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jDIII/CABE REPORT

PULMONARY VENO-OCCLUSIVE DISEASE

Marcos Calderon and John A. Burdine Baylor College of and St. Luke's Episcopal-Texas Children's Hospitals, Houston, Texas

Pulmonary veno-occiusive disease may be a of purulent sputum, and low-grade fever of 3 days not uncommon cause of pulmonary perfusion duration. The patient had experienced frequent epi defects. In the case described in this report, the sodes of pneumonia throughout the previous 15 years condition produced a unilateral absence of per and gave a history of discharge from the military fusion due to asymmetric involvement of the service due to a persistently abnormal chest roent major pulmonary . The etiology of the dis genogram. At the age of 27, he developed a produc ease is obscure although it has been associated tive cough, intermittent mild wheezing, and dyspnea with the nephrotic syndrome, viral infections, on exertion. These symptoms worsened during the otitis media, thrombophiebitis, and toxoplas next few years and two episodesof hemoptysis were mosis. Since approximately one-third of the re documented during the year before the current ad ported patients have been less than 16 years of mission. One month prior to admission, a radiograph age, the possibility of congenital malformation of the chest revealed right-sided pleural effusion and of the veins has also been considered although some evidence of pulmonary fibrosis (Fig. 1) . The this circumstance is unlikely in view of the typi patient had been a heavy smoker since his teens, cal pathologic changes. The extensive, progres averaging one to two packs per day. sive nature of the venous obstruction usually Physical examination revealed a thin, black male leads to death within a few months or years. with moderate respiratory distress. Evidence of bronchial obstruction occurs due was 100/80, rate 120/mm, and respiration 30. to of bronchial mucosa and engorgement Jugular venous pressure was elevated and breath of the bronchial veins. No effective is sounds were decreased with moist expiratory rales available. The clinician should be aware of this at the right base. The heart was enlarged with a condition in the differential diagnosis of prominent S-3, the liver was enlarged and tender, perfusion and ventihition abnormalities. and there was moderate clubbing and cyanosis of the fingers and toes as well as dependent pitting edema. An electrocardiogram showed right axis deviation, Unilateral absence of lung perfusion with relatively prominent p-waves, and incomplete right bundle unaffected ventilation has been observed as a result branch block. Vital capacity was 50% of predicted of pulmonary thromboembolism, congenital vascular and there was marked expiratory airway obstruc abnormalities, and tumors located in the hilar regions tion. Diffusing capacity was reduced. The arterial (1,2) . We have recently encounteredsuch an image ftO2 (43 mmHg) was very low and there was a pattern in a patient with pulmonary veno-occlusive slight elevation of the pCO2 (49 mmHg). Sputa were disease, an unusual and poorly recognized entity as negative for acid-fast bacilli and fungi, with cultures sociated with severe pulmonary arterial growing alpha streptococci and enterococci. An in and right heart failure. Antemortem diagnosis of this termediate strength PPD was negative. The patient condition has been rare (3,4) . Since many of the underwent cardiac catheterization with the major are common to thromboembo finding being elevation of pulmonary arterial pressure lism, an erroneous diagnosis and misguided therapy may occur.

CASE REPORT Received Oct. 2, 1973; revision accepted Dec. 3 1, 1973. A 34-year-old black male was admitted to the For reprints contact : Marcos Calderon, Dept. of Radiol ogy, Sectton of , Baylor College of Mcdi hospital with pleuritic chest , cough productive cine, Houston, Tex. 77025.

Volume 15, Number 6 455 CALDERON AND BURDINE

pathologists who have become accustomed to focus ing most of their attention on the pulmonary and bronchoalveolar system. Occurring largely among the young male population, the disease is characterized by an insidious onset with slowly pro gressiveright heart failure and respiratoryinsuffi ciency leading to death in most cases within a few years. The venous obstruction is primarily thrombotic with prominent secondary inflammatory change. No specific etiologic agent has been proven nor is there evidence of a generalized disorder of the mechanism. The disease has been observed in asso ciation with the nephrotic syndrome, viral infections, otitis media, thrombophlebitis, and toxoplasmosis (5,6) . Since approximately one-third of the patients have been less than 16 years of age, the possibility of congenital malformation of the veins has been considered. No effective therapy is available. F1G.1. Chestroentgenogram1 monthbeforeadmissionre In spiteof markedpulmonaryvenousobstruction, veals right-sidedpleural effusionand evidenceof pulmonary fibrosis.

(53 mmHg) and a normal wedge pressure (8 mmHg). Lung perfusion images obtained with D9mTc@human albumin microspheres (HAM) revealed an absence of perfusion within the right lung and some uneven ness in the distribution of perfusion on the left (Fig. 2A) . Ventilatory images obtained during inhalation and washout of 133Xe indicated diminished ventila tion throughout the right lung, more marked at the apex, but a relative preservation in comparison with perfusion (Fig. 2B). Eight days after admission the patient died from acute cardiorespiratory failure secondary to sudden

severe hemoptysis. A Postmortem examination disclosed extensive phle bitis of the pulmonary veins and venules, with marked polymorphonuclear inifitration, subintimal fibrosis, congestion, and focal hemorrhage. and ob literation of the lumina of the smaller vessels were observed and the main pulmonary veins from the right lung were severely stenosed. In addition, vas @ cular neoformation was extensive with evidence of .. recanalized thromboses in many and yen . :@‘@;r @ , .‘ @ ules but no sizable thromboemboli were noted. The ‘‘..,@. pulmonary arteries showed evidence of hypertensive change and extensive chronic bronchitis was observed in the conducting airways.

DISCUSSION B Although few casesof pulmonary venousocclusive FIG.2. (A)“mTc-HAMimage(posterior)indicatesabsenceof disease have been reported, Liebow believes that the perfusion within right lung and some irregularity in perfusion on condition is not rare (5) . Abnormalities in the pul left. (B) Single-breath “Xeventilation images (posterior) reveals marked decrease in ventilation throughout right lung but relative monary veins are probably overlooked frequently by preservation of ventilation in comparison with perfusion.

456 JOURNAL OF NUCLEAR MEDICINE PULMONARY VENO-OCCLUSIVE DISEASE the pulmonary capillary wedge pressure was normal with this disease develop progressive airway obstruc in our patient and in others described in the literature tion, produced at least in part by edema of the bron (7) . This phenomenon is most likely related to the chial mucosa and engorgement of the bronchial veins, circumstance wherein the wedged catheter registers and this phenomenon tends to equalize the influence the low pressure in collateral venules connecting with on ventilation and perfusion. the bronchial veins rather than reflecting pressure within the usual but obstructed venous channels. REFERENCES When venous obstruction occurs at the level of the 1. WHITE RI, JAMES AE, WAGNER HN : The significance left atrium, the wedged catheter records an elevated of unilateral absence of pulmonary perfusion by lung back pressure produced by the pulmonary arterial scanning. Am I Roentgenol Radium Ther Nuci Med 111: influence on interconnecting veins which remain pat 501—509,1971 ent (8) . Unilateral predominance of stenosis involv 2. STJERNHOLM MR. LANDIS GA, MARCUS Fl, et al: ing the main pulmonary veins has not been reported Perfusion and ventilation radioisotope lung scans in stenosis and remains an enigma in our patient. The abnor of the pulmonary arteries and their branches. Am Heart I 78: 37—42,1969 malities in the smaller veins and venules were dis tributed in a similar fashion throughout both . 3. BROWN CH, HARRISON CV: Pulmonary veno-occlusive disease. Lancet 2: 61—65,1966 When the pathophysiologic effects of disease in 4. C0NTIS G, FUNG RH, VAWTER GF, et al: Stenosis fluence the pulmonary ventilatory and perfusion and obstruction of the pulmonary veins associated with pub mechanismsto a dissimilar extent, the normal small monary artery hypertension. Am I Cardiol 20: 718—724, imbalance between ventilation (V) and perfusion 1967 (0) iscompounded,resultingin ineffectivegasex 5. LIEBOW AA : The Lung. Baltimore, Williams & Wilkins change. Such is often the case in uncomplicated Co. 1968, pp 360—365 bronchitis or pulmonary . Because the pul 6. Clinicopathobogicalconferenceat Royal Postgraduate . A case of veno-occlusive disease. Br Med monary arterial tree is an end-artery system, obstruc I 1: 818—821,1968 tion of either the input (arterial) or exit (venous) 7. HEATH D, SEGEL N, BISHOPJ: Pulmonary veno circuit will result in diminished perfusion. Thus, an occlusive disease. Circulation 34: 242—248, 1966 increase in V/Q ratio is not surprising in patients 8. CARRINGTON CB, LIEBOW AA : Pulmonary veno-occlu with pulmonary venous occlusion. However, patients sive disease. Hum Pathol I : 322—324,1970

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