Bland White Garland syndrome with aneurysm and collaterals of coronary arteries
Bland White Garland Syndrome (BWGS), also known as the aberrant origin of the left coronary artery from the pulmonary artery. It is an extremely rare congenital abnormality. BWGS affects around one in every 300,000 live newborns, accounting for 0.25% to 0.5% of all congenital heart disease cases.
In this anomaly, the left coronary artery (LCA) emerges from the pulmonary artery rather than the aorta. Children usually exhibit dyspnea, pallor, and failure to thrive. Moreover, when collaterals are adequate, symptoms may be absent or mild, allowing for continued development into adulthood. However, adults can occasionally be asymptomatic or, more usually, experience a variety of symptoms such as syncope, chest discomfort, and sudden death.
Echocardiography, coronary angiography, and chest computed tomography (CT) are all effective for diagnosing BWGS. When possible, surgery is recommended for patients with BWGS. The most common surgical treatment is the direct reimplantation of an aberrant left coronary artery into the aorta using a pulmonary artery button. It is highly rare for a patient to survive into their sixth or seventh decade of life without surgery.
We report on an extraordinarily unusual instance of Bland-White Garland syndrome. Along with a left coronary artery aneurysm in a 14-year-old Afghan girl.
Case Report
An Afghan 14-year-old girl was referred to Maiwand Teaching Hospital’s Pediatric Unit because she was experiencing chest pain. On arrival, she reported a 30-minute attack of localized middle chest pain, which was eased by rest. Her previous medical and family histories were ordinary. The general physical examination revealed a blood pressure of 110/70, a respiratory rate of 20/min, a heart rate of 69/min, and an oxygen saturation of 94%. A faint early systolic murmur (Levine III/VI) was detected during a systematic examination of the chest. There were no aberrant clinical findings in the other systems. Blood tests were within normal ranges.
Investigations
Blood tests were within normal ranges. The chest x-ray revealed cardiomegaly with a cardiothoracic ratio of 62.5%. Both costophrenic angles were crisp with minimal congestion (Fig. 1). The electrocardiogram (ECG) revealed a heart rate of 69 beats per minute. A normal sinus rhythm, an axis of +50, a PQ of 0.16 s, a QRS of 0.08 s, a QT/QTc of 0.36/0.36 s. And no notable ST segment shift (Fig. 2).
On day three of hospitalization, transthoracic echocardiography revealed an ectopic opening of the coronary artery. A blood-stealing phenomenon at the pulmonary artery, irregular coronary circulation. And pulsed color Doppler confirmed anomalous flow within the pulmonary artery. Pulsed-wave doppler revealed a left coronary wave pattern. The echocardiographic findings indicated BWGS.
There was also all-segment normokinesis, a left ventricular end-diastolic volume (LVEDV) of 72.9 ml, a left ventricular end-systolic volume (LVESV) of 24.9 ml, and an LVEF of 66.1%. Based on the findings of the ECG and cardiac echocardiography. BWGS was suspected; therefore, chest computed tomography (CT) and cardiac catheterization were recommended. The patient’s chest CT scan revealed that the left coronary artery originated from the pulmonary artery. As well as an aneurysm of the LCA.
Coronary angiography revealed an aneurysm or dilation of the left coronary artery (Fig. 6). She remained stable during her three-day hospital stay and after discharge. Then she was transferred to an advanced setting abroad for surgical care of BWGS.
Discussion
Bland, White, and Garland initially documented the Bland-White-Garland syndrome, or abnormal origin of the left coronary artery from the pulmonary artery, in 1933.
The anomaly is normally isolated; however, it has been linked to other congenital heart diseases like patent ductus arteriosus, ventricular septal defect, tetralogy of Fallot, and coarctation of the aorta. Antegrade flow from the pulmonary artery to the coronary artery occurs during fetal life due to high pulmonary artery pressure, allowing for adequate myocardial perfusion from the pulmonary artery via the anomalous coronary artery.
However, following birth, the pulmonary artery contains desaturated blood at a pressure rapidly lower than systemic pressure. As a result, the left ventricle, which has a high demand for oxygen, receives desaturated blood at low pressure. This predisposes to myocardial ischemia, particularly during exercise like eating or crying. Collateral vessels form between the right and left coronary arteries and may give enough blood flow to the left myocardial.
Coronary steal syndrome is a name used to characterize this condition. If the collaterals are sufficient, symptoms may be absent or moderate. Adults may not exhibit any signs, yet they are more likely to experience syncope, chest discomfort, and unexpected death.
The ultimate step occurs in late adulthood when an overloaded collateral artery encounters ischemia due to the steal phenomenon from the RCA to the LCA and pulmonary artery. BWGS can be diagnosed by computed tomography (CT) of the chest, coronary angiography, or echocardiography. Surgery is recommended for BWGS as soon as feasible.