ABSTRACT
Kartagener's syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disease, and is characterized by the triad of chronic sinusitis, bronchiectasis, and situs inversus. This paper reports the case of a 27-year-old female presenting with dyspnea on medium exertion, accompanied by chronic cough, non-productive or with clear expectoration. She had recurrent pneumonia until 15 years of age and underwent a lobectomy in the lower lobe of the left lung, probably due to bronchiectasis. Chest computed tomography showed situs inversus totalis, signs of previous surgical manipulation, and mild bronchial thickening. Computed tomography of the paranasal sinuses showed signs of chronic sinusitis due to a probable ciliary kinesis disorder. These finding suggest the diagnosis of Kartagener's syndrome. The prognosis reveals a slow rate of decline in lung function. However, repeated or chronic infections can negatively influence the quality of life of these patients.
Subject(s)
Humans , Female , Adult , Situs Inversus/diagnostic imaging , Kartagener Syndrome/complications , Dextrocardia/diagnosis , Situs Inversus/complications , Kartagener Syndrome/diagnosis , Ciliary Motility DisordersABSTRACT
INTRODUCTION: Dextrocardia is characterized by the positioning of the heart to the right of the thoracic cavity, usually with the apex oriented to the right and its inverse anatomy 1,2. It is a rare congenital pathology that can be associated with other congenital cardiac anomalies, as well as a change in position of all thoracoabdominal structures 1-4. Its diagnosis in adult life is usually incidental. We present the clinical case of a 64-year-old man with an electrocardiogram that showed deviation of the QRS complex axis to the extreme right and a physical examination compatible with dextrocardia, which was later confirmed with images. OBJECTIVES: To review the medical literature related to the diagnosis of dextrocardia through the presentation of a clinical case. METHODS: The clinical data of the case were collected by means of anamnesis, physical examination and specialized exams of the patient, as well as his clinical file. The literary review was made using the MEDLINE® search engine for scientific journals. RESULTS: Dextrocardia affects less than 1% of the general population according to the studies reviewed. Its importance lies in the association with other congenital pathologies such as cardiac and extracardiac structural malformations, which increases morbidity and mortality in this group of patients. It is imperative, when suspected, complete the study with images that allow confirming or ruling out other structural anomalies.
Subject(s)
Humans , Male , Middle Aged , Dextrocardia/diagnosis , Electrocardiography , Situs Inversus/diagnosis , Congenital Abnormalities , Informed ConsentABSTRACT
Resumen: El situs inversus totalis es la inversión congénita completa de órganos torácicos y abdominales. Se presenta el caso de una paciente de 3 años sin antecedentes médicos previos, a quien en atención primaria, y por un cuadro respiratorio agudo, se evidencia el hallazgo de dextrocardia y burbuja gástrica a derecha en la radiografía de tórax, sospechándose situs inversus totalis. Fue derivada a cardiología infantil donde se confirmó el diagnóstico con un ecocardiograma transtorácico, asociado a un hallazgo de preexcitación ventricular en el electrocardiograma. Una vez resuelto el cuadro respiratorio agudo, la paciente se mantiene controlada de manera periódica en atención primaria y por especialista de manera semestral.
Abstract: Situs inversus totalis is the complete congenital inversion of thoracic and abdominal organs. We present the case of a 3-year-old girl with no previous medical history. When seen with an acute respiratory syndrome, dextrocardia and gastric bubble on the right side led to the diagnosis of Situs inversus. She was referred to infant cardiology where the diagnosis was confirmed with a transthoracic echocardiogram. In addition, the electrocardiogram identified the presence of ventricular preexitation. Once the acute respiratory symptoms subsided, the patient remains controlled periodically in primary care and by a specialist every six months. No episodes of tachycardia have been detected.
Subject(s)
Humans , Female , Child, Preschool , Situs Inversus/complications , Situs Inversus/diagnosis , Pre-Excitation Syndromes/complications , Pre-Excitation Syndromes/diagnosis , Radiography, Thoracic , Dextrocardia/complications , Dextrocardia/diagnosis , ElectrocardiographyABSTRACT
Os autores relatam três casos de síndrome da cimitarra (SC), que em sua forma habitual cursa com drenagem venosa anômala no pulmão direito, de aspecto radiográfico característico, hipoplasia no referido pulmão e dextroposição cardíaca. Enfatizam sobre a radiografia simples do tórax, como principal exame na investigação diagnóstica, e o valor do estudo angiográfico, na elucidação de anomalias vasculares quando houver indicação cirúrgica. Outros exames complementares, como a tomografia computadorizada, são avaliados para a perfeita caracterização dessa síndrome e de suas variantes...
The authors write about three cases of scimitar syndrome which, commonly, releases as an atypical venous drainage of the right lung, with characteristic image, as well as hypoplasia of the same lung and dextropositioned heart. They emphasize the thoracic radiographic image as the most important complementary method of diagnosis and the importance of the angiographic study for the diagnose of vascular abnormalities with surgical indication. Other exams such as computerized tomography are of value in characterization of syndrome and its variants...
Subject(s)
Humans , Male , Female , Scimitar Syndrome/diagnosis , Scimitar Syndrome , Angiography , Dextrocardia/diagnosis , Drainage/methods , Medical Records , Multidetector Computed Tomography , Lung/blood supply , Radiography, Thoracic , Vena Cava, Inferior/abnormalities , Pulmonary Veins/abnormalitiesABSTRACT
Introducción: El situs inversus totalis es un hallazgo poco común. Sólo un pequeño porcentaje presentará cardiopatías asociadas, por lo que el diagnóstico suele realizarse de forma casual. Objetivo: Destacar las claves diagnósticas del situs inversus totalis y la importancia de un diagnóstico temprano. Caso clínico: Presentamos dos pacientes pediátricos de 9 y 14 años que fueron diagnosticados de forma incidental. El primer caso fue estudiado por dolor precordial, en la auscultación cardíaca se escuchaba aumento de ruidos cardíacos en precordio derecho y atenuados en el izquierdo. El electrocardiograma (ECG) mostró eje QRS y onda P de +150°, QRS estrecho de voltaje atenuado en precordiales V3-V6 y ondas T negativas en V1-V4 y aVL. La radiografía de tórax confirmó dextrocardia, además se apreció burbuja gástrica a la derecha y sombra hepática a la izquierda. La ecocardiografía evidenció dextrocardia especular clásica sin malformaciones asociadas. En el segundo caso se encontró incidentalmente dextrocardia en radiografía solicitada para evaluación de escoliosis. El ECG objetivó eje QRS +120°, eje onda P +150°, QRS estrecho de voltaje atenuado en precordiales izquierdas. La ecocardiografía doppler confirmó dextrocardia especular sin anomalías asociadas. En ecografía abdominal el hígado se encontró en hipocondrio izquierdo y el bazo en hipocondrio derecho. Conclusión: El diagnóstico temprano del situs inversus totalis es importante, dado que el abordaje quirúrgico torácico y abdominal es diferente y determinadas patologías pueden presentarse con clínica inusual. Además, tras el diagnóstico de situs inversus se puede estudiar la presencia de patologías asociadas como discinesia ciliar primaria (Síndrome de Kartagener).
Introduction: Situs inversus totalis is a rare find and only a small percentage are associated with heart disease; its diagnosis is usually made incidentally. Objective: To discuss the diagnostic features of situs inversus totalis and the importance of early diagnosis. Case reports: Two pediatric patients aged 9 and 14 years who were incidentally diagnosed are reported. The first case presented chest pain and during cardiac auscultation, increased heart sounds were heard on the right precordium and attenuated on the left. An electrocardiogram (ECG) showed P wave and QRS axis equal to +150°, narrow QRS voltage attenuated in V3-V6 precordial leads, and negative T waves in leads V1-V4 and aVL. Chest radiography confirmed dextrocardia, and gastric bubble was on the right and hepatic shadow on the left. Echocardiography showed classic mirror dextrocardia without associated malformations. In the second case, dextrocardia was found incidentally after radiography was requested for the evaluation of scoliosis. ECG showed QRS of +120°, P wave axis of +150° and narrow QRS voltage axis attenuated on left precordial leads. Doppler echocardiography confirmed dextrocardia without associated anomalies. Abdominal ultrasound found the liver in left upper quadrant and the spleen in right upper quadrant. Conclusions: Early diagnosis of situs inversus totalis is important because the thoracic and abdominal surgical approach is different and certain diseases could be presented with unusual characteristics. Also, after the diagnosis of situs inversus, the presence of associated pathologies such as primary ciliary dyskinesia can be studied (Kartagener syndrome).
Subject(s)
Humans , Male , Adolescent , Child , Kartagener Syndrome/diagnosis , Situs Inversus/diagnosis , Dextrocardia/diagnosis , Echocardiography , Electrocardiography , Incidental Findings , Radiography, ThoracicABSTRACT
Nanophthalmos is characterized by short axial length, high hypermetropia, thick sclera and a normal-sized crystalline lens. Situs inversus totalis is the mirror image of the normal morphology of the thoracic and abdominal viscera. To the best of our knowledge this is the first report of a nanophthalmic patient with situs inversus totalis. Therefore, we would like to invite the attention of our colleagues to our case and underline the importance of the systemic examination of the nanophthalmic patients to detect systemic malformations and visceral transpositions.
Subject(s)
Child, Preschool , Dextrocardia/diagnosis , Humans , Male , Microphthalmos/diagnosis , Situs Inversus/diagnosis , Tomography, X-Ray ComputedABSTRACT
Incidence of congenital cardiac anomalies in dextrocardia with situs inversus is low as compared to congenital cardiac anomalies in isolated dextrocardia. We describe the first ever case of situs inversus with dextrocardia, Lutembacher's syndrome, and pericardial effusion. The pericardial effusion in our case was acquired and was tubercular in etiology
Subject(s)
Humans , Female , Dextrocardia/diagnosis , Lutembacher Syndrome/diagnosis , Pericardial Effusion/diagnosis , Heart Defects, Congenital , EchocardiographyABSTRACT
Descreve-se o caso de uma paciente gestante de 22 anos, com uma associação incomum de anormalidades congênitas: dextrocardia em situs inversus totalis, estenose subaórtica membranosa (ESAM) e comunicação interventricular (CIV) do tipo perimembranosa. A membrana subaórtica estava aderida ao septo, estendendo-se até o anel mitral, sem envolvimento do folheto anterior mitral, apresentando mobilidade independente e obstruindo, significativamente, a via de saída do ventrículo esquerdo. O septo membranoso era aneurismático, protruindo para o ventrículo direito e apresentando comunicação interventricular. A valva aórtica apresentava insuficiência de grau direto discreto a moderado. Os achados foram diagnosticados por ecocardiograma transtorácico, transesofágico e posteriormente, confirmados por estudo hemodinâmico. Não apresentava anomalias ou obstruções coronarianas. Não foi encontrado, na literatura, associação da ESAM com alteração do situs cardíaco.
Subject(s)
Humans , Female , Pregnancy , Adult , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Dextrocardia/complications , Dextrocardia/diagnosis , Aortic Valve Stenosis/complications , Aortic Valve Stenosis/diagnosisABSTRACT
This report describes an adult male cadaver with dextrocardia and situs inversus totalis in a Nigerian cadaver. The photographic illustrations revealed transposition of some of the visceral organs such that the stomach and spleen were located on the right and the liver and gall bladder on the left. Also, the left lung was tri-lobed while the right was uni-lobed. The heart was flattened and flipped to the right thus, transposing the great vessels. The report showed that dextrocardia and situs inversus exists amongst Nigerians and possibly Africans and therefore wish to recommend early medical examination since patients with this condition are unaware of their unusual anatomy until they seek medical attention for an unrelated condition. Early detection may lead to a successful surgical management and consequently offer a safer chance of survival.
Este reporte describe a un cadáver adulto nigeriano de sexo masculino con dextrocardia y situs inversus totalis. Las ilustraciones fotográficas revelaron transposición de algunas de las vísceras, como el estómago y el bazo que se encuentra en el derecho y el hígado y la vesícula biliar a la izquierda. Además, el pulmón izquierdo era tri-lobulado, mientras que el derecho uni-lobulado. El corazón fue aplanado y situado a la derecha, por consiguiente, transposicionando los grandes vasos. El reporte demostró que la dextrocardia y el situs inversus existen entre los nigerianos y, posiblemente, los africanos, por tanto, se recomienda un temprano examen médico, ya que pacientes con esta condición no son conscientes de su inusual anatomía hasta que acuden al médico por atención por una condición no relacionada. La detección temprana puede conducir a un éxito quirúrgico y, en consecuencia, ofrecer una segura oportunidad de sobrevivir.
Subject(s)
Humans , Male , Adult , Anatomy/education , Dextrocardia/diagnosis , Dextrocardia/mortality , Cadaver , Dissection/methods , NigeriaABSTRACT
La dextrocardia es una malformación que ocurre en el período embrionario y se caracteriza por el desplazamiento de la masa y del eje cardíaco principal (la base del ápex) hacia la derecha del tórax. Se reporta el caso de una paciente de diez años de edad quien consultó inicialmente por un cuadro de faringoamigdalitis a repetición y elevación de antiestreptolisinas. La exploración física y estudios de gabinete demostraron la presencia de dextrocardia sin situs inversus. El tratamiento de la infección estreptocócica fue efectivo y la paciente evolucionó satisfactoriamente sin complicaciones asociadas a la malformación, continuando su seguimiento por consulta externa...
Subject(s)
Humans , Female , Child , Heart Defects, Congenital , Dextrocardia/diagnosis , Situs Inversus/complications , Congenital Abnormalities/diagnosisABSTRACT
La hernia diafragmática es un defecto congénito, una anomalía que aparece antes del nacimiento cuando el feto se está formando en el útero de la madre. Consiste en un orificio en el diafragma. Existen dos tipos de hernia diafragmática. La hernia de Bochdalek, consiste en un orificio sobre el lado izquierdo del diafragma, representa aproximadamente el 90 por ciento de todos los casos. La hernia de Morgagni, consiste, en cambio, en un orificio sobre el lado derecho del diafragma. El uso amplio de la ecografía obstétrica, ha producido un aumento del diagnóstico prenatal de hernia diafragmática. Presentamos caso ocurrido en el hospital militar, paciente femenino de 21 años de edad I gesta embarazo mal controlado, se realiza eco obstétrico en la semana 34 de gestación reportando hidrotórax bilateral, hernia diafragmática congénita derecha, que es poco común, representa el dos por ciento de la totalidad de los casos, la paciente es ingresada a las 37 semanas de gestación por RPM, se realiza cesárea segmentaría obteniéndose RN femenino, no lloro ni respiro al nacer, Rx de tórax a/p se evidencia asas intestinales, hígado en hemitorax derecho con pulmón hipoplasico y dextrocardia, en hemitorax izquierdo pulmón hipoplasico.
Subject(s)
Humans , Adult , Female , Pregnancy , Dextrocardia/diagnosis , Hernia, Diaphragmatic/congenital , Hernia, Diaphragmatic/diagnosis , Hydrothorax/diagnosis , Lung/abnormalities , Ultrasonography , Obstetrics , Pediatrics , Ultrasonography, Prenatal/methodsABSTRACT
Relatamos o caso de um paciente com situs inversus totalis associado a coronariopatia obstrutiva em artérias descendente anterior e posterior, coronária direita, primeiro ramo diagonal e ramo marginal esquerdo, quadro condizente com a intervenção de revascularização do miocárdio. Esse procedimento não é freqüente na literatura médica, sendo encontrado apenas um relato na literatura brasileira. A revascularização do miocárdio foi realizada com a artéria mamária interna direita para a artéria descendente anterior, e uma ponte de safena para coronária direita, marginal esquerda, primeiro ramo diagonal e descendente posterior. A cirurgia foi realizada com o auxílio de circulação extracorpórea.
We report the case of a patient with dextrocardia and situs inversus totalis associated with obstructive coronariopathy in the anterior and posterior descending arteries, right coronary artery, first diagonal branch and left marginal branch. The patient underwent coronary artery bypass grafting surgery. This surgery has been rarely reported in literature and we found only one similar case in the national medical literature. The myocardial revascularization was carried out with the right mammary artery for the anterior descending artery. The saphenous vein anastomosed the aorta to the right coronary artery, left marginal branch, fist diagonal branch and posterior descending artery. The surgery was performed with extracorporeal circulation.
Subject(s)
Humans , Male , Middle Aged , Heart Failure/etiology , Internal Mammary-Coronary Artery Anastomosis , Situs Inversus/complications , Dextrocardia/complications , Dextrocardia/diagnosis , Extracorporeal Circulation , Heart Failure/surgery , Saphenous Vein/surgery , Situs Inversus/diagnosisSubject(s)
Humans , Female , Infant , Dextrocardia/diagnosis , Dextrocardia/physiopathology , Dextrocardia/therapy , Pneumonia/diagnosis , Thoracotomy/methods , ThoracotomyABSTRACT
Successful transcatheter closure of a perimembranous ventricular septal defect with an Amplatzer device has been reported in patients with levocardia. We report a case in which the device could be deployed successfully in a child with isolated perimembranous ventricular septal defect with situs inversus and dextrocardia.
Subject(s)
Abnormalities, Multiple , Aortic Valve/abnormalities , Child , Dextrocardia/diagnosis , Echocardiography , Electrocardiography , Female , Cardiac Catheterization , Heart Septal Defects, Ventricular/diagnosis , Heart Ventricles/abnormalities , Humans , Situs Inversus/diagnosisABSTRACT
Se informa de una recién nacida de 24 horas de vida extrauterina, obtenida por vía vaginal, producto de gesta III, con 39 semanas de gestación, peso de 2600 g, y situs inversus totalis asociado con hernia de Bochdalek derecha, Desarrolló insuficiencia respiratoria secundaria a hipoplasia pulmonar derecha. Se establecio el diagnóstico por examen clínico y telerradiologia de tórax. La paciente fue intervenida quirúrgicamente y la evolución resultó satisfactoria. Revisadas fuentes documentales sobre antecedentes, embriogenia, fisiopatología,epidemiología, diagnóstico, manejo actual y análisis del tema. La hernia de Bochdalek debe operarse en cuanto se diagnostique. Su asociación con situs inversus totalis es infrecuente
Subject(s)
Humans , Female , Infant, Newborn , Situs Inversus/surgery , Situs Inversus/diagnosis , Dextrocardia/diagnosis , Hernia, Diaphragmatic/surgery , Hernia, Diaphragmatic/congenital , Hernia, Diaphragmatic/diagnosis , Lung Diseases/congenital , Lung Diseases/etiologyABSTRACT
Se describe el caso clinìco de un paciente portador de un sindrome de Poland asociado a hipoplasia costal y dextrocardia. La Importancia de presentar este caso radica en la escasa frecuencia de presentaciòn de esta patologia. Se aprovecha el tema para realizar una actualizaciòn sobre este sindrome
Subject(s)
Humans , Male , Female , Infant, Newborn , Congenital Abnormalities/diagnosis , Dextrocardia/diagnosis , Poland Syndrome/diagnosis , Thoracic Diseases/diagnosis , Focal Dermal Hypoplasia/diagnosisSubject(s)
Humans , Female , Congenital Abnormalities , Dextrocardia/diagnosis , Abdomen/diagnostic imaging , Arm/abnormalitiesABSTRACT
Apresenta-se o caso de uma paciente de 10 anos de idade, sexo feminino, assintomática, encaminhada ao serviço com diagnóstico de dextrocardia. A investigaçäo observou-se o sinal radiológico da cimitarra. A broncoscopia e a broncografia evidenciaram ausência de lobos superior e médio e anormalidades na segmentaçäo brônquica do lobo inferior direito. O cateterismo confirmou a drenagem anômala da veia pulmonar direita para a veia cava inferior, configurando a síndrome da cimitarra