RESUMEN
Truncus arteriosus (TA, also known as common arterial trunk) consists of only one great artery ("the truncus") with a semilunar valve (truncus valve) arising from the heart and an additional ventricular septal defect and (Fig. 50.1). This great artery is positioned above the ventricular septal defect and gives rise to the coronary arteries, the pulmonary arteries, and the aortic arch. Historically, TA has been classified by Collet and Edwards in three types, where in type I there was a common pulmonary artery truncus, in type II the left and right PA arise separately but close to each other, in type III both PA arise independently; in addition, there was a type IV that was later characterized as pulmonary atresia with VSD and major aortopulmonary collateral arteries arising from the descending aorta.
Asunto(s)
Tronco Arterial Persistente , Humanos , Arteria Pulmonar/fisiopatología , Arteria Pulmonar/anomalías , Arteria Pulmonar/patología , Atresia Pulmonar/terapia , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugía , Atresia Pulmonar/fisiopatología , Tronco Arterial/diagnóstico por imagen , Tronco Arterial/cirugía , Tronco Arterial Persistente/cirugía , Tronco Arterial Persistente/terapia , Tronco Arterial Persistente/fisiopatología , Tronco Arterial Persistente/diagnósticoRESUMEN
BACKGROUND: The natural history of the dilated truncal root in repaired truncus arteriosus (TA) is incompletely understood. METHODS: A single-center review of patients who underwent TA repair between January 1984 and December 2018 was performed. Echocardiographically determined root diameters and derived z scores were measured at the annulus, sinus of Valsalva (SoV), and sinutubular junction (STJ) immediately before TA repair and throughout follow-up. Linear mixed-effects models assessed trends in root dimensions over time. RESULTS: Of 193 patients who underwent TA repair at a median age of 12 days (interquartile range, 6-48 days) and survived to discharge, 34 (17.6%), 110 (57.0%), and 49 (25.4%) patients had bicuspid, tricuspid, and quadricuspid truncal valves, respectively. Median postoperative follow-up was 11.6 years (interquartile range, 4.4-22.0 years; range, 0.1-34.8 years). Truncal valve or root intervention was required in 38 patients (19.7%). The mean rates of annular, SoV, and STJ growth were 0.7 ± 0.3 mm/y, 0.8 ± 0.5 mm/y, and 0.9 ± 0.4 mm/y, respectively. Root z scores remained stable with time. At baseline, compared with patients with tricuspid leaflet anatomy, bicuspid patients had larger diameters at the SoV (P = .003) and STJ (P = .029), whereas quadricuspid patients had larger STJ diameters (P = .004). Over time, the bicuspid and quadricuspid cohorts demonstrated comparatively greater annular dilatation (both P < .05). Patients with ≥75th percentile root growth rates had a higher incidence of moderate-severe truncal regurgitation (P = .019) and truncal valve intervention (P = .002). CONCLUSIONS: Root dilatation in TA persisted for up to 30 years after primary repair. Patients with bicuspid and quadricuspid truncal valves demonstrated greater root dilatation over time and required more valve interventions. Continued longitudinal follow-up is warranted in this higher-risk cohort.
Asunto(s)
Enfermedades de las Válvulas Cardíacas , Tronco Arterial Persistente , Humanos , Recién Nacido , Tronco Arterial/diagnóstico por imagen , Tronco Arterial/cirugía , Dilatación , Tronco Arterial Persistente/complicaciones , Tronco Arterial Persistente/diagnóstico por imagen , Tronco Arterial Persistente/cirugía , Enfermedades de las Válvulas Cardíacas/complicaciones , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/cirugía , Dilatación PatológicaAsunto(s)
Arteria Pulmonar , Tronco Arterial Persistente , Humanos , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Tronco Arterial/diagnóstico por imagen , Tronco Arterial/cirugía , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Tronco Arterial Persistente/cirugía , Resultado del TratamientoRESUMEN
Truncus arteriosus, also referred to as common arterial trunk (CAT), is generally classified as a cyanotic congenital heart disease characterized by a single arterial trunk arising from the heart and supplying both pulmonary and systemic circulations. Cyanosis exists by virtue of it being an admixture lesion. We report a 13-year-old boy diagnosed to have type 1 CAT who was acyanotic at presentation and had all features of an operable lesion even at this age. He underwent a successful repair with closure of the subtruncal VSD and insertion of a hand-sewn valved right ventricle-to-pulmonary artery conduit made of bovine pericardium and Gore-Tex membrane.
Asunto(s)
Cardiopatías Congénitas , Tronco Arterial Persistente , Animales , Bovinos , Cianosis , Humanos , Masculino , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Tronco Arterial/diagnóstico por imagen , Tronco Arterial/cirugía , Tronco Arterial Persistente/diagnóstico por imagen , Tronco Arterial Persistente/cirugíaRESUMEN
We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p<.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality.
Asunto(s)
Arteria Pulmonar , Tronco Arterial Persistente , Animales , Bovinos , Ventrículos Cardíacos/cirugía , Humanos , Lactante , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Reoperación , Estudios Retrospectivos , Resultado del Tratamiento , Tronco Arterial/diagnóstico por imagen , Tronco Arterial/cirugía , Tronco Arterial Persistente/diagnóstico por imagen , Tronco Arterial Persistente/cirugíaRESUMEN
BACKGROUND: Truncus arteriosus is associated with coronary anomalies. We identified coronary artery lesions in patients undergoing repair of truncus arteriosus, defined the impact of lesions on mortality, and studied the effect of surgical intervention of coronary lesions. METHODS: A retrospective review identified 107 patients with truncus repair (1995-2019). Coronary lesions were categorized as ostial stenosis, intramural, juxtacommissural origin, and single coronary. Survival analysis characterized survival after truncus repair and studied the association of coronary lesions and mortality. RESULTS: Among 107 patients with truncus repair 34 patients had at least 1 coronary lesion. Median follow-up time was 7 years, with 85% 5-year survival. Coronary lesions including ostial stenosis, intramurality, and juxtacommissural origin were associated with increased mortality, whereas single coronaries did not impact survival. Eleven patients had 1 coronary lesion and 6 patients with 2 coronary lesions had similar (80% and 83%, respectively) 5-year survival. Eight patients with 3 coronary lesions had 24% 5-year survival (P = .0003). Among patients with 1 or 2 lesions, surgical intervention on the coronary lesions tended to be associated with longer 5-year survival (100% vs 62%, respectively; P = .06). All patients with 3 lesions underwent coronary artery intervention, with 24% 5-year survival. CONCLUSIONS: Impact of coronary lesions on mortality after truncus repair increases with the number of lesions. Coronary artery intervention may be associated with improved time-related survival among patients with 1 or 2 lesions. Patients with the most complex anomalies (3 lesions) have poor survival and warrant ongoing study of repair techniques.
Asunto(s)
Anomalías de los Vasos Coronarios/mortalidad , Vasos Coronarios/cirugía , Complicaciones Posoperatorias/mortalidad , Tronco Arterial Persistente/cirugía , Tronco Arterial/cirugía , Procedimientos Quirúrgicos Vasculares/métodos , Anomalías de los Vasos Coronarios/diagnóstico , Vasos Coronarios/diagnóstico por imagen , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Reoperación , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia/tendencias , Texas/epidemiología , Resultado del Tratamiento , Tronco Arterial/diagnóstico por imagen , Tronco Arterial Persistente/diagnóstico , Tronco Arterial Persistente/mortalidadRESUMEN
BACKGROUND: Congenital heart disease (CHD) is the most common congenital anomaly in children. Over half of the deaths due to CHD occur in the neonatal period. Most children with unrepaired complex heart lesions do not live to celebrate their first birthday. We describe the spectrum of congenital heart disease in Uganda. METHODS: We retrospectively reviewed the data of children with CHD who presented to the Uganda Heart Institute (UHI), Mulago Hospital Complex from 2007 to 2014. RESULTS: A total of 4621 children were seen at the UHI during the study period. Of these, 3526 (76.3%) had CHD; 1941(55%) were females. Isolated ventricular septal defect (VSD) was the most common CHD seen in 923 (27.2%) children followed by Patent ductus arteriosus (PDA) 760 (22%) and atrial septal defects (ASD) 332 (9.4%). Tetralogy of Fallot (TOF) and Truncus arteriosus were the most common cyanotic heart defects (7% and 5% respectively). Dysmorphic features were diagnosed in 185 children, of which 61 underwent genetic testing (Down syndrome=24, 22q11.2 deletion syndrome n=10). Children with confirmed 22q11.2 deletion had conotruncal abnormalities. CONCLUSION: Isolated VSD and Tetralogy of Fallot are the most common acyanotic and cyanotic congenital heart defects. We report an unusually high occurrence of Truncus arteriosus.
Asunto(s)
Cardiopatías Congénitas/epidemiología , Defectos del Tabique Interventricular/epidemiología , Adolescente , Niño , Preescolar , Conducto Arterioso Permeable/diagnóstico por imagen , Conducto Arterioso Permeable/epidemiología , Ecocardiografía , Femenino , Cardiopatías Congénitas/diagnóstico por imagen , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interatrial/epidemiología , Defectos del Tabique Interventricular/diagnóstico por imagen , Humanos , Lactante , Recién Nacido , Masculino , Prevalencia , Estudios Retrospectivos , Síndrome de Rubéola Congénita , Distribución por Sexo , Tetralogía de Fallot/epidemiología , Tronco Arterial/diagnóstico por imagen , Uganda/epidemiologíaAsunto(s)
Cateterismo Cardíaco , Procedimientos Quirúrgicos Cardíacos , Anomalías de los Vasos Coronarios/complicaciones , Defectos del Tabique Interatrial/terapia , Tronco Arterial/cirugía , Anomalías Múltiples , Adolescente , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Estenosis Coronaria/etiología , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interatrial/fisiopatología , Humanos , Imagen por Resonancia Magnética , Masculino , Diseño de Prótesis , Factores de Riesgo , Dispositivo Oclusor Septal , Resultado del Tratamiento , Tronco Arterial/anomalías , Tronco Arterial/diagnóstico por imagen , Tronco Arterial/fisiopatologíaRESUMEN
Truncus arteriosus (TA) or common arterial trunk is a congenital cardiac anomaly having high association with arch anomalies such as right aortic arch or aortic arch interruption. However, TA with double aortic arch (DAA) is a rare occurrence. We report a case of TA with DAA where the diagnosis of DAA was missed initially.
Asunto(s)
Anomalías Múltiples , Aorta Torácica/anomalías , Tronco Arterial Persistente/diagnóstico , Tronco Arterial/anomalías , Anillo Vascular/diagnóstico , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Humanos , Lactante , Masculino , Tomografía Computarizada por Rayos X , Tronco Arterial/diagnóstico por imagen , Tronco Arterial/cirugía , Tronco Arterial Persistente/cirugía , Anillo Vascular/cirugíaAsunto(s)
Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Angiografía por Tomografía Computarizada , Angiografía Coronaria , Defectos de los Tabiques Cardíacos/diagnóstico por imagen , Atresia Pulmonar/diagnóstico por imagen , Tetralogía de Fallot/diagnóstico por imagen , Tronco Arterial/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/fisiopatología , Defecto del Tabique Aortopulmonar/cirugía , Diagnóstico Diferencial , Femenino , Defectos de los Tabiques Cardíacos/fisiopatología , Defectos de los Tabiques Cardíacos/cirugía , Humanos , Lactante , Valor Predictivo de las Pruebas , Atresia Pulmonar/fisiopatología , Atresia Pulmonar/cirugía , Tetralogía de Fallot/fisiopatología , Tetralogía de Fallot/cirugía , Tronco Arterial/anomalías , Tronco Arterial/fisiopatología , Tronco Arterial/cirugíaAsunto(s)
Anomalías Múltiples , Ventrículos Cardíacos/anomalías , Síndrome del Corazón Izquierdo Hipoplásico/complicaciones , Síndrome de Cimitarra/complicaciones , Tronco Arterial/anomalías , Angiografía por Tomografía Computarizada , Angiografía Coronaria , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico por imagen , Lactante , Masculino , Flebografía , Síndrome de Cimitarra/diagnóstico por imagen , Tronco Arterial/diagnóstico por imagenRESUMEN
Coexistence of tricuspid atresia and truncus arteriosus (common arterial trunk) is an extremely rare combination of anomalies and requires staged palliation. Initial palliation involves disconnection of the pulmonary arteries from the common trunk and creation of a systemic-to-pulmonary artery shunt. This has generally been accomplished with the use of cardiopulmonary bypass. We describe a technique of off-pump palliation using the common trunk and a modified Blalock-Taussig shunt as sources of pulmonary blood flow at various stages of reconstruction. The child has recovered well and has undergone second-stage palliation with bilateral bidirectional superior cavopulmonary anastomoses at one year of age.
Asunto(s)
Procedimiento de Blalock-Taussing , Puente de Arteria Coronaria Off-Pump , Arteria Pulmonar/cirugía , Atresia Tricúspide/cirugía , Tronco Arterial/cirugía , Anastomosis Quirúrgica , Angiografía por Tomografía Computarizada , Humanos , Imagenología Tridimensional , Lactante , Masculino , Cuidados Paliativos , Perfusión , Arteria Pulmonar/diagnóstico por imagen , Atresia Tricúspide/complicaciones , Atresia Tricúspide/diagnóstico por imagen , Tronco Arterial/diagnóstico por imagenRESUMEN
The cardiovascular magnetic resonance imaging (CMR) features of adults with repaired truncus arteriosus (rTA) are largely undefined. We sought to explore CMR characteristics in rTA and to identify associations between imaging findings and cardiovascular outcomes. Adults with rTA and CMR were identified and anatomic subtypes (1-4) were assigned (Collett and Edwards classification). CMR characteristics, clinical data at last follow-up and adverse cardiovascular outcome were recorded. Twenty-seven adults (19% male) were studied (median age at cardiovascular magnetic resonance 26 years [interquartile range 18 to 40]) over 5.2-year duration [interquartile range 2.5 to 7.5]. With the exception of mildly increased RV mass (30 ± 12 g/m2), cardiac chamber measurements were within the normal range. In CMR measurements, only pulmonary artery peak velocity differed in subtypes (highest in subtype 3, 318 ± 26 cm/s, pâ¯=â¯0.029). Number of cardiovascular interventions in adulthood was moderately correlated with left ventricular end-diastolic volume (râ¯=â¯0.463, pâ¯=â¯0.015), left ventricular ejection fraction (râ¯=â¯0.425, pâ¯=â¯0.027) and neoaortic root size (râ¯=â¯0.398, pâ¯=â¯0.039). Cardiovascular events (nonmutually exclusive) in 5 of 27 patients (19%) included death (nâ¯=â¯1), heart failure (nâ¯=â¯1), ventricular tachycardia (nâ¯=â¯1), and atrial tachycardia (nâ¯=â¯3). Increased cardiovascular risk was associated with decreased right ventricular ejection fraction (odds ratio 1.153, confidence interval 1.003 to 1.326, pâ¯=â¯0.046) and smaller ascending aorta diameter (odds ratio 1.758, confidence interval 1.037 to 2.976, pâ¯=â¯0.036). In conclusion, decreased right ventricular ejection fraction and smaller ascending aorta on cardiovascular magnetic resonance were associated with adverse events in rTA.