DANPY (dimethylaminonaphthylpyridinium): an economical and biocompatible fluorophore.

Dyes with nonlinear optical (NLO) properties enable new imaging techniques and photonic systems. We have developed a dye (DANPY-1) for photonics applications in biological substrates such as nucleic acids; however, the design specification also enables it to be used for visualizing biomolecules. It is a prototype dye demonstrating a water-soluble, NLO-active fluorophore with high photostability, a large Stokes shift, and a favorable toxicity profile.

Development of quality metrics for ambulatory care in pediatric patients with tetralogy of Fallot.

Objective: The objective of this study was to develop quality metrics (QMs) relating to the ambulatory care of children after complete repair of tetralogy of Fallot (TOF).

Design: A workgroup team (WT) of pediatric cardiologists with expertise in all aspects of ambulatory cardiac management was formed at the request of the American College of Cardiology (ACC) and the Adult Congenital and Pediatric Cardiology Council (ACPC), to review published guidelines and consensus data relating to the ambulatory care of repaired TOF patients under the age of 18 years. A set of quality metrics (QMs) was proposed by the WT.

Development of Quality Metrics in Ambulatory Pediatric Cardiology.

The American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Section had attempted to create quality metrics (QM) for ambulatory pediatric practice, but limited evidence made the process difficult. The ACPC sought to develop QMs for ambulatory pediatric cardiology practice. Five areas of interest were identified, and QMs were developed in a 2-step review process.

Intracardiac tumor causing left-ventricular outflow-tract obstruction in a newborn.

The following report describes the case of newborn girl with an asymptomatic systolic murmur, which on imaging revealed a nearly obstructive mass in the left-ventricular outflow tract. The mass was resected and found to be consistent with a rhabdomyoma. Here, we describe the pathologic and clinical characteristics of this tumor.

Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela.

Objective: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing.

Background: Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects.

Digitalis use in children: an uncertain future.

The therapeutic indications for digoxin in the treatment of children with large left-to-right shunts continue to be reassessed. New insights into the alterations in cardiac function imposed by this hemodynamic burden have shown preserved systolic performance. Pharmacological interventions that improve cardiac output by afterload reduction or other modalities have proven useful and potentially have low risk for serious toxicity.

Treatment of transposition of the great arteries.

Today, the arterial switch operation is the treatment of choice for patients with transposition of the great arteries. Midterm follow-up confirms the initial hopes for good systemic ventricular function and lack of arrhythmias. A two-stage operation can be performed in patients seen after 1 month of age or in subjects who have had an atrial switch operation and with late systemic dysfunction.

Cervical aortic arch with aneurysm formation.

Two pediatric cases of cervical aortic arch with aneurysm formation are reported. Only female patients with cervical aortic arch have developed aneurysms, which may influence risk counseling of such patients.

Recent advances in the diagnosis and treatment of coarctation of the aorta.

Although it has been the topic of intense medical and surgical attention for over 50 years, coarctation of the aorta continues to be a major cause of cardiovascular morbidity and mortality in infants and children. Refinements in established diagnostic modalities--primarily fetal, transesophageal, and intravascular echocardiography--have improved pre- and post-treatment assessments. Aggressive and early intervention, whether by surgery, catheter, or both, have been shown to increase initial success; the results of long-term follow-up are yet to be determined.

Controlled trial to evaluate protection of high-risk infants against respiratory syncytial virus disease by using standard intravenous immune globulin.

We performed a randomized, controlled trial of intravenous immune globulin (respiratory syncytial virus [RSV] neutralizing [Nt] antibody titer of 1:950 in 5% solution) to evaluate protection against RSV-induced disease over two respiratory virus seasons. Forty-nine children (mean age at enrollment, 4.5 months) with severe congenital heart disease or bronchopulmonary dysplasia were randomized as follows.

Pulsed Doppler echocardiographic and color flow imaging detection of retrograde filling of anomalous left coronary artery from the pulmonary artery.

We report a case in which pulsed wave Doppler echocardiography and color flow imaging of blood flow direction in an anomalous coronary artery from the pulmonary artery assisted in the correct diagnosis and confirmed the adequacy of the surgical correction. Low-velocity color scales were used to show retrograde filling of the left coronary artery before surgery and antegrade filling of the left coronary artery after surgery. Detecting direction of blood flow in coronary arteries should increase the accuracy of the noninvasive diagnosis of anomalous left coronary artery from the pulmonary artery.

Clinical and noninvasive hemodynamic results after aortic balloon valvuloplasty for aortic stenosis.

Balloon valvuloplasty has been shown to be an effective treatment for adults with aortic stenosis, typically providing a 50 to 80% increase in aortic valve area and marked improvement in exertional dyspnea, angina and syncope. However, the duration of this hemodynamic and clinical improvement is uncertain. Forty-two patients were followed for 10.

Use of the postductal PaO2 as a predictor of pulmonary vascular hypoplasia in infants with congenital diaphragmatic hernia.

Infants with congenital diaphragmatic hernia (CDH) demonstrate a wide range of anatomic and physiologic abnormalities that result in decreased pulmonary perfusion. We have used the patients' ability to achieve at least one postductal PaO2 greater than 100 torr while on maximal ventilation with 100% oxygen during the first 24 hours of life as the clinical marker to identify the degree of pulmonary perfusion. Patients were grouped as follows: group 1 had at least one postductal PaO2 greater than 100 torr, and group 2 patients never had a postductal PaO2 above 100 torr.

Midterm results after the arterial switch operation for transposition of the great arteries with intact ventricular septum: clinical, hemodynamic, echocardiographic, and electrophysiologic data.

Although the short-term results of atrial level repair of transposition of the great arteries (TGA) are satisfactory, longer follow-up has disclosed a significant incidence of systemic right ventricular dysfunction and rhythm disturbances. The arterial switch operation (ASO) may represent a major improvement by restoring the left ventricle as the systemic ventricle and avoiding extensive atrial surgery. We have prospectively evaluated 49 consecutive survivors of ASO for TGA with intact ventricular septum (IVS) by clinical examination, echocardiography, cardiac catheterization, ambulatory electrocardiographic monitoring, and invasive electrophysiologic studies.

Late results in patients with tetralogy of Fallot repaired during infancy.

Between 1973 and 1985, 220 infants (age 1 day to 18 months, mean = 7 months) underwent repair of tetralogy of Fallot (TOF) or TOF/pulmonary atresia with 17 early deaths. Follow-up status was ascertained for 184 of 203 survivors (91%) at a postoperative interval of 2 to 185 months (mean = 60 months). Significant residual hemodynamic lesions included ventricular septal defect in three, and right ventricular outflow gradient in excess of 40 mm Hg in 24.

Dual balloon technique for valvuloplasty of aortic stenosis in adults.

A dual balloon technique was studied in 16 patients with aortic stenosis in whom results with a single balloon (up to 20 mm, 5.5 cm or 25 mm, 3.0 cm in diameter and length, respectively) were judged to be suboptimal.

Mechanism of aortic balloon valvuloplasty: fracture of valvular calcific deposits.

Balloon valvuloplasty has been shown to acutely reduce the hemodynamic and symptomatic severity of calcific aortic stenosis. The mechanism by which this improvement is accomplished is not known. At necropsy, three patients who died after hemodynamically successful aortic balloon valvuloplasty were found to have aortic valve calcific deposits fractured at one or more sites.

Cardiovascular findings in adolescent inpatients with anorexia nervosa.

Left ventricular function, resting electrocardiograms, and Holter recordings were systematically examined in 25 consecutively hospitalized, seriously ill, emaciated adolescents with anorexia nervosa. We failed to observe serious arrhythmias, abnormal prolongation of QT interval, conduction abnormalities, or depression in left ventricular systolic function.

In vitro analysis of mechanisms of balloon valvuloplasty of stenotic mitral valves.

Preliminary reports indicate that percutaneous balloon valvuloplasty is efficacious for treatment of mitral stenosis. The present study was designed to evaluate whether anatomic features of stenotic mitral valves in older adults affect the efficacy of balloon valvuloplasty and to determine the mechanism by which increased orifice area is accomplished. Fifteen mitral valves excised intact at the time of mitral valve replacement from patients with no more than 2+/4+ mitral a regurgitation were selected for study.

Treatment of calcific aortic stenosis by balloon valvuloplasty.

Recent reports have established the feasibility of using balloon valvuloplasty to reduce left ventricular outflow tract obstruction due to a calcified aortic valve. The present study summarizes experiences with this technique in 9 patients (7 women, 2 men, mean age 78 years) in whom balloon valvuloplasty was used to treat calcific aortic stenosis. Peak aortic valve gradient (mm Hg) decreased from 68 +/- 8 (mean +/- standard error of the mean) before valvuloplasty to 35 +/- 5 after valvuloplasty (p = 0.

Cor triatriatum associated with partial anomalous pulmonary venous connection to the coronary sinus: echocardiographic and angiocardiographic features.

An infant girl is described who had cor triatriatum and partial anomalous pulmonary venous connection of the left pulmonary veins to the coronary sinus, the first report of this combination of lesions. The infant also had a Dandy-Walker malformation and multiple facial and intrathoracic hemangiomas. The cardiac diagnosis was made by two-dimensional echocardiography.

Results of the Senning operation for transposition of the great arteries with intact ventricular septum in neonates.

In spite of an adequate balloon atrial septostomy, some neonates (less than 28 days of age) with transposition of the great arteries and intact ventricular septum (TGA, IVS) remain hypoxemic and acidotic and require operative treatment. Our entire experience with the Senning operation in neonates with TGA, IVS is reviewed. From March 1978 to March 1983 there were 104 infants who underwent the Senning operation for TGA, IVS.