Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge / Gravidez em Portadoras de Cardiopatias Congênitas Complexas: Um Constante Desafio

Abstract Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.

Resumo Fundamento: A contínua habilidade na conduta das cardiopatias congênitas complexas (CCC) tem permitido o alcance da idade fértil. Contudo, a heterogeneidade das lesões cardíacas na idade adulta limita a estimativa do prognóstico da gravidez. Objetivo: Estudar a evolução materno-fetal das gestantes portadoras de CCC e analisar as variáveis presumíveis de prognóstico. Método: No período de 10 anos, 435 gestantes portadoras de cardiopatias congênitas foram consecutivamente incluídas no Registro do Instituto do Coração (Registro-InCor). Dentre elas, foram selecionadas 42 gestações em 40 mulheres com CCC (24,5 ± 3,4 anos) que haviam sido desaconselhadas a engravidar. As cardiopatias de base distribuíram-se em: transposição das grandes artérias, atresia pulmonar, atresia tricúspide, ventrículo único, dupla via de saída de ventrículo direito, dupla via de entrada de ventrículo esquerdo e outras lesões estruturais. As cirurgias realizadas foram Rastelli, Fontan, Jatene, Senning, Mustard e outros procedimentos combinados, como tunelização, Blalock Taussing e Glenn. Oito pacientes (20%) não haviam sido operadas, e 19 (47,5%) apresentavam hipoxemia. O protocolo de atendimento incluiu: registro da saturação de oxigênio, hemoglobina sérica, hematócrito, ajuste das medicações, anticoagulação individualizada e hospitalização a partir de 28 semanas de gestação, em face da gravidade do quadro clínico e obstétrico. Na análise estatística, o nível de significância adotado foi de 0,05. Resultado: Somente 17 gestações (40,5%) não tiveram complicações maternas nem fetais. Houve 13 problemas maternos (30,9%) e 2 mortes (4,7%) causadas por hemorragia pós-parto e pré-eclâmpsia grave, ambas em pacientes que apresentavam hipoxemia. Houve 7 perdas fetais (16,6%), 17 bebês prematuros (40,5%) e 2 recém-nascidos (4,7%) com cardiopatia congênita. As complicações materno-fetais foram significativamente maiores em pacientes que apresentavam hipoxemia (p < 0,05). Conclusão: O alcance da idade reprodutiva em pacientes com CCC é crescente; contudo, a má evolução materno-fetal desaconselha a gravidez, particularmente nas pacientes que apresentam hipoxemia.

Surgical management of aortic arch obstruction associated with intracardiac anomalies in children / 中国医师杂志

Objective To summarize the clinical experience in repair of aortic arch obstruction associated with intracardiac anomalies in children retrospectively.Methods From March 2010 to March 2014,73 children diagnosed as coarctation of the aorta (CoA,n =68),interrupted aortic arch (IAA,n =3),and double aortic arch with CoA (n =2) underwent surgical management.Six of them were complicated with complex intracardiac anmalies,including tetralogy of Fallot (TOF,n =2),transposition of great arteries (TGA,n =1),total anomalous pulmonary venous connection (TAPVC,n =1),double outlet of right ventricle (DORV,n =1),and Shone's syndrome (n =1) ; the rest 67 patients were associated with ventricular septal defect (VSD) and other simple anomalies.Twenty eight cases had hypoplasia of the aortic arch.All the patients had one-stage repair except for one.The aortic arch reconstruction was end to end anastomosis between the descending aorta and the arch in 42 patients,end to side anastomosis in 22,and the aortic arch were enlarged using autologous pulmonary artery patch in 9.The associated intracardiac anomalies were repaired in the same stage.Results There were 2 deaths.The operative mortality was 2.7％.Renal failure was occurred in 2 cases who were cured afterwards by peritoneal dialysis.All survivors were followed up for 3 ～ 36 months,anastomotic restenosis was found in 1 case who underwent reoperation 14 months after the first operation.No neurological complications were occurred.Conclusions One-stage complete correction of CoA and IAA with intracardiac anomalies through median sternotomy can achieve excellent short-and mid-term surgical results.

Toxoplasmose congênita: evolução da função auditiva e da linguagem emcrianças diagnosticadas e tratadas precocemente / Congenital toxoplasmosis: auditory and language outcomes inearly diagnosed and treated children

A cross-sectional study included all children diagnosed with congenital toxoplasmosis, through the Minas Gerais State Neonatal Screening Program, from September 2006 to March 2007. All children received early treatment, initiated before the age of 2.5 months, and were periodically assisted by a team of specialists including pediatricians, ophthalmologists and speech-language therapists and audiologists. Hearing function was evaluated with the following procedures: tympanometry, transient evoked otoacoustic emissions, distortion product otoacoustic emissions, behavioral observation audiometry, and brainstem auditory evoked potentials. Hearing function and sensitivity was estimated and audiological results were classified as normal, conductive hearing loss, sensory-neural hearing loss and central dysfunction. Language performance was assessed and classified as normal or abnormal, according to test results. The following variables were studied: audiological results, neurological and ophthalmological conditions, language performance and presence of risk indicator for hearing loss other than congenital toxoplasmosis. Univariate analysis was conducted using the chi-square or Fisher?s Exact test. Results: From September 2006 to March 2007, 106 children were diagnosed with congenital toxoplasmosis through the neonatal screening program, and were included in the study. Data analysis showed normal hearing in 60 children (56.6%), while 13 children (12.3%) had conductive hearing loss, four children (3.8%) had sensory-neural hearing loss and 29 children (27.4%) presented central hearing dysfunction. There was association between hearing problems and language deficits. The comparison between children with additional risks for hearing loss other than toxoplasmosis and children who only presented toxoplasmosis as a risk factor showed no differences. This finding suggests that audiological problems were due to congenital toxoplasmosis alone.

Um estudo transversal descritivo incluiu todas as crianças diagnosticadas com toxoplasmose congênita (TC) pelo Prog. Est. de Tria. Neonatal de MG entre set. 2006 e mar. de 2007. Todas as crianças foram submetidas ao protocolo de tratamento com pirimetamina e sulfadiazina iniciado antes dos 2,5 meses de idade e com duração de 12 meses, tendo realizado acompanhamento pediátrico, oftalmológico e fonoaudiológico periódico. Para avaliar a audição foram usados, como instrumentos diagnósticos, medidas de imitância acústica, emissões otoacústicas evocadas por estímulo transiente e produto de distorção, potencial evocado auditivo de tronco encefálico e observação do comportamento auditivo. Foi avaliada a acuidade auditiva e as alterações auditivas foram classificadas em condutivas, neurossensoriais e retrococleares. O desempenho de ling. foi avaliado usando-se um instrumento de aval. do desenvolvimento da ling., e os resultados foram classificados como normais ou alterados. As seguintes variáveis foram estudadas: resultados audiológicos, condições neurológicas e oftalmológicas, linguagem e presença de fator de risco para perda auditiva além da TC. Foi realizada análise univariada pelo qui-quadrado ou teste exato de Fisher. Resultados: entre set. 2006 e mar. 2007, 106 crianças foram diagnos. com TC pelo programa de triagem neonatal, sendo incluídas no estudo. A análise dos dados mostrou que 60 crianças apresentavam audição normal (56,6%) e 46 crianças apresentavam audição alterada, sendo 13 crianças (12,3%) com alteração condutiva, 4 (3,8%) com perda auditiva neurossensorial e 29 (27,4%) com comprometimento retrococlear. Houve associação entre presença de alteração auditiva e déficit de linguagem. A comparação entre crianças que apresentavam outro fator de risco além da TC e crianças que apresentavam somente a toxoplasmose como fator de risco para alteração auditiva não mostrou diferenças.

Toxoplasmose congênita em crianças sul-americanas: [revisão] / Congenital toxoplasmosis in south American children: [review]

Aims: To review the present knowledge about congenital toxoplasmosis in South America and to advance some hypothesis for future research. Source of data: Medline and Scielo database search for papers reporting clinical characteristics of cohorts of children in South America and comparative studies between South America and other continents. Summary of findings: Systematic analysis of primary data obtained during screening programs showed that the risk of ocular lesions in congenital toxoplasmosis was much higher in the South American cohorts (47%; 18/38) than in Europe (14%, 79/550). The crude risk of intracranial lesions was much higher in the cohorts from South America (53%, 20/38) than those from Europe (9%, 49/550). In a Colombian cohort it was found 11% of mortality. Additionally, a comparative prospective cohort of congenitally infected children from Brazil and Europe found that in Brazilian children eye lesions were larger, more numerous and more likely to affect the area of the retina responsible for central vision that their counterpart in Europe. The presence of Toxoplasma strains genetically different to those found in North America and Europe could explain the higher severity of congenital toxoplasmosis in South America. Conclusions: Congenital toxoplasmosis in South America seems to be more frequent and infected children are more symptomatic than in Europe and in North America. Research for new drugs and candidate vaccines are a priority to improve indicators of health in children of South America.

Objetivos: revisar o conhecimento atual sobre toxoplasmose congênita na América do Sul e traçar algumas hipóteses para futura pesquisa. Fonte de dados: busca nas bases de dados Pubmed e Scielo por artigos sobre características clínicas de coortes de crianças com toxoplasmose congênita na América do Sul e estudos comparativos entre América do Sul e outros continentes. Síntese dos dados: uma análise sistemática de dados primários obtidos durante programas de triagem mostrou que o risco de lesões oculares foi muito maior na coorte de crianças da América do Sul (47%, 18/38) do que nas européias (14%, 79/550). O risco bruto de lesões intracranianas foi muito maior na coortes da América do Sul (53%, 20/38) do que nas da Europa (9%, 49/550). Em uma coorte colombiana constatou-se 11% de mortalidade. Adicionalmente, uma coorte prospectiva, que comparou crianças com toxoplasmose congênita do Brasil e da Europa, mostrou que nas crianças brasileiras as lesões oculares foram maiores, mais numerosas e com maior probabilidade de atingir o polo posterior da retina do que nas européias. A presença de cepas de Toxoplasma gondii diferentes das da Europa e dos Estados Unidos pode explicar a maior gravidade da toxoplasmose congênita na América do Sul. Conclusões: a toxoplasmosis congênita na América do Sul parece ser mais frequente e as crianças infectadas são mais sintomáticas do que na Europa e na América do Norte. A pesquisa sobre novas drogas e vacinas deve ser prioritária, para melhorar os indicadores de saúde nas crianças da América do Sul.