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[Corrected transposition of the great vessels. A clinical review of 21 cases (author's transl)].

Clinical and instrumental features of 21 cases of congenitally corrected transposition of the great vessels are reviewed. We confirm the rarity of the isolated form of the disease and its frequent association with ventricular septal defect and pulmonary stenosis. Electrocardiographically, the inversion of the first vectors is represented more frequently by the absence of the q wave on the left precordial leads than by the presence of the q wave on the right precordial leads; a very frequent electrocardiographic feature is a positive T wave on the right precordial leads. The angiographic image of the two ventricles in the lateral projection where they appear as crossing each other in their upper part were described. We confirm the poor results of the surgical treatment of the disease and the non-benign prognosis, because of the other associated cardiac anomalies.

Adolescent↗

Congenitally corrected transposition of the great vessels: localization of the site of complete atrioventricular block using his bundle electrograms.

Complete atrioventricular block proximal to the bundle of His in a patient with congenitally corrected transposition of the great vessels was documented using His bundle electrograms. The spontaneous rhythnm probably originated from the bundle of His and was responsive to carotid sinus massage, atropine and isometric and treadmill exercise. These electrophysiologic observations are consistent with recent anatomic studies of congenitally corrected transposition of the great vessels.

Adult↗

[Current results of the treatment of transposition of the great vessels. Apropos of a series of 168 cases including 138 physiologic corrections].

This study takes stock of the current results of physiological correction (Mustard's or Senning's operation) in simple transposition of the great vessels, at a time when anatomical corrections, or detranspositions, are developing. Between January 1, 1974 and December 31, 1984, 168 neonates with simple transposition of the great vessels were operated upon and followed up for a mean period of 3.67 years (up to 11 years and 10 months). Thirty of them died before correction (12.7% mortality rate with palliative surgery) and 15 immediately after corrective surgery (11.2%). Among the 111 children who survived corrective surgery, the results were satisfactory in 62.7%, fair in 17.8% and poor in 10.2%; the late mortality rate was 9.3%. Post-correction morbidity mainly consisted of mechanical complications suspected in one-third of the patients (with 3 consecutive deaths) and heart rhythm disorders (50% of patients in this series had abnormal Holter recordings), with predominance of atrial rhythm disorders (regression of sinus rhythm was 3.82% per annum). This, after correction the survival curve underwent an actuarial regression of 1.31% per annum. This primary and secondary morbidity and mortality justifies a switch to anatomical corrections the results of which remain to be fully evaluated.

Arrhythmias, Cardiac↗

[Caudal-angle aortography. Its usefulness for defining the coronary anatomy in cases of transposition of the great vessels].

With the purpose of obtaining an accurate definition of coronary arteries anatomy in complete transposition of the great arteries (concordant atrio-ventricular connection and discordant ventriculo-arterial connection), we performed a new angiographic technique in 14 patients with d-transposition of the great arteries and 1 patient with double-outlet right ventricle. This technique consists of using a balloon catheter with the frontal X ray tube cine-angiographic camera in caudal angulation (about 45 degrees), with balloon occlusion of the ascending aorta during the injection of the contrast medium. The results were compared with echocardiograms and surgical results. In all our cases, except 2, the technique gave us a very clear definition of the coronary anatomy and we were able to define the origin and course of the coronary arteries without difficulty. In the most common coronary artery pattern, the left coronary artery arose from sinus #1 (left aortic sinus) and gave origin to the anterior descending and left circumflex arteries, while the right coronary artery arose from aortic sinus #2 (right aortic sinus). In our series, the coronary arteries always originated from one or both facing sinuses of the aorta and the angiographic correlation with the surgical reports was accurate in all the cases. The anatomical and angiographic findings of this series are discussed. In conclusion, we find the aortogram with caudal angulation a very useful technique to improve the visualization of coronary anatomy in d-transposition of the great arteries, which has surgical significance in those cases which are candidates for anatomical correction.

Aortography↗

[Univentricular heart; angiocardiographic study].

Authors present angiocardiographic study of 19 patients with univentricular heart. It was realized during the neonatal period in 13 cases and between the six first six months in the rest. To determine the existence of an only ventricle contrast was injected in the right and left atrium and the ventricle through the right and left atrioventricular valve. The bulbo-ventricular foramen was orientated in all cases from back-front wards. Those of left ventricle type had a right anterior, anterior-superior or left-anterior rudimentary chamber. Depending on its' morphology they were classified in three types: left, right or indeterminate. In these series fourteen patients had left ventricle morphology. All cases had a rudimentary chamber: 10 with transposition great vessel and four with normal related great vessels. Six presented normal situs (five with levocardia , and eight ambiguous situs (six with asplenia, four with dextrocardia). In nine atrio ventricular valves were anomalous. In the indeterminate type, two had a normal situs, two with polysplenia and two asplenia. Cardiac apex was oriented to the right in two (one asplenia). The two patients with polysplenia and one with normal situs and single atrium had a common ventricular valve. Four had an anterior aorta and three pulmonary obstruction.

Abnormalities, Multiple↗

The changing strategies in operation for transposition of the great vessels.

Between July 3, 1985, and February 24, 1994, a total of 55 infants underwent arterial switch procedures for the repair of transposition of the great vessels. Thirty-five infants had an intact ventricular septum and 20 had ventricular septal defects. To date, there have been three late deaths, one in the group with an intact ventricular septum and two in the group with a ventricular septal defect. Early postoperative complications included atrial dysrhythmias, prolonged ventilation, inability to close the sternum, and tension on the coronary arteries. Follow-up echocardiographic data for 44 patients indicate that pulmonary artery gradients are a worrisome postoperative problem, especially in infants who have ventricular septal defects.

Child, Preschool↗

Stenting of superior vena cava and inferior vena cava for symptomatic narrowing after repeated atrial surgery for D-transposition of the great vessels.

Double venous stenting of the superior vena cava and inferior vena cava was successfully performed after failed balloon angioplasty in a young woman who had venoatrial narrowing and kinking after repeated Mustard procedure for D-transposition of great vessels. Self-expanding metallic stents were used. Angiography showed that the first stent was fully patient after six months when a second stent was implanted. This operation was followed by sustained clinical improvement. The present report shows the feasibility and success of stenting of the great veins when postoperative narrowing or kinking is unresponsive to balloon angioplasty alone.

Adolescent↗

Scintigraphic imaging of a case of congenitally corrected transposition of the great vessels and an adult case of single atrium and single ventricle.

We report on the clinical utility of radionuclide angiography and gated blood pool single emission computed tomography (gated blood pool SPECT) in two patients having congenital heart disease. Both conventional equilibrium radionuclide angiography and gated blood pool SPECT demonstrated the connection of the great vessels with both ventricles in a 15-year-old male patient with a congenitally corrected transposition of the great vessels. In particular, the latter procedure could provide very useful information about the ventricular morphology and inversion which is important for diagnosing this disorder. The second case is an extremely rare 42-year-old female patient with a single atrium and single ventricle. She underwent first-pass and multiple gated blood pool angiography from the anterior, right and left oblique views. The combination of these scintigraphic techniques revealed an insufficiency in anatomical correlations among the single atrium, atrioventricular valve, single ventricle and the great vessels in addition to the connection of superior vena cava with the single atrium, and the atrioventricular valve. Thus, conventional equilibrated angiography from multiple views and gated blood pool SPECT seems to be very reliable not only for anatomical evaluation but also for clinical course observation in patients with complicated congenital heart disease.

Abnormalities, Multiple↗