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At least 37 records · Page 2Linked to original sources
PubMed1995

22q11 deletions in isolated and syndromic patients with tetralogy of Fallot.

Tetralogy of Fallot (TF) is a congenital conotruncal heart defect commonly found in DiGeorge (DGS) and velo-cardio-facial (VCFS) syndromes. The deletion of chromosome 22q11 (del22q11) is a well established cause of DGS and VCFS, and it has been demonstrated also in sporadic or familial cases of TF. In order to investigate the prevalence of del22q11 in patients with TF, we analyzed the DNA of 137 consecutive patients with syndromic and isolated TF, using the HD7k probe, which detects hemizygosity for the D22S134 locus. Del22q11 has been detected in 11/26 (42%) syndromic patients. Evidence for hemizygosity was obtained in all patients with DGS and in 8/15 patients with VCFS. None of the 107 patients with isolated TF had del22q11. Our experience suggests that children with TF and del22q11 always present major or minor extracardiac anomalies. These features, including subtle facial dysmorphisms, should be checked routinely in patients with TF and other conotruncal heart defects.

Adolescent↗
PubMedDate not supplied

[Myocardial fatty dystrophy in patients with Fallot tetralogy].

Fatty degeneration (FD) was studied histochemically and ultrastructurally in surgical biopsies from 147 patients aged 22 days to 40 years with Fallot's tetralogy. FD was most pronounced in patients aged 3 to 6 years when the main part of cardiomyocytes completed the program of ontogenetical growth and started growing as hypertrophic mature cells. An increase of FD is considered as manifestation of an initial stage of hypertrophy of mature cardiomyocytes.

Adipose Tissue↗
PubMed2004

[Double disposition of the major vessels from the right ventricle of the Fallot tetralogy type].

Forty-six patients with double disposition of the major vessels from the right ventricle in combination with stenosis of the pulmonary artery, in sub-aortal and bi-committed defects of the interventricular septum have undergone biventricular intraventricular correction. The most specific and accurate anatomic characteristics were: 1) position of the major vessels (particularly side-to-side); 2) location of two and a part of the third aortal sinuses above the right ventricle; 3) bilateral cone; 4) absence of aorto-mitral contact; 5) location of valves of aorta and pulmonary artery on the same level. One (2,2%) patient died during surgery due to progressed cardiac insufficiency. One (2,5%) patient died 2 years after radical correction of the defect due to progressive dysfunction of the right ventricle.

Adolescent↗
PubMed1978

[Surgical correction of tetralogy of Fallot with an iatrogenic obstruction on 1 branch of the pulmonary artery].

The au;hors report 12 cases of Fallot' tetralogy with stenosis or obstruction of one of the two branches of the pulmonary artery following palliative anterior anastomosis. The complications of correction by open heart surgery include the frequent incidence of pulmonary hypertension, which was responsible for 4 deaths. No satisfactory explanation could be found for these poor results. It is desirable to avoid the creation of asymetry in pulmonary blood supply in Fallot's tetralogy and, if such should be found, suggestions are made for its correction by open heart surgery in order to improve the prognosis.

Child↗