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.

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: a propósito de 2 casos clínicos / Situs inversus totalis: 2 case reports

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).

Left-sided appendicitis: diagnosis and minimally invasive treatment / Left-sided appendicitis: diagnosis and minimally invasive treatment.

Intestinal malrotation and situs inversus can have important repercussions if acute abdominal pain develops. Intraabdominal structures can have inverted position and thus may easily mislead the surgeon during physical examination. Fortunately, radiological exams have improved the preoperative diagnosis of these patients. However, in difficult cases when an underlying surgical disease is suspected, laparoscopy remains the gold standard in order to diagnose and treat if possible the suspected disorder. We present a case of acute left-side appendicitis in a child with unknown congenital intestinal malrotation. In addition, this case stressed the value of laparoscopy in daily practice to evaluate patients with atypical abdominal pain.

A case of Kartagener’s syndrome: Importance of early diagnosis and treatment.

Kartagener’s syndrome is a very rare congenital malformation comprising of a classic triad of sinusitis, situs inversus and bronchiectasis. Primary ciliary dyskinesia is a genetic disorder with manifestations present from early life and this distinguishes it from acquired mucociliary disorders. Approximately one half of patients with primary ciliary dyskinesia have situs inversus and, thus are having Kartagener syndrome. We present a case of 12 year old boy with sinusitis, situs inversus and bronchiectasis. The correct diagnosis of this rare congenital autosomal recessive disorder in early life is important in the overall prognosis of the syndrome, as many of the complications can be prevented if timely management is instituted, as was done in this in this case.

Monocular elevation deficiency with associated dextrocardia and situs inversus

A patient having monocular elevation deficiency with associated dextrocardia and situs inversus is reported. Review of the literature regarding ocular features described in association with dextrocardia is also presented

Cardiopulmonary resuscitation in undiagnosed situs inversus totalis in emergency department: an intensivist challenge

Situs inversus totalis is a rare congenital condition. A 34-year-old woman with undiagnosed situs inversus was referred to our emergency department with cardiac arrested state. She underwent cardiopulmonary resuscitation [CPR] and defibrillation with a modified approach. We faced different challenging aspects during intensive care management. Ultrasonography in CPR in our patient was very helpful. We restricted our discussion on special aspect of SIT in emergency and intensive care unit

Nanophthalmos and situs inversus totalis.

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.

Revascularização do miocárdio em paciente com situs inversus totalis / Myocardial revascularization in a patient with situs inversus totalis

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.

Biliary atresia with situs inversus: an experience shared

Biliary Atresia [BA] is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of Biliary atresia with situs inversus [SI] has not been documented in Pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation had dextrocardia, SI, gross hydronephrosis [HN] of left kidney and stasis at pelvi ureteric junction [PUJ]. Liver biopsy showed biliary cirrhosis secondary to extra hepatic biliary atresia [EHBA]. The second baby presented at two months of age. Ultrasound abdomen and hepatobiliary scintigraphy confirmed liver in left hypochondrium [SI] and findings suggestive of BA. Echocardiography confirmed SI with mesocardia. In this paper we have described the association of BA with SI in two patients presenting at the pediatric Gastroenterology, hepatology and nutrition unit

Transcatheter closure of perimembranous ventricular septal defect in a patient with situs inversus and dextrocardia.

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.

Situs inversus: reporte de dos casos / Situs inversus: report of two cases

El objetivo del presente estudio es dar a conocer dos casos de situs inversus diagnosticados en el Hospital Juárez de México, tercer nivel de atención, en un periodo de 5 años. Se encontraron dos casos con el diagnóstico de situs inversus en 118,012 pacientes ingresados en un periodo de 5 años (0.001 por ciento).De estos dos casos, uno (50 por ciento) fue hombre, otro (50 por ciento) mujer, y el margen de edad que se obtuvo fue de 33 a 52 años con una media de 42.5 años. Los dos pacientes recibieron tratamiento quirúrgico. El primer caso fue el de una paciente de 52 años con diagnóstico de coledocolitiasis residual, y el segundo caso fue de un paciente con hipertensión portal y choque hipovólemico.El diagnóstico de situs inversus pasa inadvertido en aproximadamente 45 por ciento de los casos, llegando al diagnóstico incidentalmente durante el transoperatorio o como hallazgo imagenológico. Por lo tanto, es necesario realizar una historia clínica adecuada con una minuciosa exploración física para sospechar el diagnóstico y posteriormente corroborarlo por medio de imagenología (RX, USG y TAC), con el fin de planificar adecuadamente la estrategia quirúrgica que más convenga al paciente y al cirujano, para así evitar riesgos o complicaciones durante la cirugía.

Tratamento video-laparoscopico da hipertensao portal no situs inversus totalis / Video-laparoscopy therapy of portal hypertension in the \"situs inversus totalis\"

O situs inversus e uma anomalia congenita caracterizada pela alteracao dos planos de simetria do embriao. Um paciente de 32 anos, sexo masculino, admitido no hospital por hematemese devido a ruptura de varizes esofago-gastricas, foi tratado pela primeira vez por cirurgia video-laparoscopica. Apresentava hipertensao portal por cirrose de Laenec. O diagnostico do situs inversus foi obtido por radiografia toracica, eletrocardiograma, ecocardiograma, ultrassonografia abdominal e tomografia computadorizada. O exame endoscopico mostrava varizes esofagicas e de fundo gastrico. A cirurgia (desconexao azigo-portal com ligadura da arteria esplenica, sem esplenectomia; sutura das varizes esofagicas e gastricas sem abertura do esofago e colecistectomia) foi realizada por video-laparoscopia...

Situs inversus totalis asociado con hernia de Bochdalek derecha / Situs inversus totalis asociated with right sided Bochdalek hernia

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

Carcinoma Gall bladder with situs inversus totalls

We report a case of carcinoma gall bladder with situs inversus totalis in a 65 years old lady. She presented very late with obstructive jaundice and gastric outlet obstruction. Different imaging tests were done to accurately outline vital structures. At laparotomy the carcinoma was inoperable, hence a palliative percutaneous external biliary drainage and gastrojejunostomy was done. She survived for three months but ultimately died of disseminated cancer

Colestase intra-hepática en situs inversus do fígado / Intrahepatic cholestasis with situs inversus of the liver

Em nossa pesquisa bibliográfica näo encontramos nenhum trabalho sobre colestase intra-hepática em situs inversus do fígado. OBJETIVO. Relatar um caso de seguimento por um de nós (MM) durante aproximadamente, 14 anos após o desaparecimento da mencionada colestase. MÉTODOS. Foram estudados aspectos clínicos, realizados diversos testes laboratoriais e efetuados três exames ultra-sonográficos e uma tomografia computadorizada. Tentou-se realizar colangiografias transparieto-hepática e endoscópica. RESULTADOS. Quatro dias após a paciente ter sido submetida à prótese aórtica (com circulaçäo extracorpórea) surgiu icterícia, urina escura e fezes claras. As bilirubinas, fosfatase alcalina, gamaglutamil transferase e colesterol séricos estavam muito elevados. Os exames ultra-sonográficos e a tomografia sem evidências de obstruçäo extra-hepática. Neste exame, em especial, ficou caracterizada a existência de situs inversus do estômago e fígado. Näo houve êxito na realizaçäo das colangiografias. Os dados clínicos e laboratoriais referentes à colestase normalizaron após quatro meses. Boa evoluçäo após 14 abos. CONCLUSAO. O diagnóstico diferencial entre colestase intra e extra-hepática em pacientes com situs inversus do fígado pode, realmente, ser dificil, exigindo associar dados clínicos e vários exames complementares, entre os quais a colangiopancreatografia endoscópica retrógrada. A realizaçäo desse exame pode, porém, ser difícil nos mencionados pacientes