RESUMO
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.
Assuntos
Persistência do Tronco Arterial , Humanos , Persistência do Tronco Arterial/cirurgia , Persistência do Tronco Arterial/terapia , Persistência do Tronco Arterial/fisiopatologia , Persistência do Tronco Arterial/diagnóstico , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/anormalidades , Artéria Pulmonar/patologia , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/cirurgia , Atresia Pulmonar/terapia , Atresia Pulmonar/diagnóstico por imagem , Atresia Pulmonar/cirurgia , Atresia Pulmonar/fisiopatologiaRESUMO
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.
Assuntos
Doenças das Valvas Cardíacas , Persistência do Tronco Arterial , Humanos , Recém-Nascido , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/cirurgia , Dilatação , Persistência do Tronco Arterial/complicações , Persistência do Tronco Arterial/diagnóstico por imagem , Persistência do Tronco Arterial/cirurgia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Dilatação PatológicaAssuntos
Artéria Pulmonar , Persistência do Tronco Arterial , Humanos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Persistência do Tronco Arterial/cirurgia , Resultado do TratamentoRESUMO
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.
Assuntos
Cardiopatias Congênitas , Persistência do Tronco Arterial , Animais , Bovinos , Cianose , Humanos , Masculino , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/cirurgia , Persistência do Tronco Arterial/diagnóstico por imagem , Persistência do Tronco Arterial/cirurgiaRESUMO
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.
Assuntos
Artéria Pulmonar , Persistência do Tronco Arterial , Animais , Bovinos , Ventrículos do Coração/cirurgia , Humanos , Lactente , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Reoperação , Estudos Retrospectivos , Resultado do Tratamento , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/cirurgia , Persistência do Tronco Arterial/diagnóstico por imagem , Persistência do Tronco Arterial/cirurgiaRESUMO
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.
Assuntos
Anomalias dos Vasos Coronários/mortalidade , Vasos Coronários/cirurgia , Complicações Pós-Operatórias/mortalidade , Persistência do Tronco Arterial/cirurgia , Tronco Arterial/cirurgia , Procedimentos Cirúrgicos Vasculares/métodos , Anomalias dos Vasos Coronários/diagnóstico , Vasos Coronários/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Reoperação , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Texas/epidemiologia , Resultado do Tratamento , Tronco Arterial/diagnóstico por imagem , Persistência do Tronco Arterial/diagnóstico , Persistência do Tronco Arterial/mortalidadeRESUMO
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.
Assuntos
Cardiopatias Congênitas/epidemiologia , Comunicação Interventricular/epidemiologia , Adolescente , Criança , Pré-Escolar , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/epidemiologia , Ecocardiografia , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/epidemiologia , Comunicação Interventricular/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Masculino , Prevalência , Estudos Retrospectivos , Síndrome da Rubéola Congênita , Distribuição por Sexo , Tetralogia de Fallot/epidemiologia , Tronco Arterial/diagnóstico por imagem , Uganda/epidemiologiaRESUMO
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.
Assuntos
Anormalidades Múltiplas , Aorta Torácica/anormalidades , Persistência do Tronco Arterial/diagnóstico , Tronco Arterial/anormalidades , Anel Vascular/diagnóstico , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Humanos , Lactente , Masculino , Tomografia Computadorizada por Raios X , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/cirurgia , Persistência do Tronco Arterial/cirurgia , Anel Vascular/cirurgiaAssuntos
Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos , Anomalias dos Vasos Coronários/complicações , Comunicação Interatrial/terapia , Tronco Arterial/cirurgia , Anormalidades Múltiplas , Adolescente , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Estenose Coronária/etiologia , Anomalias dos Vasos Coronários/diagnóstico por imagem , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Desenho de Prótese , Fatores de Risco , Dispositivo para Oclusão Septal , Resultado do Tratamento , Tronco Arterial/anormalidades , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/fisiopatologiaAssuntos
Anormalidades Múltiplas , Ventrículos do Coração/anormalidades , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome de Cimitarra/complicações , Tronco Arterial/anormalidades , Angiografia por Tomografia Computadorizada , Angiografia Coronária , Ventrículos do Coração/diagnóstico por imagem , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Lactente , Masculino , Flebografia , Síndrome de Cimitarra/diagnóstico por imagem , Tronco Arterial/diagnóstico por imagemAssuntos
Defeito do Septo Aortopulmonar/diagnóstico por imagem , Angiografia por Tomografia Computadorizada , Angiografia Coronária , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Atresia Pulmonar/diagnóstico por imagem , Tetralogia de Fallot/diagnóstico por imagem , Tronco Arterial/diagnóstico por imagem , Defeito do Septo Aortopulmonar/fisiopatologia , Defeito do Septo Aortopulmonar/cirurgia , Diagnóstico Diferencial , Feminino , Defeitos dos Septos Cardíacos/fisiopatologia , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Lactente , Valor Preditivo dos Testes , Atresia Pulmonar/fisiopatologia , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/cirurgia , Tronco Arterial/anormalidades , Tronco Arterial/fisiopatologia , Tronco Arterial/cirurgiaRESUMO
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.
Assuntos
Procedimento de Blalock-Taussig , Ponte de Artéria Coronária sem Circulação Extracorpórea , Artéria Pulmonar/cirurgia , Atresia Tricúspide/cirurgia , Tronco Arterial/cirurgia , Anastomose Cirúrgica , Angiografia por Tomografia Computadorizada , Humanos , Imageamento Tridimensional , Lactente , Masculino , Cuidados Paliativos , Perfusão , Artéria Pulmonar/diagnóstico por imagem , Atresia Tricúspide/complicações , Atresia Tricúspide/diagnóstico por imagem , Tronco Arterial/diagnóstico por imagemRESUMO
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.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Ventrículos do Coração/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética/métodos , Persistência do Tronco Arterial/cirurgia , Tronco Arterial/diagnóstico por imagem , Adolescente , Adulto , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Persistência do Tronco Arterial/diagnóstico , Persistência do Tronco Arterial/fisiopatologia , Adulto JovemAssuntos
Aorta/patologia , Artéria Pulmonar/patologia , Tronco Arterial/patologia , Anastomose Cirúrgica , Aorta/anormalidades , Aorta/diagnóstico por imagem , Angiografia por Tomografia Computadorizada/métodos , Vasos Coronários/anatomia & histologia , Vasos Coronários/diagnóstico por imagem , Ecocardiografia/métodos , Ventrículos do Coração/cirurgia , Humanos , Recém-Nascido , Masculino , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Tronco Arterial/diagnóstico por imagem , Tronco Arterial/cirurgiaRESUMO
Common arterial trunk (CAT), or truncus arteriosus, is a rare form of cyanotic congenital heart disease and is highly associated with DiGeorge syndrome (microdeletion 22q11.2). Prenatal diagnosis is highly feasible, allowing proper delivery planning and postnatal management. The clinical presentation is highly variable depending on the anatomical variation; however, most commonly presenting with mild cyanosis and significant tachypnea, although these patients can often go undetected in the immediate newborn period. Transthoracic echocardiography is adequate for diagnosis and detailed anatomical delineation in the majority. Additional imaging modalities such as cardiac catheterization, computed tomography angiography, or cardiac magnetic resonance imaging can be helpful in those with more complex pulmonary artery (PA) or aortic anatomy, or in the older repaired. The surgical management of CAT is complete repair in the neonatal period with resection of branch PAs from the CAT with placement of a right ventricular (RV)-to-PA conduit and patch closure of the ventricular septal defect. Overall surgical outcomes are excellent in most centers, with the expectation that the child will eventually outgrow the RV-to-PA conduit and require reoperation. Other potential reoperations or postsurgical interventions in addition to the RV-to-PA conduit may involve the truncal valve or branch PAs.