Clinical Cases: Congenital heart disease800Late diagnosis of double chambered right ventricle in an adult: diagnostic pitfalls and the role of multimodality imaging801Anomalous origin of left pulmonary artery from ascending aorta: an unusual cause of cardiac failure802An uncommon cause of right ventricular failure803Staged severe evolution and treatment dilemmas in a patient with Marfan syndrome804A rare presentation of coarctation of the aorta: transient ischemic attack due to thrombus formation in the coarcted segment which was treated with oral anticogulation805Penetrating cardiac trauma resulting in a ventricular septal defect, a flail mitral valve leaflet and a right middle cerebral artery infarct, with percutaneous closure of the ventricular septal defect

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Introduction.Double-chambered right ventricle (DCRV) is a very rare congenital defect causing right ventricular outflow tract (RVOT) obstruction where the right ventricle (RV) is divided by aberrant muscle bundles into a high-pressure and low-pressure chamber.Case report.A 44-year-old man was referred to our center for progressive exertional dyspnea.He was previously diagnosed with Eisenmenger syndrome secondary to an uncorrected ventricular septal defect (VSD).Clinical evaluation showed cyanosis, a harsh systolic murmur along the left sternal border and hepatomegaly.The electrocardiogram revealed atrial fibrillation, right bundle branch block and right ventricular hypertrophy.Transthoracic echocardiography (TTE) showed the presence of a discrete obstruction in the RVOT (Fig. A), adjacent to the pulmonary valve, caused by an anomalous muscle bundle, consistent with DCRV.Both the right atrium (RA) and the RV were severely dilated, with marked RV trabeculations and moderate to severe RV systolic dysfunction.Moderate to severe secondary tricuspid regurgitation was also identified.The RV-RA systolic gradient (108 mmHg) was used as a surrogate for the intraventricular gradient.The left ventricular ejection fraction was normal.The patient had a large circumferential pericardial effusion with no signs of cardiac tamponade.The patient underwent right heart catheterization that showed severely increased right ventricular systolic pressure (108 mmHg) but normal pressure and arterial resistance in the pulmonary artery.The right ventriculogram identified an obstruction between the RV and the pulmonary valve (Fig. B).There was no VSD.Since the patient was cyanotic, the presence of an intracardiac shunt was suspected.Transoesophageal echocardiography (TOE) confirmed the diagnosis of DCRV and identified a right-to-left atrial shunt at the level of a patent foramen ovale.For further anatomical assessment of the RV obstruction and RV systolic function, a cardiac magnetic resonance (CMR) study was performed.It showed subinfundibular muscular obstruction and severe RV systolic dysfunction (Fig. C).Surgery was indicated (with a high perioperative risk), and the patient underwent resection of the muscle bundle and tricuspid annuloplasty.Unfortunately, the postoperative evolution was unfavorable, with nonresponsive RV failure and death.Discussion.DCRV is frequently associated (80-90%) with VSD/pulmonary valve stenosis.Isolated DCRV is very uncommon, therefore ruling out associated lesions is paramount.This disease is usually diagnosed in childhood.Hence, there is little data available in adults.Moreover, the presentation is frequently nonspecific.Differential diagnosis must be made with infundibular/ pulmonary valve stenosis.TTE is diagnostic in a few patients (15%), while TOE is superior, defining the full morphological details, including associated defects.Since surgical correction before RV failure carries a small risk and is curative, misdiagnosis must be prevented by accurate imaging of the whole RV, RVOT and pulmonic valve.While TTE is the first-line imaging tool, use of additional imaging techniques: TOE, cardiac catheterization, ventriculography and CMR adds useful information in many cases.Conclusion.This is a very rare presentation of an isolated DCRV in an adult patient.It underlines the importance of using multiple and complementary imaging techniques to establish the correct diagnosis.

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