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Biomedical subjects

J M Quaegebeur

Publications and source records attributed to J M Quaegebeur.

64 records · Page 4Linked to original sources

Nine years' experience of physiological correction of tricuspid atresia: long-term results and current surgical approach.

Fontan's correction has been performed without early death in 24 consecutive patients with tricuspid atresia. Eighteen patients had ventriculoarterial concordance (group I) and six discordance (group II). Late death related to infections occurred in three patients. The follow-up time in group I (16 survivors) ranges from 1 year 10 months to 7 years 5 months (mean 3 years 8 months) and in group II (five survivors) from 2 years 1 month to 5 years 6 months (mean 3 years 6 months). Early postoperative cardiac catheterisation showed arterial pulsatile pressure recordings in the pulmonary artery only in two patients in group I, in whom a valved conduit was used to connect the right atrial appendage with the outlet chamber. Patients with a valved conduit connected with either the pulmonary artery (group II) or the outlet chamber (two patients of group I) showed better preservation of right atrial contractions angiocardiographically and scored better in exercise tests than did patients with a non-valved conduit (14 patients of group I). In the latter patients evidence of regurgitant bloodflow from the outlet chamber into the right atrium was found. Reoperation was necessary in two patients. The outlet chambers showed a substantial increase in diameter after operation. No disturbances of kidney or liver function were found up to seven years after operation. The clinical condition has improved considerably in all survivors. It is concluded that the use of a valved conduit is preferable for connecting the pulmonary artery directly in cases of tricuspid atresia with ventriculoarterial discordance or the outlet chamber when there is ventriculoarterial concordance.

Adolescent↗

The trousers-shaped baffle for use in the Mustard operation.

The trousers-shaped baffle has been found very useful in intraatrial correction of transposition of the great arteries. Reasons are given to explain the particular form of the patch, and details of the operative technique are described.

Child, Preschool↗

Takedown and reconstruction of cavopulmonary anastomosis.

Takedown and reconstruction of a previous Glenn anastomosis at the time of repair was performed in 1 child with pulmonary atresia and ventricular septal defect and in 3 children with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. All 4 survived and showed excellent clinical results. The surgical technique and postoperative hemodynamic and lung scintigraphic data are presented.

Adolescent↗

Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: case report and review of the literature.

The pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect is highly variable. Several cases have been reported in the literature in which a coronary artery to pulmonary artery fistula, alone or in combination with additional vessels, supplies the pulmonary circulation. We report a case of successful repair during early infancy, with unique hemodynamic, clinical, and anatomic features. The literature is reviewed.

Arterio-Arterial Fistula↗

Expression of adenylyl cyclase V/VI mRNA and protein is upregulated in cyanotic infant human myocardium.

We have previously demonstrated that both basal and isoproterenol-stimulated activities of myocardial adenylyl cyclase were greater in cyanotic patients with tetralogy of Fallet (TOF) than those in acyanotic patients. However, it was not determined whether increased enzyme activity was related to a similar increase in adenylyl cyclase protein and mRNA expression. In the current study, we examined the mRNA and protein expression of cardiac adenylyl cyclase, types V and VI, in cyanotic and acyanotic patients with TOF. Ribonuclease protection assays and immunoblotting were performed on myocardial specimens obtained from cyanotic patients with TOF and acyanotic patients with TOF or ventricular septal defect. We demonstrated that in both cyanotic and acyanotic patients, there was more type V adenylyl cyclase mRNA than type VI. Types V and VI cardiac adenylyl cyclase mRNA were significantly increased in myocardium of the cyanotic group compared to the acyanotic group. Protein expression of both V and VI adenylyl cyclases was correspondingly upregulated in cyanotic patients compared to acyanotic patients. Our results indicate that gene and protein expression of cardiac adenylyl cyclases, types V and VI, is increased in the cyanotic myocardium. These results suggest that chronic hypoxemia may regulate the expression of adenylyl cyclase enzymes.

Adenylyl Cyclases↗

Double-outlet right ventricle (tetralogy of Fallot type) associated with anomalous origin of the left coronary artery from the right pulmonary artery: report of successful total repair in a 2-month-Old infant.

The case reported herein demonstrates the rare association of double-outlet right ventricle (tetralogy of Fallot type) with anomalous origin of the left coronary artery from the pulmonary artery. It is the first reported successful total surgical repair in an infant.

Aorta, Thoracic↗

Left ventricular assist device options in pediatric patients.

Left ventricular assist devices can be successfully used in the pediatric population if currently available devices are used appropriately. To highlight their management strategies, the authors' experience with pediatric left ventricular assist devices is reviewed, with case studies documenting options for device use. A retrospective study was conducted for all patients supported at the authors' institutions. Nine pediatric patients received devices during this time, with ages ranging from 12 days to 15 years. There was considerable size differences in our patients, with weights ranging between 2.5 and 73 kg. Either the Abiomed BVS 5000 (Abiomed Cardiovascular, Inc, Danvers, MA) or the Biomedicus centrifugal pump (Bio-Medicus, Inc, Eden Prairie, MN) were used for all patients. Patients were supported for an average of 5 days (range 1-11 days). Eight patients were successfully weaned from the device, with four undergoing transplant. Four patients were discharged and are long-term survivors, and one is still recovering post transplant. Neurologic sequelae were the most common complications in these patients, with four patients having events. Pediatric ventricular assist devices are limited because of size considerations, although current devices can be successfully used for bridging to recovery or transplantation. New devices are needed to meet the flow and size characteristics for pediatric patients. Successful use of assist devices, as has been recognized in adults, is possible in pediatric patients if these requirements are met.

Adolescent↗

The need for follow-up after surgical correction of anomalous left coronary artery arising from the pulmonary artery.

Four patients are described with an anomalous origin of the left coronary artery from the pulmonary artery. Three were treated by ligation of the abnormal left coronary artery at its anomalous origin; one of them died during surgery. One patient was treated with reimplantation of the left coronary artery into the aorta. Of the survivors 2 are in normal health, but 1 developed aortic valve incompetence after reimplantation of the left coronary artery into the aorta and 1 had persistent left ventricular wall motion abnormalities and developed mitral valve prolapse. The third survivor has impaired health with diffuse left ventricular wall motion abnormality. Careful follow-up after surgery for an anomalous left coronary artery arising from the pulmonary artery is necessary for secondary prevention of problems due to incomplete recovery of myocardium or problems as a consequence of surgical therapy.

Adolescent↗