Cor biloculare

Cor biloculare is a rare congenital heart defect characterized by the presence of a heart with only two chambers, instead of the normal four. This condition is also known as a "bilocular heart" or "univentricular heart." In individuals with cor biloculare, the typical separation between the two atria and the two ventricles does not occur, leading to significant physiological challenges and health implications.

Etiology

The exact cause of cor biloculare is not fully understood, but it is believed to involve a combination of genetic and environmental factors. During the early stages of fetal development, the heart initially forms as a single tube, which then divides into the four chambers seen in a healthy heart. In cor biloculare, this division process is incomplete, resulting in a heart that has only one atrial and one ventricular chamber.

Pathophysiology

In a normal heart, the right side pumps deoxygenated blood to the lungs, while the left side pumps oxygenated blood to the rest of the body. However, in cor biloculare, the mixing of oxygenated and deoxygenated blood occurs within the single ventricular chamber. This mixing leads to inefficient oxygen delivery to the body, causing symptoms associated with oxygen deprivation and increased workload on the single ventricular chamber.

Clinical Manifestations

Individuals with cor biloculare may present with a variety of symptoms, including:

• Cyanosis (a bluish discoloration of the skin due to low oxygen levels)
• Difficulty breathing
• Fatigue
• Poor growth and development in infants and children
• Heart murmurs

Diagnosis

Diagnosis of cor biloculare typically involves a combination of clinical evaluation and imaging studies. Echocardiography is the primary diagnostic tool, providing detailed images of the heart's structure and function. Other imaging modalities, such as magnetic resonance imaging (MRI) of the heart, may also be used to confirm the diagnosis and assess the condition's severity.

Treatment

Treatment of cor biloculare is challenging and focuses on managing symptoms and preventing complications. Options may include:

• Medications to support heart function
• Oxygen therapy
• Surgical interventions to improve blood flow and oxygenation, although the specific procedures depend on the heart's unique anatomy and the presence of other defects

Prognosis

The prognosis for individuals with cor biloculare varies widely and depends on several factors, including the severity of the defect, the presence of other congenital anomalies, and the timeliness and effectiveness of treatment. Early diagnosis and intervention can improve outcomes, but many affected individuals may require lifelong medical care.

See Also

• Congenital heart defect
• Heart
• Echocardiography