[ca] Introducció La herni diafragmàtica congènita (HDC) afecta a 1 de cada 2500 naixements vius, amb una mortalitat del 50%. La principal causa de mort és la hipertensió pulmonar i el subdesenvolupament pulmonar. La severitat de la HDC pot predir-se per diversos indicadors, incloent la relació entre l'àrea pulmonar ipsilateral i la circumferència cefàlica, el volum pulmonar total, i la posició de l'estómac i el fetge. La hipertensió pulmonar, definida quan la pressió pulmonar excedeix 2/3 de la sistèmica, és crucial, sent diagnòsticable per ecocardiografia neonatal primerenca a causa de la limitada circulació fetal cap als pulmons. Aquesta condició complica el tractament prenatal de l'HDC. L'organització anormal de la xarxa vascular pulmonar s'evidencia post mortem. Hipòtesi Proposem que un índex de ramificació vascular pulmonar prenatal correlaciona amb la severitat de la hipertensió pulmonar postnatal, sent un predictor clau de supervivència. Materials i Mètodes Es van utilitzar fetus de rates Sprague Dawley sans i tractats amb nitrofè. Mitjançant tomografia computada micro i tècniques d'intel·ligència artificial, es van reconstruir en 3D els arbres arterials pulmonars. Aquest marc va permetre comparar les estructures vasculars pulmonars de mostres d'HDC i controls sans, obtenint característiques morfològiques per a la caracterització de l'HDC. Resultats De 70 fetus examinats, 40 van demostrar la viabilitat de l'ús de Lugol per a la imatgeria detallada de la vasculatura pulmonar. La segmentació va assolir un 86% de precisió, 82% de sensibilitat i 84% de puntuació F1. La caracterització de l'HDC va revelar un nombre significativament menor de segments vasculars en els fetus afectats, especialment en el primer ordre de ramificació, desviant-se de la Llei de Horton, la qual cosa indica una disminució en la ramificació arterial. Conclusió La reducció en la ramificació de les artèries, a més de la coneguda disminució en la ramificació de les vies aèries en pulmons hipoplàsics, va ser notable en fetus amb HDC. Les tècniques d'imatgeria radiològica van permetre definir l'arbre vascular pulmonar prenatal, estimar el grau de hipertensió pulmonar i, potencialment, facilitar la determinació prenatal de la severitat de la malaltia per orientar teràpies prenatals.

[en] Introduction Congenital diaphragmatic hernia (CDH) is seen at 1 in every 2500 live births. Its overall mortality is still as high as 50%. Pulmonary hypertension and underdevelopment are the primary causes of death in CDH. Per our current knowledge, the severity of the CDH could be predicted by the ratio of ipsilateral lung area to head circumference, total lung volume, left/right ventricle ratio, modified McGoon index, location of the stomach with the existence of polyhydramnios, and the location of the liver. When the blood pressure in the pulmonary system exceeds 2/3 of the pressure in the systemic circulation, it is said to be pulmonary hypertension. Although the main pathology emerged in utero, this could only be documented by echocardiography in the early neonatal period. This is mostly because the blood flow into the lungs is limited in utero for the sake of fetal blood circulation. This is a significant barrier for treating CDH patients in-utero. Nonetheless, the aberrant organization of the pulmonary vascular network in pulmonary hypertension can only be shown in postmortem subjects by histopathological analyses. Hypothesis Since pulmonary hypertension is one of the predictors of the survival of these patients, this proposal seeks to test the general hypothesis that a prenatal pulmonary vascular branching index correlates with the severity of postnatal pulmonary hypertension. Materials and Methods Sprague Dawley rat fetuses that were both healthy and nitrofen-induced were used. The fetuses underwent micro-computed tomography scanning. Using artificial intelligence techniques, the pulmonary artery tree was 3D reconstructed. Moreover, a framework is developed to generate 3D pulmonary vascular tree structures by using Computed Tomography (CT) scans. Then, the developed framework is used to compare pulmonary vascular trees of CDH samples and healthy controls to determine the characterization of the CDH disease. Finally, by using pulmonary vascular trees, morphological features are obtained, and characterization of the disease is achieved. Results There were 70 fetuses examined throughout the study, 40 of which were used in the first part to demonstrate the feasibility of the Lugol application while the rest were used in the second part. Lugol diffused uniformly throughout the capillaries in the pups with partially removed skin, allowing fine-grained imaging of the lung vasculature. The segmentation part of this project had 86% precision, 82% sensitivity, and 84% F1 score. For the characterization of the CDH, the required features are obtained from the segmented output, and they were prioritized by their impact on the classification. The number of segments in CDH was significantly lower than the control group on the left (U=2.5, p=0.004) and right (U=0, p=0.001) sides for order 1(O1), whereas there was a significant difference only on the right side for O2 and O3. The pooled element numbers in the control group obeyed Horton's Law (R2=0.996 left and R2=0.811 right lungs), while the CDH group broke it. Connectivity matrices showed that the average number of elements of O1 springing from elements of O1 on the left side and the number of elements of O1 springing from elements of O3 on the right side were significantly lower in CDH samples. Conclusion Lung vasculature in CDH is of particular interest for pulmonary development and is the main underlying factor that determines the disease severity. We observed that, besides the known reduced airway branching of the hypoplastic lungs, arterial branching was decreased in fetuses with CDH as well. Herein, we have demonstrated that the radiological imaging techniques were able to define the prenatal lung vascular tree, which could potentially estimate the degree of pulmonary hypertension and enable physicians to determine the severity of the disease prenatally and promote prenatal therapy.