RESUMEN
The objective of this study is to compare quality of life (QOL) in patients with mild pulmonary insufficiency (PI) after Tetralogy of Fallot (TOF) repair or after balloon dilation for isolated valvar pulmonary stenosis (VPS). A cross-sectional study of patients with TOF (n = 12) and VPS (n = 19), ages 8-18 years, who underwent cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) was conducted. Patients with genetic syndromes were excluded. The groups were matched by severity and duration of PI using propensity scores. PI was greater than mild if the regurgitant fraction by CMR was >20%. Health status and QOL assessment included Child Health Questionnaire Child Self-Report (CHQ-CF87), Child Health Questionnaire Parent Report (CHQ-PF50), and Pediatric Cardiac QOL Instrument (parent and patient). QOL scores were compared between groups. Due to propensity matching, the groups had, at worst, mild PI and normal right ventricular ejection fraction on CMR. Parental perception of QOL was significantly worse in TOF as compared to VPS in the domains of general perception of health (P = 0.03), physical functioning (PF; P = 0.004), and family cohesion (P = 0.048). There were no differences in self-reported QOL between groups. There was no association between QOL and right ventricular function on CMR or percent-predicted maximal oxygen consumption on CPET in both groups. Parent-perceived QOL, in the domains of general health perception, PF, and family cohesion, is significantly reduced in patients with TOF with mild residual disease suggesting that the psychosocial impact of congenital heart disease may be significant even with successful repair and satisfactory medical status.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/efectos adversos , Insuficiencia de la Válvula Pulmonar/psicología , Estenosis de la Válvula Pulmonar/psicología , Calidad de Vida/psicología , Tetralogía de Fallot/cirugía , Adolescente , Procedimientos Quirúrgicos Cardíacos/métodos , Niño , Estudios Transversales , Prueba de Esfuerzo , Femenino , Estado de Salud , Corazón/fisiopatología , Humanos , Imagen por Resonancia Cinemagnética , Masculino , Consumo de Oxígeno , Puntaje de Propensión , Insuficiencia de la Válvula Pulmonar/fisiopatología , Insuficiencia de la Válvula Pulmonar/cirugía , Estenosis de la Válvula Pulmonar/cirugía , Autoinforme , Encuestas y Cuestionarios , Tetralogía de Fallot/psicología , Función Ventricular DerechaRESUMEN
OBJECTIVE: Percutaneous pulmonary valve implantation (PPVI) has emerged as a new approach to treat patients with dysfunctional pulmonary valve conduits. Short- and midterm results have outlined hemodynamic improvements and increase in exercise performance. However, there is a lack of knowledge about quality of life at short term follow-up. PATIENTS AND METHODS: From July 2007 to March 2013, we investigated 59 patients (17 female; median age 22.8 years) undergoing PPVI in our institution. 46 had predominant pulmonary stenosis (PS) and 13 had predominant pulmonary regurgitation (PR). They answered the quality of life questionnaire (SF-36) and underwent a cardiopulmonary exercise test and Cardiovascular Magnetic Resonance before and 6 months after PPVI. RESULTS: Peak oxygen uptake improved significantly from 27.2 (18.9; 34.0) ml/min/kg to 29.2 (22.4; 35.3) ml/min/kg (p<.0001), and from 69.6 (55.9; 83.6) %predicted to 76.3 (67.9; 92.7) %predicted, respectively. Improvements were seen in both, the PS (71.9 to 78.3 %predicted; p<.0001) and PR (62.7 to 73.0 %predicted; p<.0001) group. Self-estimated quality of life was good already before PPVI but increased in almost all domains 6 months after PPVI in PS and PR group. Significant improvements developed in "physical function", "general health perception" and "health transition" in both groups, and "physical role functioning", "vitality" and "mental health" only in the PS group. CONCLUSIONS: In patients with dysfunctional pulmonary valve conduits exercise performance and quality of life improve substantially 6months after successful percutaneous pulmonary valve implantation.
Asunto(s)
Tolerancia al Ejercicio/fisiología , Implantación de Prótesis de Válvulas Cardíacas/tendencias , Insuficiencia de la Válvula Pulmonar/cirugía , Estenosis de la Válvula Pulmonar/cirugía , Calidad de Vida , Adulto , Femenino , Estudios de Seguimiento , Implantación de Prótesis de Válvulas Cardíacas/psicología , Humanos , Masculino , Estudios Prospectivos , Insuficiencia de la Válvula Pulmonar/fisiopatología , Insuficiencia de la Válvula Pulmonar/psicología , Estenosis de la Válvula Pulmonar/fisiopatología , Estenosis de la Válvula Pulmonar/psicología , Calidad de Vida/psicología , Adulto JovenRESUMEN
OBJECTIVE: Patients with cyanotic congenital heart disease without corrective surgery or palliation survive into adulthood, if they have a balanced pulmonary blood flow facilitated by pulmonary stenosis (PS) or Eisenmenger syndrome (ES). Both groups show cyanosis, diminished exercise performance and impaired quality of life. This study aimed to compare the functional outcome of those two cohorts directly. PATIENTS AND METHODS: In total fifty-eight cyanotic patients with cardiac shunts (28 male, 30 female, aged 14-55 years) were investigated, twenty-three of them with PS and thirty-five of them with ES. They completed the health related quality of life questionnaire SF-36 and performed a symptom limited cardiopulmonary exercise test. RESULTS: At exercise, oxygen saturation decreased severely and similarly in both groups (PS: 90% to 65% vs. ES: 87 % to 64%). Moreover, hemoglobin levels were comparable in both subgroups. Exercise capacity was markedly reduced, but more diminished in ES (PS: 20.3 (11.9;24.6) ml/min/kg vs. ES: 11.3 (9.7;14.5) ml/min/kg; p < 0.001) and ventilatory inefficiency expressed as V(E)/V(CO2) slope was more enhanced in ES (PS: 45.7 (37.6;52.9) vs. ES: 54.6 (43.4;68.7); p = 0.005). Oxygen saturation at rest was correlated to peak V(O2) (r = 0.436; p = 0.001) and V(E)/V(CO2) slope (r = -0.388; p = 0.003). Self estimated quality of life was poor, with worse results in physical and psychosocial domains in ES group. CONCLUSIONS: Despite similar cyanosis, patients with ES show less exercise performance, more ventilation-perfusion-mismatch and a worse quality of life compared to complex cyanotic congenital heart disease patients with PS. Moreover, oxygen saturation at rest predicts exercise capacity and ventilatory efficiency in this cohort.
Asunto(s)
Cianosis/psicología , Complejo de Eisenmenger/psicología , Prueba de Esfuerzo/psicología , Cardiopatías Congénitas/psicología , Estenosis de la Válvula Pulmonar/psicología , Calidad de Vida/psicología , Adolescente , Adulto , Cianosis/complicaciones , Cianosis/fisiopatología , Complejo de Eisenmenger/fisiopatología , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Estenosis de la Válvula Pulmonar/complicaciones , Estenosis de la Válvula Pulmonar/fisiopatología , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVE: To describe the long-term prognosis after repair of Tetralogy of Fallot with pulmonary stenosis beyond 20 years. METHODS: One hundred and eighty five patients underwent corrective repair of Tetralogy of Fallot at Rigshospitalet in Copenhagen between January 1960 and July 1977. Ninety seven patients had undergone a palliative operation prior to Tetralogy of Fallot repair. All the 125 patients who were discharged from the hospital were traced through the population register and the patients alive July 1997 were contacted by mail and/or telephone and questioned about use of medicine, professional status, family status and ability to perform sport activities. RESULTS: Sixty patients died in hospital and 125 patients, 78 males and 47 females, were discharged alive. Among operative survivors, median age at operation was 12.8 years (range 0.4-41 years). Thirteen patients required a reoperation, the main indication was failed VSD closure. There were 16 late cardiac deaths, out of which seven were sudden and unexpected and three were in immediate relation to reoperations. One hundred and nine patients were alive at follow-up. The mean follow-up time was 25.5 years (range 20-38 years). Sixteen percent used cardiac drugs, 89% were, or had been, working normally (all professions from academics to hard manual labors were represented), 53% (64% of women) had given birth after the repair and 51% performed sport activities regularly. No patients were lost to follow-up. CONCLUSIONS: The vast majority of the patients seemed to live normal lives 20-37 years after Tetralogy of Fallot repair. Late deaths were cardiac in origin, including sudden death from arrhythmias. The number of late reoperation has been low. Considering the natural history of the disease, Fallot repair has proven to be a beneficial procedure even including the very early experience short after introduction of open heart surgery.