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Thrombosed Giant Left Atrium Mimicking a Mediastinal Tumor

1977; Elsevier BV; Volume: 71; Issue: 3 Linguagem: Inglês

10.1378/chest.71.3.406

1931-3543

D Rimon, Leon Cohen, Joseph B. Rosenfeld,

Cardiac Structural Anomalies and Repair

A patient with rheumatic heart disease, mitral stenosis, and mitral insufficiency is described. The thrombosed giant left atrium paralyzed the left vocal cord and completely obstructed the bronchi to the middle and lower lobes of the right lung. The giant left atrium mimicked a mediastinal tumor on the chest x-ray film. A patient with rheumatic heart disease, mitral stenosis, and mitral insufficiency is described. The thrombosed giant left atrium paralyzed the left vocal cord and completely obstructed the bronchi to the middle and lower lobes of the right lung. The giant left atrium mimicked a mediastinal tumor on the chest x-ray film. A huge left atrium is a rare finding in rheumatic mitral disease. The reported incidence is 0.2 percent of patients with rheumatic heart disease.1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar Most of the cases have combined mitral disease with predominant insufficiency.1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar, 2Priest EA Finlayson JK Short DS The x-ray manifestations in the heart and lungs of mitral regurgitation.Prog Cardiovasc Dis. 1962; 5: 219Abstract Full Text PDF PubMed Scopus (3) Google Scholar, 3Plaschkes J Borman JB Merin G et al.Giant left atrium in rheumatic heart disease.Ann Surg. 1971; 174: 194Crossref PubMed Scopus (25) Google Scholar Only in a minority of patients is pure insufficiency3Plaschkes J Borman JB Merin G et al.Giant left atrium in rheumatic heart disease.Ann Surg. 1971; 174: 194Crossref PubMed Scopus (25) Google Scholar, 4Parsonnet AE Bernstein A Martland HS Massive left auricle with special reference to its etiology and mechanism.Am Heart J. 1946; 31: 438Abstract Full Text PDF PubMed Scopus (6) Google Scholar or pure stenosis5Best PB Heath D The right ventricle and small pulmonary arteries in aneurysmal dilatation of the left atrium.Br Heart J. 1964; 26: 312Crossref PubMed Google Scholar, 6Kent EM Fischer DL Ford WB et al.Mitral valve surgery and left heart catheterization in giant left atrium.Arch Surg. 1956; 73: 503Crossref Scopus (7) Google Scholar, 7Venner A Massive left atrium and mitral valvotomy.Br Med J. 1954; 1: 1359Crossref PubMed Scopus (1) Google Scholar of the mitral valve found. Most of the cases show a conspicuous elevation of the left main bronchus and a wide angle of the carina on the x-ray films. Different grades of pressure can be seen on the main bronchi.8Sosman MC Roentgenological aspects of acquired valvular heart disease.Am J Roentgenol. 1939; 42: 47Google Scholar, 9Nichols CF Ostrum HW Unusual dilatation of the left auricle.Am Heart J. 1933; 8: 205Abstract Full Text PDF Scopus (7) Google Scholar Only very rarely10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar, 11Bach F Keith TS Enlargement of the left auricle of the heart.Lancet. 1929; 2: 766Abstract Scopus (3) Google Scholar has complete bronchial obstruction with atelectasis been reported. A more common finding is atelectasis caused by direct pressure of the enlarged heart on the lungs.1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar, 12Ashworth H Jones AM Aneurysmal dilatation of the left auricle with erosion of the spine.Br Heart J. 1946; 9: 207Crossref Scopus (7) Google ScholarA patient with a giant left atrium is described, in whom paralysis of the left vocal cord and obstruction of the main bronchi of the middle and lower lobes of the right lung were found. These findings, because of their rarity, can lead to the erroneous diagnosis of a mediastinal tumor.Case ReportA 61-year-old man was hospitalized because of dyspnea, productive cough, and chest pain. He was a heavy smoker and suffered from chronic cough.Twelve years previously, a heart murmur, irregular pulse, and congestive heart failure were found. A year before the present hospitalization, progressive hoarseness appeared. Physical examination revealed a man in a bad nutritional state, with hoarseness, cyanosis of the lips, dyspnea, and orthopnea. The chest was emphysematous. The respiratory sounds were well heard, even with heavy breathing. The blood pressure was 120/85 mm Hg. The pulse was completely irregular, with an average rate of 100 beats per minute. An uplift was found at the cardiac apex and at the right ventricle, with retraction in between the two. A pansystolic murmur was heard at the apex and lower sternum. The anterior lower part of the right side of the chest was flat on percussion. The liver was tender and was palpable 4 cm under the right costal margin. The electrocardiogram revealed atrial fibrillation. Cultures of the sputum grew Pseudomonas aeruginosa and Escherichia coli; no malignant cells were found.On the x-ray films of the chest, congested lungs, enlarged hili and, a bilateral pleural effusion were seen. The vascularization at the periphery of the lungs was poor. The cardiac silhouette was enormous (Fig 1). Tomograms of the right lung revealed a wide angle of the carina. The bronchus to the middle lobes narrowed progressively and was obstructed at its middle portion. The bronchus to the lower lobe was obstructed at its proximal part. Both of the obstructions seemed to be due to a mass in the mediastinum (Fig 2). On the upper gastrointestinal series, the lower esophagus and the fundus of the stomach were displaced by a space-occupying lesion on the right side.Figure 2Tomogram showing obstruction of bronchi to middle and lower lobes of right lung.View Large Image Figure ViewerDownload (PPT)The patient was treated with digoxin, furosemide, spironolactone (Aldactone), and ampicillin. On the third day, purpura appeared on the gluteal surface and the extensor surfaces of the limbs, accompanied by arthralgia and rectal bleeding. The blood urea level rose from 60 mg/100 ml to 200 mg/ml (normal, ≤ 40 mg/100 ml). A Henoch-Schölein syndrome was diagnosed. The therapy with ampicillin was discontinued, and treatment with hydrocortisone was started. The congestive heart failure progressively worsened, and despite increasing doses of furosemide, the patient died in a state of pulmonary edema.At the postmortem examination, the heart was extremely enlarged and occupied most of the thoracic cage. After the pericardium was opened, the right atrium and both ventricles were seen. The left atrium could not be seen from this anterior view of the heart. Only after lifting the heart could the enormous left atrium be seen. This giant left atrium reached dimensions of 15 × 15 × 20 cm. The whole heart was rotated posteriorly and to the right and pressed the main bronchus of the right lung. The heart weighed 1.3 kg. The left atrium was almost completely filled with a mural thrombus (Fig 3). The mitral valve showed both stenosis and insufficiency. Hypertrophy and enlargement of the right atrium and both ventricles, with a relative tricuspid insufficiency, were observed. No tumor was found in the mediastinum. The kidney showed focal segmental necrotizing glomerulonephritis.Figure 3Huge thrombus in left atrium.View Large Image Figure ViewerDownload (PPT)DiscussionThe differential diagnosis in this case was between rheumatic heart disease with mitral insufficiency with a giant left atrium and a mediastinal tumor which paralyzed the left recurrent laryngeal nerve, obstructed the lower and middle bronchi of the right lung, and displaced the lower esophagus and fundus of the stomach. Paralysis of the left vocal cord in patients with a giant left atrium is very rare. Only one out of ten patients described by DeSantis et al1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar and one out of 15 patients described by Daley and Franks10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar had hoarseness.Complete bronchial obstruction by a giant left atrium is even rarer. Daley and Franks10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar described a patient with complete obstruction of the bronchus to the right middle lobe. This finding was proved by bronchoscopic and bronchographic studies. Paralysis of the left vocal cord and bronchial obstruction caused by a giant left atrium have never been described in the same patient.Only mitral insufficiency was diagnosed before death; however, the autopsy revealed the presence of severe mitral stenosis. Surawicz and Nierenberg13Surawicz B Nierenberg MA Association of “silent” mitral stenosis with massive thrombi in the left atrium.N Engl J Med. 1960; 263: 423Crossref PubMed Scopus (5) Google Scholar described four patients with mitral stenosis and a large thrombus in the left atrium, which were not diagnosed ante mortem because no diastolic murmur was heard; in two of their cases, even the opening snap was absent. The big thrombus in the left atrium of our patient could have been one of the reasons for the disappearance of the characteristic auscultatory findings of mitral stenosis. There is no doubt that echocardiographic studies would have been of value in making the correct diagnosis. Unfortunately, no echocardiograph was available at that time.The absence of the clinical findings of mitral stenosis, the absence of thromboembolic phenomena, syncope, and changing murmurs did not permit us to diagnose the presence of a thrombus in the left atrium. No doubt, the right heart failure and the massive left atrial thrombosis present in our patient contributed to the pleural effusion.14Dorney ER Cabaud PG Massive left atrial thrombosis and recurring pleural effusion.Am Heart J. 1954; 48: 459Abstract Full Text PDF PubMed Scopus (1) Google Scholar It is to be emphasized that the huge atrial thrombus so filled the enlarged left atrium that it was impossible to differentiate it from a mediastinal tumor.The case described suggests that in the differential diagnosis of a mediastinal mass, the possibility of a massive left atrial thrombus should be considered. A huge left atrium is a rare finding in rheumatic mitral disease. The reported incidence is 0.2 percent of patients with rheumatic heart disease.1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar Most of the cases have combined mitral disease with predominant insufficiency.1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar, 2Priest EA Finlayson JK Short DS The x-ray manifestations in the heart and lungs of mitral regurgitation.Prog Cardiovasc Dis. 1962; 5: 219Abstract Full Text PDF PubMed Scopus (3) Google Scholar, 3Plaschkes J Borman JB Merin G et al.Giant left atrium in rheumatic heart disease.Ann Surg. 1971; 174: 194Crossref PubMed Scopus (25) Google Scholar Only in a minority of patients is pure insufficiency3Plaschkes J Borman JB Merin G et al.Giant left atrium in rheumatic heart disease.Ann Surg. 1971; 174: 194Crossref PubMed Scopus (25) Google Scholar, 4Parsonnet AE Bernstein A Martland HS Massive left auricle with special reference to its etiology and mechanism.Am Heart J. 1946; 31: 438Abstract Full Text PDF PubMed Scopus (6) Google Scholar or pure stenosis5Best PB Heath D The right ventricle and small pulmonary arteries in aneurysmal dilatation of the left atrium.Br Heart J. 1964; 26: 312Crossref PubMed Google Scholar, 6Kent EM Fischer DL Ford WB et al.Mitral valve surgery and left heart catheterization in giant left atrium.Arch Surg. 1956; 73: 503Crossref Scopus (7) Google Scholar, 7Venner A Massive left atrium and mitral valvotomy.Br Med J. 1954; 1: 1359Crossref PubMed Scopus (1) Google Scholar of the mitral valve found. Most of the cases show a conspicuous elevation of the left main bronchus and a wide angle of the carina on the x-ray films. Different grades of pressure can be seen on the main bronchi.8Sosman MC Roentgenological aspects of acquired valvular heart disease.Am J Roentgenol. 1939; 42: 47Google Scholar, 9Nichols CF Ostrum HW Unusual dilatation of the left auricle.Am Heart J. 1933; 8: 205Abstract Full Text PDF Scopus (7) Google Scholar Only very rarely10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar, 11Bach F Keith TS Enlargement of the left auricle of the heart.Lancet. 1929; 2: 766Abstract Scopus (3) Google Scholar has complete bronchial obstruction with atelectasis been reported. A more common finding is atelectasis caused by direct pressure of the enlarged heart on the lungs.1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar, 12Ashworth H Jones AM Aneurysmal dilatation of the left auricle with erosion of the spine.Br Heart J. 1946; 9: 207Crossref Scopus (7) Google Scholar A patient with a giant left atrium is described, in whom paralysis of the left vocal cord and obstruction of the main bronchi of the middle and lower lobes of the right lung were found. These findings, because of their rarity, can lead to the erroneous diagnosis of a mediastinal tumor. Case ReportA 61-year-old man was hospitalized because of dyspnea, productive cough, and chest pain. He was a heavy smoker and suffered from chronic cough.Twelve years previously, a heart murmur, irregular pulse, and congestive heart failure were found. A year before the present hospitalization, progressive hoarseness appeared. Physical examination revealed a man in a bad nutritional state, with hoarseness, cyanosis of the lips, dyspnea, and orthopnea. The chest was emphysematous. The respiratory sounds were well heard, even with heavy breathing. The blood pressure was 120/85 mm Hg. The pulse was completely irregular, with an average rate of 100 beats per minute. An uplift was found at the cardiac apex and at the right ventricle, with retraction in between the two. A pansystolic murmur was heard at the apex and lower sternum. The anterior lower part of the right side of the chest was flat on percussion. The liver was tender and was palpable 4 cm under the right costal margin. The electrocardiogram revealed atrial fibrillation. Cultures of the sputum grew Pseudomonas aeruginosa and Escherichia coli; no malignant cells were found.On the x-ray films of the chest, congested lungs, enlarged hili and, a bilateral pleural effusion were seen. The vascularization at the periphery of the lungs was poor. The cardiac silhouette was enormous (Fig 1). Tomograms of the right lung revealed a wide angle of the carina. The bronchus to the middle lobes narrowed progressively and was obstructed at its middle portion. The bronchus to the lower lobe was obstructed at its proximal part. Both of the obstructions seemed to be due to a mass in the mediastinum (Fig 2). On the upper gastrointestinal series, the lower esophagus and the fundus of the stomach were displaced by a space-occupying lesion on the right side.The patient was treated with digoxin, furosemide, spironolactone (Aldactone), and ampicillin. On the third day, purpura appeared on the gluteal surface and the extensor surfaces of the limbs, accompanied by arthralgia and rectal bleeding. The blood urea level rose from 60 mg/100 ml to 200 mg/ml (normal, ≤ 40 mg/100 ml). A Henoch-Schölein syndrome was diagnosed. The therapy with ampicillin was discontinued, and treatment with hydrocortisone was started. The congestive heart failure progressively worsened, and despite increasing doses of furosemide, the patient died in a state of pulmonary edema.At the postmortem examination, the heart was extremely enlarged and occupied most of the thoracic cage. After the pericardium was opened, the right atrium and both ventricles were seen. The left atrium could not be seen from this anterior view of the heart. Only after lifting the heart could the enormous left atrium be seen. This giant left atrium reached dimensions of 15 × 15 × 20 cm. The whole heart was rotated posteriorly and to the right and pressed the main bronchus of the right lung. The heart weighed 1.3 kg. The left atrium was almost completely filled with a mural thrombus (Fig 3). The mitral valve showed both stenosis and insufficiency. Hypertrophy and enlargement of the right atrium and both ventricles, with a relative tricuspid insufficiency, were observed. No tumor was found in the mediastinum. The kidney showed focal segmental necrotizing glomerulonephritis.Figure 3Huge thrombus in left atrium.View Large Image Figure ViewerDownload (PPT) A 61-year-old man was hospitalized because of dyspnea, productive cough, and chest pain. He was a heavy smoker and suffered from chronic cough. Twelve years previously, a heart murmur, irregular pulse, and congestive heart failure were found. A year before the present hospitalization, progressive hoarseness appeared. Physical examination revealed a man in a bad nutritional state, with hoarseness, cyanosis of the lips, dyspnea, and orthopnea. The chest was emphysematous. The respiratory sounds were well heard, even with heavy breathing. The blood pressure was 120/85 mm Hg. The pulse was completely irregular, with an average rate of 100 beats per minute. An uplift was found at the cardiac apex and at the right ventricle, with retraction in between the two. A pansystolic murmur was heard at the apex and lower sternum. The anterior lower part of the right side of the chest was flat on percussion. The liver was tender and was palpable 4 cm under the right costal margin. The electrocardiogram revealed atrial fibrillation. Cultures of the sputum grew Pseudomonas aeruginosa and Escherichia coli; no malignant cells were found. On the x-ray films of the chest, congested lungs, enlarged hili and, a bilateral pleural effusion were seen. The vascularization at the periphery of the lungs was poor. The cardiac silhouette was enormous (Fig 1). Tomograms of the right lung revealed a wide angle of the carina. The bronchus to the middle lobes narrowed progressively and was obstructed at its middle portion. The bronchus to the lower lobe was obstructed at its proximal part. Both of the obstructions seemed to be due to a mass in the mediastinum (Fig 2). On the upper gastrointestinal series, the lower esophagus and the fundus of the stomach were displaced by a space-occupying lesion on the right side. The patient was treated with digoxin, furosemide, spironolactone (Aldactone), and ampicillin. On the third day, purpura appeared on the gluteal surface and the extensor surfaces of the limbs, accompanied by arthralgia and rectal bleeding. The blood urea level rose from 60 mg/100 ml to 200 mg/ml (normal, ≤ 40 mg/100 ml). A Henoch-Schölein syndrome was diagnosed. The therapy with ampicillin was discontinued, and treatment with hydrocortisone was started. The congestive heart failure progressively worsened, and despite increasing doses of furosemide, the patient died in a state of pulmonary edema. At the postmortem examination, the heart was extremely enlarged and occupied most of the thoracic cage. After the pericardium was opened, the right atrium and both ventricles were seen. The left atrium could not be seen from this anterior view of the heart. Only after lifting the heart could the enormous left atrium be seen. This giant left atrium reached dimensions of 15 × 15 × 20 cm. The whole heart was rotated posteriorly and to the right and pressed the main bronchus of the right lung. The heart weighed 1.3 kg. The left atrium was almost completely filled with a mural thrombus (Fig 3). The mitral valve showed both stenosis and insufficiency. Hypertrophy and enlargement of the right atrium and both ventricles, with a relative tricuspid insufficiency, were observed. No tumor was found in the mediastinum. The kidney showed focal segmental necrotizing glomerulonephritis. DiscussionThe differential diagnosis in this case was between rheumatic heart disease with mitral insufficiency with a giant left atrium and a mediastinal tumor which paralyzed the left recurrent laryngeal nerve, obstructed the lower and middle bronchi of the right lung, and displaced the lower esophagus and fundus of the stomach. Paralysis of the left vocal cord in patients with a giant left atrium is very rare. Only one out of ten patients described by DeSantis et al1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar and one out of 15 patients described by Daley and Franks10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar had hoarseness.Complete bronchial obstruction by a giant left atrium is even rarer. Daley and Franks10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar described a patient with complete obstruction of the bronchus to the right middle lobe. This finding was proved by bronchoscopic and bronchographic studies. Paralysis of the left vocal cord and bronchial obstruction caused by a giant left atrium have never been described in the same patient.Only mitral insufficiency was diagnosed before death; however, the autopsy revealed the presence of severe mitral stenosis. Surawicz and Nierenberg13Surawicz B Nierenberg MA Association of “silent” mitral stenosis with massive thrombi in the left atrium.N Engl J Med. 1960; 263: 423Crossref PubMed Scopus (5) Google Scholar described four patients with mitral stenosis and a large thrombus in the left atrium, which were not diagnosed ante mortem because no diastolic murmur was heard; in two of their cases, even the opening snap was absent. The big thrombus in the left atrium of our patient could have been one of the reasons for the disappearance of the characteristic auscultatory findings of mitral stenosis. There is no doubt that echocardiographic studies would have been of value in making the correct diagnosis. Unfortunately, no echocardiograph was available at that time.The absence of the clinical findings of mitral stenosis, the absence of thromboembolic phenomena, syncope, and changing murmurs did not permit us to diagnose the presence of a thrombus in the left atrium. No doubt, the right heart failure and the massive left atrial thrombosis present in our patient contributed to the pleural effusion.14Dorney ER Cabaud PG Massive left atrial thrombosis and recurring pleural effusion.Am Heart J. 1954; 48: 459Abstract Full Text PDF PubMed Scopus (1) Google Scholar It is to be emphasized that the huge atrial thrombus so filled the enlarged left atrium that it was impossible to differentiate it from a mediastinal tumor.The case described suggests that in the differential diagnosis of a mediastinal mass, the possibility of a massive left atrial thrombus should be considered. The differential diagnosis in this case was between rheumatic heart disease with mitral insufficiency with a giant left atrium and a mediastinal tumor which paralyzed the left recurrent laryngeal nerve, obstructed the lower and middle bronchi of the right lung, and displaced the lower esophagus and fundus of the stomach. Paralysis of the left vocal cord in patients with a giant left atrium is very rare. Only one out of ten patients described by DeSantis et al1DeSantis RW Dean DC Bland EF Extreme left atrial enlargement.Circulation. 1964; 29: 14Crossref PubMed Scopus (27) Google Scholar and one out of 15 patients described by Daley and Franks10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar had hoarseness. Complete bronchial obstruction by a giant left atrium is even rarer. Daley and Franks10Daley R Franks R Massive dilatation of left auricle.Q J Med. 1949; 18: 81PubMed Google Scholar described a patient with complete obstruction of the bronchus to the right middle lobe. This finding was proved by bronchoscopic and bronchographic studies. Paralysis of the left vocal cord and bronchial obstruction caused by a giant left atrium have never been described in the same patient. Only mitral insufficiency was diagnosed before death; however, the autopsy revealed the presence of severe mitral stenosis. Surawicz and Nierenberg13Surawicz B Nierenberg MA Association of “silent” mitral stenosis with massive thrombi in the left atrium.N Engl J Med. 1960; 263: 423Crossref PubMed Scopus (5) Google Scholar described four patients with mitral stenosis and a large thrombus in the left atrium, which were not diagnosed ante mortem because no diastolic murmur was heard; in two of their cases, even the opening snap was absent. The big thrombus in the left atrium of our patient could have been one of the reasons for the disappearance of the characteristic auscultatory findings of mitral stenosis. There is no doubt that echocardiographic studies would have been of value in making the correct diagnosis. Unfortunately, no echocardiograph was available at that time. The absence of the clinical findings of mitral stenosis, the absence of thromboembolic phenomena, syncope, and changing murmurs did not permit us to diagnose the presence of a thrombus in the left atrium. No doubt, the right heart failure and the massive left atrial thrombosis present in our patient contributed to the pleural effusion.14Dorney ER Cabaud PG Massive left atrial thrombosis and recurring pleural effusion.Am Heart J. 1954; 48: 459Abstract Full Text PDF PubMed Scopus (1) Google Scholar It is to be emphasized that the huge atrial thrombus so filled the enlarged left atrium that it was impossible to differentiate it from a mediastinal tumor. The case described suggests that in the differential diagnosis of a mediastinal mass, the possibility of a massive left atrial thrombus should be considered.