Severe diffuse hypoplasia of the aorta associated with multiple vascular abnormalities

Area of Science:

• Cardiovascular Medicine
• Vascular Surgery
• Pediatric Cardiology

Background:

• Aortic hypoplasia, a rare vascular pathology, typically manifests as severe, uncontrolled hypertension in adolescents and young adults.
• Medical management of hypertension secondary to aortic hypoplasia often requires high doses of antihypertensive drugs.
• Surgical revascularization is the treatment of choice for refractory hypertension due to aortic hypoplasia.

Observation:

• This report details a rare case of severe, diffuse hypoplasia of the aorta, extending from the aortic isthmus to the bifurcation.
• The patient presented with multiple vascular abnormalities, including an aberrant celiac trunk and superior mesenteric artery.
• Unusually, the extensive vascular malformation's only clinical manifestation was medicamentously controllable hypertension.

Findings:

• The case describes a severe and diffuse hypoplasia of the thoracic and abdominal aorta with associated aberrant arteries.
• Despite the extensive nature of the vascular anomaly, hypertension was the sole clinical manifestation.
• The hypertension, though severe, was effectively managed with medication, which is atypical for such a condition.

Implications:

• This case expands the known clinical spectrum of aortic hypoplasia, demonstrating that severe presentations can have minimal symptoms.
• It underscores the importance of considering rare vascular pathologies even when clinical manifestations appear mild.
• Further research into the genetic and developmental factors of extensive aortic hypoplasia with minimal clinical impact is warranted.