Clinical Presentation and Therapy of Truncus Arteriosus.

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.

Hemitruncus Arteriosus in a 10-Day-Old Neonate with Patent Ductus Arteriosus and Thrombocytopenia.

Hemitruncus arteriosus refers to an uncommon congenital cardiovascular abnormality. It usually presents in infancy and leads to the development of pulmonary hypertension, heart failure, and in severe cases maybe death. Herein, we reported hemitruncus arteriosus in a 10-day-old neonate with respiratory distress, patent ductus arteriosus, and thrombocytopenia.

[Immediate and far remote results of the truncus arteriosus communis radical correction].

The results of surgical treatment of 59 patients, having truncus arteriosus communis (TAC), were analyzed. The hospital lethality indices were reduced essentially due to the diagnosis, surgical technique and the patients postoperative management improvement.

Root dilation in patients with truncus arteriosus.

OBJECTIVE: A dilated aortic root is a common finding in children and adults with some forms of congenital heart defects. No data exist on root dilation in truncus arteriosus. We sought to delineate root dimensions across a population of patients with truncus arteriosus. DESIGN: We performed a single-center retrospective review of all patients with truncus arteriosus. Demographic information, clinical history, and most recent echocardiographic data were evaluated. RESULTS: We identified 76 patients whose most recent study was at a median age of 5.4 years (range 0--32.7 years). Mean truncal root z-score was 5.1 ± 2.3. All but three patients had truncal root z-scores greater than or equal to 2. Truncal root z-scores remained stable with increasing body surface area and age. There were no cases of dissection or rupture. Six patients underwent truncal root surgery, typically for indications of root dilation with significant truncal valve insufficiency and left ventricular dilation. CONCLUSIONS: In conclusion, mean truncal root z-score was 5, and all but three patients had truncal root z-scores greater than or equal to 2. Although repeat surgical intervention was rare and major complications related to root dilation did not occur in our cohort, further studies with longitudinal follow-up into adulthood are needed.

Stenting of ductus arteriosus in a neonate with truncus arteriosus and interrupted aortic arch associated with a right aortic arch.

This report describes a case in which successful stenting of ductus arteriosus (DA) was performed for a 27-day-old boy with truncus arteriosus (TA) and interrupted aortic arch (IAA). The patent DA was associated with a right aortic arch. During the balloon catheter crossing of the ductus, the DA and descending aorta shifted toward the left side, making appropriate stent placement difficult. Additionally, the DA was longer than in previously reported cases with left aortic arch, thus requiring a longer stent. This experience suggests that DA stenting in neonates with TA and IAA averts surgical repair during the early neonatal period.

[Common truncus arteriosus in adults]. / Tronco arterioso común en adultos.

The purpose of this study is to describe the factors that allow for the survival of patients with persistent truncus arteriosus until adult life and cared for at the National Institute of Cardiology "Ignacio Chávez". From May 2003 to June 2007, six adult patients with clinical suspicion of truncus arteriosus were studied. All patients were subjected to clinical history, electrocardiogram of twelve leads, echocardiography, and cardiac catheterism. In these patients, the clinical symptoms began early of life. The ecocardiographic findings demonstrated the presence of truncus arteriosus type I in all cases and the most frequent associated anomalies were: hypoplasia of pulmonary branches, isolated coronary ostium, persistent ductus arteriosus and right aortic arch. Almost all patients had pulmonary hypertension. Based on this work, we conclude that until now we do not known the actual survival time of adult patients with complex congenital heart disease, such as truncus arteriosus. There are factors, such as pulmonary hypertension and hypoplasia of the pulmonary branches that are deleterious for life in some cardiovascular diseases, but in others allow for the survival of patients, as occurred in these patients with truncus arteriosus.

An adult with truncus arteriosus and unilateral pulmonary hypertension.

We report a young man who has persistent truncus arteriosus (TA), severe truncal regurgitation and unilateral pulmonary hypertension. Our patient had palliative main pulmonary artery (PA) banding done during infancy that was not followed by definitive corrective surgery. Unilateral irreversible left sided pulmonary hypertension developed due to migration of the PA band to the right PA. The patient presented to us with infective endocarditis of the truncal valve. This had resolved with medical treatment. Discussion was made on general management of TA and specific difficult management issues of palliated TA in adult, as found in our patient.

Severe truncal valve stenosis: diagnosis and management.

Severe truncal valve stenosis, due to dysplastic valve, may be found in a small number of patients with truncus arteriosus. Its presence complicates the surgical management of this anomaly due to lack of optimal therapeutic options and associated ventricular dysfunction. We report a case of truncal root replacement using a homograft for the management of such condition, and review the literature related to its management and outcome.

Cyanotic congenital heart disease with decreased pulmonary blood flow in children.

Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.

Cyanotic congenital heart disease with increased pulmonary blood flow.

Pediatricians daily encounter children with systemic cyanosis. The numerous reasons for cyanosis in neonates and infants include pulmonary, hematologic, toxic, and cardiac causes. Congenital heart defects may cause cyanosis. Often, an obvious cardiac reason for cyanosis is decreased PBF; however, several congenital heart defects cause systemic cyanosis with increased PBF, such as TGA, truncus arteriosus, and TAPVR. Because neonates are discharged from the hospital soon after birth, this magnifies the importance of each physical examination. Pediatricians need to remain alert for children who have symptoms of increased PBF with or without cyanosis. With advances in the diagnosis and treatment of patients with CHD, corrective procedures can be performed at many ages.

Asistencia respiratoria en recién nacidos / Artificial respiration in newborn infants

El presente trabajo es la descripción de la evolución presentada por 243 Recién nacidos que durante un período de 30 meses consecutivos requieron terapia respiratoria. Ingresaron 103 en P.P.C. y 140 en A.R.M., con una tasa de mortalidad global del 24.6% (9.7% en P.P.C. y 35.7% en A.R.M.). No hubo diferencias en la sobrevida de pacientes nacidos en el Hospital versus derivados. La mortalidad fue mayor en RN que necesitaron A.R.M. y presentaron el antecedente de severa depresión al nacer (apgar de 3 o menor al minuto de vida), o ingresaron en dicha técnica con un pH menor de 7.10 o una paCO2 mayor de 80 mm Hg. Las complicaciones observadas en A.R.M. fueron: pulmonares (en especial extubación espontánea) 34.2%, neumotórax 19.2%, ductus arterioso persistente 15.7% e infección intrahospitalaria 6.6 por ciento. Se realiza una auditoría de la aplicación de las normas del servicio concluyendo que en la asistencia de 30 RN hubo fallas potencialmente evitables (AU)

Asistencia respiratoria en recién nacidos / Artificial respiration in newborn infants

El presente trabajo es la descripción de la evolución presentada por 243 Recién nacidos que durante un período de 30 meses consecutivos requieron terapia respiratoria. Ingresaron 103 en P.P.C. y 140 en A.R.M., con una tasa de mortalidad global del 24.6% (9.7% en P.P.C. y 35.7% en A.R.M.). No hubo diferencias en la sobrevida de pacientes nacidos en el Hospital versus derivados. La mortalidad fue mayor en RN que necesitaron A.R.M. y presentaron el antecedente de severa depresión al nacer (apgar de 3 o menor al minuto de vida), o ingresaron en dicha técnica con un pH menor de 7.10 o una paCO2 mayor de 80 mm Hg. Las complicaciones observadas en A.R.M. fueron: pulmonares (en especial extubación espontánea) 34.2%, neumotórax 19.2%, ductus arterioso persistente 15.7% e infección intrahospitalaria 6.6 por ciento. Se realiza una auditoría de la aplicación de las normas del servicio concluyendo que en la asistencia de 30 RN hubo fallas potencialmente evitables