culation 34 :38,1966 12 Suzuki H: Spontaneous closure of ventricular defects: Anatomic evidence of six adult patients. Am J Clin Palbol 52:391, 1969 13 Suzuki H, Lucas RV: Spontaneous closure of ventricular septal defects: Anatomic closures in tIuee patients. Arch Pathol84:31, 1967 14 Schott GO: Documentation of spontaneous functional closure of a ventricular septal defect during adult life. Br Heart J 35: 1214, 1973 15 Mesko ZJ, JooesJE, Nadas AS: Dimuoition and closure of large ventricular septal defects after pulmonary artery banding. Circulation 48:847,1973 16 Van Praagh R, McNamara JJ: Anatomic types of ventricular septal defect with aortic insufBclency; diagnostic and surgical considerations. Am Heart J 75:604, 1968
(hemoglobin level of 10.3 gm/100 ml and hematocrit reading of 32 percent). Levels of electrolytes, blood urea nitrogen and the results of immunoelectrophoresis and urinalysis were normal, as were levels of muscle enzymes, antistreptolysin 0, and CHSO. The antinuclear antibody test, the latex wtion test, Farr's test, and Coombs' test were negative, and viral titers were normaI. The erythrocytic sedimentation rate was elevated to 82 mm/hr. An electroencephalogram (EEG) and eIectromyogram were normal . A muscle biopsy showed slight atrophy, which was suggestive of denervation. An electrocardiogram and chest roentgenogram were normal. Consultants on the neuromuscular service agreed that the disease was consistent with a collagen vascular disease-1iIte syndrome. The eryt:hrocyte sedimentation rate remained above 70 mm/hr, but the patient's condition improved with bed rest for a month. Over the next five months, be was
"•
.J,r-----'~,..---.-J'.,.----J'.r--
Atrial Myxoma Mimicking a Collagen Disorder· Marc E. Komimky, MD.; KlIthf'Jln H. Ehlen, MD.; MtIt'fI
Allen Engle, M.D., F.C.C.P.; ArtIwr A. KleIn, M.D.; Aaron lL Leoin, M.D.; andValaranu,A. Subrarnanlan. M.D.
A 14-year-01d boy had a protncted IOneIs cbancterized by bOatenI tenderness and weaknesB of the extremities; the iDDess was cOIISidered to be • couectift tiIIIIIe disease similar to poIyIIIyo8tlL Not 1IIItiI Dille IDODtbI later, wheD the patient ..... !leW C8J'diac mlll'lll1ll'l, .... IIlOIIIlI'J edema, and then a cerebro~ lICddellt, the true nat1U'e of IliI mne.1IIIpected. The diIIgnoIIIB 01 left atrial myxoma with the trIIMI of coastitutioIIII obItractive, 8Dd embolic dedi was coaftnned by echoc:ardiopaphk studies and cardJac eatbetertzatioI. 11Ie Iamor was IIDCCaIfdy remoftd.
w.
the most common intracardiac tumor M yxoma, adults, rare in children. These tumors, while in
is
histologically benign. are fatal if unrecognized and untreated. Coodwin! pointed out that most patients have a triad of obstructive, embolic, and constitutional effects. The case presented is unusual in the severity and dominance of the constitutional features, which were so outstanding that they mimicked a collagen disorder.
CAsE REPoRT This 14-year old boy was first examined in the emergency room in October 1974 for bilateral pains, decreased strength, and tenderness of both legs. He was thought to have myositis. The symptoms disappeared without treatment. In January 1976, the patient again complained of the sudden onset of pain and wealcness in the arms and legs and of a brief syncopal episode. The involvement of the legs was so severe that he was unable to stand. The patient had sustained a weight loss of 9.1 kg (20 lb) over the previous three months. He was hospitalized for elucidation of his muscular disease. The patient was gaunt, with weak and tender extremities that were not inflamed. The findings from examination were otherwise unremarkable, except for a soft systolic murmur. A complete blood cell count revealed a microcytic anemia ·From the Divisions of Pediatric Cardiology and Cardiovascular Surgery, the New York Hospital-Cornell University Medical College, New York. Reprint requem: Dr. Engle, New York HOIPitGl, New York Cit" 10021
CHEST, 75: I, JANUARY, 1979
LA
.
-
I. Preopera tive echocard iograms , A (top ). Dens e cloud of mult ipl echoes is demonstra ted behind anterior mitral leaflet ( A)'IL) ill dias to le_L ft ventricular dim ens ions. th ickness of left vcntrfcular wall ( L VW ), a nd left ventricu lar Inn n OD are normal. S, Septum; an d P~II~, p o tenor mitral leaflet. B (bottom), In same sweep as in Figure lA, transducer is angled to view aortic root (Ao) and left atrium (LA). Left abial dimension is markedly increased (40.9 mm). Dense abnormal echoes are demonstrated in left atrium behind aortic root in systole and diastole. F l(; URF.
ATRIAL MYXOMA IIIliCKING COWGn DISORDER 93
FiGmm 2. EcbocardJogram obtained ODe week after surgery. Left atrial dimemion is still enlarged (33.3 mm), although it is decreased compared to before surgery. Now there are DO aboormal echoes visoaIized either behind anterior mitralleaftet (AMI..) or in left atrium (LA). S, Septum; PM!., posterior mitral leaflet; LVW, left venbicular wall; and Ao, aortic root.
asymptomatic, and the findings from his physical eumination in May were oormal, although he had gained DO weight and remained anemic while receiving iron supplements. On July 12, 1976, the patient was readmitted because of severe pains in his legs, pain in the chest, shortness of breath, even at rest, and recent episodes of epitaxis. A holosytolic murmur at the apex radiated to the uiIla. The chest roentgeoogram revealed slight cardiomegaly and Kerley B Jines. The ECG showed prolonged time for atrioventricular conduction. The hemoglobin level was 8.9 gm/l00 ml, and the hematocrit reading was 29 percent. The erythrocyte sedimentation rate was 90 mm/hr, Findings from immunoelectrophoresis, CHSO, levels of muscle enzymes, results of urinalysis, and electrolytes levels were normal, while the lats tbation test, Farr's test, antinuclear antibody test. cold antibody test, and antiheart antibody test were all negative. Cardiac consultation was obtained. In addition to a grade 2/6 holosystolic mmmur radiating to the axilla, a grade 2/6 middiastolic rumble was heard and confirmed by phonocardiogram. The diagnosis of atrial myxoma was entertained, and an ecbocardiogram was scheduled; however, on that day the patient became unresponsive. As he became more alert. ueurologic examination revealed that the right pupil was larger than the left and that there was left gaze preference and an inability to voluntarily raise the eyes above midline except on caloric testing. The findings from lumbar puncture were normal. An EEG showed bilateral cerebral dysfunction. The &ndings were coosistent with multiple scattered emboli. An ecbocardiogram (Fig 1) revealed a large left atrium containing a mass which protruded into the left ventricle in ventricular diastole, a finding consistent with left atrial myxoma. On the next morning, cardiac catheterization with contrast visualization revealed a large radiolucent mass within the left atrium, causing mitral valve obstruction; the left pulmonary arterial wedge pressure was 45/18 mm Hg (mean, 35 mm Hg), the right pulmonary arterial wedge pressure was 43/17 mm Hg (mean, 30 mm Hg), and the mean pulmonary resistance was 10.53 mm Hg/L/min/sq m. On the next morning, with the patient on cardiopulmonary bypass, the left atrium was opened. and a large left Iltrial tumor was found, with the Ita1lc attached to the posteroin-
94 IWIINSKY ET AL
ferior area of the left atrium near the entrance of the right inferior pulmonary vein. Some tumor extended into the pulmonary veins. The tumor was excised in toto, along with the mouth of the right inferior pulmonary vein. The grey gelatinous tumor measured 5 X 4 X 3 em and weighed 35 gm. The microscopic diagnosis was atrial myxoma. The patient made a full neurologic and cardi0vascular recovery. The findings from physical ell:BJl1inations through the next two years have been normal . Serial echocardiograms (Fig 2) revealed no evidence of tumor and demonstrated that the left atrium progressively diminished in size so that by eight months after surgery the left atrial dimension was normal. An ECG revealed T waves consistent with a myocardial or pericardial reaction. The chest roentgenogram was normal. The antiheart antibody test remained negative, and the erythrocyte sedimentation rate became normal, as did the complete blood cell count. There has been DO recurrence of constitutional symptoms, and the patient has regained a oormal weight. DISCUSSION
Myxomas may originate in any chamber of the heart, but 75 percent of these pedunculated tumors arise from the left side of the atrial septum, often near the fossa ovalis. As this boy illustrated, atrial myxomas show themselves clinically in three ways: ( 1) by constitutional effects; (2) by obstruction to the blood flow; and (3) by embolizatfon.vs It is highly unusual for constitutional manifestations to be dominant enough to mimic a collagen disease. These symptoms were present in this child for nine months before obstructive and embolic symptoms occurred. The factors causing constitional disturbances are not clear. Evaluation of this patient for levels of muscular enzymes, abnonnality of the complement cascade, and for heart-reactive antibodies showed normal findings on repeated determinations. The utility of ultrasound in the diagnosis of atrial
CHEST, 75: 1, JANUARY, 1979
myxoma has been confirmed by many authors since Schattenberg's- first report in 1968. In prolapsing left atrial tumors, a collection of wavy echoes is seen behind the anterior mitral leaflet in diastole. These echoes disappear in systole as the tumor returns to the left atrium. In nonprolapsing left atrial tumors, abnormal echoes are seen in the left atrial cavity. Because of the risks of embolization and sudden death, surgery under direct vision should closely foRow the diagnosis.
REFERENCES
1 Goodwin IF: Diagnosis of left abial myxoma. Lancet 1:464,1963 2 Peters MN, Hall RF, Cooley DA, et al: The clinical syndrome of atrial myxoma. JAMA 230:69.5, 1974 3 Schattenberg TI: Echocardiographic diagnosis of left atrial myxoma. Mayo Clin Proc 43:620,1968
Unique Massive Pulmonary Hamartoma· Case Report with Review of Hamartomata Treated at Brompton Hospital in 27 Years Ian S. Petheram, M.B., B.Ch.;" and Brian E. Heard, M.D.t A JO.cm tumor was removed from a 32-year-Gld man. It coasisted of a c:oIIec:tioa of multiple IlIII8D tuIIIon, each bIlItololPcaDy typical of a dIoocboDlldolls ....... tomL SixteeD yeuw earlier, ideIllicaI bistoIoIk fiDdiIIp were DOted in "cysts" in die ript lower lobe. A review of other cases of cartiJaIiDoas hamartoma treated at Brompton Hospital siDce 1950 aad a study of die Uterature .... lest that tbilI tIuDor is uique.
F'Ictlu 1. Lateral roentgenogram, showing rounded shape of
tumor.
SWeen years later, the patient bad two attacks of acute bronchitis and persistent right-slded pain in the chest. A chest roentgenogram (Fig 1) showed a massive round lesion in the right lower and middle zones. At thoracotomy a tumor 30 em in diameter extended across the mediastinum into the
ulmonary hamartomata are usually single, with a Ppreponderance of cartilage.' They are often discov-
ered by routine radiologic studies for other conditions. Multiple hamartomata have been reported only rarely and have a preponderance of smooth muscle with no cartilage. The occurrence of a massive example of a cartilaginous hamartoma that appeared to be a collection of multiple small tumors prompted a review of the experience with these tumors at Brompton Hospital, London, in the past 27 years.
CASE REPoRT In 1961, a fit 16-year-()ld boy was found to have a rightsided pneumothorax. At thoracoscopic examination, six small bullae, each about 2 inches in diameter, were seen on the surface of the right lower lobe. Following poudrage with talc, the lung reexpanded. Eighteen months later, because of progressive dyspnea on exertion attributed to residual bullae, a right thoracotomy was pedormed. Two large aair cystswere removed with a wedge resection of part of the right lower lobe. Macroscopically, the excised tissue consisted of large cysts infiltrated by cartilaginous tumor. HJstologic studies showed cartilage, bronchial epithelium, and smooth muscle, appearances typical of a cartilaginous hamartoma. Six months after the operation, the patient was asymptomatic and was lost to follow-up. o From Brompton Hospital, London, EDg)aDd --Registrar. tcoDsuItant Pathologist
CHEST, 75: I, JANUARY, 1979
-"
2
J
5
10
II
2
showing discrete isJanda of cartilage surrounded by fibrous CODDeCtive tissue. F'IctlIlJ: 1. Cut smfaoe of part of tumor,
UIliGUE MASSIVE PULMONARY HAMARTOMA 95
(hemoglobin level of 10.3 gm/100 ml and hematocrit reading of 32 percent). Levels of electrolytes, blood urea nitrogen and the results of immunoelectrophoresis and urinalysis were normal, as were levels of muscle enzymes, antistreptolysin 0, and CHSO. The antinuclear antibody test, the latex wtion test, Farr's test, and Coombs' test were negative, and viral titers were normaI. The erythrocytic sedimentation rate was elevated to 82 mm/hr. An electroencephalogram (EEG) and eIectromyogram were normal . A muscle biopsy showed slight atrophy, which was suggestive of denervation. An electrocardiogram and chest roentgenogram were normal. Consultants on the neuromuscular service agreed that the disease was consistent with a collagen vascular disease-1iIte syndrome. The eryt:hrocyte sedimentation rate remained above 70 mm/hr, but the patient's condition improved with bed rest for a month. Over the next five months, be was
"•
.J,r-----'~,..---.-J'.,.----J'.r--
Atrial Myxoma Mimicking a Collagen Disorder· Marc E. Komimky, MD.; KlIthf'Jln H. Ehlen, MD.; MtIt'fI
Allen Engle, M.D., F.C.C.P.; ArtIwr A. KleIn, M.D.; Aaron lL Leoin, M.D.; andValaranu,A. Subrarnanlan. M.D.
A 14-year-01d boy had a protncted IOneIs cbancterized by bOatenI tenderness and weaknesB of the extremities; the iDDess was cOIISidered to be • couectift tiIIIIIe disease similar to poIyIIIyo8tlL Not 1IIItiI Dille IDODtbI later, wheD the patient ..... !leW C8J'diac mlll'lll1ll'l, .... IIlOIIIlI'J edema, and then a cerebro~ lICddellt, the true nat1U'e of IliI mne.1IIIpected. The diIIgnoIIIB 01 left atrial myxoma with the trIIMI of coastitutioIIII obItractive, 8Dd embolic dedi was coaftnned by echoc:ardiopaphk studies and cardJac eatbetertzatioI. 11Ie Iamor was IIDCCaIfdy remoftd.
w.
the most common intracardiac tumor M yxoma, adults, rare in children. These tumors, while in
is
histologically benign. are fatal if unrecognized and untreated. Coodwin! pointed out that most patients have a triad of obstructive, embolic, and constitutional effects. The case presented is unusual in the severity and dominance of the constitutional features, which were so outstanding that they mimicked a collagen disorder.
CAsE REPoRT This 14-year old boy was first examined in the emergency room in October 1974 for bilateral pains, decreased strength, and tenderness of both legs. He was thought to have myositis. The symptoms disappeared without treatment. In January 1976, the patient again complained of the sudden onset of pain and wealcness in the arms and legs and of a brief syncopal episode. The involvement of the legs was so severe that he was unable to stand. The patient had sustained a weight loss of 9.1 kg (20 lb) over the previous three months. He was hospitalized for elucidation of his muscular disease. The patient was gaunt, with weak and tender extremities that were not inflamed. The findings from examination were otherwise unremarkable, except for a soft systolic murmur. A complete blood cell count revealed a microcytic anemia ·From the Divisions of Pediatric Cardiology and Cardiovascular Surgery, the New York Hospital-Cornell University Medical College, New York. Reprint requem: Dr. Engle, New York HOIPitGl, New York Cit" 10021
CHEST, 75: I, JANUARY, 1979
LA
.
-
I. Preopera tive echocard iograms , A (top ). Dens e cloud of mult ipl echoes is demonstra ted behind anterior mitral leaflet ( A)'IL) ill dias to le_L ft ventricular dim ens ions. th ickness of left vcntrfcular wall ( L VW ), a nd left ventricu lar Inn n OD are normal. S, Septum; an d P~II~, p o tenor mitral leaflet. B (bottom), In same sweep as in Figure lA, transducer is angled to view aortic root (Ao) and left atrium (LA). Left abial dimension is markedly increased (40.9 mm). Dense abnormal echoes are demonstrated in left atrium behind aortic root in systole and diastole. F l(; URF.
ATRIAL MYXOMA IIIliCKING COWGn DISORDER 93
FiGmm 2. EcbocardJogram obtained ODe week after surgery. Left atrial dimemion is still enlarged (33.3 mm), although it is decreased compared to before surgery. Now there are DO aboormal echoes visoaIized either behind anterior mitralleaftet (AMI..) or in left atrium (LA). S, Septum; PM!., posterior mitral leaflet; LVW, left venbicular wall; and Ao, aortic root.
asymptomatic, and the findings from his physical eumination in May were oormal, although he had gained DO weight and remained anemic while receiving iron supplements. On July 12, 1976, the patient was readmitted because of severe pains in his legs, pain in the chest, shortness of breath, even at rest, and recent episodes of epitaxis. A holosytolic murmur at the apex radiated to the uiIla. The chest roentgeoogram revealed slight cardiomegaly and Kerley B Jines. The ECG showed prolonged time for atrioventricular conduction. The hemoglobin level was 8.9 gm/l00 ml, and the hematocrit reading was 29 percent. The erythrocyte sedimentation rate was 90 mm/hr, Findings from immunoelectrophoresis, CHSO, levels of muscle enzymes, results of urinalysis, and electrolytes levels were normal, while the lats tbation test, Farr's test, antinuclear antibody test. cold antibody test, and antiheart antibody test were all negative. Cardiac consultation was obtained. In addition to a grade 2/6 holosystolic mmmur radiating to the axilla, a grade 2/6 middiastolic rumble was heard and confirmed by phonocardiogram. The diagnosis of atrial myxoma was entertained, and an ecbocardiogram was scheduled; however, on that day the patient became unresponsive. As he became more alert. ueurologic examination revealed that the right pupil was larger than the left and that there was left gaze preference and an inability to voluntarily raise the eyes above midline except on caloric testing. The findings from lumbar puncture were normal. An EEG showed bilateral cerebral dysfunction. The &ndings were coosistent with multiple scattered emboli. An ecbocardiogram (Fig 1) revealed a large left atrium containing a mass which protruded into the left ventricle in ventricular diastole, a finding consistent with left atrial myxoma. On the next morning, cardiac catheterization with contrast visualization revealed a large radiolucent mass within the left atrium, causing mitral valve obstruction; the left pulmonary arterial wedge pressure was 45/18 mm Hg (mean, 35 mm Hg), the right pulmonary arterial wedge pressure was 43/17 mm Hg (mean, 30 mm Hg), and the mean pulmonary resistance was 10.53 mm Hg/L/min/sq m. On the next morning, with the patient on cardiopulmonary bypass, the left atrium was opened. and a large left Iltrial tumor was found, with the Ita1lc attached to the posteroin-
94 IWIINSKY ET AL
ferior area of the left atrium near the entrance of the right inferior pulmonary vein. Some tumor extended into the pulmonary veins. The tumor was excised in toto, along with the mouth of the right inferior pulmonary vein. The grey gelatinous tumor measured 5 X 4 X 3 em and weighed 35 gm. The microscopic diagnosis was atrial myxoma. The patient made a full neurologic and cardi0vascular recovery. The findings from physical ell:BJl1inations through the next two years have been normal . Serial echocardiograms (Fig 2) revealed no evidence of tumor and demonstrated that the left atrium progressively diminished in size so that by eight months after surgery the left atrial dimension was normal. An ECG revealed T waves consistent with a myocardial or pericardial reaction. The chest roentgenogram was normal. The antiheart antibody test remained negative, and the erythrocyte sedimentation rate became normal, as did the complete blood cell count. There has been DO recurrence of constitutional symptoms, and the patient has regained a oormal weight. DISCUSSION
Myxomas may originate in any chamber of the heart, but 75 percent of these pedunculated tumors arise from the left side of the atrial septum, often near the fossa ovalis. As this boy illustrated, atrial myxomas show themselves clinically in three ways: ( 1) by constitutional effects; (2) by obstruction to the blood flow; and (3) by embolizatfon.vs It is highly unusual for constitutional manifestations to be dominant enough to mimic a collagen disease. These symptoms were present in this child for nine months before obstructive and embolic symptoms occurred. The factors causing constitional disturbances are not clear. Evaluation of this patient for levels of muscular enzymes, abnonnality of the complement cascade, and for heart-reactive antibodies showed normal findings on repeated determinations. The utility of ultrasound in the diagnosis of atrial
CHEST, 75: 1, JANUARY, 1979
myxoma has been confirmed by many authors since Schattenberg's- first report in 1968. In prolapsing left atrial tumors, a collection of wavy echoes is seen behind the anterior mitral leaflet in diastole. These echoes disappear in systole as the tumor returns to the left atrium. In nonprolapsing left atrial tumors, abnormal echoes are seen in the left atrial cavity. Because of the risks of embolization and sudden death, surgery under direct vision should closely foRow the diagnosis.
REFERENCES
1 Goodwin IF: Diagnosis of left abial myxoma. Lancet 1:464,1963 2 Peters MN, Hall RF, Cooley DA, et al: The clinical syndrome of atrial myxoma. JAMA 230:69.5, 1974 3 Schattenberg TI: Echocardiographic diagnosis of left atrial myxoma. Mayo Clin Proc 43:620,1968
Unique Massive Pulmonary Hamartoma· Case Report with Review of Hamartomata Treated at Brompton Hospital in 27 Years Ian S. Petheram, M.B., B.Ch.;" and Brian E. Heard, M.D.t A JO.cm tumor was removed from a 32-year-Gld man. It coasisted of a c:oIIec:tioa of multiple IlIII8D tuIIIon, each bIlItololPcaDy typical of a dIoocboDlldolls ....... tomL SixteeD yeuw earlier, ideIllicaI bistoIoIk fiDdiIIp were DOted in "cysts" in die ript lower lobe. A review of other cases of cartiJaIiDoas hamartoma treated at Brompton Hospital siDce 1950 aad a study of die Uterature .... lest that tbilI tIuDor is uique.
F'Ictlu 1. Lateral roentgenogram, showing rounded shape of
tumor.
SWeen years later, the patient bad two attacks of acute bronchitis and persistent right-slded pain in the chest. A chest roentgenogram (Fig 1) showed a massive round lesion in the right lower and middle zones. At thoracotomy a tumor 30 em in diameter extended across the mediastinum into the
ulmonary hamartomata are usually single, with a Ppreponderance of cartilage.' They are often discov-
ered by routine radiologic studies for other conditions. Multiple hamartomata have been reported only rarely and have a preponderance of smooth muscle with no cartilage. The occurrence of a massive example of a cartilaginous hamartoma that appeared to be a collection of multiple small tumors prompted a review of the experience with these tumors at Brompton Hospital, London, in the past 27 years.
CASE REPoRT In 1961, a fit 16-year-()ld boy was found to have a rightsided pneumothorax. At thoracoscopic examination, six small bullae, each about 2 inches in diameter, were seen on the surface of the right lower lobe. Following poudrage with talc, the lung reexpanded. Eighteen months later, because of progressive dyspnea on exertion attributed to residual bullae, a right thoracotomy was pedormed. Two large aair cystswere removed with a wedge resection of part of the right lower lobe. Macroscopically, the excised tissue consisted of large cysts infiltrated by cartilaginous tumor. HJstologic studies showed cartilage, bronchial epithelium, and smooth muscle, appearances typical of a cartilaginous hamartoma. Six months after the operation, the patient was asymptomatic and was lost to follow-up. o From Brompton Hospital, London, EDg)aDd --Registrar. tcoDsuItant Pathologist
CHEST, 75: I, JANUARY, 1979
-"
2
J
5
10
II
2
showing discrete isJanda of cartilage surrounded by fibrous CODDeCtive tissue. F'IctlIlJ: 1. Cut smfaoe of part of tumor,
UIliGUE MASSIVE PULMONARY HAMARTOMA 95
