RESUMEN
With congenitally corrected transposition of the great arteries, the right ventricle (RV) supports the systemic circulation. These patients have an increased risk of developing heart failure. Implantation of a ventricular assist device may be the only therapeutic option for patients who are not transplantation candidates. The technical aspects of implanting a mechanical device into the RV have not been well described, however. We describe our experience with one such case and describe our operative strategy in obtaining optimal placement of the inflow cannula.
Asunto(s)
Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/rehabilitación , Ventrículos Cardíacos/cirugía , Corazón Auxiliar , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/rehabilitación , Anciano , Transposición Congénitamente Corregida de las Grandes Arterias , Insuficiencia Cardíaca/diagnóstico , Humanos , Masculino , Resultado del TratamientoRESUMEN
BACKGROUND: We report neuropsychological and structural brain imaging assessments in children 16 years of age with d-transposition of the great arteries who underwent the arterial switch operation as infants. Children were randomly assigned to a vital organ support method, deep hypothermia with either total circulatory arrest or continuous low-flow cardiopulmonary bypass. METHODS AND RESULTS: Of 159 eligible adolescents, 139 (87%) participated. Academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition were assessed. Few significant treatment group differences were found. The occurrence of seizures in the postoperative period was the medical variable most consistently related to worse outcomes. The scores of both treatment groups tended to be lower than those of the test normative populations, with substantial proportions scoring ≥1 SDs below the expected mean. Although the test scores of most adolescents in this trial cohort are in the average range, a substantial proportion have received remedial academic or behavioral services (65%). Magnetic resonance imaging abnormalities were more frequent in the d-transposition of the great arteries group (33%) than in a referent group (4%). CONCLUSIONS: Adolescents with d-transposition of the great arteries who have undergone the arterial switch operation are at increased neurodevelopmental risk. These data suggest that children with congenital heart disease may benefit from ongoing surveillance to identify emerging difficulties. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00000470.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/rehabilitación , Puente Cardiopulmonar/rehabilitación , Cognición/fisiología , Paro Cardíaco Inducido/rehabilitación , Transposición de los Grandes Vasos/rehabilitación , Adolescente , Atención/fisiología , Encéfalo/anatomía & histología , Encéfalo/fisiología , Procedimientos Quirúrgicos Cardíacos/métodos , Procedimientos Quirúrgicos Cardíacos/estadística & datos numéricos , Puente Cardiopulmonar/métodos , Puente Cardiopulmonar/estadística & datos numéricos , Niño , Escolaridad , Función Ejecutiva/fisiología , Estudios de Seguimiento , Paro Cardíaco Inducido/métodos , Paro Cardíaco Inducido/estadística & datos numéricos , Humanos , Hipotermia Inducida/métodos , Hipotermia Inducida/estadística & datos numéricos , Lactante , Imagen por Resonancia Magnética/métodos , Memoria/fisiología , Pruebas Neuropsicológicas , Complicaciones Posoperatorias/epidemiología , Desempeño Psicomotor/fisiología , Factores de Riesgo , Conducta Social , Transposición de los Grandes Vasos/epidemiología , Transposición de los Grandes Vasos/cirugíaRESUMEN
BACKGROUND: Due to better early and long-term outcome, the increasing population of grown-ups with congenital heart disease (GUCH) brings up unexpected quality of life (QoL) issues. The cardiac lesion by itself is not always the major problem for these patients, since issues pertaining to QoL and psychosocial aspects often predominate. This study analyses the QoL of GUCH patients after cardiac surgery and the possible impact of medical and psychosocial complications. PATIENTS AND METHODS: A questionnaire package containing the SF-36 health survey (health related QoL), the HADS test (anxiety/depression aspects) and an additional disease specific questionnaire was sent to 345 patients (mean 26+/-11 years) operated for isolated transposition of the great arteries (TGA), tetralogy of Fallot (TOF), and ventricular septal defect (VSD). The scores were compared with age- and gender-matched standard population data and in relation to the underlying congenital heart disease (CHD). RESULTS: In all SF-36 and HADS health dimensions the GUCH patients showed excellent scores (116+/-20), which are comparable to the standard population (100+/-15), regardless of the initial CHD (p=0.12). Eighty-two percent of the patients were found to be in NYHA class I and 83% patients declared that they do not consider their QoL to be limited by their malformation. Complications like reoperations (p=0.21) and arrhythmias (p=0.10) do not show significant impact on the QoL. The additional questionnaire revealed that 76% of adult patients have a fulltime job, 18% receive a full or partial disability pension, 21% reported problems with insurances, most of them regarding health insurances (67%), and 4.4% of adult patients declared to have renounced the idea of having children due to their cardiac malformation. CONCLUSION: QoL in GUCH patients following surgical repair of isolated TOF, TGA and VSD is excellent and comparable to standard population, this without significant difference between the diagnosis groups. However, these patients are exposed to a high rate of complications and special psychosocial problems, which are not assessed by standardized questionnaires, such as the SF-36 and HADS. These findings highlight the great importance for a multidisciplinary and specialized follow-up for an adequate management of these complex patients.
Asunto(s)
Cardiopatías Congénitas/cirugía , Calidad de Vida , Adolescente , Adulto , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/psicología , Cardiopatías Congénitas/rehabilitación , Defectos del Tabique Interventricular/psicología , Defectos del Tabique Interventricular/rehabilitación , Defectos del Tabique Interventricular/cirugía , Humanos , Masculino , Escalas de Valoración Psiquiátrica , Psicometría , Reoperación/rehabilitación , Tetralogía de Fallot/psicología , Tetralogía de Fallot/rehabilitación , Tetralogía de Fallot/cirugía , Transposición de los Grandes Vasos/psicología , Transposición de los Grandes Vasos/rehabilitación , Transposición de los Grandes Vasos/cirugía , Adulto JovenRESUMEN
OBJECTIVE: To analyze size and function of aortic root and left ventricle as well as quality of life in patients 20 years after arterial switch procedure. METHODS: Thirty-nine patients who underwent arterial switch operation between 1977 and 1989 were examined. Perioperative and follow-up data were analyzed. Evaluation included clinical assessment, ECG, echocardiography and quality of life questionnaire. RESULTS: Patients had simple transposition (24), transposition with ventricle septal defect (7), Taussig-Bing anomaly (4) or transposition with ventricle septal defect and aortic arch obstruction (4). Mean age at evaluation was 19.9+/-2.6 years. Seven patients required reintervention for pulmonary stenosis (4), coarctation (2) and subaortic stenosis, followed by valve replacement 10 years later (1). Arrhythmia occurred in four patients. Patients were in New York Heart Association functional class I (38) or II (1). Quality of life scores were comparable to normal controls except for the lower score in the domains of vitality, aggressive and depressive mood. Diameters of aortic annulus, sinus of Valsalva and sinotubular junction were 26.5+/-4.1, 36.5+/-5.6 and 29.2+/-6.6mm respectively. Sixty-five percent of sinus of Valsalva and 38% of sinotubular junction indexed to body surface area fall outside the 95% confidence interval. Aortic regurgitation was absent in 72%, mild in 13% and moderate in 15%. No patient had severe regurgitation. Patients without regurgitation had smaller diameters of annulus (p=0.005) and sinus of Valsalva (p=0.01). Left ventricular end-diastolic and end-systolic diameters were 51+/-7mm and 34+/-6mm respectively. Fractional shortening was 34+/-5%; no regional wall motion abnormalities were observed. CONCLUSIONS: Clinical outcome is good 20 years after arterial switch operation and aortic valve function remains preserved in most patients. However, aortic root dilatation is present in two thirds of patients emphasizing the need for careful follow-up.
Asunto(s)
Aorta/patología , Transposición de los Grandes Vasos/cirugía , Función Ventricular Izquierda/fisiología , Adolescente , Adulto , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/etiología , Insuficiencia de la Válvula Aórtica/patología , Arritmias Cardíacas/etiología , Electrocardiografía , Estudios de Seguimiento , Cardiopatías Congénitas/cirugía , Humanos , Complicaciones Posoperatorias , Calidad de Vida , Reoperación , Seno Aórtico/patología , Transposición de los Grandes Vasos/fisiopatología , Transposición de los Grandes Vasos/rehabilitación , Resultado del Tratamiento , Ultrasonografía , Adulto JovenRESUMEN
BACKGROUND: Pharmacological blockade of the renin-angiotensin system improves exercise tolerance in patients with left ventricular dysfunction, yet its impact on patients with systemic right ventricles (RVs) remains unknown. METHODS AND RESULTS: A multicenter, randomized, double-blind, placebo-controlled, crossover clinical trial was performed to assess the effects of losartan on exercise capacity and neurohormonal levels in patients with systemic RVs. Of 29 patients studied (age, 30.3+/-10.9 years), 21 had transposition of the great arteries with a Mustard baffle, and 8 had congenitally corrected transposition of the great arteries. Baseline values were as follows: VO2max, 29.8+/-5.6 mL.kg(-1).min(-1) (73.5+/-12.9% predicted value); RV ejection fraction, 41.6+/-9.3%; N-terminal pro brain natriuretic peptide (NT-proBNP), 257.7+/-243.4 pg/mL (normal <125 pg/mL); and angiotensin II, 5.7+/-4.9 pg/mL (normal <5.0 pg/mL). Comparing losartan to placebo showed no differences in VO2max (29.9+/-5.4 versus 29.4+/-6.2 mL.kg(-1).min(-1); P=0.43), exercise duration (632.3+/-123.0 versus 629.9+/-140.7 seconds; P=0.76), and NT-proBNP levels (201.2+/-267.8 versus 229.7+/-291.5 pg/mL; P=0.10), despite a trend toward increased angiotensin II levels (15.2+/-13.8 versus 8.8+/-12.5 pg/mL; P=0.08). CONCLUSIONS: In adults with systemic RVs, losartan did not improve exercise capacity or reduce NT-proBNP levels. Minimal baseline activation of the renin-angiotensin system may explain this lack of benefit and imply an alternative pathophysiological mechanism for the progressive ventricular dysfunction and impaired exercise capacity observed in such patients.
Asunto(s)
Bloqueadores del Receptor Tipo 1 de Angiotensina II/uso terapéutico , Ejercicio Físico , Losartán/uso terapéutico , Consumo de Oxígeno/fisiología , Transposición de los Grandes Vasos/rehabilitación , Disfunción Ventricular Derecha/rehabilitación , Adulto , Angiotensina II/sangre , Estudios Cruzados , Método Doble Ciego , Ecocardiografía Transesofágica , Epinefrina/sangre , Humanos , Péptido Natriurético Encefálico/sangre , Norepinefrina/sangre , Fragmentos de Péptidos/sangre , Transposición de los Grandes Vasos/tratamiento farmacológico , Transposición de los Grandes Vasos/cirugía , Resultado del Tratamiento , Disfunción Ventricular Derecha/tratamiento farmacológicoRESUMEN
We report the case of a 20-year-old man born with transposition of the great arteries who underwent emergency balloon septostomy and subsequently a Mustard procedure. When aged 20 years, he had several syncopal attacks due to sinoatrial disease for which he was simply and successfully paced transvenously in VVI mode.