[Criss-cross heart with atrioventricular discordance, straddling A-V valve, double outlet right ventricle and preexcitation].

We report the case of a newborn with cyanosis, dyspnea, and supraventricular tachycardia due to reentry with good response to medical therapy. The diagnosis was made by echocardiography. The patient died suddenly at 6 days of follow-up. Necropsy confirmed the following anomaly: criss-cross heart with straddling right atrioventricular valve, and left atrioventricular valve stenosis connected to the anterior trabeculated ventricle giving rise to the pulmonary artery and aorta.Precocious rotation when septal closure is not concluded favors atrioventricular valve anomalies and rhythm disorders.