Proporção de Largura de Distribuição de Hemoglobina/Glóbulos Vermelhos (HRR): Um Novo e Promissor Parâmetro de Glóbulos Vermelhos no Fechamento Ductal / Hemoglobin/Red Cell Distribution width Ratio (HRR): A Novel and Promising Red Cell Parameter in Ductal Closure

Resumo Fundamento Ainda não está clara a eficácia dos parâmetros hematológicos no fechamento da persistência do canal arterial (PCA). Objetivos O objetivo principal do nosso estudo é investigar o efeito da proporção (HRR) de largura de distribuição de hemoglobina (HB) para glóbulos vermelhos (RDW) no fechamento do PCA. Métodos Bebês prematuros com muito baixo peso ao nascer (MBPN: <1.500 g) e <32 semanas gestacionais foram incluídos no estudo, e todos os dados foram registrados retrospectivamente. Características demográficas, resultados clínicos, parâmetros de hemácias e HRR e suas proporções foram comparados entre grupos de PCA hemodinamicamente significativa (hsPDA) e não-hsPDA. Todos os resultados foram analisados estatisticamente, e p<0,05 foi considerado estatisticamente significativo. Resultados Um total de 677 bebês prematuros, 269 no grupo hsPDA e 408 no grupo não-hsPDA, foram incluídos no estudo. Hemoglobina (HB), hematócrito (HCT), volume celular médio (VCM), glóbulos vermelhos (RBC), largura de distribuição dos glóbulos vermelhos (RDW), volume plaquetário médio (VPM), relação VCM/RBC, relação HB/RBC, RDW A razão /RBC e a razão RDW/VPM foram semelhantes entre os grupos hsPDA e não hsPDA, (p>0,05). HRR foi significativamente menor no grupo hsPDA [mediana (Quartil 1 (Q1) - Q3) (Q1 - Q3): 0,93 (0,8-1,0)] em comparação com não-hsPDA [mediana (Q1 - Q3): 1,07 ( 1,0-1,2)] (p<0,001). A AUC para o valor diagnóstico de HRR em hsPDA foi de 0,816 e o valor de corte foi ≤0,98 (p<0,001, 95% [IC]: 0,785-0,845, sensibilidade: 90%, especificidade: 92%). Conclusões O valor de HRR foi considerado um parâmetro eficaz e poderoso no diagnóstico de hsPDA.

Abstract Background It is still unclear how effective hematological parameters are in the closure of patent ductus arteriosus (PDA). Objectives The primary aim of our study is to investigate the effect of hemoglobin (HB)-to-red cell distribution width (RDW) ratio (HRR) on the closure of PDA. Methods Premature babies with very low birth weight (VLBW: <1500 g) and <32 gestational weeks were included in the study, and all data were recorded retrospectively. Demographic characteristics, clinical results, red cell parameters, and HRR and their ratios were compared between hemodynamically significant PDA (hsPDA) and non-hsPDA groups. All results were statically analyzed, and P<0.05 was considered statistically significant. Results A total of 677 premature babies, 269 in the hsPDA group and 408 in the non-hsPDA group, were included in the study. Hemoglobin (HB), hematocrit (HCT), mean cell volume (MCV), red blood cell (RBC), red cell distribution width (RDW), mean platelet volume (MPV), MCV/RBC ratio, HB/RBC ratio, RDW/RBC ratio, and RDW/MPV ratio were found to be similar between hsPDA and non-hsPDA groups, (p>0.05). HRR was found to be significantly lower in the hsPDA group [median (Quartile 1 (Q1) - Q3) (Q1 - Q3): 0.93 (0.8-1.0)] compared to non-hsPDA [median ( Q1 - Q3): 1.07 (1.0-1.2)] (p<0.001). The AUC for the diagnostic value of HRR in hsPDA was 0.816, and the cutoff value was ≤0.98 (p<0.001, 95% [CI]: 0.785-0.845, sensitivity: 90%, specificity: 92%). Conclusions HRR value was found to be both an effective and powerful parameter in diagnosing hsPDA.

Qual Deve Ser o Tratamento de Primeira Linha para o Fechamento de Persistência de Canal Arterial Hemodinamicamente Significativo em Bebês Prematuros? / What Should be the First-line Treatment for the Closure of Hemodynamically Significant Patent Ductus Arteriosus in Premature Infants?

Resumo Fundamento É importante saber qual medicamento usar como tratamento de primeira linha para fechar o duto. Objetivos O objetivo deste estudo é comparar a eficácia e os efeitos colaterais das formas intravenosas (IV) de ibuprofeno e paracetamol e contribuir para a literatura investigando o primeiro medicamento selecionado no tratamento clínico da persistência do canal arterial (PCA). Métodos Nosso estudo foi realizado entre janeiro de 2017 e dezembro de 2019. Foram incluídos no estudo bebês prematuros com peso ao nascer (PN) ≤1500 g e idade gestacional (IG) ≤32 semanas. No período do estudo, todos os bebês com persistência do canal arterial hemodinamicamente significativa (hsPCA) receberam ibuprofeno intravenoso (IV) como resgate como tratamento clínico primário ou tratamento com paracetamol IV se houvesse contraindicações para o ibuprofeno. Os pacientes foram divididos em dois grupos: pacientes que receberam ibuprofeno IV e pacientes que receberam paracetamol IV. Resultados Desses pacientes, 101 receberam paracetamol IV e 169 receberam ibuprofeno IV. A taxa de sucesso do fechamento da PCA com o primeiro curso do tratamento foi de 74,3% no grupo de paracetamol IV e 72,8% no grupo de ibuprofeno IV (p=0,212). Conclusões Nossos resultados mostram que o paracetamol IV é tão eficaz quanto o ibuprofeno IV no tratamento de primeira linha de hsPCA, podendo se tornar o tratamento preferencial para o controle de hsPCA.

Abstract Background It is important which medicine to use as a first-line treatment to close the duct. Objectives The aim of this study is to compare the effectiveness and side effects of intravenous (IV) forms of ibuprofen and paracetamol and to contribute to the literature investigating the first drug selected in the medical treatment of patent ductus arteriosus (PDA). Methods Our study was conducted between January 2017 and December 2019. Premature infants with birth weight (BW) ≤1500 g and gestational age (GA) ≤32 weeks were included in the study. In the study period, all infants with hemodynamically significant patent ductus arteriosus (hsPDA) were given rescue intravenous (IV) ibuprofen as a primary medical treatment or IV paracetamol treatment if there were contraindications for ibuprofen. The patients were divided into two groups: patients receiving IV ibuprofen and patients receiving IV paracetamol. Results Of these patients, 101 were given IV paracetamol and 169 were given IV ibuprofen. The success rate of PDA closure with first-course treatment was 74.3% in the IV paracetamol group and 72.8% in the IV ibuprofen group (p=0.212). Conclusions Our results show that IV paracetamol is as effective as IV ibuprofen in the first-line treatment of hsPDA, and can become the preferred treatment for the management of hsPDA.

Efficacy and safety of initial treatment with ibuprofen or paracetamol and rescue treatment with high-dose ibuprofen in preterm infants with patent ductus arteriosus / 中华全科医师杂志

Objective:To investigate the efficacy and safety of initial treatment with ibuprofen or paracetamol and rescue treatment with high-dose ibuprofen in preterm infants with haemodynamically significant patent ductus arteriosus (hsPDA).Methods:The preterm infants (gestational age ≤ 32 weeks) with hsPDA who were admitted to neonatal intensive care unit (NICU) of Xuzhou Central Hospital from October 2016 to November 2019 were enrolled in the study. A total of 110 eligible cases were included and randomly divided into three groups for initial treatment: 38 cases received oral ibuprofen 10 mg/kg, and 5 mg/kg after 24 h and 48 h (ibuprofen group), 37 cases received oral paracetamol 15 mg/kg, q.8.h for 3 d (paracetamol group) and 35 cases received oral injection water 1 ml/kg, and 0.5 ml/kg after 24 h and 48 h (conservative management group). The preterm infants who failed in the initial treatment were given high-dose ibuprofen for rescue treatment (oral ibuprofen 20mg/kg, and 10 mg/kg after 24 h and 48 h). Serum creatinine, cystatin C, glutamic-pyruvic transaminase (GPT), total bilirubin, fecal occult blood and urinary prostaglandin E 2 were measured; echocardiography and brain color Doppler ultrasonography examinations were performed before and after treatment. Urine output and complications were recorded. The data were analyzed by ANOVA, t-test, non-parametric test, chi-square test and Pearson correlation coefficient with SPSS 20.0 statistical software. Results:During initial treatment, the success rates of ibuprofen group and paracetamol group were higher than that of conservative management group [71.1% (27/38) and 70.3%(26/37) vs. 40.0% (14/35), P=0.008 and 0.010]. Thirty one patients, who failed in initial treatment, received rescue treatment (8, 7, 16 cases from ibuprofen, paracetamol and conservative groups, respectively). The success rate of rescue treatment with high-dose ibuprofen was 58.1% (18/31). During initial treatment, there were no significant differences in the incidence of oliguria, upper gastrointestinal bleeding, positive fecal occult blood tests, Ⅲ-Ⅳ grade intraventricular hemorrhage, and ≥Ⅱ stage necrotizing enterocolitis among the three groups (all P>0.05). There were no significant differences in the incidence of above complications between rescue treatment and initial treatment [6.5% (2/31) vs. 6.4%(7/110), 3.2%(1/31) vs. 4.5%(5/110), 12.9%(4/31) vs. 6.4%(7/110), 0 vs. 4.5%(5/110), 3.2%(1/31) vs. 1.8%(2/110), all P>0.05]. The changes of serum creatinine and GPT before and after treatment were not significant in all groups ( P>0.05). Serum cystatin C were increased in both ibuprofen group[(0.44±0.17)μmol/L] and paracetamol group [(0.18±0.09)μmol/L] after treatment ( t=-15.70, -14.64; P<0.001), and the increase in ibuprofen group was greater than that in paracetamol group ( P<0.001). Urinary prostaglandin E 2 were decreased in both ibuprofen group [(-11.63±3.70)ng/L] and paracetamol group[(-4.89±1.91)ng/L] after treatment ( t=15.57, 7.03; P<0.001), and the decrease in ibuprofen group was greater than that in paracetamol group ( P<0.001). Serum cystatin C was not significantly increased after high dose ibuprofen rescue treatment [(1.67±0.17)mg/L vs.(1.71±0.21)mg/L; t=-1.12, P=0.273]. Conclusion:Both initial treatment with ibuprofen or paracetamol and rescue treatment with high-dose ibuprofen can effectively promote hsPDA closure in preterm infants without increase of complications. However, renal function indexes such as urine output and serum cystatin C should be monitored. The high-dose ibuprofen is relatively safe, and can be used as one of rescue treatment.

Caso Clínico: Resolución quirúrgica de coartación de aorta en un paciente pediátrico / Case Report: Surgical resolution of aortic coarctation in pediatric patient

INTRODUCCIÓN: La coartación de aorta es una cardiopatía congénita con una incidencia de 4 por cada 10 000 nacidos vivos y puede asociarse o no a ductus arterioso persistente u otras malformaciones. Suele ser asintomático, normalmente se diagnostica mediante los signos clásicos; con gradiente de presión arterial entre extremidades, pulsos reducidos en extremidades inferiores, hipertensión arterial en miembros superiores o, en casos graves, fallo cardiaco izquierdo. Su resolución puede ser percutánea o quirúrgica, dependiendo de la edad y sus características. CASO CLÍNICO: Paciente masculino de 6 años, asintomático, con sospecha de coartación aórtica, por presentar una diferencia entre presiones arteriales de miembros superiores e inferiores, extremidades inferiores con pulsos reducidos y soplo sistólico en foco aórtico. Se solicitó ecocardiograma que informó válvula aórtica bicúspide con rafe, insuficiencia ligera y coartación de aorta; angiotomografía evidenció coartación de aorta yuxtaductal; cateterismo cardiaco objetivó casi nulo paso de contraste a través del defecto, por lo que se programó tratamiento quirúrgico. EVOLUCIÓN: La corrección quirúrgica se realizó mediante coartectomía con anastomosis termino-terminal más cierre del conducto arterioso. Posterior a la intervención se logró objetivar una mejora relevante en el gradiente de presión entre las extremidades. En el postquirúrgico el paciente presentó hipertensión que logró ser controlada, evolucionó favorablemente y recibió el alta a los 4 días sin tratamiento antihipertensivo ambulatorio. CONCLUSIÓN: La expectativa de vida en pacientes intervenidos para corregir su cardiopatía congénita es superior en relación a aquellos que no son intervenidos, por lo que el diagnóstico oportuno supone una herramienta importante para mejorar la calidad y esperanza de vida.

BACKGROUND: Coarctation of the aorta is a congenital heart disease with an incidence of 4 per 10 000 live births, it may or may not be associated with patent ductus arteriosus as well as other malformations. It is usually asymptomatic and diagnosed by its classic signs such as; arterial pressure gradient between extremities, reduced pulses in the lower extremities, arterial hypertension in the upper extremities or, in severe cases, left heart failure. Its resolution can be percutaneous or surgical, depending on the patient's age and the characteristics of the defect. CASE REPORTS: A 6-year-old male patient, asymptomatic, with suspected aortic coarctation, due to a difference between arterial pressures in the upper and lower limbs, lower limbs with reduced pulses, and a systolic murmur in the aortic focus. An echocardiogram was requested, which reported a bicuspid aortic valve with raphe, mild regurgitation, and coarctation of the aorta; CT angiography showed coarctation of the juxtaductal aorta; cardiac catheterization showed almost no passage of contrast through the defect, so surgical treatment was scheduled. EVOLUTION: Surgical correction was performed by coartectomy with end-to-end anastomosis and closure of the ductus arteriosus. After the intervention, a relevant improvement in the pressure gradient between the extremities was observed. In the postoperative period the patient presented hypertension, that we managed to control, the patient progressed favorably and was discharged after 4 days without antihypertensive treatment. CONCLUSION: Life expectancy in patients who underwent surgery to correct a congenital heart disease is higher than in those who don't, so timely diagnosis is an important tool to improve life quality and life expectancy.

Patent ductus arteriosus closure: experience from a tertiary referral center / Experiência de uma maternidade terciária no fechamento do canal arterial patente

ABSTRACT Objective: To characterize the number and methods of closure of Persistent Ductus Arteriosus (PDA) over a span of 16 years in a third level maternity hospital. Methods: Retrospective study of neonates born between January 2003 and Deccember 2018, who underwent ductus arteriosus closure by pharmacological, surgical and/or transcatheter methods. Gestational age, birth weight, number and methods of closures per year were evaluated. The success rate of the pharmacologic method was calculated, as well as the mortality rate. The association between mortality and birthweight, treatment used and treatment failure was explored. Results: There were 47,198 births, 5,156 were preterm, 325 presented PDA and 106 were eligible for closure (median gestational age - 27 weeks, birthweight <1000 g - 61%). Frequency of PDA closure decreased during the study period, especially starting in 2010. Success rate with pharmacologic treatment was 62% after the first cycle and 74% after the second. After drug failure, 12 underwent surgical ligation and two underwent transcatheter closure. Exclusive surgical ligation was indicated in four infants. Ibuprofen replaced indomethacin in 2010, and acetaminophen was used in three infants. Among the 106 infants, hospital mortality was 12% and it was associated with birthweight <1000 g (13/65 <1000 vs. 0/41 >1000 g; p=0.002) and with failure in the first pharmacologic treatment cycle (13/27 with failure, vs. 0/75 without failure; p<0.001). Conclusions: The national consensus published in 2010 for the diagnosis and treatment of PDA in preterm infants led to a decrease in the indication for closure. Pharmacological closure was the method of choice, followed by surgical ligation. Birthweight <1000 g and first cycle of pharmacologic treatment failure were associated with higher mortality.

RESUMO Objetivo: Caraterizar o número e métodos de fechamento de canal arterial durante 16 anos numa maternidade de nível terciário. Métodos: Estudo retrospetivo de nascidos entre 01 de janeiro de 2003 a 31 de dezembro de 2018 submetidos a fechamento do canal arterial por métodos farmacológico, cirúrgico e/ou percutâneo. Avaliaram-se idade gestacional, sexo, peso ao nascimento, número de fechamentos por ano e método utilizado. Aferiram-se as taxas de sucesso de método farmacológico e de mortalidade e sua associação com peso ao nascer, fármaco utilizado e insucesso do fechamento. Resultados: Verificaram-se 47.198 recém-nascidos, 5.156 prematuros, dos quais 325 com canal arterial patente, sendo 106 com indicação para fechamento (idade gestacional mediana 27 semanas, peso <1000 g em 61%). Verificou-se diminuição do número de fechamentos ao longo dos anos, sobretudo a partir de 2010. O fechamento ocorreu em 62% após primeiro ciclo de tratamento farmacológico e em 74% após segundo. Após insucesso farmacológico, 12 realizaram ligadura cirúrgica e dois, fechamento percutâneo. Houve indicação de ligadura cirúrgica exclusiva em quatro. O ibuprofeno substituiu a indometacina em 2010. O acetaminofen foi usado em três doentes. A mortalidade nos 106 pacientes foi de 12%, associando-se ao peso ao nascer (13/65 <1000 vs. 0/41 >1000 g; p=0,002) e à falha do primeiro ciclo de tratamento farmacológico (13/27 com falha vs. 0/75 com sucesso; p<0,001). Conclusões: Consenso nacional de 2010 para diagnóstico e tratamento do canal arterial nos prematuros levou à diminuição do número de fechamentos desse canal. O fechamento farmacológico foi o método mais utilizado, seguido da ligadura cirúrgica. Peso <1000 g e falha no primeiro ciclo de fechamento farmacológico se associaram à maior mortalidade.

Alternative treatment for patent ductus arteriosus: a therapeutic challenge / Tratamento alternativo do canal arterial pervio: um desafio terapêutico

Abstract The ductus arteriosus is a fetal structure that spontaneously closes in 90% of newborns. Patency 3 months after birth is considered a congenital heart disease that, if untreated, can progress to serious cardiovascular complications. This report aims to review an alternative treatment with an aortic endoprosthesis in a 49-year-old man who presented with dyspnea on moderate exertion associated with a heart murmur. He was diagnosed with persistent ductus arteriosus (PDA) with cardiac complications. Clinical management was unsuccessful and surgical treatment was indicated. Endovascular treatment with a thoracic endoprosthesis was indicated and performed successfully. Percutaneous closure is the preferred method in adult patients. Endovascular intervention using an endoprosthesis is a safe and effective option, in addition to being applicable regardless of the anatomy of the PDA. This case demonstrates the natural history of the pathology and presents a safe and effective alternative for its management.

Resumo O canal arterial é uma estrutura fetal que se fecha espontaneamente em 90% dos recém-nascidos. A permeabilidade por mais de 3 meses é considerada uma cardiopatia congênita que, se não tratada, pode evoluir para complicações cardíacas graves. Este trabalho relata um tratamento alternativo com endoprótese aórtica. Trata-se de um homem, 49 anos, que apresentou dispneia aos moderados esforços, associada com um sopro cardíaco. Foi diagnosticado com canal arterial persistente (PCA) já com complicações cardíacas. Tentou-se manejo clínico sem sucesso, sendo indicado tratamento cirúrgico. Optou-se pelo tratamento endovascular com uma endoprótese torácica, o qual foi realizado com sucesso. O fechamento percutâneo é o método preferido em pacientes adultos. A intervenção endovascular com o uso da endoprótese é uma possibilidade segura e eficaz, além de ser aplicável independentemente da anatomia do PCA. Este caso, além de demonstrar a história natural desta patologia, apresenta uma alternativa segura e eficaz empregada no seu manejo.

Extrapleural closure of patent Ductus Arteriosus: how we do it

Abstract Patent ductus arteriosus (PDA) is a clinical condition mostly found in premature newborns. Among several medical, surgical and interventional treatment options, extrapleural ligation through a left minithoracotomy is recognized as a safe, efficient and less expensive technique. In fact, it requires short surgical times, grants good exposure of the duct and nearby structures (e.g., thoracic duct, left recurrent laryngeal nerve), and avoids pleural space opening and subsequent pulmonary complications in preterm patients. This approach seems ideal due to its lower costs, especially in developing countries with a high birth rate and limited resources.

Primary study on the mechanism of ductus arteriosus closure in rats: impact of aging pathway / 中华心血管病杂志

Objective: To explore the role and mechanism of aging pathway in patent ductus arteriosus closure of rats. Methods: Thirty outbreeding Sprague Dawley rats(20 females, 10-15 weeks old, 270-330 g) underwent random mating and conception. The primary Ductus Arteriosus smooth muscle cells (DASMCs) of pregnant 19 days(E19 group), 21 days(E21 group) and newborn(Day0 group) fetus were extracted and cultured. mRNA expression of cell senescence related markers p16, 21 and 53 genes in each group were detected by real-time fluorescent quantitative PCR(RT-PCR) after 48 hours culture. After hypoxic culture on DASMCs for 3 days, the DASMCs were divided into 3 groups: hypoxic control group(G0 group), 3 hours normal oxygen concentration treatment group(G3 group) and 6 hours normal oxygen concentration treatment group(G6 group). After intervention, mRNA expression of p16, 21 and 53 RT-PCR was detected. The DASMCs of newborn rats(Day0 group) were extracted and divided into 3 groups:low-oxygen culture control group, low-oxygen+siRNA culture group and normal oxygen concentration culture group. The DASMCs migration ability was tested experimentally by Transwell method. Result: The mRNA levels of p16, 21 and 53 in DASMCs were higher in E19 group than in Day0 group(all P<0.01), and the mRNA levels of p16, 21 and 53 in DASMCs were also higher in E21 group than those in Day0 group (all P<0.01). The mRNA levels of p16, 21 and 53 in DASMC were all higher in G0 group than those in G3 group (P<0.05 or 0.01), and the mRNA levels of p16, 21 and 53 in DASMCs were all higher in G0 group than those in G6 group (all P<0.01), and the mRNA levels of p16, 21 and 53 in DASMCs were all higher in G3 group than those in G6 group (all P<0.05). DASMCs migration ability of newborn rats was higher in normal oxygen concentration culture group than that in low-oxygen culture group (P<0.01), and DASMCs migration ability of newborn rats was also higher in low-oxygen+siRNA culture group than that in low-oxygen culture group (P<0.01). Conclusion: The expression of senescence marker of DASMCs decreases with the birth in rats during the process of ductal closure, and the aging pathway may affect ductal closure by inhibiting DASMCs migration in rats.

Caso Clínico: Resección Quirúrgica de un Rabdomioma Cardíaco Fetal / Clinical Case Report: Surgical Resection of a Fetal Cardiac Rhabdomyom

INTRODUCCIÓN: Los tumores cardiacos fetales son extraordinariamente raros; su prevalencia varía entre 0.027% y 0.14%. Entre ellos, los más comunes son los rabdomiomas, siendo el 90% benignos y asintomáticos. Se mencionan menos de 300 casos en la bibliografía médica internacional y, en nuestro país, existe un solo caso publicado que no requirió corrección quirúrgica. CASO CLÍNICO: Paciente masculino, recién nacido a término, adecuado para la edad gestacional, con diagnóstico prenatal ecográfico de masa intracavitaria cardiaca, observado en ecografía a las 20 semanas de gestación. Estudio ecográfico a las pocas horas de vida evidenció comunicación interatrial, persistencia de conducto arterioso e imágenes compatibles con rabdomiomas biventriculares con repercusión hemodinámica y signos de disfunción biventricular de predominio derecho por obstrucción del tracto de salida, por lo que se decidió intervención quirúrgica con corrección total. EVOLUCIÓN: El paciente fue ingresado en la Unidad de Cuidados Intensivos Neonatal para monitoreo hemodinámico y a las 36 horas de vida se realizó cirugía correctiva con resección de masa tumoral de ventrículo derecho preservando válvula pulmonar, cierre de conducto arterioso y de la comunicación interauricular. Recibió el alta a los 10 días con buena evolución; ecocardiografías posteriores muestran involución de rabdomiomas intracavitarios de ventrículo izquierdo y adecuada función hemodinámica. CONCLUSIÓN: El diagnóstico prenatal permitió planificar parto por cesárea y corrección quirúrgica total temprana. Existe dificultad para diagnóstico de patologías con prevalencias tan bajas, por lo que los controles ecográficos realizados por profesionales experimentados fueron de vital importancia. El esfuerzo multidisciplinario para el diagnóstico, tratamiento y control hicieron posible un resultado favorable.(au)

BACKGROUND: Fetal cardiac tumors are extraordinarily rare; the prevalence varies between 0.027% and 0.14%. Among them, the most common are rhabdomyomas, 90% being benign and asymptomatic. Less than 300 cases in the international medical literature have been described; in our country there is only one published case report that did not require surgical correction. CASE REPORT: 2 day-old full-term newborn, male, adequate for gestational age, with prenatal ultrasound diagnosis of cardiac intracavitary mass at 20 gestational weeks. Ultrasound study performed within a few hours after birth evidenced interatrial communication, persistence of ductus arteriosus and images compatible with biventricular rhabdomyomas with hemodynamic repercussion and signs of biventricular dysfunction, predominantly right, due to obstruction of the outflow tract. Total correction surgery was decided as treatment EVOLUTION: The patient was admitted to the Neonatal Intensive Care Unit for hemodynamic monitoring; 36 hours after birth, corrective surgery was performed with resection of the right ventricular tumor mass, preservation of pulmonary valve and closure of the ductus arteriosus and interatrial communication. The patient was discharged 10 days later with good progress; follow-up echocardiogram showed regression of left ventricular intracavitary rhabdomyoma and adequate hemodynamic function. CONCLUSIONS: Prenatal diagnosis allowed planning cesarean delivery and early total surgical correction. There is difficulty in the diagnosis of prenatal pathologies of low prevalence. The ultrasound performed by experienced professionals was crucial. The multidisciplinary effort for diagnosis, treatment and control made possible a favorable outcome(au)

Tratamiento farmacológico del conducto arterioso permeable en recién nacidos prematuros / Pharmacological treatment of patent ductus arteriosus in premature infants

Resumen Introducción. Por lo general, el manejo farmacológico del conducto arterioso permeable (CAP) comprende inhibidores no selectivos de la enzima ciclooxigenasa, en especial indometacina e ibuprofeno. En años recientes también se ha sugerido al acetaminofén como alternativa terapéutica. Objetivo. Realizar una revisión narrativa de la literatura acerca del manejo farmacológico del CAP. Materiales y métodos. Se realizó una búsqueda estructurada de la literatura en las bases de datos ProQuest, EBSCO, ScienceDirect, PubMed, LILACS, Embase, Trip Database, SciELO y Cochrane Library con los términos "Ductus Arteriosus, patentAND therapeutics"; "Ductus Arteriosus, patent AND indometacin"; "Ductus Arteriosus, PatentAND ibuprofen", y "Ductus Arteriosus, patent AND acetaminophen". La búsqueda se hizo en inglés con sus equivalentes en español. Resultados. Se encontraron 69 artículos con información relevante para llevar a cabo la presente revisión. Conclusiones. En neonatos prematuros, la base del tratamiento farmacológico del CAP continúa siendo los inhibidores no selectivos de la ciclooxigenasa, indometacina e ibuprofeno, ambos con perfiles similares de seguridad y eficacia. La evidencia disponible sugiere que el acetaminofén podría constituir una alternativa útil para el manejo, pero resulta insuficiente para realizar recomendaciones definitivas respecto a la eficacia y seguridad de este medicamento.

Abstract Introduction: Pharmacological management ofpatent ductus arteriosus (PDA) usually includes non-selective inhibitors of the enzyme cyclooxygenase, especially indomethacin and ibuprofen. Recently, acetaminophen has also been suggested as a therapeutic alternative. Objective: To conduct a narrative review of the literature on pharmacological management of PDA. Materials and methods: Structured literature search conducted on the ProQuest, EBSCO, ScienceDirect, PubMed, LILACS, Embase, Trip Database, SciELO and Cochrane Library databases, with the terms "Ductus Arteriosus, patentAND therapeutics"; "Ductus Arteriosus, patent AND indometacin"; "Ductus Arteriosus, Patent AND ibuprofen", and "Ductus Arteriosus, patent AND acetaminophen", and their equivalents in Spanish. Results: 69 articles had relevant information to carry out the present review. Conclusions: In premature infants, the mainstay of pharmacological treatment for PDA continues to be non-selective cyclooxygenase inhibitors, indomethacin and ibuprofen, all with similar safety and efficacy profiles. The available evidence suggests that acetaminophen may be a useful alternative for management, but it is insufficient to make definitive recommendations regarding the efficacy and safety of this drug.

A case control study of patent ductus arteriosus in preterm infants treated with acetaminophen and platelet-rich plasma / 中国新生儿科杂志

Objective To study the possible mechanisms,efficacy and safety of acetaminophen and platelet-rich plasma (PRP) on promoting the closure of ductus arteriosus in preterm infants.Method From January 2016 to May 2018,preterm infants (gestational age＜34 weeks) with symptomatic patent ductus arteriosus (sPDA) admitted to our neonatal intensive care unitwere enrolled prospectively.15 mg/kg of acetaminophen was orally given every 6 hours for three days (acetaminophen group).If acetaminophen was contraindicated (PRP group) or acetaminophen therapy failed (PRP rescue group),PRP transfusion was given at a single dose of 20 ml/kg.Echocardiogram,platelet-derived growth factors (PDGF) and urinary prostaglandin E2 (PGE2) were examined before and 72 hours after treatment.The data were analyzed using ANOVA,student's t test,Kruskal-Wallis H method and chi-square test.Result A total of 70 cases were enrolled.61 cases were treated with acetaminophen.The success rate (67.2％,41/61) was similar to PRP group (6/9) (P=1.000),and significantly higher than PRP rescue group (6/17) (P=0.017).The complications in acetaminophen group included 22 cases of hyperbilirubinemia (36.1％),5 cases of upper gastrointestinal hemorrhage (8.2％),4 cases of positive fecal occult blood test (6.6％),3 cases of oliguria (4.9％),1 case of Grade Ⅲ ～ Ⅳ intraventricular hemorrhage (IVH) (1.6％),and 1 case of≥ Ⅱ stage necrotizing enterocolitis (NEC) (1.6％).A total of 3 cases of hyperbilirubinemia were in PRP group.One case of hyperbilirubinemia,one oliguria,one Grade Ⅲ～ Ⅳ IVH and one ≥ Ⅱ stage NEC were in PRP rescue group.The urinary PGE2 level in post-treatment was lower than pre-treatment in acetaminophen group (t=6.878,P＜0.001).The blood platelet count and PDGF level in post-treatment were higher than pre-treatment in PRP group (t=-2.496,-8.906;P=-0.037,＜0.001) and PRP rescue group (t=-3.374,-2.503;P=0.004,0.024).Conclusion Oral acetaminophen and PRP transfusion had similar efficacy in promoting the closure of ductus arteriosus in preterm infants.If oral acetaminophen was contraindicated or failed,PRP transfusion could be one of the candidates for rescue therapy to promote the closure of ductus arteriosus in preterm infants.

Clinical study of transcervical occlusion of patent ductus arteriosus via femoral artery guided by ultrasound / 中国医师杂志

Objective To evaluate the efficacy and safety of transthoracic echocardiography (TTE) guided by transcatheter closure of patent ductus arteriosus (PDA) through the femoral artery.Methods From February 2016 to December 2017,53 patients with simple PDA were selected,the funnel type in 30 cases,cast type in 23 cases.All patients were guided by TTE and transcatheter closure of PDA through the femoral artery.The femoral artery was guided by TTE by establishing the orbit of the femoral artery-catheter-pulmonary artery to the right ventricle.The right ventricular septal defect was placed under the femoral artery approach,and the therapeutic effect was evaluated by echocardiography after occlusion.The outpatient visits were made at 1,3,and 6 months after operation.Results PDA closure was successfully completed under TTE guidance in 53 patients.In one adult PDA,the ultrasonic diagnosis of the inner diameter of the catheter was incorrect due to the image quality,which resulted in the replacement of the occluded device several times,but the occlusion was successful.The operative time was (30 ± 10)min.And the back diameter of the transcatheter closure device was (12 ± 6) mm and the murmur of the transmission sheath disappeared.There was no shunt between the main pulmonary artery and the main pulmonary artery,and the length of hospitalization was 3-4 days.No shunt signal was found at 1,3 and 6 months follow-up.Left atrial diameter (LAD) [(25.8 ± 6.1) mm vs (30.6 ± 8.4) mm] and left ventricular end diastolic diameter (LVEDD) [(38.5 ±9.1)mm vs (45.2 ± 11.5)mm],were decreased significantly (P ＜0.05).Conclusions It is safe and effective to block PDA by using femoral artery approach under the guidance of TTE,which avoids the damage of X-ray and contrast agent,and its prospect of clinical application is promising.

The predictive value of platelet associated indicators on hemodynamically significant patent ductus arteriosus in preterm infants / 中国新生儿科杂志

Objective To study the predictive value of platelet index on hemodynamically significant patent ductus arteriosus (hsPDA) in preterm infants.Method The data of 120 preterm infants born between January 2015 and December 2016 were retrospective analyzed,including 60 cases of non-hsPDA (nhsPDA) group and 60 cases of hsPDA group.The clinical features and platelet associated indicators between nhsPDA group and nhsPDA group were compared.Multivariate Logistic regression was used to analyze the effects of various factors on the occurrence of hsPDA.Receiver operating characteristic (ROC) curve was used to evaluate the early predictive value of platelet associated indicators for hsPDA.Result The hsPDA group had statistically significant differences in many parameters (P ＜ 0.05) comparing with the nhsPDA group,including smaller gestational age,lower birth weight and Apgar's score (1 min and 5min),lower proportion of cesarean section and preeclampsia,lower mean platelet volume and platelet distribution width (PDW),bigger PDA diameter and diameter2/birth weight,more serious respiratory distress syndrome,and a higher rate of pulmonary surfactant use.Multivariate Logistic regression analysis indicated that the risk of hsPDA was increased as the PDW decreased (OR =1.240,95％ CI 1.011 ～ 1.521).The ROC curve analysis showed that the best diagnostic value of PDW was 12.9％,and the sensitivity of early prediction of hsPDA was about 53.3％,and the specificity was 78.3％.Conclusion PDW has certain early predictive value for hsPDA in preterm infants.Preterm infants with PDW ＜ 12.9％ need to be monitored closely for the occurrence of hsPDA.

Significance of monitoring non-invasive cardiac output parameters on the treatment of premature infants with patent ductus arteriosus / 中国新生儿科杂志

Objective To evaluate the value of monitoring non-invasive cardiac output parameters in medical treatment of patent ductus arteriosus (PDA) in premature infants.Method Premature infants with PDA diagnosed three days after birth (gestational age:28 ～ 31 weeks or birth weight of 1 000 ～ 1 799 g) admitted to the neonatal intensive care unit (NICU) of our Hospital from February 2016 to August 2016 were enrolled in the study.These premature infants were assigned into treated PDA group (the treatment group) and untreated PDA group (the observation group) based on results of non-invasive cardiac output parameters CI and MD,with aorta CI ≥2.95 L/(min · m2),MD ≥21.50 m/min and pulmonary artery CI ≥4.55 L/(min · m2),MD ≥26.50 m/min as cut-off values.Statistical analysis was carried out using t test,x2 test.The closure rate of arterial duct of two groups and changes in non-invasive cardiac output parameters before and after the closure of arterial duct in the treatment group were compared.Result The overall closure rate of arterial duct was 85.1％ (57/67).The closure rate of arterial duct of the treatment group was 70.8％ (17/24),that of the observation group was 93.0％ (40/43),and the difference had statistical significance (P ＜ 0.05);Comparing the following parameters before and after ductal closure in the treatment group,the difference of pulmonary artery flow time (FT),aorta stroke volume index (SVI) and the integral of the flow profile (Vti) had statistical significance (P ＜ 0.05) [(217.6±19.3) ms vs.(235.8 ±21.4) ms,(22.4±6.0)ml/m2 vs.(25.2 ±7.7)ml/m2,(15.1 ± 4.1) cm vs.(17.2 ±5.3) cm].In the treatment group,after arterial duct was closed,aorta and pulmonary artery CI,MD decreased to some degree,but the difference had no statistical significance (P ＞ 0.05).Conclusion Non-invasive cardiac output parameters including aorta and pulmonary artery CI,MD have certain guiding significance for PDA drug treatment among premature infants;after PDA drug treatment,arterial duct closure condition cannot be judged simply by the changes of aorta and pulmonary artery CI,MD,ultrasonic cardiogram examination results should also be considered.

Hemodynamic Effects on Systemic Blood Flow and Ductal Shunting Flow after Loading Dose of Intravenous Caffeine in Preterm Infants according to the Patency of Ductus Arteriosus

BACKGROUND: In preterm infants, caffeine citrate is used to stimulate breathing before they are weaned from mechanical ventilation and to reduce the frequency of apnea. In recent studies, effects of caffeine on the cardiovascular system have been emphasized in preterm infants with patent ductus arteriosus (PDA). METHODS: This study aimed to assess the short-term hemodynamic effects on systemic blood flow and ductal shunting flow after loading standard doses of intravenous caffeine in preterm infants. Echocardiographic studies were performed by a single investigator, before and at 1 hour and 4 hours after an intravenous infusion of a loading dose as 20 mg/kg caffeine citrate for 30 minutes. RESULTS: In 25 preterm infants with PDA, left ventricular output decreased progressively during 4 hours after caffeine loading. Superior vena cava (SVC) flow decreased and ductal shunting flow increased at 1 hour and then recovered at 4-hour to baseline values. A diameter of PDA significantly decreased only at 4-hour after caffeine loading. There were no significant changes of these hemodynamic parameters in 29 preterm infants without PDA. CONCLUSION: In preterm infants with PDA, a standard intravenous loading dose of 20 mg/kg caffeine citrate was associated with increasing ductal shunting flow and decreasing SVC flow (as a surrogate for systemic blood flow) 1 hour after caffeine loading, however, these hemodynamic parameters recovered at 4 hours according to partial constriction of the ductus arteriosus. Close monitoring of hemodynamic changes would be needed to observe the risk for pulmonary over-circulation or systemic hypo-perfusion due to transient increasing ductal shunting flow during caffeine loading in preterm infants with PDA.

Multisystemic smooth muscle dysfunction syndrome in children: a case report and literature review / 中华儿科杂志

Objective@#To analyze the clinical characteristics and diagnosis of multisystemic smooth muscle dysfunction syndrome(MSMDS).@*Method@#Clinical data of a case diagnosed as MSMDS and hospitalized in our hospital in July 2016 was retrospectively analyzed. Literature search was performed at databases of PubMed, Wanfang, China National Knowledge Infrastructure and VIP with the key words "multisystemic smooth muscle dysfunction syndrome" "ACTA2" . The literature retrieval was confined from January 1980 to November 2016.The characteristics of MSMDS were summarized through review of literature.@*Result@#A girl aged 1.6 years had recurrent cough and wheeze for more than 1 year, complicated with congenital fixed dilated pupils, patent ductus arteriosus, pulmonary hypertension, chronic lung disease, and cerebrovascular abnormalities. We had done gene analysis for the patient and found ACTA2 c. 536C>T(p.R179H) heterozygous mutations, but her parents were normal. Totally 11 reports were retrieved from foreign language literature and no report from Chinese literature could be found; the retrieved articles reported a total of 25 cases of multiple system smooth muscle dysfunction syndrome. The minimum age was 11 months, 17 cases were female, 8 were male. The clinical common characteristic is congenital fixed dilated pupils, patent ductus arteriosus, cerebrovascular disease, pulmonary hypertension, chronic lung disease, and so on.@*Conclusion@#Genetic testing for ACTA2 gene mutations should be considered in infants presenting with congenital fixed dilated pupils and patent ductus arteriosus.

Evaluation of Pulmonary Hypertension Using Tricuspid Regurgitation Spectrum / 中国医学影像学杂志

Purpose The mean pulmonary artery (MPAP) has been widely used as an important parameter to diagnose and evaluate pulmonary hypertension (PH).The purpose of this paper is to compare the efficacy of two methods in evaluating PH,including estimating pulmonary artery systolic pressure (PASP) using Doppler ultrasonography to measure tricuspid regurgitation (TR) velocity,and directly using the peak velocity of TR.Materiasl and Methods From January 2012 to June 2013,eighty patients with left-to-right shunt congenital heart diseases (CHD) planned for closure procedure in Tangdu Hospital of the Fourth Military Medical University and the General Hospital of Shenyang Military region were included in this prospective study,who underwent right heart catheterization to measure pulmonary artery pressure,and underwent Doppler ultrasonography to measure the peak velocity ofTR.Results Using catheter-measured MPAP of≥ 25 mmHg as diagnostic reference,the false positive rate was 62.96％,and the false negative rate 0％ when the estimated PASP of ＞30 mmHg determined by TR method was used to diagnose PH.There was high diagnostic agreement when peak velocity of TR was used to diagnose PH.When 320 cm/s and 340 crn/s were used as diagnostic cutoff values,false positive rates were 14.81％ and 7.41％,and false negative rates were 15.91％ and 20.45％,respectively.Conclusion In patients with left-to-right shunt CHD,peak velocity of TR measured on echocardiography can be used to diagnose PH which overcomes the high false positive rate in estimation method.It is more suitable to diagnose PH when the MPAP is used as the diagnostic criterion.

The use of B-type natriuretic peptide and N-terminal pro-B-type natriuretic peptide concentrations to guide diagnosis and treatment of patent ductus arteriosus in preterm infants / 中国医师杂志

The high morbidity and compliments of patent ductus arteriosus (PDA) in preterm infants are reported,but the diagnostic and prognostic appraisal of PDA is still debatable.The B-type natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) are found efficient in diagnosis of hemodynamically significant patent ductus arteriosus (hsPDA) in preterm infants,and also helpful in therapy decisions and prediction of treatment response.However,the type of assay and cut-off values have not yet reached a consent,so that further researches are needed.