Bland-White-Garland syndrome

Overview

Bland-White-Garland syndrome (BWG syndrome), also known as Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA), is a rare congenital heart defect in which the left coronary artery arises abnormally from the pulmonary artery instead of the aorta. This anomaly disrupts the normal oxygen supply to the heart muscle and can lead to myocardial ischemia, heart failure, or sudden cardiac death if left untreated. The syndrome was first described by Drs. Bland, White, and Garland in 1933. It is most commonly diagnosed in infancy, although some rare cases may present later in life.

Causes

Bland-White-Garland syndrome is a congenital condition, meaning it develops during fetal life. The exact cause is not fully understood, but it results from an abnormal development of the coronary arteries during embryogenesis. In a normal heart, both coronary arteries originate from the aorta and supply oxygen-rich blood to the heart muscle. In BWG syndrome, the left coronary artery arises from the pulmonary artery, which carries oxygen-poor blood, leading to insufficient oxygen delivery to the left side of the heart.

Symptoms

Symptoms of BWG syndrome typically become apparent in the first few months of life as the pulmonary vascular resistance decreases after birth, leading to reduced oxygen supply to the myocardium. Common symptoms include:

• Feeding difficulties

• Poor weight gain (failure to thrive)

• Excessive sweating during feeding or activity

• Rapid breathing (tachypnea)

• Irritability and fatigue

• Signs of heart failure – including enlarged heart and pulmonary congestion

• In older patients – chest pain, exercise intolerance, arrhythmias, or sudden cardiac arrest may occur

Diagnosis

Early diagnosis of BWG syndrome is crucial to prevent irreversible heart damage. Diagnostic approaches may include:

• Electrocardiogram (ECG) – may show signs of myocardial ischemia or infarction

• Chest X-ray – may reveal cardiomegaly (enlarged heart) and pulmonary edema

• Echocardiogram – non-invasive imaging to visualize coronary artery origins and assess cardiac function

• Cardiac catheterization or angiography – to confirm the abnormal origin of the left coronary artery

• Cardiac MRI or CT angiography – for detailed anatomical assessment, especially in older children and adults

Treatment

Surgical correction is the definitive treatment for Bland-White-Garland syndrome. The goal is to restore normal blood flow to the left coronary artery. Common surgical procedures include:

• Coronary reimplantation – transferring the left coronary artery from the pulmonary artery to the aorta, restoring normal blood supply

• Takeuchi procedure – creating a tunnel within the pulmonary artery to redirect aortic blood into the left coronary artery (used when direct reimplantation is not feasible)

• Mitral valve repair – in cases with associated mitral valve insufficiency due to ischemic damage

• Postoperative management – includes medications such as inotropes, diuretics, and beta-blockers as needed during recovery

Timely surgery significantly improves survival and cardiac function.

Prognosis

With early diagnosis and successful surgical repair, the prognosis for individuals with Bland-White-Garland syndrome is excellent. Most patients experience normalization of heart function and a significant improvement in quality of life. However, delayed diagnosis can lead to severe complications such as irreversible myocardial damage, arrhythmias, or sudden death. Lifelong follow-up with a cardiologist is essential to monitor heart function, especially in patients who had significant ischemic injury prior to treatment.