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Right Heart Dilatation in Adults: Congenital Causes

Amanda L. Cook1, Lynne M. Hurwitz2,3, Anne Marie Valente1,3,4 and J. René Herlong1,3

1 Department of Pediatrics, Division of Pediatric Cardiology, Duke University Medical Center, Durham, NC.
2 Department of Radiology, Duke University Medical Center, Box 3808, Durham, NC 27710.
3 Duke Cardiovascular Magnetic Resonance Center and Duke University Medical Center, Durham, NC.
4 Department of Medicine, Division of Adult Cardiology, Duke University Medical Center, Durham, NC.


Figure 1
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Fig. 1 Diagram shows atrial septum and atrial septal defects (ASDs). In this view, free right atrial wall has been removed. R. = right. (Courtesy of Michael Stevens, Winston-Salem, NC)

 

Figure 2
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Fig. 2A —48-year-old asymptomatic man with secundum atrial septal defect (ASD). Posteroanterior (A) and lateral (B) chest radiographs show shunt vascularity (arrows, A) with normal-sized cardiac silhouette.

 

Figure 3
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Fig. 2B —48-year-old asymptomatic man with secundum atrial septal defect (ASD). Posteroanterior (A) and lateral (B) chest radiographs show shunt vascularity (arrows, A) with normal-sized cardiac silhouette.

 

Figure 4
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Fig. 2C —48-year-old asymptomatic man with secundum atrial septal defect (ASD). Contrast-enhanced axial CT images of heart obtained from abdominal CT reveal enlargement of right ventricle (RV). LV = left ventricle.

 

Figure 5
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Fig. 2D —48-year-old asymptomatic man with secundum atrial septal defect (ASD). Contrast-enhanced axial CT images of heart obtained from abdominal CT reveal enlargement of right ventricle (RV). LV = left ventricle.

 

Figure 6
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Fig. 2E —48-year-old asymptomatic man with secundum atrial septal defect (ASD). Still images from cine MRI four-chamber (E) and short-axis (F) views of heart show large secundum ASD (arrow). RV = right ventricle, LV = left ventricle, RA = right atrium, LA = left atrium.

 

Figure 7
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Fig. 2F —48-year-old asymptomatic man with secundum atrial septal defect (ASD). Still images from cine MRI four-chamber (E) and short-axis (F) views of heart show large secundum ASD (arrow). RV = right ventricle, LV = left ventricle, RA = right atrium, LA = left atrium.

 

Figure 8
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Fig. 3A —19-year-old woman with complaints of chest discomfort with exertion. Still images from cine MRI four-chamber (A) and short-axis (B) views of heart show communication (arrow) between left atrium (LA) and right atrium (RA) just above level of left atrioventricular valve consistent with primum atrial septal defect. RV = right ventricle, LV = left ventricle.

 

Figure 9
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Fig. 3B —19-year-old woman with complaints of chest discomfort with exertion. Still images from cine MRI four-chamber (A) and short-axis (B) views of heart show communication (arrow) between left atrium (LA) and right atrium (RA) just above level of left atrioventricular valve consistent with primum atrial septal defect. RV = right ventricle, LV = left ventricle.

 

Figure 10
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Fig. 4A —58-year-old woman with symptoms of increasing dyspnea. Still image from cine MRI four-chamber view of heart shows enlargement of right atrium (RA) and right ventricle (RV). LV = left ventricle, LA = left atrium.

 

Figure 11
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Fig. 4B —58-year-old woman with symptoms of increasing dyspnea. Bright blood axial image shows direct communication (arrow) between superior vena cava (asterisk), right upper lobe pulmonary veins, and left atrium (LA) consistent with sinus venosus defect. AA = ascending aorta.

 

Figure 12
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Fig. 4C —58-year-old woman with symptoms of increasing dyspnea. Dark blood axial image from different patient with same diagnosis shows direct communication (arrow) between superior vena cava (asterisk) and left atrium (LA) consistent with sinus venosus defect. AA = ascending aorta.

 

Figure 13
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Fig. 5A —65-year-old woman with history of breast cancer and persistent complaints of dyspnea. Axial contrast-enhanced images of chest show enlargement of right atrium (RA), right ventricle (RV), and coronary sinus (asterisk, B). LA = left atrium, LV = left ventricle.

 

Figure 14
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Fig. 5B —65-year-old woman with history of breast cancer and persistent complaints of dyspnea. Axial contrast-enhanced images of chest show enlargement of right atrium (RA), right ventricle (RV), and coronary sinus (asterisk, B). LA = left atrium, LV = left ventricle.

 

Figure 15
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Fig. 5C —65-year-old woman with history of breast cancer and persistent complaints of dyspnea. Still images from cine MRI four-chamber (C and D) and short-axis (E and F) views of heart show enlarged right atrium (RA) and right ventricle (RV). Interatrial septum is intact. Direct connection (arrow, C, D, F) between left atrium (LA) and coronary sinus (asterisk, F) was identified and was confirmed on cardiac catheterization to be a defect in coronary sinus septum. LV = left ventricle.

 

Figure 16
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Fig. 5D —65-year-old woman with history of breast cancer and persistent complaints of dyspnea. Still images from cine MRI four-chamber (C and D) and short-axis (E and F) views of heart show enlarged right atrium (RA) and right ventricle (RV). Interatrial septum is intact. Direct connection (arrow, C, D, F) between left atrium (LA) and coronary sinus (asterisk, F) was identified and was confirmed on cardiac catheterization to be a defect in coronary sinus septum. LV = left ventricle.

 

Figure 17
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Fig. 5E —65-year-old woman with history of breast cancer and persistent complaints of dyspnea. Still images from cine MRI four-chamber (C and D) and short-axis (E and F) views of heart show enlarged right atrium (RA) and right ventricle (RV). Interatrial septum is intact. Direct connection (arrow, C, D, F) between left atrium (LA) and coronary sinus (asterisk, F) was identified and was confirmed on cardiac catheterization to be a defect in coronary sinus septum. LV = left ventricle.

 

Figure 18
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Fig. 5F —65-year-old woman with history of breast cancer and persistent complaints of dyspnea. Still images from cine MRI four-chamber (C and D) and short-axis (E and F) views of heart show enlarged right atrium (RA) and right ventricle (RV). Interatrial septum is intact. Direct connection (arrow, C, D, F) between left atrium (LA) and coronary sinus (asterisk, F) was identified and was confirmed on cardiac catheterization to be a defect in coronary sinus septum. LV = left ventricle.

 

Figure 19
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Fig. 6A —57-year-old man with persistent left superior vena cava. Four-chamber (A) and sagittal oblique (B) contrast-enhanced CT images of heart show presence of IV contrast material in left superior vena cava (LSVC). Coronary sinus (asterisk) is enlarged, and right atrium (RA) and right ventricle (RV) are normal size. LA = left atrium.

 

Figure 20
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Fig. 6B —57-year-old man with persistent left superior vena cava. Four-chamber (A) and sagittal oblique (B) contrast-enhanced CT images of heart show presence of IV contrast material in left superior vena cava (LSVC). Coronary sinus (asterisk) is enlarged, and right atrium (RA) and right ventricle (RV) are normal size. LA = left atrium.

 

Figure 21
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Fig. 7A —47-year-old asymptomatic woman with partially anomalous pulmonary venous connection. Sequential axial contrast-enhanced images of chest show enlargement of right atrium (RA) and right ventricle (RV). There is abnormal drainage of right upper and right middle lobe pulmonary veins to superior vena cava (arrow, A-C) consistent with partially anomalous pulmonary venous connection. AA = ascending aorta, PA = pulmonary artery, LA = left atrium, LV = left ventricle.

 

Figure 22
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Fig. 7B —47-year-old asymptomatic woman with partially anomalous pulmonary venous connection. Sequential axial contrast-enhanced images of chest show enlargement of right atrium (RA) and right ventricle (RV). There is abnormal drainage of right upper and right middle lobe pulmonary veins to superior vena cava (arrow, A-C) consistent with partially anomalous pulmonary venous connection. AA = ascending aorta, PA = pulmonary artery, LA = left atrium, LV = left ventricle.

 

Figure 23
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Fig. 7C —47-year-old asymptomatic woman with partially anomalous pulmonary venous connection. Sequential axial contrast-enhanced images of chest show enlargement of right atrium (RA) and right ventricle (RV). There is abnormal drainage of right upper and right middle lobe pulmonary veins to superior vena cava (arrow, A-C) consistent with partially anomalous pulmonary venous connection. AA = ascending aorta, PA = pulmonary artery, LA = left atrium, LV = left ventricle.

 

Figure 24
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Fig. 7D —47-year-old asymptomatic woman with partially anomalous pulmonary venous connection. Sequential axial contrast-enhanced images of chest show enlargement of right atrium (RA) and right ventricle (RV). There is abnormal drainage of right upper and right middle lobe pulmonary veins to superior vena cava (arrow, A-C) consistent with partially anomalous pulmonary venous connection. AA = ascending aorta, PA = pulmonary artery, LA = left atrium, LV = left ventricle.

 

Figure 25
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Fig. 7E —47-year-old asymptomatic woman with partially anomalous pulmonary venous connection. Sequential axial contrast-enhanced images of chest show enlargement of right atrium (RA) and right ventricle (RV). There is abnormal drainage of right upper and right middle lobe pulmonary veins to superior vena cava (arrow, A-C) consistent with partially anomalous pulmonary venous connection. AA = ascending aorta, PA = pulmonary artery, LA = left atrium, LV = left ventricle.

 

Figure 26
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Fig. 7F —47-year-old asymptomatic woman with partially anomalous pulmonary venous connection. Sequential axial contrast-enhanced images of chest show enlargement of right atrium (RA) and right ventricle (RV). There is abnormal drainage of right upper and right middle lobe pulmonary veins to superior vena cava (arrow, A-C) consistent with partially anomalous pulmonary venous connection. AA = ascending aorta, PA = pulmonary artery, LA = left atrium, LV = left ventricle.

 

Figure 27
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Fig. 8 20-year-old woman with partially anomalous pulmonary venous return (scimitar syndrome). Posteroanterior chest radiograph shows small right hemithorax and small right hilum with abnormal curvilinear opacity that represents anomalous vein in right lower hemithorax (arrows) and dextroposition of heart due to hypoplastic right lung.

 

Figure 28
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Fig. 9A —69-year-old man with coronary artery-to-coronary sinus fistula. Axial ECG-gated CT images of heart show enlarged right atrium (RA). There is enlarged left main coronary artery connecting to large serpiginous left circumflex artery (arrow, B-D) that connects to coronary sinus (asterisk on oblique image, D). AA = ascending aorta, LA = left atrium, LV = left ventricle, RV = right ventricle.

 

Figure 29
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Fig. 9B —69-year-old man with coronary artery-to-coronary sinus fistula. Axial ECG-gated CT images of heart show enlarged right atrium (RA). There is enlarged left main coronary artery connecting to large serpiginous left circumflex artery (arrow, B-D) that connects to coronary sinus (asterisk on oblique image, D). AA = ascending aorta, LA = left atrium, LV = left ventricle, RV = right ventricle.

 

Figure 30
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Fig. 9C —69-year-old man with coronary artery-to-coronary sinus fistula. Axial ECG-gated CT images of heart show enlarged right atrium (RA). There is enlarged left main coronary artery connecting to large serpiginous left circumflex artery (arrow, B-D) that connects to coronary sinus (asterisk on oblique image, D). AA = ascending aorta, LA = left atrium, LV = left ventricle, RV = right ventricle.

 

Figure 31
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Fig. 9D —69-year-old man with coronary artery-to-coronary sinus fistula. Axial ECG-gated CT images of heart show enlarged right atrium (RA). There is enlarged left main coronary artery connecting to large serpiginous left circumflex artery (arrow, B-D) that connects to coronary sinus (asterisk on oblique image, D). AA = ascending aorta, LA = left atrium, LV = left ventricle, RV = right ventricle.

 

Figure 32
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Fig. 10A 8-year-old noncyanotic boy with history of tricuspid valve dysplasia. Posteroanterior (A) and lateral (B) chest radiographs show markedly enlarged right atrium (asterisk, A) and right ventricle (arrow, B). Pulmonary vascularity is normal.

 

Figure 33
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Fig. 10B 8-year-old noncyanotic boy with history of tricuspid valve dysplasia. Posteroanterior (A) and lateral (B) chest radiographs show markedly enlarged right atrium (asterisk, A) and right ventricle (arrow, B). Pulmonary vascularity is normal.

 

Figure 34
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Fig. 11A —30-year-old man with Ebstein's anomaly. Still images from cine MRI short-axis (A and B) and long-axis (C and D) views of heart in systole and diastole show enlarged right atrium and right ventricle (RV) with apical displacement of septal tricuspid leaflet (arrowhead, C). Anterior leaflet (arrow, C and D) is elongated and sail-like.

 

Figure 35
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Fig. 11B —30-year-old man with Ebstein's anomaly. Still images from cine MRI short-axis (A and B) and long-axis (C and D) views of heart in systole and diastole show enlarged right atrium and right ventricle (RV) with apical displacement of septal tricuspid leaflet (arrowhead, C). Anterior leaflet (arrow, C and D) is elongated and sail-like.

 

Figure 36
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Fig. 11C —30-year-old man with Ebstein's anomaly. Still images from cine MRI short-axis (A and B) and long-axis (C and D) views of heart in systole and diastole show enlarged right atrium and right ventricle (RV) with apical displacement of septal tricuspid leaflet (arrowhead, C). Anterior leaflet (arrow, C and D) is elongated and sail-like.

 

Figure 37
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Fig. 11D —30-year-old man with Ebstein's anomaly. Still images from cine MRI short-axis (A and B) and long-axis (C and D) views of heart in systole and diastole show enlarged right atrium and right ventricle (RV) with apical displacement of septal tricuspid leaflet (arrowhead, C). Anterior leaflet (arrow, C and D) is elongated and sail-like.

 

Figure 38
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Fig. 12A —47-year-old man who underwent repair of tetralogy of Fallot at age of 6 years. Posteroanterior (A) and lateral (B) chest radiographs show enlargement of right atrium (asterisk, A), right ventricle (arrow, B), and left pulmonary artery (arrowhead, A).

 

Figure 39
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Fig. 12B —47-year-old man who underwent repair of tetralogy of Fallot at age of 6 years. Posteroanterior (A) and lateral (B) chest radiographs show enlargement of right atrium (asterisk, A), right ventricle (arrow, B), and left pulmonary artery (arrowhead, A).

 

Figure 40
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Fig. 12C —47-year-old man who underwent repair of tetralogy of Fallot at age of 6 years. Still images from cine MRI long-axis views of heart show enlarged right atrium (RA) and right ventricle (RV) with severe pulmonary valve insufficiency jet (arrow, D). PA = pulmonary artery.

 

Figure 41
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Fig. 12D —47-year-old man who underwent repair of tetralogy of Fallot at age of 6 years. Still images from cine MRI long-axis views of heart show enlarged right atrium (RA) and right ventricle (RV) with severe pulmonary valve insufficiency jet (arrow, D). PA = pulmonary artery.

 

Figure 42
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Fig. 13A 60-year-old man with recurrent right ventricular tachycardia. Still images from cine MRI short-axis (A) and long-axis (B) views of heart in systole show enlargement of right ventricle (RV) with focal aneurysm (arrow, B) consistent with diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. LV = left ventricle.

 

Figure 43
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Fig. 13B 60-year-old man with recurrent right ventricular tachycardia. Still images from cine MRI short-axis (A) and long-axis (B) views of heart in systole show enlargement of right ventricle (RV) with focal aneurysm (arrow, B) consistent with diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. LV = left ventricle.

 

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