RESUMO
Situs inversus with dextrocardia is both a clinical and diagnostic challenge for emergency physicians to properly identify acute coronary syndrome. While dextrocardia itself does not independently increase the risk of coronary artery disease, mirrored symptoms, including right-sided and rightward radiating chest pain in any patient with cardiac risk factors should raise suspicion for acute coronary syndrome. In patients with a reversed cardiac silhouette on a chest radiograph, a reversed electrocardiogram, to include both the precordial and limb leads, is necessary to evaluate for cardiac ischemia in presumed dextrocardia. The authors present a case of a 66-year-old man with dextrocardia who presented with shortness of breath and hypotension. Rapid application of a reversed electrocardiogram resulted in the timely diagnosis of ST-segment elevation myocardial infarction and activation of the cardiac catheterization laboratory resulting in the preservation of this patient's life.
Assuntos
Síndrome Coronariana Aguda/diagnóstico , Dextrocardia/diagnóstico , Dextrocardia/etiologia , Infarto do Miocárdio com Supradesnível do Segmento ST/diagnóstico , Síndrome Coronariana Aguda/complicações , Idoso , Diagnóstico Diferencial , Eletrocardiografia/métodos , Humanos , Masculino , Radiografia Torácica , Infarto do Miocárdio com Supradesnível do Segmento ST/etiologia , Situs Inversus/complicações , Situs Inversus/diagnósticoRESUMO
BACKGROUND: Kartagener's syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disorder characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. Abnormal ciliary structure or function leading to impaired ciliary motility is the main pathophysiologic problem in Kartagener's syndrome. CASE PRESENTATION: A 24-year-old man from Gondar town, North-West Ethiopia, presented to University of Gondar Hospital with recurrent episodes of nasal congestion with itching and paranasal discomfort, and productive cough for more than a decade. Clinical and imaging findings revealed chronic sinusitis, bronchiectasis, dextrocardia, and situs inversus. He was treated with orally administered antibiotics, mucolytic, and chest physiotherapy. He was symptomatically better with the above therapy, and started on a long-term low-dose prophylactic antibiotic. CONCLUSIONS: Patients with Kartagener's syndrome exist in Ethiopia as cases of chronic recurrent sinopulmonary infections. As there is no easy, reliable non-invasive diagnostic test for Kartagener's syndrome and the correct diagnosis is often delayed by years, it may cause chronic respiratory problems with reduced quality of life. Genetic counseling and fertility issues should be addressed once Kartagener's syndrome is diagnosed.
Assuntos
Antibacterianos/administração & dosagem , Bronquiectasia , Dextrocardia , Expectorantes/administração & dosagem , Síndrome de Kartagener , Qualidade de Vida , Sinusite , Situs Inversus , Exercícios Respiratórios/métodos , Bronquiectasia/diagnóstico , Bronquiectasia/etiologia , Bronquiectasia/fisiopatologia , Bronquiectasia/terapia , Doença Crônica , Dextrocardia/diagnóstico , Dextrocardia/etiologia , Diagnóstico Diferencial , Aconselhamento Genético , Humanos , Síndrome de Kartagener/diagnóstico , Síndrome de Kartagener/fisiopatologia , Síndrome de Kartagener/psicologia , Síndrome de Kartagener/terapia , Masculino , Administração dos Cuidados ao Paciente/métodos , Sinusite/diagnóstico , Sinusite/tratamento farmacológico , Sinusite/etiologia , Situs Inversus/diagnóstico , Situs Inversus/etiologia , Adulto JovemRESUMO
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 VentricularAssuntos
Dextrocardia/etiologia , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética/métodos , Síndrome de Poland/complicações , Síndrome de Poland/diagnóstico por imagem , Adulto , Dextrocardia/diagnóstico por imagem , Eletrocardiografia/métodos , Humanos , Hipertensão/complicações , Hipertensão/diagnóstico , Hipertensão/tratamento farmacológico , Masculino , Monitorização Fisiológica/métodos , Fotografação/métodos , Prognóstico , Radiografia Torácica/métodos , Medição de RiscoRESUMO
El Síndrome PAGOD es un acrónimo de hipoplasia de pulmón y arterias pulmonares, agonadismo, onfalocele / defecto diafragmático y dextrocardia. Se describe una serie de 21 pacientes, en la cual, 90,5 % presentó un cariotipo 46,XY y solo dos casos 46,XX; el 66,6 % exhibió un fenotipo femenino y 28,6 % genitales ambiguos. La ocurrencia de dos paciente 46,XX excluye al cromosoma Y como portador del defecto genético y plantea la posibilidad de una herencia recesiva ligada al cromosoma X, sin descartar que los casos observados en hermanos puedan deberse a mutaciones en otros genes como STRA6, VEGFA, VEGFB, VEGFC, transcritos de empalmes alternativos de VEGFA, HIF1, HIF2, entre otros. Las malformaciones congénitas observadas en los pacientes fueron: genitales y gónadas 85,7 %, diafragma y pared 66,6 %, cardíaco 80,9 %, pulmonar 71,4 %, vascular 80,9 % y abdomen 42,8 %. La revisión de los pacientes ha demostrado un alto grado de variabilidad en la expresividad de malformaciones de órganos, aparatos o sistemas. Las malformaciones vasculares representan un componente importante y característico del síndrome PAGOD y cuya base morfogenética del síndrome pueda deberse a un defecto de la angiogénesis embrionaria temprana con repercusión en la organogénesis de aparatos y sistemas. Dentro de los genes relacionados con el remodelamiento vascular durante la embriogénesis, regeneración tisular y carcinogénesis está el Factor de Crecimiento del Endotelio Vascular D (VEGFD), localizado en Xp22.31, con expresión en pulmón, corazón, intestino delgado, pulmón fetal, útero, mamas, tejido neural y neuroblastoma, el cual representa un fuerte candidato para su análisis molecular como una de las posibles causa del síndrome.
PAGOD Syndrome is an acronym for lung and pulmonary arteries hypoplasia, agonadism, omphalocele / diaphragmatic defect and dextrocardia. A series of 21 patients is described, where 90.5% had a 46,XY karyotype and only two cases 46,XX; 66.6% exhibited a female phenotype and 28.6% ambiguous genitalia. The occurrence of two patients 46,XX excludes the Y chromosome as a carrier of the genetic defect and raises the possibility of a recessive X-linked inheritance, without ruling out that the observed cases in siblings may be due to mutations in other genes as Stra6, VEGFA, VEGFB, VEGFC, and alternative splicing of transcripts VEGFA, HIF1, HIF2, among others. Congenital malformations were observed in patients genitals and gonads 85.7%, 66.6% in diaphragm and abdominal wall , heart 80.9%, 71.4% lungs, blood vessels 80.9% and 42.8% in abdomen. The review of patients has demonstrated a high degree of variability in the expression of malformations of organs and organ systems. Vascular malformations represent an important and characteristic component of PAGOD syndrome and whose base morphogenetic syndrome may be due to a defect in early embryonic angiogenesis with impact on organogenesis and system development. Among genes related to vascular remodeling during embryogenesis, tissue regeneration and carcinogenesis, the Endothelial Growth Factor D Vascular (VEGFD), located in the Xp22.31 region, with expression in lung, heart, small intestine, uterus, breast, neuroblastoma and neural tissue, represents a strong candidate for molecular analysis as a cause of the syndrome.
Assuntos
Criança , Feminino , Humanos , Vasos Sanguíneos/anormalidades , Dextrocardia/etiologia , Hérnias Diafragmáticas Congênitas/etiologia , Genitália Feminina/anormalidades , Dextrocardia/genética , Hérnias Diafragmáticas Congênitas/genética , Neovascularização Patológica/embriologiaRESUMO
A 73-year-old woman, who had a history of chronic sinusitis, presented to the emergency department with shortness of breath. Additional imaging demonstrated situs inversus totalis and multiple bronchiectases. The clinical triad of sinusitis, situs inversus totalis and bronchiectasis is often characteristic of Kartagener's syndrome, a subset of primary ciliary dyskinesia.
Assuntos
Dextrocardia/etiologia , Dispneia/etiologia , Síndrome de Kartagener/complicações , Síndrome de Kartagener/diagnóstico , Sinusite/etiologia , Idoso , Doença Crônica , Feminino , HumanosRESUMO
PAGOD Syndrome is an acronym for lung and pulmonary arteries hypoplasia, agonadism, omphalocele / diaphragmatic defect and dextrocardia. A series of 21 patients is described, where 90.5% had a 46,XY karyotype and only two cases 46,XX; 66.6% exhibited a female phenotype and 28.6% ambiguous genitalia. The occurrence of two patients 46,XX excludes the Y chromosome as a carrier of the genetic defect and raises the possibility of a recessive X-linked inheritance, without ruling out that the observed cases in siblings may be due to mutations in other genes as Stra6, VEGFA, VEGFB, VEGFC, and alternative splicing of transcripts VEGFA, HIF1, HIF2, among others. Congenital malformations were observed in patients' genitals and gonads 85.7%, 66.6% in diaphragm and abdominal wall , heart 80.9%, 71.4% lungs, blood vessels 80.9% and 42.8% in abdomen. The review of patients has demonstrated a high degree of variability in the expression of malformations of organs and organ systems. Vascular malformations represent an important and characteristic component of PAGOD syndrome and whose base morphogenetic syndrome may be due to a defect in early embryonic angiogenesis with impact on organogenesis and system development. Among genes related to vascular remodeling during embryogenesis, tissue regeneration and carcinogenesis, the Endothelial Growth Factor D Vascular (VEGFD), located in the Xp22.31 region, with expression in lung, heart, small intestine, uterus, breast, neuroblastoma and neural tissue, represents a strong candidate for molecular analysis as a cause of the syndrome.
Assuntos
Vasos Sanguíneos/anormalidades , Dextrocardia/etiologia , Genitália Feminina/anormalidades , Hérnias Diafragmáticas Congênitas/etiologia , Criança , Dextrocardia/genética , Feminino , Hérnias Diafragmáticas Congênitas/genética , Humanos , Neovascularização Patológica/embriologiaRESUMO
Herein, we describe a temporal correlation between the electrocardiographic changes and the chest computed tomographic findings in a 73-year-old woman who underwent a right pneumonectomy (RP) for lung adenocarcinoma.
Assuntos
Adenocarcinoma/cirurgia , Dextrocardia/diagnóstico , Dextrocardia/etiologia , Eletrocardiografia , Neoplasias Pulmonares/cirurgia , Pneumonectomia/efeitos adversos , Tomografia Computadorizada por Raios X , Idoso , Feminino , Humanos , Fatores de TempoRESUMO
The combined Poland and Mobius syndrome occurs rarely and with a wide range of features. There is no consensus on the etiology of this syndrome; familial, sporadic cases and likely environmental insult cases have been reported. This sporadic case represents a unique variant in the spectrum of this syndrome.
Assuntos
Cromossomos Humanos Par 3/genética , Dextrocardia/etiologia , Síndrome de Möbius/complicações , Síndrome de Poland/complicações , Humanos , Recém-Nascido , MasculinoRESUMO
We report a case of severe mitral regurgitation (MR) with dextrocardia and 180° counterclockwise rotated situs solitus heart. We describe the technique for mitral valve surgery in a patient with dextrocardia and agenesis of the right lung.
Assuntos
Dextrocardia/etiologia , Cardiopatias Congênitas/etiologia , Implante de Prótese de Valva Cardíaca/métodos , Pulmão/anormalidades , Anuloplastia da Valva Mitral/métodos , Insuficiência da Valva Mitral/cirurgia , Adulto , Ponte Cardiopulmonar , Diagnóstico por Imagem , Humanos , Masculino , Insuficiência da Valva Mitral/diagnóstico , Insuficiência da Valva Mitral/etiologia , Resultado do TratamentoAssuntos
Síndrome de Kartagener/diagnóstico , Bronquiectasia/diagnóstico por imagem , Bronquiectasia/etiologia , Dextrocardia/etiologia , Eletrocardiografia , Feminino , Humanos , Síndrome de Kartagener/complicações , Síndrome de Kartagener/patologia , Síndrome de Kartagener/fisiopatologia , Pessoa de Meia-Idade , RadiografiaRESUMO
A case of unilateral pulmonary hypoplasia in association with abdominal situs inversus (Situs Inversus Partialis) is described here in a 2-mo-old baby. The normally related heart (levocardia) is dextroposed due to the hypoplastic right lung and compensatory hyper-inflation of the opposite lung which clinically mimicked a mirror-image situs inversus totalis. Such a combination, to the best of authors' knowledge, has never been reported in the world literature.
Assuntos
Dextrocardia/etiologia , Pneumopatias/complicações , Pulmão/anormalidades , Anormalidades Múltiplas/diagnóstico por imagem , Dextrocardia/diagnóstico por imagem , Diagnóstico Diferencial , Humanos , Lactente , Pulmão/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Masculino , Tomografia Computadorizada por Raios X , UltrassonografiaRESUMO
A 22-year-old man was admitted with a rapidly enlarging soft mass on the left chest wall, which was diagnosed as lipoma by postoperative pathology. A chest roentgenogram revealed a defect of the fourth rib, scoliosis, dextrocardia, and diaphragmatic hernia. A computed tomographic scan showed maldevelopment of the pectoralis major and minor muscles. This is the first reported case of Poland syndrome with ipsilateral lipoma of the chest wall. Dextrocardia associated with Poland syndrome may be considered dextroposition, rather than a dextroinversion, and it may arise as a result of Poland syndrome.
Assuntos
Dextrocardia/etiologia , Lipoma/etiologia , Síndrome de Poland/complicações , Parede Torácica/patologia , Adulto , Humanos , Lipoma/patologia , Masculino , Tomografia Computadorizada por Raios XRESUMO
We report here on an unusual late postoperative presentation of extreme post-pneumonectomy dextrocardia and spontaneous contralateral pneumothorax presenting as late complications occurring approximately 2 years after right-sided pneumonectomy. Computed tomography is the diagnostic modality of choice to obtain information on anatomical changes within the post-pneumonectomy space. Knowledge of the spectrum of cardiopulmonary, pleural, and other complications after lung resection is important to properly manage complications in post-pneumonectomy patients.
Assuntos
Dextrocardia/diagnóstico por imagem , Doenças do Mediastino/complicações , Doenças do Mediastino/diagnóstico por imagem , Pneumonectomia/efeitos adversos , Pneumotórax/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Carcinoma Pulmonar de Células não Pequenas/cirurgia , Dextrocardia/etiologia , Feminino , Humanos , Neoplasias Pulmonares/cirurgia , Doenças do Mediastino/etiologia , Pessoa de Meia-Idade , Pneumotórax/etiologia , Pneumotórax/terapia , Valor Preditivo dos Testes , Sensibilidade e EspecificidadeRESUMO
Pacemaker implantation after pneumonectomy is rare and there have been no previously reported cases of acquired dextrocardia after implantation. The authors report the case of a pacemaker implantation in a patient with complete heart block, impaired left ventricular function, sclerosis of heart valves and radiation induced vasculopathy resulting in ostial stenosis of the right coronary artery 30 years after radiochemotherapy in childhood. Acquired dextrocardia after right pneumonectomy for mucoepidermoid carcinoma made implantation a challenge due to and poor fluoroscopic visualization of the heart and increased radio-opacity of the right chest when compared to congenital dextrocardia.