Very rare disease characterized by anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA) which may result in myocardial infarction, congestive heart failure and sometimes death during the early infantile period.
- WBGS affects 1 in every 300 000 live births.
History:
In 1933, three doctors from the Massachusetts General Hospital (MGH) in Boston, William Franklin Bland, Paul Dudley White, and Joseph Garland, described a case of an anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) in a three-month-old boy. The infant died following two weeks of hospitalization. The authors stressed the credibility of information obtained from the infant’s parents, i.e. the alarming symptoms they observed at home before leaving for hospital. The infant’s father, Dr. Aubrey Hampton, a radiologist, was White, Bland, and Garland’s colleague at MGH.
Clinical presentation
Onset of symptoms is usually observed in the neonatal period. Beginning at 1 to 3 months, feeding or crying induces dyspnea, profuse sweating, pallor, fatigue and a semblance of pain. Between attacks, physical examination is frequently normal.
- Failure to thrive
- Difficulties in breast-feeding
Long-term complications:
Patients with Bland-White-Garland (BWG) syndrome who survive past childhood often have varying symptoms cardiac complications depending on the development of collateral circulation. However, a majority of patients die in infancy.
- Myocardial ischaemia
- Impaired left ventricular function
- Progressive heart failure
- Pulmonary hypertension: Gradually develops because of combined left-to-right shunting, left ventricular dysfunction and mitral regurgitation
- Mitral regurgitation: Result from dysfunction of ischemic papillary muscles and adjacent myocardium
Case study:
Diagnosis
The health care professional should take a full medical history, including history of pregnancy, infections, diet, and psychosocial state, and perform a full physical examination, because BWGS is a life-threatening clinical syndrome that must be included in the differential diagnosis.
Electrocardiography (EKG):
In theory, the more extensive the coronary collaterals, the better preserved the myocardial function due to lack of ischemia, although the degree of coronary steal may then become significant.
- Left axis deviation
- Abnormal Q-waves in leads I and aVL
- Poor R-wave progression resembling lateral myocardial infarction and left ventricular hypertrophy (with/without ST-segment depression)
Coronary angiography:
Management
Surgical management:
Surgery is recommended in patients with BWG syndrome, even in the absence of symptoms or a significant left-to-right shunt syndrome, given the risk of ventricular arrhythmias and sudden death.
- Procedures preferred in children:
- Establishment of a 2-coronary system: By the direct implantation of the left coronary artery into the ascending aorta
- Procedures preferable in adults:
- Ligation of left coronary artery ostium combined with aortocoronary bypass grafting using venous grafts, the internal mammary artery or the radial artery
- Intrapulmonary technique