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At least 19 recordsLinked to original sources
PubMed1997

Caesarean delivery of a patient with truncus arteriosus.

Truncus arteriosus Type IV is a rare congenital cardiac malformation characterized by agenesis of the pulmonary arteries. Pulmonary perfusion is ensured by bronchial arteries. One common arterial trunk arises from both ventricles and systemic pulmonary and coronary arteries originate from this common vessel. Survival is rare without surgical correction. We report a case of uncorrected truncus arteriosus Type IV in a 28-year-old primigravid woman who underwent caesarean section.

Journal Article↗
PubMed2006

Complete autologous reconstruction of the aorta and the pulmonary bifurcation in truncus arteriosus communis.

Truncus arteriosus communis is a congenital heart malformation, which is usually repaired in the neonatal period or early infancy. Although results of repair are good, there is long-term morbidity caused by reoperations mainly owing to right ventricle to pulmonary artery conduit exchange or stenosis at the pulmonary artery bifurcation as recently reported for Contegra conduits. We present a new technique for complete autologous reconstruction of the aorta and the pulmonary bifurcation in truncus arteriosus type I and II.

Anastomosis, Surgical↗
PubMed1977

Surgical correction of types II and III truncus arteriosus.

In truncus arteriosus types II and III, one or both pulmonary arteries arise independently from either side of the truncus. In the surgical correction of this anomaly, we have utilized on operative technique in which the essential features are as follows: ventricular septal defect (VSD) closure, which directs left ventricular outflow into the truncus: (2) anastomosis of a Dacron tube containing a glutaraldehyde-preserved procine aortic heterograft to the right ventriculotomy: (3) removal of a circumferential band of the truncus containing both pulmonary artery orifices; (4) tailoring of the band of truncus tissue into a generous cuff which is anastomosed to the distal end of the valved Dacron conduit; and (5) restoration of aortic continuity with a tubular Dacron graft. Since 1971, 4 children ages 2 to 9 years have undergone successful correction of truncus arteriosus types II or III by this technique. In one patient with marked pulmonary hypertension and congestive heart failure preoperatively, the pulmonary vascular resistance had reverted to normal by 3 years after the operation. In one patient in whom bronchial collaterals to the right pulmonary artery were present, postoperative left ventricular failure required reoperation for ligation of the collaterals. All 4 patients are asymptomatic and fully active 5 to 60 months postoperatively. None has evidence of stenosis or insufficiency of the heterograft valve.

Animals↗
PubMed1991

Dizygotic twins concordant for truncus arteriosus.

Persistent truncus arteriosus (TA) is an uncommon congenital cardiovascular malformation, which comprises between 0.4% and 4% of all congenital heart defects. Occurrence of TA in siblings has been reported infrequently. Twins concordant for isolated TA appear to have been reported only once previously. In this paper, we describe dizygotic twin females who were concordant for isolated TA.

Diseases in Twins↗
PubMed1978

Surgical significance of the coronary arterial anatomy in truncus arteriosus communis.

In truncus arteriosus communis, as in other anomalies of conotruncal development, the coronary arterial anatomy not only differs from that found in the normal heart but also is subject to unpredictable variations. A consistently distinctive pattern was found in roughly two thirds of 31 cases, a frequency approximating that with which a distinctive coronary arterial pattern occurs in complete transposition of the great vessels. Surgical injury to a major coronary artery was identified in 2 of the 30 hearts from patients with truncus arteriosus communis who died in the early postoperative period. Of particular significance are large branches of the right coronary artery crossing the upper anterior surface of the right ventricle to supply the anterobasal surface of both ventricles and the upper part of the interventricular septum. These arteries are at special risk in surgical procedures utilizing a conduit anastomosed to a right ventriculotomy.

Adolescent↗
PubMed2003

Chromosome 22q11 deletion in patients with truncus arteriosus.

The association between truncus arteriosus and chromosome 22q11 deletion is well recognized, but the frequency of a chromosome 22q11 deletion has not been characterized in a large series of patients with truncus arteriosus, and little is known about cardiovascular morphologic features associated with a chromosome 22q11 deletion in this group of patients. We prospectively enrolled 50 consecutive patients with truncus arteriosus who were admitted to The Children's Hospital of Philadelphia between November 1991 and December 2001. Patients were studied for chromosome 22q11 deletion using fluorescence in situ hybridization. Correlations between anatomic features and chromosome 22q11 deletion were assessed. A chromosome 22q11 deletion was detected in 20 of the 50 patients (40%). Anatomic features that were significantly associated with a chromosome 22q11 deletion included a right-sided aortic arch, an abnormal aortic arch branching pattern, both abnormal sidedness and branching of the aortic arch, and the combined category of either abnormal sidedness or branching of the aortic arch. There was a trend toward the association of discontinuous pulmonary arteries with a chromosome 22q11 deletion. Interruption of the aortic arch and truncal valve morphology and function did not correlate significantly with the presence of a chromosome 22q11 deletion. In conclusion, a chromosome 22q11 deletion is common in patients with truncus arteriosus, and those with abnormal sidedness and/or branching of the aortic arch are significantly more likely to have a deletion. Clinically important anatomic variables, such as abnormalities of the truncal valve and interrupted aortic arch, were not associated with a chromosome 22q11 deletion in this series.

Chi-Square Distribution↗
PubMed1992

[Total repair for truncus arteriosus].

Total repair for truncus arteriosus using an external conduit was performed in 12 patients from 1978 through 1989. Six cases were infants (mean age: 3.4 months) and 6 were children (mean age; 1 years 9 months). Two cases had Collet-Edwards type II truncus and the other 10 cases had type I truncus. One of the infants was associated with an interruption of the aorta and another had a severe regurgitation of the truncal valve (TrV). For external conduits, we used a non-valved conduit in one infant, a composite valved conduit of Dacron containing a heterograft valve in 4 children and a valved pericardial roll made of an autologous or porcine pericardium in 5 infants and 2 children. One infant with a severe regurgitation of the TrV needed valve replacement along with enlargement of the annulus of the TrV. One infant who had replacement of the TrV died early postoperatively. Another infant died 10 months after total repair due to an infection of an external conduit. Cardiac catheterization was performed in all 10 survivors. The mean value for the systolic pulmonary/systemic pressure ratio decreased from 0.98 +/- 0.09 preoperatively to 0.36 +/- 0.09 postoperatively. Replacement of an external conduit was performed due to a conduit stenosis in 2 children and 1 infant, 10 years and 2 months, 7 years and 9 months, and 1 year and 8 months after the total repair, respectively. In one of these 2 children, replacement of the aortic valve was performed due to a severe aortic regurgitation. We conclude that our results of total repair for truncus arteriosus were satisfactory. However, it remains to be solved how to manage an infant with truncus arteriosus associated with a severe regurgitation of the TrV.

Bioprosthesis↗
PubMed1997

Truncus arteriosus with patent ductus arteriosus and normal aortic arch.

In hearts with a common arterial trunk (truncus arteriosus), there is almost always an inverse development of the aortic arch and the ductus arteriosus. Truncus with a normal aortic arch and a patent ductus is a rare echocardiographic and surgical finding. In this report, we describe 2 neonates in whom truncus arteriosus with a normal aortic arch and a medium or large patent ductus was diagnosed by preoperative echocardiography (without catheterization) and confirmed intraoperatively.

Aorta, Thoracic↗
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↗
PubMedDate not supplied

Postoperative hemolysis and its prevention in corrective surgery of truncus arteriosus.

Two cases of truncus arteriosus type I associated with truncal valve insufficiency were operated upon with the Rastelli's procedure. The first case of 3-year-old boy died from severe hemolysis which was caused by the regurgitant jet from the truncal valve running against the rough-surfaced Dacron patch for the VSD. The second case of 12-month-old girl was treated with a Dacron patch lined with an autologous pericardial pedicle. Her postoperative course was uneventful. The authors discuss the potential risk and prevention of postoperative hemolysis in corrective surgery of the truncus arteriosus with truncal valve insufficiency.

Child, Preschool↗
PubMed1993

Fate of small homograft conduits after early repair of truncus arteriosus.

Neonatal repair of truncus arteriosus is being performed in a number of centers, often with the use of small homograft conduits. The fate of the homograft and the risk of replacement were the subjects of this study. Between January 1987 and October 1991, 43 infants aged less than 3 months (range, 3 to 90 days) underwent primary repair of truncus arteriosus including implantation of a valved homograft conduit (diameter, 7 to 12 mm). Twenty-nine had follow-up of more than 6 months (range, 6 to 65 months; mean, 21.9 months). After a mean period of 31 months (range, 8 to 65 months), 7 patients showed obstruction with right ventricular pressures at least 75% systemic and underwent either a conduit change (n = 5) or a patch augmentation (n = 2). Mean cardiopulmonary bypass time at reoperation was 99 minutes; mortality was zero. Five other children are known to have a right ventricular pressure of 50% to 60% systemic, 2 having undergone balloon dilation. Statistical comparison of the patients with conduit reoperation or high right ventricular pressure (n = 12) with the rest of the population (n = 17) revealed an elevated pulmonary artery to right ventricular pullback gradient on postoperative day 1 after the repair (7.7 versus 1.3 mm Hg; p = 0.001) and choice of an aortic over a pulmonary homograft (100% versus 64.7%; p = 0.065) as significant risk factors. Age and weight at repair, postoperative pulmonary artery pressure, length of follow-up, and size of the homograft showed no significant differences between the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗
PubMed1992

Current method of repair of truncus arteriosus.

Surgical treatment of truncus arteriosus has been demonstrated to be optimized when approached in early infancy. In the past, the materials available, namely the 12-mm porcine valve Dacron conduit, restricted the size of the patient in whom early repair could be performed with increasing risk associated with decreasing age and size. The availability of homografts for reconstruction of right ventricular pulmonary artery continuity has allowed repair even in the neonatal period. Two hundred forty-four patients with truncus arteriosus have been operated on at the University of California San Francisco since 1975. In the past 5 years, 49 have been repaired using homograft reconstruction. The method of repair differs markedly from that of Dacron conduits, and specific points concerning homograft insertion including orientation, method of proximal anastomosis, and sizing are discussed in detail and the difference in insertion from the Dacron bovine heterograft conduit are discussed.

Age Factors↗
PubMed1982

Two-dimensional echocardiographic prospective diagnosis of common truncus arteriosus in infants.

Two-dimensional echocardiographic prospective diagnosis of truncus arteriosus was made in 7 infants. Two infants had truncus arteriosus type I, 3 patients had truncus arteriosus type II, 1 infant had truncal valve stenosis with an interrupted aortic arch, and 1 had type IV truncus arteriosus with pulmonary hypertension. Multiple imaging views were utilized to confirm the diagnosis. The parasternal long-axis view demonstrated the great vessel-ventricular septal override and the origin of the pulmonary arteries from the posterior aspect of the ascending truncus. The suprasternal notch image facilitated identification of the left- or right-side aortic arch and the origin of the pulmonary arteries from the truncus. Transverse imaging sections at the base of the heart facilitated identification of the pulmonary artery origin of truncus arteriosus type I. Subcostal coronal and sagittal views imaged the common truncus and the ventricular septal defect. These echocardiographic images were contrasted with and discriminated from those of an infant with aorticopulmonary window with intact ventricular septum. Although cardiac catheterization and angiography may be required to assess pulmonary arterial pressure, pulmonary vascular resistance, and the distal pulmonary arterial anatomy in truncus arteriosus, 2-dimensional echocardiography can be used to correctly establish the morphologic diagnosis of truncus arteriosus in infants.

Angiocardiography↗
PubMed1975

Current status of the surgical treatment of truncus arteriosus.

Persistent truncus asteriosus is now correctable surgically in patients with favorable anatomy. Given pulmonary arteries of reasonable size arising from any source, successful correction is possible so long as irreversible pulmonary vascular disease has not occurred. Although the majority of children with this defect demonstrate increased pulmonary blood flow, systemic-pulmonary artery shunts can be used. Also, banding of the pulmonary artery, followed subsequently by successful total correction, has been described. Recent reports of a few successful total corrections in infancy, performed with the aid of deep hypothermia and circulatory arrest, may modify the current approach. Although the majority of the reported corrections have involved aortic homograft reconstruction of the pulmonary artery, we strongly favor a synthetic prosthesis containing a heterograft valve. Based upon our clinical experience and this review of the literature, a suggested management protocol is presented.

Age Factors↗
PubMedDate not supplied

A case of common truncus arteriosus with rare cardiac abnormalities.

Truncus arteriosus communis is a well-known, congenital, cardiac abnormality. In this report, we describe a very rare variant in which the truncus arose from the right ventricle, the atrial and ventricular septa were intact, the left ventricle was hypoplastic and the small right atrium was draining to the truncus arteriosus through a fibrose tunnel-like connection.

Heart Ventricles↗