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Background. Poor maternal self-rated health in healthy women is associated with adverse neonatal outcomes, but knowledge about self-rated health in pregnant women with congenital heart disease (CHD) is sparse. This study, therefore, investigated self-rated health before, during, and after pregnancy in women with CHD and factors associated with poor self-rated health. Methods. The Swedish national registers for CHD and pregnancy were merged and searched for primiparous women with data on self-rated health; 600 primiparous women with CHD and 3062 women in matched controls. Analysis was performed using descriptive statistics, chi-square test and logistic regression. Results. Women with CHD equally often rated their health as poor as the controls before (15.5% vs. 15.8%, p =.88), during (29.8% vs. 26.8% p =.13), and after pregnancy (18.8% vs. 17.6% p =.46). None of the factors related to heart disease were associated with poor self-rated health. Instead, factors associated with poor self-rated health during pregnancy in women with CHD were ≤12 years of education (OR 1.7, 95%CI 1.2–2.4) and self-reported history of psychiatric illness (OR 12.6, 95%CI 1.4–3.4). After pregnancy, solely self-reported history of psychiatric illness (OR 5.2, 95%CI 1.1–3.0) was associated with poor self-rated health. Conclusion. Women with CHD reported poor self-rated health comparable to controls before, during, and after pregnancy, and factors related to heart disease were not associated with poor self-rated health. Knowledge about self-rated health may guide professionals in reproductive counselling for women with CHD. Further research is required on how pregnancy affects self-rated health for the group in a long-term perspective. [ABSTRACT FROM AUTHOR]

Objective: To describe and evaluate the outcomes of ductal angioplasty with stent placement at a single high-complexity center during the period 2016-2022. Method: A retrospective descriptive cross-sectional study was conducted, including patients under 3 months of age who underwent ductal stent implantation as initial palliative treatment. Demographic, clinical, and anatomical data were collected before the intervention. Mortality, intra- and post-procedural complications, need for re-intervention, intensive care requirements, and hospital stay were recorded. The characteristics at the time of definitive surgery are described. Discrete variables are presented as percentages, and continuous variables are presented with their medians and respective interquartile ranges. Results: Twenty patients who underwent this treatment were reviewed, revealing a success rate of 80%. Complications due to stent dysfunction required surgical resolution. 95% of patients were discharged from the institution after the procedure, and 17 patients reached a second definitive surgical stage. Three patients died after the procedure, but with no direct relation to it. Conclusions: Indications for ductal angioplasty with stent as an alternative treatment to systemic-pulmonary anastomosis by surgery are not yet fully defined; the strategy represents a valid alternative in appropriately selected patients. The presented experience shows results similar to international reference centers. [ABSTRACT FROM AUTHOR]

Abstract Background The number of pregnant women with congenital heart disease (CHD) is rising, and the disease poses increased risks of cardiovascular and obstetric complications during pregnancy, potentially impacting breastfeeding success. This study aimed to investigate breastfeeding in primiparous women with CHD compared to primiparous women without CHD, and to examine potential hindering factors for breastfeeding in women with CHD. Methods The data were gathered between 2014 and 2019 and obtained by merging the Swedish Congenital Heart Disease Register (SWEDCON) with the Swedish Pregnancy Register. Primiparous women ≥ 18 years of age with CHD (n = 578) were matched by age and municipality to 3049 women without CHD, giving birth after 22 gestational weeks. Multivariable logistic regression analysis was used to identify factors associated with non-breastfeeding in women with CHD. Results Fewer women with CHD breastfed than women without CHD two days (94% vs. 97%, p = 0.001) and four weeks after birth (84% vs. 89%, p = 0.006). When all women were analysed, having CHD was associated with non-breastfeeding at both two days and four weeks after birth. For women with CHD, body mass index (BMI) ≥ 30 (OR 3.1; 95% CI 1.4, 7.3), preterm birth (OR 6.4; 95% CI 2.1, 19.0), self-reported history of psychiatric illness (OR 2.4; 95% CI 1.2, 5.1), small for gestational age (OR 4.2; 95% CI 1.4, 12.2), and New York Heart Association Stages of Heart Failure class II − III (OR 6.0; 95% CI 1.4, 26.7) were associated with non-breastfeeding two days after birth. Four weeks after birth, factors associated with non-breastfeeding were BMI ≥ 30 (OR 4.3; 95% CI 2.1, 9.0), self-reported history of psychiatric illness (OR 2.2; 95% CI 1.2, 4.2), and preterm birth (OR 8.9; 95% CI 2.8, 27.9). Conclusions The study shows that most women with CHD breastfeed, however, at a slightly lower proportion compared to women without CHD. In addition, factors related to the heart disease were not associated with non-breastfeeding four weeks after birth. Since preterm birth, BMI ≥ 30, and psychiatric illness are associated with non-breastfeeding, healthcare professionals should provide greater support to women with CHD having these conditions.

Background: The number of pregnant women with congenital heart disease (CHD) is rising, and the disease poses increased risks of cardiovascular and obstetric complications during pregnancy, potentially impacting breastfeeding success. This study aimed to investigate breastfeeding in primiparous women with CHD compared to primiparous women without CHD, and to examine potential hindering factors for breastfeeding in women with CHD. Methods: The data were gathered between 2014 and 2019 and obtained by merging the Swedish Congenital Heart Disease Register (SWEDCON) with the Swedish Pregnancy Register. Primiparous women ≥ 18 years of age with CHD (n = 578) were matched by age and municipality to 3049 women without CHD, giving birth after 22 gestational weeks. Multivariable logistic regression analysis was used to identify factors associated with non-breastfeeding in women with CHD. Results: Fewer women with CHD breastfed than women without CHD two days (94% vs. 97%, p = 0.001) and four weeks after birth (84% vs. 89%, p = 0.006). When all women were analysed, having CHD was associated with non-breastfeeding at both two days and four weeks after birth. For women with CHD, body mass index (BMI) ≥ 30 (OR 3.1; 95% CI 1.4, 7.3), preterm birth (OR 6.4; 95% CI 2.1, 19.0), self-reported history of psychiatric illness (OR 2.4; 95% CI 1.2, 5.1), small for gestational age (OR 4.2; 95% CI 1.4, 12.2), and New York Heart Association Stages of Heart Failure class II − III (OR 6.0; 95% CI 1.4, 26.7) were associated with non-breastfeeding two days after birth. Four weeks after birth, factors associated with non-breastfeeding were BMI ≥ 30 (OR 4.3; 95% CI 2.1, 9.0), self-reported history of psychiatric illness (OR 2.2; 95% CI 1.2, 4.2), and preterm birth (OR 8.9; 95% CI 2.8, 27.9). Conclusions: The study shows that most women with CHD breastfeed, however, at a slightly lower proportion compared to women without CHD. In addition, factors related to the heart disease were not associated with non-breastfeeding four weeks after birth. Since preterm birth, BMI ≥ 30, and psychiatric illness are associated with non-breastfeeding, healthcare professionals should provide greater support to women with CHD having these conditions. [ABSTRACT FROM AUTHOR]

ABSTRACT Objective: To analyze the factors associated with the indication of echocardiography for the investigation of congenital heart disease among newborns. Method: Retrospective sectional study through the collection of 848 medical records of patients admitted to maternity hospitals in Rio de Janeiro-Brazil, respecting the time frame from September to December 2022. Results: The average age of mothers was 26.5±6.3 years; 52.7% were classified as brown. The average age of the newborns was 3.5±5.6 days. Maternal variables: gestational age (OR=6.93, CI:3.76-12.80), number of gestational risk factors (1.90: 1.47-2.45) and number of medications (1. 97: 1.40-2.77); and neonatal variables: age (1.07: 1.03-1.02), prematurity (10.55: 5.29-21.03) and number of risk factors (2.62: 2.03-3 .38) were significantly associated with the indication for echocardiography (p

Heart diseases are potentially fatal pathologies considered a risk factor for anxiety disorders. This paper aims to present a systematic literature review on anxiety in cardiology. Five databases were selected: Scielo, PsycINFO, Web of Science, and PubMed. The descriptors "cardiologia" and "ansiedade" and their equivalents in English, "anxiety" and "cardiology" were used. The inclusion criteria were papers published in Portuguese, English, or Spanish between 2017 and 2021. Twenty-six studies were selected, and four topics stood out: socio-demographic profile, anxiety and cardiac rehabilitation, anxiety-related risk and protective factors, and anxiety and depression. The results indicate that healthy anxiety levels play a protective role in promoting patient self-care. However, maladaptive anxiety levels hinder patients from coping with the disease. [ABSTRACT FROM AUTHOR]

Heart diseases are potentially fatal pathologies considered a risk factor for anxiety disorders. This paper aims to present a systematic literature review on anxiety in cardiology. Five databases were selected: SciElo, PsycINFO, Web of Science, and PubMed. The descriptors "cardiologia" and "ansiedade" and their equivalents in English, "anxiety" and "cardiology" were used. The inclusion criteria were papers published in Portuguese, English, or Spanish between 2017 and 2021. Twenty-six studies were selected, and four topics stood out: socio-demographic profile; anxiety and cardiac rehabilitation; anxiety-related risk and protective factors; and anxiety and depression. The results indicate that healthy anxiety levels play a protective role in promoting patient self-care. However, maladaptive anxiety levels hinder patients from coping with the disease. [ABSTRACT FROM AUTHOR]

Introducción. La atresia pulmonar con comunicación interventricular es una cardiopatía compleja que enmarca grandes desafíos en su etapa pre y postquirúrgica; el uso del soporte vital extracorpóreo con membrana de oxigenación restablece la oxigenación y perfusión al organismo para permitir recuperación y complementar estudios. El objetivo de este caso clínico es determinar la atención de enfermería en la fase aguda post quirúrgica. Se expondrá usando el modelo teórico de Dorothea Orem: teorías de déficit de autocuidado y teoría de sistemas. Esta cardiopatía es la forma más severa de la tetralogía de Fallot. Tiene una incidencia del 2% entre todas las cardiopatías. Metodología. Es el caso de una preescolar con atresia pulmonar con comunicación interventricular, se describe los procesos realizados desde el diagnóstico, la intervención percutánea y reparo quirúrgico, así como el manejo de lesión residual en el marco del uso de diferentes tecnologías. Se resalta como elemento clave el uso del soporte con oxigenación con membrana extracorpórea usada como puente a decisión. Resultados. El plan de atención de enfermería en esta fase crítica logró los resultados planteados como la adecuada perfusión y oxigenación, recuperación de la función ventricular, estabilización hemodinámica para para ser llevada al reparo de la lesión residual. Este novedoso soporte fue implementado dos veces durante la misma hospitalización y con resultados exitosos. Conclusiones. Caso de difícil manejo con terapias convencionales, pero con aplicación de una atención integral de enfermería; el uso de tecnología y de diversas especialidades permitió un egreso de la menor sin complicaciones. Introduction. The pulmonary atresia with ventricular septal defect is a complex heart disseas that possess great challenges in pre and post-surgical stages; the use of vital support extracorporeal with membrane oxygenation restores oxygen and perfusion to the body to allow recovery and complement studies. The objetive of this case report is to determinate the nurse attention in the acute post quirulgical phase. It will be presented using Dorothea Orem’s theoretical model: theories of self-care deficits and systems theory. This heart disease is the most severe form of tetralogy Fallot. It has an incidence of 2% among all heart diseases. Methodology. This is the case of a kindergarten with pulmonary atresia with ventricular septal defect, the processes carried out from diagnosis, percutaneous intervention and surgical repair are described, as well as the management of residual injury within the framework of the use of different technologies. The use of extracorporeal membrane oxygenation support used as a decision bridge is highlighted as a key element. Results. The nursing care plan in the critical phase achieved the results proposed as adequate perfusion and oxygenation, recovery of the ventricular function, hemodynamic stabilization to be carried out to repair the residual injury. This newfangled support was implemented twice during the same hospitalization with sucessful result. Conclusions. Case report with struggle managment with conventional therapies but with the application of comprehensive nursing care; the use of technology and the work of various specialities allowed the minor to be discharged without complications. Introdução. A atresia pulmonar com comunicação interventricular é uma cardiopatia complexa que apresenta grandes desafios em sua fase pré e pós-cirúrgica. O uso de suporte de vida extracorpóreo com membrana de oxigenação restaura a oxigenação e a perfusão do corpo para permitir a recuperação e complementar os estudos. O objetivo deste caso clínico é determinar os cuidados de enfermagem na fase aguda pós-cirúrgica. Será apresentado utilizando o modelo teórico de Dorothea Orem: teorias do déficit de autocuidado e teoria de sistemas. Esta doença cardíaca é a forma mais grave de tetralogia de Fallot. Tem uma incidência de 2% entre todas as doenças cardíacas. Metodologia. É o caso de uma criança em idade pré-escolar com atresia pulmonar com comunicação interventricular, são descritos os processos realizados desde o diagnóstico, intervenção percutânea e reparação cirúrgica, bem como o manejo da lesão residual no âmbito da utilização de diferentes tecnologias. Destaca-se, como elemento-chave, a utilização de suporte com oxigenação por membrana extracorpórea como ponte para a decisão. Resultados. O plano de cuidados de enfermagem nesta fase crítica alcançou os resultados propostos como perfusão e oxigenação adequadas, recuperação da função ventricular, estabilização hemodinâmica a ser realizada para o reparo da lesão residual. Este novo suporte foi implementado duas vezes durante a mesma hospitalização e com resultados bem-sucedidos. Conclusões. Caso de difícil manejo com terapias convencionais, mas com aplicação de cuidados integrais de enfermagem, o uso da tecnologia e de diversas especialidades permitiu que a criança recebesse alta sem complicações.

Resumen: La cirugía de reemplazo de raíz aórtica con preservación valvular se ha erigido como una técnica estándar para el tratamiento de pacientes con aneurismas de raíz aórtica. Presentamos nuestra experiencia en la población de pacientes con cardiopatías congénitas.Desde julio de 2012 a diciembre de 2020, 9 pacientes con antecedentes de cardiopatías congénitas fueron intervenidos según la técnica de Yacoub (remodelado aórtico). Ocho de ellos eran varones y la mediana de edad fue de 28 años. Seis pacientes habían sido intervenidos previamente. El 67% presentaban insuficiencia aórtica grado III o IV. La mediana de diámetro máximo de raíz aórtica previo a la cirugía fue de 50 mm (rango, de 42 a 55 mm). Con una mediana de seguimiento 85 meses, la supervivencia global ha sido del 89%. Un paciente falleció durante el postoperatorio inmediato a causa de una tumoración en el tronco coronario izquierdo. Durante el seguimiento, todos los pacientes presentaban insuficiencia aórtica grado ≤ II (7 de ellos grado ≤ I). Ningún paciente ha presentado endocarditis aórtica ni ha sido reintervenido por insuficiencia aórtica.La cirugía de reemplazo de raíz aórtica con preservación valvular muestra unos resultados excelentes a corto y medio plazo en la población de pacientes con cardiopatías congénitas, si bien la casuística propia es baja y habrá que esperar unos años a obtener datos sobre una población mayor y con un mayor seguimiento. Abstract: Valve-sparing aortic root replacement surgery has emerged as a standard technique for the treatment of patients with aortic root aneurysms. We present our experience in patients with congenital heart disease.From July 2012 to December 2020, 9 patients with a history of congenital heart disease were operated on according to the Yacoub technique (aortic root remodeling). Eight of them were male and the median age was 28 years. Six patients underwent a prior cardiac surgery. 67% had grade III or IV aortic regurgitation. The median maximum diameter of the aortic root prior to surgery was 50 mm (range, 42 to 55 mm). With a median follow-up of 85 months, overall survival was 89%. One patient died during the immediate postoperative period due to a tumor in the left main coronary artery. During follow-up, all patients had grade ≤ II aortic regurgitation (7 of them grade ≤ I). No patient suffered from aortic endocarditis or underwent reoperation for aortic regurgitation.Valve-sparing aortic root replacement surgery shows excellent results in the short and medium term in the population of patients with congenital heart disease. Nonetheless, our casuistry is low, so we need to wait a few years to get data based on a larger population and with a longer follow-up.

Background: There is variability in the impact of adult congenital heart disease (ACHD) on health-related quality of life (HRQoL). A greater insight into the impact of ACHD may be gained from investigating HRQoL in various diagnostic groups and considering the importance of psychosocial risk factors for poor HRQoL. Objective: We compared the HRQoL of people with ACHD with normative data from the general population and among 4 diagnostic groups and identified risk factors for poor HRQoL in ACHD from a comprehensive set of sociodemographic, clinical, and psychosocial factors. Methods: We conducted a cross-sectional study with 303 participants from 4 diagnostic groups Simple, Tetralogy of Fallot, Transposition of the Great Arteries, Single Ventricle who completed measures of illness perceptions, coping, social support, mood, and generic and disease-specific HRQoL. Data were analyzed using 1-sample t tests, analysis of variance, and hierarchical multiple regressions. Results: There was diminished psychosocial HRQoL in the Simple group compared with the general population. Consistently significant risk factors for poor HRQoL included younger age, a perception of more severe symptoms due to ACHD, depression, and anxiety. Clinical factors were poor predictors of HRQoL. Conclusions: The findings highlight the need to develop intervention studies aiming to improve HRQoL in people with ACHD and the routine assessment of illness perceptions and mood problems during key periods in people's lives. This will help address patient misconceptions that could be tackled by clinicians or specialist nurses during routine outpatient appointments and identify people in need of psychological support. [ABSTRACT FROM AUTHOR]

Introduction: Incontinentia pigmenti (IP) is a rare genodermatosis. It classically has manifestations of linear vesicular lesions, evolving into verrucous lesions within few weeks, followed by a peculiar swirled pigmentation lasting for many years. In addition, IP can affect other ectodermal tissues such as the teeth, eyes, bones, and the central nervous system. Case report: We report a case of IP in a newborn female baby who presented to us with verrucous lesions at birth and on examination was found to have congenital heart disease (atrial septal defect, with pulmonary hypertension, tricuspid regurgitation, and predominant right to left shunt). Discussion: We wish to draw attention to the association of IP with congenital heart disease.

To build a serial album about congenital heart diseases as an instrument for Health Education. The construction took place in two stages: Integrative review on the topic and elaboration of a serial album based on the data obtained and the researcher's experience. The study followed Resolution 466/12, is approved by the ethics committee with opinion number 2931.261. The album consists of 10 illustrations, which are exposed to the participants, and 10 corresponding script sheets, which are aimed at the professional. Consisting of the following pathologies: Interventricular Communication (IVC), Interatrial Communication (CIA), Arterial Canal Persistence (PCA), Transposition of the Great Arteries (TGA), Tetralogy of Fallot (T4F), Aortic Stenosis (AS), Pulmonary Atresia (AP), Atrioventricular Septal Defect (DSAV). development of the serial album was intended to provide the multidisciplinary team with educational material that enables the family to understand their child's congenital heart disease and clarify the doubts of parents and family members regarding the child's treatment and follow-up by the specialized service, minimizing stress and encouraging empowerment and confidence in the care. [ABSTRACT FROM AUTHOR]

Introduction: Incontinentia pigmenti (IP) is a rare genodermatosis. It classically has manifestations of linear vesicular lesions, evolving into verrucous lesions within few weeks, followed by a peculiar swirled pigmentation lasting for many years. In addition, IP can affect other ectodermal tissues such as the teeth, eyes, bones, and the central nervous system. Case report: We report a case of IP in a newborn female baby who presented to us with verrucous lesions at birth and on examination was found to have congenital heart disease (atrial septal defect, with pulmonary hypertension, tricuspid regurgitation, and predominant right to left shunt). Discussion: We wish to draw attention to the association of IP with congenital heart disease. [ABSTRACT FROM AUTHOR]

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Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent singlestage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups. [ABSTRACT FROM AUTHOR]

Objective: To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients. Methods: In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients’ medical records and echocardiographic database. Crossed ventricular inflow streams was identified when there was inability to visualize both atrio-ventricular valves in a single imaging plane in cardiac four chamber view. Results: CCH was diagnosed in five patients from 10,500 pediatric echocardiographic studies. The age at diagnosis ranged from one month to 8 years. Cyanosis was present in all but one of the five cases. Crossed ventricular inflow streams was present by definition in all cases, whereas superior-inferior ventricular relationship was present in only three cases. All cases were associated with ventricular septal defects. Atrio-ventricular discordance was seen in three cases and concordance in two. Ventriculo-arterial discordance was seen in three cases, concordance in one and double outlet right ventricle in one. Three cases had pulmonary stenosis and the other two had pulmonary arterial hypertension. Straddling of AV valve was observed in four cases and hypoplastic aortic arch in one case. Conclusion: CCH is an extremely rare congenital cardiac anomaly. Superior-inferior ventricular relationship often co-exists with CCH, but is not necessarily present in all cases. CCH requires early diagnosis because of its common association with diverse cardiac anomalies.

Objective: Antonovsky coined sense of coherence (SOC) as the central concept of his salutogenic theory focusing on the origins of well-being. SOC captures the degree to which one perceives the world as comprehensible, manageable, and meaningful. Life events and resources are considered to be the building blocks of a person's SOC. However, mainly quantitative studies have looked into the role of life events and resources. Therefore, the present study aims to gain a deeper insight into the experiences of patients with congenital heart disease (CHD) regarding resources and life events. Method: For this qualitative study, patients were selected from the sample of a preceding study on development of SOC (n = 429). In total, 12 young individuals with CHD who had either a weak (n = 6) or strong SOC (n = 6) over time were interviewed (8 women, median age of 20 years). Data analysis was based on the constant comparative method as detailed in the Qualitative Analysis Guide of Leuven. Commonalities and differences between patients from both groups were explored. Results: The following themes emerged: (1) self-concept; (2) social environment; (3) daytime activities; (4) life events and disease-related turning points; (5) stress and coping; and (6) illness integration. Additionally, the degree of personal control was identified as an overarching topic that transcended the other themes when comparing both groups of patients. Conclusion: These results may have implications for the structure and content of interventions improving well-being in young people with CHD.

The Congenital Heart Disease Standards for England indicate that parents and children should have access to a 24-h telephone advice service, however, little is known about existing services. This paper presents phase two of a mixed-methods service evaluation, which aimed to evaluate staff experiences of telephone communication with these parents. All nursing and support staff in a single specialist children's cardiac surgical centre were invited to participate in an online survey during July-November 2019. Data were descriptively and thematically analysed. Participants ( N = 39) were predominantly nurses ( n = 32, 82%) with 64.1% ( n = 25) working in the speciality >10 years. Positive experiences included: signposting and preventing further deterioration; supporting families to get expert advice quickly; providing reassurance. Challenging experiences included: offering advice without being able to see the child, dealing with telephone calls alongside busy workload; and parents running out of medications and telephoning out of hours. In conclusion, taking telephone calls were perceived to be time consuming and are potentially high risk. A standardised approach to assessment, intervention and documentation was deemed necessary. Implementation of an updated parental early warning tool was recommended, along with staff and parental education., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background Despite a lack of evidence supporting the use of angiotensin-converting enzyme (ACE) inhibitors in patients with a Fontan circulation, their use is frequent. We decided to identify the rationale for ACE inhibitor therapy in patients within the Australia and New Zealand Fontan Registry. Methods All patients in the Registry taking an ACE inhibitor at last follow up were identified, and a review of medical records was undertaken to determine the rationale for treatment initiation and reasons for treatment continuation or dose increase. Results In 2015, 36% of the surviving patients in the Registry (462/1268) were taking an ACE inhibitor. Indications for initiation of therapy were ventricular systolic or diastolic dysfunction (29%), atrioventricular valve regurgitation (19%), preservation of normal ventricular function (7%), prolonged effusions at Fontan (6%), hypertension (6%), other (6%) and unknown (2%). No indication was stated in the remaining patients (25%). Those with hypoplastic left heart syndrome were more likely to be on an ACE inhibitor than those with an alternative primary morphology (70% vs 32%; p < 0.001). Only 36% of the patients treated with an ACE inhibitor at last follow up (166/462) had an indication that would generally justify treatment in a two-ventricle circulation. Conclusion It is likely that the use of ACE inhibitors in patients with a Fontan circulation is excessive within our region. The coordination of prospective, multicentre studies and initiatives such as the Australia and New Zealand Fontan Registry will facilitate further investigations to guide treatment decisions in the growing Fontan population. [ABSTRACT FROM AUTHOR]

Background: There are no clear guidelines for the use of angiotensin-converting enzyme (ACE) inhibitors in patients with single ventricle physiology, especially after the Fontan procedure. Despite this, ACE inhibitors are widely prescribed within the single ventricle population.Objective: We decided to review the literature to better establish the efficacy of ACE inhibitors in the single ventricle population and summarise their indications.Methods: A review of the literature was conducted to identify relevant articles describing use of ACE inhibitors in single ventricle patients both before and after the Fontan procedure.Results: A search in the Ovid MEDLINE and Embase databases identified a total of 140 articles, of which 31 were deemed relevant to the review.Conclusions: There is little published evidence supporting the use of ACE inhibitors in patients with single ventricle physiology. Based on the current literature, it is possible that ACE inhibitors are overprescribed in patients with single ventricle physiology. The coordination of large, prospective studies through initiatives such as the Australia and New Zealand Fontan Registry is necessary to guide the appropriate use of ACE inhibitors in the single ventricle population. [ABSTRACT FROM AUTHOR]

Supravalvar mitral ring is a rare congenital heart defect of surgical importance. The condition is characterized by an abnormal ridge of the connective tissue on the atrial side of the mitral valve. It often substantially obstructs the mitral valve inflow. We herein introduce a case of a supravalvar mitral ring in a 17-year-old male, who was admitted to our hospital with cardiac syncope. He had undergone a cardiac operation for ventricular septal defect (VSD) closure and mitral valve repair 15 years before. Transthoracic echocardiography, transesophageal echocardiography, and finally cardiac catheterization revealed a neglected supravalvular mitral ring. The ring was resected in a second operation, and the patient was discharged from the hospital symptom free.

Objetivo. Determinar la frecuencia y factores asociados a desnutrición en niños con cardiopatía congénita (CC). Material y métodos. Estudio transversal analítico, hecho entre agosto de 1997 y mayo de 1998, en el servicio de cardiopatías congénitas del hospital de Cardiología del Centro Médico Nacional Siglo XXI, del Instituto Mexicano del Seguro Social de la Ciudad de México, México, a 244 menores de 17 años, de ambos sexos, con diagnóstico de CC y sin otras malformaciones. El estado de nutrición se evaluó mediante los índices peso/edad (P/E), talla/edad (T/E) y peso/talla (P/T), y se definió desnutrición con puntuaciones Z mayores a -2. Se consideraron factores de riesgo: edad, sexo, antecedentes perinatales, historia de alimentación, administración de suplementos nutricios, estado socioeconómico y funcionalidad y composición de la familia. Se formaron cuatro grupos de CC: acianógenas con flujo pulmonar aumentado (AFPA) o con flujo pulmonar normal (AFPN); cianógenas con flujo pulmonar aumentado (CFPA) o disminuido (CFPD). Análisis estadístico: ji cudrada, U-Mann Whitney, Kruskal-Wallis. Se aplicó regresión logística para el control de las variables de confusión y se calculó razón de momios (RM) e intervalos de confianza al 95% (IC 95%). Resultados. El grupo de AFPA (62.7%) fue el más frecuente, seguido por CFPD (15.6%), AFPN (11.5%) y CFPA (10.2%); con P/E, 40.9% tuvieron desnutrición; con T/E, 24.6%; y con P/T, 31.1%. Los grupos más afectados fueron los lactantes y los ninos con CFPA. Los factores asociados a desnutrición fueron: presencia de cardiopatía cianógena (RM 2.54; IC 95% 0.98-6.58) y la falta de administración de algún complemento nutricio (RM 2.38; IC 95% 1.06-5.34). Entre mayor número de miembros en una familia (RM 1.42; IC 95% 0.99-2.05) mayor frecuencia de desnutrición; a mayor edad menor riesgo de desnutrición (RM 0.92; IC 95% 0.89-0.96). Conclusiones. La desnutrición en niños con CC es frecuente; es más común en niños más pequeños y con cardiopatías cianógenas. Se necesitan programas orientados a las familias para prevenir y disminuir la desnutricion en estos niños. El texto completo en inglés de este artículo está disponible en: http://www.insp.mx/salud/index.htmlObjectives. To assess the frequency and risk factors of malnutrition among children with congenital heart disease (CHD). Material and methods. Between August 1997 and May 1998, a cross-sectional survey was conducted among 244 children, at the congenital heart disease ward of the Cardiology Hospital, National Medical Center "Siglo XXI", Mexican Institute of Social Security, in Mexico City. Study subjects were male and female children younger than 17 years, diagnosed with CHD and without any other congenital malformation. Weight/Age (W/A), Height/Age (H/A) and Weight/Height (W/H) were used to measure nutritional status; Z scores greater than -2 was the case definition of malnutrition. Risk factors investigated were age, sex, perinatal history, dietary factors and nutritional supplementation, socioeconomic status, and family composition and functionality. Four CHD groups were studied: acyanotic with and without pulmonary hypertension (APH, AWPH) and, cyanotic with and without pulmonary hypertension (CPH, CWPH). Statistical analysis consisted of the chi-squared, Mann Whitney's U, and Kruskal-Wallis tests. Confounding variables were controlled for with a logistic regression model; odds ratios (OR) and 95% confidence intervals (95%CI) were calculated. Results. APH was the most frequent CHD (62.7%), followed by CWPH (15.6%), AWPH (11.5%), and CPH (10.2%). Malnutrition was identified in 40.9% children with the W/A index, in 24.6% with the H/A index; and in 31.1% with the W/H index. Infants and the CPH group had the worst nutritional status. Risk factors associated with malnutrition were: having a cyanotic CHD (OR 2.54; 95%CI, 0.98-6.58), lack of nutritional supplementation (OR 2.38; 95%CI, 1.06-5.34), and a greater number of family members (OR,1.42; 95%CI, 0.99-2.05). Older children were more likely to be well-nourished (OR 0.92; 95%CI, 0.89-0.96). Conclusions. Malnutrition is frequent among children with CHD; it is more common in younger children and in those with cyanotic CHD. Educational programs directed to the families of these children are needed to prevent and decrease the frequency of malnutrition. The English version of this paper is available at: http://www.insp.mx/salud/index.html

Objective: Antonovsky coined sense of coherence (SOC) as the central concept of his salutogenic theory focusing on the origins of well-being. SOC captures the degree to which one perceives the world as comprehensible, manageable, and meaningful. Life events and resources are considered to be the building blocks of a person's SOC. However, mainly quantitative studies have looked into the role of life events and resources. Therefore, the present study aims to gain a deeper insight into the experiences of patients with congenital heart disease (CHD) regarding resources and life events. Method: For this qualitative study, patients were selected from the sample of a preceding study on development of SOC (n = 429). In total, 12 young individuals with CHD who had either a weak (n = 6) or strong SOC (n = 6) over time were interviewed (8 women, median age of 20 years). Data analysis was based on the constant comparative method as detailed in the Qualitative Analysis Guide of Leuven. Commonalities and differences between patients from both groups were explored. Results: The following themes emerged: (1) self-concept; (2) social environment; (3) daytime activities; (4) life events and disease-related turning points; (5) stress and coping; and (6) illness integration. Additionally, the degree of personal control was identified as an overarching topic that transcended the other themes when comparing both groups of patients. Conclusion: These results may have implications for the structure and content of interventions improving well-being in young people with CHD. [ABSTRACT FROM AUTHOR]

Interventricular communication (VSD) is considered a more frequent heart disease, an important cause of mortality in the first year of life. Generally associated with pulmonary atresia. Patients with this pathology, severe condition, being the fast identification and intervention. [ABSTRACT FROM AUTHOR]

Congenital heart defects (CHD) occur in less than one percent of all newborns. Echocardiography represents the imaging modality of choice for morphological and functional assessment. In childhood the different CHD types can be diagnosed trustfully and can be performed bedside. In the follow-up of CHD cross sectional imaging plays an important role and therefore it is essential for the radiologist to know the features, challenges and limitations of echocardiography. Within this review article a systematic approach for morphological and functional assessment of the heart will is given along with representative example images. In addition, typical echocardiographic findings in common CHD is presented. In older children, adolescents and grown-ups with CHD (GUCH) echocardiography suffers from limitations - partially due to skeletal deformations and lung emphysema. In particular right ventricular function assessment is not always possible by echocardiography. Therefore strengths and limitations of echocardiography will be discussed the role of cardiac magnetic resonance imaging (cMRI) and cardiac computed tomography (cCT) emphasized. [ABSTRACT FROM AUTHOR]

Introduction: ectopia cordis is a rare congenital malformation, with an estimated incidence of 5 to 8 per million live births. It is defined as a malformation in which the heart is located in an extra-thoracic position. Ectopia cordis may occur as an isolated malformation or associated with other anomalies such as omphalocele, congenital heart disease or integrating Cantrell syndrome. The size and location of the defect influence the prognosis. Description: we report a case of a 24-year-old nulliparous woman, with no relevant family or personal history, in which the prenatal fetal ultrasound, performed at 21 weeks of gestation, revealed a defect of the anterior chest wall with exteriorization of the heart. Discussion: fetal echocardiography revealed a severe congenital heart disease. The parents decided to continue the pregnancy, after being duly informed by a multidisciplinary team. Delivery occurred at 37 weeks of gestation but the female newborn died one hour afterwards. Pathological examination confirmed the sonographic findings. [ABSTRACT FROM AUTHOR]

Supravalvar mitral ring is a rare congenital heart defect of surgical importance. The condition is characterized by an abnormal ridge of the connective tissue on the atrial side of the mitral valve. It often substantially obstructs the mitral valve inflow. We herein introduce a case of a supravalvar mitral ring in a 17-year-old male, who was admitted to our hospital with cardiac syncope. He had undergone a cardiac operation for ventricular septal defect (VSD) closure and mitral valve repair 15 years before. Transthoracic echocardiography, transesophageal echocardiography, and finally cardiac catheterization revealed a neglected supravalvular mitral ring. The ring was resected in a second operation, and the patient was discharged from the hospital symptom free.

Abstract Introduction This resource is a team-based learning module on congenital heart defects designed for first-year medical students. By using this resource, students will be able to understand and appreciate the value of a systems-based approach to exploring and discussing information with a patient who has a congenital heart defect. Methods Team-based learning cases utilize a specific written format and method of facilitating to produce very effective small-group and class discussions. There is a detailed description of this process in the facilitator's version of the module. Students should have an understanding of basic cardiovascular diagnostic tools such as an echocardiogram, electrocardiogram, wedge pressure, and cardiac catheterization. They should also be able to recognize how to utilize these diagnostic tools to diagnose a basic cardiovascular pathology. Results The purpose of this module was to introduce clinical materials to first-year medical students to stimulate group discussion and critical thinking. This case was very effective in achieving all of these goals, and the student feedback was overall positive. The case was particularly successful in helping students to “see the big picture” and aided them in their overall understanding of cardiovascular physiology as a whole. Discussion In our experience, the students found this case to be interesting and a positive learning experience. They liked interpreting the diagnostic tests. The discussion was excellent, particularly for the second question of the clinical case which was difficult enough to stimulate a lot of discussion as to why some of the answer choices were incorrect.

Background: seroepidemiological surveys suggest that approximately 20 % of women of childbearing age are susceptible to rubella. It is necessary to detect congenital rubella cases. Our objective was to determine the frequency of perinatal infection by rubella virus (RV) in infants with congenital heart disease. Methods: prospective, cross-sectional study. We studied hospitalized and outpatients from September 2007 to December 2008. Neonates and infants under one year of age with congenital heart disease were included. A blood sample of 3 mL was taken from mother-child binomial and micro-ELISA for IgG and IgM against rubella were performed Results: 80 patients were studied, 56 % were female, with a median age of 3 months. More frequent congenital heart disease was ventricular septal defect (28.5 %), followed by atrial septal defect (17.5 %). Median maternal age was 28 years old. A history of febrile illness and rash during pregnancy was positive in 1.25 %. 7 cases of perinatal infection by RV were detected, three met the criteria for congenital rubella syndrome, and four had only congenital heart disease. Conclusions: the search for cases of congenital rubella syndrome in newborns and infants with heart disease could be used as a strategy to detect non-obvious cases. [ABSTRACT FROM AUTHOR]

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The article presents a study on the safety and efficacy of stenting of the left ventricular outflow tract (LVOT) and the creation or enlargement of a ventricular septal defect (VSD) using a stent in patients with congenital heart disease. It cites stent fracture as the well-known complication after the placement of balloon-expendable stents. It also notes that stenting may reduce the degree of obstruction and ventricular pressure.

Objectives: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates. Background: The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007. Methods: A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios. Results: Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41. Conclusions: Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease. [ABSTRACT FROM AUTHOR]