A 78-year-old case of Bland–White–Garland syndrome with conservative therapy

Abstract

A 78-year-old woman was referred to our echo laboratory for shortness of breath and chest oppression during exercise last year. The symptoms occurred during her childhood, but were well tolerated without treatment. The patient's height was 150 cm and her weight was 42 kg, almost the average of the age-matched Japanese women group. Physical examination showed a blood pressure of 118/64 mmHg and a regular pulse rate of 72/min. Auscultation revealed a grade 3 systolic ejection murmur and a grade 1 continuous murmur at the left sternal border and apex. Electrocardiography showed sinus rhythm with high voltage in I, aVL, and chest leads, accompanied by negative T wave in I and aVL.Transthoracic echocardiography demonstrated a dilated, hypertrophic left ventricle (LV) with preserved global ejection fraction, in spite of the hypokinesis of the anteroseptal wall. There was no significant valvular regurgitation or stenosis. Color Doppler echocardiography showed remarkable continuous flow inside all segments of the LV wall. Reverse flow, from the posterior to the anterior side, was observed in the septal branch as well as the main stream of the left anterior descending coronary artery (LAD) (Fig. 1a, b), suggesting collateral flow from the right coronary artery (RCA) to the LAD. Also, blood flow from the atrioventricular branch of the RCA connected to a branch of the left circumflex coronary artery (LCx) was recorded (Fig. 1c). Thus, both the LAD and LCx were fed by collateral vessels from the RCA. Coronary sinus was not dilated and no flow signals drained from the myocardium into any cardiac chamber. Pulsed-wave Doppler demonstrated a diastole-dominant, high flow velocity of about 1 m/s in a collateral vessel (Fig. 1d). The short-axis view at the pulmonary artery level demonstrated that the left main coronary artery (LMCA) was drained into the proximal main pulmonary artery (Fig. 2a). This anomaly was confirmed by cardiac artery computed tomography (CACT, Fig. 2b). CACT also revealed some collateral connections of epicardial coronary arteries with tortured and dilated RCA as well as LAD (Fig. 3).Since the patient had only limited symptoms and was also complicated by Alzheimer's disease, conservative medication had been chosen instead of surgery, and the symptoms were well controlled. DiscussionThe prognosis of non-operated Bland-White-Garland (BWG) syndrome depends on the development of collateral vessels [1]. Compared to the majority of patients who died or received surgical repair during their childhood or early adulthood [2,3], to our knowledge, our patient is the eldest alive without having undergone surgery. The major