Disease relevance of Dextrocardia

• Associated anomalies included atrial septal defect (n = 11), double-outlet right ventricle (n = 7), dextrocardia (n = 4), and mesocardia (n = 2) [1].
• Eighty-two patients (77%) had a ventricular septal defect, 57 (53%) had pulmonary stenosis, 35 (34%) had tricuspid insufficiency and 24 (23%) had dextrocardia [2].
• The 2 girls had similar related internal malformations, including agonadism, hypoplasia of the right pulmonary artery, hypoplasia of the right lung, isolated dextrocardia with complex vitium cordis, and diaphragmatic hernia (only sib 1) or omphalocele (only sib 2) [3].
• All three patients had atrioventricular concordance, ventriculoatrial concordance, dextrocardia, superoinferior ventricles, crisscross atrioventricular relations, underdevelopment of the right ventricle, a large ventricular septal defect, and an inverted tetralogy of Fallot type of malformation of the infundibulum and great arteries [4].
• In this article, the authors observed a ranitidine-mediated sinus bradycardia in a man with dextrocardia (situs inversus) who had acute bleeding from a duodenal ulcer [5].

High impact information on Dextrocardia

• In all three patients the crossover segment was associated with a variant of the scimitar syndrome: right lung hypoplasia with dextrocardia, aberrant systemic arterial supply to the right lower lobe, and anomalous venous drainage of the whole of the right lung, and in two patients with eventration of the right diaphragm [6].
• In addition, patients with hypertrophic cardiomyopathy, septal defects, dextrocardia, and anomalous pulmonary venous drainage have been reported anecdotally [7].
• Secondary dextrocardia (39 cases) was due to intrathoracic displacement and, when caused by diaphragmatic hernia, was associated with cardiac malformations in 31% of cases [8].
• In one patient with dextrocardia and the cardiac type of total anomalous pulmonary venous return, the abnormality was not recognized [9].
• Right abnormal pulmonary venous return into the inferior vena cava associated with abnormal fissure, dextrocardia, and systemic arterial supply of a variable degree, are the characteristics of the scimitar syndrome [10].

Chemical compound and disease context of Dextrocardia

• The patient probably had Ivemark syndrome with dextrocardia (confirmed by chest radiograph), horseshoe kidney (confirmed by 99Tcm DTPA scintigraphy), and biliary atresia (confirmed on the HIDA scan) [11].
• We have recently 'challenged' a neonate with dextrocardia, vesceral heterotaxia, presumed asplenia, and complex congenital heart disease including pulmonary atresia, with an infusion of prostaglandin E1 to increase pulmonary blood flow via his ductus arteriosus [12].

Biological context of Dextrocardia

• Fetal diagnosis of atrioventricular septal defect with dextrocardia in trisomy 18 [13].
• Additionally, we observed some remarkable phenotypes, i.e., dextrocardia with E359Xfs (c.1075delG) and cribriform type ASD with R190C, both of which are expected to be clues for further investigations [14].
• Out of the latter 2 had syndromal (Osteogenesis Imperfecta and Achondroplasia), 16 nonsyndromal and 3 sequence multiple (Pierre Robin, diaphragmatic hernia with dextrocardia and breech presentation sequence) malformations [15].

Anatomical context of Dextrocardia

• Isolated left superior vena cava to the left atrium with situs solitus and dextrocardia: extracardiac repair facilitated by juxtaposition of the atrial appendages [16].
• Successful surgical correction of transposition of the great arteries in a nine-year-old girl with dextrocardia, primum atrial septal defect, bilateral venae cavae, and azygos continuation of inferior vena cava is reported [17].
• When ventricular septal defect exists in hearts with atrioventricular discordant connection and dextrocardia, closure of the defect can most easily be accomplished by exposure through the left atrium and retracted tricuspid valve leaflets [18].
• In addition, our case had anomalies not previously described in this condition, including abnormal lung lobation with bilateral left bronchial morphology, dextrocardia, total anomalous pulmonary venous return, a left superior vena cava, and a right aortic arch [19].
• PATIENTS: 81 consecutive fetuses with a fetal dextrocardia presenting at Guy's Hospital, London, between 1983 and 2003 and at Hôpital Robert Debré, Paris, between 1988 and 2003 [20].

Gene context of Dextrocardia

• All patients had a previous Glenn shunt; five patients had dextrocardia and a midline inferior vena cava [21].
• Growth hormone deficiency, wormian bones, dextrocardia, brachycamptodactyly, and other midline defects [22].
• Systemic lupus erythematosus and dextrocardia [23].
• Familial dextrocardia, divergent strabismus and situs inversus of optic disc [24].
• Congenital diaphragmatic hernia usually presents with cyanotic attacks, dyspnoea and dextrocardia, a typical triad which is almost considered pathognomonic [25].

References