Role of perioperative echocardiography in repair of incomplete shone complex: A case series

Multilevel obstruction of left-sided heart structures was originally characterized by Shone et al. The formulation of an appropriate operative strategy remains challenging and needs to be individualized for this complex subset of patients. Intraoperative transesophageal echocardiography (TEE) not only helps in delineating spatial anatomy but also reveals associated anomalies that help in decision-making regarding operative strategies for these patients. Here, we discuss five such cases of Shone's anomaly presenting at varied age group with different associated anomaly in which intraoperative TEE played a pivotal role in the management.

A Rare Case of Shone Complex, Where Coarctation was Treated Successfully with Covered Cheatham Platinum Stent

Shone complex is an extremely rare and severe congenital heart disease characterized by left -heart obstruction at multiple levels namely supravalvular mitral ring, parachute mitral valve, subaortic stenosis and coarctation of the aorta. We in the present case describe a patient of shone complex who underwent successful coarctoplasty with the help of BIB (balloon in balloon) dilatation catheter and covered CP (cheatham platinum) stent. The role of interventional cardiologist is to diagnose and choose the right approach for the specific patient, whether surgery, balloon or stent.

Incomplete Form of Shone Complex in an Adult Congenital Heart Disease Patient / 대한흉부외과학회지

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.

Supra-Annular Mitral Valve Replacement in Two Infants / 日本心臓血管外科学会雑誌

When mitral valve dysfunction occurs in infants and mitral valve repair is difficult, mitral valve replacement (MVR) is required. However, commercially available prosthetic heart valves can be too large to implant in infants with a small annulus. In these children, the technique of supra-annular MVR is useful. Here we report two cases of supra-annular MVR, which were performed using an expanded polytetrafluoroethylene (ePTFE) graft as a skirt for a prosthetic valve. This method has been previously reported by Sung et al. The first case was a 16-month-old, 6.7-kg male infant who suffered from Shone' syndrome, mitral stenosis (MS) with a parachute mitral valve, coarctation of the aorta (CoA), and ventricular septal defect (VSD). MS progressed after CoA repair and VSD closure and a supra-annular MVR was performed. The second case was a 5-month-old, 4.9-kg female infant who suffered from polysplenia, intermediate atrioventricular septal defect (AVSD), and severe left atrioventricular valve regurgitation. AVSD repair was performed at the age of 3 months. However, valve stenosis and regurgitation gradually progressed postoperatively and consequently, a supra-annular MVR was performed. Postoperative prosthetic valve function was good in both cases. We believe that this method of performing supra-annular MVR is useful for infants with a small annulus.

Obstructive membrane in arch of aorta in a case of Shone’s complex.

Shone’s complex is a rare congenital heart disease consisting of multisite obstruction on the left side of the heart. The obstructive membrane in the arch of aorta is never described among these obstructions. We report echocardiographic findings in a patient with Shone’s complex with the obstructive membrane in the arch of aorta.