The longitudinal effect of the quality of participation experiences in a voluntary organization on youth psychological empowerment.

Research shows its limitations by not capturing the specificities of individual experiences, which result either from the participation of or interaction among different people in a given context across time. This study explores the effect that the quality of youth participation experiences in a Portuguese voluntary organization can have on the development of their psychological empowerment (PE). This study adopted a longitudinal, quantitative design where the same cohort of 481 participants (62.4% female; Mage = 17.26 years) was followed across three time points. Latent growth curve modeling was performed to examine the effect of the quality of participation experiences (QPE) on the developmental trajectories of PE components. Findings show that the QPE had the most powerful effect on developmental trajectories in PE. The study shows how youth perceptions of opportunities for reflection and action in interaction in a climate of openness to dialogue and the emergence of different ways of thinking and doing affect their PE.

Benign Presentation of a Potentially Fatal Disease.

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiovascular defect that can range from being fatal early in life to presenting in adulthood asymptomatically. We report the case of a teenager whose diagnosis was incidental and underwent surgery, consisting in coronary artery button transfer, with excellent result.

A origem anómala da artéria coronária esquerda a partir da artéria pulmonar é uma cardiopatia congénita que pode ser fatal precocemente ou apresentar-se na vida adulta de forma assintomática. Apresentamos o caso clínico de um adolescente cujo diagnóstico foi incidental e que foi submetido a cirurgia, com translocação da artéria coronária, com excelente resultado.

The developmental quality of participation experiences: beyond the rhetoric that "participation is always good!".

A powerful rhetoric regarding the importance of adolescents' civic engagement and political participation is common in contemporary societies, whilst citizens, both adolescent and adults, seem to express a growing scepticism and alienation regarding politics. Even if this disengagement is debatable, as there are simultaneous signs of an increasing involvement in a variety of emerging and broadly-defined civic and political activities, we argue that the benefits of these experiences should be scrutinized using psychological evidence-based criteria. We rest on classical contributions from developmental psychology, educational theory and political science to define criteria that could inform the quality of participation experiences, and then present two studies that explore its adequacy. Study 1 is a cross-sectional study that observes that higher quality civic and political experiences are connected with more complex modes of thinking about politics. In Study 2, a two-wave longitudinal design, the quality of participation experiences is a significant predictor of change patterns of political attitudes; moreover, results support the argument that participation is not good in itself and that some experiences, with lesser developmental quality, might have a detrimental effect on adolescents' political development.

The relationship between the levels of stress and the age and years of service of military firefighters from the fire rescue corps of the metropolitan area of the state of São Paulo.

PURPOSE: To verify the relationship between stress indicators and the age and years of service of military firefighters from the fire rescue corps of the metropolitan area of the state of São Paulo. METHODS: Forty military firefighters with the mean age of 37.9 ± 5.4 years, from the 1st, 2nd and 8th fire brigades of the metropolitan area of São Paulo participated in this study. All underwent clinical, physical and psychological evaluations to verify the relationship between the age and years of service with the variables of levels of stress (tension, depression, anger, vigor, fatigue, confusion and total stress) on workdays and on days off. RESULTS: On workdays, the mean scores for tension, depression, fatigue and stress were overall higher compared to those on days off. The older the firefighter, the higher the levels of stress, depression, anger and total stress. The more years of service, the greater the depression and anger. CONCLUSIONS: The levels of stress were high on workdays. Chronological age and years of service influenced the increase in the scores of stress.

Primary adrenal insufficiency due to bilateral adrenal infarction in COVID-19: a case report.

CONTEXT: Coronavirus disease 2019 (COVID-19) is a proinflammatory and prothrombotic condition, but its impact on adrenal function has not been adequately evaluated. CASE REPORT: A 46-year-old woman presented with abdominal pain, hypotension, skin hyperpigmentation after COVID-19 infection. The patient had hyponatremia, serum cortisol <1.0 ug/dL, ACTH of 807 pg/mL and aldosterone <3 ng/dL. Computed tomography (CT) findings of adrenal enlargement with no parenchymal and minimal peripheral capsular enhancement after contrast were consistent with bilateral adrenal infarction. The patient had autoimmune hepatitis and positive antiphospholipid antibodies, but no previous thrombotic events. The patient was treated with intravenous hydrocortisone, followed by oral hydrocortisone and fludrocortisone. DISCUSSION: Among 115 articles, we identified nine articles, including case reports, of new-onset adrenal insufficiency and/or adrenal hemorrhage/infarction on CT in COVID-19. Adrenal insufficiency was hormonally diagnosed in five cases, but ACTH levels were measured in only three cases (high in one case and normal/low in other two cases). Bilateral adrenal non- or hemorrhagic infarction was identified in five reports (two had adrenal insufficiency, two had normal cortisol levels and one case had no data). Interestingly, the only case with well-characterized new-onset acute primary adrenal insufficiency after COVID-19 had a previous diagnosis of antiphospholipid syndrome. In our case, antiphospholipid syndrome diagnosis was established only after the adrenal infarction triggered by COVID-19. CONCLUSIONS: Our findings support the association between bilateral adrenal infarction and antiphospholipid syndrome triggered by COVID-19. Therefore, patients with positive antiphospholipid antibodies should be closely monitored for symptoms or signs of acute adrenal insufficiency during COVID-19.

[Percutaneous embolization of coronary fistulas: a single-center experience]. / Embolização percutânea de fístulas coronárias - Experiência de um centro.

INTRODUCTION: A coronary fistula is a connection between one of the coronary arteries and a cardiac chamber or great artery. It is a rare defect and usually occurs in isolation. Two-dimensional echocardiography has an important role in diagnosis but coronary or CT angiography is essential to delineate the anatomy. Surgery is the traditional therapeutic approach but percutaneous closure is now the recommended method, with excellent results and few complications in experienced centers. METHODS: We describe our experience with percutaneous treatment of 15 coronary fistulas in 12 patients between 1996 and 2011. Eight (67%) were male and median age was 25 years. The most frequent symptoms were murmur and/or fatigue. All fistulas were congenital. Five patients (42%) had concomitant cardiac disease: pulmonary atresia with intact ventricular septum (1), patent ductus arteriosus (1), ostium secundum atrial septal defect (1), stenotic bicuspid aortic valve (1), and critical pulmonary stenosis operated in the neonatal period (1). Three patients had two fistulas, while the others had a single lesion. All fistulas were hemodynamically significant. They originated in the territory of the right coronary (10), left coronary (3) and circumflex (2), draining into the right ventricle (5), pulmonary artery (6), right atrium (2) coronary sinus (1) and left ventricle (1). Embolization materials included standard coils, controlled-release coils, microcoils (standard, GDC or IDC) and an Amplatzer(®) duct occluder. RESULTS: Embolization was achieved in all patients. There was no mortality. One patient with a large fistula and a very small right coronary artery distally to the origin of the fistula had a right ventricular infarction. In three patients there were minor complications: inadvertent coil embolization, recovered in the same procedure (1), transient arrhythmia (1) and femoral pseudo-aneurysm (1). In a mean follow-up of 4.9 years (one month to 14 years), there were no procedure-related complications. Echocardiographic and/or angiographic control showed complete and permanent occlusion in ten patients and minimal residual flow in two patients through small collaterals with no hemodynamic significance. CONCLUSION: Percutaneous embolization represents an effective form of treatment for selected coronary fistulas. A wide range of embolization devices must be available to ensure the best therapeutic approach.

University Social Responsibility, Service Learning, and Students' Personal, Professional, and Civic Education.

The long-standing vision of universities as the "alma mater" of students and graduates is a demonstration of its role as sustaining the person, the expert/professional, and the citizen. This role has persisted in the face of rising global challenges such as the emergence of new learning spaces, the growing diversity of publics, the call for productivity and performativity, and the hope for a significant engagement with the community and the public good. These sometimes conflicting tendencies have also stimulated higher education institutions to further pedagogical strategies that articulate in novel ways the classical elements of learning: action/experience and reflection/theory. In this context, service learning received a new impetus, particularly in the post-Bologna European Higher Education Area, as universities were looking for ways in which to articulate the social dimension of HEI and their "third mission" as institutions not only committed to addressing and solving societal problems, but also committed to fostering public-minded alumni through powerful experiences of engagement for both the students and the community. This paper is based on the experience of Erasmus+project ESSA, a service earning based project focused on University social responsibility (USR). ESSA engaged four groups of students from three European universities (Edinburgh, Porto, and Kaunas) in conducting a 1-week on-site USR audit based on an ecologic and situated concept of social responsibility. We will consider the perceived impact of ESSA on 44 students through a thematic analysis of focus group discussions and student self-assessment reports produced during and after their participation in the USR audit.

Engagement in civic organisations in old age: Motivations for participation and retention.

Although the importance of older citizens' civic engagement has been highlighted in research and policy, the topic remains underexplored. In this study, we discuss older Portuguese citizens' motivations for civic engagement. The research is based on interviews with eighteen participants aged over 60, who are retired but active in civic organisations, in which they hold or have held leadership positions. We employed thematic analysis to examine the qualitative data. The findings suggest a range of motivations for participation, with personal and ideological/political motives emerging as relevant for involvement, retention and leaving, and interpersonal relationships motivating involvement and retention. However, the specific motivations differ as a function of not only the stages of involvement/retention/leaving, but also the type of organisation and the participants' life experiences. There are notable differences between the motivations for involvement in cultural and recreational organisations, neighbourhood organisations and volunteer programmes, where the emphasis is on fostering interpersonal relationships, and the more ideological/political motivations that appear as determinant within political and activist organisations. As such, this study brings a more complex understanding of the range of motivations for civic engagement in later life, with significant implications for fostering older citizens' mobilisation and sustained engagement.

Pediatric cardiology and telemedicine: seven years' experience of cooperation with remote hospitals.

BACKGROUND: A telemedicine program was initiated in November 2000 between the Pediatric Cardiology Department of a tertiary care hospital in Lisbon and pediatricians and obstetricians from three remote Portuguese hospitals: two in mainland Portugal and one in the Azores. Images were transmitted by a telemedicine link over three integrated service digital network (ISDN) lines to a regional pediatric cardiology unit for interpretation by a consultant pediatric cardiologist. METHODS: We performed a retrospective review of all teleconsultations at our Department between November 2000 and December 2007. We analyzed the population (fetal, neonatal and pediatric), indications for teleconsultation, diagnoses, impact of the teleconsultation and medical management. RESULTS: Over seven years, a total of 577 real-time teleconsultations were carried out in 500 patients, corresponding to 201 fetal exams (35%), 249 evaluations of newborns (43%) and 127 of children (22%). A total of 103 transmissions were urgent (18%). There were 364 positive diagnoses (63%) in 209 newborns (56%), 85 children (23%) and 71 fetuses (21%). Structural congenital heart disease was the most frequent diagnosis, complex in 95 patients. In all of the complex anomalies, the segmental arrangement and the main diagnosis were correctly assessed by telemedicine, with the exception of one case of a telemedicine diagnosis of atrial septal defect which was not subsequently confirmed. Nineteen patients required urgent transfer to Lisbon, while a medical team from our department traveled to the local hospitals and performed surgical ligation of a large patent ductus arteriosus in three premature newborns and a percutaneous atrioseptostomy in one newborn with transposition of the great arteries and severe desaturation unresponsive to prostaglandins, thus avoiding the transfer of unstable patients. The other patients were referred for follow-up in local clinics or for specialist consultation, either locally or at our hospital. CONCLUSIONS: In our experience, real-time telemedicine with on-line echocardiography, conducted by a pediatric cardiologist, is an important tool in the diagnosis or exclusion of pediatric cardiovascular diseases in patients admitted to remote hospitals. It plays an important role in continuous medical training for the staff of those hospitals, especially in the field of pre-and post-natal echocardiography. Telemedicine has significant medical, economic and social benefits for patients, families and institutions in remote areas, particularly in the field of pediatric cardiology.

Percutaneous closure of perimembranous ventricular septal defects: initial experience in Portugal.

INTRODUCTION: Closure of ventricular septal defects (VSDs) with significant shunt is indicated due to the risks associated with increased pulmonary flow leading to left chamber dilatation, the possibility of cardiac dysfunction and arrhythmias, and the risk of bacterial endocarditis. Percutaneous VSD closure is an effective and safe alternative to surgery in selected patients. However, perimembranous VSD (PMVSD) constitutes a special case since the technique for percutaneous closure is more complex and hence warrants individual evaluation. OBJECTIVES: To assess the efficacy and safety of percutaneous closure of PMVSD based on the initial experience of our center, the first to use this technique in Portugal. METHODS: Five patients, aged between 5 and 23 years, with PMVSD of a suitable size for percutaneous closure, were selected since they showed evidence of a significant left-to-right shunt together with left chamber dilatation. The procedure was performed under general anesthesia, guided by fluoroscopy and transesophageal echocardiography (TEE). A femoro-femoral arteriovenous loop was established and an Amplatzer occluder implanted, the characteristics and dimensions of which were chosen according to the angiographic and TEE findings. Besides therapeutic efficacy and complications during the procedure and follow-up, left ventricular dimensions and function and degree of mitral, aortic and tricuspid regurgitation were also assessed pre- and post-procedure, during a follow-up of 5 to 23 months. RESULTS: All patients had a Qp:Qs ratio of > 2:1. In one case, the procedure was abandoned as the VSD diameter was over 17 mm, and the patient was referred for surgical closure. In the other four patients, two membranous (8 and 16 mm) and two muscular (6 and 12 mm) VSD occluders were used. On final angiographic assessment, no residual shunt was observed in any patient. One patient presented a transient junctional rhythm during the procedure. During follow-up, there was no prolongation of the PQ interval or de novo aortic regurgitation in any patient. Catheterization time ranged between 90 and 176 minutes and fluoroscopy time between 10 and 17 minutes. CONCLUSIONS: 1) Percutaneous closure of PMVSD is an effective and safe method of treatment in selected patients. 2) It is a complex procedure, but is likely to be refined and extended to a larger number of patients in suitably qualified centers.

Primary cardiac tumors in children: a 16-year experience.

INTRODUCTION: Primary cardiac tumors are rare in children. The majority are benign and noninvasive, although they may have significant hemodynamic consequences depending on their location. The most frequent type in children is rhabdomyoma, usually multiple and intraventricular. OBJECTIVES: To assess the presentation and outcome of patients with cardiac tumors followed by the Department of Pediatric Cardiology of Hospital de Santa Cruz. METHODS: A retrospective analysis was performed of the clinical records of patients diagnosed with primary cardiac tumors between January 1992 and March 2008. RESULTS: Eleven children with a diagnosis of primary cardiac tumor were identified, three of them detected antenatally. In the remaining cases, median age at diagnosis was 27.5 months, ranging from two days to 13 years. Seven (64%) were male. In three cases diagnosis was made during fetal echocardiography, and in the others by transthoracic echocardiogram. In six patients magnetic resonance imaging was used to confirm the diagnosis. All were benign (eight rhabdomyomas, one papillary fibroelastoma, one myxoma and one pericardial tumor with histological features of Castleman disease). Six patients were asymptomatic at the time of diagnosis; two cases presented with arrhythmias; one with heart failure; and two were diagnosed following stroke, one with right hemiparesis, and the other with paresthesias of the lip and fingers of the right hand. In four cases there was total or partial tumor regression. Surgery was performed in four patients, due to tumor location and/ or significant left ventricular outflow tract obstruction. One of these patients died following surgery due to multiorgan system failure. FINAL REMARKS: Primary cardiac tumors are uncommon in children. Their treatment depends mainly on location and histological type. The most serious complications are ventricular outflow tract obstruction and thromboembolic events, which are associated with a poor prognosis even when surgery is attempted.

Hemoptysis in a single lung Fontan: percutaneous approach.

BACKGROUND: The Fontan procedure is sometimes the last palliative surgery for complex congenital heart disease with single-ventricle physiology. According to some authors, atrial-baffle fenestrations with right-to-left shunts have improved the surgical outcome of the Fontan operation. Percutaneous fenestration closure at a later stage may increase saturations and reduce the risk of embolic complications. A case could be made that the fenestration should be left patent if the patient, having lost the continuity of the pulmonary artery to one lung, only has a single functional lung. CASE REPORT: The case of an 11-year-old boy with a severe form of Ebstein anomaly is reported. He had undergone a fenestrated Fontan procedure and had lost the continuity to the left pulmonary artery. Further intervention was discouraged at other centers but he was eventually referred to our institution due to increasing cyanosis and severe hemoptysis. Several right-sided arteriovenous pulmonary malformations and collateral arteries were embolized percutaneously; a major right-to-left shunt at the fenestration in the atrial baffle was also occluded with an Amplatzer device. His saturation rose from 78 to 94% and his exercise tolerance increased, without further hemoptysis.

How do we live with chronic disease? A rights-based approach promoting the wellbeing of children with chronic disease.

This paper focuses on the life experiences of children with chronic disease, a group whose invisibility involves particular challenges in their relationship with professionals in important life contexts, such as family, school and hospital. The study includes two complementary phases: i) Phase 1, composed of 15 interviews with parents, education and health professionals and two focus group discussions with children, and children and their mothers; and ii) Phase 2, which included self-report questionnaires administered to parents (n = 152) and children with chronic disease (n = 176). Based on a mixed methodology, this study combines quantitative and qualitative methods assuming that plural approaches allow for a deeper understanding of the life conditions of children with chronic disease and their families. The results reinforce the reproduction of social stereotypes and the tendency to focus on the individual ability to solve problems, which still remain to be circumscribed to the people's chronic disease sphere. Moreover, this paper reveals the central role that inclusive contexts have on children's wellbeing.

Aortopulmonary window--a review of nine cases.

INTRODUCTION: Aortopulmonary window (APW) is a rare anomaly, accounting for 0.1% of congenital heart defects. It consists of a communication between the ascending aorta and the pulmonary artery in the presence of normal separate aortic and pulmonary valves. Early treatment is usually required in order to prevent the development of irreversible pulmonary hypertension. OBJECTIVE: To assess the results of treatment in all patients diagnosed at our institution between January 1994 and November 2007, based on a retrospective longitudinal study. RESULTS: Nine patients treated for APW were identified. Their ages at diagnosis ranged from two days to 23 years; eight were infants aged 2 +/- 2.9 months. In this group clinical presentation was congestive heart failure in all cases. Five patients had associated lesions (interrupted aortic arch: 2; coarctation of the aorta: 2; VSD: 1; ASD: 3). In seven cases the diagnosis was made on the basis of echocardiography only. In the other two it was by cardiac catheterization, one patient with coarctation of the aorta and the other an adult patient with a smaller lesion who was initially misdiagnosed as having ductus arteriosus. All patients had corrective surgery, via a transaortic approach, with implantation of an autologous pericardial patch. All patients are alive and only one case has a small residual shunt at the correction site. CONCLUSION: A diagnosis of APW should always be kept in mind in the differential diagnosis of a child with congestive heart failure. Associated congenital heart anomalies should be excluded. Surgical repair appears to provide good short- and long-term results.

From an oppressed citizenship to affirmative identities: lesbian and gay political participation in Portugal.

In this paper we intend to articulate a multidimensional perspective on citizenship with a psychological understanding of lesbian and gay identities' development in the context of a Southern European country: Portugal. We begin by reviewing some legal statements and institutional regulations around gay and lesbian issues and the lack of opportunities for the affirmation of a non-hegemonic (sexual) identity in Portugal. Next, we describe participation efforts developed by the Portuguese LGBT nongovernmental organizations (NGOs) and the actual results that such efforts already produced in the political and cultural attitudes toward gay men and lesbians: particularly, the legal approval of domestic same-sex partnership is emphasized as a symbolic achievement of such political struggle. Finally, we explore the implications of communitarian participation for gay and lesbian identities' development, not just in terms of collective empowerment but also in what concerns individual development and well-being.

[Health and school: thoughts on the medicalization of education]. / Saúde e escola: reflexões em torno da medicalização da educação.

Children and youth reach school with different starting points. It is not known for sure how far these children and youth will go, and what path the school holds for them, particularly at a stage in which teachers are divided in multi-tasking (with some tasks that are merely administrative). Meanwhile, it is increasingly common to explain students' "inappropriate" behavior in biomedical terms. The increasing emergence of disorders and deficits calls for critical reflection on what they actually involve in public health terms. Thisarticle addresses the school's role in the educational achievement and comprehensive development of students flagged for or with clinical indication of medication based on "fuzzy" diagnoses. The concept of medicalization acquires a central position, and the article discusses its implications based on a set of field notes and interviews with parents and teachers in the North of Portugal.

Linking Learning Contexts: The Relationship between Students' Civic and Political Experiences and Their Self-Regulation in School.

This paper considers the relationship between self-regulation strategies and youth civic and political experiences, assuming that out-of-school learning can foster metacognition. The study is based on a sample of 732 Portuguese students from grades 8 and 11. Results show that the quality of civic and political participation experiences, together with academic self-efficacy, are significant predictors of young people's self-regulation, particularly regarding cognitive and metacognitive strategies (elaboration and critical thinking). Such effects surpass even the weight of family cultural and school variables, such as the sense of school belonging. Therefore, we argue that the pedagogical value of non-formal civic and political experiences is related to learning in formal pedagogical contexts. This is because civic and political participation with high developmental quality can stimulate higher-order cognitive engagement and, thus, contribute to the development of learning strategies that promote academic success.

[Congenital Heart Disease in Children with Down Syndrome: What Has Changed in the Last Three Decades?] / Cardiopatia Congénita em Crianças com Síndrome de Down: O que Mudou nas Últimas Três Décadas?

INTRODUCTION: The prevalence of Down syndrome has increased in the last 30 years; 55% of these children have congenital heart disease. MATERIAL AND METHODS: A retrospective longitudinal cohort study; clinical data from 1982 to 2013 databases with the diagnosis of Down syndrome or trisomy 21 in a reference hospital in pediatric cardiology and cardiac surgery. OBJECTIVE: to assess the progress in the last three decades of cardiological care given to children with Down syndrome and congenital heart disease. RESULTS: We studied 102 patients with Down syndrome and congenital heart disease subjected to invasive therapy: corrective or palliative cardiac surgery and therapeutic catheterization. The referral age was progressively earlier in patients referred in the first year of life. The most frequent diagnosis was complete atrioventricular sptal defect (41%). There was a trend towards increasingly early corrective surgery in patients under 12 months (p < 0.001). Since 2000, the large majority of patients were operated before reaching six months of age. The main cardiac complications were rhythm dysfunction and low output. More frequent noncardiac complications were pulmonary and infectious. The 30-day mortality rate was 3/102 cases (2.9%). Of patients in follow-up, 89% are in NYHA class I. DISCUSSION AND CONCLUSION: The early surgical correction seen over the past 15 years follows the approach suggested in the literature. The observed 30-day mortality rate is overlapping international results. Patients with Down syndrome subjected to corrective surgery of congenital heart disease have an excellent long-term functional capacity.

Introdução: A prevalência da síndrome de Down tem aumentado nos últimos 30 anos; 55% destas crianças apresentam cardiopatia congénita. Material e Métodos: Estudo retrospetivo longitudinal de coorte; dados clínicos obtidos em bases de dados de 1982 a 2013 com o diagnóstico de síndrome de Down ou trissomia 21 num hospital de referência em cardiologia pediátrica e cirurgia cardíaca. Objetivo: Avaliar a evolução, nas últimas três décadas, dos cuidados cardiológicos prestados às crianças com síndrome de Down ecardiopatia congénita.Resultados: Estudámos 102 doentes com síndrome de Down e cardiopatia congénita submetidos a terapêutica invasiva: cirurgiacardíaca corretiva, paliativa e cateterismo terapêutico. Em doentes referenciados no primeiro ano de vida, a referenciação foi cada vez mais precoce. O diagnóstico mais frequente foi o defeito completo do septo aurículo-ventricular (41%). Verificou-se uma tendência para cirurgia corretiva cada vez mais precoce em doentes abaixo dos 12 meses (p < 0,001). A partir de 2000, a grande maioria dos doentes foi operada antes dos seis meses de idade. As principais complicações cardíacas foram alterações de ritmo e baixo débito e as principais não cardíacas foram pulmonares e infeciosas. A taxa de mortalidade a 30 dias foi de 3/102 casos (2,9%). Dos doentes em follow-up, 89% estão em classe funcional I da NYHA. Discussão e Conclusão: A correção cirúrgica mais precoce verificada nos últimos 15 anos vai ao encontro do proposto na literatura. A taxa de mortalidade a 30 dias verificada é sobreponível aos resultados internacionais. Os doentes com síndrome de Down submetidos a cirurgia corretiva de cardiopatia congénita apresentam uma excelente capacidade funcional a longo prazo.

[Complex Congenital Heart Disease: The Influence of Prenatal Diagnosis]. / Cardiopatias Congénitas Complexas: Influência do Diagnóstico Pré-Natal.

INTRODUCTION: Complex congenital heart disease is a group of severe conditions. Prenatal diagnosis has implications on morbidity and mortality for most severe conditions. The purpose of this work was to evaluate the influence of prenatal diagnosis and distance of residence and birth place to a reference center, on immediate morbidity and early mortality of complex congenital heart disease. MATERIAL AND METHODS: Retrospective study of complex congenital heart disease patients of our Hospital, born between 2007 and 2012. RESULTS: There were 126 patients born with complex congenital heart disease. In 95%, pregnancy was followed since the first trimester, with prenatal diagnosis in 42%. There was a statistically significant relation between birth place and prenatal diagnosis. Transposition of great arteries was the most frequent complex congenital heart disease (45.2%), followed by pulmonary atresia with ventricular septal defect (17.5%) and hypoplastic left ventricle (9.5%). Eighty-two patients (65.1%) had prostaglandin infusion and 38 (30.2%)were ventilated before an intervention. Surgery took place in the neonatal period in 73%. Actuarial survival rate at 30 days, 12 and 24 months was 85%, 80% and 75%, respectively. There was no statistically significant relation between prenatal diagnosis and mortality. DISCUSSION: Most patients with complex congenital heart disease did not have prenatal diagnosis. All cases with prenatal diagnosis were born in a tertiary center. Prenatal diagnosis did not influence significantly neonatal mortality, as already described in other studies with heterogeneous complex heart disease. CONCLUSION: prenatal diagnosis of complex congenital heart disease allowed an adequate referral. Most patients with complex congenital heart disease werenâÄôt diagnosed prenatally. This data should be considered when planning prenatal diagnosis of congenital heart disease.

Introdução: As cardiopatias congénitas complexas são patologias graves, e o diagnóstico pré-natal poderá ter implicações sobre a morbilidade e a mortalidade. O objetivo deste trabalho foi estudar a influência do diagnóstico pré-natal e da distância do local de parto a um centro de referência, na morbilidade imediata e mortalidade precoce de um grupo de doentes com cardiopatias congénitas complexas.Material e Métodos: Análise retrospetiva dos doentes com cardiopatias congénitas complexas, seguidos no nosso hospital, nascidos entre 2007 e 2012.Resultados: Identificaram-se 126 doentes com cardiopatias congénitas complexas. Em 95% a gravidez foi vigiada desde o primeiro trimestre existindo diagnóstico pré-natal em 42%. Houve relação estatisticamente significativa entre o local do parto e a existência de diagnóstico pré-natal. A cardiopatia congénita complexa mais frequente foi a transposição das grandes artérias (45,2%), seguida da atresia da pulmonar com comunicação interventricular (17,5%) e ventrículo esquerdo hipoplásico (9,5%). Oitenta e dois doentes (65,1%) foram medicados com prostaglandinas e 38 (30,2%) foram ventilados antes de uma intervenção. A cirurgia ocorreu no período neonatal em 73%. A sobrevida atuarial aos 30 dias, 12 e 24 meses foi 85%, 80% e 75%, respetivamente. Não houve relação estatisticamente significativa entre diagnóstico pré-natal e mortalidade.Discussão: A maioria dos doentes com cardiopatias congénitas complexas não teve diagnóstico pré-natal. Nos casos com diagnóstico pré-natal houve referenciação e parto num centro terciário. Não houve associação estatisticamente significativa entre diagnóstico prénatal e mortalidade neonatal, como já descrito em séries heterogéneas de cardiopatia congénita complexa.Conclusão: A maioria dos doentes com cardiopatias congénitas complexas não teve diagnóstico pré-natal. Estes dados devem ser tomados em conta no planeamento do diagnóstico pré-natal das cardiopatias congénitas.