Chest X-ray quiz. Transposition of the great vessels.
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We present a new approach for anatomic correction of transposition of the great arteries. The two coronary arteries, with a piece of the aortic wall attached, are transposed to the posterior artery. The two aortic openings are closed with a patch. The aorta and pulmonary artery are transected, contraposed, ant then anastomosed. The interventricular septal defect is closed with a patch, through a right ventriculotomy approach, because the right ventricle is no longer part of the systemic circulation. Two patients, aged 3 months and 40 days weighing 4,200 and 3,700 grams, respectively, were operated upon with deep hypothermia and total circulatory arrest. There was good recovery from the operation, with normal cardiocirculatory conditions. Renal failure developed in the first patient, and she died on the third postoperative day. During this time the cardiocirculatory conditions were good. The second patient made an uneventful recovery. Hemodynamic studies 20 days after the operation showed complete correction of the malformation. Five and one-half months after the operation, he weighs 7,500 grams, and his development is very good. We believe that this operation will be reproducible by most cardiovascular septal defect and pulmonary hypertension.
His bundle electrogram was recorded in a young adult male with corrected transposition of the great vessels and single ventricle. Prolonged PA and AH intervals, wide diphasic His spike, and normal HV intervals were found. These findings are suggestive of the diffuse nature of conduction delay in this congenital lesion involving intra-atrial, AV nodal, and intra-His regions.
Echocardiography can provide important diagnostic information that identifies the transposition complexes, evaluates relative pressure levels in the pulmonic and systemic circuits, describes subpulmonic obstruction, and identifies some associated congenital anomalies. Following the Mustard procedure, baffle dysfunction may be recognized, and the function of the procine heterograft used in the Rastelli procedure studied. Echocardiography can also demonstrate other congenital cardiac defects that may mimic transposition of the great vessels or reveal normal cardiac and great vessel anatomy and function that can reliably rule out the transposition complexes.
Eight of 43 patients with transposition of the great vessels (TGV) and either an intact ventricular septum or very small ventricular septal defect were found to have dynamic left ventricular outflow tract obstruction (LVOTO). The preoperative left ventricle-pulmonary artery (LV-PA) pressure gradient ranged between 14 and 60 mm Hg. In 3 patients the pulmonary artery could not be catheterized. They had a left ventricular pressure of 43, 45, and 62 mm Hg, respectively, no evidence of pulmonary vascular disease, and either echocardiographic or angiocardiographic demonstration of LVOTO. The outflow tract was explored in 3 patients at the time of the Mustard operation. Exposure of the LVOT was difficult, particularly in patients less than 6 months of age. A shallow septal myectomy was performed in 1 patient; a thin fibrous endocardial scar was resected in 2; and no procedure was performed in the third. All 8 patients survived and remain asymptomatic 1 to 11 years (mean, 5 years) after operation. The LV-PA pressure gradient has either remained the same or has increased in every patient except 1 in whom there was a slight reduction in the gradient. We conclude that dynamic LVOTO persists after the Mustard operation and is not amenable to direct surgical relief. However, an excellent clinical result can be obtained for up to 11 years after operation.
Contrast bidimensional echocardiographic (2DE) studies were performed in eight patients with d-transposition of the great vessels in the postoperative period of the Senning technique. Contrast was injected into a peripheral vein of all patients, and into the arterial atrium in five in the postoperative period. The 2DE projections used were the four-chamber apical view and four-chamber subcostal view. In all patients it was possible to see and identify the new atrial cavities which greatly resembled the actual anatomy. Early postoperative alternating injections of contrast in both new atria not only achieved the main purpose of delineating the real anatomy, but also permitted more definitive identification of residual shunts.
The vectorcardiograms (VCGs) of two groups of patients with corrected transposition of the great vessels (CTGV) were studied; the first, group A, included 17 patients with CTGV in "situs solitus,", characterized by leftward orientation of the cardiac apex; the second group, group B, with three patients, presented CTGV in "situs solitus" and apex to the right. All cases had one or more associated defects: ventricular septal defect, atrial septal defect, pulmonic stenosis or tricuspid insufficiency.
The presenting chest radiographs of 27 new-born patients with D-transposition of the great vessels (D-TGV) were evaluated for the degree of pulmonary flow as well as other findings classically described in D-TGV (narrow superior mediastinum, radiographically absent thymus, inapparent main pulmonary artery, non-visualization of the malpositioned aortic arch, asymmetric pulmonary blood flow, and cardiomegaly). Of the 27 patients, 22 (82%) demonstrated normal or decreased flow. The majority of the D-TGV patients also failed to demonstrate any of the other classically described radiographic findings. A normal chest radiograph is the most common presenting scenario in the neonate with D-TGV.
In the period between april 1984 and march 1989, 246 newborn children with transposition of the great vessels benefited from anatomical repair of their heart disease. 221 children had simple transposition with normal interventricular septa. These were aged 2 to 23 days (mean age 7.8 +/- 3.5 standard deviation). 25 had transposition of the great vessels associated with a large interventricular septum defect. These were aged 7 to 30 days (mean age 18 +/- 8.3 S.D.). Associated lesions included a small interventricular septum defect in 20 cases which was not treated at the time of the operation; tricuspid insufficiency in 3 cases; and isthmic coarctation of the aorta in 10 cases. According to Yacoub's classification relative to the anatomical distribution of the coronary arteries, all the children fell into the following groups: type A, 172 cases; type B, 8 cases; type C, 12 cases; type D, 40 cases; type E, 14 cases. Preoperative catheterization was carried out in all the children. 96% benefited from Rashkin's atrioseptostomy, 90% had a prostaglandin infusion continuing through surgery. The surgical technique included the use of continuous extracorporal circulation at 20 degrees C, without circulatory arrest. Protection of the myocardium was ensured during aortic clamping by injecting of a crystalloid cardioplegia-inducing solution. The main steps of surgical repair were repositioning of the coronary arteries onto the ancient aortic stump and reconstruction of a pulmonary shunt. Repositioning of the coronary arteries was carried out in accordance with Yacoub's technique in all cases except for the 10 first patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Coronary artery obstruction is the main late complication of the so-called arterial switch operation designed to repair transpositions of the great arteries in newborn infants by switching the great vessels and transferring the coronary ostia onto the posterior vessel. Our aim was to study the links between myocardial perfusion and coronary artery anatomy after the arterial switch operation. Forty-five patients (5.863 years) underwent a 201Tl myocardial SPECT and a selective coronary artery angiography. The latter was normal in 20 children: 13 had also a normal myocardial scan but 7 had myocardial perfusion defects including 2 with angina who had a very low coronary reserve at positron emission tomography. Twenty-five patients had severe coronary artery lesions: 5 with a normal myocardial scan and 20 with perfusion defects. Twelve out of these 20 underwent surgical revascularization and the SPECT images went back to normal in all within 6 months after surgery. Specificity and sensitivity of myocardial SPECT in detecting coronary artery lesions were 78% and 69% whereas positive and negative predictive values were 74 and 73%. We conclude that myocardial SPECT imaging is not the right way to detect late post arterial switch coronary artery lesions. It is helpful in decision making as to submit these children to surgical revascularization and in assessing its postoperative effectiveness.
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A case of native valve endocarditis caused by Enterococcus durans in a patient with transposition of the great vessels is reported. The patient was treated initially with gentamicin and ceftriaxone; after isolation of enterococci, ceftriaxone was switched to ampicillin. The only virulence factors established in the strain were haemolytic activity and biofilm formation.
Heart block has been a frequent complication of ventricular septal defect closure in L-transposition of the great vessels. Intraoperative mapping of the conduction tissue combined with accurate suture placement should significantly reduce the incidence of heart block produced by direct injury to the conduction tissue.
Congenitally corrected transposition of the great arteries is a rare defect characterized by discordant atrioventricular and ventriculoarterial connections. Symptoms result from one or a combination of associated cardiovascular malformations, including ventricular septal defect, pulmonary stenosis or atresia, tricuspid valve dysfunction, dextrocardia, hypoplastic left or right ventricle. Correcting exclusively the associated defects, leaving the morphologic right ventricle in systemic position, will determine the patient's life-long prognosis. Anatomic repair by double switch technique may improve survival of patients with congenitally corrected transposition of the great arteries by establishing the morphologic left ventricle in the systemic circulation. A 3-year-old girl with congenitally corrected transposition of the great arteries, ventricular septal defect, hypoplastic right ventricle, and previous palliative procedure was corrected by double switch technique and patch closure of ventricular septal defect. The authors prefer the double switch procedure inspite of its many surgical challenges because it has very good long-term outcome. The more simple surgery namely the operation of only associated cardiac defects will involve the possibility of deterioration of right ventricular function.