Massive Hemoptysis Secondary to Pulmonary Arteriovenous Fistula

and

minimal-change

type-3

disease.5

pneumococcal

patients with antithymocytic normal

has

thymoma

to the

not

been

best

occurs

predicted knowledge,

in the

in

thymoma.9

coccal 9 Lee

on

of ab-

these

clin-

10

minimal-change by

Shalhoub;

this

association

therapy.b0 in

this

elderly

relation

patient,

was

other

disease

to

is

the

responsive

minimal-change resistant

and

disease

it is usually

suggesting a possible interdependence athy with the malignant thymoma, spond to steroid therapy.” Remission

the

thymic

malignant

the mutant

the

end

of the

clone course

In conclusion, dence

that an

induced

ated, lying

our this

disease.

An

of lymphocytes of the

disease.

case

provides

minimal-change

an imbalance until

this

disease steroid

and

1966;

64:41-51.

Simon

MN,

erular

diseases.

pre-

Cliii

JD, Forman WH. 1974; 65:114-6.

Massive

Hemoptysis

of this nephropwhich does not reof Hodgkin’s dis-

explanation

further might

immunity. for

might

experimental

model

by

of cell-mediated

be

towards

indirect be

Pulmonary

treatment

Medical

Am

North Response

1978;

of glom-

62:1157-81.

of thymoma

to steroids.

William

Secondary

by a Catheterization S. Hoffman,

Ring,

to

Fistula*

Arteriovenous

Treatment

L. Henry

the

evi-

result

of

Nevertheless,

minimal-change immunity

assumption concerning the mechanism clinical observation remains hypothetical.

disease is cre-

under-

Procedure

Paul M. Weinberg,

M.D.;

M.D.;

and

M.D.;

Edmunds,

Jr.,

M.D.,

F.C.C.P.

Massive pulmonary hemorrhage secondary to an acquired arteriovenous fistula is a rare event associated with high mortality. Cotton wads mounted on steel coils were inserted by percutaneous catheter and successfully occluded a pulmonary arteriovenous fistula in a patient who had massive hemoptysis and contraindications to thoracotomy.

M

assive

pulmonary

and operation.

The

tuberculosis, ectasis, but single,

We wish to thank T. Moalem, M. Becker, Ph.D., and Professor A. Klajman for perdeterminations of levels of circulatory immune com-

with

to 100

in 50

occurs

hemorrhage

associated

high

percent

most

is

life-threatening

mortality.12

Suffocation

of patients

treated

causes

of hemoptysis

common

without are

arpergillosis, bronchial pulmonary arteriovenous well-documented causes

tumors,

nonoperative treatments been attempted. None

of massive hemophas been uniformly

are

and

fistulas, of

bronchi-

multiple

massive

or

hemop-

tysis.1

Various have

ACKNOWLEDGMENT:

successful,

and

Operation

is the

mortality

rates

treatment

85 percent.2

approximate

of

choice,

but

contraindica-

to surgery include inadequate pulmonary function, inability to locate the site of bleeding, bronchial carcinoma with involvement of the mediastinum, multiple bleeding sites, or severe coagulation deficiencies. The following case illustrates a new method of managing massive hemoptysis from a pulmonary arteriolions

REFERENCES 1 Eagen JW, Lewis EJ. Glomerulopathies of neoplasia. Kidney mt 1977; 11:297-306. 2 Becker M, Klajman A, Moalem T, et al. Circulatory immune complexes in sera from patients receiving procainamide. Clin Immunol Immunopathol 1979; 12:220227. 3 Sherman RL, Susin M, Weksler ME, Becker EL. Lipoid nephrosis in Hodgkin’s disease. 1972; Am J Med 52:699706. 4 Shalhoub RJ. Pathogenesis of lipoid nephrosis: a disorder ofT-cell function. Lancet 1974; 2:556-9. 5 Michael AF, McLean RH, Roy LP, et al. Immunologic aspects of the nephrotic syndrome. Kidney mt 1973; 3:105-15. 8 Mota CH. Infantile Hodgkin’s disease: remission after

measles. Br Med J 1973; 2:421. 7 Elisabeth MS, Summer B. In vitro suppression lymphocyte response to tuberculin by live measles

of the virus.

Proc Soc Exp Biol Med 1968; 123:276-a 8 Baker JP, Stashak WP, Amsbaugh FD, et al. Evidence for the existence of two functionally distinct types of cells

CHEST,

JM.

Med

Chest

tysis M.Sc., forming plexes.

Rosenberg

treatment,

originated

disease

in cell-mediated disorders

cancer

Ernest

ease also results in a remission of minimal-change disease in these patients.’ Unfortunately, in our case, this was not achieved, since the thymoma was unresectable. The minimal-change disease developed late in the course of that

polysacchride. JC, Yaunauchi

11 Green

is rare

minimal-change The

case

patients

to malignant

elderly

the antibody response to type III pneuxnoJ Inimunol 1970; 105:1581-3. H, Hopper J Jr. The association of the nephrotic syndrome. Ann Intern Med

regulate

literature.

in

When in the

steroid

sented

was

described

disease

to

between

of our

disease

generally only

which

case, additional circumstantial evidence supfact that minimal-change disease was a paraprocess, rather than a coincidental event.

Minimal-change than

to

in with

Based

an association

and

In our ports the neoplastic

can

T-lymphocytes.8

ical observations however,

response be enhanced

Antibody

minimal-change disease by treatment globulin, suggesting the presence

suppressor

disease

(4)

polysaccharide

77: 5, MAY, 1980

venous

fistula

without

thoracotomy. CASE

A diagnosis great

of

arteries,

and

catheterization been

cyanosis,

first

and

episode

#{176}Fromthe

of the

of

age of

1977,

In

Surgery

and

boy

who

Durhe

Divisions

Children’s

Lankenau

had

his

spontaneously.

Radiology, the

had was

dyspnea,

1977,

and

the

cardiac

polycythemia. progressive

and

Cardiology,

at

anastomosis

terminated

of Surgery

of

made

October

Pennsylvania,

Philadelphia, Philadelphia. Reprint requests: Dr. Hoffman, Philadelphia

and

developed

which

of

transposition

was a Glenn

cyanosis

he

polycythemia.

Departments

L

in a 15-year-old five,

of hemoptysis

University

diothoracic

years

At

palliation

summer

ventricle, stenosis

four

at birth. for

the

REPORT

left

pulmonic

at age

cyanotic

performed ing

single

Medical

Hospital of Car-

of

Hospital

Building,

19151

MASSIVE HEMOPTYSIS AND PULMONARY AV FISTULA

697

time, and platelet count were all within normal limits. Cough produced only minimal amount of blood-stained sputum until 9:30 i,s. At this time, the patient coughed up 400 ml of bright red blood in a ten-minute period, but again bleeding stopped spontaneously. The pulmonary arteriovenous flstula was the most probable source of hemorrhage. Bronchoscopy was not done because of the rapidity and amount of hemorrhage. The two episodes of massive hemoptysis demonstrated an urgent need for definitive therapy. There were several considerations, however, which weighed against resection. First, because of cyanotic heart disease and the previous Glenn operation, many tortuous thin-walled vessels were expected in the adhesions between the right lung and chest wall. Second, the patient needed all of his lung for oxygenation; it was uncertain whether or not he could tolerate lobectomy. Lastly, the open sternal wound increased the possibility of infection. These

considerations

teriovenous

Ficun 1. Pulmonary right lower lobe.

angiogram

of artenovenous

fistula

in

At repeat cardiac catheterization in January 1978, the previous diagnosis was confirmed, and in addition, he had severe aortic regurgitation with marked dilatation of the aortic root, and a patent Glenn anastomosis which drained into an arteriovenous fistula in the right lower lobe (Fig 1). Mean atrial pressure was 12 mm Hg and aortic saturation was 87 percent. On examination, he weighed 64 kg and was moderately cyanotic with clubbed digits. He had murmurs of aortic insuEciency and pulmonic stenosis. There was no evidence of heart failure. Lungs were clear without murmurs or bruits. Hemoglobin value was 19.4. On Feb 23, 1978, the aortic valve was replaced with a No. 31 porcine heterograft. A 6-mm polytetrafluoroethylene graft was inserted between the ascending aorta and the proximal stump of the right pulmonary artery such that flow entered the left lung. The patient was extubated immediately after operation and was started on acetylsalicylic acid and dipyrimadole to prevent thromboemboli. On the sixth postoperative day, superficial tissues of the sternal wound were opened because of persistent fever and serous drainage. The wound was packed. At 4 as of the 15th postoperative day, the patient awoke with a cough that produced approximately 300 ml of bright red blood over a 20-minute period. This terminated spontaneously after the patient expectorated a clotted cast of a lower lobe bronchus. He was sedated and transferred to the intensive care unit. Acetylsalicylic acid and dipyrirnadole were discontinued. The prothrombin time, partial thromboplastin

698

HOFFMAN El AL

favored

malformation

an

attempt

to

occlude

the

ar-

with

Gianturco stainless steel coils. Under fluoroscopic control, a 6.5 French cobra-shaped catheter was introduced percutaneously through the left internal jugular vein into the right pulmonary artery branch supplying the fistula. Seven stainless steel coils with attached wads of cotton-wool were then selectively passed through a nontapered 7F Teflon catheter into the subsegmental arterial branches supplying the fistula until repeated injections of contrast material had confirmed that flow to the fistula had been completely obliterated (Fig 2). The patient coughed up small amounts of blood over the next 48 hours and complained of right pleural pain. He also developed prominent neck veins, pitting edema of both upper extremities, and swelling of the soft tissues of the head. Chest x-ray film showed consolidation of the right lower lobe and a right pleural effusion. These findings were consistent with the superior vena caval syndrome and infarction of the right lower lobe and were direct consequences of acute occlusion of the pulmonary arteriovenous fistula with the Gianturco

coils. Over recovery,

the following days, but occasionally

Ficun coils.

2.

Closure

of

the

patient

produced

arteriovenous

made a progressive blood tinged sputum.

fistula

with

Gianturco

CHEST, 77: 5, MAY, 1980

Ficuuz

3. Gross pathology

His upper body edema the 29th postoperative Seventeen sented

weeks

with

ously

but

had

no

resolved

hospital

discharge

on

right

occlusion venous vena

his

heart

in intractable

recurrent

by

mechanical

day. following

massive died

had

demonstrating

surgery,

failure. failure

hemoptysis.

the

He on

June

Autopsy

patient

pre-

was treated 23, 1978,

vigorhaving

showed

venous fistula was totally obliterated and demonstrated multiple areas of infarction organization (Fig 3).

the

arteno-

the lung distally with beginning

fistula cava

Glenn

of

Hemoptysis

secondary

is uncommon,

to

for

less

arteriovenous than

of cases in most series.1’2 In those patients a fistula, which is a recognized complication superior vena cava-right pulmonary arterial the incidence of hemorrhage complications mon in the second and third decades, and is ly 20

2 percent

who develop of the Glenn anastomosis,3 is most comapproximate-

percent.2

Silastic

spheres,

blood

clot,

autologous

tissue,

tissue

gelfoam have all been used in the past in an attempt to stop localized hemorrhage.4’5 These materials, however, when injected into an arteriovenous fistula, can be life-threatening because of possible passage of the material into the pulmonary veins with resultant systemic embolization. In 1976, Wallace et al reported the use of a stainless steel occluding device in 24 patients, two of whom had renal arteriovenous flstulas. The coil successfully occluded the fistula in each case. The Gianturco coil was adhesives,

subsequently

and

used

in surgically

created

fistulas

between

the carotid artery and jugular vein in dogs where it produced a fixed obstruction at the point of placement and complete occlusion of the fistula within 5 minutes.6 Fibrous encasement eventually occurred and resulted in permanent closure. This same technique has been utilized,

clinically,

arteriovenous

In

our

to correct

hypoxemia

resulting

from

fistulas.8

patient,

occlusion

CHEST, 77: 5, MAY, 1980

of

the

pulmonary

arterio-

lung

the

of vessels

progressively

After

in ventilated more

but

of the

arterial

increased

right-to-left

resistance

shunt

In

of the

ventilated

portions

this

unoxygen-

hypoxemia.

disclosed

a por-

increases.

increased,

resistance

superior

infarction.

blood was shunted through the arteriovenous the pulmonary veins. This prevented systemic

vascular

pulmonary

accounting

complications:

pulmonary

resistance

right

hypertension

DISCUSSION

two and

as vascular

clusion

fistula

the

patient,

fistulas.

produced

syndrome

anastomosis,

tions ated into

of arteriovenous

fistula venous Acute

oc-

the

increased of the right syndrome.

lung and produced the superior vena cava Occlusion of a pulmonary artery does not generally cause hemorrhage consolidation unless the affected lung also becomes atelectatic. If the affected lung remains inflated, infarction does not occur.9 In this patient, hemorrhagic atelectasis of the right lower lobe developed after occlusion of the fistula in those areas of the right lower lobe not supplied by bronchial arteries. Cianturco steel coils may have wider application in patients with multiple pulmonary arteriovenous fistulas or in patients with other causes of hemoptysis in whom thoracotomy patients with

is contraindicated arteriovenous

or unduly fistulas and

secondary

to a Glenn

anastomosis,

the

preferable

to ligation

of the

pulmonary

reanastomosis

of the

right

superior

vena

hazardous.

severe

technique cava

In

hypoxia may artery

to

the

be

and right

atrium.10 REFERENCES 1 Zavarella C, Jaffe A, Tellez G, Ruthilanchas JJ, Agosti 5, Figuera D: Pulmonary artenovenous fistula: a review. Vasc Surg 1975; 9:244-256 2Chocco JA, Rooney JJ, Frankushem DS, DiBenedetto RS, Lyons HA: Massive hemoptysis. Arch Intern Med 1968; 121:495-498 3 Glenn WWL, Liebow AA, Lindskog GE: Thoracic and cardiovascular surgery. 3rd ed. New York: AppletonCentury Crafts, 1975; 911-917 4 Remy J, Arnaud A, Fardou H, Giraud R, Voisin C: Treat-

MASSIVE HEMOPTYSIS AND PULMONARY AV FISTULA 699

5

6

7

8

9

ment of hemoptysis by embolization of bronchial arteries. Radiology 1977; 122:33-37 Wallace S, Gianturco C, Anderson JH, Goldstein HM, Davis U, Bree RL: Transcatheter intravascular steel coil occlusion. Am J Roentgenol 1976; 127: 381-387 Gianturco C, Anderson JH, Wallace S: Mechanical devices for arterial occlusion. Am J Roentgenol 1975; 124: 428-435 Anderson JH, Wallace 5, Gianturco C: Transcatheter intravascular coil occlusion of experimental arteriovenous fistulas. Am J Roentgenol 1977; 129:795-798 Taylor BG, Cockerill EM, Manfredi F, Klatte EC: Therapeutic embolization of the pulmonary artery in pulmonary arteriovenous fistulas. Am J Med 1978; 64:360365 Edmunds LH Jr, Holm JC: Effect of atelectasis on lung changes after pulmonary arterial ligation. J Appl Physiol 1968; 25:115

10 Van Den Bogaert-Van Hesvelde AM, Derom Y, Kunnen M, Van Egmond H, Devloo-Blancquaert A: Surgery for arteriovenous fistulas and dilated vessels in the right lung after the Glenn procedure. Thorac Cardiovasc Surg 1978;

76:195

Echocardiographic Unruptured Sinus

Features

Aneurysm

Shulman,

Barbara

M.D.;

Ray,

I.

of the

Shukri

Khuri,

and Alfred

B.A.;

Right

*

of Valsalva

Robert

of an

M.D.;

F. Parisi,

M.D.

A 2-cm aneurysm of the right sinus of Valsalva was documented in a patient with a prosthetic aortic valve. The M-mode findings differed from prior reports and mimicked those of aortic root dissection or a catheter placed in the right ventricular outflow tract. Two-dimensional echocardiograms readily distinguished the aneurysm of the right sinus of Valsalva from the alternative

A

possibilities.

neurysms

of the

tomatic

until

sinus

of Valsalva

rupture.1

They

are result

usually from

asympa defect

in

attachment of the aortic root to the fibrous ring connecting it to the aortic valve and left ventricle. In the case of an aneurysm of the right sinus, growth of the sac and retraction from its initial position lead to its pointing toward the right ventricular outflow tract, anterior to the the

ascending cle just

aorta. below

Rupture the

is usually

pulmonic

into

the

right

ventri-

valve.

We describe a case of a presumably acquired aneurysm of the right sinus of Valsalva secondary to bacterial endocarditis, a known cause of such a condition.2 Special attention to heretofore unreported echocardiographic features indicates that findings on M-mode echocardiograms with

an

can

ascending

#{176}Fromthe

Administration Peter Bent Boston. Supported

closely

aortic

Medical by the

Center,

Hospital Medical

Administration.

700

dissecting

those aneurysm.

consistent Real-time

of Medicine

Departments Brigham

simulate

SHULMAN ET AL

and Surgery, Veterans West Roxbury Mass, and and Harvard Medical School,

Research

Service

of the

Veterans

two-dimensional curate

echocardiograms

noninvasive

help make the two

can

between

distinction

an accondi-

tions.

m

MATERIALS

Both

M-mode

and

METEODS echocardiograms

two-dimensional

were

the patient in a semirecumbent 30#{176} left lateral decubitus position. M-mode recordings were made with a multichannel recorder (Irex Continutrace 101) interfaced with a cardiac ultrasonic module (Irex 150-149) using a 2.25MHz transducer (Aerotech) at a paper speed of 25 mm/sec. Two-dimensional study was performed with a phased-array obtained

sector

with

scanner

(Varian

V3000). CASE

In white

1969,

after

man

without

intravenous

oped

bacterial

known

endocarditis.

REPORT

abuse

of drugs,

antecedent Medical

heart therapy

a 23-year-old disease was

develsuccessful

in controlling

the infection, but the patient was left with residual aortic regurgitation. He suffered repeated bouts of endocarditis in 1970 and 1972. Cardiac catheterization in June 1973 showed severe aortic regurgitation and an aneurysm of the right sinus of Valsalva. In September 1973, at another hospital, the patient underwent aortic valvular replacement with a No. 2 Kaye-Suzuki prosthesis. At surgery, a nonperforated aneurysm of the right sinus of Valsalva was described. In March 1976, the patient again developed endocarditis; and four months later, after successful medical treatment, a soft murmur of aortic regurgitation was noted. Catheterization in July 1976 revealed 1 + aortic insufficiency with a paravalvular leak in the area of the aneurysm of the right sinus of Valsalva, without evidence for a left-to-right shunt The right sinus of Valsalva was aneurysmal and calcified. Surgery was subsequently planned, due to fear of rupture of the aneurysm. Furthermore, the patient had suffered several episodes of cerebral embolization. The patient refused surgery but underwent repeat catheterization in 1978 when, after suffering repeated embolic episodes, he agreed to undergo surgery. Preoperative M-mode echocardiograms demonstrated an abnormal thick echocardiographic density 21 mm anterior to the anterior aortic wall. Its motion was identical to that of the aorta (Fig 1, left). Scanning showed this structure to converge inferiorly with the upper interventricular septum. On grams

the longitudinal view, two-dimensional demonstrated an echo-dense saccular

structure

echocardio2 cm in

diameter at the base of the anterior aortic root (Fig 1, top right). When the two-dimensional cursor was used to derive an M-mode record, a pattern identical to that described previously herein was obtained. A representative frame from the patient’s aortogram on his most recent catheterization is shown to compare with the two-dimensional image (Fig 1,

bottom

right).

1979, the patient successfully underwent repeat aortic valvular replacement with a prosthesis (No. 21 Bj#{246}rkShiley), as well as aneurysmal repair by exclusion with a In January

Dacron patch. Postoperative echocardiograms were essentially without change from the preoperative studies, with the exception of better definition of the (new) aortic valvular poppet. DISCUSSION

The sinus

few echocardiographic of Valsalva have indicated

reports variable

of aneurysm findings.

of a Roth-

CHEST, 77: 5, MAY, 1980