Severe aortic complications in a patient with variant Shone's complex and bicuspid aortic valve.

Ahmed M, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-221348 Description A 47-year-old man presented with new onset exertional dyspnoea. He received a surgical repair of coarctation of the aorta (COA) at the age of 21 years and aortic root grafting with mechanical aortic valve replacement for type A aortic dissection and bicuspid aortic valve (BAV) at the age of 28 years. He had been otherwise healthy and did not follow-up with a cardiologist or surgeon after his last surgery. An echocardiogram (Echo) revealed normal left ventricular size and wall thickness, with an ejection fraction of 45%–50%. There was a parachute mitral valve (MV) (figure 1), with only one severely underdeveloped lateral papillary muscle. The mechanical aortic valve was functioning normally. There was a massively dilated distal aortic arch and proximal descending aorta with maximum dimension of up to 65 mm (figure 2). There was no Doppler evidence of recurrent COA. The CT angiogram confirmed the giant aneurysm, which was absent from the previous site of COA repair (figure 3). With the current Echo finding of parachute MV and history of COA, he was diagnosed with variant Shone’s complex. He then underwent a stent graft repair of the aneurysm. Shone’s complex is a rare congenital disorder consisting of four obstructive lesions of the left side of the heart which are (1) supra-MV membrane, (2) parachute MV, (3) subaortic stenosis and (4) COA. Since its initial reporting in 1963, a limited number of cases had been reported in English literature. In contrast to the classic Shone’s complex, incomplete forms with two or three lesions were also reported as variant or partial Shone’s complex. Our case is a variant Shone’s complex with parachute MV and COA. Most of the cases of Shone’s complex were diagnosed during childhood with left-sided outflow obstruction with resultant downstream left heart underdevelopment. Patients were usually symptomatic during early childhood. Typical symptoms include dyspnoea, tachypnoea, poor feeding, failure to thrive, fatigue and signs and symptoms of heart failure and low output syndrome. Recurrent episodes of wheezing and respiratory tract infections with pulmonary congestion and acute pulmonary oedema had been reported as well. Transthoracic Echo was used as diagnostic and follow-up tool. Generally, patients with Shone’s complex undergo a number of left heart interventions, both surgical and transcatheter approaches. COA was associated with an increased likelihood for repeat interventions. The 10-year transplant-free survival was 86%. The presence of Severe aortic complications in a patient with variant Shone’s complex and bicuspid aortic valve