Challenging Combination; Pseudocoarctation with Subaortic and Subpulmonic Stenosis

Combined left and right ventricular outflow tract obstruction is an extremely rare congenital anomaly. Pseudocoarctation (PcoA), also known as kinking or buckling of the aorta, is an uncommon anomaly of the aorta. A 4-year-old girl presented to our hospital with progressive complaints of exertional dyspnea and intermittent dizziness for about one year. She was diagnosed with severe combined left and right ventricular outflow tract obstructions associated with pseudocoarctation of the aorta. Echocardiographic estimated peek pressure gradients across the right and left ventricular outflow tracts were 151 mmHg and 109 mmHg respectively. There was no significant pressure gradient across the pseudocoarctaion. Aggressive surgical myomectomy for both outflow tracts, combined with reconstruction of right ventricular outflow tract using pericardial patch and iatrogenic ventricular septal defect (VSD) closure using PTFE patch was performed. No intervention was done for pseudocoarctation of the aorta (PcoA). Association of PCoA with different types of cardiac anomalies has been reported. However to our knowledge, combined left and right ventricular outflow obstruction associated with PCoA has not been described before.