Hybrid treat-and-repair strategy for large patent ductus arteriosus: a proof-of-concept case report.

Background: In cases of atrial septal defect with pulmonary arterial hypertension (PAH), a treat-and-repair strategy that adopts pulmonary vasodilator therapy and subsequent defect closure is postulated to be effective. However, this strategy has not been applied to the large patent ductus arteriosus (PDA) with PAH. Case summary: A 10-year-old girl with trisomy 21 was referred to our hospital for the treatment of a large PDA with PAH. Cardiac catheterization and angiography revealed a type C tubular PDA with a minimal diameter of 8.1 mm, an increase in mean pulmonary artery pressure (mPAP) of 60 mmHg, a ratio of pulmonary to systemic blood flow (Qp/Qs) of 2.7, and pulmonary artery resistance (Rp) of 7.1 U/m2. Because she was categorized in the grey zone for operability, we adopted a hybrid treat-and-repair strategy in which palliative surgical duct banding was performed before pulmonary vasodilator therapy to prevent excessive pulmonary blood flow and was followed by transcatheter closure of the PDA. Postoperatively, we confirmed the flow-restricted duct with a minimal diameter of 3.3 mm, decreased Qp/Qs 1.38, high mPAP 40 mmHg, and Rp 7.3 U/m2. Six months after treatment with macitentan and tadalafil, we confirmed a decrease in Rp 4.1 U/m2 as well as low Qp/Qs 1.12, which was low enough for the duct occlusion. The transcatheter occlusion of the surgically created type A conical duct was easily and safely performed. In the mid-term follow-up, favourable haemodynamics and improved exercise were confirmed. Discussion: This is the first proof-of-concept case report to show the successful hybrid treat-and-repair strategy for large PDA, which warrants further investigation.

Case Report: Patent ductus arteriosus with tuberous sclerosis complex.

A 33-year-old patient presented with a chief complaint of patent ductus arteriosus (PDA) persisting for over 30 years. Physical examination revealed bilateral facial angiofibromas, multiple nail fibromas, intraoral fibromas, and a 'shagreen patch' on the left lumbar region. Genetic testing was performed using a peripheral venous blood sample, which confirmed the diagnosis of Tuberous Sclerosis Type 2 (TSC2). Subsequently, the patient underwent cardiac color Doppler ultrasound and chest computed tomography angiography, which confirmed the presence of PDA. Tuberous sclerosis complex (TSC) is associated with cardiovascular diseases. The initial clinical manifestation of TSC is usually cardiac rhabdomyoma in children, and it is rarely reported in adults with PDA. In this case, the patient was diagnosed with PDA when he was young, and the genetic test showed heterozygous variation of TSC2 gene. The purpose of this article is to explore the correlation between TSC and PDA at the gene level through literature review.

Effects of Total Parenteral Nutrition on Serum Osmolality and Patent Ductus Arteriosus.

BACKGROUND: The persistence of high serum osmolality in the early postnatal period is a risk for developing patent ductus arteriosus (PDA). Early aggressive nutrition (EAN), involving total parenteral nutrition (TPN), by which enough concentrations of glucose and amino acids are administered intravenously, is recommended postnatally to improve the neurological prognosis in preterm infants. However, the effects of EAN involving TPN on serum osmolality and the development of a PDA have not been adequately studied. OBJECTIVES: Thus, in this study, we aimed to investigate the impact of TPN on serum osmolality and determine whether increased serum osmolality could be associated with a higher incidence of PDA in preterm infants. METHODS: In this single-center retrospective observational study, preterm infants born at <28 weeks of gestation who had been admitted to our neonatal intensive care unit (NICU) before (pre-TPN period) and after the introduction of TPN (post-TPN) were included. We reviewed the medical records of these patients, compared the changes in serum osmolality from birth to five days after birth, the clinical background, and the incidence of PDA between these two periods, and analyzed the risk factors. Additionally, the factors affecting the serum osmolality in very preterm infants were examined. The patients who met the intervention criteria of our NICU and received a cyclooxygenase (COX) inhibitor, Indacin® (Nobelpharma, Tokyo, Japan), within seven days after birth were classified as PDA+; those who could not be identified to have PDA flow by echo and did not receive a COX inhibitor were classified as PDA-. RESULTS: The postnatal day and serum sodium (Na+) were statistically significantly correlated with a higher serum osmolality. Serum osmolality remained statistically significantly higher in the PDA+ cohort compared with the PDA- cohort after the first day of life. However, no statistically significant differences were observed in serum osmolality after 24 hours of age, weeks of gestational age, birth weight, or incidence of PDA between the pre- and post-TPN periods. The results of the multiple logistic regression analyses revealed that the increased serum osmolality correlated with PDA development. CONCLUSIONS: In this study, the serum Na+ statistically significantly correlated with a higher serum osmolality. Moreover, the increased serum osmolality correlated with PDA development. Thus, the prevention of hypernatremia might reduce the incidence of PDA. Nonetheless, the findings in this study revealed that no statistically significant differences in serum osmolality were observed between the pre-and post-TPN periods, indicating that TPN had little effect on serum osmolality.

Left Pulmonary Artery Occlusion Following Device Closure of Patent Ductus Arteriosus in Premature Infants.

Background: Device closure of a patent ductus arteriosus (PDA) is rapidly evolving, with the Amplatzer Piccolo Occluder (Abbott) receiving US Food and Drug Administration approval and becoming the first device approved for PDA closure in patients ≥700 g. We report on the first known cases of complete left pulmonary artery (LPA) occlusion following Piccolo closure of a PDA in premature infants. Methods: Retrospective chart analysis of PDA closures. Results: We have performed over 50 cases of Piccolo device closure of the PDA in preterm neonates in the past 2 years, with these 2 cases representing our only complications (4%). This represents a total complication rate similar to or lower than most centers that have published data for this procedure. Conclusions: Although rare, severe LPA obstruction can be seen in premature infants following device closure of the PDA. The Piccolo device is designed to ideally remain entirely intraductal. Although our device selection appeared to provide a device short enough for the given ductal length, we recommend, whenever possible, giving consideration to using the shortest possible device. We also recommend increasing the frequency of echocardiographic surveillance to weekly studies if at any time the imaging demonstrates an increase in the degree of obstruction/turbulence.

Comparing Neurodevelopmental Outcomes in Infants With Patent Ductus Arteriosus Stenting Versus Blalock-Taussig-Thomas Shunt: A Pilot Study.

Background: Patent ductus arteriosus stenting (PDAS) is a nonsurgical alternative to Blalock-Taussig-Thomas shunt (BTTS) for infants with ductal-dependent congenital heart disease. In this single-center study, we aimed to compare neurodevelopmental outcomes in children who underwent BTTS as initial palliation versus PDAS. Methods: Bayley Scales of Infant and Toddler Development Screening Test (Bayley-III) reports and mode of feeding data were collected for any patient who underwent PDAS or BTTS at Rady Children's Hospital from 2013 to 2021. We also prospectively administered the Parents' Evaluation of Development Status questionnaire (PEDS) to parents of children aged 2-8 years in this patient population. Results: Of the 99 patients, 64 received a Bayley-III assessment and/or PEDS screen. Of the 35 who had a Bayley-III, there was a higher proportion of patients with PDAS who scored as developmentally appropriate compared with BTTS. PEDS screen showed that a higher proportion of patients with PDAS had no parental concern for delay than that of patients with BTTS (63% vs 30%). Patients with BTTS were more likely to undergo gastrostomy tube placement than patients with PDAS. Conclusions: Our study suggests that neurodevelopmental measures are feasible, clinically relevant, and should be included in comparative effectiveness studies of infant congenital interventions. Whether PDAS offers neurodevelopmental benefit over BTTS should be confirmed in a prospective powered randomized controlled clinical trial.

Decrease in the Internal Cerebral Vein Pulsation With Improvement of Patent Ductus Arteriosus in Premature Infants at the Risk of Intraventricular Hemorrhage: Two Interesting Case Reports.

Recently, augmenting the pulsation of the internal cerebral vein (ICV) has been reported to be a predictor of premature intraventricular hemorrhage (IVH); however, prophylaxis for IVH has not yet been established. Venous pulsation is a marker of central venous pressure elevation and may be improved after heart failure treatment. Herein, we report two cases of low-birth-weight infants (29 weeks and 31 weeks of gestational age), who exhibited improvements in ICV pulsation with relief of hemodynamically significant patent ductus arteriosus (hs-PDA) following indomethacin administration. ICV flow patterns were continuously flat early after birth. Thereafter, both patients demonstrated ICV pulsation augmentation with PDA progression and brain natriuretic peptide (BNP) elevation at 52 h and 39 h after birth (in infants born at 29 and 31 weeks of gestational age, respectively). After relieving PDA with indomethacin administration, both infants exhibited an improvement in ICV pulsation with decreased BNP levels. In both cases, ICV pulsation increased when PDA became hemodynamically significant with BNP elevation, and the pulsation improved by reduction in ductal flow with decreasing BNP when PDA was relieved by indomethacin administration. The association between hs-PDA and elevated ICV pulsation indicates that hs-PDA likely leads to heightened central venous pressure. Additionally, indomethacin treatment was effective in reducing the exacerbated ICV pulsation caused by heart failure due to hs-PDA. These cases suggest that treatment for heart failure might improve the augmented ICV pulsation, which is related to the development of premature IVH. However, further studies are needed to confirm this association.

Perinatal Factors Associated with Successful Pharmacologic Closure of the Patent Ductus Arteriosus in Premature Infants.

The patent ductus arteriosus (PDA) is associated with significant morbidity and mortality in preterm infants. While pharmacologic closure of the PDA is common and effective, it can be difficult to identify which patients will respond. As such, the objective of this study was to identify factors associated with successful pharmacologic closure of the PDA. We hypothesized that clinical factors such as gestational age, birth weight, and hypertensive disorders of pregnancy would be associated with successful closure. We performed a retrospective cohort study of preterm infants who received pharmacologic treatment for a PDA at two large neonatal intensive care units in Boston, MA between January 2016 and December 2021. Infants were excluded if they received prophylactic indomethacin, had early termination of therapy, did not have an echocardiogram prior to therapy, or had congenital heart disease. The primary outcome was closure after initial course. Relevant perinatal data were collected on enrolled infants. Of the 215 enrolled infants, 131 (61%) had successful closure. Older gestational age (OR, 1.23; 95% CI,1.03-1.47), male sex (OR, 2.17; 95% CI,1.18-3.99), and maternal preeclampsia (OR, 2.75; 95% CI,1.07-7.02) were associated with successful closure. Infants who received postnatal steroids (OR, 0.49; 95% CI,0.25-0.96) were less likely to have had successful closure. In this study, we identified previously established associations of gestational age and male sex with successful pharmacologic closure. However, the associations with maternal preeclampsia and postnatal steroids are novel. While further investigation is warranted, these associations can help inform decision-making around management of the PDA.

Evaluation of Serum Acetaminophen Concentration Utility for Closure of Patent Ductus Arteriosus.

OBJECTIVE: Acetaminophen for patent ductus arteriosus (PDA) closure has gained popularity over the last decade; however, therapeutic drug monitoring for this indication remains uncertain. The exact timing and goal trough serum acetaminophen concentration ranges are not well defined. The purpose of our study is to evaluate the impact of therapeutic drug monitoring on both PDA closure rates and identify real-world risk of hepatotoxicity. METHODS: Retrospective single-center chart review of neonates admitted to the neonatal intensive care unit (NICU) between April 2016 and August 2022 with at least 1 serum acetaminophen concentration to monitor for PDA closure. Acetaminophen was initiated at 15 mg/kg administered intravenously every 6 hours and a trough serum concentration was obtained prior to the sixth or seventh dose. PDA closure was confirmed radiographically with corresponding provider documentation. Associations of efficacy to closure were -analyzed using descriptive statistics. RESULTS: Thirty-eight neonates were included in the analysis, of which 18 (47%) achieved PDA closure. First serum acetaminophen trough concentration was obtained before the seventh dose [IQR, 6-8] and ranged from undetectable (< 5 mg/L) to 30.8 mg/L. Subgroup analysis of first concentrations revealed therapeutic trough, defined as 10 to 20 mg/L, did not correlate to PDA closure (no closure median concentration = 14.7 [IQR, 13-15.6] vs closure median concentration = 15.4 [IQR, 11.4-18.5], p = 0.42), or duration of treatment. No neonate experienced acetaminophen-associated toxicity. CONCLUSIONS: PDA closure did not correlate to serum acetaminophen trough concentration. The regimen of 15 mg/kg every 6 hours appears safe as no neonate experienced acetaminophen toxicity or discontinued treatment early.

Impact of a Multidisciplinary Service Line and Program for Transcatheter Device Closure of the Neonatal Ductus Arteriosus.

Outline a quality initiative establishing an institutional service line for neonatal transcatheter device closure of the patent ductus arteriosus (TDC-PDA). A retrospective descriptive observational study surrounds programmatic approach to TDC-PDA in premature neonates with process measure spanning education, implementation, referral, and post-procedural care. Metrics tracked pre- and post-program creation with statistical analyses performed. Neonatal TDC-PDA referrals increased exponentially since program inception (n = 13 in year prior; n = 42 year 1; n = 74 year 2), especially in patients weighing less than 1.3 kg (12.5%; 55%; 50%), and were associated with an increased procedural success rate (81%; 95%; 99%). Procedural checklist creation decreased procedural "out of isolette" time (median 93 min; 59; 52), and procedural-related complication or clinical sequelae (19%; 12%; 4%). A multidisciplinary service line and program dedicated to neonatal TDC-PDA can result in a significant increase in referrals as well as procedural efficacy and safety for this medically fragile population.

International treatment outcomes of neonates on extracorporeal membrane oxygenation (ECMO) with persistent pulmonary hypertension of the newborn (PPHN): a systematic review.

BACKGROUND: PPHN is a common cause of neonatal respiratory failure and is still a serious condition and associated with high mortality. OBJECTIVES: To compare the demographic variables, clinical characteristics, and treatment outcomes in neonates with PHHN who underwent ECMO and survived compared to neonates with PHHN who underwent ECMO and died. METHODS: We adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline and searched ProQuest, Medline, Embase, PubMed, CINAHL, Wiley online library, Scopus and Nature for studies on the development of PPHN in neonates who underwent ECMO, published from January 1, 2010 to May 31, 2023, with English language restriction. RESULTS: Of the 5689 papers that were identified, 134 articles were included in the systematic review. Studies involving 1814 neonates with PPHN who were placed on ECMO were analyzed (1218 survived and 594 died). Neonates in the PPHN group who died had lower proportion of normal spontaneous vaginal delivery (6.4% vs 1.8%; p value > 0.05) and lower Apgar scores at 1 min and 5 min [i.e., low Apgar score: 1.5% vs 0.5%, moderately abnormal Apgar score: 10.3% vs 1.2% and reassuring Apgar score: 4% vs 2.3%; p value = 0.039] compared to those who survived. Neonates who had PPHN and died had higher proportion of medical comorbidities such as omphalocele (0.7% vs 4.7%), systemic hypotension (1% vs 2.5%), infection with Herpes simplex virus (0.4% vs 2.2%) or Bordetella pertussis (0.7% vs 2%); p = 0.042. Neonates with PPHN in the death group were more likely to present due to congenital diaphragmatic hernia (25.5% vs 47.3%), neonatal respiratory distress syndrome (4.2% vs 13.5%), meconium aspiration syndrome (8% vs 12.1%), pneumonia (1.6% vs 8.4%), sepsis (1.5% vs 8.2%) and alveolar capillary dysplasia with misalignment of pulmonary veins (0.1% vs 4.4%); p = 0.019. Neonates with PPHN who died needed a longer median time of mechanical ventilation (15 days, IQR 10 to 27 vs. 10 days, IQR 7 to 28; p = 0.024) and ECMO use (9.2 days, IQR 3.9 to 13.5 vs. 6 days, IQR 3 to 12.5; p = 0.033), and a shorter median duration of hospital stay (23 days, IQR 12.5 to 46 vs. 58.5 days, IQR 28.2 to 60.7; p = 0.000) compared to the neonates with PPHN who survived. ECMO-related complications such as chylothorax (1% vs 2.7%), intracranial bleeding (1.2% vs 1.7%) and catheter-related infections (0% vs 0.3%) were more frequent in the group of neonates with PPHN who died (p = 0.031). CONCLUSION: ECMO in the neonates with PPHN who failed supportive cardiorespiratory care and conventional therapies has been successfully utilized with a neonatal survival rate of 67.1%. Mortality in neonates with PPHN who underwent ECMO was highest in cases born via the caesarean delivery mode or neonates who had lower Apgar scores at birth. Fatality rate in neonates with PPHN who underwent ECMO was the highest in patients with higher rate of specific medical comorbidities (omphalocele, systemic hypotension and infection with Herpes simplex virus or Bordetella pertussis) or cases who had PPHN due to higher rate of specific etiologies (congenital diaphragmatic hernia, neonatal respiratory distress syndrome and meconium aspiration syndrome). Neonates with PPHN who died may need a longer time of mechanical ventilation and ECMO use and a shorter duration of hospital stay; and may experience higher frequency of ECMO-related complications (chylothorax, intracranial bleeding and catheter-related infections) in comparison with the neonates with PPHN who survived.

Blood pressure increase preceded spontaneous and paracetamol-induced, early ductal closure in very preterm infants.

AIM: To investigate the blood pressure (BP) and ductal calibre patterns associated with early ductal closure in very low gestational age (<32 weeks) infants. METHODS: Blood pressure was continuously measured intra-arterially among 43 infants participating in a randomised, double-blind trial of 4 days prophylactic intravenous paracetamol. Early closure of the ductus was verified by daily ultrasound examinations. The BP recordings, before and after the verified ductal closure, were examined and compared to control infants without ductal closure. In the 24-h preceding the ultrasound-verified ductal closure, periods of increased BP were searched and identified as suggestive ductal closure time points. In control infants without ductal closure, BP trends were examined during the corresponding age. RESULTS: Mean arterial BP increased significantly before ductal closure (p < 0.001) and stayed higher than in the control group. Mean BP during the 8 h preceding closure significantly predicted the ductal closure [OR 2.094 (95% CI 1.209-3.630), p = 0.008], and multiple potential confounders failed to abolish this association. A linear decrease pattern in ductal calibre was significantly associated with the ductal closure. CONCLUSION: Blood pressure increased and stabilised before the early ductal closure, offering potential implications on treatment strategies of very premature infants.

Patent ductus arteriosus and the association between lung ultrasound score and bronchopulmonary dysplasia: a secondary analysis of a prospective study.

Moderate-to-large patent ductus arteriosus (PDA) has been linked to increased risk of bronchopulmonary dysplasia (BPD), while lung ultrasound score (LUS) has been demonstrated to accurately predict BPD. We aimed to investigate the correlation of LUS as a marker of interstitial pulmonary edema and the severity of the ductal shunt in predicting future BPD development in very preterm infants. This secondary analysis of a prospective study recruited preterm infants with gestational age < 30 weeks. LUS on postnatal days 7 and 14, and echocardiographic data [PDA diameter and left atrium-to-aortic root ratio (LA/Ao)] near LUS acquisition were collected. Correlation coefficient, logistics regression analysis, and the area under the receiver operating characteristic (AUROC) procedure were used. A statistically significant and positive correlation existed between LUS and PDA diameter (ρ = 0.415, ρ = 0.581, and p < 0.001) and LA/Ao (ρ = 0.502, ρ = 0.743, and p < 0.001) at postnatal days 7 and 14, respectively, and the correlations of LUS and echocardiographic data were generally stronger in the non-BPD group. In the prediction of BPD, LUS incorporating echocardiographic data at postnatal days 7 obtained significantly higher predictive performance compared to LUS alone (AUROC 0.878 [95% CI 0.801-0.932] vs. AUROC 0.793 [95% CI 0.706-0.865]; Delong test, p = 0.013). CONCLUSIONS: There is a statistically significant correlation between LUS and echocardiographic data, suggesting their potential role as early predictors for respiratory outcomes in very preterm infants. WHAT IS KNOWN: • Lung ultrasound score (LUS) has shown good reliability in predicting bronchopulmonary dysplasia (BPD) development. • Some echocardiographic data that characterized ventricular function was reported to be used to predict severe BPD. WHAT IS NEW: • There is a positive and statistically significant correlation between LUS and echocardiographic data at postnatal days 7 and 14. • The integrated use of LUS and echocardiographic data may have potential value in predicting BPD.

Comparison of Immediate Intensive Care Outcomes of Patent Ductus Arteriosus Stenting Versus Modified Blalock-Taussig-Thomas Shunt in Infants With Ductal-Dependent Pulmonary Circulation.

Patent Ductus Arteriosus (PDA) stenting is an alternative to Modified Blalock-Taussig-Thomas Shunt (BT) to increase pulmonary blood flow in ductal-dependent pulmonary circulation. The objective of this study is to compare the immediate Intensive Care Unit (ICU) outcomes of PDA stent versus BT shunt in infants with ductal-dependent pulmonary circulation. This is a single center, retrospective study in infants less than 6 months with ductal-dependent pulmonary circulation palliated with either PDA stenting or BT shunt from January 2015 till December 2023. Data were reviewed from pediatric ICU database. Demographics details, ICU parameters, mortality and morbidity were compared. The propensity score with inverse probability weighting was used to control the effect of covariates. A total of 302 patients with PDA stenting and 100 patients with BT shunt were included. Majority of the patients had intervention at first month of life. 126 (41.7%) patient underwent PDA stenting while 36 (36%) had BTS. The PDA stenting group has shorter ICU stay, 3 (2.0,6.0) days versus 8 (4.8,13.0) days (p < 0.001), shorter length of hospital stay, 9 (6.8,15.0) days versus 16 (11.0,22.0) days (p < 0.001), shorter ventilation days, 2 (1.0,4.0) days versus 3 (2.0,7.0) days (p < 0.001) and lower mortality, 7 (2.3%) versus 16 (16.0%) (p < 0.001). At 3 months follow-up, the survival was higher in the PDA stenting group (p < 0.001). Conclusion: PDA stenting in infants with ductal-dependent pulmonary blood flow has a more favorable intensive care outcomes with shorter ICU and hospital stay. PDA stenting has a better survival outcome.

Premature Closure of the Ductus Arteriosus and Veno-Arterial Extracorporeal Membrane Oxygenation in Critically Ill Neonates: A 10-Year Single-Center Retrospective Study.

To describe critically ill neonates with premature closure of the ductus arteriosus (DA) and assess the frequency as well as predictive factors for extracorporeal membrane oxygenation (ECMO) support in the latter. This was a monocentric retrospective observational study conducted in the NICU of a French academic medical center between 01/01/2013 and 01/01/2023. All neonates diagnosed with premature closure of the DA were included. Exclusion criteria were associated congenital heart disease amenable to urgent surgery or contraindication for ECMO. Eleven neonates with complete premature closure of the DA were included. Births were full-term with a weight of 3.60 kg [3.16-3.89]. Only one case (9%) was diagnosed antenatally. Premature closure of the DA was idiopathic in seven neonates (64%) and associated to maternal exposure to non-steroidal anti-inflammatory drugs (NSAIDs) in three (27%). All newborns had pulmonary hypertension (PH) and right ventricular hypertrophy. Three neonates (27%) were supported on veno-arterial ECMO and appeared to have more pathological adaptation to extra-uterine life, greater need for vasopressor and/or inotropic support, and higher frequency of bi-ventricular failure (100%) or morphological anomaly of the tricuspid valve (67%). No patient died but more than half had non-cardiological sequelae. Veno-arterial ECMO support is not uncommon in critically ill neonates with premature DA closure and appeared to be associated with more pathological adaptation to extra-uterine life, greater need for vasoactive-inotropic support and higher frequency of bi-ventricular failure or morphological anomaly of the tricuspid valve. These parameters can help clinicians to identify neonates likely to require such an assistance.

Hybrid palliation to promote growth of left ventricle and left ventricular outflow tract.

OBJECTIVES: In patients with borderline left hearts or a severe left ventricular outflow tract obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study, we analysed growth of left-sided structures and outcomes of these patients. METHODS: We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analysed. RESULTS: In 38 patients, hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross-Konno/Yasui procedure, while 23 patients received conventional BVR. In patients with a conventional BVR, a significant increase was found in left ventricular volume indexed by body surface area, Z-score of aortic valve and left ventricular outflow tract between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up, only increase of the aortic valve Z-scores and left ventricular volume indexed by body surface area was found significant. Of all included patients (n = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, with no mortality in the conventional BVR group. CONCLUSIONS: Hybrid palliation as part of a staged BVR is a safe and effective initial step and promotes the growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed.