Primary Dissecting Aneurysm of the Right Coronary Artery with Survival

FORKER ET AL Allowing recollapse and/or occluding the right pulmonary artery as suggested by Sautter et a14 may have proved helpful, and would have been tried had the patient not gradually rallied. Alcohol nebulization was used but was of no help, a finding also noted by o t h e ~ s . ~ The high-flow air leak prevented re-expansion and vented fluid into his chest bottle. The protein-rich edema did not then soil the left lung, but it clotted frequently and blocked the chest tubes causing the loculated cortex for which decortication was necessary. This external protein loss does not fully explain the hemoconcentration, hypovolemia or the severe hypoalbuminemia. Albumin production is depressed with hypoxia, shock and stress;' but liver functions remained normal, and the transdiaphragmatic liver biopsy at decortication was also normal. The serum albumin gradually rose to normal levels by the third month after re-expansion, when he returned to the outpatient clinic. The precise etiology of this form of pulmonary edema is unclear. Warren et alRdemonstrated fairly conclusively that hypoxia and increased negative pressure in the chest produce increased pulmonary lymphatic flow. It has long been known that atelectasis is associated with decreased perfusion to that area. Wahrenbrock et ale suggested that this shift in perfusion stabilized after a few hours, but with complete atelectasis over a long period this decrease in perfusion may be more profound. Surfactant levels decrease in the atelectatic lung if the pulmonary artery is occluded. Surfactant deficiency would decrease compliance and increase the transthoracic pressure required to re-expand the lung, and therefore promote lung lymph flow or overflow as edema fluid. It is curious that in this and other experiences the lung re-expanded fully initially, and when the edema developed, compliance deteriorated as manifest by partial recollapse. This suggests that the surfactant levels were not decreased with initial re-expansion. Hypoalbuminemia facilitates the formation of pulmonary

edema." although alhuminemia does not cause spont t ~ ~ ~ c opulmonary ux edema,' It is probable that this constellution of edemu-predisposing factors is responsible for. tllc prodrrction of the pulmonary edema seen in this syndrome, rather than any one causative factor. The fever ocn~rringduring this process is interesting and ~rnexplnined," It remains u curiosity that all infants do not develop this syndrome shortly after birth; that children with (*ventration of the diaphragm do not dsvelap it when that condition is corrected; and that only an occasional ndult patient with complete sudden re-expansion does dt*vslopit. In lieu of a good nnimal model and further elucidatian or the etiology and effective therapy of this entity, there is, st present, no way to predict this prohlem, and its mnllsgeme~ltmllst remain symptomatic.

3

4

5 6 7 8

9 10 11

lapsed lung due to a pneumothorax: A clinical and experimental study. Surg Forum 9:367-371, 1958 Ziskind MM, Weill H, George RA: Acute pulmonary edema following the treatment of spontaneous pneumothorax with excessive negative intrapleural pressure. Am Rev Resp Dis 92:632-636, 1965 Sautter RD, Dreher WH, MacIndoe JH, et al: Fatal pulmonary edema and pneumonitis after reexpansion of chronic pneumothorax. Chest 60:399-401, 1971 Trapnell DH, Thurston JGB: Unilateral pulmonary edema after pleural aspiration. Lancet 1: 1367-1369, 1970 Humphreys RL, Berne AS: Rapid reexpansion of pneumothorax. Radiology 96:509-512, 1970 Rothschild MA, Oratz M, Schriber SS: Albumium synthesis (11) N. Engl J Med 286:816-821, 1972 Warren MF, Peterson DK, Drinker CK: The effects of heightened negative pressure in the chest together with further experiments upon anoxia in increasing the flow of lung lymph. Am J Physiol 137:641-648, 1942 WahrenBrock EA, Camco CJ, Arnundsen DA, et al: Increased atelectatic pulmonary shunt during hemorraghic shock in dogs. J Appl Physiol29:615-621, 1970 Finley TN, Tooley WH, Swenson EW, et al: Pulmonary surface tension in experimental atelectasis. Am Rev Resp Dis 89:372-378, 1964 Guyton AC, Lindsey AW: Effect of elevated left atrial pressure and decreased plasma protein concentration on the development of pulmonary edema. Circ Res 7:649657. 1959

Primary Dissecting Aneurysm of the Right Coronary Artery with survival* Alan D. Forker, M.D.; Robert C . Rosenlof, M.D.,O0 Walt F . Weaver, M.D., F.C.C.P.; Stephen W . Caroeth, M.D., F.C.C.P.; and Herbert E . Reese, M.D.

Following acute inferior wall myocardial infarction, a

56i-yarrc-~ldman gradually dsvslapd madtsd cardiac anlargement due to hemoperlcardlum. In addition to an occluded rlght coronary artery, coronary arterlography damondrated extralumlnal dye around the artery. Surglcal lmpectloa revealed adventltlol dlaoectlon of blood and reroivlng hematoma. Three and one-half yean later, he b asymptomatic and b the fi* known rurvlvor of a primary d b c t l n g aneuryrm of a coronary artery. wenty-four cases of primary dissecting aneurysm of a T coronary artery were racently reviewed hy Claudon et a1.I No previaus patient with this condition has been

correctly diagnosed during life, and none haa had coronary arteriography, The purpose of this report is to describe the clinical, angiographic, and surgical Bndings in tho Brst known survivor with a primary dissecting aneurysm of a coronary artery,

r rob

I P~~~lcnrt A : Citetl by Carlson et al (ref 2 ) 2 Cnrluon RI, Klnssrn KL, Gollan F, et al: P~~lnlonary

c.dt\n~ufollowing the rnpid rc*expnnuion of a totally col-

the Cardiovascular Labarato and I3epart&ent " f Thoradc Surgery, Bryan Memorial %r ital, Lincoln, Neb. *Conatlltant in Internal Medicine, & a d !amadtan Hospital, Kearney, Neb. Re1)rint rw rrssts: Dr. Wsaoer, 1512 First National Bank Build-

ing, ~ i n c o % 68508

CHEST, VOL, 64, NO, 5, NOVEMBER, 1973

PRIMARY DISSECTING ANEURYSM

FIGURE1. PA chest x-ray film on March 13, 1969. showing moderate cardiac enlargement with a globular contour, compatible with a pericardial effusion.

A 56-year-old white man was admitted to Good Samaritan Hospital, Kearney, Nebraska, on February 18, 1969, with a three-day history of substernal chest pain compatible with myocardial ischemia. The admission electrocardiogram showed evidence of acute inferior wall myocardial infarction. Complete heart block developed during the first week of hospitalization, with an adequate ventricular rate of 50 per minute and a normal QRS duration of 0.08 second. Pacemaker therapy was not utilized. The patient was treated in the usual manner for acute myocardial infarction, including coumadin anticoagulation. Gradually over the next four weeks, evidence of rightsided fluid retention developed, as manifested by dyspnea, distended neck veins, and a mildly enlarged liver. He was treated with digoxin and furosemide. but in spite of this, progressive cardiac enlargement developed ( Fig 1 ) . He was referred to Bryan Memorial Hospital for further evaluation, with the presumptive diagnosis of pericardial effusion. On initial evaluation a 26 mm Hg pulsus paradoxus and moderate cardiac enlargement by chest x-ray examination were present. Pericardiocentesis removed approximately 200 ml of blood, which would not clot. On March 27, 1969, this patient underwent left heart catheterization and selective coronary arteriography without difficulty. The percutaneous Seldinger technique via the right femoral artery was utilized, filming on 16 mm cine film (illustrations could not adequately be reproduced from this film). The left ventricular pressure was 106/14 mm Hg. The left ventriculogram revealed fairly good contractility of the anterior wall, but the inferior wall appeared to be akinetic. The extraluminal shadow (pericardial space) was moderately increased in size. On selective catheterization of the right coronary artery ostia, arterial pressure was noted to damp quite easily. Injected contrast media appeared to wash out very slowly. The right coronary artery was totally occluded at the level of the acute marninal - branch. No aneurvsmal dilatation was seen. However, loose dye appeared in a space surrounding the right coronary ostial area, and appeared to spread out for several centimeters. The dye was not washed away, as one would expect when entering an atrial chamber

CHEST, VOL. 64, NO. 5, NOVEMBER, 1973

or aorta. An area of 50-70 percent stenosis of the proximal left circumflex coronary artery was seen. A 70-90 percent stenosis was present in the proximal left anterior descending coronary artery, just proximal to the origin of the main diagonal branch. Aortic root cine angiogram revealed no aortic insufficiency or dissection of the ascending aorta; however, dye was noted to remain in portions of the right coronary artery for 30 to 45 seconds following cessation of injection. Even though no ascending aortic dissection was demonstrated by aortic root angiography, the final clinical impression was that the patient had suffered an aortic dissection with involvement of the right coronary artery. On April 3, 1969, the patient underwent open heart surgery. Upon entering the pericardial space, 100-150 ml of serous fluid was found. The parietal and visceral pericardiurn was covered with a fibrinous exudate. Upon exposure of the right coronary artery, the usual findings of a dissection were found, with adventitial dissection of blood and resolving hematoma (Fig 2). The vessel was also highly tortuous and arteriosclerotic, with a thickened wall. The posterior aspect of the heart revealed a fresh myocardial infarction high on the posterior wall. There was no evidence of dissection of the ascending aorta. No aneurysmal dilatation of the right coronary artery could be found, nor was there a localized or definite site of leakage. It was decided not to ligate the right coronary artery. Bilateral internal mammary artery implants were placed into the anterior and posterior aspects of the left ventricle. The patient tolerated the procedure well, and the postoperative course was uncomplicated. No further evidence of hemopericardium developed. In November, 1970, the patient returned for a routine restudy of his internal mammary artery implants by the Seldinger percutaneous technique. Selective visualization of the internal mammary implants was technically unsuccessful. Repeat selective coronary arteriograms were obtained on 35 mm cine 6lm. The left main coronary artery showed some minor insignificant narrowing. The proximal left anterior descending coronary artery was severely diseased, showing an area of 95 percent stenosis just prior to the origin of the main diagonal branch, followed by an area of 90 percent stenosis, with aneurysmal dilatation between the two high grade obstructions. A good sized normal distal vessel was

FIGURE2. Exposed right coronary artery demonstrating the surrounding hematoma.

MARIN-GARCIA ET AL visualized. The left circumflex coronary artery showed an area of 50 percent narrowing prior to its bifurcation into the obtuse marginal branch. In addition, there was a significant 70-80 percent stenosis at the origin of the obtuse marginal branch, with no distal circumflex coronary artery visualized. The right coronary artery was a large vessel and was totally occluded at the acute margin, with no distal vessel visualized. The left ventriculogram was still abnormal, showing an akinetic proximal inferior wall, but the apex and anterior wall appeared to contract normally. No further surgical therapy was recommended because the patient was asymptomatic. At the present time, three and one half years after myocardial infarction and dissection of the right coronary artery, the patient is without cardiac symptoms, and is employed full time as a maintenance worker in a county highway department.

After reviewing the available autopsy data on the previously reported cases of primary dissecting aneurysm of the coronary artery, Claudon et all postulated that in the presence of acute myocardial infarction, the following features may suggest a dissecting aneurysm of a coronary artery: 1) relative youth of a patient, since only five of the patients were older than 45 years of age; 2) frequent occurrence in a woman, since 20 of the reported cases were in women; 3) frequently present post partum, since eight of the women were postpartum. Also, they emphasized that "atherosclerosis appears to be highly uncommon" among the patients with primary dissection of a coronary artery. Findings in our patient did not correlate with these criteria. He was a 56-year-old man with severe three vessel arteriosclerotic coronary artery disease who presented with pericardial effusion secondary to hemopericardium. If a patient presents with acute myocardial infarction and bloody pericardial effusion, not only should a dissecting aneurysm of the ascending aorta be considered, but also primary dissection of the coronary artery. Both are indications for emergency coronary arteriography. The relationship of coumadin anticoagulation therapy to the dissection is unknown, but the presence of anticoagulants probably allowed hemopericardium to develop more easily. If this patient had been seen at the present time, most likely the surgical approach would have been ligation of the proximal right coronary artery, with construction of an aortocoronary saphenous vein graft to the distal right coronary artery.

Congenital Absence of the Pulmonary Valve Associated with Tricuspid Atresia and Intact Ventricular septum* Jose Matin-Garcia, M.D.; Juan Roca, M.D.; Leonard C . Blieden, M.B.B. Ch.; RusseU V . Lucos, Jr., M.D.; and Jesse E. Edwards, M.D.

A case of congenital absence of the pulmonary valve associated with tricuspid atresia and intact ventricular sep turn is presented. In this case, the presence of a patent ductus arteriosus and absent pulmonary valve allowed the right ventricle to receive blood from the pulmonary artery. The right ventricle, however, represented a blind pouchlike structure which was not an essential part of the circulation.

I

n tricuspid atresia, when two ventricles are present, usually the right ventricle is filled through a ventricular septa1 defect. Uncommonly, the ventricular septum is intact. Under this condition, the pulmonary valve is also atretic and the right ventricle is a nonfunctioning, slitlike structure in the wall of the left ventricle. We have observed a situation which, to the best of o w knowledge, has not previously been reported. In this case, the tricuspid valve was atretic and, although two ventricles were present, these were separated by an intact ventricular septum. An additional unusual feature was the absence of the pulmonary valve, allowing filling of the right ventricle through the pulmonary artery. The right ventricle was an appendage of the pulmonary arterial system, having no hemodynamic significance. In view of the unusual nature of this condition, we are prompted to place it on record. 'From The Children's Hospital, Seguridad Social, Barcelona, Spain, The Department of Patholo United Hospitals-Miller Division, St. Paul. Minnesota, a n r d e Departments of Pediatrics and Pathology, University of Minnesota, Minneapolis, Minn. This study was sup orted b Public Health Service Research Grant 5 R01 ~ ~ 0 8 3 anhesearch 94 Training Grant 5 TO1 HUE570 from the National Heart and Lun Institute. Reptint requests: Dr. Edwards, 125 West C o h g e Awnue, St. Paul 551 02

REFERENCE 1 Clar~donDC,Claudon DB, Edwards JE: Primary,dissecting aneurysm of mronary artery, a cause of acute myocardial ischemia. Circi~lation45:259-266, 1972

FIGURE 1. In frontal plane note evidence of right atrial enlargement and biventricular hypertrophy. QRS axis is +Boo.

CHEST, VOL. 64, NO. 5, NOVEMBER, 1973