Corrected Transposition of the Great Arteries with Ebstein’s Anomaly, Dysplasia of the Mitral Leaflets and Persistence of Left Superior Vena Cava in an Adult

Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult popula-tion, especially if it is associated with other abnormalities such as Ebstein’s anomaly and abnormal origin of coronary arteries. Case presentation: Twen-ty-two-year-old woman admitted to intensive care unit with acute decom-pensated heart failure. The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein’s anomaly. The computed tomography corroborated these findings and additionally showed a left superior vena cava and an abnormal origin of the coronary arteries. The patient is in NYHA functional class II, receiving treatment with diuretic, digitalis, beta-blockers and angiotensin-converting enzyme (ACE) inhibitor. Conclusion: This is a very interesting case of an adult in the third decade of life with cc-TGA associated with abnormalities not described in the literature that had reached adulthood. Noninvasive images play an important role in its diagnosis.


Introduction
The incidence of grown up congenital heart disease is still unknown. According to the 32 nd Bethesda Conference reported in 2000, there were approximately 2800 adults with congenital heart disease (CHD) per 1 million population, with more than half of them having moderate or high complexity of their defect [1].
Congenitally corrected transposition of the great arteries (cc-TGA or L-TGA) is an uncommon entity, accounting for <1% of all CHD [2]. The commonest associated defect is ventricular septal defect, found in two-thirds of cases, followed by stenosis of the outflow tract of the morphologically left ventricle found in 50%. Tricuspid valve is abnormal with varying degrees of apical displacement into the ventricular cavity resembling Ebstein's anomaly [3]. According to the literature, there are few reports of L-TGA associated with Ebstein's anomaly in adult population, given that the majority of patients die within the first years of life if they are not surgically treated. We present a rare case of an adult woman with L-TGA associated with Ebstein's anomaly, left superior vena cava and pulmonary hypertension diagnosed by non-invasive imaging and computed tomography.

Case Presentation
A 22-year-old-woman from Ayacucho-Peru came to her local hospital with signs of systemic and pulmonary congestion. The patient was initially managed with inotropic and vasopressor drugs, diuretic therapy and high flow oxygen and then was referred to our hospital. She was hospitalized in the intensive care unit with tachycardia (heart rate: 101 bpm), tachypnea (respiratory rate: 24/min), hypotension (BP: 92/62 mmHg) and blood oxygen saturation of 98% (FiO 2 36%). At physical exam, signs of systemic and pulmonary congestion, with painful hepatomegaly, jugular ingurgitation and edema in lower limbs and bi-basal rales were found. On auscultation, the heart sounds were arrhythmic, with an increased second cardiac sound, systolic murmur III/VI in mitral focus and systolic murmur IV/VI in tricuspid focus. Electrocardiogram in sinus rhythm with heart rate of 75 bpm. QS wave in DII, DIII, aVF and RS wave from V2 -V6 ( Figure 1). Chest X-ray showed a severe cardiomegaly and signs of pulmonary congestion.
The transthoracic echocardiogram demonstrated situs solitus in levocardia. Ostium secundum atrial septal defect of 1.1 cm with left to right shunt, great vessels side to side, the pulmonary artery located to the right and aorta to the left. In four-chamber view, atrioventricular discordance was detected with severe tri-  He is currently in NYHA functional class II with a follow-up every 2 months at the Congenital Heart Disease Adult Clinic of our institution.

Discussion
Congenitally corrected transposition of great arteries (cc-TGA) accounts for 0.5% of all CHD [4]. It is commonly associated with ventricular septal defect in   the 60% to 80% of cases, pulmonary stenosis or atresia in 30% to 50% and abnormalities of tricuspid valve with varying degrees of tricuspid regurgitation in 14% to 56% [4]. However, it is rarely associated with an authentic Ebstein anomaly and persistence of the left superior vena cava that drains into the azygos vein. The persistence of left superior vena cava (SVC) is a rare congenital anomaly and can be seen in conjunction with other cardiac or vascular abnormalities. Identification of inferior vena cava with azygos continuation is of great importance before catheterization access from the femoral veins and any surgical procedures to complex congenital heart disease [5]. In fact, Celermajer et al. over a 20-year period from 1968 to 1988 found 10 cases with coexisting cc-TGA and Ebstein's anomaly; none of them had left SVC. Unlike our patient who has reached adulthood, those patients were neonates (7 cases) or children (3 cases) and 5 of them die within first year of life; these patients had severe systemic atrioventricular valve regurgitation and aortic arch obstruction [6]. On the other hand, pulmonary hypertension is rare in the absence of a large ventricular septal defect [4]. In our case, despite no ventricular septal defect, a moderate pulmonary hypertension was detected, probably as a result of the volume overload due to severe tricuspid regurgitation. Also, severe mitral regurgitation caused by dysplasia of the mitral leaflets was evident, which is a less common condition associated with cc-TGA [4]. Mitral valve straddling occurs in hearts with double inlet ventricles or a double outlet RV, criss-cross hearts and in some rare cases of ccTGA [7] [8] [9]. As described in literature, our patient had situs solitus atrial (like 95% of cases) and normal heart position (nearly 80% of cases are in levocardia) [4]. The relationship of the great vessels showed a distinct pattern to the usual, the great vessels were in parallel or side to side [3].
With regard to the possibilities of surgical treatment we must emphasize that this type of complex CHD is rarely seen in adults, so there is quite few expertise in repairing this type of defects in our country. However, in experienced centers, if the ejection fraction is depressed despite afterload therapy [4], options such as the double switch operation or transplantation should be considered [3]. When the systemic ventricle shows depressed systolic function and severe tricuspid regurgitation, and the double-switch procedure is not feasible, one alternative in

Conclusions
This patient has certain features that are common to the usual presentation of cc-TGA, but there are no current reports of cc-TGA associated with authentic Ebstein's anomaly, dysplasia of the mitral leaflets, left SVC and pulmonary hypertension, being even more interesting the fact that the patient reached adulthood.
Non-invasive images (echocardiography and computed tomography) played a very important role in the precise characterization of this complex congenital heart disease.