The findings suggest a picture of left atrial isomerism (LAI) or polysplenia syndrome ( Table 30-1 ). There is a fair degree of overlap between defects associated with right and left atrial isomerism. Cardiac anomalies associated with RAI and LAI overlap a great deal. c. The heart is mildly enlarged (cardiothoracic ratio = 0.47). Asplenia, which … In left isomerism, vena caval connections are necessarily to a morphologic left atrium, so the sinus node is absent or hypoplastic. The pulmonary veins enter the posterosuperior chamber, also termed accessory LA chamber. overall results have improved substantially over time, with current-era outcomes suggesting 7% to 15% early mortality, and long-term survival of 75% Non cardiac abnormality contribute to mortality and morbidity RESULTS Interruption of the inferior vena cava with azygous continuation is almost universal. Complete atrioventricular septal defect, common atrium, ventricular septal defect, bilateral superior vena cava and complete heart block are frequent findings in left isomerism. 1 4 5 6 7 8 Left isomerism is associated with paired left-sided viscera, while right-sided structures may be absent. Methods: Determinants of survival were analyzed for the group as a whole as well as separately for left and right atrial isomerism. Abnormal cardiac development typically leads to atrial appendage isomerism, resulting in either bilateral paired right atria (right atrial isomerism) or paired left atria (left atrial isomerism). The term atrial heterotaxy includes right atrial isomerism (RAI) and left atrial isomerism (LAI) sometimes collectively labelled as situs ambiguous. 12 patients had a poor outcome; 6 patients died, 1 underwent cardiac transplantation and 5 had an NYHA functional class of >III. The associated findings with left isomerism include polysplenia, bilateral morphologic left atrial appendages, bilateral bilobed lungs, interruption of the IVC with azygos continuation into the SVC, midline symmetric liver, stomach on either right or left side of the abdomen, and absence of the gallbladder. This topic will review the management and outcome of patients with heterotaxy (isomerism). Left atrial isomerism is associated with the presence of “double” left-sided structures with the underdevelopment or absence of right-sided structures. Individuals with right atrial isomerism develop 2 sinoatrial nodes, as they have 2 mirrored right atria, whereas those with left atrial isomerism fail to develop a sinus node at all. The term "heterotaxy" is from the Greek words "heteros," meaning "other than," and "taxis," meaning "arrangement." 10. syndrome. antenatally. In right atrial isomerism (RAI), compared to left atrial isomerism (LAI), there is a higher incidence of a single ventricle physiology, complete atrioventricular septal defect, total anomalous pulmonary venous drainage (APVD) and pulmonary stenosis.3 The long-term outcome of patients with heterotaxy syndrome is most often dictated by the severity of their cardiac anomalies. There is dextrocardia (cardiac axis = 60 degrees), and the stomach is on the right side of the fetal abdomen as well. 1 – Case 3—isomeric left atrial appendages (LAA—left atrial appendage, RAA—right atrial appendage). Some authors instead refer to heterotaxy syndrome as a specific subset of situs ambiguus, such as that with the presence of In patients with right atrial isomerism, there were 36 males and 12 females with age ranging from 15 days to 31 years (median age 3.5 years). This can result in many different organ systems being affected, and also means that each individual with heterotaxy is unique. Orientation is assessed from a transverse section of the fetal abdomen. The distance be- tween the two papillary muscles was significantly smaller in hearts with right isomerism than in either those with left isomerism or those with usual atrial arrangement (Fig. patients with isomeric left atrial appendages frequently have bilobed lungs bilaterally and each with a long bronchus 1. Pre-EC-TCPC (left), post-EC-TCPC (center), and post-reoperation (right) axial computed tomography scans of a case with right isomerism, dextrocardia, and PVs draining to the left atrium. Paired organs, such as the lungs or kidneys, are often mirror images of one another instead of having the unique characteristics of right and left that are normally present. Someone with heterotaxy may have multiple spleens. Objectives: To identify determinants of adverse outcomes in patients with atrial isomerism. The following symptoms also sometimes occur in coronary sinus ASD: 1. Heterotaxy syndrome is a complex syndrome that occurs when the axes of the body fail to rotate correctly when developing in the womb. between the right and left ventricles as well as a moder-ate-sized ventricular septal defect (Figure 4). 3, A). Of the hearts with right isomerism studied histologically, only 1 has been reported to be associated with a complete heart block. Another name is "asplenia-cardiovascular defect-heterotaxy". The term Heterotaxy syndrome is derived from two words – HETERO (means “different”) and TAXY (means “arrangement”). Heterotaxy is split into two types, left atrial isomerism and right atrial isomerism, sometimes called Ivemark’s Syndrome. Nearly all patients with right atrial isomerism, and some patients with left atrial isomerism, will require a series of major heart surgeries. Surgeons will reconfigure the heart and circulatory system so that the heart functions with one ventricle (pumping chamber), instead of two. There is a midline liver and multilobed right-sided spleen. Duplication of the right atrial appendages, one on each side, was designated as isomerism of the right atrial appendages (RAA) and similarly, duplication of the left morphology as isomerism of the left atrial appendages (LAA). Abnormal cardiac development typically leads to atrial appendage isomerism, resulting in either bilateral paired right atria (right atrial isomerism) or paired left atria (left atrial isomerism). In Heterotaxy syndrome, the defective arrangement of a specific organ or more than one organs is involved. Heterotaxy syndrome is a condition in which the internal organs are abnormally arranged in the chest and abdomen. On occasion, however, even infants develop clinically important symptoms of congestive heart failure, generally in conjunction with other contributing factors. a. If the 16 The presence of a twin AV conduction system in right atrial isomerism might explain the infrequent occurrence of heart block. We aim to assess the impact of gut abnormalities on outcome in HS. Heterotaxy syndrome is a congenital rare defect that can affect many organs, including heart, liver and other organs. Inside the uterus, incorrect rotation along with the body axis of the devel… Left atrial isomerism is generally associated with polysplenia. The treatment approach depends on the complexity of the symptoms and range of organ abnormality involves. Heterotaxy Syndrome affected patients with left atrial isomerism need surgical interventions to repair septal defects. Pacemaker insertion can assist to control abnormal heart rhythms. Left isomerism Mirror image of the structures on the left side of the chest along the left-right axis of the body, i.e. One of the most common associations with left isomerism is the absence of the intrahepatic part of the inferior vena cava, a finding that is present in 80% to 90% of cases (2, 4). Patients with left atrial isomerism had a much better survival outcome than patients with right atrial isomerism: 18 years survival 74% (95% CI, 63%-82%) versus 50% (95% CI, 38%-60%), presumably due to the higher prevalence of obstructed TAPVD (23% for right vs 0% left) and asplenia (73% for right vs 10% for left) amongst The atrial pacemaker is therefore ectopic, and the P-wave axis is abnormal. 5 Venous malformations of left atrial isomerism include bilateral superior vena cavae; however, in cases of a single superior vena cava, it may be located on either the right or the left.
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