Daubeney, offers essential guidance on the anatomical issues, clinical presentation, diagnosis, and treatment options available to practitioners today. [books.google.de]

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]

Presenting signs and symptoms of the congenital heart defect may include cyanosis, breathlessness, lethargy and poor feeding. [en.wikipedia.org]

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 spleen is almost always affected, and there is syndromic clustering of the malformations corresponding to the type of splenic abnormality present. [smile4riley.com]

• Atrial Septal Defect

  A primary diagnosis was made based on these findings as AM Type Ib with severe PH, heterotaxy syndrome of left-sided polysplenia, and an atrial septal defect. [medical-dictionary.thefreedictionary.com]

  Ostium Primum Atrial Septal Defect Please see Atrial Septal Defect for further information. Ostium Secundum Atrial Septal Defect Please see Atrial Septal Defect for further information. [nicklauschildrens.org]

  The heart defects that are associated with Heterotaxy include: dextocardia, single atrium/ atrial septal defect, single ventricle/ventricular septal defect, transposition of the great vessels, common AV valve, total/partial anomalous pulmonary venous [chd-uk.co.uk]

  I was diagnosed with hypoplastic right heart syndrome, atrial septal defect, transposition of the great vessels, and isthmus coarctation of the aorta. This combination of defects in one baby is incredibly rare. [cdc.gov]

• Asymptomatic

  To the best of our knowledge, this is the third reported case of heterotaxy with polysplenia associated with dextrocardia in an asymptomatic adult patient. [lirias.kuleuven.be]

  The most likely reason that our patient with heterotaxy syndrome was asymptomatic and felt well until she was 16 years of age was that she had no relevant structural or functional cardiac defect. [medical-dictionary.thefreedictionary.com]

  We report a unique case of agenesis of the dorsal pancreas in association with polysplenia/heterotaxy syndrome incidentally identified in an otherwise asymptomatic patient with a prior diagnosis of aortic coarctation. [pancreas.imedpub.com]

  Some individuals with left atrial isomerism may be asymptomatic too. [dovemed.com]

  Children with left atrial isomerism may be asymptomatic until later in childhood and even into adulthood. Diagnosis of Heterotaxy Syndrome Heterotaxy syndrome can be diagnosed before or after birth. [rileychildrens.org]

• Congestive Heart Failure

  Presence of coarctation of aorta elevates systemic systolic pressure and increases the pulmonary blood flow, thus worsening failure. An increase in PVR helps regulate pulmonary blood flow and improves congestive heart failure. [japi.org]

  […] also Failure, heart ICD-10-CM Diagnosis Code I50.9 Heart failure, unspecified 2016 2017 2018 2019 Billable/Specific Code Applicable To Cardiac, heart or myocardial failure NOS Congestive heart disease Congestive heart failure NOS Type 2 Excludes fluid [icd10data.com]

  Ventricular septal defects occur in about one third of babies with transposition (see Figure 10-7 A ), and when present, may result in congestive heart failure from the large blood flow. [clinicalgate.com]

• Poor Feeding

  Presenting signs and symptoms of the congenital heart defect may include cyanosis, breathlessness, lethargy and poor feeding. [en.wikipedia.org]

  Over time, your baby may develop poor feeding, fast breathing, increased sweating when they eat, or poor weight gain. [chdbabies.com]

• Pulmonary Valve Stenosis

  Classically, Heterotaxy comprises of: Cardiac looping malformations – commonly Fallot’s Tetralogy, Transposition of the Great Vessels, Pulmonary Valve Stenosis, and ventricular and atrial septal defects. [chdbabies.com]

  MVP = mitral valve prolapse. PAPVR = partial anomalous pulmonary venous return. PDA = patent ductus arteriosus. PPS = peripheral pulmonary stenosis. PS = pulmonary stenosis. PVS = pulmonary valve stenosis. SVAS = supravalvular aortic stenosis. [mja.com.au]

• Tachypnea

  Less commonly children who have increased blood flow to lungs may present with symptoms of excessive pulmonary blood flow, for example tachypnea (rapid breathing) or poor growth. [pediatricheartspecialists.com]

• Hepatomegaly

  There was a tender midline hepatomegaly. Chest X-ray PA view (Figure 3) confirmed dextrocardia with the fundic gas shadow on the left side (Figure 4). [japi.org]

  Physical exam demonstrated a non-tender, nondistended abdomen without notable hepatomegaly, splenomegaly, palpable masses, or audible bruits. Laboratory values revealed normal pancreatic and liver biochemistry, fasting glucose and lipid profile. [pancreas.imedpub.com]

• Heart Disease

  Designed to meet the needs of clinicians working with adults with congenital heart disease, Diagnosis and Management of Adult Congenital Heart Disease, by Drs. Michael A. Gatzoulis, Gary D. Webb, and Piers E. F. [books.google.de]

  The origin of most congenital heart disease is thought to be multifactorial, implying both anomalous expression of genes and the influence of epigenetic factors. [semanticscholar.org]

  Specific treatment of congenital heart disease can be seen in their separate articles. situs classification situs solitus situs inversus isomerism congenital heart disease Promoted articles (advertising) [radiopaedia.org]

  Guidelines for the outpatient management of complex congenital heart disease. Congenital Heart Disease, 1(1-2), 10-26. doi:10.1111/j.1747-0803.2006.00002.x [Context Link] Woodward C. S. (2011). Keeping children with congenital heart disease healthy. [nursingcenter.com]

  The incidence of congenital heart disease. J. Am. Coll. Cardiol. 2002; 39(12):1890-900. PMID: 12084585 Nakhleh, N, et al. High prevalence of respiratory ciliary dysfunction in congenital heart disease patients with heterotaxy. [invitae.com]

• Heart Failure

  Presence of coarctation of aorta elevates systemic systolic pressure and increases the pulmonary blood flow, thus worsening failure. An increase in PVR helps regulate pulmonary blood flow and improves congestive heart failure. [japi.org]

  All of these findings are signs that the heart is not able to pump enough blood to the body to meet its needs and is called “heart failure.” [chdbabies.com]

  […] with growth and eating, developmental delays, difficulty with exercise, heart rhythm problems, heart failure, sudden cardiac arrest or stroke Most causes of CHDs are unknown. [nicksheart.org]

  […] also Failure, heart ICD-10-CM Diagnosis Code I50.9 Heart failure, unspecified 2016 2017 2018 2019 Billable/Specific Code Applicable To Cardiac, heart or myocardial failure NOS Congestive heart disease Congestive heart failure NOS Type 2 Excludes fluid [icd10data.com]

  Complications include: Blocked intestines (due to a condition called intestinal malrotation) Heart failure Infection (heterotaxy with no spleen) Infertility in males (Kartagener syndrome) Repeated pneumonias Repeated sinus infections (Kartagener syndrome [medlineplus.gov]

• Hypertension

  Features the addition of four new chapters : A Historic Perspective; Quality of Life in Patients with Pulmonary Hypertension; Psychosocial Issues in ACHD; Supportive and Palliative Care for End-Stage ACHD. [books.google.de]

  Diagnosis of heterotaxy syndrome (with polysplenia, azygous continuation of the IVC, repaired atrial septal defect and pulmonary arterial hypertension) was made, with incidental associated mild aortic coarctation and left renal dysplasia. [medical-dictionary.thefreedictionary.com]

  Pulmonary Atresia Pulmonary Hypertension Hypertension means high blood pressure, but in the case of pulmonary hypertension, the high pressure affects the pulmonary arteries that take blood from the heart to the lungs. [nicklauschildrens.org]

  Heterotaxy syndrome with severe pulmonary hypertension in an adult. South Med J. 2002;95:536–8. Google Scholar 24. Cho YH, Jin SJ, Je HC, et al. A case of noncompaction of the ventricular myocardium combined with situs ambiguous with polysplenia. [link.springer.com]

  The cause of premature delivery was attributed to maternal illness- pregnancy induced hypertension. His parents were of non-consanguineously married. [biopublisher.ca]

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]

Daubeney, offers essential guidance on the anatomical issues, clinical presentation, diagnosis, and treatment options available to practitioners today. [books.google.de]

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]

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]

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]

(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]

While much progress has been made, we need to develop new treatments for CHDs so that children undergo fewer invasive surgeries and improve the long-term prognosis for children with CHDs.” [news.heart.org]

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]

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]

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]

(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]

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]

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]

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]