Acute Severe Left Atrioventricular Valve Regurgitation Secondary to Spontaneous Rupture of Chordae Tendinae in a Patient with Down Syndrome

We present a unique case of spontaneous chordal rupture in a young infant with Down syndrome and unrepaired transitional atrioventricular septal defect. This resulted in abrupt onset of severe left atrioventricular valve regurgitation, cardiogenic shock, and multiorgan dysfunction. Our case highlights the need for a high index of suspicion for a ruptured chord in a young infant presenting with acute onset of pulmonary edema.