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Bland-White-Garland syndrome (BWGS)

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.

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
Coronary angiography in right anterior oblique view showed the giant coronary aneurysm (yellow arrow) and significantly enlarged collateral circulation supplied to left coronary artery (Video is also available as online supplemental data). A pig tail catheter with an autoinjector was used to assess right coronary aneurysm because it was hard to visualize using Judkins catheter. | LAO, left anterior oblique; RAO, right anterior oblique. | Tobita, T., Arashi, H., Fukushima, K., & Hagiwara, N. (2017). An adult case of Bland-White-Garland syndrome with collaterals from giant right coronary artery and bronchial artery. Journal of cardiology cases, 16(2), 41–43. https://doi.org/10.1016/j.jccase.2017.04.006

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)
Resting ECG—abnormal Q waves in leads I and aVl, negative T-waves in leads I, aVl, V2, V3. | Petr Parizek, Ludek Haman, Jan Harrer, Miloslav Tauchman, Vladimir Rozsival, Ivo Varvarovsky, Miloslav Pleskot, Miroslav Mestan, Josef Stasek, Bland–White–Garland syndrome in adults: sudden cardiac death as a first symptom and long-term follow-up after successful resuscitation and surgery, EP Europace, Volume 12, Issue 9, September 2010, Pages 1338–1340, https://doi.org/10.1093/europace/euq087

Coronary angiography:

Coronary angiography in Patient 2. Right coronary mega artery with a left-to-right shunt to the pulmonary artery because of anomalous origin of the left coronary artery from the pulmonary artery (right anterior oblique view). | Petr Parizek, Ludek Haman, Jan Harrer, Miloslav Tauchman, Vladimir Rozsival, Ivo Varvarovsky, Miloslav Pleskot, Miroslav Mestan, Josef Stasek, Bland–White–Garland syndrome in adults: sudden cardiac death as a first symptom and long-term follow-up after successful resuscitation and surgery, EP Europace, Volume 12, Issue 9, September 2010, Pages 1338–1340, https://doi.org/10.1093/europace/euq087

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

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