Restrictive Atrial Septum Research Articles

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia

Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

Fetal cardiac interventions: an update of therapeutic options.

Objective This article aims to present updated therapeutic options for fetal congenital heart diseases. Methods Data source for the present study was based on comprehensive literature retrieval on fetal cardiac interventions in terms of indications, technical approaches and clinical outcomes. Results About 5% of fetal congenital heart diseases are critical and timely intrauterine intervention may alleviate heart function. Candidates for fetal cardiac interventions are limited. These candidates may include critical aortic valve stenosis with evolving hypoplastic left heart syndrome, pulmonary atresia with an intact ventricular septum and evolving hypoplastic right heart syndrome, and hypoplastic left heart syndrome with an intact or highly restrictive atrial septum as well as fetal heart block. The advocated option are prenatal aortic valvuloplasty, pulmonary valvuloplasty, creation of atrial communication and fetal cardiac pacing. Conclusion Fetal cardiac interventions are feasible at midgestation with gradually improved technical success and fetal/postnatal survival due mainly to a well-trained multidisciplinary team, sophisticated equipment and better postnatal care.

Optimization of Preoperative Status in Hypoplastic Left Heart Syndrome With Intact Atrial Septum by Left Atrial Decompression and Bilateral Pulmonary Artery Bands

Hypoplastic left heart syndrome (HLHS) with intact (IAS) or highly restrictive atrial septum (RAS) has extremely poor outcomes largely related to pulmonary pathology. At birth, immediate left atrial (LA) decompression is required to remain viable, but there is a tradeoff between residual increase in LA pressure and pulmonary overcirculation, either of which exacerbates the pulmonary status. From August 2010 to April 2013, a retrospective chart review was performed on consecutive patients with a prenatal diagnosis of HLHS with IAS/RAS presenting to a single center. The management strategy was immediate LA decompression followed by placement of bilateral pulmonary artery bands (bPAB) and subsequent conventional Norwood procedure. Six patients were born with HLHS with IAS/RAS during this time period with this planned management strategy. Four patients underwent LA decompression and subsequently developed low cardiac output with pulmonary overcirculation. bPAB were used with improvement in cardiac output and pronounced diuresis. These patients all survived the Norwood and subsequent Glenn procedures and remain alive [median follow-up 2.2 years (range 11 months-2.7 years)]. Two patients did not survive with therapy being withdrawn before the Norwood procedure. It is hypothesized that a strategy of total LA decompression followed by bPAB maximizes preoperative systemic perfusion and minimizes ongoing injury to the pulmonary system. This may enhance patient candidacy for the Norwood procedure and long-term survival.

Transient serosal enhancement mimicking pneumoperitoneum

Sir, In a prior issue of this journal, we reported two cases of a novel enhancement pattern involving the pericardium, peritoneum and soft tissues seen on radiographs in the early post-cardiac catheterization period [1]. Knowledge of this phenomenon is clinically important because the enhancement pattern renders a relative lucency over the liver. We report a third case of this radiographic phenomenon that mimicked the appearance of pneumoperitoneum and led to an exploratory laparotomy. A baby girl with hypoplastic left heart variant including a double-outlet right ventricle and restrictive atrial septum was born by cesarean section at 37 2/7 weeks to a gravida I para I woman. Cardiac catheterization was performed on the day the child was born for stent placement into veins decompressing into the left atrium. At 5 days old, the newborn was taken for a Norwood/Sano procedure and atrial septectomy. A complicated post-operative course followed, including renal insufficiency, respiratory failure, poor cardiac function, acidosis and delayed sternotomy closure. The infant was taken back to the cardiac catheterization lab to evaluate the pulmonary veins. A radiograph of the chest and abdomen obtained within 24 h of completion of the cardiac catheterization suggested the presence of free intraperitoneal air, characterized by a conspicuous relative lucency over the right upper quadrant (Fig. 1). A left lateral decubitus view did not confirm the presence of pneumoperitoneum. However, given the suggestive findings of pneumoperitoneum on the supine radiograph and the infant’s clinical condition, the child was taken to the operating room. Findings during the exploratory laparotomy were negative. The classic findings of pneumoperitoneum on supine abdominal radiography include radiolucency over the liver [2].

Fetal Interventions for Congenital Heart Disease in Brazil

Fetal interventions have been performed for some congenital heart diseases. However, these procedures have not gained wide acceptance due to concerns about their efficacy and safety. The aim of this study was to report on a preliminary experience with fetal cardiac interventions in Brazil. Twenty-two cardiac interventions were performed in 21 fetuses. Thirteen fetuses had critical aortic stenosis (CAS), 4 had hypoplastic left heart syndrome (HLHS) and intact interatrial septum or small patent foramen ovale, 1 had pulmonary atresia with intact ventricular septum (IVS), and 3 had critical pulmonary stenosis (CPS). The main outcome variables evaluated were technical success and procedural complications as well as pregnancy and postnatal outcomes. Success was achieved in 20 of 22 procedures (91%) with 1 failed aortic and 1 failed pulmonary valvuloplasties. There was 1 fetal death. No maternal complications occurred. One patient with CAS, severe mitral regurgitation, and hydrops died postnatally within 5 months of age. All patients with HLHS and restrictive atrial septum died after interventional or surgical procedures and prolonged hospitalizations. All patients with CPS/IVS survived and achieved a biventricular (BV) circulation after neonatal valvuloplasty and ductal stenting. A BV circulation was achieved in 4 of 8 patients with CAS and evolving HLHS (one still in utero), including 2 with initial borderline left ventricles (LV) in whom surgical LV overhaul was performed at 9 months of age. In this preliminary experience, the feasibility of fetal cardiac interventions and their outcomes were similar to those previously reported.

Balloon Atrial Septostomy by a Right Internal Jugular Venous Approach in a Newborn with Hypoplastic Left Heart Syndrome with a Restrictive Atrial Septum

Hypoplastic left heart syndrome with an intact or highly restrictive atrial septum requires urgent decompression of the left atrium. Catheter-based interventions from the femoral or umbilical veins represent the standard method of atrial decompression. Restrictive atrial septal defects located at the superior portion of the fossa ovalis can be difficult to cross from these access sites. Here, we describe a successful Rashkind balloon atrial septostomy performed from an internal jugular approach.

Fetal stenting of the atrial septum: Technique and initial results in cardiac lesions with left atrial hypertension

Hypoplastic left heart syndrome with a highly restrictive or intact atrial septum (HLHS-RAS) has a very high mortality. Fetal left atrial (LA) hypertension results in abnormal lung development with lymphangiectasia and pulmonary vein muscularization. We report our initial experience with percutaneous ultrasound-guided stenting of the fetal atrial septum to decompress the LA. Retrospective review of fetuses with HLHS-RAS or a variant that underwent active perinatal management from 2000 to 2012. Ten fetuses were identified. Two died in utero (33, 29 weeks). Four required the urgent creation of an atrial communication immediately after birth but died subsequently (5-54 days). Four fetuses (28-36 weeks) underwent percutaneous stenting of the atrial septum, with ultrasound guidance and intravenous maternal sedation. Elevated LA pressure, pulmonary vein dilation and MRI estimated pulmonary perfusion all improved after stenting. Three of four stented fetuses were delivered vaginally. Atrial septectomy was performed within 48 h of delivery to ensure complete LA decompression, rather than for hypoxemia. Intraoperative lung biopsy demonstrated muscularized pulmonary veins and lymphangiectasia in all four. Two fetuses developed stent stenosis in utero and died after birth, from pulmonary hypertension and sepsis respectively. Two are alive, representing an improved outcome over our previous experience (p=0.03). Fetal atrial septal stenting is feasible without maternal complications and allows vaginal delivery of a more stable neonate. Fetal LA decompression ameliorates rather than reverses lung injury, and is one component of an approach that may improve survival in HLHS-RAS.

Current Outcomes of Hypoplastic Left Heart Syndrome With Restrictive Atrial Septum: A Single-Center Experience

Advances in the management of hypoplastic left heart syndrome (HLHS) have resulted in improved survival. However, short and long-term mortality in patients with a restrictive atrial septum remains high. All neonates diagnosed with HLHS from 2003 to 2010 at our institution were evaluated. Patients who underwent atrial septostomy within the first 72 h conformed the restrictive atrial septum group (HLHS-RS). Patients with a non-restrictive communication (HLHS-NRS) formed the control group. Outcomes and survival status were determined from review of medical records. Of the 141 newborns diagnosed with HLHS, 20 (14 %) required intervention for a restrictive atrial septum. Procedural success was achieved in 17/20 (85 %) patients. Complications occurred in ten procedures, two of which were life threatening. No procedural deaths occurred. Overall median follow up was 35.5 months (0.4-104). Initial hospitalization survival was 16/20 (80 %) for the HLHS-RS group and 114/121(94 %) for the HLHS-NRS (p = 0.028). Twenty (14 %) patients were lost to follow up and 9 (6 %) underwent heart transplant. Overall survival was 10/16 (62 %) for HLHSRS patients and 77/95 (81 %) for HLHS-NRS (p = 0.1). Survival after initial discharge was 10/12 (83 %) for the HLHS-RS group and 77/88 (87 %) for the HLHS-NRS (p = 0.67). No predictors for HLHS-RS outcome were identified. Mortality at first-stage palliation in HLHS neonates with a restrictive atrial septum remains higher than in those with an unrestrictive communication. However, survival after initial hospital discharge is similar.

Hypoplastic left heart syndrome with intact or highly restrictive atrial septum outcome after neonatal transcatheter atrial septostomy

Hypoplastic left heart syndrome with intact or highly restrictive atrial septum outcome after neonatal transcatheter atrial septostomy

Bilateral pulmonary artery banding for resuscitation in high-risk, single-ventricle neonates and infants: A single-center experience

Bilateral pulmonary artery banding with or without ductal stenting has been performed as a resuscitative intervention for patients considered at too high risk for conventional single ventricle palliation. The purpose of the present study was to determine the outcomes using this strategy. We performed a retrospective review of 24 patients with single ventricle anatomy who were younger than 3 months who had undergone bilateral pulmonary artery banding and ductal stenting or maintenance of prostaglandin E(1) from January 2007 to October 2011 at our institution. The echocardiographic, angiographic, operative, and clinical data were reviewed. Follow-up data were available for 100% of the patients. All 24 patients (13 male patients) underwent bilateral pulmonary artery banding at a median age of 8days (range, 2-44 days). Their gestational age was 38 weeks (range, 27-41 weeks), and their weight was 3.01 kg (range, 1.5-4.4 kg). The cardiac diagnoses included hypoplastic left heart syndrome/variant hypoplastic left heart syndrome in 18, unbalanced atrioventricular canal in 4, and tricuspid atresia in 2. In the hypoplastic left heart syndrome group, 9 (50%) had an intact or a highly restrictive atrial septum requiring open (n=1) or transcatheter (n=8) atrial septostomy with or without atrial stent placement (n=4). Ductal stenting was performed in 14 patients, and 10 patients were continued with prostaglandin E(1). Fifteen patients (62.5%) survived to undergo a Norwood procedure (n=7), comprehensive stage 2 (n=1), or primary cardiac transplantation (n=7). Of the 9 who died, support was withdrawn in 5 because of a contraindication to transplantation, 1 because of sepsis and/or multiorgan system failure, and 1 for whom palliative care was desired. Two died awaiting transplantation. All 7 patients who underwent a conventional Norwood operation survived to discharge, and 6 of the 7(85.7%) underwent bidirectional Glenn shunt placement. Of the 7 patients who underwent transplantation, 6(85.7%) were alive at a median follow-up of 33.6 months. Bilateral pulmonary artery banding with or without ductal stenting is an effective method of resuscitation for high-risk neonates and infants with a single ventricle, allowing for reasonable survival to conventional first-stage palliation or primary transplantation.

Abstract 19087: Improved Survival After Percutaneous Stenting of the Atrial Septum in Human Fetal Left Atrial Hypertension

Hypoplastic left heart syndrome with a highly restrictive or intact atrial septum (HLHS-RAS) has a very high mortality. The associated fetal LA hypertension results in abnormal lung development with lymphangiectasia and pulmonary vein muscularization. We report our experience of ultrasound-guided stenting of the human fetal atrial septum. Methods and Results. Retrospective review of fetuses with HLHS-RAS or a variant that underwent active perinatal management from 2000-January 2012. Ten fetuses were identified. Two fetuses became hydropic and died in utero (33, 29 weeks). Four fetuses required the urgent creation of an atrial communication immediately after birth but subsequently died (5-54 days), secondary to pulmonary hypertension. More recently, the atrial septum was stented in four fetuses at 28-36 weeks without maternal general anesthesia. Elevated left atrial pressure, massive dilatation of the pulmonary veins and MRI estimated pulmonary perfusion all improved after stenting. Two fetuses developed stent stenosis with recurrence of LA hypertension in utero for approximately four weeks and 1 day respectively. Three of four stented fetuses were born by vaginal delivery. Atrial septectomy and additional procedures were performed within 72 hours of birth. Intraoperative lung biopsy demonstrated muscularized pulmonary veins and lymphangiectasia in all four. Two died at 17 and 53 days of age from pulmonary hypertension and sepsis, respectively. The other two are still alive, a significant improvement in survival (p=0.03). Conclusion. Fetal left atrial decompression by stenting may improve survival in HLHS-RAS.

Pulmonary Overcirculation Following Left Atrial Decompression in Hypoplastic Left Heart Syndrome with Intact/Restrictive Atrial Septum

Patients with hypoplastic left heart syndrome (HLHS) with intact or severely restrictive atrial septum have slow resolution of their elevated pulmonary vascular resistance after left atrial decompression. We report two neonates who required urgent surgical intervention because of unusually rapid fall in their pulmonary vascular resistance following left atrial decompression leading to pulmonary overcirculation and severe congestive heart failure. HLHS with intact or severely restrictive atrial septum is a predictor of poor outcome. The need for left atrial decompression can be predicted by well defined fetal echocardiographic indicators. However evolution of pulmonary resistance can be difficult to predict. In some patients decompression of the left atrium can lead to early excessive pulmonary blood flow and congestive heart failure requiring urgent intervention. Here, we report two cases of HLHS with highly restrictive atrial septum that after atrial septostomy had an accelerated transition requiring urgent intervention. Because of retrospective nature of the study, Children's National Medical Center approved waiver of informed consent.

OC01.02: Cerebrovascular impedance in the fetus with transposition of the great arteries: variability based on atrial septal restriction

OC01.02: Cerebrovascular impedance in the fetus with transposition of the great arteries: variability based on atrial septal restriction

Outcome and Resource Utilization of Infants Born With Hypoplastic Left Heart Syndrome in the Intermountain West

The objective of the present study was to characterize the outcomes and resource utilization of all infants born with hypoplastic left heart syndrome (HLHS) in the Intermountain West. This was a retrospective cohort study of all infants born with HLHS in the Intermountain West from January 1995 and January 2010. The cohort was divided into 3 eras: era 1, 1995 to 1999; era 2, 2000 to 2004; and era 3, 2005 to 2010. Cox proportional hazards regression analysis was performed to assess mortality. The lifetime hospitalization days and charges were also determined. Of the 245 infants identified, 65% were male infants and 172 (70%) underwent Stage 1 palliation. The transplant-free survival rate for the entire cohort was 33% at 14 years. The 1-year transplant-free survival rate for the surgical cohort was 60% in era 3. The infants whose initial presentation included shock, restrictive or intact atrial septum, chromosomal defects, or multiorgan dysfunction had an increased risk of death. A recent era of birth, greater birthweight, and older gestational age were associated with improved survival. The factors associated with mortality after stage 1 included surgical procedure type (Blalock-Taussig vs Sano shunt, hazard ratio 2.1), requirement for postoperative extracorporeal membrane oxygenation (hazard ratio 4.2), postoperative renal dysfunction (hazard ratio 3.0), anomalous pulmonary venous return (hazard ratio 2.9), and moderate or greater tricuspid valve regurgitation at any point (hazard ratio 2.0). For patients who had undergone stage 1, 2, or 3 palliation, the median cumulative lifetime hospitalization was 32, 48, and 65 days, and the median cumulative lifetime charges for hospitalization were $201,812, $253,183, and $296,213, respectively. In conclusion, although hospital-based studies of HLHS have shown significantly improved survival after surgical palliation, population-based studies have shown that HLHS continues to have a high mortality and high resource utilization.

Prenatal diagnosis of atrial restriction in hypoplastic left heart syndrome is associated with decreased 2‐year survival

To compare the course of hypoplastic left heart syndrome (HLHS) patients diagnosed prenatally with any degree of atrial restriction with those without evidence of atrial restriction. Retrospective, cohort. Prenatally diagnosed HLHS patients from August 1999 to January 2009 were categorized as nonrestrictive versus restrictive, defined by left atrial hypertension on pulmonary venous Doppler and/or an intact interatrial septum. Of 73 total fetal patients identified, 49 were live-born. Survival at 2 years was 29/35 [83% confidence interval (CI): 59.5%-88.9%] for the nonrestrictive group and 6/14 (43% CI: 17.7%-6.0%) for the restrictive group (p<0.0001). Of those who underwent stage 1 palliation (35 with nonrestrictive and 10 with restrictive atrial septa), both groups had a similar incidence of preoperative acidosis and need for ventilation and inotropic support. Postoperatively, there was no difference between groups in ventilator days, length of stay, or survival to discharge. There was decreased survival at 2 years in the restrictive group, 60% (CI: 26.2%-87.8%) versus 83% (CI: 66.4%-93.4%) in the nonrestrictive group. Furthermore, a disproportionate number of interstage deaths was evident in the restrictive group. Prenatal presence of any degree of atrial septal restriction in the setting of HLHS confers a significant survival disadvantage, with increases in both early and late mortality.

Evidence-Based Fetal Referrals for Congenital Heart Surgery

Background. The outcomes of fetal referrals to congenital heart disease centers for delivery and postnatal surgery prior to discharge over a two-year period were reviewed. Cost differences between fetal referrals and neonatal transports were investigated. Methods. A retrospective chart review was conducted on 17 fetal referrals to two congenital heart disease centers from 01/01/2007 to 12/31/2008. The two centers were contacted to obtain their neonatal transport charges. Results. Of the 17 fetal referrals, 10 patients underwent congenital heart surgery prior to postnatal discharge. Only one patient who underwent surgery died. Third party payers saved approximately $13,600 or $36,600 in neonatal transport costs to these centers. Conclusions. There was only one death of a patient with hypoplastic left heart syndrome and a restrictive atrial septum, which has a poor prognosis. There was a significant cost differential between fetal referral and neonatal transport.

Hypoplastic left heart syndrome with restrictive atrial septum and advanced heart block documented with a novel fetal electrocardiographic monitor

Hypoplastic left ventricle with congenital heart block has been reported previously in a fetus with concurrent left atrial isomerism and levo-transposition of the great arteries. We present the unusual case of an infant diagnosed in utero with hypoplastic left heart syndrome, a restrictive atrial septum and advanced heart block but with D-looping of the ventricles and no atrial isomerism. In addition, fetal heart rhythm was documented with the assistance of a new fetal electrocardiographic monitor.

360 Fetal pulmonary vein doppler flow pattern: Predicting the need for immediate left atrial decompression in hypoplastic left heart syndrome with restrictive atrial septum

BACKGROUND: Fetal pulmonary venous (PV) Doppler flow has been correlated with the severity of atrial septal restriction (ASR) and the need for emergent atrial septostomy (EAS) in newborns with HLHS. According to Michelfelder et al (JTCS 2011), a fetal forward/reverse PV Doppler flow velocity-time integral (PV VTI) ratio 5 predicted the need for EAS, required in 21% of their 39 HLHS cases, with a sensitivity of 100% and a specificity of 94%. We reviewed our contemporary institutional experience with this entity. METHODS: Echocardiograms and outcomes of 67 consecutive fetal HLHS cases with active postnatal management were reviewed. PV Doppler assessments included maximal S-, Dand A-wave flow velocities (cm/s); A-wave duration (ms), S/D wave and forward/reverse PV VTI ratios. EAS 1 days of age and survival to infancy were used as clinical outcome variables. RESULTS: Four (6%) of the 67 cases required an EAS for an intact (n 1) or severely restrictive atrial septum. The table indicates the median (range) fetal PV Doppler indices at the pre-delivery echocardiogram of newborns with/without EAS:

Perioperative Care of a Child with Transposition of the Great Arteries

Because a minority of patients with D-transposition of the great arteries are diagnosed in utero by ultrasound, most present after delivery with cyanosis. In the absence of apparent lung disease, cyanotic neonates suspected of having a cardiac lesion should be immediately transferred to an intensive care unit at a pediatric tertiary care center for monitoring, resuscitation, and to define the cardiac anatomy and physiology. A prostaglandin E-1 infusion is usually initiated to maintain ductal patency and promote intra-cardiac mixing. In the past, balloon atrial septostomy (BAS) was routinely performed to enlarge the atrial septal defect and improve intra-cardiac mixing while the infants awaited surgery. Recent literature has reported an increase risk of stroke in neonates who undergo BAS, although more recent studies refute this. Our current practice is to perform BAS in neonates who have both echocardiographic evidence of a restrictive atrial septum and hypoxia or instability that is unresponsive to other interventions. The occasional patient who does not respond to initial management may have elevated pulmonary vascular resistance and may stabilize with pulmonary vasodilators, such as inhaled nitric oxide. Rarely, a child does not respond to interventional and pharmacologic resuscitation and requires mechanical support pre-operatively with extracorporeal membrane oxygenation (ECMO). In our experience, ECMO has been a successful bridge to corrective surgery with excellent outcomes. After pre-operative stabilization, arterial switch procedure is typically performed in the first week of life with very favorable early results.

Impact of Fetal Cardiac Intervention on Congenital Heart Surgery

Fetal cardiac interventions performed by interventional cardiologists are currently in a clinical experimental phase. The 3 most frequent interventions are: 1) aortic balloon valvuloplasty for critical aortic stenosis with a small left ventricle or with a normal size left ventricle but poor function; 2) atrial septostomy for highly restrictive or intact atrial septum in hypoplastic left heart syndrome; and 3) pulmonary valvuloplasty for pulmonary atresia and hypoplastic right ventricle. Current impact on everyday congenital heart surgery practice is small, but may increase in the future.