Combined Supravalvular Aortic and Pulmonic Stenosis.

نویسندگان

  • M G BOURASSA
  • L CAMPEAU
چکیده

SIGNIFICANT outflow obstruction of both ventricles on a congenital basis is rare. Only 11 cases have been reported1-7 and, in most instances, the stenosis was either valvular or subvalvular on both sides. Supravalvular stenosis occurring in the pulmonary arteries or in the root of the aorta, although uncommon, has been reported frequently in recent years. The association of these two lesions in the same patient, however, has not, to our knowledge, been previously described. Two unusual cases recently reported by Soulie and co-workers" involved the coexistence of stenosis or hypoplasia of the primary pulmonary artery branches and hypoplasia of the thoracic and abdominal aorta, in the absence of supravalvular aortic stenosis. These authors also mention a similar case reported by Bulgarelli9 in which narrowing of the main pulmonary artery and its branches was associated with hypoplasia of the entire aorta. The purpose of this report is to describe the combination of supravalvular aortic stenosis and postvalvular pulmonic stenosis seen in three patients during the past 3 years and to stress the common features of the two conditions. Case Reports Case 1 S.M., a 16-year-old boy, was admitted to the hospital in February 1960, complaining of marked diminution of his exereise tolerance because of dyspnea, fatigue, cough, and hemoptysis. A heart murmur had been discovered at the age of 18 months, following an attack of eyanosis. At the age of 10 years, he began to experience dyspnea upon moderate exertion. After the age of 13, he was unable to participate in competitive athletics. Five months prior to admission, after running 200 yards, he experienced intense dyspnea and gross hemoptysis. From then on, he had bouts of hemoptysis almost daily following slight exer-

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Balloon dilation of congenital supravalvular pulmonic stenosis in a dog

Percutaneous balloon valvuloplasty is considered the standard of care for treatment of valvular pulmonic stenosis, a common congenital defect in dogs. Supravalvular pulmonic stenosis is a rare form of pulmonic stenosis in dogs and standard treatment has not been established. Although, there have been reports of successful treatment of supravalvular pulmonic stenosis with surgical and stenting t...

متن کامل

Calcific pulmonic stenosis.

C ALCIFIC STENOSIS of the aortic valve is a well-recognized and relatively common clinical and pathological entity. In contrast, calcific pulmonic stenosis is rarely encountered either at operation or necropsy. We have observed small deposits of calcium in one or more pulmonic valve cusps in patients who lived into adulthood with left-toright intracardiac shunts, and in older persons with sever...

متن کامل

Combined percutaneous treatment of atrial septal defect and pulmonic or aortic stenosis in adult patients

Combined atrial septal defect and pulmonic or aortic stenosis are relatively uncommon conditions in adult patients, with few reported cases of percutaneous treatment. We present two patients with secundum type atrial septal defect and concomitant pulmonic or aortic stenosis and their treatment by transcatheter techniques.

متن کامل

Computerized Tomography Use in Williams–Beuren Syndrome Aortopathy

Williams-Beuren syndrome is a multisystem genetic disorder caused by hemizygous deletion on chromosome 7q11.23, encompassing about 28 genes including the elastin gene, ELN. Cardiovascular abnormalities are frequent and are related to elastin insufficiency. These abnormalities include supravalvular aortic stenosis (SVAS) in 70% of case, pulmonic valve stenosis, and renal artery stenosis. Definit...

متن کامل

An atypical 7Q11.2-Q21.11 deletion in a Williams-Beuren syndome patient

Results The proband, a female neonate, is the first child of healthy nonconsanguineous Chinese parents. She was born by uterine-incision delivery with intrauterine distress after 41 weeks of gestation. Her birth weight was 2.4 kg. She showed an distinctive facies including broad brow, periorbital fullness, epicanthal folds, short nose, long philtrum, small jaw and prominent earlobes. The cardio...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Circulation

دوره 28  شماره 

صفحات  -

تاریخ انتشار 1963