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Objectives Tricuspid valve regurgitation (TR) is a common finding in children with hypoplastic left heart syndrome (HLHS) undergoing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation due to annular dilatation. The purpose of this study was to determine the incidence of severe TR and to evaluate the effect of surgically treated tricuspid valve (TV) disease on overall survival. Methods Between December 1988 and December 2012, 86 patients with HLHS underwent a Norwood procedure (mean age/weight, 11.1 days/3.5 kg). Thirty-five percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 65 patients underwent a bidirectional Glenn procedure or a hemi-Fontan (stage II) and 42 patients a modified Fontan procedure (stage III). The mean follow-up was 6.6 years. All patients were evaluated preoperatively and postoperatively by Doppler echocardiography to determine the degree of TR. Results At the time of discharge following the Norwood procedure, 64% (n = 47) of the patients had none/trivial, 32% (n = 24) had mild, 3% had moderate (n = 2), and 1% (n = 1) had severe TR on their post-Norwood Doppler echocardiograms. Among 74 Norwood survivors, 11 children (15%) with severe TR underwent TV repair at a median interval after the Norwood procedure of nine months (range, 2–70). There were one surgical and one late death, giving an overall mortality of 18%. The overall survival rate between TR and non-TR repair groups was not significantly different (p = 0.18). Four patients (36%) underwent reoperation for persistent TR. Conclusions Severe TR as a risk factor for mortality in children undergoing palliative procedures for HLHS can be diminished by successful TV procedures. Survival at intermediate follow-up of patients with successful TV procedures that eliminate severe TR and patients without a history of severe TR are similar. Further follow-up is required to determine the impact of surgically treated TR on long-term survival and the durability of surgical techniques. doi: 10.1111/jocs.12414 (J Card Surg 2014;29:698–704)

Background Autograft dilatation (AD) and aortic insufficiency (AI) after the Ross procedure are the most common causes of late autograft failure. The purpose of this study was to examine the results of valve-sparing root replacement (modified David) and composite root replacement. Methods We performed a retrospective review of all children (n = 78) undergoing a Ross procedure at our Center from 1993 to 2011. Results Median follow-up was 10 years (1to 18 years). Freedom from autograft reoperation was 94% at 5 years, and 65% at 15 years. Freedom from greater than 2+ autograft AI was 93% at 5 years and 76% at 15 years. Autograft reoperation was necessary in 22 patients, at a median interval of 8.7 years after the original procedure. Indications for reoperation were AI with autograft dilatation in 15 patients, AI without dilatation in 2 patients, and AD without AI in 5 patients. Surgical procedures used at reoperation included valve-sparing root replacement in 14 patients, root replacement either mechanical or biologic valved conduit in 6 patients, and valve replacement in 2 patients. At a mean follow-up of 5.8 years after reoperation, 4 patients from the valve-sparing group underwent second reoperation (valve replacement). Freedom from second autograft reoperation was 71% for patients after a valve sparing procedure and 100% for patients after an aortic valve or root replacement (Bentall procedure) at 5 years. Conclusions Autograft valve-sparing root replacement and composite aortic root replacement are effective treatments for aortic root dilation and AI after the Ross procedure. The potential of late autograft insufficiency after valve-sparing root replacement warrants annual follow-up.

Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p \ 0.001). The follow- up data did not differ between the groups (HLHS 3.6 ± 3.2 years vs non-HLHS 4.8 ± 3.3 years, nonsignif- icant difference). The overall operative survival was 85 %: 83 % for the patients with HLHS and 89 % for the patients with non-HLHS (nonsignificant difference). The Kaplan- Meier transplant-free survival estimate at 10 years was 73 % and did not differ between the HLHS patients (67 %) and the non-HLHS patients (84 %, nonsignificant differ- ence). The timing of stages 2 and 3 palliation (bidirectional Glenn and external conduit Fontan) did not differ signifi- cantly (HLHS, 7.0 ± 2.8 months and 3.0 ± 0.5 years vs non-HLHS 6.1 ± 1.7 months and 2.8 ± 0.8 years). The two groups were similar with respect to the need for post- Norwood extracorporeal membrane oxygenation support, aortic arch reinterventions, atrioventricular valve repair, pulmonary artery reconstruction, pacemaker implantation, and progression to bidirectional Glenn and Fontan com- pletion. The modified Norwood reconstruction provides good palliation for HLHS as well as other forms of func- tional single-ventricle anatomy. Survival, postoperative morbidity, and progression to Glenn and Fontan are not significantly different.

Objective: Patients with double inlet left ventricle (DILV)/transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. The aim of this study is to evaluate the outcomes of initial pulmonary artery banding (PAB) compared with the Norwood-type reconstruction in neonates. Methods: A retrospective study of children with DILV and similar morphologies presenting between 1982 and 2012. The outcomes of initial PAB (n = 17) arecomparedwithaNorwood-typereconstruction(n = 20).Thetwogroupsweresimilarwithrespecttoage, gender, weight, noncardiac syndromes, and Fontan completion. Mean follow-up was longer in PAB patients (13.3 W 9.8years)versusNorwood(4.5 W 3.0years,p = 0.001).Results:Survivalwas75%ateightyearsinthe Norwood group versus 71% in the PAB group (p = 0.76). Mortality in the Norwood group was higher before 2002 (p = 0.06). The age of patients who underwent a bidirectional Glenn shunt was significantly higher for PAB group (PAB, 9.1 W 1.4 months vs. Norwood, 6.1 W 1.6 months; p < 0.001). Freedom from any type of reintervention (systemic outflow obstruction or coarctation) was similar (Norwood, 83% vs. PAB, 71%; p = 0.62). Freedom from heart block with a pacemaker insertion was significantly better for Norwood patients (Norwood, 89% vs. PAB, 41%; p = 0.001). Conclusions: The Norwood-type reconstruction provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining an unobstructed systemic outflow tract and avoiding the risk of heart block. Survival does not differ depending on the type of procedure. Patients with PAB show comparable satisfactory early and long- term results, with an increasing reoperation risk and heart block remaining a major concern. doi: 10.1111/ jocs.12171 (J Card Surg 2013;28:569-575)

In patients undergoing the Ross procedure, the right ventricular outflow tract (RVOT) conduit is inserted in an orthotopic position rather than in the more heterotopic position used in the repair of complex congenital RVOT obstruction. The study aim was to compare the authors' institutional mid-term experience of large-sized homografts (19 mm) in patients with Ross and non-Ross RVOT reconstructions.The outcome was reviewed of all homografts implanted for Ross (n = 72) or non-Ross (n = 64) RVOT reconstruction at a single center between 1993 and 2012. Echocardiographic data were reviewed to evaluate valve performance. Homograft dysfunction was defined as RVOT obstruction with peak echo-Doppler gradient40 mmHg and/or gradeIII/IV conduit valve regurgitation. Homograft failure was defined as the need for conduit replacement or catheter or surgical reintervention.The age, body weight, conduit diameter and previous surgery were significantly higher in patients with Ross compared to the non-Ross group (p = 0.002, 0.003 and0.001, respectively). The mean follow up duration was similar in both groups. There were 35 re-interventions (Ross, n = 17; non-Ross, n = 18). The data acquired showed actuarial survival, freedom from conduit dysfunction and conduit failure to be similar in both cohorts. Freedom from any type of reoperation was worse for the Ross group (58%) than for the non-Ross group (72%) (p = 0.05).During the first 15 years after Ross or non-Ross pulmonary homograft implantation, the survival rate, freedom from failure and dysfunction, and RVOT gradient were statistically similar. Freedom from any type of reoperation was significantly higher in the non-Ross group, however.

The objective of this prospective, observational study with consecutive sampling was to assess the reliability, bias, and precision of Nellcor N-395 (N) and Masimo SET Radical (M) pulse oximeters in children with cyanotic congenital heart disease and children with congenital heart disease recovering from cardiopulmonary bypass-assisted surgery admitted to a cardiovascular operating suite and pediatric intensive care unit at a tertiary care community hospital. Forty-six children with congenital heart disease were studied in 1 of 2 groups: (1) those recovering from cardiopulmonary bypass with a serum lactic acid > 2 mmol/L, and (2) those with co-oximetry measured saturations (SaO 2) < 90% and no evidence of shock. Measurements of SaO 2 of whole blood were compared to simultaneous pulse oximetry saturations (SpO 2). Data were analyzed to detect significant differences in SpO 2 readout failures between oximeters and average SpO 2 - SaO 2 ± 1 SD for each oximeter. A total of 122 SaO 2 measurements were recorded; the median SaO 2 was 83% (57 - 100%). SpO 2 failures after cardiopulmonary bypass were 41% (25/61) for N versus 10% (6/61) for M ( P < .001). There was a significant difference in bias (ie, average SpO 2 - SaO 2) and precision (± 1 SD) between oximeters (N, 1.1 ± 3.3 vs M, -0.2 ± 4.1; P < .001) in the postcardiopulmonary bypass group but no significant difference in bias and precision between oximeters in the cyanotic congenital heart disease group (N, 2.9 ± 4.6 vs M, 2.8 ± 6.2; P = .848). The Nellcor N-395 pulse oximeter failed more often immediately after cardiopulmonary bypass than did the Masimo SET Radical pulse oximeter. SpO2 measured with both oximeters overestimated SaO2 in the presence of persistent hypoxemia.

Background/Purpose: Hospital mortality rate among children with hypoplastic left heart syndrome (HLHS) after cardiac repair is well documented, but comparable data after noncardiac, surgical procedures are unknown. The authors hypothesized an increasing number of noncardiac procedures were being performed on children with HLHS, less than 2 years of age, from 1988 to 1997, and that these procedures were associated with a substantial mortality rate. Methods: A retrospective review of hospital discharge data for 2,457 children less than 2 years of age with HLHS for 1988 through 1997 was performed. The authors examined the outcomes of HLHS children undergoing only noncardiac surgical procedures during their hospital stay. Differences in hospital mortality rates between 1988 through 1992 versus 1993 through 1997 were assessed using the X 2 square statistic. Results: Nineteen percent of the 147 children with HLHS undergoing noncardiac, surgical procedures died (95% CI, 13% to 25%). Comparing the 2 study periods, there was no significant change in outcome among HLHS children undergoing noncardiac, surgical procedures (78% v. 83%; P >.1). There was no significant difference in the percentage of hospital discharges with noncardiac, surgical procedures performed per year. Conclusions: Although children with HLHS were not undergoing an increase in the number of noncardiac surgical procedures performed annually, even minor surgical procedures were associated with considerable mortality. Outcomes after noncardiac surgery in high-risk children with congenital heart disease warrant further investigation. J Pediatr Surg 37:1399-1403. Copyright 2002, Elsevier Science (USA). All rights reserved.

Tricuspid valve regurgitation (TR) is a common finding in children with hypoplastic left heart syndrome (HLHS) undergoing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation due to annular dilatation. The purpose of this study was to determine the incidence of severe TR and to evaluate the effect of surgically treated tricuspid valve (TV) disease on overall survival.Between December 1988 and December 2012, 86 patients with HLHS underwent a Norwood procedure (mean age/weight, 11.1 days/3.5 kg). Thirty-five percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 65 patients underwent a bidirectional Glenn procedure or a hemi-Fontan (stage II) and 42 patients a modified Fontan procedure (stage III). The mean follow-up was 6.6 years. All patients were evaluated preoperatively and postoperatively by Doppler echocardiography to determine the degree of TR.At the time of discharge following the Norwood procedure, 64% (n = 47) of the patients had none/trivial, 32% (n = 24) had mild, 3% had moderate (n = 2), and 1% (n = 1) had severe TR on their post-Norwood Doppler echocardiograms. Among 74 Norwood survivors, 11 children (15%) with severe TR underwent TV repair at a median interval after the Norwood procedure of nine months (range, 2-70). There were one surgical and one late death, giving an overall mortality of 18%. The overall survival rate between TR and non-TR repair groups was not significantly different (p = 0.18). Four patients (36%) underwent reoperation for persistent TR.Severe TR as a risk factor for mortality in children undergoing palliative procedures for HLHS can be diminished by successful TV procedures. Survival at intermediate follow-up of patients with successful TV procedures that eliminate severe TR and patients without a history of severe TR are similar. Further follow-up is required to determine the impact of surgically treated TR on long-term survival and the durability of surgical techniques.

Background . Early implantation of centrifugal devices in patients with postcardiotomy cardiogenic shock may provide a bridge to recovery and allow subsequent long-term survival. Methods . Since January 1989, 62 patients were supported with centrifugal pumps because of failure to wean from cardiopulmonary bypass. Indications were postcardiotomy cardiogenic shock (PCCS) (n = 60), bridge to cardiac retransplantation (n = 1), and right ventricular failure (n = 1). Patients' ages ranged from 23 to 78 years; 40 were men (65%), and 22 were women (35%). Twenty-two patients (35%) had a left ventricular assist device; 9 patients (15%) had a right ventricular assist device; and 31 patients (50%) had a biventricular assist device. Length of support ranged from 1 day to 19 days. Results . Forty-two patients (68%) were weaned successfully; 27 patients survived to discharge (44%). Complications included bleeding (n = 41, 66%), renal failure (n = 28, 45%), and respiratory failure (n = 26, 42%). Currently, 23 patients survived 10 or more years (n = 1), 6 to 10 years (n = 7), 1 to 5 years (n = 10), and less than 1 year (n = 5). Conclusions . Centrifugal pumps are available, easy to use, and relatively inexpensive. Our experience justifies their continued use as a bridge to recovery for patients with postcardiotomy cardiogenic shock, despite the availability and increasing use of more expensive devices.

Allograft valves have been used in cardiac valve replacement for over 40 years. Early failures of allograft mitral valve replacement (AMVR) were attributed to inadequate allograft preservation or unsuccessful implantation techniques. Due to an improved understanding of mitral valve function gained from experience in mitral valve reconstruction, improved imaging techniques for preoperative assessment, and improved methods of allograft preservation, there has been a resurgence of interest in AMVR. The use of bioprosthetic valves in children and young adults is characterized by reduced durability and early valve failure. With the use of mechanical valves, there is a significant long-term risk of morbidity and mortality resulting from thrombosis or hemorrhagic complications from anticoagulation. Additionally, anticoagulation is often difficult to manage in children. The advantages of using allograft mitral valves include the avoidance of anticoagulation and the preservation of the subvalvar apparatus and its role in ventricular function. At our institution, successful early results with AMVR have been obtained in eight children and young adults with previous atrioventricular septal defects, previous placement of mitral valve prostheses, rheumatic valvular disease, and bacterial endocarditis. Our early results and those of others are encouraging for the continued use of AMVR in children and young adults. Long-term follow-up will be necessary to assess the durability and function of these allograft mitral valves. Copyright 1999 by W.B. Saunders Company

Patients with double inlet left ventricle (DILV)/transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. The aim of this study is to evaluate the outcomes of initial pulmonary artery banding (PAB) compared with the Norwood-type reconstruction in neonates.A retrospective study of children with DILV and similar morphologies presenting between 1982 and 2012. The outcomes of initial PAB (n=17) are compared with a Norwood-type reconstruction (n=20). The two groups were similar with respect to age, gender, weight, noncardiac syndromes, and Fontan completion. Mean follow-up was longer in PAB patients (13.3 ± 9.8 years) versus Norwood (4.5 ± 3.0 years, p=0.001).Survival was 75% at eight years in the Norwood group versus 71% in the PAB group (p=0.76). Mortality in the Norwood group was higher before 2002 (p=0.06). The age of patients who underwent a bidirectional Glenn shunt was significantly higher for PAB group (PAB, 9.1 ± 1.4 months vs. Norwood, 6.1 ± 1.6 months; p0.001). Freedom from any type of reintervention (systemic outflow obstruction or coarctation) was similar (Norwood, 83% vs. PAB, 71%; p=0.62). Freedom from heart block with a pacemaker insertion was significantly better for Norwood patients (Norwood, 89% vs. PAB, 41%; p=0.001).The Norwood-type reconstruction provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining an unobstructed systemic outflow tract and avoiding the risk of heart block. Survival does not differ depending on the type of procedure. Patients with PAB show comparable satisfactory early and long-term results, with an increasing reoperation risk and heart block remaining a major concern.

A variety of valve substitutes has been used for right ventricular outflow tract (RVOT) reconstruction in children and young adults following previous RVOT surgery that has led to significant pulmonary insufficiency and/or stenosis. Herein, the authors' experience with pulmonary valve replacement (PVR) using a stented bovine pericardial xenograft late after previous RVOT surgery was reviewed.Between 2000 and 2011, a total of 49 patients (mean age 22.4 +/- 12.3 years; range: 5-50 years) underwent PVR using stented bovine pericardial xenografts. All patients had prior RVOT reconstruction; these patients averaged two prior operations (range: 1-4) for the following diagnoses: tetralogy of Fallot (n = 28), pulmonary atresia (n = 6), pulmonary stenosis (n = 6), transposition of the great arteries (n = 3), truncus arteriosus (n = 1), and others (n = 5). Of these patients, 24 (49%) underwent additional procedures at the time of pulmonary valve insertion.There was no early death, but six late deaths. The overall survival rate was 88% at 10 years. All surviving patients were well at a mean follow up of 4.0 +/- 2.3 years (range: 6 months to 10 years). Echocardiography showed trivial or no pulmonary insufficiency in 22 patients (56%). The calculated mean peak systolic RVOT gradient at the last follow up by echocardiography was 22.7 +/- 6.6 mmHg. Freedom from RVOT reintervention and xenograft explantation was 94% and 98% at 10 years, respectively.The stented bovine pericardial xenografts demonstrated excellent intermediate-term results for repeat RVOT reconstruction. The hemodynamic characteristics of this valve are comparable to those of allografts, and the xenograft represents is an attractive alternative given the limited availability of allografts. The stented bovine pericardial xenograft remains an ideal valve choice for teenagers and young adults with congenital abnormalities of the RVOT, especially for redo as a second or third choice.

Background We analyzed the outcome of children and young adults (younger than 40 years) with aortic valve disease who underwent allograft or autograft aortic root replacement (ARR) in our institution and evaluated whether there is a preference for either valve substitute. Methods One-hundred fifty patients younger than 40 years underwent ARR between January 1990 and July 2011. Forty-four patients, aged 18.8 ± 12.4 years, had ARR with allograft conduit (allograft group), whereas 106 patients, aged 17.9 ± 11 years (p = 0.63), had a Ross ARR during the same period of time (autograft group). Echocardiographic data were reviewed to evaluate valve performance. The 2 groups were similar with respect to age, gender, etiology, and previous and concomitant procedures. Results Operative deaths were 3 in the autograft group. There were 6 late deaths in the autograft group and 5 in the allograft group. Survival was 92% and 84% at 5 and 15 years, respectively, in the allograft group versus 93% and 91% in the autograft group (p = 0.42). Freedom from any type of reintervention and from reoperation on aortic valve were similar (autograft, 64% and 72% versus allograft, 66% and 66%; p = not significant) at 15 years. Freedom from explantation were significantly better for Ross patients (autograft, 82% versus allograft, 66%; p = 0.05). Conclusions Aortic valve replacement with either the autograft or allograft provides good clinical results in children and young adults during an intermediate duration of observation. Survival early after ARR does not differ depending on the type of prosthesis. In patients with aortic valve disease, autograft and allograft ARR show comparable satisfactory early and long-term results, with the increasing reoperation risk in the second decade after operation remaining a major concern.

For children who require aortic valve replacement, the pulmonary autograft (Ross procedure) may be the ideal substitute. However, performing a modified Konno procedure at the time of autograft implantation (Ross-Konno) may be associated with significant morbidity and mortality. A retrospective study was undertaken to compare the outcomes of Ross-Konno (RK) and the Ross (R) procedures including the need for reinterventions and long-term survival.Between 1993 and 2011, 78 children (mean age, 11.1 ± 5.6 years; range, 1 week to 18 years) underwent the Ross procedure. Modified Konno-type enlargement of the left ventricular outflow tract was performed in 18 of those patients.There was no statistically significant difference between the groups with respect to pathologic process, sex, concomitant procedures, and aortic gradient. Our data demonstrate that mean age (R, 12.9 years versus RK, 5.3 years; p0.001), mean size of allograft (R, 23.3 mm versus RK, 20.1 mm; p0.001), previous surgery (R, 51% versus RK, 83%; p = 0.05), and postoperative morbidity (R, 3% versus RK, 28%; p = 0.003) were significantly different between the groups. There were 3 hospital deaths (all RK with mitral valve anomalies). Actuarial survival at 10 years was significantly better for Ross patients than Ross-Konno (R, 96% versus RK, 72%; p = 0.001). Freedom from autograft, right ventricular outflow tract obstruction, and cumulative reoperations at 10 years were not significantly different between groups.The risk of death and postoperative complications after the Ross-Konno procedure is higher than for the Ross procedure. Preoperative complexity (including mitral valve anomalies) is associated with significantly higher morbidity and mortality. Autograft insufficiency and right ventricular outflow tract obstruction are common postoperative complications, requiring reoperation in one quarter of patients, but these were not significantly different between the groups.

Postpump chorea (PPC) is the development of choreoathetoid movements within 2 weeks following cardiopulmonary bypass. Over a 10-year period, 668 children underwent open cardiac surgery, of whom 8 (1.2%) developed PPC. Age at surgery ranged from 8 to 34 months. The onset of chorea was 3 to 12 days following surgery. Computed tomography and magnetic resonance imaging showed atrophy but no focal lesions. Cerebral positron emission tomography using [18F]fluorodeoxyglucose in a patient following 12 months of chorea showed patchy areas of decreased glucose metabolism. None of the patients were developmentally normal 22 to 130 months following surgery. Three patients have had transient and 5 have persistent chorea. Neurological deficits ranged from a mild learning disability to progressive hypotonia and obtundation ending in death. One of 4 patients who received haloperidol had a decrease in the severity of chorea. We compared PPC patients with 39 randomly selected controls. During surgery, affected patients spent significantly more time on pump and at temperatures under 36 degrees C, were cooled to lower temperatures than controls, and were more likely to have had a circulatory arrest. One patient developed chorea without a history of circulatory arrest. We conclude that (1) there is a strong association between PPC, deep hypothermia, and circulatory arrest, (2) absence of characteristic macroscopic changes suggests a biochemical or microembolic etiology in some cases, (3) chorea is frequently associated with developmental delay, and (4) the prognosis for complete resolution of chorea is guarded.

We report the case of a 29-year-old man who suffered sub-arachnoid bleeding while stabilized on a biventricular assist device as a bridge to cardiac transplantation. We adjusted his anti-coagulation therapy to control the bleeding and to concurrently minimize thrombosis while on support. He underwent 2 craniotomy operations to evacuate sub-arachnoid hematomas, and he underwent a subsequent operation to debride and close the dura. Eighteen days later, he underwent successful orthotopic heart transplant and was discharged to home 3 weeks post-transplant.

A 29-year-old gravid female presented at 22 weeks gestation with an acute Type I aortic dissection and coarctation of the aorta. She underwent emergent repair of her aortic dissection using cardiopulmonary bypass and hypothermia. At 25 weeks gestation, she underwent repair of her coarctation of the aorta. The patient had a cesarean delivery of a viable, normal male infant at 39 weeks gestation.

Objective Children's digits are often too small for proper attachment of oximeter sensors, necessitating sensor placement on the sole of the foot or palm of the hand. No study has determined what effect these sensor locations have on the accuracy and precision of this technology. The objective of this study was to assess the effect of sensor location on pulse oximeter accuracy (i.e., bias) and precision in critically ill children. Design Prospective, observational study with consecutive sampling. Setting Tertiary care, pediatric intensive care unit. Patients Fifty critically ill children, newborn to 2 yrs of age, with an indwelling arterial catheter. Forty-seven of 50 (94%) patients were postcardiac surgery. Interventions None. Measurements and main results Co-oximeter-measured arterial oxygen saturation (Sao2) was compared with simultaneously obtained pulse oximetry saturations (Spo2). A total of 98 measurements were obtained, 48 measurements in the upper extremities (finger and palm) and 50 measurements in the lower extremities (toe and sole). The median Sao2 was 92% (66% to 100%). There was a significant difference in bias (i.e., average Spo2 - Sao2) and precision (+/-1 sd) when the sole and toe were compared (sole, 2.9 +/- 3.9 vs. toe, 1.6 +/- 2.2, p = .02) but no significant difference in bias and precision between the palm and the finger (palm, 1.4 +/- 3.2 vs. finger, 1.2 +/- 2.3, p = .99). There was a significant difference in bias +/- precision when the Sao2 was or=90% in the sole (6.0 +/- 5.7 vs. 1.8 +/- 2.1, p = .002) and palm (4.5 +/- 4.5 vs. 0.7 +/- 2.4, p = .006) but no significant difference in the finger (1.8 +/- 3.8 vs. 1.1 +/- 1.8, p = .95) or toe (1.9 +/- 2.9 vs. 1.6 +/- 1.9, p = .65). Conclusions The Philips M1020A pulse oximeter and Nellcor MAX-N sensors were less accurate and precise when used on the sole of the foot or palm of the hand of a child with an Sao2

Techniques for pediatric cardiac surgery requiring cardiopulmonary bypass (CPB) have significantly improved over the years. The use of fresh whole blood (FWB) and pre-bypass ultrafiltration (PBUF) has been suggested as means for improving perioperative and postoperative outcomes. It is the intent of this study to show that fresh whole blood along with PBUF will result in balanced CPB prime that can offer a reduction in blood product exposures and blood loss along with improving several measured postoperative outcomes. After institutional review board approval, a retrospective review was conducted on 100 patients to analyze the benefits of FWB and PBUF on outcomes in neonatal and pediatric cardiac surgery. Data analysis included preoperative and CPB data, perioperative inotrope and blood product exposure, and postoperative blood loss and blood product exposure measured for up to a 12-hour period in the intensive care unit (ICU). The three groups compared were FWB prime vs. packed red blood cell (PRBC) prime

Pediatric patients who have preoperative hemodynamic instability or postoperative cardiac decompensation may frequently require the use of extracorporeal membrane oxygenation (ECMO) for stabilization of cardiac and respiratory function. While ECMO can be a therapeutic treatment for the congenital pediatric patient, it does not allow the additional functions of a complete cardiopulmonary bypass (CPB) circuit should subsequent surgical revision in the operating room be required. This paper will discuss our approach to converting the ECMO circuit to total cardiopulmonary bypass allowing the use of cardioplegia, cardiotomy suction, and modified ultrafiltration. This technique allows the conversion to CPB without ceasing support to the critically ill patient or exposing them to additional blood products or surface area in the priming of a new extracorporeal circuit. In addition, this circuit design allows for the resumption of ECMO support utilizing the same circuit if the patient necessitates it.

A 13-year-old Haitian girl presented with complex congenital heart disease consisting of transposition of the great arteries with total anomalous venous return. The patient exhibited clinical findings of long-standing congestive heart failure and cyanosis. She underwent complete repair with resolution of her symptoms.

Background. Recent reports have demonstrated successful early outcomes using mitral valve homografts in adults. We report our early results after homograft mitral valve replacement in 4 children with previous atrioventricular septal defects, previous placement of a prosthetic valve, and rheumatic valvular disease. Methods. Between May 1996 and June 1997, 4 children (ages 5, 11, 13, and 15 years) underwent mitral valve replacement with cryopreserved mitral valve homografts at our institution. Preoperative echocardiography confirmed moderately severe to severe mitral regurgitation, stenosis, or both in all 4 patients. Results. Successful homograft valve replacement was achieved in all 4 patients. Based on symptoms, physical examinations, and echocardiographic follow-up, all four homograft mitral valves are functioning well with normal hemodynamics. None of these patients are receiving warfarin. Follow-up has been limited to 10 months. Conclusions. In children requiring mitral valve replacement, the use of mitral valve homografts offers advantages over prosthetic valves, such as the avoidance of complications associated with thrombosis and anticoagulation. Homograft mitral valve replacement is technically feasible in children with congenital and rheumatic heart disease and previous prosthetic valves.

Ninety-two children underwent aortic (AVR) or mitral valve replacement (MVR), or both, at the Hospital for Sick Children in Toronto from 1963 to February, 1980. No early or late deaths occurred in 39 children having AVR. However, in 50 children having MVR, the operative mortality was 32% and the actuarial survival rate 5 years after operation was only 50%. Major complications occurred with almost equal frequency in the two groups; 50% of children surviving AVR or MVR experienced major complications within 6 years of operation. Retrospective comparison of results with tissue and mechanical valves showed no clear advantage with either type of prosthesis. Outgrowth of a prosthetic valve was satisfactorily managed in children with AVR but presented a difficult problem in those with MVR, who required frequent reoperation to increase the prosthetic valve to adult size. Prosthetic valve replacement in children is a palliative procedure at best, and every effort should be made to preserve the natural valve by a more conservative repair technique.

Background Standard cryopreserved valved allografts (SCAs) are recognized as the benchmark for reconstruction of the right ventricular outflow tract (RVOT). However, SCAs frequently demonstrate early valve deterioration and elicit an immune response. Decellularized cryopreserved valve allografts (SynerGraft, SG) are less immunogenetic and may be more durable. This study analyzed our results of RVOT reconstruction using SGs and compared it with the SCAs used during the same period. Methods We reviewed the outcome of all allografts (SG and SCA) that were implanted for RVOT reconstruction at a single center from 2000 to 2005. Echocardiographic data were reviewed to evaluate valve performance. Conduit failure is defined as the need for conduit replacement or reintervention in either the catheterization laboratory or operating room. Conduit dysfunction is defined as RVOT obstruction with peak echocardiographic Doppler gradient greater than 40 mm Hg and/or grade III/IV or greater conduit valve regurgitation. Data were compared using the Wilcoxon rank sum and Fisher's exact test. Results From January 2000 to April 2005, 100 patients (mean age 18.6 ± 16.8 years) received SG (n = 39) or SCA (n = 61) conduits. The 2 retrospective nonrandomized cohorts were similar with respect to age, gender, weight, conduit indication, bypass and crossclamp time, and conduit size. Follow-up time was not significant between the 2 groups (SG, 5.7 ± 2.5 years vs SCA, 5.8 ± 2.8 years; P = .83). Early and late mortality were similar (SG, 13%; SCA, 10%; P = .75). No death was graft related. Freedom from dysfunction was superior with SG (SG, 74%, vs SCA, 52%; P = .05). Freedom from failure was also better in patients with SG (SG, 87%, vs SCA, 68%; P = .05). Freedom from explantation and more than moderate pulmonary insufficiency were significantly better for SG patients (SG, 92% and 90%, vs SCA, 78% and 68%; P = .02). Conclusions This study suggests that the midterm performance of SGs may be superior to that of SCAs. Decellularization of the cryopreserved allografts may provide a more durable option for patients who need RVOT reconstruction. Further long-term follow-up is needed to see whether this decellularization process improves long-term allograft durability.

A case is reported of acute thrombosis of the superior vena cava (SVC) resulting from infusion of antibiotics via a subclavian catheter with its tip in the SVC. The patient was treated by operative thrombectomy instead of thrombolytic drugs. Eight months postoperativel)’, he remains asymptomatic.

Noncoronary collateral flow is a potential hazard when cold cardioplegia is used for myocardial protection. In this study, noncoronary collateral flow was observed during coronary revascularization in 58 patients. It was distributed significantly (p less than 0.005) toward arteries demonstrating intercoronary collateral flow angiographically - 1.0 of 35 with and 12 of 121 without. Preponderance of coronary collateral flow in hearts with more extensive disease may complicate cardioplegic myocardial protection for such patients.

Three infants developed greatly accelerated junctional ectopic tachycardia with a heart rate greater than 200 beats/min after open heart surgery. When the heart rate exceeded 200 beats/min for 5 hours, all the infants had congestive heart failure and clinical signs of low cardiac output. Conventional therapy (cardioversion, lidocaine, verapamil, digoxin and ice to face) has been shown in the past to be unsuccessful in controlling the heart rate. Because hypothermia is known to decrease automaticity of the heart, these patients were treated with induced hypothermia. The goal was to arbitrarily decrease the junctional ectopic rate to less than 180 beats/min to increase cardiac filling time. The duration of the junctional ectopic tachycardia greater than 180 beats/min ranged from 0.5 to 17 hours after cooling began. The duration of the hypothermia ranged from 4 to 24 hours. Spontaneous reversion to sinus rhythm occurred either during the hypothermia or shortly thereafter in all three patients. The blood pressure and urinary output remained stable during hypothermia. Hypothermia is an effective means of controlling the rate of greatly accelerated junctional ectopic tachycardia after open heart surgery in infants. Although hypothermia does not convert junctional ectopic tachycardia to sinus rhythm, it slows the rate to a more acceptable level, allowing the infants' survival and eventual recovery of sinus rhythm.

• Jejunoileal bypass was performed in 50 morbidly obese patients. The morbidity encountered compared favorably with that of other series. All patients manifesting hepatic failure demonstrated hyperbilirubinemia within the first three months postoperatively. Hyperbilirubinemia, if uncorrected, resulted in a mortality of 75%. Augmentation jejunal interposition was performed in three patients who demonstrated hepatic decompensation or severe electrolyte imbalance or both. This resulted in rapid correction of electrolyte disturbances, liver function measurements, and patient symptoms without significant postoperative weight gain. Persistent hyperbilirubinemia or recalcitrant electrolyte problems or both are indications for augmentation jejunal interposition. (Arch Surg111:1362-1365, 1976)