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1.
Pediatr Cardiol ; 2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38438790

RESUMO

Ventricular septal defect (VSD) is a common congenital heart disease. However, consensus on the utility of echocardiography in predicting spontaneous closure (SC) of VSD remains lacking. This study aimed to identify and validate significant predictors of SC through a predictive scoring system. This retrospective study included medical records of 712 echocardiography instances performed on 304 patients diagnosed with VSD from 2016 to 2020 in their first year of life. A novel scoring system for predicting the SC of VSD was developed and validated using another dataset from different hospitals. Of the 304 patients, 215 (70.7%) had perimembranous (PM) VSDs and 89 had muscular (29.3%) VSDs. The median follow-up periods were 36.2 (interquartile range [IQR], 13-59) months and 13.7 9 (IQR, 5-37.4) days for PM and muscular VSDs, respectively. The overall SC rate during follow-up was 29.3%. Pulmonary hypertension (HTN), concomitant left ventricle (LV)-right atrium (RA) shunt, VSD size to aortic valve (AV) annulus size ratio, and left ventricular end-diastolic dimension (LVEDD) z-score were significant risk factors affecting SC of VSD. The "P-VSD" score, a new scoring system, demonstrated an area under the curve for predictability of 0.769. Pulmonary HTN, concomitant LV-RA shunt, LVEDD z-score, and VSD size-to-AV annulus size ratio at diagnosis were significantly associated with non-SC VSD after infancy. The P-VSD score can predict the SC of VSD in clinical settings and simplify the identification and appropriate management of high-risk patients.

2.
J Cardiovasc Nurs ; 38(1): 52-59, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-35020708

RESUMO

BACKGROUND: As more than 85% of patients with congenital heart disease (CHD) have grown to adulthood through improvement in treatment and surgery, the difficulties they experience are expanding into areas related to daily life. Accordingly, adjustment to school in adolescents and young adults (AYAs) with CHD is of increasing interest and is influenced by familial factors. OBJECTIVE: This was a cross-sectional descriptive study to examine the effects of parental positive emotional expressiveness and sibling relationships on school adjustment of AYAs with CHD. METHODS: In this study, a self-reported questionnaire survey was used to collect the data. The participants were 104 AYAs with CHD aged 13 to 21 years who were attending school and had siblings. RESULTS: Maternal positive emotional expressiveness ( r = 0.584, P < .01), paternal positive emotional expressiveness ( r = 0.584, P < .01), and sibling warmth/closeness ( r = 0.478, P < .01) were significantly correlated with school adjustment. However, the results of multiple regression analysis showed that only maternal positive emotional expressiveness (ß = 0.459, P < .05) and sibling warmth/closeness (ß = 0.236, P < .05) were significantly associated with school adjustment. CONCLUSIONS: Adolescents and young adults with CHD who reported higher maternal positive emotional expressiveness and sibling warmth/closeness exhibited better school adjustment. Findings suggest that intervention programs to increase parental positive expressiveness and enhance warmth/closeness of sibling relationships may contribute to improving school adjustment.


Assuntos
Adaptação Psicológica , Cardiopatias Congênitas , Adolescente , Adulto Jovem , Humanos , Adulto , Estudos Transversais , Relações Familiares , Cardiopatias Congênitas/psicologia , Instituições Acadêmicas
3.
Europace ; 20(6): 1043-1049, 2018 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-29016830

RESUMO

Aims: In patients with Fontan circulation, the conduit may be punctured for electrophysiological procedures. We evaluated the feasibility and safety of a stepwise approach to conduit puncture in adults who have undergone Fontan operation. Methods and results: We included 13 consecutive patients with lateral tunnel or extracardiac conduit Fontan circulation [median age (interquartile range), 24.0 (16.0-25.0) years; seven men] who had undergone electrophysiological procedures. We performed a stepwise approach to conduit puncture: 1st, Brockenbrough needle; 2nd, Brockenbrough needle with snare; 3rd, extra-steep Brockenbrough needle with/without snare; 4th radiofrequency transseptal needle with/without snare; 5th, wiring through the puncture; 6th, conduit dilation with angioplasty balloon; 7th, non-compliant or cutting balloon; and 8th, Inoue dilator. In 12 patients, conduit puncture was successful. In two, one, and two patients with a lateral tunnel made of the pericardium or right atrial wall, conduit puncture was performed by steps 1st, 2nd, and 4th, respectively. In one, three, two, and one patient with the Goretex lateral tunnel or extracardiac conduit, conduit puncture was performed by steps 1st, 6th, 7th, and 8th, respectively. Puncture time was significantly longer in patients with Goretex conduits than with pericardial conduits [62.0 (50.0-120.0) and 11.5 (10.0-14.8) min, respectively; P < 0.001]. A snare was necessary in patients with angles ≤ 35° between the conduit wall and vertical line. Conclusion: A stepwise conduit puncture approach is feasible and safe in patients with lateral tunnel and extracardiac conduit Fontan circulation. Goretex conduit puncture was more difficult than pericardial conduit puncture.


Assuntos
Técnicas Eletrofisiológicas Cardíacas/métodos , Técnica de Fontan/métodos , Cardiopatias Congênitas , Punções/métodos , Adolescente , Adulto , Circulação Sanguínea , Estudos de Viabilidade , Feminino , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Período Pós-Operatório
4.
Pediatr Cardiol ; 39(6): 1144-1155, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29671004

RESUMO

Few hemodynamic comparison studies on various types of Fontan operation have been reported. The objective of this study was to perform hemodynamic comparisons for flow size and volume in three types of Fontan operation: atriopulmonary connection (APC), lateral tunnel (LT), and extracardiac conduit (ECC). Forty patients with Fontan operation (8 with APC Fontan, 22 with LT Fontan, and 10 with ECC Fontan) were enrolled. Velocity time integral (VTI) and average peak velocity (APV) were assessed according to cardiac and respiratory cycles in SVC, IVC, hepatic vein, conduit, LPA, and RPA using direct intravenous Doppler echocardiography. During each cardiac cycle in APC, VTI and APV between inspiration and expiration did not show significant differences in SVC, IVC, HV, LPA, or RPA. During each cardiac cycle in LT and ECC, VTI and APV between inspiration and expiration showed significant differences in all native vessels. The gap between S and D wave in APC was the highest, followed by that in LT. It was the lowest in ECC regardless of inspiration or expiration. Hepatic reverse VTI and APV in APC showed significant decreases compared to those in VC and PA during inspiration and expiration. Flow size and volume in APC were more influenced by cardiac cycle. Those in LT were moderately influenced by both respiratory cycle and cardiac cycle while those in ECC were more influenced by respiratory cycle. APC Fontan has hemodynamic inefficiency with prominent reverse flow. However, total cavopulmonary connection (TCPC) Fontan has more hemodynamic efficiency without prominent reverse flows.


Assuntos
Ecocardiografia Doppler/métodos , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Hemodinâmica/fisiologia , Adolescente , Adulto , Criança , Feminino , Coração/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Veias Hepáticas/fisiopatologia , Humanos , Fígado/fisiopatologia , Fígado/cirurgia , Masculino , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/cirurgia , Veias Cavas/fisiopatologia , Veias Cavas/cirurgia , Adulto Jovem
5.
Pediatr Cardiol ; 37(8): 1404-1408, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27394424

RESUMO

Recently, the prevalence of infant Kawasaki disease (KD) has increased. However, the myocardial functional analysis of infant KD can be difficult and rarely reported. The purpose of this study was to investigate layer specific myocardial strain analysis for better assessment of the acute period in infant KD. The study retrospectively reviewed the echocardiographic data of 25 infant patients with KD at the acute phase. With advanced imaging, pulsed tissue Doppler velocity data, myocardial strain with three layers specific analysis was performed. Then the data were compared with 25 age-matched healthy control infants. The measures of longitudinal strain and radial strain were decreased in infant KD compared to healthy controls. The circumferential strain was significantly decreased in infant KD at all three myocardial layers, especially in the endocardial layer (KD: -20.5 ± 6.4 % vs. CONTROL: -25.6 ± 7.6 %, endocardium, p = 0.00001; -14.6 ± 4.4 % vs. -18.1 ± 4.0 %; middle myocardium, p = 0.01; -9.7 ± 3.3 % vs. -11.4 ± 3.8 %; epicardium, p = 0.04). The acute phase of infant KD demonstrated decreased myocardial strain measurement. Circumferential strain was the lowest in the endocardial layer. Further continuous long-term follow up for myocardial assessment should be recommended even after recovery with appropriate treatment.


Assuntos
Síndrome de Linfonodos Mucocutâneos , Ecocardiografia , Endocárdio , Humanos , Lactente , Miocárdio , Pericárdio
6.
Pediatr Int ; 57(4): 706-8, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26315201

RESUMO

Erythromelalgia is a rare clinical syndrome characterized by erythema, increased temperature, and severe burning pain that can be aggravated by warmth or relieved by cold. Erythromelalgia occurs either as a primary, idiopathic form, or secondary to a number of diseases and conditions. Although fairly well studied in adults, the characteristics, pathogenesis, and natural history are poorly characterized in the pediatric age group. Different therapeutic options have been tried, but no optimal treatment has been suggested for erythromelalgia. We report a rare case of linear erythromelalgia in a 12-year-old girl involving her central body from the peripheral extremities, which seemed to be secondary due to vasculitis. Clinical progress waxed and waned on maintenance aspirin and propranolol.


Assuntos
Aspirina/uso terapêutico , Eritromelalgia/diagnóstico , Dor/etiologia , Propranolol/uso terapêutico , Vasculite/complicações , Antagonistas Adrenérgicos beta/uso terapêutico , Anti-Inflamatórios não Esteroides/uso terapêutico , Criança , Quimioterapia Combinada , Eritromelalgia/complicações , Eritromelalgia/tratamento farmacológico , Feminino , Humanos , Dor/diagnóstico , Dor/tratamento farmacológico , Vasculite/tratamento farmacológico
7.
J Korean Med Sci ; 30(10): 1429-38, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26425039

RESUMO

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 ± 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.


Assuntos
Bases de Dados Factuais , Hipertensão Pulmonar Primária Familiar/epidemiologia , Artéria Pulmonar/fisiopatologia , Sistema de Registros , Adulto , Idoso , Doenças do Tecido Conjuntivo/complicações , Coleta de Dados , Hipertensão Pulmonar Primária Familiar/mortalidade , Hipertensão Pulmonar Primária Familiar/terapia , Feminino , Cardiopatias Congênitas/complicações , Humanos , Internet , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , República da Coreia/epidemiologia , Taxa de Sobrevida , Adulto Jovem
8.
J Clin Res Pediatr Endocrinol ; 14(1): 102-106, 2022 03 03.
Artigo em Inglês | MEDLINE | ID: mdl-33251784

RESUMO

Cushing syndrome (CS) is a rare disease caused by hypercortisolemia. Although surgical treatment is the first-line treatment in CS, the appropriate medication for the patient's condition should be selected when medical treatment is needed. Etomidate is an adrenal-blocking drug used to treat CS and the most suitable for severe hypercortisolemia and adrenocortical carcinoma (ACC), due to cardiovascular stability and an anti-tumorigenic effect. However, its use and safe recommended dosage in infants with CS is unreported. Here we describe the case of a 2-month-old girl treated with etomidate for CS caused by ACC. Even though radical mass excision was performed, severe hypercortisolemia persisted, resulting from metastatic lesions in the liver, and medical treatment was considered. The etomidate doses, no bolus dose and infusion rate of 0.03 mg/kg/hour, may be an appropriate dose for severe hypercortisolemia in infants. This case will help determine future treatment strategies for similar cases in infants.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Síndrome de Cushing , Etomidato , Neoplasias do Córtex Suprarrenal/complicações , Neoplasias do Córtex Suprarrenal/tratamento farmacológico , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/complicações , Carcinoma Adrenocortical/tratamento farmacológico , Carcinoma Adrenocortical/cirurgia , Síndrome de Cushing/induzido quimicamente , Síndrome de Cushing/tratamento farmacológico , Etomidato/efeitos adversos , Feminino , Humanos , Hiperplasia/complicações , Hiperplasia/tratamento farmacológico , Lactente
9.
Transplant Proc ; 54(10): 2800-2802, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36371280

RESUMO

Alström syndrome is a rare, multisystemic genetic disorder, and dilated cardiomyopathy occurs in approximately two-thirds of patients with this condition. Because of donor organ shortage and unfavorable prognosis of multiple organ dysfunction, heart transplant is not the most desirable therapeutic option for patients with dilated cardiomyopathy with Alström syndrome. However, eliminating heart dysfunction elements at an appropriate time itself plays a pivotal role in preventing or even reversing other organ failures. Herein, we report the case of a 17-year-old boy who underwent successful isolated heart transplant despite severe liver dysfunction.


Assuntos
Síndrome de Alstrom , Cardiomiopatias , Cardiomiopatia Dilatada , Transplante de Coração , Masculino , Humanos , Adolescente , Síndrome de Alstrom/complicações , Síndrome de Alstrom/diagnóstico , Síndrome de Alstrom/genética , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/cirurgia , Cardiomiopatias/complicações
10.
Yonsei Med J ; 63(12): 1144-1146, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36444551

RESUMO

Here we report a case of successful heart transplantation (HT) in a patient with high risk on HT due to her complex congenital heart disease and its consequent complications; physiologic single lung and severe pulmonary arterial hypertension. HT was successfully performed in our patient using meticulous perioperative management techniques, such as control of collateral vessels, appropriate donor selection, pulmonary vasodilator therapy, post-transplant extracorporeal membranous oxygenation support, and atrial septostomy for right ventricular unloading.


Assuntos
Cardiopatias Congênitas , Transplante de Coração , Hipertensão Pulmonar , Humanos , Feminino , Hipertensão Pulmonar/cirurgia , Tórax , Pulmão , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia
11.
J Clin Med ; 11(9)2022 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-35566520

RESUMO

We aimed to determine the feasibility, efficacy, success, and safety of intracardiac echocardiography (ICE) in transcatheter multiple atrial septal defect (ASD) closure. Of 185 patients with multiple ASDs who underwent transcatheter closure, 140 (76%) patients who weighed <30kg with a narrow distance between defects or in whom single device closure was anticipated were guided by ICE and 45 patients were guided by three-dimensional (3D) transesophageal echocardiography (TEE) with or without ICE. Patients in the ICE group were relatively younger and weighed less than those in the 3D TEE group (p < 0.0001). The ratio of the distance between defects >7 mm was high, and more cases required ≥2 devices in the 3D TEE group than those in the ICE group (p < 0.0001). All patients in the 3D TEE group and seven patients (5%) in the ICE group were operated on under general anesthesia (p < 0.0001). The fluoroscopic time was shorter in the ICE group (13.98 ± 6.24 min vs. 24.86 ± 16.47 min, p = 0.0005). No difference in the complete closure rate and complications was observed. ICE-guided transcatheter and 3D TEE were feasible, safe, and effective in successful multiple ASD device closures, especially for young children and patients at high risk under general anesthesia.

12.
Circ Cardiovasc Imaging ; 15(7): e014138, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35861980

RESUMO

BACKGROUND: Although the transcatheter closure of atrial septal defect was established as the treatment of choice several decades ago, the process of device neoendothelialization (NE) in humans is not well understood. We aimed to measure the extent of device NE using cardiac computed tomography angiography and analyze its risk factors. METHODS: Between January 2005 and February 2021, we retrospectively reviewed 164 devices of 112 patients on cardiac computed tomography angiography. We investigated device shape, contrast opacification within the device that differentiated device NE, and device-related thrombosis or vegetation. Risk factor analysis for major adverse cardiovascular events and incomplete NE according to the postprocedural period was performed. RESULTS: Seventy patients (62.5%) were women, with a median (range) age at the time of device closure of 44.5 (0.6-79.2) years. The mean (±SD) defect size was 16.6 (±7.8) mm, and patients were followed for 35.9±33.9 months. After 6 months of device implantation, 35% of the devices (42/120) had incomplete NE. The intensity of intradevice opacification shifted from complete to partial or nonopacification over time (P<0.001), and a similar pattern was observed in the shunt flow (P<0.001). The bulkiness of devices also decreased in proportion to the postprocedural period (P<0.001). Risk analysis revealed device diameter (hazard ratio, 1.18 [95% CI, 1.04-1.27]; P<0.001) as the only significant factor of incomplete NE and major adverse events. CONCLUSIONS: Incomplete NE of atrial septal defect devices was identified on cardiac computed tomography angiography in significant numbers after 6 months of the procedure. The device diameter was related to incomplete NE and major adverse events. Further prospective and multicenter studies are warranted to validate this new assessment of device NE.


Assuntos
Angiografia por Tomografia Computadorizada , Comunicação Interatrial , Cateterismo Cardíaco , Feminino , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/cirurgia , Humanos , Masculino , Próteses e Implantes , Estudos Retrospectivos , Resultado do Tratamento
13.
Clin Transl Sci ; 15(2): 501-513, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34719115

RESUMO

On October 2020, the US Food and Drug Administration (FDA) approved remdesivir as the first drug for the treatment of coronavirus disease 2019 (COVID-19), increasing remdesivir prescriptions worldwide. However, potential cardiovascular (CV) toxicities associated with remdesivir remain unknown. We aimed to characterize the CV adverse drug reactions (ADRs) associated with remdesivir using VigiBase, an individual case safety report database of the World Health Organization (WHO). Disproportionality analyses of CV-ADRs associated with remdesivir were performed using reported odds ratios and information components. We conducted in vitro experiments using cardiomyocytes derived from human pluripotent stem cell cardiomyocytes (hPSC-CMs) to confirm cardiotoxicity of remdesivir. To distinguish drug-induced CV-ADRs from COVID-19 effects, we restricted analyses to patients with COVID-19 and found that, after adjusting for multiple confounders, cardiac arrest (adjusted odds ratio [aOR]: 1.88, 95% confidence interval [CI]: 1.08-3.29), bradycardia (aOR: 2.09, 95% CI: 1.24-3.53), and hypotension (aOR: 1.67, 95% CI: 1.03-2.73) were associated with remdesivir. In vitro data demonstrated that remdesivir reduced the cell viability of hPSC-CMs in time- and dose-dependent manners. Physicians should be aware of potential CV consequences following remdesivir use and implement adequate CV monitoring to maintain a tolerable safety margin.


Assuntos
Monofosfato de Adenosina/análogos & derivados , Alanina/análogos & derivados , Antivirais/efeitos adversos , Tratamento Farmacológico da COVID-19 , Doenças Cardiovasculares/induzido quimicamente , Farmacovigilância , SARS-CoV-2 , Monofosfato de Adenosina/efeitos adversos , Alanina/efeitos adversos , Bases de Dados Factuais , Humanos , Miócitos Cardíacos/efeitos dos fármacos , Estudos Retrospectivos , Organização Mundial da Saúde
14.
Circ J ; 75(9): 2228-34, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21747192

RESUMO

BACKGROUND: Idiopathic cardiomyopathies (CMPs) are an important heterogeneous group of diseases. With the advance of therapeutic strategies, epidemiologic data on CMP have become very important, but only a few have been reported in Asian children. We conducted a retrospective epidemiologic study of primary CMP in Korean children. METHODS AND RESULTS: Using a multicenter survey, we studied primary CMP among Korean children from January 1998 to December 2006 based on classification (2006) of CMP by the American Heart Association. A total of 277 primary CMP patients were reported from 17 cardiovascular centers. The average annual occurrence of new cases of primary CMP was 0.28 per 100,000 Korean children younger than 15 years of age (95% confidence interval (CI) 0.24-0.31). Dilated CMP (DCMP) was 66.43%, hypertrophic CMP (HCMP) 23.47%, restrictive CMP (RCMP) 6.50% and others 3.61%. The point prevalence of primary CMP at the end of the study was estimated as 2.11/100,000 (95%CI 1.83-2.43), DCMP 1.39/100,000, HCMP 0.51/100,000, RCMP 0.16/100,000 and others 0.04/100,000. Survival rates over 9 years were 69.8% in DCMP, 90.3% in HCMP, and 47.2% in RCMP. CONCLUSIONS: Recent point prevalence of childhood primary CMP in Korea was estimated as 2.11/100,000. Further epidemiologic study with a nationwide survey is necessary.


Assuntos
Cardiomiopatia Dilatada/mortalidade , Coleta de Dados , Adolescente , Povo Asiático , Cardiomiopatia Dilatada/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Prevalência , República da Coreia/epidemiologia , Estudos Retrospectivos , Taxa de Sobrevida
15.
Artigo em Inglês | MEDLINE | ID: mdl-33800133

RESUMO

Adolescents with congenital heart disease (CHD) continuously need family support because of their repeated follow ups, treatments, and complications. However, sibling relationships have not been well studied among adolescents with CHD. The purpose of the present study was to explore the relationships between adolescents with CHD and their siblings, and to examine these relationships according to birth order and age. Adolescents aged from 13 to 21 years who had been diagnosed with CHD and had siblings were included as participants. The Sibling Relationship Questionnaire (SRQ) was used. The SRQ consists of four factors: warmth/closeness, conflict, relative power/status, and rivalry. A univariate general linear model was conducted to identify the sibling relationship factors according to birth order and sibling ages. The score for relative power/status of participants who were the eldest sibling was higher than that of younger siblings. The score for rivalry increased as sibling age increased. Therefore, healthcare providers need to investigate sibling relationships and to explain the importance of self-identity and power balance between adolescents with CHD and their siblings to parents.


Assuntos
Cardiopatias Congênitas , Irmãos , Adolescente , Idoso , Cardiopatias Congênitas/epidemiologia , Humanos , Relações entre Irmãos , Inquéritos e Questionários
16.
J Clin Med ; 9(9)2020 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-32859019

RESUMO

Percutaneous pulmonary valve implantation (PPVI) is used to treat pulmonary stenosis (PS) or pulmonary regurgitation (PR). We described our experience with PPVI, specifically valve-in-valve transcatheter pulmonary valve replacement using the Melody valve and novel self-expandable systems using the Pulsta valve. We reviewed data from 42 patients undergoing PPVI. Twenty-nine patients had Melody valves in mostly bioprosthetic valves, valved conduits, and homografts in the pulmonary position. Following Melody valve implantation, the peak right ventricle-to-pulmonary artery gradient decreased from 51.3 ± 11.5 to 16.7 ± 3.3 mmHg and right ventricular systolic pressure fell from 70.0 ± 16.8 to 41.3 ± 17.8 mmHg. Thirteen patients with native right ventricular outflow tract (RVOT) lesions and homograft underwent PPVI with the new self-expandable Pulsta valve-a nitinol wire stent mounted with a trileaflet porcine pericardial valve. Following Pulsta valve implantation, cardiac magnetic resonance imaging showed a decreased PR fraction and that the right ventricular end-diastolic volume index decreased from 166.1 ± 11.9 to 123.6 ± 12.4 mL/m2. There were no mortality, severe procedural morbidity, or valve-related complications. At the mean 14.2 month (4-57 months) follow-up, no patients had more than mild PR. PPVI using Melody and Pulsta valves was first shown to provide excellent early outcomes without serious adverse event in most patients with RVOT dysfunction in Korea.

17.
Korean J Thorac Cardiovasc Surg ; 53(2): 79-81, 2020 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-32309207

RESUMO

Treatment options for children with end-stage heart failure are limited. We report the first case of a successful pediatric heart transplantation bridged with a durable left ventricular assist device in Korea. A 10-month-old female infant with dilated cardiomyopathy and left ventricular non-compaction was listed for heart transplantation. During the waiting period, the patient's status deteriorated. Therefore, we decided to provide support with a durable left ventricular assist device as a bridge to transplantation. The patient was successfully bridged to heart transplantation with effective support and without any major adverse events.

18.
Korean Circ J ; 49(10): 975-986, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31165594

RESUMO

BACKGROUND: This study aimed to compare procedural, early and long-term outcome of device closure of atrial septal defect (ASD) between large ASD patients and very large ASD patients. METHODS: We carried a retrospective study of adult large ASD (defined as ≥25 mm) treated by percutaneous closure using Amplatzer septal occluder during 12-year period (May 2003-February 2015) at a single tertiary center. A total of 269 patients were divided into 2 groups, according to the pre-procedural maximal ASD diameter; 25 mm≤ASD<35mm, group 1 (n=216) and 35 mm≤ASD, group 2 (n=53). We compared procedural parameters, early complications and long-term follow-up results between 2 groups. RESULTS: The need of modified implantation techniques (MITs) was higher group 2 (23.6% vs. 37.7%, p=0.034). Procedural success rate was considerably high in both groups (99.1% in group 1 vs. 100% in group 2, p=0.620). Major complications were occurred in 4 (1.5%) patients (1.4% vs. 1.9%, p=0.804). Minor complication rate was not different between 2 groups. During long-term follow-up (47.2±32.0 months, range, 6.0-135.5), there was one major complication (0.4%) of stroke. Most common long-term minor event was migraine headache (3.9%) followed by arrhythmias (1.9%) without statistical difference between 2 groups. CONCLUSIONS: Although MIT was more frequently required in very large ASD groups, the procedural, early and long-term outcomes after percutaneous ASD closure were similar in both groups. This suggested that percutaneous ASD closure for very large ASD could be considered a good treatment option.

19.
Korean J Radiol ; 20(9): 1334-1341, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31464112

RESUMO

OBJECTIVE: To evaluate flow pattern characteristics in the ascending aorta (AA) with four-dimensional (4D)-flow MRI and to determine predictors of aortic dilatation late after tetralogy of Fallot (TOF) repair. MATERIALS AND METHODS: This study included 44 patients with repaired TOF (25 males and 19 females; mean age, 28.9 ± 8.4 years) and 11 volunteers (10 males and 1 female, mean age, 33.7 ± 8.8 years) who had undergone 4D-flow MRI. The aortic diameters, velocity, wall shear stress (WSS), flow jet angle (FJA), and flow displacement (FD) at the level of the sinotubular junction (STJ) and mid-AA were compared between the repaired TOF and volunteer groups. The hemodynamic and clinical parameters were also compared between the aortic dilatation and non-dilatation subgroups in the repaired TOF group. RESULTS: The diameters of the sinus of Valsalva, STJ, and AA were significantly higher in the repaired TOF group than in the volunteer group (p = 0.002, p < 0.001, and p = 0.013, respectively). The FJAs at the STJ and AA were significantly greater in the repaired TOF group (p < 0.001 and p = 0.003, respectively), while velocities and WSS parameters were significantly lower. FD showed no statistically significant difference (p = 0.817). In subgroup analysis, age at TOF repair was significantly higher (p = 0.039) and FJA at the level of the AA significantly greater (p = 0.003) and mean WSS were significantly lower (p = 0.039) in the aortic dilatation group. FD were higher in the aortic dilatation group without statistical significance (p = 0.217). CONCLUSION: Patients with repaired TOF have an increased FJA, dilated AA, and secondarily decreased WSS. In addition to known risk factors, flow eccentricity may affect aortic dilatation in patients with repaired TOF.


Assuntos
Aorta/fisiologia , Imageamento por Ressonância Magnética , Tetralogia de Fallot/diagnóstico , Adulto , Aorta/diagnóstico por imagem , Estudos de Casos e Controles , Feminino , Hemodinâmica , Humanos , Interpretação de Imagem Assistida por Computador , Masculino , Resistência ao Cisalhamento , Seio Aórtico/fisiologia , Tetralogia de Fallot/diagnóstico por imagem , Adulto Jovem
20.
Korean Circ J ; 49(4): 326-335, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30808079

RESUMO

BACKGROUND AND OBJECTIVES: This study aimed to describe our early to mid-term experience with transcatheter atrial septal defect (ASD) closure using the Occlutech Figulla® Flex II device (FSO), Gore® Cardioform septal occluder (GSO), and Amplatzer® septal occluder (ASO) after they were first approved in Korea in 2014, and to compare the three aforementioned kinds of ASD closure devices. METHODS: Between September 2014 and August 2016, 267 patients underwent transcatheter ASD closure in our institution. Baseline characteristics, hemodynamic features, comorbidities, and procedural success and complication rates were analyzed retrospectively. The unpaired Student t-test or variance analysis was used in the statistical analysis. RESULTS: The FSO was most commonly used (n=152, 56.9%), followed by the ASO (n=98, 36.7%) and GSO (n=17, 6.4%). Baseline characteristics and hemodynamic features were similar between the devices, except that the defect size and pulmonary flow-to-systemic flow ratio were lower in the GSO group than in the other groups. Overall, the procedural success rate remained at 100%, and major complication rate was <1%. No late complication occurred during the follow-up. CONCLUSIONS: The FSO and GSO are feasible, safe options for use in transcatheter ASD closure, and they are comparable to the ASO.

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