Levocardia with visceral heterotaxy—isolated levocardia: Pathologic anatomy and its clinical implications

Liberthson, Richard R.; Hastreiter, Alois R.; Sinha, Sumit; Bharati, Saroja; Novak, Gertrude M.; Lev, Maurice

doi:10.1016/0002-8703(73)90524-3

Cited by 53 publications

(27 citation statements)

References 40 publications

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“…For the purpose of assessing the haemodynamic state, a common atrium was held to be present when the maximum extent of the atrial septum was a superior rim or extended strand. Thus common atrium was present in 25 (56-8%/) cases, 21 with right isomerism and four with left isomerism. Fig.…”

Section: Atrial Septationmentioning

confidence: 96%

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: Atrial Septationmentioning

confidence: 96%

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

Macartney¹,

Zuberbuhler²,

Anderson³

1980

Heart

148

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show abstract

“…[11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] With their clinical colleagues, they compiled clinicopathological correlations and published extensively on specimens that constituted their cardiac registries. Collectively, they described and characterized the entire spectrum of congenitally malformed hearts and produced a number of classifications of various cardiac anomalies, including tricuspid atresia, interruption of the aortic arch, truncus arteriosus, ventricular septal defect, double-inlet ventricle, and double-outlet right ventricle.…”

Section: Cardiac Anatomy and The Nosology Of Congenital Heart Diseasementioning

confidence: 99%

Pediatric Cardiology and Cardiovascular Surgery: 1950-2000

Freedom

Lock²,

Bricker³

2000

Circulation

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“…Van Mierop, on many occasions, has pointed out that the atria in these conditions cannot be classified as either right or left on anatomical criteria, since both atria show characteristics of either a right or a left atrium (Van Mierop et al, 1964, 1972a. He coined the term 'isomerism' to indicate the symmetrical appearance of the atrial appendages under these circumstances (Van Mierop et al, 1964), while other investigators employed the terms 'uncertain' (Van Praagh et al, 1964b) or 'indeterminate' (Lev et al, 1968;Liberthson et al, 1973). The latter observers used the term 'indeterminate' for the situs of the atria when the atrial anatomy was highly abnormal, not necessarily associated with a symmetrical appearance of the atrial appendages.…”

Section: Terminology Of Atrial Situsmentioning

confidence: 99%

“…Indeed, in patients with a normal spleen only 2 exceptions to this rule have as yet been reported (Clarkson et al, 1972;Liberthson et al, 1973;Brandt and Calder, 1977). However, anatomical studies have disclosed several other exceptions to this 'rule' in the presence of splenic abnormalities, albeit that the various authors do not always emphasise the discrepancy (Freedom and Fellows, 1973;Liberthson et al, 1973;Freedom and Harrington, 1974;Anderson et al, 1976). These exceptions necessitate a reconsideration of the criteria that determine the classification of atrial situs.…”

mentioning

confidence: 99%

“…In these circumstances it has been suggested that atrial situs can be inferred from the configuration of the tracheobronchial tree (Landing et al, 1971; Received for publication 27 July 1978 Partridge et al, 1975), since it is widely accepted that atrial situs corresponds with that of the thoracic organs (Van Praagh et al, 1964a;Van Mierop et al, 1970). Indeed, in patients with a normal spleen only 2 exceptions to this rule have as yet been reported (Clarkson et al, 1972;Liberthson et al, 1973;Brandt and Calder, 1977). However, anatomical studies have disclosed several other exceptions to this 'rule' in the presence of splenic abnormalities, albeit that the various authors do not always emphasise the discrepancy (Freedom and Fellows, 1973;Liberthson et al, 1973;Freedom and Harrington, 1974;Anderson et al, 1976).…”

mentioning

confidence: 99%

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How to determine atrial situs? Considerations initiated by 3 cases of absent spleen with a discordant anatomy between bronchi and atria.

Caruso¹,

Becker²

1979

Heart

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Three cases have been documented in which abdominal heterotaxy with absent spleen was accompanied by a discordant anatomy between the tracheobronchial tree and the atria. In 1 instance the tracheobronchial anatomy was as in situs inversus but the venous connections and the atrial appendages suggested situs solitus. The second case exhibited a situs solitus anatomy of the tracheobronchial tree, but the morphological features of the atria were mixed up in the sense that the venous connections and atrial septum suggested situs inversus, whereas the atrial appendages were of a right isomeric type. The third case was interesting in showing a symmetrical tracheobronchial tree not accompanied by isomeric atrial appendages. The latter and the atrial septum exhibited situs solitus characteristics, though the venous connections suggested 'situs ambiguus'. Such observations have led us to reconsider the criteria that determine the classification of atrial situs. It is our contention that in cases characterised by major abnormalities in atrial anatomy, particularly with respect to the venous connections, the prime feature for identification of atrial situs would be the morphology of the atrial appendages, which represent the 'true' atrial Anlagen. Positive identification of atrial appendages would also enable a more precise definition of the term 'atrial situs ambiguus'. This term can then be defined as the situs characterised by either bilateral right-or bilateral left-sided morphological characteristics of the atrial appendages, irrespective of the venous connections or atrial septal anatomy.

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Levocardia with visceral heterotaxy—isolated levocardia: Pathologic anatomy and its clinical implications

Cited by 53 publications

References 40 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.

Pediatric Cardiology and Cardiovascular Surgery: 1950-2000

How to determine atrial situs? Considerations initiated by 3 cases of absent spleen with a discordant anatomy between bronchi and atria.

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