The question of screening organ donors for hepatitis e virus: a case report of transmission by kidney transplantation in France and a review of the literature.

BACKGROUND: Hepatitis E is a potentially serious infection in organ recipients, with an estimated two-thirds of cases becoming chronic, and with a subsequent risk of cirrhosis and death. In Europe, transmission occurs most often through the consumption of raw or undercooked pork, more rarely through blood transfusion, but also after solid organ transplantation. Here we describe a case of Hepatitis E virus (HEV) infection transmitted following kidney transplantation and review the literature describing cases of HEV infection transmitted by solid organ transplantation. CASE PRESENTATION: Three weeks after kidney transplantation, the patient presented with an isolated minimal increase in GGT and hepatic cytolysis 6 months later, leading to the diagnosis of genotype 3c hepatitis E, with a plasma viral load of 6.5 log10IU/mL. In retrospect, HEV RNA was detected in the patient's serum from the onset of hepatitis, and in the donor's serum on the day of donation, with 100% identity between the viral sequences, confirming donor-derived HEV infection. Hepatitis E had a chronic course, was treated by ribavirin, and relapsed 10 months after the end of treatment. DISCUSSION: Seven cases of transmission of HEV by solid organ transplantation have been described since 2012 without systematic screening for donors, all diagnosed at the chronic infection stage; two patients died. HEV organ donor transmission may be underestimated and there is insufficient focus on immunocompromised patients in whom mild liver function test impairment is potentially related to hepatitis E. However, since HEV infection is potentially severe in these patients, and as evidence accumulates, we believe that systematic screening of organ donors should be implemented for deceased and living donors regardless of liver function abnormalities, as is already the case in the UK and Spain. In January 2024, the French regulatory agency of transplantation has implemented mandatory screening of organ donors for HEV RNA.

Imaging and surgical management of congenital heart diseases.

Congenital heart disease affects approximately 1% of live births per year. In recent years, there has been a decrease in the morbidity and mortality of these cases due to advances in medical and surgical care. Imaging plays a key role in the management of these children, with chest radiography, echocardiography and chest ultrasound the first diagnostic tools, and cardiac computed tomography, catheterization and magnetic resonance imaging reserved to assess better the anatomy and physiology of the most complex cases. This article is a beginner's guide to the anatomy of the most frequent congenital heart diseases (atrial and ventricular septal defects, abnormal pulmonary venous connections, univentricular heart, tetralogy of Fallot, transposition of the great arteries and coarctation of the aorta), their surgical management, the most common postsurgical complications, deciding which imaging modality is needed, and when and how to image gently.

Early Presentation of Patients with Abnormal Origin of Left Coronary Artery from the Pulmonary Artery is a Predictor of Poor Mid-term Outcomes.

Abnormal origin of left coronary artery from the pulmonary artery (ALCAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. This study aimed to determine the correlation between age at clinical presentation, level of ventricular dysfunction, and post-repair outcomes. This is retrospective study from 1993 to 2018 including thirty-one patients. The study cohort was divided into two groups according to age (< 6 months, > 6 months). The significance level was set at p 0.05.The median follow-up time was 72 [24-168] months. Median age was 4.7 [2.3-16] months. Median weight was 6.2 [4.3-9] kg. There was severe left ventricular (LV) dysfunction (ejection fraction < 35%) in 64.5% of patients. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Two patients (6.4%) required extracorporeal membrane oxygenation (ECMO) support before surgery and 6 (19.4%) after correction. Age < 6 months was significantly associated with severe clinical presentation, severe LV dysfunction, delayed sternal closure, prolonged respiratory mechanical support, and prolonged length of ICU stay (p = 0.024, p = 0.042, p = 0.002, p = 0.042, p = 0.022, respectively). After surgery, ejection fraction improved to a median of 57% [50.7-60.5]. MR regressed in 12 patients (92.3%). Mortality rate after surgery was 9.7%. All patients were free from reoperation at the last follow-up. Young age at diagnosis was significantly associated with a more severe clinical presentation and poorer outcomes. After re-establishment of a two-coronary circulation, both ventricular function and MR tend to normalize over time regardless of age at repair.

Berlin Heart EXCOR Paediatric Ventricular Assist Device: Does Weight Matter?

BACKGROUND: Berlin Heart EXCOR (BH) ventricular assist devices provide mechanical long-term circulatory support in children with end-stage heart failure, as a bridge to transplantation or to recovery. Most studies are from large-volume paediatric cardiac centres. AIM: The aim of this study was to analyse the experiences of three French centres and to compare these with available published data. METHOD: We performed a retrospective observational study of three paediatric cardiac intensive care units. All children supported with BH devices were included. Morbidity and mortality data were collected and risk factors analysed. RESULTS: Fifty-four (54) patients (54% male) were included. Survival rate was 73% while on a BH device. Median age at BH device implantation was 17 months (range 2-180 months). The predominant indication was dilated cardiomyopathy (61%). Bi-ventricular assist device was used in 25 (46%) cases. The total length of long-term circulatory support was 3,373 days, with a mean length per patient of 62.5 days (range 5-267 days). Thirty-two (32) patients were transplanted (59%) and seven (13%) were successfully weaned. Type and length of support did not influence morbidity. Main complications were renal dysfunction (57%), bleeding (41%), and infection (39%). In multivariate analysis, a weight <5 kg was significantly associated with higher mortality. CONCLUSIONS: The weight seems to be the most important risk factor of mortality in this precarious condition.

Pulmonary atresia with ventricular septal defect and tetralogy of Fallot: transannular path augmentation versus systemic to pulmonary artery shunt for first-stage palliation.

BACKGROUND: Pulmonary atresia with ventricular septal defect and severe tetralogy of Fallot require a palliative procedure for pulmonary artery rehabilitation. For first-stage palliation, two main surgical options are still debated: right ventricle to pulmonary artery connection and modified Blalock-Taussig shunt. We compared the clinical outcomes of the two procedures. METHODS: From 1995 to 2018, 88 patients needed palliation (pulmonary atresia with ventricular septal defect n = 47; tetralogy of Fallot n = 41). Among these patients, 70 modified Blalock-Taussig shunt and 18 transannular path augmentation were performed before 6 months of age. Using a 1:1 propensity score match analysis, 20 patients were included in the analysis. The primary outcome was in-hospital mortality and pulmonary artery growth. RESULTS: After matching, the pre-operative Nakata was smaller in transannular path augmentation 54 ± 24 mm2/m2 than modified Blalock-Taussig shunt 109 ± 31 mm2/m2 (p < 0.001). The age and weight were similar (p = 0.31 and p = 0.9, respectively). There was no difference in in-hospital mortality (p = 0.3). The Nakata index before biventricular repair and delta Nakata were smaller in modified Blalock-Taussig shunt group (206 ± 80 mm2/m2, 75 ± 103 mm2/m2) than transannular path augmentation (365 ± 170 mm2/m2, 214 ± 165 mm2/m2; p = 0.03; p < 0.001). Median time to biventricular repair was similar (p = 0.46). The rate of interstage reintervention was similar (p = 0.63). CONCLUSIONS: The transannular path augmentation is better for the rehabilitation of the native pulmonary artery. Despite a smaller pulmonary artery, right ventricle to pulmonary artery connection is equivalent to modified Blalock-Taussig shunt for rate of biventricular repair and time to biventricular repair.

Can computed tomodensitometry predict ventricular pseudoaneurysm occurrence after heart valve endocarditis?

Ventricular pseudoaneurysm is a rare but well-known complication after valvular endocarditis. The lesion was localised exactly where pre-operative CT scan showed lack of enhancement in the postero-lateral wall of the left ventricle. This case demonstrates how much attention must be paid to myocardial CT images and emphazises the need of close follow-up in patients with infective endocartitis.

Impact of education and training course for ECMO patients based on high-fidelity simulation: a pilot study dedicated to ICU nurses.

INTRODUCTION: Medical and para-medical education is one of the key points of healthcare strategy. Training and education based on high-fidelity simulation is one of the gold standards in modern healthcare institutions. We describe a model of training dedicated to ICU nurses in charge of patients with ECMO. The aim of our educational tool was to teach ICU nurses ECMO basic knowledge and skills. METHODS: An ECMO Specialist Course Committee implemented the training programme. It was on two consecutive days and consisted of theoretical, practical and high-fidelity, simulation-based teaching. A content expert implemented each scenario and learning objectives were defined. Participants were assessed pre- and post-test (Group 1 and Group 2). RESULTS: In two years, seven sessions took place and 40 volunteers were enrolled. High-fidelity, simulation-based teaching consisted of seven scenarios. There was a significant improvement in mean score between pre- and post-test. Moreover, we noticed that the basic level (pre-test) of participants was improving over the time. The mean pre-test scores of Group 2 were significantly higher than Group1. CONCLUSION: The implementation of education and training course for ICU nurses in charge of patients on ECMO is feasible and reliable. It improves nurse personal levels, but also shares in improving the global level of the team to which they belong.

Mobile extracorporeal membrane oxygenation for children: single-center 10 years' experience.

OBJECTIVES: Extracorporeal membrane oxygenation has become a gold standard in treatment of severe refractory circulatory and/or pulmonary failure. Those procedures require gathering of competences and material. Therefore, they are conducted in a limited number of reference centers. Emergent need for such treatments induces either hazardous transfers or a mobile pediatric extracorporeal membrane oxygenation team able to remote implantation and transportation. The aim of this work is not to focus on pediatric extracorporeal membrane oxygenation outcomes or indications, which have been extensively discussed in the literature. This study would like to detail the implementation, safety, and feasibility, even in a middle-size pediatric cardiac surgery reference center. PATIENTS: This is a retrospective analysis of a series of patients initiated on extracorporeal membrane oxygenation in a peripheral center and transferred to a reference center. The data were collected from 10 consecutive years: from 2006 to 2016. RESULTS: A total of 57 pediatric patients with a median weight of 6.00 (3.2-14.5) kg and median age of 2.89 (0.11-37.63) months were cannulated in peripheral center and transported on extracorporeal membrane oxygenation. We did not experience any adverse event during transport. The outcomes were comparable to our literature-reported on-site extracorporeal membrane oxygenation series with 42 patients (74%) weaned from extracorporeal membrane oxygenation and a 30-day survival of 60%. Neither patient's age nor weight, indication for extracorporeal membrane oxygenation or length of transport, was statistically significant in terms of outcomes. CONCLUSION: Offsite extracorporeal membrane oxygenation implantation and ground or air transport for pediatric patients on extracorporeal membrane oxygenation appeared to be safe when performed by a dedicated and experienced team, even within a mid-size center.

Outcome Comparison in Children Undergoing Extracorporeal Life Support Initiated at a Local Hospital by a Mobile Cardiorespiratory Assistance Unit or at a Referral Center.

PURPOSE: To compare characteristics and outcome in children undergoing extracorporeal life support initiated in an extracorporeal life support center or at the patient's bedside in a local hospital, by means of a mobile cardiorespiratory assistance unit. METHODS: A retrospective study in a single PICU during 6 years. Extracorporeal life support was started either in our center (control group) or in the local hospital (mobile cardiorespiratory assistance unit group). The data collected were demographics, markers of patient's preextracorporeal life support condition, and outcome. RESULTS: One hundred twenty-six children underwent extracorporeal life support, 105 in the control group and 21 in the mobile cardiorespiratory assistance unit group. There was no difference between groups in terms of age, weight, or Pediatric Risk of Mortality II score. There was a significant difference in organ failure etiology between groups, with more respiratory cases in the mobile cardiorespiratory assistance unit group (76.2%) and more cardiac surgery cases in the control group (60%; p < 0.001). The duration of extracorporeal life support was longer in the mobile cardiorespiratory assistance unit group than in the control group (10 [1-36] vs 5 [0-33] d; p = 0.003). PICU length of stay and mortality (60% vs 47.6%; p = 0.294) were not significantly different between the two groups. To allow comparison of a more homogenous population, a subgroup analysis was performed including only respiratory failure patients from the two groups (R-control group [n = 22] and R-mobile cardiorespiratory assistance unit group [n = 16]). PICU length of stay was 17 (3-64) days in the R-control group and 23 (1-45) days in the R-mobile cardiorespiratory assistance unit group (p = 0.564), and PICU mortality rate was 54.5% in the R-control group and 43.8% in the R-mobile cardiorespiratory assistance unit group (p = 0.511). There was no difference between the R-groups for age, weight, Pediatric Risk of Mortality II score, and markers of kidney or liver dysfunction, and lactate blood levels. CONCLUSION: Extracorporeal life support can be safely initiated at children's bedside in the local hospital and then transported to the specialized referral center. Our results support the validity of an interregional organization of mobile cardiorespiratory assistance unit teams.

Risk of infective endocarditis after hybrid melody mitral valve replacement in infants: the French experience.

OBJECTIVES: Surgical management of mitral valve disease is challenging in infants <1 year old. We aimed at reviewing the French experience with Melody mitral valve replacement in critically ill infants. METHODS: A retrospective cohort study reporting the French experience with Melody mitral valve replacement. RESULTS: Seven symptomatic infants [complete atrioventricular septal defect (n = 4, Down syndrome: n = 3), hammock valve (n = 3)] underwent Melody mitral valve replacement [age: 3 months (28 days to 8 months), weight: 4.3 kg (3.2-6.4 kg)] because of severe mitral valve regurgitation (6) or mixed valve disease (1) and 14 mm (11-16 mm) mitral valve annulus. In 2 patients whose valve was felt irreparable, Melody mitral valve replacement was performed straightaway. The others underwent 2 (1-3) previous attempts of valve repair; 3 were on extracorporeal membrane oxygenation. Melody mitral valve replacement led to competent valve and low gradient [3 mmHg, (1-4 mmHg)]. One patient died 3 days post-implant from extracorporeal membrane oxygenation-related stroke. Of the 6 discharged home patients, 3 (50%) were readmitted for a definite diagnosis (1) or high suspicion (2) of infective endocarditis, of which 2 died. Over the follow-up, 1 underwent balloon expansions of the valve at 9- and 16-months post-implant, and mechanical mitral valve replacement at 2 years; another is currently planned for transcatheter Melody valve dilation. CONCLUSIONS: Melody mitral valve replacement may be considered in selected infants with small mitral valve annulus as an alternative to mechanical mitral valve replacement. Our experience highlights a high-risk of late infective endocarditis that deserves further consideration.

Stenting right ventricular outflow in an infant with tetralogy of Fallot and well-developed pulmonary arteries.

A right ventricular outflow tract stent was implanted in a 1-month-old (3.5 kg) baby boy with tetralogy of Fallot and worsening cyanosis to relieve infundibular and pulmonary valve stenosis in the setting of well-developed pulmonary artery branches. This management allowed a symmetric and optimal growth of the pulmonary artery branches, with right and left pulmonary arteries measuring 7-mm (z-score, +1.3 SD) and 7.3-mm (z-score, +1.7 SD), respectively, 3 months after stent implantation. No signs of pulmonary overcirculation developed, and complete surgical repair was performed at the age of 6 months.

Morphological analysis of ventricular septal defect by echocardiography for prediction of aortic regurgitation in pediatric population.

Ventricular septal defects (VSD) are the most common congenital heart diseases in children. Among them, perimembranous VSD (pm-VSD) have a higher risk of complications, including aortic valve prolapse and aortic regurgitation (AR). The aim of our study was to assess echocardiographic criteria associated with AR during follow-up of pm-VSD. Forty children with restrictive pm-VSD, followed-up in our unit and who underwent a workable echocardiographic evaluation between 2015 and 2019 were included and retrospectively analyzed. The propensity score was used to match 15 patients with AR to 15 patients without AR. Median age was 2.2 year [1.4-5.7]. Median weight was 14 kg [9.9-20.3]. Aortic annulus z-score, Valsalva sinus z-score, sinotubular junction z-score, valve prolapse and commissure commitment were significantly different between the two groups (p = 0.047, p = 0.001, p = 0.010, p = 0.007, p < 0.001 respectively). Aortic root dilatation, aortic valve prolapse and commissure commitment to a perimembranous VSD are associated to aortic regurgitation.

Right Ventricular Outflow Tract Reconstruction in Truncus Arteriosus: A 30-Year Two-Center Comparison between Homografts and Bovine Jugular Vein.

INTRODUCTION: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. METHODS: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. RESULTS: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). CONCLUSION: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

Impact of cardiac surgical timing on the neurodevelopmental outcomes of newborns with Complex congenital heart disease (CHD).

Background: More than half of infants with complex congenital heart disease (CHD) will have a neurodevelopmental disorder of multifactorial causes. The preoperative period represents a time-window during which neonates with complex CHD are in a state of hypoxia and hemodynamic instability, which fosters the emergence of brain injuries and, thus, affects early brain networks and neurodevelopmental outcomes. Currently, there is no consensus regarding the optimal age for cardiac surgery in terms of neurodevelopmental outcomes, and its definition is a real challenge. Our aim is to determine the relationship between cardiac surgical timing and long-term neurodevelopmental outcomes for various types of complex CHD. Methods: We hypothesize that earlier surgical timing could represent a neuroprotective strategy that reduces perioperative white matter injuries (WMIs) and postoperative morbidity, leading to improved neurodevelopmental outcomes in infants with complex CHD. Firstly, our prospective study will allow us to determine the correlation between age at the time of surgery (days of life) and neurodevelopmental outcomes at 24 months. We will then analyze the correlation between age at surgery and (i) the incidence of WMIs (through pre- and postoperative MRIs), (ii) postoperative morbidity, and (iii) the duration of the hospital stay. Implications and Dissemination: This research protocol was registered in the Clinical Trial Registry (National Clinical Trial: NCT04733378). This project aims to help launch the first Neurocardiac Investigation Clinic in Marseille - AP-HM - to propose an overall personalized monitoring and treatment program for patients operated on for complex CHD.

Severe aortic valve regurgitation after percutaneous ventricular septal defect closure.

Percutaneous closure of ventricular septal defect is now commonly used. We report a rare case, and especially a rare image of aortic leaflet damage caused by percutaneous ventricular septal device.

The Lugones Procedure for Surgical Repair of Scimitar Syndrome: Preserved Growth in a Young Infant.

Surgical repair of Scimitar syndrome is challenging, especially in small patients. Our images demonstrate that the pericardial tunnel technique is feasible even in low-weight patient and that it provides a good growth potential.

Anterior Minithoracotomy vs. Transcatheter Closure of Patent Ductus Arteriosus in Very Preterm Infants.

Introduction: Patent ductus arteriosus (PDA) is common in preterm infants and contributes to morbidity and mortality. Several studies have shown the feasibility and safety of percutaneous PDA closure. Minimally invasive surgical ligation by anterior thoracotomy is an alternative, bedside technique for PDA closure in very low birth weight preterm infants. Our study aimed to compare short- and medium-term morbidity and mortality between anterior minithoracotomy and transcatheter PDA closure. Methods: From 2010 to 2020, 92 preterm infants <1,600 g underwent PDA closure in two centers: 44 surgical anterior minithoracotomies (center 1) and 48 transcatheter closures (center 2). Using a 1:1 propensity score match analysis, 22 patients in each group were included. The primary outcome was time to extubation after intervention. Results: Preoperative characteristics were similar in both groups after propensity matching (mean weight at procedure, 1,171 ± 183 g; p = 0.8). Mean time to extubation was similar: 10 ± 15 days in the surgical group vs. 9 ± 13 days in the transcatheter group (p = 0.9). Mean age at hospital discharge was 114 ± 29 days vs. 105 ± 19 days (p = 0.2). Two deaths occurred in the surgical group and one in the transcatheter group (p = 0.61). Five complications (pneumothorax n = 2, chylothorax n = 2, phrenic nerve injury n = 1) occurred in three patients after surgery. Three complications (chylothorax n = 1, endocarditis n = 1, renal vein thrombosis n = 1) occurred in two patients after percutaneous closure (p = 0.63). Conclusion: Equivalent efficiency and safety of surgical mini-invasive vs. transcatheter PDA closure in preterm infants <1,600 g are in favor of applying these alternative techniques according to centers' facilities and competences.

Quality of life of transplanted children and their parents: a cross-sectional study.

BACKGROUND: Transplantation is a saving therapeutic that has heavy consequences. The quality of life (QoL) of transplanted children and their parents has been little studied and should help physicians better manage these patients. The objectives of the study were to assess: (1) the QoL of transplanted children and parents and compare it with that of children with other chronic conditions associated with long-term consequences, and (2) potential variables modulating the QoL. METHODS: This cross-sectional study was performed in a multidisciplinary paediatric unit (Timone Hospital, Marseille, France). Children were less than 18 years old; had a liver, kidney or heart transplant; and had a time since transplantation of 1-10 years. Socio-demographics and clinical data were recorded from medical forms. The QoL was assessed using the VSP-A (Vécu et Santé Perçue de l'Adolescent et de l'Enfant) and the WhoQoL self-reported questionnaires. RESULTS: Forty-five families were included (response rate: 76%). The transplanted organs were the liver for 20 children, the kidney for 15 children, and the heart for 10 children. The QoL of transplanted children reported by their parents was better than that of children with inborn errors of metabolism and similar to that of childhood leukaemia survivors. The QoL of parents of transplanted children was better than that of parents of children with inborn errors of metabolism and did not differ from French norms. The QoL did not differ according to the nature of the transplanted organ, sex or the main sociodemographic data. The main modulators decreasing QoL were residual treatment level, medications switch and the presence of another regular treatment. CONCLUSION: Transplanted children and their families reported a fairly preserved QoL compared to children with other chronic health conditions. Special attention should be given to QoL modulators related to therapeutic management (medication switches, regular treatments) that might be amenable to improve the QoL. Trial registration Ethics committee of Aix-Marseille University, France (reference number: 2014-08-04-03, 24/4/2015; https://www.univ-amu.fr/fr/public/comite-dethique ).

Anomalous Left Coronary Artery Connected to the Pulmonary Artery in a 15-Year-Old Girl: Case Report and Discussion on Secondary Prevention of Sudden Death.

Background. Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) is a rare congenital heart disease. Adaptive development of sufficient heterocoronary collaterality in the newborn may allow survival to a later age. In older children or adults, malignant ventricular arrhythmias can reveal the disease. Case Report. A 15-year-old girl was referred to the local hospital after a resuscitated out-of-hospital cardiac arrest. CT scan and coronary angiography revealed an ALCAPA. Direct aortic reimplantation of the left coronary artery was performed. Postoperative ECG monitoring showed short episodes of nonsustained ventricular tachycardia. Transthoracic echocardiography and cardiac MRI revealed subendocardial fibrosis of the anterolateral papillary muscle. Beta-blockade therapy was initiated at first intention. After hospital discharge, the patient reported several fainting without loss of consciousness. Considering sudden death nonrelated to effort, episodes of nonsustained ventricular tachycardia, and areas of myocardial fibrosis, the patient underwent subcutaneous cardioverter-defibrillator implantation. 6-month follow-up is satisfactory without clinical or rhythmic abnormalities. Discussion. Indication for surgical correction of ALCAPA is well defined, but rhythmic secondary prevention after resuscitated cardiac arrest is less consensual. Cardiac MRI is an essential tool in the identification of a potential rhythmic substrate and should be taken into account in the discussion of a preventive cardioverter-defibrillator implantation.

Anterograde blood flow associated with modified Blalock-Taussig shunt does not modify pulmonary artery growth compared with modified Blalock-Taussig shunt alone.

BACKGROUND: The main difference between extreme tetralogy of Fallot (TOF) and pulmonary atresia with ventricle septal defect (PA/VSD) is anterograde pulmonary blood flow (APBF). It is speculated that the association of modified Blalock-Taussig shunt (mBTS) with APBF favours shunt thrombosis, but promotes better pulmonary artery growth. AIM: To compare pulmonary artery growth after mBTS between TOF and PA/VSD. METHODS: From 1995 to 2018, 77 mBTS procedures were performed in infants (aged<1 year): 45 for TOF; 32 for PA/VSD. Using a 1/1 propensity score-matched analysis, 38 patients were included (19 per group). Delta Nakata was defined as the difference in the Nakata index before biventricular repair and before mBTS. RESULTS: After matching, the preoperative Nakata index was similar in the two groups (TOF 101±34 vs. PA/VSD 106±35 mm2/m2; P=0.75). Age and weight were similar (TOF 24±20 days, 3.3±0.6kg vs. PA/VSD 24±33 days, 3.3±0.9kg; P=0.84 and P=0.77, respectively). There was no difference in rates of in-hospital mortality (TOF 0% vs. PA/VSD 10%; P=0.13) or mBTS thrombosis (TOF 15% vs. PA/VSD 10%; P=0.63). The left and right pulmonary artery diameters at time of biventricular repair were similar (TOF 7.5±2.2 and 6.7±2.1 vs. PA/VSD 8±2.7 and 7.1±2.5mm; P=0.43 and P=0.78, respectively), as were delta Nakata (TOF 112±102 vs. PA/VSD 107±66 mm2/m2; P=0.89), median age for biventricular repair (P=0.83) and reintervention rates (TOF 10% vs. PA/VSD 15%; P=0.67). CONCLUSIONS: We found no difference in pulmonary artery growth between APBF with mBTS versus mBTS alone. Thus, we could not show an increase in mBTS thrombosis with APBF.