What is a Shone complex?
What is Shone’s complex? Shone’s complex is a congenital (present at birth) heart disease. It affects how blood flows both into and out of the left side of the heart. The multiple sites of blockage in the blood flow through the left side of the heart differentiates Shone’s syndrome from other isolated heart defects.
Is Shone’s complex hereditary?
The team found that mutations in the gene encoding myosin, a contractile protein that is highly expressed during development accounted for about 11 percent of Shone syndrome. The team also reports a shared mutation among some CHD patients with Ashkenazian ancestry.
What is Supravalvular mitral ring?
Supravalvar mitral ring is a rare congenital heart defect of surgical importance. The condition characterized by an abnormal ridge of connective tissue on the atrial side of the mitral valve.
How many people have Shone’s Complex?
Results: Among 4189 adults with congenital heart disease, 28 (0.67%) patients (mean age, 35 ± 11 years; 50% women) had complete or incomplete Shone complex and were followed for a median of 8 years. Only 39% were previously diagnosed as having Shone complex.
Can your heart be on the right?
Dextrocardia is a condition in which the heart is pointed toward the right side of the chest. Normally, the heart points toward the left. The condition is present at birth (congenital).
How long can you live with Shone’s Complex?
The average follow-up of 24 patients was 6.3 years (range, 1 to 16 years). Overall survival was 93%. All surviving patients are in class I or II congestive heart failure. Conclusions: Long-term survival of patients diagnosed with Shone complex is excellent.
What is Supravalvular aortic stenosis?
Supravalvular aortic stenosis (SVAS) is a heart defect that develops before birth. This defect is a narrowing (stenosis) of the large blood vessel that carries blood from the heart to the rest of the body (the aorta).
How common is subaortic stenosis?
Subvalvular aortic stenosis (SAS), also called subaortic stenosis, is a rare disorder seen in infants. In most cases, it involves the presence of a membrane that is typically muscular just below the aortic valve, which causes a fixed obstruction to blood flow across the left ventricular outflow tract.
Does mitral stenosis increase stroke risk?
Mitral stenosis was not associated with stroke risk (adjusted odds ratio 1.07, 95% CI 0.67-1.72, P=0.764). Conclusion: The performance of the CHA2DS2-VASc score was modest in AF patients both with and without RHD. In this cohort, moderate-to-severe mitral stenosis was not an independent risk factor for stroke.
What is the shone complex?
Shone complex is a rare congenital cardiac malformation characterized by a complex of four obstructive lesions of the left heart: supravalvular mitral membrane, parachute mitral valve, muscular or membranous subvalvular aortic stenosis and coarctation of aorta.
Is the shone complex an under-recognized entity?
Conclusions: Shone complex is an under-recognized entity associated with relatively low mortality in adulthood but substantial morbidity related to arrhythmias, heart failure, and interventions. Increased awareness of this condition and associated complications may allow for more tailored follow-up.
What is the mortality and morbidity associated with shone complex?
Shone complex is an under-recognized entity associated with relatively low mortality in adulthood but substantial morbidity related to arrhythmias, heart failure, and interventions. Increased awareness of this condition and associated complications may allow for more tailored follow-up.
What is coarctation in shone’s complex?
Shone’s Complex. A bicommissural aortic valve is a common finding in patients with Shone’s syndrome, and typically results in valvar aortic stenosis. Coarctation of the aorta is described as narrowing of the aortic arch, typically at the level of the aortic isthmus. The transverse arch may be elongated and hypoplastic.