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PubMed1992

The influence of coronary anatomy on the arterial switch operation in neonates.

To determine whether coronary anatomy influences the outcome of the neonatal arterial switch operation, we examined the results in all newborn infants (n = 70) with D-transposition of the great arteries who had a corrective operation at our institution between March 1987 and April 1991. The origin and distribution of coronary arteries were identified preoperatively by echocardiography, aortic root angiography, or selective coronary arteriography and intraoperatively by direct inspection. However, the arterial switch operation was performed independent of the coronary anatomy in all but two candidates for the operation. Four early deaths occurred and five surviving patients had symptoms of impaired cardiac function. No late deaths have occurred in patients followed up for 2 to 50 months. Evidence of myocardial ischemia was present in three of the four deaths and in four of the five patients with cardiovascular symptoms. Patients with commissural or intramural coronary origins between the great arteries had significantly greater cardiovascular morbidity and mortality because of coronary ischemia than patients with the most common coronary pattern. Thus coronary anatomy may influence surgical management and the postoperative course of newborn infants with transposition.

Age Factors↗
PubMed2005

Left and right ventriculo-arterial coupling in a patient with congenitally corrected transposition.

The single beat method was used to evaluate right and left ventriculo-arterial coupling in an asymptomatic patient with congenitally corrected transposition. The ratio of ventricular end-systolic to arterial elastances was normal for the left ventricle coupled to the pulmonary circulation, and depressed for the right ventricle coupled to the systemic circulation. This result suggests that chronic uncoupling of the right ventricle to the systemic circulation might play a role in the pathophysiology produced by congenitally corrected transposition.

Adolescent↗
PubMed1978

Aortic levopositions without ventricular inversion.

A multi-center series of aortic levoposition (aorta anterior and to the left of the pulmonary artery) without ventricular inversion is presented and analyzed from an anatomical viewpoint. All the cases are exceptions to the 'loop rule' and amount to 0.9% of the total number or cases reviewed. A combined study of our own cases and those reported in the literature is presented. There is a high incidence of anomalies (malrotations, juxtaposition of the atrial appendages, atrioventricular valve anomalies, ventricular septal defects, pulmonary outflow tract obstructions and abnormal conal types) which may be related to the primary cause of the aortic levoposition, suggesting that they may form part of a new syndrome of diagnostic and surgical importance. In some cases, absence of coronary sinus was noted without asplenia or left superior vena cava draining into the left atrium. The major coronary artery pattern in cases of antomically corrected malposition with two well-developed ventricles was similar to that seen with ventricular inversion. Most of these cases can be helped surgically provided accurate and detailed preoperative evaluation and operative assessment and treatment is carried out.

Aorta↗
PubMedDate not supplied

Pediatric heart transplantation: surgical considerations for congenital heart diseases.

Congenital anomalies of the atrium, of the pulmonary and systemic venous return, and of the great vessels are sometimes regarded as contraindications to heart transplantation. Among 10 children who underwent heart transplantation in our institution, five, aged 4 to 15 years and weighting 9 to 32 kg, previously operated on, had congenital anomalies. These lesions, encountered singly or in association, were single atrium (2), previous Mustard operation (1), hypoplastic left atrium (1), anomalous systemic venous return (3), anomalous pulmonary venous return (2), transposition or malposition of the great vessels (5), pulmonary artery hypoplasia (1). To enable correction of these lesions and to perform heart transplantation, the donor heart preparation was modified in four ways. The surgical techniques used for correcting these anomalies in this group were atrial septation, atrial enlargement, superior and inferior reroofing, double venous rerouting, septal realignment, full-length mobilization of the great vessels, and pulmonary artery reconstruction. All children survived operation and left the hospital in excellent condition. Follow-up ranged from 6 months to 3 years. All children are totally asymptomatic. As demonstrated by echocardiography, heart catheterization, and angiography, there are no stenoses, no shunts, and there is good atrial size and good orientation of the great vessels. On the basis of our experience, we conclude that with appropriate surgical techniques, most atrial, venous return, and intrapericardial great vessel anomalies are correctable at the time of orthotopic transplantation and that these techniques allow for a successful outcome in children.

Anastomosis, Surgical↗
PubMed1996

MR imaging of conotruncal abnormalities.

MR imaging has been shown to be accurate in the diagnosis and follow-up of congenital abnormalities of the heart and great vessels in infants and children [1-3]. Although echocardiology, in many cases, remains the primary imaging technique in congenital heart disease, MR imaging is a useful adjunct [1-3]. The advantages of MR imaging include a wide field of view and multiplanar capabilities [1-3]. Echocardiology is often suboptimal in evaluating the superior mediastinum because of the narrow imaging window [3]. This fact is especially true in patients after surgery and in older children. Therefore, MR imaging often plays a role in the evaluation of the great vessels.

Adolescent↗
PubMed1994

Coronary echocardiography in 406 patients with d-loop transposition of the great arteries.

OBJECTIVES: The reliability of two-dimensional echocardiography for determining the proximal coronary artery anatomy in d-loop transposition of the great arteries was investigated in 406 infants who underwent surgical repair at one institution. BACKGROUND: The origin and proximal course of the main coronary arteries can affect the surgical results of the arterial switch operation. Preoperative determination of the coronary artery anatomy appears to be advantageous for the surgeon. METHODS: All infants with d-loop transposition who underwent a two-dimensional echocardiogram and primary surgical repair at our institution between 1987 and 1992 were identified, and the echocardiographic, operative and, when available, autopsy reports were reviewed for coronary artery anatomy, presence of a ventricular septal defect and the spatial relation between the arterial roots. The two-dimensional echocardiographic findings were compared with surgical or autopsy findings. The relation between proximal coronary artery anatomy and 1) a ventricular septal defect, and 2) the spatial orientation of the arterial roots was investigated. Twenty-seven infants diagnosed with an intramural coronary artery were not included because they are the subjects of another report. RESULTS: Excluding intramural coronary artery patterns, 10 different types of coronary artery anatomy were seen in these 406 patients. The coronary arteries were imaged adequately in 387 (95%) of the 406 patients. The coronary artery anatomy was determined correctly by two-dimensional echocardiography in 369 (95.4%) of the 387 patients, with 18 errors in diagnosis. During the most recent 2.5 years, 193 (98.5%) of 196 patients were diagnosed correctly, with three diagnostic errors. Patients with a ventricular septal defect or side-by-side great arteries are more likely to have an unusual coronary pattern. CONCLUSIONS: Echocardiography appears to be highly reliable for determining proximal coronary artery anatomy in d-loop transposition of the great arteries. An unusual coronary artery pattern is more likely in patients with side-by-side great arteries or posterior aorta or a ventricular septal defect, or both.

Child↗
PubMed1988

Fate of pulmonary artery after anatomic correction of simple transposition of great arteries in newborn infants.

From April 1984 to April 1987, surgical anatomic correction was performed in 86 newborn infants, 2-23 days old (6.8 +/- 3.6 days, mean +/- SD) with simple transposition of the great arteries. In all patients, the pulmonary artery was reconstructed by end-to-end anastomosis according to the Lecompte maneuver, including eight patients with side-by-side position of the great arteries. Three different approaches were used. In the first 10 patients (group 1, six survivors), two separate patches of preserved tanned pericardium were used to reconstruct the pulmonary artery, whereas in the next 15 patients (group 2, 13 survivors), a single patch of the same material was used, and in the last 61 patients (group 3, 56 survivors), surgery was performed with a single patch of fresh autologous pericardium. Among the 75 survivors, 68 (including six in group 1, 12 in group 2, and 50 in group 3) were followed serially for at least 6 months (6-48 months, 26 +/- 9 months) with sequential noninvasive evaluations. At follow-up, all were asymptomatic with normal growth. Two patients with severe pulmonary artery stenosis (group 1) were successfully reoperated on. Four infants with moderate pulmonary artery stenosis have been followed medically and have had stable right ventricular pressures. The last 62 patients have normal or near-normal right ventricular pressures. The spatial relation of the great arteries did not affect the quality of the results. Group 1 had clearly the worst results.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗
PubMed2004

Congenitally corrected transposition of the great arteries: clues for prenatal diagnosis.

Congenitally corrected transposition of the great arteries (ccTGA) is an uncommon cardiac defect characterized by the atria connecting with the anatomically discordant ventricles and the ventricles connecting with discordant and transposed great arteries. Parallel vessels are evident in corrected TGA, but as this sign is also present in complete TGA, a heart anomaly requiring major cardiac surgery in the postnatal period, it is important to differentiate between the entities prenatally. Most cases of ccTGA have associated anomalies but isolated forms or those with a mild associated cardiac anomaly are infrequently detected prenatally. We report on three cases detected between 21 and 25 weeks' gestation on screening ultrasound with associated mild findings. One fetus had an isolated ventricular septal defect (VSD) first detected at 34 weeks. The child developed heart block at 4 years of age. The second case was associated with a small VSD, a tiny pulmonary trunk and a persistent right umbilical vein. After birth, mild pulmonary stenosis was found as an additional cardiac finding at 4 months of age. The third fetus had no additional cardiac anomalies prenatally, but after birth a bicuspid aortic valve was detected. The first case needed pacemaker implantation but the other two children required no cardiac surgery. Two of the cases were referred because abnormal vessel anatomy was detected on screening ultrasound. As prenatal detection of TGA is becoming a more frequent occurrence, this paper aims to present clues aiding in the prenatal diagnosis of atrioventricular and ventriculoarterial discordance, especially in its differentiation from complete transposition. These details are crucial for counseling and perinatal management.

Adolescent↗
PubMed1986

Congenitally corrected transposition of the great arteries associated with the pre-excitation syndrome.

A case with congenitally corrected transposition of the great arteries associated with the pre-excitation syndrome is presented. A gated-blood-pool study and Tl-201 myocardial imaging were performed using SPECT. The tomographic gated-blood-pool images showed the shapes of the right ventricle (RV) and left ventricle (LV), i.e., anatomically LV and RV respectively, to be reversed from that of normal patients. The relationships of the great vessels and ventricles also were easily visualized. RV hypertrophy (anatomical LV) was visualized on the Tl-201 images. Phase analysis showed the earliest excitation was in the anteroseptal region near the base of the LV, which was consistent with the site of the accessory conduction pathway. The gated-blood-pool and Tl-201 tomographic studies were helpful for demonstrating the corrected transposition of the great arteries and the pre-excitation syndrome.

Female↗
PubMedDate not supplied

Three-dimensional reconstruction of abnormal ventriculoarterial relationship by electron beam CT.

PURPOSE: Assessment of the relationships of the ventricles and great vessels is critical in evaluating cardiac anomalies before surgery. We investigated the usefulness of 3D CT in their demonstration. METHOD: From July 1995 through June 1996, 20 patients with abnormal ventriculoarterial relationships were analyzed by electron beam CT. A 3D gradient shading surface rendering was done on a workstation. We focused on the morphologies of the atria, ventricles, and great vessels and particularly their spatial relationships on the 3D images. RESULTS: There were two cases of isolated ventricular inversion, three of anatomically corrected malposition, seven of congenitally corrected transposition, and eight of complete transposition. Anatomic presentations of 3D surface images in all were superior to cineangiography and echocardiography presentations and were consistent with intraoperative findings. CONCLUSION: 3D CT images provide useful information that is not available by sectional or projective imaging. Independent segmental analysis of the anomalies by this method provides a complement for surgeons to understand the spatial relationships of the vessels and chambers of the heart, which may be of value in preoperative planning.

Adolescent↗