Síndrome de Kartagener y artritis reumatoide. Reporte de caso / Kartagener Syndrome and Rheumatoid Arthritis. Case Report

Presentación del caso. Se trata de una mujer de 26 años de edad, en seguimiento por la especialidad de reumatología desde los 17 años, cuando consultó con historia de un año de evolución de síndrome poliarticular de grandes y pequeñas articulaciones, aditivo, simétrico acompañado de fatiga, rigidez matutina mayor de una hora. Se reportó además factor reumatoide positivo. La radiografía de ambas manos presentó erosiones, que confirmó el diagnóstico de artritis reumatoide. Adicionalmente, la paciente tenía el antecedente de procesos sinobronquiales a repetición desde su infancia. En la evaluación médica se identificó dolor en los senos paranasales, dextrocardia y bronquiectasias, confirmados por los estudios de imágenes, que permitió concluir en el diagnóstico de síndrome de Kartagener. Intervención terapéutica. La paciente presentaba actividad clínica severa de la artritis reumatoide, se inició el tratamiento con metotrexato 10 mg vía oral un día a la semana, prednisona 5 mg al día y ácido fólico 5 mg a la semana y citas periódicas, controlando los datos de actividad y efectos adversos de los medicamentos, con pruebas hepáticas, hemograma y transaminasas. La especialidad de neumología recomendó la inclusión de la paciente en un programa de rehabilitación respiratoria, así como el uso de azitromicina 500 mg cada día por tres días en los períodos de agudización. Evolución clínica. El tratamiento logró mantener una actividad leve de la artritis reumatoide y sin exacerbación de los síntomas respiratorios

Case presentation. A 26-year-old woman, under follow-up by the rheumatology specialty since she was 17 years old, when she consulted with a history of one year of evolution of polyarticular disease of large and small joints, additive, symmetrical, accompanied by fatigue and morning stiffness for more than one hour. Positive rheumatoid factor was also reported. Additionally, the patient had a history of repeated sinobronchial processes since childhood. Medical examination revealed sinus pain in the paranasal sinuses, dextrocardia, and bronchiectasis, confirmed by imaging studies, which led to the diagnosis of Kartagener's syndrome. Treatment. The patient presented the severe clinical activity of rheumatoid arthritis. The treatment was started with methotrexate 10 mg orally one day a week, prednisone 5 mg a day, and folic acid 5 mg a week and periodic appointments, controlling the activity data and adverse effects of the drugs, with liver tests, hemogram, and transaminases. The pneumology department recommended the inclusion of the patient in a respiratory rehabilitation program as well as the use of azithromycin 500 mg every day for three days during periods of exacerbation. Outcome. The treatment was successful in maintaining a mild activity of the rheumatoid arthritis and without exacerbation of respiratory symptoms

Anesthetic management of a pediatric patient with situs solitus and dextrocardia

Dextrocardia with situs inversus is a rare heart condition with a genetic predisposition. Although most individuals lead a normal healthy life and usually, it is an incidental finding. Due to their unique anatomical variations and associated congenital variations, they may pose challenges to attending clinicians. We are hereby reporting a successful anesthetic management of the case of a 2.5-year-old child who presented for emergency laparotomy and on investigations, was found to have dextrocardia along with situs solitus.

Dextrocardia y herniación intratorácica del hígado. Una rara entidad / Dextrocardia and Intrathoracic Herniation of the Liver. A Rare Entity

Introducción: la incidencia de dextrocardia como anomalía congénita es menor del 0.01% y la combinación con herniación intratorácica del hígado semejando una neoplasia benigna sin antecedente de trauma toracoabdominal abierto o contuso lo hace aún menos frecuente. Caso clínico: se presenta el caso de paciente femenina de 34 años de edad que consulta por dolor de espalda. Al examen físico, se auscultan ruidos cardíacos en el hemitórax derecho y la radiografía de tórax evidencia dextrocardia e imagen que semeja masa supra diafragmática derecha, la TAC trifásica confirma la presencia de protrusión de un segmento del hígado de forma redondeada a través de un defecto no abierto del hemidiafragma derecho. Su tratamiento ha sido conservador. Conclusión: la combinación de dextrocardia acompañada de herniación de una porción del hígado a través de un defecto del diafragma derecho es una asociación extremadamente rara y los reportes de caso publicados son escasos

Revascularización miocárdica completa con injertos arteriales en un paciente con dextrocardia / Complete coronary artery bypass grafting in a patient with dextrocardia

Introducción: la dextrocardia es una rara anomalía cardiaca congénita. Aunque la incidencia de enfermedad coronaria es similar a la población en general, son pocos los casos documentados en la literatura. La disposición anatómica en esta anomalía constituye un desafío a la hora de planificar la técnica quirúrgica y los injertos a utilizar. Presentación del caso: paciente de 59 años con enfermedad coronaria de 2 vasos que fue sometido a una cirugía de revascularización arterial completa.

Introduction: dextrocardia is a rare congenital heart abnormality. Although the incidence of coronary heart disease is similar to that in the general population, few cases have been documented in the literature. Anatomic variants in this anomaly are a challenge when planning the surgical technique and the choice of graft configuration to be used. Case presentation: a 59 -year- old patient with 2-vessel coronary artery disease who underwent coronary artery bypass grafting

Implante de marcapaso en paciente con dextrocardia y situs inversus. Desafíos y soluciones / Pacemaker implant in a patient with dextrocardia and situs inversus. Challenges and solutions

Resumen La dextrocardia es una anomalía congénita rara que puede o no asociarse a otras malformaciones congénitas; los adultos con esta condición pueden presentar enfermedad sinusal o trastornos de la conducción auriculoventricular. Se debe analizar la configuración visceroatrial asociada a la dextrocardia para definir las herramientas necesarias (ultrasonido vascular, angiograma venoso, proyecciones fluoroscópicas modificadas, etc.) para la realización exitosa del implante de dispositivos transvenosos en estos pacientes. A continuación, se presenta el caso de una paciente con dextrocardia y situs inversus en quien, por disfunción sinusal, se realizó el implante de un marcapaso bicameral definitivo.

Abstract Dextrocardia is a rare congenital anomaly that may or may not be associated with other congenital malformations; adults with this condition may have sinus disease or atrioventricular conduction disorders. The viscero-atrial configuration associated with dextrocardia should be analyzed to define the necessary tools (vascular ultrasound, venous angiogram, modified fluoroscopic views, etc.) to successfully perform the implantation of transvenous devices in these patients. The following is the case of a patient with dextrocardia and situs inversus who underwent implantation of a permanent dual chamber pacemaker due to sinus dysfunction.

Coronary Artery Bypass Grafting in a Patient with Situs Inversus Totalis / 日本心臓血管外科学会雑誌

A 75-year-old man with situs inversus totalis, presented to our hospital complaining of chest pain. ECG showed ST depression in the leads of Ⅰ, aVL, V5, and V6. Blood tests showed cardiac enzyme (Troponin I) elevation. Coronary angiography revealed severe calcification and stenosis of the anatomical left anterior descending artery (LAD) , and the anatomical right coronary artery (RCA). The Patient underwent coronary artery bypass grafting (CABG) using the right internal thoracic artery and saphenous vein graft. The postoperative course was uneventful. CABG in a patient with situs inversus totalis is very rare. We present this case with a review of the relevant literature.

Kartagener's syndrome: a case report

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.

Dextrocardia con doble salida del ventrículo derecho con comunicación interventricular no relacionada asociada a malposición de grandes vasos / Right ventricular double outlet dextrocardia with unrelated ventricular septal defect associated with great vessel malposition

Resumen En el siguiente reporte se expone un caso complejo en el que coexisten malformaciones cardiacas congénitas de presentación atípica con un doble tracto de salida del ventrículo derecho, en un paciente con múltiples anomalías menores en la exploración física y un cariotipo normal. La atipia de cada una de estas y su coexistencia hacen pensar en posibles alteraciones genéticas que aún son desconocidas. Lo anterior supone un reto terapéutico con el fin de restaurar una fisiología cardiaca compatible con la vida, lo cual se logra en este caso mediante un cerclaje exitoso de la arteria pulmonar.

Abstract The following report presents a complex case in which congenital cardiac malformations of atypical presentation coexist with a double outlet right ventricle, in a patient with multiple minor abnormalities on the physical examination and a normal karyotype. The atypia of these and their coexistence suggest possible genetic alterations that are still unknown. Therefore, a therapeutic challenge in order to restore a cardiac physiology compatible with life is proposed, which is achieved in this case through a successful banding of the pulmonary artery.

A patient with dextrocardia and chagas disease: case report and literature review

ABSTRACT Introduction: About half a million patients in Colombia are currently infected with Trypanosoma cruzi. However, little is known about patients with Chagas disease and anatomical defects such as dextrocardia. Case presentation: A 52-year-old male patient with a 4-year history of dyspnea, chest pain, lower limb edema and syncope (requiring hospitalization), arrhythmias and dextrocardia, underwent serological tests for T. cruzi that were positive. A literature review was conducted to find case reports of patients with dextrocardia or situs inversus and Chagas disease in order to determine the proper treatment. Conclusion: Cases of patients with dextrocardia and Chagas disease are rare. Besides the reported case, only three other cases were found in the literature, which were relatively similar, although they could be considered more severe. According to the findings, the use of etiological treatment is acceptable in patients with coronary anatomic abnormalities and T. cruzi infection. The present case draws attention to the importance of adequately approaching and monitoring this type of patient.

RESUMEN Introducción. En la actualidad, en Colombia hay aproximadamente medio millón de personas infectadas con Trypanosoma cruzi; sin embargo, no hay mucha información sobre pacientes que viven con enfermedad de Chagas y anomalías anatómicas como la dextrocardia. Presentación del caso. Paciente masculino de 52 años con cuadro clínico de aproximadamente cuatro años de evolución consistente en disnea, dolor torácico, edema de extremidades inferiores, síncope (que requirió hospitalización), arritmias y dextrocardia, a quien se le practicaron pruebas serológicas para T. cruzi que resultaron positivas. Con el fin de establecer el tratamiento adecuado, se realizó una revisión de la literatura buscando reportes de casos de pacientes con dextrocardia o situs inversus y enfermedad de Chagas. Conclusión. Los casos de pacientes con dextrocardia y enfermedad de Chagas son poco frecuentes: además del caso reportado, en la literatura solo se encontraron tres reportes adicionales, los cuales fueron relativamente similares, aunque podrían considerarse más severos. Según los hallazgos, el uso de tratamiento etiológico es adecuado en pacientes con anormalidades anatómicas cardiovasculares e infección por T. cruzi. El presente caso llama la atención sobre la importancia de tener un enfoque y seguimiento adecuados en este tipo de pacientes.

Exceptionally Rare Krukenberg Tumour in a Patient With Dextrocardia and Situs Inversus Totalis

@#Krukenberg tumour is a rare clinical entity and accounts for about 1-2% of all ovarian tumours. It is a metastatic ovarian tumour in which accurate diagnosis requires proper endoscopic evaluation and histopathological confirmation to exclude primary ovarian tumours. Dextrocardia with situs inversus totalis is another unusual clinical entity, with an incidence of 1 in 10,000 of the general population. Many patients with this unusual condition are unaware of their structural abnormality and mostly are diagnosed incidentally when they present with other medical conditions. Herein, we report a case of 47 years old woman who presented with bilateral ovarian tumour and incidental diagnosis of Dextrocardia with situs inversus totalis. She underwent surgery and histopathological examination confirms Krukenberg tumour. Both conditions existing together, an exceptionally rare clinical entity.

Pseudodextrocardia: una nueva defifinición / Pseudo-dextrocardia: a new defifinition

La pseudodextrocardia se ha definido como un desplazamiento irregular del corazón y sus estructuras vasculares hacia la derecha por causas extracardiacas. Es un hallazgo ocasional en estudios imagenológicos de tórax o abdomen y varía acorde con las diferentes relaciones causales. Se presenta el caso de un paciente con una neumopatía crónica, quien a su ingreso presenta pseudodextrocardia como hallazgo incidental.

Pseudo-dextrocardia has been defined as an unusual displacement of the heart and its vascular structures to the right secondary to extracardiac causes. It is an occasional finding in thoracic or abdominal imaging studies and varies according to the various causal relationships. We report a case of pseudo-dextrocardia as an incidental finding in a patient admitted with chronic pulmonary disease.

Dextrocardia como hallazgo incidental: revisión a partir de un caso / Dextrocardia as incidental finding: review from a case

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.

Situs inversus totalis asociado a síndrome de preexcitación ventricular: reporte de un caso / Situs inversus totalis and pre excitation syndrome

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.

Recurrent Peumonia in an Infant With an Esophageal Lung

Esophageal lung is a rare variety of communicatingbronchopulmonary foregut malformation with anomaolouscommunication between an isolated portion of respiratory tissueand esophagus. Children present in early life with recurrentcough and pneumonia. Majority of the reported cases areassociated with other anomalies like tracheoesophageal fistula.We report a case of a 7-month-old girl with right sidedesophageal lung who was misdiagnosed as dextrocardia withright sided pneumonitis

Severe haemoptysis in a 5 year old child with Kartagener's syndrome: case report

Kartagener`s syndrome, a rare autosomal recessive disorder is a type of Primary Ciliary Dyskinesia (PCD) associated situs inversus, bronchiectasis, sinusitis and male infertility. We present a case of a 5-year-old girl who came with features of bilateral glue ear, recurrent sinusitis, recurrent hemoptysis and dextrocardia. She was diagnosed to have Kartagener`s syndrome and was evaluated for recurrent hemoptysis.

Dextrocardia and situs inversus totalis in a Turkish subject: a case report / Dextrocardia y situs inversus totalis en un sujeto turco: reporte de un caso

Dextrocardia with situs inversus is an uncommon anomaly affecting about 1 to 2 per 10,000 in the general population. This report describes an adult male patient with dextrocardia and in a Turkish subject. The photographic illustrations revealed transposition of some of the visceral organs such as the spleen was located right and the liver and gall bladder on the left. The heart was flattened and flipped to the right. Many people with situs inversus totalis are unaware of their unusual anatomy until they seek medical attention for an unrelated condition. So, early detection may lead to a successful surgical management and consequently offer a safer chance of survival. This report showed that dextrocardia and situs inversus can be seen amongst Turkish subjects.

La dextrocardia con situs inversus es una anomalía poco frecuente que afecta aproximadamente de 1 a 2 personas por 10.000 en la población general. Este informe describe un paciente masculino adulto con dextrocardia. Las figuras revelaron que la transposición de algunos de los órganos viscerales, como el bazo, se ubicada a la derecha y el hígado y la vesícula biliar a la izquierda. El corazón fue aplastado y girado hacia la derecha. Muchas personas con situs inversus totalis desconocen su anatomía inusual hasta que buscan atención médica por una afección no relacionada. Por lo tanto, la detección temprana puede llevar a un manejo quirúrgico exitoso y, en consecuencia, ofrecer una posibilidad más segura de supervivencia. Este informe mostró que la dextrocardia y el situs inversus se pueden encontrar entre los sujetos turcos.

Three cases of orthotopic cardiac transplantation for congenital dextrocardia / 中华器官移植杂志

Objective To summarize the surgical strategies of orthotopic cardiac transplantation for congenital dextrocardia .Methods Three patients with congenital dextrocardia suffered from endstage heart failure and underwent orthotopic cardiac transplantation from March 2014 to September 2017 .They were aged 10 ,29 ,13 years respectively .Donor hearts were from brain death donors and procured with extra length on inferior vena cava , aorta and pulmonary artery tissues . After cardiectomy , left atrial-atrial anastomosis was performed initially between donor ' s left-upper pulmonary vein orifices and recipient's left-lower pulmonary vein orifices .Apex was orientated at a 90 degrees' clockwise to right . Then aorta ,inferior and superior vena cava and last pulmonary artery were anastomosed continuously . The prosthetic conduits were also used owing to a lack of tissue . Results All operations were successful . The cold ischemic time was (130-375 ) (251 .00 ± 122 .53) min ,cardiopulmonary bypass time (127-212 )(179 .67 ± 55 .72 ) min and aortic clamp time (38-105 ) (65 .33 ± 35 .166) min . Two patients had stable hemodynamics and recovered well after HTx .During a follow-up period of 1 .5-3 .5 years , echocardiography showed excellent cardiac functions without blood flow obstruction . Chest radiology showed well-placed donor heart in right mediastinum .One left-sided patient with total cavopulmonary connection before HTx died at 59 days after HTx because of pneumonia and multiple organ failure .Conclusions Heart transplantation is curative for patients with congenital dextrocardia and surgical strategies are the key factor of successful treatment .

Double Outlet Right Ventricle with Dextrocardia and Situs Inversus in a Three-Year-Old Boy: A Case Report and Review of Literature

Aims: To highlight the rare occurrence of double outlet right ventricle (DORV) with Dextrocardia and Situs Inversus in a three-year-old boy viz-a-viz what has been reported in the literatures. Presentation of Case: N.A is a three year old boy who presented with easy fatiguebility, bluish discolouration of lips and tongue and occasional dyspnoea on exertion noticed since about 12 months of life. There was no associated history of cough, leg swelling or frequent hospitalization. Physical examination revealed a small for age, centrally cyanosed boy with conjuctival ejection, and grade 4 digital clubbing. Pulse rate was 100 beats /min regular and synchronous with other peripheral pulses. There was a praecordial bulge to the right with apex beat at the 4th right intercostal space mid-clavicular line. Heart sounds were 1st and 2nd with a grade 2 systolic murmur at the right upper sternal border. The liver was 4 cm palpable below the left costal margin firm non-tender. Pulse oximetry showed SPO2 value of 75-79% in ambient air. He had chest radiograph, electrocardiogram, abdominal ultrasound and two-dimensional echocardiography which confirmed a diagnosis of DORV with Situs totalis. Discussion and Conclusion: Situs Inversus totalis (Dextrocardia co-existing with Situs inversus) is associated with lower incidence of congenital heart defects as compared with Dextrocardia and Situs solitus. The defects commonly associated with it, are a transposition of great arteries (TGA) and ventricular septal defects (VSD) unlike in the index patient where DORV is being reported.