As comunicações interventriculares (CIV), na forma isolada, são, de longe, a comunicação interventricular com atresia pulmonar (CIV/AP), a transposição das . La atresia pulmonar es una enfermedad del corazón presente ya en el momento del nacimiento, por lo que se incluye dentro del grupo de enfermedades. Atresia Pulmonar con Septo Interventricular cerrado. Doble Emergencia del pulmonar a la prueba de oxígeno: Cierre de CIV o Si RPT > 7 uds y posible.

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Recognizing a common genetic syndrome: Frequency of 22q11 deletions in patients with conotruncal defects. All the patients in this subgroup achieved DT, independently of the PAI, demonstrating that there was no correlation between this characteristic and the treatment. The year mortality was Three patients presented with agenesis of the left pulmonary artery, two of the right pulmonary artery, two fistulae of the left coronary branch to the pulmonary branch and one had hypoplastic CPA.

Atresia pulmonar | American Heart Association

Of those with confluent CPA, four presented with stenosis pulmoanr the left pulmonary artery and four in the CPA bilaterally. Among the Barbero-Marcial classification groups, it was possible to identify nine subgroups of patients: Similarly, Reddy et al.

The 22q11 ;ulmonar syndrome, also called DiGeorge syndrome, velocardiofacial syndrome and CATCH22, stands out as one of the main known causes of congenital heart defects.

Chromosome 22 microdeletion by F. This fact might be explained by the small number of patients in group C, as these are more seriously sick patients who were submitted to more difficult surgical therapies.

Thus, with basis in the analysis of cine angiocardiograms of patients suffering from PA with VSD, the present study aims at identifying within the groups proposed by the Barbero-Marcial classification, subgroups with pulmonary vascular blood supplies that present similar morphological characteristics, to assess their CPA and MAPCA, to attempt to establish implications involved in surgical treatment.


Results Of the total of 63 patients, 15 Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: There was no association among the numbers of MAPCA, the presence of stenosis and the treatment stages.

PT was defined for patients who were submitted to interventions without closing the VSD and with future perspectives of conclusion or not of the treatment. Am J Med Genet ;A: Analysis of group B The cardiac cineangiographic studies of all the patients of this group were submitted to morphometric analysis as can be seen in Table 2.

Chromosome 22q11 deletions in patients with conotruncal heart defects. The evolution of diagnostic trends in congenital heart disease: Clin Chim Acta ; Cardiac surgery of the neonate and infant. The mortality of disease in this subgroup of patients was The origin, course and distribution of the MAPCA for the pulmonary segments should also be known, obtaining a complete and detailed map of the pulmonary vascular blood supply, fundamental in the adequate planning of a surgical strategy [5].

Incidence and significance of 22q Chromosomal abnormalities among children born with conotruncal cardiac defects.

The morphological aspects of the CPA had a greater influence in the surgical conduct. Schematic representation of the B1 subgroup with central pulmonary arteries supplying the left superior and right inferior lobes. Clinical features of 78 adults pjlmonar 22q11 Deletion Syndrome. Typical phenotypic spectrum of velocardiofacial syndrome occurs independently of deletion size in chromosome 22q The incidence of a deletion in chromosome 22Q11 in sporadic and familial conotruncal heart disease.

New York, Churchill- Livingstone, ; The mortality presented larger correlation with the morphologic characteristics that with the morphometric. Arch Dis Child ; All the other lobes were irrigated by the major aortopulmonary collateral arteries.


Indian J Pediatr ; The parametric student-t test and the non-parametric Kruskal-Wallis and Wilcoxon tests were used. Services on Demand Journal. The lack of confluence of the CPA was not a relevant factor for DT and survival, however it should be noted that this only included two patients and both of whom presented with mean PAI atrseia The profile and outcome of patients admitted to a pediatric intensive care unit.

Frequency of a 22q11 deletion in patients with conotruncal cardiac malformations: Morphometric characteristics of the pulmonary arteries, surgical procedures atrssia stages of treatment in relation to the age and the body surface area in Group A. The other lobes were irrigated by major aortopulmonary collateral arteries.

Atresia pulmonar

Of these, four also presented with stenosis of the right pulmonary artery and only one achieved DT, showing that the presence of stenosis in both the CPA is an important factor in relation to surgical correction [12].

Am J Cardiol ; Semin Thorac Cardiovasc Surg ; 2: Rosa I ; Paulo Ricardo G. Among the groups A, B e C cov possible to identifiy nine subgroups, the morphologic and morphometric characteristics allowed to suggest the surgical treatment in the patients of the group A had larger chance of TD, the group B of TP and the group C of TPD. Haworth SG, Macartney FJ – Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Marino B, Digilio MC. Cardiac defects and results of cardiac surgery in 22q