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At least 235 records · Page 13Linked to original sources
PubMed1999

Expression of the Mf1 gene in developing mouse hearts: implication in the development of human congenital heart defects.

The transcription factor FKHL7 gene has recently been associated with the anterior segment dysgenesis disorder of the eye known as Axenfeld-Rieger anomaly (ARA). A growing body of evidence indicates that mutations in FKHL7 cause not only defects in the anterior segment of the eye but defects in the heart valves and septa as well. In order to evaluate its contribution to normal heart septation and valve formation, expression of the mouse homologue Mf1 in embryonic hearts was analyzed by in situ hybridization. A weak but significant level of Mf1 expression could be detected in the endocardium of mouse embryos as early as day 8.5 post-conception (p.c.). Mf1 expression was undetectable in the hearts of day 9.5 p.c. embryos, but by day 10.5-11 p.c., Mf1 transcripts could be found again in the endocardium of both the atrium and ventricle and a relatively strong signal was observed in the dorsal portion of the septum primum, in what appeared to be the spinal vestibule. At day 13 p.c. when aortic and pulmonary trunks are separated, relatively more Mf1 transcripts were detected in the leaflets of aortic, pulmonary, and venous valves, the ventral portion of the septum primum, as well as in the single layer of cells on the edges of the atrioventricular cushion tissues. Surprisingly, there was no signal detected in the developing interventricular septum. At day 15 p.c., overall Mf1 signals were greatly decreased. However, significant levels of expression could still be observed in the atrial septum, the tricuspid valve, the mitral valve, and in the venous valve but not in the interventricular septum. The temporal and spatial expression patterns of the Mf1 gene in developing mouse hearts suggest that Mf1 may play a critical role in the formation of valves and septa with the exception of the interventricular septum. This is further supported by our studies showing that mutations in the FKHL7 gene were associated with defects in the anterior segment of the eye as well as atrial septal defects or mitral valve defects. Dev Dyn 1999;216:16-27.

Animals↗
PubMed1983

Cardiovascular responses to PEEP and CPAP following repair of complicated congenital heart defects.

Fourteen infants and children ranging in age from 7 months to 8 years were studied in a hemodynamically stable condition following repair of various heart defects. Changes in cardiac index, stroke index, heart rate, systemic vascular resistance, mean arterial pressure, and central venous pressure were evaluated at levels of 0, 5, and 10 cm H2O using positive end-expiratory pressure (PEEP) in 14 patients and continuous positive airway pressure (CPAP) in 3 patients. No significant changes were found in any of the measurements taken at the different levels.

Blood Pressure↗
PubMedDate not supplied

Ruptured aneurysm of the sinus of Valsalva into the right atrium. An uncommon congenital heart defect.

Rupture of an aneurysm of the sinus of Valsalva is an uncommon heart defect. A continuous murmur may be the first clinical sign of this rupture. Additional imaging techniques, and in the first place echocardiography, can be used to confirm the diagnosis. We present a case where, at the age 53 years, the diagnosis was made of a ruptured sinus of Valsalva into the right atrium, with a typical windsock image on echocardiogram. Although several cases of a ruptured sinus of Valsalva are reported in the literature, it is still an underdiagnosed condition because it is frequently missed during physical examination.

Aortic Aneurysm↗
PubMedDate not supplied

[The regional contractile function of the normal right ventricle and in congenital heart defects].

The authors have developed a method for studying the regional contractile function of the right ventricle (RV) performed by cineangiocardiogram in two projections. It allowed an analysis of the regional contractile function of the RV to be performed in patients with hypervolemic exercise of RV and hyperkinetic load to RV, and to compare them with normal conditions. It was established that the regional contractile function of the input and output parts are not equivalent and that there are reliable differences in changes of contractility of the output part of the RV in patients with stenosis of the pulmonary artery as compared with normal state.

Cineangiography↗
PubMedDate not supplied

[Residual postoperative murmurs in children with congenital heart defects].

Fifty-four children without postoperative complications were admitted to the Department of Pediatrics, University Hospital Rebro, following a complete surgical correction of ventricular septal defect (VSD), atrial septal defect - secundum type (ASD II) and patent ductus arteriosus (PDA) in the period from June, 1989, to February, 1991. Twenty-two patients were treated surgically for VSD, 17 for ASD II and 15 for PDA. There were 23 male and 31 female children. In the early postoperative period, 57% of all the examinees had a murmur with the innocent or functional characteristics. The murmur was significantly more frequent among children with VSD than among children of other groups (ASD II, PDA). Sex, age and hematocrit did not influence this difference. There were 8 anemic children (3 with operated VSD, 4 with ASD II, and 1 with PDA). From this, it may be concluded that anemia also did not cause this difference. However, anemic children represented 50% of the examinees with a murmur among the operated for ASD II and 33% among those treated surgically for PDA. When these anemic children were eliminated, the others operated on for ASD II and PDA probably had the innocent murmurs, since the proportion of examinees with a murmur fits into the incidence of innocent murmurs in the population. The children operated for VSD have had a greater proportion of examinees with a murmur, and most likely, some of these murmurs do not belong to a group of innocent or functional murmurs. Their cause is unknown, as yet (probably, small organic lesion).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗