Anomalous Origin of the Left Main Coronary Artery from the Right Coronary Artery with a Preaortic Course

We report the case of a 51-year-old woman who presented with stable angina pectoris and Canadian Cardiovascular Society class II functional capacity. An electrocardiogram during a treadmill exercise test showed substantial ST-segment depression in the inferolateral leads. Coronary angiograms revealed an anomalous origin of the left main coronary artery from the opposite sinus of Valsalva and an interarterial course between the ascending aorta and pulmonary artery. Although this phenomenon is dangerous, the patient refused further examination. We discuss the diagnosis and treatment of patients who have an anomalous origin of a coronary artery from the opposite sinus of Valsalva.     

Sudden Cardiac Death Associated with Anomalous Origin of the Left Main Coronary Artery from the Right Sinus,with an Intramural Course

Anomalous origin of the left main coronary artery from the right sinus of Valsalva is extremely rare and can lead to sudden cardiac death. We report a case in which an 18-year-old college student collapsed immediately after a long-distance run of 10 km. After cardiopulmonary resuscitation and electrical shock for ventricular fibrillation, she experienced a return of spontaneous circulation. Cardiac catheterization and cardiac computed tomographic angiography revealed an unusually long intramural course of the left main coronary artery from the right sinus of Valsalva. The young woman underwent a successful unroofing operation for coronary artery correction. She remained asymptomatic upon exercise during 2.5 years of follow-up.     

Multimodal Imaging after Sudden Cardiac Arrest in an 18-Year-Old Athlete

We report the case of a previously healthy 18-year-old male athlete who twice presented with sudden cardiac arrest. Our use of electrocardiography, echocardiography, cardiac magnetic resonance, coronary angiography, coronary computed tomographic angiography, and nuclear stress testing enabled the diagnoses of apical hypertrophic cardiomyopathy and anomalous origin of the right coronary artery. We discuss the patient''s treatment and note the useful role of multiple cardiovascular imaging methods in cases of sudden cardiac arrest.     

Primary Prevention of Sudden Cardiac Death in Adults with Transposition of the Great Arteries: A Review of Implantable Cardioverter-Defibrillator Placement

Transposition of the great arteries encompasses a set of structural congenital cardiac lesions that has in common ventriculoarterial discordance. Primarily because of advances in medical and surgical care, an increasing number of children born with this anomaly are surviving into adulthood. Depending upon the subtype of lesion or the particular corrective surgery that the patient might have undergone, this group of adult congenital heart disease patients constitutes a relatively new population with unique medical sequelae. Among the more common and difficult to manage are cardiac arrhythmias and other sequelae that can lead to sudden cardiac death. To date, the question of whether implantable cardioverter-defibrillators should be placed in this cohort as a preventive measure to abort sudden death has largely gone unanswered. Therefore, we review the available literature surrounding this issue.     

Papillary Fibroelastoma of the Aortic Valve: Analysis of 21 Cases,Including a Presentation with Cardiac Arrest

Cardiac papillary fibroelastoma is a rare, benign tumor, arising predominantly from cardiac valves. This tumor can cause a variety of symptoms due to thromboembolism. We describe our single-center surgical experience with papillary fibroelastoma of the aortic valve.From April 2004 through June 2013, 6,530 patients underwent cardiac surgery. Of those, 6,098 patients were included in the final analysis. Twenty-one patients (0.34%) underwent surgical resection of 30 papillary fibroelastomas of the aortic valve.Most patients (67%) were incidentally diagnosed to have cardiac papillary fibroelastoma. The usual symptom was cerebral infarction (in 5 of 7 symptomatic patients). A rare presentation of papillary fibroelastoma in one patient was cardiac arrest caused by left main coronary artery ostial obstruction. Tumor size was not related to patient age (Pearson correlation coefficient, 0.34; P=0.13). Neither the number of tumors (1.43 ± 0.72 vs 1.43 ± 0.62) nor tumor size (8.14 ± 2.42 vs 8.07 ± 3.31 mm) was significantly different between symptomatic and asymptomatic patients. All lesions were resected by means of the simple shave technique. There were no operative or 30-day deaths. Follow-up echocardiograms showed no tumor recurrence (mean follow-up duration, 17 ± 14 mo).We identified no significant relationship among tumor size, number of tumors, symptoms, or patient age. Because simple shave excision of the tumor can be safely achieved without evidence of tumor recurrence, we conclude that surgical resection can be reasonable in asymptomatic patients.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.