AnatomyDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and Equipment
Heterotaxy SyndromeSitus InversusCardiovascular AbnormalitiesDextrocardiaSyndromeBlalock-Taussig ProcedureAbnormalities, MultipleHeart Defects, CongenitalRespiratory System AbnormalitiesCiliary Motility DisordersSpleenGenetic Diseases, X-LinkedAxonemal Dyneins
Hypoplastic Left HeaIsomerismAtrioventricular CanalDouble outlet riCongenitalAbnormalitiesSitusAortic archDiGeorgePatientsAbnormalAutosomalPulmonary veinsAbnormalityInvolvesMidline liverAbnormallySevereCanalArrangementSpectrumGeneticConditionYearsFeaturesFamiliesConnection
Hypoplastic Left Hea3
  • Outcomes for the superior cavopulmonary connection in children with hypoplastic left heart syndrome: a 30-year experience. (chop.edu)
  • Savannah is an 18-month-old with hypoplastic left heart syndrome, heterotaxy, and cleft lip/palate. (wubbanub.com)
  • Hypoplastic left heart syndrome (HLHS) is the most common functionally univentricular (single ventricle) anomaly. (sts.org)
Isomerism5
  • Institute Jantung Negara (IJN) confirmed Ariana has Heterotaxy Syndrome - Right Atrial Isomerism with severe mutiple heart defects. (theasianparent.com)
  • A rare birth defect, heterotaxy syndrome (isomerism) usually involves heart defects of varying types and severity. (chop.edu)
  • The CT scan identified the features of heterotaxy syndrome with left isomerism and an abnormal blush from the branch of splenic artery supplying the greater curvature of the stomach. (springeropen.com)
  • left isomerism, i.e. polysplenia syndrome and right isomerism, i.e. asplenia syndrome. (springeropen.com)
  • We are reporting the first case of left isomerism variant of heterotaxy syndrome presenting with upper gastrointestinal bleed (non-variceal) from the greater curvature of stomach which was managed successfully with radiological intervention. (springeropen.com)
Atrioventricular Canal1
  • Double Choker: Double Aortic Arch with Bilateral Aortic Coarctation Associated with Heterotaxy-Asplenia Syndrome and Complex Atrioventricular Canal Defect. (rchsd.org)
Double outlet ri1
  • Amber was diagnosed with heterotaxy syndrome and double outlet right ventricle (DORV) with pulmonary atresia soon after she was born. (chop.edu)
Congenital6
  • My focus was on the gene RAPGEF5 , which was previously identified as a candidate gene for heterotaxy, a congenital disease affecting heart development and the spatial arrangement of organs. (biologists.com)
  • It is estimated that 1 in 10,000 people are diagnosed with heterotaxy, and is the cause of 3% of all congenital heart cases [1]. (biologists.com)
  • According to the Heterotaxy Connection, a non-profit organization dedicated to to supporting, educating, and empowering families affected by Heterotaxy Syndrome: "Heterotaxy syndrome is a congenital condition that disrupts the normal left-right asymmetry of the body. (localanchor.com)
  • The International Nomenclature Committee for Pediatric and Congenital Heart Disease defines heterotaxy syndrome as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body (Jacobs et al. (springeropen.com)
  • Autosomal visceral heterotaxy-7 is an autosomal recessive developmental disorder characterized by complex congenital heart malformations and/or situs inversus and caused by defects in the normal left-right asymmetric positioning of internal organs. (nih.gov)
  • Some form of complex congenital heart disease is almost always present in Down syndrome. (msdmanuals.com)
Abnormalities4
  • Life expectancy for left sided heterotaxy is better than for its right sided counterpart due to association with less severe cardiac abnormalities. (radiopaedia.org)
  • Heterotaxy syndrome is a complex set of abnormalities related to arrangements of the internal thoracic-abdominal structures across the left-right axis. (heraldopenaccess.us)
  • Does loss of RAPGEF5 lead to any developmental abnormalities such as heterotaxy? (biologists.com)
  • Heterotaxy refers to developmental abnormalities of "sidedness" of the thoracic and abdominal organs. (msdmanuals.com)
Situs6
  • The discovery of kindreds in which both heterotaxy and situs inversus totalis occur strongly suggests that these are not truly separate diseases. (medscape.com)
  • Because PCD is present in about a quarter of situs inversus totalis patients and due to the fact that situs inversus totalis is present in nearly half of individuals with PCD, this article covers both heterotaxy syndrome and PCD. (medscape.com)
  • [ 9 ] That is, the ratio of the heterotaxy phenotype to the situs inversus totalis phenotype decreased with progressive inbreeding. (medscape.com)
  • Since PCD is present in about a quarter of situs inversus totalis patients and since situs inversus totalis is present in nearly half of individuals with PCD, this article covers both heterotaxy syndrome and PCD. (medscape.com)
  • The "heterotaxy syndrome" also called "situs ambiguus" is a rare condition in which the internal organs of the chest and abdomen are abnormally arranged. (springeropen.com)
  • recessive disorder consisting of the triad of Approximately 50 % of patients with SCD sinusitis, bronchiectasis and situs inversus with have laterality defects (including situs inversus dextrocardia and represents a subgroup of totalis and, less frequently, heterotaxy and primary ciliary dyskinesia (PCD). (bvsalud.org)
Aortic arch1
  • We present a case of left isomeric heterotaxy, a right aortic arch, and a left brachiocephalic artery arising from a left ductus arteriosus. (heraldopenaccess.us)
DiGeorge1
  • Also, your child doesn't need antibiotics to help prevent infections with a few notable exceptions, including children with heterotaxy syndromes like asplenia, or, in some cases of DiGeorge syndrome . (heart.org)
Patients5
  • [ 3 ] At least 12% of primary ciliary dyskinesia (PCD) patients have heterotaxy. (medscape.com)
  • [ 5 , 6 ] a patient's splenic phenotype should be viewed as merely one phenotypic aspect of an underlying laterality disorder, even though many prior reviews of heterotaxy syndrome have divided patients into two groups based on only splenic phenotype. (medscape.com)
  • Upper gastrointestinal bleed in the patients with heterotaxy syndrome is a rare presentation of a rare condition. (springeropen.com)
  • AV septal defect is also common among patients with heterotaxy syndromes. (msdmanuals.com)
  • and Arrhythmias in Patients with Genetic Arrhythmia Syndromes. (123library.org)
Abnormal1
  • Heterotaxy is an abnormal arrangement of viscera across the left-right axis of the body often associated with a variety of complex cardiac lesions. (heraldopenaccess.us)
Autosomal1
  • Kartagener syndrome (KS) is an autosomal prevalence is 1/15 000 to 1/30 000 live births1. (bvsalud.org)
Pulmonary veins1
  • 7) 2 major anomaly of the systemic and/or pulmonary veins, as in the heterotaxy syndrome with asplenia. (nih.gov)
Abnormality1
  • Down Syndrome (Trisomy 21) Down syndrome is an abnormality of chromosome 21 that can cause intellectual disability, microcephaly, short stature, and characteristic facies. (msdmanuals.com)
Involves1
Midline liver1
  • Her baby girl was born with Heterotaxy Syndrome (HS), with: half a functioning heart and a midline liver instead of on the right like other children. (theasianparent.com)
Abnormally1
  • When Katie Stanley and Betsy Olesen found out their soon-to-be-born baby, William, had heterotaxy syndrome, a rare condition where organs form abnormally, it meant he needed a heart transplant shortly after birth. (morningsun.net)
Severe1
  • Sung was born with a severe heart condition called Heterotaxy Syndrome. (htcflorida.org)
Canal1
  • She has complete AV canal, pulmonary atresia, supraventicular tachycardia, and Heterotaxy syndrome with malrotation. (gofundme.com)
Arrangement1
  • the word heterotaxy is derived from the Greek heteros, meaning "other" and taxis , meaning "arrangement. (medscape.com)
Spectrum2
  • This syndrome is usually associated with a spectrum of health-related conditions involving birth defects in organs like the heart, spleen, liver, lungs and others. (springeropen.com)
  • We'll be gathering together to learn from some of the foremost medical experts in heterotaxy and offering workshops, breakout sessions, and panel discussions packed with diverse experiences from across the spectrum of heterotaxy. (weaversorchard.com)
Genetic1
  • 2015). For a discussion of the genetic heterogeneity of visceral heterotaxy, see HTX1 (306955). (nih.gov)
Condition1
  • For example, he developed the unique CHOP Scoring System for Twin-Twin Transfusion Syndrome, a potentially fatal condition where twins sharing a placenta experience uneven blood flow. (chop.edu)
Years1
  • Ethan was diagnosed with heterotaxy syndrome when I was 25 weeks pregnant and home on maternity leave from the charity hospital ship where our family had been serving for nearly six years. (weaversorchard.com)
Features2
  • An incidental note was made of multiple features of left sided heterotaxy syndrome - multiple aberrant nodules of splenic tissue, an azygous continuation of the IVC, midgut malrotation. (radiopaedia.org)
  • This prompted review of previous imaging to identify further features of left sided heterotaxy syndrome. (radiopaedia.org)
Families1
  • More than anything, we'll be giving heterotaxy families the chance to be together with people who truly understand. (weaversorchard.com)
Connection1
  • Here we are presenting a case with left isomeric heterotaxy and a left brachiocephalic artery arising from a left ductus arteriosus in connection with the left pulmonary artery. (heraldopenaccess.us)