Ventricular Outflow Tract Stenting Research Articles

Palliative Right Ventricular Outflow Tract Stenting in Tetralogy of Fallot with Severe Cyanosis: Experience from a Single Center in Southern India

Background: This study conducted at an Indian tertiary care hospital aimed to characterize the clinical profile of tetralogy of Fallot (ToF) patients undergoing right ventricular outflow tract (RVOT) stenting and assess their immediate postprocedural outcomes until discharge. Methods: In this retrospective analysis, the clinical and procedural outcomes were analyzed for 20 patients who underwent palliative stenting in the RVOT for severe cyanosis associated with ToF during 2021–2023. Results: The median age for the patients was 2.3 years (range 3 months–21 years), two being adults (>18 years). Fourteen (70%) patients underwent elective RVOT stent and 30% underwent emergency stenting. The most common indication of RVOT stenting was hypoplastic branch pulmonary arteries (PAs) (n = 11, 78.6%) and cyanotic spell (n = 4, 66.7%) for elective and emergency surgeries, respectively. The average improvement in SpO2 was 27.2% (95% confidence interval 23.4–31.0, P < 0.001). Six (30%) patients had complications including reperfusion injury of lungs (n = 2, 33.4%) sepsis (n = 1, 16.7%), stent embolization (n = 1, 16.7%), re-stenting (n = 1, 16.7%), transient complete heart block (n = 1, 16.7%) during the procedure, and. One child went through RVOT stent postoperative due to severe residual stenosis and child developed severe tricuspid regurgitation and succumbed to death due to culture positive sepsis with multi-organ dysfunction. Rest (n = 19, 95%) were discharged with an average 6.7 days of hospital stay. Conclusion: ROVT stenting is a safe and effective palliative management in ToF enhancing oxygen saturation prior to definitive surgical repair.

The Current Strategy in Managing Congenital and Structural Heart Disease: Find and Treat Them Appropriately

Significant progress has been made in diagnosing and treating congenital heart defects (CHDs) over the past five decades. Notable advancements include pulse oximetry screening for critical CHDs in newborns and percutaneous, catheter-based procedures, improving outcomes even in developing nations. Recent studies demonstrate favorable outcomes with device closure of acyanotic CHDs, with non-fluoroscopy techniques adopted to minimize radiation risks. Positive results are observed with right ventricular outflow tract (RVOT) stent palliation for late-presenting cyanotic congenital heart diseases such as tetralogy of Fallot (TOF). Novel interventions like reverse Potts shunt anastomosis show promise in managing pediatric pulmonary arterial hypertension (PAH), while prophylactic treatments effectively reduce postoperative arrhythmias. Fontan procedure significantly enhances survival rates for individuals with single functional ventricles, although challenges arise with aging populations. In developed countries, fetal cardiac interventions utilizing advanced imaging techniques allow for early detection and preventive interventions, reducing secondary complications. Keyword: Fetal Intervention, Fontan, Non-Fluoroscopy, Percutaneous, Reverse Potts, Rheumatic Heart Disease, RVOT Stenting.

Personalized Approach in Transcatheter Palliation of Congenital Heart Disease with Duct-Dependent Pulmonary Circulation: Right Ventricular Outflow Tract Stenting vs. Arterial Duct Stenting.

Despite significant improvements in techniques, the treatment of neonates and infants with congenital heart disease resulting in duct-dependent pulmonary circulation is still significantly challenging. Despite current trends toward early primary surgical repair, temporary palliation is still necessary in those patients who are at high surgical risk for complete correction due to unfavorable clinical or anatomic characteristics. Recent advances in interventional cardiology have led to the emergence of right ventricular outflow tract and arterial duct stenting as cost-effective alternatives to surgical palliation in high-risk surgical candidates or whenever short-term pulmonary blood flow support is anticipated. This review aims to explore the evolving landscape of these transcatheter approaches, highlighting their role, efficacy and potential complications in the context of duct-dependent pulmonary circulation anatomies.

Right ventricular outflow tract stenting for late presenter unrepaired Fallot physiology: a single-center experience.

The purpose of this study was to assess the clinical outcome after right ventricular outflow tract (RVOT) stenting in late presenter patient with unrepaired Fallot physiology. In younger patients, RVOT stenting is an alternative to mBTT shunt; however, there have been few reports of this palliative technique in late presenter population, including adults. This was a single-center, retrospective study of nonrandomized, palliated Fallot patients. Clinical outcomes such as left ventricular ejection fraction and saturation were measured in 32 individuals following RVOT stenting in adults (n = 10) and children (n = 22). The Statistical Package for Social Science (SPSS) 26.0 software was used to analyze the statistical data. During the procedure, the average stent diameter and length were 8.84 ± 1.64 mm and 35.46 ± 11.23 mm, respectively. Adult patients received slightly longer stents than pediatric patients (43.60 ± 11.64 mm vs. 31.77 ± 9.07 mm). Overall, patients' saturation increased from 58.56 ± 19.03% to 91.03 ± 8.98% (p < 0.001), as did their left ventricular ejection fraction (LVEF) from 64.00 ± 18.21% to 75.09 ± 12.98% (p = 0.001). Three patients improved their LVEF from 31 to 55%, 31 to 67%, and 26 to 50%. The median length of stay was 8 (2-35) days, with an ICU stay of 2 (0-30) days. The median time from RVOT stent palliation to total repair was 3 months (range: 1 month-12 months). RVOT stenting is a safe and effective method for increasing saturation and ejection fraction not only in newborn infants but also in late presenters, including adults with unrepaired Fallot physiology.

Outcomes of Right Ventricular Outflow Tract Stenting as a Palliative Procedure in Tetralogy of Fallot Patients

Background: Certain groups of tetralogy of Fallot (TOF) patients require a palliative procedure until factors permit a full surgical repair. An aorto-pulmonary shunt is the gold standard palliative procedure but requires a cardiothoracic surgeon in a well-equipped facility. In the government sector in a developing country this limited resource has an overwhelming caseload. Right ventricular outflow tract (RVOT) stenting via cardiac catheterization by a trained paediatric cardiologist is a possible alternative. Objectives: To demonstrate that RVOT stenting is a safe and effective palliative procedure in TOF patients in a resource limited setting. Method: A retrospective, cohort observational study at Steve Biko Academic Hospital from January 2014 to March 2021. Results: 37 patients required RVOT stent placement. Mean oxygen saturation increased from 65% to 95% post-stent insertion. Mean pulmonary artery (PA) growth, measured by McGoon ratio, increased from 1.36 to 2.05. Average Intensive Care Unit stay was 2 days with a zero 30-day mortality. Three stents fractured requiring replacement. Conclusion: Stenting the RVOT in TOF patients who present at an older age, with multiple comorbidities and often in extremis, has yielded good results. Significant improvement in oxygen saturations and PA growth permits majority of our patients to a full TOF surgical repair.

Bridge to Surgery with Pulmonary Valve Perforation and Right Ventricular Outflow Tract Stenting in an Infant with PA-VSD

Pulmonary atresia (PA) with ventricular septal defect (VSD) is a rare congenital heart disease. Blood flow to pulmonary arteries is provided by patent ductus arteriosus or major aorto-pulmonary collateral arteries (MAPCAs). Palliative interventions are required to maintain the blood supply to the pulmonary arteries in order to achieve for the complete surgical repair. In this case report, we present a patient who underwent pulmonary valve perforation and right ventricular outflow tract (RVOT) stenting as an alternative to a systemic-pulmonary artery shunt (SPS). Subsequently, surgical repair was performed ten months later.

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.

Stenting of native right ventricular outflow tract obstructions in symptomatic infants: histological work-up of explanted specimen.

Stenting of stenotic right ventricular outflow tract is a palliative measure for severely impaired small babies with Tetralogy of Fallot or similar pathologies. Little is known about the histopathological fate of the stents in the right ventricular outflow tract. Eight samples of surgically removed right ventricular outflow tract stents were histologically analysed according to a predefined protocol. The most frequent diagnosis was Tetralogy of Fallot in four patients, pulmonary atresia with ventricular septal defect in two patients, double outlet right ventricle with pulmonary obstruction in one patient, and muscular obstruction of the right ventricular outflow tract in one patient with a syndromic disease with hypertrophic cardiomyopathy. Stents mean implantation duration was 444 days ranging from 105 to 1117 days (median 305.5 days). Histology revealed a variable degree of pseudointima formation consisting of fibromuscular cells surrounded by extracellular matrix. Four of the specimen contained adjacent myocardial tissue fragments, which showed regressive changes. Neither myocardium nor pseudointima tissue or tissue parts locally related to stent struts were infiltrated by inflammatory cells. Histological analysis after explantation of early-in-life implanted right ventricular outflow tract stents revealed predominantly pronounced neo-intimal proliferation with a visible endothelial layer, no signs of inflammation, and no prolapse of muscular tissue through the stent struts. Thus, implantation of stents in early life seems to interfere little with the hosts' immune system and might help to open up the right ventricular outflow tract by mechanical forces and regressive changes in adjacent muscular tissue.

Right Ventricular Outflow Tract Stenting is a Safe and Effective Bridge to Definitive Repair in Symptomatic Infants With Tetralogy of Fallot1.

Right ventricular outflow tract (RVOT) stent angioplasty is a palliative procedure for neonates and infants with symptomatic tetralogy of Fallot prior to surgical repair. We review our institutional outcomes of RVOT stenting. Retrospective review of all infants with tetralogy of Fallot under 3 months of age who underwent primary native RVOT stent angioplasty at The Children's Hospital at Westmead, Sydney, Australia between January 2010 and December 2020. Demographics and echocardiographic pulmonary artery dimensions were collected pre-stent angioplasty and prior to surgical repair. Twenty (20) infants underwent primary RVOT stenting. Median age at stent was 14 days (interquartile range [IQR] 7-32) and median weight 2.7 kg (IQR 2.1-3.4). Three patients underwent hybrid per-ventricular procedures. Indication for RVOT stenting was recurrent hyper-cyanotic spells in 12 (60%) and duct-dependent pulmonary blood flow in 8 (40%). Saturations increased from a median of 80% (IQR 75-85) to 91% (IQR 90-95) post procedure (P<0.001). A single major complication occurred: transient complete atrioventricular dissociation requiring isoprenaline infusion for <24 hours. Twelve (12, 60%) required catheter re-intervention prior to definitive repair for further augmentation of pulmonary blood flow. There were two non-cardiac deaths distant from the stent procedure, but prior to surgical repair. Median right and left pulmonary artery Z-scores increased respectively from -2.06 (IQR -2.99 to -0.17) and -1.2 (IQR -2.59 to -0.14) prior to RVOT stent, to -0.74 (IQR [-1.21 to 0.26], P=0.01) and 0.06 (IQR [-1.87 to 1.15], P=0.006) by the time of definitive repair. Eighteen (18) patients achieved definitive repair at a median age of 6.1 months (IQR 4.7-7.3). Palliation with more than one RVOT stent was associated with an increased duration of cardiac bypass (P=0.035) and cross-clamp (P=0.044) time at definitive repair. In symptomatic neonates and infants with tetralogy of Fallot at high-risk of peri-operative complications, RVOT stent angioplasty can safely and effectively augment pulmonary blood flow prior to definitive repair.

Right ventricular outflow tract stenting promotes pulmonary artery development in tetralogy of fallot.

Right ventricular outflow tract (RVOT) stenting seems to be suggested as a promising treatment option and an alternative to modified Blalock-Taussig shunt (mBTS) in the initial palliation of patients with Fallot-type lesions in recent years. This study sought to assess the effect of RVOT stenting on the growth of the pulmonary artery (PA) in patients with Tetralogy of Fallot (TOF). Retrospective review analyzing 5 patients with Fallot-type congenital heart disease with small pulmonary arteries who underwent palliative with RVOT stenting and 9 patients underwent modified Blalock-Taussig shunt within 9 years period. Differential left PA (LPA) and right PA (RPA) growth was measured by Cardiovascular Computed Tomography Angiography (CTA). RVOT stenting improved arterial oxygen saturation from median of 60% (interquartile range [IQR]: 37% to 79%) to 95% (87.5% to 97.5%) (p = 0.028). The LPA diameter Z-score improved from -2.843 (-3.51-2.037) to -0.78 (-2.3305-0.19) (p = 0.03), the RPA diameter Z-score improved from median -2.843 (-3.51-2.037) to -0.477 (-1.1145-0.459) (p = 0.002), the Mc Goon ratio increased from median 1 (0.8-1.105) to 1.32 (1.25-1.98) (p = 0.017). There were no procedural complications and all 5 patients have undergone final repair in the RVOT stent group. In the mBTS group, the LPA diameter Z-score improved from -1.494 (-2.242-0.6135) to -0.396 (-1.488-1.228) (p = 0.15), the RPA diameter Z-score improved from median -1.328 (-2.036-0.838) to 0.088 (-0.486-1.223) (p = 0.007), and there were 5 patients occur different complications and 4 patients was not attained the standards of final surgical repair. RVOT stenting, compared with mBTS, seems to better promote pulmonary artery growth, improve arterial oxygen saturations, and have less procedure complications in patients with TOF who being absolute contraindicated for primary repair due to high risks.

Comparison between the modified Blalock-Taussig shunt and right ventricular outflow tract stent in the palliative treatment for tetralogy of Fallot.

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. 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. 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). 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.

Rescue right ventricular outflow tract stenting for refractory hypoxic spells.

While right ventricular outflow tract stenting (RVOTS) has become an acceptable alternative to palliative surgery in Tetralogy of Fallot (TOF) and similar physiologies, its utility for relief of refractory hypoxic spells is unclear. Patients who underwent RVOTS for emergency relief of refractory hypoxic spells were identified. Specific modifications to enable expeditious RVOTS included use of stent delivery systems (guiding catheter or long sheath) upfront to minimize catheter exchanges; using coronary wires to cross RVOT initially; stabilizing the catheter with a wire in the aorta while crossing RVOT with a second wire. From 2015 to 2022, 11 patients underwent RVOTS for hypoxic spells refractory to medical management. Their median age was 27 days (IQR 8.5-442.5); weight 3.27 kg (2.7-8.96); 9 males. Median pulmonary annulus Z score was -4.13 (IQR-4.85 to-0.86). Thirteenstents with median diameter 5 (4-6.5) mm and length 19 (16-19.75) mm were implanted, fluoroscopy time:13.6 (11-26.3) min; procedure time (60, 30-70 min). All were ventilated. Oxygen saturations improved from 45% (40-60) to 90% (84-92); (p < 0.0001) with no major complications. Postprocedure ventilation was needed for 21 (20-49) hours and 4 required diuretic infusion for pulmonary over-circulation. Four needed re-stenting 13 days to 5 months later. At median follow-up of 7 (4-17) months; 2 died from unrelated causes, 3 underwent surgery (two correction and one aorto-pulmonary shunt) and 6 await surgery. RVOTS enables safe, expeditious and effective short-term palliation for refractory hypoxic spells. Specific technical modifications facilitate safety, ease and swiftness.

Right Ventricular Outflow Tract Stenting as the First Option for Palliation of Neonates with Severe Tetralogy of Fallot

Background: Neonates with Tetralogy of Fallot (TOF) might be prostaglandin dependent and cannot be discharged from the hospital except after complete correction or palliation. Palliation could be achieved by modified Blalock-Taussig Shunt (BTS), ductal stenting, or right ventricular outflow tract (RVOT) stenting. Objective: The objective of this study was to present our experience of palliating neonates with severe TOF who require augmentation of the pulmonary blood flow by RVOT stenting. Methods: This study was conducted on a series of patients who had RVOT stenting in PSCC-Qassim, Saudi Arabia, from August 2016 till December 2019. Cases that had RVOT stenting are included. The patient's clinical data, echocardiography, cardiac catheterization, and follow-up data were recorded. Data were analyzed using The Microsoft Excel data analysis tool. Results: During the study period, four consecutive patients with a diagnosis of TOF with significant cyanosis underwent RVOT stenting. The mean age and weight were 2.8±1.7 days and 2.9±0.1 kg respectively. All were on prostaglandin infusion before the intervention. The mean saturation before and after RVOT stenting was 63% (±11%) and 82% (±4%) respectively (p = 0.018). One patient required RVOT stent dilatation after 4 months. All patients are alive and had complete TOF repair at a median age of 6.5 months (range 6 to 12 months). The median follow-up period is 13 months. The median branch PAs diameter and Z-score before RVOT stenting and immediately before complete TOF repair were 2.8 mm (Z score: -2.5) and 5.5 mm (Z score: -0.5 to -1.16) respectively (p = 0.001). Conclusion: RVOT stenting is a reasonable alternative palliation for patients with severe TOF. The technique of stent deployment in the RVOT is challenging and requires pre-intervention planning and discussion. Using soft catheters, coronary wires, and the premounted coronary stents facilitate crossing of the RVOT and pulmonary valve and then stent deploymen

Preservation of Antegrade Pulmonary Blood Flow in Kawashima Procedure With Prior Right Ventricular Outflow Tract Stent.

Surgical management of single ventricle with interrupted inferior vena cava and azygos continuation typically requires a Kawashima procedure with subsequent completion of Fontan. However, this group is at risk of development of pulmonary arteriovenous malformations. Evidence suggests preservation of hepatic venous flow into the pulmonary circulation can potentially delay this development. We hereby describe a method of preserving antegrade pulmonary blood flow during the Kawashima procedure in the setting of prior right ventricular outflow tract stents.

Immediate and Mid-term Outcomes of Blalock-Taussig Shunt versus Right Ventricular Outflow Tract Stenting in Symptomatic Neonates with Tetralogy of Fallot

Background: The initial management of symptomatic neonate patients with tetralogy of Fallot (TOF) associated with unfavorable anatomy and significantly reduced pulmonary vascular flow is controversial and continues to be a clinical challenge. Objective: The aim of this study was to describe the clinical evolution and to compare branch pulmonary artery growth in symptomatic neonatal TOF patients who received a modified Blalock Taussig (mBT) shunt versus right ventricular outflow tract stent placement (RVOTs) at the Department of Cardiology, Hospital de Niños de Córdoba, between March 2011 and March 2021. Methods: A retrospective, observational study identified 113 patients with TOF, 20 of which (18%) were symptomatic neonates requiring initial palliative intervention. Categorical variables are expressed as percentage and continuous variables as median and interquartile range (IQR). A p value &lt;0.05 was considered significant. Results: Among the 20 patients included in the study, 11 (55%) formed the mBT shunt group and 9 (45%) the RVOTs group. In the mBT shunt group, pre-palliation right pulmonary artery (RPA) Z score was -3 (IQR 4.20) and increased to -1.6 (IQR 1.56) (p= 0.11), and left pulmonary artery (LPA) Z score of -2.5 (IQR 4.8) increased to -1.80 (IQR 2.36) (p= 0.44). In the RVOTs group, RPA Z score prior to the palliative procedure was -3.45 (IQR 3.83) and increased to -2.5 (IQR 3.58) (p= 0.021) and LPA Z score of -4.10 (IQR 2.51) increased to -2.00 (IQR 3.75) (p= 0.011). Pre-intervention O2 saturation of 75% (IQR 6) increased to 87% (IQR 9) in the mBT shunt group (p= 0.005) and from 75% (IQR 16) to 91% (IQR 13) in the RVOTs group (p= 0.008). Mean length of hospital stay after the procedure was 10 days (IQR 11) in the mBT shunt group and 6 days (IQR 2) in the RVOTs group (p= 0.095). Conclusions: In symptomatic neonates with TOF, both palliative strategies improved the clinical condition. In patients who received RVOTs, there was greater branch pulmonary artery growth. A larger number of cases and longer-term follow-up will be necessary to confirm these findings.

M13. Stenting of right ventricular outflow tract as palliation for patient with right ventricular outflow tract obstruction with ventricular shunt physiology: a systematic review and meta-analysis

Abstract Aims Right ventricular outflow tract (RVOT) stenting is emerging as an effective alternative to surgical palliation in selected patients. We aim to assess the outcome of RVOT stenting in patients suffered from RVOT obstruction with ventricular shunt physiology such as tetralogy of Fallot (ToF) and other type of lesions, also to compare its clinical outcomes with modified Blalock-Taussig shunt (mBTS) procedure. Method and Results A systematic review and meta-analysis was conducted according to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) statement. We systematically searched the relevant studies from date of inception to June 2021. Outcomes of interest were pulmonary artery (PA) growth and oxygen saturation after palliation. Eleven retrospective studies (384 patients) were included for qualitative analysis, and four studies (193 patients) were eligible for meta-analysis. From our study, RVOT stenting can be accomplished safely and effectively in severe cyanosis or high risk patients with ToF physiology. Right PA z-score and oxygen saturation post RVOT stenting was significantly higher compared with post mBTS (MD 0.50; 95% CI 0.06 – 0.93, p = 0.03 and MD 3.46; 95% CI 1.42 – 5.49, p = 0.0009 respectively). Our analysis shows no significant difference of left PA z-score after palliation between two groups (MD -0.05; 95% CI -0.89 – 0.79, p = 0.90). Conclusion RVOT stenting effectively improves oxygen saturation and promotes proper pulmonary artery growth in patients with ToF physiology. The larger and randomized-controlled studies are required to better evaluate the efficacy of RVOT stenting and its superiority compared to mBTS.

Evaluation of Right Ventricular Outflow Tract Stenting as Palliative Treatment in Severely Cyanotic Tetralogy of Fallot: A Systematic Review and Meta-Analysis of Observational Studies.

Background: Tetralogy of Fallot (ToF) is a cardiac malformation that accounts for up to 1/10 of all congenital diseases. Although surgical repair serves as a treatment of choice, it cannot be performed unless weight and anatomical key factors are favorable. The stenting of the right ventricular outflow tract (RVOT) has become an alternative palliative procedure for ToF as an option to alleviate infundibular obstruction with minimal invasion. Methods: A literature search was conducted through 7 databases, followed by the screening and independent assessment of 6 final studies, using the Newcastle-Ottawa Quality Assessment Scale (NOS). Analysis was then conducted using inverse variance analysis, and cumulative data were presented with forest and funnel plots. Results: Studied patients were referred for RVOT stenting due to the marked obstruction of the pulmonary blood flow, a low birth weight, or a small pulmonary artery size. The analysis revealed a significant increase in O2 saturation (mean difference [MD=18%; 13–23.78). The same trend was observed concerning an increase in the Nakata index (MD=54.59; 10.05–99.14), the right pulmonary artery diameter (MD=2.28; 1.20–3.36), and the left pulmonary artery diameter (MD=1.77; 0.22–3.32). Several complications were found, including tricuspid regurgitation and frequent premature beats. Conclusion: RVOT stenting is considered a feasible palliative treatment for ToF, with its high effectiveness in improving patients’ condition, especially their pulmonary flow. While complications are scarce, several conditions should be noted, particularly for fatal complications. Finally, this study has limitations as catheterization details in studies and diverse infants’ conditions may have caused potential bias

Emergency Department Presentation and Outcome of Children With Cyanotic Congenital Heart Diseases.

ObjectiveThis study aimed to assess the outcome and see the spectrum of presenting symptoms of children with cyanotic congenital heart diseases (CHDs) admitted through the emergency department in Lady Reading Hospital (LRH) in Peshawar, Pakistan.Materials and methodsThis cross-sectional study included 104 children with cyanotic CHD admitted through the emergency department in the pediatric cardiology unit of LRH from February 2019 to January 2020. Diagnosis of cyanotic CHD was confirmed through two-dimensional echocardiography. Patients were treated according to standard protocols for their presenting symptoms. The data were analyzed using Statistical Package for the Social Sciences (SPSS), version 20.0 (IBM Corp., Armonk, NY) and frequencies were expressed as percentages.ResultsThe study included 65 male cases (62.5%) and 39 female cases (37.5%), and participant ages ranged from 10 days to 15 years (mean, 2.7 ± 3.4 years). Of 104 patients, 70 presented with cyanotic spells (84.5%), 53 with fever (10.7%), 28 with respiratory distress (26.5%), 11 with loose stools (10.5%), 10 with hemiplegia (9.6%), and eight with seizures (7.6%). Pneumonia was a precipitating factor in 13 patients (12.5%), infective endocarditis in 12 patients (11%), gastroenteritis in 11 (11%), brain abscess and cerebrovascular accidents in seven patients each (6% each), meningitis in six patients (6%), and tuberculous pericardial effusion in one patient (<1%). The primary CHD was tetralogy of Fallot (TOF) in 52 patients, tricuspid atresia in 14 patients, pulmonary atresia in 13 patients, double outlet right ventricle in 10 patients, transposition of great arteries (TGA) in seven patients, and total anomalous pulmonary venous return (TAPVR), truncus arteriosus, congenitally corrected TGA, and critical pulmonary stenosis in two patients each. Twenty-six patients received treatment of the acute problem and instructions for follow-up monitoring. Twelve patients died during their hospital stay, and three left against medical advice. Fifteen patients received patent ductus arteriosus stenting, and six received right ventricular outflow tract stenting. One patient received a Blalock-Taussig (BT) shunt stent, and one received a left pulmonary artery stent. Two patients received a balloon pulmonary valvotomy, and one received pericardial effusion drainage. Eleven patients received surgical correction of TOF, 11 received surgical correction for BT shunt, four received surgical correction for brain abscess drainage, and two received TAPVR repair.ConclusionTOF was the most common cyanotic heart disease in our study. Cyanotic spells or increased cyanosis was the most common presenting concern. Cyanotic CHDs present with variable extracardiac signs and symptoms in emergencies. Appropriate assessment, immediate management of the acute problem, and timely intervention or surgical management result in good outcomes.

Surgical versus transcatheter palliation for insufficient pulmonary blood supply in infants with cyanotic CHD.

Infants with complex cyanotic CHD can become symptomatic from insufficient pulmonary blood supply following either ductal closure or due to outflow tract obstruction. Blalock-Taussig shunt mortality remains significant and recent studies have highlighted the advantages of using transcatheter alternatives. We present here our experience in changing our primary choice of palliation from the Blalock-Taussig shunt to transcatheter palliation with either a ductal stent or, if antegrade flow is present, a right ventricular outflow tract stent.This is a retrospective, single-unit cohort study. Eighty-seven infants underwent palliation for insufficient pulmonary blood flow at under 3 months of age between 2012 and 2019. On an intention-to-treat basis, 29 underwent insertion of a Blalock-Taussig shunt, 36 duct stents, and 22 right ventricular outflow tract stents at median ages of 15, 9, and 32 days, respectively, and median weights of 3.3, 3.1, and 3.1 kg, respectively. No primary Blalock-Taussig shunts have been performed in our institution since 2017.At 30-days there had been one death in each group (univariable p = 0.93) and deaths prior to repair totalled three in the shunt group, four in the ductal stent group, and two in the right ventricular outflow tract stent group (univariable p = 0.93). Reintervention on the pulmonary circuit prior to next stage of surgery was more frequent in those undergoing transcatheter intervention, reaching statistical significance by logrank (p = 0.012).In conclusion, within this work we provide further evidence of the safety and efficacy of transition from a primary surgical to primary transcatheter palliation pathway in infants with insufficient pulmonary blood supply.

Right Ventricular Outflow Tract Stenting as Palliation of Critical Tetralogy of Fallot: Techniques and Results

Background. Despite current trends toward early primary repair, the surgical systemic-to-pulmonary shunt is still considered the first-choice palliation in patients with critical tetralogy of Fallot (TOF) and duct-dependent pulmonary circulation unsuitable for primary repair. However, stenting of the right ventricular outflow tract (RVOT) is nowadays emerging as an effective alternative to surgical palliation in selected patients. Methods and results. RVOT stenting is usually performed from a venous route, either femoral or, in selected cases, the right internal jugular vein. Less frequently, mostly in pulmonary infundibular/valvar atresia, this procedure can be performed using a hybrid surgical/interventional approach by surgical exposure of the RVOT, puncture of the atretic valve, and stent deployment under direct vision. The size and type of the most appropriate stent may be chosen, based on ultrasound measurements of the RVOT, to cover the right ventricular infundibulum completely and, at the same time, sparing the pulmonary valve, unless significant pulmonary valve annulus hypoplasia and/or supra-valvular stenosis is a significant component of the obstruction. In the large series so far published, early mortality of RVOT stenting is less than 2%, comparing favourably with either Blalock-Thomas-Taussig shunt or early primary repair. In addition, morbidity and clinical sequelae of this approach do not significantly differ from surgical palliation, even if RVOT stenting shows lesser durability and a higher rate of trans-catheter re-interventions over a mid-term follow-up. Finally, similar but more balanced pulmonary artery growth than surgical palliation following RVOT stenting is reported over a mid-term follow-up. Conclusions. RVOT stenting is a technically feasible, well-tolerated, and effective palliation in critical TOF. This approach is cost-effective with respect to surgical palliation either in high-risk neonates or whenever a short-term pulmonary blood flow source is anticipated due to the early surgical repair. It effectively increases pulmonary blood flow, improves arterial saturation, and promotes balanced pulmonary artery growth over a mid-term follow-up.