Isomerism of the left atrial appendages associated with bronchoatrial discordance and unusual systemic venous drainage

Jewel, Francis M.; Joffe, H. S.; Wilde, Peter

doi:10.1017/s1047951100001608

Cited by 4 publications

(3 citation statements)

References 8 publications

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“…Other authors [21][22][23][24] take thoracic structures into consideration, such as the bronchuses and the lungs, for the diagnosis of the overall bodily arrangement. They recognize, nonetheless, that there are discordances between the bronchopulmonary and the atrial situs.…”

Section: Left Isomorphism (5 Cases)mentioning

confidence: 99%

“…Those who have previously noted such discordances, albeit in a limited number of cases, have emphasized the problems they produce in terms of both concepts and nomenclatures. 21,22 The association of cardiovascular malformations in each type of heterotaxy; be it right or left isomorphism, cannot be used as an absolute reference for classification of "situs". This is because the incidence of malformations, although greater in a given type of arrangement, is far from constant.…”

Section: Left Isomorphism (5 Cases)mentioning

confidence: 99%

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Discordances between the different types of atrial arrangement and the positions of the thoraco-abdominal organs

et al. 2001

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Numerous investigations have addressed the most appropriate anatomical structures to be used for the identification of segmental arrangement ("situs"). Variations from normal have been termed "heterotaxy syndromes". The assumption is frequently made that, in such instances, all the organs in a given individual case follow the same rules for segmental morphology. This leads to confusion. The starting point for cardiac analysis is the structure of the atriums. On this basis, we can define viscero-atrial discordance when, in usual arrangement, mirror imagery, and heterotaxy, the atriums and their appendages are no longer concordant with the remaining thoracic and abdominal organs. With this in mind, we examined a large number of autopsied specimens to identify all possible variations of thoracic structures and abdominal organs relative to atrial arrangement. We analyzed 1100 malformed hearts. Of these, 1046 had usual arrangement with normally situated heart (95%); 10 cases had usual arrangement with right-sided heart (0.90%); 25 showed mirror imagery (2.27%), and 19 were found with heterotaxy (1.72%). There was a total of 54 hearts of usual arrangement with right-sided heart, mirror imagery and heterotaxy, (4.90%) and, amongst them, 17 showed viscero-atrial discordances (31.48%). In those having usual arrangement with right-sided heart, there were 2 cases (20%); 8 in those with mirror imagery (32%); and 7 in heterotaxy (36.8%). In the 1046 cases of usual arrangement with normally situated heart, there were 12 examples of viscero-atrial discordances (1.14%). In all, we found a total of 29 cases of viscero-atrial discordances. Taken together, the discordances are best dealt with by describing separately, in every case of normal or abnormal atrial arrangement, the thoracic structures, the atrial appendages, and the abdominal organs. In the final analysis, the diagnosis of the cardiac arrangement rests on the location and morphology of the atriums and their appendages.

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Section: Left Isomorphism (5 Cases)mentioning

confidence: 99%

Section: Left Isomorphism (5 Cases)mentioning

confidence: 99%

Discordances between the different types of atrial arrangement and the positions of the thoraco-abdominal organs

et al. 2001

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“…1 " 2 But there is considerable diversity in these patterns, and sometimes there is incongruity. 3 " 5 Heterotaxy, in most organs, is generally of no clinical significance. Involvement of the atrial segment of the heart, however, is almost invariably accompanied by major malformations.…”

Section: H Eterotaxy or Disturbances Of Later-mentioning

confidence: 99%

Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases.

Gerlis

Durà-Vilà

2000

Cardiol Young

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We present two cases in which there was isomeric arrangement of the left atrial appendages in association with multiple spleens and complete heart block. In both of these, the venous connections were normal. In one case the arrangement of the bronchial tree and the lungs was normal, the liver was left-sided, and the stomach was right-sided. In the other case, there was left isomeric broncho-pulmonary morphology, and both liver and stomach were right-sided. These unusual combinations show the need for full description of the morphology of other organs, and the venoatrial connections, in cases with isomeric arrangement of the atrial appendages. They also demonstrate that connection of the inferior caval vein cannot be taken as a reliable marker of an atrium having a morphologically right appendage.

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Thoracic Situs As an Indicator of Atrial Appendage Morphology: A Postmortem Study of 306 Specimens With Situs Solitus in 250 and Heterotaxy in 56 Cases

Calder

2011

Pediatr Cardiol

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Symmetry of bronchi, lung lobation, and atrial appendages is frequently found in patients with heterotaxy syndromes. To evaluate the reliability of thoracic situs as an indicator of atrial appendage morphology, thoracic situs was assessed in 306 postmortem cases: 250 with atrial situs solitus and 56 with heterotaxy syndromes. Five features that indicate thoracic situs were assessed: (1) lung lobation, (2) lengths of main bronchi, (3) ratio of left to right (L/R) bronchial lengths, (4) relationship of bronchi to ipsilateral pulmonary artery, and (5) number of cartilage rings in each main bronchus. In the group with heterotaxy, the expected symmetrical lung lobation, lengths of bronchi, number of cartilage rings, and relations to pulmonary arteries were found in 77, 77, 77, and 95% of cases, respectively. The ratios of L/R bronchial lengths were ≤ 1.5 in 90% of cases. The relations of the bronchi to the pulmonary arteries were the best predictors of symmetrical atrial appendages or splenic syndromes. Bronchial-atrial discordance occurred in ten cases: in situs solitus in one case and in heterotaxy in nine cases. Detection of heterotaxy syndromes is important because of the associated severe congenital cardiac defects. No single feature of thoracic situs is completely reliable. All available data should be used to make the diagnosis.

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Isomerism of the left atrial appendages associated with bronchoatrial discordance and unusual systemic venous drainage

Cited by 4 publications

References 8 publications

Discordances between the different types of atrial arrangement and the positions of the thoraco-abdominal organs

Discordances between the different types of atrial arrangement and the positions of the thoraco-abdominal organs

Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases.

Thoracic Situs As an Indicator of Atrial Appendage Morphology: A Postmortem Study of 306 Specimens With Situs Solitus in 250 and Heterotaxy in 56 Cases

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