Hybrid stage 1 Norwood combined with reverse Blalock-Taussig shunt and sutureless repair of total anomalous pulmonary venous connections.

The patient is a 35-week-old, 2.6-kg neonate who was diagnosed prenatally with hypoplastic left heart syndrome and a supracardiac variant of total anomalous pulmonary venous connection. We present the technique of stage 1 hybrid palliation combined with repair of total anomalous pulmonary venous connection. Due to the risk of retrograde aortic arch obstruction from the ductal stents, a reverse Blalock-Taussig shunt was performed as well.

Precision in Norwood Shunt Sizing: Single Ventricle Reconstruction Trial Public Dataset Analysis.

BACKGROUND: Morbidity and mortality after the Norwood procedure remains high. Shunt size selection is not standardized and the impact of shunt size on outcomes is poorly understood. The Single Ventricle Reconstruction trial randomized infants to modified Blalock-Taussig-Thomas shunt (MBTTS) or right ventricle-to-pulmonary artery shunt at the Norwood procedure. We assessed shunt size distribution and its association with postoperative outcomes. METHODS: We included 544 patients, excluding 5 with ambiguous shunt crossover data. Normalized shunt diameter 1 and 2 were calculated as shunt diameter divided by patient's weight and body surface area, respectively. The primary outcome was 30-day mortality after Norwood. Secondary outcomes were intensive care and total length of stay, and survival to Glenn procedure. Logistic and ordinal regression models evaluated the association of normalized shunt diameter with outcomes. RESULTS: Thirty-day mortality after Norwood was 11.4% (n = 62), survival to Glenn procedure was 72.6% (n = 395), median length of stay was 14.0 (interquartile range, 9.0-27.7) days and 24.0 (interquartile range, 16.0-41.0) days in the intensive care and total, respectively. Normalized shunt diameters exhibited variation in both shunt types but were not associated with 30-day mortality. Right ventricle-to-pulmonary artery shunt size was not associated with secondary outcomes. However, a MBTTS diameter ≥1.5 mm/kg predicted longer Norwood (odds ratio, 4.89; 95% CI, 1.41-16.90) and intensive care (odds ratio, 4.11; 95% CI, 1.25-13.49]) duration. CONCLUSIONS: Shunt size selection was variable. Right ventricle-to-pulmonary artery shunt had a wider size range seen with favorable outcomes compared with MBTTS. A MBTTS either too large or too small is associated with worse postoperative outcomes. Refining shunt sizing practices can improve surgical outcomes after the Norwood procedure.

Comparison between the modified Blalock-Taussig shunt and right ventricular outflow tract stent in the palliative treatment for tetralogy of Fallot. / 改良Blalock-Taussigåˆ†æµæœ¯ä¸Žå³å¿ƒå®¤æµå‡ºé“æ”¯æž¶æ¤å ¥æœ¯å§‘æ¯æ€§æ²»ç–—æ³•æ´›å››è”ç—‡çš„æ¯”è¾ƒ.

OBJECTIVES: For patients with tetralogy of Fallot (TOF) who are not suitable candidates for primary corrective surgery or have a high surgical risk, transcatheter right ventricular outflow tract (RVOT) stent implantation is considered a safe and effective palliative intervention. This study aims to investigate the therapeutic outcomes of RVOT stent implantation in neonates and infants with TOF in comparison with the modified Blalock-Taussig shunt (mBTS) and to compare the impact of the 2 palliative interventions on arterial oxygen saturation and pulmonary artery development in pediatric patients. METHODS: Clinical data of 32 patients with TOF admitted to the Second Xiangya Hospital of Central South University from March 2011 to March 2021 were retrospectively collected. The patients were divided into an mBTS group (undergoing mBTS, n=15) and a stent implantation group (undergoing RVOT stenting, n=17) according to the surgical procedures. The 2 groups were assessed and compared in the surgical-related arterial oxygen saturation, postoperative complication rate, mortality rate, and re-intervention rate. The development of the patients' main pulmonary artery, right pulmonary artery, and left pulmonary artery was assessed by z-scores according to echocardiographic results. RESULTS: The children in the stent implantation group were younger and less weighed compared with the mBTS group (both P<0.05). Compared with the preoperative period, children in the stent implantation group had significantly higher arterial oxygen saturation [(75±17)% vs (96±3)%, P=0.026]; z-scores of pulmonary trunk [(-2.82±1.27) points vs (0.86±0.77) points, P=0.014], right pulmonary artery [(-1.88±0.59) points vs (-0.28±0.71) points, P=0.011], and left pulmonary artery [(-2.34±0.36) points vs (-1.67±0.36) points, P=0.036] were significantly increased. However, there were no significant differences in arterial oxygen saturation and pulmonary artery z-scores between pre- and post-mBTS procedures (all P>0.05). CONCLUSIONS: RVOT stent would have good surgical outcomes used in TOF patients with low weight and severe comorbidities. It also leads to an higher postoperative oxygen saturation and better promotion of pulmonary artery growth with RVOT stent compared to mBTS.

Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome: 2021 vs 2011.

We sought to examine current practices and changes in practice regarding initial counseling for families of patients with hypoplastic left heart syndrome (HLHS) given the evolution of options and outcomes over time. Counseling (Norwood with Blalock-Taussig-Thomas shunt (NW-BTT), NW with right ventricle to pulmonary artery conduit (NW-RVPA), hybrid palliation, heart transplantation, or non-intervention/hospice (NI)) for patients with HLHS were queried via questionnaire of pediatric care professionals in 2021 and compared to identical questionnaire from 2011. Of 322 respondents in 2021 (39% female), 299 respondents were cardiologists (92.9%), 17cardiothoracic surgeons (5.3%), and 6 were nurse practitioners (1.9%). Respondents were largely from North America (96.9%). In 2021, NW-RVPA procedure was the preferred palliation for standard risk HLHS patient (61%) and was preferred across all US regions (p < 0.001). NI was offered as an option by 71.4% of respondents for standard risk patients and was the predominant strategy for patients with end-organ dysfunction, chromosomal abnormality, and prematurity (52%, 44%, and 45%, respectively). The hybrid procedure was preferred for low birth-weight infants (51%). In comparison to the identical 2011 questionnaire (n = 200), the NW-RVPA was endorsed more in 2021 (61% vs 52%, p = 0.04). For low birth-weight infants, hybrid procedure was more recommended than in 2011 (51% vs 21%, p < 0.001). The NW-RVPA operation is the most recommended strategy throughout the US for infants with HLHS. The hybrid procedure for low birth-weight infants is increasingly recommended. NI continues to be offered even in standard risk patients with HLHS.

A Single-Centre Retrospective Review of Modified Blalock-Taussig Shunts: A 22-Year Experience.

INTRODUCTION: This single-centre retrospective study explores demographics and outcomes of patients who underwent a modified Blalock-Taussig shunt (MBTS) over a 22-year period. The predominant surgical approach in this study is a lateral thoracotomy, in contrast to a midline sternotomy. Risks and outcomes of this approach are compared with national and international literature. MATERIALS AND METHODS: Demographic, anatomical, clinical, surgical and outcome data of all patients who underwent a MBTS between 2000 and 2022 were collected and analysed, excluding Norwood procedures, which are not performed at this institution. Short- and long-term morbidity and mortality is described. RESULTS: Over the 22-year study period, 185 MBTS were performed in 162 patients, at a median age of 16 days (interquartile range [IQR] 5-59 days) and weight of 3.47 kg (IQR 3-4.25 kg, minimum weight 2 kg). Of these, 79% of patients had a biventricular circulation. Cardiac diagnoses included both univentricular and biventricular anatomy; tetralogy of Fallot (TOF) (36%), transposition of the great arteries/ventricular septal defect/pulmonary stenosis (TGA/VSD/PS) (11%), pulmonary atresia with intact ventricular septum (PA/IVS) (23%), pulmonary atresia with ventricular septal defect (PA/VSD) (14%), other (16%). The most common size of MBTS was 4 mm (71%); 93% were performed via a lateral thoracotomy. There were 47 cases of major operative morbidity, which did not differ significantly with cardiac diagnosis. Overall all-cause mortality was 13.5%. Early operative mortality was 4.3%. Mortality varied with cardiac diagnosis, 6% with TOF and 19% with PA/IVS. There was no era effect on mortality rates, however a lower frequency of major morbidity (23% vs 7%, p=0.03) was observed in the most recent third of the study period. Risk factors for shunt reintervention or mortality included weight <2.5 kg (HR=2.79 [1.37, 5.65], p=0.005), and pre- (HR=3.31 [1.86, 5.9], p<0.001) or postoperative lactic acidosis (HR=1.37 [1.25,1.5], p<0.001). These rates are comparable to those in the literature, with the predominant approach a midline sternotomy. CONCLUSION: Mortality rates and risk factors for adverse outcomes are comparable to those previously reported for both univentricular and biventricular groups. These results highlight that outcomes of MBTS performed via lateral thoracotomy are comparable to those by midline sternotomy as reported in the literature. Operating via the lateral approach may be advantageous as it avoids the complications of a midline sternotomy.

Increasing Use of the Right Ventricle-to-Pulmonary Artery Shunt for Stage 1 Palliation.

BACKGROUND: Stage 1 palliation of hypoplastic left heart syndrome entails use of the Norwood operation with a modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt, or the hybrid procedure. Use trends and factors influencing palliation selection remain unclear. We aimed to evaluate these questions and to compare outcomes between types of stage 1 palliation. METHODS: The National Pediatric Cardiology Quality Improvement Collaborative phase 1 (June 2008-August 2016) and phase 2 (August 2016-September 2019) databases were used. Procedure type was assessed by operation year. Baseline characteristics and annual hospital volume were evaluated. Postsurgical admission duration and outcomes were compared. RESULTS: A total of 3497 patients were included, 30.8% with modified Blalock-Taussig shunt, 59.7% with right ventricle-to-pulmonary artery shunt, and 9.5% with hybrid. Use of the right ventricle-to-pulmonary artery shunt increased over time (P = .02). This increase was similar among all hospital volumes. Higher hospital volume (odds ratio [OR], 1.2; 95% CI, 1.1-1.4; P = .003), male sex (OR, 1.3; 95% CI, 1.1-1.6; P = .01), and isolated cardiac disease (OR, 1.33; 95% CI, 1.01-1.55; P = .05) were associated with relatively higher likelihoods of a modified Blalock-Taussig shunt. Mortality/transplant rates before stage 2 palliation were higher with the modified Blalock-Taussig shunt than with the right ventricle-to-pulmonary artery shunt (12.3% vs 9.6%, P = .03). CONCLUSIONS: In stage 1 palliation, use of right ventricle-to-pulmonary artery shunts has increased over time, use of modified Blalock-Taussig shunts has decreased, and use of hybrids was unchanged. The modified Blalock-Taussig shunt has a higher likelihood of use in higher-volume centers, males, and less complex patients but is associated with longer hospitalizations and lower transplant-free survival to stage 2 palliation.

Blalock-Taussig Shunt versus Ductal Stenting as Palliation for Duct-Dependent Pulmonary Circulation.

Objectives: There is limited data published from outside North America and Europe comparing the outcomes of a modified Blalock-Taussig shunt (MBTS) and ductal stenting as the first palliative procedure for infants with duct-dependent pulmonary circulation. This study reports the National Heart Center's, in Muscat, Oman, experience in comparing the outcomes of these 2 interventions. Methods: This retrospective study included all infants with duct-dependent pulmonary circulation who received either a MBTS or ductal stenting from 2016-2019. The primary outcomes were death or re-interventions. Secondary outcomes included death, subsequent re-interventions, survival to subsequent surgical intervention, survival to hospital discharge, post-procedural mechanical ventilation and duration of intensive care unit stay. Results: A total of 71 patients were included in the study, 33 (46%) of whom received ductal stenting. The prevalence of the primary outcome (death or re-intervention) in the patent ductus arteriosus (PDA) stent group was 54.5% versus 31.6% in the MBTS group but this was not statistically significant (P = 0.06). There was no difference between the 2 groups in terms of time to next surgical intervention (P = 0.233). The PDA stent group had shorter post-procedural, mechanical ventilation and intensive care unit stay durations (P <0.05). Syndromic patients were at higher risk of mortality compared to non-syndromic patients. Conclusion: MBTS and ductal stenting are both acceptable modalities as a palliative intervention for infants with duct-dependant pulmonary circulation. Syndromic patients are at higher risk of mortality. This can be considered an important factor for patient selection.

Technical Aspects and Common Pitfalls of Norwood With Modified Blalock Taussig Shunt.

The Stage 1 Norwood procedure is the first of 3 stages in the surgical palliation of hypoplastic left heart syndrome and certain other single ventricle lesions with systemic outflow obstruction. In this article, we address some technical aspects and common pitfalls of the Norwood procedure with systemic to pulmonary shunt for HLHS palliation. We report our results with the Norwood with Blalock Taussig shunt in a cohort of 44 patients over a 7-year period in 2 institutions in Argentina. The results of the Norwood procedure have improved significantly through the understanding and refinement of the surgical techniques. Procedures must be technically perfect since residual lesions are poorly tolerated. Norwood with a modified Blalock Taussig shunt can be performed with low mortality and may provide excellent long-term outcomes.

Outcomes of duct stenting and modified Blalock-Taussig shunt in cyanotic congenital heart disease with duct-dependent pulmonary circulation.

BACKGROUND: Transcatheter ductus arteriosus stenting (DS) is emerging as an alternative method to modified Blalock-Taussig shunt (MBTS) in providing pulmonary blood flow in cyanotic congenital heart disease (CCHD) with duct-dependent pulmonary circulation. OBJECTIVE: To evaluate post-procedural outcomes and survival between patients undergoing DS and MBTS. METHODS: All infants ≤ 60 days of age having CCHD with diminished pulmonary blood flow who underwent palliative procedure either with MBTS or DS at King Chulalongkorn Memorial Hospital during January 1st, 2013 and December 31th, 2017 were retrospectively reviewed. RESULTS: 98 patients were included; 34 patients underwent a transcatheter DS and 64 patients underwent MBTS. There was no significant difference in post-procedural outcomes and overall mortality rate between two groups (17.6% in MBTS group and 6.1% in DS group, p = 0.09). Single ventricle morphology was the major risk factor associated with increased mortality compared with biventricular morphology (aHR 3.9, 95% CI 1.49-10.2, p = 0.01). There was similar number of early and pre-repair additional interventions focusing on MBTS/DS patency between two groups. The MBTS group had a greater number of early interventions on PA branch stenosis related to baseline diagnosis. Risk factors associated with additional intervention were pre-existing pulmonary branch stenosis (aHR 2.54, 95% CI 1.3-4.97, p = 0.006) and body weight less than 2.5 kg (aHR 3.33, 95% CI 1.57-7.08, p = 0.003). Having pulmonic valve perforation or balloon pulmonary valvuloplasty to promote antegrade pulmonary blood flow could result in a lower number of additional interventions required before definitive repair. CONCLUSION: Duct stenting is a feasible and safe alternative to MBTS in cyanotic infants with duct-dependent pulmonary circulation. However, mortality rate was significantly higher in patients with single ventricle that required careful follow-up after procedure.

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

OBJECTIVE: The 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. METHODS: A 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. RESULTS: Six 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. CONCLUSIONS: Duct 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.

Alternate Approach to Hypoplastic Left Heart Syndrome Stage 1 Surgery.

Hypoplastic left heart syndrome is almost always fatal in neonates unless it is palliated surgically. The conventional Norwood procedure with a Blalock-Taussig shunt or a Sano modification and a hybrid approach with branch pulmonary artery banding and ductal stenting are common surgical options. An alternate technique with a different strategy to restore pulmonary blood flow is described in this report.

A novel unidirectional-valved shunt approach for end-stage pulmonary arterial hypertension: Early experience in adolescents and adults.

OBJECTIVES: Despite advances in treatment of idiopathic pulmonary arterial hypertension (IPAH), there remains no medical cure, and patients can experience disease progression leading to right heart failure, progressive exercise intolerance, and death. The reversed Potts shunt (left pulmonary artery to descending aorta) was reintroduced for treatment of end-stage IPAH to permit decompression of the suprasystemic right ventricle by right to left shunting, with preservation of upper body oxygenation. The shunt has the potential to delay the need for lung transplantation and offer a treatment for those who are transplant ineligible. To optimize shunt design and avoid the potential complications of bidirectional shunting, we developed a novel approach using a unidirectional-valved shunt (UVS) in patients with IPAH with suprasystemic pulmonary arterial pressure and poor right ventricular function. METHODS: A single-center retrospective review was performed of UVS cases done at Columbia University Medical Center-New York Presbyterian between November 1, 2016, and May 1, 2019. RESULTS: Five patients (4 female; ages 12-22 years) underwent UVS. All had suprasystemic pulmonary arterial pressure, poor right ventricular function, and World Health Organization functional class IV symptoms at baseline. All patients are alive and transplant-free at latest follow-up (range 3-33 months; median 6 ± 11 months). CONCLUSIONS: The UVS may offer an alternative solution to lung transplantation in adolescents and young adults with IPAH. Longer-term follow-up is needed to determine the ultimate impact of unidirectional unloading of the right ventricle in these patients and to determine whether the UVS will enable a broader approach to the treatment of patients with IPAH.

Clinical Outcomes after Biologic Graft Use for the Creation of Blalock-Taussig Shunt in Critically Ill Patients with Thrombophilia.

BACKGROUND: The modified systemic to pulmonary artery shunt (mSPS) is an effective palliative procedure in children with cyanotic congenital heart disease (CCHD) who are not suited for total correction. Early graft failure related to hereditary thrombophilic disorder is one cause of mortality. The aim of this study is to compare the clinical outcomes and rate of graft failure after mSPS in cyanotic infants with hereditary thrombophilia using bovine mesenteric venous graft (BMVG) and polytetrafluoroethylene (PTFE). METHODS: 60 cyanotic patients (28 neonates, mean age 19 ± 11.3 days; range 1 to 27) who had thrombophilic risk factors were divided into 2 groups: BMVG (n = 30) and PTFE (n = 30). Preoperative thrombophilic factors were measured for each patient. The most common thrombophilic factors were protein C and S deficiency and Factor V Leiden mutation. We also investigated D-dimer, positivity of prothrombin G20210A, factor XII and antithrombin III deficiency, and homocysteinemia in both groups. The mean age of patients was 4.6 ± 1.09 months (range 1 day to 6 months) in the BMVG group and 3.9 ± 1.02 months (range 2 days to 9 months) in the PTFE group (P = .67). mSPS procedures were performed via left thoracotomy (n = 19 in the BMVG group and n = 22 in the PTFE group) or right anterior thoracotomy (n = 3 in the BMVG group and n = 3 in the PTFE group). Median sternotomy was performed to create a central shunt in 8 neonates in the BMVG group. In the PTFE group, we performed a central shunt in 5 patients via median sternotomy. Low molecular weight heparin in combination with acetylsalicylic acid (aspirin) were administered after surgery in both groups. The patients received aspirin combined with warfarin (Coumadin) after being discharged from hospital. We performed revision surgery to observe whether any patient had a significant drop in saturation with inaudible mSPS murmur. RESULTS: 7 patients died early after surgery (n = 2 in the BMVG group [6.6%] and n = 5 in the PTFE group [16.5%]; P = .022). 53 patients were discharged home in good clinical condition. Early graft thromboses were observed in 2 patients in the BMVG group (6.6%) and 8 patients in the PTFE group (26.6%) (P = .001). In a case from the BMVG group, the reason for graft thrombosis was entanglement of the graft. Revision surgery was performed successfully without any complication. Cil et collegues has been reported a successful percutaneous balloon angioplasty after an acute thrombosis of BMVG previously [Cil 2010]. In another patient who had acute BMVG thrombosis, we performed transluminal graft angioplasty using successful thrombolytic administration in the catheterization laboratory. There were no complications due to graft materials such as hematoma, seroma, or infection in the BMVG group. Bleeding from the needle hole was seen in 1 patient in the BMVG group. PTFE thrombosis developed in 3 patients within 24 hours (10%). We detected total or partial PTFE graft thrombosis in 5 patients during the follow-up period (20%). Revision surgeries in 3 patients were performed immediately after diagnosis. Transluminal balloon angioplasty combined with thrombolysis was performed in infants with partial or total PTFE occlusion in 5 patients. In the PTFE group, perigraft seroma (n = 5 [16.6%)] and hematoma (n = 2 [6.6%]) were detected. We performed revision surgery because of bleeding from the needle hole in 3 patients in the PTFE group (10%) in the early period after surgery. We detected a graft infection in 1 patient (3.3%) 6 months after surgery in the PTFE group. The rate of overall complications including pseudoaneurysm, seroma formation, graft infection, or partial or total graft occlusion in the early and follow-up periods was 6.6% in the BMVG group and 53.3% in the PTFE group (P = .0001). The rate of freedom from shunt failure was 92.6% ± 2.1% and 76.2% ± 4.8% during follow-up in BMVG and PTFE groups, respectively (P = .034). The rate of shunt-related mortality was 10.7% (n = 3) in the BMVG group and 20% in the PTFE group (P = .01). Regular physical examinations, transcutaneous oxygen saturation, and echocardiographic study were performed for shunt control during follow-up. Shunt occlusion or thrombosis was not seen in the BMVG group; 5 patients in the PTFE group (20%) had shunt occlusion during follow-up (P = .001). CONCLUSION: Our study shows that BMVG, as a biological material, may be used as an alternative material for creating mSPS. It decreases postoperative life-threatening complications after shunt procedures, including graft thrombosis, bleeding from the needle hole, perigraft hematoma, and seroma in patients with hereditary thrombophilia. To our knowledge, we report the first clinical comparison of the 2 grafts in our case series with thrombophilic risk factors.

Modified Blalock-Taussig Shunt: A Single-Center Experience and Follow-Up.

BACKGROUND: The modified Blalock-Taussig shunt (MBTS) is used to palliate patients with restrictive pulmonary blood flow in complex cardiac anomalies. We describe the immediate and follow-up results of patients with MBTS in our center. METHODS: Patients who received MBTS (excluding those with hypoplastic left heart syndrome) from May 2008 to December 2018 were retrospectively identified. Hospital records were evaluated to determine patient demographics, diagnoses, and perioperative data. Patients were followed up by echocardiograph to evaluate the patency of the graft until stage II procedure or death. RESULTS: MBTS was performed in 25 patients by 2 surgeons; 16% were neonates, and 60% had pulmonary atresia and 24% tetralogy of Fallot. The patients' median age was 2.6 months (range 0.2 to 372), and median weight was 5.3 kg (range 1.9 to 45). Preoperative oxygen saturation (SaO2) was 68.7% ± 7.8%. Forty-eight percent of patients received a 3.5-mm graft, and 20% received a concomitant pulmonary arterioplasty with cardiopulmonary bypass. Postoperative SaO2 was 83.2% ± 3.6%, significantly different from preoperative SaO2 (P < .05). Follow-up duration was 1.2 years (range 0.3 to 7.8), with no graft blockage. Three patients died in hospital from cardiorespiratory decompensation after MBTS with concomitant pulmonary arterioplasty. The median age of patients receiving a stage II procedure was 1 year (range 0.4 to 17.4). Actuarial 1-year survival was 79.7% (95% confidence interval 53.1% to 92.2%). CONCLUSION: MBTS continues to be valuable for palliation of complicated cyanotic congenital heart disease, yet mortality was considerable with concomitant pulmonary arterioplasty. With effective coagulation, the patency rate of grafts was high.

Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.

Because of remarkable surgical and medical advances over the past several decades, there are growing numbers of infants and children living with single ventricle congenital heart disease (SV), where there is only one functional cardiac pumping chamber. Nevertheless, cardiac dysfunction (and ultimately heart failure) is a common complication in the SV population, and pharmacological heart failure therapies have largely been ineffective in mitigating the need for heart transplantation. Given that there are several inherent risk factors for ventricular dysfunction in the setting of SV in addition to probable differences in molecular adaptations to heart failure between children and adults, it is perhaps not surprising that extrapolated adult heart failure medications have had limited benefit in children with SV heart failure. Further investigations into the molecular mechanisms involved in pediatric SV heart failure may assist with risk stratification as well as development of targeted, efficacious therapies specific to this patient population. In this review, we present a brief overview of SV anatomy and physiology, with a focus on patients with a single morphological right ventricle requiring staged surgical palliation. Additionally, we discuss outcomes in the current era, risk factors associated with the progression to heart failure, present state of knowledge regarding molecular alterations in end-stage SV heart failure, and current therapeutic interventions. Potential avenues for improving SV outcomes, including identification of biomarkers of heart failure progression, implications of personalized medicine and stem cell-derived therapies, and applications of novel models of SV disease, are proposed as future directions.

Near-infrared spectroscopy: An important tool during the blalock-taussig shunt.

Near infra red spectroscopy (NIRS) is a noninvasive diagnostic tool for measuring regional oxygen saturation (rSO2). Cerebral oxygenation measured with NIRS is used to corroborate mixed venous oxygenation and hence considered an indicator of tissue perfusion. We describe NIRS guiding an anatomical variation leading to inadequate cerebral circulation or any impairment in cerebral oxygen delivery during Blalock Taussig shunt.

Pulmonary artery growth after Modified Blalock-Taussig shunt: A single center experience.

OBJECTIVE: This study evaluates growth of pulmonary artery (PA) between different age groups after Modified Blalock-Taussig shunt (MBTS) based on a single center experience. METHODS: We retrospective analyzed outcomes of 90 patients undergoing MBTS in our institute from January 1, 2010 to May 1, 2018. Patients were divided into three groups: Group 1, ≦2 months, Group 2, >2 months, ≦24 wmonths, Group 3, >24 months. The outcome included PA growth, overall survival rates and postoperative complications. RESULTS: 23 patients were involved in Group 1, while 49 in Group 2 and 18 in Group 3. The diameter of the PA, McGoon ratio, Nakata index and the oxygen saturation increased significantly after MBTS. Compared to pre-MBTS, there was no statistically significant increase of McGoon ratio in Group 3 after performing MBTS, but it witnessed less secondary cardiac surgery rate compared with Group 2 (11.11% vs. 48.98%, P = 0.005). Cumulative survival rates of three groups were 60.53%, 85.70%, 94.40% at 1 year; 60.53%, 78.30%, 87.75% at 3 years and 60.53%, 78.30%, 87.75% at 5 years. Multivariable analysis showed diastolic blood pressure <30 mmHg [OR 14.14 (1.92-104.32), P = 0.009], cardiopulmonary bypass use [OR 16.79 (2.05-137.67), P = 0.009] and single ventricle anomaly [OR 8.80 (1.18-65.54), P = 0.034] were predictors of perioperative mortality. CONCLUSION: MBTS in our institute is a conventional and effective procedure for growth of PA especially for patients younger than 24 months. Patients with biventricular anomaly, no cardiopulmonary bypass use in surgery and relatively high postoperative diastolic blood pressure will likely offer a good prognosis.

Outcomes of Multistage Palliation of Infants With Single Ventricle and Atrioventricular Septal Defect.

BACKGROUND: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results. METHODS: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention. RESULTS: Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients. CONCLUSIONS: Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

Use of a Modified Classic BT Shunt in a Patient With Multiple Conduit Obstructions With Positive Lupus Anticoagulant.

The use of a right ventricle-to-pulmonary artery conduit has re-emerged as a popular alternative to a systemic artery-to-pulmonary artery shunt in the Norwood procedure. Both proximal obstruction secondary to dynamic compression and distal obstruction at the anastomosis site with the pulmonary arteries are well described. In an effort to prevent complications, a technique where in the conduit is placed through the entire full thickness, or dunked, through the RV free wall has been described. We report a case of a patient with HLHS and positive lupus anticoagulant who developed recurrent conduit obstructions. The "Sano" was abandoned in favor of an autologous source of pulmonary blood flow-the modified Blalock-Taussig shunt using the "turn down" of the carotid artery.