Raghib syndrome

Raghib Syndrome is a rare congenital disorder characterized by the combination of congenital heart disease with abnormalities of the sinus node and atrioventricular node, and a persistent left superior vena cava. It was first described by Raghib et al. in 1965.

Etiology

The exact cause of Raghib Syndrome is unknown. However, it is believed to be a result of abnormal development of the cardiac neural crest cells during embryogenesis. These cells contribute to the formation of the heart and the great vessels.

Clinical Features

The clinical features of Raghib Syndrome can vary greatly among affected individuals. The most common features include:

• Bradycardia: A slower than normal heart rate.
• Sinus node dysfunction: A condition that slows down the heart's natural pacemaker.
• Atrioventricular block: A delay or complete block in the electrical signals between the upper and lower chambers of the heart.
• Congenital heart defects: These can include atrial and ventricular septal defects, patent ductus arteriosus, and tetralogy of Fallot.
• Persistent left superior vena cava: A condition where the vein that carries deoxygenated blood from the upper half of the body to the heart remains on the left side instead of the right.

Diagnosis

Diagnosis of Raghib Syndrome is based on the clinical features, echocardiography, and electrocardiogram (ECG). Genetic testing may also be performed to rule out other conditions.

Treatment

Treatment of Raghib Syndrome is symptomatic and supportive. It may include medications to manage heart rhythm abnormalities, surgery to correct congenital heart defects, and in some cases, the implantation of a pacemaker.

Prognosis

The prognosis for individuals with Raghib Syndrome varies depending on the severity of the heart defects and the presence of other health problems. With appropriate treatment and management, many individuals with Raghib Syndrome can lead normal lives.

See Also

• Congenital heart disease
• Sinus node dysfunction
• Atrioventricular block

NIH genetic and rare disease info

• NIH info on Raghib syndrome

Raghib syndrome is a rare disease.

Raghib syndrome Resources
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