Cardiac and Non-Cardiac Abnormalities in Heterotaxy Syndrome

@article{Mishra2015CardiacAN,
  title={Cardiac and Non-Cardiac Abnormalities in Heterotaxy Syndrome},
  author={Smita Mishra},
  journal={The Indian Journal of Pediatrics},
  year={2015},
  volume={82},
  pages={1135-1146}
}
  • Smita Mishra
  • Published 26 November 2015
  • Medicine
  • The Indian Journal of Pediatrics
Thoraco-abdominal viscera have unique morphological asymmetry, unlike the body’s external organs. Heterotaxy syndrome is a disorder in which there is a loss of normal left to right asymmetry of thoraco-abdominal viscera and their naturally proscribed spatial relationship. It has multiple anatomical alterations, culminating into physiological and hemodynamic consequences. It is divided into two groups on the basis of morphology of the two atrial appendages. These subgroups are - 1) Isomerism of… 
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References

SHOWING 1-10 OF 48 REFERENCES
Characterization of atrial morphology and sinus node morphology in heterotaxy syndrome: an autopsy-based study of 41 cases (1950-2008).
TLDR
The findings indicate that the sinus node is not a morphologically right-sided structure, and its presence therefore is not consistently related to the sidedness of the atria.
Extracardiac anomalies in the heterotaxy syndromes with focus on anomalies of midline-associated structures.
TLDR
The high incidence of midline-associated defects supports the theory that the midline plays a critical role in establishing left-right asymmetry in the body and suggests that mutations that disrupt the transforming growth factor beta pathway may result in heterotaxy.
Dorsal pancreas agenesis and polysplenia/heterotaxy syndrome: a novel association with aortic coarctation and a review of the literature.
TLDR
In patients with congenital heart disease, there is increased likelihood for the presence of other congenital malformations including polysplenia/heterotaxy syndrome and agenesis of the dorsal pancreas, including late-onset diabetes mellitus, pancreatitis, and intestinal volvulus.
Intrauterine diagnosis of heterotaxy syndrome.
TLDR
Heterotaxy syndrome is usually detected in fetuses with the sonographic cardiac abnormalities and visualization of the pulmonary venous return and outflow obstruction and characterization of the rhythm disturbances are feasible, however, in spite of prenatal diagnosis, the prognosis remains poor.
Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis.
TLDR
The study indicated that the cardiac anomalies in polysplenia were less severe than they were in asplenia and therefore the prognosis in the former syndrome is likely to be more favourable.
Asplenia syndrome: insight into embryology through an analysis of cardiac and extracardiac anomalies.
TLDR
It was hypothesized that most defects originate at Streeter Horizon XIII; patients averaged 3.2 Horizon XIII defects, more than at any other stage, and specific anomalies were less frequent, although much more prevalent than in the general population.
Congenital Cardiac Disease Associated with Polysplenia: A Developmental Complex of Bilateral “Left‐Sidedness”
TLDR
This study suggests that the developmental complex of multiple spleens is closely related to the asplenic syndrome, with the important difference being left- sided symmetry rather than right-sided symmetry.
Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation.
TLDR
The atrial morphology and venous connections were assessed "blind" in necropsy specimens from patients with visceral heterotaxy and suggest that atrial situs can be defined as solitus inversus, right isomersism, and left isomerism.
Heterotaxy Syndrome With Severe Pulmonary Hypertension in an Adult
TLDR
This case exhibits 2 particular and partially novel features: primary diagnosis of heterotaxy syndrome may be delayed until adulthood, andheterotaxy Syndrome may be associated with pulmonary hypertension, possibly on the basis of longstanding portosystemic shunts.
Abdominal manifestations of situs anomalies in adults.
  • A. Fulcher, M. Turner
  • Medicine
    Radiographics : a review publication of the Radiological Society of North America, Inc
  • 2002
TLDR
Recognition of the spectrum of situs anomalies is important because the altered anatomy associated with these anomalies may result in confusing imaging findings when seen in conjunction with acquired diseases.
...
1
2
3
4
5
...