Complex venous malformation in an elderly female: A case report.

Area of Science:

• Cardiology
• Vascular Malformations
• Medical Imaging

Background:

• Heterotaxy syndrome is a rare congenital disorder characterized by abnormal arrangement of visceral organs.
• Venous anomalies, particularly involving the superior and inferior vena cava, can occur in heterotaxy syndrome.
• Congenital cardiovascular abnormalities are common in heterotaxy syndrome, but complex venous malformations without other cardiac defects are less frequently reported in adults.

Observation:

• A 76-year-old female presented with bilateral leg edema, revealing an enlarged coronary sinus (40 mm) on echocardiography.
• Contrast-enhanced computed tomography identified absence of the left innominate vein, a persistent left superior vena cava, and absence of the hepatic segment of the inferior vena cava.
• The interrupted inferior vena cava connected to the hemiazygos vein, which also received the persistent left superior vena cava, leading to reflux into the coronary sinus.

Findings:

• The patient was diagnosed with heterotaxy syndrome with polysplenia, complicated by right cardiac failure due to atrioventricular valve regurgitation and chronic atrial fibrillation.
• Absence of the hepatic segment of the inferior vena cava was noted as a pathognomonic malformation in left isomerism.
• Edema resolved with oral medication, despite the complex venous anomalies.

Implications:

• This case highlights the potential for complex congenital venous malformations to remain undiagnosed until late in life, presenting with symptoms like edema.
• The findings underscore the importance of advanced imaging techniques in diagnosing rare vascular anomalies, even in elderly patients.
• Understanding these venous anomalies is crucial for managing cardiac complications and surgical planning in patients with heterotaxy syndrome.