Suprasystemic Pulmonary Hypertension Research Articles

(870) - Intermediate-Term Outcomes of Reversed Potts Shunt in Children with Suprasystemic Pulmonary Arterial Hypertension: A Short Series of Three Chinese Patients

(870) - Intermediate-Term Outcomes of Reversed Potts Shunt in Children with Suprasystemic Pulmonary Arterial Hypertension: A Short Series of Three Chinese Patients

Hemolysis during Venovenous Extracorporeal Membrane Oxygenation in Neonates with Congenital Diaphragmatic Hernia: A Prospective Observational Study

To investigate the incidence of hemolysis and its association with outcome in neonates with congenital diaphragmatic hernia (CDH) requiring venovenous extracorporeal membrane oxygenation (ECMO) treatment using a Medos Deltastream circuit with a DP3 pump, a hilite 800 LT oxygenator system, and a ¼' tubing. Plasma free hemoglobin (PFH) was prospectively measured once daily during ECMO using spectrophotometric testing. Patients (n=62) were allocated into two groups according to presence or absence of hemolysis. Hemolysis was defined as PFH ≥ 50mg/dL on at least 2 consecutive days during ECMO treatment. Hemolysis was classified as either moderate with a maximum PFH of 50-100mg/dL or severe with a maximum PFH >100mg/dL. Hemolysis was detected in 14 patients (22.6%). Mortality was 100% in neonates with hemolysis compared with 31.1% in neonates without hemolysis (P<.001). In 21.4% hemolysis was moderate and in 78.6% severe. Using multivariable analysis, hemolysis (hazard ratio: 6.8; 95%CI: 1.86-24.86) and suprasystemic pulmonary hypertension (PH) (hazard ratio: 3.07; 95%CI: 1.01-9.32) were independently associated with mortality. Hemolysis occurred significantly more often using 8 French (Fr) cannulae than 13 Fr cannulae (43% vs 17%; P=.039). Cutoff for relative ECMO flow to predict hemolysis were 115ml/kg/ minute for patients with 8 Fr cannulae (Area under the curve [AUC] 0.786, P=.042) and 100ml/kg/ minute for patients with 13 Fr cannulae (AUC 0.840, P<.001). Hemolysis in CDH neonates receiving venovenous ECMO is independently associated with mortality.

Pulmonary Hypertension in Infants and Children with Vein of Galen Malformation and Association with Clinical Outcomes

To assess the extent and resolution of pulmonary hypertension (PH), cardiovascular factors, and echocardiographic findings associated with mortality in infants and children with vein of Galen malformation (VOGM). We performed a retrospective review of 49 consecutive children with VOGM admitted to Boston Children's Hospital from 2007 to 2020. Patient characteristics, echocardiographic data, and hospital course were analyzed for 2 cohorts based on age at presentation to Boston Children's Hospital: group 1 (age ≤60days) or group 2 (age >60days). Overall hospital survival was 35 of 49 (71.4%); 13 of 26 (50%) in group 1 and 22 of 23 (96%) in group 2 (P<.001). High-output PH (P=.01), cardiomegaly (P=.011), intubation (P=.019), and dopamine use (P=.01) were significantly more common in group 1 than group 2. Among patients in group 1, congestive heart failure (P=.015), intubation (P<.001), use of inhaled nitric oxide (P=.015) or prostaglandin E1 (P=.030), suprasystemic PH (P=.003), and right-sided dilation were significantly associated with mortality; in contrast, left ventricular volume and function, structural congenital heart disease, and supraventricular tachycardia were not associated. Inhaled nitric oxide achieved no clinical benefit in 9 of 11 treated patients. Resolution of PH was associated with overall survival (P<.001). VOGM remains associated with substantial mortality among infants presenting at ≤60days of life owing to factors associated with high output PH. Resolution of PH is an indicator associated with survival and a surrogate end point for benchmarking outcomes.

Intraoperative prophylactic right ventricular assist device in prevention of postcardiotomy failure.

In patients at risk of developing right ventricular failure after cardiac surgery, right ventricular support with a ventricular assist device may be a promising strategy to reduce mortality. We present our experience with intraoperative right ventricular assist device implantation as a prevention strategy of right ventricular failure after cardiac surgery. Between 2016 and 2022, we implanted four right ventricular assist devices prophylactically in a series of patients with surgical indication for valvular heart disease and high risk of postoperative right ventricular failure. Indications for the right ventricular assist device were suprasystemic pulmonary hypertension or severe right ventricular dysfunction. Externalization of the device cannulas through intercostal spaces was performed in three patients, allowing early mobilization and withdrawal without resternotomy. Removal of the device ocurred on the eighth postoperative day. ICU and hospital length of stay was 12 (±1.6) and 23 days (±4.2) respectively. Hospital mortality was null. No patient died during follow-up, mean follow-up was 32.5months [1-72]. Patients improved their NYHA functional class up to grade II during follow-up. Acute right ventricular failure after cardiac surgery remains a significant cause of morbidity and mortality. Prophylactic strategies to prevent postoperative right ventricular dysfunction may decrease the incidence of refractory postoperative right ventricular failure. We propose a novel approach to prevent right failure after cardiac surgery with prophylactic intraoperative ventricular assist device implantation.

Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension.

Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. To evaluate long-term clinical and procedural outcomes of TPS intervention. Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge (p < 0.001) and stayed better at the last visit (p < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional class across follow-up was significant (p < 0.001). There was, however, no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The median follow-up was 77.4 months (IQR, 70.7-113.4). Survival was 100% at 1 year and 92.3% at 6 years. Freedom from reinterventions was 77% at 1 year and 21% at 6 years. Nine (69.2%) patients had stent reinterventions at a median of 25 months (IQR, 9.5-56) postoperative. Balloon dilatation and restenting were performed in 53.8% and 46.2% of patients, respectively. High-pressure post-dilatation of implanted stents was performed in 53.8% of patients during TPS intervention for incomplete stent expansion and/or residual pressure gradient and was associated with higher rates of reinterventions (p = 0.021). Stent malfunctioning was present in 46.2% of patients at last follow-up. Two patients are listed for heart-lung transplantation. Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Six-year survival is, however, satisfactory.

Tricuspid regurgitation and left ventricular eccentricity as a measure of heart failure in the newborn patient with a vein of Galen malformation: illustrative case.

BACKGROUNDSuccessful management of a vein of Galen malformation (VoGM) in the newborn patient requires a highly coordinated team approach involving neonatologists, pediatric cardiologists, pediatric neurologists, neurosurgeons, and interventionalists. Indication and timing of catheter intervention are topics of ongoing debate.OBSERVATIONSThe authors highlighted two key echocardiographic markers believed to be practical indicators regarding the need for urgent catheter embolization in neonates with a VoGM. The first and preferred parameter was the tricuspid valve regurgitation (TR) gradient, an estimate of pulmonary artery hypertension. If the TR gradient exceeds systolic blood pressure (suprasystemic pulmonary hypertension [PH], i.e., >60 mm Hg), urgent intervention should be considered in eligible newborns. The second parameter was the left ventricular end-systolic eccentricity index (EI), a newly emerging echocardiographic marker and indirect correlate of PH. As an alternative to the TR gradient, an increased eccentricity index (>1.6) suggests severe right heart compromise, requiring emergency catheter embolization of the malformation. Postoperatively, the progressive reduction of both the TR gradient and the EI correlated with recovery.LESSONSIn eligible newborns, urgent embolization of a VoGM is recommended in the presence of suprasystemic TR gradients and/or increased EI >1.6.

Long-term outcomes of Transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension

Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge ( P < 0.001) and stayed better at the last visit ( P < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional status across follow-up was significant ( P < 0.001). There was no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The Median follow-up was 62.7 months (IQR, 32.2–96.5). Survival was 100% at 1 year and 92.3% at 5 years. Freedom from reinterventions was 77% at 1 year and 17% at 5 years. Eight (61.5%) patients had stent reinterventions at a median of 22 months (IQR, 8.8–49.8) postoperative. Balloon dilatation and restenting were performed in 46.1% and 38.5% of patients, respectively. Stent malfunctioning was present in 46.2% of patients at the last follow-up. One patient is listed for heart-lung transplantation. Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Five-year survival is however satisfactory.

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Multiscale modelling of Potts shunt as a potential palliative treatment for suprasystemic idiopathic pulmonary artery hypertension: a paediatric case study

Potts shunt (PS) was suggested as palliation for patients with suprasystemic pulmonary arterial hypertension (PAH) and right ventricular (RV) failure. PS, however, can result in poorly understood mortality. Here, a patient-specific geometrical multiscale model of PAH physiology and PS is developed for a paediatric PAH patient with stent-based PS. In the model, 7.6mm-diameter PS produces near-equalisation of the aortic and PA pressures and Q_p/Q_s (oxygenated vs deoxygenated blood flow) ratio of 0.72 associated with a 16% decrease of left ventricular (LV) output and 18% increase of RV output. The flow from LV to aortic arch branches increases by 16%, while LV contribution to the lower body flow decreases by 29%. Total flow in the descending aorta (DAo) increases by 18% due to RV contribution through the PS with flow into the distal PA branches decreasing. PS induces 18% increase of RV work due to its larger stroke volume pumped against lower afterload. Nonetheless, larger RV work does not lead to increased RV end-diastolic volume. Three-dimensional flow assessment demonstrates the PS jet impinging with a high velocity and wall shear stress on the opposite DAo wall with the most of the shunt flow being diverted to the DAo. Increasing the PS diameter from 5mm up to 10mm results in a nearly linear increase in post-operative shunt flow and a nearly linear decrease in shunt pressure-drop. In conclusion, this model reasonably represents patient-specific haemodynamics pre- and post-creation of the PS, providing insights into physiology of this complex condition, and presents a predictive tool that could be useful for clinical decision-making regarding suitability for PS in PAH patients with drug-resistant suprasystemic PAH.

Supra-systemic pulmonary hypertension after complicated percutaneous mitral balloon valvuloplasty: a case report and review of literature

BackgroundThe World Symposium of Pulmonary Hypertension in 2018, updated the definition of pulmonary hypertension (PH) as mean pulmonary artery pressures (PAP) > 20 mmHg. Pulmonary venous hypertension secondary to left-heart disease, constitutes the most common cause of PH, and the determination of a co-existent pre-capillary (primary) PH becomes paramount, particularly at the moment of evaluating and managing patients with heart failure. Pulmonary artery pressures above the systemic pressures define supra-systemic PH and generally leads to frank right ventricular failure and high mortality.Case presentationWe present the perioperative management of a patient with rheumatic mitral valve disease, initially found to have severe PH due to pulmonary venous hypertension, who underwent percutaneous mitral balloon valvuloplasty complicated with mitral chordae rupture, severe mitral regurgitation and supra-systemic PH. Multiple medical therapies and an intra-aortic balloon pump were used as means of non-surgical management of this complication.ConclusionsThis case report illustrates the perioperative implications of combined pre- and post-capillary PH and supra-systemic PH, as this has not been widely discussed in previous literature. A thorough literature review of the clinical characteristics of PH, methods to determine co-existent pre- and post-capillary PH components, as well as concomitant right ventricular failure is presented. Severe PH has known detrimental effects on the hemodynamic status of patients, which can ultimately lead to a decrease in effective cardiac output and poor tissue perfusion.

Anesthetic management for percutaneous reverse Potts shunt creation in children with refractory idiopathic pulmonary arterial hypertension: A case series.

Percutaneous reverse Potts shunt improves right ventricular function in patients with suprasystemic idiopathic pulmonary arterial hypertension. There are no data regarding the anesthesia in this high-risk procedure. We report our experience of the anesthetic management for the creation of percutaneous reverse Potts shunt in children with suprasystemic idiopathic pulmonary arterial hypertension. This study included 10 patients presenting with symptomatic idiopathic pulmonary arterial hypertension despite undergoing medical treatment. All patients underwent gradual induction of anesthesia to maintain hemodynamic stability (etomidate, n=8; ketamine, n=4). Four patients needed extracorporeal life support: 2 were rescued after cardiac arrest and 2 had elective extracorporeal life support due to preprocedural dysfunctional right ventricle and/or small left ventricle volumes with reduced cardiac output. All patients were admitted to the pediatric cardiac intensive care unit (4 [2-5] days). All patients with extracorporeal life support died. None of the six survivors needed pulmonary transplantation. Both ketamine and etomidate support hemodynamics. High-dose opioid technique has the advantage of blunting noxious stimuli and subsequent increase in pulmonary vascular resistance. We recommend using multimodal monitoring with transesophageal echocardiography. The 100% mortality of extracorporeal life support patients, probably too sick to undergo such procedure, may question its usefulness. Further studies should identify suitable candidates for percutaneous reverse Potts shunt creation.

Two cases of different genetic variants of alveolar capillary dysplasia associated with left-sided obstructive CHDs.

Alveolar capillary dysplasia with misalignment of the pulmonary veins is an uncommon disorder that affects the lung vasculature development in the neonatal period and leads to pulmonary hypertension. We describe two patients with alveolar capillary dysplasia associated with left-sided obstructive heart defects with two different genetic variants. Our cases highlight the importance of early recognition of this disease in the setting of persistent and supra-systemic pulmonary hypertension despite surgical correction of the associated lesions. Identification of these cases will facilitate the development of a multidisciplinary approach and provide guidance to the affected families.

Who are at high risk of mortality and morbidity among children with congenital heart disease undergoing noncardiac surgery?

With advances in the development of surgical and medical treatments for congenital heart disease (CHD), the population of children and adults with CHD is growing. This population requires multiple surgical and diagnostic imaging procedures. Therefore, general anesthesia is inevitable. In many studies, it has been reported that children with CHD have increased anesthesia risks when undergoing noncardiac surgeries compared to children without CHD. The highest risk group included patients with functional single ventricle, suprasystemic pulmonary hypertension, left ventricular outflow obstruction, and cardiomyopathy. In this review, we provide an overview of perioperative risks in children with CHD undergoing noncardiac surgeries and anesthetic considerations in patients classified as having the highest risk.

Midterm outcomes of the Potts shunt for pediatric pulmonary hypertension, with comparison to lung transplant

ObjectiveFor children with severe pulmonary hypertension, addition of Potts shunt to a comprehensive palliation strategy might improve the outcomes afforded by medications and delay lung transplantation. MethodsA prospective analysis was conducted of all children undergoing Potts shunt (first performed in 2013) or bilateral lung transplant for pulmonary hypertension from 1995 to present. ResultsA total of 23 children underwent Potts shunt (20 surgical, 3 transcatheter), and 31 children underwent lung transplant. All children with Potts shunt had suprasystemic right ventricle pressures despite maximal medical treatment. In the majority of patients, the Potts shunt was performed through a left thoracotomy approach (90%, 18/20), by direct anastomosis (65%, 13/20), and without the use of extracorporeal support (65%, 13/20). Perioperative outcomes after Potts shunt were superior to lung transplant including mechanical ventilation time (1.3 vs 10.2 days, P = .019), median hospital length of stay (9.8 vs 34 days, P = .012), and overall complication rate (35% [7/20] vs 81% [25/31], P = .003). Risk factors for operative mortality after Potts shunt (20%, 4/20; compared with 6%, 2/31 for lung transplant, P = .195) included preoperative extracorporeal membrane oxygenation and significant right ventricle dysfunction. In midterm follow-up (median 1.8, maximum 6.1 years), patients with Potts shunt had durable equalization of right ventricle/left ventricle pressures and improved functional status. There was no significant survival difference in patients with Potts shunt and patients with lung transplant (P = .258). ConclusionsPotts shunt is an effective palliation for children with suprasystemic pulmonary hypertension that may become part of a strategy to maximize longevity and functional status for these challenging patients.

Potts anastomosis in children with severe pulmonary arterial hypertension and atrial septal defect.

AimsPotts shunt has been proposed as a bridge or alternative to lung transplantation for children with severe and drug‐refractory suprasystemic pulmonary arterial hypertension (PAH). We describe the management of the atrial shunt when a Potts shunt is planned in refractory PAH.Methods and resultsWe report a case series of children in whom a Potts shunt was done for severe PAH associated with an atrial septal defect to illustrate the different clinical and haemodynamic scenarios. Five children (2 to 13 years) underwent a Potts shunt: three surgical, one percutaneous Potts shunt, and one percutaneous stenting of a restrictive arterial duct. All had associated atrial septal defect. Those who had generalized cyanosis before the procedure had a complicated postoperative course and required longer ventilatory and inotropic support, except the one who had atrial septal defect closure before the Potts shunt. One of the three cyanotic patients died. Two patients with left‐to‐right shunt before the Potts shunt had an uncomplicated postoperative course.ConclusionsShunt physiology is only partially predictable after the Potts shunt in children with PAH and atrial septal defect. Abrupt drop in left ventricle preload while the right ventricle is decompressed can potentially be prevented by atrial septal defect closure prior to the Potts shunt.

Transcatheter creation of a Potts shunt with the Occlutech Atrial Flow Regulator: Feasibility in a pig model

BackgroundCreation of a Potts shunt, a connection between the left pulmonary artery (LPA) and descending aorta (DAo), improves functional status and survival in drug-refractory suprasystemic pulmonary arterial hypertension. We investigated a new approach to transcatheter Potts shunt creation in pigs. Methods and resultsIn six pigs, a steerable SureFlex sheath was used to optimize the trajectory of perforation from the DAo into LPA using a 0.035″ radiofrequency wire. The combination of a larger perforation, stiffer radiofrequency wire and smooth dilator-to-sheath transition allowed sheath entry into the LPA without requiring an arterio-venous wire circuit. The Occlutech Atrial Flow Regulator (AFR), a double-disc device with a central fenestration, was deployed through this sheath with apposition of the distal disc to the posterior LPA wall and the proximal disc to the anterior DAo wall. The AFR is compliant and crumpling of the central fenestration was resolved by balloon dilation. It was feasible to implant a stent within the fenestration (n = 3). Aortography confirmed a left-to-right shunt through the AFR without contrast extravasation. Autopsy demonstrated anchoring of both discs against the vessel walls, patency of the fenestration and secure placement of the stent with no intra-thoracic bleeding. ConclusionsIn an acute pig model, we have demonstrated the feasibility of creating a transcatheter Potts shunt with a simplified technique using a steerable sheath, a double-disc device with a central fenestration that acts as the shunt channel and optional stenting of the fenestration.

A case series of transcatheter Potts Shunt creation in a pediatric population affected with refractory pulmonary artery hypertension: focus on the role of ECMO.

Patients with suprasystemic idiopathic pulmonary hypertension (S-PAH) have a poor prognosis. Therapeutic options are limited. Reverse Potts shunt creation modifies physiology transforming patients with PAH into Eisenmenger physiology with a better outcome. Percutaneous transcatheter stent secured aortopulmonary connection (transcatheter Potts Shunt, TPS) is a feasible very high-risk procedural option in such patients. We report our experience with patients undergoing TPS at our institution requiring extracorporeal membrane oxygenation (ECMO) support. A prospective observational study of patients with drug-refractory PAH, worsening NYHA class, and right ventricular failure undergoing TPS. Two patients required rescue ECMO for cardiac arrest during the procedure. Subsequently, "standby ECMO" was available in all the following cases and elective support was provided in patients with extremely poor conditions. Ten pediatric patients, underwent TPS at our institution. Two patients were rescued by ECMO after cardiac arrest during the shunt creation. This occurred as a result of the acute loading of the left ventricle (LV) after retrograde aortic arch filling through the Potts shunt. Following this, another two patients underwent elective ECMO after the uneventful induction of anesthesia. They all died postoperatively despite a successful TPS procedure. The causes of death were not related to the use of ECMO, but the complication of severe PAH. Six patients with successful TPS did not require ECMO and survived. TPS is a pioneering procedure offering the opportunity to treat high-risk idiopathic drug-refractory PAH patients. Acute LV failure is a complication of TPS in patients with S-PAH. Elective ECMO, an option to avoid circulatory arrest and acute profound hypoxia secondary to exclusive right-to left shunt systemic perfusion by Potts shunt and LV dysfunction with resulting pulmonary edema, may be used at the early stage of the learning curve, but it does not influence the prognosis of these patients which remains poor.

Congenital diaphragmatic hernia: postnatal predictors of mortality.

Congenital diaphragmatic hernia (CDH) prevalence is low while its associated morbidity and mortality rates are high. Postnatal prognostic factors on the first day of life are useful for predicting the outcome. To determine the mortality predictive ability of postnatal echocardiographic, clinical, and biochemical factors among newborn infants with CDH in their first day of life. Observational analytical study of a retrospective cohort. Patients with CDH were consecutively included between March 2012 and November 2018. On the first day of life, analyzed predictors were the oxygenation index (OI), the highest partial pressure of carbon dioxide (pCO2) level in blood, the SNAPPE II severity score, the echocardiography, and the N-terminal pro-B-type natriuretic peptide (NTproBNP) value. The population consisted of 178 patients with CDH. Survival was 75 %. Extracorporeal membrane oxygenation was used in 24 %. The early onset of systemic or suprasystemic pulmonary hypertension showed no predictive ability (OR: 2.2, 95 % CI: 0.8-8), p = 0.1. NT-proBNP did not show good discrimination either (area under the curve [AUC]: 0.46, p = 0.67). The OI, SNAPPE II score, and the highest pCO2 level showed adequate discrimination power, AUC for OI: 0.82, AUC for SNAPPE II: 0.86, and AUC for pCO2: 0.75, p < 0.001. The SNAPPE II score, the OI, and the highest pCO2 level measured on the first day of life, showed a good predictive ability in terms of the course of the disease; the SNAPPE II score was better than the OI and the highest pCO2 level.

Prostaglandin E1 in infants with congenital diaphragmatic hernia (CDH) and life-threatening pulmonary hypertension

BackgroundSuprasystemic pulmonary hypertension (PH) is highly predictive of pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH). ObjectivesTo report the effects and tolerability of Prostaglandin E1 (PGE1) in newborns with severe CDH and life-threatening PH. MethodsNewborn infants with isolated CDH and life-threatening PH defined by an acute worsening of the cardiorespiratory function, and bidirectional or exclusive right-to-left shunting across the ductus arteriosus (DA) with an acceleration of the blood flow >1.5 m.s−1 assessed by Doppler echocardiography. Serial measurements of cardiorespiratory variables have been recorded before and after PGE1. Results18 infants (out of 102 in the cohort) were included in the study (gestational age: 39 ± 2 weeks). The median FiO2, and preductal and postductal SpO2 were 80% [50; 100], 91% [88; 95] and 86% [82; 91], respectively, before treatment. FiO2 decreased to 35% [30–40] (p = 0.001) at H6. Maximal blood flow velocities in the DA decreased after starting PGE1 from 2.2 m.s−1 [1.5–2.5] to 1 m.s−1 [0.55–1.2] (p < 0.001). ConclusionsPGE1 treatment improved oxygenation and circulatory function in newborn infants with severe CDH and life-threatening PH. Our data provide evidence that restrictive DA may result in suprasystemic pulmonary hypertension in CDH infants, and that PGE1 may improve cardiorespiratory failure through reopening of the DA. Type of studyTreatment study. Level of evidenceLevel III.

Long-term outcome of vein of Galen malformation.

To describe the long-term outcomes of children by the time they reached school age with vein of Galen aneurysmal malformation (VGAM). This was a retrospective observational study on a consecutive cohort of patients with VGAM. We included patients with at least one Francophone parent, aged between 6 and 11 years at the time of long-term evaluation. The neurological outcome was assessed with the King's Outcome Scale for Childhood Injury score and eight neurological and behavioural items from the Rivermead Postconcussion Symptoms questionnaire. All 52 patients (17 females, 32 males [data missing for n=3]) with at least one Francophone parent (5 fetuses and 47 children) were included. At the long-term evaluation time-point, 33 patients were alive and 19 patients had died. Risk of postnatal death was associated with severe neonatal cardiac failure (p=0.007) or isosystemic or suprasystemic pulmonary hypertension (p=0.014). Among survivors, 19 had a good outcome with normal schooling and 14 had a poor outcome. Moreover, among the good outcome patients, a large proportion had neurodevelopmental alterations. Long-term outcome of patients with VGAM appears to be less favourable than outcome described at the short- and medium-term, even in the absence of encephalomalacia at birth. Even patients with good outcome often have neuropsychological disorders that may have repercussions on learning and requiring appropriate rehabilitation or medical management. Long-term outcome appears to be less favourable than described at short- and medium-term follow-up. Even patients with good outcome at these time-points often have minor neuropsychological disorders.