Szmigielska Agnieszka, Roszkowska-Blaim Maria, Gołąbek-Dylewska Małgorzata, Tomik Agnieszka, Brzewski Michał, Werner Bożena
Department of Pediatric Nephrology, Medical University of Warsaw, Warsaw, Poland.
Am J Case Rep. 2013 Sep 16;14:370-2. doi: 10.12659/AJCR.889112. eCollection 2013.
Male, 0 FINAL DIAGNOSIS: Bland-White-Garland syndrome Symptoms: Cardiomegaly, feeding problems Medication: - Clinical Procedure: Reimplantation of the left coronary artery to the aorta Specialty: Pediatrics and Neonatology.
Rare disease.
Bland-White-Garland syndrome (BWGS) is a very rare disease characterized by anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA). WBGS affects 1 in every 300 000 live births. Children typically present with dyspnea, pallor, and failure to thrive. Without surgical repair, most of these children die during the first months of life.
This case report describes 3-month-old boy admitted to the hospital because of feeding problems. The boy was born at term, with birth weight 3200 g, and was 10 points in Apgar score. He was breast-fed from birth. From the seventh week of age, his mother observed his increasing difficulties with feeding. Physical examination revealed pale skin, diminished heart sounds, tachycardia, cardiomegaly, and hepatomegaly. Results of urine and blood tests and ultrasonography of the central nervous system and abdomen were normal. The chest radiography showed cardiomegaly and electrocardiogram revealed anterolateral myocardial infarction. On echocardiography, an anomalous left coronary artery arising from the pulmonary artery was found. The life-saving treatment of choice was immediate surgical reimplantation of the left coronary artery to the aorta.
Children with congenital heart disease are often prone to malnutrition, but in rare cases failure to thrive and breast-feeding problems can be the first symptoms of life-threatening diseases like myocardial infarction secondary to Bland-White-Garland syndrome (BWGS).
男性，0岁 最终诊断：布兰德 - 怀特 - 加兰综合征 症状：心脏扩大、喂养困难 用药：- 临床操作：左冠状动脉再植至主动脉 专科：儿科与新生儿科。
罕见病。
布兰德 - 怀特 - 加兰综合征（BWGS）是一种非常罕见的疾病，其特征为左冠状动脉起源于肺动脉干（ALCAPA）。BWGS在每30万例活产中约有1例发病。患儿通常表现为呼吸困难、面色苍白及发育不良。若不进行手术修复，这些患儿中的大多数会在出生后的头几个月内死亡。
本病例报告描述了一名因喂养困难入院的3个月大男婴。该男婴足月出生，出生体重3200克，阿氏评分10分。他从出生起就进行母乳喂养。从7周龄起，他的母亲发现他喂养困难日益加重。体格检查发现皮肤苍白、心音减弱、心动过速、心脏扩大及肝脏肿大。尿液和血液检查结果以及中枢神经系统和腹部超声检查均正常。胸部X线检查显示心脏扩大，心电图显示前外侧心肌梗死。超声心动图检查发现左冠状动脉起源于肺动脉。首选的挽救生命的治疗方法是立即进行左冠状动脉再植至主动脉的手术。
先天性心脏病患儿常易发生营养不良，但在罕见情况下，发育不良和母乳喂养问题可能是诸如布兰德 - 怀特 - 加兰综合征（BWGS）继发心肌梗死等危及生命疾病的首发症状。
