SummaryBackground and hypothesis: Valvar pulmonary stenosis is a common congenital heart defect. Progression of stenosis over time, even when mild initially, has been shown by serial cardiac catheterization studies in children and adults. We studied the natural history of asymptomatic valvar pulmonary stenosis diagnosed in infancy with two-dimensional echocardiography and Doppler method.Method.v: Between November 1986 and March 1993,51 infants in the Northeast Tennessee and Southwest Virginia region were clinically diagnosed to have isokated valvar pulmonary stenosis. In 40 patients, the diagnosis was confirmed by two-dimensional echocardiogram/Doppler and color-flow mapping study at the time of presentation, and only their course is reported. Of 40 infants, six asymptomatic infants ( 15%) showed rapid progression of pulmonary stenosis over a relatively short period of time. Within the first 6 months of life, three of the six infants showed worsening of the stenosis needing intervention (one had surgical valvectomy and the others had percutaneous balloon valvuloplasty). The three other infants showed a more gradual increase of pulmonary stenosis over the fist 2 years of life.Results: Pulmonary stenosis even when mild can worsen in infancy, and it is not possible to predict which patients will follow this course. In our group of asymptomatic infants with initial mild pulmonary stenosis, 15% developed significant stenosis that needed intervention.Conclusion: We recommend frequent follow-up of asymptomatic infants with mild pulmonary stenosis during the first 2 years of life to detect rapid progression that may need intervention.

We report our experience with asymptomatic valvar aortic stenosis diagnosed during infancy. During the period between November 1, 1986 and October 31, 1992 a total of 13 infants were diagnosed with asymptomatic aortic stenosis, 5 of whom showed rapid progression over the first 2 years of life. Two of these asymptomatic infants had severe aortic stenosis by 2 months of age, requiring intervention. We agree that aortic stenosis is a progressive lesion even in mild cases, but we emphasize the need for close clinical and echocardiographic follow-up of these asymptomatic children during infancy to prevent congestive heart failure and sudden death.

The Children's Heart Center, Atlanta, 83 infants with congenital heart defects were diagnosed by echocardiography and underwent surgery without cardiac catheterization. The diagnostic categories included 46 infants with left heart obstructive lesions, 19 infants with cyanotic heart lesions, and 18 infants with miscellaneous lesions. Fortyfive infants (55%) underwent surgery under cardiopulmonary bypass. There were three errors in diagnosis, yielding a diagnostic accuracy of 95%. Many infants with congenital heart disease can be accurately and completely diagnosed by echocardiography and can safely undergo surgery without cardiac catheterization. upright imaging. The latter has permitted echocardiography-angiography-heart specimen correlation and has thereby greatly enhanced appreciation of echocardiography.Similarly, at The Children's Heart Center, the credibility of echocardiography was established by extensive angiography+chocardiography correlation. Referral of selected patients to surgery with an echocardiographic diagnosis followed. This proved to be successful, and as our confidence increased a great variety of lesions were submitted for surgery. Currently, echocardiography is used reliably in our institution to make a complete and categorical diagnosis of structural congenital heart disease.In this report we describe our experience with use of echocardiography in the surgical management of patients with congenital heart disease. Key words: echo, congenital infant heart surgery Methods IntroductionSince the early 1980s, several centers1-' have reported successful submission of patients with congenital heart defects to surgery based on echocardiographic diagnosis. This has been possible due to many factors including (a) application of segmental approach to obtain complete anatomical diagnosis, (b) delineation of hemodynamics by pulse wave, continuous wave, and color flow Doppler, and (c) On review of the surgical records between January 1988 and August 1991 of the Emory Department of Cardiothoracic Surgery, 83 infants under 6 months were identified as having had cardiac surgery based on echocardiographic diagnosis. These infants had previously undergone exhaustive echocardiograms using orthogonal sweeping techniques from subcostal, parasternal, and suprasternal windows. A complete anatomical diagnosis was sought in each by employing the segmental approach. All relevant hemodynamic information was obtained by pulse wave, continuous wave, and color flow Doppler. Appropriate measurements of chambers and great vessels was made from the two-dimensional image. The studies were stored on 1/2" VHS tapes and reviewed collectively by the echocardiologist, the attending cardiologist, and the surgeon. The patients were referred for surgery only when a complete anatomical diagnosis and relevant hemodynamic and functional information had been obtained to the satisfaction of the reviewers. Frequently, echocardiograms were repeated for clarification or for obtaining additional information. If, however, any uncertainty exis...

Propranolol, a first-generation nonselective beta-adrenoceptor blocking agent, is commonly used to treat pediatric arrhythmias. Atenolol, relatively long-acting, cardioselective beta-adrenoceptor blocking agent, has been successfully used in adults with supraventricular tachycardia (SVT). There is only one report on the use of atenolol in children with SVT; and our report is on the first long-term prospective study to evaluate the use of atenolol in children. A group of 22 children < 18 years of age with clinical SVT were enrolled in the study. The tachycardia was documented on electrocardiograms in each case and was confirmed by electrophysiologic studies in some. Once-a-day oral atenolol was started as a monotherapy. Of the 22 children with various types of SVT, 13 (59%) were well controlled on long-term oral atenolol therapy. The effective dose of atenolol ranged between 0.3 and 1.3 mg/kg/day (median effective dose 0.7 mg/kg/day). Five children had some adverse effects. However, none in the successful group of 13 patients required drug discontinuation because of such effects. Once-a-day oral atenolol as a monotherapy is effective and relatively safe for long-term management of SVT during childhood. It is an attractive alternative beta-adrenoceptor blocking agent for the management of pediatric arrhythmias.

Propranolol, a first-generation nonselective beta-adrenoceptor blocking agent, is commonly used to treat pediatric arrhythmias. Atenolol, relatively long-acting, cardioselective beta-adrenoceptor blocking agent, has been successfully used in adults with supraventricular tachycardia (SVT). There is only one report on the use of atenolol in children with SVT; and our report is on the first long-term prospective study to evaluate the use of atenolol in children. A group of 22 children < 18 years of age with clinical SVT were enrolled in the study. The tachycardia was documented on electrocardiograms in each case and was confirmed by electrophysiologic studies in some. Once-a-day oral atenolol was started as a monotherapy. Of the 22 children with various types of SVT, 13 (59%) were well controlled on long-term oral atenolol therapy. The effective dose of atenolol ranged between 0.3 and 1.3 mg/kg/day (median effective dose 0.7 mg/kg/day). Five children had some adverse effects. However, none in the successful group of 13 patients required drug discontinuation because of such effects. Once-a-day oral atenolol as a monotherapy is effective and relatively safe for long-term management of SVT during childhood. It is an attractive alternative beta-adrenoceptor blocking agent for the management of pediatric arrhythmias.