Definition: Corrected transposition of the great arteries (CTGA) is a malformation of the heart in which the ventricular artery connections are incongruent with the atrioventricular connections, allowing the heart to correct its physiological function. Almost all CTGAs are combined with other cardiac malformations.
Morbidity.
Morbidity.
CTGA is a relatively uncommon congenital heart malformation, accounting for only 0.8% to 1.4% of congenital heart disease. From our statistics, it accounts for only 0.46% of our congenital heart disease surgery patients.
Clinical presentation.
The combination of different cardiac malformations will have different clinical manifestations. If combined with a large ventricular septal defect and severe left ventricular outflow tract stenosis, the same symptoms and signs as in tetralogy of Fallot will appear, such as cyanosis, dyspnea, and heart murmur.
Diagnosis.
Since the clinical manifestations are not characteristic, definite diagnosis mainly relies on echocardiography and cardiac catheterization and angiography. 1985, Attie et al. concluded that echocardiography and hemodynamic examination are the best means to confirm the diagnosis of the disease by analyzing the clinical features and diagnosis of 73 cases with inconsistent atrioventricular connections, while electrocardiography, X-ray chest radiography and isotope examination can be used as auxiliary. During the diagnostic examination, the issues that must be clarified as clearly related to the surgical procedure are.
1. The relationship and location of the atrioventricular and ventricular aortic connections must be clarified, as well as whether the heart is in an abnormal position. Abnormal heart position such as right rotation or horizontal axis transposition of the heart can make surgical operation exceptionally difficult.
2.To clarify whether other cardiac abnormalities are combined, and whether there are hemodynamic significance and meaningful pathophysiological changes to decide whether there are surgical indications and the timing of surgery.
3.Define the origin and location of the coronary artery to facilitate the choice of surgical approach.
4.In the case of pulmonary hypertension or pulmonary stenosis, to understand the extent of pulmonary vascular lesions, as well as to have a clear judgment of the site and extent of stenosis in the case of pulmonary stenosis and/or left ventricular outflow tract stenosis to help decide the surgical modality and the choice of surgical timing.
5. To evaluate the morphology and function of the atrioventricular valves of the heart, especially those bearing the body circulation.
Natural course of disease.
According to Friedbery in 1970 and Lev in 1963, 5% to 10% of children with corrected transposition of the great arteries are born with a third-degree AV block. In adults, Huhta in 1983 showed that about 30% of patients had combined third-degree AV block. In addition, 40% to 50% of patients have first- or second-degree AV block. In addition, because the ventricles of the body circulation are morphologically right ventricular, their cardiac function has been of great concern. There have been reported cases of long-term progressive decline in cardiac function, congestive heart failure and death in morphologic right ventricles with corrected transposition of the great arteries as the body circulation ventricle.
Treatment.
Corrective transposition of the great arteries alone does not require surgical treatment. Surgical treatment is required only when combined with other cardiac malformations with hemodynamic significance and meaningful pathophysiologic changes. In general, the surgical treatment of corrective transposition of the great arteries is basically surgery for combined malformations. As for the best surgical indication for correction of corrective transposition, Imai reported in 1994 that it is when corrective transposition is combined with ventricular insufficiency of the corporal circulation and/or atrioventricular regurgitation of the corporal circulation.
Surgical complications.
(i) Third-degree AV block.
(ii) Low cardiac output syndrome.
(iii) Left atrioventricular valve insufficiency.
Surgical results.
In the early years, the surgical outcome of corrected transposition of the great arteries combined with cardiac malformations was poor, but has improved significantly since the 1980s. In China, the early mortality rate was 29% in 24 cases before 1987 and 6.9% in 29 cases after 1988. This shows a significant improvement in the surgical outcome of corrective transposition of the great arteries. The surgical outcome of corrected transposition of the great arteries varies with the combined malformation. Postoperative in-hospital mortality was less than 5% for combined ventricular septal defect, 10% to 20% for combined ventricular septal defect and pulmonary stenosis, and up to 15% to 25% for simultaneous surgery including left atrioventricular valve replacement, with long-term survival rates of 80% and 76% at 5 and 10 years, respectively. Patients still need to pay attention to the maintenance of cardiac function after corrective surgery, and as long as cardiac function allows, they can live and study normally, generally should not engage in heavy work, and can have a family.