Pleuroperitoneal shunts for refractory chylothorax after operation for congenital heart disease.

Between 1980 and 1990, 10 of 12 children with a symptomatic chylothorax after operation for congenital heart disease failed to respond to traditional medical therapy (thoracentesis, tube thoracostomy, low-fat diet). All 10 patients underwent placement of a pleuroperitoneal shunt, with complete resolution of the chylothorax in 9 patients (90%). Cardiac catheterization, performed before placement of the pleuroperitoneal shunt in 5 patients, demonstrated elevated right atrial pressure in all patients (range, 10 to 25 mm Hg). The pleuroperitoneal shunt functioned effectively in 4 patients with moderately elevated right atrial pressures (range, 10 to 16 mm Hg; median, 13.5 mm Hg) but not in 1 patient with a right atrial pressure of 25 mm Hg. Pleuroperitoneal shunting as treatment for chylothorax after operation for congenital heart disease is safe and effective, even in the face of moderate elevations in right atrial pressure.

Intrapericardial teratoma causing nonimmune hydrops fetalis and pericardial tamponade: a case report.

A case of fetal anasarca secondary to an intrapericardial teratoma is reported. The clinical, echocardiographic, and histologic features are described, along with a review of intrapericardial lesions.

Internal mammary artery bypass after the arterial switch operation.

Myocardial ischemia may ensure after coronary artery translocation during the arterial switch operation. We report the successful use of a right internal mammary artery to right coronary artery bypass graft in an infant with angiographic documentation of persistent graft patency 6 months postoperatively.

Incidence and risk of reintervention after coarctation repair.

We examined the need for intervention after coarctation repair in a retrospective study of 197 procedures performed between 1967 and 1989. Reintervention was required in 23 patients. No technique of coarctation repair was free from complications. Although there were only two stenoses in the group receiving Dacron patch angioplasty, only seven of these procedures were performed in children under the age of 1 year. The risk of stenosis was inversely correlated to the age at primary repair, with children less than 1 year old being at greater risk than those more than 1 year of age (p less than 0.05). Subclavian flap angioplasty had a lower risk of reoperation than end-to-end anastomosis (p less than 0.02). Formation of true aneurysms was confined to the Dacron patch angioplasty group. The morbidity and mortality for reintervention was low in all groups, with only one procedure-related death and no incidence of paraplegia. Although no technique is free from risk, subclavian flap angioplasty leads to fewer reinterventions in younger patients.

Interrelationship of atrial natriuretic peptide, atrial volume, and renal function in premature infants.

Infants experience dramatic changes in fluid balance during the first few days of life, which provides an opportunity to observe the interrelationships of changing atrial size, atrial natriuretic peptide (ANP) secretion, and renal function during a relatively short period. To study these relationships, we examined nine infant boys (mean birth weight 1180 gm and gestational age 30 weeks) at 20 to 28 hours of age and then at four 24-hour intervals. Measurements included plasma ANP concentration, two-dimensional echocardiographic estimations of left and right atrial volumes, Doppler determination of ductus arteriosus patency, creatinine clearance, urine flow rate, urinary sodium excretion, and cyclic guanosine monophosphate (cGMP) excretion. Plasma ANP concentration was found to decrease with age and to correlate with decreasing size of the right atrium, closure of the ductus arteriosus, urinary cGMP excretion, and sodium excretion. We speculate that elevated plasma ANP values in a preterm neonate reflect an expanded volume state. As volume contraction, reflected by decreasing atrial volume and body weight occurs, ANP levels decrease, which may diminish diuresis. These findings are compatible with a significant role for ANP in volume homeostasis of newborn infants.

Treatment of absent pulmonary valve syndrome with homograft.

We report the successful treatment of absent pulmonary valve syndrome in an infant. The treatment consisted of intracardiac repair of pulmonary regurgitation with a homograft valve, which allowed for early extubation and survival.

Usefulness of adenosine for arrhythmias in infants and children.

Adenosine was administered to 25 infants and children (11 patients after presenting with a sustained arrhythmia, and 14 during a diagnostic electrophysiologic study) to determine its electrophysiologic effects. Adenosine was given as an intravenous bolus (starting dose 37.5 micrograms/kg, and increased by 37.5 micrograms/kg increments until an effect was seen). Adenosine caused tachycardia termination or transient increased atrioventricular (AV) block in all 25 patients. Seven patients had tachycardia requiring only the atria for perpetuation and developed increased AV nodal block (minimum effective adenosine dose range 37.5 to 350 micrograms/kg, mean 131). Thirteen had AV reciprocating tachycardia or AV node reentry tachycardia (minimum effective adenosine dose range 37.5 to 225 micrograms/kg, mean 114). Four other patients received adenosine to rule out preexcitation (minimum effective adenosine dose range 37.5 to 375 micrograms/kg, mean 165). One of the 25 patients had junctional ectopic tachycardia and adenosine administration caused retrograde AV block. Six of the 25 (24%) had noticeable but minor side effects. One patient had sustained bradycardia (2 to 3 minutes requiring temporary pacing). Adenosine is a safe and effective agent in the evaluation and treatment of infants and children with arrhythmias.

Kawasaki syndrome.

Kawasaki syndrome is a recently recognized acute febrile illness of childhood. The etiology is unknown, and diagnosis depends on fulfilling certain clinical criteria. The significance of the disorder rests primarily on possible cardiovascular involvement, which occurs in about 50 per cent of patients. At least 20 per cent develop coronary artery aneurysms. Therapy remains nonspecific and incompletely effective.

Baffle obstruction following the Mustard operation: cause and treatment.

Baffle obstruction developed in 11 patients after they had undergone the Mustard procedure. Eight of them required operative revision. The cause of the baffle obstruction seemed to be related to patient age (less than 1 year) and to the use of Dacron for the baffle but not to the shape of the baffle. A technique of revision that involves widely opening the previously placed baffle and enlarging it and the atriotomy with polytetrafluoroethylene was employed for the last 6 patients. All 5 survivors of this operation had good long-term results without recurrence.

Chylothorax: an assessment of current surgical management.

The development of chylothorax is a serious and often life-threatening clinical entity. Optimal management of this problem has not been well defined to date. We reviewed our experience with chylothorax in patients of all ages during the past 10 years. Ages ranged from 2 days to 69 years. The etiologies were traumatic in 17 and congenital or idiopathic in three. Six patients (five infants) were treated nonoperatively with either repeated thoracenteses or chest tube drainage. Fourteen patients (11 infants) underwent operative treatment: transthoracic thoracic duct ligation (five patients), pleuroperitoneal shunting (seven), pleuroperitoneal shunting combined with reoperation on a patient with congenital heart disease (one), and reoperation alone on a patient with congenital heart disease (one). Duration of preoperative therapy ranged from 9 days to 2 months (average 3.3 weeks). Five of six (83.3%) patients treated nonoperatively died. Of the surgically treated group, only two of 14 (14.3%) died, and 11 of the 12 survivors had resolution of the chylothorax and immediate clinical improvement. Our experience suggests that both pediatric and adult patients respond poorly to nonoperative treatment of chylothorax and that this treatment has a high mortality rate. Post-traumatic and congenital chylothorax should be treated operatively after a limited trial (1 to 2 weeks) of nonoperative therapy. Pleuroperitoneal shunting may offer a reasonable and effective alternative to thoracotomy and thoracic duct ligation.

A simple, rapid technique for operative closure of patent ductus arteriosus in the premature infant.

A rapid, simplified clip technique for operative closure of the ductus arteriosus in premature infants is presented.

Ductal origin of the left pulmonary artery in severe tetralogy of Fallot: problems in management.

Cardiac catheterization in a neonate demonstrated tetralogy of Fallot and absence of anatomic origin of the left pulmonary artery from the main pulmonary artery. A central aortopulmonary shunt was performed in order to increase pulmonary blood flow. Because of the concern that the left pulmonary artery was actually being supplied by a ductus arteriosus, repeat catheterization was performed and this revealed closure of the ductus arteriosus with obliteration of arterial supply to the left pulmonary artery. A left Blalock-Taussig shunt was subsequently performed and this reestablished blood supply to the left pulmonary artery. This case report describes problems in management of congenital heart disease with absence of anatomic origin of the left pulmonary artery from the main pulmonary artery.