Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity. Case presentation: We were involved in the assessment, treatment, and pathological evaluation of two adult ALCAPA patients who were rescued from ventricular fibrillation and then surgically treated to establish a dual coronary artery system. Histological studies indicated various chronic ischemic changes in the myocardium, patchy fibrosis, and severely thickened arteriolar walls in both ventricles. The first patient is alive and well 11.5 years after surgical correction without any implantable cardioverter defibrillator (ICD) activations. The second patient required redo surgery 9 months after the initial operation but subsequently died. Histologically, chronic ischemic alteration of the myocardium and thickened arteriolar walls persisted even after surgical correction, and coronary angiography (CAG) showed an extremely slow flow phenomenon even after surgical correction in both patients. The average postoperative opacification rate in the first case was 7.36 + 1.12 (n = 2) in the RCA, 3.81 + 0.51 (n = 3) in the left anterior descending (LAD) artery, and 4.08 + 0.27 (n = 4) in the left circumflex (LCx) artery. The slow flow phenomenon may represent persistent high arteriolar resistance in both ventricles. Conclusions: Seldom reported or new findings in adult ALCAPA were identified in two cases. More frequent diagnosis of adult ALCAPA can be expected because of the widespread availability of resuscitation and more advanced diagnostic modalities. Accumulation of pathological and clinical findings and confirmation of the longterm follow-up results after treatment may contribute to expanding our knowledge of this rare entity and establishing optimal treatment.
IntroductionAnomalous origin of the left coronary artery from the pulmonary artery (ACLAPA) is widely called BWG syndrome, and was first reported by Bland, White, and Garland in 1933 [1]. This is a rare congenital heart disease with prevalence of one in 300,000 births or 0.25-0.5% of all congenital heart disease [2]. The left main coronary artery (LMCA) originates from the main pulmonary artery in more than 90% of cases of BWG syndrome. We herein report two notable adult BWG syndrome cases that presented with ventricular tachycardia (VT) during exertion in daily life, and recovered well with cardiopulmonary resuscitation (CPR) by appropriate use of an automated external defibrillator (AED).
Case 1A 42-year-old man suddenly collapsed while jogging. Consciousness level was Glasgow Coma Scale (GCS) 1-1-1 at the time the rescue team arrived, and bystander CPR had been performed until then. The electrocardiographic (ECG) monitor showed VT and ventricular fibrillation (VF) when the AED was attached. The AED was operated 7 min after the patient collapsed. ECG showed normal sinus rhythm with ST depression in the chest leads on arrival at our hospital.After administration of amiodarone, VT did not reoccur during the course of admission. Multi-detector computed tomography coronary angiography (CTA) was performed because of suspected ischemic arrhythmia, and showed that the left coronary artery (LCA) originated from the pulmonary artery with marked collateral flow from the right coronary artery (RCA) to the LCA, consistent with BWG syndrome (Fig. 1a).Invasive coronary angiography (CAG) showed that the RCA and conus branch were well developed and provided good collateral flow to the left anterior descending artery and left circumflex Journal of Cardiology Cases xxx (2016) xxx-xxx Anomalous origin of left coronary artery from pulmonary artery Ventricular tachycardia Congenital
A B S T R A C TWe 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.
The early diastolic tricuspid annular velocity determined by TDI is a noninvasive and sensitive index for diagnosing RVMI.
A 27-year-old man was referred to our hospital for rightsided heart failure. At the age of 19 years, he had undergone tricuspid valve replacement (TVR) with a bioprosthesis, a Carpentier-Edwards pericardial (CEP) valve, because of infective endocarditis of the tricuspid valve (TV). On examination, he was afebrile, with a blood pressure of 110/66 mmHg and a heart rate of 84 bpm. Auscultation of the heart was notable for a grade 3/6 holosystolic regurgitant murmur and diastolic murmur at the 4th left sternal border. Jugular venous distention and peripheral edema were observed.Dilatation of the right atrium was observed in transthoracic echocardiography and a tricuspid regurgitation (TR) jet was seen on a color Doppler image. Continuouswave Doppler showed a mean pressure gradient of about 12 mmHg across the bioprosthetic TV. Real-time threedimensional transesophageal echocardiography (RT3D-TEE) (iE33; Philips, Bothell, WA, USA) was performed in order to evaluate the prosthetic valve function. The RT3D-TEE demonstrated tricuspid stenosis with restricted leaflet motion, incomplete closing bioprosthetic TV, and obvious TR ( Figs. 1 and 2). The leaflets are thickened and fibrocalcific, with decreased mobility. A redo TVR was performed for valve dysfunction of the CEP.The causes of bioprosthetic valve dysfunction in this case were concluded to be fibrotic and sclerotic degeneration with calcification based on the pathological findings of the extracted bioprosthesis. The RT3D-TEE findings were consistent with these pathological findings. DiscussionRight-sided valve replacement is less common than leftsided valve replacement. The major disadvantage of the tissue valve is structural deterioration. The prevalence of primary valve failure 15 years after valve implantation is 20% for aortic bioprostheses and 40% for mitral bioprostheses [1,2]. One study demonstrated a 68.7% survival rate at 18 years after TVR and a 34% rate of redo TVR in surviving patients [3]. Valve failure is caused by the following conditions: (1) tissue deterioration, which results in a loss of leaflet integrity and consequent regurgitation, and (2) tissue calcification, which results in valve stenosis or regurgitation. The risk of fibrocalcific degeneration is higher in younger patients than in older ones. A previous study reported that the CEP is more susceptible to pannus formation and tissue overgrowth on the cusps than other types of bioprostheses. The characteristics of our case were: (1) a young age and (2) an implanted CEP. We suspected that these factors had accelerated the prosthetic valve deterioration.Two-dimensional transesophageal echocardiography (2D-TEE) is almost wholly incapable of visualizing structural valve deterioration. Compared to 2D-TEE, RT3D-TEE furnishes a clearer view of the anatomy and better facilitates our understanding of the mechanism and severity of bioprosthesis dysfunction [4,5]. In this case, RT3D-TEE could directly visualize morphologic abnormalities, such as leaflet
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