Estenose aórtica supravalvar em adulto com anomalia de vasos da base e insuficiência aórtica / Supravalvular aortic stenosis in adult with anomalies of aortic arch vessels and aortic regurgitation

A estenose aórtica supravalvar é uma rara cardiopatia congênita, bastante incomum em adultos. Apresentamos um caso de estenose aórtica supravalvar em adulto com anomalia de vasos do arco aórtico, já com presença de insuficiência aórtica importante, tratado com êxito por meio de plastia da aorta ascendente e troca valvar aórtica.

The supravalvular aortic stenosis is a rare congenital heart defect being very uncommon in adults. We present a case of supravalvular aortic stenosis in adult associated with anomalies of the aortic arch vessels and aortic regurgitation, which was submitted to aortic valve replacement and arterioplasty of the ascending aorta with a good postoperative course.

Puérpera com trombose de prótese mecânica e estenose supravalvar aórtica adquirida / Postpartum patient with thrombosis of mechanical prostheses and acquired supravalvular aortic stenosis

A hipercoagulabilidade sanguínea proporcionada na gravidez aumenta consideravelmente a incidência de trombose de valvas mecânicas. A estenose supravalvar aórtica adquirida é extremamente rara. Relata-se o caso de uma puérpera imediata, portadora de prótese mecânica aórtica e estenose supravalvar aórtica adquirida, submetida à cirurgia cardíaca de emergência, com instabilidade hemodinâmica grave, por meio de técnica operatória adaptada para a correção da estenose supravalvar aórtica, com evolução clínica e resultados ecocardiográficos pós-operatórios satisfatórios.

The blood hypercoagulability in pregnancy increases significantly the incidence of thrombosis of mechanical valves. Acquired supravalvular aortic stenosis is extremely rare. We report the case of an immediate postpartum patient with aortic mechanical prostheses and acquired supravalvular aortic stenosis who underwent emergency heart surgery, with severe hemodynamic instability, using adapted surgical technique for correction of supravalvular stenosis with satisfactory clinical and echocardiography results.

Correção cirúrgica da estenose aórtica supravalvar com modificação da técnica de Sousa / Supravalvular aortic stenosis surgical repair using modified Sousa's technique

Relato de uma paciente de sete anos apresentando progressiva intolerância ao esforço. A criança apresentava taquicardia e sopro pansistólico de maior intensidade no foco aórtico. O ecocardiograma evidenciava hipertrofia ventricular esquerda e estenose aórtica supravalvar com gradiente sistólico de 190 mmHg. A angioressonância e o cateterismo cardíaco confirmaram o diagnóstico, sugerindo estenose do óstio da artéria coronária esquerda. A correção foi realizada modificando a técnica descrita por Sousa. A evolução pósoperatória transcorreu sem intercorrências, com gradiente pós-operatório de 23 mmHg e boa mobilidade da valva aórtica.

Report of a patient with seven years old and effort intolerance progressing. The child had tachycardia, pansystolic murmur in the aortic focus. Echocardiography showed left ventricle hypertrophy and aortic narrowing at the level of sinotubular junction with sistolic gradient of 190 mmHg. Cardiac catheterization confirmed the diagnostic suggesting left coronary ostium stenosis. The surgery was performed modification the technique, described by Sousa. Immediate post-operative has presented no complications, with gradient of 23 mmHg, and good leaflets mobility.

Sudden cardiac death under anesthesia in pediatric patient with williams syndrome: A case report and review of literature.

Williams syndrome is a complex syndrome characterized by developmental abnormalities, craniofacial dysmorphic features, and cardiac anomalies. Sudden death has been described as a very common complication associated with anesthesia, surgery, and procedures in this population. Anatomical abnormalities associated with the heart pre-dispose these individuals to sudden death. In addition to a sudden and rapid downhill course, lack of response to resuscitation is another significant feature seen in these patients. The authors report a five-year-old male with Williams syndrome, hypothyroidism, and attention deficit hyperactivity disorder. He suffered an anaphylactic reaction during CT imaging with contrast. Resuscitation was unsuccessful. Previous reports regarding the anesthetic management of patients with Williams are reviewed and the potential for sudden death or peri-procedure related cardiac arrest discussed in this report. The authors also review reasons for refractoriness to defined resuscitation guidelines in this patient population.