RESUMO
BACKGROUND: Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. METHODS: Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. RESULTS: The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7⯱â¯6.4% vs. 48.5⯱â¯7.2%, Pâ¯=â¯.81) and exercise capacity (% predicted peak VO2:82.5⯱â¯17.7% vs. 75.6⯱â¯20.4%, Pâ¯=â¯.27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:râ¯=â¯-0.39, Pâ¯=â¯.04, rToF:râ¯=â¯-0.40, Pâ¯=â¯.03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:râ¯=â¯-0.77, Pâ¯<â¯.001, rToF:râ¯=â¯-0.69, Pâ¯<â¯.001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1⯱â¯3.9 vs.-25.7⯱â¯4.4, Pâ¯<â¯.001). CONCLUSIONS: Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.
Assuntos
Tolerância ao Exercício/fisiologia , Hipertrofia Ventricular Direita/fisiopatologia , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/fisiopatologia , Função Ventricular Direita/fisiologia , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia , Teste de Esforço , Feminino , Humanos , Hipertrofia Ventricular Direita/diagnóstico por imagem , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/patologia , Imageamento por Ressonância Magnética , Masculino , Análise por Pareamento , Consumo de Oxigênio , Complicações Pós-Operatórias/fisiopatologia , Atresia Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/complicações , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Volume Sistólico , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologiaRESUMO
The authors present a case of a seventeen-year-old white male who suffered from a knife chest wound and secondarily developed a traumatic coronary arteriovenous fistula communicating the left main coronary artery to the pulmonary artery, associated with pulmonary valvular insufficiency and endocarditis.