Congenital diaphragmatic hernia increases the sensitivity of pulmonary arteries to nitric oxide

Bressan, Alecsander F.; Maia, Victoria Oliveira; Rodrigues, Beatriz de Souza; Bertozi, Giuliana; Batah, Sabrina Setembre; Fabro, Alexandre Todorovic; Pelizzo, Glória; Pierucci, Ugo Maria; Tostes, Rita C.; Sbragia, Lourenço; Carneiro, Fernando S.

doi:10.1016/j.phrs.2023.106749

Cited by 1 publication

(1 citation statement)

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“…In these cases, despite the escalation of vasoactive medication and targeted vasodilators for PH, which specifically address the functionality of smooth muscle cells in the pulmonary arteries, the empirical substantiation delineating their efficacy remains regrettably circumscribed. 16 Nevertheless, our meticulous microscopic observations showed no significant decline in the number of muscular arteries, corroborating the insights presented by Stainsby et al 17 They discerned a proportional relationship between the reduction in arterial growth and a corresponding decrease in lung size. The absence of response to vasodilators is attributed to this physiological deficit, giving rise to persistently heightened resistance following birth in infants with CDH.…”

Section: Diaphragm Muscle and Other Organs' Findingssupporting

confidence: 91%

Pathological findings in congenital diaphragmatic hernia on necropsy studies: A single‐center case series

Meng,

Zou,

Wang

et al. 2023

Pediatric Pulmonology

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IntroductionCongenital diaphragmatic hernia (CDH) is associated with high mortality rates and significant pulmonary morbidities. The objective of this study was to delineate the histopathological features observed in necropsies of CDH patients and correlate these with their clinical manifestations.MethodsWe retrospectively reviewed the postmortem findings and corresponding clinical characteristics in eight CDH cases from 2017 to July 2022.ResultsThe median survival time was 46 (8–624) hours. Autopsy reports showed that diffuse alveolar damage (congestion and hemorrhage) and hyaline membrane formation were the primary pathological lung changes observed. Notably, despite significant reduction in lung volume, the lung development appeared normal in 50% of the cases, while lobulated deformities were present in three (37.5%) cases. All patients displayed a large patent ductus arteriosus (PDA) and a patent foramen ovale, resulting in increased right ventricle (RV) volume, and myocardial fibers appeared slightly congested and swollen. The pulmonary vessels indicated thickening of the arterial media and adventitia. Lung hypoplasia and diffuse lung damage resulted in impaired gas exchange, while PDA and pulmonary hypertension led to RV failure, subsequent organ dysfunction and ultimately death.ConclusionsPatients with CDH typically succumb to cardiopulmonary failure, a condition driven by a complex interplay of pathophysiological factors. This complexity accounts for the unpredictable response to currently available vasodilators and ventilation therapies.

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Section: Diaphragm Muscle and Other Organs' Findingssupporting

confidence: 91%