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PubMed1980

[Surgical treatment of truncus arteriosus in the 1st year of life].

80% of patients with Truncus arteriosus died in the first year of life by cardiac insufficiency from left to right shunt, high level of pulmonary vascular disease, truncal valve insufficiency. The palliative procedure, banding of the pulmonary arteries, is technically difficult, with high hospital mortality (50%) and increases the risk of the second stage repair (70%). Eight infants (1 to 9 months) have been corrected with 3 operative deaths and two late deaths. Three patients are doing well 12, 12 and 36 months post-operatively. Two technical problems are of concern: 1: The size of the valved extracardiac conduit comparing with the size of the infants thorax and the long term follow-up of such conduit. 2: The management of the truncal valve insufficiency: no plasty or replacement have be done, but in three cases of truncal insufficiency one pericardial patch has been used to double the VSD's dacron patch to avoid hemolysis. The one-stage repair of truncus arteriosus in infancy is one acceptable approach.

Humans↗
PubMed1994

[Brain abscess in patients with cyanotic heart defects].

As a result of hypoxia following right-to-left shunts, cerebral bacterial spreading and an altered blood-brain-barrier permeability, brain abscesses (BA) are typical complications in patients with cyanotic congenital heart disease. In 483 prospectively followed patients the incidence of BA was 0.45%/year. It was higher (0.57%/year) for patients with tetralogy of Fallot where the cumulative risk within the first two decades of life was 12.1 +/- 1.7%. The risk of BA complicating cyanotic heart disease is inconstant and continuously increasing up to approximately age 12 (instantaneous risk at that time: 1.75 +/- 0.12%), decreasing thereafter. With respect to etiology, infectious endocarditis, infections per continuitatem, bacterial meningitis, bacterial lung diseases with intrapulmonary shunts, and thromboembolic complications of systemic infections have to be differentiated. The stepwise diagnosis includes CCT to demonstrate the typical contrast enhancement and a lumbar puncture which shows granulocytic pleocytosis. If the cerebral spinal fluid fails to demonstrate the typical findings, cerebral angiography may be necessary to exclude a malignant vascularized neoplasma. In cases of doubt, stereotactic cerebral biopsy should be performed. Optimal antibiotic therapy after determining the minimal bactericidal concentration and combination of antibiotics is of utmost prognostic significance. Cranial computed tomography should be repeated after 6, 14, and 24 days. Infections resistant to antibiotics may necessitate local instillation of antibiotics.

Adolescent↗
PubMed1995

Performance of right ventricle to pulmonary artery conduits after repair of truncus arteriosus: a comparison of Dacron-housed porcine valves and cryopreserved allografts.

Repair of complex congenital heart defects often requires the use of extracardiac conduits. These repairs can be technically accomplished with excellent early results. However, the long-term performance of various conduits is less than optimal. In this report, we examined the long-term outcome of xenograft valved Dacron conduits and cryopreserved allograft valved conduits used for reconstruction of the right ventricular outflow tract in young patients with truncus arteriosus. A retrospective review was performed on 222 patients who underwent primary surgical repair of truncus arteriosus between January 1975 and December 1994 using a xenograft valved synthetic conduit (group I; n = 175) or with a cryopreserved valved allograft conduit (group II; n = 47). Median age at repair was 121 days in group I and 70 days in group II; median weight of patients in both groups was the same (4.2 kg). Conduit-related early deaths occurred in 5.7% (10/175) of patients in group I and none in group II. In a cohort of age-matched patients, actuarial freedom from conduit-related reintervention at 5 years follow-up was significantly better (P = .037) for the cryopreserved valved allograft conduits when compared with xenograft valved synthetic conduits. However, this difference was not apparent by 7 years. Multivariate analysis showed conduit size (P = .0005) as a significant predictor of early conduit-related reintervention. This study shows that mid-term performance of cryopreserved allografts (which have the advantage of technical ease of insertion of availability of small sizes for use in neonates), is better than the xenograft Dacron conduits.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗
PubMed1995

Valved conduits of bovine pericardium for right ventricle to pulmonary artery connections.

Between February 1991 and October 1994, 29 patients received right heart bovine pericardial valved conduits. Patients' ages ranged from 19 days to 18 years (mean 3.5 years), with 8 patients younger than 1 year of age. Ten patients had truncus arteriosus type II or III, 7 had pulmonary atresia with ventricular septal defect (VSD), 6 had transposition of the great arteries and pulmonary stenosis (PS), 3 had corrected transposition with VSD and PS, 2 had double-outlet right ventricle, and 1 had aortic atresia with normal left ventricle and VSD. Two hospital deaths (6.9%) occurred, but no late deaths. The postoperative serial echocardiographic studies showed a mean transconduit gradient of 16 mm Hg with a range of 2 to 37 mm Hg. All patients are in functional class I. Although the intermediate results are satisfactory, considering the late complications known from others conduits, long-term follow-up is necessary.

Adolescent↗
PubMed1995

The use of a glutaraldehyde-preserved ovine pulmonary valve, as a pulmonary valve substitute in infants.

Reconstruction of the right ventricular outflow tract with a valved conduit is often necessary to repair some forms of congenital heart disease. We have used a 12- to 13-mm sized glutaraldehyde-preserved lamb's (ovine) valve for this purpose. The valves were harvested from the local abattoir, dissected, and stored in glutaraldehyde at 4 degrees C for 2 weeks before use. The valve was placed in four infants, two with truncus arteriosus, one with aortic valve atresia and ventricular septal defect, and one with absent pulmonary valve syndrome. In three patients, the valve had to be replaced in the second year of life, and in one patient in the fourth year of life. All patients are alive and well at follow-up. In the short-term, this valve has functioned well and could be considered where commercially available conduits or homografts are not routinely available.

Animals↗
PubMed1995

The 'Rastelli' operation--results at the Singapore General Hospital.

Reconstruction of the right ventricular outflow tract in patients with right ventricle-pulmonary artery discontinuity with a valved conduit is well established. The aim of this study was to review our results at the Singapore General Hospital. Between October 1989 and May 1993, 13 patients underwent the 'Rastelli' operation. The mean age at definitive repair was 68 months. Ten patients had pulmonary atresia with ventricular septal defect, two patients truncus arteriosus (one patient each with type 3 and type 2) and one patient had tetralogy of Fallot with absent pulmonary valve syndrome. In 12 patients cryo-preserved pulmonary artery homografts with a mean diameter of 21mm (19-23mm) were used, and a 11mm composite Hancock dacron valved conduit in one patient. There were no operative deaths. The mean duration of follow-up is 37 months (23-48 months). All patients were well and in NYHA class I on recent follow-up. On echocardiographic assessment 12 patients had trivial or no gradient across the right ventricular outflow tract, while one patient had a gradient of 25mmHg. The "Rastelli" operation can be undertaken with low mortality and morbidity and early results are encouraging.

Blood Vessel Prosthesis↗
PubMed1994

[Hemitruncus arteriosus in an asymptomatic young man--diagnostic value of transesophageal echocardiography].

The aortic origin of one pulmonary artery, also called hemitruncus arteriosus, is a rare congenital anomaly with poor prognosis. In this report the case of an asymptomatic 18-year-old man with a systolic precordial murmur and the electrocardiographic finding of biventricular hypertrophy is described. The diagnosis of hemitruncus was confirmed by cardiac catheterization. Echocardiography showed biventricular hypertrophy and moderate enlargement of the right atrium and ventricle. In contrast to reports in children, the diagnosis could not be verified by transthoracic echocardiography because of the lack of a satisfactory acoustic window. On the other hand, the aortic origin of the right pulmonary artery and the continuation of the main pulmonary artery as left pulmonary artery were visualized by transesophageal echocardiography. However, the shunt-volume could not be determined by means of the velocity-time-integral and the diameter of the right pulmonary artery because of the turbulent blood-flow in its proximal part.

Adolescent↗
PubMed1994

[Surgical repair of common truncus arteriosus with homograft. Short- and mid-term results].

Between July 1987 and July 1993, 25 consecutive children with truncus arteriosus underwent complete surgical correction with homografts. Nineteen were under 6 months of age and 4 had an associated interruption of the aortic arch. Ten pulmonary and fifteen aortic homografts were implanted. They were cryopreserved with the exception of two, fresh aortic homografts. The diameters of the homografts varied from 8 to 19 mms. Two children who had an interruption of the aortic arch, aged 24 and 31 days, died in the peroperative period. The postoperative course was uncomplicated in only 3 cases; 20 patients had complications, including 9 cardiogenic shocks and 8 pulmonary hypertensive crises. The medium-term results included one death 4 months after surgery in an infant with an interruption of the aortic arch, and 2 successful homograft replacements 3 and 12 months after the initial repair for mycotic infection in 1 case and valvular stenosis in the other. The mean follow-up of the other 20 patients was 23 months: 14 were in NYHA functional Class I, 4 are on the waiting list for replacement of their homograft for obstruction after an average period of 47 months. The authors strategy is to perform surgical correction of truncus arteriosus very early: immediately in the neonatal period in cases with uncontrollable cardiac failure, at 6 months at the latest. Small homografts make this aggressive attitude feasible, but with an operative mortality in the first 6 months of life of 10.5% (2/19).(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve↗
PubMed1995

[A case report of the radical correction of a truncus arteriosus and peripheral pulmonary stenosis in association with Holt-Oram syndrome].

A one year and 6 months old female infant was admitted to our department for a radical correction of a truncus arteriosus (Collett-Edwards type II). She also had a peripheral pulmonary stenosis and Holt-Oram syndrome. Under a cardiopulmonary bypass with deep hypothermia, PFO was closed directly. First of all, the pulmonary arteries were dissected from the aorta, the defect of the aorta was closed directly and VSD was closed with Dacron fabric patch. The reconstruction of the pulmonary arteries were performed with valved conduit which consisted of Dacron graft and 14 mm Björk-Shily valve. The post-operative course was uneventful. Cardiac catheterization study, done 77 days after surgery, revealed 44 mmHg of pressure gradient between RV outflow tract and right PA. The patient was doing well and two years have passed since the surgery. She should be observed carefully at the out-patient clinic due to the mechanical valve and the presence of pressure gradient at RV outflow tract.

Blood Vessel Prosthesis↗
PubMed1994

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.

Abnormalities, Multiple↗
PubMed1994

[Intracardiac repair of truncus arteriosus with severe truncal valve regurgitation in neonate].

The majority of infants born with truncus arteriosus associated with truncal valve regurgitation are seen within the first week of life with the manifestation of severe congestive heart failure. The patient reported herein was a 6-day-old male with severe congestive heart failure due to massive truncal valve regurgitation. Rastelli operation with simultaneous truncal valve plasty was undertaken. The truncal valve consisted of four cusps. One left cusp was small and prolapsed. The valvuloannuloplasty was performed to the small cusp. The patient was discharged and doing well at six months postoperatively, though mild degree of the truncal valve regurgitation was recognized. We believe that the plasty of truncal valve is an effective surgical procedure for the correction of sever truncal valve regurgitation in neonate.

Heart Failure↗
PubMed1994

[Autologous pulmonary arterial flap for repair of truncus arteriosus in neonate and small infants].

Complete correction without external conduit repair by autologous pulmonary arterial flap was performed for the truncus arteriosus in one neonate and two small infants. In this study, we investigated advantages of postoperative hemodynamics and pulmonary artery growth from the data on truncus arteriosus repaired by the autologous flap technique. Patient in this study was classified as type I or II truncus arteriosus and age ranged from 19 days to 95 days after birth. One of the patients died of unexpected gastric bleeding 25 days after surgery. However, all showed excellent hemodynamics in the early postoperative period. The reconstructed pulmonary tract positioned rather left ward and posterior to the level of the systemic root than the technique with extra cardiac conduit repair. Compression did not occur at the created pulmonary tract and hemodynamics was fairly stable after closure of the chest. The postoperative RVP/LVP ratio ranged among 0.45 to 0.65 and the pressure gradient across the right ventricular out flow tract demonstrated only 10-15 mmHg. The size of the pulmonary arterial flap was sufficient even in type II truncus arteriosus to create right ventricle-to-pulmonary tract. However, the first patient underwent subsequent surgery 20 months after the initial surgery, because of left pulmonary artery stenosis due to intimal tissue overgrowth distal to the outflow patch. Surgical findings revealed that the posterior wall of the pulmonary tract was smooth and well developed. Traction of pulmonary arterial flap toward the right ventricular outflow tract shortens the distance between monocusp and left pulmonary arterial orifice than expected.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans↗
PubMed1994

[Recurrence of common truncus arteriosus. Prenatal diagnosis of a case report].

We report a case of isolated truncus arteriosis diagnosed prenatally which recurred during a subsequent pregnancy. This observation would suggest an increased risk of recurrent single trunk malformation as compared with other congenital heart diseases, in agreement with our understanding of the genetic processes involved. A prenatal screening can be achieved with a systematic examination of the fetal morphology. Prognosis is severe and prenatal diagnosis is difficult.

Abortion, Therapeutic↗
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↗