Hemodynamic simulation of complete transposition of the great arteries for optimal treatment strategies based on its circulatory physiology.

Our study aimed to elucidate the role of different shunts and provide novel insights into optimal treatment approaches for complete transposition of the great arteries (TGA), which is characterized by unique and complicated circulatory dynamics. We constructed a computational cardiovascular TGA model and manipulated cardiovascular parameters, such as atrial septal defect (ASD) and patent ductus arteriosus (PDA) sizes, to quantify their effects on oxygenation and hemodynamics. In addition, ASD flow patterns were investigated as innovative indications for balloon atrial septostomy (BAS). Our model of TGA with an intact ventricular septum (TGA-IVS) showed that a large ASD can achieve sufficient mixing for survival without PDA, and the presence of PDA is detrimental to oxygen delivery. A treatment strategy for TGA-IVS that enlarges the ASD as much as possible by BAS and PDA closure would be desirable. In TGA with a ventricular septal defect (TGA-VSD), the VSD allows for higher oxygenation and reduces the detrimental effects of PDA on systemic circulation. In TGA-VSD, both strategies of enlarging the ASD by BAS with a closed PDA and adjusting the PDA in response to pulmonary vascular resistance (PVR) reduction without BAS may be effective. The simulated ASD flow patterns showed that the sharp peak left-to-right flow pattern in systole (σ-wave) reflected the hemodynamically significant ASD size, independent of PDA, VSD, and PVR. The ASD flow pattern visualized by Doppler echocardiography provides clinical insights into the significance of an ASD and indications for BAS, which are not readily apparent through morphological assessment.NEW & NOTEWORTHY Complete transposition of the great arteries (TGA) represents complex and unique circulation that is dependent on blood mixing through multiple interacting shunts. Consequently, the role of each shunt and the treatment strategy remain unclear. We developed a mathematical model of TGA circulation, revealing the significant influence of atrial septal defect (ASD) on oxygenation and hemodynamics. The blood flow pattern through the ASD reflects its hemodynamic impact and helps determine treatment strategies.

Balloon atrial septostomy for transposition of the great arteries: Safety and experience with the Z-5 balloon catheter.

BACKGROUND: Balloon atrial septostomy (BAS) is an emergent and essential cardiac intervention to enhance intercirculatory mixing at atrial level in deoxygenated patients diagnosed with transposition of the great arteries (TGA) and restrictive foramen ovale. The recent recall of several BAS catheters and the changes in the European legal framework for medical devices (MDR 2017/745), has led to an overall scarcity of BAS catheters and raised questions about the use, safety, and experience of the remaining NuMED Z-5 BAS catheter. AIMS: To evaluate and describe the practice and safety of the Z-5 BAS catheter, and to compare it to the performance of other BAS catheters. METHODS: A retrospective single-center cohort encompassing all BAS procedures performed with the Z-5 BAS catheter in TGA patients between 1999 and 2022. RESULTS: A total of 182 BAS procedures were performed in 179 TGA-newborns at Day 1 (IQR 0-5) days after birth, with median weight of 3.4 (IQR 1.2-5.7) kg. The need for BAS was urgent in 90% of patients. The percentage of BAS procedures performed at bedside increased over time from 9.8% (before 2010) to 67% (2017-2022). Major complication rate was 2.2%, consisting of cerebral infarction (1.6%) and hypovolemic shock (0.5%). The rate of minor complications was 9.3%, including temporary periprocedural AV-block (3.8%), femoral vein thrombosis (2.7%), transient intracardiac thrombus (0.5%), and atrial flutter (2.2%). BAS procedures performed at bedside and in the cardiac catheterization laboratory had similar complication rates. CONCLUSIONS: BAS using the Z-5 BAS catheter is both feasible and safe at bedside and at the cardiac catheterization laboratory with minimal major complications.

Fetoscopic laser versus amnioreduction, septostomy, and expected management for the treatment of twin-twin transfusion syndrome (TTTS): an economic evaluation analysis in Iran.

BACKGROUND: Twin-twin transfusion syndrome (TTTS) affects 10-15% of monochorionic twin pregnancies. Without treatment, their mortality rates would be considerable. There are differences in survival rate between different therapeutic modalities. This study aims to compare the cost-effectiveness of Fetoscopic laser versus amnioreduction, septostomy, and expected management in the treatment of twin-to-twin transfusion syndrome (TTTS). METHODS: This is a cost-effectiveness analysis of the treatment strategies in patients with TTTS. A decision tree model was used to estimate the clinical and economic outcomes with a pregnancy period time horizon. Medical direct costs were extracted in a quantitative study, and survival rates were determined as effectiveness measures based on a review. A probabilistic sensitivity analysis was used to measure the effects of uncertainty in the model parameters. The TreeAge, Excel and R software were used for analyzing data. RESULTS: In the first phase, 75 studies were included in the review. Based on the meta-analysis, a total of 7183 women treated with Fetoscopic laser, the perinatal survival of at least one twin-based pregnancy was 69%. In the second phase, the results showed that expected management and amnioreduction have the lowest (791.6$) and highest cost (2020.8$), respectively. Based on the decision model analysis, expected management had the lowest cost ($791.67) and the highest rate in at least one survival (89%), it was used only in early stages of TTTS. Fetoscopic laser surgery, with the mean cost 871.46$ and an overall survival rate of 0.69 considered the most cost-effectiveness strategy in other stages of TTTS. CONCLUSION: Our model found Fetoscopic laser surgery in all stages of TTTS to be the most cost-effective therapy for patients with TTTS. Fetoscopic laser surgery thus should be considered a reasonable treatment option for TTTS.

Effect of Atrial Septostomy on Left Ventricular Function in Pediatric Dilated Cardiomyopathy Requiring ECMO.

Balloon atrial septostomy (BAS) reduces left ventricular (LV) hypertension during extracorporeal membrane oxygenation (ECMO). However, the acute effect of BAS on LV function as measured by echocardiography is unknown. This was a Retrospective analysis of clinical outcome, LV dimensions, and LV function in dilated cardiomyopathy patients 0-18 years old who underwent BAS on ECMO. In 13 patients with median (IQR) age of 2.3 (0.6-10.9) years, there were no differences in clinical markers of cardiac output at intervals between 12 h before and 6 days after BAS. In addition, BAS was associated with a low rate of periprocedural complications (0.0%), acute kidney injury (7.7%), and worsening radiographic pulmonary vascular congestion (30.7%). There was a significant worsening in LV end systolic diameter (LVIDs; 3.6 [2.9-4.8] cm vs 4.2 [3.2-5.6] cm vs 3.3 [2.6-4.6] cm, p = 0.025), LV end systolic posterior wall thickness (LVPWs; 0.7 [0.5-0.9] cm vs 0.6 [0.5-0.9] cm vs 0.8 [0.6-1.2] cm, p = 0.038), fractional shortening (FS; 17.6% [8.4-20.4%] vs 6.3% [2.0-9.9%] vs 13.2% [3.6-23.4%], p = 0.013), and ejection fraction (EF; 13.1% [8.7-18.9%] vs 5.3% [2.5-11.1%] vs 9.2% [6.0-16.3%], p = 0.039) following BAS that improved in approximately 1 week. There were no differences in LV global longitudinal strain following BAS. We conclude that BAS was associated with low procedural and clinical adverse event rates in our cohort. The worsening LVIDs, LVPWs, FS, and EF seen immediately after the procedure suggests that BAS causes altered loading conditions affecting LV function in pediatric patients with dilated cardiomyopathy requiring ECMO.

D-Transposition of the great arteries with restrictive foramen ovale in the fetus: the dilemma of predicting the need for postnatal urgent balloon atrial septostomy.

OBJECTIVE: Restrictive foramen ovale (FO) in dextro-transposition of the great arteries (d-TGA) with intact ventricular septum may lead to severe life-threatening hypoxia within the first hours of life, making urgent balloon atrial septostomy (BAS) inevitable. Reliable prenatal prediction of restrictive FO is crucial in these cases. However, current prenatal echocardiographic markers show low predictive value, and prenatal prediction often fails with fatal consequences for a subset of newborns. In this study, we described our experience and aimed to identify reliable predictive markers for BAS. METHODS: We included 45 fetuses with isolated d-TGA that were diagnosed and delivered between 2010 and 2022 in two large German tertiary referral centers. Inclusion criteria were the availability of former prenatal ultrasound reports, of stored echocardiographic videos and still images, which had to be obtained within the last 14 days prior to delivery and that were of sufficient quality for retrospective re-analysis. Cardiac parameters were retrospectively assessed and their predictive value was evaluated. RESULTS: Among the 45 included fetuses with d-TGA, 22 neonates had restrictive FO postnatally and required urgent BAS within the first 24 h of life. In contrast, 23 neonates had normal FO anatomy, but 4 of them unexpectedly showed inadequate interatrial mixing despite their normal FO anatomy, rapidly developed hypoxia and also required urgent BAS ('bad mixer'). Overall, 26 (58%) neonates required urgent BAS, whereas 19 (42%) achieved good O2 saturation and did not undergo urgent BAS. In the former prenatal ultrasound reports, restrictive FO with subsequent urgent BAS was correctly predicted in 11 of 22 cases (50% sensitivity), whereas a normal FO anatomy was correctly predicted in 19 of 23 cases (83% specificity). After current re-analysis of the stored videos and images, we identified three highly significant markers for restrictive FO: a FO diameter < 7 mm (p < 0.01), a fixed (p = 0.035) and a hypermobile (p = 0.014) FO flap. The maximum systolic flow velocities in the pulmonary veins were also significantly increased in restrictive FO (p = 0.021), but no cut-off value to reliably predict restrictive FO could be identified. If the above markers are applied, all 22 cases with restrictive FO and all 23 cases with normal FO anatomy could correctly be predicted (100% positive predictive value). Correct prediction of urgent BAS also succeeded in all 22 cases with restrictive FO (100% PPV), but naturally failed in 4 of the 23 cases with correctly predicted normal FO ('bad mixer') (82.6% negative predictive value). CONCLUSION: Precise assessment of FO size and FO flap motility allows a reliable prenatal prediction of both restrictive and normal FO anatomy postnatally. Prediction of likelihood of urgent BAS also succeeds reliably in all fetuses with restrictive FO, but identification of the small subset of fetuses that also requires urgent BAS despite their normal FO anatomy fails, because the ability of sufficient postnatal interatrial mixing cannot be predicted prenatally. Therefore, all fetuses with prenatally diagnosed d-TGA should always be delivered in a tertiary center with cardiac catheter stand-by, allowing BAS within the first 24 h after birth, regardless of their predicted FO anatomy.

Diagnosis and Management of 2 Cases of Spontaneous Septostomy: Monochorionic/Dichorionic Hybrid Twin Gestation and Dichorionic Triamniotic Triplet Gestation.

INTRODUCTION: Spontaneous septostomy is a rare complication of multiple gestations. Related complications include cord entanglement and preterm delivery. Limited data exist to guide the management of these high-risk patients. The majority of spontaneous septostomy cases have been reported in monochorionic diamniotic twins. We present 2 cases of spontaneous septostomy occurring in a monochorionic/dichorionic hybrid twin gestation (chorionicity transitions from dichorionicity to monochorionicity within the placenta) and in a dichorionic triamniotic triplet gestation. CASE PRESENTATION: Case 1 was a monochorionic/dichorionic hybrid twin gestation with a septostomy complicated by fetal parts of one twin protruding into the co-twin's sac as well as symptomatic polyhydramnios. Fetal magnetic resonance imaging confirmed the septostomy. Case 2 was a dichorionic triamniotic triplet gestation with septostomy and cord entanglement. Both patients were managed akin to a pseudo-monoamniotic gestation with serial ultrasound surveillance and eventual inpatient admission for heightened fetal monitoring. Case 1 underwent elective scheduled cesarean delivery at 33 weeks, and case 2 underwent emergent cesarean delivery for fetal heart rate decelerations at 28 weeks. CONCLUSION: With a high degree of clinical suspicion, spontaneous septostomy can be diagnosed in uncommon settings such as hybrid twin gestations and higher order multiples. Management of such patients is individualized and may include a combination of heightened outpatient and inpatient surveillance.

Cerebroplacental hemodynamics in fetuses with transposition of the great arteries and usefulness in predicting neonatal condition.

OBJECTIVES: Literature on cerebroplacental hemodynamics in fetuses with transposition of the great arteries (TGA) is scarce and provides conflicting results regarding the presence of a brain-sparing effect. The aims of this study were to examine Doppler parameters in the middle cerebral artery (MCA) and umbilical artery (UA) in a large cohort of fetuses with TGA, and to assess their possible utility in predicting the need for urgent balloon atrial septostomy (BAS) in the neonate. METHODS: This was a retrospective observational study of fetuses diagnosed with TGA between 2008 and 2022 and an age-matched cohort of normal fetuses, conducted in a single tertiary fetal cardiology center. Medical records and echocardiographic examinations were reviewed to collect demographic, sonographic and follow-up data. Selected Doppler parameters were compared between fetuses with TGA and normal fetuses, as well as between TGA fetuses with and those without an associated ventricular septal defect (VSD), to assess the impact of this congenital heart defect on cerebroplacental circulation. Additionally, Doppler indices in patients with a restrictive foramen ovale (FO) were analyzed to identify potential predictors of the need for urgent BAS. RESULTS: A total of 541 examinations of 159 fetuses with TGA performed between 19 and 40 weeks' gestation and 1300 examinations of 1215 age-matched normal fetuses were included in the study. MCA pulsatility index (PI) and UA-PI followed expected trends throughout pregnancy, with slightly higher values observed in TGA fetuses, albeit within the limits for the normal population. Cerebroplacental ratio (CPR) values were similar in normal and TGA fetuses. The presence of a small VSD did not have a clinically significant impact on Doppler parameters. Peak systolic velocity (PSV) in the MCA increased gradually after 35 weeks' gestation, especially in fetuses that did not develop restriction of the FO after birth. MCA-PSV values below 1.16 multiples of the median measured at 38 weeks or later predicted the need for urgent BAS with 81.4% sensitivity and 52.4% specificity. CONCLUSIONS: MCA-PI, UA-PI and CPR values in fetuses with TGA usually fall within normal limits throughout pregnancy. The presence of a small VSD does not affect the Doppler parameters significantly. MCA-PSV increases in TGA fetuses after 35 weeks, and its value measured at the last prenatal examination (ideally after 37 weeks) may serve as an additional predictive factor for the need for urgent BAS. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Speckle tracking analysis to evaluate the size, shape, and function of the atrial chambers in fetuses with d-transposition of the great arteries to predict the need for neonatal urgent balloon atrial septostomy.

INTRODUCTION: Speckle tracking analysis was used to evaluate right (RA) and left (LA) atria size, shape and contractility to create a probability calculator to identify fetuses at risk for urgent neonatal balloon atrial septostomy (BAS). METHODS: The study group consisted of 39 fetuses with D-TGA, of which 55% (N = 22) required neonatal BAS and 45% (N = 17) did not. The RA and LA end-diastolic areas, lengths, widths, and sphericity indices as well as global, longitudinal, and transverse contractility were measured with speckle tracking analysis. The z-scores of the measurements were compared to 200 controls. Logistic regression analysis of the computed z-score measurements was performed to separate fetuses requiring urgent neonatal atrial BAS from those who did not. RESULTS: The following z-score values for all fetuses with D-TGA, irrespective of whether they required neonatal BAS, that were significantly less than controls: RA base sphericity index, basal-apical length fractional shortening, fractional area change, lateral wall annular plane systolic excursion (APSE), and longitudinal reservoir strain; LA mid-chamber width, fractional shortening, ejection fraction, basal-apical length fractional shortening, atrial ejection volume, septal wall APSE, and reservoir strain. The following z-score values were significantly larger than control values: RA/LA mid-chamber width, RA/LA base width, and RA mid-chamber length. Logistic regression analysis identified the following five measurements that correctly identified 19 of 22 fetuses requiring urgent neonatal atrial BAS with a sensitivity of 86.4%, a false-positive rate of 11.8% and a positive predictive value of 90.4%: (1) LA mid-chamber transverse fractional shortening, (2) RA mid-chamber end-diastolic width, (3) RA basal-apical length fractional shortening, (4) RA mid-chamber fractional shortening, and (5) RA fractional area change. CONCLUSION: Using the measurements described in this study identified significant differences between all fetuses with D-TGA and controls, as well as identified measurements that predicted the probability of D-TGA fetuses requiring neonatal septostomy.

Neuroendoscopic septostomy in unilateral and bilateral ventricular hydrocephalus.

PURPOSE: This study describes the results of septostomy (SPT) in terms of success and analysis of follow-up in a series of pediatric patients diagnosed with unilateral ventricular hydrocephalus (UHV) and biventricular hydrocephalus (BVH). METHODS: A total of 29 pediatric patients diagnosed with UVH or BVH were included in this study. In UVH, a neuroendoscopic SPT was performed (sometimes accompanied by NEFPFMO). In those diagnosed with BVH, an SPT combined with VPS was carried out. Demographic, etiological, clinical, and diagnostic variables and percentage of treatment success were collected. RESULTS: SPT was successful during follow-up when no VPS was required in UVH and only unilateral VPS was implanted in BVH. At the time of surgery, 16 patients needed a ventriculoperitoneal shunt. The first SPT was successful in 22 patients, requiring a second surgery in 7 patients, from the oncology group diagnosed with BHV. CONCLUSIONS: The surgical management of UVH and BVH still has some disclosure points to be reviewed. However, SPT seems to be a secure, non-traumatic, and efficient procedure.

Timing of Balloon Atrial Septostomy in Patients with d-TGA and Association with Birth Location and Patient Outcomes.

Patients with d-looped transposition of the great arteries (d-TGA), especially those without an adequate atrial septal defect, can experience severe hypoxemia and hemodynamic compromise in the neonatal period. This can be mitigated by urgent balloon atrial septostomy (BAS). However, some patients with d-TGA are born at centers without this capability. The aim of this retrospective study of d-TGA patients who had urgent or emergent BAS at our institution between 2010 and 2021 was to evaluate time from birth to BAS for infants born at a tertiary care center as compared to those requiring transport from other institutions and to examine correlation between time to BAS and patient outcomes. Our primary outcome was time from birth to BAS. Secondary outcomes included hospital and ICU length of stay, mortality, and evidence of pulmonary or neurologic abnormalities including pulmonary hypertension, abnormal neuroimaging, or seizures. Of 96 patients, 67 (70%) were born at our institution. The median time to BAS was 4 h for patients born at our institution vs. 14.1 h for those born elsewhere (p < .0001). A longer time from birth to BAS was associated with longer ICU (r = 0.21, p = 0.046) and hospital length of stay (r = 0.24, p = 0.02) and increased likelihood of elevated right ventricular pressure on post-operative discharge echocardiogram (p = 0.01). There were no differences in mortality between the groups. Therefore, prenatal planning for patients with known d-TGA should include a delivery plan with access to urgent BAS.

Speckle Tracking Analysis in Fetuses with D-Transposition: Predicting the Need for Urgent Neonatal Balloon Atrial Septostomy.

INTRODUCTION: Speckle tracking analysis of the endocardium of the right (RV) and left (LV) ventricles was used to evaluate the size, shape, and contractility of these chambers in fetuses with D-Transposition of the great arteries (D-TGA) to identify fetuses that would require emergent balloon atrial septostomy (BAS) after birth. METHODS: This was a retrospective analysis of fetuses with D-TGA and intact ventricular septum that were divided into 2 groups. Group 1 underwent urgent BAS after birth because of a restrictive atrial septum and group 2 did not. Using speckle tracking analysis, the end-diastolic and end-systolic RV and LV areas, lengths, widths, sphericity indices, and contractility were computed. Logistic regression analysis was performed to identify fetuses who would require urgent neonatal BAS. RESULTS: Of the 39 fetuses with D-TGA, 55% (n = 22) required urgent neonatal BAS (group 1) and 45% (n = 17) (group 2) did not. When comparing D-TGA groups 1 and 2, differences were seen in RV and LV area, sphericity index for segment 1 of the LV, LV fractional area of change and free wall annular plane systolic excursion, fractional shortening for LV segment 12, and RV free wall strain. Regression analysis of these measurements identified 91% of neonates who underwent BAS, with a false-positive rate of 12%. CONCLUSION: Using speckle tracking analysis to evaluate the RV and LV, measurable differences were identified for the RV and LV size, shape, and contractility between fetuses who underwent neonatal urgent BAS vs. those who did not require this procedure.

In vivo study of a reserved atrial septal puncture area patent foramen ovale occluder.

PURPOSE: After patent foramen ovale interventional closure, puncture of the interatrial septum through the occluder is difficult but sometimes needed for further interventional treatment. This paper presents findings from an in vivo experimental study of a reserved atrial septal puncture area patent foramen ovale occluder. MATERIALS AND METHODS: A patent foramen ovale model was established in canines using trans-septal puncture of the fossa ovale and high-pressure balloon dilation. Then, patent foramen ovale closure was performed with a reserved atrial septal puncture area and all canines were raised for 3 months. Then, the occluder was crossed and left atrial angiography was performed on the septal area with the occluder. Finally, DSA angiography, echocardiography, and histology were used to evaluate the performance and feasibility of the reserved atrial septal puncture area. RESULTS: A patent foramen ovale model was successfully established in 10 canines using the atrial septal puncture method. The average diameter of the patent foramen ovale was 3.77 ±0.19 mm, and the patent foramen ovale was successfully closed in all canines using a reserved atrial septal puncture area. As assessed using transoesophageal echocardiography, the new occluder exhibited an ideal position and was occluded entirely without a residual shunt intraoperatively and postoperatively. A 100% success rate of atrial septum puncture was achieved across the new occluder. The occluders were completely endothelialised 3 months post-implantation. CONCLUSIONS: The reserved atrial septal puncture area was effective in patent foramen ovale closure and exhibited positive sealing performance and biological compatibility. Trans-septal puncture was feasible and effective after reserved atrial septal puncture area patent foramen ovale closure.

Out-of-hours versus in-hours delivery of antenatally diagnosed transposition of the great arteries: outcomes from a United Kingdom Tertiary Centre.

OBJECTIVE: To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries. SETTING: Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit. PATIENTS: Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00-08:00 and weekends) versus in-hours between 2015 and 2020. OUTCOME: The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy. RESULTS: Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42-32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03-0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37-10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00-23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003). CONCLUSION: Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.

Improvised bespoke technique for atrial septostomy in the shortage of atrioseptostomy balloon catheters.

Atrioseptostomy balloon catheter is an essential item to have on our shelves. However, the recall and shortage in production of the commonly used balloon atrioseptostomy catheters posed an imminent threat to our patients. Herein, we present the case of a newborn with a post-natal diagnosis of simple transposition of great arteries and restrictive atrial communication where repeated static balloon atrial septostomy using a 9 mm x 20 mm Sterling balloon failed to improve his status. We had to improvise per-operatively a new bespoke technique to perform a vital pull-through balloon atrial septostomy. The distal third of a 10 mm x 20 mm semi-compliant Cristal balloon was exteriorised out the tip of a 6-Fr 55 cm Cook Flexor sheath in the left atrium, and both were simultaneously pulled back to the right atrium to create an 8 mm septal defect. The procedure was successful without any complications. The baby was weaned off prostaglandin on day 3 and surgically repaired on day 5 with excellent results.

Idiopathic membranous occlusion of the foramen of Monro: an unusual cause of hydrocephalus and headache in adults.

Occlusion of foramen of Monro is an uncommon clinical entity that usually presents in children. Common causes are obstructing mass, infectious etiologies or vascular malformation. Rarely, it may be an idiopathic stricture or membrane. We report a case of idiopathic membranous obstruction of the foramen of Monro in a 45-year-old male with no past medical or surgical history. He presented with new intermittent dull and burning bifrontal severe headache for 2 d, which was alleviated slightly by non-steroidal anti-inflammatory medication. Imaging showed marked dilation of the lateral ventricles with normal third and fourth ventricles. The patient was discharged initially with conservative medical management and close follows up; however, the headache continued to progress and neurosurgical intervention was offered. The patient underwent endoscopic exploration, fenestration of the septum pellucidum, and right ventriculoperitoneal shunt placement. Bilateral membranous obstruction of foramina of Monro and an auto-fenestrated cavum septum pellucidum were identified intraoperatively. The patient reported resolution of headache post-operatively without recurrence on 1-month follow up. This case is unusual in that the patient presented without any known neurologic history or prior intracranial infections. It became apparent at the time of surgery that chronic obstruction of the bilateral foramina with collapse of the third ventricle had developed, and the safest durable treatment for him was septostomy and cerebrospinal fluid (CSF) shunt placement.

Balloon atrial septostomy versus left atrial cannulation for left heart decompression in children with dilated cardiomyopathy and myocarditis on extracorporeal membrane oxygenation: An ELSO registry analysis.

INTRODUCTION: In children with myocarditis or dilated cardiomyopathy (DCM) on extracorporeal membrane oxygenation (ECMO) for cardiogenic shock, it is often necessary to decompress the left heart to minimize distension and promote myocardial recovery. We compare outcomes in those who underwent balloon atrial septostomy (BAS) versus direct left atrial (LA) drainage for left heart decompression in this population. METHODS: Retrospective study of the Extracorporeal Life Support Organization (ELSO) multicenter registry of patients ≤ 18 years with myocarditis or DCM on ECMO who underwent LA decompression. Descriptive and univariate statistics assessed association of patient factors with decompression type. Multivariable logistic regression sought independent associations with outcomes. RESULTS: 369 pediatric ECMO runs were identified. 52% myocarditis, 48% DCM, overall survival 74%. 65% underwent BAS and 35% LA drainage. Patient demographics including age, weight, gender, race/ethnicity, diagnosis, pre-ECMO pH, mean airway pressure, and arrest status were similar. 89% in the BAS group were peripherally cannulated onto ECMO, versus 3% in the LA drainage group (p < .001). On multivariable analysis, LA drainage (OR 3.96; 95% CI, 1.47-10.711; p = .007), renal complication (OR 2.37; 95% CI, 1.41-4.01; p = .001), cardiac complication (OR 3.14; 95% CI, 1.70-5.82; p < .001), and non-white race/ethnicity (OR 1.75; 95% CI, 1.04-2.94; p = .035) were associated with greater odds of mortality. There was a trend toward more episodes of pulmonary hemorrhage in BAS (n = 17) versus LA drainage group (n = 3), p = .08. Comparing only those with central cannulation, LA drainage group was more likely to be discontinued from ECMO due to recovery (72%) versus the BAS group (48%), p = .032. CONCLUSIONS: In children with myocarditis or DCM, there was a three times greater likelihood for mortality with LA drainage versus BAS for LA decompression. When adjusted for central cannulation groups only, there was better recovery in the LA drainage group and no difference in mortality. Further prospective evaluation is warranted.

Practice and Safety of Static Balloon Atrial Septostomy Based on a Nationwide Registry Data.

BACKGROUND: Balloon atrial septostomy (BAS) is an essential catheterization procedure for congenital heart lesions. Recently, a balloon catheter for static BAS was approved for the first time in Japan as an alternative to the conventional pull-through BAS. Despite the expected increase in the use of static BAS, reports on its safety are scarce worldwide.Methods and Results: Data on static and pull-through BAS registered in a national registry between 2016 and 2018 were collected. During the study period, 247 sessions of static BAS and 588 sessions of pull-through BAS were performed on a total of 674 patients. Patients who underwent static BAS were older (P<0.001). The incidence of serious adverse events (4.3% vs. 0.9%, P=0.03) and the overall incidence of adverse events (8.1% vs. 3.2%, P=0.03) were higher in static BAS than in pull-through BAS. Among patients who underwent static BAS, the risk factor for adverse events was a body weight <3 kg at the time of the procedure (odds ratio: 4.3 [confidence interval: 1.7-11], P=0.003). CONCLUSIONS: This nationwide study revealed differences in patient background between static and pull-through BAS, as well as a higher incidence of adverse events related to static BAS. Patients weighing <3 kg are at high risk for adverse events after static BAS and may require surgical and circulatory support backup.

Iatrogenic chorioamniotic separation and septostomy following fetoscopic laser photocoagulation for twin-twin transfusion syndrome.

OBJECTIVE: To compare the perinatal outcomes of pregnancies complicated by chorioamniotic separation (CAS) vs septostomy following fetoscopic laser photocoagulation (FLP) for twin-twin transfusion syndrome (TTTS). METHODS: This was a retrospective cohort analysis of monochorionic diamniotic twin pregnancies with TTTS that underwent FLP at one of two university-affiliated tertiary medical centers between January 2012 and December 2020. CAS and septostomy were diagnosed either during the procedure or by ultrasonography within 24-48 h after FLP. Data on procedure and postprocedure parameters, pregnancy outcomes and survival were collected from the patients' electronic medical records. Pregnancies were stratified according to the presence of CAS, septostomy or neither. Patients diagnosed with both CAS and septostomy were analyzed separately. RESULTS: Of the 522 women included in the cohort, 38 (7.3%) were diagnosed with CAS, 68 (13.0%) with septostomy and 23 (4.4%) with both CAS and septostomy. The remaining 393 (75.3%) women comprised the control group. Groups did not differ in demographic characteristics. The septostomy group had a lower rate of selective fetal growth restriction than did the CAS and control groups (24.2% vs 36.8% vs 42.7%, respectively; P = 0.017). Moreover, intertwin size discordance was lower in the septostomy group (15.1% vs 23.4% in the CAS group and 25.5% in the control group; P = 0.001). Median gestational age at FLP was significantly lower in the CAS group (19.3 weeks vs 20.4 weeks in controls and 20.9 weeks in the septostomy group; P = 0.049). The rate of delivery prior to 34 weeks was significantly higher in the CAS group (89.2%), followed by the septostomy group (80.9%), compared with the control group (69.0%) (P = 0.006). A secondary analysis demonstrated that patients with both CAS and septostomy presented the highest rates of delivery prior to 34 weeks (100%) and 32 weeks (68.2%). CONCLUSIONS: CAS and septostomy following laser surgery for TTTS are independently associated with higher rates of preterm delivery. The presence of these two findings in the same patient enhances the risk of prematurity. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Atrial septoplasty through internal jugular venous access in an infant with transposition of great arteries: technical challenges and solutions.

We describe a 3-month-old infant with transposition of great arteries and restrictive foramen ovale who presented with severe cyanosis. Child had a large thrombus causing near-total occlusion of the inferior caval vein. An emergency atrial septoplasty was performed via internal jugular venous access. The case emphasises the technical challenges faced while performing this procedure through jugular approach and plausible solutions to overcome these challenges.

Balloon atrial septostomy through azygos vein in two cases of D-TGA with left atrial isomerism.

Balloon atrial septostomy is a palliative procedure that is performed in D-transposition of great arteries when surgery is not immediately available. Although D-TGA and left isomerism association are rare, it is an important condition as the BAS procedure approach is unique. In this case report, we present two cases of D-TGA with left isomerism where BAS was performed due to restrictive atrial septal defect and lack of immediate availability of the paediatric cardiac surgeon.