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1.
J Med Case Rep ; 17(1): 499, 2023 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-38042875

RESUMO

BACKGROUND: Situs inversus with levocardia is a rare anomaly in which the heart is present in the left chest but the abdominal viscera are transposed. It is caused by a single incomplete penetration of an autosomal recessive gene. It is unclear what exactly causes situs inversus with levocardia. Even if situs inversus can be identified following a comprehensive physical examination, it is now possible to validate the results and search for further information and pathologies since medical imaging is so widely accessible. CASE: A 15-year-old Oromo male child from a remote area of Bale Zone presented to the Goba Referral Hospital's medical emergency outpatient department complaining of periumbilical pain that had persisted for 4 months. He frequently came to our hospital and was admitted three times with the same problem. Objectively, there was tenderness over the left lower quadrant and periumbilical area. The sonographic evaluation discovered the transposition of the liver and spleen with cardiac apex on the left side. He received conservative treatment with ceftriaxone 1 g intravenous twice a day and metronidazole 500 mg intravenous for 5 days, and he went home improved. CONCLUSION: Isolated levocardia is a rare form of situs inversus in which the heart is in the traditional levo position while the abdominal organs are in the dextro position. What causes situs inversus with levocardia is unknown. Despite the fact that situs inversus can be diagnosed after a thorough physical examination, medical imaging has allowed us to confirm the findings as well as understand more about diseases. Due to the severity of an underlying heart defect, situs inversus with levocardia has a dismal prognosis.


Assuntos
Cardiopatias Congênitas , Levocardia , Situs Inversus , Criança , Masculino , Adolescente , Humanos , Situs Inversus/complicações , Situs Inversus/diagnóstico por imagem , Vísceras , Abdome
5.
J Card Surg ; 35(10): 2798-2799, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32652587

RESUMO

We present a case of a 12-year-old boy with discordance between the atrial arrangement and the thoracoabdominal arrangement in the setting of twisted atrioventricular connections. This case highlights the importance of a separate description of all visceral organs and venous drainage for an accurate description of visceroatrial arrangement in cases of congenital heart defects.


Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interventricular/diagnóstico por imagem , Ventrículos do Coração/anormalidades , Levocardia/diagnóstico , Estenose da Valva Pulmonar/diagnóstico por imagem , Criança , Angiografia por Tomografia Computadorizada , Átrios do Coração/anormalidades , Átrios do Coração/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino
6.
Br J Radiol ; 92(1102): 20190231, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31271542

RESUMO

Viscero-atrial situs encompasses the laterality, relative position and configuration of the abdominal viscera, the atria of the heart and the tracheobronchial tree. Determining the situs and cardiac position is the first step in the commonly used sequential, segmental approach to the imaging evaluation of congenital heart defects (CHD). Abnormalities of visceroatrial situs and cardiac position are frequently associated with the presence of complex CHDs and accurate assessment of situs abnormalities can help predict the probability and type of the defect. Multidetector CT (MDCT) angiography, with its multiplanar reformatting and volume rendering techniques, offers accurate information about the morphology and three-dimensional relationships of the various cardiac and extra cardiac structures. In this pictorial essay, we present the MDCT imaging findings of the spectrum of abnormalities of visceroatrial situs and cardiac position, using a third generation dual source CT scanner.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Tomografia Computadorizada Multidetectores , Vísceras/diagnóstico por imagem , Brônquios/anormalidades , Brônquios/diagnóstico por imagem , Coração/embriologia , Átrios do Coração/anormalidades , Átrios do Coração/diagnóstico por imagem , Cardiopatias Congênitas/embriologia , Humanos , Levocardia/diagnóstico por imagem , Fígado/anormalidades , Fígado/diagnóstico por imagem , Estômago/anormalidades , Estômago/diagnóstico por imagem , Traqueia/anormalidades , Traqueia/diagnóstico por imagem , Vísceras/anormalidades
8.
Tex Heart Inst J ; 44(6): 416-419, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-29276443

RESUMO

Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.


Assuntos
Anormalidades Múltiplas , Ruptura Aórtica/etiologia , Síndrome de Heterotaxia/complicações , Levocardia/complicações , Seio Aórtico , Baço/anormalidades , Esplenopatias/complicações , Ruptura Aórtica/diagnóstico , Ecocardiografia Transesofagiana , Feminino , Humanos , Levocardia/diagnóstico , Pessoa de Meia-Idade , Baço/diagnóstico por imagem , Esplenopatias/congênito , Esplenopatias/diagnóstico , Tomografia Computadorizada por Raios X
9.
BMC Pregnancy Childbirth ; 17(1): 393, 2017 Nov 23.
Artigo em Inglês | MEDLINE | ID: mdl-29169330

RESUMO

BACKGROUND: To investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia. METHODS: Clinical demographics, prenatal features, postnatal characteristics and the outcomes of fetuses with levocardia or dextrocardia were recorded and analyzed. RESULTS: Sixty-five fetuses with dextrocardia and thirty-eight fetuses with levocardia were enrolled. Right ventricle outlet obstruction, atrioventricular septal defect and intestinal malrotation were common in both groups. Univentricular physiology, transposition of the great arteries and esophageal atresia were more frequent in fetuses with levocardia, whereas abnormal pulmonary venous connection, double outlet of right ventricle, left ventricle outlet obstruction and brain abnormalities were more frequent in the dextrocardia group. The accuracy of evaluating cardiac malformations was high, but the sensitivity in assessing extracardiac abnormalities was low. CONCLUSIONS: Although the disorders have certain overlapping features, there are several differences between fetuses with levocardia and dextrocardia. These findings might improve patient counseling and perinatal management.


Assuntos
Anormalidades Múltiplas/fisiopatologia , Dextrocardia/fisiopatologia , Coração Fetal/fisiopatologia , Síndrome de Heterotaxia/fisiopatologia , Levocardia/fisiopatologia , Anormalidades Múltiplas/embriologia , Adulto , Dextrocardia/embriologia , Dextrocardia/etiologia , Feminino , Defeitos dos Septos Cardíacos/embriologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/embriologia , Síndrome de Heterotaxia/complicações , Síndrome de Heterotaxia/embriologia , Humanos , Levocardia/embriologia , Levocardia/etiologia , Gravidez , Veias Pulmonares/anormalidades , Veias Pulmonares/embriologia , Transposição dos Grandes Vasos/embriologia , Função Ventricular
12.
Obstet Gynecol Surv ; 71(1): 33-8, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26819133

RESUMO

The incidence of congenital heart disease is most commonly reported in 8 per 1000 live births. Situs anomalies are among the least common forms of congenital heart disease. This study defines situs and describes the variations of fetal situs anomalies. Situs refers to the arrangement of the viscera, atria, and vessels within the body. Situs solitus describes the normal arrangement; situs inversus describes inverted arrangement of the viscera and atria; and situs ambiguous (also referred to as heterotaxy) describes disturbances in arrangements that can neither be identified as solitus nor inversus. This review will concentrate on heterotaxy, as it is the most complicated situs abnormality to define, classify, and study. Prognosis of heterotaxy is variable but most correlated with cardiac anatomy. Management is concentrated on numerous cardiac operations and requires a multidisciplinary approach to address coexisting congenital anomalies.


Assuntos
Síndrome de Heterotaxia/complicações , Síndrome de Heterotaxia/epidemiologia , Adulto , Dextrocardia/epidemiologia , Feminino , Aconselhamento Genético , Síndrome de Heterotaxia/classificação , Síndrome de Heterotaxia/diagnóstico , Síndrome de Heterotaxia/terapia , Humanos , Incidência , Levocardia/epidemiologia , Gravidez , Prognóstico , Terminologia como Assunto , Ultrassonografia Pré-Natal
13.
Surg Radiol Anat ; 38(3): 369-71, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25737490

RESUMO

Situs inversus incompletus is a rare congenital condition in which the major abdominal organs are reversed or mirrored from their normal positions. It is often associated with multiple congenital anomalies. We present the case of a 38-year-old woman with dyspnea and a clinical history of chronic kidney disease and kidney transplantation. Echocardiography showed a right atrial mass, and analysis of multidetector computed tomography angiography revealed the interruption of the inferior vena cava with an increase of the azygos vein and azygos continuation. These congenital malformations are often associated with deep vein thrombosis and/or pulmonary thromboembolism and explained the occurrence of dyspnea. Cardiac magnetic resonance with contrast medium confirmed the presence of the right atrial mass, the characteristics of which were attributed to interatrial thrombus, which was further confirmed by the success of thrombolytic therapy and the remission of symptoms. In conclusion, we described a case of situs inversus with levocardia in association with infrahepatic interruption of the inferior vena cava, and azygos continuation with cardiac thrombus and chronic renal failure.


Assuntos
Veia Ázigos/anatomia & histologia , Levocardia/complicações , Trombose/etiologia , Veia Cava Inferior/anormalidades , Adulto , Feminino , Humanos
14.
BMJ Case Rep ; 20152015 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-26628311

RESUMO

A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet. There was no asymmetric septal hypertrophy. A possible mechanism of the LOVT obstruction in the present case may have been related to an abnormally long and bent outflow tract resulting from overriding of the right ventricle over the LVOT due to a congenital malposition of the heart. Mitral valve replacement with septal myectomy was performed in order to eliminate systolic anterior motion. The postoperative course has been excellent.


Assuntos
Levocardia/complicações , Levocardia/diagnóstico por imagem , Obstrução do Fluxo Ventricular Externo/complicações , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Idoso , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Levocardia/cirurgia , Masculino , Tomografia Computadorizada por Raios X , Obstrução do Fluxo Ventricular Externo/cirurgia
16.
J Radiol Case Rep ; 9(2): 16-23, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25926924

RESUMO

When approaching a case with a situs abnormality, using the proper terminology, making a specific diagnosis, and understanding the other often associated abnormalities that need to be excluded are of great importance. We present a case of situs ambiguous in the presence of intestinal nonrotation and an obstructing duodenal web. Our patient initially presented at two days old with bilious emesis and failure to pass meconium after birth. An abdominal radiograph demonstrated an unusual bowel gas pattern, a reversed "double bubble" sign. A subsequent thorough imaging survey was crucial to further characterize our patient's unique anatomy. Overall, our case demonstrates many of the unusual plain radiographic and sonographic findings associated with our patient's situs abnormality and allows for review of situs abnormalities and their significance.


Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Duodeno/anormalidades , Intestinos/anormalidades , Levocardia/diagnóstico por imagem , Estômago/anormalidades , Anormalidades Múltiplas/cirurgia , Diagnóstico Diferencial , Duodeno/diagnóstico por imagem , Feminino , Humanos , Recém-Nascido , Intestinos/diagnóstico por imagem , Radiografia , Estômago/diagnóstico por imagem , Ultrassonografia
17.
Singapore Med J ; 56(4): 198-201; quiz 202, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25917470

RESUMO

Levocardia (left-sided cardiac apex) with abdominal situs inversus is extremely rare. This is also known as isolated levocardia and is almost always associated with severe forms of congenital heart defects with poor prognosis. We report isolated levocardia in a 13-year-old symptomatic male patient. The purpose of this paper is to outline the imaging features of isolated levocardia and to highlight the role of cardiovascular magnetic resonance imaging (CMR) in the diagnosis and management of such cases. Other forms of cardiac malposition, including dextrocardia, mesocardia and criss-cross heart, with chest radiograph and CMR correlation, are also discussed.


Assuntos
Anormalidades Múltiplas/diagnóstico , Diagnóstico por Imagem , Levocardia/diagnóstico , Situs Inversus/diagnóstico , Adolescente , Humanos , Imagem Cinética por Ressonância Magnética , Masculino , Radiografia Torácica , Tomografia Computadorizada por Raios X
18.
Eur J Hum Genet ; 23(9): 1262-5, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25469542

RESUMO

The laterality in the embryo is determined by left-right asymmetric gene expression driven by the flow of extraembryonic fluid, which is maintained by the rotary movement of monocilia on the nodal cells. Defects manifest by abnormal formation and arrangement of visceral organs. The genetic etiology of defects not associated with primary ciliary dyskinesia is largely unknown. In this study, we investigated the cause of situs anomalies, including heterotaxy syndrome and situs inversus totalis, in a consanguineous family. Whole-exome analysis revealed a homozygous deleterious deletion in the WDR16 gene, which segregated with the phenotype. WDR16 protein was previously proposed to play a role in cilia-related signal transduction processes; the rat Wdr16 protein was shown to be confined to cilia-possessing tissues and severe hydrocephalus was observed in the wdr16 gene knockdown zebrafish. The phenotype associated with the homozygous deletion in our patients suggests a role for WDR16 in human laterality patterning. Exome analysis is a valuable tool for molecular investigation even in cases of large deletions.


Assuntos
Sequência de Bases , Proteínas de Transporte/genética , Síndrome de Heterotaxia/genética , Hidrocefalia/veterinária , Levocardia/genética , Deleção de Sequência , Proteínas de Transporte/metabolismo , Cílios , Consanguinidade , Exoma , Feminino , Síndrome de Heterotaxia/metabolismo , Síndrome de Heterotaxia/patologia , Homozigoto , Humanos , Hidrocefalia/genética , Hidrocefalia/metabolismo , Hidrocefalia/patologia , Lactente , Levocardia/metabolismo , Levocardia/patologia , Dados de Sequência Molecular , Fenótipo , Análise de Sequência de DNA
19.
Turk Kardiyol Dern Ars ; 42(6): 564-7, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25362949

RESUMO

Anatomically corrected malposition of the great arteries (ACMGA) is defined as parallel arising of aorta and main pulmonary artery (PA) roots although ventriculoarterial connection is normal. Abnormally related aorta arises from the left ventricle, while abnormally related PA arises from the right ventricle. It can be diagnosed with via echocardiography. In some cases, additional imaging modalities such as computerized tomographic angiography and magnetic resonance are required. In this article, we presented two cases of ACMGA, 5-month-old boy and 1-month-old girl. We wanted to point out the importance of differential diagnosis of other great artery anomalies from this rare pathology.


Assuntos
Levocardia/diagnóstico , Transposição dos Grandes Vasos/diagnóstico , Diagnóstico Diferencial , Ecocardiografia , Feminino , Humanos , Lactente , Recém-Nascido , Levocardia/diagnóstico por imagem , Levocardia/cirurgia , Masculino , Radiografia , Tomografia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia
20.
BMC Pregnancy Childbirth ; 13: 79, 2013 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-23530545

RESUMO

BACKGROUND: Fetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs. The present study describes a systematic approach for the diagnosis of fetal unilateral lung agenesis and cardiac malpositions in routine prenatal screening. METHODS: A total of 18 cases of fetal unilateral lung agenesis complicated with cardiac malposition were reviewed. A systematic method was proposed to identify the fetal left side and right side according to the fetal head position and posture by acquiring a long axis and transverse view of the fetus. Fetal unilateral lung agenesis was diagnosed by evaluation of the ipsilateral pulmonary artery. The diagnosis was confirmed by postnatal echocardiography, digital radiology, and computed tomography after birth or by autopsy findings. RESULTS: The left-sided fetal heart with the cardiac apex rotating to the left and posterior were confirmed in all 7 left lung agenesis cases, while the rightward shifting of the fetal heart together with the cardiac axis deviating to the right were confirmed in all 11 cases of right lung agenesis. The disappearance of the ipsilateral pulmonary artery was confirmed in all 18 cases of unilateral lung agenesis. Cardiac anomalies were present in a total of 7 of the 18 cases of lung agenesis with 4 of 7 in cases of left lung agenesis and 3 of 11 in cases of right agenesis. CONCLUSIONS: The systematic approach introduced in the current report is helpful in the diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition. The information provided by this study may be helpful to better understand unilateral lung agenesis anatomically and to facilitate its potential examination.


Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Levocardia/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Pulmão/anormalidades , Ultrassonografia Pré-Natal/métodos , Ecocardiografia Doppler em Cores , Feminino , Humanos , Pulmão/diagnóstico por imagem , Gravidez , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos
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