Presentation
Known for sickle cell disease, presents with hypertension and difficulty in breathing, thus placed on oxygen support.
Patient Data
Cardiac configuration and size appear normal. The pulmonary vasculature and aeration are within normal. No mediastinal or lung lesion is seen. Both hemidiaphragm and lateral costophrenic sulci are well-defined. The tracheobronchial tree is unremarkable. Thoracic bony cage and spine are normal.
There is spontaneous echo contrast (SEC) seen in the left ventricle. Good biventricular systolic function.
Haematology lab. results.
Case Discussion
Sickle cell disease is an inherited genetic illness characterised by abnormalities in haemoglobin structure and function in the blood. This raises the risk of blood vessel blockages and disrupted blood flow, both of which can lead to major problems. When the vascular system lacks oxygen, sickle haemoglobin becomes more insoluble, increasing polymer production in the blood, its total viscosity and density affecting blood flow and endothelial vessel wall integrity. Erythrocyte adhesion increases, followed by the formation of heterocellular aggregates, as observed in the apical four-chamber of the aforementioned ultrasound images as spontaneous echocardiographic contrast.
This induces minor artery blockage and hypoxia leading to conditions such as stroke, pulmonary embolism, pulmonary hypertension, occlusion of the peripheral retinal vasculature, and priapism among others.
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