Double Discordance with Pulmonary Stenosis: About a Clinical Case

Introduction: Double discordance or corrected transposition of the great vessels is a rare congenital heart disease. It is an atrioventricular and ventriculo-arterial mismatch. It is a complex and unusual form of congenital heart disease. Often asymptomatic, in its isolated form, bradycardia, murmur and cyanosis can be a mode of revelation. Case presentation: We report the case of an 11-year-old child, asymptomatic at birth, after he started to have dyspnea on exertion, recurrent bronchitis, motivating a cardiological consultation. On cardiac physical examination, the heart sounds regular, not rapid at 81 BPM with a grade 3-4/6 systolic murmur at the 4th left EIC (Intercostal space). EKG (electrocardiogram) shows PR interval at 0.20 seconds. Cardiac ultrasound reveals atrioventricular discordance, vascular malposition, anterior aorta, the two vessels placed side by side, minimal pulmonary insufficiency, a small leak at the levels of the mitral and tricuspid valves, dystrophic pulmonary valves with an average gradient of 91 mmHg, max at 158 mmHg. Regular follow-up has been recommended through the performance of a clinical examination and cardiac ultrasound. Conclusion: The double discordance can be asymptomatic, and survival can be long in the isolated forms, but the evolution is not always benign, especially in the associated forms. It depends on the function of the systemic right ventricle and associated abnormalities.