Long-term results after atrial repair of transposition of the great arteries in early infancy.

Abstract

SUMMARY Fifty-two patients younger than age 100 days who had an intact interventricular septum or a small ventricular septal defect underwent atrial repair of d-transposition of the great arteries (d-TGA). No patient died. To assess long-term results, we evaluated all 36 patients who had been followed for at least 1.5 years (mean 2.7 years) after surgery. The physical findings, chest roentgenograms, ECGs and echocardiograms were reviewed. Catheterization was done 6-60 months (mean 15 months) after surgery in 28 patients. Growth was normal in all but three patients. Neurologic development was abnormal in six patients (delayed speech in one patient, learning disability in three patients and preoperative cerebral infarction in two patients). The ECG showed sinus rhythm in 24 patients, minor abnormalities in nine and major dysrhythmias in three. Catheterization showed a normal cardiac index in all 28 patients. No intracardiac shunt was detected in 19 of 24 patients in whom complete oximetry data were available. One patient required reoperation for persistent atrial shunt and subsequent pacemaker placement. Two patients required baffle revision for symptoms related to superior vena caval obstruction. Right ventricular end-diastolic pressure was less than 15 mm Hg in all of 18 patients evaluated. We conclude that atrial repair of d-TGA in early infancy can be performed with a low mortality rate and a low incidence of late complications.THE SURVIVAL rate of children with d-transposition of the great arteries (d-TGA) (concordant atrioventricular and discordant ventriculoarterial connections) and an intact interventricular septum or a small ventricular septal defect improved dramatically after balloon atrial septostomy and the atrial baffle operation were introduced. 1-5 Balloon atrial septostomy in the first days of life provides palliation until an atrial baffle operation can be performed.The optimal age for an atrial baffle operation is controversial. Early repair has been recommended for several reasons. First, although many infants are clinically stable after successful balloon atrial septostomy, before atrial repair they are at risk of death7 and complications including moderately severe hypoxemia, polycythemia, cerebrovascular accidents, 1 12 growth retardation,'3 and early onset of pulmonary vascular disease. '4 In addition, infants who do not improve after balloon atrial septostomy require urgent further inter-

Intermediate outcome after Mustard and Senning procedures: A study by the Congenital Heart Surgeons Society

“…Both early and late mortality are known to be increased with TGA/ VSD. 4,[13][14][15] Early mortality for this subgroup undergoing atrial switch has been variously reported, but averages around 20%. However, analysis of the mix of simple versus complex cases in this series shows little difference between the 2 groups of atrial switch procedures (Senning 14% complex v Mustard 10%).…”

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

“…164,166,212 Preoperative cerebral infarctions have been noted in 4% of children with congenital heart defects and are associated with subsequent abnormal neurological development. 105,[213][214][215] Higher bilirubin levels and low birth weight may be associated with a higher rate of neurological complications postoperatively. 216 The abnormal neurology and growth deficits 217 identified in these newborns may either be due to the congenital heart defect or be the result of an earlier factor that has led to both the heart defect and other neurological sequelae, but as yet causal pathways are poorly elucidated.…”

Stent implantation for superior vena cava occlusion after the mustard operation

“…For many years the Mustard operation was the preferred operative repair for transposition of the great arteries [1][2][3]. It was associated with complications such as obstruction of the systemic and/or pulmonary venous pathways, which usually required surgical revision and which carried a risk of morbidity and mortality [4 -6].…”

“…Obstruction of the superior and inferior vena cava after Mustard repair for transposition of the great arteries is a well-documented complication that occurs in 10%-40% of patients [1][2][3]. When both the superior and the inferior systemic venous pathways are obstructed, symptoms are more likely to occur in the form of superior vena cava syndrome, ascites, protein losing enteropathy, and generalized edema [4,5].…”