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Cystic Medionecrosis of the Pulmonary Arteries Ethan A. Natelson, H. David Watts and Herbert L. Fred Chest 1970;57;333-335 DOI 10.1378/chest.57.4.333 The online version of this article, along with updated information and services can be found online on the World Wide Web at: http://chestjournal.chestpubs.org/content/57/4/333 CHEST is the official journal of the American College of Chest Physicians. It has been published monthly since 1935. Copyright 1970 by the American College of Chest Physicians, 3300 Dundee Road, Northbrook, IL 60062. All rights reserved. No part of this article or PDF may be reproduced or distributed without the prior written permission of the copyright holder. (http://chestjournal.chestpubs.org/site/misc/reprints.xhtml) ISSN:0012-3692 Downloaded from chestjournal.chestpubs.org by guest on July 29, 2010 1970, by the American College of Chest Physicians Cystic Medionecrosis Pulmonary Arteries* of the Ethan A. Natelson, M.D.,*O H. David and Herbert L. Fred, M.D., F.C.C.P. Watts, M.D.,t Cystic medionecrosis (CMN) characteristically affects the aorta. That it also may involve vessels in the lung is not widely appreciated. Two patients are presented in whom cystic medionecrosis of the pulmonary arteries accounted for dramatic clinical and pathologic findings. One had massive dilatation of both main pulmonary arteries together with a healed dissecting aneurysm extending enormous from the aortic saccular aneurysm arch to the iliac bifurcation. The other of the left main pulmonary artery. had an C ystic medionecrosis (CMN) is a specific type of degeneration involving elastic fibers and muscle elements within the media of the arterial wall.12 It is characterized histopathologically by numerous mucoid cysts filled with metachromatically staining material.3 The affected artery is predisposed to an- eurysmal dilatation, medial dissection, and sponta- neous rupture.4" Cystic medionecrosis characteristically occurs in the aortae of patients with the Marfan syndrome.347 Sometimes, however, it appears as an isolated arter- ial lesion.8#{176} This communication concerns two patients in whom CMN of the pulmonary arteries caused strik- ing clinicopathologic findings. CASE REPORTS CASE 1 A 63-year-old Negro woman entered the hospital because of increasing pedal edema and breathlessness of three weeks' duration. Additional historical information never became available. The patient appeared critically ill. Her blood pressure was 150/70 mm Hg; pulse rate, 70 heats per minute; respiratory rate, 40 per minute; and oral temperature, 99#{176}FS. he weighed 155 pounds and her body habitsis was normal. She had no specific skeletal or ocular manifesta- tions of the Marfan syndrome. Her jugular venous pres- sure was greatly elevated. The precordium was hyperdy- namic with an apical impulse in the midaxillary line. A grade ILI/VI systolic ejection nuurmur and a IV/VI diastolic decrescendo murmur were loudest to the left of the #{176}Fromthe Department of Internal lege of Medicine, Houston, Texas. #{176}#{176}ResiPdheynstician in Medicine. Formerly Senior Medical Student, Medicine. Associate Professor of Medicine. Medicine, Baylor Baylor Col- College of sternum in the third and fourth interspaces. Rales were audible at the bases of both lungs. A nontender edge was palpable 10 cm below the right costal hepatic margin. Both legs were massively edematous. Carotid, brachial, and femoral arterial pulsations were normal, bilaterally. An electrocardiogram showed atrial fibrillation, occa- sional ventricular premature contractions, and a pattern of right ventricsilar hypertrophy. Chest roentgenOgram (Fig 1) disclosed cardiomegaly, pulmonary venous con- gestion, and large, bilateral hilar masses. On the second hospital day catheterization of the right side of the heart revealed a pulmonary arterial pressure of 98/35 mm Hg (mean, 60 mm Hg) and a simultaneous brachial arterial pressure of 142/59 mm Hg (mean, 89 mm Hg). Attempts to measure pulmonary capillary wedge pressure were unsuccessful. strated massive dilatation visualization of the more The patient's status Angiography (Fig 2) demon- of the major pulmonary arteries; distal branches was poor. rapidly worsened. Multiple psi1- monary thromboemboli seemed likely. Three days after admission, thoracotomy vealed no thromboemboli. with pulmonary arteriotomy re- Shortly thereafter, the patient suffered cardiac arrest and died. Autopsy disclosed aneurysmal dilatation of the main pulmonary arteries and a healed dissecting aneurysm extending from the arch of the aorta to the iliac bifurca- tion. Cystic medionecrosis was evident throughout the pulmonary branches. arterial tree as well as the aorta Cause of death was not apparent. and its major CASE 2 A 19-year-old Negro man was admitted to the hospital complaining of shortness of breath and easy fatiguing of eight years' duration. He had suffered from recurrent sipper respiratory infections throughout childhood. On physical examination the patient appeared much younger than his chronological age. He was 62 inches tall and weighed 80 pounds. His pulse rate was 70 heats per minute; respiratory rate, 19 per minute; blood pressure, 100/70 mm Hg; and oral temperature, 99#{176}F.He had a webbed neck and an asymmetrical chest with prominence 333 Downloaded from chestjournal.chestpubs.org by guest on July 29, 2010 1970, by the American College of Chest Physicians 334 NATELSON, WATTS AND FRED FIGURE masses. monary 1 (Case 1), left. Chest roentgenogram FIGURE 2 (Case 1), right. Angiogram arteries. of the left hemithorax. The precordium was active with a right and left ventricular lift. A low pitched to-and-fro murmur was audible in the fourth left intercostal space. Chest roentgenogram (Fig 3) demonstrated dilatation of the right pulmonary artery and a large density filling the anterior mediastinum and much of the left hemi- thorax. Electrocardiogram showed a sinus rhythm and a depicting demonstrating cardiomegaly enormously and bilateral hilar dilated major pul- pattern of biventricular hypertrophy with right atrial enlargement. Cardiac catheterization revealed a pulmonary arterial pressure of 50/25 mm Hg (mean, 40 mm Hg) and a simultaneous aortic pressure of 95/70 mm Hg. Angiocardiogram demonstrated a left-to-right shunt through a ventricular septal defect; the catheter delineated a large, saccular aneurysm of the left main pulmonary artery (Fig 4). 4 FIGURE 3 (Case 2). Chest roentgenogram showing dilatation of right large mass in left hemithorax. FIGURE 4 (Case 2). Chest roentgenogram coursing through right atrium and right ventricle before entering Essentially all of catheter to left of spine lies within large saccular pulmonary artery and disclosing angiocatheter main pulmonary artery. aneurysm of left main pulmonary artery. CHEST, VOL. 57, NO. 4, APRIL 1970 Downloaded from chestjournal.chestpubs.org by guest on July 29, 2010 1970, by the American College of Chest Physicians CYSTIC MEDIONECROSIS OF PULMONARY ARTERIES 335 - FIGURE 5 (Case 2). Photomicrograph demonstrating extensive microcystic (Hematoxylin and eosin, original of pulmonary change of the magnification X artery media. 360.) At thoracotomy an interventricular septal defect 1.5 cm in diameter was closed. The aneurysm of the left main pulmonary artery was repaired by partial resection and angioplasty; its wall contained changes typical of cystic medionecrosis (Fig 5). Con'sirs These two patients are noteworthy because pul- monary arterial disease incident to CMN dominated their clinical presentation. Moreover, successful re- pair of a pulmonary arterial aneurysm resulting from CMN has not been documented previously. Detailed information on CMN involving the pul- monary vasculature is sparse. In the Marfan syn- drome the spectrum of such lesions ranges from idiopathic dilatation of the pulmonary arteries" and minimal abnormalities of the elastic fibers of the media312-14 to typical CMN.48 Only rarely do such changes overshadow those in the aorta.15-17 Cystic medionecrosis of the pulmonary arteries also occurs in association with long-standing pulmonary hypertension6 and leads occasionally to dissection and rupture of the affected vessels.6,16,ls The rela- tion between pulmonary hypertension and CMN of the pulmonary vasculature remains unclear. Widely disseminated cystic medionecrosis in our first patient and phenotypic resemblance of the sec- ond patient to others with unusual manifestations of the Marfan syndrome1#{176} suggest congenital rather than acquired arterial defects. We recognize, how- ever, that the vascular lesions in both patients may have had no connection with the Marfan syndrome. ACKNOWLEDGMENT:Drs. J. 0. F. Roehm, present the second Jr., and patient. J. F. Howell, S. A. Kinard, R. E. Fechner permitted us to 1 CSELL, REFERENCES 0.: Wandnekrosen der Aorta als selbstandige Erkrankung Virchow 2 ERDHEIM, und ihre Beziehung sur spontanruptur, Arch. Path. Anat., 270:1, 1928. J.: Tvledionecrosis aortae idiopathica, Virchow Arch. Path. Anat., 273:454, 1929. 3 ROARK, J.W.: The Marfan syndrome. Report of one case with autopsy, special histological study, and re- view of the literature, Arch. Intern. Med., 103:123, 1959. 4 MCKUSICK, V.A.: The cardiovascular aspects of Mar- fan's syndrome: tissue, Circulation, a heritable disorder 11:321, 1955. of connective 5 GRONDIN, CM., STEINBERG, CL., AND EDWARDS, J.E.: Dissecting aneurysm complicating Marfan's syndrome (arachnodactyly) 77:301, 1969. in a mother and son, Amer. Heart 1., 6 LEVY, H.: Partial rupture of pulmonary artery with lesions of medionecrosis in a case of mitral stenosis, Amer. Heart 1., 62:31, 1961. 7 MARFAN, A.B.: Un cas de deformation cong#{233}nitale des quatre membres, plus prononc#{233}e aux extr#{233}mit#{23ca3r}asc- t#{233}ris#{p2ar33}e l'allongement des os avec un certain degr#{233} d'amincissement, Bull. Soc. Med. Hop. Paris, 13:220, 1896. 8 WEAVER, W.F., EDWARDS, J.E., AND BRANDENBURG, R.O.: Idiopathic dilatation of the aorta with aortic valvular syndrome, insufficiency: a possible forme fruste Mayo Clin. Proc., 34:518, 1959. of Marfan's 9 CHAPMAN, D.W., BEAZLEY, H.L., PETanSON, P.K., WEBB, J.A., AND COOLEY, D.A.: Annulo-aortic with cystic medial necrosis, Amer. I. Cardiol., ectasia 16:679, 1965. 10 GOLDEN, R.L., AND LAKIN, H.: The forme fruste in Marfan's syndrome, New Eng. I. Med., 260:797, 1959. 11 BEFELER, B., MACLEOD, C.A., BAUM, G.L., AND SCHWARTZ, H.: Idiopathic dilatation of the pulmonary artery, 12 TUNC, Amer. I. Med. Sci., 254:667, H., AND LIEBOW, A.A.: 1967. Marfan's syndrome. Observations at necropsy: with special reference to medionecrosis of the great vessels, Lab. Invest., 1:382, 1952. 13 BAER, R.W., TAUSSIG, Congenital aneurysmal H.B., AND OPPENHEIMER, E.H.: dilatation of the aorta asso- ciated with arachnodactyly, Bull. Hopkins Hosp., 72: 309, 1943. 14 HEADLY, Unusual postmortem RN., CARPENTER, H.M., AND features of Marfan's syndrome studies, Amer. I. Cardiol., SAWYER, CC.: including two 11:259, 1963. 15 ANDERSON, M., AND PRA1-r-TH0MA5, HR.: Marfan's syndrome, Amer. Heart 1., 46:911, 1953. J.:16 BEST, Dissecting aneurysm of the pulmonary artery with multiple cardio-vascular abnormalities and pul- monary hypertension, Med. J. Australia, 2:1129, 1967. 17 KAPANCI, Y.: MCdionCcrose et an#{233}vrysmes diss#{233}quants des art#{233}res intra-pulmonaires. Description d'un cas chez nourisson et #{233}tuden-iorphog#{233}n#{233}tiqudee Ia m#{233}dion#{233}crose type Csell-Erdheim, Frankfurt Z. Path., 74:425, 1965. 18 RAvINES, H.T.: Dissecting hematomas monary arteries in a case of pulmonary associated with patent ductus arteriosus, Cardiovasc. Surg., 39:760, 1960. of intrapulhypertension I. Thorac. Reprint requests: Dr. Fred, Director St. Joseph Hospital, 1919 LaBranch, of Medical Education, Houston, Texas 77002. CHEST, VOL. 57, NO. 4, APRIL 1970 Downloaded from chestjournal.chestpubs.org by guest on July 29, 2010 1970, by the American College of Chest Physicians Cystic Medionecrosis of the Pulmonary Arteries Ethan A. Natelson, H. David Watts and Herbert L. 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