A case of 15-year misdiagnosed heart failure: congenitally corrected transposition of the great arteries.

Introduction

Congenitally corrected transposition of the great arteries (CCTGA) is a rare condition, accounting for about 1% of all congenital heart diseases, and more than 90% of patients have associated anomalies like ventricular septal defect, pulmonary stenosis, and systemic atrioventricular valve anomalies. European Society of Cardiology (ESC) guidelines recommend systemic atrioventricular valve surgery for severe regurgitation before systemic ventricular function deteriorates (before right ventricular ejection fraction <45%).

Case presentation

A 54-year-old lady presented in 2000 with fatigue. Her cardiac catheterizations in 2000 and 2005 revealed normal coronary arteries, 3+ mitral regurgitation (Supplementary material online, Video S1); a significantly elevated pulmonary artery pressure (90/55 mmHg), right ventricular pressure (90/20 mmHg), pulmonary capillary wedge pressure (33 mmHg), and left ventricular end-diastolic pressure (25 mmHg) were recorded. She was kept under annual surveillance, but she developed exertional dyspnoea in time. She had multiple echocardiograms until 2015, which reported ‘severe mitral regurgitation, mild left ventricular systolic dysfunction, and severe pulmonary artery hypertension’. The heart team decided on medical treatment due to high-risk surgery. In 2015, she was admitted to our hospital in severe heart failure. The rhythm was atrial fibrillation, and there was right bundle branch block in electrocardiogram. Our TTE disclosed apically displaced left atrioventricular valve with dysplastic, thickened morphology, heavily trabeculated morphologically right ventricle on the left side of the heart, and parallel course of the great arteries (; Supplementary material online, Videos S2–S4). The systolic function of the systemic ventricle was moderately impaired, and both atrioventricular valves were moderately to severely regurgitant (; Supplementary material online, Video S5). There was no intracardiac shunt present. The atrioventricular and ventriculoarterial discordance were consistent with CCTGA, and the patient was judged as inoperable in accordance with ESC guidelines.

Figure 1

Apical four-chamber view demonstrating congenitally corrected transposition of the great arteries. Note the trabeculated morphologically right ventricle with the moderator band on the left side of the heart. The arrow indicates the tricuspid valve, which is dysplastic, thickened, stenotic, and more apically positioned than the mitral valve. Both atria are dilated. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

Figure 2

Anterior sweep of a modified apical four-chamber view showing situs solitus and parallel course of the great arteries. Ao, aorta; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle.

This case illustrates how a late diagnosis of CCTGA has an impact on a patient’s life. Marked tricuspid regurgitation and systolic dysfunction of the systemic ventricle probably contributed to congestive heart failure.

Supplementary material

Supplementary material is available at European Heart Journal - Case Reports online.

Consent: The author/s confirm that written consent for submission and publication of this case report including image(s) and associated text has been obtained from the patient in line with COPE guidance.

Conflict of interest: none declared.

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