Clinical and hemodynamic diagnosis of pulmonary venous obstruction due to sclerosing mediastinitis

Clinical and Hemodynamic Diagnosis of Pulmonary Venous Obstruction Due to Sclerosing Mediastinitis* WILLIAM K . NASSER, M .D ., HARVEY FEIGENBAUM, M .D . and CHARLES FISCH, M .D ., E . .A .C .C .

Indianapolis, Indiana

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She denied the ingestion of drugs mr toxins prior to her illness, and there were no known allergies . Physical examination revealed a well developed young woman with a blood pressure of 105/70 mm . Hg . Numerous moist rales were present in the bases of both lungs . No pleural friction rubs were present . Examination of the heart revealed a left parasternal lifting impulse . The first heart sound was of normal intensity . The second sound was physiologically split with an accentuated pulmonic component . No munnurs, gallops, opening snaps, nor pericardial friction rubs were heard . There was no evidence of cyanosis, peripheral edema, clubbing, calf tenderness or jugular venous distension . Most of the laboratory tests, including complete blood count, blood urea nitrogen, blood sugar, serology and protein electrophoresis, were within normal limits . Numerous cultures of sputum, urine and lung tissue from the biopsy specimen for bacteria, acidfast bacilli and fungi revealed no growth . No lupus erythematosus cells were found during examination of several blood smears . Skin tests for tuberculosis and histoplasmosis were negative . An electrocardiogram showed right axis deviation but was otherwise not remarkable (Fig . 1) . Chest roentgenograms revealed the heart to he normal in size and configuration . A reticular infiltration throughout both lung fields partially obliterated tike left cardiac border (Fig . 2) . Phonocardiograms supported the cardiac findings . An ultrasoundcardiogram showed normal movement of the anterior mitral valve leaflet (Fig . 3) . The pulmonary function studies are listed in fable i . The findings were compatible with a moderate degree of restrictive pulmonary disease . Percutaneous lung biopsy resulted in insufficient tissue for adequate histologic examination .

ULMONARY hypertension due to occlusion of

the pulmonary veins is a rare disease . Its diagnosis has usually been made inadvertently at postmortem examination or during exploratory thoracotomy . However, recent reports have shown that the diagnosis can be suspected during life .' -4 A patient recently seen at the Indiana University Medical Center with this disease was diagnosed during life by means of cardiac catheterization . This paper presents the findings on this patient, describes a hemodynamic method for diagnosing this disease and suggests clinical factors important in the differentiation of pulmonary veno-occlusive disease from pulrnonary hypertension due to mitral valve disease or primary pulmonary hypertension . CASE REPORT

The patient, a 21 year old woman, was admitted to the Indiana University Medical Center in December 1965 because of hemoptysis and dyspnea . She was in good health until one year prior to admission when she coughed up bright red blood during her eighth month of pregnancy . Marked respiratory distress with cough and dyspnea was present during this time . A full-term, normal, viable fetus was delivered . The patient did relatively well following her pregnancy until three months prior to admission when fatigue and dyspnea with exertion became progressively worse . Orthopnea and paroxysmal nocturnal dyspnea were present for two weeks prior to admission . Complaints of chest pain, ankle edema, dizziness, syncope and recent fever were denied . There was no recurrent hemoptysis . Past history of known thrombophlebitis, bacterial endocarditis, or exposure to granulomatous diseases was denied .

Right, retrograde left, and transseptal left heart cath-

* From the Department of Medicine, Indiana University School of Medicine, and the Krannert Institute of Cardiology, Marion County General Hospital, Indianapolis, Ind . 46207 . This study was supported in part by the Herman C . Krannert Fund, U . S . Public Health Service Grants HE-6308, HTS-5363 and IIE-5749 and the Indiana Heart Association. voLUME 20, NOVEMBER 1967

725

Nasser, Feigenbaum and Fisch

726

eterization with selective eineangiocardiography was performed (Table it) . Pulmonary hypertension was found (Fig . 4) . The presence of a diastolic pressure gradient between the pulmonary capillary wedge and left ventricle and the absence of a diastolic pressure gradient between the left atrium and left ven-

urn" MEdr r M

.

NO

10

I

N

tricle suggested an obstruction to the flow of blood at the level of the pulmonary veins (Fig . 5) . Selective cineangiocardiography with injection of radiopaque dye into the left atrium showed a normalsized chamber without thrombi or abnormal membranes (Fig . 6) . The mitral valve opened well, and a normal-sized left ventricle was visualized . An injection of radiopaquc dye into the right atrium visualized normal pulmonary arteries without evidence of delayed opacification of the left atrium . Course : Because the patient's problem seemed to be due to an obstruction of the pulmonary veins for which surgical correction would be difficult, it was decided to follow the patient's course and ad-

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till 0 .5

Flu . 1 . Electrocardiogram shows right axis deviation but is otherwise not remarkable .

FIG . 3 . Ultrasoundcardiogram (UCG) with simultaneous edectrocardiogrom and phonocardiogram . Note the normal movement of the anterior leaflet of the mitral valve in diastole (points Fl, F and A) . The sudden descent from E to F essentially rules out immobility of the anterior mitral valve leaflet .

TABLE I Pulmonary Function Studies Predicted

Flo . 2 . Roentgenogram of the theist, pasteroanterior view . The heart is normal in size and configuration . A reticular infiltration throughout both lung fields partially obliterates the left cardiac border .

Inspiramry capacity (ml .) Inspiratnry reserve volume (ml .) F.xpiratory reserve volume (ml .1 Tidal volume (ml .) vital capacity (ml .) 3080 Residual volume (ml .) 1140 total Iungcapacit y (ml .) 4220 RV/TLC (`7,d <35 Functional residual capacity pnl .)* 2610 . ./mm ./min Diffusing capacity (H_) (ml 21 .8 IIg) Maximal breathing capacity (L . F11111.) 94 Forced expiratory volume. (1 sec.) (%) 83 400 Peak flow (L./min.) Maxivsal midcxpirarory Mw Forced expiratory flow (L ./min .) 200-1200 Airway resistance (cm . H,O/ 4/sec .) <2

Observed 1140 490 440 650 1580 1060 2640 40 1500 16 .2 55 92 280 130 117 2 .9

*By ptelhysmugraph. RV/TLC: = residual volume/total lung capacity . 'THE AMERICAN JOURNAL OF CARDIOLOGY



Diagnosis

of

Pulmonary

minister steroids if her clinical condition worsened . However, she moved to a different area and approximately four months later was admitted to another hospital because of marked dyspnea . 11 . was believed that, a definitive diagnosis was necessary, and the patient was subjected to operation for an open lung biopsy . The pleural surface was described as hyperemic, but no nodules not other abnormalities were seen . The right lung appeared quite thick and boggy, and the surface showed increased pleural lymphatic vessels . Biopsy specimens of the lung and pleura were obtained. However, following operation the patient experienced increasing dyspnea, became cyanotic and died . Postmortem Examination : The pertinent findings were limited to the middle mediastinum . A hard, white, fibrotic mass extended from the tracheal carina throughout the middle mediastinum involving lymph nodes, nerves and the pulmonary vessels'I 'here were organized, partially occlusive thrombi in the right pulmonary veins at their entrance into the left atrium. Microscopically, the mass consisted enrircly of intertwining bundles of fibrous tissue and chronic inflammatory cells . The left atrium and mitral valve were normal except for obvious thrombi at the site of entrance of the pulmonary veins . The left ventricle was normal . There was intimal hyperplasia of the pulmonary arteries and arterioles as well as hypertrophy of the right ventricle. Pulmonary edema and lymph stasis were also present in the pulmonary lobular septums . Passive congestion of the liver, spleen and kidneys was moderate . I here was no gross nor histologic evidence for granulomatous or other infectious disease processes in the pulmonary parenchyma .

Venous

TABLE n C.athetetization Data

Pressure lin m . Hni S

D

RA RV LA

2

LV

103 103

BA

na

diastolic pressure gradient between the pulmonary capillary wedge pressure and left ventricle and the absence of a diastolic pressure gradient between the left atrium and left ventricle suggest the presence of an obstruction to flow of blood at the level of the pulmonary veins

l ao

VOLUME 20 . NOVEMBER 1967

EE

70

w

i'2) 74 74 74 96 e7

50

a_

cardiac inde, it. ~ m,n .at= Resr Exorcise

PVR sI Resi (PA-PCW I Resr (PA-LA) E ...ri a PA-t.A :

2 .4 7s

5 = syxmliq 1) = diastolic ; Pot = e right v,niririe PA - puhnunarv RV . LA=lenavium ; LV=leftcentride ; BA

; R .A = right a arcry n ; P = pulmonary, rhrachlalarierv ; PVR=

pulmonary vascular resistance .

60

s0 Q

40

S

E E

30 20

0

with pulmonary capillary wedge pressure (PCW) and B, left atrial pressure (LA). 'I'hc presence of a

Pre

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11

Although sclerosing mediastinitis usually presents as obstruction to blood flow in the superior vena cava,s y it has been reported to cause obstruction to blood flow in the pulmonary veins . ] ('

10

M

PA P wedg r .7 .

0

Frc . 5 . A, recording of rimrdtaaeour left ventricular pressure (L V)

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2

COMMENTS

so

7 27

Obstruction

Fte. 4. 5'iroultaneoas recording of ere rocardiogram and pulmonary artery pressure (PA) showing pulmonary hypertension .

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Nasser, Feigenbaum and Fisch The present case is an example of pulmonary venous obstruction due to sclerosing mediastinitis without involvement of the superior vena cava . Clinically, there are several points which should aid in the differentiation of pulmonary hypertension due to obstruction of the pulmonary veins from pulmonary hypertension due to structural changes in the pulmonary vasculature or mitral valve disease (Table in) . However, the results of the present case would suggest that cardiac catheterization may be the best method available for diagnosing this condition . It is well known that an accurate pulmonary capillary wedge pressure is occasionally difficult to obtain in severe pulmonary hypertension . However, the presence of a good contour to the wedge pressure in pulmonary hypertension correlates well with an elevated direct left atrial pressure in patients with mitral valve disease

Selective cineangfocardiograrn with injection of radiopaque dye into the left atrium shows a normalsized chamber without thrombi or abnormal membranes. The mitral valve opens well, and the left ventricle is normal in size. Fm . G .

without pulmonary venous obstruction . This fact indicates that the wedge pressure can be accurate in pulmonary hypertension . Since the pulmonary capillary wedge pressure represents

TABLE III

Differential Diagnosis of Pulmonary Hypertension Due to Pulmonary Veno-occlusive Disease, Primary Pulmonary Hypertension and Mitral Valve Disease Pulmonary Veno-occlusive Disease History Previous rheumatic fever Hemoptysis Orthopnea PND Physical findings Intensity of SI Intensity of P, Murmurs Opening snap Pulmonary roles Electrocardiogram Atrial fibrillation LA hypertrophy Chest roentgenogram LV enlargement LA enlargement PV distension Kerley lines Mitral valve calcium Cardiac catheterization Pulmonary hypertension Elevated PCW pressure Elevated LA pressure

Primary Pulmonary Hypertension

Mitral Valve Disease

Uncommon Common Common Common

Uncommon Rare Uncommon Uncommon

Common Common Common Common

Normal Increased Usually absent Absent Frequently present

Normal Increased Usually absent Absent Usually absent

Increased Increased Usually present Frequently present Frequently present

Uncommon Absent

Uncommon Absent

Common May be present

Absent Absent Present Frequently present Absent

Absent Absent Absent Absent Absent

May be present Present Present Frequently present Frequently present

Present Present Absent

Present Absent Absent

Present Present Present

PND = paroxysmal nocturnal dyspnea ; S, = tint heart sound ; P, = pulmonic valve closure ; LA = left atrium ; LV = left ventricle ; PV = pulmonary vein ; PCW = pulmonary capillary wedge . THE AMERICAN JOURNAL OF CAROIOLOCY

Diagnosis of Pulmonary Venous Obstruction an indirect measurement of pulmonary venous pressure," a significant difference between the wedge pressure and direct left atrial pressure (Fig . 5) should immediately suggest possible obstruction at the level of the pulmonary veins . Botticelli et al 2 also found an elevated pulmonary capillary wedge pressure and a normal left atrial pressure in their patient with this disease . Brown and Harrisont suggested that the diagnosis may be made when the wedged catheter is flushed with saline solution and a high pressure falls gradually to the base level because the saline solution is trapped between the catheter tip and the occluded vein and slowly leaks away through the remaining patent vessels and lymphatics . Pulmonary venous hypertension is usually due to left ventricular failure or mitral valve disease and, rarely, to left atrial myxoma, cor triatriatuuu, or pulmonary vein stenosis . The normal left atrial pressure (Fig . 5) and left atrial cineangiocardiogram (Fig. 6) in the present case immediately placed the obstruction at the level of the pulmonary veins . Since the main pathologic lesion was an obliteration of the pulmonary venous beds - '- 10,12-17 either by fibrous proliferation of the media and intima or by thrombosis, or both, and since surgical palliative procedures are difficult, we recommended medical treatment . SUMMARY

A young woman was admitted to this hospital because of dyspnea, easy fatigability, hemoptysis, orthopnea and paroxysmal nocturnal dyspnea . Cardiac catheterization revealed an elevated pulmonary capillary wedge pressure and a normal left atrial pressure, suggesting an obstruction to blood flow at the levell of the pulmonary veins . Postmortem examination confirmed this diagnosis . The clinical differentiation of pulmonary hypertension due to pulmonary venoocclusive disease from pulmonary hypertension due to structural changes in the pulmonary vasculaturc or mitral valve disease is attempted . ACKNOWLEDGMENT We express our appreciation to the Department of Pathology, Washington University Medical School, for supplying us with the postmortems findings .

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REFERENCES 1 . IlEAsH, D ., SECEL, N . and BssHor . .1 . Pulmonary veno-occlusive disease. Circulation, 34 : 242, 1966 . 2 . BonsceLI.I, 7. T., SCHLUETER, D . P. and LANCE, K . L . Pulmonary venous and arterial hypertension due to chronic fibrous mediastinitis : Ilemodynamics and pulmonary function . Circulation, 33 : 862, 1966 . 3 . BROWN, C . H . and HARRISON, C . V. Pulmonary veno-occlusive disease . Lancet, 2 : 21, 1966 . 4 . STOVIN, P . G . I . and MITCHINSON, M . J . Pulmonary hypertension d ue. to obstruction of the intrapulmonary veins. Thorax, 20 : 106, 1965 . 5 . ERGANTAN, .1, and WADE, I . . J . Chronic librous mediastinitis with obstruction of the superior vena cava. J . Thoracic Sung ., 12 : 275, 1943 . 6 . FERGUSON, L. II ., JR . and WERNER, W . A . Obstruction of superior vena cava . Ois . ( .'here, 19 : 234, 1951 . 7 . GRAY, H . K . and SKINNER, 1. C . Constrictive occlusion of the superior vena cava ; report of 3 cases in which patients were treated surgically . Sung. Gynec . & Obst., 72 : 923, 1941 . 8 . KLASsEN, K . P ., ANDREws, N. C . and CURTIS, G . M . Diagnosis and treatment of superior vena eava obstruction . A .M.A . Arch. Surg ., 63 : 311, 1951 . 9 . SANCETTA, S . M . and McDONALD, H . E . Superior vena cava obstruction due to chronic mediastinitis and phlebitis. Ohio M . J ., 46 : 1173, 1950 . 10 . BINDELCLASS, J . L . and'FRUBOWiTZ, S . Pulmonary vein obstruction : An uncommon sequel to chronic fibrous mediastinitis, Ann . Inc . Med ., 48 : 876, 1958 . 11 . WOOD, P . Diseases of the Heart and Circulation, p . 176 . Philadelphia, 1956 . .f . B . Lippineott . 12 . EDWARDS, J, E . and BORCHELL, If . B . Multilobular pulmonary venous obstruction with pulmonary hypertension, A .M.A . Arch . Int . Med ., 87 : 372, 1951 . 13 . ANDREws, E . C ., JR . Five cases of an undescribed form of pulmonary interstitial fibrosis caused by obstruction of the pulmonary veins . Bull . Johns Hophins Hasp ., 100 : 28, 1957 . 14 . BREWER, D . B . and HUMPHREYS, D . R . Primary pulmonary hypertension with obstructive venous lesions . Brit . Heart J., 22 : 445, 1960. 15. HEATH, D. and EDWARDS, J . E. Histological changes in the lung in diseases associated with pulmonary venous hypertension. Brit. J . Die Chest, 53 : 8, 1959 . 16 . MALLORY, T. B. Case records of the Massachusetts General Hospital : Case 23511 . New England J. Med., 217 : 1045, 1937. 17 . CRANE, J . T . and GRIMES, O . F . Isolated pulmonary venous sclerosis : A cause of cor pulmonale . J . Thoracic & Cardiovas . Surg ., 40 : 410 .. 1960 .