Recommendations for starting a grown up congenital heart disease (GUCH) unit / Recomendações para a implantação de uma Unidade de Cardiopatias Congênitas no Adulto

AbstractDuring the last decades, advances in diagnosis and treatment of congenital heart disease have allowed many individuals to reach adulthood. Due mainly to the great diagnostic diversity and to the co-morbidities usually present in this age group, these patients demand assistance in a multidisciplinary facility if an adequate attention is aimed. In this paper we reviewed, based in the international literature and also on the authors’ experience, the structural conditions that should be available for these patients. We highlighted aspects like the facility characteristics, the criteria usually adopted for patient transfer from the paediatric setting, the composition of the medical and para- medical staff taking into account the specific problems, and also the model of outpatient and in-hospital assistance. We also emphasized the importance of patient data storage, the fundamental necessity of institutional support and also the compromise to offer professional training. The crucial relevance of clinical research is also approached, particularly the development of multicenter studies as an appropriate methodology for this heterogeneous patient population.

ResumoDurante as últimas décadas, os avanços verificados no diagnóstico e tratamento das cardiopatias congênitas têm permitido que muitos indivíduos cheguem à idade adulta. Devido principalmente à grande diversidade diagnóstica e também às comorbidades habitualmente presentes nesse grupo etário, esses pacientes necessitam ser atendidos numa unidade multidisciplinar, se o objetivo for proporcionar uma assistência adequada. Neste trabalho revisamos, com base na experiência dos autores e na literatura internacional, as condições estruturais que devem estar disponíveis para esses pacientes. Procuramos ressaltar aspectos como as características da unidade, o critério usualmente adotado para transferência desses pacientes da unidade pediátrica, a composição das equipes médica e paramédica levando em consideração os problemas específicos dos pacientes e também o modelo de assistência ambulatorial e hospitalar. Enfatizamos, ainda, a importância do armazenamento dos dados dos pacientes, a necessidade fundamental de apoio institucional e a importância de oferecer treinamento profissional. A relevância da pesquisa clínica é também abordada, particularmente a importância da confecção de estudos multicêntricos, como uma metodologia apropriada para essa heterogênea população de pacientes.

Adult congenital heart disease: experience with the surgical approach / Cardiopatia congênita no adulto: experiência com a abordagem cirúrgica

OBJECTIVE: To report the institution experience with the surgical treatment of adults with congenital heart disease due to the increasing number of these patients and the need for a better discussion of the subject. METHODS: Retrospective analysis describing demographic data, risk factors and results. RESULTS: 191 patients between 16 and 74 years old were operated on. Primary correction was done in 171 cases, 93 (55%) for atrial septal defect repair. Among 20 (12%) reoperations, pulmonary valve replacement was done in six cases. The mean intensive care and hospital stay were 2.7 and 8.5 days respectively, significantly greater for the reoperated cases (P=0.001). The mean bypass and clamping times were 68.6 and 44.7 minutes respectively, greater for the reoperated cases (P<0.0001 and P=0.0003 respectively). Hospital mortality was 4.2% and male sex, functional class III-IV and older age at operation were predictive risk factors. Significant complications were more frequent in the reoperated cases (P<0.003), mainly atrial flutter and fibrillation. Among 183 patients discharged, 149 (82%) are being followed and atrial flutter and fibrillation are common. The mean functional class value improved significantly after operation (1.66 to 1.11; P<0.0001). The estimated survival was 96.2% in six years. CONCLUSION: Heart surgery in adults with congenital heart disease can be accomplished with low mortality and functional class improvement. Immediate and late complications are frequent. Multicenter studies are important to better characterize this patient population in the country.

OBJETIVO: Relatar a experiência da instituição com o tratamento cirúrgico de adultos com cardiopatia congênita devido ao crescente aumento no número desses pacientes e consequentes necessidades de maior discussão do tema. MÉTODOS: Análise retrospectiva dos pacientes operados, com análise de dados demográficos, fatores de risco e resultados. RESULTADOS: Cento e noventa e um pacientes, com idade entre 16 e 74 anos, foram operados. Cirurgia primária foi realizada em 171 pacientes, 93 (55%) com comunicação interatrial. Dentre 20 (12%) reoperações, substituição de valva pulmonar ocorreu em seis casos. Os tempos médios de unidade de terapia intensiva e hospitalar foram 2,7 e 8,5 dias, respectivamente, maiores nas reoperações (P=0,001). Os tempos médios de circulação extracorpórea e pinçamento aórtico foram 68,6 e 44,7 minutos, respectivamente, maiores nas reoperações (P<0,0001 e P=0,0003, respectivamente). Mortalidade hospitalar foi 4,2%, sem relação com reoperação. Sexo masculino, classe funcional III-IV e idade avançada foram fatores preditivos de risco. Complicações importantes foram mais frequentes nas reoperações (P<0,003), principalmente fibrilação e flutter atrial. Entre 183 pacientes com alta hospitalar, 149 (82%) foram seguidos e a fibrilação e flutter atrial foram bastante prevalentes. O valor médio da classe funcional mudou significativamente após a cirurgia (1,66 para 1,11; P<0,0001). A estimativa de sobrevida geral foi de 96,2% em seis anos. CONCLUSÃO: Cirurgia em adultos com cardiopatia congênita pode ser realizada com baixa mortalidade e melhora funcional na maioria dos pacientes. Complicações imediatas e tardias são frequentes. Estudos multicêntricos são importantes para melhor caracterização dessa população de indivíduos no país.

Avaliaçäo pós-operatória imediata da influência da desinserçäo da valva tricúspide no tratamento da comunicaçäo interventricular / Immediate postoperative evaluation of the tricuspid valve takedown insuficiency in the treatment of the ventricular septal defect

Although the tricuspid valve and its attachments can sometimes obscure the margins of the ventricular septal defect from the vision of the surgeon, concern for valvular competence has made surgeons hesitate to take down the tricuspid valve. In this paper, the records of 34 patients divided into two groups (comparable by gender, age and associated anomalies) were reviewed retrospectively. Treatment of the ventricular septal defect was done without (Group I, with 19 patients) and with (Group II, with 15 patients) takedown of the tricuspid valve to determine the degree of the tricuspid regurgitation and also the incidence of residual ventricular septal defects and atrioventricular block. The anterior and septal leaflets of the valve were taken down at the annulus and resuspended after VSD repair with running 6-0 polypropylene sutures. The degree of valvular regurgitation and the presence of residual VSD were determined by echo done on post-operative day one and at the the patient's discharge. Valvular regurgitation was graded as none in 12 patients of the Group I and in 10 patients of the Group II; trivial regurgitation in 5 patients of Group I and 4 of Group II; mild to moderate regurgitation in 2 cases of Group I and 1 in Group II. No patient had severe regurgitation. Even with a small number of cases, there was no statistically significant difference between the groups. There was only one case of residual VSD in Group I with spontaneous closure. The mortality in Group I was 10.5 per cent and 6.6 per cent in Group II. The authors conclude that takedown and re-suspension of the tricuspid valve in VSD repair surgery does not adversely affect valve competence neither increases the incidence of residual VSD or the incidence of A-V block.