Presentation
Daubeney, offers essential guidance on the anatomical issues, clinical presentation, diagnosis, and treatment options available to practitioners today. [books.google.de]
Presenting signs and symptoms of the congenital heart defect may include cyanosis, breathlessness, lethargy and poor feeding. [en.wikipedia.org]
The case review also discussed short pancreas and malrotation in some patients, neither of which were visualized in the case presented here. 5 Conclusion Heterotaxy syndrome with polysplenia presents uniquely on radiography, and physicians should consider [appliedradiology.com]
Cyanotic congenital heart disease is the main presentation in right isomerism. Left isomerism tends to present later in childhood or even in adulthood since it is less associated with complex congenital heart disease. [radiopaedia.org]
The case presented had features with LI with duodenal atresia. [biopublisher.ca]
Workup
A minimal workup should include 1 : chest radiograph echocardiogram: to assess for congenital heart disease abdominal ultrasound: to assess intra-abdominal contents (especially spleen) upper GI series: to rule out malrotation Treatment and prognosis Treatment [radiopaedia.org]
Treatment
Daubeney, offers essential guidance on the anatomical issues, clinical presentation, diagnosis, and treatment options available to practitioners today. [books.google.de]
A minimal workup should include 1 : chest radiograph echocardiogram: to assess for congenital heart disease abdominal ultrasound: to assess intra-abdominal contents (especially spleen) upper GI series: to rule out malrotation Treatment and prognosis Treatment [radiopaedia.org]
Treatments Treatments Every case of heterotaxy syndrome is unique, as are each patient’s treatments. The exact nature of the heart defects, the degree of splenic involvement and the extent of other organ abnormalities dictate the treatment. [rileychildrens.org]
What are the treatment options for heterotaxy syndrome? Treatment for heterotaxy syndrome varies depending on the severity of the heart defects. [ourheartprincess.blogspot.com]
Children with Kartagener syndrome will need repeated treatment with antibiotics for sinus infections. Children with a missing or abnormal spleen need long-term antibiotics. [medlineplus.gov]
Prognosis
(Outcomes/Resolutions) The prognosis of Heterotaxy Syndrome is dictated by the organ affected, as well as the type and severity of symptoms in an individual Generally, children diagnosed before birth or in infancy have a poorer prognosis than when the [dovemed.com]
These results will provide important information on the causes, management, and prognosis of heterotaxy and related congenital heart defects. [clinicaltrials.gov]
Prognosis Without corrective surgery, most children with heterotaxy syndrome and significant heart problems will not survive beyond the first year of life. [pediatricheartspecialists.com]
A minimal workup should include 1 : chest radiograph echocardiogram: to assess for congenital heart disease abdominal ultrasound: to assess intra-abdominal contents (especially spleen) upper GI series: to rule out malrotation Treatment and prognosis Treatment [radiopaedia.org]
The prognosis and treatment of patients with cardiac malposition are determined by underlying heart diseases. Asplenia increases the risk of severe infections such as bacterial sepsis, which makes daily antibiotic prophylaxis necessary. [ncbi.nlm.nih.gov]
Etiology
The etiology is really poorly assumed but it is believed to also be linked to: Family history of malformations Maternal diabetes mellitus Maternal cocaine use This would seem to suggest that both environmental factors as well as genetic mutations play [syndrome.org]
There are reports of families having several members with HTS and the etiology remains unclear ( Cesko et al., 1998). The case reported did not have any significant dysmorphism and had complex CHD of the LI. [biopublisher.ca]
The etiology of congenital heart block when the heart is structurally normal is most often a fetal immune inflammatory response, caused by the binding of antibodies to the fetal cardiac conduction system. [fetalsono.com]
She had extensive evaluation to determine the etiology for the pulmonary embolism and was noted to have an anatomic variation consistent with heterotaxy syndrome on imaging studies. [bmchematol.biomedcentral.com]
(Etiology) Heterotaxy Syndrome may be caused by mutations in over 60 genes, which may be inherited in an autosomal recessive, autosomal dominant, or X-linked manner. [dovemed.com]
Epidemiology
Epidemiologic studies suggest that a genetic or environmental cause for CHD is identifiable in approximately 20%–30% of cases, with 3%–5% of CHD due to a detectable single-gene disorder. [invitae.com]
Laterality defects in the national birth defects prevention study (1998-2007): birth prevalence and descriptive epidemiology. Am J Med Genet A. 2014 Oct;164A(10):2581-91. doi: 10.1002/ajmg.a.36695. Epub 2014 Aug 6. [ghr.nlm.nih.gov]
Heterotaxia as an outcome of maternal diabetes: an epidemiological study. Am J Med Genet 2001; 99 : 142–6 [ PubMed ] [ Google Scholar ] 13. Bartram U, Wirbelauer J, Speer CP. [ncbi.nlm.nih.gov]
This significant shift in the epidemiology of CHD requires nurses to take action in preparing children with CHD and their families for their teenage years and young adulthood. [nursingcenter.com]
Pathophysiology
A discussion on pathophysiology of venous thromboembolism in pregnancy and heterotaxy syndrome has been undertaken along with treatment approach in such situations. [bmchematol.biomedcentral.com]
The pathophysiology of syringomyelia changes with different types. In the cases associated with the malformation of the cranium cervical, the cerebrospinal fluid cannot flow freely from the ventricle IV towards the subarachnoid space. [ncbi.nlm.nih.gov]
Prevention
Your doctor will help you prevent infections if you’re at a higher risk of getting sick. If you have a more complicated case of dextrocardia, you may face health problems throughout your life. [healthline.com]
Prevention of neural tube defects: results of the Medical Research Council Vitamin Study. Seite 332 - Crane JP, LeFevre ML, Winborn RC, et al. [books.google.de]
Subsequently she was placed on low dose aspirin (81 mg) to prevent recurrent VTE (especially given her heterotaxy syndrome) based on the Warfarin and Aspirin (WARFASA) and Aspirin to Prevent Recurrent Venous Thromboembolism (ASPIRE) trials as well as [bmchematol.biomedcentral.com]
Heterotaxy syndrome can’t be prevented, but a lot can be done to improve and often completely repair children's hearts at any age. [nemours.org]