PedAM
Pediatric Disease Annotations & Medicines
| Disease | heterotaxy |
| Phenotype | C0018799|heart disease |
| Sentences | 9 |
| PubMedID- 26162269 | Orthotropic heart transplantation for adult congenital heart disease: a case with heterotaxy and dextrocardia. |
| PubMedID- 26557043 | Asplenia has been shown to be one of the postoperative risk factors influencing mortality when operated due to congenital heart disease, while with heterotaxy, morbidity and mortality increases [11]. |
| PubMedID- 25298693 | Management of complex congenital heart disease in patients with heterotaxy syndrome (hs) has steadily improved. |
| PubMedID- 22387996 | Ineffective airway mucociliary clearance usually manifests within the first year of life with recurrent infections, sinusitis, rhinitis and otitis media, causing a chronic respiratory condition, and progressing to permanent lung damage (bronchiectasis)1,2. half of pcd patients have laterality defects reflecting randomized left-right organogenesis due to embryonic nodal cilia dysfunction, usually situs inversus totalis (kartagener syndrome) with rarer incidence of complex heterotaxy defects often associated with congenital heart disease3,4, 5. subfertility arises from dysmotile sperm flagella and oviduct cilia, and hydrocephalus occasionally arises 6 from reduced cerebrospinal fluid flow due to ependymal cilia dysmotility7,8. |
| PubMedID- 21768105 | In addition, galnac-t14 has been suggested as a negative regulator of apoptotic signaling (17), galnac-t16 as a regulator of tgfβ signaling (18), and galnac-t11 as a candidate gene for congenital heart disease with heterotaxy (19). |
| PubMedID- 23691403 | Out of the 109 cases of visceral heterotaxy with congenital heart disease seen at autopsy, 53% had asplenia, 42% had polysplenia, and 5% had a single, normal-sized spleen that was abnormally located in the upper right side of the abdomen [7]. |
| PubMedID- 24192704 | Defects within the nodal cilia pathways cause left-right laterality defects, such as situs inversus totalis, situs ambiguus, and heterotaxy associated with congenital heart disease, asplenia, and polysplenia (26, 27). |
| PubMedID- 23804943 | We review the physiology of heterotaxy syndrome with congenital heart disease and justify our approach in the context of a review of previous surgical strategies used in this patient population. |
| PubMedID- 20971273 | Medium-term outcomes of kawashima and completion fontan palliation in single-ventricle heart disease with heterotaxy and interrupted inferior vena cava. |
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