RESUMO
ABSTRACT Objective: Pediatric cardiac tumors are rare and, among them, 90% are benign. Cardiac fibroma is the second most frequent tumor, after rhabdomyoma. The objective of this study is to report a case of cardiac fibroma diagnosed incidentally in a patient admitted with acute viral bronchiolitis. Case description: A 5-month-old male infant was admitted to the pediatric emergency department with acute viral bronchiolitis requiring hospitalization. He presented a detectable respiratory syncytial virus in oropharyngeal swab, blood test with lymphocytosis and a chest radiography revealed cardiomegaly. Further cardiologic testing was performed detecting elevation of cardiac biomarkers, an electrocardiogram with alteration of left ventricular repolarization and echocardiogram with a heterogeneous mass in the left ventricular, with areas of calcification. A chest angiotomography suggested rhabdomyosarcoma or cardiac fibroma and a magnetic resonance showed a mass, with characteristics suggesting fibroma. The final diagnosis was made after two cardiac catheterizations for biopsy of the lesion, confirming cardiac fibroma by anatomopathological examination. Because the patient had moderate to severe systolic dysfunction, he was submitted to heart transplant. Comments: One third of cardiac fibromas are asymptomatic, generally diagnosed late through tests ordered for other reasons. The gold-standard test for definitive diagnosis is biopsy. Cardiac fibroma usually does not present spontaneous regression and, in most cases, partial or total surgical resection is necessary. When tumors are unresectable, heart transplantation should be indicated. It is essential to have detailed characterization of the cardiac mass to establish the most appropriate therapeutic approach for each patient.
RESUMO Objetivo: Tumores cardíacos pediátricos são raros e, entre eles, 90% são benignos. O fibroma cardíaco é o segundo mais frequente, após o rabdomioma. O objetivo deste trabalho é relatar um caso de fibroma cardíaco, cujo diagnóstico foi incidental em um paciente internado por bronquiolite viral aguda. Descrição do caso: Lactente do sexo masculino com cinco meses foi atendido em um pronto atendimento pediátrico com quadro de bronquiolite viral aguda, necessitando de hospitalização. Em exames complementares, apresentou vírus sincicial respiratório detectável, hemograma com linfocitose, além de radiografia de tórax com cardiomegalia. Em investigação cardiológica, as enzimas cardíacas encontravam-se elevadas, o eletrocardiograma apresentava alteração de repolarização de parede lateral de ventrículo esquerdo e o ecocardiograma demonstrou massa heterogênea em ventrículo esquerdo, com áreas de calcificação. A angiotomografia de tórax sugeriu rabdomiossarcoma ou fibroma cardíaco e a ressonância magnética demonstrou massa cuja localização e características sugeriram fibroma. O diagnóstico foi conclusivo após dois cateterismos para biópsia da lesão, confirmando fibroma cardíaco pelo anatomopatológico. Em razão da disfunção sistólica moderada a grave, o paciente foi submetido a transplante cardíaco. Comentários: Um terço dos fibromas cardíacos é assintomático, geralmente diagnosticado tardiamente por meio de exames solicitados por outro motivo. O padrão-ouro para o diagnóstico definitivo é a biópsia. O fibroma cardíaco não costuma apresentar regressão espontânea, sendo submetido, na maioria dos casos, a ressecção cirúrgica parcial ou total. Nos tumores irressecáveis, o transplante cardíaco deve ser indicado. A caracterização detalhada da massa cardíaca é fundamental para traçar a conduta terapêutica mais adequada para cada paciente.
RESUMO
CLINICAL DATA: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. CHEST RADIOGRAPHY: Cardiomegaly with prevalence of pulmonary vascular network. ELECTROCARDIOGRAM: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. ECHOCARDIOGRAM: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. COMPUTED TOMOGRAPHY: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. DIAGNOSIS: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. OPERATION: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.
Assuntos
Cardiopatias Congênitas , Malformações Vasculares , Lactente , Humanos , Feminino , Veia Porta/diagnóstico por imagem , Veia Porta/anormalidades , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Veia Cava Inferior/diagnóstico por imagem , Cianose/complicações , Angiografia por Tomografia Computadorizada , Malformações Vasculares/diagnóstico , Malformações Vasculares/diagnóstico por imagemRESUMO
ABSTRACT Clinical data: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. Chest radiography: Cardiomegaly with prevalence of pulmonary vascular network. Electrocardiogram: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. Echocardiogram: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. Computed tomography: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. Diagnosis: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. Operation: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.
RESUMO
Resumo Fundamento Foi relatado que o RNA 1 antisenso 1 (SLC26A4-AS1) do membro 4 da família de transportadores de soluto 26 está altamente relacionado à hipertrofia cardíaca. Objetivo Esta pesquisa visa investigar o papel e o mecanismo específicos de SLC26A4-AS1 na hipertrofia cardíaca, fornecendo um novo marcador para o tratamento da hipertrofia cardíaca. Métodos Angiotensina II (AngII) foi infundida em cardiomiócitos ventriculares (NMVCs) de camundongos neonatos para induzir hipertrofia cardíaca. A expressão gênica foi detectada por PCR quantitativo em tempo real (RT-qPCR). Os níveis de proteína foram avaliados por western blot. Ensaios funcionais analisaram o papel de SLC26A4-AS1. O mecanismo de SLC26A4-AS1 foi avaliado por imunoprecipitação de proteína de ligação a RNA (RIP), pull-down de RNA e ensaios de luciferase repórter. O valor de p < 0,05 foi identificado como significância estatística. O teste t de Student avaliou a comparação dos dois grupos. A diferença entre os diferentes grupos foi analisada por análise de variância (ANOVA) de uma via. Resultados SLC26A4-AS1 é regulado para cima em NMVCs tratados com AngII e promove hipertrofia cardíaca induzida por AngII. SLC26A4-AS1 regula o membro 4 da família de transportadores de soluto 26 (SLC26A4) por meio do funcionamento como um RNA endógeno competitivo (ceRNA) para modular o microRNA (miR)-301a-3p e o miR-301b-3p em NMVCs. SLC26A4-AS1 promove hipertrofia cardíaca induzida por AngII via regulação para cima de SLC26A4 ou absorção de miR-301a-3p/miR-301b-3p. Conclusão SLC26A4-AS1 agrava a hipertrofia cardíaca induzida por AngII via absorção de miR-301a-3p ou miR-301b-3p para aumentar a expressão de SLC26A4.
Abstract Background It has been reported that solute carrier family 26 members 4 antisense RNA 1 (SLC26A4-AS1) is highly related to cardiac hypertrophy. Objective This research aims to investigate the role and specific mechanism of SLC26A4-AS1 in cardiac hypertrophy, providing a novel marker for cardiac hypertrophy treatment. Methods Angiotensin II (AngII) was infused into neonatal mouse ventricular cardiomyocytes (NMVCs) to induce cardiac hypertrophy. Gene expression was detected by quantitative real-time PCR (RT-qPCR). Protein levels were evaluated via western blot. Functional assays analyzed the role of SLC26A4-AS1. The mechanism of SLC26A4-AS1 was assessed by RNA-binding protein immunoprecipitation (RIP), RNA pull-down, and luciferase reporter assays. The P value <0.05 was identified as statistical significance. Student's t-test evaluated the two-group comparison. The difference between different groups was analyzed by one-way analysis of variance (ANOVA). Results SLC26A4-AS1 is upregulated in AngII-treated NMVCs and promotes AngII-induced cardiac hypertrophy. SLC26A4-AS1 regulates its nearby gene solute carrier family 26 members 4 (SLC26A4) via functioning as a competing endogenous RNA (ceRNA) to modulate the microRNA (miR)-301a-3p and miR-301b-3p in NMVCs. SLC26A4-AS1 promotes AngII-induced cardiac hypertrophy via upregulating SLC26A4 or sponging miR-301a-3p/miR-301b-3p. Conclusion SLC26A4-AS1 aggravates AngII-induced cardiac hypertrophy via sponging miR-301a-3p or miR-301b-3p to enhance SLC26A4 expression.
RESUMO
A foetal echocardiogram, in a 27-week foetus referred for cardiomegaly, demonstrated dextrocardia, absence of the ductus venosus, and an unrestricted unusual umbilical venous drainage to a left posterior intercostal vein, which continued to left hemiazygos vein and drained into the coronary sinus. Progressive cardiomegaly led to early delivery. To the best of our knowledge, no case with similar umbilical venous drainage has been previously reported.
Assuntos
Seio Coronário , Ultrassonografia Pré-Natal , Seio Coronário/diagnóstico por imagem , Ecocardiografia , Feminino , Feto , Humanos , Gravidez , Veias UmbilicaisRESUMO
Resumo Em 2019, um artigo publicado no European Heart Journal reconheceu pela primeira vez a insuficiência cardíaca (IC) com fração de ejeção do ventrículo esquerdo (FEVE) ≥ 65% como um novo fenótipo de IC, ou a insuficiência cardíaca com fração de ejeção supranormal (ICFEsn), com o objetivo principal de promover a investigação desta nova categoria. Eles analisaram a mortalidade em pessoas com IC e descobriram que havia uma relação em forma de U entre a mortalidade e a FEVE. Sendo assim, os pacientes com ICFEsn tinham uma mortalidade geral mais alta em comparação com outros pacientes diagnosticados com IC com fração de ejeção preservada (ICFEp). Este artigo descreve a situação atual da ICFEsn e discute as perspectivas futuras com base nos resultados preliminares de nosso grupo. Para melhor tratar os pacientes com ICFEsn, é fundamental que cardiologistas e médicos entendam as diferenças e semelhanças desse novo fenótipo.
Abstract In 2019, an article published in the European Heart Journal recognized for the first time heart failure (HF) with left ventricular ejection fraction (LVEF)≥ 65% as a new HF phenotype, heart failure with supra-normal left ventricular ejection fraction (HFsnEF), with the main purpose of promoting research on this new category. They analyzed mortality in people with HF and found that there was a u-shaped relationship between mortality and LVEF. Accordingly, HFsnEF patients had a higher all-cause mortality compared with other patients diagnosed with HF with preserved ejection fraction (HFpEF). This article describes the current situation of HFsnEF and discusses future perspectives based on the preliminary results of our group. To better treat patients with HFsnEF, it is fundamental that cardiologists and physicians understand the differences and similarities of this new phenotype.
Assuntos
Humanos , Função Ventricular Esquerda , Insuficiência Cardíaca , Prognóstico , Volume Sistólico , Fatores de TempoRESUMO
Resumen Paciente masculino de 18 años, quien es valorado en el Servicio de Cardiología del Hospital Víctor Manuel Sanabria Martínez referido del primer nivel de atención por cuadro crónico de dolor torácico atípico acompañado de pectus excavatum y desplazamiento izquierdo del latido de punta a la exploración física. La radiografía de tórax posteroanterior evidencia una cardiomegalia moderada con levocardia máxima. El ecocardiograma transtorácico con función sistólica biventricular conservada, dilatación moderada del ventrículo derecho, sin signos de hipertensión pulmonar e imagen cardiaca de "corazón en lágrima". Angiotomografía computarizada de tórax con contraste que documenta malformación de la caja torácica con hundimiento de la región esternal, en relación con pectus excavatum. Corazón aumentado de tamaño en relación con cardiomegalia grado I y desplazamiento hacia el hemitórax izquierdo. No se observa pericardio en ninguna región. Aurícula derecha levemente dilatada con un ventrículo derecho con diámetro aumentado en su porción media y deformidad de su tracto de salida debido a la forma de la caja torácica, de tal manera que se corrobora el diagnóstico de una agenesia pericárdica completa.
Abstract An 18-year-old male patient who is evaluated in the Cardiology Department of the Víctor Manuel Sanabria Martínez Hospital, referred to the first level of attention due to chronic symptoms of atypical chest pain accompanied by pectus excavatum and left displacement of the peak beat. Chest X-ray Posteroanterior with moderate cardiomegaly with maximum levocardia. Transthoracic echocardiogram with preserved biventricular systolic function, moderate dilatation of the right ventricle, without signs of pulmonary hypertension and cardiac image of "tear heart". Thoracic Computed Angiotomography with contrast documenting malformation of the rib cage with sinking of the external region, in relation to pectus excavatum. Heart enlarged in relation to grade I cardiomegaly and displacement towards the left hemithorax. No pericardium is observed in any region. Slightly dilated right atrium with a right ventricle with an increased diameter in its middle portion and deformity of its outflow tract due to the shape of the rib cage. Corroborating the diagnosis of complete pericardial agenesis.