Restrictive Interatrial Communication Research Articles

Virtual reality modelling for planning of percutaneous first step palliation in a newborn with heterotaxy syndrome.

We report the first-stage percutaneous palliation in a newborn with a rare heterotaxy syndrome variant including interrupted inferior vena cava, partial anomalous pulmonary venous drainage, and restrictive interatrial communication. Virtual reality imaging aided visualisation, decision-making, and planning. Successful atrial septoplasty performed via the internal jugular vein and anomalous pulmonary vein was followed by stenting of ductus arteriosus.

Evolving Approach in Hypoplastic Left Heart Syndrome With Restrictive and Intact Septum.

Background: Hypoplastic left heart syndrome (HLHS) with either intact atrial septum (IS) or highly restrictive interatrial communication (HRIC) is associated with poor survival. Immediate postpartum access to cardiac therapy and timely left atrial decompression (LAD) are paramount to a successful outcome. We describe herein our evolving approach to LAD and report interstage and longer-term results. Methods: We retrospectively identified neonates with HLHS IS/HRIC requiring LAD between 2005 and 2019. All babies had prenatal/postnatal echocardiography. Our LAD strategy evolved over time from attempt at transcatheter balloon atrial septostomy (BAS) to surgical septectomy with inflow occlusion, to hybrid trans-atrial stent implantation. Results: Twelve neonates required LAD at a median time of 14 (0.5-31) hours after birth. Five patients underwent BAS that proved successful in 2 cases. Of the 3 unsuccessful cases, 2 required extra-corporeal membrane oxygenation (ECMO) support and died subsequently; one underwent hybrid trans-atrial stent implantation. Of the remaining 7 patients, 3 underwent surgical septectomy with inflow-occlusion and 4 underwent hybrid trans-atrial stent implantations. Overall, 8 patients survived LAD and reached Norwood palliation. Three of the 8 required ECMO postoperatively. There was no hospital mortality after Norwood stage 1 palliation and interstage survival was 100%. Six patients successfully underwent Glenn shunt (superior cavopulmonary anastomosis) and 5 have completed total cavopulmonary connection. Conclusions: Our experience suggests that prompt postnatal LAD can be safely achieved with careful multidisciplinary planning and accurate antenatal diagnosis. In our hands, hybrid trans-atrial septal stent insertion appears to be a safe approach which combines the versatility of transcatheter techniques together with the effectiveness of surgical control.

Ближайшие и отдаленные результаты временной баллонной окклюзии общих подвздошных артерий при врастании плаценты

Objective. To study immediate and long-term results of endovascular reduction of blood flow in placental ingrowth. Patients and methods. The study included 70 pregnant women with ingrowth of the placenta into the uterine scar. The gestational age at time of delivery ranged from 30 to 40 weeks, averaging 35.8 ± 2.9 weeks. In all cases, the reduction of blood flow was performed using balloon catheters placed in the common iliac arteries. Admiral (Invatec) peripheral balloon catheters were used in 55 patients; in 15 patients – balloon catheters used in pediatric practice for dilatation of restrictive interatrial communication (Rashkind balloon). Results. There were no fatal outcomes. Endovascular complications were observed in 4 (5.7%) parturient women. In the longterm period the results were studied in 31 patients. In 22 (71%) patients menstrual function normalized within the first 3 months after the end of lactation. Recovery of menstrual function occurred in 3 (9.7%) women between 3 and 6 months, 2 (6.5%) cases each were in the subgroups with menstrual function recovery between 6 and 9 months and more than 9 months. Menstrual function was not recovered in 2 (6.5%) patients. Conclusion. Temporary balloon occlusion of the common iliac arteries significantly reduces the volume of blood loss, provides better visualization of the surgical area, and creates favorable conditions for subsequent pregnancy. Key words: balloon catheter, placental ingrowth, temporary occlusion of common iliac arteries, bleeding

Maternal hyperoxygenation in congenital heart disease.

The importance of prenatal diagnosis and fetal intervention has been increasing as a preventative strategy for improving the morbidity and mortality in congenital heart disease (CHD). The advancements in medical imaging technology have greatly enhanced our understanding of disease progression, assessment, and impact in those with CHD. In particular, there has been a growing focus on improving the morbidity and mortality of fetuses diagnosed with left-sided lesions. The disruption of fetal hemodynamics resulting from poor structural developmental of the left outflow tract during cardiogenesis is considered a major factor in the progressive lethal underdevelopment of the left ventricle (LV). This positive feedback cycle of inadequate flow and underdevelopment of the LV leads to a disrupted fetal circulation, which has been described to impact fetal brain growth where systemic outflow is poor and, in some cases, the fetal lungs in the setting of a restrictive interatrial communication. For the past decade, maternal hyperoxygenation (MH) has been investigated as a diagnostic tool to assess the pulmonary vasculature and a therapeutic agent to improve the development of the heart and brain in fetuses with CHD with a focus on left-sided cardiac defects. This review discusses the findings of these studies as well as the utility of acute and chronic administration of MH in CHD.

Prevalence and Outcomes of Balloon Atrial Septostomy in Neonates With Transposition of Great Arteries.

Transposition of the great arteries is the most common cyanotic congenital heart defect. Surgical correction usually occurs in the first week of life; presence of restrictive interatrial communication and severe hypoxemia warrants urgent intervention with balloon atrial septostomy and medical stabilization prior to surgery. The main objective of this study is to compare the characteristics, outcomes, and mortality risks in patients with transposition of the great arteries who underwent balloon atrial septostomy during their hospitalization versus transposition of the great arteries patients who have not undergone this procedure. Retrospective analysis of administrative data. Data from Kids' Inpatient Database complemented with the National Inpatient Sample dataset for the years 1998-2014, this includes data from participating hospitals in 47 U.S. States and the District of Columbia. Neonates admitted with transposition of the great arteries. None. A total of 17,392 neonates with diagnosis of transposition of the great arteries were captured in the databases we used. Male-to-female ratio was 2:1. The rate of balloon atrial septostomy in patients with transposition of the great arteries was 27.7% without significant change over the years. There was no significant difference in mortality between balloon atrial septostomy and no balloon atrial septostomy (6.3% vs 6.7%; p = 0.29). Neonates with balloon atrial septostomy had a two-fold increase in their length of stay compared with no balloon atrial septostomy (16 d vs 7 d; p < 0.0001). Stroke was present in 1.1% of balloon atrial septostomy group versus 0.6% in those who did not have balloon atrial septostomy (odds ratio, 1.85; 95% CI, 1.29-2.65; p < 0.0001). Extracorporeal membrane oxygenation was used more in balloon atrial septostomy group (5.1% vs 3.1%; p < 0.0001). There was no difference in mortality rate between balloon atrial septostomy and no balloon atrial septostomy patients. The prevalence of the diagnosis of stroke in this study was higher in patients who underwent balloon atrial septostomy. Furthermore, comparison of in-hospital mortality in balloon atrial septostomy and no balloon atrial septostomy revealed increased mortality risk in no balloon atrial septostomy patients transferred from other institution, no balloon atrial septostomy patients supported with extracorporeal membrane oxygenation, and balloon atrial septostomy patients diagnosed with stroke. Finally, length of stay and charges were higher in balloon atrial septostomy patients.

Creation of a restrictive atrial left-to-right shunt: a novel treatment for heart failure

Elevated left atrial (LA) filling pressures are associated with poor prognosis in patients with heart failure (HF). Recent evidences have shown that, in this setting, the presence of restrictive small atrial defect may protect from an extreme increase in LA pressure and prevent consecutive pulmonary congestion and acute decompensation, without the expense of an unfavourable significant left-to-right shunt. Therefore, decompression of LA by creating a unidirectional but restrictive left-to-right interatrial shunt has been emerging as a new therapeutic strategy in patients suffering from both HF with preserved and reduced ejection fraction. Two dedicated devices (V-Wave and interatrial septal device system-IASD) are currently available to create a restrictive interatrial communication. Several clinical studies have demonstrated the safety of this approach, with encouraging results regarding the improvements in haemodynamic parameters, functional status and quality of life in treated patients. We aim to summarize the present knowledge concerning this novel therapeutic approach for HF, describe the currently available devices and depict their potential future clinical indications.

Left ventricular regression after balloon atrial septostomy in d-transposition of the great arteries.

Balloon atrial septostomy (BAS) is an effective palliative procedure in children with transposition of the great arteries and poor intercirculatory mixing. While the subpulmonary left ventricle (LV) is known to regress with time in these newborns due to the declining afterload, it has not been studied how it behaves following BAS and a consequent decrease in preload. The study was designed to examine the effects of BAS on the LV in infants with simple d-transposition of the great arteries by serial 2D echocardiographic monitoring. This was a prospective echocardiographic follow-up study of all consecutive children with simple d-transposition of the great arteries who underwent BAS for restrictive interatrial communication and oxygen saturation below 75% between January 2014 and June 2015. Left ventricular mass estimation was done by M-mode transthoracic echocardiography before balloon septostomy and serially on follow-up till surgery. The median age of the 25 children studied was 4 days (1-95 days) when they underwent BAS. Twenty patients underwent arterial switch operation at a mean of 9 days from balloon septostomy. The mean baseline left ventricular mass was 47.9 g/m2, which decreased to 38.5, 36.2, 32.1, 32.4, 25.7 and 25.2 g/m2 on Days 1, 3, 6, 9, 12 and 15, respectively. The left ventricular mass decreased by 1.5 g/m2 every day during the first 2 weeks following balloon septostomy adjusted for the age of the child in days. Children who underwent BAS beyond 3 weeks of life had faster LV regression than those who underwent the procedure earlier (unstandardized regression coefficient β 0.892, P < 0.001). BAS is associated with accelerated regression of the LV in infants with simple d-transposition of the great arteries in the first 2 weeks after the procedure. Regression of the LV is faster in children who underwent BAS after 3 weeks of age.

140 Evolving Technical Approach and Results in Hypoplastic Left Heart Syndrome with Intact or Highly Restrictive Atrial Septum

Background We describe our current unit approach and report interstage results in the HLHS with Intact atrial septum (IAS) or highly restrictive interatrial communication (HRIC). Method A retrospective review of the institutional HLHS programme (2005–2014). Advances in fetal diagnosis lead to delivery planning in hybrid theatre: median sternotomy and interventional defect creation/enlargement. Results 9 neonates (4 IAS and 5 HRIC) and 5 required immediate intervention postnatally. Delayed/insufficient septostomy in 3 HRIC resulted in death before stage I Norwood even with ECMO support in 2/3. Of the other 6, 2 had trans-atrial stent placement and 1 trans-atrial balloon septostomy (3 had surgical septectomy). 5/6 underwent concomitant bilateral pulmonary artery banding. All the 6 patients reached the Norwood procedure after 27 ± 21 days and 50% required ECMO postoperatively. Trans-atrial stenting resulted in less pre Norwood morbidities. There was no stent-related complications. There was no hospital mortality after Norwood and current inter-stage survival is 100%: 5 patients underwent successfully second-stage palliation, 1 of them had the Fontan completion and subsequently transplanted. Conclusion Together with advanced fetal diagnosis, effective left atrial decompression especially with trans-atrial stunting using hybrid technique and availability of mechanical support can improve the outcome of HLHS/IAS/HRIC with low interstage morbidity and excellent survival.

Left ventricular obstruction with restrictive inter-atrial communication leads to retardation in fetal lung maturation.

Intact atrial septum or highly restrictive inter-atrial communication (I/HRAS) combined with either severe aortic stenosis (SAS) or hypoplastic left heart syndrome (HLHS), respectively, is associated with adverse outcome. This study focusses on changes in alveolo-septal lung parenchyma due to increased left atrial pressure. In a retrospective cross-sectional autoptic study, we investigated fetal/neonatal lung specimens of 18 patients with SAS/HLHS with I/HRAS, 11 patients with SAS/HLHS and unrestrictive inter-atrial communications and 18 controls. Pulmonary maturation was investigated by means of morphometric and immunohistochemical analyses. In a comparison of all three groups, alveolo-capillary membrane maturation was significantly disturbed in I/HRAS fetuses from week 23 of pregnancy on. I/HRAS lungs showed angiomatoid hyper-capillarisation and significantly wider inter-airspace mesenchyme. Differences in width ranged between 34.58 µm (95% CI: 11.41-57.75 µm) and 46.74 µm (95% CI: 13.97-79.50 µm) in the second and third trimesters. In I/HRAS infants with HLHS, inter-airspace mesenchymal diameters steadily normalised with age; however, significant fibroelastosis of alveolar septae developed. Fetal lung maturation with respect to alveolo-capillary membrane formation is severely disordered in patients with SAS/HLHS with I/HRAS. Our findings indicate that, from a morphological point of view, timing of fetal invention in fetuses with I/HRAS should be fixed within the second trimester of pregnancy.

Impact of novel balloon catheter on static balloon atrial septostomy with double balloon technique in infants with congenital heart disease.

The aim of this study was to investigate the feasibility of static balloon atrial septostomy (BAS) with the double balloon technique for infants. TMP PED balloon catheter, newly designed for static BAS in small children, was used in 3 infants. The balloon catheter has a low profile, short and round shoulder, and smooth deflation without slippage. Three infants (transposition of the great arteries, n=2; pulmonary atresia with intact ventricular septum, n=1) underwent static BAS with double balloon for restrictive interatrial communication between December 2014 and March 2015. Hemodynamic and echocardiographic assessment was done before and after the procedure. Pressure gradient between left and right atrium decreased from 6, 7 and 9 mmHg to 2, 2 and 1 mmHg, respectively. Oxygen saturation in systemic artery increased from 72, 68 and 73% to 78, 70 and 79%, respectively. Maximum defect diameter increased from 3.5, 3.0 and 3.3 mm to 6.6×5.2, 9.0×6.2 and 8.1×5.1 mm, respectively. No complication was recorded. Static BAS with double balloon technique using the novel TMP PED balloon catheter was safe and effective in producing sufficient interatrial communication for 8-20 weeks in infants. Static BAS is a promising procedure to create interatrial communication in infants.

Fetal Pulmonary Venous Flow and Restrictive Foramen Ovale in Hypoplastic Left Heart

Hypoplastic left heart (HLH) with intact or restrictive interatrial communication (HLH-IAS/RAS) is associated with high mortality rates. The object was to correlate pulmonary venous (PV) Doppler spectra and direct foramen ovale (FO) assessment with the neonatal need for early atrial septostomy (EAS) and neonatal outcome. We reviewed all prenatal echocardiograms and outcomes of 51 fetuses with HLH and information about the interatrial communication between 1994 - 2011. IAS/RAS was defined as a small/absent interatrial shunt on 2-dimensional imaging. Three PV Doppler spectra were observed: type A: continuous forward flow with a small a wave reversal; type B: continuous forward flow with increased a-wave reversal; type C: brief to-and-fro flow. Three of 51 neonates with type C pulmonary venous flow pattern and suspicion of IAS/RAS on 2-dimensional (2-DE) evaluation required EAS. In one fetus pulmonary venous flow changed from type B to type C spectra throughout gestation. Fetuses with type C spectra showed a 71.4 % survival after 30 days compared to 92.3 % in fetuses with type A spectra. Short term survival after EAS was 33 %. Prenatal PV flow pattern and 2-DE of the FO size help in identifying the fetus at risk for neonatal EAS and patient selection for fetal cardiac intervention. Most late second trimester values will not change over time.

Right Ventricle-to-Pulmonary Artery Shunt in Norwood Procedure: Early Results

The aim of this study was to evaluate experience and predictors of early mortality in patients with hypoplastic left heart syndrome (HLHS)-type defects undergoing Norwood procedure (NP) with right ventricle-to-pulmonary artery (RV-PA) shunt. Between 2001 and 2009, a consecutive series of 229 children with HLHS-type single ventricle underwent NP with application of RV-PA shunt. Demographic, echocardiographic, and clinical perioperative data were retrospectively analyzed. The mean duration of follow-up of survivors was 4.5 ± 2.1 years (60 days to 8.1 years). Follow-up was complete for 92.1% of patients. Major early postoperative complications included sepsis/generalized infection in 40 (17.5%), pericardial effusion in 9 (3.9%), and wound infection in 8 (3.5%). The early (30-day) survival was 87.8% (n = 201). In the late postoperative period, 12 (5.9%) died. Early nonsurvivors were more frequently older than 14 days (P = .045) at initial surgery, had lower operative weight (P = .024), had more frequent associated cardiac (P < .001) and/or extracardiac anomalies (P < .001), and were more likely to have a restrictive interatrial communication before operation (P = .024). Use of the right RV-PA shunt has helped to mitigate some previously described predictors of early death after NP. Longer follow-up will be required to determine whether the RV-PA shunt modification confers an important survival benefit.

Intralimbic Hematoma Formation within an Atrial Septal Aneurysm in a Neonate with Hypoplastic Left Heart Syndrome

Atrial septal restriction is estimated to occur in up to 22% of patients with hypoplastic left heart syndrome and is associated with poor prognosis postnatally. Atrial septal aneurysms are known to be more prevalent in patients with congenital heart disease and atrial septal restriction due to elevated atrial pressures in fetal life. We report the case of a newborn with hypoplastic left heart syndrome, restrictive interatrial communication, and atrial septal aneurysm. The latter led to formation of a large intralimbic hematoma within it, which resulted in complete obstruction of the interatrial communication after birth, which led to death. We suggest that in addition to the known associated problems encountered in patients with hypoplastic left heart syndrome and restrictive interatrial communication, aneurysmal formation of the interatrial septum should alert the fetal and postnatal echocardiographer further, and careful assessment and follow-up is recommended.

Levoatrial cardinal vein in mitral atresia and closed foramen ovale: prenatal diagnosis and perinatal management

A levoatrial cardinal vein is a rare cardiovascular anomaly that may be present in malformed hearts with severe left heart obstruction and restrictive interatrial communication. We report the prenatal diagnosis at 23 weeks of a fetus with mitral atresia, double-outlet right ventricle, premature closure of the foramen ovale and a levoatrial cardinal vein draining into the innominate vein. In a prior examination performed elsewhere the levoatrial cardinal vein had been interpreted as an aortic arch perfused retrogradely, and hypoplastic left heart syndrome with aortic atresia had been diagnosed. Prenatal management, induction at 38 weeks and postnatal examinations and treatment are reported. To the best of our knowledge, this is the first reported prenatal diagnosis of this embryological vessel, presenting a potential pitfall for prenatal echocardiography.

Fetal intervention for cardiac disease: The cutting edge of perinatal care

Fetal cardiac valvuloplasty has been proposed for progressive cardiac disease with a poor prognosis, such as critical aortic stenosis and pulmonary atresia with intact ventricular septum and balloon atrial septostomy for hypoplastic left heart syndrome, or simple transposition of the great arteries with closed or restrictive inter-atrial communication. It is anticipated that early rescue of ventricles or the pulmonary veins from an unfavourable environment may promote healthier ventricular and vascular growth and improve postnatal outcomes. While close collaboration between the fetal medicine specialist and perinatal cardiologist may optimize the chances of technical success, obstacles to progress include the relative rarity of suitable cases and late referral for therapy. In common with other interventions in fetal medicine, there is a learning curve, and it would benefit progress if the procedures were initially concentrated in just a few centres to enable them to develop skills and experience. Following careful evaluation, it may then be desirable to train further centres and roll out best practice models.

Abstracts for the British Congenital Cardiac Association Annual Meeting: The Barbican, London, 24–25 November 2005: Poster Presentations: Assessment of the need for elective balloon atrial septostomy in patients with tricuspid atresia. a 25 year experience at the Yorkshire Heart Centre

Introduction: Significant variation exists in the perceived indications for balloon atrial septostomy in children with tricuspid atresia (TA). Many units advocate early, elective balloon atrial septostomy due to potential progressive narrowing of the interatrial opening, especially after the insertion of an arterial shunt. Our practice is to perform an atrial septostomy only in children with clinical and/or echocardiographic features of a restrictive interatrial communication. The aim of the study was to assess our practice by retrospective evaluation of patients born with tricuspid atresia with particular reference to the clinical fate of the atrial septum. More specifically the frequency of emergency atrial septostomy, the complication rate, the need for delayed septostomy or surgical resection and the longer term outcome were evaluated. Materials and methods: The study was a retrospective review (1980–2005) of the data on 49 live-born patients in our departmental database with a diagnosis of TA. One patient was excluded from the study due to referral to another centre and subsequent loss to follow up. By reviewing the medical notes of the remaining 48 patients we collected data on indications for, and timing of balloon atrial septostomy and subsequent complications and long term outcome. Results: The median time to follow up was 9.5 years (0.7–23.7). The diagnosis group was heterogeneous with 38 (79%) patients diagnosed with TA and normally related great arteries with or without pulmonary stenosis or atresia. The remaining 10 patients had more complex anatomy. Of the 48 patients, 5 (10%) were deemed to require balloon atrial septostomy for a clinically and/or echocardiographically restrictive atrial septum. Three procedures were performed within the first 24 hrs of life and 2 within the first month. One of the patients developed NEC following the septostomy and subsequently died of overwhelming sepsis. One patient required delayed surgical septectomy at 6 years of age, during a bidirectional Glenn operation. Of the remaining 42 patients there were 7 deaths (16%) but none were attributed to a restrictive atrial septum (1–at birth, severe acidosis and multi organ failure due to interrupted aortic arch and poor response to IV Prostin; 1–5 days with Group B streptococcal sepsis; 1–post operative sepsis following insertion of a BT shunt; 1–9 years, acute shunt obstruction; 1–withdrawal of care due to complex anatomy deemed to have a poor prognosis; 2-sudden death at home at 3½ years and 11 years). 26 patients required palliation with an arterial shunt (19–modified BT shunt, 6–Waterston Shunt, 1–ductal stent). None of the patients developed a clinically and/or echocardiographically restrictive atrial septum post procedure or during long term follow-up. Conclusions: We conclude that elective balloon atrial septostomy at presentation may not be necessary in patients born with tricuspid atresia. Accepting the limitations of retrospective data collection we also conclude that progressive narrowing of the interatrial septum at a later date is an unusual occurrence, even after an arterial shunt operation. We recommend that atrial septostomy should only be performed in patients who show obvious clinical and/or echocardiographic evidence of restrictive interatrial communication.

Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle

Pulmonary venous Doppler (PVD) flow patterns in the fetus with hypoplastic left heart syndrome (HLHS) have been correlated with restrictive interatrial communication or intact atrial septum (RAS) postnatally; however, the ability of PVD to identify the neonate requiring emergent atrial septoplasty (EAS) for severe left atrial hypertension and hypoxemia has not been critically evaluated. It was the purpose of this study to determine the predictive power of fetal PVD in identifying the need for EAS in newborns with HLHS and RAS. Forty-one patients with fetal PVD flow analysis and postnatally confirmed HLHS were studied. Pulsed-wave assessment of PVD flow included S-, D-, and A-wave velocity, time-velocity integral (VTI) of forward and reverse flow, and S/D velocity and forward/reverse VTI ratio. Neonatal EAS was used as the primary clinical outcome variable. Receiver operating characteristic curves were used to determine cutpoints at which PVD indices best predicted EAS. Cutpoints were evaluated for clinical accuracy and usefulness by use of Bayesian analysis. Eight of 41 subjects underwent EAS. Need for EAS was most accurately predicted by forward/reverse VTI ratio <5 (sensitivity, 0.88, 95% CI, 0.49 to 0.99; specificity, 0.97, 95% CI, 0.82 to 0.99), which, when present, increases the posttest likelihood of EAS to 74%, assuming a pretest prevalence of 10%. Accuracy and usefulness of other PVD indices were affected by false-positive results. In the fetus with HLHS, a PVD forward/reverse VTI ratio of <5 is the strongest predictor of the need for EAS in the newborn period. These observations should improve our ability to identify and expectantly manage the fetus with HLHS and RAS.

Restrictive interatrial communication in hypoplastic left heart syndrome after modified Fontan repair

The occurrence of pulmonary venous obstruction after total cavopulmonary connection with intraatrial lateral tunnel is a rare occurrence. We present two cases of hypoplastic left heart syndrome with restrictive interatrial communication presenting late after this type of modified Fontan repair. This occurred even after complete excision of the atrial septum at the time of Stage 1 Norwood in both cases. A novel approach to this problem of resecting the roof of the coronary sinus was utilized to enlarge the interatrial communication.

Scoring system to determine need for balloon atrial septostomy for restrictive interatrial communication in infants with hypoplastic left heart syndrome

Background Restrictive interatrial communication (IAC) causes morbidity and mortality in infants with hypoplastic left heart syndrome awaiting cardiac transplantation. We sought to create a scoring system, based on echocardiographic and clinical findings, to serve as a guide for determining the need for balloon atrial septostomy (BAS). Methods We retrospectively reviewed echocardiograms of 44 infants with hypoplastic left heart syndrome. Infants were studied from the time of admission to the final end-point of transplantation, Norwood procedure, or death. Seventeen infants underwent BAS for clinical indications of oxygen saturation <80% in room air. Data collected included age at BAS, maximum velocity (V max), and IAC diameter throughout the clinical course. We assigned higher IAC scores to smaller IAC diameter, greater V max through the IAC, and lower oxygen saturation value. The minimum score was 3, and the maximum score was 9. Results Only 10% of infants with a score <6 at presentation required BAS, whereas 67% of those with scores ≥6 required BAS. Higher IAC scores at presentation were associated with earlier need for BAS ( p = 0.04). Conclusions The IAC scoring system can serve as a reliable clinical guide for identifying infants with hypoplastic left heart syndrome who are likely to require BAS for relief of critically restrictive IAC while awaiting cardiac transplantation.

Anastomosis between the left atrial appendage and left superior vena cava in a patient with mitral atresia.

A 5-day-old boy with mitral atresia and restrictive interatrial communication underwent an anastomosis between the left atrial appendage and left superior vena cava draining to the right atrium after unsuccessful balloon atrial septostomy. Consequent reduction of left atrial pressure improved the child's clinical condition, enabling palliative surgery. This procedure may be done safely without cardiopulmonary bypass in a seriously ill patient with combined elevated left atrial pressure and persistent left superior vena cava.