Ascites and weight loss in a child: due to congenital division of the right atrium

Abstract

Congenital division of an atrial chamber is a very rare congenital malformation that more commonly affects the left atrium but which may, in rare circumstances, involve the right atrium. Such a divided right atrium may present with symptoms consistent with increased portal venous pressure. Reported is a case with unusual clinical presentation. The patient underwent resection of the dividing shelf with good postoperative results.

Cor triatriatum dexter in children: Literature review and case report

“…2,12,17 (6) Predominant obstruction of inferior vena cava flow by the RA membrane with no associated ASD. 6,7 Eleven of the 14 pediatric cases reported in the literature presented with cyanosis early in life due to the presence of obstructive sinoatrial orifice(s) and an associated ASD of varying sizes. [1][2][3][4][5][8][9][10][11][12] Two of these 14 cases also presented with Budd-Chiari syndrome with exudative enteropathy in one case 6 and ascites in the other 7 due to obstruction of the inferior vena cava flow by the RA membrane, in the context of an intact interatrial septum.…”

“…6,7 Eleven of the 14 pediatric cases reported in the literature presented with cyanosis early in life due to the presence of obstructive sinoatrial orifice(s) and an associated ASD of varying sizes. [1][2][3][4][5][8][9][10][11][12] Two of these 14 cases also presented with Budd-Chiari syndrome with exudative enteropathy in one case 6 and ascites in the other 7 due to obstruction of the inferior vena cava flow by the RA membrane, in the context of an intact interatrial septum. As a sole symptom, progressive fatigue and dyspnea on exertion is consistent with associated TV dysfunction in the context of CTD and can contribute to its incidental diagnosis by echocardiography, especially in cases of unobstructive sinoatrial orifice(s) and no associated ASD, as in our case.…”

“…4 After 1980, the reported 9 cases, including ours, survived thanks to the complete resection of the membrane at the time of surgery. [4][5][6][7][8][9][10][11][12] In the last 6 cases including ours, which were reported after 2009, accurate preoperative diagnosis of CTD relied entirely on TTE performance. [8][9][10][11][12] Nowadays, cardiac magnetic resonance imaging may be very helpful in the diagnostic assessment of CTD, as an additional valuable tool, to delineate the membrane and assess better right ventricular volume and function.…”

Cor Triatriatum Dexter versus Prominent Eustachian Valve in an Adult Congenital Heart Disease Patient

“…CTD has usually a perforated membrane that allows some flow across the membrane into the downstream chamber of the right atrium and through the tricuspid valve into the right ventricle 2 and is frequently associated with right‐side defects such as stenosis or atresia of the pulmonary valve, tricuspid valve abnormalities, or an atrial septal defect 3 . In the presence of an obstructive CTD, there is cyanosis if there is an associated interatrial right to left shunt (foramen ovale or atrial septal defect), and there are symptoms consistent with increased portal venous pressure—such as ascitis, hepatomegaly, or protein‐losing enteropathy—secondary to intraatrial obstruction caused by the presence of such an abnormal partition 4 …”

“…3 In the presence of an obstructive CTD, there is cyanosis if there is an associated interatrial right to left shunt (foramen ovale or atrial septal defect), and there are symptoms consistent with increased portal venous pressuresuch as ascitis, hepatomegaly, or protein-losing enteropathy-secondary to intraatrial obstruction caused by the presence of such an abnormal partition. 4 The EV is considered to be a benign finding; however, on rare occasions, a large prominent EV can be associated with obstruction of the IVC, bacterial endocarditis and thrombosis with subsequent pulmonary embolism or systemic embolism via a patent foramen ovale. 5,6 Though in our case no complication was associated with the EV, due to the existence of a large interatrial septal defect with evidence of right volume overload, a net leftto-right shunting and a significant tricuspid regurgitation associated with a reduced exercise tolerance our patient was sent for surgical closure of the atrial septal defect and the implantation of a tricuspid ring.…”