Sabine Kleinert

Anatomic Features and Surgical Strategies in Double-Outlet Right Ventricle

BACKGROUNDnThe objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality.nnnMETHODS AND RESULTSnRecords were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years.nnnCONCLUSIONSnBiventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.

Treatment of critical pulmonary valve stenosis by balloon dilatation in the neonate

Critical pulmonary valve stenosis represents an emergency, and immediate treatment is mandatory. The purpose of this study was to evaluate the immediate and medium-term results of pulmonary valve dilatation. We report 18 neonates in whom pulmonary valvuloplasty was attempted. The procedure could be accomplished in 14 patients. The angiographically determined diameters of the pulmonary and tricuspid valve at the time of procedure were 5.6 +/- 1.5 mm and 14.0 +/- 5.4 mm. The mean Doppler gradient decreased from 71 +/- 27 mm Hg to 27 +/- 14 mm Hg. Perforation of the right ventricular outflow tract was the major complication in three patients with one fatal event. Infusion of prostaglandin E1 could be discontinued 1 to 5 days after the procedure. On follow-up three children required a second balloon dilatation with good results. Seven patients monitored for more than 9 months with a mean follow-up time of 34.4 +/- 16 months had a residual gradient of 11.6 +/- 6.7 mm Hg. In spite of a hypoplastic pulmonary valve annulus in seven of the patients, results were good and surgery could be avoided.

Surgical commissurotomy of the aortic valve: outcome of open valvotomy in neonates with critical aortic stenosis.

Early intervention is necessary in neonates with critical aortic stenosis. The advent of alternate therapy, particularly balloon aortic valvuloplasty, requires a reappraisal of the traditional surgical approach, including the efficacy of initial transvalvar gradient reduction and freedom from recurrence of obstruction in the longer term. This report describes a series of 33 consecutive infants who underwent surgical aortic valvotomy in the first month of life. The hospital mortality was 18 percent with a 5-year probability of survival of 66 percent (90 percent CI, 50 percent to 79 percent). Fourteen reinterventions, nine reoperations and five balloon dilatations, were required at a median age of 0.8 years (range 9 days to 6 years). Three patients died after reintervention (one early and two late). The median follow-up time was 5.8 years (range 0.2 to 14 years). At last follow-up Doppler investigation the average Doppler mean and peak gradients were 34 mm Hg and 18 mm Hg, respectively. Open valvotomy in neonatal aortic valve stenosis allows the use of an appropriate surgical approach with low initial mortality and satisfactory 5-year freedom from reintervention.

Clinical, electrocardiographic, and histologic correlations in children with dilated cardiomyopathy

OBJECTIVEnTo determine whether presenting electrocardiography is related to histologic findings and clinical outcomes in children with dilated cardiomyopathy.nnnBACKGROUNDnLymphocytic myocarditis is an important cause of childhood dilated cardiomyopathy, the outcome of which is unclear. The results of non-invasive investigations are often used to infer the presence or absence of lymphocytic myocarditis.nnnMETHODSnThirty-four children, presenting acutely with dilated cardiomyopathy, underwent both early electrocardiography and endomyocardial biopsy. The parameters examined included heart rate, PR, QRS, and corrected QT intervals, R-wave voltages in Leads V(1) and V(6), S-wave voltages in Leads V(1) and V(6), and sum of SV(1) and RV(6). We expressed measurements as Z scores, based on published normal values for age and gender.nnnRESULTSnA total of 15 patients had lymphocytic myocarditis on endomyocardial biopsy (Group I), and 19 had non-specific histologic findings (Group II). We did not distinguish the 2 groups by age, time to endomyocardial biopsy, or duration of follow-up. Group I patients had significantly smaller R-wave Z scores in Leads V(1) and V(6), and combined S in V(1) and R in V(6) Z scores (p < 0.02 for each). The positive and negative predictive values of an R-wave amplitude in V(6) < 5th percentile were 75% and 65%, respectively, for the diagnosis of lymphocytic myocarditis. An R-wave amplitude in V(6) > 95th percentile had a positive and negative predictive value of 80% and 63%, respectively, for the diagnosis of idiopathic dilated cardiomyopathy. Survival and freedom from late cardiac dysfunction were more common among Group I patients compared with Group II (p <or= 0.02 for both).nnnCONCLUSIONnMyocardial histology cannot reliably be inferred from the presenting electrocardiogram. Survival and outcome for children with lymphocytic myocarditis is better than for those with non-specific histology.

Pulmonary vascular resistance and reactivity in children with end-stage cardiomyopathy

Pulmonary vascular resistance (PVR) and reactivity were compared in 63 children with end-stage cardiomyopathy (CM) referred for cardiac transplantation. Diagnostic category of CM was the sole determinant of PVR. Compared with other patients, children with restrictive CM were younger at diagnosis and had a significantly higher pulmonary vascular resistance index (PVRI). Children with a baseline PVRI of up to 11.8 units per meter squared (U.m(2)) who showed reactivity underwent successful orthotopic cardiac transplantation.

985-55 Surgical Repair of Double Outlet Right Ventricle with Non-committed Ventricular Septal Defect

Between January 1978 and June 1993, 32 patients presented for management of double outlet RV with non-committed VSD. 29 patients had definitive palliation or repair with 3 early and 1 late deaths (hospital mortality: 10.4%). The VSD type was muscular in 4, inlet in 12 and perimembranous but distant from both great arteries in 16 patients. Complicating anomalies with a major influence on management were present in 23 children (72%). These included aortic arch interruption (3), coarctation of the aorta (4), pulmonary stenosis (11), pulmonary atresia (2). multiple VSOs (3), AV discordance (2), left atrial isomerism (3), unbalanced ventricles (8) and criss-cross AV connection (2) with 11 children having more than one of these lesions. Initial palliation was performed in 20 patients and consisted of pulmonary artery banding in 8 (with repair of an interrupted aortic arch in 2), systemic-pulmonary artery shunt in 9 and repair of coarctation of the aorta in 3. Of these, 17 subsequently had definitive surgery: Fontan procedure in 9 and biventricular repair in 8 (arterial switch in one, intraventricular baffle in 7). 3 patients died following attempted biventricular repair. 12 patients underwent repair as the initial procedure: Fontan procedure in 2 and biventricular repair in 10 (arterial switch in 3 and intraventricular baffle in 7) with no deaths. Thus, of the 29 patients who had definitive surgery, 11 had Fontan procedures and 18 biventricular repair with no death in the Fontan group and 3 early deaths in the biventricular repair group (not statistically significant by Fischers exact test, pxa0=xa00.27). Only 2 patients have required reoperations, both for residual VSDs. 9 of the Fontan procedures were undertaken in patients who had complex anatomy (e.g. AV discordance, unbalanced ventricles, multiple VSDs, atrial isomerism or pulmonary atresia), whereas 14 children of the biventricular repair group had non-complex anatomy (pxa0lxa00.005). Surgery for double outlet RV with non-committed VSD needs to be tailored to individual patients, but biventricular repair was achieved in a substantial majority of patients with non-complex anatomy with relatively low risk and a low incidence of reoperations.