Interrupted right aortic arch and origin of the left pulmonary artery from the aorta in DiGeorge syndrome.

We describe a neonate with DiGeorge syndrome undergoing diagnosis and successful repair of interrupted right aortic arch and origin of the left pulmonary artery from the aorta. We discuss a link between this lesion and persistence of a left fifth arch.

The evolution of ductus arteriosus treatment.

The treatment of patent ductus arteriosus (PDA) has evolved over the years. We reviewed 231 non-premature children (group 1) undergoing surgical closure of a PDA between January 1985 and December 1997, and 30 children (group 2) undergoing transcatheter closure from May 1995 to December 1998. The median age and weight at operation in group 1 were 13 months (range, 0.5-174 months) and 9.5 kg (range, 1.9-49.7 kg), respectively. There was one intra-operative death (0.4%) secondary to hemorrhage. Immediate extubation was performed in 208 patients (90%). Intra-operative chest tube use decreased from 73.3% to 10% between the 1985-88 and 1996-97 periods (P < 0.001). Postoperative pneumothoraces occurred in 33/131 (25%) patients with only one patient (0.7%) requiring drainage. Eleven patients had complications including wound infection in four, vocal cord paralysis in three, and left pulmonary artery stenosis in one. The median length of stay (LOS) was 5 days (range, 2-43 days). Follow-up echocardiogram was performed in 146/230 patients (63%) and revealed a residual PDA in six (4%); two being re-ligated, two remaining clinically insignificant, and two spontaneously resolved at 7 and 28 months follow-up. The remaining 84 patients had no clinical signs of a residual PDA. In group 2, where a transcatheter coil occlusion technique was used, the median age and weight at procedure were 31 months (range, 9-320 months) and 14.9 kg (range, 9-69.7 kg), respectively. Vascular complications occurred in four patients (13.3%). One patient developed hemolysis and hemoglobinuria requiring hospital admission. Four patients required a second intervention. At the most recent echocardiographic assessment, four patients (13.3%) had a residual PDA.

Extracardiac Fontan operation with tube fenestration allowing transcatheter coil occlusion.

A fenestration may improve the immediate postoperative course after a Fontan procedure by preserving the cardiac output. We describe a simple and safe technique of fenestration amenable to coil occlusion, which can be carried out in most cardiac catheterization laboratories.

Intermediate follow-up of right ventricular outflow tract reconstruction with allograft conduits.

BACKGROUND: Allograft conduits are among many varieties of material used for right ventricular outflow tract reconstruction. They invariably need to be replaced due to growth of the patient or conduit failure. METHODS: From June 1984 to June 1996, a total of 76 patients underwent reconstruction of the right ventricular outflow tract with an allograft conduit: 51 aortic and 25 pulmonary. The median age, weight and conduit size at surgery were 37 months (range, 0.2 to 228 months), 12.4 kg (range, 2.9 to 61.4 kg), and 17 mm (range, 8 to 26 mm), respectively. RESULTS: The hospital mortality was 5.3% (4 of 76 patients) and 2 patients died at 9 and 78 months follow-up. The median follow-up was 61 months (range, 2 to 132 months). Reoperation was necessary in 22 patients (28.9%) at a median interval of 50.5 months (range, 3 to 109 months) and the median conduit size was 21 mm (range, 12 to 23 months). There was no mortality. Freedom from reoperation at 64 months was 49.5% for conduits 15 mm and smaller, and 73.3% for conduits 16 mm and larger. Analysis by age shows freedom from reoperation at 64 months of 49.4% and 74.5% for patients younger than and older than 2 years, respectively. At 54 months there was no statistical difference in freedom from reoperation between pulmonary and aortic allografts. CONCLUSION: Right ventricular outflow tract reconstruction with allograft conduits results in a high reoperation rate at 4 years but provides significantly longer freedom from reoperation with conduits larger than 15 mm or in patients over 24 months of age.

Is surgical closure of patent ductus arteriosus a safe procedure in premature infants?

Despite indomethacin therapy, many premature infants require surgical closure of their patent ductus arteriosus (PDA). Between January 1985 and December 1997, 176 premature infants underwent surgical closure of PDA by vascular clip after failure of medical treatment. The median gestational age and birth weight were 26 weeks (range 23-36 weeks) and 847.5 g (range 400-2300 g), respectively. The median age at diagnosis and at surgery was 4 days (range, 1-37) and 21 days (range, 4-60) respectively. The median weight at surgery was 982.5 g (range 475-2740 g). Of these infants, 168 (95%) were intubated prior to surgery and the median time to extubation was 21 days (range 1-273 days). There were no operative deaths but 11 infants (6.4%) died from complications of prematurity (sepsis, bronchopulmonary dysplasia and pulmonary hemorrhage). The frequency of chest tube insertion at surgery decreased from 41.7% to 10% between the 1985-88 and 1996-97 periods (P<0.01). Three infants (1.7%) developed vocal cord paralysis directly related to the position of the vascular clip. Echocardiography confirmed PDA closure in 43 infants (24.4%) while the remaining 133 had no clinical signs of PDA. Surgical closure of PDA by vascular clip carries a very low morbidity in premature infants.

Risks of repeat sternotomy in pediatric cardiac operations.

BACKGROUND: Repeat sternotomy has been associated with increased perioperative risks. METHODS: We reviewed 165 patients undergoing 192 repeat sternotomies between January 1985 and January 1997 (group 1) and a control group of 184 patients (group 2). The operations in group 1 were valve procedures in 94 patients, Fontan procedure in 46, ventricular septal defect closure in 10, pulmonary arterioplasty in 17, and others in 25; in group 2 ventricular or atrial septal defect closure in 120 patients, tetralogy of Fallot repair in 26, valve procedures in 16, bidirectional Glenn anastomosis in 7, repair of transposition of the great arteries in 7, pulmonary arterioplasty in 4, and others in 4. RESULTS: The hospital mortality was 2.6% in group 1 and 3.8% in group 2. Cardiac laceration occurred in 10 of 192 patients (5.2%), requiring emergent femorofemoral bypass in 6 patients. Two patients sustained an air embolism that was successfully treated with a hyperbaric chamber. Median total blood loss and requirements were not significantly different between the two groups. The length of stay in the intensive care unit and in the hospital were 4 days (range, 1 to 80 days) and 11 days (range, 1 to 135 days) in group 1, and 2 days (range, 1 to 87 days) and 7 days (range, 1 to 131 days) in group 2 (p < 0.02 and p < 0.002, respectively). The rate of complications was not significantly different in group 1 versus group 2. Overall survival was 97% (group 1) and 95% (group 2) at 120 months' follow-up (not significant). CONCLUSIONS: With careful surgical technique and judicious use of femorofemoral bypass, the risk of repeat sternotomy is minimized.

Effect of glutamate-aspartate reperfusion on postischemic neonatal myocardium.

OBJECTIVE: We postulated that L-glutamate- and L-aspartate-enriched perfusate would improve functional recovery of postischemic neonatal rabbit hearts. METHODS: Isolated working neonatal rabbit hearts were perfused with Krebs-Henseleit buffer and then subjected to 1 hour of hypothermic cardioplegic arrest with St. Thomas' Hospital solution. Hearts were then reperfused with L-glutamate- and L-aspartate-enriched (20 mmol/L) Krebs-Henseleit buffer (AA-enriched Krebs-Henseleit buffer). Hearts reperfused with Krebs-Henseleit buffer alone acted as controls (experiment A). Another group of hearts underwent a similar protocol but were reperfused with the AA-enriched Krebs-Henseleit buffer with correction of the sodium content (experiment B). RESULTS: Hearts reperfused with AA-enriched Krebs-Henseleit buffer showed a significant decrease in aortic flow at both 15 (p = 0.04) and 30 (p = 0.025) minutes compared with controls. Arrhythmias were frequent. Sodium content of the AA-enriched Krebs-Henseleit buffer was 174 +/- 0.5 mmol/L. In experiment B, hearts reperfused with the AA-enriched Krebs-Henseleit buffer with correction of the sodium content exhibited no difference in aortic flow and cardiac output at either 15 or 30 minutes (p = 0.95 and 0.5 and 0.48 and 0.78, respectively) compared with controls. No arrhythmias were observed. The sodium content of the AA-enriched Krebs-Henseleit buffer was 146 +/- 0.7 mmol/L. CONCLUSIONS: A beneficial effect on functional recovery of neonatal hearts reperfused with AA-enriched Krebs-Henseleit buffer was not demonstrated.

Right atrial lipoma.

The case of a patient undergoing successful surgical resection of a huge lipoma of the right atrium is presented. The diagnosis was established preoperatively by magnetic resonance imaging. The tumor was involved intimately with the right coronary artery, and careful identification and dissection were required to preserve the vessel. The tumor was removed successfully, and follow-up at 1 year showed no evidence of recurrence.

Prosthetic valve endocarditis. Experience with porcine bioprostheses.

Prosthetic valve endocarditis remains an infrequent but serious complication of cardiac valvular replacement. Prosthetic valve endocarditis was diagnosed in 56 (1.8%) of 3200 patients in whom one or more porcine bioprostheses were implanted between 1975 and 1988. Of the 56 patients with prosthetic valve endocarditis, there were 40 men and 16 women, with a mean age at initial implantation of 57 years (27 to 81 years). Of the 56 patients, 6 were initially treated for native valve endocarditis. There were 8 cases of early prosthetic valve endocarditis (defined as occurring less than 60 days after initial surgical intervention) and 48 cases of late prosthetic valve endocarditis (occurring after 60 days). The overall mortality rate of the 56 patients was 32% (18 patients). Of the 8 patients with early prosthetic valve endocarditis, 6 (75%) died. Of the 48 patients with late prosthetic valve endocarditis, 12 (25%) died. The predominant organisms were Staphylococcus epidermidis (12 cases), Streptococcus viridans (8 cases) and Staphylococcus aureus (7 cases). The presence of hemodynamic compromise, including congestive heart failure, septic embolism, persistent sepsis, and echocardiographic evidence of vegetations, dictated the mode and timing of the addition of surgical intervention to medical therapy. The survival rate for medically and surgically treated patients with late prosthetic valve endocarditis was 91% (20 patients); none of the patients with early prosthetic valve endocarditis survived (all had severe hemodynamic compromise). We analyzed 18 factors for the prediction of early and late death. The predictors of death by univariate analysis for both early and late prosthetic valve endocarditis were age, diagnosis time, renal status, sepsis, management mode, fever, dental procedures, and dental prophylaxis. The predictors by multivariate analysis were age, diagnosis time, renal status, and management mode for early prosthetic valve endocarditis, and only diagnosis time for late prosthetic valve endocarditis. Annular abscess formation occurred in 27% of the patients. There were no complex aortic or mitral reconstructions. There was one reoperation for recurrent and residual endocarditis. There was one late death as a result of recurrent prosthetic valve endocarditis. We advocate early diagnosis and aggressive combined medical and surgical treatment before the development of hemodynamic compromise and other characteristic signs when the culprit organisms are Staphylococcus aureus, gram-negative organisms, and Candida albicans.

Antiplatelet therapy in children with left-sided mechanical prostheses.

Valve replacement in children has always been associated with a high mortality, outgrowth of the prosthetic valve and difficulty in managing anticoagulation. Between January 1985 and April 1991, 20 patients (14 males and 6 females) underwent replacement of a left-sided cardiac valve. The median age at diagnosis was 21.6 months (1 to 120 months) and the median age at surgery was 85 months (11 to 213 months). Six patients were under 4 years of age. The site of valve replacement was aortic in 11 patients and left atrioventricular (AV) valve in 9 patients. The indications for aortic valve replacement were stenosis (6) and incompetence (5). The left AV valve was replaced in three patients following repair of AV septal defect, in one patient with corrected transposition of the great arteries (LTGA), in one patient with severe dysplasia and insufficiency, and in four patients for congenital mitral stenosis. A Bjork-Shiley prosthesis was used in three mitral and one aortic position, all the other patients receiving a St. Jude prosthesis: six mitral and ten aortic. All patients were anticoagulated (warfarin) for 3 months post implantation and then switched to a regimen of aspirin and persantine. There was no early or late death. Median follow-up was 12.3 months (4 to 72 months). Total follow-up was 59 patient-years or 708 months. There was one incident of thromboembolism (1.7%/patient-year) and four instances of bleeding (6.8%/patient-year). There was no valve thrombosis. Our regime of early warfarin followed by long-term antiplatelet therapy has been associated with a low incidence of thromboembolism and no valve thrombosis.

Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases.

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Children's Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.

Univentricular atrioventricular connection with subaortic stenosis: a staged surgical approach.

A staged surgical approach was developed for the management of hearts with univentricular atrioventricular connection (double-inlet left ventricle or tricuspid atresia) and discordant ventriculoarterial connection with anatomical substrate for the development of subaortic stenosis. This consisted of initial palliation with pulmonary artery banding, followed by early elective relief of subaortic obstruction using a proximal pulmonary artery to ascending aorta anastomosis in infancy. Pulmonary blood flow was maintained at this time by creating a bidirectional superior cavopulmonary anastomosis. Over an 18-month period, 5 children, including 4 seen in the first week of life with aortic arch obstruction, were palliated with this approach. All patients survived operation and are asymptomatic with transcutaneous oxygen saturations of 80% to 85%. Completion of cavopulmonary repair is planned at 2 years of age. Although some authors have considered pulmonary artery banding contraindicated in these infants, the current staged approach offers an attractive alternative to the construction of a pulmonary artery to aorta anastomosis in the neonatal period.

Aortic pseudoaneurysm secondary to celiac plexus block.

Traumatic pseudoaneurysm of the abdominal aorta has been infrequently reported in the literature. We report a case of an infected pseudoaneurysm of the supraceliac aorta which we believe to be secondary to celiac plexus block performed for pain from chronic pancreatitis. The aneurysm was successfully repaired using a Dacron graft through a thoracoabdominal approach. The possible mechanism of aortic injury from celiac plexus block is discussed.

Penetrating chest wounds: a 10-year review.

From January 1975 to December 1984, 93 patients with penetrating chest wounds were admitted to three hospitals in Regina. Sixty-three percent of the wounds were caused by knives and 34% by firearms. Sixty-three patients were treated conservatively, 18 patients had thoracotomy and 12 others underwent laparotomy. Of the 18 patients, 16 had wounds between the nipples; 8 of the 16 had injuries to the heart or great vessels. Whereas the majority of penetrating wounds to the chest may be treated by observation or thoracostomy alone, a surgical approach is recommended when penetrating injuries are thought to have traversed the mediastinum, because of the high incidence of associated cardiac injuries. In doubtful cases the decision should favour early thoracotomy.