Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of anomalous left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome, present the challenges of performing a differential diagnosis, and discuss the treatment of the syndrome. Although ALCAPA is a rare congenital heart disease, it is one of the most common causes of myocardial ischemia in childhood and presents a diagnostic challenge. A four-year-old girl was referred to a pediatric cardiologist for evaluation of mitral valve regurgitation murmur and heart failure. The transthoracic echocardiogram demonstrated the left coronary artery (LCA) not arising from the aorta, presence of coronary collateral circulation, and moderate mitral valve regurgitation. ALCAPA was confirmed using angiotomography. The LCA was surgically reimplanted into the aorta. After 3 years of postoperative follow-up, the patient developed an LCA aneurysm. Diagnosis of cardiac ischemia in childhood remains a challenge, and careful evaluation of coronary arteries on the echocardiogram is an important tool. In this report, we present a case of ALCAPA with an uncommon postoperative outcome.
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PMID:Aneurysm of the Left Coronary Artery in Postoperative Bland-White-Garland Syndrome. 2677 Aug 39

Bland-White-Garland syndrome (BWG) is a congenital heart disease characterized by anomalous origin of left coronary artery from main pulmonary artery. The anomaly causes significant myocardial ischemia, mitral insufficiency, and high infant mortality. Several investigators have reported that the subjects who survived to adulthood showed extensive collateral flow from right coronary artery (RCA). We report a case of newly diagnosed BWG in an elderly patient who had well-developed collaterals from aneurysmal giant RCA and bronchial artery, which were identified by coronary angiography with multi-detector computed tomography. <Learning objective: Structural heart disease is occasionally hard to assess with invasive angiography. In this case, multi-detector computed tomography demonstrated complicated structure of anomalous coronary morphology including collateral sources from extra-cardiac anastomoses. The severity of anomaly related myocardial ischemia was evaluated by image fusion with radionuclide myocardial perfusion. Multimodal imaging approach can provide comprehensive and incremental information.>.
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PMID:An adult case of Bland-White-Garland syndrome with collaterals from giant right coronary artery and bronchial artery. 3027 93

We experienced two adult cases of anomalous origin of the left coronary artery from the pulmonary artery, so-called Bland-White-Garland (BWG) syndrome, that presented with ventricular tachycardia (VT) and ventricular fibrillation during exertion in daily life. They presented to our hospital with syncope due to VT, and recovered following application of an automated external defibrillator with cardiopulmonary resuscitation. We diagnosed BWG syndrome by multi-detector computed tomography angiography and coronary angiography. We analyzed the mechanisms of lethal arrhythmias in relation to myocardial ischemia on exertion. Coronary flow modification and implantable cardioverter defibrillator implantation were performed in order to prevent future lethal arrhythmia due to myocardial ischemia. It is important to be aware of congenital heart disease in ordinary cases. <Learning objective: We experienced two rare cases of patients who were admitted with ventricular tachycardia and ventricular fibrillation who had adult type Bland-White-Garland syndrome during exertion in daily life. It is important to be aware of congenital heart disease in ordinary cases.>.
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PMID:Two adult cases of Bland-White-Garland syndrome with lethal arrhythmia due to coronary steal phenomenon during physical or mental stress. 3054 46

Bland-White-Garland (BWG) syndrome is a rare congenital heart disease in which the left coronary artery originates from the pulmonary artery (PA). Surgical treatment to rebuild a dual coronary system is recommended at the time of the diagnosis. However, no effective operative procedure has been established for adult-type BWG patients because of the paucity of such cases. We herein report a case of adult-type BWG that was successfully treated by patch closure of the orifice of the left main tract from the main PA and coronary artery bypass grafting. 123I-15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) and 201thallium (Tl) dual myocardial single-photon emission computed tomography (SPECT) were performed before surgery, early after surgery, and at three months after surgery. Before surgery, dual SPECT showed myocardial perfusion defects in the anterior and septal wall, which corresponded to the cardiovascular magnetic resonance imaging findings. Early after surgery, only 201Tl images demonstrated an improvement in the defect area. At three months after surgery, both the 201Tl and 123I-BMIPP imaging findings demonstrated an improvement in the defect area, which was correlated with the recovery of the left ventricular function. These results showed the effectiveness of this surgical approach for BWG syndrome. <Learning objective: Adult-type Bland-White-Garland syndrome is a rare congenital disease and no effective operative procedure has been established because of the paucity of such cases. The patient in this report underwent patch closure of the orifice of the left main tract from the main pulmonary artery and coronary artery bypass grafting. Myocardial dual single-photon emission computed tomography was considered to be effective for evaluating the results of revascularization surgery, and an improved uptake on 123I-15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid images was closely related to an improvement in the left ventricular function.>.
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PMID:123I-BMIPP/201Tl dual myocardial SPECT proves the efficacy of surgical treatment for an adult with Bland-White-Garland syndrome. 3193 6