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1.
Zhejiang Da Xue Xue Bao Yi Xue Ban ; 49(5): 597-602, 2020 Oct 25.
Artigo em Chinês | MEDLINE | ID: mdl-33210486

RESUMO

OBJECTIVE: To investigate the morbidity of congenital heart defects(CHDs) in children with anorectal malformation, and to summarize appropriate treatment. METHODS: The clinical data and echocardiographic findings of 155 children with congenital anorectal malformations from the Third Affiliated Hospital of Zhengzhou University during January 2016 and October 2019 were reviewed. According to the surgical findings of anorectal malformations, the patients were categorized as the high/intermediate group and the low group; the CHDs were classified as minor CHDs and major CHDs. Multiple logistic regression was used to analyze the correlation of wingspread classification, and extracardiac malformations with the severity of CHDs. RESULTS: Out of 155 children with anorectal malformations, 47 (30.3%) had different types of cardiac structural malformations, including 18 cases of minor CHDs (11.6%) and 29 cases of major CHDs (18.7%). Sixty children (38.7%) had extracardiac malformations, of which 38 cases (24.5%) had a single extracardiac malformation, 15 cases (9.7%) had multiple extracardiac malformations, 6 had trisomy 21 syndrome, and 1 had VATER syndrome. Multivariate logistic regression analysis showed that wingspread classification of anorectal malformation and extracardiac disorders were independent risk factors for major CHDs. The probability of major CHDs in children with high/intermediate anorectal malformation was 4.709 times higher than that with low anorectal malformation (OR=4.709, 95% CI: 1.651-13.432, P<0.01). The probability of major CHDs was 3.85 times higher for increasing each additional grade of extracardiac malformations(without, with single, or multiple malformations) (OR=3.850, 95% CI: 2.065-7.175, P<0.01). According to the presence and severity of CHDs, children with anorectal malformations were classified into three categories: without CHDs, with minor CHDs and with major CHDs, for differential treatment and management. Anorectal malformations would be treated and managed in children without CHDs and with minor CHDs following the original plan; however, children with minor CHDs may require cardiac follow-up after surgery. In children with major CHDs, the personalization tactics were developed based on the principle of emergency first. There were increased perioperative infection rate (P<0.05), longer hospital days (P<0.01), reduced cure rate (P<0.05) and increased mortality (P<0.05) in children with major, compared with those without CHDs and minor CHDs. CONCLUSIONS: The morbidity of major CHDs is higher in severe cases with high/intermediate anorectal malformation and acute cases without fistula or with obstructed fistula and cases with multiple congenital disorders. Echocardiography can define the type and severity of CHDs, which are useful to develop the optimal diagnosis and treatment plan for children with anorectal malformation.


Assuntos
Malformações Anorretais , Cardiopatias Congênitas , Anormalidades Múltiplas , Malformações Anorretais/complicações , Malformações Anorretais/terapia , Criança , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Humanos , Estudos Retrospectivos
2.
Kyobu Geka ; 73(10): 789-794, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-33130767

RESUMO

A large population of patients with congenital heart disease with untreated systemic to pulmonary shunts( left to right shunts) will develop pulmonary arterial hypertension( PAH). There are 2 different statuses of an increase in pulmonary arterial pressure. One is high resistance due to high pulmonary blood flow (high flow with high resistance), another one is low pulmonary flow due to high resistance (low flow with high resistance). Chronic large left-to-right shunt induced severe pulmonary vascular disease and pulmonary hypertension. This was then subsequence of low pulmonary blood flow with high pulmonary vascular resistance. We have to avoid this situation and have to do intervention within the pulmonary vascular reactivity has been left. For this reason, preoperative treatment for avoidance of high flow, appropriate timing of interventions and postoperative various managements are important factors as aiming of low pulmonary resistance in this group. Recent advances in PAH-specific drugs have dramatically changed the therapeutic strategy for PAH. A strategy that includes "treatment" with PAH-specific drugs initially and then "repair" by closure of the cardiac defect (i.e. "treat and repair") was devised, and has been attempted, in patients with PAH associated with a cardiac defect.


Assuntos
Cardiopatias Congênitas , Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Hipertensão Pulmonar/complicações , Pulmão , Circulação Pulmonar
3.
Kyobu Geka ; 73(10): 795-799, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-33130768

RESUMO

Congenital anomaly of respiratory or digestive system have a significant impact on the perioperative management of congenital heart disease. Therefore, it is important to make a long-term treatment strategy. It has been reported that 1-stage surgery is effective for pulmonary artery sling. Simultaneous repair is also useful if significant airway stenosis is present prior to cardiac surgery. In the case of congenital heart disease associated with gastrointestinal anomaly, gastrointestinal surgery precedes in more than 80% of cases. In recent years, though treatments of gastrointestinal atresia have a good outcome, the results of esophageal atresia are still bad. If we choice multi-stage surgery, there is a possibility that we cannot perform cardiac surgery at appropriate time due to postoperative mediastinitis. Therefore, we must consider the timing and method of operation individually. The important thing is to keep a good relationships with related departments and to provide close informed consent to the patient's parents.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Atresia Esofágica , Cardiopatias Congênitas , Sistema Digestório , Atresia Esofágica/complicações , Atresia Esofágica/cirurgia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Lactente
4.
J Biol Regul Homeost Agents ; 34(4 Suppl. 2): 99-106, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33000608

RESUMO

This study examined the prevalence and distribution of elevated systolic pulmonary arterial pressure, measured by echocardiography, in young patients with down syndrome associated or not with congenital heart disease and surgical correction during childhood. Pulmonary artery systolic pressure, computed by regurgitant tricuspid flow velocity evaluation, is the most frequently used parameter for the screening of pulmonary hypertension. Down syndrome and congenital heart disease often coexist and the probability to detect elevated systolic pulmonary arterial pressure in this setting is high. However, little is known about the evaluation of pulmonary arterial pressure during growth of patients with down syndrome with or without congenital heart disease. We enrolled 47 young patients (55% of male sex; mean age: 18.4 ± 6.0 years), 40 with congenital heart disease and 7 without a cardiac defect. Systolic pulmonary arterial pressure was assessed by echocardiography. No difference was found in the population dichotomized by presence or absence of CHD. Only male sex (p=0.000), highly sensitive troponin-T (P=0.027), tricuspid annular plane systolic excursion (TAPSE, p=0.045) and sPAP (p=0.004) were elevated in surgical group. The ASD was found as, the most common structural abnormality in our patients (50%), followed by VSD (27.5%) and complex CHD (such as complete atrioventricular canal defect, CAVC = 25% and Fallot disease = 15%). Furthermore, about 45% of patients had the combined defect. Only 37.5% of patients underwent to corrective surgery during the first months of life. We observed a significantly increase of sPAP values in patients with complex CHD, such as CAVC (p=0.019) and Fallot disease (p=0.001) but, in the following multivariate analysis performed in the patients with CHD, only Fallot disease remains as independent predictors of elevated values of sPAP (p=0.022). An elevated systolic pulmonary arterial pressure may represent the key screening tool in the diagnostic assessment of suspect pulmonary arterial hypertension in high risk population with down syndrome regardless the presence of congenital heart disease.


Assuntos
Síndrome de Down , Cardiopatias Congênitas , Adolescente , Pressão Sanguínea , Criança , Síndrome de Down/complicações , Síndrome de Down/diagnóstico por imagem , Síndrome de Down/epidemiologia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Masculino , Prevalência , Artéria Pulmonar/diagnóstico por imagem , Adulto Jovem
5.
J Biol Regul Homeost Agents ; 34(4 Suppl. 2): 91-97, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33000607

RESUMO

Congenital hypothyroidism (CH) is the most common endocrine disease in children, according to literature, infants with CH have an increased risk of associated congenital malformations (CM), especially cardiac defects (CD), compared to the general population. We retrospectively analyzed medical records of 255 patients with a positive screening result for CH in the period 1991-2016 followed at our Center. At the time of enrollment, the clinical examination included looking for the presence of heart murmurs and dysmorphic features. In all patients an echocardiography with cardiological evaluation were performed. Of all patients, 191 were included in the final analysis. Of these, 51.3% (98/191) presented an eutopic normally sized thyroid gland while 48.7% (93/191) showed a thyroid dysgenesis. Among the studied infants, 13.6% (26/191) presented CD. The most frequent cardiac anomaly was atrial septal defect (ASD) which was found in 65.4% (17/26) of patients with CD. Other defects were ventricular septal defect (VSD), patent ductus arteriosus (PDA), pulmonary valve stenosis (PvS), transposition of the great vessels (TGV), aortic valve stenosis (AvS). Six patients had multiple defects. In the analysed group, there was no significant relation with sex, type of CH, median blood-TSH (b-TSH) and serum-TSH (s-TSH) values and frequency of CD. There is a high prevalence of CD in CH, indicating the need of routine echocardiography in these patients to achieve an early diagnosis and management of CD.


Assuntos
Hipotireoidismo Congênito , Cardiopatias Congênitas , Criança , Hipotireoidismo Congênito/diagnóstico por imagem , Hipotireoidismo Congênito/epidemiologia , Ecocardiografia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Estudos Retrospectivos
6.
Arch Cardiovasc Dis ; 113(10): 607-616, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33039325

RESUMO

BACKGROUND: Red blood cell distribution width (RDW) is a predictor of adverse outcomes in patients with heart disease. AIM: To establish predictors of high RDW values in patients with congenital heart disease (CHD), and their relationship with cardiovascular events. METHODS: Overall, 561 patients with stable CHD who attended a single outpatient clinic and a matched control population of 2128 patients were studied. Exclusion criteria were renal failure, anaemia, receiving iron therapy and cyanosis. Blood tests included glucose, creatinine, iron, apoferritin, liver enzymes and a complete blood count. C-reactive protein and N-terminal prohormone of B-type natriuretic peptide (NT-pro-BNP) concentrations were also measured in patients with CHD. Major adverse cardiac events (MACE) were defined as cardiovascular/total mortality, arterial thrombotic events, arrhythmias, major bleedings, pulmonary embolism or heart failure needing hospital admission. RESULTS: The median age in patients with CHD was 23 (17-36) years and the median follow-up time was 5.8 (3.2-8.7) years; 103 (4.8%) controls and 40 (7.1%) patients with CHD had an RDW>15% (P=0.032). During follow-up, MACE were reported in 48 patients. CHD of great complexity, cardiovascular risk factors, low haemoglobin concentration and high NT-pro-BNP concentration were risk factors for an RDW>15%. Kaplan-Meier analysis showed a significantly worse cardiovascular outcome in patients with CHD with an RDW>15% (P<0.001). The multivariable survival analysis determined that age, CHD of great complexity, high NT-pro-BNP concentration and an RDW>15% were independent predictive factors for MACE. CONCLUSION: RDW and NT-pro-BNP concentration are independent analytical predictors of MACE in patients with CHD.


Assuntos
Índices de Eritrócitos , Cardiopatias Congênitas/diagnóstico , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Adolescente , Adulto , Biomarcadores/sangue , Estudos de Casos e Controles , Feminino , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Medição de Risco , Fatores de Risco , Adulto Jovem
8.
J Cardiothorac Surg ; 15(1): 297, 2020 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-33008444

RESUMO

BACKGROUND: While the aneurysms of the membranous septum (AVS) are rare, the possibility that they lead to obstruction is even rarer. To the best of our knowledge, 11 similar cases have been reported since 1982. CASE PRESENTATION: Initially, the five-year-old boy was evaluated for dyspnoea that had been present since birth. He did not receive any medical treatment until the previous year. At the age of four, the transthoracic echocardiography showed a large aneurysm extending to the right ventricular outflow tract (RVOT) and causing RVOT stenosis. Complete surgical resection of the aneurysmal tissue was performed, and the boy was discharged home in satisfactory condition. CONCLUSIONS: As the occurrence of RVOT obstruction by a membranous ventricular septal aneurysm is very rare, we are reporting the second case in which an aneurysm of the membranous septum dynamically obstructed the RVOT in a child. We are also reviewing all the previously reported similar cases in the literature. Further studies are needed to obtain a more comprehensive understanding of aneurysms of the membranous septum (AVS).


Assuntos
Aneurisma Cardíaco/cirurgia , Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Septo Interventricular/cirurgia , Pré-Escolar , Dispneia/etiologia , Ecocardiografia , Aneurisma Cardíaco/complicações , Cardiopatias Congênitas/complicações , Comunicação Interventricular/complicações , Ventrículos do Coração , Humanos , Masculino
9.
Anesth Analg ; 131(4): 1083-1089, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32925328

RESUMO

BACKGROUND: The objective of this study is to estimate the surgical risk of noncardiac procedures on the incidence of 30-day mortality in children with congenital heart disease. METHODS: Children with congenital heart disease undergoing noncardiac surgery from 2012 to 2016 and included in the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) Pediatric database were included in the derivation cohort, while the 2017 database was used as a validation cohort. Intrinsic surgical risk quartiles were built utilizing 30-day mortality rates for each Current Procedural Terminology code and relative value units to create 2 groups defined as low surgical risk (quartiles 1-3) and high surgical risk procedures (quartile 4). We used multivariable logistic regression to determine the predictors for 30-day mortality including patient comorbidities and intrinsic surgical risk. A partially external validation of the model was performed using the 2017 version of the database. RESULTS: We included 37,658 children with congenital heart disease undergoing noncardiac surgery with an incidence of overall 30-day mortality of 1.7% in the derivation cohort and 1.5% in the validation cohort (n = 13,129). Intrinsic surgical risk of procedures represented by Current Procedural Terminology procedural codes and relative value units risk quartiles was significantly associated with 30-day mortality (unadjusted P < .001). Predicted probability of 30-day mortality ranges from 0.2% (95% confidence interval [CI], 0.2-0.2) with no comorbidities to 39.6% (95% CI, 23.2-56.0) when all comorbidities were present among high surgical risk procedures and from 0.3% (95% CI, 0.3-0.3) to 54.8% (95% CI, 39.4-70.1) among low surgical risk procedures. An excellent discrimination was reported for the multivariable model with area under the curve (AUC) of 0.86 (95% CI, 0.85-0.88). High surgical risk was not associated with increased odds of 30-day mortality after adjustment for all other predictors (adjusted odds ratio [OR]: 0.75, 95% CI, 0.62-0.91). We also estimated the discriminative ability of a model that does not include the surgical risk (0.86 [95% CI, 0.84-0.88], with P value for the direct comparison of the AUC of the 2 models = 0.831). The multivariable model obtained from an external validation cohort reported an optimism corrected AUC of 0.88 (95% CI, 0.85-0.91). CONCLUSIONS: Our study demonstrates that integration of intrinsic surgical risk to comorbidities and severity of cardiac disease does not improve prediction of 30-day mortality in children undergoing noncardiac surgery. In children with congenital heart disease, patient comorbidities, and severity of the cardiac lesion are the predominant predictors of 30-day mortality.


Assuntos
Comorbidade , Cardiopatias Congênitas/mortalidade , Medição de Risco/métodos , Procedimentos Cirúrgicos Operatórios/mortalidade , Área Sob a Curva , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Cardiopatias Congênitas/complicações , Mortalidade Hospitalar , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Modelos Estatísticos , Resultados Negativos , Valor Preditivo dos Testes , Análise de Sobrevida
10.
Pediatr Clin North Am ; 67(5): 903-921, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32888689

RESUMO

Pulmonary hypertension (PH), the syndrome of increased pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children and is associated with a variety of potential underlying causes. Several pulmonary arterial hypertension-targeted therapies have become available to reduce pulmonary artery pressure and improve outcome, but there is still no cure for most patients. This review provides a description of select causes of PH encountered in pediatrics and an update on the most recent data pertaining to evaluation and management of children with PH. Available evidence for specific classes of PH-targeted therapies in pediatrics is discussed.


Assuntos
Diagnóstico por Imagem , Cardiopatias Congênitas/complicações , Hipertensão Arterial Pulmonar , Artéria Pulmonar/fisiopatologia , Pressão Propulsora Pulmonar/fisiologia , Criança , Cardiopatias Congênitas/diagnóstico , Humanos , Prognóstico , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/etiologia , Hipertensão Arterial Pulmonar/fisiopatologia
12.
Medicine (Baltimore) ; 99(27): e21092, 2020 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-32629741

RESUMO

RATIONALE: Univentricular dextrocardia is a rare congenital heart disease that usually presents cyanotic manifestations from childhood. Due to the sustained dysfunction of blood oxygenation, it is very difficult to keep an asymptomatic survival. Herein, we described an interesting case of univentricular dextrocardia who suffered from initial symptoms in his middle age. PATIENT CONCERNS: A 54-year-old male patient with numbness and tingling of limbs was admitted to hospital due to the secondary manifestations of congenital heart disease. DIAGNOSIS: The patient was diagnosed as univentricular dextrocardia with pulmonary hypertension and secondary erythrocytosis based on computed tomography (CT) scan, echocardiography, and laboratory examinations. INTERVENTIONS: Intravenous hydration therapy with normal saline successfully eliminated his hyperviscosity associated symptoms. In view of socio-economic reasons, this patient refused surgical evaluation and further medical interventions. OUTCOMES: During 18-month follow up, he received no drug except for regular water intake. Fortunately, his life quality was satisfactory, and no other symptoms emerged except for mild numbness of limbs. LESSONS: In univentricular dextrocardia, it is possible to keep a long-term asymptomatic period due to the slow progress of pathophysiology. In this population, regular cardiac function evaluation and avoiding dehydration may help improve the quality of life.


Assuntos
Desidratação/prevenção & controle , Dextrocardia/diagnóstico por imagem , Cardiopatias Congênitas/complicações , Assistência ao Convalescente , Grupo com Ancestrais do Continente Asiático/etnologia , Dextrocardia/fisiopatologia , Dextrocardia/terapia , Ecocardiografia/métodos , Hospitalização , Humanos , Hipertensão Pulmonar/etiologia , Masculino , Pessoa de Meia-Idade , Policitemia/etiologia , Policitemia/terapia , Qualidade de Vida , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
14.
World J Pediatr Congenit Heart Surg ; 11(5): 548-556, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32662334

RESUMO

As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees' experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Cardiopatias Congênitas/cirurgia , Pneumonia Viral/complicações , Recuperação de Função Fisiológica , Infecções por Coronavirus/epidemiologia , Cardiopatias Congênitas/complicações , Humanos , Pandemias , Pneumonia Viral/epidemiologia
15.
J Pediatr ; 225: 246-248, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32610168

RESUMO

With increasing information available about the epidemiology, pathophysiology, and management of patients affected with severe acute respiratory syndrome corona virus-2 infection, patients with Down syndrome, congenital heart disease, airway obstruction, and pulmonary hypertension present a unique challenge. This case series describes 3 patients with Down syndrome and respiratory failure secondary to coronavirus infection.


Assuntos
Infecções por Coronavirus/complicações , Síndrome de Down/complicações , Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/complicações , Pneumonia Viral/complicações , Adulto , Betacoronavirus , Pré-Escolar , Feminino , Humanos , Masculino , Pandemias , Fatores de Risco , Adulto Jovem
16.
J Card Surg ; 35(8): 2027-2028, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32557801

RESUMO

We hereby present a case of divided left atrium with mitral valve prolapse and discuss the hemodynamic consequences that ensue. This case demonstrates the utility of computed tomography angiography in identifying such associations.


Assuntos
Angiografia por Tomografia Computadorizada , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Prolapso da Valva Mitral/complicações , Prolapso da Valva Mitral/diagnóstico por imagem , Adulto , Átrios do Coração , Humanos , Masculino , Insuficiência da Valva Mitral/genética , Adulto Jovem
17.
Am J Cardiol ; 129: 42-45, 2020 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-32540168

RESUMO

Studies have shown that highly selected patients who underwent combined heart-kidney (HK) and heart-liver transplants (HLv) have short- and long-term outcomes comparable to those observed in primary heart transplantation (HT). Adults patients with stage D heart failure that underwent combined HK, HLv, and heart-lung (HL) were identified in the United Network for Organ Sharing registry from 1991 to 2016, with follow-up through March 2018. We conducted inverse probability of treatment weighting survival analysis of long-term survival stratified by type of combined organ transplant, accounting for donor, recipient, and operative characteristics. We identified 2,300 patients who underwent combined organ transplant (HK 1,257, HLv 212, HL 831). HL recipients were more likely white (77%), women (58%), with congenital heart disease (44.5%), and longer waiting list time (median 195 days). HK transplant increased significantly during the study period where as HL decreased significantly. Median survival was 12.2 years for HK (95% confidence intervals [CI] 10.8 to 12.8), 12 for HLv (95% CI 8.6 to 17.6) but significantly lower at 4.5 years for HL (95% CI 3.6 to 5.8). Combined HK and HLv transplantation rates are increasing and long-term survival is comparable to primary HT, unlike HL which is associated with decreasing trends and significantly lower survival.


Assuntos
Insuficiência Cardíaca/cirurgia , Transplante de Coração , Transplante de Rim , Transplante de Fígado , Mortalidade , Adulto , Afro-Americanos , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Restritiva/complicações , Grupo com Ancestrais do Continente Europeu , Feminino , Rejeição de Enxerto , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/complicações , Transplante de Coração-Pulmão , Hispano-Americanos , Humanos , Nefropatias/complicações , Nefropatias/cirurgia , Hepatopatias/complicações , Hepatopatias/cirurgia , Pneumopatias/complicações , Pneumopatias/cirurgia , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Sistema de Registros , Reoperação , Taxa de Sobrevida
18.
Arch. argent. pediatr ; 118(3): 173-179, jun. 2020. tab, ilus
Artigo em Inglês, Espanhol | LILACS, BINACIS | ID: biblio-1102723

RESUMO

La hernia diafragmática congénita (HDC) es una enfermedad de baja prevalencia, con elevada morbimortalidad. Los factores pronósticos posnatales, durante el primer día de vida, son útiles para la toma de decisiones.Objetivos. Determinar la capacidad predictiva de los factores posnatales ecocardiográficos, clínicos y bioquímicos de mortalidad en los recién nacidos con HDC durante el primer día de vida.Método. Estudio observacional analítico de cohorte retrospectiva. Se incluyeron los pacientes con HDC, en forma consecutiva, desde marzo de 2012 a noviembre de 2018. Se analizaron como predictores el índice de oxigenación (IO), valor más alto de presión parcial de dióxido de carbono en sangre (pCO2), puntaje de gravedad SNAPPE II, ecocardiograma, dosaje de NT-pro péptido natriurético B (NT-proPNB), todos medidos en el primer día de vida.Resultados. La población fue de 178 pacientes con HDC. La sobrevida, del 75 %. El 24 % recibió oxigenación por membrana extracorpórea. La presencia precoz de hipertensión pulmonar sistémica o suprasistémica no mostró capacidad predictiva (OR 2,2; IC 95 %: 0,8-8), p = 0,1. NT-proPNB tampoco mostró buena discriminación (área bajo la curva (ABC) 0,46, p = 0,67). El IO, SNAPPE II y el valor más alto de pCO2 mostraron buena discriminación, ABC IO 0,82, ABC SNAPPE II 0,86 y ABC pCO2 0,75, p < 0,001.Conclusión.SNAPPE II, IO y valor más alto de pCO2, medidos el primer día de vida, mostraron buena capacidad predictiva con respecto a la evolución; SNAPPE II fue superior al IO y al valor más alto de CO2.


Introduction: Congenital diaphragmatic hernia (CDH) prevalence is low while its associated morbidity and mortality rates are high. Postnatal prognostic factors on the first day of life are useful for predicting the outcome. Objectives: To determine the mortality predictive ability of postnatal echocardiographic, clinical, and biochemical factors among newborn infants with CDH in their first day of life. Method: Observational analytical study of a retrospective cohort. Patients with CDH were consecutively included between March 2012 and November 2018. On the first day of life, analyzed predictors were the oxygenation index (OI), the highest partial pressure of carbon dioxide (pCO2) level in blood, the SNAPPE II severity score, the echocardiography, and the N-terminal pro-B-type natriuretic peptide (NTproBNP) value. Results: The population consisted of 178 patients with CDH. Survival was 75 %. Extracorporeal membrane oxygenation was used in 24 %. The early onset of systemic or suprasystemic pulmonary hypertension showed no predictive ability (OR: 2.2, 95 % CI: 0.8-8), p = 0.1. NT-proBNP did not show good discrimination either (area under the curve [AUC]: 0.46, p = 0.67). The OI, SNAPPE II score, and the highest pCO2 level showed adequate discrimination power, AUC for OI: 0.82, AUC for SNAPPE II: 0.86, and AUC for pCO2: 0.75, p < 0.001. Conclusion: The SNAPPE II score, the OI, and the highest pCO2 level measured on the first day of life, showed a good predictive ability in terms of the course of the disease; the SNAPPE II score was better than the OI and the highest pCO2 level.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Hérnias Diafragmáticas Congênitas/mortalidade , Mortalidade Infantil , Estudos Retrospectivos , Fatores de Risco , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Hérnia Diafragmática/diagnóstico por imagem
19.
Middle East Afr J Ophthalmol ; 27(1): 28-33, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32549721

RESUMO

OBJECTIVE: The objective of the study was to asses ocular findings' prevalence in children with primary congenital heart disease (CHD). PATIENTS AND METHODS: This is a prospective cross-sectional study of children with CHD treated at a specialized center in the South of Brazil between 2013 and 2015. They underwent a complete ocular examination, including measurement of visual acuity, refraction test, external motility, anterior and posterior biomicroscopy, and binocular indirect fundoscopy with retinal photographs. Two experienced examiners independently assessed fundus findings: one at the time of examination and image capture, while the other assessed only the captured images. RESULTS: Of a total of 146 children examined, 124 were included in this analysis (16% loss). Seventy children were male (55.5%). The average age was 9.3 years (minimum 1 month and maximum 15 years). Caucasians race were 81.2%, African Descendants race were 11.1%, and others were 7.7%. About 57.1% had already had heart surgery. About 14.8% had visual acuity below 0.6 and 2.8% below 0.1. Strabismus was found in 7.4% and cataracts in 1.7%. Retinal alterations were recognized in 13.5%, of which 4.8% were related to vascular narrowing or dilation and/or abnormal arteriovenous crossing; 7.14% were related to increased vascular tortuosity, while 1.6% were related to active toxoplasmic chorioretinitis lesions. Concomitant abnormalities in ocular motility, biomicroscopy, or ophthalmoscopy were detected in 24% of the cases. CONCLUSION: Children under the age of 15 years old with primary CHD have a high prevalence of ocular alterations, with external ocular and retinal manifestations, with higher occurrence rate among cyanotic cases. This leads us to strongly recommend the performance of a complete ophthalmological examination in such cases.


Assuntos
Catarata/etiologia , Cardiopatias Congênitas/complicações , Transtornos da Motilidade Ocular/etiologia , Doenças Retinianas/etiologia , Estrabismo/etiologia , Adolescente , Catarata/diagnóstico , Criança , Pré-Escolar , Estudos Transversais , Feminino , Fundo de Olho , Humanos , Lactente , Masculino , Transtornos da Motilidade Ocular/diagnóstico , Oftalmoscopia , Estudos Prospectivos , Refração Ocular/fisiologia , Doenças Retinianas/diagnóstico , Estrabismo/diagnóstico , Acuidade Visual/fisiologia
20.
Expert Rev Cardiovasc Ther ; 18(6): 331-342, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32476525

RESUMO

INTRODUCTION: Infective endocarditis (IE) is a life-threatening adverse event for patients with congenital heart disease (CHD). Its incidence has changed little over time despite progress in techniques for diagnosis and treatment, and guidelines for prophylaxis. AREAS COVERED: The review sought for key-words: 'congenital heart disease,' 'infective endocarditis,' 'microbial diagnosis,' 'imaging diagnosis,' 'surgical techniques,' 'prognosis,' 'prophylaxis.' Objectives were to investigate epidemiology, novel techniques for imaging and microbial diagnosis, therapeutic management and prognosis, and guidelines for prophylaxis in patients with CHD. The incidence of IE is increasing in adults with CHD. Morbidity caused by a broad clinical spectrum of cardiac and extracardiac episode-related complications is high. Surgical management is increasingly required in the early phase of the disease. Despite new techniques for diagnosis and microbiological therapy, mortality rate is still up to 10-20%. EXPERT OPINION: IE has increased in the growing cohort of adults with complex heart disease, living with residual cardiac lesions and prosthetic materials. Diagnosis is challenging for complex heart defects. Pet-scan technique can provide beneficial information to locate intracardiac lesions and embolic foci. Identification of the microbiological agents is improving. Innovative surgical techniques aim to avoid prosthetic material. Guidelines for prophylaxis currently emphasize oral and skin daily hygiene.


Assuntos
Endocardite/cirurgia , Cardiopatias Congênitas/cirurgia , Adulto , Criança , Endocardite/complicações , Endocardite/diagnóstico por imagem , Endocardite/microbiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Prognóstico
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