Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review.

Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review / Púrpura trombocitopênica idiopática em paciente com situs inversus totalis: relato de caso e revisão da literatura

ABSTRACT Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.

RESUMO Situs inversus totalis é uma anormalidade congênita autossômica recessiva rara em que os órgãos mediastinais e abdominais encontram-se em posição espelhada em relação à topografia habitual. A literatura relata alguns casos de concomitância do situs inversus totalis com outras condições: anomalias espinhais, malformações cardíacas e doenças hematológicas, como púrpura trombocitopênica idiopática, que é uma doença autoimune com plaquetopenia, devido à destruição dos trombócitos ou supressão da sua produção. Esse artigo teve o objetivo de relatar coexistência de situs inversus totalis e púrpura trombocitopênica idiopática.

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.

Casuística. Vesícula biliar siniestra (sinistraposición): Caso clínico / Left-sided Gallbladder (Sinistraposition of the gallbladder)

A rare case of left-sided gallbladder (sinistraposition) is reported with review of the literature. Left-sided gallbladder is very unusual, with a frequency of 0.3% of the cases, being generally associated to situs inversus. The aim of this invesigation was to establish the association between left-sided gallbladder and right-sided round ligaments. Left-sided gallbladder is a rare anomaly and has been classified into two situations: 1) gallbladder migration to the left side, and 2) development of a second gallbladder with atrophy of the original one. Left-sided gallbladder were reported to be associated with right-sided round ligaments.

Criss-cross con concordancia aurícular-ventricular y discordancia ventrículo-arterial: presentación clínica / Criss-cross with atrioventricular concordance and ventriculoarterial discordance: clinical presentation

El corazón con entrecruzamiento de la circulación venosa pulmonar y sistémica a nivel auriculoventricular, denominada criss-cross, puede coexistir en presencia de situs solitus o situs inversus con concordancia o discordancia aurículo-ventricular dependiendo de la rotación cardíaca sobre el eje longitudinal ventricular, a favor o en contra de las manecillas del reloj. Presentamos el caso clínico de un lactante menor masculino con diagnóstico de corazón en criss-cross con concordancia aurículo-ventricular y discordancia ventrículo-arterial que ingresó a nuestro centro con disnea y cianosis. En un primer abordaje se le practica atrioseptostomía quirúrgica, sin embargo, en su evolución intrahospitalaria presenta falla cardíaca global refractaria a tratamiento convencional, por lo cual es llevado a nuevamente a cirugía donde se le realiza intervención de Damus-Kaye-Stansel. En el presente artículo se analizan los detalles de la evaluación ecocardiográfica, el cateterismo cardíaco, la resonancia magnética cardíaca y se plantean las opciones quirúrgicas de esta fascinante entidad patológica.

A heart with cross circulation (criss – cross) can coexist in the presence of situs solitus or situs inversus, with atrioventricular concordance or discordance, depending on the cardiac rotation over the ventricular longitudinal axis, clockwise or clock counterwise. This is the presentation of the clinical case of a male lactant, with diagnosis of criss–cross heart with atrioventricular concordance and ventriculoarterial discordance, who was admitted to these facilities with dyspnoea and cyanosis. In a first approach, a surgical atrioseptectomy was performed on the patient. However, in his intrahospitalary evolution, the patient had a global heart failure refractory to conventional treatment, causing his admission into the operating room for surgery, where the Damus–Kaye–Stensel procedure was performed on him. This article presents an analysis of the details of the echocardiographic evaluation, the cardiac catheterization, and the cardiac magnetic resonance of the clinical case, raising the surgical options for this fascinating pathological entity.

[Sequential segmental analysis in 100 cardiopulmonary specimens]. / Aplicación del análisis secuencial segmentario en 100 especímenes cardiopulmonares.

OBJECTIVE: To evaluate the feasibility and accuracy of the sequential segmental analysis (SSA) to diagnose congenital heart diseases. MATERIAL AND METHODS: 100 cardiopulmonar specimens were analyzed according with the sequential segmental analysis. The atrial situs, atrioventricular (AVC) and ventriculoarterial connections (VAC) were defined, finally associated lesions were observed. RESULTS: From 100 specimens, 91 had situs solitus (SS), 6 inversus (SI) and 3 isomerism (2 right isomerism and 1 left isomerism). The AVC in these hearts were: 89 biventricular, 80 (90%) concordant, 6 (6.7%) discordant and 3 (3.3%) ambiguous (2 right topology an 1 left topology). The univentricular AVC were 11, 4 (36%) had absent left AVC (mitral atresia), 3 (27%) absent right AVC (tricuspid atresia), 2 (18%) double inlet right ventricle (DIRV), 1 double inlet left ventricle (DILV) and 1 (9%) double inlet undetermined ventricle. The VAC in SS were: 52 (57%) concordant, 13 (14%) discordant, 6 (6.6%) double outlet right ventricle (DORV), 20 (22%) with single outlet (13 pulmonary atresia , 5 aortic atresia and 2 common arterial trunk). The VAC in SI were: concordant in one, (16.7%), discordant 3 (50%) and DORV in 2 (33.3%). In the hearts with isomerism, the VAC were concordant in one, DORV in one and single outlet in another (pulmonary atresia). The associated anomalies were ASD (67%), PAD (51%) and VSD (53%). CONCLUSIONS: The SSA was and effective and accurate tool to codify clear and completely the congenital heart disease. It is suggested that if the SSA is normal to describe this situation as a Normal Basic Heart Organization and then to describe the associated anomalies.

Aplicación del análisis secuencial segmentario en 100 especímenes cardiopulmonares / Sequential segmental analysis in 100 cardiopulmonary specimens

OBJECTIVE: To evaluate the feasibility and accuracy of the sequential segmental analysis (SSA) to diagnose congenital heart diseases. MATERIAL AND METHODS: 100 cardiopulmonar specimens were analyzed according with the sequential segmental analysis. The atrial situs, atrioventricular (AVC) and ventriculoarterial connections (VAC) were defined, finally associated lesions were observed. RESULTS: From 100 specimens, 91 had situs solitus (SS), 6 inversus (SI) and 3 isomerism (2 right isomerism and 1 left isomerism). The AVC in these hearts were: 89 biventricular, 80 (90%) concordant, 6 (6.7%) discordant and 3 (3.3%) ambiguous (2 right topology an 1 left topology). The univentricular AVC were 11, 4 (36%) had absent left AVC (mitral atresia), 3 (27%) absent right AVC (tricuspid atresia), 2 (18%) double inlet right ventricle (DIRV), 1 double inlet left ventricle (DILV) and 1 (9%) double inlet undetermined ventricle. The VAC in SS were: 52 (57%) concordant, 13 (14%) discordant, 6 (6.6%) double outlet right ventricle (DORV), 20 (22%) with single outlet (13 pulmonary atresia , 5 aortic atresia and 2 common arterial trunk). The VAC in SI were: concordant in one, (16.7%), discordant 3 (50%) and DORV in 2 (33.3%). In the hearts with isomerism, the VAC were concordant in one, DORV in one and single outlet in another (pulmonary atresia). The associated anomalies were ASD (67%), PAD (51%) and VSD (53%). CONCLUSIONS: The SSA was and effective and accurate tool to codify clear and completely the congenital heart disease. It is suggested that if the SSA is normal to describe this situation as a Normal Basic Heart Organization and then to describe the associated anomalies.

Radiofrequency catheter ablation of an accessory pathway in a patient with Wolff-Parkinson-White and Kartagener's syndrome.

We report a case of a manifest left free wall accessory pathway in a patient with Kartagener's syndrome and recurrent episodes of orthodromic atrioventricular reentrant tachycardia. To the best of our knowledge, it is the first report of Wolff-Parkinson-White syndrome associated with Kartagener's syndrome. Situs inversus and mirror image dextrocardia occurred with no additional detectable cardiac structural abnormalities. Diagnostic and therapeutic electrophysiological study was carried out via transaortic approach and a left-to-right reversal of monoplane fluoroscopic image.

[Absence of right and left atrioventricular connexion]. / Ausencia de conexión atrioventricular derecha e izquierda.

Fifty seven hearts with absence of atrioventricular (A-V) connection were studied morphologically to specify their types of ventriculoarterial connection and their associated anomalies; the anatomic features of the hearts were correlated with their echocardiographic and cardioangiographic images in order to establish their mutual correspondence. Fifty six hearts had situs solitus; fifty specimens had right absent A-V connection and six had left absent A-V connection. One had situs inversus. All the specimens had: A deep A-V sulcus at the site of the absent A-V connection, a dimple in the muscular floor of the involved atrium connected with the dilated and hypertrophic left ventricle, incomplete right ventricle without inlet portion, ventricular septal defect of variable dimensions (it was obliterated in two), atrial septal defect, the ventricular septum deviated from the crux cordis. The left absent A-V connection had ventricular inversion and discordant ventriculoarterial connection. In the right absent A-V connection the ventriculoarterial connections were concordant in thirty eight hearts, from which thirty four had pulmonary stenosis both infundibular and valvular (five had the tetrad of Fallot), two had pulmonary valve atresia and two had a dilated pulmonary artery; discordant in nine hearts, one with aortic atresia; double outlet, from the right ventricle in two, (one with the tetrad of Fallot) and from the left ventricle in one. The heart in situs inversus had ventricular inversion, right absent A-V connection (left-sided), single (right) ventricle and atresia of the left ventricle. The correlations between cardiac morphology and imaging were precise. Developmentally, this cardiopathy is the result of an ectopic unequally lateralized septation of the common atrioventricular canal, which separates two canals, one stenotic leading to atresia and the other which develops too wide.

[Double outlet right ventricle with discordant atrioventricular connection. Clinical study]. / Doble cámara de salida del ventrículo derecho con conexión atrioventricular discordante. Estudio clínico.

The clinical and anatomic findings were reviewed in 17 patients with double-outlet right ventricle and atrioventricular discordance. Ten cases had atrial situs solitus, seven with right-sided heart three with left-sided heart. Seven cases had atrial situs inversus, five with left-sided heart and two with right-sided heart. All cases presented ventricular septal defect, 13 subvalvar pulmonary stenosis, two tricuspid regurgitation and two complete atrioventricular block. The spatial relationship between the arterial valves are variable. Most cases in atrial situs solitus had a left-sided and anterior aorta and all patients in atrial situs inversus had a right-sided and anterior aorta. In this study we compared the anomalies found in our cases with double outlet right ventricle with those in 58 patients with corrected transposition. Absolute, relative and attributable risks were calculated for the presence of subvalvular pulmonary stenosis, ventricular septal defect, tricuspid regurgitation and atrioventricular block for each the two groups. We concluded that patients with double-outlet right ventricle are more prone to present ventricular septal defect and subvalvar pulmonary stenosis, while those with corrected transposition have a greater likelihood of presenting with tricuspid regurgitation and atrioventricular block. There is no typical clinical picture for the malformations. Symptoms depend upon the associated anomalies. The final diagnosis is best achieved by the echocardiographic and angiocardiographic studies, but electrocardiogram and chest radiograph may suggest the presence of a discordant atrioventricular connection.

[Crossed atrioventricular connection with straddling of the right atrioventricular valve]. / Conexion atrioventricular cruzada con cabalgamiento de la valvula atrioventricular derecha.

We describe one patient with crossed atrioventricular connection in situs inversus, concordant atrioventricular connection, double outlet right ventricle, and straddling of the right atrioventricular valve. Angiographic features are analyzed, pointing out the importance of selective angiography in order to define the atrioventricular and ventriculoarterial connections. The utility of bidimensional echocardiographic study before cardiac catheterization is emphasized in order to know the associated malformations.

[Anatomy of atrioventricular discordances]. / Anatomía en las discordancias atrioventriculares.

Nineteen anatomic specimens were revised, 10 in situs solitus and 9 in situs inversus. The type of atrioventricular connection, atrioventricular relation, the characteristics of atrioventricular valves and ventricular cavities, the atrial and ventricular septa, the conducting tissue, the type of arterio-ventricular connections, the anatomy of the coronary arteries and associated defects were studied. The anatomic features of the ventricular cavities differ from those in the normal heart with respect to the orientation of the atrio-ventricular valve rings, the implantation of the papillary muscles in both ventricles and the extention of the membranous septum. The most frequently found type of arterioventricular connection was discordant, followed by double-outlet right ventricle, concordant connections and finally single outlet. We did not find any case of double-outlet left ventricle. It is important to know the anatomy of the conducting tissue that is in close contact with the ventricular septal defects, the most common associated anomaly in our series, and with infundibular pulmonary stenosis.