Neurodesarrollo a los 2 años en cardiopatía congénita: utilidad pronóstica de los marcadores de daño cerebral / Neurodevelopmental outcomes in congenital heart disease: Usefulness of biomarkers of brain injury

Introducción: En la actualidad, las alteraciones del neurodesarrollo son la complicación más frecuente en los pacientes con cardiopatía congénita (CC) en edad escolar. Analizamos la incidencia de eventos neurológicos agudos (ENA) en pacientes con CC sometidos a cirugía cardiaca y la utilidad de los neuromarcadores para predecir el neurodesarrollo. Métodos: Estudio prospectivo observacional en recién nacidos (RN) con CC diagnosticada prenatalmente, y sometidos a cirugía el primer año de vida. Se evaluaron: 1) biomarcadores sanguíneos de lesión cerebral (S100B, enolasa neuronal específica) en sangre de cordón y periquirúrgicos; 2) datos clínicos y analíticos perinatales y periquirúrgicos; 3) tratamientos y complicaciones, y 4) neurodesarrollo (escala de Bayley III) a los 2 años. Resultados: Se incluyeron 84 RN con CC de diagnóstico fetal, confirmada posnatalmente, sometidos a cirugía cardiaca en el primer año de vida. Diecisiete pacientes tenían corazón univentricular, 20 pacientes obstrucción izquierda y 10 síndromes genéticos. Fallecieron en el periodo posquirúrgico 5 pacientes (5,9%) y 9 pacientes presentaron ENA (10,7%). Las puntuaciones medias en el test de Bayley III fueron normales, pero el 31% tuvieron alteración cognitiva, motora o en el lenguaje. Los pacientes con síndromes genéticos, ENA y CC univentriculares tuvieron peor neurodesarrollo. La elevación de S100B en el postoperatorio inmediato se correlacionó con peores puntuaciones. Conclusiones: Los pacientes con CC sometidos a cirugía tienen mayor riesgo de sufrir alteraciones del neurodesarrollo. Los pacientes con síndromes genéticos o corazones univentriculares presentan peores resultados. Presentar ENA posquirúrgico puede contribuir a peores resultados. Niveles de S100B elevados en el postoperatorio se correlacionan con peores resultados en los test de neurodesarrollo a los 2 años...(AU)

Introduction: At present, neurodevelopmental abnormalities are the most frequent type of complication in school-aged children with congenital heart disease (CHD). We analysed the incidence of acute neurologic events (ANEs) in patients with operated CHD and the usefulness of neuromarkers for the prediction of neurodevelopment outcomes. Methods: Prospective observational study in infants with a prenatal diagnosis of CHD who underwent cardiac surgery in the first year of life. We assessed the following variables: (1) serum biomarkers of brain injury (S100B, neuron-specific enolase) in cord blood and preoperative blood samples; (2) clinical and laboratory data from the immediate postnatal and perioperative periods; (3) treatments and complications; (4) neurodevelopment (Bayley-III scale) at age 2 years. Results: The study included 84 infants with a prenatal diagnosis of CHD who underwent cardiac surgery in the first year of life. Seventeen had univentricular heart, 20 left ventricular outflow obstruction and 10 genetic syndromes. The postoperative mortality was 5.9% (5/84) and 10.7% (9/84) patients experienced ANEs. The mean overall Bayley-III scores were within the normal range, but 31% of patients had abnormal scores in the cognitive, motor or language domains. Patients with genetic syndromes, ANEs and univentricular heart had poorer neurodevelopmental outcomes. Elevation of S100B in the immediate postoperative period was associated with poorer scores. Conclusions: Children with a history of cardiac surgery for CHD in the first year of life are at risk of adverse neurodevelopmental outcomes. Patients with genetic syndromes, ANEs or univentricular heart had poorer outcomes. Postoperative ANEs may contribute to poorer outcomes. Elevation of S100B levels in the postoperative period was associated with poorer neurodevelopmental outcomes at 2 years...(AU)

Neurodevelopmental outcomes in congenital heart disease: Usefulness of biomarkers of brain injury.

INTRODUCTION: At present, neurodevelopmental abnormalities are the most frequent type of complication in school-aged children with congenital heart disease (CHD). We analysed the incidence of acute neurologic events (ANEs) in patients with operated CHD and the usefulness of neuromarkers for the prediction of neurodevelopment outcomes. METHODS: Prospective observational study in infants with a prenatal diagnosis of CHD who underwent cardiac surgery in the first year of life. We assessed the following variables: (1) serum biomarkers of brain injury (S100B, neuron-specific enolase) in cord blood and preoperative blood samples; (2) clinical and laboratory data from the immediate postnatal and perioperative periods; (3) treatments and complications; (4) neurodevelopment (Bayley-III scale) at age 2 years. RESULTS: the study included 84 infants with a prenatal diagnosis of CHD who underwent cardiac surgery in the first year of life. Seventeen had univentricular heart, 20 left ventricular outflow obstruction and 10 genetic syndromes. The postoperative mortality was 5.9% (5/84) and 10.7% (9/84) patients experienced ANEs. The mean overall Bayley-III scores were within the normal range, but 31% of patients had abnormal scores in the cognitive, motor or language domains. Patients with genetic syndromes, ANEs and univentricular heart had poorer neurodevelopmental outcomes. Elevation of S100B in the immediate postoperative period was associated with poorer scores. CONCLUSIONS: children with a history of cardiac surgery for CHD in the first year of life are at risk of adverse neurodevelopmental outcomes. Patients with genetic syndromes, ANEs or univentricular heart had poorer outcomes. Postoperative ANEs may contribute to poorer outcomes. Elevation of S100B levels in the postoperative period was associated with poorer neurodevelopmental outcomes at 2 years. Studies with larger samples and longer follow-ups are needed to define the role of these biomarkers of brain injury in the prediction of neurodevelopmental outcomes in patients who undergo surgery for management of CHD.

Malestar psicológico, ajuste diádico y dinámica familiar tras el diagnóstico prenatal de cardiopatía congénita / Psychological distress, dyadic adjustment and family dynamics after prenatal diagnosis of congenital heart disease

Introducción: Las cardiopatías congénitas (CC) se encuentran entre las enfermedades congénitas más frecuentes. Cuando se informa a los padres y las madres del diagnóstico de una CC durante el embarazo, tienen que afrontar una serie de cambios psicológicos a nivel individual e interpersonal. El objetivo del estudio es evaluar el impacto psicológico que produce en los padres el diagnóstico de una CC en el periodo prenatal. La muestra incluyó 214 participantes, madres y sus parejas (grupo de casos: 57 gestantes con fetos diagnosticados de CC y sus parejas; grupo control: 50 gestantes y sus parejas). Método: Administración del BSI-18, el DAS y el FACES-III tras el diagnóstico prenatal. Resultados: Muchos progenitores (alrededor del 83,6%) pasaron por fases de choque emocional y negación tras recibir el diagnóstico de una enfermedad que necesitaban afrontar y aceptar. A nivel individual, el 35,1% de los padres y el 47,4% de las madres tuvieron puntuaciones significativas en el malestar psicológico. A nivel de la pareja, tanto los padres (77%) como las madres (82,4%) presentaron un ajuste diádico idealizado. Por último, el 43,9% de los padres y el 42,2% de las madres reflejaron una dinámica familiar moderada. Conclusión: Los resultados ilustran la complejidad de la etapa inicial que atravesaban las parejas participantes. (AU)

Introduction: Congenital heart disease (CHD) is one of the most frequent congenital diseases. When expectant fathers and mothers are informed of a CHD during pregnancy, they have to confront a series of individual and interpersonal psychological changes. The aim of the study was to evaluate the psychological impact of a prenatal diagnosis of CHD on expectant parents. The sample included 214 participants, mothers and their partners (case group, 57 pregnant women carrying a foetus with CHD and their partners; and control group, 50 pregnant women and their partners). Method: Administration of BSI-18, DAS and FACES-III following prenatal diagnosis. Results: Many parents (approximately 83.6%) went through stages of shock and denial in response to the diagnosis of disease that they had to confront and accept. At the individual level, 35.1% of fathers and 47.4% mothers had clinically significant scores of psychological distress. At the couple level, both fathers (77%) and mothers (82.4%) had expressed an idealized dyadic adjustment. Lastly, the perception of the family dynamic by 43.9% of fathers and 42.2% of mothers was in the mid-range. Conclusion: The results highlight the complexity of the initial stage that these couples were going through. (AU)

Copy-number intratumor heterogeneity increases the risk of relapse in chemotherapy-naive stage II colon cancer.

Optimal selection of high-risk patients with stage II colon cancer is crucial to ensure clinical benefit of adjuvant chemotherapy. Here, we investigated the prognostic value of genomic intratumor heterogeneity and aneuploidy for disease recurrence. We combined targeted sequencing, SNP arrays, fluorescence in situ hybridization, and immunohistochemistry on a retrospective cohort of 84 untreated stage II colon cancer patients. We assessed the clonality of copy-number alterations (CNAs) and mutations, CD8+ lymphocyte infiltration, and their association with time to recurrence. Prognostic factors were included in machine learning analysis to evaluate their ability to predict individual relapse risk. Tumors from recurrent patients displayed a greater proportion of CNAs compared with non-recurrent (mean 31.3% versus 23%, respectively; p = 0.014). Furthermore, patients with elevated tumor CNA load exhibited a higher risk of recurrence compared with those with low levels [p = 0.038; hazard ratio (HR) 2.46], which was confirmed in an independent cohort (p = 0.004; HR 3.82). Candidate chromosome-specific aberrations frequently observed in recurrent cases included gain of the chromosome arm 13q (p = 0.02; HR 2.67) and loss of heterozygosity at 17q22-q24.3 (p = 0.05; HR 2.69). CNA load positively correlated with intratumor heterogeneity (R = 0.52; p < 0.0001). Consistently, incremental subclonal CNAs were associated with an elevated risk of relapse (p = 0.028; HR 2.20), which we did not observe for subclonal single-nucleotide variants and small insertions and deletions. The clinico-genomic model rated an area under the curve of 0.83, achieving a 10% incremental gain compared with clinicopathological markers (p = 0.047). In conclusion, tumor aneuploidy and copy-number intratumor heterogeneity were predictive of poor outcome and improved discriminative performance in early-stage colon cancer. © 2022 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.

Usefulness of transaortic approach for a complex double-outlet right ventricle.

Surgical procedures for double-outlet right ventricle with ventricular septal defect are based on rerouting the blood flow of the left ventricle to the aorta through the ventricular septal defect (VSD) with an intraventricular baffle. The right atriotomy is the most common approach combined with a right ventriculotomy in some cases, particularly in pulmonary stenosis association. However, in complex cases, this standard operative strategy may not provide an adequate exposure. We describe the transaortic approach as an alternative procedure to repair a complex case of double-outlet right ventricle (DORV) with subaortic stenosis. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12055-021-01261-7.

Psychological distress, dyadic adjustment and family dynamics after prenatal diagnosis of congenital heart disease.

INTRODUCTION: Congenital heart disease (CHD) is one of the most frequent congenital diseases. When expectant fathers and mothers are informed of a CHD during pregnancy, they have to confront a series of individual and interpersonal psychological changes. The aim of the study was to evaluate the psychological impact of a prenatal diagnosis of CHD on expectant parents. The sample included 214 participants, mothers and their partners (case group, 57 pregnant women carrying a foetus with CHD and their partners and control group, 50 pregnant women and their partners). METHOD: Administration of the BSI-18, the DAS and FACES-III following prenatal diagnosis. RESULTS: Many parents (approximately 83.6%) went through stages of shock and denial in response to the diagnosis of disease that they had to confront and accept. At the individual level, 35.1% of fathers and 47.4% mothers had clinically significant scores of psychological distress. At the couple level, both fathers (77%) and mothers (82.4%) had expressed an idealized dyadic adjustment. Lastly, the perception of the family dynamic by 43.9% of fathers and 42.2% of mothers was in the mid range. CONCLUSION: The results highlight the complexity of the initial stage that these couples were going through.

[Psychological distress, dyadic adjustment and family dynamics after prenatal diagnosis of congenital heart disease]. / Malestar psicológico, ajuste diádico y dinámica familiar tras el diagnóstico prenatal de cardiopatía congénita.

INTRODUCTION: Congenital heart disease (CHD) is one of the most frequent congenital diseases. When expectant fathers and mothers are informed of a CHD during pregnancy, they have to confront a series of individual and interpersonal psychological changes. The aim of the study was to evaluate the psychological impact of a prenatal diagnosis of CHD on expectant parents. The sample included 214 participants, mothers and their partners (case group, 57 pregnant women carrying a foetus with CHD and their partners; and control group, 50 pregnant women and their partners). METHOD: Administration of BSI-18, DAS and FACES-III following prenatal diagnosis. RESULTS: Many parents (approximately 83.6%) went through stages of shock and denial in response to the diagnosis of disease that they had to confront and accept. At the individual level, 35.1% of fathers and 47.4% mothers had clinically significant scores of psychological distress. At the couple level, both fathers (77%) and mothers (82.4%) had expressed an idealized dyadic adjustment. Lastly, the perception of the family dynamic by 43.9% of fathers and 42.2% of mothers was in the mid-range. CONCLUSION: The results highlight the complexity of the initial stage that these couples were going through.

Effect of In utero Non-steroidal anti-inflammatory drug therapy for severe ebstein anomaly or tricuspid valve dysplasia (NSAID therapy for fetal ebstein anomaly)

Abstract: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental non-steroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010-2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy 4 at a median gestational age (GA) of 30.0 weeks (range: 20.9-34.9). Most (15/21=71%) mothers received NSAIDs, and 12/15 (80%) achieved DA constriction after a median of 2.0 days (1.0-6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome was 36.1 weeks (30.7-39.0) in fetuses with DA constriction vs. 33 weeks (23.3-37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p=0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4/9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p=0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly).

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21 = 71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p = 0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry.

INTRODUCTION: Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience. OBJECTIVES: Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR). METHODS: We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics. RESULTS: Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9-31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgitation, and pulmonary valve patency. There were fetal complications in 55% of cases, including 7 deaths and 2 delayed fetal losses. Among those who underwent successful FCI, the absolute measurement of the TV increased by 0.32 (±0.17) mm/week from intervention to birth. Among 60 liveborn with known outcome, there was a higher percentage having a biventricular circulation following successful FCI (87 vs. 43%). CONCLUSIONS: Our data suggest a possible benefit to fetal therapy for PAIVS/PS, though rates of technically unsuccessful procedures and procedure-related complications, including fetal loss were substantial. FCI criteria are extremely variable, making direct comparison to nonintervention patients challenging and potentially biased. More uniform FCI criteria for fetuses with PAIVS/PS are needed to avoid unnecessary procedures, expose only fetuses most likely to sustain a benefit, and to enable comparisons to be made with nonintervention patients.

Quantitative analysis of somatically acquired and constitutive uniparental disomy in gastrointestinal cancers.

Somatically acquired uniparental disomies (aUPDs) are frequent events in solid tumors and have been associated with cancer-related genes. Studies assessing their functional consequences across several cancer types are therefore necessary. Here, we aimed at integrating aUPD profiles with the mutational status of cancer-related genes in a tumor-type specific manner. Using TCGA datasets for 1,032 gastrointestinal cancers, including colon (COAD), rectum (READ), stomach (STAD), esophageal adenocarcinoma (EAC) and esophageal squamous cell carcinoma (ESCC), we show a non-random distribution of aUPD, suggesting the existence of a cancer-specific landscape of aUPD events. Our analysis indicates that aUPD acts as a "second hit" in Knudson's model in order to achieve biallelic inactivation of tumor suppressor genes. In particular, APC, ARID1A and NOTCH1 were recurrently inactivated by the presence of homozygous mutation as a consequence of aUPD in COAD and READ, STAD and ESCC, respectively. Furthermore, while TP53 showed inactivation caused by aUPD at chromosome arm 17p across all tumor types, copy number losses at this genomic position were also frequent. By experimental and computationally inferring genome ploidy, we demonstrate that an increased number of aUPD events, both affecting the whole chromosome or segments of it, were present in highly aneuploid genomes compared to near-diploid tumors. Finally, the presence of mosaic UPD was detected at a higher frequency in DNA extracted from peripheral blood lymphocytes of patients with colorectal cancer compared to healthy individuals. In summary, our study defines specific profiles of aUPD in gastrointestinal cancers and provides unequivocal evidence of their relevance in cancer.

Evolución ondulante de la miocardiopatía no compactada / Undulating clinical course of noncompaction cardiomyopathy

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Surgical Atrioventricular Valve Replacement With Melody Valve in Infants and Children.

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.

Acute Myocarditis with Infarct-like Presentation in a Pediatric Population: Role of Cardiovascular Magnetic Resonance.

Chest pain is a typical symptom of acute myocarditis in adolescents. It may be indistinguishable from myocardial ischemia so it is called "infarct-like pattern." Cardiovascular magnetic resonance has an important role as a non-invasive diagnostic tool. The aim of our study is to provide a description of an acute myocarditis series with infarct-like pattern and to evaluate the cardiovascular magnetic resonance role in a pediatric population. We included all pediatric patients (0-16 years) admitted to our hospital (May 2007-May 2016) with clinical diagnosis of acute myocarditis and infarct-like presentation (chest pain, EKG alterations, and released cardiac biomarkers). Diagnosis was confirmed with cardiovascular magnetic resonance using Lake Louise criteria. Seven patients (five males, two females) with a median age of 14 years (12.5-15.2) were included. All patients showed ST-segment changes and released cardiac biomarkers. Three patients had left ventricular hypertrophy and two presented mild systolic left ventricular dysfunction. All patients had at least two positive Lake Louise criteria. Late gadolinium enhancement was positive in all of them. With a median follow-up of 23 months (8-47), all of them are alive, with no cardiac symptoms and normal ventricular function. Infarct-like pattern is a typical presentation of acute myocarditis in adolescents. CMR should be performed in this population and may be considered as a first-line diagnostic tool. Its high sensitivity in infarct-like acute myocarditis may allow us to avoid endomyocardial biopsy. Unlike what was described in adults, late gadolinium enhancement does not imply worse outcome in our series.

Vena cava superior izquierda persistente con agenesia de la vena cava superior derecha / Persistent left superior vena cava with absent right superior vena cava

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Placenta-related complications in women carrying a foetus with congenital heart disease.

INTRODUCTION: Recent studies pointed to an intrinsically angiogenic imbalance in CHD in the maternal and foetal circulation suggestive of impaired placentation. OBJECTIVES: To assess whether pregnant women with a CHD foetus are at greater risk of placenta-related complications. METHODS: Perinatal results of women with a CDH foetus were compared with those of a non-selected population followed up at our centre. Multiple pregnancies and chromosomal abnormalities were excluded from the analysis. RESULTS: About 279 pregnancies with CHD foetuses were included. Mothers were classified in three groups according to the foetal cardiac defect: 104 (37.3%) atrioventricular defect, 102 (36.5%) conotruncal anomalies and 73 (26.2%) left-ventricular outflow tract obstruction. A significantly higher incidence of pre-eclampsia was observed in the CHD group compared with the normal population (5.7% versus 1.2% p < 0.0001) [OR 5.96 (95% CI - 3.19-10.54)]. About 9.7% of foetuses with CHD had < 3rd birth weight percentile compared with 3% for the normal population [OR 3.32 (95% CI - 2.39-4.56)]. A higher incidence of stillbirth was also observed in the CHD group compared with the normal population (2.5% versus 0.4%) [OR 9.45 (95% CI - 3.35-23.3)]. CONCLUSIONS: Women carrying a foetus with CHD have a high risk of pre-eclampsia and intrauterine growth restriction. The relationship between CHD and placenta-related complications could be an encouraging topic for future research.