Prenatal diagnosis of hypoplastic left heart syndrome: current knowledge / Diagnóstico pré-natal da síndrome hipoplásica do coração esquerdo: conhecimentos atuais

Abstract Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the left-sided heart structures. The prenatal diagnosis of this congenital heart disease is crucial because a newborn with undiagnosed HLHS often presents with clinical signs of low cardiac output once the ductus arteriosus begins to close. With that in mind, the aim of this article was to perform a non-systematic review focusing on the key ultrasound features that can be used in the prenatal diagnosis of HLHS. Severe forms of HLHS are characterized by a markedly abnormal four-chamber view of the fetal heart (small left atrium, hypoplastic left ventricle, or abnormal mitral valve). The left ventricular outflow tract view allows the degree of hypoplasia in the tract to be evaluated and the diameter of the ascending aorta to be measured. The Z-scores are intended to aid in the diagnosis and follow-up of HLHS. In mild forms of HLHS, a right ventricle/left ventricle length ratio > 1.28 was the strongest predictor of a univentricular outcome.

Resumo A síndrome do coração esquerdo hipoplásico (SCEH) é caracterizada pelo subdesenvolvimento das estruturas cardíacas do lado esquerdo. O diagnóstico pré-natal dessa cardiopatia congênita é crucial, uma vez que recém-nascido com SCEH não diagnosticado apresenta, frequentemente, sinais clínicos de baixo débito cardíaco, quando o canal arterial começa a se fechar. Por isso, o objetivo deste artigo foi realizar uma revisão não sistemática sobre as principais características ultrassonográficas que podem ser usadas no diagnóstico pré-natal da SCEH. As formas graves de SCEH são caracterizadas por plano de quatro câmaras marcadamente anormal (átrio esquerdo pequeno, ventrículo esquerdo hipoplásico, válvula mitral anormal) do coração fetal. A visualização da via de saída do ventrículo esquerdo permite a avaliação do grau de hipoplasia dessa via e a mensuração da aorta ascendente. Os escores Z têm como objetivo auxiliar no diagnóstico e acompanhamento da SCEH. Nas formas leves da SCEH, a relação comprimento do ventrículo direito/comprimento do ventrículo esquerdo > 1,28 foi a variável mais forte para identificar o desfecho univentricular.

Use of Extracorporeal Membrane Oxygenation and Cerebral Oximetry in a Stage 1 Norwood Repair for Hypoplastic Left Heart Syndrome

The role of extracorporeal membrane oxygenation (ECMO) and its indications in stage I Norwood palliation are controversial. The decision to initiate ECMO and its timing remains difficult with no definitive cut?off points or evidence?based guidelines. It varies on a case?to?case basis. We report a case where the use of ECMO was beneficial after stage I Norwood palliation with severe ventricular dysfunction. The systemic?to?pulmonary artery shunt was kept open to balance the systemic and pulmonary circulations. Cerebral oximetry can be useful as an additional monitoring modality to guide management, monitor cerebral perfusion, and help detect cerebral steal.

Associated Factors with Congenital Heart Disease in the Most Populated State of Brazil Between 2010 and 2018

Abstract Background Cardiac anomalies are the most prevalent congenital malformations among live births in the world. In Brazil, it is estimated that nearly 25,757 new cases occur each year, and the Southeast region presents the highest prevalence, with 10 new cases/1,000 live births. Objective The aim of this study is to evaluate the epidemiology of congenital heart disease (CHD) in the state of São Paulo. Methods This is a cross-sectional and time-series observational study with data from the Brazilian Information System on Live Births. Heart-related cardiac malformation cases — International Classification of Diseases (ICD) 10th revision Q20.0 to Q24.9 — were selected from January 2010 to December 2018, in the state of São Paulo, Brazil. This study analyzed rates of malformations per year and investigated associated factors, using single and multiple logistic regression models. The significance level adopted in this study was 5%. Results The highest cardiac malformation rate was in the São Paulo metropolitan region (2.84:1000), while the lowest was found in the region of Franca (0.3:1000). The most frequent defect was interatrial communication (38.2%). The main associated factors observed in this study were prematurity of 22 to 27 weeks (OR=4,401 95% CI: 3,796-5,104) — CI: Confidence Interval; OR: Odds ratio —, mother's age between 35 and 49 years of age (OR=1,602 95% CI: 1,525-1,682), yellow race (OR=1,481 95% CI: 1,235-1,775), triple or more pregnancy (OR=1,438 95% CI: 1,004-2,060), and history of a dead child (OR=1,213 95% CI: 1,152-1,277). Conclusion The main factors associated with this outcome, which are part of the obstetric history of mothers, should be addressed and considered when pregnancy is a planned event.

A Systematic Review of Benefits and Risks of Fetal Surgery for Congenital Cardiac Defects Such as Pulmonary Valve Stenosis and Critical Aortic Stenosis

ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

Resultados Precoces do Procedimento de Norwood em um Centro de Referência no Brasil / Early Outcomes of the Norwood Procedure in a Reference Center in Brazil

Resumo Fundamento Apenas dois artigos abordam os resultados precoces de pacientes com síndrome do coração esquerdo hipoplásico (SHCE) submetidos à operação de Norwood, no Brasil. Objetivos Avaliamos pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood para identificar os fatores preditivos de mortalidade precoce (nos primeiros 30 dias após a cirurgia) e intermediária (desde a sobrevida precoce até o procedimento de Glenn). Métodos Foram incluídos pacientes com SHCE submetidos em nosso serviço ao primeiro estágio da operação de Norwood de janeiro de 2016 a abril de 2019. Dados demográficos, anatômicos e cirúrgicos foram analisados. Os desfechos foram mortalidade precoce (nos primeiros 30 dias após a cirurgia), mortalidade intermediária (desde a sobrevida precoce até o procedimento de Glenn) e a necessidade de suporte pós-operatório com ECMO. Foram realizadas análises univariadas e multivariadas e calculados odds ratios, com intervalos de confiança de 95%. Um valor de p < 0,05 foi considerado estatisticamente significativo. Resultados Um total de 80 pacientes com SHCE foram submetidos ao primeiro estágio da operação de Norwood. A taxa de sobrevida em 30 dias foi de 91,3% e a taxa de sobrevida intermediária foi de 81,3%. Quatorze pacientes (17,5%) necessitaram de suporte com ECMO. Menor peso (p=0,033), estenose aórtica (vs atresia aórtica; p=0,036) e necessidade de suporte pós-operatório com ECMO (p=0,009) foram fatores preditivos independentes para mortalidade em 30 dias. A estenose da valva mitral ( vs atresia da valva mitral; p=0,041) foi um fator preditivo independente para mortalidade intermediária. Conclusão O presente estudo inclui a maior coorte brasileira de pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood na era recente. Nossas taxas de sobrevida foram comparáveis às mais altas taxas de sobrevida relatadas globalmente. Baixo peso corporal, estenose valvar aórtica e necessidade de suporte pós-operatório com ECMO foram preditores independentes para mortalidade em 30 dias. A estenose da valva mitral foi o único fator preditivo independente para mortalidade intermediária.

Abstract Background Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil. Objectives We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality. Methods Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled. Demographic, anatomical, and surgical data were analyzed. Endpoints were early mortality (within the first 30 days after surgery), intermediate mortality (from early survival up to the Glenn procedure) and the need for postoperative ECMO support. Univariate and multivariate analyses were performed, and odds ratios, with 95% confidence intervals, were calculated. A p-value <0.05 was considered statistically significant. Results A total of 80 patients with HLHS underwent the stage I Norwood procedure. The 30-day survival rate was 91.3% and the intermediate survival rate 81.3%. Fourteen patients (17.5%) required ECMO support. Lower weight (p=0.033), aortic stenosis (vs aortic atresia; p=0.036), and the need for postoperative ECMO support (p=0.009) were independent predictive factors for 30-day mortality. Mitral valve stenosis (vs mitral valve atresia; p=0.041) was an independent predictive factor for intermediate mortality. Conclusion The present study includes the largest Brazilian cohort of patients with HLHS undergoing the stage I Norwood procedure in the recent era. Our survival rates were comparable to the highest survival rates reported globally. Low body weight, aortic valve stenosis, and the need for postoperative ECMO support were independent predictors for 30-day mortality. Mitral valve stenosis was the only independent predictive factor for intermediate mortality.

Spinal Anesthesia for Open Gastrostomy in an Infant after Stage I Norwood for Hypoplastic Left Heart

Infants with hypoplastic left heart are at increased risk of adverse events including mortality when they undergo procedures with general anesthesia in the inter?stage period after stage I Norwood. This is primarily caused by an imbalance between pulmonary and systemic blood flows augmented by decreased function of the single ventricle. These factors can be aggravated by general anesthesia, hence the increased risk. Many of these infants experience feeding dysfunction and require a gastrostomy to optimize nutrition. We report a case of open gastrostomy in an infant with Norwood physiology under spinal anesthesia with an excellent outcome.

Manejo anestésico en síndrome corazón izquierdo hipoplásico: Reporte de un caso / Anesthetic management in hypoplastic left heart syndrome: A case report

Introducción: El síndrome corazón izquierdo hipoplásico puede presentarse con signos clínicos inmediato al nacimiento, siendo responsable de las muertes neonatales en la primera semana de vida. El reconocimiento clínico y los hallazgos ecocardiográficos son claves para un diagnóstico y tratamiento oportuno. Reporte de caso: describe el manejo anestésico de un neonato con inestabilidad hemodinámica que requirió cirugía de emergencia con banding pulmonar bilateral para mantener el equilibrio entre la relación del flujo sanguíneo pulmonar y sistémico cercano a la unidad, junto a maniobras de ventilación mecánica, medicamentos anestésicos, inotrópicos y adyuvantes con el objetivo de manipular las resistencias vasculares y el flujo sanguíneo logrando estabilidad hemodinámica del paciente que permitió al quinto día ser sometido a una cirugía electiva de Norwood Sano, tolerando procedimiento con extubación a los 15 días de la cirugía. Conclusión: el momento oportuno de la indicación quirúrgica aumenta la sobrevida de los pacientes síndrome corazón izquierdo hipoplásico.

Background: Hypoplastic left heart syndrome may appear with clinical signs immediately at birth, being responsible for neonatal deaths within the first week of life. Clinical recognition and echocardiographic findings are key to timely diagnosis and treatment. Case report: describes the anesthetic procedure of a neonate with hemodynamic instability who required emergency surgery for bilateral pulmonary banding to maintain the balance between pulmonary and near-unit systemic flow ratio, along with mechanical ventilation maneuvers, anesthetic, inotropic and adjuvant drugs to control vascular resistance and blood flow, thus achieving hemodynamic stability of the patient, which allowed him to undergo an elective Norwood Sano procedure on the fifth day, and which was tolerated with extubation 15 days after surgery. Conclusion: The right timing of the surgical indication increases the survival of patients with hypoplastic left heart syndrome.

The study on brain biometry and cerebral hemodynamics in fetuses with hypoplastic left heart syndrome in the second trimester of gestation / 中华超声影像学杂志

Objective:To explore the changes of biparietal diameter, head circumference and cerebrovascular hemodynamics in fetuses with hypoplastic left heart syndrome (HLHS) during middle pregnancy.Methods:The biparietal diameter, head circumference, middle cerebral artery pulsatility index (MCA-PI), umbilical artery pulsatility index (UA-PI) and MCA-PI/UA-PI (CPR) of 41 fetuses with HLHS(HLHS group) were retrospectively analyzed from January 2015 to December 2019 in Beijing Anzhen Hospital, and were compared with those of 82 normal fetuses matched for gestational age at the same period (control group).Results:The Z-scores of head circumference, MCA-PI and CPR in with HLHS group were lower than in control group(all P<0.05); Head circumference in HLHS group were weakly and positively correlated with the MCA-PI and CPR ( r=0.385, 0.416; all P<0.05). Conclusions:There are some changes in the head circumference and cerebral hemodynamics in fetuses with HLHS during mid-gestational age, and the head circumference is weakly and positively correlated with MCA-PI and CPR, which has clinical significance.

Surgical Treatment for Occlusion of Left Coronary Artery Ostium by an Aortic Valve Cusp / 日本心臓血管外科学会雑誌

The patient was a 4-year-old boy, with no indication of heart disease at birth. When he was 4 years and 3 months old, he underwent surgery for cryptorchidism, and persistent changes after crying, consisting of ST-segment depression, were noted on the ECG. Therefore, it was decided that a detailed examination would be carried out. Aortography revealed that a small left coronary cusp had formed a pouch and that there was a delayed washout of the contrast material in the left coronary artery compared with the right coronary artery. Right coronary angiography showed retrograde flow to the left coronary artery. There was no significant aortic regurgitation, and no stenosis in the left coronary artery. Intraoperative findings demonstrated that the hypoplastic left coronary cusp had adhered to the aortic wall and covered the left sinus of Valsalva. Enlargement of the Valsalva was performed by placing a patch from the aortic transection site to an area directly above the coronary artery ostium. Postoperatively, the patient recovered satisfactorily and was discharged on the eleventh postoperative day. Cardiac catheterization performed 6 months after surgery showed no aortic regurgitation, no significant stenosis in the left coronary artery, and no retrograde flow from the right coronary artery. We report our experience with the rare case described above.

Diagnóstico Prenatal de Fibroelastosis Endocardica

Resumen La fibroelastosis endocárdica es una cardiopatía congénita caracterizada por presentar un engrosamiento difuso del endocardio, esto condiciona una miocardiopatía dilatada en la mayoría de los casos o menos frecuente una miocardiopatía restrictiva. Puede ser una patología primaria o secundaria a otras malformaciones cardíacas, sobre todo a una estenosis o atresia de la aorta. Se estima que la incidencia global al nacimiento es de 1 de cada 5 000 y de 4 por cada 100 cardiopatías congénitas. Se presenta el caso clínico de una gestante de 37 años de edad, quien fuera ingresada a las 19.1 semanas de gravidez en el Hospital Obrero N°2 de Cochabamba-Bolivia, por el diagnóstico ecográfico prenatal de cardiopatía fetal estructural, fibroelastosis endocárdica.

Abstract Endocardial fibroelastosis is a congenital heart disease characterized by diffuse thickening of the endocardium, this conditions a dilated cardiomyopathy in most cases or less frequent restrictive cardiomyopathy. It can be a primary pathology or secondary to other cardiac malformations, especially a stenosis or atresia of the aorta. The global incidence at birth is estimated to be 1 in 5 000 and 4 in 100 congenital heart disease. The clinical case of a 37-year-old pregnant woman is presented, who was admitted at 19.1 weeks of pregnancy in Hospital Obrero N ° 2 in Cochabamba-Bolivia, due to the prenatal ultrasound diagnosis of structural fetal heart disease, endocardial fibroelastosis.

Cleıdocranıal Dysplasıa: A Rare Skeletal Dysplasıa

ABSTRACT Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal dysplasia. Hypoplastic clavicles, delayed closure of the cranial sutures, dental abnormalities, and short stature are main features in this syndrome. RUNX2 is the responsible gene for CCD. Here we reported two cases with CCD; they are admitted to clinic for short stature and dental abnormalities. We report these cases to increase the awareness of clinicians

Extubación fallida postoperación de Norwood en pacientes con síndrome de hipoplasia de ventrículo izquierdo / Extubation failure after Norwood procedure in hypoplastic left heart syndrome patients

Resumen: Introducción: En pacientes con Síndrome de Hipoplasia de Ventrículo Izquierdo (SHVI) la primera etapa hacia una fisiología univentricular de Fontan es la operación de Norwood, cuya mortalidad es 10-30%. En estos pacientes la extubación fallida se presenta en un 18% y se ha asociado a aumento de la mortali dad. Objetivo: Describir la frecuencia de extubación fallida y sus factores de riesgo en pacientes con SHVI operados (Norwood). Pacientes y Método: Estudio de casos y controles que incluyó a todos los pacientes con SHVI manejados con cirugía de Norwood en el Hospital Clínico de la Pontificia Universidad Católica (enero-2000 a febrero-2018). Se define como casos a pacientes con falla en la extubación y como controles a los pacientes sin esta complicación. Se registraron variables demográficas, quirúrgicas, y post-quirúrgicas, y se realizó análisis univariado y multivariado (regresión logística) para determinar los factores de riesgo asociados a la falla en la extubación. Resultados: De un total de 107 pacientes, 26 pacientes tuvieron extubación fallida (24,3%). En el análisis univariado los factores asociados a extubación fallida fueron: mayor tiempo de ventilación mecánica postquirúrgico, desarrollar atelectasias, derrame pleural, quilotórax, tener otras comorbilidades respiratorias (apnea y traqueítis), y mayor tiempo de uso de morfina y midazolam. En el análisis multivariado, la presencia de quilotórax, otras comorbilidades respiratorias, y mayor tiempo de uso de midazolam fueron variables asociadas a extubación fallida (p<0,03). La extubación fallida no se asoció a mayor mortalidad. Conclusiones: La presencia de quilotórax, complicaciones respiratorias y uso de mida zolam prolongado deben considerarse para definir el momento de la extubación, con el objetivo de evitar su fracaso.

Abstract: Introduction: Hypoplastic left heart syndrome (HLHS) is the main cause of mortality due to congenital heart disea se. The Norwood surgery is the first procedure of the surgical staging process towards a single ventri cle physiology or Fontan-type operation and has a mortality rate of 10% to 30%. Extubation failure during the postoperative period occurs in up to 18% of these patients and is associated with increased mortality. Objective: To describe extubation failure rates and risk factors in pediatric patients with HLHS who underwent Norwood procedure. Patients and Method: Case-control study that included all the patients with HLHS managed with Norwood surgery at the Hospital Clínico de la Pontificia Universidad Catolica between January 2000 and February 2018. Cases and controls were defined as patients with extubation failure and as patients without this complication, respectively. The following variables were recorded demographic, surgical, and post-surgical ones, and univariate and multivariate analyses (logistic regression) were performed to determine risk factors associated with extubation failure. Results: Out of 107 patients, 26 of them presented extubation failure (24.3%). In the univariate analysis, longer mechanical ventilation time during the postsurgical period, atelectasis, pleural effusion, chylothorax, other respiratory morbidities (i.e. apneas and tracheitis), and longer infusion times of morphine and midazolam, all were associated with a higher extubation failure rate in this population. In the multivariable analysis, chylothorax, other respiratory comorbidities, and longer infusion time of midazolam remained associated with this complication, however, it was not associated with higher mortality. Conclusions: Chylothorax, respiratory comorbidities, and longer use of Midazolam should be addressed before planning airway extubation in order to avoid failure.

Síndrome del corazón izquierdo hipoplásico / Hypoplastic Left Heart Syndrome

Introducción: El síndrome del corazón izquierdo hipoplásico es una rara anormalidad que se presenta en el periodo prenatal. Sus manifestaciones clínicas se relacionan con la presencia de hipoxemia a nivel sistémico. Objetivo: Socializar las manifestaciones clínicas y elementos diagnósticos del síndrome del corazón izquierdo hipoplásico. Caso clínico: Recién nacido masculino, a término, que presenta manifestaciones clínicas y ecosonográficas que permiten llegar al diagnóstico de un síndrome del corazón izquierdo hipoplásico. Conclusiones: A pesar de los avances de la ciencia y la técnica, el síndrome del corazón izquierdo hipoplásico presenta una elevada mortalidad. Una adecuada planificación de la gestación, el seguimiento preciso del embarazo, un correcto asesoramiento genético y la utilización de técnicas quirúrgicas para corregir las deformidades resultantes parecen ser las herramientas más eficaces para enfrentar esta mortal afectación(AU)

Introduction: Hypoplastic left heart syndrome is a rare abnormality that occurs in the prenatal period. Its clinical manifestations are related to the presence of hypoxemia at the systemic level. Objective: To describe the clinical manifestations and diagnostic elements of hypoplastic left heart syndrome. Clinical case: A male newborn, at term, who presented clinical and echographic manifestations that permitted the diagnosis of hypoplastic left heart syndrome. Conclusions: Despite advances in science and technology, hypoplastic left heart syndrome presents a high mortality. Proper planning for pregnancy, accurate pregnancy monitoring, proper genetic counseling, and the use of surgical techniques to correct the resulting deformities seem to be the most effective tools to deal with this deadly condition(AU)

Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle

Minor alleles of genetic variants in second heart field increase the risk of hypoplastic right heart syndrome

Hypoplastic right heart syndrome(HRHS) is characterized by hypoplastic right ventricle (RV); Numerous transcriptional cascades in the second heart field (SHF) regulateRVdevelopment. The relationship of SHF gene variants with humanHRHS remains unknown. The whole lengths of 17 SHF genes were sequenced in 16 HRHS, and the selected single-nucleotide variants (SNVs) were then genotyped in HRHS, other congenital heart disease (CHD) and healthy control. Luciferase assay was performed to verify the effect of FOXC2: rs34221221A>GandTBX20: rs59854940C>Gat the transcription level. There were 151 (12.86%) novel SNVs after sequence analysis, of which three were in exons (one was synonymous SNV and two were nonsynonymous SNVs), two in promoter, and most SNVs (89.95%) were in intronic regions. Genotype analyses revealed that the minor alleles of FOXC2: rs34221221 A>G and TBX20: rs59854940 C>G could increase HRHS risk (P<0.05), but not in other CHD or healthy control. Luciferase assay showed that the minor G allele in rs34221221 significantly increased FOXC2 transcription while in rs59854940 it decreased TBX20 transcription significantly. Novel variants of SHF gene associated with HRHS were identified. Minor alleles in two variants from FOXC2 and TBX20 could increase the risk of HRHS.

A Morphometric Study Of The Intracranial Vertebral Artery In Human Cadavers

Background: Vertebral artery radically contributes to the blood supply of brain. Any variation in its form willsignificantly alter the circulation dynamics in the brain leading to fatal consequences. The aim of the study wasto measure the diameter of the intracranial part of vertebral artery on both right and left sides.Materials and method: The study was conducted in Anatomy Department on 30 brains from the cadavers donatedto the Medical College Baroda. The diameter of the artery was measured bilaterally with digital vernier caliperand results were statistically analyzed. The data is presented as mean+ SD. Students’ t test has been applied andobservation with P<0.05 is considered significant.Result: The current study observed the mean diameter (mm) on the left side to be greater than on the right. One ofarteries on the left side was also found to be hypoplastic.Conclusion: The results will have wider implications on medical field as any variation in structure of the vertebralartery will compromise the blood flow to the brain. The study will be useful to neurologists, neurosurgeons,radiologists and anatomists alike

Emergency Pulmonary Artery–to-Systemic Artery Shunt to Break the Positive Feedback Loop of a Pulmonary Hypertensive Crisis after Neonatal Coarctation Repair / 대한흉부외과학회지

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.

Reconsideration of urine culture for the diagnosis of acute pyelonephritis in children: a new challenging method for diagnosing acute pyelonephritis / 소아과

Acute pyelonephritis (APN) should be detected and treated as soon as possible to reduce the risk of the development of acquired renal scarring. However, in the medical field, urine culture results are not available or considered when the prompt discrimination of APN is necessary and empirical treatment is started. Furthermore, urine culture cannot discriminate APN among children with febrile urinary tract infection (UTI) (pyelitis, lower UTI with other fever focus). Therefore, the usefulness of urine culture for diagnostic purposes is small and the sampling procedure is invasive. Congenital hypoplastic kidney is the most common cause of chronic kidney injury in children. Thus, it is desirable that a main target be detected as early as possible when imaging studies are performed in children with APN. However, if APN does not recur, no medical or surgical treatment or imaging studies would be needed because the acquired renal scar would not progress further. Therefore, the long-term prognosis of APN in young children, particularly infants, depends on the number of recurrent APN, not other febrile UTI. New methods that enable prompt, practical, and comfortable APN diagnosis in children are needed as alternatives to urinary catheterization for urine culture sampling.