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Resumen Introducción: El tronco arterial persistente es una rara malformación cardíaca congénita que provoca diversas complicaciones en el sistema cardiovascular. Se caracteriza por la presencia de un tabique ventricular defectuoso, una única válvula troncal y un tronco arterial común entre la arteria pulmonar y aorta, conllevando a una mezcla entre la sangre arterial y venosa, debido a un cortocircuito cardíaco bidireccional predominante de izquierda a derecha que compromete el suministro de flujo sanguíneo, nutrientes y oxigenación sistémica. Las manifestaciones clínicas incluyen desaturación con cianosis, hipoxemia, taquicardia, taquipnea, alteraciones en la contractilidad cardíaca, pulsos distales anómalos, pérdida de peso, fatiga y hepatomegalia. Objetivo: El propósito de esta investigación es establecer hipótesis sobre los diversos mecanismos compensatorios que se activan a nivel sistémico para contrarrestar los efectos de esta malformación. Reflexión: Se sugiere que se producen respuestas biomoleculares similares en los sistemas cardiovascular, pulmonar y renal, reduciendo la producción de óxido nítrico y provocando respuestas vasoconstrictoras. A nivel hepático, se generan factores de crecimiento y se inician procesos de angiogénesis para aumentar la perfusión sanguínea. En el cerebro, se activan enzimas para incrementar el flujo sanguíneo y proporcionar oxígeno y nutrientes esenciales. Conclusión: A pesar de estos mecanismos compensatorios, no logran contrarrestar por completo las manifestaciones clínicas, conduciendo a una serie de problemas de salud, como hipertensión pulmonar, insuficiencia cardíaca, hepatomegalia, hipoperfusión de órganos y déficits neurológicos. Estos factores convergen para generar una compleja condición cardíaca que desencadena respuestas adaptativas en el cuerpo que terminan siendo una afección médica desafiante y potencialmente grave.
Abstract Introduction: Persistent truncus arteriosus is a rare congenital cardiac malformation that causes various complications in the cardiovascular system. It is characterized by the presence of a defective ventricular septum, a single truncal valve and a common truncus arteriosus between the pulmonary artery and aorta, leading to a mixture between arterial and venous blood, due to a predominantly left-to-right bidirectional cardiac shunt that compromises the supply of blood flow, nutrients, and systemic oxygenation. Clinical manifestations include desaturation with cyanosis, hypoxemia, tachycardia, tachypnea, alterations in cardiac contractility, abnormal distal pulses, weight loss, fatigue, and hepatomegaly. Aim: The purpose of this research is to establish hypotheses about the various compensatory mechanisms that are activated at a systemic level to counteract the effects of this malformation. Reflection: It is suggested that similar biomolecular responses occur in the cardiovascular, pulmonary, and renal systems, reducing nitric oxide production and causing vasoconstrictive responses. At the liver level, growth factors are generated and angiogenesis processes are initiated to increase blood perfusion. In the brain, enzymes are activated to increase blood flow and provide oxygen and essential nutrients. Conclusion: Despite these compensatory mechanisms, they fail to completely counteract the clinical manifestations, leading to a series of health problems such as pulmonary hypertension, heart failure, hepatomegaly, organ hypoperfusion, and neurological deficits. These factors converge to generate a complex cardiac condition that triggers adaptive responses in the body that end up being a challenging and potentially serious medical condition.
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ABSTRACT Introduction: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. Methods: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. Results: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). Conclusion: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.
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@#Objective To evaluate the early- and mid-term outcomes of surgical repair of persistent truncus arteriosus in children in a single institution of China. Methods The clinical data of 27 consecutive patients with persistent truncus arteriosus undergoing surgical repair in Guangzhou Women and Children’s Medical Center from November 2009 to May 2018 were retrospectively reviewed. There were 14 males and 13 females. Median age was 3.0 months (range: 13 days -11 years), of whom 10 (37.0%) were older than 6 months. Results There were three early deaths with a mortality of 11.1%. The main complications included VSD partial repair in 2 patients, complete atrioventricular block in one patient. The mean follow-up time was 24.5±19.3 months (range: 1–76 months). There were three late deaths, and two patients lost follow. Echocardiology showed seven patients of right heart outflow tract obstruction, including three in pulmonary artery trunk, and four of pulmonary artery branches. One patient showed moderate aortic valve regurgitation. None required re-intervention during the follow-up. Survival estimates for the entire cohort following surgery were both 76.1% (95%CI 59.2% to 92.9%) at 1 year and 5 years. Conclusion The surgical repair of persistent truncus arteriosus (PTA) remains challenges. The early- and mid-term outcomes of surgical repair of persistent truncus arteriosus are acceptable. For older children with severe pulmonary artery hypertension and/or trunk valve regurgitation, the risk of death is still higher. Some children have the higher risk of late right heart obstructive lesions.
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Objective To evaluate prenatal diagnosis value of echocardiography in pathological types,differential diagnosis and accompanied malformations of fetal persistent truncus arteriosus(PTA).MethodsTwenty-four cases of PTA selected from 1 392 cases were analysed,who were definitely diagnosed to be suffered from cardiovascular malformation by fetal echocardiography.The ultrasound findings,pathological results and followed up were analysed.According to Van Praagh classification,the type IV PTA was excluded in this study which was classified into pulmonary artery atresia.Results The total PTA were 24 cases,in which 10 cases of A1 type,3 cases of A2 type,9 cases of A3 type,and 2 cases of A4 type.Nine cases of PTA accompanied other cardiac anomalies,and 1 case of PTA accompanied both cardiac anomalies and extracardial malformations.Two PTA cases were born,one was A1 type underwent surgical intervention,and the other was died due to multiple organ-failure.Fourteen PTA cases were termination and 7 cases were confirmed by pathology.Seven women pregnant again,of which 5 cases were born while only one was diagnosed atrial septal defect after birth,2 pregnant women were still during follow-up.Eight PTA cases follow-up were lost.Conclusions A1 type and A3 type of PTA have high incidence in fetus.Accompanied cardiac anomalies is certainly related to different types.Combination of multiple ultrosund techniques can diagnose PTA prenatally,make accurate classification and detect accompanying malformations,which is of great significance to offer proper pregnancy counselling and postpartum treatment.
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Objective Evaluating a new continuity between the right ventricle and the pulmonary artery for repair of persistent truncus arteriosus(PTA).Methods Between January 2000 and December 2012,we performed operation of 53 children with without a conduit:sleeve and direct right ventricle-pulmonary artery anastomosis [age,40 day to 2.3 years; median age,(0.9 ± 0.5) years] in 86 cases of PTA.The other 33 cases were performed with valved conduit.we used different methods to form the posterior floor of the new pulmonary arterial trunk.The edge of the floor was attached directly to the superior margin of an oblique incision made in the left-anterior wall of the right ventricle.A Gortex monocusp was attached to the lower half margin of the right ventricular incision in 26 cases.Results There were high pulmonary hypertension (PH) in 6 cases with the treatment of anti-PH.There were 2 death postoperatively because of pulmonary hypertension and right heart disfunction.The other patients were discharged from hospital.Cardiac ultrasound at hospital discharge showed pulmonary regurgitation that was trivial in three cases,mild in twentyseven cases,and moderate in twentyone case.During follow-up(36-60 months,median 54 months),there were no death.Conclusion This simple modification for surgical correction of persistent truncus arteriosus may be an effective alternative that overcomes conduit-related problems from the mid-followup period.
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A neonate, presenting with cyanosis, received the diagnosis of persistent truncus arteriosus with truncal valve stenosis with insufficiency. Her disease was classified as persistent truncus arteriosus Van Praagh type A1, or Collett and Edwards type I. At the age of 2 months, she underwent a modified Blalock-Taussig shunt, and her operative team was waiting for adequate body weight gain before performing further surgery. At the age of 1 year, however, she began to have repeated episodes of congestive heart failure due to severe tricuspid valve regurgitation and truncal valve insufficiency. When she reached 18 months of age, she underwent a definitive operation including a truncal valve plasty, VSD patch closure, and a right ventricular outflow tract reconstruction. Postoperative echocardiography 6 months after surgery showed a good truncal valve function with minimal regurgitaion. Truncal valve surgery is a challenging operation ; we report a successfully treated case, though the patient will require extensive follow-up.