Resolution of acquired pulmonary arteriovenous malformations in a patient with total anomalous systemic venous return.

An 11-year-old male with total anomalous systemic venous return had surgical repair except for the hepatic venous return, which drained to the left atrium. He developed progressive cyanosis and fatigue and was diagnosed with large pulmonary arteriovenous malformations (PAVMs) during cardiac catheterization with the use of bubble contrast echocardiography. After surgical redirection of hepatic venous flow to the right heart and pulmonary arterial system, resolution of these PAVMs was demonstrated clinically and by contrast echocardiography. This unique case report demonstrates the development of PAVMs with exclusion of hepatic venous return through the pulmonary vascular bed while pulsatile pulmonary blood flow remains intact. It reinforces the likelihood of the absence of an as yet unidentified hepatic vasoactive substance as the source for development of PAVMs.

Use of peak Doppler gradient across ventricular septal defects leads to underestimation of right-sided pressures in patients with "sloped" Doppler signals.

In patients with "sloped" appearance of the Doppler signal across a ventricular septal defect (VSD), the peak Doppler velocity seems to overestimate the catheterization-derived peak-to-peak gradient, resulting in underestimation of right-sided heart pressures. In 11 patients with sloped Doppler signals across the VSD, ventricular pressure tracings were compared with simultaneous recordings of the Doppler signal. The average peak Doppler gradient (40.2 +/- 19.2 mm Hg) overestimated the catheterization-derived peak-to-peak gradient (20.2 +/- 13.6 mm Hg) significantly (P < or =.001). Doppler mean gradient (20.2 +/- 11.3 mm Hg; P = ns) and end-systolic gradient (17.0 +/- 12.5 mm Hg; P < or =.05) were closer estimates of the catheterization peak-to-peak gradient. All Doppler gradients showed good correlation to the catheterization peak-to-peak gradient with r2 values of 0.77, 0.73, and 0.91. We conclude that Doppler mean or end-systolic gradients should be used for calculation of right-sided heart pressures in this patient population.

Course of right and left ventricular function in patients with pulmonary insufficiency after repair of Tetralogy of Fallot.

Surgical repair of tetralogy of Fallot (TOF) frequently results in pulmonary valve insufficiency. Nevertheless, no serial information is available on the long-term impact of the valvular insufficiency on right and left ventricular function. Right and left ventricular ejection fraction was measured serially by radionuclide angiocardiography in 21 patients with at least moderate pulmonary insufficiency after repair of TOF. A baseline study was obtained an average of 1.2 years after repair, and a follow-up study was performed an average of 10.2 years after surgery. Changes in ventricular function over time and deviations from the normal range were analyzed. At baseline evaluation the mean right ventricular ejection fraction (RVEF; 0.52 +/- 0.10) and left ventricular ejection fraction (LVEF; 0.68 +/- 0.10) were normal. At the time of follow-up the mean RVEF had significantly decreased to 0.45 +/- 0.09 (p < 0.01). The mean LVEF had decreased to 0.60 +/- 0.11 (p < 0.02). This change was independent of the RVEF (r = -0.13). Eleven patients (52%) had an abnormal RVEF or LVEF at follow-up. Nineteen patients (90%) showed a decrease of 0.05 or more in RVEF, LVEF, or both between studies. These data suggest a negative impact of long-standing pulmonary insufficiency on right and left ventricular systolic function after repair of TOF. Therefore, continued surveillance of biventricular function in this patient population appears warranted.

Successful balloon angioplasty of a coarctation in an infant <500 g.

We describe the first successful balloon angioplasty of a coarctation in a 460-g newborn infant with coarctation of the aorta and heart failure. A coronary angioplasty catheter was positioned across the coarctation via a transumbilical approach. The waist of the balloon disappeared on maximal inflation and there was an increase in blood pressure distal to the coarctation and the clinical status improved. A ductus arteriosus was ligated 4 days after angioplasty.

Stevens-Johnson syndrome and respiratory failure in a 9-year-old boy.

The etiology of respiratory failure associated with Stevens-Johnson syndrome may be multifactorial, including upper airway involvement, pneumothorax/pneumomediastinum, and direct involvement of the respiratory mucosa. Respiratory failure from direct involvement of the respiratory mucosa is relatively uncommon. We describe a 9-year-old boy who had respiratory failure associated with Mycoplasma pneumoniae-induced Stevens-Johnson syndrome. Bronchoscopic examination of the airways revealed sloughed mucosa, ulcerative lesions, and inspissated secretions indicative of lower airway involvement with Stevens-Johnson syndrome. Although the mainstay of therapy is supportive care with controlled ventilation, rigid bronchoscopy with bronchoalveolar lavage to clear the airways of the debris was an invaluable adjunct to this patient's care.

Cardiac emergencies in children.

Pediatric cardiac emergencies require very specific treatment in the emergency room setting. Considering the possibility of a cardiac problem as the cause for the presenting symptoms is the initial step in successful management. Many patients present with what is initially considered a primary pulmonary disorder such as pneumonia, asthma, or bronchiolitis. Airway stabilization and ventilatory support, if needed, remain the first steps in stabilizing the patient. Many neonates with acutely decompensating heart disease may require the patency of the ductus arteriosus for survival. Prostaglandin E given as continuous infusion is the treatment of choice. Congestive heart failure can present at any age. In older patients, it is often due to myocarditis and is characterized by low cardiac output. Supportive measures, fluid restriction, and inotropic support are the basic concepts for initial treatment. Supraventricular tachycardia is a frequent arrhythmia, especially in young children. If the patient is unstable, immediate intravenous administration of adenosine or synchronized cardioversion are the initial interventions. In stable patients, vagal maneuvers may be attempted to abort the arrhythmia.