of the coronary arteries, maximal dilator capacity of the coronary microvasculature and cold pressor-induced MBF response capacity remain substantially impaired in young adults after ASO compared to healthy controls.


Assuntos
Fibras Adrenérgicas/fisiologia , Transposição das Grandes Artérias/tendências , Velocidade do Fluxo Sanguíneo/fisiologia , Circulação Coronária/fisiologia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Transposição das Grandes Artérias/métodos , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/inervação , Vasos Coronários/fisiologia , Feminino , Humanos , Masculino , Tomografia por Emissão de Pósitrons/métodos , Tomografia por Emissão de Pósitrons/tendências , Transposição dos Grandes Vasos/metabolismo , Adulto Jovem
18.
Am J Cardiol ; 124(9): 1454-1459, 2019 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-31474329

RESUMO

Fontan associated liver disease is associated with morbidity and mortality in palliated single-ventricle congenital heart disease patients. Magnetic resonance elastography (MRE) provides a quantitative assessment of liver stiffness in Fontan patients. We hypothesized that MRE liver stiffness correlates with liver enzymes, hemodynamics, portal hypertension, and Fontan failure (FF). All adult Fontan patients who had MRE between 2011 and 2018 were included. Radiologic portal hypertension was defined as splenomegaly, ascites, and/or varices. FF was defined as death, transplantation, or heart failure symptoms requiring escalation of diuretics. Seventy patients with a median age of 24.7 years and a median follow-up from MRE of 3.9 years were included. The median liver stiffness was 4.3 kPa (interquartile range [IQR]: 3.8 to 5.0 kPa). There was a weak, positive correlation between liver stiffness and Fontan pathway pressure (r = 0.34, p = 0.03). There was a moderate negative correlation of liver stiffness with ventricular ejection fraction (r = -0.52, p = 0.03). Liver stiffness was weakly positively correlated with liver transaminases and gamma glutamyl transferase. Patients with portal hypertension had higher liver stiffness compared to patients without (5.2 ± 1.3 vs 4.2 ± 0.8 kPa, p = 0.03). At MRE or during follow-up, 13 patients (19%) met definition of FF and had significantly higher liver stiffness compared to patients without FF (5.1 [IQR: 4.3 to 6.3] vs 4.2 [IQR: 3.7 to 4.7] kPa, p = 0.01). Liver stiffness above 4.5 kPa differentiated FF with a sensitivity of 77% and specificity of 77%. In conclusion, elevated MRE-derived liver stiffness is associated with worse hemodynamics, liver enzymes and clinical outcomes in Fontan patients. This measure may serve as a global imaging biomarker of Fontan health.


Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Hipertensão Portal/diagnóstico por imagem , Hepatopatias/diagnóstico por imagem , Fígado/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico por imagem , Adulto , Ascite/diagnóstico por imagem , Progressão da Doença , Técnicas de Imagem por Elasticidade , Varizes Esofágicas e Gástricas/diagnóstico por imagem , Feminino , Insuficiência Cardíaca/epidemiologia , Transplante de Coração/estatística & dados numéricos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Hepatopatias/etiologia , Imageamento por Ressonância Magnética , Masculino , Programas de Rastreamento , Mortalidade , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Esplenomegalia/diagnóstico por imagem , Falha de Tratamento , Atresia Tricúspide/cirurgia , Adulto Jovem
19.
Int J Cardiol ; 292: 258-264, 2019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-31178224

RESUMO

AIM: Amongst patients with coronary artery disease (CAD), women experience relatively worse outcomes as compared to men. Evidence to date has failed to explore unique female imaging targets as major determinants of cardiovascular risk. We sought to assess the prognostic value of epicardial (EFV) and intrathoracic fat volume (IFV) quantification in women and men with suspected and known CAD. METHODS AND RESULTS: Intrathoracic fat volume and EFV were calculated from non-contrast CT and analyzed in a propensity-matched cohort of 190 patients (95 women, mean age 62.5 ±â€¯11.3 years) undergoing myocardial perfusion imaging (MPI) and coronary computed tomography angiography (CCTA) for evaluation of CAD. IFV and EFV were significantly lower in women as compared to men (198.2 ±â€¯78.4 vs 293.2 ±â€¯114.7 cm3 and 105.6 ±â€¯48.9 vs 135.8 ±â€¯60.9 cm3, p < 0.001) and showed a strong association with coronary artery calcium score (CACS) and obstructive CAD in women (p < 0.05), but not in men. Fat volumes were not related to abnormal MPI in either population (p = NS). During a median follow-up of 2.8 years, high IFV was associated with reduced event free survival (log rank = 0.019 vs low IFV) in women, but not in men. Accordingly, a multivariate Cox regression model adjusted for cardiovascular risk factors, CACS, CCTA, and MPI findings selected IFV as a significant predictor of major adverse cardiovascular events (MACE) in women (HR 1.32, 95%CI 1.18-1.55, p = 0.001). CONCLUSION: Quantification of IFV provides incremental prognostic value for MACE in women, beyond that provided by traditional risk factors and imaging findings.


Assuntos
Tecido Adiposo/diagnóstico por imagem , Distribuição da Gordura Corporal/métodos , Doença da Artéria Coronariana/diagnóstico por imagem , Imagem de Perfusão do Miocárdio/métodos , Pericárdio/diagnóstico por imagem , Tecido Adiposo/metabolismo , Idoso , Estudos de Coortes , Angiografia por Tomografia Computadorizada/métodos , Doença da Artéria Coronariana/metabolismo , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Pericárdio/metabolismo , Prognóstico , Tomografia Computadorizada com Tomografia Computadorizada de Emissão de Fóton Único/métodos , Cavidade Torácica/diagnóstico por imagem , Cavidade Torácica/metabolismo
20.
J Nucl Cardiol ; 26(2): 561-568, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-28685251

RESUMO

BACKGROUND: To assess the functional relevance of a coronary artery stenosis, corrected coronary opacification (CCO) decrease derived from coronary computed tomography angiography (CCTA) has been proposed. The present study aims at validating CCO decrease with quantitative 13N-ammonia positron emission tomography (PET) myocardial perfusion imaging (MPI). METHODS AND RESULTS: This retrospective study consists of 39 patients who underwent hybrid CCTA/PET-MPI. From CCTA, attenuation in the coronary lumen was measured before and after a stenosis and corrected to the aorta to calculate CCO and its decrease. Relative flow reserve (RFR) was calculated by dividing the stress myocardial blood flow (MBF) of a vessel territory subtended by a stenotic coronary by the stress MBF of the reference territories without stenoses. RFR was abnormal in 11 vessel territories (27%). CCO decrease yielded a sensitivity, specificity, negative predictive value, positive predictive value, and accuracy for prediction of an abnormal RFR of 73%, 70%, 88%, 47%, and 70%, respectively. CONCLUSIONS: CCTA-derived CCO decrease has moderate diagnostic accuracy to predict an abnormal RFR in PET-MPI. However, its high negative predictive value to rule out functional relevance of a given lesion may confer clinical implications in the diagnostic work-up of patients with a coronary stenosis.


Assuntos
Angiografia por Tomografia Computadorizada , Estenose Coronária/fisiopatologia , Imagem de Perfusão do Miocárdio , Radioisótopos de Nitrogênio , Tomografia por Emissão de Pósitrons , Adulto , Idoso , Idoso de 80 Anos ou mais , Aorta/diagnóstico por imagem , Constrição Patológica , Estenose Coronária/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Curva ROC , Reprodutibilidade dos Testes , Estudos Retrospectivos
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...