Chordal Systolic Anterior Motion of the Mitral Valve in Dextro-Looped Transposition of the Great Vessels After Mustard Procedure

Patient: Male, 41-year-old Final Diagnosis: Chordal SAM Symptoms: Dyspnea • dyspnea on exertion • fatigue Medication: — Clinical Procedure: Alfieri stitch • Mustard procedure • subpulmonic stenosis resection Specialty: Cardiology OBJECTIVE: Congenital defects/diseases BACKGROUND: D-transposition of the great vessels (D-TGA) was once a fatal diagnosis within the first year of life. The Mustard and Senning procedures were invented to redirect the blood flow via intra-atrial baffles. The complicated nature of the clinical course and presence of chordal systolic anterior motion of the mitral valve in a patient with D-TGA and prior subpulmonic resection and Alfieri stitching is presented. CASE REPORT: A 41-year-old man presented to the clinic with a chief concern of dyspnea on exertion and chronic chest pain. Diagnosed with D-TGA as an infant, he underwent balloon septostomy and later a Mustard procedure at 3 months of age and subpulmonic resection and Alfieri stitching as an adolescent. The patient now presented with transthoracic echocardiogram-revealed severe turbulence in native left ventricular outflow tract to the pulmonary circulation. Doppler velocities indicated this was originating from chordal systolic anterior motion of the mitral valve. CONCLUSIONS: This case reinforces the need for practitioners caring for such patients to become familiarized with and educated in the field of adult congenital heart disease, as patients once plagued with shorter life expectancies are living longer. Repeat surgical intervention or catheter-based therapies may be considered in the future should medical therapy fail to control our patient’s symptoms. A multidisciplinary approach and further monitoring of these patients for best practice guidelines would be ideal and beneficial for the patients and practitioners alike.