Williams?Beuren Syndrome with Pseudoaneurysm of Aortic Arch and Infective Vegetations for Modified Broms Procedure: Anesthetic Concerns & Echocardiographic Illustrations

Williams?Beuren syndrome is a rare genetic malformation with predilection for supravalvular aortic stenosis. Apart from cardiovascular malformation, hypocalcemia, developmental delay, and elfin facies, challenging airway make perioperative management more eventful. Association of infective endocarditis within the aortic arch and pseudoaneurysm formation is infrequent. We, hereby report a case of pseudoaneurysm formation and infective vegetation within the aortic arch in a patient with Williams syndrome and the role of transthoracic echocardiography in its perioperative management.

Treatment and effect of cardiovascular surgery in patients with Williams syndrome / 中国胸心血管外科临床杂志

@#Objective    To analyze the effects of cardiovascular surgery on Williams syndrome (WS). Methods    The clinical data of 68 WS patients undergoing cardiovascular surgery in the Department of Cardiac Surgery, Guangdong Provincial People's Hospital from January 2010 to January 2020 were retrospectively analyzed. There were 48 males and 20 females with a median age of 2.8 years ranging from 3 months to 33 years. Except one patient undergoing the coarctation repair, the rest 67 patients underwent surgical interventions to correct supravalvular aortic stenosis (SAVS) and pulmonary artery stenosis with hypothermic cardiopulmonary bypass, concommitant with 3 patients of relief of left ventricular outflow tract obstruction, 2 patients of relief of right ventricular outflow tract obstruction, 2 patients of mitral valvuloplasty, 3 patients of ventricular septal defect repair and 1 patient of arterial catheter ligation. Results    Two (2.9%) patients died of sudden cardiac arrest on the next day after surgery. One (1.5%) patient died of cardiac insufficiency due to severe aortic arch stenosis 3 years after surgery. The effect of SAVS was satisfactory. Two (2.9%) patients progressed to moderate aortic valvular regurgitation during postoperative follow-up. A total of 5 (7.4%) patients were re-intervened after operation for arch stenosis or pulmonary stenosis. Conclusion    WS patients should be diagnosed early, followed up and  assessed for cardiovascular system diseases, and timely surgical treatment has a good clinical effect.

Right coronary ostia displacement——maybe an important factor of peri-operative mortality after SVAS correction in infant / 中华胸心血管外科杂志

Objective Explore the influence of different surgical methods on right coronary artery opening after congeni-tal supravalvular aortic stenosis correction surgery, to avoid right coronary artery obstruction caused by surgery.Methods A retrospective analysis was made on the surgical methods and results of continuous records of 91 supravalvular aortic stenosis sur-gery cases operated from 2008 to 2015, while analyzing the cause of early death, early reoperation, and ECMO assisted.Re-sults 4 cases of perioperative death,6 cases of ECMO assisted,8 cases of reoperation,all above are concerned with early ma-lignant arrhythmia.The surviving cases were followed up for 15 months,no death cases during the followed-up.Conclusion Different surgical methods have different influences on right coronary artery, related to the operation of right coronary incision. If the patch is oversize,it will cause obstruction of right coronary artery opening and cause malignant arrhythmia,however,un-dersized patch can not relieve aortic stenosis.So different surgical methods should be used according to different age and weight of patients.

Estenosis aórtica supravalvular: reporte de caso clínico y revisión bibliográfica / Supravalvular aortic stenosis: clinical case report and review

Resumen La estenosis aórtica supravalvular es una lesión congénita obstructiva del tracto de salida del ventrículo izquierdo. Es la forma menos frecuente dentro de este grupo de lesiones, las cuales, a su vez representan el 6% de las cardiopatías congénitas en pacientes pediátricos. Esta cardiopatía se relaciona más con pacientes pediátricos, sin embargo, presentamos el caso de un paciente masculino de 23 años de edad quien acudió al hospital por haber presentado episodio de disnea súbita en reposo, acompañada de diaforesis, dolor torácico opresivo sin irradiaciones y síncope. Se realizó un ecocardiograma transtorácico con el cual se diagnosticó estenosis aórtica supravalvular.

Abstract Supravalvular aortic stenosis is a left ventricular outflow tract obstructive lesion. It is the least common form of this group of lesions, which only represents 6% of congenital heart disease in children. This condition is commonly diagnosed during childhood; however we present the case of a 23 year old man who was taken to the hospital for having presented a sudden dyspnea episode with diaphoresis, chest pain without radiation and syncope. He was diagnosed with supravalvular aortic stenosis after echocardiography.

Surgical treatment for congenital supravalvular aortic stenosis in children with Doty procedure / 中华实用儿科临床杂志

Objective To summarize the clinical effect of Doty procedure on surgical treatment for patients with congenital supravalvular aortic stenosis (SVAS).Methods Between Jan.2009 and Jun.2013,12 cases of consecutive patients underwent surgical corrections of SVAS using Doty procedure.Among them,Williams' syndrome were present in 5 patients.Nine cases were male,and 3 cases were female.Mean age was(5.3 ±4.2) years(9 months to 12 years) and mean weight was (11.5 ± 5.3) kg (7.5-32.0 kg).Echocardiography and Computed Tomography confirmed the diagnosis.SVAS was discrete in 9 patients and diffuse in 3 patients.Mean peak preoperative gradient was 6.86-16.23 (11.07± 3.68) kPa,with 3 patients above 13.33 kPa.Bicuspid aortic valve was present in 2 patients,mild aortic valve stenosis in 1 patient,mild aortic regurgitation in 2 patients,1 patient with subaortic membrane,3 patients with stenosis of the left or the right pulmonary artery,1 patient with pulmonary valve stenosis,2 patients with PDA,moderate to severe mitral insufficiency in 1 patients.Surgery was performed using Doty procedure through median sternotomy with cardiopulmonary bypass.Results Mean time of cardiopulmonary bypass was (85 ± 26)min (68-129 min).Mean time of aortic clamp was (51 ± 16) min(43-68 min).There was no early death and low cardiac output syndrome.Mild ST segment change was present in 5 patients and occasional premature beat was in 3 patients.Ventilator was applied for 7 to 38 h and Cardiac Intensive Care Unit treatment was done for 1 to 4 d.The mean peak supravalvular gradient measured postoperatively was (2.18 ± 1.09) kPa.Follow-up data were available for 11 patients,ranged from 6 months to 3 years.The gradients across supravalvular were lower than 3.33 kPa,except for 2 patients with severe SVAS preoperatively.There was no late death,dizzy or palpitation during follow-up.Conclusion Doty produce for SVAS can get satisfactory clinical results.

Supravalvular Aortic Stenosis Combined with Right Coronary Artery Ostial Obstruction

Supravalvular aortic stenosis is an uncommon congenital narrowing of the ascending aorta that may be localized or diffuse, originating at the superior margin of the sinuses of Valsalva just above the level of the coronary arteries. The most common complication of supravalvular aortic stenosis is early onset of intimal hyperplasia and atherosclerosis of the coronary arteries. The coronary arterial lesions of supravalvular aortic stenosis are dilatation or coronary artery ostial obstruction. We experienced a case of supravalvular aortic stenosis combined with right coronary artery ostial obstruction. A 21 year-old female patient was admitted because of exertional dyspnea and chest pain for 2 months. Cardiac catheterization showed a narrowing of ascending aorta with prominent calcification in the lesion and moderate aortic valve insufficiency. The peak to peak left ventricular-supravalvular aortic pressure gradient was 54 mmHg. Selective coronary angiography revealed as a complete obstruction of the ostium of the right coronary artery. Surgical correction was performed successfully. Postoperative left ventricular-supravalvular aortic pressure gradient was decreased to 22 mmHg. Postoperative clinical course was favorable and she was discharged with good condition. We present a case of supravalvular aortic stenosis combined with right coronary artery ostial obstruction with a review of literatures.

Williams Syndrome with Megacoronary Artery

Supravalvular aortic stenosis is a rare cause of left ventricular outflow obstruction in adults. It occurs as an isolated defect sporadically or as a hereditary basis with an autosomal dominant trait without further phenotypical anomalies, or as a part of the Williams syndrome with mental retardation and multiple anomalies. In this report, we present a case of williams syndome associated with megacoronary artery.

A Case of Supravalvular Aortic Stenosis

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta whitch originates just distal to the level of the origins of the coronary arteries, and whitch includes a wide spectrum of pathologic changes. A 27 years-old-female was admitted because of further evaluation of known some heart disease. Clinical diagnosis of supravalvular aortic stenosis was made by echocardiography and angiography. We presented a case of supravalvular aortic stenosis with a review of literature.

A Case of Supravalvular Aortic Stenosis (Williams Syndrome)

Williams syndrome(Williams-Beuren Syndrome, Williams elfin facies syndrome) is a rare syndrome and this is the first case in Korea. We experienced a case of Williams syndrome who was a 11 year old male child. He showed a characteristic facial appearance of this syndrome. A systolic thrill was palpable over the upper sternal borders and in the suprasternal notch. A Grade 3 (on the basis of 6) ejection systolic murmur was heard maximally over the right and left upper sternal borders and radiated over the entire precordium and into the neck. No manifestations of congestive cardiac failure were present. Pectus excavatum and inguinal hernia were observed. he also had mental retardation (IQ=66). Diagnosis was established by angiocardiography, and also aided by EKG and phonocardiography. A brief review of the literatures was done.