European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (1) pre-transplant evaluation.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment in end-stage liver disease. Imaging is a key element for successful organ-transplantation to assist surgical planning. So far, only limited data regarding the best radiological approach to prepare children for liver transplantation is available. OBJECTIVES: In an attempt to harmonize imaging surrounding pediatric liver transplantation, the European Society of Pediatric Radiology (ESPR) Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phase. This paper reports the responses on preoperative imaging. MATERIAL AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted and 22 institutions from 11 countries returned the survey. From 2018 to 2020, the participating centers collectively conducted 1,524 transplantations, with a median of 20 transplantations per center per annum (range, 8-60). RESULTS: Most sites (64%) consider ultrasound their preferred modality to define anatomy and to plan surgery in children before liver transplantation, and additional cross-sectional imaging is only used to answer specific questions (computed tomography [CT], 90.9%; magnetic resonance imaging [MRI], 54.5%). One-third of centers (31.8%) rely primarily on CT for pre-transplant evaluation. Imaging protocols differed substantially regarding applied CT scan ranges, number of contrast phases (range 1-4 phases), and applied MRI techniques. CONCLUSION: Diagnostic imaging is generally used in the work-up of children before liver transplantation. Substantial differences were noted regarding choice of modalities and protocols. We have identified starting points for future optimization and harmonization of the imaging approach to multicenter studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (2) intraoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of intraoperative and postoperative complications. So far, only limited data regarding the best radiological approach to monitor children during liver transplantation is available. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra- and postoperative phase. This paper reports the responses related to intraoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: Intraoperative ultrasound (US) is used by all sites to assess the quality of the vascular anastomosis in order to ensure optimal perfusion of the liver transplant. Vessel depiction is commonly achieved using color Doppler (95.3%). Additional US-based techniques are employed by fewer centers (power angio mode, 28.6%; B-flow, 19%; contrast-enhanced US, 14.3%). Most centers prefer a collaborative approach, with surgeons responsible for probe handling, while radiologists operate the US machine (47.6%). Less commonly, the intraoperative US is performed by the surgeon alone (28.6%) or by the radiologist alone (23.8%). Timing of US, imaging frequency, and documentation practices vary among centers. CONCLUSION: Intraoperative US is consistently utilized across all sites during pediatric liver transplantation. However, considerable variations were observed in terms of the US setup, technique preferences, timing of controls, and documentation practices. These differences provide valuable insights for future optimization and harmonization studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (3) postoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiologic approach to monitor children after liver transplantation. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phases. This paper reports the responses related to postoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: All sites commence ultrasound (US) monitoring within 24 h after liver transplantation. Monitoring frequency varies across sites, ranging from every 8 h to 72 h in early, and from daily to sporadic use in late postoperative phases. Predefined US protocols are used by 73% of sites. This commonly includes gray scale, color Doppler, and quantitative flow assessment. Alternative flow imaging techniques, contrast-enhanced US, and elastography are applied at 31.8%, 18.2%, and 63.6% of sites, respectively. Computed tomography is performed at 86.4% of sites when clarification is needed. Magnetic resonance imaging is used for selected cases at 36.4% of sites, mainly for assessment of biliary abnormalities or when blood tests are abnormal. CONCLUSION: Diagnostic imaging is extensively used for postoperative surveillance of children after liver transplantation. While US is generally prioritized, substantial differences were noted in US protocol, timing, and monitoring frequency. The study highlights potential areas for future optimization and standardization of imaging, essential for conducting multicenter studies.

Transjugular Intrahepatic Portosystemic Shunt Insertion for the Management of Portal Hypertension in Children.

OBJECTIVES: the aim of the study was to describe our 20-year experience with transjugular intrahepatic portosystemic shunt (TIPSS) procedures for children with resistant portal hypertension (PHTN). METHODS: Retrospective review of all children that had a TIPSS performed at Birmingham Children's Hospital from January 1, 1995 to January 1, 2015. RESULTS: Forty children underwent 42 attempted TIPSS for resistant PHTN with recurrent variceal bleeding (n = 35), refractory ascites (n = 4), and hypersplenism (n = 1). Median age at operation was 12 years (range 7 months-17 years). Thirty-four procedures were elective and 8 were emergency cases. TIPSS was established in 33 cases (79%). Median portal venous pressure reduction was 10 mmHg. Variceal bleeding ceased in 27 (96%) and ascites improved in all. Clinical improvement following TIPSS enabled 7 children to be bridged to transplantation and 7 others to become suitable for transplantation. The 1-year and 5-year survival with TIPSS was 57% and 35%, respectively. Child-Pugh score C was an independent risk factor for adverse outcome (Likelihood ratio (LR) = 8.0; 95% confidence interval (CI) 2.7 to 23.5; P = 0.001). There were 6 major complications: hepatic artery thrombosis and infarction (n = 1), hepatic artery pseudoaneurysm (n = 1), bile leak (n = 1), and hepatic encephalopathy (n = 3). Encephalopathy was resistant to medical treatment in 2 cases, necessitating staged closure in 1. Ten patients (30%) required intervention to maintain TIPSS patency. The 1-year and 5-year freedom from reintervention was 71% and 55%, respectively. CONCLUSIONS: A TIPSS is highly successful in controlling symptoms in children with resistant PHTN and facilitating liver transplantation. It is technically demanding and not without risk. Patients must be appropriately selected and counselled.

Autonomic cardio-respiratory reflex reactions and superselective ophthalmic arterial chemotherapy for retinoblastoma.

OBJECTIVE: To describe our experience with superselective ophthalmic artery chemotherapy (SOAC) in retinoblastoma and to report the serious adverse cardio-respiratory reactions we have observed. METHODS: SOAC was performed using a standardized protocol for general anesthesia, ophthalmic artery catheterization, and pulsed infusion of melphalan. Adverse reactions were defined as those in which the patient required active treatment to maintain cardio-respiratory stability. RESULTS: Between December 2008 and May 2012, 54 eyes in 52 patients were treated. 143 catheterization procedures were performed, with a technical success rate of 93% (n = 133). There were no deaths or major complications. Adverse cardio-respiratory reactions developed during 35 procedures (24%; 95% CI, 18-32%). All reactions occurred during second or subsequent catheterization procedures (39%; 95% CI, .29-49%) and were characterized by hypoxia, reduced lung compliance, systemic hypotension and bradycardia. Adverse events were successfully treated in all patients. One procedure was abandoned due to prolonged hemodynamic instability. CONCLUSION: Adverse cardio-respiratory reactions are commonly observed in SOAC for retinoblastoma. We believe that the adverse clinical signs represent an autonomic reflex response, akin to the trigemino-cardiac or oculo-respiratory reflexes, and all patients should be considered at-risk. Reactions occur only during second or subsequent procedures and can be life-threatening. The routine use of intravenous atropine does not seem to have altered the incidence or severity of these reactions. Anesthetists and interventional neuroradiologists involved in SOAC must be vigilant to ensure adverse reactions, when they develop, are treated quickly and effectively.

Bone Mineral Density and Vascular Calcification in Children and Young Adults With CKD 4 to 5 or on Dialysis.

Introduction: Older adults with chronic kidney disease (CKD) can have low bone mineral density (BMD) with concurrent vascular calcification. Mineral accrual by the growing skeleton may protect young people with CKD from extraosseous calcification. Our hypothesis was that children and young adults with increasing BMD do not develop vascular calcification. Methods: This was a multicenter longitudinal study in children and young people (5-30 years) with CKD stages 4 to 5 or on dialysis. BMD was assessed by tibial peripheral quantitative computed tomography (pQCT) and lumbar spine dual-energy X-ray absorptiometry (DXA). The following cardiovascular imaging tests were undertaken: cardiac computed tomography for coronary artery calcification (CAC), ultrasound for carotid intima media thickness z-score (cIMTz), pulse wave velocity z-score (PWVz), and carotid distensibility for arterial stiffness. All measures are presented as age-adjusted and sex-adjusted z-scores. Results: One hundred participants (median age 13.82 years) were assessed at baseline and 57 followed up after a median of 1.45 years. Trabecular BMD z-score (TrabBMDz) decreased (P = 0.01), and there was a nonsignificant decrease in cortical BMD z-score (CortBMDz) (P = 0.09). Median cIMTz and PWVz showed nonsignificant increase (P = 0.23 and P = 0.19, respectively). The annualized increase in TrabBMDz (ΔTrabBMDz) was an independent predictor of cIMTz increase (R 2 = 0.48, ß = 0.40, P = 0.03). Young people who demonstrated statural growth (n = 33) had lower ΔTrabBMDz and also attenuated vascular changes compared with those with static growth (n = 24). Conclusion: This hypothesis-generating study suggests that children and young adults with CKD or on dialysis may develop vascular calcification even as their BMD increases. A presumed buffering capacity of the growing skeleton may offer some protection against extraosseous calcification.

The burden of subclinical cardiovascular disease in children and young adults with chronic kidney disease and on dialysis.

BACKGROUND: Cardiovascular disease (CVD) is a common cause of morbidity and mortality even in young people with chronic kidney disease (CKD). We examined structural and functional CV changes in patients ˂30 years of age with CKD Stages 4 and 5 and on dialysis. METHODS: A total of 79 children and 21 young adults underwent cardiac computed tomography for coronary artery calcification (CAC), ultrasound for carotid intima-media thickness (cIMT), carotid-femoral pulse wave velocity (cfPWV) and echocardiography. Differences in structural (CAC, cIMT z-score, left ventricular mass index) and functional (carotid distensibility z-score and cfPWV z-score) measures were examined between CKD Stages 4 and 5 and dialysis patients. RESULTS: Overall, the cIMT z-score was elevated [median 2.17 (interquartile range 1.14-2.86)] and 10 (10%) had CAC. A total of 16/23 (69.5%) patients with CKD Stages 4 and 5 and 68/77 (88.3%) on dialysis had at least one structural or functional CV abnormality. There was no difference in the prevalence of structural abnormalities in CKD or dialysis cohorts, but functional abnormalities were more prevalent in patients on dialysis (P < 0.05). The presence of more than one structural abnormality was associated with a 4.5-fold increased odds of more than one functional abnormality (95% confidence interval 1.3-16.6; P < 0.05). Patients with structural and functional abnormalities [cIMT z-score >2 standard deviation (SD) or distensibility <-2 SD) had less carotid dilatation (lumen:wall cross-sectional area ratio) compared with those with normal cIMT and distensibility. CONCLUSIONS: There is a high burden of subclinical CVD in young CKD patients, with a greater prevalence of functional abnormalities in dialysis compared with CKD patients. Longitudinal studies are required to test these hypothesis-generating data and define the trajectory of CV changes in CKD.

Bilateral hydroureteronephrosis: A neonatal presentation of bladder duplication.

In a male neonate with bilateral hydroureteronephrosis, the most common surgical diagnosis is posterior urethral valves. This case report describes a male infant with the same presentation, but caused by a very uncommon congenital anomaly. The summation of different imaging modalities allowed a multidisciplinary team of colleagues to define the anatomy: bilateral duplex kidneys draining into separate urinary bladders. Only one of the bladders had an outlet, hence the obstructive uropathy to the right kidney led to total loss of function. The distended tortuous ureters produced a mass effect at presentation. This case acts as a reminder that complex congenital anomalies can mimic the presentation of more common conditions, and that they often require input from various specialists to diagnose the condition and guide its management.

Morphology of vascular ring arch anomalies influences prognosis and management.

OBJECTIVE: This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention. METHODS: This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data. RESULTS: Of those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery. CONCLUSION: Individuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.

Arterial switch operation in patients with Taussig-Bing anomaly--influence of staged repair and coronary anatomy on outcome.

OBJECTIVE: This study evaluated the results of arterial switch operation and closure of ventricular defects (ASO+VSDc) for double outlet right ventricle with sub-pulmonary ventricular septal defect (Taussig-Bing anomaly). METHODS: Between 1988 and 2003, 33 patients (25 male, 76%) with Taussig-Bing anomaly underwent ASO+VSDc (median age 39 days, 1 day-2.1 years). The relationship of the great arteries was antero-posterior (Group I, n=19) or side-by-side (Group II, n=14). Coronary anatomy (Yacoub's classification) was exclusively type A or D in Group I and predominantly type D or E in Group II (64%). Incidence of sub-aortic obstruction and aortic arch obstruction was similar in Group I and II (37% vs 57%, p=0.25 and 84% vs 79%, p=0.98, respectively). Twenty-five patients (76%) had one-stage total correction. Risk factors were analysed using multivariable analysis. Follow-up was complete (median interval of 6.2 years; range, 0.6-15.2 years). RESULTS: There were three early (9%) and one late death. Actuarial survival was 88+/-6% at 1 and 10 years. There were two early and four late re-operations. Freedom from re-operation was 90+/-5% and 75+/-9% at 1 and 10 years. Four patients required cardiological re-interventions. Freedom from re-intervention at 5 and 10 years was 79+/-9%. On multivariable analysis, complex coronary anatomy (type B and C) was a risk for early mortality (p<0.001) but all other anatomical variables and staged strategy did not influence early or actuarial survival. CONCLUSIONS: The ASO+VSDc approach can be applied to Taussig-Bing anomaly with acceptable mortality and morbidity and it is the procedure of choice at our institution. Anatomical variables did not influence outcomes with this strategy. A staged strategy is still appropriate in complex cases.

Creation of aorto-pulmonary window with pulmonary artery band is not good palliation for hypoplastic left heart syndrome.

OBJECTIVE: A small sub-group of patients with hypoplastic left heart syndrome (HLHS) have normal-sized ascending aorta and arch. An alternative to the Norwood I procedure in these patients is the creation of an aorto-pulmonary (AP) window with a distal pulmonary artery band (PAB). We reviewed our experience with this technique and compared outcomes to the Norwood procedure for HLHS. METHODS: All patients treated for HLHS in a single institution between 1992 and 2005 were analysed. This identified 13 patients treated with AP window and PAB compared to 333 patients undergoing stage I Norwood procedure. An unrestrictive AP window was created and the main PA was banded. Patient records and echocardiograms were analysed. Median follow-up was 10 (IQR 0-655) days and 100% complete. RESULTS: There were seven early deaths (54%) in the AP window group and two conversions to Norwood circulation. This was a significantly worse outcome than for the Norwood procedure over the same period, which had an early mortality of 29% (p=0.03). Kaplan-Meier actuarial analysis demonstrated a continued survival benefit of the Norwood group at 6 months (p=0.0005). Deaths were due to either low cardiac output syndrome (n=4) or sudden unheralded arrest (n=3). This occurred despite aortic cross-clamp and circulatory arrest times being significantly lower in the AP window group compared to the Norwood group (35+/-27 vs 55+/-16 min, p<0.01 and 16+/-29 vs 55+/-20 min, p<0.01, respectively). No differences in arterial saturations or systolic blood pressure existed between the groups, but diastolic blood pressure was significantly lower in the AP window group at 27+/-10 mmHg compared to 42+/-8 mmHg in the Norwood group (p=0.01) with evidence of flow reversal in the descending aorta. Differences in diastolic blood pressure between groups were abolished after conversion to stage II. CONCLUSIONS: Despite favourable anatomy and shorter ischaemic times, the AP window/PAB technique has a poor outcome compared to the Norwood procedure for HLHS. Low diastolic blood pressure with reversal of descending aortic flow in diastole was a feature of the AP window/PAB circulation. We recommend the Norwood procedure for these sub-types. This may have implications for newer 'hybrid' procedures for HLHS which create a similar palliative circulation.

Washing of irradiated red blood cells prevents hyperkalaemia during cardiopulmonary bypass in neonates and infants undergoing surgery for complex congenital heart disease.

OBJECTIVE: High concentrations of potassium and lactate in irradiated red cells transfused during cardiopulmonary bypass may have detrimental effects on infants and neonates undergoing cardiac surgery. The effects of receiving washed and unwashed irradiated red cells from the cardiopulmonary circuit on serum potassium and lactate concentrations were compared. METHODS: The study population included neonates and infants undergoing heart surgery for complex congenital heart disease. A control group (n=11) received unwashed irradiated red cells and the study group (n=11) received irradiated red cells washed in a cell saver (Dideco Electa) using 900ml of 0.9% saline prior to pump priming. Potassium and lactate concentrations were compared before, during and after bypass. RESULTS: Washing irradiated red cells reduced donor blood [potassium] from>20 to 0.8+/-0.1mmol/l, and [lactate] from 13.7+/-0.5 to 5.0+/-0.3mmol/l (p<0.001). The resulting prime had significantly lower [potassium] and [lactate] than the unwashed group (potassium 2.6+/-0.1 vs 8.1+/-0.4mmol/l, p<0.001; lactate 2.6+/-0.2 vs 4.6+/-0.3mmol/l, p<0.001). Peak [potassium] in the unwashed group occurred 3 minutes after going on bypass (4.9+/-0.3mmol/l) and during rewarming (4.9+/-0.4mmol/l). These were significantly higher than the washed group (3.1+/-0.1, p<0.001 and 3.0+/-0.1mmol/l, p<0.001). The [potassium] was greater than 6.0mmol/l for 4 out of these 11 unwashed patients compared with none of the washed group. Immediately post-bypass the washed group had significantly lower serum [potassium] (3.2+/-0.1 vs 4.2+/-0.2mmol/l, p=0.002). There was no significant difference in [lactate] between groups during and after cardiopulmonary bypass. CONCLUSIONS: The washing of irradiated red cells reduces potassium and lactate loads and prevents hyperkalaemia during cardiopulmonary bypass. The washing of irradiated red cells should be considered in neonates and infants undergoing cardiac surgery for complex congenital heart disease.

Factors influencing early and late outcome following the Fontan procedure in the current era. The 'Two Commandments'?

OBJECTIVE: This study was undertaken to identify the factors affecting early and late outcome following the Fontan procedure in the current era. We have examined whether conventional selection criteria, the 'Ten Commandments', are still applicable in the current era. MATERIALS AND METHODS: Between January 1988 and July 2004, 406 patients underwent a modified Fontan procedure at a median age of 4.7 years (IQR, 3.8-7.1 years). The single functional ventricle was of left (n=241, 59%) or right ventricular morphology (n=163, 40%). The modified Fontan procedure was performed using an atriopulmonary connection (n=162, 40%) or total cavopulmonary connection (TCPC) involving a lateral atrial tunnel (n=50, 12%) or extracardiac conduit (n=194, 48%). They were fenestrated in 216 patients (53%). RESULTS: The early mortality was 4.4% (n=18) and four other patients required takedown of the Fontan circulation. On multivariable analysis, early outcome was adversely influenced by two factors (p<0.05): preoperative impaired ventricular function and elevated pulmonary artery pressures. Two risk models were constructed for early outcome based on preoperative and predictable operative variables (Model 1) and all preoperative and operative data (Model 2). Both models were calibrated across all deciles (p=0.83, p=0.25) and discriminated well. The area under the ROC curve was 0.85 and 0.89, respectively. There were 21 late deaths, 1 patient required late takedown of the Fontan circulation and 3 required orthotopic cardiac transplantation. Actuarial survival was 90+/-2%, 86+/-2% and 82+/-3% at 5, 10 and 15 years, respectively. Multivariable analysis identified that outcome was influenced by preoperatively impaired ventricular function, elevated preoperative pulmonary artery pressures and an earlier year of operation. The freedom from reintervention was 83+/-4%, 76+/-4% and 74+/-8% at 5, 10 and 15 years, respectively. Additional risk factors for reintervention were right atrial isomerism and preoperative small pulmonary artery size. CONCLUSIONS: Late outcome of the Fontan circulation is encouraging. Ventricular morphology, surgical technique and fenestration do not appear to influence early or late outcome. Preoperatively impaired ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor.

Intention-to-treat analysis of pulmonary artery banding in conditions with a morphological right ventricle in the systemic circulation with a view to anatomic biventricular repair.

BACKGROUND: Some patients with a morphological right ventricle (mRV) in the systemic circulation require early intervention because of progressive systemic ventricular dysfunction or atrioventricular valve regurgitation. They may be eligible for anatomic repair (correction of atrioventricular and ventriculoarterial discordance) but require prior training of the morphological left ventricle (mLV). METHODS AND RESULTS: Forty-one patients with congenitally corrected transposition of the great arteries or a previous atrial switch procedure embarked on a protocol of pulmonary artery (PA) banding with a view to anatomic repair. All had an mRV in the systemic circulation and a subpulmonary mLV that was not conditioned by either volume or pressure load. Two patients were not banded, and 39 were followed up for a median of 4.3 years (range, 25 days to 12.6 years). Sixteen patients achieved anatomic repair, with 3 in the early stages of the training protocol. After 2 years, 12 patients were not suitable for anatomic repair and persisted with palliative banding; 8 were functionally improved; and 4 died, underwent transplantation, or required debanding. PA banding improved functional class but did not improve tricuspid regurgitation in the long term for patients not achieving anatomic repair. mLV function was a critical determinant of survival with a PA band as well as survival after anatomic repair. Patients >16 years were unlikely to achieve anatomic repair. CONCLUSIONS: PA banding is a safe and effective method of training the mLV before anatomic repair. It is also an effective palliative procedure for those who do not attain this goal.

Risk assessment and early outcome following the Norwood procedure for hypoplastic left heart syndrome.

BACKGROUND: This study was undertaken to identify risk factors for early mortality following the Norwood procedure for hypoplastic left heart syndrome (HLHS) and develop a predictive risk model to monitor clinical performance. METHODS AND RESULTS: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure at a single institution. The early mortality was 29% (n=95). Estimated early mortality improved progressively and was 10% at the end of the series. Multivariable analysis identified that body surface area at operation, size of the ascending aorta, preoperative right ventricular function and source of pulmonary blood flow established at operation were risk factors for early mortality (P<0.05). These variables were included in a preoperative risk model. The duration of cardiopulmonary support was an independent risk factor, which was included in a separate operative risk model. The performance of the risk models was evaluated by goodness-of-fit analyses, using the Hosmer-Lemeshow test and receiver operating characteristic (ROC) curve. Both models were well calibrated across all deciles (P=0.64, P=0.77) and discriminated moderately well. The area under the ROC curve was 0.71 for Model 1 and 0.75 for Model 2. Risk adjustment broadly accounted for the variation in early mortality observed during this series. CONCLUSIONS: Patient-related and predetermined operative variables have a major influence on the early outcome following the Norwood procedure for HLHS. The identification of these risk factors allows the risk of early mortality to be calculated. This information could be applied as part of a risk-adjusted performance-monitoring system to enable early identification of meaningful changes in practice.

Fate of pulmonary arteries following Norwood Procedure.

OBJECTIVE: This study evaluated the requirement for surgical reoperation and catheter-based reintervention to central pulmonary arteries (CPAs) following Norwood Procedure (NP). We sought to identify the influence of various surgical techniques employed during NP on subsequent interventions. METHODS: Between 1993 and 2004, 226 patients underwent Stage II following NP. Ninety-eight patients (43%) had completion of Fontan circulation (Stage III) and a further 107 (47%) are on course for Fontan completion with 21 (9%) inter-stage deaths. During NP, the aortic arch was reconstructed without additional material (n = 91, 40%) or with a pulmonary homograft patch (n = 135, 60%). Pulmonary blood flow was supplied by modified Blalock-Taussig shunt (n = 177, 78%) or right ventricle to pulmonary artery conduit (RV-PA; n = 49, 22%). The CPAs defect was closed directly (n = 69, 31%) or with a patch (n = 157, 69%). Complete resection of coarctation was performed in 126 patients (56%). RESULTS: Ninety-seven patients (43%) required surgical reoperation to CPAs during Stage II. Actuarial freedom from reoperation was 60+/-3%, 52+/-4% and 50+/-4% at 1, 5 and 10 years, respectively. On multivariable analysis, NP with RV-PA increased risk of reoperation (LR 8.3, 5.3-13.2; p < 0.001). Forty-one patients (18%) required catheter-based reintervention on CPAs. Actuarial freedom from reintervention was 98+/-1%, 72+/-4% and 58+/-6% at 1, 5 and 10 years, respectively. CPA problems were almost exclusively limited to the proximal Left pulmonary artery. On multivariable analysis, catheter-based reintervention became more common with time. Complete resection of coarctation increased risk of reintervention (LR 3.9, 1.6-9.6; p < 0.005). Arch reconstruction and CPAs repair techniques did not affect risk of reoperation or reintervention on CPAs. CONCLUSIONS: CPA stenoses and hypoplasia need surgical attention in approximately half of all patients undergoing the NP. The need for reoperation is increased when using the RV-PA conduit technique (although the majority of these are performed as part of the Stage II procedure). Catheter reinterventions are almost exclusively confined to the left CPA and are increased when the arch is shortened by resection of the coarctation tissue at time of NP.

The RV-PA conduit stimulates better growth of the pulmonary arteries in hypoplastic left heart syndrome.

OBJECTIVE: This retrospective study compared the size of the central pulmonary arteries in patients with hypoplastic left heart syndrome (HLHS) following either a classical Norwood or Norwood procedure with a right ventricle to pulmonary artery (RV-PA) conduit. METHODS: Between May 2001 and May 2003, 30 patients with HLHS underwent cardiac catheterization prior to stage II palliation. Patients were initially palliated with a classical Norwood (Classical group, n=18) or Norwood procedure with RV-PA conduit (RV-PA group; n=12). Indexed maximum and minimum diameters of the LPA and RPA were measured using the McGoon ratio. Cardiac catheterisation was performed at a median age of 4.0 months. There was no difference in the time interval to catheterisation (P=0.13), Qp:Qs (P=0.41) or median haemoglobin (P=0.42) between the groups. RESULTS: The combined PA diameter was larger in the RV-PA group (B) than the classical group (A) (1.99+/-0.38 versus 1.63+/-0.29, P<0.05). There were marked differences in the relative size of the pulmonary arteries between the two groups. In RV-PA patients, the LPA and RPA sizes were comparable (0.99+/-0.22 versus 1.00+/-0.31, P=1.00) whereas, in the classical group, the LPA was smaller than the RPA (0.75+/-0.15 versus 0.88+/-0.17, P<0.05). Both techniques were also associated with discrete PA stenoses at the site of shunt insertion. Stenoses were more severe in RV-PA group (RV-PA), causing a 42+/-16% reduction in the combined PA diameter compared with a 28+/-18% reduction in Classical group (classical) (P<0.05). CONCLUSIONS: The Norwood procedure with RV-PA conduit is associated with better and more evenly distributed central pulmonary artery growth. Nevertheless, it is also associated with central PA stenoses, which may require subsequent reconstruction.

The influence of pulmonary artery morphology on the results of operations for major aortopulmonary collateral arteries and complex congenital heart defects.

OBJECTIVE: Congenital heart defects with major aortopulmonary collateral arteries show marked variability in the size and distribution of native pulmonary arteries. We sought to classify the size and distribution of native pulmonary arteries and to determine their influence on surgical outcome. METHODS: Between 1989 and 2002, 164 patients underwent surgical intervention for congenital heart defects with major aortopulmonary collateral arteries (median age, 10 months). Three patterns of native pulmonary arteries were identified: intrapericardial native pulmonary arteries present (group I); confluent intrapulmonary native pulmonary arteries without intrapericardial native pulmonary arteries (group II); and nonconfluent intrapulmonary native pulmonary arteries (group III). Thirty-seven (23%) patients had single-stage and 76 (47%) patients had multistage complete repair. Thirty (18%) patients await septation, and 8 (5.0%) patients are not septatable. Follow-up is 98% complete (median follow-up, 5.8 years). RESULTS: In the 164 patients there were 15 (9.1%) early and 12 (7.3%) late deaths. Early mortality after complete repair was 4.4% (n = 5). Actuarial survival was 90% +/- 3% and 85% +/- 4% at 1 and 10 years, respectively. Actuarial freedom from surgical or catheter reintervention in septated patients was 77% +/- 4% and 45% +/- 8% at 1 and 10 years, respectively. On multivariate analysis, the morphology of the native pulmonary arteries was the only factor that influenced actuarial survival after complete repair (P =.04). Group III had the highest risk of death after septation (P =.008). Group II fared better than group III after the initial operation (P <.05). CONCLUSIONS: Current classifications of congenital heart defects with major aortopulmonary collateral arteries are based on the presence or absence of intrapericardial pulmonary arteries. We have identified a subgroup without intrapericardial native pulmonary arteries but with confluent intrapulmonary native pulmonary arteries. This group has a better outcome than those with nonconfluent intrapulmonary native pulmonary arteries.