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1.
Circulation ; 2024 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-39319469

RESUMO

BACKGROUND: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD). METHODS: Children with HF attributable to LVSD were randomized to sacubitril/valsartan versus enalapril to assess the efficacy and safety of sacubitril/valsartan at 52 weeks of follow-up. The primary end point of the study was to determine whether sacubitril/valsartan was superior to enalapril for the treatment of pediatric patients with HF attributable to systemic LVSD, assessed using a primary global rank end point consisting of ranking patients from worst to best on the basis of clinical events such as death, listing for urgent heart transplant, mechanical life support requirement, worsening HF, New York Heart Association (NYHA)/Ross class, Patient Global Impression of Severity (PGIS), and Pediatric Quality of Life Inventory physical functioning domain. The change from baseline to 52 weeks in NT-proBNP (N-terminal pro-B-type natriuretic peptide) was an exploratory end point. RESULTS: A total of 375 children (mean age, 8.1±5.6 years; 52% female) were randomized to sacubitril/valsartan (n=187) or enalapril (n=188). At week 52, no significant difference was observed between the 2 treatment arms in the global rank end point (Mann-Whitney probability, 0.52 [95% CI, 0.47-0.58]; Mann-Whitney odds, 0.91 [95% CI, 0.72-1.14]; P=0.42). At week 52, clinically meaningful reductions were observed in both treatment arms in NYHA/Ross, PGIS, Patient Global Impression of Change, and NT-proBNP, without significant differences between groups. Adverse events were similar between treatment arms (incidence: sacubitril/valsartan, 88.8%; enalapril, 87.8%), and the safety profile of sacubitril/valsartan was acceptable in children. CONCLUSIONS: In this study, sacubitril/valsartan did not show superiority over enalapril in the treatment of children with HF attributable to systemic LVSD using the prespecified global rank end point. However, both treatment arms showed clinically meaningful improvements over 52 weeks. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02678312.

2.
Pediatr Int ; 58(7): 662-5, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27460401

RESUMO

Propranolol, 2 mg/kg/day, is effective in the treatment of infantile hemangioma. We report the response to propranolol in infants with hemangioma at a dose of 1 mg/kg/day. Sixteen infants with newly diagnosed infantile hemangioma were given propranolol at a dose titrated from 0.5 mg/kg/day then increased to 1 or 2 mg/kg/day based on response to treatment until the lesions showed clinical stability for 3 consecutive months. Five out of 16 patients (31.2%) responded to propranolol at 1 mg/kg/day, while the remainder required 2 mg/kg/day for response. Vascular endothelial growth factor significantly decreased after treatment (median, 117.8 pg/mL; range, 35.3-468.7 pg/mL vs 59.2 pg/mL; range, 26.3-133.0 pg/mL; P = 0.016). Therefore, we recommend initiating treatment at 0.5 mg/kg/day for 2 days, then 1 mg/kg/day for 1 month. If the hemangioma has not decreased in size by 1 month follow up, the dose is subsequently increased to 2 mg/kg/day.


Assuntos
Hemangioma/tratamento farmacológico , Propranolol/administração & dosagem , Antagonistas Adrenérgicos beta/administração & dosagem , Biópsia , Relação Dose-Resposta a Droga , Feminino , Hemangioma/diagnóstico , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Pele/patologia , Neoplasias Cutâneas , Tomografia Computadorizada por Raios X , Resultado do Tratamento
3.
Jpn J Radiol ; 39(12): 1149-1158, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34181176

RESUMO

PURPOSE: The study sought to determine coronary artery diameter in congenital coronary-cameral fistula (cCCF), factors associated with coronary artery aneurysm, coronary artery changes after fistula closure, and computed tomographic (CT) findings after treatment. MATERIALS AND METHODS: We retrospectively reviewed CT findings of the cCCF for origins, terminations, fistula length, complexities, and Sakakibara classification. Coronary artery diameter was expressed as coronary artery Z score. Fistula features associated with coronary artery aneurysm were analyzed. Post-fistula closures were analyzed for coronary artery dilatation, coronary thrombosis, complete fistula closure, and fistula thrombosis. RESULTS: Twenty-five patients (median age 33 months, interquartile range, IQR 25-48) were included. Coronary feeders and terminations were frequently right coronary artery (48%) and right ventricle (56%), respectively. Fistula aneurysm occurred in 52% of cases. Mean coronary artery Z score was 13.03 ± 6.36 with a high incidence of giant coronary artery aneurysm (68%). We found no statistically significant risk factors associated with coronary artery aneurysm (p value range 0.075-0.370). Median duration of the follow-up CT after closure of the fistulas was 6.4 months (IQR 5.0-8.7). Coronary artery Z score significantly decreased by 0.82 (IQR 0.28-1.35), p = 0.006 and coronary thrombosis occurred in 23% of cases during follow-up. CONCLUSIONS: Large coronary aneurysm is common in cCCF. No characteristic feature of the fistula influencing coronary artery aneurysm is identified. There is a diminution in coronary artery Z score after fistula closure. Coronary thrombosis is a major complication after treatment.


Assuntos
Anomalias dos Vasos Coronários , Fístula Vascular , Angiografia , Pré-Escolar , Angiografia Coronária , Anomalias dos Vasos Coronários/diagnóstico por imagem , Humanos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Fístula Vascular/diagnóstico por imagem
4.
Tomography ; 7(3): 412-423, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34564298

RESUMO

BACKGROUND: The aim was to determine the effect of pulmonary artery (PA) morphology on the branch pulmonary artery-regurgitation fraction (BPA-RF), the relationship of pulmonary insufficiency (PI) to BPA-RF and PA-distensibility, and factors (BPA-RF and PA-distensibility) associated with right ventricular function (RVF) in repaired tetralogy of Fallot (rTOF). METHODS: A total of 182 rTOF patients (median age 17.1 years) were analyzed for length, angle of PA, BPA-RF, PI, and PA-distensibility, using magnetic resonance imaging. RESULTS: The left PA had a significant greater RF than the right PA (median (interquartile range)): LPA 43.1% (32.6-51.5) and RPA 35.2% (24.7-44.7), p < 0.001. The LPA was shorter with a narrower angle than the RPA (p < 0.001). The anatomy of the branch-PA was not a factor for the greater LPA-RF (odds ratio, 95% confidence interval: CI, p-value): length 0.44 (0.95-2.00), p = 0.28; angle 0.63 (0.13-2.99), p = 0.56. There was a strong positive correlation between PI and BPA-RF-coefficients (95% CI), p-value: LPA 0.78% (0.70-0.86), p < 0.001; RPA 0.78% (0.71-0.84), p < 0.001 and between BPA-RF and distensibility-coefficients (95%CI), p-value: LPA 0.73% (0.37-1.09), p < 0.001; RPA 1.63% (1.22-2.03), p < 0.001, respectively. The adjusted BPA-RF did not predict RVF, RPA (p = 0.434), LPA (p = 0.268). CONCLUSIONS: PA morphology is not a significant factor for the differential BPA-RF. The vascular wall in rTOF patients responds to chronic increased intravascular volume by increasing distensibility. BPA-RF is not a determinant of RVF.


Assuntos
Insuficiência da Valva Pulmonar , Tetralogia de Fallot , Adolescente , Humanos , Imageamento por Ressonância Magnética , Artéria Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/etiologia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Função Ventricular Direita
5.
Jpn J Radiol ; 39(8): 774-782, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33866518

RESUMO

PURPOSE: To determine the prevalence and degree of aortic dilatation (Adilatation), severity of aortic stiffness (Astiff), factors for Adilatation, and level of aortic root most sensitive to Astiff in patients with repaired tetralogy of Fallot (rTOF). MATERIALS AND METHODS: 269 patients with rTOF (mean age 14.9 ± standard deviation 5.0 years) were analyzed for Adilatation at annulus, sinus, sinotubular junction, and ascending aorta (aAo). Aortic size index was graded as Z score < 2, 2-2.99, 3-4.99 and ≥ 5. Aortic distensibility (aAdis) was categorized according to 4 aortic levels and dilatation severity. Factors for Adilatation and level of aortic root most sensitive to Astiff were analyzed. RESULTS: Sinus and aAo were the two most common sites of Adilatation, with a prevalence of 84% and 76%, respectively. A decreased aAdis was found (mean 5.38 ± 1.79 10-3 mmHg-1). aAdis only declined significantly at the sinus level (p = 0.009). Male sex, age-at-repair and aortic regurgitation were significant factors for Adilatation, with male sex as the strongest factor (odds ratio 2.94). There was a significant decline in aAdis at sinus level (p = 0.002) as Adilatation progressed. CONCLUSIONS: We observed a high prevalence of Adilatation and Astiff in patients with rTOF. Male sex is the strongest factor for Adilatation. The sinus is the most sensitive area for determining a negative aAdis effect.


Assuntos
Tetralogia de Fallot , Adolescente , Aorta/diagnóstico por imagem , Criança , Dilatação , Dilatação Patológica/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Adulto Jovem
6.
Eur J Radiol Open ; 8: 100354, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34026947

RESUMO

PURPOSE: The study sought to determine the rate of aortic expansion and correlation with somatic growth in patients with repaired tetralogy of Fallot (rTOF), and predictors for determining the annual growth rate of the aorta (Ao-AGR). METHODS: Ninety-four rTOF patients (mean age 14.5 ± 4.4 years) with two cardiac magnetic resonance tests (CMR) (median duration 52 months, interquartile range, IQR 24-71) were analyzed for aortic diameter (AoD) at the annulus, the sinus of Valsalva (SoV), the sinotubular junction, and the ascending aorta (AAo), and compared with the normal limit AoD (NL-AoD) values. The median age-at-repair was 60 months (IQR 36-84). Ao-AGR and its index (Ao-AGRI) were derived from changes of the AoD and AoD-index, respectively, divided by the duration between the two studies. Three potential predictors (baseline AoD, sex, and age-at-repair) for the progression of Ao-AGR were analyzed. RESULTS: There was a significant larger AoD than NL-AoD (p < 0.001). Slow aortic growth was encountered in 78-85 % of patients. The Ao-AGR was slow, the median AGR ranged from 0.37 mm (IQR 0.13-0.72) at annulus to 0.56 mm (IQR 0.22-0.91) at AAo. There was a regression in Ao-AGRI, ranged from -1.41 mm (IQR -1.94, -0.87) at annulus to -2.36 mm (IQR -3.09, -1.63) at SoV. The three predictors were not correlated with severity of Ao-AGR. CONCLUSION: Most adolescents with rTOF show significant aortic dilatation. There is a slow Ao-AGR with regression of Ao-AGRI, which may suggest that the rate of aortic growth is slower than the somatic growth. There are no significant predictors of the progression of Ao-AGR.

7.
J Investig Med High Impact Case Rep ; 8: 2324709620910645, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32131639

RESUMO

Actinomyces species are gram-positive, facultative anaerobic bacilli. Infection caused by Actinomyces species is usually limited to cervicofacial, thoracic, and abdominopelvic regions. Infective endocarditis due to Actinomyces species is extremely rare with only 30 reported cases since 1939. We report a case of Actinomyces oris endocarditis in a 14-year-old boy who had a 2-week history of dyspnea on exertion without other constitutional signs. Transthoracic echocardiography was suggestive of perforation of the right coronary cusp of aortic valve. No organisms were isolated from blood cultures. The patient underwent surgical valve repair due to deteriorated cardiac function. Valve tissue culture did not initially identify the organism. However, the terminal subculture in a thioglycolate broth grew gram-positive bacilli. The matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF MS) was compatible with Actinomyces oris. After 6 weeks of intravenous ampicillin, the patient remained well with improved cardiac function. We reviewed all reported cases of infective endocarditis caused by Actinomyces species, commenting on clinical characteristics and factors associated with unfavorable outcomes in infective endocarditis due to Actinomyces species. Although infective endocarditis caused by Actinomyces spp is rare, it could be considered in a case of culture-negative endocarditis since the clinical features might be indistinguishable from other bacterial endocarditis. Additionally, MALDI-TOF MS is a useful diagnostic tool for the identification of Actinomyces spp to improve the accuracy of diagnosis.


Assuntos
Actinomyces/isolamento & purificação , Actinomicose/diagnóstico , Endocardite Bacteriana/diagnóstico , Perfuração Espontânea/cirurgia , Actinomicose/tratamento farmacológico , Actinomicose/microbiologia , Adolescente , Antibacterianos/uso terapêutico , Valva Aórtica/patologia , Ecocardiografia , Endocardite Bacteriana/tratamento farmacológico , Endocardite Bacteriana/microbiologia , Humanos , Masculino , Valva Mitral/patologia , Resultado do Tratamento
8.
Southeast Asian J Trop Med Public Health ; 39(6): 1088-91, 2008 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19062700

RESUMO

There is a paucity of data regarding the treatment of endocarditis caused by penicillin-resistant viridans group streptococci (PR-VGS). We report a 16-year-old girl who had native-valve endocarditis due to PR-VGS which was identified as Streptococcus mitis. She also had unusual reactions to vancomycin. Eighteen hours after initiation of 50 mg/kg/day vancomycin, she developed a maculopapular rash, then at 48 hours she developed an intermittent high fever and a progressive decrease in peripheral leukocytes and platelets. She developed hypotension on Day 8. Her serum C-reactive protein and procalcitonin levels were high. All reactions improved after vancomycin was discontinued and oral prednisolone was started. This unusual combination of reactions to vancomycin was likely caused by immune and nonimmune mechanisms. Her endocarditis was successfully treated with cefotaxime 200 mg/kg/ day for 4 weeks.


Assuntos
Antibacterianos/efeitos adversos , Endocardite Bacteriana/tratamento farmacológico , Infecções Estreptocócicas/tratamento farmacológico , Streptococcus mitis/isolamento & purificação , Vancomicina/efeitos adversos , Adolescente , Antibacterianos/uso terapêutico , Cefotaxima/uso terapêutico , Endocardite Bacteriana/microbiologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Resistência às Penicilinas , Prednisolona/uso terapêutico , Infecções Estreptocócicas/microbiologia , Vancomicina/uso terapêutico
10.
Expert Rev Pharmacoecon Outcomes Res ; 16(4): 525-36, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26566873

RESUMO

OBJECTIVE: This study aims to compare the lifetime costs and health outcomes of both first-line and sequential combination treatments with standard treatment for pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) (PAH-CHD) patients. METHODS: A cost-utility analysis was performed using a Markov model based on a societal perspective. One-way and probabilistic sensitivity analyses were performed to investigate the effect of parameter uncertainty. RESULTS: As first-line treatments, both beraprost (incremental cost-effectiveness ratio (ICER) = 192,752 and 201,308 Thai baht (THB) per quality-adjusted life year (QALY) gained) and sildenafil (ICER = 249,770 and 226,802 THB per QALY gained) seemed cost-effective for PAH-CHD patients aged ≤30 years in functional classes II and III, respectively, while no treatment was cost-effective for the sequential combination therapy. CONCLUSIONS: Sildenafil should be included in the National Drug List of Essential Medicines as the first-line treatment for PAH-CHD, and its price per dose should be negotiated to be reduced by 43-57%.


Assuntos
Epoprostenol/análogos & derivados , Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/tratamento farmacológico , Citrato de Sildenafila/administração & dosagem , Adulto , Orçamentos , Análise Custo-Benefício , Custos de Medicamentos , Medicamentos Essenciais/economia , Epoprostenol/administração & dosagem , Epoprostenol/economia , Humanos , Hipertensão Pulmonar/economia , Hipertensão Pulmonar/etiologia , Cadeias de Markov , Anos de Vida Ajustados por Qualidade de Vida , Citrato de Sildenafila/economia , Tailândia , Vasodilatadores
11.
J Am Soc Echocardiogr ; 15(10 Pt 2): 1189-96, 2002 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-12411904

RESUMO

OBJECTIVE: Our study was intended to test the accuracy of a 3-dimensional (3D) digital color Doppler flow convergence (FC) method for assessing the effective orifice area (EOA) in a new dynamic orifice model mimicking a variety of mitral regurgitation. BACKGROUND: FC surface area methods for detecting EOA have been reported to be useful for quantifying the severity of valvular regurgitation. With our new 3D digital direct FC method, all raw velocity data are available and variable Nyquist limits can be selected for computation of direct FC surface area for computing instantaneous flow rate and temporal change of EOA. METHODS: A 7.0-MHz multiplane transesophageal probe from an ultrasound system (ATL HDI 5000) was linked and controlled by a computer workstation to provide 3D images. Three differently shaped latex orifices (zigzag, arc, and straight slit, each with cutting-edge length of 1 cm) were used to mimic the dynamic orifice of mitral regurgitation. 3D FC surface computation was performed on parallel slices through the 3D data set at aliasing velocities (14-48 cm/s) selected to maximize the regularity and minimize lateral dropout of the visualized 3D FC at 5 points per cardiac cycle. Using continuous wave velocity for each, 3D-calculated EOA was compared with EOA determined by using continuous wave Doppler and the flow rate from a reference ultrasonic flow meter. Simultaneous digital video images were also recorded to define the actual orifice size for 9 stroke volumes (15-55 mL/beat with maximum flow rates 45-182 mL/s). RESULTS: Over the 9 pulsatile flow states and 3 orifices, 3D FC EOAs (0.05-0.63 cm(2)) from different phases of the cardiac cycle in each pump setting correlated well with reference EOA (r = 0.89-0.92, SEE = 0.027-0.055cm(2)) and they also correlated well with digital video images of the actual orifice peak (r = 0.97-0.98, SEE = 0.016-0.019 cm(2)), although they were consistently smaller, as expected by the contraction coefficient. CONCLUSION: The digital 3D FC method can accurately predict flow rate, and, thus, EOA (in conjunction with continuous wave Doppler), because it allows direct FC surface measurement despite temporal variability of FC shape.


Assuntos
Velocidade do Fluxo Sanguíneo/fisiologia , Ecocardiografia Doppler em Cores , Processamento de Sinais Assistido por Computador , Diagnóstico Diferencial , Humanos , Insuficiência da Valva Mitral/diagnóstico , Insuficiência da Valva Mitral/epidemiologia , Insuficiência da Valva Mitral/fisiopatologia , Variações Dependentes do Observador , Estatística como Assunto , Volume Sistólico/fisiologia
12.
J Med Assoc Thai ; 85 Suppl 4: S1259-65, 2002 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-12549804

RESUMO

Pulmonary artery (PA) trunk or branch PA stenosis is commonly found in patients with congenital heart disease. The aim of the present study was to evaluate the freehand "Flock of Birds" color Doppler three-dimensional (3D) reconstruction on a modeled-segment imitating PA stenosis. First, a PA model was created from latex tubes to simulate the main PA and its main branches with baseline cross-sectional areas (CSA) of 0.7 cm2. A series of narrowed segments in the right and left PA were created. The cross-sections of the smallest area ranged from 0.13 to 0.59 cm2 and stenotic segmental length ranged from 0.17 to 1.80 cm. The dimensions of these elements mounted on to the model were verified by intravascular ultrasound (IVUS) imaging. Next, pulsatile flows at 60 beats/ min were generated through the system. A GE/VingMed System FiVe with magnetic locator system (Flock of Birds) on a 3.5 MHz transducer was used to acquire a freehand sweep for ECG gated 3D data acquisition of color Doppler flows through the model. The images were reconstructed by EchoPac 3D software and the morphology of the stenotic elements were determined. The results revealed that the narrowest CSA determined by the 3D color flow cast of the pulmonary artery were in excellent agreement with IVUS CSA (r = 0.98, p < 0.001, SEE = 0.04 cm2). The stenotic length estimated from 3D was also in good agreement with the IVUS (r = 0.98, p < 0.001, SEE = 0.03 cm). In addition, complex morphology of the stenosis was well visualized by this technique. As a result, the noninvasive free-hand digital color 3D echocardiography can be adopted for the accurate assessment of the severity and morphology of PA stenosis in patients with congenital heart diseases.


Assuntos
Ecocardiografia Doppler em Cores/métodos , Ecocardiografia Tridimensional/métodos , Processamento de Imagem Assistida por Computador/métodos , Artéria Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/diagnóstico por imagem , Processamento de Sinais Assistido por Computador , Humanos , Técnicas In Vitro , Modelos Cardiovasculares
13.
J Med Assoc Thai ; 85 Suppl 4: S1266-74, 2002 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-12549805

RESUMO

BACKGROUND: Surgical repair of tetralogy of Fallot (TOF) with reconstruction of the right ventricular (RV) outflow tract invariably results in pulmonary regurgitation (PR). Chronic PR has been associated with RV dysfunction and ventricular arrhythmia. Pericardial monocusp has recently been used at Ramathibodi Hospital to preserve pulmonary valve function. OBJECTIVES: First, to study the competency of the pericardial monocusp, one-year after correction. Second, to assess the right and left ventricular (LV) functions after surgery. Third, to assess correlation between severity of PR and the characters of electrocardiography (ECG) and chest X-ray (CXR) after correction. METHOD: A cross-sectional study was conducted in patients who, had undergone total correction for TOF at least one year ago. The past medical history was retrospectively reviewed from the medical records. The patients who underwent surgical correction with and without pericardial monocusp were recruited into group I and group II, respectively. The clinical symptoms, QRS duration from ECG, and cardio-thoracic (CT) ratio from CXR were analyzed. From the echocardiographic standpoint, the LV systolic function was determined by LV fractional shortening (LVFS), whereas the RV systolic function was determined by the tricuspid annular plane systolic excursion (TAPSE). Restrictive physiology of the RV was determined by presence of antegrade flow across the pulmonary valve during diastole. RESULTS: Sixty four patients were enrolled in the study, 7 in group I and 57 in group II. The median follow-up time after the surgery was 6.5 years, which was 3 years in group I and 7 years in group II (p < 0.01). All patients in group I (100%) and 45 (80.4%) in group II had moderate or severe PR. The severity of PR, the RV and LV systolic functions were not statistically significantly different between the two groups (p > 0.01). The median of the LVFS was 32.4 per cent, and of the TAPSE was 10.5 mm. There was no restrictive physiology of the RV in all patients. There were no significant correlations between symptoms, CT-ratio, QRS duration and the severity of PR. CONCLUSIONS: The pericardial monocusp could neither reduce severity of PR nor improve right and left ventricular functions after 3 years follow-up post-operatively. However, the right and left ventricular performances in mid-term period remained insignificantly changed and severity of PR could not be predicted from symptoms and simple laboratory investigations.


Assuntos
Prótese Vascular , Pericárdio/fisiopatologia , Pericárdio/cirurgia , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/cirurgia , Função Ventricular Esquerda/fisiologia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Estudos Transversais , Feminino , Seguimentos , Humanos , Masculino
14.
J Med Assoc Thai ; 85 Suppl 4: S1121-6, 2002 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-12549785

RESUMO

BACKGROUND: Kawasaki disease is an acute febrile illness recognized most often in young children. Coronary abnormality is the most serious complication preventable with intravenous immunoglobulin (IVIG) administration. Various treatment regimens of IVIG have been reported. OBJECTIVE: To determine initial treatment failure and prevalence of coronary artery abnormality (CAA) in Kawasaki disease (KD) treated with a moderate dose (1 g/kg) of intravenous immunoglobulin (IVIG). METHOD: All patients with a diagnosis of KD who had initial treatment with 1 g/kg of IVIG at Ramathibodi Hospital between 1994 and 1998 were reviewed retrospectively. RESULTS: Thirty-one of 41(76%) patients responded completely to a single treatment with a moderate dose of IVIG (group A). The second dose of 1 g/kg of IVIG was required in 7 patients (17%) due to persistent fever more than 48 hours after the initial treatment (group B), and 3 patients (7%) required 3 doses of 1 g/kg of IVIG due to persistent fever after the second dose (group C). During the convalescent phase, there were 19 per cent, 29 per cent and 100 per cent of the patients in group A, B and C, respectively who developed CAA with an overall rate of 27 per cent. After 1-year follow-up, the prevalence of CAA had decreased to 3 per cent, 0 per cent and 67 per cent in the according groups with overall rate of 9.6 per cent. Only 1 patient in group C developed a giant aneurysm of the right coronary artery. CONCLUSION: The efficacy of a moderate dose (1 g/kg) of IVIG in preventing CAA is lower than that of the high dose regimen (2 g/kg) reported previously. Short duration of fever before starting IVIG and low hemoglobin level may be the risk factors of unresponsiveness to moderate-dose IVIG.


Assuntos
Doença da Artéria Coronariana/etiologia , Doença da Artéria Coronariana/prevenção & controle , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Pré-Escolar , Relação Dose-Resposta a Droga , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos
15.
J Med Assoc Thai ; 85 Suppl 2: S648-57, 2002 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-12403243

RESUMO

UNLABELLED: Myocardial diseases are among the important causes of mortality and morbidity in children. This drew the authors attention to the study of myocardial diseases in children to find out the outcome, factors affecting the outcome, and management strategies. The authors retrospectively studied children who had been diagnosed with primary myocardial diseases at six university hospitals in Thailand from January 1996 to December 2000. The total number of cases was 209 which accounted for 1.2 per cent of cardiovascular diseases in children. The patients' ages ranged from 0.1-15 years. These myocardial diseases included dilated cardiomyopathy (DCM) 45 per cent, acute myocarditis 27.3 per cent, hypertrophic cardiomyopathy (HCM) 18.2 per cent, hypertrophic obstructive cardiomyopathy (HOCM) 8.1 per cent and restrictive cardiomyopathy (RCM) 1.4 per cent. Fifty-six per cent of the patients were female. Congestive heart failure was the most common presenting symptom (75%). Median ejection fraction (EF) of acute myocarditis was 42 per cent (15-79%) which was significantly higher than DCM (33.5%, 10-57%). Serum cardiac troponin T (cTnT) was also significantly higher in acute myocarditis than in DCM (0.08 ng/ml, 0.01-0.16 vs 0.01 ng/ml, 0.01-0.10). Within the follow-up period of 1 year (0.1-5.5 years), the mortality rates were 18.8 per cent, 17.0 per cent, 5.4 per cent and 33.3 per cent in DCM, acute myocarditis, HCM and RCM respectively. Factors associated with the mortality rate in acute myocarditis were admission to ICU and low left ventricular EF at presentation while IVIG administration and cTnT level did not. CONCLUSION: Primary myocardial diseases are uncommon. Most of the patients had compromised cardiovascular reserve. Admission to ICU and low EF were factors that affected the mortality in acute myocarditis while intravenous immunoglobulin administration did not. Mortality rate in the subacute follow-up period was high in all groups.


Assuntos
Cardiomiopatias/diagnóstico , Cardiomiopatias/epidemiologia , Adolescente , Distribuição por Idade , Cardiomiopatias/terapia , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/epidemiologia , Cardiomiopatia Dilatada/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/epidemiologia , Cardiomiopatia Hipertrófica/terapia , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Estudos Multicêntricos como Assunto , Miocardite/diagnóstico , Miocardite/epidemiologia , Miocardite/terapia , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Distribuição por Sexo , Taxa de Sobrevida , Tailândia/epidemiologia
16.
Clin Rheumatol ; 32(6): 899-904, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23483294

RESUMO

Macrophage activation syndrome (MAS) is a fatal complication in rheumatic diseases. It is characterized by prolonged fever, pancytopenia, and hepatosplenomegaly, which are consequences of uncontrolled macrophage activation. MAS in children is most commonly associated with systemic juvenile idiopathic arthritis. Its association with systemic lupus erythematosus (SLE) is relatively rare, so we report a Thai boy who initially presented with MAS and eventually was diagnosed as having SLE. He also had recurrent MAS during the course of therapy. Hyperferritinemia is one of the abnormal laboratory findings in MAS and it has been used as an inflammatory marker. However, its correlation with disease activity remains unclear. Therefore, a review of literature regarding MAS-associated SLE in children and ferritin level in this disease was carried out.


Assuntos
Lúpus Eritematoso Sistêmico/fisiopatologia , Síndrome de Ativação Macrofágica/fisiopatologia , Adolescente , Ferritinas/sangue , Humanos , Lúpus Eritematoso Sistêmico/complicações , Síndrome de Ativação Macrofágica/complicações , Masculino , Obesidade/complicações , Reprodutibilidade dos Testes , Tailândia , Fatores de Tempo
17.
Cardiol J ; 19(5): 543-6, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23042324

RESUMO

We present the cases of three children with patent ductus arteriosus (PDA), pulmonary arterial hypertension (PAH), and desaturation. One of them had desaturation associated with atrial septal defect (ASD). His ASD, PAH, and desaturation improved after successful device closure of the PDA. The other two had desaturation associated with Down syndrome. One had desaturation only at room air oxygen (21% oxygen) but well saturated with 100% oxygen, subsequently underwent successful device closure of the PDA. The other had experienced desaturation at a younger age but spontaneously recovered when he was older, following attempted device closure of the PDA, with late embolization of the device.


Assuntos
Permeabilidade do Canal Arterial/complicações , Hipertensão Pulmonar/etiologia , Oxigênio/sangue , Adolescente , Aortografia , Pressão Arterial , Cateterismo Cardíaco/instrumentação , Criança , Pré-Escolar , Síndrome de Down/complicações , Permeabilidade do Canal Arterial/sangue , Permeabilidade do Canal Arterial/diagnóstico , Permeabilidade do Canal Arterial/fisiopatologia , Permeabilidade do Canal Arterial/terapia , Hipertensão Pulmonar Primária Familiar , Comunicação Interatrial/complicações , Humanos , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Masculino , Circulação Pulmonar , Dispositivo para Oclusão Septal , Resultado do Tratamento , Resistência Vascular
18.
J Pediatr Intensive Care ; 1(3): 153-160, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31214401

RESUMO

To determine the cardiovascular changes in children with dengue shock syndrome. Echocardiography was performed in 8 children (5 females) with dengue shock syndrome, median age 6.5, 4.2-13.7 yr and weight 34, 12-66 kg. All had massive bleeding with low initial hematocrit in most cases (median 31%), thrombocytopenia (median platelet 37,000/µL), and coagulopathy with massive pleural effusion. Seven (87.5%) developed acute renal failure and hepatic failure. All patients were in either compensate or decompensate shock with alteration of consciousness, tachycardia, poor tissue perfusion, and prolonged capillary refill (>4 s) with mean arterial pressure 65, 39-94 mm Hg. The cardiac dimension was normal to low normal except one had dilated left ventricle. Seven patients had normal left ventricular systolic function (5 with inotrope infusion). One patient had impaired systolic function even with inotrope. All had normal cardiac index (4.14, 3.51-6.37 L/min/m2) with increased heart rate (141.5, 110-160/min) but low stroke volume index (30.72, 25.37-42.49 mL/m2) and low systemic vascular resistance index (1,072, 223-2,880 dyne/sec/cm-5/m2). Decreased preload from bleeding and vascular leakage into the third space play an important role in shock in Dengue. However, decreased stroke volume and low systemic vascular resistance may be additional causes of shock.

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