Interposition of a modified aortic homograft conduit as main pulmonary trunk in anatomic correction of transposition of the great arteries. Report of two cases and technical considerations.

Anatomic correction of transposition of the great arteries and ventricular septal defect has been performed successfully in two patients. The technique employed differed in several aspects from the so-called Jatene procedure: (1) Both great arteries were transected at low level; (2) the switch of the coronary arteries was carried out after transection of both great arteries; (3) a modified aortic homograft conduit was used to bridge the gap between the proximal aortic root and the distal main pulmonary artery; (4) the "new" main pulmonary arterial trunk was positioned on the right side of the ascending aorta; and (5) the ascending aorta was enlarged in order to match the dilated proximal pulmonary stump. The advantages of these modifications over other techniques reported in the literature are discussed.

Aortoplasty with the left subclavian flap in older children.

Ten patients between 8 months and 9 years of age (average 4.3 years) underwent surgical relief of coarctation of the aorta by the technique of left subclavian flap aortoplasty. Before operation, nine patients had systolic hypertension in the upper extremity (average 145 mm Hg) and all had a significant pressure gradient across the coarctated aortic segment (average 45 mm Hg). The hospital and late mortality were zero. The follow-up over 7 years has shown complete relief of the coarctation and no ischemic impairment or functional limitation of the left upper extremity. The blood pressure returned to normal in all patients. Hemodynamic and angiographic studies, performed in one case, demonstrated adequate growth of the repaired segment and absence of a pressure gradient across the previously narrowed area. The time interval between the peak of the femoral pulse and the dicrotic notch of the carotid pulse, recorded postoperatively, was normal. We consider the left subclavian flap technique to be the operation of choice in infancy and also in older children, whenever the anatomic configuration of the coarctation suggests that subsequent growth would be severely restricted if other techniques were used.

Late effects on the left upper limb of subclavian flap aortoplasty.

The subclavian flap aortoplasty gives excellent short-term and medium-term results when performed on infants with coarctation of the aorta. This study was devised to determine whether there were any detrimental effects to the sacrifice of the left subclavian artery in infancy. Sixteen patients were studied 2 to 9 years post-operatively. Clinical evaluation revealed no major symptoms in the left upper limb. However, in seven children, minor symptoms had been noted by the parents. Anthropometric measurements revealed a shortening of the left upper limb in all patients; when these measurements were compared with those in normal subjects, the shortening was localized to the left upper arm. Four papers which mention the effect on the left arm of ligation of the subclavian artery in childhood during the Blalock-Taussig operation are discussed. It is concluded that the excellent results from the subclavian flap aortoplasty are not compromised by the minor effects of ligating the left subclavian artery in infancy.

Appraisal of the Mustard procedure for the physiological correction of "simple" transposition of the great arteries. Eighty consecutive cases, 1970-1980.

Eighty successive patients underwent the Mustard operation for "simple" transposition of the great arteries (TGA) between January, 1970, and January, 1980. Ages ranged from 42 days to 12.4 years (mean 14.7 months). Balloon atrial septostomy (BAS) with balloon volumes of more than 2.5 ml produced significant increases of arterial oxygen saturation, although the initial benefit of BAS was greater than that found at follow-up catheterization. In these patients the left ventricular (LV) pressure tended to decrease during the first months of life, but this drop was less marked at the end of the first year. Deep hypothermia and cardiocirculatory arrest were employed in all cases. Several modifications were adapted to the original surgical technique in order to reduce last sequelae. There were two early and three late deaths. At follow-up (mean 4.5 years) 74% of patients were symptom free. Sinoatrial and atrioventricular (AV) conduction disturbances were present in five and six patients, respectively. Caval or pulmonary venous obstruction occurred in 13 patients, seven of whom required reoperation. Isolated tricuspid valve incompetence was present in one patient only, but in four it was associated with other defects. Primary right ventricular (RV) failure occurred in one patient. These findings, in relation to mortality, complications, and the excellent clinical results in the majority of survivors, compare favorably with results published for patients with "simple" TGA who have been subjected to other types of physiological or anatomic correction. At the present time, there seems to be no good reason to alter our surgical policy in this group of patients.

Early and late results of aortoplasty with a left subclavian flap for coarctation of the aorta in infancy.

Between February, 1969, and December, 1976, 45 consecutive infants younger than 6 months old underwent aortoplasty with a subclavian flap for the relief of coarctation of the aorta. All infants had persistence of the ductus arteriosus, and 58 percent had associated intracardiac anomalies. The over-all hospital mortality rate was 24 percent, nad no deaths occurred in infants with coarctation associated with patent ductus arteriosus only. All the deaths were in the group of patients under 2 months of age who had associated intracardiac defects. Follow-up over a 7 year period shows no clinical, hemodynamic, or angiographic evidence of recoarctation in any of the survivors.

Surgical correction of a case of common atrium with anomalous systemic and pulmonary venous drainage.

A case of common atrium with anomalous systemic and pulmonary venous connections and pulmonary stenosis is described. Surgical correction was performed by pulmonary valvotomy and the insertion of an intra-atrial pericardial baffle. To our knowledge this is the first reported case of surgical correction of total anomalous connections of both systemic and pulmonary veins. The embryologic development of this condition is discussed.

Double connections in total anomalous pulmonary venous connection.

Three infants who underwent operation for total anomalous pulmonary venous connection had unobstructed drainage of all the pulmonary veins to both the coronary sinus and left vertical vein. In one patient the anatomy was demonstrated preoperatively and complete correction accomplished without difficulty, but in two patients the second connection was discovered only at postoperative reinvestigation. Arbitrary classification of total anomalous pulmonary venous connection into cardiac, supracardiac, infracardiac, and mixed types does not lend itself to description of such lesions and may obscure their clinical importance. For these less common variants of total anomalous pulmonary venous connection, a more detailed and precise definition of morphology facilitates accurate surgical repair.

Total anomalous pulmonary venous drainage. Seventeen-year surgical experience.

Between 1968 and 1985, 80 children underwent correction of total anomalous pulmonary venous drainage. There were 47 boys and 33 girls whose ages ranged from 3 days to 16 years (median 2 months, interquartile range 5 years). Seventy (87.5%) were less than 1 year of age at operation. Fifty-eight (72.5%) weighed less than 5 kg, the range being 1.6 to 42 kg (median 3.7 kg, interquartile range 2.4 kg). Forty-five (56%) patients had supracardiac, 14 (17.5%) cardiac, 15 (19%) infracardiac, and 6 (7.5%) had mixed total anomalous pulmonary venous drainage. Follow-up was complete in 78 (97.5%) and ranged from 6 to 189 months (median 58 months, interquartile range 59 months). There were 14 (17.5%) early and six (7.5%) late deaths. Analysis by various factors revealed year of operation as the only factor to affect survival at the 5% level of significance. Early mortality was 29% between 1968-1977 and 11% between 1978-1985 (p = 0.04). Postoperative pulmonary venous obstruction occurred in five (6%) patients between 6 weeks and 3 months after operation. All 5 died, three after reoperation. Five (6%) other children had reoperations, four for residual shunts and one for superior vena caval obstruction.

A changing policy for the surgical treatment of tetralogy of Fallot: early and late results in 235 consecutive patients.

Between February, 1969, and March, 1980, 235 consecutive patients underwent repair of tetralogy of Fallot. Of these, 94 patients were less that 4 years of age, including 40 less than 2 years of age. Previous palliative procedures had been performed in 46 patients. Conservative technique to relieve the right ventricular outflow tract (RVOT) obstruction was employed in 194 patients; transannular approach was performed in the remaining 41. There were 18 hospital deaths (7.6%) and 3 late deaths. The early and late results were significantly related to the ratio of the right ventricular to left ventricular systolic peak pressure after repair. Until 1976, the age of patients of operation constituted an operative risk factor. Since 1977, none of the 17 infants who underwent operation died after the repair. In the overall series, the transannular approach to relieve the RVOT obstruction affected the early mortality and the late results. During the last 3 years, only 1 of the 12 patients who received an aortic homograft monocusp transannular gusset died in the hospital, and the 11 survivors were completely symptom free at follow-up.

Morphology of pulmonary atresia with intact ventricular septum in patients dying after operation.

In 1976, we adopted staged surgical management of pulmonary atresia with intact ventricular septum: stage 1 = establishment of a systemic to pulmonary artery shunt; stage 2 = open reconstruction of the right ventricular outflow tract and pulmonary valve; and stage 3 = closure of the shunt and interatrial communication. The morphological features of nine specimens obtained from 10 patients who died were reviewed. Special attention was given to features that might have influenced the poor surgical outcome in these patients. Survival after stage 1 depends on adequate systemic to pulmonary artery blood flow, initially as a combination of ductus arteriosus and shunt flow, with subsequent modification if the ductus closes. After stage 2, survival is influenced by left ventricular function and mitral valve function. The success of final correction (stage 3) depends largely on the morphology of both ventricles and their atrioventricular valves. It appears that the behavior of the ductus arteriosus and the size of the shunt are of vital importance for the survival of the infant. In 3 of the specimens, no right ventricular outflow tract was present, and in 2 others, short chordal attachments of the mitral valve were observed. Staged surgical correction appears to be a satisfactory approach if these considerations are taken into account.

Determinants of survival following repair of interrupted aortic arch in infancy.

Between January 1971 and March 1987, surgery was performed in 26 infants with interrupted aortic arch. At operation the 14 boys and 12 girls weighted between 1.71 and 4.23 kg (mean +/- SD = 3.1 +/- 0.63 kg) and ranged in age from 2 to 90 days (13 +/- 18 days). The interruption was distal to the left subclavian artery in 4 (15%), between the left carotid and subclavian arteries in 20 (77%) and between the brachiocephalic (innominate) and left carotid arteries in 2 (8%). Associated complex cardiac lesions in 8 patients included complete transposition (2), common arterial trunk (2), aortopulmonary window (2), double inlet left ventricle (1) and tricuspid atresia (1). The remaining patients had an isolated ventricular septal defect. The arch was reconstructed with a prosthetic conduit in 14 patients; by a direct anastomosis in 6; using the subclavian artery in 3; and with the pulmonary trunk and the arterial duct in 2. Twenty patients (77%) underwent palliative surgery as the first stage of management, and banding of the pulmonary trunk was also performed in 16 of these. Five patients (19%) underwent primary complete repair of the interruption and intracardiac anomalies. One patient (4%) died soon after thoracotomy for palliative surgery. Of the 15 (57%, 70% confidence limits CL = 46-69%) early deaths, 7 occurred in patients with complex associated defects and 4 occurred when single stage repair was attempted. Survival following first-stage palliative surgery for arch interruption with isolated ventricular septal defect was 64% (9/14) [70% CL = 47-79%]. All of these patients subsequently underwent complete repair. Chi-squared and t-tests showed the year of operation and the type of operation (two-stage repair) to be associated with improved survival. It is concluded that a two-stage repair of interrupted aortic arch offers a reasonable alternative to primary complete correction and will lead to satisfactory subsequent repair in most cases.

An anatomical study of the patterns of the coronary arteries and sinus nodal artery in complete transposition.

The origin and course of the main coronary arteries and the sinus nodal artery are relevant to both intra-atrial and arterial relocation operations for complete transposition (the combination of concordant atrioventricular and discordant ventriculo-arterial connexions). The patterns of the main coronary arteries in 148 hearts with complete transposition have been documented together with the patterns of the sinus nodal artery in 105 of these. The terminology which has been used to describe these arteries has been explained. The findings illustrate the wide variation which exists in the origin and course of all these arteries in complete transposition and indicate areas where they are most at risk.

Pulmonary venous obstruction following correction for total anomalous pulmonary venous drainage: a challenge.

Pulmonary venous obstruction after surgical correction of total anomalous pulmonary venous drainage (TAPVD) is a serious condition. Pulmonary venous obstruction can be the result of a primary developmental error or is due to post-operative anastomotic stricture and is usually manifest within 6 months of surgery. Prompt restudy is indicated and if a stricture is present, urgent surgical relief is indicated. However, the results are often disappointing with a high early mortality and a significant chance of restenosis.

Traumatic intercostal hernia due to a nonpenetrating injury in a child.

Herniation of the lung is a recognized, though rare, complication of thoracotomy and penetrating chest injury. Closed chest injury is usually associated with rib fractures, laceration of the lung, and rupture of the diaphragm, bronchus, and aorta. It is unusual for the intercostal muscles to be ruptured in a closed chest injury. In this case, herniation of the lung may result.

Surgical correction of congenital heart defects in adults.

From April 1987 to April 1991, 30 patients over the age of 15 years underwent surgery for congenital heart defects in the adult department of the Professorial Unit of Cardiothoracic Surgery, Royal Infirmary of Edinburgh. Eighteen were females (60%) and 12 were males (40%). Ages ranged from 15 to 68 years (mean 38.1). Six patients 20% had undergone previous cardiac surgery. Operative and hospital mortality was zero. Major postoperative complications occurred in three patients. In the follow-up period between three months and four years, there has been one late death. The spectrum of patients with congenital heart defects over the age of 15 was reviewed. The relatively late presentation of these defects and the indications for operating in adult life are discussed.

Reliability of a Behavioral Avoidance Test for the assessment of dog phobic children.

The temporal stability of approach scores on a Behavioral Avoidance Test designed for dog phobic children was confirmed. Small gains in scores among the 14 children were observed at retest without treatment only for those displaying relatively mild fears.