Background: Williams syndrome (WS) is a rare genetic condition frequently associated with cardiovascular anomalies, particularly supravalvular aortic stenosis (SVAS). The coexistence of SVAS, aneurysmal dilation, and infective endocarditis (IE) with vegetations in the aortic arch is exceedingly rare.Case presentation: We describe an an 8-year-old Arab boy from Syria with WS who presented with fatigue, tachycardia, dyspnea, fever, chills, and night sweats. Transthoracic echocardiography revealed a dilated aortic arch with large vegetation at the origin of the brachiocephalic trunk and SVAS. Despite 10 days of intravenous antibiotic therapy, vegetations persisted, necessitating urgent surgery. The ascending aorta was replaced using the McGoon technique with complete excision of infected tissue. Postoperative recovery was uneventful, and follow-up imaging showed good left ventricular function without aortic gradient.Conclusion: This case underscores the importance of early recognition and timely surgical intervention in preventing catastrophic outcomes in patients with WS and complex cardiovascular involvement.Keywords: Williams syndrome, supravalvular aortic stenosis, aneurysm, vegetations, endocarditis, case report.

Unroofed coronary sinus syndrome may be associated with persistent left superior vena cava (PLSVC). Herein, we present a case of a 2-year-old patient who underwent an operation for repair of a coronary sinus-type ASD; however, PLSVC was detected intraoperatively. total repair was performed by creating an intra-atrial tunnel.

Konno-Rastan operation is the treatment of choice for relief of complex left ventricular outflow obstruction, especially in those with a small aortic annulus. In this report, we present a case of a ten-year-old boy with the diagnosis of recurrent diffuse subaortic stenosis and dextrocardia who underwent Konno-Rastan operation successfully.

Total anomalous pulmonary venous connection is a rare congenital anomaly, and has four anatomical subtypes of which the mixed type represents diagnostic and therapeutic challenge. Herein, we present a rare case of obstructed mixed type total anomalous pulmonary venous connection with successful surgical repair

Congenital coronary artery fistula is a relatively rare cardiac anomaly accounting for 0.2-0.4% of all congenital heart defects, and when isolated, is commonly asymptomatic. We present a case of an asymptomatic 9-year-old boy who underwent successful surgical repair of a right coronary artery fistula to the right ventricle.

Ellis--van Creveld syndrome is a rare autosomal recessive disorder. We describe the case of a 7-year-old girl with Ellis--van Creveld Syndrome with the diagnosis of common atrium and partial atrioventricular septal defect. She underwent a successful surgical repair, and intraoperatively, a double orifice mitral valve was diagnosed as well.

Situs inversus totalis associated with partial anomalous pulmonary venous connection is a very rare congenital heart malformation. Herein, we describe successful surgical repair of the case of a 30-month-old girl who had situs inversus totalis, partial anomalous pulmonary venous connection , large atrial septal defect, and severe pulmonary hypertension.

Cardiac Hydatid Cysts are uncommonly encountered entity of hydatid disease. Presentation may be with non-specific symptoms and sometimes with life threatening events. We present a rare case of a 9-year-old female who was diagnosed with a Cardiac Hydatid Cyst in the right ventricle, and underwent successful surgical excision

Congenital left atrial aneurysm is a very rare congenital cardiac anomaly, and very few cases have been reported with neonatal presentation. Despite the congenital origin of this condition, it may remain asymptomatic and patients may not present until their third decade of life. Even in asymptomatic cases, prompt surgical intervention is indicated for the prevention of fatal thromboembolic events such as stroke. In neonates and infants presenting early in life, surgical intervention is urgent to relieve respiratory distress symptoms. Herein, we introduce the case of a 3-week-old neonate who presented with severe respiratory distress symptoms requiring prompt intubation and mechanical ventilation. Diagnosis of congenital left atrial aneurysm was established and confirmed by transthoracic echocardiography and computed tomography angiography. The patient underwent surgical resection of the aneurysm using cardiopulmonary bypass and cardiac arrest. The patient was discharged from the hospital after three weeks with normal echocardiography.