VolumeORIGINAL - 9 | Issue - 8 RESEARCH | August - 2020 PAPER Volume - 9 | Issue - 8 | August - 2020 | PRINT ISSN No. 2277 - 8179 | DOI : 10.36106/ijsr INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH
TOTAL SITUS INVERSUS WITH COR TRIATRIATUM SINISTER WITH SUPRAMITRAL RING IN AN ADOLESCENT
Cardiology Consultant interventional cardiologist, Oscar superspeciality hospital and trauma center, Ashish Kumar D.M Rohtak, Haryana-124001 ABSTRACT 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 denitive cure for these anomalies although percutaneous approach can also be tried. KEYWORDS Cor triatriatum sinister; supramitral ring; complete situs inversus; 2D Echocardiography INTRODUCTION: Cor triatriatum is rare congenital cardiac malformation with an estimated incidence of 0.1% of all congenital heart disease and it most commonly occurs on left atrium (Cor triatriatum sinister). Total situs inversus is a rare syndrome, with overall frequency estimated at 1/10,000 births, resulting from abnormal rotation of the cardiac tube during embryogenesis, is characterised by heart on right side of the midline while the liver and the gall bladder are on the left side. In Total situs inversus, heart is structurally normal in 90-95% of cases, in contrast to dextroversion (dextrocardia with situs solitus), which has high incidence of structural cardiac defects. Here we report an Figure 2-Parasternal view showing turbulent flow across extremely rare case of Cor triatriatum sinister with supramitral ring cortriatriatum membrane which is associated with Total situs inversus.
CASE REPORT: A 12 year old female was referred for cardiac murmur evaluation. History of non progressive dyspnea was present since toddler age. There was no family history of congenital heart disease. On examination, pulse rate of 78 beats/min, blood pressure of 100/60 mmHg with baseline oxygen saturation of 96%. On auscultation, S1 was normal, loud P2 with narrow splitting, 4/6 sysolic murmur on right parasternal border, faint diastolic murmur on right side of chest. Chest X-ray demonstrated dextrocardia and prominent vascular markings Figure 3-Parasternal view with pressure gradient across muscular with gastric air bubble on right side and liver shadow on left side. ECG VSD. revealed sinus rhythm with dextrocardia with biventricular hypertrophy. Abdominal ultrasound showed the presence of liver on DISCUSSION: the left side and spleen on the right side. The Aorta was towards right 80% of Cor triatriatum cases have associated cardiac defects (ostium side of spine and IVC was on the left side. Transthoracic secundum ASD, TOF, Bicuspid aortic valve, Anomalous pulmonary echocardiography demonstrated {I,L,I}(I,situs visceroatrial inversus; venous return, Coarctation of aorta, DORV, VSD, Common AV L, L-loop of ventricles; I, normally related inverted great arteries). canal).[1] It was rst described by Church in 1868 and was named by Borst in 1905.[2] It also showed two bromuscular membranes in the left atrium, one attached above the left atrial appendage dividing it into 2 chambers of It involves usually the left atrium (Cor triatriatum sinister) and rarely Cor triatriatum sinister (gure 1). The two chambers communicated the right atrium (Cor triatriatum dexter). The atrium is divided into two through a small restrictive orice with turbulent ow (gure 2) and a chambers by a thick bro-muscular or membranous septum into (i) a peak pressure gradient of 21 mm Hg. Another membrane was also seen proximal or posterior-superior chamber which drains the pulmonary in the left atrium attached laterally below the left atrial appendage venous blood and (ii) a distal or anterior-inferior chamber (true atrium) (gure 1). It was non restrictive in nature with a large central orice. A which is in contact with the AV valve and contains the atrial appendage small muscular ventricular defect (peak gradient 95 mm Hg)(gure 3) and the true atrial septum.[3] Clinical features mimic those of mitral was also present. Pulmonary veins were dilated. No ASD, PDA, stenosis (but absence of loud S1 and opening snap). anomalous pulmonary venous return was noted. Pathophysiologically the obstructive membrane leads to a pressure gradient with an associated rise in pulmonary arterial and venous pressure.[4,5] Cor triatriatum sinister was classied in1949 by Loefer,[6] according to the number and size of the orices in the bromuscular septum: Group 1-without an opening, Group 2- having one or more small openings, Group 3- having a single large opening.
Supramitral ring also has similar clinical presentation and is a close differential of Cor triatriatum sinister. The embryonic origin of supramitral ring is unclear, but it has been postulated that it results from incomplete division of endocardial cushion tissue.[7] Cor triatriatum sinister is believed to be a result of incomplete absorption of the Figure 1-Parasternal view shows Supramitral ring, Cor primary pulmonary vein during the fth embryonic week.[8] triatriatum sinister Occurrence of defects with varied embryological basis together is very
56 International Journal of Scientific Research Volume - 9 | Issue - 8 | August - 2020 PRINT ISSN No. 2277 - 8179 | DOI : 10.36106/ijsr rare.
Age and symptoms at the time of diagnosis are highly variable and depend on the degree of obstruction to the pulmonary venous return and presence of associated cardiac lesion.[9] In the case of mild obstruction, most blood will ow through the mitral valve, creating the picture of mitral stenosis. Moderate to severe obstruction results in left to right shunting across the atrial septal defect, which is inversely proportional in volume to that of the antegrade ow across the left atrial obstructing lesion. The absence of an associated ASD, although unusual in this entity, leads to the hemodynamic consequences and clinical picture simulating total anomalous pulmonary venous return. The denitive treatment of Cor triatriatum sinister is surgical excision of the membrane..Balloon dilatation can be an alternative in selected cases as a bridge to surgical repair.[10] Our case was successfully managed with surgical repair
CONCLUSION: Cor triatriatum sinister and supramitral ring and complete situs inversus can be adequately diagnosed with transthoracic echocardiography and surgical correction is the denitive cure for these anomalies. To the best of our knowledge, this is the only case report of these rare defects occurring in combination.
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