Blalock-Taussig Research Articles

Factors Related to Early Postoperative Modified Blalock-Taussig Shunt Thrombosis and In-Hospital Mortality in Infants

Objective: The modified Blalock-Taussig shunt (mBTs) is used palliatively in cyanotic congenital heart diseases when definitive surgery is not possible. However, factors contributing to shunt thrombosis and in-hospital mortality in infancy remain unclear. The present study aimed to identify those risk factors. Materials and Methods: The authors conducted a retrospective review of medical records from the infants diagnosed with cyanotic congenital heart disease who underwent isolated mBTs procedures at Chiang Mai University Hospital between March 1, 2011 and April 30, 2015. Patient characteristics, operative data, and treatment outcomes were extracted. The primary outcome assessed was shunt thrombosis, with in-hospital mortality within 30 days as the secondary outcome. Risk regression analysis was used to identify factors associated with both outcomes. Results: Forty-nine infants were included in the present study, with a mean weight of 3.81 kilograms (range of 2.05 to 7.70 kg) and an average age of 66 days (range of 3 to 349 days). Early shunt thrombosis occurred in eight patients (16.33%), and five patients (10.2%) died. Multivariable analysis revealed only sepsis as a significant predictor (RR 7.93, 95% CI 1.86 to 33.69). Neither weight at surgery nor shunt size showed a significant association with shunt thrombosis. Prognostic factors for in-hospital mortality in the univariable analysis included prematurity (p=0.01), size of pulmonary artery (p=0.033), preoperative intubation (p=0.043) shunt thrombosis (p=0.002), and body weight at surgery of less than 3 kg (p=0.002). However, multivariable analysis could not demonstrate any difference. Conclusion: Postoperative sepsis emerged as the sole statistically significant predictor for early shunt thrombosis following isolated mBTs. However, larger studies are needed to ascertain prognostic factors for in-hospital mortality in infants with cyanotic congenital heart disease.

Role of BT shunt in tetralogy of Fallot.

Modified Blalock-Taussig shunt (BT shunt) is a palliative operation used for cyanotic heart diseases with decreased pulmonary blood supply. The definitive management of tetralogy of Fallot (TOF) is total corrective surgery, but these patients can be palliated with BT shunt. In the modern world, the BT shunt is getting out of favour in patients with TOF. In this article, we will share our 5-year experience at our institute, which also shows a decreasing trend. It is a retrospective study. Files of all the patients admitted in our department from January 2019 to December 2023 were reviewed. Age, weight, hospital stay, inotropic support duration, mechanical ventilation duration, and outcomes were studied. From January 2019 to December 2023, 173 patients underwent BT shunt for TOF. The mean age was 31 months, and the mean weight was 9.3 kg. The overall mortality for BT shunt was 15% after BT shunt. Hypercyanotic spell not controlled by medical management was the most common indication for BT shunt in our setup. Most of the patients with hypercyanotic spells were also candidates for total correction but due to the emergency, BT shunt was performed. The role of BT shunt in patients with TOF is decreasing due to PDA/RVOT stenting, it is likely that the BT shunt in TOF will become a thing of the past in the future even in developing countries like ours.

Impact of Shunt/Pulmonary Artery Ratio on Outcomes in Modified Blalock-Taussig Shunt Patients

Background: The modified Blalock-Taussig shunt (mBTT shunt) is a critical procedure for managing congenital heart defects by improving pulmonary circulation. Traditionally, shunt size is determined based on patient weight and standard guidelines. However, recent evidence indicates that the shunt size relative to the pulmonary artery diameter (S/PA ratio) may be a more accurate predictor of outcomes. This study evaluates the impact of the S/PA ratio on mortality and other clinical outcomes in patients undergoing the mBTT shunt. Methods: This retrospective study analyzed the medical records of 36 patients who underwent mBTT shunt at Soetomo General Academic Hospital between 2021 and 2023. Data on demographics, clinical characteristics, procedural details, and outcomes were collected, focusing on variables such as age, weight, McGoon ratio, pre- and post-operative SpO2 levels, and S/PA ratio. Statistical analyses included Chi-square tests for categorical variables and independent t-tests for continuous variables, with a p-value of <0.05 considered significant. Results: The cohort had a mean age of 54.81 months and a mean weight of 12.18 kg. Preoperative SpO2 averaged 71.44%, improving to 79.26% post-operatively. Patients with an S/PA ratio >1.00 had a significantly higher mortality rate (37.5%) compared to those with an S/PA ratio <1.00 (7.7%) (p = 0.029). These patients also experienced longer ICU stays (4.92 vs. 2.06 days, p = 0.005) and more ventilation days (3.40 vs. 1.68 days, p = 0.047). The hospital stay was also extended in the higher ratio group (8.60 vs. 5.50 days, p = 0.076). No significant differences were observed in pre- and post-operative SpO2 levels or inotropic use. Conclusion: A higher S/PA ratio is associated with increased mortality, longer ICU and hospital stays, and greater ventilation needs following the mBTT shunt. These findings suggest that optimizing shunt size relative to pulmonary artery diameter could lead to better outcomes. Further research is needed to confirm these results and refine surgical strategies for enhanced patient care.

Comparative Analysis of Modified BT Shunt and Central Shunt in Pediatric Patients.

Cyanotic congenital heart diseases constitute 40-45% of all congenital heart diseases. In patients who are not suitable for primary repair, modified BT (MBT) shunt and central shunt (CS) procedures are still frequently used. This study included 62 pediatric patients who underwent MBT shunt or CS via median sternotomy. Patients' demographic, echocardiographic, operative, and postoperative data were collected retrospectively. The patients were classified as single ventricle and bi-ventricle according to their cardiac anatomy, and the presence of prematurity and heterotaxy was noted. Procedure details of the patients who underwent endovascular intervention prior to the surgery were investigated, and operation data were accessed from the surgery notes. Data regarding postoperative follow-ups were obtained and comparatively analyzed. Of the total 62 patients, 32 (51.6%) were newborns and 16 (25.8%) had a body weight < 3 kg. MBT shunt was applied to 48 patients (77.4%), while CS was applied to 14 patients (22.6%). There was no significant difference between the two surgical procedures in terms of requirement for urgent shunt or cardiopulmonary bypass, additional simultaneous surgical intervention, need for high postoperative inotropes, and in-hospital mortality (P>0.05). The rate of congestive heart failure in patients with in-hospital mortality was determined as 66.7% and it was significantly higher than in patients without heart failure (P<0.001). MBT shunt and CS are still frequently used in cyanotic patients. The use of small-diameter shunts, particularly when centrally located, can prevent the onset of congestive heart failure and lower mortality.

May 2024 Medical Image of the Month: Hereditary Hemorrhagic Telangiectasia in a Patient on Veno-Arterial Extra-Corporeal Membrane Oxygenation

No abstract available. Manuscript truncated after 150 words. A 54-year-old man with a complex cardiac history, including Tetralogy of Fallot requiring Blalock-Taussig shunt in infancy, infundibular patch repair at age 7, and bioprosthetic tricuspid valve replacement at age 52, had ongoing frequent hospitalizations with decompensated right ventricular heart failure secondary to native pulmonary valve mixed stenosis plus regurgitation and left pulmonary artery stenosis. His case was further complicated by his history of hereditary hemorrhagic telangiectasia (HHT) with recurrent epistaxis and recent GI bleeds with multiple angiodysplastic lesions throughout the stomach, duodenum, and descending colon which were previously treated with argon plasma coagulation. The patient was admitted to our hospital in NYHA class IV heart failure receiving a continuous dopamine infusion and aggressive diuresis. Upon admission, a right heart catheterization demonstrated severe pulmonary valve regurgitation, left pulmonary artery stenosis, and systemic hypoxemia suggestive of an intrapulmonary shunt. Admission transthoracic echocardiogram demonstrated normal left ventricular ejection fraction of 55-60%, a …

Surgically repaired tetralogy of Fallot in the 7th decade: a late presentation of severe pulmonic regurgitation.

Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical management in adults with TOF once pulmonary regurgitation occurs. A 73-year-old male with surgically corrected TOF presented with heart failure symptoms. He underwent ToF repair with a classic right Blalock-Taussig shunt at 2years of age with transannular patching at 18years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular dilatation, and pulmonary regurgitation. Our patient's new-onset right-sided heart failure was managed medically with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization with resolution of symptoms and improved tricuspid regurgitation. It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs; however, medical optimization should take place first. Following adequate RV load optimization, our patient underwent successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.

Internal thoracic artery as a growing conduit for pulmonary shunt.

Studies suggest the internal thoracic artery as a shunt option due to its growth potential. However, long-term data are lacking. Here, a patient with a failing single ventricle shunt had an enlarged internal thoracic artery. We followed the patient for 12 years after converting this artery into a Blalock-Taussig shunt, analysing its growth to assess its effectiveness.

Change in Pulmonary Arteries after Modified Blalock-Taussig Shunt Procedure: Analysis Based on Computed Tomography.

Although the modified Blalock-Taussig shunt remains the mainstay method of palliation for augmenting pulmonary blood flow in various congenital heart diseases, the shunt must be carefully designed to achieve the best outcomes. This study investigated the effect of shunt configuration on pulmonary artery growth and growth discrepancy. Twenty patients with successful modified Blalock-Taussig shunt takedown were analyzed. Pulmonary artery and shunt characteristics were obtained using computed tomography scans. Differences in the baseline and follow-up diameter ratios and growth in the ipsilateral and contralateral arteries were calculated. The angle between the shunt and pulmonary artery, as well as the distance from the main pulmonary artery bifurcation, were measured. Correlations between pulmonary arteries and shunt configurations were analyzed. The median interval time between shunt placement and takedown was 154.5 days (interquartile range, 113.25-276.25 days). Follow-up values of the ipsilateral-to-contralateral pulmonary artery diameter ratio showed no significant correlation with the shunt angle (ρ=0.429, p=0.126) or distance (ρ=0.110, p=0.645). The shunt angle and distance from the main pulmonary bifurcation showed no significant correlation (ρ=-0.373, p=0.189). Pulmonary artery growth was negatively correlated with shunt angle (ipsilateral, ρ=-0.565 and p=0.035; contralateral, ρ=-0.578 and p=0.030), but not with distance (ipsilateral, ρ=-0.065 and p=0.786; contralateral, ρ=-0.130 and p=0.586). Shunt configuration had no significant effect on growth imbalance. The angle and distance of the shunt showed no significant correlation with each other. A more vertical shunt was associated with significant pulmonary artery growth. We suggest a more vertical graft design for improved pulmonary artery growth.

Anesthesia Management in Blalock-Taussig Shunt Procedure

Background: The systemic to pulmonary artery shunts are done as palliative procedures for complex cyanotic congenital heart diseases. Blalock-Taussig shunt (BT shunt) provide regulated blood flow to the lungs allowing growth of pulmonary arteries until the patient reaches proper age and body weight suitable for definitive corrective repair. BT shunts are first line management in patients with critical cyanotic conditions.Case: A 12-month-old boy diagnosed with PA-VSD subaortic, L-R shunt PDA and critical PDA stenosis experienced a recurrent spell condition with the lowest oxygen saturation 40%, underwent urgent BT shunt surgery. Oxygen saturation increases to 80-85% after shunt procedure.Discussion: Anesthesia management includes optimizing preoperative condition and patient hydration state, providing balance anesthesia during surgery, maintaining balance of pulmonary and systemic blood flow. High oxygen fraction can be given to maintain oxygen saturation before BT shunt anastomosis. Mechanical ventilation, heart rate with sinus rhythm, preload and contractility is maintained to obtain normal cardiac output. After BT shunt anastomosis, the oxygen fraction is reduced with a saturation target of 70–85%. Postoperative management includes anticoagulant administration and monitoring postoperative complications. The patient developed complications of increased pulmonary blood flow postoperatively and was admitted to the PICU for 3 days. The patient was discharged in good condition from ward on day 7.Conclusion: Understanding the physiology of heart defects and perioperative management determine the success of BT shunt surgery, reducing patient morbidity and mortality. Optimizing intraoperative and postoperative oxygen delivery with oxygenation targets PaO2 40-45 mmHg and saturation 70-80% reflects the balance of pulmonary blood flow and systemic blood flow (Qp:Qs=0.7-1.5:1).

Early outcomes after post-cardiotomy extracorporeal membrane oxygenation in paediatric patients: a contemporary, binational cohort study.

The aim of this study was to assess the early outcomes and risk factors of paediatric patients requiring extracorporeal membrane oxygenation after cardiac surgery (post-cardiotomy). Retrospective binational cohort study from the Australia and New Zealand Congenital Outcomes Registry for Surgery database. All patients younger than 18 years of age who underwent a paediatric cardiac surgical procedure from 1 January 2013 to 31 December 2021 and required post-cardiotomy extracorporeal membrane oxygenation (PC-ECMO) in the same hospital admission were included in the study. Of the 12290 patients included in the study, 376 patients required post-cardiotomy ECMO (3%). Amongst these patients, hospital mortality was 35.6% and two-thirds of patients experienced a major complication. Hypoplastic left heart syndrome was the most common diagnosis (17%). The Norwood procedure and modified Blalock-Taussig shunts had the highest incidence of requiring PC-ECMO (odds ratio of 10 and 6.8 respectively). Predictors of hospital mortality after PC-ECMO included single-ventricle physiology, intracranial haemorrhage and chylothorax. In the current era, one-third of patients who required PC-ECMO after paediatric cardiac surgery in Australia and New Zealand did not survive to hospital discharge. The Norwood procedure and isolated modified Blalock-Taussig shunt had the highest incidence of requiring PC-ECMO. Patients undergoing the Norwood procedure had the highest mortality (48%). Two-thirds of patients on PC-ECMO developed a major complication.

Technical Details of a Novel Surgical Procedure for Construction of the Modified Blalock–Taussig Shunt (UKC’s Modification): A Video Presentation

We describe herein the surgical technique on a 2.5-years-old male child weighing 10 kilograms diagnosed with a functionally univentricular heart, disproportionately small right subclavian artery, and hypoplastic pulmonary arteries (Mc Goon’s ratio of less than 1.0).5 We interposed a short segment of polytetrafluroethylene graft between the right brachiocephalic artery and the intrapericardial portion of the right pulmonary artery closest to the bifurcation of the pulmonary trunk on the right side.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Norwood procedure.

To evaluate longitudinal systemic ventricular function and atrioventricular valve regurgitation in patients after the neonatal Norwood procedure. Serial postoperative echocardiographic images before Fontan completion were assessed in neonates who underwent the Norwood procedure between 2001 and 2020. Ventricular function and atrioventricular valve regurgitation were compared between patients with modified Blalock-Taussig shunt and right ventricle to pulmonary artery conduit. A total of 335 patients were identified including 273 hypoplastic left heart syndrome and 62 of its variants. Median age at Norwood was 8 (7-12) days. Modified Blalock-Taussig shunt was performed in 171 patients and the right ventricle to pulmonary artery conduit in 164 patients. Longitudinal ventricular function and atrioventricular valve regurgitation were evaluated using a total of 4352 echocardiograms. After the Norwood procedure, ventricular function was initially worse (1-30 days) but thereafter better (30 days to stage II) in the right ventricle to pulmonary artery conduit group (P < 0.001). After stage II, the ventricular function was inferior in the right ventricle to the pulmonary artery conduit group (P < 0.001). Atrioventricular valve regurgitation between the Norwood procedure and stage II was more frequent in the modified Blalock-Taussig shunt group (P < 0.001). After stage II, there was no significant difference in atrioventricular valve regurgitation between the groups (P = 0.171). The effect of shunt type on haemodynamics after the Norwood procedure seems to vary according to the stage of palliation. After the Norwood, the modified Blalock-Taussig shunt is associated with poorer ventricular function and worse atrioventricular valve regurgitation compared to right ventricle to pulmonary artery conduit. Whereas, after stage II, modified Blalock-Taussig shunt is associated with better ventricular function and comparable atrioventricular valve regurgitation, compared to the right ventricle to pulmonary artery conduit.

Transcatheter dilation and stenting of the modified blalock-taussig shunt in cyanotic heart diseases: points to consider. A single-center experience.

Our study focuses on the technique and results of interventional dilation and stenting of the modified Blalock-Taussig shunt (MBTS) performed in our center, providing a comprehensive review of our practice over the past 4 years. 42 catheter interventions on MBTS performed on 32 patients between January 2020 and May 2024 included 11 balloon dilatations and 31 stenting procedures. They were analyzed retrospectively. We documented early and late complications, the need for in-shunt reintervention or surgical revision, in-stent thrombotic events, and sudden death. The median age, weight, body surface area, and procedural radiation time at the time of the intervention were 100 days (15-870 days), 5 kg (2.6-12 kg), 0.3 m2 (0.19-0.54 m2), and 12 min (7-28 min). Four interventions were performed as an emergency in three patients, of which three were performed with ECMO support. The interventions were semi-emergent for severe hypoxia in 22 patients and elective for mild desaturation in the rest. All interventions were successfully performed without any intervention-related complications or death. Eighty-two percent of the shunt dilations led to saturation improvement. Of the 31 shunt stentings performed in 26 patients, saturation improvement was documented in 97% of the cases. The late complication with stent thrombosis was documented in one patient. In two patients, sudden death and cardiac decompensation with the need for resuscitation were documented. MBTS interventions are effective in emergent and semi-emergent situations with severe hypoxia. While serial balloon dilatations improved the luminal diameter in clipped MBTS, most patients needed stenting as a definitive procedure. In some cases, enlargement of the shunt via stenting may be an alternative to the surgical shunt revision to accommodate the shunt size and weight and delay the subsequent operation when there are contraindications. Dual antiplatelet therapy is strongly recommended to reduce thrombotic events, especially in shunts with more than one stent and those that need reinterventions.

Clinical outcomes of right ventricular outflow tract stenting compared to surgical shunting in late-presenting children.

Right ventricular outflow tract (RVOT) stenting as an alternative palliation for patients with stenotic RVOTs is increasingly recognized. However, a notable gap remains in the literature regarding the efficacy and the comparative outcomes between RVOT stenting and the modified Blalock-Taussig shunt (mBTS) in children older than one year. We conducted a retrospective review of clinical data from patients aged one year to 18 years with stenotic RVOTs who underwent RVOT stenting or mBTS procedures at our institution between December 2019 and October 2022. We compared major adverse cardiovascular events (MACE) including re-hospitalization, re-intervention, and mortality, hospital and ICU length of stay, and discharge oxygen saturation between the groups. We identified 58 patients (51.7% male) with a median age of 2.6 years (IQR: 2-8.1) and a median weight of 9.7 kg (IQR: 7.5-17.5). Among them, 18 (31%) patients received RVOT stenting, and 40 (68.9%) patients had mBTS. The median age for the RVOT stenting group was 92.5 months (IQR: 31.2-152) compared to 26.5 months (IQR: 23.0-54.0) for the mBTS group (p = 0.218). MACEs occurred in 4 (22.2%) patients with RVOT stents and 8 (20%) patients with mBTS (p = 0.624). Patients with mBTS had a longer ICU stay (median 3.5 days, IQR, 2-5) compared to those with RVOT stents (median 2 days, IQR: 1-2) (p = 0.295). Conversely, the hospital stay for patients with mBTS was shorter (median 10 days, IQR, 7.5-13.7) than for those with RVOT stents (median 11.5 days, IQR, 7-19) (p = 0.045). The median discharge oxygen saturation was 87% (IQR: 83-88) in the mBTS group and 80% (IQR: 75-87) in the RVOT stenting group (p = 0.212). RVOT stenting as palliation to stenotic RVOTs in children older than one year demonstrated outcomes comparable to mBTS in terms of MACE and achieving oxygen saturation targets.

Outcomes after surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: a series of 104 cases

Objective: To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies. Methods: A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children's Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged (M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results: In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95%CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95%CI: 89.9% to 100%). Conclusions: Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

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

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. 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. 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. 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.

Pulmonary Annulus Growth Pattern in Patients with Tetralogy of Fallot Prior to Surgical Repair.

The size of the pulmonary valve annulus often determines the feasibility of pulmonary valve preservation at the time of intracardiac repair of Tetralogy of Fallot. Currently, there is limited available data regarding the growth pattern and the determining factors that contribute towards pulmonary valve annulus growth. This retrospective study included patients who underwent surgical repair of Tetralogy of Fallot with or without prior palliation. These patients had an echocardiogram at the time of initial diagnosis and a second echocardiogram prior to intracardiac repair. The sizes of the pulmonary annulus, the right and left pulmonary arteries with z-scores were recorded. Patients with improvement in the pulmonary annulus z-scores between the 2 echocardiographic examinations were allocated in Group I (n = 46) and Group II (n = 68) were those with no improvement. A total of 114 patients were included in the study. The right and left pulmonary arteries size and z scores improved significantly between the 2 echocardiograms. Although the median size of the pulmonary annulus increased between the 2 echocardiograms (6 and 7.9 mm; P<0.001), there was no significant change in the z-score (-2.2, -2.34; P = 0.185). Multivariate logistic regression analysis showed that gender, blood group, presence of collaterals, and palliation with Blalock-Taussig shunt had no impact on the improvement in pulmonary annulus z-score. In Tetralogy of Fallot, the pulmonary valve annulus z-score may not change significantly prior to the intracardiac repair. Although in certain subgroups there may be an improvement, there was no specific factor that could be identified and had an influence on this improvement.

Assessment of the pulmonary arterial growth and results of two-stage repair in infants with severe form of tetralogy of Fallot

Aim: assess of the pulmonary artery growth and the outcomes of complete repair after palliative treatment in infants with critical form of Tetralogy of Fallot.Methods. This was prospective randomized two-center study (2019 to 2022). Its included infants with Tetralogy of Fallot who underwent palliation with either stenting of the right ventricular outflow tract (stent group, n=21) or modified Blalock-Taussig shunt (shunt group, n=21).Results. In the stent group Nakata index increase from median 104.2 to 208.6 mm2/m2, while in the shunt group, it increased from 107.3 to 169.4 mm2/m2 (p&lt;0,01). Mixed model analysis showed that in stent group the right pulmonary artery growth rate was 2.05*10-2 z scores/day, which was 3.01 times higher than in the shunt group. In stent group the left pulmonary artery growth rate was 2.3*10-2 z scores/day, which was 1.47 times higher than in the shunt group. In the stent group, there was one noncardiac-related mortality during the intermediate period. Transannular patch repair of the right ventricular outflow tract was performed in 12 patients (60%) in the stent group and in 15 patients (71.4%) in the shunt group (p=0.52) during complete repair. At 8 cases (40%) in the stent group and 6 cases (28.6%) in the shunt group, pulmonary artery replacement was performed. Time to surgical repair was shorter in the stent group (p=0.046), while the aortic cross clamp time (p&lt;0,01) and cardiopulmonary bypass time (p&lt;0,01) were significantly shorter in the shunt group.Conclusions. Right ventricular outflow tract stenting provides hemodynamic stabilization and uniform growth of the pulmonary artery compared to modified Blalock-Taussig shunt.

Cardiac Laterality: Surgical Results of Right Atrial Isomerism.

Right atrial isomerism (RAI) is a complex entity with varying diagnostic and treatment outcomes due to its rarity. Treatment options range from palliative to corrective surgeries, resulting in heterogeneous outcomes. The aim of this study was to analyze the results obtained after cardiac surgery in patients with RAI. A retrospective study was conducted, including patients diagnosed with RAI who underwent cardiac surgery. Their follow-up was from 1 January 2010 to 31 March 2020. Demographic characteristics and perioperative conditions were described. Thirty-eight patients were included, the median age was 4 years (IQR 2-9.2) and 57.9% were men. The main diagnoses were atrioventricular canal (63.2%) and pulmonary stenosis (55.3%). The most common surgical procedures were modified Blalock-Taussig shunt (65.8%) and total cavopulmonary connection with an extracardiac conduit fenestrated without cardiopulmonary bypass (15.9%). We did not find any factors associated with negative outcomes in these patients. The overall survival was 86.8%, with a better outcome in those who did not require reintubation (log rank, p < 0.01). The survival of RAI was similar to other centers. Individuals with RAI should be evaluated rigorously to determine an adequate repair strategy, considering high morbidity and mortality.

Three-dimensional-printed heart model can determine univentricular repair strategy in borderline double-outlet right ventricle

BackgroundAs double-outlet right ventricle has a wide pathophysiology spectrum, its comprehensive treatment strategy is determined based on relevant factors, such as the location and size of the ventricular septal defect, ventricular volume, and relationship of the great arteries. However, for borderline double-outlet right ventricle cases, it is occasionally difficult to decide the treatment strategy preoperatively. Recently, advances in 3D printing technology based on computed tomography have enabled the creation of 3D heart models of congenital heart disease that can precisely reproduce the anatomical structure of each patient even for complex anomalies. Herein, we describe a young patient in whom univentricular repair could be decided after confirming the 3D heart model and intracardiac structure under direct vision.Case presentationWe describe a 3-year-old girl who was diagnosed with double-outlet right ventricle and severe pulmonary valve stenosis at birth and who underwent a left modified Blalock–Taussig shunt at 2 years of age. Preoperative examination revealed a borderline condition for biventricular repair characterized by a small left ventricle volume and side-by-side relationship of the great artery. After a preoperative discussion using a 3D heart model, we concurred that an intraoperative assessment would be made as to whether biventricular repair was possible or not. After confirming the intracardiac structure under direct vision, we assessed that intraventricular rerouting was not possible owing to the high risk of subvalvular aortic stenosis as there was no tissue that could be incised between the right ventricular free wall and the primary interventricular foramen, as indicated in the 3D heart model. Thus, atrial septostomy and Glenn anastomosis were performed.ConclusionsWe report a 3-year-old girl with a borderline double-outlet right ventricle in whom a univentricular repair strategy could be decided after confirming the 3D heart model and intracardiac structure under direct vision. A 3D-printed heart model can be useful in patients whose repair strategy is difficult to judge for the borderline double-outlet right ventricle.