Displacement of the Heart by a Giant Mediastinal Cyst
PAUL C . KOCH ITZHAK KRONZON, MD HOWARD E . WINER, MD PETER ADAMS, MD MAX TRUBEK, MD
A giant mediastinal cyst caused marked cardiac displacement, factitious cardiomegaly and clinical and hemodynamic findings suggestive of constrictive pericarditis. The correct diagnosis was established with echocardiography and confirmed with angiography and surgery .
New York, New York Echocardiography is of proved benefit in diagnosing a wide variety of structural cardiac anomalies . Its value in assessing cardiomegaly has been emphasized and it has also been successfully used to identify extracardiac anomalies simulating primary cardiac disease . This report exemplifies its utility in the latter setting .
Case Report
From the Departments of Medicine and Surgery, New York University Medical Center, New York, New York . Manuscript received December 27, 1976 ; revised manuscript received February 14, 1977, accepted February 16, 1977 . Address for reprints : Itzhak Kronzon, MD, Room 618, 550 First Avenue, New York, New York 10016 .
A 57 year old white woman was referred for evaluation of chest discomfort, dyspnea on exertion and ankle edema of 1 year's duration . There was no history of acute rheumatic fever, heart murmur, hypertension, myocardial infarction or chest trauma . Physical examination revealed a regular pulse of 80/min . The blood pressure was 130/80 mm Hg with 6 mm Hg of paradoxical pulse . The neck veins were distended 6 cm above the clavicles at 45° . The hepatojugular reflux was absent . The chest was clear to percussion and auscultation . A systolic impulse was present at the second right intercostal space and right sternal border. A pulsatile mass and a systolic thrill were noted in the right supraclavicular fossa . The heart sounds were normal but decreased in intensity from the left sternal border to the normal location of the cardiac apex . A loud systolic ejection murmur was audible over the entire precordium and was maximal over the pulsating mass in the right supraclavicular region . A smooth liver edge was palpable 3 cm below the right costal margin and was nonpulsatile . There was no ascites, but mild ankle edema was present . The peripheral pulses were all normal . The electrocardiogram revealed normal sinus rhythm, low voltage in leads V2 to V6 and mild nonspecific T wave changes with little change in configuration from leads VZ to Vs. The chest roentgenogram (Fig . 1A) revealed apparent cardiomegaly, a mass in the right upper lung field and a left pleural effusion. Initial diagnostic considerations included cardiomyopathy, aortic aneurysm and pericardial tamponade . A standard M mode single transducer echocardiogram was obtained with the transducer located in the third and fourth left intercostal spaces, 1 cm from the left sternal border. Instead of the normal cardiac echoes, a large sharply demarcated echo-free space was identified . It measured 10 cm in the anteroposterior diameter and 8 cm in the lateral diameter (Fig . 2) . With the transducer located over the pulsating mass in the second right interspace, a normal echocardiogram was recorded . It revealed normal chamber size, wall thickness and motion and normal aortic and mitral valve motion (Fig . 3) . Pericardial effusion was not present. This study suggested that a huge cystic mass had displaced the heart to the right infraclavicular region . Cardiac catheterization was performed to investigate the anatomy and hemodynamic consequences of this cystic mass . The study revealed equalization of diastolic intracardiac pressures . Right atrial mean pressure (mm Hg) was 14,
September 1977 The American Journal of CARDIOLOGY Volume 40
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GIANT MEDIASTINAL CYST-KOCH ET AL.
FIGURE 1 . Chest roentgenograms . A, preoperative . The cyst margin is designated by the black arrows ; the white arrows mark the displaced cardiac silhouette . B, postoperative .
right ventricular pressure 34/14, pulmonary arterial pressure 36/15, pulmonary capillary wedge pressure 14 and left ventricular pressure 130/14 . The right atrial pressure had a rapid y descent, and the right ventricular diastolic curve conformed to the "dip and plateau" pattern of pericardial constriction . The displacement of the heart indicated in the echocardiogram was confirmed with left ventricular angiography . The left ventricle was deformed by a large nonopacified mass . The patient underwent thoracotomy and a large anterior med iastinal mass was removed . The heart gradually returned to a more normal position (Fig. 113) . Examination of the mass (Fig . 4) revealed it to be a large benign fibrous cyst with organized hemorrhage on its inner surface . Grayish-red mucilaginous debris was found in the center of the cyst. No epithelial lining was present . The final pathologic diagnosis was
organized hemorrhage that had undergone cavitation and fibrosis, The patient did well postoperatively. Her symptoms were completely relieved and the murmur disappeared . Central venous pressure returned to normal immediately after operation . A repeat echocardiogram, with the transducer in the usual location, revealed normal cardiac structures and motion . The electrocardiogram also returned to normal . The patient was discharged on the 10th postoperative day .
Discussion The differential diagnosis of mediastinal masses remains a difficult clinical problem . Mediastinal and pericardial masses may simulate many cardiac anom-
FIGURE 2 . Echocardiogram obtained with the transducer placed in the fourth intercostal space at the left sternal border, showing a large echo-free space but no cardiac structures .
4 46
September 1977 The American Journal of CARDIOLOGY Volume 40
GIANT MEDIASTINAL CYST-KOCH ET AL .
FIGURE & Echocardiograms obtained with the transducer placed in the second intercostal space, right midclavicular line showing normal aorta (Ao), aortic valve (A0V), left atrium (LA), left ventricle (LV) and mitral valve (MV) .
alies and have been misinterpreted as representing cardiomegaly, aortic aneurysm' and pericardial effusion . 2 The origin of these masses can be lymphoreticular, neurogenic, teratomatous, pericardial, vascular, bronchogenic, enteric and mesenchymal. 3 ' 4 A case has been reported of an esophageal duplication cyst that caused precordial and upper abdominal pain .' Cardiovascular hemodynamics may be significantly altered by mediastinal masses . Tumors located near the heart and great vessels can produce signs and symptoms that mimic tricuspid stenosis, 6 pulmonary stenosis 7 and constrictive pericarditis . 8 Invasive techniques are often required to provide accurate evaluation of these tumors . Several recent reports, 8 - 1° including one from this laboratory, have stressed the use of M and B mode echo-
cardiography in the differential diagnosis of such tumors . This case was remarkable because of the displacement of the heart into the right upper quadrant of the chest . The correct diagnosis was inapparent from the chest roentgenogram because the cyst could not easily be differentiated from a normally located but enlarged heart . Echocardiography demonstrated the actual location of the heart and revealed the cystic nature of the tumor and provided accurate data on its internal dimensions . Our case also demonstrates that mediastinal masses may produce hemodynamic alterations . Increased diastolic intracardiac pressures, identical to the pattern seen in constrictive pericarditis, have been seen in a case of cystic thymoma.8 In our case, the heart
September 1977
The American Journal of CARDIOLOGY
Volume 40
447
GIANT MEDIASTINAL CYST-KOCH ET AL.
FIGURE 4 . The opened cyst after removal from the thorax .
was pinioned between the cyst and the chest wall. Although the right heart pressures were only moderately elevated, the diastolic equalization of right and left heart pressures suggests an element of constriction . The patient's murmur may have been due to "kinking" of the great vessels by abnormal cardiac position or pressure on the right or left ventricular outflow tracts . The dyspnea may he explained by the increased right atrial pressure and the large intrathoracic mass that decreased
vital capacity . The origin of this lesion is obscure . It is unclear how long the patient had this mass in her chest ; no history of trauma, previous surgery or bleeding diathesis was elicited . Our case illustrates the valuable diagnostic role of echocardiography in clarifying the actual location and normalcy of the displaced heart and the cystic nature of the mass that shifted it .
References 1 . Oldham HN, Sabiston DC : Primary tumors and cysts of the mediastinum presenting as cardiovascular abnormalities . Arch Surg 96 :71-75, 1968 2 . Allee G, Logue B, Mansour K : Thymic cyst simulating multiple cardiovascular abnormalities and presenting with pericarditis and pericardial tamponade . Am J Cardiol 31 :377-380, 1973 3 . Benjamin SP, McCormack LJ, EKler DE, et al : Primary tumors of the mediastinum . Chest 62 :297-303, 1972 4 . Fine G : Primary tumors of the pericardium and heart . Cardiovasc Clin 5 :207-238, 1973 5 . Fallazadeh H, Haiderer 0 : Esophageal duplication cyst with unusual manifestations . Chest 63 :827-828, 1973 6 . Pitt A, Cutforth RH, Bender HW, et al : Intrapericardial cyst for-
4 48
September 1977 The American Journal of CARDIOLOGY
mation in constrictive pericarditis simulating tricuspid stenosis . Circulation 40 :665-672, 1969 7 . Shaver VC, Bailey WR, Marrangoni AG : Acquired pulmonic stenosis due to external cardiac compression . Am J Cardiol 16 : 256-261,1965 8 . Schloss M, Kronzon I, Gelber PM, at al : Cystic thymoma simulating constrictive pericarditis . J Thorac Cardiovasc Surg 70 :143-146, 1975 9 . Peterson DT, Zatz LM, Popp RL : Pericardial cyst ten years after acute pericarditis . Chest 67:719-721, 1975 10 . Friday RO : Paracardiac cyst : diagnosis by ultrasound and puncture . JAMA 226 :82-83, 1973
Volume 40
PAUL C . KOCH ITZHAK KRONZON, MD HOWARD E . WINER, MD PETER ADAMS, MD MAX TRUBEK, MD
A giant mediastinal cyst caused marked cardiac displacement, factitious cardiomegaly and clinical and hemodynamic findings suggestive of constrictive pericarditis. The correct diagnosis was established with echocardiography and confirmed with angiography and surgery .
New York, New York Echocardiography is of proved benefit in diagnosing a wide variety of structural cardiac anomalies . Its value in assessing cardiomegaly has been emphasized and it has also been successfully used to identify extracardiac anomalies simulating primary cardiac disease . This report exemplifies its utility in the latter setting .
Case Report
From the Departments of Medicine and Surgery, New York University Medical Center, New York, New York . Manuscript received December 27, 1976 ; revised manuscript received February 14, 1977, accepted February 16, 1977 . Address for reprints : Itzhak Kronzon, MD, Room 618, 550 First Avenue, New York, New York 10016 .
A 57 year old white woman was referred for evaluation of chest discomfort, dyspnea on exertion and ankle edema of 1 year's duration . There was no history of acute rheumatic fever, heart murmur, hypertension, myocardial infarction or chest trauma . Physical examination revealed a regular pulse of 80/min . The blood pressure was 130/80 mm Hg with 6 mm Hg of paradoxical pulse . The neck veins were distended 6 cm above the clavicles at 45° . The hepatojugular reflux was absent . The chest was clear to percussion and auscultation . A systolic impulse was present at the second right intercostal space and right sternal border. A pulsatile mass and a systolic thrill were noted in the right supraclavicular fossa . The heart sounds were normal but decreased in intensity from the left sternal border to the normal location of the cardiac apex . A loud systolic ejection murmur was audible over the entire precordium and was maximal over the pulsating mass in the right supraclavicular region . A smooth liver edge was palpable 3 cm below the right costal margin and was nonpulsatile . There was no ascites, but mild ankle edema was present . The peripheral pulses were all normal . The electrocardiogram revealed normal sinus rhythm, low voltage in leads V2 to V6 and mild nonspecific T wave changes with little change in configuration from leads VZ to Vs. The chest roentgenogram (Fig . 1A) revealed apparent cardiomegaly, a mass in the right upper lung field and a left pleural effusion. Initial diagnostic considerations included cardiomyopathy, aortic aneurysm and pericardial tamponade . A standard M mode single transducer echocardiogram was obtained with the transducer located in the third and fourth left intercostal spaces, 1 cm from the left sternal border. Instead of the normal cardiac echoes, a large sharply demarcated echo-free space was identified . It measured 10 cm in the anteroposterior diameter and 8 cm in the lateral diameter (Fig . 2) . With the transducer located over the pulsating mass in the second right interspace, a normal echocardiogram was recorded . It revealed normal chamber size, wall thickness and motion and normal aortic and mitral valve motion (Fig . 3) . Pericardial effusion was not present. This study suggested that a huge cystic mass had displaced the heart to the right infraclavicular region . Cardiac catheterization was performed to investigate the anatomy and hemodynamic consequences of this cystic mass . The study revealed equalization of diastolic intracardiac pressures . Right atrial mean pressure (mm Hg) was 14,
September 1977 The American Journal of CARDIOLOGY Volume 40
445
GIANT MEDIASTINAL CYST-KOCH ET AL.
FIGURE 1 . Chest roentgenograms . A, preoperative . The cyst margin is designated by the black arrows ; the white arrows mark the displaced cardiac silhouette . B, postoperative .
right ventricular pressure 34/14, pulmonary arterial pressure 36/15, pulmonary capillary wedge pressure 14 and left ventricular pressure 130/14 . The right atrial pressure had a rapid y descent, and the right ventricular diastolic curve conformed to the "dip and plateau" pattern of pericardial constriction . The displacement of the heart indicated in the echocardiogram was confirmed with left ventricular angiography . The left ventricle was deformed by a large nonopacified mass . The patient underwent thoracotomy and a large anterior med iastinal mass was removed . The heart gradually returned to a more normal position (Fig. 113) . Examination of the mass (Fig . 4) revealed it to be a large benign fibrous cyst with organized hemorrhage on its inner surface . Grayish-red mucilaginous debris was found in the center of the cyst. No epithelial lining was present . The final pathologic diagnosis was
organized hemorrhage that had undergone cavitation and fibrosis, The patient did well postoperatively. Her symptoms were completely relieved and the murmur disappeared . Central venous pressure returned to normal immediately after operation . A repeat echocardiogram, with the transducer in the usual location, revealed normal cardiac structures and motion . The electrocardiogram also returned to normal . The patient was discharged on the 10th postoperative day .
Discussion The differential diagnosis of mediastinal masses remains a difficult clinical problem . Mediastinal and pericardial masses may simulate many cardiac anom-
FIGURE 2 . Echocardiogram obtained with the transducer placed in the fourth intercostal space at the left sternal border, showing a large echo-free space but no cardiac structures .
4 46
September 1977 The American Journal of CARDIOLOGY Volume 40
GIANT MEDIASTINAL CYST-KOCH ET AL .
FIGURE & Echocardiograms obtained with the transducer placed in the second intercostal space, right midclavicular line showing normal aorta (Ao), aortic valve (A0V), left atrium (LA), left ventricle (LV) and mitral valve (MV) .
alies and have been misinterpreted as representing cardiomegaly, aortic aneurysm' and pericardial effusion . 2 The origin of these masses can be lymphoreticular, neurogenic, teratomatous, pericardial, vascular, bronchogenic, enteric and mesenchymal. 3 ' 4 A case has been reported of an esophageal duplication cyst that caused precordial and upper abdominal pain .' Cardiovascular hemodynamics may be significantly altered by mediastinal masses . Tumors located near the heart and great vessels can produce signs and symptoms that mimic tricuspid stenosis, 6 pulmonary stenosis 7 and constrictive pericarditis . 8 Invasive techniques are often required to provide accurate evaluation of these tumors . Several recent reports, 8 - 1° including one from this laboratory, have stressed the use of M and B mode echo-
cardiography in the differential diagnosis of such tumors . This case was remarkable because of the displacement of the heart into the right upper quadrant of the chest . The correct diagnosis was inapparent from the chest roentgenogram because the cyst could not easily be differentiated from a normally located but enlarged heart . Echocardiography demonstrated the actual location of the heart and revealed the cystic nature of the tumor and provided accurate data on its internal dimensions . Our case also demonstrates that mediastinal masses may produce hemodynamic alterations . Increased diastolic intracardiac pressures, identical to the pattern seen in constrictive pericarditis, have been seen in a case of cystic thymoma.8 In our case, the heart
September 1977
The American Journal of CARDIOLOGY
Volume 40
447
GIANT MEDIASTINAL CYST-KOCH ET AL.
FIGURE 4 . The opened cyst after removal from the thorax .
was pinioned between the cyst and the chest wall. Although the right heart pressures were only moderately elevated, the diastolic equalization of right and left heart pressures suggests an element of constriction . The patient's murmur may have been due to "kinking" of the great vessels by abnormal cardiac position or pressure on the right or left ventricular outflow tracts . The dyspnea may he explained by the increased right atrial pressure and the large intrathoracic mass that decreased
vital capacity . The origin of this lesion is obscure . It is unclear how long the patient had this mass in her chest ; no history of trauma, previous surgery or bleeding diathesis was elicited . Our case illustrates the valuable diagnostic role of echocardiography in clarifying the actual location and normalcy of the displaced heart and the cystic nature of the mass that shifted it .
References 1 . Oldham HN, Sabiston DC : Primary tumors and cysts of the mediastinum presenting as cardiovascular abnormalities . Arch Surg 96 :71-75, 1968 2 . Allee G, Logue B, Mansour K : Thymic cyst simulating multiple cardiovascular abnormalities and presenting with pericarditis and pericardial tamponade . Am J Cardiol 31 :377-380, 1973 3 . Benjamin SP, McCormack LJ, EKler DE, et al : Primary tumors of the mediastinum . Chest 62 :297-303, 1972 4 . Fine G : Primary tumors of the pericardium and heart . Cardiovasc Clin 5 :207-238, 1973 5 . Fallazadeh H, Haiderer 0 : Esophageal duplication cyst with unusual manifestations . Chest 63 :827-828, 1973 6 . Pitt A, Cutforth RH, Bender HW, et al : Intrapericardial cyst for-
4 48
September 1977 The American Journal of CARDIOLOGY
mation in constrictive pericarditis simulating tricuspid stenosis . Circulation 40 :665-672, 1969 7 . Shaver VC, Bailey WR, Marrangoni AG : Acquired pulmonic stenosis due to external cardiac compression . Am J Cardiol 16 : 256-261,1965 8 . Schloss M, Kronzon I, Gelber PM, at al : Cystic thymoma simulating constrictive pericarditis . J Thorac Cardiovasc Surg 70 :143-146, 1975 9 . Peterson DT, Zatz LM, Popp RL : Pericardial cyst ten years after acute pericarditis . Chest 67:719-721, 1975 10 . Friday RO : Paracardiac cyst : diagnosis by ultrasound and puncture . JAMA 226 :82-83, 1973
Volume 40
