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PubMed1990

Ventricular inversion with truncus arteriosus.

We report clinical, angiocardiographic, and anatomic findings of an infant with ventricular inversion (atrioventricular discordance) and truncus arteriosus. The patient was in congestive heart failure and the injections into the left ventricle, right ventricle, and truncus arteriosus allowed a precise diagnosis. The autopsy confirmed this very unusual combination of anomalies and in particular it showed a truncus type 2 completely surrounded by muscular infundibulum and mainly aligned with the right ventricle. Surgical correction of this malformation is a challenge and so far, to our knowledge, has never been reported in the literature.

Abnormalities, Multiple↗
PubMed2007

Rastelli and Norwood combination for the treatment of type I truncus arteriosus and hypoplastic aortic arch: a case report.

Although truncus arteriosus is often treated with low mortality and morbidity rates, truncal valve patency and aortic arch and coronary artery anomalies are factors that can contribute to a worse outcome. In this report, we present our experience with the combination of Rastelli and Norwood procedures for the treatment of Type I truncus arteriosus that was complicated by a hypoplastic aortic arch.

Aorta, Thoracic↗
PubMed1978

Association of atrioventricular valve atresia with single ventricle, truncus arteriosus communis and transposition. A basic reorientation in the approach to the definition of congenital heart defects.

A heart specimen is presented which showed a rare combination of atrioventricular valve atresia with single ventricle, truncus arteriosus communis with an anteriorly placed trunk, ie transposition. Because of the problems involved in the classification of this heart, the types of single ventricle are revised with particular attention to the rare cases of atrioventricular valve atresia and single ventricle reported in the literature. Also the rare type of truncus arteriosus communis with truncoatrioventricular valve discontinuity is excluded from the so-called transposition complexes, and properly classified as a form of truncus. The literal meaning of transposition is stressed and it is urged to approach congenital heart defects in a purely anatomic sense with definition of each segment as accurately as possible. Thus the present confusion in terminology and further plethora of new terms are avoided.

Child↗
PubMed2005

Index of suspicion.

Explore the source record for details and available documents.

Child, Preschool↗
PubMed1980

Aortic arch interruption with truncus arteriosus or aorticopulmonary septal defect.

Interruption of the aortic arch is commonly associated with cardiac malformations. The angiocardiographic appearance in the presence of coexistent truncus arteriosus or an aorticopulmonary septal defect may be very similar. Five patients with arch interruption, three of whom also had truncus arteriosus and two with an aorticopulmonary septal defect are analyzed and the pertinent literature reviewed. In the presence of a truncus arteriosus with arch interruption the aorta appears as an appendage arising anteriorly from a large "pulmonary trunk" with a single semilunar valve, whereas when there is an aorticopulmonary septal defect two separate great artery roots and two semilunar valves are visible. The angiographic features are best defined by a truncal root angiogram when there is a truncus arteriosus or by an aortic root angiogram in the presence of an aorticopulmonary fenestration.

Angiocardiography↗
PubMed1981

CT of anomalies of the mediastinal vessels.

Computed tomographic (CT) studies of the thorax were performed on 12 patients with congenital anomalies of the mediastinal great vessels. CT correctly identified 11 of 12 abnormalities including left superior vena cava, right aortic arch with aberrant left subclavian artery, L- and D-transposition of the great vessels, pulmonary valvular stenosis, and truncus arteriosus. CT obviated further invasive procedures in those anomalies not associated with intracardiac defects and supplemented the angiographic findings in some patients with intracardiac defects requiring catheterization.

Adolescent↗
PubMed1996

MR imaging of conotruncal abnormalities.

MR imaging has been shown to be accurate in the diagnosis and follow-up of congenital abnormalities of the heart and great vessels in infants and children [1-3]. Although echocardiology, in many cases, remains the primary imaging technique in congenital heart disease, MR imaging is a useful adjunct [1-3]. The advantages of MR imaging include a wide field of view and multiplanar capabilities [1-3]. Echocardiology is often suboptimal in evaluating the superior mediastinum because of the narrow imaging window [3]. This fact is especially true in patients after surgery and in older children. Therefore, MR imaging often plays a role in the evaluation of the great vessels.

Adolescent↗
PubMedDate not supplied

Syndromal associations of common origin of the carotid arteries.

The term "common origin of the carotid arteries" (COCA) has been proposed to replace the older terms "origin of the left carotid artery from the innominate stem" and "bicarotid trunk with anomalous right or left subclavian artery." These anatomic patterns are usually reported to occur in about 11% of whites and 20-25% of blacks and have been reported to have increased frequency in patients with esophageal atresia-tracheoesophageal fistula, DiGeorge anomaly, and anomalous origin of the left coronary artery from the pulmonary artery. COCA is a significant, if not invariant, feature of the great arteries in the condition usually called in the more recent literature "anomalous origin of the innominate artery," the most frequent cause of symptomatic tracheal compression by anomalous systemic arteries. Analysis of associations of COCA with various other congenital cardiovascular lesions showed, in addition, significant association with congenital polyvalvular disease, truncus arteriosus, aorticopulmonary window, trisomy 13, 18, and 21 syndromes, acrocephalosyndactyly (especially Apert syndrome), tetralogy of Fallot not associated with DiGeorge anomaly, and clinical Noonan phenotype. Pentalogy of Cantrell was associated with no increase in incidence of COCA.

Carotid Artery, Common↗
PubMedDate not supplied

Outflow tract abnormalities in atrioventricular canal malformations.

Atrioventricular canal malformations are commonly regarded as being arrests of development of normal cardiac septation since their morphology strongly resembles the normal heart of Carnegie stages 14 through 18. Similarly, the spectrum of outflow tract abnormalities, which includes truncus arteriosus, transposition of the great arteries, double outlet right ventricle, and tetralogy of Fallot, has been interpreted as a developmental arrest because these malformations resemble the normal embryonic outflow tract in Carnegie stages 14 through 18, respectively. The causes of the developmental arrests in these two classes of malformations are unknown. Observation of an unusual autopsy case with features of both atrioventricular canal defect and tetralogy of Fallot prompted us to review the outflow tract morphology in 38 hearts with an atrioventricular canal malformation and two semilunar valves to determine if there was evidence to support the idea that both conditions may have the same pathogenesis. In all 38 hearts there was fibrous continuity between an anterior mitral leaflet and an aortic valve, which by anatomic definition means that no case had truncus arteriosus, transposition of the great arteries, or double outlet right ventricle. There were three cases with coarctation of the aorta, a lesion which arises when blood flow in the pulmonary trunk exceeds aortic flow during early development. There were four cases with pulmonary to aortic valve caliber ratios similar to those found in tetralogy of Fallot but the aortic-to-pulmonary valve angle relative to the heart base ranged over the full spectrum of angles seen in normal hearts and those with tetralogy of Fallot. Furthermore, this angle showed the same range of values for partial and complete canal defects, and did not correlate with patient age or ratio of pulmonary to aortic valve caliber. We conclude that the pathogenesis of the spectrum of outflow tract developmental arrests is different from that of atrioventricular canal malformations.

Adult↗
PubMedDate not supplied

A case of truncus arteriosus type II.

A case of truncus arteriosus type II is reported. Truncus arterious is an uncommon congenital cardiac defect where a single great vessel exits the heart. Truncus arteriosus is usually fatal, if untreated. This defect occurs when the conus arteriosus and the truncus divide erroneously in the embryo. Palliative surgery in truncus arteriosus has been unsuccessful. Pulmonary banding has been tried and was ineffective and usually fatal. We operated on a nine-month-old (6200 g) male infant with a type II (Edwards-Collett) defect and a large ventricular septal defect. The pulmonary artery average pressure was 51 mmHg. We performed a cardiopulmonary bypass in the usual manner. Pulmonary arteries were resected from the truncal root, and primary end-to-end anastomosis of the truncal root to the ascending aorta was performed. Right ventricle to pulmonary artery continuity was provided using a valveless Gore-Tex graft. We lost our patient due to intractable pulmonary hypertension on the first postoperative day.

Anastomosis, Surgical↗
PubMed1976

[Reconstruction of the pulmonary outflow tract with a valved conduit (author's transl)].

The authors describe 25 patients who underwent open heart surgery for reconstruction of the pulmonary outflow tract with a valved conduit. The following anomalies were observed: six cases of great vessels transposition, five cases of pulmonary artery atresia, five cases of truncus, three cases of tetralogy of Fallot and six cases of single ventricle. Two cases of single ventricle were treated in order to be transformed into a systemic ventricle. The pulmonary outflow was reconstructed with a valved conduit anastomosed between right atrium and pulmonary artery. This study includes pre- and postoperative hemodynamic values, surgical evaluation and a 5-months to 3-years follow-up. The authors emphasize that it is important that the surgical indications are closely related to the morphology and hemodynamics of pulmonary circulation for a better result.

Adolescent↗
PubMed1999

[Primary reconstruction of the common truncus arteriosus in infants, using modified pulmonary homograft].

Truncus arteriosus communis (TAC) is a complex heart disease, it presents 1% of the congenital heart defects. A 7 weeks old infant with cardiac failure was operated on with TAC. The bicuspidized (14 mm diameter) pulmonary homograft valved conduit was used to reconstruct the right ventricular outflow tract (RVOT). The sternum closure was delayed. The important points of the indications for the surgical management of TAC: 1. Management or prevention of the pulmonary hypertensive crisis. 2. Patient's age. 3. Reconstruction of the RVOT (choices: homograft, dacron valve conduit, autologous pericardial valve conduit, porcine aortic root, direct anastomosis of the pulmonary trunc to the right ventricle). The surgical management is based on the optimal combination of these three important points. This is the first successfully performed primary surgical repair of an infant with TAC using modified pulmonary homograft in Hungary.

Echocardiography↗