Ventriculotomy Decreases Agreement Between Assessment of Right Ventricular Function by Echocardiography and Cardiac Magnetic Resonance Imaging in Patients with Hypoplastic Left Heart Syndrome.

Accurate assessment of the right ventricular (RV) volume and function is important in patients with hypoplastic left heart syndrome (HLHS). We sought to investigate the effect of ventriculotomy on the correlation of RV functional assessments by two-dimensional echocardiography (2DE) to cardiac magnetic resonance (CMR)-derived RV ejection fraction (EF) in patients with HLHS. A retrospective re-analysis of CMR imaging with matched 2DE was performed from the institutional HLHS registry. Echocardiographic RV functional parameters were analyzed and correlated with CMR-derived EF. Intraclass correlation coefficient was used to determine interobserver reliability. A total of 58 matched echocardiograms and CMR imaging studies from 46 patients was evaluated. Median duration between CMR imaging and echocardiogram was 1 day (range 0-6 days). No significant difference was seen in CMR RV EF between patients with and without a ventriculotomy (EF - 43.6% vs 44.7%, p = 0.85). The presence of a ventriculotomy significantly decreased the correlation of biplane FAC (r = 0.86 vs 0.52; p = 0.02), triplane FAC (r = 0.84 vs 0.49; p = 0.03), and 2DE visually estimated EF (r = 0.83 vs 0.49; p = 0.02). The correlation of circumferential and longitudinal strains to CMR-derived EF was not significantly affected by the presence of a ventriculotomy. A prior ventriculotomy significantly affected correlation between 2DE FAC and visually estimated EF with CMR-derived EF. The dyskinetic myocardial segment due to ventriculotomy, which is often not visualized by 2DE, may be the reason for this discrepancy.

Negative Impact of the Left Ventricular Remnant Morphology on Systemic Right Ventricular Myocardial Deformation in Hypoplastic Left Heart Syndrome.

Left ventricular (LV) morphology may affect right ventricular (RV) function before and after Fontan palliation in patients with hypoplastic left heart syndrome (HLHS). We sought to assess the potential impact of LV morphology on RV function in patients with HLHS using cardiac magnetic resonance (CMR) imaging. A retrospective analysis of available CMR scans from all patients with HLHS was performed. LV morphology was categorized as absent/slit-like or globular/miniaturized. Volumetric analysis was performed using manual disc-summation method on steady-state free precession (SSFP) stack obtained in short-axis orientation of the ventricles. 4-chamber and short-axis SSFP images were used to measure strain on a semi-automated feature-tracking (FT) module. Two sample t-test was used to compare the groups. A total of 48 CMR scans were analyzed. Of those, 12 patients had absent/slit-like and 36 had globular/miniaturized LV morphology. Averaged 4-chamber longitudinal RV strain was significantly higher for absent/slit-like (- 17.6 ± 4.7%) than globular/miniaturized (- 13.4 ± 3.5; P = 0.002). Averaged 4-chamber radial RV strain was also significantly higher for absent/slit-like (33.1 ± 14.9%) than globular/miniaturized (21.6 ± 7.1; P = 0.001). For globular/miniaturized LV morphology, the decreases of 4-chamber longitudinal and radial strains were mainly attributable to the septal basilar and septal mid-ventricular segments. No differences were found in short-axis RV global circumferential strain between the morphologic subtypes (absent/slit-like - 15.0 ± 6.5, globular/miniaturized - 15.7 ± 4.7; P = 0.68). Larger LV remnants, with globular/miniaturized LV morphology, demonstrated diminished strain in the septal base and mid-ventricle segments. Patients with globular/miniaturized LV morphology may benefit with closer monitoring and lower threshold to start heart failure medications. These results exemplify the utility of including both septal and regional deformation in systemic RV strain analysis.

Ebstein anomaly, left ventricular non-compaction, and early onset heart failure associated with a de novo α-tropomyosin gene mutation.

Ebstein anomaly of the tricuspid valve (EA) can be associated with left ventricular non-compaction (LVNC), a rare congenital cardiomyopathy. We report a 2 year-old female with EA and severe tricuspid regurgitation, LVNC, pulmonary hypertension, and chronic biventricular systolic heart failure, who died during evaluation for cardiac transplantation. Gene panel testing revealed a heterozygous de novo missense mutation in TPM1, which encodes the cardiac sarcomeric thin filament protein α-tropomyosin. The c.475G>A variant results in a p.Asp159Asn substitution, altering a highly conserved residue predicted to be damaging to protein structure and function. TPM1 is the second gene linked to EA with LVNC in humans, implicating overlap in the molecular basis of structural and myopathic heart disease. © 2016 Wiley Periodicals, Inc.

Diagnostic Value of Computed Tomography Angiography for Infective Endocarditis After Right Ventricle Outflow Tract Repair.

Congenital heart disease patients with pulmonary valve replacement or right ventricle-pulmonary artery conduit have increased risk of pulmonary valve endocarditis. We present a 6-patient case series illustrating the diagnostic utility of computed tomography angiography to provide definitive visualization of pulmonary valve vegetation to aid in the diagnosis of endocarditis. (Level of Difficulty: Intermediate.).

The Gerbode defect: the significance of a left ventricular to right atrial shunt.

BACKGROUND: The so-called Gerbode ventriculo-atrial defect is a rare defect that permits shunting from the left ventricle to the right atrium. It takes 2 forms, either a deficiency of the atrioventricular membranous septum, or shunting initially through a ventricular septal defect, with atrial shunting occurring through a deficiency in the septal leaflet of the tricuspid valve. In this review, we describe the natural history and outcomes of surgical repair for the variant with shunting through a deficiency at the site of the atrioventricular membranous septum. METHODS: From 1990 to 2008, we identified 6 patients from our departmental database who had undergone surgical closure of a congenital defect of the atrioventricular component of the membranous septum. Median age at repair was 1.6 years, with a range, from 0.4 to 19 years. All patients were symptomatic, with 3 having congestive cardiac failure, 2 failing to thrive, and 2 having intolerance to exercise. All had a dilated right atrium demonstrated by echocardiogram, with a mean preoperative gradient measured by echocardiogram to be 109 millimetres of mercury, with a range from 65 to 150 millimetres of mercury. RESULTS: There was no operative or late mortality. The mean size of the defect was 6.2 +/- 2.0 millimetres, with a range from 4 to 8 millimetres. All were closed by insertion of a patch. The mean period of cardiopulmonary bypass was 90.5 +/- 11.3 minutes, the mean time of aortic cross-clamping 54.8 +/- 6.9 minutes, and the mean length of stay in hospital 4.3 +/- 1.0 days. No patient had a residual defect, and only trivial regurgitation of the tricuspid valve was evident by postoperative echocardiography. There were no complications or reoperations. CONCLUSION: The membranous ventriculo-atrial defect can be recognized echocardiographically on the basis of dilation of the right atrium in the setting of an unusually high Doppler echocardiogram gradient compared to the ventricular septal defect with shunting only at ventricular level. Since all patients in our series were symptomatic, we recommend surgical closure of all these defects.

Echocardiographic Evaluation of Patients Undergoing Transcatheter Tricuspid Valve-In-Valve Replacement.

BACKGROUND: Transcatheter tricuspid valve-in-valve replacement (TVIV) is an emerging therapy for dysfunctional surgical valves in patients with congenital and acquired TV disease. The present study was performed to establish baseline quantitative data for echocardiographic and invasive parameters obtained pre- and immediately post-TVIV. METHODS: Patients were drawn from the VIVID Registry. This study included two cohorts. The registry cohort included all patients entered in the VIVID registry through February 2017 who had both echocardiographic and invasively measured gradients across the TV. The focused cohort comprised a subset of patients from a single institution who had both pre- and post-TVIV echocardiogram images reviewed offline by a single investigator. The echocardiographic variables measured were based on published guidelines from the American Society of Echocardiography. RESULTS: Assessment of paired pre- and/or postimplant echocardiographic and invasive pressure measurements (n = 199) showed reasonable correlation between mean TV gradient measured invasively with cardiac catheterization and noninvasively both pre- and post-TVIV (R = 0.72, P < .001), although there was a bias toward the echocardiographic gradient being higher than the invasively measured gradient and sizable discrepancies were reported in several patients. In the focused cohort (n = 42), the mean TV inflow gradient was 9.3 ± 5.0 mm Hg pre- and 5.6 ± 2.3 mm Hg post-TVIV (P < .001). The TV pressure halftime and TV:left ventricular outflow tract Doppler velocity index were 215 ± 94 msec and 3.4 ± 1.2, respectively, at baseline, and 170 ± 44 msec and 2.4 ± 0.6 post-TVIV. Both the Doppler velocity index and the TV E velocity correlated with the mean TV inflow gradient. CONCLUSIONS: This study provides benchmark data for the echocardiographic assessment of valve function after TVIV. In this population, the significance of an inflow gradient after TVIV should be interpreted in the clinical context. The appropriate threshold for defining dysfunction may differ from the levels proposed for assessment of native or newly placed surgical valves.

Cardiac transplantation in children and adolescents with long QT syndrome.

BACKGROUND: Long QT syndrome (LQTS) is a potentially lethal, yet highly treatable, cardiac channelopathy. Cardiac transplantation has been reported anecdotally for patients with severe LQTS refractory to standard therapies. OBJECTIVE: The purpose of this study was to evaluate the incidence of and risk factors for cardiac transplantation in children evaluated and treated in an LQTS specialty center. METHODS: This was a retrospective review of 349 children with LQTS (mean age at diagnosis, 8.0 ± 5.7 years; mean corrected QT interval, 469 ± 51 ms; long QT syndrome type 1 [LQT1] in 46%, LQT2 in 31%, and LQT3 in 9%) evaluated from 2000 to 2013. A subset analysis was performed on patients referred for cardiac transplantation. RESULTS: Only 3 patients (0.9%; all LQT3; 2 female) underwent cardiac transplantation at ages 4, 11, and 17 years. Overall, 90 of 349 (26%) were symptomatic (exhibited LQTS-associated cardiac events) before LQTS diagnosis, including those who ultimately underwent transplant. Age at sentinel event was associated with transplantation (3 of 26 [12%] with an event at <1 year of life were transplanted vs 0 of 64 with an event after age 1; P = .02). Genotype was also a risk factor (3 of 32 patients with LQT3 were transplanted [9.4%] vs 0 of 270 patients with LQT1 or LQT2; P = .001). Before transplant, all patients had recurrent ventricular fibrillation-terminating shocks despite combination drug therapy and bilateral sympathetic denervation. All transplanted patients are alive at follow-up. CONCLUSION: Cardiac transplantation is seldom necessary for the management of LQTS. However, patients with LQT3 and in utero/neonatal expressivity are at higher risk of treatment failure and refractory ventricular arrhythmias with standard therapy, and cardiac transplantation should be considered for this malignant subset of LQTS.

Cell-Based Therapy for Myocardial Dysfunction After Fontan Operation in Hypoplastic Left Heart Syndrome.

Myocardial dysfunction after Fontan palliation for univentricular congenital heart disease is a challenging clinical problem. The medical treatment has a limited impact, with cardiac transplant being the ultimate management step. Cell-based therapies are evolving as a new treatment for heart failure. Phase 1 clinical trials using regenerative therapeutic strategies in congenital heart disease are ongoing. We report the first case of autologous bone marrow-derived mononuclear cell administration for ventricular dysfunction, 23 years after Fontan operation in a patient with hypoplastic left heart syndrome. The cells were delivered into the coronary circulation by cardiac catheterization. Ventricular size decreased and several parameters reflecting ventricular function improved, with maximum change noted 3 months after cell delivery. Such regenerative therapeutic options may help in delaying and preventing cardiac transplant.

Familial Incidence of Cardiovascular Malformations in Hypoplastic Left Heart Syndrome.

Obstructive left-sided congenital heart lesions exhibit familial clustering, and familial echocardiographic screening for bicuspid aortic valve has become standard practice. Hypoplastic left heart syndrome (HLHS) is a severe left-sided obstructive lesion; however, familial screening is not universally recommended. The purpose of this study was to define the incidence of cardiovascular malformations (CVMs) in first-degree relatives of HLHS probands. First-degree relatives were screened for CVM by transthoracic echocardiography. Screening was completed in 152 family members (97 parents and 55 siblings) of 52 probands. Of these, 17 of 152 (11%) had CVM. Anomalies detected included: bicuspid aortic valve in 5 (3%), isolated dilated ascending aorta in 4 (3%), coarctation of the aorta in 1, partial anomalous pulmonary venous connection in 1, anomalous, intramural coronary artery in 1, bicuspid pulmonary valve in 1, and other anomalies in 4. Most were previously undiagnosed (11 of 17, 65%). Fourteen of 52 families (27%) had ≥1 relative with CVM. Overall, 7 of 55 siblings (13%), 5 of 46 fathers (11%) and 5 of 51 mothers (10%) had CVM. Although the incidence of CVM in first-degree relatives of HLHS probands was lower in this cohort than previously reported, it remained substantial, with at least one additional member having CVM in 27% of families. The frequent occurrence of undiagnosed CVM highlights the importance of routine familial screening in HLHS. In fact, even if screening was done in childhood, it may be appropriate to screen again in the third or fourth decade to exclude isolated enlargement of the ascending aorta.

Total anomalous pulmonary venous connection: results of surgical repair of 100 patients at a single institution.

OBJECTIVE: Surgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome. METHODS: Our cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990-2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0-4 years), and the median weight was 3.5 kg (range, 1.3-15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy. RESULTS: There were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 +/- 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days). CONCLUSION: Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.

Tracheal reconstruction in children with unilateral lung agenesis or severe hypoplasia.

BACKGROUND: Infants with congenital tracheal stenosis may also have unilateral lung agenesis or severe lung hypoplasia. The purpose of this review is to evaluate our results with these patients and compare their presentations and outcomes to those of tracheal stenosis patients with two lungs. METHODS: Our database was queried for patients undergoing tracheal stenosis repair since 1982. Patients were divided into two groups based on pulmonary anatomy of single lung (SL = unilateral lung agenesis or severe hypoplasia) or two lungs (BL = bilateral lungs) and analyzed to compare presentation and outcomes. RESULTS: From 1982 to 2008, 71 patients had tracheal stenosis repair. Bilateral lungs were present in 60 patients; 9 patients had an absent (4) or severely hypoplastic (5) right lung, and 2 patients had an absent left lung (SL = 11). Age at repair was similar between groups; median age 0.42 years in the SL group (mean 0.80 +/- 1.0 years) versus 0.37 years in the BL group (mean 0.91 +/- 2.1 years, p = not significant [ns]). In the SL group 8 of 11 (73%) were intubated preoperatively versus 15 of 60 (25%) in the BL group (p = 0.004). In the SL group 4 of 11 (36%) patients had pulmonary artery sling versus 20 of 60 (33%) of BL patients (p = ns). In the SL group 2 of 11 (18%) versus 14 of 60 (23%) in the BL group had intracardiac anomalies requiring simultaneous repair (p = ns). Procedures included pericardial tracheoplasty (2 vs 26), tracheal autograft (4 vs 16), slide tracheoplasty (3 vs 8), and tracheal resection (2 vs 10). Overall mortality (operative and late) was 2 of 11 (18%) SL versus 10 of 60 (17%) BL (p = ns). Median postoperative length of stay was 43 days SL (mean 48.6 +/- 40) versus 30 days BL (mean 52.2 +/- 65) (p = ns). The incidence of postoperative tracheostomy (SL group) was 0 of 3 for slide tracheoplasty and 5 of 8 for the other techniques (p = 0.12). CONCLUSIONS: Despite the increased severity of pathology and increased critical presentation of tracheal stenosis patients with unilateral lung agenesis or severe hypoplasia, outcome measures of mortality and length of stay were similar to patients with two lungs. The incidence of associated pulmonary artery sling (1 of 3) and intracardiac anomalies (1 of 4) was similar. Unilateral lung agenesis or severe hypoplasia should not preclude operative repair of tracheal stenosis. Slide tracheoplasty is our current procedure of choice for these infants.

Complete atrioventricular canal: comparison of modified single-patch technique with two-patch technique.

BACKGROUND: The purpose of this study was to compare the modified single-patch technique to the two-patch technique for infants with complete atrioventricular canal (CAVC) defects. METHODS: Between January 2000 and June 2006, 55 infants underwent CAVC repair. Twenty-six patients had a modified single-patch technique; 29 patients had a two-patch technique. Trisomy 21 was present in 23 of 26 and 26 of 29 patients (p = not significant [ns]). Mean age was 4.4 +/- 1.3 months (single-patch) versus 5.5 +/- 1.9 months (two-patch, p < 0.02). Mean weight was 4.74 +/- 0.92 versus 5.28 +/- 1.67 kilograms (p = ns). RESULTS: There was one death in the modified single-patch group (postoperative day 130, liver failure) and no deaths in the two-patch group. Cross-clamp times and cardiopulmonary bypass times were shorter in the modified single-patch group (97.3 +/- 19.9 vs 123.3 +/- 28.2 minutes, p < 0.0003; 128 +/- 25 vs 157 +/- 37, p < 0.03). Rastelli classification was type A (18 vs 14), B (1 vs 0), and C (7 vs 15). Mean size of the ventricular septal defect as assessed by transesophageal echocardiogram was 9 +/- 2 mm, (single-patch) versus 10 +/- 3 mm (two-patch) (p = ns). Median postoperative length of stay did not differ (10 vs 8 days). There was no difference in the degree of postoperative left or right AV valve insufficiency as assessed by serial echocardiography. One patient (4%) required reoperation for mitral insufficiency in the modified single-patch versus three patients in the two-patch group (10%, p = ns). There were no patients with third degree atrioventricular block or that required reoperation for residual VSD in the modified single-patch group. There was one patient with third-degree AV block that required a pacemaker and one patient who had reoperation for a residual ventricular septal defect in the two-patch group (p = ns). No patient in either group required reoperation for left ventricular outflow tract obstruction. CONCLUSIONS: The modified single-patch technique produced results comparable with the two-patch technique in younger patients with similarly sized ventricular septal defects. Furthermore, the modified single-patch technique was performed with significantly shorter cross-clamp and cardiopulmonary bypass times.

Evolving surgical strategy for sinus venosus atrial septal defect: effect on sinus node function and late venous obstruction.

BACKGROUND: Our surgical strategy for repair of sinus venosus atrial septal defect has evolved chiefly to avoid sinus node dysfunction. We reviewed our experience with the single-patch, two-patch, and Warden repairs. METHODS: We identified 54 patients with repair of sinus venosus atrial septal defect from 1990 to 2006. Mean age was 9.5 +/- 12.6 years; median age was 4.2 years. Partial anomalous pulmonary venous connection was found in 52 patients (96%); drainage was to the right atrium in 8, right atrial-superior vena cava (SVC) junction in 17, and directly to the SVC in 27. Techniques were single-patch repair (24), two-patch repair (25), and Warden repair (5). Autologous pericardium was used in all patients. Echocardiogram and electrocardiogram follow-up were available for 48 patients (89%). RESULTS: There were no early or late deaths and no reoperations. No patient had pulmonary vein stenosis. Five patients had SVC stenosis: 2 mild after two-patch repair; 1 moderate and 1 mild after single-patch repair; and 1 severe stenosis after Warden procedure (p = 0.3). The incidence of rhythm change from sinus to low atrial or junctional rhythm was 35% and was significantly greater among patients with two-patch repair (12 of 22, 55%) compared with single-patch repair (5 of 21, 24%), or the Warden repair (0 of 5, p = 0.02). CONCLUSIONS: Repair of sinus venosus atrial septal defect with autologous pericardium is associated with a low incidence of late SVC or pulmonary vein stenosis with all techniques. Use of the two-patch technique, however, is associated with a significantly greater incidence of sinus node dysfunction. For the patients with partial anomalous pulmonary venous connection entering the SVC, the Warden procedure avoids interfering with the sinus node and should be used preferentially. The single-patch technique remains the procedure of choice for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection entering the right atrium or right atrium-SVC junction.

Dual-chamber epicardial pacing in neonates with congenital heart block.

OBJECTIVE: This review evaluates the outcome of a treatment strategy using dual-chamber pacemakers for neonates with congenital heart block. METHODS: From 1989 to 2006, 10 neonates had dual-chamber epicardial pacemaker placement for congenital heart block. Mean age and weight were 4.4 +/- 5.6 days and 2.8 +/- 0.6 kg. Four patients had heterotaxy syndrome and required concomitant cardiac procedures. One patient had fetal hydrops, myocarditis, and cardiomyopathy. Five patients had structurally normal hearts. Sternotomy (2 partial, 8 full) and unipolar leads (2 non-steroid eluting, 18 steroid-eluting) were used in all. Generators were placed in submuscular bilateral rectus sheath pockets. RESULTS: Successful atrioventricular synchrony was established in all patients. Mean P-wave sensing was 4.7 +/- 2.6 mV and atrial voltage threshold was 0.8 +/- 0.3 V. Mean R-wave sensing was 13.0 +/- 5.7 mV and ventricular voltage threshold 0.9 +/- 0.5 V. There were no wound complications or acute lead failures. Median postoperative stay was 14 days. Three of the 4 patients with heterotaxy died at 3 days, 14 days, and 15 months postoperatively. The patient with cardiomyopathy died suddenly at 6 months of acute myocarditis. No patient with a structurally normal heart died (P < .05). Mean follow-up interval in survivors is 6.1 +/- 7.1 years with 1 patient lost to follow-up. CONCLUSIONS: Implantation of a dual-chamber epicardial pacemaker in neonates with congenital heart block is technically feasible and results in excellent outcomes in patients with structurally normal hearts. System longevity at 6 years is excellent. Patients with congenital heart block and heterotaxy syndrome have a poor prognosis despite dual-chamber pacing.

Aprotinin is safe in pediatric patients undergoing cardiac surgery.

OBJECTIVE: Aprotinin, a serine protease inhibitor, decreases transfusion requirements and inflammatory response after cardiopulmonary bypass. This study was done to determine whether aprotinin is associated with adverse outcomes, particularly mortality and acute kidney failure, in pediatric patients (<18 years of age) undergoing cardiopulmonary bypass. METHODS: We compared a cohort of all pediatric cardiopulmonary bypass operations from 1994-1999, when aprotinin was not used (n = 1230), with a cohort from 2000-2006, when all patients received high-dose aprotinin (n = 1251). Primary end points were operative and late mortality, acute kidney failure, need for dialysis, and neurologic complications. Association of aprotinin with primary end points was assessed by means of univariate analysis, multivariate logistic regression, and Cox regression analysis, where appropriate. RESULTS: The aprotinin group was younger (mean age, 3.49 +/- 1.84 vs 3.64 +/- 4.75 years; P = .019) and had a higher Aristotle score (7.8 +/- 2.3 vs 7.2 +/- 2.6, P < .001). Univariate and multivariate analysis showed no significant difference between the no-aprotinin and aprotinin groups for operative mortality (55 [4.5%] vs 47 [3.8%], P = .508), acute kidney failure (68 [6.0%] vs 69 [5.7%], P = .77), need for temporary dialysis (6 [0.49%] vs 12 [0.96%], P = .17), or neurologic complications (14 [1.1%] vs 17 [1.4%], P = .62). By means of Cox regression analysis, aprotinin had no influence on late mortality (24 vs 10 deaths, P = .078). CONCLUSION: In this retrospective cohort study of pediatric patients undergoing cardiopulmonary bypass, there was no association between the use of aprotinin and acute kidney failure, need for dialysis, neurologic complications, and operative or late mortality. We continue to use aprotinin for all pediatric patients undergoing cardiopulmonary bypass.

Use of partial cardiopulmonary bypass for coarctation repair through a left thoracotomy in children without collaterals.

BACKGROUND: Paraplegia is a devastating complication of coarctation of the aorta (COA) repair. Since 1990 we have used left atrium-to-descending aorta cardiopulmonary bypass (CPB) for COA repair in patients with inadequate collaterals. We reviewed the results with that strategy and compared this CPB group with COA repairs in which CPB was not used to see whether there was any increase in morbidity or delay in recovery. METHODS: From 1990 to 2006, 11 patients with COA were identified to have inadequate collaterals based on preoperative examination and intraoperative arterial monitoring and test clamp. Left thoracotomy with left atrium-to-descending aorta CPB was used in all. Age ranged from 4.2 to 17.4 years (mean, 8.7 +/- 4.6 years). Two were reoperations for recurrent COA, 3 patients had four prior transcatheter balloon dilatations. One patient had aberrant origin of the right subclavian artery. Operative techniques included resection with extended end-to-end anastomosis (n = 6), interposition graft (n = 4), and patch repair (n = 1). During the same period 71 patients older than 1 year of age had COA repair without CPB. Age ranged from 1.1 to 46.1 years (mean, 7.6 +/- 7.1 years; p = 0.6). RESULTS: Preoperative imaging of CPB patients demonstrated absence of collaterals (n = 7), possible collaterals (n = 2), small collaterals (n = 1), and anomalous origin of the right subclavian artery (n = 1). Preoperative arm leg gradient in CPB patients was 36.0 +/- 9.0 mm Hg versus 49.9 +/- 15 mm Hg in non-CPB patients (p < 0.01). Mean distal femoral artery pressure with aortic test clamp was 34.3 +/- 4.8 mm Hg in CPB patients versus 49.8 +/- 12.4 mm Hg in non-CPB patients (p < 0.01). Mean CPB flow was 53% +/- 7.3% of calculated total flow. Cardiopulmonary bypass time ranged from 17 to 46 minutes (mean, 27.5 +/- 9.7 minutes). Aortic clamp time in CPB patients ranged from 15 to 33 minutes (mean, 21.6 +/- 6.3 minutes). In the non-CPB group aortic clamp time ranged from 10 to 50 minutes (mean, 23.4 +/- 7.5 minutes; p = 0.5). In the CPB group length of stay ranged from 3 to 7 days (mean, 4.9 +/- 1.3 days), and in the non-CPB group length of stay ranged from 3 to 12 days (mean, 4.7 +/- 1.4 days; p = 0.5). No patient had a neurologic complication. There were no other major complications in the CPB group (eg, bleeding, recurrent laryngeal nerve injury, re-COA). CONCLUSIONS: Preoperative imaging and a lower arm-to-leg gradient in this series of COA patients suggested inadequate collateral circulation with the potential need for CPB. A femoral artery pressure of less than 45 mm Hg during test clamp was used as an indication for partial CPB. The use of left atrium-to-descending aorta CPB with just over 50% calculated total flow protected the spinal cord in a safe and expeditious fashion. Use of left heart bypass did not affect morbidity or recovery time as compared with COA repair in non-CPB patients.