This study aimed to demonstrate that congenital diaphragmatic hernia (CDH) results in
vascular abnormalities that are directly associated with the severity of pulmonary
hypoplasia and hypertension. These events increase right ventricle (RV) afterload and
may adversely affect disease management and patient survival. Our objective was to
investigate cardiac function, specifically right ventricular changes, immediately
after birth and relate them to myocardial histological findings in a CDH model.
Pregnant New Zealand rabbits underwent the surgical procedure at 25 days of gestation
(n=14). CDH was created in one fetus per horn (n=16), and the other fetuses were used
as controls (n=20). At term (30 days), fetuses were removed, immediately dried and
weighed before undergoing four-parameter echocardiography. The lungs and the heart
were removed, weighed, and histologically analyzed. CDH animals had smaller total
lung weight (P<0.005), left lung weight (P<0.005), and lung-to-body ratio
(P<0.005). Echocardiography revealed a smaller left-to-right ventricle ratio
(LV/RV, P<0.005) and larger diastolic right ventricle size (DRVS, P<0.007).
Histologic analysis revealed a larger number of myocytes undergoing mitotic division
(186 vs 132, P<0.05) in CDH hearts. Immediate RV dilation of CDH
hearts is related to myocyte mitosis increase. This information may aid the design of
future strategies to address pulmonary hypertension in CDH.