Rationalising the nomenclature of common arterial trunk.

Hearts having a common arterial trunk belong to a family of congenital cardiac malformations for which traditional systems of classification and nomenclature are plagued by internal paradoxes, incompatibility between systems due to the lack of potential for identification of synonyms, or irreconcilable inconsistencies with our current knowledge of cardiac development and morphology. A simplified categorisation that classifies these hearts on the basis of pulmonary or aortic dominance reconciles the existing disparate categorisations, is in keeping with recent findings concerning cardiac development, and emphasises the principal morphologic determinant of surgical outcome.

Surgery for common arterial trunk.

Surgery for common arterial trunk has evolved over the past 30 years. Current management involves total repair during the neonatal period with excellent expected results. The presence of truncal valve insufficiency or interrupted aortic arch may increase the surgical risk for morbidity and mortality. Current therapy and management continues to evolve.

Prenatal diagnosis of truncus arteriosus using multiplanar display in 4D ultrasonography.

Prenatal diagnosis of truncus arteriosus with two-dimensional sonography requires expertise in fetal echocardiography. Indeed, truncus arteriosus shares with tetralogy of Fallot and pulmonary atresia with a ventricular septal defect (VSD) the sonographic finding of a single arterial trunk overriding a VSD. The diagnosis of truncus arteriosus can be confirmed when either the main pulmonary artery or its branches are visualized arising from the truncus itself. This requires sequential examination of multiple scanning planes and a process of mental reconstruction of their spatial relationships. The advantage of multiplanar imaging in three-dimensional and four-dimensional ultrasonography is that it allows for the simultaneous visualization of three orthogonal anatomic planes, which can be very important in diagnosing cardiac abnormalities. We report, first, a case of truncus arteriosus diagnosed in utero where the multiplanar display modality provided important insight into the differential diagnosis of this conotruncal anomaly, and then, review the diagnosis of truncus arteriosus on ultrasound.

Persistent truncus arteriosus: twenty years experience in a tertiary care center in Taiwan.

BACKGROUND: Persistent truncus arteriosus (PTA) is a rare congenital heart disease. The disease spectrum and outcome in the Oriental are still unclear. METHODS AND RESULTS: A total of 35 patients with PTA were identified from the Pediatric Cardiology Database of this institution, giving an incidence of 0.47%. According to the Van Praagh classification, we found type A1 in 16, A2 in 10, A3 in 4 and A4 in 5 patients. The most common truncal valves were still tricuspid (57.1%) and quadricuspid (28.5%), with the latter being associated with moderate to severe truncal regurgutation. Eleven patients had not received surgery and all died. We classified the era of operation as early (between 1980 and 1995) or late (1996-2001), and further classified the type of PTA as simple (type A1 or A2) or complex (interrupted aortic arch-A4, absent orifice of one PA from truncal root-A3 or moderate truncal valve insufficiency). The overall surgical mortality was 67%. Statistical analysis revealed that age at operation (older than 6 months), early operation era and complex PTA were risk factors for survival. CONCLUSIONS: PTA is a rare form of congenital heart disease in Taiwan and probably also in the Oriental. The surgical reparation seems to improve with experience. Early operation may prevent pulmonary vasculopathy. However, the results in complex PTA remain poor.

Assessment of operability for common arterial trunk without cardiac catheterisation.

Pulmonary vascular disease is a risk factor for the surgical management of common arterial trunk. Surgical repair, therefore, is usually performed in early infancy, before irreversible changes can occur in the epulmonary vasculature. Because of this, there has been an increasing tendency to dispense with cardiac catheterisation as a means of assessing pulmonary vascular disease. Cardiac catheterisation, nonetheless, is still performed when there is a risk of pulmonary vascular disease, such as in older children. There are no clear guidelines, however, as to who should be catheterised. We have developed a simple screening test to help make this decision.

Truncus arteriosus with anomalous pulmonary venous connection.

The anatomic, diagnostic, and management findings of 6 patients with truncus arteriosus and anomalous pulmonary venous connections are described. Additional risk factors indicative of poor prognosis were found in 3 of 4 patients with truncus arteriosus and totally anomalous pulmonary venous connection and in 1 patient with partially anomalous pulmonary venous connection.

Truncus arteriosus communis associated with chromosome 22q11 deletion.

OBJECTIVES: The purpose of this study was to clarify characteristics of truncus arteriosus communis associated with chromosome 22q11 deletion (del 22q11). BACKGROUND: DiGeorge syndrome and conotruncal anomaly face syndrome are associated with del 22q11 (hemizygosity). In 30% of cases, truncus arteriosus communis is associated with the deletion. METHODS: Fifteen consecutive patients with truncus arteriosus communis were checked for 22q11 with fluorescent in situ hybridization using an N25 probe (Oncor). Cardiovascular anomalies were studied with cardiac catheterization, cineangiography and echocardiography. RESULTS: Five patients had del 22q11. Two had a rare type of truncus arteriosus: type A3 of Van Praagh and Van Praagh with major aortopulmonary collateral arteries and pulmonary ostial stenosis. The other three had type A1 truncus arteriosus and pulmonary artery stenosis. One of them had major aortopulmonary collateral arteries. Ten patients with truncus arteriosus had no del 22q11. The types of truncus arteriosus in these 10 patients were type A1 in 7, type A2 in 2 and type A3 with closed ductus in 1. None of nine patients with type 1 or type 2 truncus arteriosus had pulmonary stenosis. CONCLUSIONS: In truncus arteriosus communis, the rare type A3 with major aortopulmonary collateral arteries and pulmonary ostial stenosis and type A1 with pulmonary artery stenosis are associated with del 22q11.

Management strategy and long-term outcome for truncus arteriosus.

OBJECTIVE: Evaluation of a consistent policy of elective repair of truncus arteriosus at 2-3 months of age for the patients who are independent of hospital ward care, and long-term outcome. METHODS: Retrospective study of 82 patients with truncus arteriosus who underwent total repair at the Victorian Paediatric Cardiac Surgical Unit between 1979 and December 1995. The timing was based on a consistent policy of elective repair at 2-3 months of age for patients who were independent of hospital ward care. Earlier repair was performed when the patients were in uncontrolled congestive heart failure. RESULTS: Follow-up was complete for all patients with a mean of 76 months (1-183). There were 11 hospital deaths (13.4% CL 9-18.5), and five late deaths, actuarial survival at 80 months was 81% (CL 70-88%) with 39 patients uncensored at that point. For the purpose of this presentation, patients have been grouped according to their age at repair; 1, neonates n = 17 (hospital mortality = 5); 2, infants 1-6 months of age n = 48 (hospital mortality = 4); 3, patients beyond 6 months n = 17 (hospital mortality = 2). This series includes 10 patients with interrupted aortic arch with no mortality, and 10 patients with discontinuous pulmonary artery (hospital mortality = 2). Thirty-seven patients have had 54 conduits replaced to date. Using multiple regression, body weight < 3 kg was the only significant independent risk factor for hospital mortality. CONCLUSIONS: Our management policy tended to gather patients with risk factors described elsewhere into presentation group 1 and low risk truncus patients into presentation group 2. Deferral of surgery to 2-3 months of age is possible and lowers the surgical risk.

Anatomy of the proximal coronary arteries as a risk factor in primary repair of common arterial trunk.

The objective of this study was to investigate whether the proximal coronary arterial anatomy is a risk factor in surgical treatment of common arterial trunk, with special focus on the value of preoperative angiocardiography. A retrospective analysis was performed of all 22 patients who underwent primary surgical repair of the common arterial trunk, with a mean follow-up of 5.1 years. In 18 patients preoperative angiocardiography was performed. Anatomical features (angiocardiographical, surgical as well as post-mortem) of the proximal coronary arteries were investigated. With standard biplane angiocardiography single and dual coronary arterial systems could adequately be distinguished. However, the position of the coronary orifices in relation to the sinus of Valsalva could not adequately be identified. Three patients had coronary abnormalities without surgical consequences. In 2 cases the surgical approach had to be modified due to the coronary anatomy. Early mortality was 23% (5/22) and was correlated with worse functional class (p < 0.05) and earlier date of operation (p < 0.05). Late mortality was 5% (1/22). Five patients were reoperated, without mortality. Fourteen surviving patients are in functional class I, and 2 in class II. A further improvement of the surgical therapy of common arterial trunk might be provided by adequate appreciation of the proximal coronary arterial anatomy at surgery.

Persistent truncus arteriosus--an autopsy study of 16 cases.

Sixteen specimens of heart with persistent truncus arteriosus were studied to evaluate the anatomic features. Using the Collet and Edwards classification, type I truncus arteriosus was the most frequent (62.5%). Using Van Praagh's classification type A1 was the most common (43.7%). There were two cases which could not be classified according to the Collet and Edwards classification. The truncal valve was tricuspid in 75% of the cases and bicuspid in the remaining 25%. In all 6 cases with interruption of the aorta, the truncal valve was committed to the right ventricle. The ventricular septal defect was subtruncal in all except 1 case. There was variation in the thickness of the posterior limb of the septal band and the ventriculo-infundibular fold. Absence of the ventriculo-infundibular fold in 3 cases led to truncal tricuspid continuity. Right-sided aortic arch and interruption of the aorta were frequently associated arch anomalies.

Truncus arteriosus communis associated with interrupted aortic arch: a report on two uncommon cases.

The paper presents two infants with the A-4 type of truncus arteriosus communis (according to Van Praagh's classification). One patient who survived a surgical procedure demonstrated a rare variant of aortic arch interruption to the left off the left subclavian artery (type A according to Celoria and Patton), whereas the second presented an uncommon anomaly in which the right subclavian artery originated from the descending aorta with associated severe truncal valve incompetency. The authors describe the clinical picture along with the surgical treatment of the first infant who being six days old was subjected to a correction employing the wide patent ductus arteriosus to reconstruct the aortic arch, following the method described by Gomes and McGoon. Subsequently an aortic homograft was implanted in order to connect the right ventricle and the pulmonary artery.

Truncus arteriosus revisited: an angiographic demonstration.

The 1965 reclassification of truncus arteriosus by Van Praagh and Van Praagh greatly enhanced our understanding of this interesting anomaly. This brief review article attempts to illustrate the various types of truncus arteriosus identified in this classification by demonstrating their angiographic features. Reemphasis of the usefulness of this classification should help students of congenital heart disease recognize the advantages of a uniform diagnostic approach to this entity.

Truncus arteriosus type A3: complex repair with cryopreserved pulmonary homograft.

A 6-year-old girl with truncus arteriosus type A3, one of the rarest anatomic types, underwent corrective surgery using a cryopreserved valved pulmonary homograft. The special anatomic features in this case were individual origin of the pulmonary arteries. The right pulmonary artery came directly off the truncal vessel, whereas the left pulmonary artery was connected to the aortic arch via a stenotic ductus or ductus-like vessel. The preoperative pulmonary blood flow distribution was 94% on the right versus 6% on the left side. The underperfusion of the left lung may have been related to pulmonary hypoplasia due to long lasting stenosis at the left pulmonary artery take off. The postoperative pulmonary blood flow distribution was 67% on the right versus 33% on the left side. The operative and postoperative course has been uneventful; presently, 6 months after the operation, the patient is in improved clinical condition.