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At least 577 records · Page 32Linked to original sources
PubMed1977

Reconstruction of the right ventricular outflow tract with a conduit in congenital heart disease.

A series of five patients with complex cyanotic congenital cardiac malformations underwent surgical reconstruction of the right ventricular outflow tract using a Dacron conduit with a porcine aortic valve. All patients survived and all have shown clinical improvement. At cardiac catheterization postoperatively, a pressure gradient of between 20 and 50 mm Hg across the conduit was found in all patients. This surgical approach to patients necessitating reconstruction of the right ventricular outflow tract is effective and appears to have the best long-term results.

Adolescent↗
PubMed1992

The role of coronary artery abnormalities in the prognosis of truncus arteriosus.

A high incidence of coronary ostial and arterial abnormalities was found in a study of 30 pathologic specimens of classic truncus arteriosus at Children's Hospital of Pittsburgh. The following were of special note: (1) left coronary ostium in a posterior and high position; (2) close relation of the left coronary ostium to the pulmonary artery segment in three-leaflet truncal valves; (3) stenosis of the coronary ostium caused by small size, slitlike shape, or the location of the ostium above or in a commissure; (4) the acute angle takeoff of the coronary artery; (5) the position of the left anterior descending artery as it courses posteriorly and close to the truncal wall, and then to the left of the interventricular septum; (6) the size and course of the conal and diagonal arteries from the right coronary artery across the right ventricular outflow area; (7) other coronary abnormalities, including a single coronary artery or ostium with branches crossing the right ventricle below the truncus, the circumflex arising from the right coronary artery and coursing behind the truncus, and the right coronary artery originating from the left anterior descending artery and vice versa. Eight heart specimens with conduit repair were reviewed, and all had injury to coronary arteries, possibly responsible for or contributing to the deaths of six of the eight patients. Coronary abnormalities, often several occurring in combination, may contribute to high operative mortality rate and may be a cause of late sudden death in truncus arteriosus. Surgical procedures should be planned with a view to protecting coronary arteries in the region of the right ventricular outflow tract below the truncus. Coronary artery obstruction (ostial or luminal) can occur and may need to be addressed as a separate issue during surgical procedures.

Abnormalities, Multiple↗
PubMed1992

[Suspected SIDS diagnosis--van Praagh I truncus communis as a nonfatal incidental finding].

Autopsy of a baby suspicious to SIDS. Most interesting postmortem finding was a congenital malformation of the great vessels, a truncus arteriosus communis typus van Praagh A I. That one is very rare with a clinically frequency of only 1.7%. The malformation was not cause of death and was statistically survived for 2 months. The baby died of aspiration resultant from an incarcerated inguinal hernia.

Asphyxia↗
PubMedDate not supplied

Tricuspid atresia associated with common arterial trunk and 22q11 chromosome deletion.

The case of a four-months old male with coexistent tricuspid atresia and common arterial trunk is presented. The diagnosis was made by cardiac catheterization and selective angiocardiography. Clinical considerations are discussed and the review of the available literature reveals this patient to be the tenth case reported of this very unusual association of cardiovascular defects, and the first with positive deletion of the 22q11 chromosome.

Abnormalities, Multiple↗
PubMed2004

[Results of the surgery of the truncus arteriosus: present implications].

We report our results of truncus arteriosus surgical reparation in a period of 10 years. From January 1993 to November 2003, 17 patients were operated with a median age and weight of 66 days and 3.5 kg respectively. According to the Van Praagh classification there were 13 cases of type A1, 2 cases of type A2 and 2 cases of A4. The connection between the right ventricle and the pulmonary artery was performed by a homograft (n = 12), a Contegra tube (n = 1) or a Barbero-Marcial intervention (n = 4). In one case, a plasty of the truncus was performed. Patients were classified in two groups: group A for those operated between 1993 and 1997 (n = 8) and group B for those operated after 1997 (n = 9). Five patients died (29%) in the post-operative period, 4 in the group A (50%) and one in group B (11%). The mean duration of intensive care stay was 12.2 (+ 7.4) days. Statistical analysis did not reveal any difference between both groups, especially concerning post-operative treatment or prognosis. In the multivariate analysis, an age below 30 days was a risk factor of post-operative death (OR = 16.5, 95% CI = 1.09 - 250; p = 0.043). After a mean follow-up of 3.9 (+ 3.5) years, 2 patients required a redo intervention for replacement of the pulmonary artery homograft. All 12 suvivors are asymptomatic without any pulmonary hypertension. In conclusion, the results of surgical reparation of the truncus arteriosus seem to improve with experience. According to recent progresses in surgery and intensive care, the intervention can be scheduled beyond the neonatal period without additive risk and with potentially less consecutive redo interventions.

Female↗
PubMed1992

Pain upon eye movement following digoxin absorption.

A patient who had received digoxin for 6 months experienced glare and ocular pain induced by eye movement. The symptoms lasted for 2 days and disappeared within hours after the treatment was discontinued. Four months later, digoxin was readministered. After 2 days, the symptoms reappeared but disappeared again within hours of stopping treatment. The origin of the pain is uncertain, although mechanisms related to optic nerve or ocular muscle involvement should be considered. Digoxin toxicity should be included in the differential diagnosis of pain evoked by ocular movements.

Adult↗
PubMedDate not supplied

DiGeorge syndrome with truncus arteriosus: report of one case.

DiGeorge syndrome is a rare disorder characterized by a spectrum of thymic and parathyroid gland abnormalities, conotruncal cardiac defects, and typical facial dysmorphism. We report a male infant with partial DiGeorge syndrome characterized by truncus arteriosus, typical facial dysmorphism, hypocalcemia, lymphocytopenia with T-cell deficiency, and chromosome 22q11.2 deletion. Transient lymphocytopenia was noted for 5 days after birth and hypocalcemia was corrected with calcium gluconate administration. Surgical correction of the truncus arteriosus was performed at the age of 3 months. Unfortunately, the patient subsequently had an unwitnessed cardiac arrest, and despite resuscitation, died at the age of 4 months.

Chromosome Deletion↗
PubMedDate not supplied

Bilateral percutaneous stent implantation in the pulmonary vasculature: a modality now available in Lebanon.

We report a child with Truncus Arteriosus who developed bilateral proximal branch pulmonary stenosis, following total surgical repair of her condition with construction of the right ventricular outflow tract utilizing a Contegra conduit (Medtronic Inc., Minneapolis, Minn.). The obstruction was relieved completely utilizing bilateral percutaneous stent implantation. To the best of our knowledge, this is the first reported case of bilateral stent implantation to relieve branch pulmonary artery stenosis in Lebanon. This technique could be applied to similar cases of obstruction in the pulmonary tree.

Child, Preschool↗
PubMed2005

[22q11 deletion in conotruncal anomalies].

22q11 deletions in conotruncal anomalies. Two children are reported in whom conotruncal anomalies (truncus arteriosus communis, tetralogy of Fallot) were associated with chromosome 22q11 deletion. In both cases, which represent the first published cases in Hungary according to the knowledge of the author, deletions were suspected on the basis of phenotype termed Di George syndrome. Nowadays the role of molecular genetics is growing in the etiology of congenital heart defects and the chromosome 22q11 deletions constitute one of the most frequent genetic mutations associated with congenital heart defects. The author emphasizes that clinicians must know about this disease, and in case of suspicion they should request for molecular genetic investigation. 22q11 deletions have an important roles in the prognosis of congenital heart disease, in counseling and in the prenatal diagnosis as well.

Chromosome Deletion↗
PubMed1992

[Total correction of truncus arteriosus with severe truncal valve insufficiency in neonate].

The prognosis of infants with truncus arteriosus associated with severe truncal valve insufficiency is quite poor. Total correction was successfully performed in a neonate with such a complicated anomaly. The patient was 21 days old female with anuria due to severe congestive heart failure preoperatively in spite of medical treatment. She underwent Rastelli operation and pulmonary artery was reconstructed using autologous pericardial 3 valved conduit. Truncal valve was 4 cusps with the malformed nodular margins and one cusp had cleft. This cleft was closed suturing the cleft cusp and adjacent cusp each other and annuloplasty was added in 4 commissures. She survived and her truncal valve insufficiency was still mild at 2 years post-operative period. Although total correction with truncal valve repair for such a severely ill neonate and young infant with truncus arteriosus as this patient has not been reported, our experience suggests that severe truncal valve insufficiency could be fairly well repaired by valvulo-annuloplasty.

Cardiac Surgical Procedures↗
PubMed1992

[Prenatal diagnosis of truncus arteriosus communis (type I) in diabetic pregnancy].

Recently Ferencz et al. [6] using the datas of the Baltimore-Washington Infant Study found a predominance of double outlet righ ventricle and truncus arteriosus communis among the congenital heart diseases of newborns of diabetic mothers. This paper reports about the prenatal diagnosis of a truncus arteriosus communis Type I in a 23 year old pregnant with insulin-dependent diabetes mellitus (White B). The diagnosis was performed in the 25th week using Real-Time- and Color-Doppler-sonography. The important features in the prenatal differentialdiagnosis from a tetralogy of Fallot are explained. The optimal management of pregnancy, the delivery at a perinatal center with subsequent operative correcture could improve the prognosis of the newborn.

Diabetes Mellitus, Type 1↗
PubMed1991

[Criteria of the operability of patients with a common arterial trunk based on hemodynamic data].

Criteria of operability of patients with a common arterial trunk were evaluated from a complex of hemodynamic, clinical, and morphological data. The patients were divided into 2 main groups according to the value of the pulmonary blood flow (PBF): group 1 with the pulmonary to systemic blood flow ratio (PBF/SBF) greater than 1; group 2 with PBF/SBF less than 1. The indications for operation in group 1 depended on the degree of PBF increase. For instance, an operation was indicated for patients with marked increase of PBF (PBF/SBF greater than 2); in cases with moderate PBF growth [formula: see text] the indications for operation were determined only after additional lung biopsy. The operation was not indicated for group 2 patients (PBF/SBF less than 1.0) because of the severity of their condition due to obstructive pulmonary vascular disease.

Adolescent↗
PubMed1991

[Immediate and mid-term results of complete repair of truncus arteriosus during the first year of life].

Twenty five infants with truncus arteriosus underwent complete surgical correction in the first year of life between January 1984 and June 1990 at Marie Lannelongue Hospital. All had cardiac failure and pulmonary hypertension. Another severe cardiac malformation was present in 6 cases. Complete repair was carried out under cardiopulmonary bypass with moderate hypothermia. After closing the ventricular septal defect the continuity of the right ventricle and pulmonary artery was reestablished by a valved Dacron conduit with a bioprosthesis (13 patients), by an autologous pericardial conduit with the same type of prosthesis (5 patients), by a valveless conduit (1 patient) or by direct insertion of the pulmonary artery (6 patients). Eight children (32%) died shortly after surgery. Seventy one per cent of children operated in the first month of life died compared with only 17% of those operated after one month of life (p less than 0.05). The seventeen survivors have been followed up for an average of 21 +/- 22 months. Three secondary deaths were observed at 33 days, 2 and 10 months after surgery: the first child died of left ventricular failure and pulmonary vascular disease related to the complexity of the associated cardiac malformations; the other 2 deaths were unexpected. The one and three year survival rate is 54%. Pulmonary stenosis with a systolic pressure gradient of more than 30 mmHg was found in 7 patients of whom 6 had valved Dacron conduits (p less than 0.01). One child was successfully operated 60 months after the total correction and another child is on the waiting list for reoperation 69 months after the total correction.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗