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1.
Respirar (Ciudad Autón. B. Aires) ; 16(3): 303-309, sept.2024.
Article in Spanish | LILACS, UNISALUD, BINACIS | ID: biblio-1570683

ABSTRACT

Introducción: Entre 0,3-0,5% de niños nacidos presentan una vena cava superior izquierda persistente, lo que lo hace una de las malformaciones congénitas más frecuentes del drenaje venoso. El drenaje de esta en la aurícula derecha, además del drenaje de la vena cava superior derecha en la aurícula izquierda, es extremadamente infrecuente. Caso Clínico: Se presenta el caso de un infante de 8 meses asintomático que es llevado a consulta por presentar desaturaciones. El examen físico es normal. El ecocardiograma de contraste con solución salina muestra una vena cava superior izquierda persistente que drena en la aurícula derecha y un retorno anómalo de la vena cava superior derecha. Se realiza una corrección quirúrgica y evoluciona de forma favorable. Conclusión: La presentación en simultáneo de una vena cava superior izquierda persistente que drena en la aurícula derecha y una vena cava derecha que drena en la aurícula izquierda es extremadamente rara. La mayoría de los casos registrados se diagnosticaron de manera incidental en personas adultas al no presentar síntomas.


Introduction: Between 0.3-0.5% of children born have a persistent left superior vena cava, which makes it one of the most frequent congenital malformations of venous drainage. Drainage of this vein into the right atrium, in addition to drainage of the right superior vena cava into the left atrium, is extremely rare. Clinical case: We present a case of an asymptomatic 8-month-old infant who was taken to the clinic for desaturations. Physical examination was normal. The contrast echocardiogram with saline solution showed a persistent left superior vena cava draining into the right atrium and an anomalous return of the right superior vena cava. Surgical correction was performed and the patient evolved favorably. Conclusion: The simultaneous presentation of a persistent left superior vena cava draining into the right atrium and a right superior vena cava draining into the left atrium is extremely rare. Most of the reported cases were diagnosed incidentally in adults in the absence of symptoms.


Subject(s)
Humans , Male , Infant , Persistent Left Superior Vena Cava/diagnosis , Heart Defects, Congenital/surgery , Diagnostic Imaging , Foramen Ovale, Patent/diagnosis , Oxygen Saturation , Heart Atria/abnormalities , Hypoxia
3.
ABC., imagem cardiovasc ; 35(3): eabc280, 2022. tab, ilus
Article in Portuguese | LILACS | ID: biblio-1411955

ABSTRACT

Cor triatriatum é um anomalia cardíaca congênita rara frequentemente diagnosticada na primeira infância. Este estudo de caso apresenta um adulto com um achado acidental de cor triatriatum sinistrum. Com base na apresentação clínica, o paciente foi tratado de forma conservadora. São apresentados achados de imagens ecocardiográficas de cor triatriatum sinistrum deste paciente juntamente de revisão narrativa da literatura sobre essa doença.(AU)


Cor triatriatum is a rare congenital heart anomaly often diagnosed in early childhood. This case study features an adult with an incidental finding of cor triatriatum sinistrum. Based on the clinical presentation, the patient was treated conservatively. Cor triatriatum sinistrum echocardiographic image findings of this patient are presented along with a narrative review of the literature about this disease. (AU)


Subject(s)
Humans , Male , Middle Aged , Cor Triatriatum/complications , Cor Triatriatum/diagnostic imaging , Incidental Findings , Heart Atria/abnormalities , Magnetic Resonance Spectroscopy/methods , Echocardiography, Doppler/methods , Echocardiography, Transesophageal/methods , Echocardiography, Three-Dimensional/methods , Fatty Liver/complications , Heart Septal Defects, Atrial/complications , Kidney/injuries , Myocardial Infarction/genetics
4.
ABC., imagem cardiovasc ; 34(4): eabc220, 2021. ilus
Article in Portuguese | LILACS | ID: biblio-1361783

ABSTRACT

Fístulas de artérias coronárias têm incidência baixa, sendo diagnosticadas mais frequentemente pelo ecocardiograma ou angiotomografia coronariana, embora a cineangiocoronariografia seja o método padrão-ouro. Têm origem mais comumente na artéria coronária direita, sendo a drenagem para câmaras de baixa pressão o mais habitual. O tratamento pode ser expectante, cirúrgico ou percutâneo. Este relato descreve o caso de uma rara apresentação de insuficiência cardíaca, dada por fístula da coronária circunflexa com drenagem para o átrio esquerdo. (AU)


Coronary artery fistulas have a low incidence and are often diagnosed by echocardiography or coronary computed tomography angiography, although coronary angiography is the gold standard. They commonly originate in the right coronary artery, with drainage to low-pressure chambers being the most frequent finding. Treatment can be expectant, surgical, or percutaneous. This report describes a case of a rare presentation of heart failure due to a fistula of the circumflex coronary artery with drainage into the left atrium. (AU)


Subject(s)
Humans , Male , Aged , Arterio-Arterial Fistula/diagnosis , Arterio-Arterial Fistula/etiology , Coronary Vessels/pathology , Heart Atria/abnormalities , Heart Failure/diagnosis , Atrial Fibrillation/congenital , Cineangiography/methods , Echocardiography/methods , Magnetic Resonance Spectroscopy/therapeutic use , Echocardiography, Doppler , Arterio-Arterial Fistula/diagnostic imaging , Electrocardiography/methods
6.
Rev. bras. cir. cardiovasc ; Rev. bras. cir. cardiovasc;34(1): 104-106, Jan.-Feb. 2019. tab, graf
Article in English | LILACS | ID: biblio-985251

ABSTRACT

Abstract Congenital aneurysm or enlargement of the right atrium is a rare condition. Two children operated on at the age of 14 months and 11 years old for congenital aneurysm of the right atrium are reported. Both presented cardiomegaly and symptoms of paroxysmal supraventricular tachycardia. Diagnosis was established by echocardiography. Surgical resection was successful. Both patients are free of symptoms and their chest X-ray and echocardiogram are normal. The first patient is now in her 17th postoperative year. The patients' evolution suggests that the surgery is a curative procedure.


Subject(s)
Humans , Male , Female , Infant , Adolescent , Heart Aneurysm/surgery , Heart Aneurysm/congenital , Echocardiography , Radiography, Thoracic , Treatment Outcome , Dilatation, Pathologic , Electrocardiography , Heart Aneurysm/diagnostic imaging , Heart Atria/abnormalities , Heart Atria/surgery , Heart Atria/diagnostic imaging
8.
Arq. bras. cardiol ; Arq. bras. cardiol;106(5): 367-372, May 2016. tab, graf
Article in English | LILACS | ID: lil-784170

ABSTRACT

Abstract Background: Isolated cleft mitral valve (ICMV) may occur alone or in association with other congenital heart lesions. The aim of this study was to describe the profile of cardiac lesions associated with ICMV and their potential impact on therapeutic management. Methods: We conducted a descriptive study with data retrieved from the Congenital Heart Disease (CHD) single-center registry of our institution, including patients with ICMV registered between December 2008 and November 2014. Results: Among 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had Down syndrome. Seventeen patients (77.3%) had associated lesions, including 11 (64.7%) with accessory chordae in the left ventricular outflow tract (LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD), three had secundum atrial septal defect, and four had patent ductus arteriosus. Thirteen patients (59.1%) required surgical repair. The decision to proceed with surgery was mainly based on the severity of the associated lesion in eight patients (61.5%) and on the severity of the mitral regurgitation in four patients (30.8%). In one patient, surgery was decided based on the severity of both the associated lesion and mitral regurgitation. Conclusion: Our study shows that ICMV is rare and strongly associated with Down syndrome. The most common associated cardiac abnormalities were VSD and accessory chordae in the LVOT. We conclude that cardiac lesions associated with ICMV are of major interest, since in this study patients with cardiac lesions were diagnosed earlier. The decision to operate on these patients must take into account the severity of both mitral regurgitation and associated cardiac lesions.


Resumo Fundamento: A fissura isolada da valva mitral (FIVM) pode ocorrer isoladamente ou em associação com outras lesões cardíacas congênitas. O objetivo deste estudo foi descrever o perfil das lesões cardíacas associadas à FIVM e o potencial impacto dessas lesões na terapêutica. Métodos: Realizamos um estudo descritivo com dados obtidos do registro unicêntrico Congenital Heart Disease (CHD) de nossa instituição incluindo pacientes com FIVM registrados entre dezembro de 2008 e novembro de 2014. Resultados: Entre 2177 pacientes identificados no registro CHD, 22 (1%) apresentavam FIVM. A mediana de idade ao diagnóstico foi de 5 anos (6 dias a 36 anos). Nove pacientes (40,9%) apresentavam síndrome de Down. Dezessete pacientes (77,3%) apresentavam lesões associadas, incluindo 11 (64,7%) com cordoalha acessória na via de saída do ventrículo esquerdo (VSVE) sem obstrução, 15 (88,2%) com comunicação interventricular (CIV), três com comunicação interatrial do tipo ostium secundum e quatro com persistência do canal arterial. Treze pacientes (59,1%) necessitaram reparo cirúrgico. A decisão de prosseguir com a cirurgia foi baseada principalmente na gravidade da lesão associada em oito pacientes (61,5%) e na gravidade da regurgitação mitral em quatro pacientes (30,8%). Em um paciente, a decisão por cirurgia foi baseada na gravidade tanto da lesão associada quanto da regurgitação mitral. Conclusão: Nosso estudo mostra que a FIVM é rara e está fortemente associada à síndrome de Down. As anormalidades cardíacas associadas mais comuns foram a CIV e cordoalha acessória na VSVE. Concluímos que as lesões cardíacas associadas à FIVM são de grande interesse, já que neste estudo, pacientes com lesões associadas foram diagnosticados mais precocemente. A decisão cirúrgica deve levar em conta a gravidade tanto da regurgitação mitral quanto das lesões cardíacas associadas.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Adult , Young Adult , Heart Defects, Congenital/complications , Mitral Valve/abnormalities , Mitral Valve Insufficiency/complications , Severity of Illness Index , Echocardiography, Doppler, Color , Heart Atria/abnormalities , Heart Defects, Congenital/surgery , Heart Septal Defects, Atrial/surgery , Mitral Valve/surgery , Mitral Valve/diagnostic imaging , Mitral Valve Insufficiency/surgery , Mitral Valve Insufficiency/diagnostic imaging
10.
ABC., imagem cardiovasc ; 29(1): 28-32, jan.-mar.2016. ilus
Article in Portuguese | LILACS | ID: lil-777624

ABSTRACT

A dilatação idiopática do átrio direito (AD) é uma condiçãorara, frequentemente assintomática, com tratamentocontroverso, podendo se restringir ao acompanhamentoclínico ou tratamento cirúrgico. Descrevemos aqui um caso deum adolescente com achados ecocardiográficos compatíveiscom essa doença...


Subject(s)
Humans , Male , Adult , Adolescent , Heart Atria/abnormalities , Heart Atria/surgery , Dilatation, Pathologic/diagnosis , Echocardiography, Transesophageal/methods , Echocardiography/methods , Treatment Outcome
12.
Rev. Soc. Cardiol. Estado de Säo Paulo ; 25(4): 182-186, out.-dez.2015. ilus
Article in Portuguese | LILACS | ID: lil-789228

ABSTRACT

As arritmias cardíacas apresentam comportamento eletrofisiológico, manifestação clínica, prognóstico e resposta terapêutica diferentes na população. Sendo assim, a compreensão dos mecanismos envolvidos na gênese da anormalidade do ritmo cardíaco é fundamental para nortear a terapêutica. O sistema nervoso autônomo participa ativamente da eletrofisiologia da célula cardíaca e dos mecanismos relacionados ao desencadeamento das arritmias cardíacas. Dentre as arritmias supraventriculares, a fibrilação atrial merece destaque. Estudos avaliando a variabilidade da frequência cardíaca demonstram importante modificação autonômica antecedendo um quadro defibrilação atrial. Tanto a ativação simpática quanto a estimulação parassimpática podem participar da manutenção dessa arritmia. Além disso, métodos que reduzem a inervação autonômica também reduzem a incidência de arritmias atriais, sugerindo que a neuromodulação possa ser uma ferramenta promissora no controle dessa arritmia. As arritmias ventriculares também se relacionam ao comportamento autonômico. O infarto agudo do miocárdio ocasiona denervação das fibras simpáticas na cicatriz e as áreas de intensa fibrose perdem a capacidade de responderem a estimulação simpática; entretanto asregiões próximas a cicatriz apresentam um encurtamento do período refratário para a estimulação simpática de forma heterogênica predispondo a ocorrência da ectopias ventriculares. Dessa forma, a investigação do sistema nervoso autônomo no desencadeamento e manutenção das arritmias cardíacas é uma linha de pesquisa promissora e a maior compreensão dos mecanismos autonômicos irá propiciar novas ferramentas de controle das anormalidades do ritmo cardíaco...


Cardiac arrhythmias present varying electrophysiological behavior, clinical manifestation, prognosis, and therapeutic response in the population. An understanding of the mechanisms involved in the genesis of heart rhythm disorders is there fore essential for guiding the therapy. The autonomic nervous system plays an active role in the electrophysiology of the cardiac cells and the mechanisms that trigger cardiac arrhythmias. Atrial fibrillation, one of the supraventricular arrhythmias, deserves special attention. Studies to evaluate heart rate variability have demonstrated important modifications preceding an episode of atrial fibrillation. Both sympathetic activation and parasympathetic stimulation can play a role in the persistence of this arrhythmia. More over, methods that reduce autonomic innervation also reduce the incidence of atrial arrhythmias, suggesting that neuromodulation can be a promising tool in the control of this arrhythmia. Ventricular arrhythmias are also related to autonomic behavior. Acute myocardial infarction leads to denervation of the sympathetic fibers in the scar tissue, and the areas of intense fibrosis lose their capacity to respond to sympathetic stimulation, while the regions close to thescar tissue present heterogeneous refractory period shortening for sympathetic stimulation, predisposing to the occurrence of ventricular ectopic beats. Thus, the investigation of the autonomic nervous system in the triggering and persistence of heart arrhythmias is a promising line of research, and a better understanding of the autonomic mechanisms involved will provide new tools for the control of heart rhythm disorders...


Subject(s)
Humans , Arrhythmias, Cardiac/complications , Arrhythmias, Cardiac/therapy , Electrophysiology/methods , Autonomic Nervous System/physiology , Sleep Apnea, Obstructive/complications , Sleep Apnea, Obstructive/diagnosis , Bradycardia/complications , Bradycardia/diagnosis , Risk Factors , Heart Rate , Heart Atria/abnormalities
14.
RELAMPA, Rev. Lat.-Am. Marcapasso Arritm ; 28(3): 123-125, jul.-set.2015. ilus
Article in Portuguese | LILACS | ID: lil-777956

ABSTRACT

A policondrite recidivante é uma doença rara, multissistêmica, de etiologia desconhecida, provavelmenteautoimune, que acomete estruturas cartilaginosas e tecidos ricos em proteoglicanos. Orelha, nariz, articulações e árvore traqueobrônquica são os locais mais afetados durante os surtos inflamatórios, ocasionando destruição tecidual, deformidades permanentes e complicações respiratórias. Menos comumente, afeta olhos, coração e vasos sanguíneos.O diagnóstico é essencialmente clínico e o tratamento envolve o uso de corticoides e imunossupressores. Relatamos um caso de policondrite recidivante, em que a manifestação do segundo surto da doença ocorreu por meio de bloqueio atrioventricular com recuperação da condução cardíaca após tratamento com corticoide...


The relapsing polychondritis is a rare, multisystem disease, whose etiology is unknown. It is probably autoimmune and affects cartilaginous structures and proteoglycan-rich tissues. The ear, nose, joints and tracheobronchial tree are the most affected sites during inflammatory outbreaks causing tissue destruction,permanent deformities and respiratory complications. Less commonly, it affects the eyes, heart and blood vessels. The diagnosis is essentially clinical and treatment involves the use of steroids and immunosuppressive agents.We report a case of relapsing polychondritis where the second outbreak of the disease was manifested by na atrioventricular block with recovery of cardiac conduction after treatment with steroids...


Subject(s)
Humans , Female , Adult , Atrioventricular Block/complications , Autoimmune Diseases/diagnosis , Autoimmune Diseases/therapy , Polychondritis, Relapsing/complications , Polychondritis, Relapsing/etiology , Heart Atria/abnormalities , Adrenal Cortex Hormones/therapeutic use , Prednisone/administration & dosage , Heart Ventricles/abnormalities
15.
Ann Card Anaesth ; 2014 Oct; 17(4): 318-319
Article in English | IMSEAR | ID: sea-153709

ABSTRACT

Left atrial fibromuscular band is a rare congenital cardiac anomaly. We present a patient with an incidental finding of left atrial band on an intra‑operative transesophageal echocardiogram and characterize its appearance on two‑dimensional and three‑dimensional echocardiograms.


Subject(s)
Adult , Cardiopulmonary Bypass/methods , Echocardiography, Three-Dimensional/methods , Echocardiography, Transesophageal/methods , Heart Atria/abnormalities , Heart Atria/diagnostic imaging , Heart Defects, Congenital/diagnostic imaging , Humans , Incidental Findings , Male
16.
Arch. argent. pediatr ; 111(3): e58-e61, jun. 2013. ilus
Article in Spanish | LILACS | ID: lil-694631

ABSTRACT

El síndrome de Ellis-Van Creveld (SEVC) o displasia condroectodérmica se debe a una mutación de transmisión autosómica recesiva en el brazo corto del cromosoma 4 y afecta múltiples órganos. Descrito como una tétrada clásica de condrodisplasia, displasia ectodérmica, polidactilia y defectos cardíacos congénitos, sólo se conoce a partir de informes y series de casos. Se describe el caso de un varón de 3 meses, sin antecedentes familiares de importancia, que presentó un cuadro de condrodisplasia, labio superior fusionado al paladar, simpolidactilia posaxial bilateral en las manos, displasia del desarrollo de las caderas, tórax estrecho con costillas cortas y compromiso cardíaco. El presente caso sería la primera comunicación del SEVC en el Perú.


Ellis-Van Creveld Syndrome or chondrectodermal dysplasia is produced by an autosomal recessive inheritance secondary to mutation in the short arm of chromosome 4. The syndrome affects multiple organs. It is described as a clinical tetrad that involves chondrodysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. It is only known from reports and case series. We present a three months old male, without relevant family history, who presented chondrodysplasia, upper lip merged to palate, bilateral sinpolydactyly in the hands, developmental dysplasia of the hip, narrow chest with short ribs, and heart defects. This case is the first report of EVC in Peruvian literature.


Subject(s)
Humans , Infant , Male , Abnormalities, Multiple , Ellis-Van Creveld Syndrome/complications , Endocardial Cushion Defects/complications , Heart Atria/abnormalities , Tricuspid Atresia/complications
18.
Ann Card Anaesth ; 2012 Apr; 15(2): 134-137
Article in English | IMSEAR | ID: sea-139655

ABSTRACT

Total anomalous systemic venous connection (TASVC) to the left atrium (LA) is a rare congenital anomaly. An 11-year-old girl presented with complaints of palpitations and cyanosis. TASVC with left isomerism and noncompaction of LV was diagnosed after contrast echocardiogram and computed tomography angiogram. The knowledge of anatomy and pathophysiology is essential for the successful management of these cases. Anesthetic concerns in this case were polycythemia, paradoxical embolism and rhythm abnormalities. The patient was successfully operated by rerouting the systemic venous connection to the right atrium.


Subject(s)
Anesthesia, General/methods , Anesthetics , Arrhythmias, Cardiac/etiology , Cardiac Surgical Procedures/methods , Child , Coronary Thrombosis/complications , Cyanosis/etiology , Echocardiography , Female , Heart Atria/abnormalities , Heart Atria/surgery , Heart Ventricles/diagnostic imaging , Humans , Polycythemia/complications , Preanesthetic Medication , Pulmonary Veins/abnormalities , Sternotomy , Tomography, X-Ray Computed , Veins/abnormalities , Vena Cava, Superior/abnormalities
19.
Rev. bras. ecocardiogr. imagem cardiovasc ; 25(1): 44-47, jan.-mar. 2012. ilus
Article in Portuguese | LILACS | ID: lil-613934

ABSTRACT

As doenças malignas do coração e em especial dos grandes vasos são extremamente raras. Esses tumores fazem o seu diagnóstico clínico e a suaterapia difíceis, uma vez que seus sintomas são na maioria dos casos inespecíficos e geralmente ocorrem em estágios mais avançados da doença.Relata-se o caso de paciente feminina, 17 anos, com diagnóstico prévio de osteossarcoma femural, curado há cinco anos. Compareceu ao hospital onde fazia acompanhamento com queixa de cefaleia holocraniana, associada a vomitos e mal-estar. Ao exame físico mostrava-se discretamentehipocorada, sem outras alterações. Foi identificada por meio de uma tomografia computadorizada de crânio a presença de uma massa compatível com neoplasia maligna. Foi realizado um ecocardiograma transtorácico pré-operatório que evidenciou uma massa em átrio esquerdo, confirmada pelo exame transesofágico. Em pouco tempo a paciente apresentou um acidente vascular encefálico hemorrágico, sendo então procedida uma neurocirurgia de urgência. O histopatológico confirmou tratar-se de um osteossarcoma. A paciente evoluiu com deterioração clínica antes que pudesse ser submetida a uma biópsia da lesão torácica. Após um curto período de tempo, sem perspectivas de melhora, evoluiu a óbito.


Subject(s)
Humans , Female , Adolescent , Heart Atria/abnormalities , Echocardiography, Transesophageal , Heart Neoplasms/complications , Heart Neoplasms/diagnosis , Diagnosis, Differential , Tomography
20.
Article in English | WPRIM | ID: wpr-72922

ABSTRACT

We present an unusual case of an intracardiac Eustachian valve cyst observed concurrently with atresia of the coronary sinus ostium, a persistent left superior vena cava (LSVC) and a bicuspid aortic valve. There have been several echocardiographic reports of Eustachian valve cysts; however, there is no report of multidetector computed tomography (MDCT) findings related to a Eustachian valve cyst. Recently, we observed a Eustachian valve cyst diagnosed on MDCT showing a hypodense cyst at the characteristic location of the Eustachian valve (the junction of the right atrium and inferior vena cava). MDCT also demonstrated additional cardiovascular anomalies including atresia of the coronary sinus ostium and a persistent LSVC and bicuspid aortic valve.


Subject(s)
Aged , Humans , Male , Aortic Valve/abnormalities , Cysts/diagnostic imaging , Echocardiography, Transesophageal , Heart Atria/abnormalities , Heart Defects, Congenital/diagnostic imaging , Tomography, X-Ray Computed , Vena Cava, Inferior/abnormalities , Vena Cava, Superior/abnormalities
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