Cases Of Hypoplastic Left Heart Syndrome Research Articles

Critical Organ Dysfunction and Preoperative Mortality in Newborns with Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome (HLHS) is fatal without surgical intervention. An important subset of HLHS patients die prior to surgical intervention, but this population is underevaluated. The neonatal sequential organ failure assessment score (nSOFA) is an operational definition of organ dysfunction that can identify those with a high risk of mortality among neonatal intensive care unit (NICU) patients. The utility of the nSOFA to predict preoperative mortality in the unique HLHS population is unknown and could inform care, particularly care provided by neonatology staff. We performed a multicenter retrospective cohort study of HLHS cases across three level IV NICUs from January 1, 2009 to December 3, 2023. Patients were classified as either survived or died prior to surgical intervention. Demographic variables were curated from medical records including the maximum nSOFA (nSOFAmax) before surgical intervention or death. We identified 265 patients with HLHS over the study period. The nSOFAmax was greater in patients who died preoperatively (14/265; 5%) compared with survivors to surgical intervention (median 8 [interquartile range, 6, 12] vs. 2 [0, 4]; p < 0.001). The area under receiver operating characteristics curve for the nSOFAmax to discriminate for mortality was 0.93 (95% confidence interval, 0.88-0.98; p < 0.001). Compared with an nSOFAmax of 0, the likelihood ratio for preoperative death doubled at 2, tripled at 4, and was 10-fold at 9. This is the first demonstration of nSOFA utility in specific to congenital heart disease and HLHS. The nSOFAmax represents a novel, electronic health record-compatible, and generalizable method to identify patient-level organ dysfunction and risk for preoperative mortality in HLHS patients. KEY POINTS: · An important subset of HLHS patients die preoperatively.. · nSOFA can be used to measure preoperative HLHS severity.. · nSOFA predicts preoperative mortality risk in HLHS patients..

Human Genetics of Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome (HLHS) is a severe congenital cardiovascular malformation characterized by hypoplasia of the left ventricle, aorta, and other structures on the left side of the heart. The pathologic definition includes atresia or stenosis of both the aortic and mitral valves. Despite considerable progress in clinical and surgical management of HLHS, mortality and morbidity remain concerns. One barrier to progress in HLHS management is poor understanding of its cause. Several lines of evidence point to genetic origins of HLHS. First, some HLHS cases have been associated with cytogenetic abnormalities (e.g., Turner syndrome). Second, studies of family clustering of HLHS and related cardiovascular malformations have determined HLHS is heritable. Third, genomic regions that encode genes influencing the inheritance of HLHS have been identified. Taken together, these diverse studies provide strong evidence for genetic origins of HLHS and related cardiac phenotypes. However, using simple Mendelian inheritance models, identification of single genetic variants that "cause" HLHS has remained elusive, and in most cases, the genetic cause remains unknown. These results suggest that HLHS inheritance is complex rather than simple. The implication of this conclusion is that researchers must move beyond the expectation that a single disease-causing variant can be found. Utilization of complex models to analyze high-throughput genetic data requires careful consideration of study design.

Perinatal Outcomes in Hypoplastic Left Heart Syndrome

Aim: To review our experience in fetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS).&#x0D; Materials and Method: Retrospective study of fetuses prenatally diagnosed with HLHS between 2020 and 2022 in a tertiary referral center.&#x0D; Results: 29 fetuses with HLHS were identified. 13 of all 29 cases (44.8%) had associated cardiac abnormalities and 8 cases (27.5%) had coexisting extracardiac abnormalities. Only a small minority of cases underwent invasive diagnostic testing (20%), and all of which had a normal karyotype. Most cases were delivered at term (58.6%), median gestational week at delivery was 37 (range, 34-39), and the mean standard deviation (SD) birthweight was 3099±455 grams. In addition, HLHS was more common in male than in female fetuses (69% vs 31%). Termination of pregnancy (TOP) was selected in 4 (13.7%) of the diagnosed 29 cases and performed before at ≤ 26 weeks of pregnancy. There were 3 intrauterine fetal demise (IUFD) at 23,29, and 34 weeks of gestation. Among the 29 cases of HLHS, 22 (75.8%) were live born. 3 of 22 live born infants were managed by medical procedures as they were not appropriate surgical candidates and later died at 22nd, 23rd and 25th of postnatal day. Surgery was performed in 15 cases and only one of them is are still alive. Mortality rate in the first three months of neonatal period was 96.5%. &#x0D; &#x0D; Conclusion: Fetal echocardiography allows an accurate diagnosis of HLHS, which is made even&#x0D; in the late first trimester. Despite early prenatal detection and postnatal surgical interventions, the results from our center in last two years reflect poor outcomes for HLHS.

Prenatal diagnosis of hypoplastic left heart syndrome on ultrasound using artificial intelligence: How does performance compare to a current screening programme?

Artificial intelligence (AI) has the potential to improve prenatal detection of congenital heart disease. We analysed the performance of the current national screening programme in detecting hypoplastic left heart syndrome (HLHS) to compare with our own AI model. Current screening programme performance was calculated from local and national sources. AI models were trained using four-chamber ultrasound views of the fetal heart, using a ResNet classifier. Estimated current fetal screening programme sensitivity and specificity for HLHS were 94.3% and 99.985%, respectively. Depending on calibration, AI models to detect HLHS were either highly sensitive (sensitivity 100%, specificity 94.0%) or highly specific (sensitivity 93.3%, specificity 100%). Our analysis suggests that our highly sensitive model would generate 45,134 screen positive results for a gain of 14 additional HLHS cases. Our highly specific model would be associated with two fewer detected HLHS cases, and 118 fewer false positives. If used independently, our AI model performance is slightly worse than the performance level of the current screening programme in detecting HLHS, and this performance is likely to deteriorate further when used prospectively. This demonstrates that collaboration between humans and AI will be key for effective future clinical use.

CELSR1 Risk Alleles in Familial Bicuspid Aortic Valve and Hypoplastic Left Heart Syndrome.

Whole-genome sequencing in families enables deciphering of congenital heart disease causes. A shared genetic basis for familial bicuspid aortic valve (BAV) and hypoplastic left heart syndrome (HLHS) was postulated. Whole-genome sequencing was performed in affected members of 6 multiplex BAV families, an HLHS cohort of 197 probands and 546 relatives, and 813 controls. Data were filtered for rare, predicted-damaging variants that cosegregated with familial BAV and disrupted genes associated with congenital heart disease in humans and mice. Candidate genes were further prioritized by rare variant burden testing in HLHS cases versus controls. Modifier variants in HLHS proband-parent trios were sought to account for the severe developmental phenotype. In 5 BAV families, missense variants in 6 ontologically diverse genes for structural (SPTBN1, PAXIP1, and FBLN1) and signaling (CELSR1, PLXND1, and NOS3) proteins fulfilled filtering metrics. CELSR1, encoding cadherin epidermal growth factor laminin G seven-pass G-type receptor, was identified as a candidate gene in 2 families and was the only gene demonstrating rare variant enrichment in HLHS probands (P=0.003575). HLHS-associated CELSR1 variants included 16 missense, one splice site, and 3 noncoding variants predicted to disrupt canonical transcription factor binding sites, most of which were inherited from a parent without congenital heart disease. Filtering whole-genome sequencing data for rare, predicted-damaging variants inherited from the other parent revealed 2 cases of CELSR1 compound heterozygosity, one case of CELSR1-CELSR3 synergistic heterozygosity, and 4 cases of CELSR1-MYO15A digenic heterozygosity. CELSR1 is a susceptibility gene for familial BAV and HLHS, further implicating planar cell polarity pathway perturbation in congenital heart disease.

Geographic Distribution of Congenital Heart Disease: A Single Surgical Center Experience

To determine presence of spatial clustering or dispersion of pre and postnatally detected hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (TGA) cases. This retrospective study examined all patients with a prenatal or postnatal diagnosis of HLHS or TGA who had an initial visit or hospitalization at our tertiary care center over a 5-year period from 2012 to 2016 (n=105). Using geographic information systems software, the nearest neighbor ratio (NNR) tool was used to determine whether statistically significant clustering or dispersion occurred. Geographic clustering was observed among prenatally diagnosed pooled cases of HLHS and TGA and all total cases (NNR=0.73 and 0.66, respectively), but not postnatally detected cases (NNR=1.08). Notably, there was significant dispersion of postnatally detected TGA cases (NNR=1.22) There was no pattern for prenatally detected TGA or HLHS when analyzed individually. The spatial distribution of HLHS and TGA is not random; these conditions occur in geographic clusters. Clustering of all patients in the study population and dispersion of postnatal diagnosis of TGA represent opportunities for improved delivery of fetal cardiac care.

Seasonality of Hypoplastic Left Heart Syndrome and Single Ventricle Heart in Poland in the Context of Air Pollution.

Hypoplastic left heart syndrome (HLHS) and single ventricle (SV) remain a significant cause of cardiac deaths occurring in the first week of life. Their pathogenesis and seasonal frequency are still unknown. Therefore, we attempt to look at the genesis of the HLHS and SV in the context of territorial distribution as well as seasonality. A total of 193 fetuses diagnosed with HLHS and 92 with SV were selected. The frequency was analyzed depending on the year, calendar month, quarter and season (fall-winter vs. spring-summer). The spatial distribution of HLHS and SV in Poland was analyzed. We observed a statistically significant overrepresentation of HLHS formation frequency in March: 27 (14.00%) in comparison to a monthly median of 15 (IQR: 13.75–16.25; p = 0.039), as well as a significantly higher frequency of HLHS in 2007−2009: 65 cases (33.68%) in comparison to the annual mean of 13.79 ± 6.36 (p < 0.001). We noted a higher frequency of SV among parous with the last menstrual period reported in the fall/winter season of 58 vs. 34 in the spring/summer season (p = 0.014). The performed analysis also revealed significant SV overrepresentation in 2008: 11 cases (12.00%) in comparison to the annual mean of 6.57 ± 2.71 (p = 0.016). Every single case of HLHS was observed when the concentration of benzo(a)pyrene and/or PM10 exceeded the acceptable/target level. Our research indicates that both the season and the level of pollution are significant factors affecting the health of parous women and their offspring. The reason why HLHS and SV develop more frequently at certain times of the year remains unclear, therefore research on this topic should be continued, as well as on the effects of PM10 and benzo(a)pyrene exposure.

The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales.

Background: Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (CHD) characterised by the underdevelopment of the left side of the heart with varying levels of hypoplasia of the left atrium, mitral valve, left ventricle, aortic valve and aortic arch. In the UK, age 12 survival for cases born between 1991 and 1993 was 21%. UK survival estimates corresponding to cases born between 2000 and 2015 were improved at 56%, but survival was examined up to age five only. Contemporary long-term survival estimates play a crucial role in counselling parents following diagnosis. The aim of this study was to report survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle with additional CHD in England and Wales between 1998 and 2012.Methods: Cases of HLHS notified to four congenital anomaly registers in England and Wales during 1998–2012, matched to Office for National Statistics mortality information, were included. Kaplan-Meier survival estimates to age 15 were reported. Cox regression models were fitted to examine risk factors for mortality.Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality.Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.

Fluid mechanics of the left atrial ligation chick embryonic model of hypoplastic left heart syndrome

Left atrial ligation (LAL) of the chick embryonic heart at HH21 is a model of the hypoplastic left heart syndrome (HLHS) disease, demonstrating morphological and hemodynamic features similar to human HLHS cases. Since it relies on mechanical intervention without genetic or pharmacological manipulations, it is a good model for understanding the biomechanics origins of such HLHS malformations. To date, however, the fluid mechanical environment of this model is poorly understood. In the current study, we performed 4D ultrasound imaging of LAL and normal chick embryonic hearts and 4D cardiac flow simulations to help shed light on the mechanical environment that may lead to the HLHS morphology. Results showed that the HH25 LAL atrial function was compromised, and velocities in the ventricle were reduced. The HH25 LAL ventricles developed a more triangular shape with a sharper apex, and in some cases, the atrioventricular junction shifted medially. These changes led to more sluggish flow near the ventricular free wall and apex, where more fluid particles moved in an oscillatory manner with the motion of the ventricular wall, while slowly being washed out, resulting in lower wall shear stresses and higher oscillatory indices. Consequent to these flow conditions, at HH28, even before septation is complete, the left ventricle was found to be hypoplastic while the right ventricle was found to be larger in compensation. Our results suggest that the low and oscillatory flow near the left side of the heart may play a role in causing the HLHS morphology in the LAL model.

Timing of Transfer and Mortality in Neonates with Hypoplastic Left Heart Syndrome in California.

Maternal race/ethnicity is associated with mortality in neonates with hypoplastic left heart syndrome (HLHS). We investigated whether maternal race/ethnicity and other sociodemographic factors affect timing of transfer after birth and whether timing of transfer impacts mortality in infants with HLHS. We linked two statewide databases, the California Perinatal Quality Care Collaborative and records from the Office of Statewide Health Planning and Development, to identify cases of HLHS born between 1/1/06 and 12/31/11. Cases were divided into three groups: birth at destination hospital, transfer on day of life 0–1 (“early transfer”), or transfer on day of life ≥ 2 (“late transfer”). We used log-binomial regression models to estimate relative risks (RR) for timing of transfer and Cox proportional hazard models to estimate hazard ratios (HR) for mortality. We excluded infants who died within 60 days of life without intervention from the main analyses of timing of transfer, since intervention may not have been planned in these infants. Of 556 cases, 107 died without intervention (19%) and another 52 (9%) died within 28 days. Of the 449 included in analyses of timing of transfer, 28% were born at the destination hospital, 49% were transferred early, and 23% were transferred late. Late transfer was more likely for infants of low birthweight (RR 1.74) and infants born to US-born Hispanic (RR 1.69) and black (RR 2.45) mothers. Low birthweight (HR 1.50), low 5-min Apgar score (HR 4.69), and the presence of other major congenital anomalies (HR 3.41), but not timing of transfer, predicted neonatal mortality. Late transfer was more likely in neonates born to US-born Hispanic and black mothers but was not associated with higher mortality.Supplementary InformationThe online version of this article (10.1007/s00246-021-02561-w) contains supplementary material, which is available to authorized users.

Detection screening and seasonality evaluation of hypoplastic left heart syndrome in the polish national registry for fetal cardiac anomalies from the years 2004 to 2016.

To evaluate the incidence of hypoplastic left heart syndrome (HLHS) and the efficiency of the screening program using data from the Polish National Registry for Fetal Cardiac Anomalies. To investigate whether HLHS incident rates in Poland are seasonally variable. Data on 791 cases of HLHS from the Registry collected between 2004 and 2016 was analyzed. The median gestational age for the 734 cases of HLHS detected was 23 weeks. Comparing the age at time of HLHS detection between 2004 and 2016, a decrease from 26 to 20.8 weeks was observed. We noted a rapid increase in HLHS incidence during the initial years of the Registry data, the annual percentage change during that period was 22.0% and this trend lasted until 2010. In the following years, the Registry became representative of the general population which has an estimated incidence of HLHS of 20.93 cases per 100 000 live births. We observed no clear seasonal patterns of HLHS incidence in our population. The Registry reached a plateau state in terms of diagnosing new cases of HLHS. No evidence of seasonality has been noted. The average gestational age of patients identified as having HLHS decreased significantly during the study period.

Impact of MYH6 variants in hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a clinically and anatomically severe form of congenital heart disease (CHD). Although prior studies suggest that HLHS has a complex genetic inheritance, its etiology remains largely unknown. The goal of this study was to characterize a risk gene in HLHS and its effect on HLHS etiology and outcome. We performed next-generation sequencing on a multigenerational family with a high prevalence of CHD/HLHS, identifying a rare variant in the α-myosin heavy chain (MYH6) gene. A case-control study of 190 unrelated HLHS subjects was then performed and compared with the 1000 Genomes Project. Damaging MYH6 variants, including novel, missense, in-frame deletion, premature stop, de novo, and compound heterozygous variants, were significantly enriched in HLHS cases (P < 1 × 10−5). Clinical outcomes analysis showed reduced transplant-free survival in HLHS subjects with damaging MYH6 variants (P < 1 × 10−2). Transcriptome and protein expression analyses with cardiac tissue revealed differential expression of cardiac contractility genes, notably upregulation of the β-myosin heavy chain (MYH7) gene in subjects with MYH6 variants (P < 1 × 10−3). We subsequently used patient-specific induced pluripotent stem cells (iPSCs) to model HLHS in vitro. Early stages of in vitro cardiomyogenesis in iPSCs derived from two unrelated HLHS families mimicked the increased expression of MYH7 observed in vivo (P < 1 × 10−2), while revealing defective cardiomyogenic differentiation. Rare, damaging variants in MYH6 are enriched in HLHS, affect molecular expression of contractility genes, and are predictive of poor outcome. These findings indicate that the etiology of MYH6-associated HLHS can be informed using iPSCs and suggest utility in future clinical applications.

Proportion of selected congenital heart defects attributable to recognized risk factors

PurposeTo assess the contribution of multiple risk factors for two congenital heart defects—hypoplastic left heart syndrome (HLHS) and tetralogy of Fallot (TOF). MethodsWe used data from the National Birth Defects Prevention Study (1997–2011) to estimate average adjusted population attributable fractions for several recognized risk factors, including maternal prepregnancy overweight–obesity, pregestational diabetes, age, and infant sex. ResultsThere were 594 cases of isolated simple HLHS, 971 cases of isolated simple TOF, and 11,829 controls in the analysis. Overall, 57.0% of HLHS cases and 37.0% of TOF cases were estimated to be attributable to risk factors included in our model. Among modifiable HLHS risk factors, maternal prepregnancy overweight–obesity accounted for the largest proportion of cases (6.5%). Among modifiable TOF risk factors, maternal prepregnancy overweight–obesity and maternal age of 35 years or older accounted for the largest proportions of cases (8.3% and 4.3%, respectively). ConclusionsApproximately half of HLHS cases and one-third of TOF cases were estimated to be attributable to risk factors included in our models. Interventions targeting factors that can be modified may help reduce the risk of HLHS and TOF development. Additional research into the etiology of HLHS and TOF may reveal other modifiable risk factors that might contribute to primary prevention efforts.

Clinical analysis of 38 infants with hypoplastic left heart syndrome

Objective To discuss the clinical features and treatment of hypoplastic left heart syndrome(HLHS). Methods A single-center retrospective study of infants with HLHS who were admitted to the Department of Pediatric Cardiology of Guangdong General Hospital from January 2004 to December 2014 was performed, and the infants were followed-up. Results There were 38 cases of HLHS, including 23 boys and 15 girls, accounting for 0.15% of the 25 698 in-patients with congenital heart disease during the corresponding period.The ages of onset ranged from 1 hour to 5 months, while the ages at admission ranged from 1 day to 11 months.Weight at admission ranged from 1.55 to 14.00 kg (the mean weight was 3.9 kg), and hospital day ranged from 1 to 88 days (the mean hospital day was 46 days). Twenty-four of the patients had comorbidities at admission, including 20 patients with pneumonia, 2 with septicemia, 3 developed multi-organ dysfumction, 1 with cytomegalovirus infection, 4 with brain injury caused by hypoxia, 1 with congenital airway constriction, and 1 with renal failure.Eighteen patients received operation with 8 survi-vals, 16 patients gave up and were discharged, 3 died before operation because of give-up, 10 patients died during or after operation.One of the children who did not receive the operation lived until 4 years old.Patients underwent operation at the ages between 2 days and 4 months, and 10 of them needed ventilation before operation, 8 needed emergency operation.Modified Blalock-Taussig shunt(MBTS) was applied in 10 patients while sano-shunt in 8 patients.In the 8 survivals, MBTS and sano-shunt was performed equally in 4 patients in Norwood stage Ⅰ, while 5 received MBTS and 3 sano-shunt who died after stage Ⅰ.After the operation, 7 patients developed severe low cardiac output, and 1 had pericardial tamponade, 3 suffered from pulmonary infection, 1 had to use extracorporeal membrane oxygenation, and 1 underwent seizure. Conclusions HLHS is a rare malformation of heart.Norwood Procedure Staged palliative surgery is the predominant management option for HLHS. Key words: Infant; Hypoplastic left heart syndrome; Treatment; Prognosis

DELIVERY PLANNING IN HYPOPLASTIC LEFT HEART SYNDROME WITH INTACT OR HIGHLY RESTRICTIVE ATRIAL SEPTUM

Hypoplastic left heart syndrome (HLHS) with intact or highly restrictive atrial septum (RS) is a rare and potentially lethal diagnosis that comprises 5-10% of all cases of HLHS. Immediate postnatal creation of an atrial communication is often necessary to decompress the left atrium (LA) and relieve

Hypoplastic Left Heart Syndrome and Ventriculocoronary Connections

Hypoplastic left heart syndrome (HLHS) is a critical congenital heart defect. There are variants of HLHS, consisting of permutations of stenosis or atresia of the mitral and aortic valves. A notable phenomenon described in all HLHS subtypes is ventriculocoronary connections (VCCs). The significance of VCCs remains undetermined. Some studies have demonstrated higher mortality associated with VCCs, while others have shown no difference in mortality compared to other HLHS variants. All variants of HLHS are associated with myocardial disarray and endomyocardial fibroelastosis, although recent evidence demonstrates a higher association of those findings with the mitral stenosis–aortic atresia (MS-AA) variant. And VCCs may have a higher incidence in MS-AA as they constitute the only egress of blood from the LV. These associations may play a role in the survival of patients with MS-AA. We present a case of HLHS with MS-AA and significant VCCs. Figure 1 depicts the diminutive LV, no outflow, and opacification of the entire coronary system. Coronary opacification can only be achieved via VCCs, given the aortic valve atresia. Figure 2 demonstrates the severely hypoplastic ascending aorta. The aorta is similar in size to the 3F catheter and the coronaries do not opacify.

Ex-utero intrapartum treatment as a novel bridging strategy to surgery in hypoplastic left heart syndrome with intact atrial septum—cross-circulation revisited

Hypoplastic left heart syndromewith an intact or nearly intact septum (HLHS-IS/NIS) constitutes 6%-20% of hypoplastic left heart syndrome cases and represents themost severe form of the disease. Unless there is a pathway for pulmonary venous decompression, hemodynamic instability and death ensue within minutes to hours after birth. Survival depends upon emergent relief of this obstruction, either by operative or interventional techniques. The optimal strategy to bridge a newborn to a stable circulation remains undefined. We describe a novel EXIT (ex-utero intrapartum treatment) strategy using maternal-placental support to bridge a fetus with HLHS-IS/NIS to palliative intervention. A 26-year-old mother was referred for a fetal echocardiogram (at 30 weeks gestation), and the diagnosis of HLHSIS/NIS (with aortic atresia, mitral stenosis) was confirmed (Figure 1, A). The family elected to proceed with fetal intervention, which was performed (at 31 weeks) in another institution. This intervention was complicated by interatrial stent malposition with no demonstrable flow across the septum, and pleural effusion (Figure 1, B). As delivery neared, the parents requested aggressive management. Although our usual approach at birth is immediate catheter intervention, this strategy would be complicated by the oblique stent position in the right atrium. An EXIT to achieve cardiopulmonary bypass (CPB) was proposed to allow the fetus to remain on placental support while undergoing median sternotomy and initiation of CPB. The strategy was the consensus from a multidisciplinary fetal care conference as the safest means of maintaining hemodynamic stability before establishing interatrial communication. After the mother suffered premature rupture of membranes, a multidisciplinary team was mobilized for an emergent EXIT procedure. General anesthesia was induced using

P20.07: Fetal balloon atrioseptostomy and interatrial stent: a single center experience

Hypoplastic left heart syndrome (HLHS) with restrictive or closed foramen ovale (Fo) is a fatal condition. In our experience the mortality among such neonates was 100%. In other series it was about 90% if the immediate surgery was performed after delivery, but the further treatment had also very high risk due to the severe damage of the pulmonary circulation. Knowing this, in an era of fetal interventions, fetal balloon atrioseptostomy or interatrial stent placement should be considered. Between June 2011 and April 2014 we performed 4 balloon atrioseptostomies and 6 stent placements in 9 fetuses. In one fetus the stent was placed after ineffective atrioseptostomy. All procedures were performed in anesthetized fetuses and mothers. There were 6 cases of HLHS and 3 cases of secondary closure of the Fo in fetuses with severe aortic stenosis and mitral insufficiency. All procedures were performed after 24 weeks. They were technically successful and improved pulmonary venous flow. There were 2 intrauterine deaths related to the procedure and 2 premature deliveries (not related to the procedure) with neonatal death. One fetus is still in utero. 4 neonates were born at term in good general condition but deteriorated quickly due to increased pulmonary flow after delivery. 3 died in spite of intensive treatment few weeks after delivery. All of them were immediately operated on. One was operated on in the 5th hour of life and his condition is stable during the first week. Atrioseptostomy and stent placement can be successfully performed in fetuses. They are potentially lifesaving procedures for otherwise lethal defects. They should always be considered in fetuses whose parents opt for active treatment. In spite of poor results, technical success and better organization for the whole team is promising for better outcome in the future.

PPO.51 Pregnancy Outcomes in Major Congenital Heart Disease

BackgroundAdvances in management have prolonged the life of many patients with major congenital heart disease (CHD). No national guidelines exist for the counselling of expectant parents with an antenatal diagnosis...

PC.01 Outcomes following the diagnosis of Hypoplastic Left Heart Syndrome; changes over a 15 year period

BackgroundAnecdotally, outcomes in patients with hypoplastic left heart syndrome (HLHS) are poor but improving. Most outcome data has come from specialist centres, excluding patients who die before transfer. Long term...