Arrangement of the systemic and pulmonary venous components of the atrial chambers in hearts with isomeric atrial appendages

Min, Joongkee; Kim, Chi-Yon; Oh, Mee‐Hye; Chun, Yi Kyeong; Suh, Yeon-Lim; Kang, I-Seok; Lee, Heung-Jae; Seo, Jeong‐Wook

doi:10.1017/s1047951100009719

Cited by 14 publications

(33 citation statements)

References 17 publications

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“…Abnormal LR cardiomyocyte compositions were frequently associated with CHDs, which suggests that the ability of different regions of the heart to become differentiated into particular structures is linked to cardiac cell lineage. For example, the left and right atria derive from a common progenitor, the common atrium, which must become divided into two chambers with distinct LR differences (Anderson, 1992;Markwald et al, 1998;Min et al, 2000). LR labeling of the primary heart fields previously suggested that this division is related to the LR origin of cells that contribute to this region (Gormley and Nascone-Yoder, 2003).…”

Section: Research Articlementioning

confidence: 99%

Left-right lineage analysis of the embryonicXenopusheart reveals a novel framework linking congenital cardiac defects and laterality disease

2006

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The significant morbidity and mortality associated with laterality disease almost always are attributed to complex congenital heart defects (CHDs), reflecting the extreme susceptibility of the developing heart to disturbances in the left-right (LR) body plan. To determine how LR positional information becomes 'translated' into anatomical asymmetry, left versus right side cardiomyocyte cell lineages were traced in normal and laterality defective embryos of the frog, Xenopus laevis. In normal embryos, myocytes in some regions of the heart were derived consistently from a unilateral lineage, whereas other regions were derived consistently from both left and right side lineages. However, in heterotaxic embryos experimentally induced by ectopic activation or attenuation of ALK4 signaling, hearts contained variable LR cell composition, not only compared with controls but also compared with hearts from other heterotaxic embryos. In most cases, LR cell lineage defects were associated with abnormal cardiac morphology and were preceded by abnormal Pitx2c expression in the lateral plate mesoderm. In situs inversus embryos there was a mirror image reversal in Pitx2c expression and LR lineage composition. Surprisingly, most of the embryos that failed to develop heterotaxy or situs inversus in response to misregulated ALK4 signaling nevertheless had altered Pitx2c expression, abnormal cardiomyocyte LR lineage composition and abnormal heart structure, demonstrating that cardiac laterality defects can occur even in instances of otherwise normal body situs. These results indicate that: (1) different regions of the heart contain distinct LR myocyte compositions; (2) LR cardiomyocyte lineages and Pitx2c expression are altered in laterality defective embryos; and (3) abnormal LR cardiac lineage composition frequently is associated with cardiac malformations. We propose that proper LR cell composition is necessary for normal morphogenesis, and that misallocated LR cell lineages may be causatively linked with CHDs that are present in heterotaxic individuals, as well as some 'isolated' CHDs that are found in individuals lacking overt features of laterality disease.

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Section: Research Articlementioning

confidence: 99%

Left-right lineage analysis of the embryonicXenopusheart reveals a novel framework linking congenital cardiac defects and laterality disease

2006

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“…2A-D). Such features are typically found in human hearts with left cardiac isomerism as well as in mouse models for this subset of the visceral heterotaxy syndrome [18][19][20].…”

Section: Discussionmentioning

confidence: 97%

Spontaneous Left Cardiac Isomerism in Chick Embryos: Case Report, Review of the Literature, and Possible Significance for the Understanding of Ventricular Non-Compaction Cardiomyopathy in the Setting of Human Heterotaxy Syndromes

Männer

2019

Preprint

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Except for a few species, the outer shape of vertebrates normally is characterized by bilateral symmetry. The inner organs, on the other hand, normally are arranged in bilaterally asymmetric patterns, which are of special importance for the normal function of the cardiovascular system of lung-breathing vertebrates. Deviations from the normal organ asymmetry can occur in the form of mirror imagery of the normal arrangement (situs inversus), or in the form of arrangements that have the tendency for development of bilateral symmetry, either in a pattern of bilateral left-sideness (left isomerism) or bilateral right-sidedness (right isomerism). The latter two forms of visceral situs anomalies are called “heterotaxy syndromes”. During the past 30 years, remarkable progress has been made in uncovering of the genetic etiology of heterotaxy syndromes. However, the pathogenetic mechanisms causing the spectrum of cardiovascular defects found in these syndromes remain poorly understood. In the present report, a spontaneous case of left cardiac isomerism found in a HH-stage 23 chick embryo is described. The observations made in this case suggest that hearts with left cardiac isomerism may have the tendency for development of a non-compaction cardiomyopathy caused by defective development of the proepicardium.

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“…This diagnosis is furthermore supported by the fact that the abnormal embryonic chick heart had striking hypoplasia of its systemic venous component (sinus venosus) in combination with anomalous pulmonary venous drainage to the right-sided atrium (Figure 2A-D). Such features are typically found in human hearts with left cardiac isomerism as well as in mouse models for this subset of the visceral heterotaxy syndrome [18][19][20]. Previous data from mice disclosed a correlation between the isomeric expression of Pitx2 and the occurrence of the double outlet from the right ventricle (DORV) [11].…”

Section: Discussionmentioning

confidence: 98%

Spontaneous Left Cardiac Isomerism in Chick Embryos: Case Report, Review of the Literature, and Possible Significance for the Understanding of Ventricular Non-Compaction Cardiomyopathy in the Setting of Human Heterotaxy Syndromes

Männer

2019

JCDD

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The outer shape of most vertebrates is normally characterized by bilateral symmetry. The inner organs, on the other hand, are normally arranged in bilaterally asymmetric patterns. Congenital deviations from the normal organ asymmetry can occur in the form of mirror imagery of the normal arrangement (situs inversus), or in the form of arrangements that have the tendency for the development of bilateral symmetry, either in a pattern of bilateral left-sidedness (left isomerism) or bilateral right-sidedness (right isomerism). The latter two forms of visceral situs anomalies are called “heterotaxy syndromes”. During the past 30 years, remarkable progress has been made in uncovering the genetic etiology of heterotaxy syndromes. However, the pathogenetic mechanisms causing the spectrum of cardiovascular defects found in these syndromes remain poorly understood. In the present report, a spontaneous case of left cardiac isomerism found in an HH-stage 23 chick embryo is described. The observations made in this case confirmed the existence of molecular isomerism in the ventricular chambers previously noted in mouse models. They, furthermore, suggest that hearts with left cardiac isomerism may have the tendency for the development of non-compaction cardiomyopathy caused by defective development of the proepicardium.

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Arrangement of the systemic and pulmonary venous components of the atrial chambers in hearts with isomeric atrial appendages

Cited by 14 publications

References 17 publications

Left-right lineage analysis of the embryonicXenopusheart reveals a novel framework linking congenital cardiac defects and laterality disease

Left-right lineage analysis of the embryonicXenopusheart reveals a novel framework linking congenital cardiac defects and laterality disease

Spontaneous Left Cardiac Isomerism in Chick Embryos: Case Report, Review of the Literature, and Possible Significance for the Understanding of Ventricular Non-Compaction Cardiomyopathy in the Setting of Human Heterotaxy Syndromes

Spontaneous Left Cardiac Isomerism in Chick Embryos: Case Report, Review of the Literature, and Possible Significance for the Understanding of Ventricular Non-Compaction Cardiomyopathy in the Setting of Human Heterotaxy Syndromes

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