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.

Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge.

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.

Influence of pregnancy on clinical course and fetal outcome of women with hypertrophic cardiomyopathy.

OBJECTIVES: To study clinical evolution of women with HCM during pregnancy; the influencing factors of gestation on natural course of HCM and the frequency of HCM in their children in early childhood. METHODS: A prospective study was conducted in 35 women with HCM; there were 23 pregnant women (PG group) and 12 nonpregnant control patients (NP group), matched for age and functional class (FC). Clinical monthly evaluations were carried out and electrocardiogram and transthoracic echocardiography tests were performed. The offspring endpoints included stillbirth and prematurity rates and investigation of HCM during childhood. RESULTS: No deaths occurred in either group. Cardiac arrhythmias were significantly (p< 0.05) more frequent in the NP group (33.3% vs. 13.4%), and no differences were observed between the groups (p>0.05) in heart failure (30.3% vs. 16.6%) or ischemic stroke (4.3% vs. 8.3%) rates. In the PG group, required hospitalization for treatment of cardiac complication was more frequent (p=0.05) in patients with family history of HCM (71.4% vs. 25.0%). Cesarean section was performed in 12 (52%) patients, for obstetrical reasons; there were 7 (30.4%) premature babies and 1 (4.3%) neonatal death. One child was clinically diagnosed as having HCM, and his genetic study identified a mutation in the beta myosin heavy chain gene, located on chromosome 14. CONCLUSION: Heart failure is a frequent cardiac complication in women with HCM during pregnancy, particularly in patients with family history of the disease, but this did not influence the natural course of HCM. In one child, clinical examination allowed HCM identification during early childhood.

Influência da gestação na evolução clínica materno-fetal de portadoras de cardiomiopatia hipertrófica / Influence of pregnancy on clinical course and fetal outcome of women with hypertrophic cardiomyopathy

OBJETIVOS: Estudar a evolução clínica de gestantes com CMH, a influência da gestação sobre o curso natural da CMH e a freqüência da doença nos filhos dessas mulheres no início da infância. MÉTODOS: Foi realizado um estudo prospectivo em 35 portadoras de CMH com a mesma idade e classe funcional (CF). Vinte e três estavam grávidas (grupo G), e 12 pacientes que não estavam grávidas serviram como controle (grupo NG). Foram realizadas avaliações clínicas mensais, bem como eletrocardiograma e ecocardiograma transtorácico Os desfechos para os filhos foram taxas de natimortalidade e prematuridade e investigação de CMH durante a infância. RESULTADOS: Não houve mortes em nenhum dos dois grupos. A ocorrência de arritmias cardíacas foi significantemente maior (p< 0,05) no grupo NG (33,3 por cento vs. 13,4 por cento), e não foram observadas diferenças nos índices de insuficiência cardíaca (30,3 por cento vs. 16,6 por cento) ou acidente vascular cerebral isquêmico (4,3 por cento vs. 8,3 por cento) entre os grupos (p > 0,05). No grupo NG, a necessidade de hospitalização para tratamento de complicações cardíacas foi mais freqüente (p = 0,05) em pacientes com história familiar de CMH (71,4 por cento vs. 25,0 por cento). Doze pacientes (52 por cento) foram submetidas à cesariana por razões obstétricas, sete bebês (30,4 por cento) nasceram prematuramente e um bebê (4,3 por cento) teve morte neonatal. Uma criança teve diagnóstico clínico de CMH, e seu estudo genético identificou mutação no gene da cadeia pesada da b-miosina, situado no cromossomo 14. CONCLUSÃO: Insuficiência cardíaca é uma complicação freqüente em portadoras de CMH durante a gravidez, principalmente em pacientes com antecedentes familiares da doença, mas não altera o curso natural da CMH. Em uma criança, o exame clínico identificou HMC no início da infância.

OBJECTIVES: To study clinical evolution of women with HCM during pregnancy; the influencing factors of gestation on natural course of HCM and the frequency of HCM in their children in early childhood. METHODS: A prospective study was conducted in 35 women with HCM; there were 23 pregnant women (PG group) and 12 nonpregnant control patients (NP group), matched for age and functional class (FC). Clinical monthly evaluations were carried out and electrocardiogram and transthoracic echocardiography tests were performed. The offspring endpoints included stillbirth and prematurity rates and investigation of HCM during childhood. RESULTS: No deaths occurred in either group. Cardiac arrhythmias were significantly (p< 0.05) more frequent in the NP group (33.3 percent vs. 13.4 percent), and no differences were observed between the groups (p>0.05) in heart failure (30.3 percent vs. 16.6 percent) or ischemic stroke (4.3 percent vs. 8.3 percent) rates. In the PG group, required hospitalization for treatment of cardiac complication was more frequent (p=0.05) in patients with family history of HCM (71.4 percent vs. 25.0 percent). Cesarean section was performed in 12 (52 percent) patients, for obstetrical reasons; there were 7 (30.4 percent) premature babies and 1 (4.3 percent) neonatal death. One child was clinically diagnosed as having HCM, and his genetic study identified a mutation in the beta myosin heavy chain gene, located on chromosome 14. CONCLUSION: Heart failure is a frequent cardiac complication in women with HCM during pregnancy, particularly in patients with family history of the disease, but this did not influence the natural course of HCM. In one child, clinical examination allowed HCM identification during early childhood.

Effect of enalapril on left ventricular diameters and exercise capacity in asymptomatic or mildly symptomatic patients with regurgitation secondary to mitral valve prolapse or rheumatic heart disease.

The effects of 12 months of therapy were evaluated in 47 mildly symptomatic patients with moderate to severe mitral valve regurgitation; 26 patients received enalapril and 21 received a placebo. Enalapril was associated with a significant reduction in left ventricular diameter and mitral regurgitation volume, with no evidence of change in systolic function indexes. However, enalapril did not hinder progressive aerobic impairment to effort.

Aspectos práticos da abordagem terapêutica de pacientes com disfunção ventricular secundária a valvopatia / Practical aspects of the management of the ventricular dysfuntion related to valvar heart disease

O tratamento das valvopatias deve sempre considerar o dano secundário ao miocárdio. Na indicação cirúrgica, deve-se sempre avaliar judiciosamente a evolução da disfunção ventricular, ocasionada pela remodelação e pela fibrose intersticial. Se não for corrigida em tempo adequado, a valvopatia irá se comportar, no futuro, como verdadeira "miocardiopatia". Após a correção da valvopatia existe o remodelamento reverso, com melhora da função global do coração. O grau desse remodelamento reverso relaciona-se com o prognóstico pós-operatório. O atual arsenal terapêutico dos inibidores da enzima de conversão, betabloqueadores, inibidores da angiotensina II e aldactone desempenha um papel na melhora da remodelação reversa no pós-operatório.

Tratamento medicamentoso da estenose aórtica / Medical treatment of aortic stenosis

A estenose aórtica importante deve implicar tratamento cirúrgico. Na evolução pré-cirúrgica deve-se avaliar a profilaxia da endocardite infecciosa e da doença reumática. Atualmente têm sido usadas estatinas para retardar a evolução da estenose aórtica. Medidas medicamentosas podem ser usadas para compensação do paciente, na preparação do tratamento cirúrgico, ou quando não há indicação deste. Entre as drogas usadas destacam-se os digitálicos e os diuréticos. O uso de vasodilatador pode ser justificado em grupo restrito de pacientes, como ponte para tratamento cirúrgico.

Pregnancy in patients with heart disease: experience with 1,000 cases.

BACKGROUND: Previously, the high maternal mortality in cardiac patients who became pregnant prompted the assertion: Women with an abnormal heart should not become pregnant. This long-standing notion needs to be revised today. HYPOTHESIS: The study was undertaken to ascertain the experience with a large series of pregnant women with cardiac disease cared for in the same referral center. METHODS: From 1989 to 1999, 1,000 pregnant women with heart disease were followed by the same clinical and obstetric team. The cardiac diseases included rheumatic heart disease (55.7%), congenital heart disease (19.1%), Chagas' disease (8.5%), cardiac arrhythmias (5.1%), cardiomyopathies (4.3%), and others (7.3%). RESULTS: Of the pregnant women studied, 765 (76.5%) experienced no cardiovascular events during the study; 235 (23.5%) patients had the following cardiovascular complications: congestive heart failure (12.3%), cardiac arrhythmias (6%), thromboembolism (1.9%), angina (1.4%), hypoxemia (0.7%), infective endocarditis (0.5%), and other complications (0.7%). Clinical treatment allowed adequate management in 161 (68.8%) patients; however, 46 (19.6%) patients underwent interventional procedures because of refractory complications. The general maternal mortality rate was 2.7%. Of the 915 (91.5%) infants who were discharged, 119 (13%) were premature. CONCLUSION: Pregnancy in women with heart disease is still associated with considerable morbidity and mortality rates, which strongly correlate to maternal underlying disease. Strict prenatal care and early risk stratification during gestation are fundamental measures to improve the prognosis of pregnancy in women with heart disease.

Pregnancy and peripartum cardiomyopathy. A comparative and prospective study.

OBJECTIVE: To assess pregnancy outcome in women with peripartum cardiomyopathy and to compare it with idiopathic cardiomyopathy. METHODS: Twenty-six pregnant women, aged 28.4+/-6.1 years, with dilated cardiomyopathy were followed. Eighteen patients had peripartum cardiomyopathy [11 with persistent left ventricular systolic dysfunction (EF=45.2+/-2) and 7 with recovered ventricular function (EF=62.3+/-3.6)]. The 8 remaining patients had idiopathic cardiomyopathy (EF= 43.5+/-4.1). During the prenatal period, limited physical activity and a low-sodium diet were recommended, and hospitalization was recommended when complications occurred. RESULTS: Of the 26 patients, 11 (42.3%) had a normal delivery; 9(35.5%) had cardiac complications, 6 (22.2%) had obstetric complications. Two patients (7.7%) died. Two preterm pregnancies occurred, with 26 health newborns (2 sets of twins). Two miscarriages took place. The cardiac complication rate during pregnancy was lower (p<0.009) in the peripartum cardiomyopathy group without ventricular dysfunction and greater (p=0.01) in the idiopathic group when compared with the peripartum group with ventricular dysfunction. Changes in left ventricular ejection fraction were not observed (p<0.05) in the postpartum period, when compared with that during pregnancy in the 3 groups. CONCLUSION: Pregnancy in patients with dilated cardiomyopathy is associated with maternal morbidity. Left ventricular function is a prognostic factor and must be the most parameter when counseling patients with peripartum cardiomyopathy about a new pregnancy.

Gravidez em portadoreas de cardiomiopatia periparto. Estudo prospectivo e comparativo / Pregnancy and peripartum cardiomyopathy. A comparative and prospective study

OBJECTIVE - To assess pregnancy outcome in women with peripartum cardiomyopathy and to compare it with idiopathic cardiomyopathy. METHODS - Twenty-six pregnant women, aged 28.4±6.1 years, with dilated cardiomyopathy were followed. Eighteen patients had peripartum cardiomyopathy [11 with persistent left ventricular systolic dysfunction (EF=45.2±2) and 7 with recovered ventricular function (EF=62.3±3.6)]. The 8 remaining patients had idiopathic cardiomyopathy (EF= 43.5±4.1). During the prenatal period, limited physical activity and a low-sodium diet were recommended, and hospitalization was recommended when complications occurred. RESULTS - Of the 26 patients, 11 (42.3 percent) had a normal delivery; 9(35.5 percent) had cardiac complications, 6 (22.2 percent) had obstetric complications. Two patients (7.7 percent) died. Two preterm pregnancies occurred, with 26 health newborns (2 sets of twins). Two miscarriages took place. The cardiac complication rate during pregnancy was lower (p<0.009) in the peripartum cardiomyopathy group without ventricular dysfunction and greater (p=0.01) in the idiopathic group when compared with the peripartum group with ventricular dysfunction. Changes in left ventricular ejection fraction were not observed (p<0.05) in the postpartum period, when compared with that during pregnancy in the 3 groups. CONCLUSION - Pregnancy in patients with dilated cardiomyopathy is associated with maternal morbidity. Left ventricular function is a prognostic factor and must be the most parameter when counseling patients with peripartum cardiomyopathy about a new pregnancy

Plástica da valva mitral. Ressecçäo quadrangular da cúspide posterior em portadores de degeneraçäo mixomatosa / Mitral valve repair. Quadrangular resection of the posterior leaflet in patients with myxomatous degeneration

Objetivo - Analisar os resultados imediatos e tardios da plástica da valva mitral com ressecção quadrangular da cúspide posterior, sem a utilização de anuloplastia com anel protético. Métodos - Com esta técnica foram estudados 118 portadores de prolapso da valva mitral submetidos à plástica da valva mitral, de janeiro/84 a dezembro/96. A idade variou de 30 a 86 (média de 59,1 + 11,8) anos, e 62,7 por cento eram do sexo masculino. Cirurgia associada foi realizada em 22 por cento dos pacientes, sendo a mais freqüente a revascularização do miocárdio em 15 (12,7 por cento). Em 20 (16,9 por cento) pacientes, foram utilizadas técnicas associadas de plástica da valva mitral, sendo a mais freqüente o encurtamento de cordas alongadas (6 pacientes). Resultados - A mortalidade imediata foi de um paciente (0,9 por cento). As taxas linearizadas para tromboembolismo, endocardite, reoperação e óbito no pós operatório tardio foram respectivamente 0,4 por cento, 0,4 por cento, 1,7 por cento e 2,2 por cento paciente/ano. A curva actuarial de sobrevida é de 83,8 + 8,6 por cento em 12 anos, livre do evento reoperação, 91,8 + 4,3 por cento, endocardite 99,2 + 0,8 por cento e tromboembolismo 99,2 + 0,8 por cento. No pós-operatório tardio, 93,8 por cento dos pacientes se encontram em classe funcional I (NYHA), com um seguinte completo de 89,7 por cento. Conclusão - Os portadores de prolapso da valva mitral submetidos a plástica mitral com esta técnica, apresentaram uma evolução satisfatória em 12 anos.

Prolapso de valvar mitral / Mitral valve prolapse

O prolapso da valva mitral,apesar de muito prevalente,apresenta complicacoes significativas em pequeno numero de casos.A mioria dos pacientes e assintomatica e com bom prognostico.Eventualmete,os pacientes podem apresentar queixas inespecificas como dor precordial,dispneia,palpitacoes,ansiedade e sindrome do panico.No entanto,a relacao do PVM contribui com numero significativo de casos que necessitam de tratamento cirurgico por insuficiencia mitral(IM)importante.Constitui tambem a patologia de base de grande numero de pacientes que apresentam endocardite infecciosa.O diagnostico clinico se baseia,principalmente,na auscuta de astalido mesossistolico e de sopro mesotelessistolico.O uso do ecocardiograma fornece subsidios para diagnostico.O atendimentp do portador de PVM deve ser diferenciado,de acordo com o grau de acometimento,geralmente refletido pelo grau da IM.Nao podemos esquecer que pode haver progressao da lesao,tanto de maneira cronica(dilatacao progressiva do anel,das lacineas e cordas)como de maneira aguda(endocardite infecciosa ou rotura de cordas)Atualmente,o PVM e a principal causa de insuficiencia mitral isolada de indicacao cirurgica.O desenvolvimento da cirurgia conservadora tem permitido que numero crascente de portadores de PVM tenham a correcao da sua insuficiencia mitral realizada sem uso de proteses

Interações medicamentosas em cardiologia / Drug interaction in cardiology

A interaçäo de drogas é importante fator a ser considerado na cardiologia atual.Doenças cardíacas usualmente requerem o uso crônico de medicações, frequentemente em associaçäo, aumentando o risco de interações medicamentosas.Grande porcentam das drogas é metabolizada pelo sistema das enzimas citocromo monoxidase.Drogas inibidoras ou indutoras dessa via podem levar à potencializaçäo ou à diminuiçäo da atividade dos fármacos de açäo cardiovascular.Outros fatores para interferência de drogas incluem alteraçäo da absorçäo ou efeito aditivo de drogas, quanto à toxicidade.Säo exemplos de interaçäo significativa:bloqueio cardíaco por uso concomitante de bloqueadores de cálcio e betabloqueadores; insuficiência renal pelo uso concomitante de "estatina" e genfibrozil e arritmias decorrentes da interaçäo de cisaprida e antifúngicos.O cardiologistas deve estar atento näo só à medicaçäo que prescreve, como também a outros fármacos que podem ser ingeridos pelo paciente, por automedicaçäo, ou por indicaçäo de colegas.

Avaliação comparative entre valvoplastia percutânea e comissurotomia a céu aberto na estenose mitral / Comparison between percutaneous balloon valvuloplasty and open commissurotomy for mitral stenosis

PURPOSE: To compare immediate and late (12 months) follow-up of clinical and Doppler echocardiographic results between percutaneous mitral balloon valvuloplasty and open commissurotomy in a prospective and randomized trial. METHODS: Eighty eight symptomatic patients with severe mitral stenosis and favorable anatomy were randomized in a prospective trial comparing the two procedures. All patients were submitted to clinical and Doppler echocardiographic evaluation before the procedures and immediate and twelve months thereafter. RESULTS: Mean mitral gradient (mmHg) decreased from 12.2 +/- 5.8 to 5.80 +/- 2.7 (p < 0.001) in commissurotomy group (CG) and from 11.7 +/- 6.1 to 5.0 +/- 2.4 (p < 0.001) in the balloon valvuloplasty group (VG). Mitral valve are (cm2) increased from 0.98 +/- 0.21 to 2.52 +/- 0.46 in CG and from 1.05 +/- 0.25 to 2.18 +/- 0.40 in VG (p < 0.001). In both groups there was a slight decrease in mitral valve area at 12 month follow-up. There was no death in either group. One patient in the VG had moderate mitral regurgitation and underwent surgery. At the 12 month follow-up, all patients in CG and 97.7of patients in VG were in New York Heart Association functional class I or II. CONCLUSION: Both procedures were safe and showed similar immediate improvement in mitral gradient and functional class. Mitral valve area had a greater increase immediately after commissurotomy, however, there was a significantly greater reduction in the CG after 12 months of follow-up, when compared to balloon valvuloplasty. In both groups, mitral gradient remained reduced and most patients did not change functional class during the follow-up.