Neonatal Shunt Research Articles

Transcatheter Ductal Stents Versus Surgical Systemic-Pulmonary Artery Shunts in Neonates With Congenital Heart Disease With Ductal-Dependent Pulmonary Blood Flow: Trends and Associated Outcomes From the Pediatric Health Information System Database.

Background Surgical systemic-to-pulmonary artery shunts have been the standard approach to establish stable pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow. More recently, transcatheter ductal stents have been performed as an alternative, less invasive intervention. We aimed to characterize trends in the utilization of surgical shunts versus ductal stents and compare associated outcomes. Methods and Results Using data from the Pediatric Health Information System, we retrospectively analyzed neonates with congenital heart disease with ductal-dependent pulmonary blood flow who underwent surgical shunt or ductal stent placement between January 2016 and December 2021. Patients were identified by International Classification of Diseases, Tenth Revision (ICD-10) diagnosis and procedure codes. The primary outcome was length of hospital stay. Secondary outcomes were reintervention risk and adjusted hospital costs. Of 936 patients included, 65.2% underwent a surgical shunt over the 6-year period. The proportion who underwent ductal stenting increased from 19% to 53.4% from 2016 to 2021. The median adjusted difference in postintervention length of hospital stay was 11 days greater for the surgical shunt cohort (95% CI, 7.2-14.8; P<0.001). The adjusted reintervention risks within 3 (odds ratio [OR], 3.37 [95% CI, 1.91-5.95], P<0.001) and 6 months (OR, 2.43 [95% CI, 1.62-3.64], P<0.001) were significantly greater in the ductal stent group. Median adjusted index hospital costs were $198 300 ($11 6400-$340 000) versus $120 400 ($81 800-$192 400) for the surgical shunt and ductal stent cohorts, respectively (P<0.001). Conclusions Ductal stenting has become an increasingly utilized palliative approach to secure pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow in the United States. Ductal stenting is associated with decreased length of hospital stay and reduced overall cost for the index hospitalization but with a greater reintervention risk than surgical shunting.

First Experience of Patent Ductus Arteriosus Stenting in Neonate with Pulmonary Atresia and Intact Ventricular Septum in North Sulawesi, Indonesia

Abstract: Stenting of patent ductus arteriosus (PDA) is a minimally invasive catheter-based temporary palliative procedure, and it serves as an alternative to surgical shunt in neonates with duct dependent pulmonary perfusion, such as pulmonary atresia. This presentation aimed to report the first experience of PDA stenting in a neonate with pulmonary atresia in North Sulawesi, Indonesia. We reported a female neonate patient, weighing 3775 grams, referred from primary hospital with suspect congenital heart disease. The patient was born at 40 weeks of gestational age by caesarean section delivery from a primigravid mother aged 23 years with inertia uteri and strangulation of nuchal cord. At birth, she cried immediately and her APGAR score was 6-7. On her first day of life, she looked cyanotic, therefore she was referred to Prof. Dr. R. D. Kandou Hospital. Physical examination showed that she had perioral cyanotic and peripheral cyanotic with SpO2 58%. Chest X-ray showed cardiomegaly. Echocardiography revealed pulmonary atresia with PDA with intact ventricular septum. PDA stenting was done on her second day of admission. After the procedure, she was clinically stable with SpO2 80%, and was treated with intravenous antibiotic, and intravenous heparin and aspirin enterally. On the third day post procedure, she was discharged and no complication was found. PDA stenting in patient with duct-dependent pulmonary circulation appeared to be an alternative to surgery. It provided bridging palliation until the time of definitive surgery. The effectiveness of the procedure was highlighted by the fact that all the patients showed significant improvement in arterial saturation and pulmonary vascular marking. In conclusion, PDA stenting is considered feasible, safe and associated with lower mortality rates and shorter hospital length of stay than systemic-pulmonary surgical shunt procedure. Early detection and timely management are imperative to save the life. Keywords: patent ductus arteriosus stenting; pulmonary atresia

Haemodynamic and clinical variables after surgical systemic to pulmonary artery shunt placement versus arterial ductal stenting.

Transcatheter stenting of the arterial duct is an alternative to surgical systemic to pulmonary artery shunt in neonates with parallel circulation. The current study compares haemodynamic and laboratory values in these patients for the first 48 hours after either intervention. Neonates with ductal dependent pulmonary blood flow who underwent surgical shunt placement or catheter-based arterial ductal stent placement between January 2013 and January 2022 were identified. Haemodynamic variables included heart rate, blood pressure, near infrared spectroscopy, central venous pressure, vasoactive inotropic score, and arterial saturation. Laboratory variables collected included blood urea nitrogen, serum creatinine, and serum lactate. Variables were collected at baseline, upon post-procedural admission, 6 hours after admission, 12 hours after admission, and 48 hours after admission. Secondary outcomes included post-procedural mechanical ventilation duration, post-procedural hospital length of stay, need for reintervention, need for extracorporeal membrane oxygenation, cardiac arrest, and inpatient mortality. Of the 52 patients included, 38 (73%) underwent shunt placement while 14 (27%) underwent a stent placement. Heart rates, renal oxygen extraction ratio, and cerebral oxygen extraction ratio were significantly lower in the stent group (p = <0.01, 0.01, and < 0.01, respectively).Haemoglobin and vasoactive inotropic scores were significantly lower in the stent group (p = <0.01, <0.01, respectively). The stent group had increased risk for cardiac arrest (p = 0.04). Patients who undergo arterial ductal stent placement have lower heart rates, haemoglobin, renal oxygen extraction ratio, cerebral oxygen extraction ratio, and vasoactive inotropic score in the first 48 hours post-procedure compared to patients with shunt placement.

Favourable Short- to Mid-Term Outcome after PDA-Stenting in Duct-Dependent Pulmonary Circulation.

Background. Stenting of patent ductus arteriosus (PDA) is a minimally invasive catheter-based temporary palliative procedure that is an alternative to a surgical shunt in neonates with duct-dependent pulmonary perfusion. Methods. An observational, single-centre, cross-sectional study of patients with duct-dependent pulmonary perfusion undergoing PDA-stenting as a stage I procedure and an analysis of short- to mid-term follow-up until a subsequent surgical procedure (stage II), with a focus on the interstage course. Results. Twenty-six patients were treated with PDA-stenting at a median (IQR) age of 7 (4–10) days; 10/26 patients (38.5%) (6/10 single pulmonary perfusion) were intended for later univentricular palliation, 16/26 patients (61.5%) (13/16 single pulmonary perfusion) for biventricular repair. PDA diameter was 2.7 (1.8–3.2) mm, stent diameter 3.5 (3.5–4.0) mm. Immediate procedural success was 88.5%. The procedure was aborted, switching to immediate surgery after stent embolisation, malposition or pulmonary coarctation in three patients (each n = 1). During mid-term follow-up, one patient needed an additional surgical shunt due to severe cyanosis, while five patients underwent successful catheter re-intervention 27 (17–30) days after PDA-stenting due to pulmonary hypo- (n = 4) or hyperperfusion (n = 1). Interstage mortality was 8.6% (2/23), both in-hospital and non-procedure-related. LPA grew significantly (p = 0.06) between PDA-stenting and last follow-up prior to subsequent surgical procedure (p = 0.06). RPA Z-scores remained similar (p = 0.22). The subsequent surgical procedure was performed at a median age of 106 (76.5–125) days. Conclusions. PDA-stenting is a feasible, safe treatment option, with the need for interdisciplinary decision-making beforehand and surgical backup afterwards. It allows adequate body and pulmonary vessel growth for subsequent surgical procedures. Factors determining the individual patient’s course should be identified in larger prospective studies.

Shunt occlusion in neonates with congenital heart disease undergoing systemic-to-pulmonary shunt surgery in the Neonatal Intensive Care Unit, Maharaj Nakorn Chiang Mai Hospital

Objectives To study occlusion of shunts and related factors in neonates after systemic-to-pulmonary shunt surgery. Methods This retrospective descriptive study reviewed the medical records of neonates in the Neonatal Intensive Care Unit who underwent a systemic-to-pulmonary shunt operation at Maharaj Nakorn Chiang Mai Hospital between January 1, 2011 and December 31, 2016. Patient characteristics and operative data were collected. Demographic data and incidence of shunt occlusion were analyzed using descriptive statistics. Factors associated with shunt occlusion were identified using Fisher’s exact test and the Mann-Whitney U test. Results Seventy-five newborns were enrolled in the study, of whom 39 (52.0%) were female. The average birth weight was 2,711.1 grams, and the average gestational age was 37.6 weeks. The number of newborns with TOF or TOF/IVS was equal to those with PA/IVS (34.7%). Sixty neonates (80%) underwent Modified Blalock Taussig shunt surgery, most (57.3%) with a 3.5 Fr. diameter shunt. The average weight at surgery was 2,898.9 grams, and the average age at surgery was 17.6 days. Anticoagulant and anti-platelet medication was used with almost all the neonates following surgery (96.0% and 93.3%, respectively). The incidence of shunt occlusion was 22.7% (17 neonates). Complete occlusion was found in 11 neonates. In-hospital shunt occlusion occurred in 10 neonates as well as in 7 neonates after discharge from the hospital. The overall mortality rate was 14.7%. Congenital heart disease diagnosis and gestational age were significantly associated with shunt occlusion (p = 0.02 and p = 0.01, respectively). Conclusion The study results can be used to provide develop guidelines for treatment of neonates with complex heart disease and low gestational age as well as nursing care guidelines for the prevention of blockage of the shunt in neonates undergoing a systemic-to-pulmonary shunt operation due to hospitalization in a hospital.

Duct stenting versus modified Blalock–Taussig shunt in neonates and infants with duct-dependent pulmonary blood flow: A systematic review and meta-analysis

ObjectiveThe aim of this systematic review and meta-analysis is to evaluate whether duct stenting is associated with better survival and other clinical outcomes compared with the modified Blalock–Taussig shunt in infants with duct-dependent pulmonary flow. MethodsA systematic search of the Medline, Embase, and Cochrane databases was performed by 4 independent reviewers from inception to March 2019. Meta-analysis was performed using the DerSimonian and Laird method with inverse-variance weighting. The quality of evidence was summarized using the Grading of Recommendations, Assessment, Development, and Evaluation framework. ResultsSix comparative observational studies were included, of which 3 were rated low risk of bias. There was no difference in 30-day mortality between the Blalock–Taussig shunt and duct stenting groups (risk ratio, 1.02; 95% confidence interval, 0.46-2.27; P = .96; I2 = 0%). However, there was benefit in favor of duct stenting for medium-term mortality (risk ratio, 0.63; 95% confidence interval, 0.40-0.99; P = .05; I2 = 0%). Duct stenting demonstrated a reduced risk for procedural complications compared with the Blalock–Taussig shunt (risk ratio, 0.50; 95% confidence interval, 0.31-0.81; P = .005; I2 = 0%). However, there was an increased risk for unplanned reintervention for duct stenting (risk ratio, 1.77; 95% confidence interval, 1.39-2.26; P < .00001; I2 = 10%). Duct stenting demonstrated shorter mean intensive care unit length of stay (mean difference, −4.69 days; 95% confidence interval, −7.30 to −2.07; P = .0004; I2 = 80%), as well as shorter hospital length of stay (mean difference, −5.78 days; 95% confidence interval, −9.27 to −2.28; P = .0009, I2 = 75%). The overall quality of evidence was rated low using the Grading of Recommendations, Assessment, Development, and Evaluation framework. ConclusionsDuct stenting demonstrated comparable early mortality, lower medium-term mortality, lower risk of procedural complications, and higher risk of reintervention compared with the Blalock–Taussig shunt.

Ministernotomy approach for modified Blalock-Taussig shunts in neonates: a feasibility study

BackgroundThe original approach to performing a modified Blalock-Taussig (MBT) shunt is via a left thoracotomy. However, the median sternotomy has become the preferred approach of many surgeons. We think that the upper ministernotomy approach provides several advantages and avoids the disadvantages of both the sternotomy and thoracotomy approaches. Here, we describe our experience in constructing neonatal MBT shunts via upper ministernotomy.MethodsA prospective study was conducted on 50 neonates who underwent modified Blalock-Taussig shunt performed through upper ministernotomy between March 2011 and December 2016. Preoperative characteristics, mortality, and morbidity were recorded.ResultsMean age was 16.9 ± 10.4 days, and weight was 3.5 ± 0.5 kg. All patients received grafts of size 3.5 mm. The mean oxygen saturation increased from 59.5 ± 7.3% preoperatively to 84.8 ± 4.2% postoperatively. There were three cases of mortality (6%). One patient suffered from an unstable sternum (2%). No patients required conversion to full sternotomy. Superficial wound infection occurred in three cases (6%), and there were no cases of mediastinitis. Mean duration of ventilation was 55.64 ± 37.5 h, mean ICU stay was 5.44 ± 3.9 days, and mean hospital stay was 14.7 ± 7.2 days.ConclusionUpper ministernotomy is a safe approach with good early results. It provides adequate exposure with limited surgical trauma. Emergency conversion to full sternotomy and initiation of cardiopulmonary bypass can be achieved easily. It avoids lung compression and respiratory compromise. Additional costs for specific instruments are not necessary.

Stenting the vertical neonatal ductus arteriosus via the percutaneous axillary approach.

Stenting the ductus arteriosus (DAS) has become an alternative to surgical systemic to pulmonary artery shunts in neonates with ductal-dependent pulmonary blood flow (PBF). Femoral approach for a vertical ductus can be difficult secondary to the acute angle and tortuous course, thus alternative access sites have been explored. Carotid access complications have been reported in 5%-10%. The extensive use of an axillary arterial approach in the United States has not been reported. The aim of this study is to describe our experience with DAS using the axillary approach. We reviewed all patients with DAS with an axillary approach in neonates with ductal-dependent PBF (May 2017-May 2018) in our institution. Procedural reports, angiograms, and clinical records of all consecutive patients were reviewed. Procedural technique, procedural outcomes, adverse events, and post-hospital courses are reported. Seven consecutive patients who received DAS utilizing axillary approach. All patients had ductal-dependent PBF through a vertical, tortuous ductus. Five had pulmonary atresia or near atresia, one had compromised PBF due to dynamic subvalvar obstruction, and one had Tetralogy of Fallot with isolated left pulmonary artery. Axillary access with 3.3 or 4 French sheath was obtained using ultrasound guidance. Bare metal coronary stents were deployed successfully in all. Intra-procedure, one developed in stent thrombus requiring re-stenting. There were no procedural mortalities or major adverse events from axillary access. There is a steep learning curve. Hemostasis was achieved with manual compression. Two patients had reintervention at 6-8weeks. All patients underwent successful planned surgeries. This series suggests DAS in neonates utilizing an axillary approach is a feasible and effective alternative for establishing PBF. Axillary arterial approach may be preferred as there is no risk to neurological sequelae and very low risk of limb complications. Larger series are needed to validate this approach.

Flow Preservation of Umbilical Vein for Autologous Shunt and Cardiovascular Reconstruction

Synthetic graft materials are commonly used for shunts and cardiovascular reconstruction in neonates, but are prone to thrombosis and scarring. The umbilical vein is a potential source of autologous, endothelialized tissue for neonatal shunts and tissue reconstruction, but requires preservation before implantation. Umbilical cords were collected in UW solution with antibiotics at 4°C until dissection. Umbilical vein segments were tested for burst pressure before and after 2 weeks of preservation. Umbilical veins segments were preserved under static or flow conditions at 4°C in UW solution with 5% human plasma lysate for 7 days. Veins were evaluated with histopathology, scanning electron microscopy, and platelet adhesion testing. Umbilical veins have no difference in burst pressure at harvest (n= 16) compared with 2 weeks of preservation (n= 11; 431 ± 229 versus 438 ± 244 mm Hg). After 1 week, static and flow-preserved veins showed viability of the vessel segments with endothelium staining positive for CD31, von Willebrand factor, and endothelial nitric oxide synthase. Scanning electron microscopy demonstrated preservation of normal endothelial morphology and flow alignment in the flow-preserved samples compared with cobblestone endothelial appearance and some endothelial cell loss in the static samples. Static samples had significantly more platelet adhesion than flow-preserved samples did. Umbilical veins have adequate burst strength to function at neonatal systemic pressures. Preservation under flow conditions demonstrated normal endothelial and overall vascular morphology with less platelet adhesion compared with static samples. Preserved autologous umbilical veins are potential source for endothelialized shunts or cardiovascular repair tissue for neonates.

Reasons for Failure of Systemic-to-Pulmonary Artery Shunts in Neonates.

Systemic-to-pulmonary artery shunt placement is an established palliative procedure for congenital heart disease. Although it is thought to be a simple operation, it is associated with significant morbidity and mortality. Data for all neonates who underwent surgery for a systemic-to-pulmonary artery shunt between 2000 and 2016 were reviewed. The study endpoints were shunt failure and shunt-related mortality. Shunt failure was defined as a shunt dysfunction because of thrombosis or stenosis requiring intervention or reoperation; shunt mortality was defined as death because of a shunt dysfunction. A total of 305 shunts (central shunt, n = 135; Blalock-Taussig shunt, n = 170) were implanted in 280 patients. The median patients' age at the time of surgery was 9 days (1-31 days). The median shunt size was 3.5 mm (3-4 mm). Twenty-four patients (8%) were diagnosed with a shunt failure, with a median time of 7 days (0-438 days). Freedom from shunt failure at 1 year was 91.6% ± 2%. A shunt-related mortality was ascertained for 12 patients (4%). Freedom from shunt-related mortality at 1 year was 96% ± 1%. Perioperative platelet transfusion (p = 0.01), central shunt (p = 0.02), 3-mm shunt size (p = 0.02), and postoperative extra corporeal membrane oxygenation (ECMO) (p < 0.01) were identified as risk factors for shunt failure. Platelet transfusion (p = 0.04) and postoperative ECMO (p < 0.01) were further identified as risk factors for shunt mortality. Based on these data, we recommend implanting a modified Blalock-Taussig shunt of at least 3.5 mm in neonates. Perioperative platelet transfusion and postoperative ECMO increase the risk of shunt failure.

Ventriculoperitoneal shunts in neonates: a retrospective study of outcomes with antibiotic-impregnated catheters and a modified peri-operative antibiotic protocol

Background: CSF infection is a significant complication of ventriculoperitoneal (VP) shunts and results in prolonged hospital stay, developmental delay and decreased quality of life. To decrease the high rates of neonatal VP shunt infections, an updated clinical guideline that included the use of antibiotic-impregnated shunts and a revised peri-operative antibiotic protocol was introduced in our neonatal unit. In this study, we evaluated the efficacy of these new guidelines in reducing the CSF shunt infection rates.Methods: A retrospective cohort study of neonates (≤28 days) who had VP shunt insertions in our unit.Results: 24 neonates in the first epoch received plain silastic shunt catheters (Feb 2002–April 2007), and 23 in the second epoch (August 2007–July 2015) received AIS catheters and a revised perioperative antibiotic protocol. Patient demographics were similar between both cohorts. Shunt related CSF infections were reduced in epoch 2 (2/23, 8.7%) compared to epoch 1 (5/24, 20.8%), but the results were not statistically significant (OR 0.36 (0.063–2.090); p = 0.256). Amongst neonates that needed VP shunt revision due to any cause, the median time interval between insertion to revision was significantly later in epoch 2 (epoch 1, 48 days (3–99); epoch 2, 148 days (20–396); p = 0.013).Conclusions: AIS catheters and a 48-hour perioperative antibiotic regimen may be beneficial in neonatal hydrocephalus. Adequately powered RCTs in the neonatal population are needed to confirm these findings.

Reasons for Failure of Systemic-to-Pulmonary Artery Shunts in Neonates

Reasons for Failure of Systemic-to-Pulmonary Artery Shunts in Neonates

Mechanically assisted bidirectional cavopulmonary shunt in neonates and infants: An acute human pilot study

ObjectivePoor survival following surgical palliation for hypoplastic left heart syndrome (HLHS) raises the question of the need for a paradigm shift. This is the first human study to investigate the possibility of primary “in-series” palliation in neonates and infants with HLHS in an acute setting with the aid of 2 types of mechanical assist: superior vena cava (SVC)–to–pulmonary artery (PA) pump assist and SVC–to–right atrium (RA) oxygenation assist. MethodsBy rearranging the cannula sites and flow rates for modified ultrafiltration, 2 types of mechanically assisted bidirectional cavopulmonary shunt (BCPS) circulation were simulated for 20 minutes. Three neonates undergoing a stage I Norwood procedure were assigned to SVC-PA pump assist, and 3 infants undergoing stage II BCPS were assigned to SVC-RA oxygenation assist. Hemodynamic parameters, blood gas values, and arterial (SaO2) and regional cerebral tissue (rCTO2) saturations were analyzed. ResultsAll 6 patients completed the study without hemodynamic compromise. In the SVC-PA pump assist group, a mean arterial pressure >40 mm Hg was maintained. SVC pressure was lower (P = .01) and cerebral perfusion pressure (CPP) was higher (P = .03) during the last 10 minutes of assist compared with Norwood physiology. SaO2 >80%, rCTO2 >60%, and mixed venous saturation ≥59% were maintained, comparable to values with Norwood physiology. In the SVC-RA oxygenation assist group, with full or 50% support, mean blood pressure >50 mm Hg, SVC pressure <15 mm Hg, mixed venous saturation >50%, and CPP >40 mm Hg were maintained, which were comparable to BCPS physiology. ConclusionsTwo types of mechanical assist to support primary in-series palliation are feasible in the acute setting. Both modes of mechanical assist maintained oxygenation, as well as systemic and cerebral perfusion.

Factors affecting death and progression towards next stage following modified Blalock-Taussig shunt in neonates.

The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair. Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies. Competing risk analysis modelled events after BTS (death or transplantation, transition to Glenn shunt or biventricular repair) and examined risk factors affecting outcomes. SV patients had a higher incidence of extracorporeal membrane oxygenation (ECMO) support requirement (12 vs 4%, P = 0.004) and unplanned cardiac reoperation (14 vs 7%, P = 0.051) than their BV counterparts. Additionally, hospital mortality was higher in SV than in BV patients (15 vs 3%, P < 0.001). In SV patients, competing risk analysis showed that, 2 years following BTS, 27% of patients had died or received transplantation and 73% had undergone the Glenn shunt. On multivariable analysis, factors associated with time until death or transplantation prior to Glenn were cardiopulmonary bypass [hazard ratio (HR) 3.6 (2.0-6.4), P < 0.001], unplanned cardiac reoperation [HR 2.4 (1.3-4.6), P = 0.007], pulmonary atresia [HR 2.0 (1.1-3.7), P = 0.026] and the shunt size/weight ratio [HR 1.3 (1.1-1.4) per 0.1 increase, P = 0.001]. In BV patients, competing risk analysis showed that, 2 years following BTS, 13% of patients had died or received transplantation, 85% had undergone biventricular repair and 2% were alive without biventricular repair. On multivariable analysis, factors associated with time until death or transplantation prior to biventricular repair were genetic syndromes and extracardiac malformations [HR 6.1 (2.0-18.2), P = 0.001], weight ≤2.5 kg [HR 5.6 (2.0-16.0), P = 0.001] and male gender [HR 3.4 (1.1-11.0), P = 0.041]. Palliation with BTS continues to be associated with significant operative morbidity and mortality. In addition to hospital death, there is an important interstage attrition risk prior to subsequent palliation or biventricular repair. Inherent patient characteristics (i.e. genetic syndromes and low weight) and anatomical details (i.e. SV, pulmonary atresia and concomitant cardiac anomalies) are associated with worse survival.

Results of Palliation With an Initial Modified Blalock-Taussig Shunt in Neonates With Single Ventricle Anomalies Associated With Restrictive Pulmonary Blood Flow

We report our current-era results after placement of a modified Blalock-Taussig shunt (mBTS) as the first surgery in the multistage palliation strategy of neonates born with single ventricle (SV) anomalies associated with restrictive pulmonary blood flow. Between 2002 and 2012, 174 neonates with SV underwent mBTS. Competing risks analysis modeled events after mBTS (death or transplantation, transition to Glenn) and subsequently after Glenn (death or transplantation, transition to Fontan) and examined risk factors affecting outcomes. Competing risks analysis showed that 2 years after mBTS 27% of patients had died or received transplantation and 73% had undergone Glenn. Five years after Glenn, 6% had died, 73% had undergone Fontan, and 21% were alive awaiting Fontan. Overall survival 8 years after mBTS was 68%. On multivariable analysis, risk factors for mortality were increased shunt size to weight ratio (hazard ratio [HR]:1.2 per 0.1 ratio increase [1.0 to 1.4], p = 0.015), postoperative extracorporeal membrane oxygenation (HR: 4.0 [2.2 to 7.4], p < 0.001), chromosomal and extracardiac malformations (HR: 2.5 [1.3 to 5.0], p = 0.008), cardiopulmonary bypass use (HR: 2.5 [1.3 to 4.5], p = 0.004), and underlying pulmonary atresia with intact ventricular septum (PAIVS) or atrial isomerism (HR: 2.1 [1.1 to 4.2], p = 0.035). Palliation outcomes with mBTS in SV patients trail behind the generally improved results of congenital heart surgery. Several anatomic and patient-related factors continue to affect survival. Mortality is high in low weight neonates in whom a balance between overcirculation due to large shunt and low pulmonary blood flow and risk of occlusion due to small shunt is difficult to attain. Patients with chromosomal and extracardiac malformations, atrial isomerism, and PAIVS continue to be the most challenging and are associated with higher hospital and interstage mortality.

Abstract 19213: Establishment of Mechanically-Assisted Primary Bidirectional Cavopulmonary Shunt in Neonates and Infants: An Acute Human Pilot Study

Background: One year survival for stage 1 palliation in hypoplastic left heart syndrome (HLHS) is 69%. We hypothesized that primary ‘in-series’ palliation (neonatal bidirectional cavopulmonary shunt - BCPS) with mechanical assist would provide more stable physiology. This is the first human study of an acute intraoperative BCPS model. Methods: Two types of intraoperative, tubing configured, pump-assisted BCPS circulations [superior vena cava (SVC) to pulmonary artery (PA) pump assist at stage I Norwood, and SVC to right atrium (RA) pump with oxygenation at stage II BCPS] were established in the operating room for 20 minutes at the time of modified ultrafiltration (Figure 1A). Hemodynamics and cerebral blood flow were continuously recorded. Arterial and venous blood gases were analyzed every 5-10 minutes. Results: All six patients (3 in each group) completed 20 minutes of mechanical support without hemodynamic compromise. In the SVC-PA pump assist (n=3), the pump generated effective pulmonary blood flow of 335+/-53ml/min while the BT shunt was occluded. Compared to Norwood physiology, the SVC-PA pump-assisted BCPS had a higher mean arterial pressure (median 33 vs. 44 mmHg, p=0.02), lower central venous pressure (CVP) (12 vs. 10 mmHg, p=0.03), and higher cerebral perfusion pressure (33 vs. 46 mmHg, p=0.01) (Figure 1B). In the SVC-RA oxygenation assist (pump and oxygenator assisted BCPS), the pump generated blood flow of 536+/176ml/min with oxygenation. Compared to baseline BCPS physiology, SVC-RA oxygenation-assisted BCPS had a lower CVP (14 vs. 7 mmHg, p=0.05). Conclusion: The study demonstrates the physiologic feasibility of mechanically assisted neonatal in-series single ventricle circulation. Both assist systems attained equivalent or better hemodynamics, stable ventilation and oxygenation, and a more favorable cerebral perfusion profile. This human proof of concept study raises the possibility of a potential paradigm shift for the treatment of HLHS.

Invited Commentary

Patients with ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries (MAPCAs) are not uncommon. There is still controversy regarding surgical treatment for these patients. The authors' experience in this article [1] was disappointing regarding patency rate, lack of growth, and the contribution to the pulmonary circulation of translocated collateral vessels. They planned to rehabilitate hypoplastic native pulmonary arteries by neonatal shunting without MAPCA translocation in 25 consecutive patients.

Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collaterals: Neonatal Pulmonary Artery Rehabilitation Without Unifocalization

This study analyzed a protocol of neonatal rehabilitation of hypoplastic pulmonary arteries in the management of pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs). Ideal management of patients with pulmonary atresia, VSD, and MAPCAs is the subject of controversy. From June 2003 to December 2008, 25 consecutive patients were diagnosed with pulmonary atresia, VSD, and MAPCAs, and 20 were entered into a neonatal shunting regimen. The median age at the first operation was 3.6 weeks (range, 0.7 to 17 weeks). All patients underwent an initial central or modified Blalock-Taussig shunt, or both. Further preparatory procedures included 26 pulmonary artery patch reconstructions, 19 right ventricle-to-pulmonary artery conduits, 4 MAPCA ligations, and 4 further shunts. No patient underwent translocation of the collateral arteries. At the latest follow-up, 12 of 20 patients have had a complete repair at a median age of 18 months (range, 11 to 48 months), 6 are awaiting repair, and 2 are considered unlikely to be repaired. No patient was missing follow-up. Median pulmonary artery indices had grown from 14.51 to 118.7 in 17 patients. Postoperative angiograms were performed at a median of 8 months (range, 1.9 to 32.7 months) in 9 of 12 completely repaired patients. The median right/left ventricular pressure ratio was 0.64 (range, 0.54 to 0.91). Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival.

PP-283. Two cases of congenital portosystemic venous shunts in neonates

PP-283. Two cases of congenital portosystemic venous shunts in neonates

Endovascular management of arteriovenous malformations and other intracranial arteriovenous shunts in neonates, infants, and children

We discuss the management of cerebral arteriovenous shunts in neonates, infants, and children, with emphasis on our experience with pediatric cerebral arteriovenous malformations (AVMs). The management of vein of Galen malformations is discussed in a separate chapter. An all-inclusive retrospective chart review of the endovascular surgery operative record database at the Hyman Newman Institute for Neurology and Neurosurgery at Roosevelt Hospital in NYC was conducted. All consecutive pediatric patients (newborn to 18 years of age) with intracranial arteriovenous shunts who presented from January 1, 2004 to June 16, 2009 were included. A total of 151 consecutive pediatric patients with intracranial arteriovenous shunts were evaluated from the period of January 1, 2004 to June 16, 2009. This included 56 patients with vein of Galen malformations, 48 cerebral AVMs, 11 patients with pial arteriovenous fistulae, six patients with dural arteriovenous malformations, and 30 patients with mixed intracranial vascular malformations. Forty-four patients underwent a total of 163 endovascular embolizations. The complications rate for endovascular embolizations was 6.7% (11 in 163), 5.5% with temporary complications and 1.2% with permanent complications. The mortality rate for the group of patients (excluding patients with vein of Galen malformations) that underwent endovascular embolizations was 0.0%. Careful clinical observation and timely intervention are important in the management of pediatric patients with intracranial arteriovenous shunts. Trans-arterial endovascular embolization with liquid embolic agents is the treatment of choice for safe stabilization and/or improvement of symptoms in the group of pediatric patients with intracranial arteriovenous malformations.