A rare congenital triad: ebsteinoid tricuspid valve, giant right atrial aneurysm, and atrial septal defect

Figures

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  Fig. 1. Chest radiograph (posteroanterior view) demonstrating marked cardiomegaly with elevated right ventricular apex and a cardiothoracic ratio of 75%. Prominent dilation of the right cardiac border indicates severe right atrial enlargement

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  Fig. 2. Transthoracic echocardiography (apical four-chamber view) showing a giant, aneurysmal right atrium measuring \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\:90.8\mathrm{\:mm}\times\:98.4\mathrm{\:mm}$$\end{document}. The white arrow indicates the lack of typical apical displacement. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle

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  Fig. 3. Transthoracic echocardiography (apical four-chamber view) detailing right ventricular dimensions (\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\:49.7\mathrm{\:mm}\times\:50.6\mathrm{\:mm}\times\:65.5\mathrm{\:mm}$$\end{document}). The white arrow points to the tethered septal leaflet of the Ebstenoid tricuspid valve without typical apical displacement. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle

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  Fig. 4. Transthoracic echocardiogram (apical four-chamber view) with color Doppler demonstrating severe, low-pressure tricuspid regurgitation (TR) filling the giant right atrium, with the regurgitant jet directed toward the interatrial septum (white arrow). LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; TR, tricuspid regurgitation

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  Fig. 5. Continuous-wave Doppler interrogation of the tricuspid regurgitation jet yielding a peak systolic velocity of \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\:3.39\mathrm{\:m/s}$$\end{document} and an estimated right ventricular systolic pressure (RVSP) gradient of \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\:46.0\mathrm{\:mmHg}$$\end{document} (excluding right atrial pressure), indicative of moderate pulmonary hypertension

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  Fig. 6. Four-dimensional transthoracic echocardiographic reconstruction viewed from the right atrial aspect, illustrating the massive aneurysmal cavity of the right atrium and abnormal tricuspid valve coaptation

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  Fig. 7. Contrast-enhanced axial cardiac computed tomography (CT) confirming a giant aneurysmal right atrium measuring \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\:93.5\mathrm{\:mm}\times\:94.5\mathrm{\:mm}$$\end{document} without evidence of intracavitary thrombus