Pulmonary artery sling: a new diagnostic tool and clinical review.

The pulmonary artery sling is a vascular anomaly that produces severe respiratory disease in infancy. The differential diagnosis is lengthy, and the diagnostic work-up frequently includes such invasive maneuvers as bronchography, bronchoscopy, and cardiac catheterization. In this paper the use of a new noninvasive diagnostic tool, computer tomography, to aid in the diagnosis of this form of vascular ring, is discussed and a review of salient features of this cardiovascular malformation is presented.

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.

Interrupted aortic arch. A conservative approach for the sick neonate.

Interrupted aortic arch with associated ventricular septal defect is a congenital cardiovascular defect which, untreated, is lethal in nearly 100% of the cases. We have treated nine patients by reconstructing the aorta with endogenous arch vessels; in five of them, concomitant pulmonary artery banding was also done. If two infants with preoperative complete renal failure are excluded, the mortality with this approach is only 29%. Long-term follow-up of these patients demonstrates excellent hemodynamic results with marked reduction of the anastomotic gradient in the older survivors. Growth of the anastomosis has been noted in the older survivors.

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.

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.

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.

Rupture of postcoarctation mycotic aneurysms of the aorta.

Sudden and usually fatal rupture is a common complication of postcoarctation mycotic aneurysms. Prompt operative treatment is always indicated, and can be performed using one of three techniques depending on the adequacy of the collateral circulation and the presence or absence of active bacterial infection. Renal dysfunction commonly is associated with bacterial endarteritis and is secondary to an immune-complex glomerulonephritis. The patient reported here was treated successfully by the placement of an emergency axillofemoral bypass graft, removal of the infected portion of the descending thoracic aorta, and delayed intrathoracic reconstruction.

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.

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.

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.

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.

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.

Near-fatal Kearns-Sayre syndrome. A case report and review of clinical manifestations.

The Kearns-Sayre syndrome is a relatively uncommon condition which occurs in childhood and is characterized by neurologic dysfunction and abnormalities in atrioventricular (AV) conduction. The high mortality rate is directly attributable to the cardiovascular manifestations of this syndrome. We report a case, with documentation of a "near-fatal" episode, and review the pertinent clinical features of the syndrome.

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.

Congenital absence of the pulmonary valve.

Congenital absence of the pulmonary valve (CAPV) is a rare anomaly usually associated with ventricular septal defect or Fallot's tetralogy. CAPV may be rapidly fatal in infancy because of severe disturbance of pulmonary ventilation resulting from bronchial compression by massively dilated pulmonary arteries. Other cases may be relatively benign. Our group of five cases with CAPV ranged from the severe to one case in which there was documented closure of a patent ductus arteriosus and a ventricular septal defect, leaving the CAPV as an apparently isolated, well-tolerated lesion. Plain chest radiographs play a major part in the diagnosis of CAPV since they almost invariably give some indication od the aneurysmal dilatation of the pulmonary arteries. On angiography, dilated main pulmonary arteries are shown giving rise to branches of normal or even diminished caliber.