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Protein-losing enteropathy (PLE) is a serious complication of the Fontan operation and is associated with pronounced mortality. Medical management of PLE has been only partially successful. A recent report noted dramatic improvement in patients with PLE within 3 weeks of subcutaneous administration of heparin. We report a case of reversal of PLE with resolution of clinical symptoms and normalization of serum albumin, total protein, and fecal alpha1-antitrypsin values after several months of heparin treatment. Our findings substantiate those recently reported but suggest that reversal of PLE may necessitate more than a few weeks of heparin therapy.

Protein-losing enteropathy (PLE) after the Fontan operation is a life-threatening complication that may be refractory to medical therapy. Herein we describe a percutaneous atrial fenestration that was performed in a 42-year-old man with a double-inlet left ventricle who had undergone a Fontan operation 9 years earlier. Severe PLE developed, and despite frequent infusions of protein, his albumin level was 1.8 g/dL. The diagnosis of PLE was confirmed by an alpha(1)-antitrypsin clearance of 425 mL in 24 hours (normal 27 or less). Percutaneous atrial fenestration resulted in dramatic clinical improvement and resolution of the PLE. At 5-month follow-up, the patient's albumin level was 4.2 g/dL, his alpha(1)-antitrypsin clearance was normal, and he was free of ascites and edema.

Objective: The current study was undertaken to assess the frequency of excessive enteric protein loss and protein-losing enteropathy in the relatively early period after the Fontan operation., Design: Protein excretion was determined in 26 of 27 consecutive patients who underwent the Fontan procedure between January and June 1990 at the Mayo Clinic., Material and Methods: At two testing intervals during the first 4 months after the Fontan operation, alpha 1-antitrypsin clearance and fecal alpha 1-antitrypsin concentration studies were done., Results: All results were normal for the first postoperative test period (2 to 8 weeks). For the second study period, all 17 patients tested had normal alpha 1-antitrypsin clearances. One of the 17 patients had an appreciably increased fecal alpha 1-antitrypsin concentration and transient protein-losing enteropathy., Conclusion: Excessive enteric protein loss and protein-losing enteropathy are relatively uncommon during the first 4 months after the Fontan operation.

Patients with various types of congenital heart disease were contacted 25 years after their original examination at the Mayo Clinic. In addition to providing their current health status, level of education achieved, and current occupation, they were asked to complete a detailed standardized questionnaire to assess their degree of psychologic stress. Of the original 463 patients, 168 completed and returned the psychologic questionnaires. These patients had evidence of psychologic stress in excess of that expected on the basis of normative data. Furthermore, the degree of stress was unrelated to the clinical severity of the original cardiac defect. In addition, the psychologic stress occurred despite "success" as defined by educational achievement and occupational level. One can speculate that as children these patients were exposed to environmental stresses that may well have been colored by parental attitudes and perceptions.

Twenty-six normal newborns (13 male, 13 female) with normal prenatal histories, no perinatal stress, and normal vaginal deliveries had creatine phosphokinase (CPK) activity and isoenzyme activities assayed in cord blood and in 24-hour postpartum serum. Total CPK activity was high in cord blood when compared with adult control values. Moreover, the total CPK was significantly higher in serum at 24 hours of age compared with cord blood. There was a significant increase in both the skeletal muscle isoenzyme and the cardiac muscle isoenzyme from birth to 24 hours of age. There was a decrease in the brain isoenzyme at 24 hours of age which was not statistically significant. These results were compared with values obtained in a group of 10 neonates with severe cardiac problems. Three of the ill neonates had significant elevation of total serum CPK and the skeletal muscle isoenzyme when compared with the normal newborns. There were no significant differences between the normal infants and the ill neonates for the cardiac isoenzyme and the brain isoenzyme. These data suggest that caution should be used in the diagnosis of certain neonatal cardiac syndromes based on serum CPK levels and isoenzyme alone.

A series of 72 patients with proved double-outlet right ventricle was studied with reference to the ability of angiocardiography to determine the location of a ventricular septal defect when it was associated with various great-artery relationships. Only 2 of the 72 patients had an intact ventricular septum. The other 70 patients had 16 possible anatomic variations based on four positions of the ventricular septal defect (subaortic, subpulmonary, subaortic and subpulmonary, and remote type) and four great-artery relationships (normal, side by side, dextromalposition, and levomalposition). Using the hemodynamic information regarding systemic and pulmonary arterial saturations, combined with biplane angiocardiographic data from the right ventricle (and if possible with left ventriculography), one can predict the location of ventricular septal defect. Because the different types of double-outlet right ventricle have different surgical approaches, this information can be important to the surgeon.

Three cases of neonatal pneumopericardium are presented. Detailed ventilatory measurements and chest roentgenographic changes are described for each of the three patients. The literature of neonatal pneumopericardium is reviewed, and based on that review and our own cases, we offer guidelines for managing these patients.

Two patients--one an infant and the other an adolescent--with transposition of the great arteries and hemophilia underwent a successful major reconstructive open-heart surgical procedure. Despite a severe coagulation deficiency, a major reconstructive cardiac operation was performed with the use of a detailed replacement plan, which took into consideration the potency of the replacement material and its potential hazards, biologic half-life, and anticipated in vivo recovery on the basis of the patient's plasma volume. To our knowledge, these are the first reports of successful surgical repair of this type in patients with severe coagulation factor deficiency.

Aortico-left ventricular tunnel is a rare congenital cardiac lesion that often results in severe aortic insufficiency in infancy. In previously reported cases, the diagnosis has often been missed initially despite use of cardiac catheterization and angiography. We describe a patient who underwent successful surgical correction of this malformation at 10 months of age after the diagnosis had been established by use of two-dimensional echocardiography, color flow Doppler imaging, and magnetic resonance imaging. Aortico-left ventricular tunnel can be diagnosed by use of these noninvasive means without cardiac catheterization.

This is a detailed clinical and autopsy documentation of a rare entity--primary cardiac myxosarcoma in a 29-month-old girl. The patient had sudden onset of right hemiplegia and angiographic evidence of multiple occlusions of the left middle cerebral artery. Subsequent M-mode and sector echocardiography showed a mobile, pedunculated left atrial tumor, which was excised. No other tumor mass was noted at the time of surgical exploration, and postoperatively, the patient received a course of chemotherapy (vincristine, dactinomycin, and cyclophosphamide). After a temporary improvement in her condition, the patient died following several days of rapid deterioration; this was 3 months after the onset of symptoms. Autopsy showed that death was due to brainstem herniation secondary to massive infiltration of the brain by tumor, and there were also widespread systemic metastases.

The use of the percutaneous subclavian approach for permanent tranvenous cardiac pacing is described in an 8.6-kg, 18-month-old child with symptomatic congenital heart block. This approach may facilitate the use of permanent transvenous cardiac pacing in infancy and childhood.