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.
The pulmonary vascular blood supply in the pulmonary atresia with ventricular septal defect and its implications in surgical treatment. Sixty three patients were classified in groups A 15B 40 and C 8 between january and june Among the groups A, B e C was 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.
The mortality presented larger correlation with the morphologic characteristics that with the morphometric. Heart septal defects, ventricular, surgery. Heart defects, congenital, surgery. Defeitos do septo interventricular, cirurgia. Pulmonary atresia PA with ventricular septal defect VSD is defined as a group of cardiopulmonary malformations of coni-truncal origin, in which there is an interruption in the continuity of the lumen and absence of blood flow between the ventricles and the central pulmonary arteries CPA.
In a biventricular heart this presents with an orifice in the interventricular septum .
Commonly this is seen with a microdeletion of the long arm of chromosome 22 q The fundamental key for surgical treatment is knowledge of the anomalies of pulmonary vascular blood supply. Utilizing cardiac cineangiographic studies it is possible to adequately identify the presence, size, existence of confluence and stenosis between the CPA. 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 .
A, B and C figure 1. In group A all the pulmonary segments are supplied by CPA and the surgical objective consists of assuring the presence of the CPA, with a size, distribution and pulmonary resistance that permits a complete correction. InNakata et al. Similarly, Reddy et al. 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.
The patients were divided into groups according to the Barbero-Marcial classification . The median age at the first cardiac cineangiographic study was 1. The cineangiographic study was considered complete when it included: The system was calibrated based on the diameter of the distal portion of the catheter. The images selected for measurement were in the posteroanterior position and in ventricular systole. The CPA were measured immediately proximal to the root of the first lobar branch  and the MAPCA was measured distally to the point in which ideally the surgical unifocalization would be possible .
With the obtained measurements, the areas of the blood vessels were calculated. Knowing the weight and height of the patients at the moment of the examination, the body surface was calculated utilizing Mosteller’s formula . 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.
These patients were considered as being within the treatment process. Of the total of 63 patients, 15 The cardiac cineangiographic studies of all the patients of this group pulmlnar submitted to morphometric analysis as can be seen in Table 1.
Morphometric characteristics of the pulmonary arteries, surgical procedures and stages of treatment in relation to the age and the body surface area in Group A. Two subgroups were identified: A1 with 5 patients In all, the CPA were artesia and did not demonstrate stenosis. Within subgroup A2, in eight patients the CPA were confluent, however presented with stenosis.
In two, the CPA were not confluent. Of those with confluent CPA, four presented with stenosis in the left pulmonary artery and four in the CPA bilaterally. Thus, the A1 subgroup was schematically represented by the patients who presented with normal-sized or hypoplastic confluent CPA without stenosis. The A2 subgroup, by patients who presented with non-confluent CPA or with stenosis figure 2.
Notice that the proportion of patients cib subgroup A1 who underwent DT was greater than those from subgroup A2. There is no indication of a relationship between the number of procedures and the treatment stage for the patients in group A.
In subgroup A2 there was a greater number of patients who required two procedures than in subgroup A1. The cardiac cineangiographic studies of all the patients of this group were submitted to morphometric analysis as can be seen in Table 2.
Morphometric characteristics of the pulmonary arteries and major aortopulmonary collateral arteries surgical procedures and treatment stages in relation to uplmonar age and the body surface area in Group B. Five subgroups were identified: In group B1, all the patients presented with CPA supplying the segments of the left pukmonar and lower right lobes. Schematic representation of the B1 subgroup with central pulmonary arteries supplying the left superior and right inferior lobes.
The other lobes were irrigated by major aortopulmonary collateral arteries. The subgroup B2 with central pulmonary arteries supplying the upper right and left lower lobes. All the other lobes were irrigated by the major aortopulmonary collateral arteries. In subgroup B3 with central pulmonary arteries supplying the left and right lower lobes A or central pulmonary arteries supplying all the lobes of the left lung and the right upper lobe B.
The other lobes being supplied by major aortopulmonary collateral arteries A, B and C. In subgroup B4 with central pulmonary arteries supplying the left and right upper lobes A. The other lobes being supplied by major aortopulmonary collateral arteries A and B. In subgroup B5 with central pulmonary arteries and major aortopulmonary collateral arteries supplying lobes without possibility of exact definition.
In subgroup B2, all the patients presented with CPA supplying the segments of the upper right and left lower lobes. In subgroup B3, all the patients presented with CPA supplying the segments of the left and right lower lobes or supplying the segments of one of the lower lobes and the majority of the lobes of the contralateral lung figure 3. In subgroup B4, all the patients presented with CPA supplying the segments of the left and right upper lobes or supplying the segments of one of the upper lobes and the majority of the segments of the lobes of the contralateral lung figure 3.
In subgroup B5, the patients presented with a great diversity in the distribution of the CPA and MAPCA, with great difficulty to define the blood supply of the pulmonary segments.
Three patients presented with agenesis of the left pulmonary artery, two of the right pulmonary artery, two fistulae of the left pulmonqr branch to the pulmonary branch and one had hypoplastic CPA. Thus, this subgroup consisted of a miscellaneous group of patients of group B figure 3. There were more patients in subgroup B2 that received DT than in the other subgroups pulmonzr group B, however without statistical significance.
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The only patients who underwent DPT were from subgroup B1. No patient who underwent three procedures achieved DT. The majority of the patients with Cif were submitted to two procedures. The presence of stenosis did not indicate any relationship with the stage of treatment of the patients.
The majority of the patients of subgroup B5 underwent only one procedure. The number of patients who underwent one, two or three procedures in the B1 and B3 subgroups is very similar. The same can be said in respect to the B2 and B4 subgroups. The greatest proportion of deaths was seen in the B1, B2 and B4 subgroups. In all of group B there were 7 The cardiac cineangiographic studies of all the patients of this group were submitted to morphometric analysis as can be seen in Table 3.
Morphometric characteristics of the major aortopulmonary collateral arteries, surgical procedures and treatment stages in relation to the age and the body surface area in Group C. C1 with 5 Thus subgroup C1 was schematically represented by patients who had a greater number of atrwsia or thick MAPCA and predominantly without stenosis and subgroup C2 was schematically represented viv patients who presented with a greater number of medium or thin MAPCA and predominantly with local or segmental stenosis figure 4.
There was no association among the numbers of MAPCA, the presence of stenosis and the treatment stages. Similarly, there was no statistical difference between group C1 and C2 in relation to the procedures performed. In group C this mortality represented The parametric student-t test and the non-parametric Kruskal-Wallis and Wilcoxon tests were used. Correlation between the A, B and C groups, atreia indices and treatment stages. There was no evidence of statistical differences among the indexes: The indices of the patients who died were lower that those who survived, however, the differences were not statistically significant.
The total mortality rate was This data is compatible with data found by Nakata et al.
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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 morphological aspects of the CPA had a greater influence in the surgical conduct.
In the A2 subgroup, eight patients presented with confluent CPA all of whom suffered stenosis of the left pulmonary artery near to the arterial canal.
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 . The seriousness of this can pulmonr seen by the greater number of procedures and the smaller number of patients who achieve DT when compared with the A1 subgroup.
The lack of confluence of the CPA was not a pumlonar factor for DT and survival, however it should be noted that this only included two patients and both of whom presented with mean PAI The morphological characteristics were more important and significant for the choice of treatment.
In the B5 subgroup, only one patient achieved DT, with a single procedure.
The other achieved PT, independently of their indices, showing that the morphologic characteristics are more important than atresiz morphometric aspects in this subgroup. The mortality of disease in this subgroup of patients was In this study, a greater mortality rate was registered in group C than in group B, different to the result in our study. 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.
In conclusion, adequate phlmonar knowledge of this severe congenital heart disease is more important than morphometric measurements which can be obtained, as diverse factors should be considered for guidance in the surgical treatment and evaluation of risk of mortality. Among the Barbero-Marcial classification groups, it was possible to identify nine subgroups of patients: The morphological and morphometric characteristics allow suggestions for the surgical therapy, as the patients from group A have a greater chance of definitive treatment, those of group B of palliative treatment and those of group C of definitive palliative treatment.
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