TOTAL SITUS INVERSUS WITH COR TRIATRIATUM SINISTER WITH SUPRAMITRAL RING IN AN ADOLESCENT

Cor triatriatum sinister is a rare congenital anomaly in which a membrane divides LA into two distinct chambers, supramitral ring is another membrane dividing LA. Combination of both these defects is very rare and these occurring in tandem with complete situs inversus with associated VSD are rarer still. 12 year old female with h/o dyspnea and a systolic murmur presented with complete situs inversus with cor triatriatum sinister with supramitral ring with VSD. Cor triatriatum sinister and supramitral ring and complete situs inversus is a very rare congenial disorder that can be confused clinically with congenital mitral stenosis but can be adequately diagnosed with transthoracic echocardiography and surgical correction is the definitive cure for these anomalies although percutaneous approach can also be tried.

Double outlet right ventricle, pulmonary stenosis and congenital mitral stenosis

A 4-year-old girl child was diagnosed with double outlet right ventricle (DORV), severe pulmonary stenosis, and supramitral ring. This case is presented to bring to light this rare association. Through this report, we aim to stress importance of assessing mitral apparatus on echocardiography during evaluation for situations like DORV and Tetralogy of Fallot (TOF). The physiological differences in such situations as opposed to their isolated counterparts and special postoperative outcomes are also discussed.

Valvular heart disease

Rheumatic valve disease remains prevalent in developing countries, but over the last 50 years there has been a decline in the incidence of rheumatic valve disease and an increase in the prevalence of degenerative valve pathology in northern Europe and North America. In all forms of valve disease, the most appropriate initial diagnostic investigation is almost always the echocardiogram. The most common cause is rheumatic valve disease. Other causes include mitral annular calcification, congenital mitral stenosis, infective endocarditis (very rarely), and systemic lupus erythematosus (Liebman–Sachs endocarditis). The important consequences of mitral stenosis are its effect on left atrial pressure, size, and the pulmonary vasculature; it commonly causes atrial fibrillation. Presenting symptoms are typically exertional fatigue and breathlessness; systemic embolism can occur. Characteristic physical signs are irregular pulse, tapping apex beat, loud first heart sound, opening snap, and an apical low-pitched rumbling mid-diastolic murmur.

A rare case of an isolated aneurysmal supravalvar mitral ring in a young Nigerian adult: a cor-triatriatum mimic

Supravalvar mitral ring is a rare congenital abnormality characterized by a ridge of connective tissue located above the mitral valve. It is a cause of congenital mitral stenosis typically presenting in childhood and usually associated with other cardiac abnormalities. We report the rare case of a 24-year-old male presenting with an isolated aneurysmal supravalvar mitral ring. He presented at the emergency room with a 2-week history of worsening heart failure symptoms and antecedent effort intolerance of 4 years duration. He was referred from a primary care facility with an echo diagnosis of cor-triatriatum. Echocardiography done at our centre revealed an isolated aneurysmal supravalvar mitral ring of the intramitral variant. This report highlights the unusual isolated presentation of a supravalvar mitral ring in a young adult and the need to carefully differentiate it from cor-triatriatum, a possible close mimic.