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Dissecting aneurysm of the pulmonary trunk
660
BRIEF
REPORTS
63). When the hearts were considered to be normal or hypertrophied, 68% (183 of268) were correctly identified, yielding a sensitivity of 57% and a specificity of 83%. The positive and negative predictive values were 81% and 60%, respectively. A cardiac surface area of 499 cm2 or less identified 80% of normal patients (94 of 1171, whereas an area greater than 499 cm2 identified 56% of patients with LV hypertrophy (33 of 59),43% with RV hypertrophy (12 of 28) and 83% with combined ventricular hypertrophy (52 of 63). When the hearts were considered to be either normal or hypertrophied, 72% (192 of 267) were correctly identified, yielding a sensitivity of 65% and a specificity of 80%. The positive and negative predictive values were 81% and 6470, respectively. A cardiac volume of 499 cm3/m2 or less identified 76% of normal patients (79 of 1041, whereas a volume greater than 499 cm3 identified 71% of patients with LV hypertrophy (39 of 55), 54% with RV hypertrophy (14 of 26) and 82% with combined ventricular hypertrophy (46 of 56). When the hearts were considered to be normal or hypertrophied, 74% (178 of 242) were correctly identified, yielding a sensitivity of72% and a specificity of 76%. The positive and negative predictive values were 80% and 6770, respectively. These results are summarized in Table I. Most clinical trials involving patients with cardiovascular disease include the chest roentgenogram as a baseline variable, reporting it to be either normal or abnormal. Some use the subjective reading of the investigator to stratify patients as having either a normal sized heart or cardiomegaly7; some studies include actual objective chest roentgenogram measurements as variables.* Although the chest roentgenogram is accepted in this way as a screening test, no data using specific ventricular weights has existed to confirm its accuracy in screening for ventricular hypertrophy.
Dissecting Aneurysm of the Pulmonary Trunk MICHIELJ. NAGELSMIT, MD FRITS EULDERINK,MD, PhD
I
n contrast to dissecting aneurysm of the aorta, dissecting aneurysm of the pulmonary trunk (PT) is rare.l-14 Most often it affects patients with congenital heart disease with pulmonary hypertension. None of the 14 previously reported cases was recognized during life, and all 14 patients died soon after the onset of symptoms. Herein, we describe another patient with dissection of the PT. From the Departments of Cardiology and Pathology, University Hospital Leiden, Rijnsburgerweg 10, 2333 AA Leiden, The Netherlands. Manuscript received January 20, 1986; revised manuscript received February 26, 1986, accepted February 27, 1986.
The 4 objective measurements of the chest roentgenogram in our study gave similar results in identification of hearts with normal ventricular mass, with 76 to 83% of normal hearts being correctly identified as normal. Specific chamber size was not recognized well by any measurement. Right ventricular hypertrophy was very poorly identified by objective measurements, ranging from 29 to 54%. Combined ventricular hypertrophy was more often identified than LV hypertrophy or RV hypertrophy although no one measurement was superior. We conclude that these 4 objective measurements on the chest roentgenogram are useful as a screening tool for the presence or absence of ventricular hypertrophy although individual chamber hypertrophy cannot be accurately determined. Transverse cardiac diameter is the method of choice because of its simplicity and accuracy (compared to the other three measurements).
References 1. Keats TE, Enge IP. Cardiac mensuration by the cardiac volume method. Radiology 1965;85:850-855. 2. Rackley CE, Dodge HT, Coble YD, Hay RE. A method for determining left ventricufor moss in man. Circulation 1964:29:666-671. 3. Reiner L, Mazzoleni A, Rodriquez FL, Freudenthal RR. The weight of the human heart. I. “Normal cases.” Arch Path01 1959;68:58-73. 4. Murphy ML, Adamson J, Hutcheson F. Left ventricular hypertrophy in patients with chronic bronchitis and emphysema. Ann Intern Med 1974; 81:307-313. 5. Murphy ML, Thenabadu PN, Blue LR, Meade J, de Soyza N, Doherty JE, Baker B. Descriptive characteristics of the electrocardiogram from autopsied men free of cardiopulmonary disease: a bosis for evaluating criteria for ventricular hypertrophy. Am J Cardiol 1983;52:1275-1280. 6. Lusted LB, Keats TE. Atlas of Roentgenographic Measurement. 3rd ed. Chicago: Year Book Medical, 1972:192-196. 7. Aspirin Myocardial Infarction Study Group. A randomized controlled trial of aspirin in persons recovering from myocardial infarction. JAMA 1980;243:661-669. 6. Detre K. Hultaren H. Takaro T. and the VA Coooerative Grouo for the Study of S&gery?o:or Coronary Arterial Occlusive Diseases. Veterans Administration Cooperative Study of Surgery for Coronary Arterial Occlusive Diseose. III. Methods and boseline characteristics, including experience with medical treatment. Am J Cardiol 1977;40:212-224.
A 20-year-old man had double-outlet right ventricle, transposition of the great arteries, ventricular septal defect and overriding of the PT (Taussig-Bing malformation) diagnosed 17 years earlier. Pulmonary pressure at age 3 was 90/60 mm Hg and the PT was dilated. Thereafter, he had frequent respiratory infections, retarded growth and development, and poor exercise tolerance. At age 20 years, exercise tolerance decreased more. The hematocrit was 77%. Shortly thereafter, he had sudden onset of severe pain in the back of the neck and was admitted to a hospital. He had no palpable peripheral pulses, was pale but cyanotic, and the heart rate was 135 beats/min. Precordial auscultation was unchanged, Blood hematocrit was 77% and white blood cell count was 35,000/mm3. The electrocardiogram showed sinus tachycardia, rightaxis deviation, and right atrial and ventricular enlargement. Chest x-ray was unaltered and showed marked cardiomegaly probably due to right ventricular enlargement and marked dilatation of the PT. Dopamine, heparin, dexamethasone and dextran were administered. Phlebotomy was performed. Shortly
September
TABLE I Aneurysm
Findings in 14 Previously of the Pulmonary Trunk References
= congenital
heart
Cases
of Dissecting
Age (yr) & Sex
PH
CHD
26F 19M 33M 21M 23M 24F 26M 43F 57M 73M 28F 13F 44F 41F
+ + + + t t t t + -
+ + t t t t 0 + 0 0 + 0 +
I 2 3 4 5 6 7 8 9 IO 11 12 13 14 CHD
Reported
disease;
PH = pulmonary
15, 1986
0 + + + hypertension.
thereafter he had an epileptic insult with subsequent respiratory and fatal cardiac arrest. Necropsy disclosed dissecting aneurysm of the PT. The immediate cause of death was a hemopericardium (500 ml). The PT overrode a 2-cm defect in the ventricular septum and it was the only outlet of the paucitrabecular left ventricle. The aorta arose from the thick-walled (16 mm] right ventricle. The left ventricular wall was 20 mm thick. The heart weighed 550 g. In addition to the ventricular septum defect, an atrial septum defect (1.5 cm] was present. Atherosclerotic plaques were present in the large pulmonary arteries and plexiform lesions were present in the lungs. The intimal entrance of PT dissection was a small rupture about 2 cm above the pulmanic valve. Adjacent to this rupture scarring of the media with interruption of the elastic membranes was found. The dissection extended from the valve to the
Pulmonary Arterial and Venous Hypertension and left Ventricular Calcification of Undetermined Etiology DEBORAH THOMAS JOEL WILLIAM
J. V. A. C.
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume
58
661
bifurcation of the PT, separating the outer half to third of the media from the inner part. At the level of the valve the blood had penetrated the epicardium and, via a small rupture, the pericardial cavity. Table I is a summary of the main features of the 14 previously reported cases. The most frequent cause of death was cardiac tamponade (13 patients). Twelve of the 14 patients had known pulmonary hypertension, most often caused by congenital heart disease (9 of 14, 1 unknown). The average age at death was 33 years and 7 were men.
References 1. Foord AG, Lewis RD. Primary dissecting aneurysms of peripheral and, pulmonary arteries. Arch Pathol 1959x%:553-577. 2. Crumpton M. Congenital heart disease and dissecting aneurysm of pulmonary artery. Br Med J 1950;1:1303. 3. Durno L, Brown WL. A case of dissecting aneurysm of the pulmonary artery; patent ductus arteriosus; rupture into the pericardium. Lancet 1908;1:1693-1694. 4. W&he WH. A Practical Treatise on the Diseases of the Heart and Great Vessels. 3rd ed. London, 1862:531. 5. Placik B, Rodbard S, McMahon J, Swaroop S. Pulmonary artery dissection and rupture in Eisenmenger’s syndrome. Vast Surg 1976;10:72-80. 6. Best J. Dissecting aneurysm of the pulmonary artery with multiple cardiovascular abnormalities and pulmonary hypertension. Med J Aust 1967; 2:1129-1130. 7. Fleming HA. Aorta-pulmonary septol defect with patent ductus arteriosus and death due to rupture of dissecting aneurysm of the pulmonary artery into the pericardium. Thorax 1956;11:71-76. 8. Odinokova VA. Dissecting aneurysm of the pulmonary artery. Arkh Patol 1956;18:87-89. 9. Shilkin KB, Low LP, Chen BTM. Dissecting aneurysm of the pulmonary artery. J Pathol 1969;98:25-29. 10. Gog B. Aneurysma dissecans de A. pulmonalis. Zschr Inn Med 1966;6:189191. 11. Plenge K. Zur Frage der Syphilis der Lungenschlagader. Virchows Arch Path Anat Physiol 1930;275:572-584. 12. Wilkinson KD. Aneurysmal dilatation of the pulmonary artery. Br Heart J 1940;2:255-259. 13. McNaught JB, Dock W. Spontaneous rupture of the pulmonary artery. Am J Pathol 1935;11:989-999. 14. Grindvald IV, Vasilev MA. Dissecting aneurysm of the pulmonary artery. Klin Med Mosk 1959;37:125-127.
phy, radionuclide angiography, pulmonary function studies, and, finally, necropsy, the cause of the severe pulmonary hypertension could not be determined. A description of the findings in this unusual patient is the purpose of this report.
BARBOUR, MD INGLESBY, MD ROTH, MD ROBERTS, MD
I
n patients with severe pulmonary hypertension, the cause can nearly always be determined clinically or, if not, by necropsy examination. We recently studied a young woman with severe pulmonary hypertension and, despite cardiac catheterization, echocardiograFrom the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, and the Departments of Medicine [Cardiology) and Pathology, Overlook Hospital, Summit, New Jersey. Manuscript received and accepted March 6,1986.
FIGURE 1. Posteroanterior and lateral chest radiographs taken 19 months before the patient’s death showing mild cardiomegaly and an intracardiac calclfic denslty (arrows) variously Interpreted at the time as involving the aortic or mitral valve but which, at necropsy, was present in the ventricular septum.
BRIEF
REPORTS
63). When the hearts were considered to be normal or hypertrophied, 68% (183 of268) were correctly identified, yielding a sensitivity of 57% and a specificity of 83%. The positive and negative predictive values were 81% and 60%, respectively. A cardiac surface area of 499 cm2 or less identified 80% of normal patients (94 of 1171, whereas an area greater than 499 cm2 identified 56% of patients with LV hypertrophy (33 of 59),43% with RV hypertrophy (12 of 28) and 83% with combined ventricular hypertrophy (52 of 63). When the hearts were considered to be either normal or hypertrophied, 72% (192 of 267) were correctly identified, yielding a sensitivity of 65% and a specificity of 80%. The positive and negative predictive values were 81% and 6470, respectively. A cardiac volume of 499 cm3/m2 or less identified 76% of normal patients (79 of 1041, whereas a volume greater than 499 cm3 identified 71% of patients with LV hypertrophy (39 of 55), 54% with RV hypertrophy (14 of 26) and 82% with combined ventricular hypertrophy (46 of 56). When the hearts were considered to be normal or hypertrophied, 74% (178 of 242) were correctly identified, yielding a sensitivity of72% and a specificity of 76%. The positive and negative predictive values were 80% and 6770, respectively. These results are summarized in Table I. Most clinical trials involving patients with cardiovascular disease include the chest roentgenogram as a baseline variable, reporting it to be either normal or abnormal. Some use the subjective reading of the investigator to stratify patients as having either a normal sized heart or cardiomegaly7; some studies include actual objective chest roentgenogram measurements as variables.* Although the chest roentgenogram is accepted in this way as a screening test, no data using specific ventricular weights has existed to confirm its accuracy in screening for ventricular hypertrophy.
Dissecting Aneurysm of the Pulmonary Trunk MICHIELJ. NAGELSMIT, MD FRITS EULDERINK,MD, PhD
I
n contrast to dissecting aneurysm of the aorta, dissecting aneurysm of the pulmonary trunk (PT) is rare.l-14 Most often it affects patients with congenital heart disease with pulmonary hypertension. None of the 14 previously reported cases was recognized during life, and all 14 patients died soon after the onset of symptoms. Herein, we describe another patient with dissection of the PT. From the Departments of Cardiology and Pathology, University Hospital Leiden, Rijnsburgerweg 10, 2333 AA Leiden, The Netherlands. Manuscript received January 20, 1986; revised manuscript received February 26, 1986, accepted February 27, 1986.
The 4 objective measurements of the chest roentgenogram in our study gave similar results in identification of hearts with normal ventricular mass, with 76 to 83% of normal hearts being correctly identified as normal. Specific chamber size was not recognized well by any measurement. Right ventricular hypertrophy was very poorly identified by objective measurements, ranging from 29 to 54%. Combined ventricular hypertrophy was more often identified than LV hypertrophy or RV hypertrophy although no one measurement was superior. We conclude that these 4 objective measurements on the chest roentgenogram are useful as a screening tool for the presence or absence of ventricular hypertrophy although individual chamber hypertrophy cannot be accurately determined. Transverse cardiac diameter is the method of choice because of its simplicity and accuracy (compared to the other three measurements).
References 1. Keats TE, Enge IP. Cardiac mensuration by the cardiac volume method. Radiology 1965;85:850-855. 2. Rackley CE, Dodge HT, Coble YD, Hay RE. A method for determining left ventricufor moss in man. Circulation 1964:29:666-671. 3. Reiner L, Mazzoleni A, Rodriquez FL, Freudenthal RR. The weight of the human heart. I. “Normal cases.” Arch Path01 1959;68:58-73. 4. Murphy ML, Adamson J, Hutcheson F. Left ventricular hypertrophy in patients with chronic bronchitis and emphysema. Ann Intern Med 1974; 81:307-313. 5. Murphy ML, Thenabadu PN, Blue LR, Meade J, de Soyza N, Doherty JE, Baker B. Descriptive characteristics of the electrocardiogram from autopsied men free of cardiopulmonary disease: a bosis for evaluating criteria for ventricular hypertrophy. Am J Cardiol 1983;52:1275-1280. 6. Lusted LB, Keats TE. Atlas of Roentgenographic Measurement. 3rd ed. Chicago: Year Book Medical, 1972:192-196. 7. Aspirin Myocardial Infarction Study Group. A randomized controlled trial of aspirin in persons recovering from myocardial infarction. JAMA 1980;243:661-669. 6. Detre K. Hultaren H. Takaro T. and the VA Coooerative Grouo for the Study of S&gery?o:or Coronary Arterial Occlusive Diseases. Veterans Administration Cooperative Study of Surgery for Coronary Arterial Occlusive Diseose. III. Methods and boseline characteristics, including experience with medical treatment. Am J Cardiol 1977;40:212-224.
A 20-year-old man had double-outlet right ventricle, transposition of the great arteries, ventricular septal defect and overriding of the PT (Taussig-Bing malformation) diagnosed 17 years earlier. Pulmonary pressure at age 3 was 90/60 mm Hg and the PT was dilated. Thereafter, he had frequent respiratory infections, retarded growth and development, and poor exercise tolerance. At age 20 years, exercise tolerance decreased more. The hematocrit was 77%. Shortly thereafter, he had sudden onset of severe pain in the back of the neck and was admitted to a hospital. He had no palpable peripheral pulses, was pale but cyanotic, and the heart rate was 135 beats/min. Precordial auscultation was unchanged, Blood hematocrit was 77% and white blood cell count was 35,000/mm3. The electrocardiogram showed sinus tachycardia, rightaxis deviation, and right atrial and ventricular enlargement. Chest x-ray was unaltered and showed marked cardiomegaly probably due to right ventricular enlargement and marked dilatation of the PT. Dopamine, heparin, dexamethasone and dextran were administered. Phlebotomy was performed. Shortly
September
TABLE I Aneurysm
Findings in 14 Previously of the Pulmonary Trunk References
= congenital
heart
Cases
of Dissecting
Age (yr) & Sex
PH
CHD
26F 19M 33M 21M 23M 24F 26M 43F 57M 73M 28F 13F 44F 41F
+ + + + t t t t + -
+ + t t t t 0 + 0 0 + 0 +
I 2 3 4 5 6 7 8 9 IO 11 12 13 14 CHD
Reported
disease;
PH = pulmonary
15, 1986
0 + + + hypertension.
thereafter he had an epileptic insult with subsequent respiratory and fatal cardiac arrest. Necropsy disclosed dissecting aneurysm of the PT. The immediate cause of death was a hemopericardium (500 ml). The PT overrode a 2-cm defect in the ventricular septum and it was the only outlet of the paucitrabecular left ventricle. The aorta arose from the thick-walled (16 mm] right ventricle. The left ventricular wall was 20 mm thick. The heart weighed 550 g. In addition to the ventricular septum defect, an atrial septum defect (1.5 cm] was present. Atherosclerotic plaques were present in the large pulmonary arteries and plexiform lesions were present in the lungs. The intimal entrance of PT dissection was a small rupture about 2 cm above the pulmanic valve. Adjacent to this rupture scarring of the media with interruption of the elastic membranes was found. The dissection extended from the valve to the
Pulmonary Arterial and Venous Hypertension and left Ventricular Calcification of Undetermined Etiology DEBORAH THOMAS JOEL WILLIAM
J. V. A. C.
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume
58
661
bifurcation of the PT, separating the outer half to third of the media from the inner part. At the level of the valve the blood had penetrated the epicardium and, via a small rupture, the pericardial cavity. Table I is a summary of the main features of the 14 previously reported cases. The most frequent cause of death was cardiac tamponade (13 patients). Twelve of the 14 patients had known pulmonary hypertension, most often caused by congenital heart disease (9 of 14, 1 unknown). The average age at death was 33 years and 7 were men.
References 1. Foord AG, Lewis RD. Primary dissecting aneurysms of peripheral and, pulmonary arteries. Arch Pathol 1959x%:553-577. 2. Crumpton M. Congenital heart disease and dissecting aneurysm of pulmonary artery. Br Med J 1950;1:1303. 3. Durno L, Brown WL. A case of dissecting aneurysm of the pulmonary artery; patent ductus arteriosus; rupture into the pericardium. Lancet 1908;1:1693-1694. 4. W&he WH. A Practical Treatise on the Diseases of the Heart and Great Vessels. 3rd ed. London, 1862:531. 5. Placik B, Rodbard S, McMahon J, Swaroop S. Pulmonary artery dissection and rupture in Eisenmenger’s syndrome. Vast Surg 1976;10:72-80. 6. Best J. Dissecting aneurysm of the pulmonary artery with multiple cardiovascular abnormalities and pulmonary hypertension. Med J Aust 1967; 2:1129-1130. 7. Fleming HA. Aorta-pulmonary septol defect with patent ductus arteriosus and death due to rupture of dissecting aneurysm of the pulmonary artery into the pericardium. Thorax 1956;11:71-76. 8. Odinokova VA. Dissecting aneurysm of the pulmonary artery. Arkh Patol 1956;18:87-89. 9. Shilkin KB, Low LP, Chen BTM. Dissecting aneurysm of the pulmonary artery. J Pathol 1969;98:25-29. 10. Gog B. Aneurysma dissecans de A. pulmonalis. Zschr Inn Med 1966;6:189191. 11. Plenge K. Zur Frage der Syphilis der Lungenschlagader. Virchows Arch Path Anat Physiol 1930;275:572-584. 12. Wilkinson KD. Aneurysmal dilatation of the pulmonary artery. Br Heart J 1940;2:255-259. 13. McNaught JB, Dock W. Spontaneous rupture of the pulmonary artery. Am J Pathol 1935;11:989-999. 14. Grindvald IV, Vasilev MA. Dissecting aneurysm of the pulmonary artery. Klin Med Mosk 1959;37:125-127.
phy, radionuclide angiography, pulmonary function studies, and, finally, necropsy, the cause of the severe pulmonary hypertension could not be determined. A description of the findings in this unusual patient is the purpose of this report.
BARBOUR, MD INGLESBY, MD ROTH, MD ROBERTS, MD
I
n patients with severe pulmonary hypertension, the cause can nearly always be determined clinically or, if not, by necropsy examination. We recently studied a young woman with severe pulmonary hypertension and, despite cardiac catheterization, echocardiograFrom the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, and the Departments of Medicine [Cardiology) and Pathology, Overlook Hospital, Summit, New Jersey. Manuscript received and accepted March 6,1986.
FIGURE 1. Posteroanterior and lateral chest radiographs taken 19 months before the patient’s death showing mild cardiomegaly and an intracardiac calclfic denslty (arrows) variously Interpreted at the time as involving the aortic or mitral valve but which, at necropsy, was present in the ventricular septum.