A constellation of cardiac anomalies: Beyond shone's complex

Abstract

Shone's anomaly is a very rare congenital cardiac malformation characterized by four serial obstructive lesions of the left side of the heart (i) Supravalvular mitral membrane (ii) parachute mitral valve (iii) muscular or membranous subaortic stenosis and (iv) coarctation of aorta. We report a unique presentation of Shone's complex in a 14-year-old adolescent male. In addition to the four characteristic lesions the patient had bicuspid aortic valve, aneurysm of sinus of valsalva, patent ductus arteriosus, ventricular septal defect, persistent left superior vena cava opening into coronary sinus and severe pulmonary artery hypertension. This case report highlights the importance of a strong clinical suspicion of the coexistence of multiple congenital cardiac anomalies in Shone's complex and the significance of a careful comprehensive echocardiography.

Prenatal Diagnosis of Shone’s Syndrome by Fetal Echocardiography

“…Shone's syndrome is a rare and under-recognized diagnostic entity, associated with substantial morbidity related to arrhythmias, heart failure, and interventions [13] . The obstructive lesions in Shone's complex have a tendency to worsen over time as compared to other congenital heart defects [14] . The antenatal recognition of Shone's syndrome is still challenging, the complex hemodynamic interactions between multiple levels of left-side obstructions challenge the clinical determination of prognoses [15] and sometimes the diagnosis at mid-gestation is misleading, as both steady state and worsening by the end point are possible [5] .…”

Prenatal diagnosis of Shone’s syndrome: ultrasonographic features and differential diagnosis with coarctation of the aorta

“…Shone's syndrome is a rare and under-recognized diagnostic entity 13 . The obstructive lesions in Shone's complex have a tendency to worsen over time as compared to other congenital heart defects 14 . The antenatal recognition of Shone's syndrome, which remains infrequent in the literature 15 , is still challenging, the complex hemodynamic interactions between multiple levels of left-side obstructions challenge the clinical determination of prognoses 16 and sometimes the diagnosis at mid-gestation is misleading, as both steady state and worsening by the end point are possible 5 .…”

Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava and Discrete Subaortic Stenosis Diagnosed in a Patient with Sick Sinus Syndrome: A Case Report and Brief Review of the Literature

“…To the best of our knowledge, there has only been one further case reported with this association of anomalies [ 9 ]. The other published cases of discrete subaortic stenosis and a left-sided vena cava do not offer details about the status of the right-sided vena cava, and several include complex malformative syndromes [ 10 , 11 , 12 , 13 , 14 ]. The purpose of the following discussion is to address the main characteristics of this venous anomaly.…”

“…The most frequently reported congenital cardiac defects are atrial and ventricular septal defects, bicuspid aortic valves, the coarctation of the aorta, coronary sinus atresia, anomalous pulmonary venous return, and tetralogy of Fallot [ 19 ]. Complex malformations, such as Shone’s syndrome, and systemic anomalies, such as VACTERL association and esophageal atresia, have also been reported [ 5 , 11 ].…”