Hypoplastic Left Heart Syndrome: About a Postnatal Death

Figures

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  Figure 1. (a) Cyanosis of the buccal rim. Anterior face (b) and posterior face (c) of the heart. The red arrow points to a small and virtual auricle-like left atrial cavity (b) which is better highlighted by the introduction of a probe (d); the black arrow indicates the emergence cone of the predominant pulmonary trunk which is predominant (b). Evidence of hypoplastic aorta after rotation of the heart towards the posterior face (e). Prevalence of the right ventricle (f): the red arrow indicates a small residual left ventricular cavity with a small residual sept; the red circle is the only identified right valve; the black circle indicates the emergence of the right and left pulmonary vessels above the emergence of the common trunk. Dissection of the right anterior ventricular wall and pulmonary trunk following the outflow route. (Images from Mansueto’s forensic archive). The missing intrauterin diagnosis of fetal defects could be the cause of unexpected newborn deaths. HLHS is a syndrome characterized by multiple congenital cardiac structural abnormalities, which was first described by Lev in 1952 [1]. Currently, the mortality rate from HLHS is approximately 2–3% of all congenital heart…

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  Figure 2. All the organs were macroscopically observed, and samples were taken for each. After formalin fixation and paraffin embedding, sections stained with hematoxylin and eosin were prepared for histological diagnosis [7]. Histology showed multiorgan congestion and lungs in the alveolar-saccular development phase with diffuse congestion with focal aspiration signs (a–f). In (a): congestion and erythrocyte extravasation in hepatic sinusoids (black arrow) (H&E×10). In (b): congestion and erythrocyte extravasation in the bowel wall (black arrow) H&E×20). In (c): spleen congestion (black arrow) (H&E×4). In (d): kidney congestion (black arrow) (H&E×20). In (e): adrenal gland congestion (black arrow) (H&E×10). In (f): Lungs diffuse congestion (black arrow) with focal intra-alveolar eosinophilic material due to aspiration (blue arrow) (H&E×63 magnification). (Images from Mansueto’s forensic archive). HLHS is a rare type of congenital heart syndrome. Due to the great number of elective terminations of pregnancy and the spontaneous abortion of affected fetuses, the reported overall incidence is likely underestimated. HLHS is not related to maternal age, ethnicity, or geographical factors. Seven…