“Asplenic syndrome” in association with rudimentary spleen∗

Layman, Thomas E.; Levine, Myron A.; Amplatz, Kurt; Edwards, Jesse E.

doi:10.1016/0002-9149(67)90120-8

Cited by 27 publications

(8 citation statements)

References 5 publications

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“…The asplenia syndrome without asplenia (sic!) has also been described by others.3 28 Finally, diagnostic "labels" affect the way in which patients are investigated. Cardiologists are becoming accustomed to being able to attach exceedingly accurate diagnostic labels to their patients.…”

Section: Discussionmentioning

confidence: 92%

Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation.

Macartney¹,

Zuberbuhler²,

Anderson³

1980

Heart

148

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SUMMARYThe atrial morphology and venous connections were assessed "blind" in 51 necropsy specimens from patients with visceral heterotaxy. This was compared with bronchial morphology as established by dissection. Six specimens were found to have both atria and bronchi in situs solitus or inversus, and were rejected. In the remainder, atrial isomerism was diagnosed, though this required minor revision of the atrial assessment in two patients. Thirty-four patients had isomeric right atria and bronchi, while 11 had isomeric left atria and bronchi.In seven cases, splenic status was unknown, but in seven of the remaining 38 (1844%) atrial isomerism was not associated with either asplenia or polysplenia. Nevertheless, right isomerism was strongly associated with total anomalous pulmonary venous drainage (as is asplenia) and left isomerism was likewise associated with interruption of the inferior vena cava (as is polysplenia). Bilateral superior venae cavae and hepatic veins, and absence of the coronary sinus, were frequent in both forms of isomerism (as they are in asplenia and polysplenia).These findings suggest that atrial situs can be defined as solitus inversus, right isomerism, and left isomerism. This determination of atrial situs is quite independent of any other abnormalities of visceral situs. The high incidence of anomalies of both venous return and common atrium resulted in presumed complete mixing of blood at atrial level in all but one patient (97-8%), making the haemodynamic connection between atria and ventricles almost always ambiguous. To describe this anatomical connection as ambiguous when there are two ventricles present is therefore no more than a recognition of anatomical and haemodynamic reality. To investigate these questions we have studied visceral situs, atrial morphology, and venous connections in 45 patients in whom the diagnosis of visceral heterotaxy was made or confirmed at necropsy.

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Section: Discussionmentioning

confidence: 92%

Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation.

Macartney¹,

Zuberbuhler²,

Anderson³

1980

Heart

148

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“…10,21,33 A rudimentary spleen cannot easily be differentiated from one that is absent. 34 Splenic anatomy can be difficult to determine both clinically and at autopsy. 11 Although not all patients with multiple spleens have isomerism of the left atrial appendages, and not all patients with absence of the spleen have isomerism of the right atrial appendages, it has become customary for many paediatric cardiologists to stratify heterotaxy into the subsets of ''asplenia syndrome'' and ''polysplenia syndrome''.…”

Section: Bodily Arrangement or ''Situs''mentioning

confidence: 99%

The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy

et al. 2007

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In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. The Nomenclature Working Group created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET]. In previous publications from the Nomenclature Working Group, unity has been produced by cross-mapping separate systems for coding, as for example in the treatment of the functionally univentricular heart, hypoplastic left heart syndrome, or congenitally corrected transposition. In this manuscript, we review the nomenclature, definition, and classification of heterotaxy, also known as the heterotaxy syndrome, placing special emphasis on the philosophical approach taken by both the Bostonian school of segmental notation developed from the teachings of Van Praagh, and the European school of sequential segmental analysis. The Nomenclature Working Group offers the following definition for the term "heterotaxy": "Heterotaxy is synonymous with 'visceral heterotaxy' and 'heterotaxy syndrome'. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as 'situs solitus', nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as 'situs inversus'." "Situs ambiguus is defined as an abnormality in which there are components of situs solitus and situs inversus in the same person. Situs ambiguus, therefore, can be considered to be present when the thoracic and abdominal organs are positioned in such a way with respect to each other as to be not clearly lateralised and thus have neither the usual, or normal, nor the mirror-imaged arrangements."The heterotaxy syndrome as thus defined is typically associated with complex cardiovascular malformations. Proper description of the heart in patients with this syndrome requires complete description of both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. The position of the heart in the chest, and the orientation of the cardiac apex, must also be described separately. Particular attention is required for the venoatrial connec...

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“…Asplenia has been described in individuals with left atrial isomerism, and on occasions, "asplenic syndrome" may be associated with a spleen. [7][8][9] Despite these rare discrepancies, asplenia commonly carries a worse prognosis than polysplenia and several investigators have described ways that aid in the prenatal diagnosis of asplenia or polysplenia syndrome. The most obvious way to differentiate between polysplenia and asplenia is to determine the presence of a spleen on gray scale ultrasonography.…”

Section: Discussionmentioning

confidence: 99%

Color Doppler of the Splenic Artery in the Prenatal Diagnosis of Heterotaxic Syndromes

Abuhamad¹,

Robinson²,

Bogdan³

et al. 1999

Amer J Perinatol

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The objective of this paper is to determine whether color and pulsed Doppler of the splenic artery is helpful in the prenatal diagnosis of polysplenia or asplenia in heterotaxic syndromes. Over a 3-year period, localization of the splenic artery by color and pulsed Doppler was attempted on all fetuses with the diagnosis of heterotaxic syndromes. Postnatal follow-up was obtained on all neonates. The diagnosis of heterotaxic syndromes was performed on eight fetuses during the study period. Mean gestational age at diagnosis was 20.1 weeks. All fetuses had situs ambiguous and complex cardiac abnormalities. All pregnancies were managed expectantly and none were terminated. The splenic artery was imaged by color and pulsed Doppler in 6 of 8 fetuses, all with one or multiple spleens confirmed postnatally. The splenic artery could not be imaged in two fetuses, both with asplenia confirmed postnatally. The perinatal mortality rate was 88% (7 of 8) and the one surviving infant is currently alive and well at 3 years of age. Color and pulsed Doppler of the splenic artery can aid in the prenatal diagnosis of heterotaxic syndromes. This information is of value and should result in improved prenatal counseling and management of affected pregnancies.

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“Asplenic syndrome” in association with rudimentary spleen∗

Cited by 27 publications

References 5 publications

Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation.

Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation.

The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy

Color Doppler of the Splenic Artery in the Prenatal Diagnosis of Heterotaxic Syndromes

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