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Results for “Heart Septal Defects”

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PubMed1979

The cause of the hemodynamic disturbances in Ebstein's anomaly based on observations at operation.

Ten adult patients with Ebstein's anomaly had open-heart operation for interruption of a Kent bundle. The 4 patients in Group 1 had arrhythmias. One patient had a patent foramen ovale, which was closed. In 3 patients the Kent pathway was identified and interrupted, but in the other only the pathway's anterograde function was interrupted, leaving the patient subject to supraventricular tachycardia (SVT). The 3 patients in Group 2 had mild to moderate cyanosis and dyspnea during SVT as well as mild impairment during strenuous physical activity. Only interruption of their Kent bundles and closure of the patent foramen ovale were done. The 3 patients in Group 3 had dyspnea and cyanosis on exertion, and 1 had overt right heart failure. In each patient, obstruction between the atrialized right ventricle (RV) and functioning RV by the displaced tricuspid valve (TV) was relieved by valve excision and replacement. Interruption of the Kent bundle was successful in 2 of the 3 patients. Operation for Ebstein's anomaly is indicated when any of the following conditions are present: arrhythmias due to Kent bundles that are refractory to medical management; a defect in the atrial septum that must be closed because of a history of cyanosis or paradoxical emboli, or an arrhythmia that must be corrected by right atriotomy; and obstruction between the atrialized and functioning RV or a small functioning RV, both of which can be corrected by TV replacement.

Adolescent↗
PubMed2000

Cardiac septal and valvular dysmorphogenesis in mice heterozygous for mutations in the homeobox gene Nkx2-5.

Heterozygous mutations in the cardiac homeobox gene, NKX2-5, underlie familial cases of atrial septal defect (ASD) with severe atrioventricular conduction block. In this study, mice heterozygous for Nkx2-5-null alleles were assessed for analogous defects. Although ASD occurred only rarely, atrial septal dysmorphogenesis was evident as increased frequencies of patent foramen ovale and septal aneurysm, and decreased length of the septum primum flap valve. These parameters were compounded by genetic background effects, and in the 129/Sv strain, septal dysmorphogenesis bordered on ASD in 17% of Nkx2-5 heterozygotes. In a proportion of neonatal heterozygotes, as well as in adults with ASD, we found that the size of the foramen ovale was significantly enlarged and altered in shape, potentially exposing the normally thin septum primum to excessive hemodynamic forces. Therefore, defective morphogenesis of the septum secundum may be one contributing factor in the generation of patent foramen ovale, septal aneurysm, and certain ASDs. Mild prolongation of P-R interval in females and an increased frequency of stenotic bicuspid aortic valves were also features of the Nkx2-5 heterozygous phenotype. Our data demonstrate that the complex effects of Nkx2-5 haploinsufficiency in mice are weaker but convergent with those in humans. As in the mouse, the phenotype of human NKX2-5 mutations may be modulated by interacting alleles.

Alleles↗