What causes situs Ambiguus?
Mutations in the zinc finger transcription factor ZIC3 gene result in situs ambiguus with severe heart malformations as a consequence of the inability of the embryo to establish the appropriate left-right asymmetry in early development.
What is normal situs?
Situs solitus is the medical term referring to the normal position of thoracic and abdominal organs. Anatomically, this means that the heart is on the left with the pulmonary atrium on the right and the systemic atrium on the left along with the cardiac apex.
How rare is situs ambiguous?
Heterotaxy syndrome with atrial isomerism occurs in 1 out of every 10,000 live births and is associated with approximately 3% of congenital heart disease cases. Additional estimation of incidence and prevalence of isomerism proves difficult due to failure to diagnose and underestimation of the disease by clinicians.
2 On the other hand, mesocardia is extremely rare with reported incidence of 0.2 per 10 000 deliveries. 3 It includes two relatively well-defined apexes defined by each ventricle with the major axis of the heart lies in the midline.
Situs inversus is a genetic condition in which the organs in the chest and abdomen are positioned in a mirror image from their normal positions.
Levocardia: The location of the heart is in the left chest, in its normal condition. This term has sometimes been used to refer to a normal position of the heart when associated with situs inversus (reversal of sidedness of abdominal and thoracic organs) or other heart diseases.
A midline liver is a classic finding. • EKG: Variable presentation, but often right ventricular hypertrophy (RVH) and/or left ventricular. hypertrophy (LVH) is present. The P axis may alternate between the lower right and left quadrants. due to two sinus nodes (two right atria).
Situs inversus (also called situs transversus or oppositus) is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement of internal organs is known as situs solitus.
Situs inversus is present in 0.01% of the population. Situs describes the position of the cardiac atria and viscera. Situs solitus is the normal position, and situs inversus is the mirror image of situs solitus (see the image below). Cardiac situs is determined by the atrial location.
Situs refers to the arrangement of the viscera, atria, and vessels within the body.
Dextrocardia affects an estimated 1 out of every 12,000 people . Dextrocardia situs inversus totalis affects approximately 1 out of every 10,000 children.
: the place where something exists or originates specifically : the place where something (such as a right) is held to be located in law.
Situs solitus is the normal arrangement of abdominal organs with levocardia (Fig. 3) and is associated with a less than 1% incidence of congenital heart disease.
These terms refer to the embryologic looping of the straight tube of the heart. A D-loop brings the cardiac apex initially to the right with the morphologic right ventricle anterior to the left ventricle; the normal heart has a D-loop.
Etiology: Cardiac location is affected by many factors including underlying cardiac malformation, abnormalities of mediastinal and thoracic structures (eg., Bronchogenic cysts, lung mass, tumors, esophageal atresia, diaphragmatic hernia, kyphoscoliosis, abnormalities of the diaphragm ecc.), Otherwise mesocardia
Disease definition. A rare, genetic, developmental defect during embryogenesis characterized by total mirror-image transposition of both thoracic and abdominal viscera across the left-right axis of the body.
Dextroposition describes a heart on the right with an apex to the left, secondary to extracardiac causes (right lung hypoplasia, pneumonectomy or diaphragmatic hernia).
Yet individuals with anatomical reversals in brain structure, due to a condition called situs inversus totalis, still retain left-sided language processing . These results suggest that, for some cognitive tasks, function may not follow structure.
Additionally, the position of the heart chambers as well as the visceral organs such as the liver and spleen is reversed (situs inversus). However, most affected individuals can live a normal life without associated symptoms or disability.
Situs inversus is a condition in which the arrangement of the internal organs is a mirror image of normal anatomy. It can occur alone (isolated, with no other abnormalities or conditions) or it can occur as part of a syndrome with various other defects.
A rare, congenital, non-syndromic, developmental defect during embryogenesis characterized by the heart located in the normal (levo) position associated with abdominal viscera located in the dextro position.
Abstract. Atrioventricular (AV) discordance with ventriculoarterial (VA) concordance is a rare form of congenital heart disease that consists of 5 different anatomic types.
Perimembranous ventricular septal defects (VSDs) are located in the left ventricle outflow tract beneath the aortic valve. They are the most common VSD subtype in the United States, occurring in 75-80% of cases. Defects may extend into adjacent portions of the ventricular septum.
Situs inversus abdominalis (SIA) is an uncommon condition that causes intestinal malrotation in the paediatric population as its primary complication (Brown, 2017). Presentations of acute surgical emergencies in adults secondary to SIA are extremely rare (Brown, 2017).
Heterotaxy syndrome is an arrangement of internal organs somewhere between situs solitus and situs inversus; this condition is also known as "situs ambiguus." Unlike situs inversus, the abnormal arrangement of organs in heterotaxy syndrome often causes serious health problems.
Spleen pain is usually felt as a pain behind your left ribs. It may be tender when you touch the area. This can be a sign of a damaged, ruptured or enlarged spleen.
There were 6 pregnancies in 3 patients with situs inversus and 9 pregnancies in 6 patients with isolated dextrocardia. There were no apparent antenatal complications. None of the patients developed any cardiac symptoms antenatally.
Dextrocardia is a rare heart condition in which your heart points toward the right side of your chest instead of the left side. Dextrocardia is congenital, which means people are born with this abnormality. Less than 1 percent of the general population is born with dextrocardia.
You can still have a fairly normal life without one of your lungs, a kidney, your spleen, appendix, gall bladder, adenoids, tonsils, plus some of your lymph nodes, the fibula bones from each leg and six of your ribs.
Dextrocardia is a condition in which the heart is pointed toward the right side of the chest. Normally, the heart points toward the left.
Although Aristotle cited two cases of transposed organs in animals, situs inversus was first discovered in Naples by the anatomist and surgeon Marco Severino, in 1643.
To determine situs, check the orientation of the fetal head and spine. If the fetus is in cephalic presentation with its spine to the maternal left, then the fetal right side is “up” (ie, closest to the maternal abdominal wall) so the cardiac apex should point “down” and the stomach should be “down” or left as well.
Placing the ultrasound probe horizontally on the maternal abdomen, a transverse view of the fetal trunk is obtained at the level of the fetal upper abdomen or the four-chamber view of the heart. The position of the fetal spine is then determined.
Primary Ciliary Dyskinesia
The underlying basis for situs inversus totalis in PCD has been attributed to dysfunction of the embryonic nodal cilia that play a key role in directing normal rotation of viscera. Without functional nodal cilia, thoracoabdominal laterality becomes random.
A Homozygous Nme7 Mutation Is Associated with Situs Inversus Totalis.