Situs inversus with levocardia in a 15-year-old male adolescent: a case report.

BACKGROUND: Situs inversus with levocardia is a rare anomaly in which the heart is present in the left chest but the abdominal viscera are transposed. It is caused by a single incomplete penetration of an autosomal recessive gene. It is unclear what exactly causes situs inversus with levocardia. Even if situs inversus can be identified following a comprehensive physical examination, it is now possible to validate the results and search for further information and pathologies since medical imaging is so widely accessible. CASE: A 15-year-old Oromo male child from a remote area of Bale Zone presented to the Goba Referral Hospital's medical emergency outpatient department complaining of periumbilical pain that had persisted for 4 months. He frequently came to our hospital and was admitted three times with the same problem. Objectively, there was tenderness over the left lower quadrant and periumbilical area. The sonographic evaluation discovered the transposition of the liver and spleen with cardiac apex on the left side. He received conservative treatment with ceftriaxone 1 g intravenous twice a day and metronidazole 500 mg intravenous for 5 days, and he went home improved. CONCLUSION: Isolated levocardia is a rare form of situs inversus in which the heart is in the traditional levo position while the abdominal organs are in the dextro position. What causes situs inversus with levocardia is unknown. Despite the fact that situs inversus can be diagnosed after a thorough physical examination, medical imaging has allowed us to confirm the findings as well as understand more about diseases. Due to the severity of an underlying heart defect, situs inversus with levocardia has a dismal prognosis.

Agenesia Pericárdica Completa. Presentación de una caso y revisión de la literatura / Complete pericardial agenesis. Presentation of a case and review of the literature

Resumen Paciente masculino de 18 años, quien es valorado en el Servicio de Cardiología del Hospital Víctor Manuel Sanabria Martínez referido del primer nivel de atención por cuadro crónico de dolor torácico atípico acompañado de pectus excavatum y desplazamiento izquierdo del latido de punta a la exploración física. La radiografía de tórax posteroanterior evidencia una cardiomegalia moderada con levocardia máxima. El ecocardiograma transtorácico con función sistólica biventricular conservada, dilatación moderada del ventrículo derecho, sin signos de hipertensión pulmonar e imagen cardiaca de "corazón en lágrima". Angiotomografía computarizada de tórax con contraste que documenta malformación de la caja torácica con hundimiento de la región esternal, en relación con pectus excavatum. Corazón aumentado de tamaño en relación con cardiomegalia grado I y desplazamiento hacia el hemitórax izquierdo. No se observa pericardio en ninguna región. Aurícula derecha levemente dilatada con un ventrículo derecho con diámetro aumentado en su porción media y deformidad de su tracto de salida debido a la forma de la caja torácica, de tal manera que se corrobora el diagnóstico de una agenesia pericárdica completa.

Abstract An 18-year-old male patient who is evaluated in the Cardiology Department of the Víctor Manuel Sanabria Martínez Hospital, referred to the first level of attention due to chronic symptoms of atypical chest pain accompanied by pectus excavatum and left displacement of the peak beat. Chest X-ray Posteroanterior with moderate cardiomegaly with maximum levocardia. Transthoracic echocardiogram with preserved biventricular systolic function, moderate dilatation of the right ventricle, without signs of pulmonary hypertension and cardiac image of "tear heart". Thoracic Computed Angiotomography with contrast documenting malformation of the rib cage with sinking of the external region, in relation to pectus excavatum. Heart enlarged in relation to grade I cardiomegaly and displacement towards the left hemithorax. No pericardium is observed in any region. Slightly dilated right atrium with a right ventricle with an increased diameter in its middle portion and deformity of its outflow tract due to the shape of the rib cage. Corroborating the diagnosis of complete pericardial agenesis.

Thoracoatrial discordance in a case of twisted atrioventricular connections: A unique configuration of visceroatrial arrangement.

We present a case of a 12-year-old boy with discordance between the atrial arrangement and the thoracoabdominal arrangement in the setting of twisted atrioventricular connections. This case highlights the importance of a separate description of all visceral organs and venous drainage for an accurate description of visceroatrial arrangement in cases of congenital heart defects.

Deciphering normal and anomalous viscero-atrial situs on multidetector CT angiography.

Viscero-atrial situs encompasses the laterality, relative position and configuration of the abdominal viscera, the atria of the heart and the tracheobronchial tree. Determining the situs and cardiac position is the first step in the commonly used sequential, segmental approach to the imaging evaluation of congenital heart defects (CHD). Abnormalities of visceroatrial situs and cardiac position are frequently associated with the presence of complex CHDs and accurate assessment of situs abnormalities can help predict the probability and type of the defect. Multidetector CT (MDCT) angiography, with its multiplanar reformatting and volume rendering techniques, offers accurate information about the morphology and three-dimensional relationships of the various cardiac and extra cardiac structures. In this pictorial essay, we present the MDCT imaging findings of the spectrum of abnormalities of visceroatrial situs and cardiac position, using a third generation dual source CT scanner.

Ruptured Sinus of Valsalva Aneurysm Associated with Situs Ambiguus, Isolated Levocardia, and Polysplenia.

Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.

Comparing levocardia and dextrocardia in fetuses with heterotaxy syndrome: prenatal features, clinical significance and outcomes.

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.

Abnormalities of Fetal Situs: An Overview and Literature Review.

The incidence of congenital heart disease is most commonly reported in 8 per 1000 live births. Situs anomalies are among the least common forms of congenital heart disease. This study defines situs and describes the variations of fetal situs anomalies. Situs refers to the arrangement of the viscera, atria, and vessels within the body. Situs solitus describes the normal arrangement; situs inversus describes inverted arrangement of the viscera and atria; and situs ambiguous (also referred to as heterotaxy) describes disturbances in arrangements that can neither be identified as solitus nor inversus. This review will concentrate on heterotaxy, as it is the most complicated situs abnormality to define, classify, and study. Prognosis of heterotaxy is variable but most correlated with cardiac anatomy. Management is concentrated on numerous cardiac operations and requires a multidisciplinary approach to address coexisting congenital anomalies.

Situs inversus with levocardia, infrahepatic interruption of the inferior vena cava, and azygos continuation: a case report.

Situs inversus incompletus is a rare congenital condition in which the major abdominal organs are reversed or mirrored from their normal positions. It is often associated with multiple congenital anomalies. We present the case of a 38-year-old woman with dyspnea and a clinical history of chronic kidney disease and kidney transplantation. Echocardiography showed a right atrial mass, and analysis of multidetector computed tomography angiography revealed the interruption of the inferior vena cava with an increase of the azygos vein and azygos continuation. These congenital malformations are often associated with deep vein thrombosis and/or pulmonary thromboembolism and explained the occurrence of dyspnea. Cardiac magnetic resonance with contrast medium confirmed the presence of the right atrial mass, the characteristics of which were attributed to interatrial thrombus, which was further confirmed by the success of thrombolytic therapy and the remission of symptoms. In conclusion, we described a case of situs inversus with levocardia in association with infrahepatic interruption of the inferior vena cava, and azygos continuation with cardiac thrombus and chronic renal failure.

Dynamic left ventricular outflow obstruction in situs inversus with levocardia.

A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet. There was no asymmetric septal hypertrophy. A possible mechanism of the LOVT obstruction in the present case may have been related to an abnormally long and bent outflow tract resulting from overriding of the right ventricle over the LVOT due to a congenital malposition of the heart. Mitral valve replacement with septal myectomy was performed in order to eliminate systolic anterior motion. The postoperative course has been excellent.

Situs ambiguous, levocardia, right sided stomach, obstructing duodenal web, and intestinal nonrotation: a case report.

When approaching a case with a situs abnormality, using the proper terminology, making a specific diagnosis, and understanding the other often associated abnormalities that need to be excluded are of great importance. We present a case of situs ambiguous in the presence of intestinal nonrotation and an obstructing duodenal web. Our patient initially presented at two days old with bilious emesis and failure to pass meconium after birth. An abdominal radiograph demonstrated an unusual bowel gas pattern, a reversed "double bubble" sign. A subsequent thorough imaging survey was crucial to further characterize our patient's unique anatomy. Overall, our case demonstrates many of the unusual plain radiographic and sonographic findings associated with our patient's situs abnormality and allows for review of situs abnormalities and their significance.

Clinics in diagnostic imaging (160). Levocardia with abdominal situs inversus.

Levocardia (left-sided cardiac apex) with abdominal situs inversus is extremely rare. This is also known as isolated levocardia and is almost always associated with severe forms of congenital heart defects with poor prognosis. We report isolated levocardia in a 13-year-old symptomatic male patient. The purpose of this paper is to outline the imaging features of isolated levocardia and to highlight the role of cardiovascular magnetic resonance imaging (CMR) in the diagnosis and management of such cases. Other forms of cardiac malposition, including dextrocardia, mesocardia and criss-cross heart, with chest radiograph and CMR correlation, are also discussed.

A human laterality disorder associated with a homozygous WDR16 deletion.

The laterality in the embryo is determined by left-right asymmetric gene expression driven by the flow of extraembryonic fluid, which is maintained by the rotary movement of monocilia on the nodal cells. Defects manifest by abnormal formation and arrangement of visceral organs. The genetic etiology of defects not associated with primary ciliary dyskinesia is largely unknown. In this study, we investigated the cause of situs anomalies, including heterotaxy syndrome and situs inversus totalis, in a consanguineous family. Whole-exome analysis revealed a homozygous deleterious deletion in the WDR16 gene, which segregated with the phenotype. WDR16 protein was previously proposed to play a role in cilia-related signal transduction processes; the rat Wdr16 protein was shown to be confined to cilia-possessing tissues and severe hydrocephalus was observed in the wdr16 gene knockdown zebrafish. The phenotype associated with the homozygous deletion in our patients suggests a role for WDR16 in human laterality patterning. Exome analysis is a valuable tool for molecular investigation even in cases of large deletions.

Clinics in diagnostic imaging (160). Levocardia with abdominal situs inversus

Levocardia (left-sided cardiac apex) with abdominal situs inversus is extremely rare. This is also known as isolated levocardia and is almost always associated with severe forms of congenital heart defects with poor prognosis. We report isolated levocardia in a 13-year-old symptomatic male patient. The purpose of this paper is to outline the imaging features of isolated levocardia and to highlight the role of cardiovascular magnetic resonance imaging (CMR) in the diagnosis and management of such cases. Other forms of cardiac malposition, including dextrocardia, mesocardia and criss-cross heart, with chest radiograph and CMR correlation, are also discussed.