PubMed Health⌕ Search

Biomedical subjects

James W Ziegler

Publications and source records attributed to James W Ziegler.

2 recordsLinked to original sources

Intracardiac yolk sac tumor and dysrhythmia as an etiology of pediatric syncope.

This report describes a new etiology of pediatric syncope. Epilepsy, brain anomalies, infection, electrolyte abnormalities, and trauma are commonly identified etiologies of seizures in the pediatric population. We report here a child with third-degree heart block and right ventricular outflow tract obstruction related to an intracardiac tumor presenting with syncope and seizure-like activity. Echocardiography revealed a large (3 x 8-cm) intracardiac mass filling the right atrium, extending across the tricuspid valve into the right ventricle and crossing the atrial septum into the left atrium, extending into the left ventricular outflow tract. She underwent emergent cardiopulmonary bypass with removal of the majority of the tumor mass, clearing both the left and right ventricular outflow tracts of obstruction and repairing the tricuspid valve. Postoperative cardiac conduction remained blocked and required permanent pacing. The initial serum alpha-fetoprotein level was grossly elevated, and the tumor showed characteristic histopathologic features of a yolk sac tumor. Four years after the completion of her chemotherapy, she remains clinically well, with no evidence of recurrent tumor by echocardiography or radiographic studies, and her alpha-fetoprotein remains in the normal range. The clinical manifestations of tumor infiltration of the heart with complete heart block resulting in loss of consciousness with tonic-clonic movements are detailed. Although rare, cardiac syncope has multiple known causes and should be suspected in any patient with sudden loss of consciousness and pallor. In the pediatric population, cardiac rhythm disturbances are typically the result, rather than the cause, of acute cardiac emergencies. Pediatricians should be aware of depressed cardiac output and dysrhythmias as etiologies of new-onset syncope. Evaluation should include a cardiac assessment with electrocardiogram to exclude a life-threatening arrhythmia as a potential cause.

Electrocardiography↗

Hepatic dysfunction following the Fontan procedure.

OBJECTIVES: The Fontan procedure offers a palliation for the hemodynamic derangements associated with congenital heart lesions characterized by a single functional ventricle, but it causes a chronically elevated systemic venous pressure that may result in hepatic congestion. The objective of this study was to characterize hepatic function and its relationship to cardiac function in children who had undergone the Fontan procedure. METHODS: In a cross-sectional study of 11 children aged 38 months to 216 months (mean, 149 months), the authors evaluated indices of cardiac and hepatic function, including galactose clearance, Doppler hepatic ultrasonography, synthetic function, and markers of liver injury, at 9 months to 176 months (mean, 100 months) after children had undergone the Fontan procedure. RESULTS: The most common biochemical abnormality of hepatic function was a prolongation of the prothrombin time and a low factor V level. There was a trend toward progressive abnormality in prothrombin time with increasing interval since the Fontan procedure. Galactose elimination half-life and galactose elimination capacity were inversely correlated with the time after Fontan (R2= 0.65, P = 0.004). There was no relationship between cardiac functional measurements and liver function. CONCLUSIONS: Prothrombin time and galactose elimination half-life are abnormal in children who have undergone the Fontan procedure and may be useful markers of hepatic function in the longitudinal assessment of these patients.

Adolescent↗