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Pulmonary diseases secondary to heart diseases
Pulmonary
Diseases Secondary Diseases
to Heart
DIAGNOSIS AND TREATMENT NATHANIEL
E. REICH, M.D.,Brooklyn,
ULMONARYcompIications are a frequent ocduring the course of manv heart diseases and are- of vita1 concern “to the thoracic surgeon as we11 as to the internist and chest specialist. Our rapidIy expanding knowIedge in both fields makes it imperative to estabIish the correct diagnosis if treatment is to be effective. SeveraI congenita1 cardiac defects, acute and chronic inflammations of the heart and aorta, degenerative Iesions of the coronary arteries and aorta and primary neoplasms of the heart are capabIe of producing a variety of puImonary compIications.
P currence
CONGENITAL CARDIAC LESIONS Interest in this field has been reawakened since it is now possible to correct a number of congenita1 cardiovascular Iesions by surgical means. These incIude patent ductus arteriosus, coarctation, tetraIogy of Fallot, “vascuIar ring” anomaIies, aberrant subcIavian artery, septa1 defects, puImonary stenosis and various transpositions. The onIy congenita1 vascuIar Iesions which directly affect the trachea or bronchi by compression are the vascular ring anoma1ies.l Other defects, such as tetraIogy of FaIIot, produce dyspnea, cyanosis, cIubbing, etc., but these are due to Iack of proper oxygenation of bIood because of the existence of abnorma1 shunts. However, a11 infants with cyanosis or dyspnea do not have congenita1 heart disease. A large diaphragmatic hernia, congenita1 air cyst, Iung anomaIy or puImonary hemorrhage may simuIate the signs and symptoms of congenita1 heart disease. In addition to insuffIcient puImonary bIood flow and congestive failure induced by some congenita1 Iesions, subacute bacteria1 infections may resuIt in embolic infarctions, multipIe abscesses and bronchopneumonia. Right Aoytic Arch. This congenita1 anomaIy may sometimes form a vascuIar ring by the constricting effect of a patent ductus arteriosus or American
Journal OJ Surgery,
Volume 89, January,
,995
252
New York
Iigamentum arteriosus. (Fig. I.) It produces symptoms in the newborn by pressure on the right upper Iobe bronchus thus producing ateIectasis, obstructive emphysema or recurrent infections (trabeobronchitis or pneumonitis). It may also cause the pulmonary artery to compress the anterior surface of the trachea. Symptoms may be initiated Iater in life owing to traction caused by eIongation and posterior displacement of the arteriosclerotic aorta. Pulmonary symptoms incIude dyspnea, cyanosis, cough and chest pain. Head retraction and stridor may occur in infants. X-ray studies of the barium-fiIIed esophagus reveal an indentation at the IeveI of the third intervertebra space. 2 Lipiodol studies in the IateraI position deIineate anterior trachea1 compression just above the carina. Angiocardiography (7 second fiIm) usuaIIy reveals the exact Iocation of the vascuIar deformity. When the obstruction produces symptoms, the Iigamentum arteriosus is divided, aIIowing the pulmonary artery to faI1 forward.3 Double Aortic Arch. This anomaIy occurs when both fourth branchia1 arteries persist. The Ieft (anterior) arch is usuaIIy much smaIIer than the right. The vascuIar ring produces encroachment upon the trachea and esophagus, resuIting in symptoms and diagnostic studies similar to those Iisted under the right aortic arch. Complete reIief is afforded by the Iigation and extirpation of the Ieft (anterior) portion of the doubIe arch. (Fig. I.) Pulmonary Stenosis. PuImonary tubercutosis has been found to occur with greater frequency in the presence of severa congenita1 heart conditions. This is especiaIIy true of puImonary stenosis and tetraIogy of FaIIot. Abbott4 found an incidence of 36 per cent and attributed this to the insuffIcient puImonary bIood flow. This expIanation appears IikeIy in view of the fact that tubercuIosis seIdom occurs in the chronicaIIy engorged Iungs of
Pulmonary
Diseases
Secondary
to Heart
Diseases
Double Aortic Arch Right Aortic Arch FIG;. I. Vascular ring anomalies.
with mitral stenosis. Superimposed patients subacute bacterial infections with pulmonary embolization may also occur. This results in infarction, pneumonitis or lung abscess. The BlaIock shunt operation has been employed with success. A direct attack on the stenosing tissue by incision or punch operation produces equally satisfactory results in other cases, especiaiIy in those with an infundibulum. Patent Ductus Arteriosus. Ordinarily the higher pressure in the aorta allows a return ilow to the pulmonary artery through this abnormal communication. Pulmonary congestion may occur eventually if the shunt is of considerable size. The most frequent complications are congestive heart failure and subacute bacteria1 endocarditis. The Iatter results in embolic infarctions of the lung. Multiple smaIl abscesses or patchy pneumonitis may ensue. Ligation and section of the ductus and the use of antibiotics are producing remarkabIe cures. The operation is contraindicated if evidences of puImonary hypertension appear during the course of this disease. ACUTE
Rheumatic
monia
RHEUMATIC
Pneumonia.
is a rare
but
FEVER
Rheumatic pneuintegra1 phase of acute
rheumatic fever since the collagen tissue and its ground substance may be affected in any part of the body. An incidence of pulmonary involvement has been reported by numerous observers as varying from z to 13 per cent of all cases. The occurrence would be noted in a much larger percentage if aTI patients with acute rheumatic fever had chest x-rays at frequent intervals but especialIy at the height of an attack of acute rheumatic fever. The pathologic changes consist essentially of irregularly scattered areas of hemorrhagic consolidation. Cough and sputum are not prominent features. The diagnosis rests on finding5 signs of transitory or migratory pulmonary exudates. This is demonstrable on serial x-ray studies. (Fig. 2.) Part of one lobe may be involved or the inAammatory process may extend to ail the lobes. The lesions cIear rapidly FolIowing the use of ACTH or cortisone, but this is di&ult to evaluate because of the evanescent character of the lesions. Pleu7i.g~. Pleurisy is probably another manifestation of inflammation of the subendothelia1 connective tissue. Although acute fibrinous or serofihrinous pleuritis is recognized clinically in only 5 to 15 per cent of a11 cases of acute rheumatic fever, it is found in half of the cases that
Pulmonary
Diseases
Secondary
to Heart
Diseases
FIG. 2. Rheumatic pneumonia. A, irreguIarIy scattered areas of soft infiItrations throughout both lower lung fields, more marked on the left side. Onset of pneumonic involvement occurred the previous day. B, marked diminution in extent and intensity of the infiltrations the foIlowing day iIIustrating the transient nature of the exudates.
come to postmortem examination. It may occur on either or both sides and usuaIIy foIlows puImonary or pericardial invoIvement.5 CharacteristicaIIy, the initial severe inspiratory pain and friction rub disappear within several days as effusion accumulates. Routine roentgenograms demonstrate tIuid Ievels in a high percentage of cases. Aspiration is seIdom required but shows a clear or cloudy serofibrinous exudate. On rarer occasions it may appear hemorrhagic, possibly due to the presence of pulmonary infarction. Although the fluid is rapidly absorbed, adhesions deveIop occasionaIIy. These may be pIeuropericardia1 adhesions which give the cardiac silhouette a scaIIoped appearance or partiaIIy obliterate the pleura1 space. MITRAL
VALVULAR
thickening of the capillary basement membrane. These changes thicken the aIveoIar wall and interfere with gaseous exchange. They eventuaIIy produce an ineIasticity of the Iung tissue which seriously interferes with ventilation. Dyspnea, orthopnea and cyanosis are major symptoms. Hemoptysis occurs in IO per cent of cases of mitra1 stenosis due to vascuIar hypertrophy. AIthough it may appear at any time during the course of the disease, it usuaIIy denotes an advanced degree of stenosis with increasing pulmonary congestion. The blood loss is seIdom massive but augurs a dire prognosis. Cough is commonIy present, and when productive the sputum may revea1 numerous “heart faiIure” ceIIs. The sputum is mucoid but may be bIood-streaked or frankIy purulent due to secondary bronchopuImonary infection. Acute Pulmonary Edema. This may occur in mitraI stenosis and other vaIvuIar lesions even when reguIar sinus rhythm exists. It usuaIIy foIIows severe exertion, operation, rapid digitalization or paroxyspregnancy, ma1 tachycardia. Attacks are precipitated by the added strain on the right heart in the presence of puImonary congestion. Treatment incIudes digitaIization, morphine and atropine, and antifoaming agents.6 oxygen, mercurials
DISEASE
Several pulmonary complications commonIy occur folIowing distortion of the mitra1 vaIve. On rare instances mitral stenosis may be associated with an interauricuIar septa1 defect (Lutembacher’s syndrome). Chronic Congestive Failure. MitraI vaIvuIar lesions, especiaIIy stenosis, may produce earIy puImonary congestion. As a resuIt of chronic congestive heart faiIure the Iungs become firm, dense, dry and red-brown in appearance. There is interstitia1 edema, increased coIIagen and 254
PuImonary
Diseases
Secondary
Subsequently, surgery may be indicated. Remarkable results are constantly being reported by numerous surgeons. Commissurotomy or finger fracture of the stenosed valve, or anastomosis bet\yeen the aorta and pulmonary arter! or between the right inferior pulmonary vein to the azygos veins will relieve puImonar> hypertension. Rupture. Rupture of a papillary muscle of chordae tendineae results in an unusually rapid form of pulmonary congestion. This may be caused by trauma, superimposed subacute bacterial endocarditis or coronary thrombosis. It produces an extremely loud murmur which is frequently audible at a distance from the chest. Pulmonan, Infarction. The effects of pulinfarction are discussed elsewhere. monary Suffice it to sa\- that this serious compIication may result from the discharge of clots from the right auricle during auricular fibrillation. It is important not to overlook other major causes such as peripheral or pelvic \.enous thrombosis. Early treattnent of the primar? heart condition and the use of anticoagulants have greatI\- reduced morbidity and mortality. Amputation or ligation of the auricular appcndages is presently employed with success in cases with recurrent embolization thladden). HtCTERl.iL
ENDOChRDITIS
The acute and subacute forms of bacterial infections ot‘ the valvular and mural endocardium may be caused by manv microorganisms. Howver, the non-hemoly& streptococcus accounts for 95 per cent of all cases. In order for emboli to reach the lungs, the right heart must obviously he affected. Septal defects, pulmonary or tricuspid stenosis, patent ductus arteriosus, tetralogy of Fallot or other combined congenital lesions may also provide a nidus. Pulmonary emboli and infarctions, congestion, bronchopneumonia or lung abscesses ma! fx encountered. The infarctions usually are too small to be visuahzed on the x-ray. PIeural effusions or hetnorrhages are not uncommonly found. Emboli in pulmonary arteria1 branches may also result in arteritis or mycotic aneurysm. Symptoms include cough, chest pain, dypsnea or bloody expectoration. Antibiotics are effective in most cases when properly selected and administered in adequate dosage.
to Heart -\CCTE
Diseases
X0&-SPECIFIC
PERICARDITIS
Acute pericarditis is usually regarded as evidence of serious underIying disease such as rheumatic fever, mvocardia1 infarction, uremia, tubercuIosia and pneumonia. There is a rarer group of disorders which may also produce acute pericarditis. However, \ve are primarily concerned with acute non-specific pericarditis. This type occurs in j-oung males as a rule and has an increasing incidence. Frequency ot recurrences has been emphasized lately. In\-ariably there is involvement of the pleura in \-iral inflammation of the pericardium. A pleural lesion must be suspected n-hen the clinical ichest pain, dyspnea, fever, rub) and laboratory findings ieIectrocardiogram and y-ray) of acute non-specific pericarditis are accompanied or followed shortly by the onset of a pleural friction rub which may last for da?-s or weeks. This ma>- progress to pleural effusion of \.arying degree (usualI\- small in amount) and, finally, progressive pleurai thickening. The aspirated fIuid is esudativc ancl non-blood\and ma>- be bilateral.; Pulmon:ir>- involvement is uncommon. Pleural fluid is demonstrable on the roentgenogram. Persistence of lever, following improvement of the pericarditis and invariable recovery further suggest this possibility. Result; with aureom!-tin, chloromvcetin’~ and terramycin@’ have been equivocal thus far. The disease is self-limited but aspiration of fluid is indicated \vhen its presence embarrasses cardiac or pulmonar>function. t Fig. 3.) CHRONIC
CONSTRICTIVE
PERICr\RDITIS
Chronic constrictive pericarditis is a dense fibrous thickening of the pericardium (Fig. 4) \vhich ma)- cause compression of the heart ancl interfere \vith normal filling. It may completeI! envelop the heart or produce a band-like or other localized strictures. It is characterized clinically by the triad of a small quiet heart, elevated venous pressure and ascites. The cause is often obscure. AIthough tuberculosis is the most common cause in cases \yith proven etiology, this disease accounts for onI\ 13 per cent of al1 cases. Rheumatic fever, pleuropulmonary disease, myocardial infarction and neoplastic involvement make up the buIk of all cases. (Fig. 4.) The Iungs usuaIIv do not revea1 significant congestion or fihros’is unIess tuberculosis is the 255
PuImonary
Diseases
Secondary
to Heart
Diseases
FIG. 3. Marked pericardia1 effusion. Reduction of pulmonary function by very marked effusion. A, note waterbottle configuration of pericardial effusion. B, in Ieft anterior obIique view of angiocardiogram the pericardia1 effusion is more sharpIy defined. Note posterior compression of Iung space.
FIG. 4. Types of chronic intrapericardial pressure. A, compIete enveloping scar. B, ventricuIar scar; note compression with bulge above. C, band over ventricIes. D, band around superior vena cava, resuIting in superior vena cava1 syndrome. E, band over auricIes. F, tamponade (bIood or effusion); note compression of auricular appendages and intrapericardia1 portions of venae cavae and puImonary veins. G, adhesive pericarditis. H, band around inferior vena cava, resuIting in inferior vena cava1 syndrome. (From REICH, N. E. The Uncommon Heart Diseases. SpringfieId, III., 1954. Charles C Thomas.)
256
PuImonary
Diseases
Secondary
to Heart
Diseases
5A
FIG. 5. Chronic constrictive pericarditis. A, eIectrocardiogram reveals negative T waves and decreased voltacre comnlrses. B. x-ravs reveal chronic tuberculous pericnrditis with biIatcra1 apical lung involve__ of ORS ment. Arrows point to calcification. 9
cause.
(Fig. 5B.)
Yet pIeura1 adhesions
sions are fairly common. pericardial auricle,
may be seriousIy around
interfered
pulmonary
congestion
owing to marked by ascites.
over the left
of this chamber
with.
Constriction
vein orifices engorgement.’
may be due to extensive monary
mainly
and filling
the pulmonary
in marked
When the constricting
scar is Iocalized
reIaxation
or effu-
pIeural
or decreased eIevation
The vita1 capacity
to 68 per cent of normal
may result Dyspnea
effusions,
signs of cardiac appear.
or puImonary
Dramatic
ing decortication practiced
by
Streptomycin, atives
of the Cutler
are of value
tuberculous
as
heart. far
embarrassment occurs This back
PAS and isonicotinic in the treatment
follow-
was first as
1924.
acid derivof active
lesions.
puISYPHILIS
vital capacity
of the diaphragm was reduced
improvement
up
in the cases reviewed
b?- Stewart and Heuer.8 Signs of cardiac fixation and evidences of the right heart faiIure sh-ndrome comprise the cardiac findings. The electrocardiogram is characteristic. (Fig. 5A.) ;IledicaI treatment is of little avai1 when
It
has been
estimated
miliion Americans yet only
2
that
a quarter
have cardiovascular
of a
syphilis,
per cent of these develop aneurysm.g
The unfavorable prognosis of cardiovascular syphiIis is we11 known. Aortic vaIvuIar lesions are generally considered to be more serious than mitra1 Iesions because they are often caused by negIected syphilis and because of
PuImonary
Diseases
Secondary
to Heart
Diseases
FIG. 6. SyphiIis.
the poor prognosis. When Ieft ventricuIar faiIure ensues, puImonary edema is rapid and reIativeIy unresponsive to therapy. SyphiIitic myocarditis is equaIIy diffIcuIt to treat in the stage of congestive faiIure. Saccular aneurysms may reach aIarming proportions and may produce symptoms and signs of compression of the Iungs, bronchi and other structures depending on the direction and extent of their diIation. (Fig. 6.) Aneurysms of the arch are most important because of their greater proximity to respiratory structures. PuImonary signs of ateIectasis, bronchiectasis and secondary infection may resuIt. Trachea1 tug may be elicited and Ieft recurrent IaryngeaI nerve compression may resuIt in hoarseness or aphonia. Phrenic nerve invoIvement may produce paraIysis of the diaphragm, thereby interfering with oxygenation. The most important of these compIications is atelectasis. UnIess the pathoIogic condition is su&cientIy extensive, tactiIe fremitus may be non-reveaIing.10 Diminished breath sounds and aItered resonance on percussion may be noted. Bronchiectasis may ensue from Iack of drainage and infection induced by bronchia compression. EndobronchiaI tumors and other Iesions must be differentiated occasionaIIy. Bronchography may revea1 the true cause of IocaIized obstructions or distortions of the bronchia tree. Tomography may excIude endobronchia1 tumor
by delineating cavitation within the aneurysmal mass. This is unreIiabIe when the aneurysm is cIotted. Kymography may show typica expansiIe puIsations when the aneurysm is uncIotted. Further differentiation of adenoma, carcinoma and inspissated mucous or granuIomatous tissue rareIy requires cautious bronchoscopy, biopsy or PapanicoIaou staining of sputum for tumor cells. SeroIogy is positive in approximateIy 85 per cent of cases. Calcification in the waI1 of the ascending aorta (Ieft anterior obIique view) is characteristically found in approximately haIf of a11 cases of Iuetic aortitis. Antibiotics now have a definiteIy estabIished position in the treatment of cardiovascular syphiIis. Aneurysms have been wired and eIectrocoaguIated, or surrounded by various ceIIophanes in order to produce a fibrotic reaction which wiI1 shrink or prevent further expansion with rupture. MYOCARDIAL
INFARCTION
This condition has aIready received the direct attention of surgeons. Not onIy are a number of surgica1 procedures avaiIabIe pertaining to revascuIarization of the heart, but aIso excision of Iarge myocardial scars and aneurysms is being practiced presentIy. A number of puImonary compIications is possibIe in patients suffering from myocardial
258
PuImonary
Diseases
Secondary
to Heart
Diseases
tion is commonly confused with pneumonia or In addition to duIIness puImonary infarction. or ffatness and bronchia breathing, a shower of fine inspiratory rales is frequentIy heard. The clinical course is usuahy severe with frank hemoptysis, marked pyrexia, and Ieukocytosis. Signs of congestive failure may ensue in some patients. The x-ray picture is diagnostic.” Pulmonary Infarction. Emboli to the Iung may be due to auricular fibrillation, peripheral and peIvic venous thrombosis, bacteria1 endocarditis and right-sided mural thrombosis owing to myocardial infarction. Emboli may enter any part of the pulmonary arteria1 system although the right base is commonIy affected. The sudden onset of chest pain, cough and bloody expectoration usually heralds this complication. Signs incIude a patch of crepitant rales, dullness and bronchia breathing. Pleural pain is present in half of the cases but a rub occurs in only IO per cent of the cases.‘l Effusions are usuahy minimal and may be clear or sanguineous. On rare occasions large recurrent effusions may dominate the cIinica1 picture. These effusions seldom require aspiration. The icterus index may be slightly eIevated on the second or third day due to absorption. The electrocardiogram is diagnostic in a large percentage of the cases, but x-ray investigation is usually disappointing. Immediate anticoagulant therapy is indicated. High venous ligation must be considered when embolization from the peripheral or pelvic veins is suspected. Inferior \‘ena caval ligation produces peripheral edema which may be quite disabhng for long periods,
infarction. They are mistakenly referred to as in most instances. Five condi“pneumonia” tions must be considered when lung findings appear following this condition. This dire pulmonary Pulmonary Edema. complication is due to acute left ventricmar faiIure, since the left ventricuIar musculature is most commonly affected by infarction. It may be the dominant manifestation of an acute myocardial infarct appearing suddenIy within hours to days folIowing an occlusion. Symptoms include thoracic oppression, dyspnea, orthopnea and cough. The attack may be characterized by episodes of paroxysmal nocturna dyspnea with wheezing respirations and rhonchi. MiId puImonary congestion is common when the dominant manifestation is pain, shock or congestive heart faiIure. BasaI raIes are heard in such cases, especiaIIy on the right side. It may become more severe with noisy respirations when copious white or pink foam appears. In advanced cases it may pour out of the oronasa1 passages and bubbling raIes may become audibIe up to the apices. DigitaIis, oxygen, morphine and atrophine, diuretics and antifoaming agents are empIoyed.6 Anticoagulants are aIso employed since they affect the ultimate prognosis. Atelectasis. It is very common to find a Iocalized patch or patches of crepitant rales at the Ieft Iung base, coinciding with x-ray, demonstration of plate-like areas of ateIectasis. However, this is found so frequently early in the attack that it is usuaIIg considered norma by WoIff and not necessaray indicative of congestive failure, pulmonary infarction or infection.” An area of dullness and bronchia breathing without temperature elevation usually indicates the presence of atelectasis. Oxy-gen and expectorants may be of vaIue in such cases. Rebreathing is obviousIy contraindicated following myocardial infarction. Pneumonia. Pneumonia is rarely associated with acute myocardia1 infarction and yet this diagnosis is made with aIarming frequency and treated as such. The possibility of other Iesions such as localized pulmonary edema, puImonary infarction, ateIectasis or chronic bronchiectasis must be investigated. When there is leukocgtosis, hyperpyrexia and mucopuruIent sputum, a therapeutic trial with an antibiotic is indicated. Localized Pu1monar.y Edema. This compIication is not uncommon in acute myocardial infarction and is usually biIatera1. The condi-
DISSECTING
ANEURYSM
There has been a marked improvement in the recognition of dissecting aneurysms during the last decade. (Fig. 7.) This has been occasioned by an awareness of the disease on the part of most clinicians. Efforts at Iimiting the pathoIogic condition to a single factor have been generahy unsuccessful although medial necrosis is recognized as the most important pathoIogic factor. 1 Pulmonary symptoms are rare in the absence of rupture ahhough cough and hemoptysis may occur. When not instantly fatal, various cIinica1 syndromes may appear depending on the site and extent of dissection or rupture. Twenty per cent of al1 dissections rupture into the left pleural cavity and present the 259
PuImonary
Diseases
FIG. 7. Dissecting aneurysm. EventuaI rupture gram prior to rupture. 6, injected specimen.
Secondary
into the left pIeuraI
characteristic signs of chest fIuid.13 However, fluid in the left pleura1 cavity must be differentiated from tubercuIosis circuIatory faiIure (which seIdom affects the left side aIone), maIignancy of the pIeura and Meig’s syndrome. The aspiration of pure bloody fluid is highIy significant for dissection. Maiignancy may revea1 characteristic ceIIs in addition, and tubercuIosis may show organisms on smear or cuIture. On rare occasions ruptures into the right pIeura1 and retroperitonea1 space, mediastinum and other structures have been described. Massive hemoptysis may indicate rupture directIy into the trachea, bronchi or Iung. Blood repIaCement and pleura1 aspiration are indicated. Enzymatic agents (tryptar,@ or streptokinase and streptodornase) are capabIe of Iiquefying bIood cIots prior to aspiration. SurgicaI repair of the site of rupture may be attempted by empIoying gelatin sponge cuffs or by applying poIythene ceIIophane (PT 300). Occasiona cases are known to hea spontaneousIy.
to Heart
Diseases
space with massive
hemothorax.
A, roentgeno-
any tissue in the body, the most common sources being the trachea, breast, Iung and stomach. On rare occasions they may produce the syndrome of chronic constrictive pericarditis. Primary neopIasms occur approximately once in every 2,000 cases that come to autopsy.14 The frequency of types has been reported in the foIlowing order: myxoma, sarcoma, rhabdomyoma, fibroma, Iipoma, angioma, cystoma, papilloma, teratoma and epicardia1 epitheIioma. Any heart chamber may be the site of neopIasm, but the right side is more commonIy invoIved. Therefore, pulmonary metastases from primary cardiac malignancies are frequently found. Diagnosis of a primary Iesion shouId be suspected in cases of heart disease of undetermined etioIogy when: (I) roentgenogram shows an unexpected or bizarre heart size or shape, (2) there are progressive eIectrocardiographic (3) pericardia1 changes otherwise unexpIained, aspiration reveaIs red ceIIs and tumor ceIIs, changing arrhythmias and and (4) frequentIy murmurs appear. PeduncuIated intracardiac tumors may produce intermittent obstruction of valve orifices and simuIate vaIvuIar lesions. The subsequent appearance of metastatic puImonary Iesions with or without pIeura1 invoIvement shouId increase the index of suspicion.
NEOPLASMS
AIthough primary tumors of the heart are reIativeIy rare, metastatic Iesions are not uncommon. Metastatic lesions may arise from 260
Pulmonary
Diseases
Secondary
to Heart
Diseases
This is especialIy true when pIeura1 transudates aIso contain tumor ceIIs or when stained bronchial secretions reveaI tumor celIs of noncarcinomatous origin. (Fig. 8.) Treatment mav be effective when the tumor is benign and pedunculated. Such tumors have Y
TABLE 1 DlSEASES OF TllE LUNG PAKEYCHYMA"
1. ln,fections Acute pneumonitis, chronic bronchitis, bronchiectasis, chronic bronchopulmonary suppuration; various virus and rickettsial diseases 2. Infectious Granulomus Tuberculosis, sarcoidosis, fungus infections 3. Parasitic Diseases are excellent Isease and schistosomiasis :~y$,“’ 4. Allergies Bronchial asthma and aIlergic bronchitis (with secondary emphysema) 5. Chemical and Physical Irritants Dusts, fumes, gases, x-ray and radium fibrosis. (!ncluded are the various pneumoconioses of which silicosis, anthracosis and berylIosis are the most important.) 6. Malignancies malignancies. Extensive primary or metastatic (Metastatic endoIymphatic carcinoma is the most common cause.) 7. ,l/liscellaneous Diseases amyIoidosis (primary systemic and Pulmonary secondary forms), pulmonary fibrosis and emphysema due to various causes, congenital cystic Iung disease, massive puImonary coIIapse, extensive pleural adhesions, chronic passive congestion (secondary sclerosis) *Tables I to IV from REICH, N. E. The Uncommon Heart Diseases, SpringfieId, III., 1954. CharIes C Thomas.
FIG. 8. Syphilis with malignancy in a sixty-one year 01~1man. Note aneurysma diIation of thoracic aorta and large bronchogenic carcinoma invading mediastinum with metastases to right upper Iung field. Autopsy corroboration.
Since we are considering the l>asic mechanism.’ pulmonary compIications of primary heart disease, the other factors wilI not be considered in any detail. However, it is significant that TABLE II I'HI\IAHY HEAHT DISE4SES I. JIitraI
2.
been removed from within the heart (Beck, Bailey). The deveIopment of a good mechanical heart will permit more extensive surgery. Radiotherapy and chemotherapy have produced no known cures thus far, aIthough life may be prolonged in some cases of malignant Iymphoma. Urethane, nitrogen mustard and certain radioactive isotopes have proven of some benefit in appropriate cases.
3. 4.
5. 6.
CHKONIC
COR
PCLMOKALE
Chronic car puImonale is a term applied to hypertrophy of the right ventricle, with or without congestive heart faiIure. Despite a varied etiology which includes a number of puImonary (TabIe I), cardiac (TabIe II) and vascular disturbances (TabIe III), and bony cage deformities (TabIe IV), increased resistance and pressure in the pulmonary circuit is the
valve involvement a. .\litral stenosis b. Lutembacher’s syndromr (mitral stenosis \\ith intcrauricular septal defect) c. Subacute bacterial endocarditis Left ventricular failure, due to myocardial infarction, amyloid disease, beriberi, etc., but most frequent1.y due to aortic disease or hypertension. (None of these wiII show right axis deviation but may have pulmanic hypertension, right ventricular hypertrophy and failure.) Auricular fibrillation (with right auricular thrombus producing pulmonary emboli) Chronic constrictive pericarditis affecting the Icft auricle Tumor of heart causing mural thrombi and pulmonarv embolism Congenital defects (patent ductus, srptal defects, aberrant pulmonary veins)
when congcXive failure supervenes, other puImonary findings become superimposed on the underlying lung disease process (e.g., pulmonarq’ emph~-scma and other bronchopulmonar) diseases). Kyphoscoliosis, funnel chest and other chest deformities are interesting in that they mav also cause hvpertrophp and dilation of the
261
PuImonary
Diseases
Secondary
to Heart
Diseases
tasis or secondary emphysema in addition to bronchopuImonary infections. The diagnosis of chronic car puImonaIe is based upon the recognition of the underIying causative factor (TabIes I to IV) and the discovery of right ventricuIar enlargement with
I.
2.
TABLE IV THORACIC BONY CAGE DEFORMITIES CongenitaI deformities of the chest (funne1 or pigeon breast) KyphoscoIiosis due to neuromuscular diseases (e.g., poIiomyeIitis) and bone diseases (e.g., rickets) 3. ThoracopIasty 4. Ankylosing SpondyIarthritis
or without evidences of faiIure. Right-sided faiIure is recognizabIe by the presence of hepatomegaly, dependent edema and ascites, eIevated venous pressure and proIonged armto-Iung circuIation time. It is important to remember that dyspnea and cyanosis are usuaIIy due to the underIying Iung or heart disease, but may be aggravated in the presence of congestive faiIure. RoentgenoIogic studies show: (I) an exaggeration of the hiIar markings due to enlargement of the puImonary artery and its major branches; (2) enIargement of the right ventricuIar outflow tract in the Ieft anterior obIique view as indicated by the posterior dispIacement ofthe interventricular groove, and in the right IateraI position by the encroachment of the right ventricIe on the retrosterna1 space; and (3) prominence of the pulmonary segment in the postero-anterior view. (Fig. IoA.) When decompensation intervenes, (I) the right auricIe may (2) there is enIargement of become enlarged, the right ventricuIar inflow tract as weI1, (3) the transverse diameter of the heart is further increased in size, (4) the Iower Iung fieIds revea1 congestive changes or fIuid. EIectrocardiograms show a progressive right ventricuIar strain pattern. (Fig. IoB.) PuImonary function tests are empIoyed in the evaIuation of the Iung status. The vita1 capacity is markedIy diminished. Dyspnea is out of a11 proportion to the cardiac state and is mainIy due to the associated pulmonary disease. Treatment must be directed at the causative lesion as we11 as myocardia1 insuffrciency. In many disorders, such as kyphoscoIiosis, it is unsatisfactory. fn others, such as patent ductus arteriosus, earIy surgica1 intervention resuIts in compIete reIief. When dyspnea and cyanosis occur due to either cardiac or puImonary fac-
FIG. g. Kyphoscohotic heart disease. Marked rachitic spina deformity in fifty-eight year old woman. Severe exertiona dyspnea. Note spinat curvature toward right eventually producing chronic car pulmonaIe. Note prominent puImonary vesseIs, atelectasis at bases with compensatory emphysema.
right vent&e. It is the consensus that the syndrome occurs in about 75 per cent of cases of severe kyphoscoIiosis. (Fig. 9.) These deformities may aIso produce compression ateIecTABLE III DISEASES OF THE PULMONARY VASCULAR SYSTE’M Arteries I. Pulmonary a. EmboIism (I) PhIebothrombosis and thrombophIebitis of the peripherat, abdominal and peIvic veins and the venae cavae. (Thromboembolic complications due to myocardial infarction are considered under primary heart disease.) (2) EmboIism due to air, fat and foreign bodies (bullets, etc.) b. Endarteritis obIiterans (Ayerza’s disease) c. Aneurysm (syphilis, congenita1, post-stenotic) d. Thrombosis due to blood dyscrasias e. Persistent truncus arteriosus f. Thromboangiitis obliterans, Iupus erythematosus disseminatus, poIyarteritis nodosa g. Congenital and acquired intravascuIar bands h. Primary neoplasms of the puImonary vascuIar system i. Neoplastic invasion or compression of Iarger branches 2. Pulmonary Veins a. Massive infectious endophIebitis b. ThrombophIebitis migrans c. NeopIastic invasion or compression of large branches d. Thrombosis due to bIood dyscrasias e. Aberrant pulmonary veins
262
Pulmonary Diseases Secondary to Heart Diseases
FIG. IO. Chronic car pulmonale. A, postero-anterior and right B, electrocardiogram views showing chamber enlargements. acteristic changes of right ventricuIar strain p:\ttern.
obIique anterior reveals char-
and aorta, degenerative lesions of the coronary arteries and aorta, primary neoplasms of the heart and chronic car pulmonale.
tors, oxygen therapy is indicated. Digitalis produces equivocal results in this type of failure but should be given a therapeutic trial. Venesection may prove benetkia1 in the presence of high pIasma and cd1 volumes. Antibit&s are indicated in the presence of underIging or intercurrent bronchopulmonar? infections. Morphine is definitely contraindrcated since it depresses respiratory activity. New t_vpes of surgica1 anastomoses are being advocated to reheve pulmonary engorgement in some cases; puImonary to azygos veins (Swan) and various operations for mitral stenosis. Orthopedic conditions require correction.
REFERENCES I.
2.
3.
4. 5.
S~LM~IARY
I. The appearance of puImonary findings during the course of many heart diseases must be evaluated carefully for proper diagnosis, prognosis and therapy. 2. Pulmonary complications ma.y appear following certain congenital cardiac defects, acute and chronic inffammations of the heart
6.
7”
263
N. E. Diseases of the Aorta, Springfield, 1949. Charles C Thomas. diagnosis of NEUHALJSEK, E. B. D. Roentgen doubk aortic arch and other anomalies of the great vessels. Am. J. Roentgenok, 56: I, 1946. GROSS, R. E. Surgical Treatment for Abnormalities of the Heart and Great Vessels. SpringfieId, III., ~947. Charles C Thomas. AISBOI1, M. E. Atlas of Congenital Cardiac Disease. New York, 1936. American Heart Association. REICH, N. E. Protean manifestations of acute rhcumatic fever. Am. Pratt., 12: 645, ~947; Acute rheumatic fwer in urlcommon sites. Am. Pratt., 2: 328, 1951. :a) REICH, N. E. The use of 2-ethylhexanol in acute pulmonary edema. Dis. of Cbesl, 23: 43. 1953. (b) REICH, N. E., ROSENBERG, B. A. and METZ, M. A new therapy for acute pubnonary edema. Neuj l’ork &ate .J. Med., fz: 2647, 1952. TOMLIN, C. E. et at. Recurrent nature of acute benign pericarditis. J. A. M. A., 149: I 215, 1952. REICH,
PuImonary
Diseases Secondary to Heart Diseases vascular
8. REICH, N. E. et aI. The Uncommon Heart Diseases. SpringfieId, III., 1954. Charles C Thomas. g. STEWART, H. J. and HEUER, G. J. Chronic constrictive pericarditis: dynamics of circulation and resuIts of surgica1 treatment. Arch. Int. Med., 63:
disease. Neu: England
J. Med., 244: 965,
‘94’. 12. SAGALL, E. L. et al. Clinica syndrome in patients with puImonary embolism. Arch. Int. Med., 76: 234, ‘945. 13. REICH, N. E. Dissecting aneurysm of the aorta: a chnico-pathoIogic correIation of rg cases. Clinics,
504. 1939. 10. REICH, N. E. Method of appIying flask to chest in tactile fremitus. New York State J. Med., 39:
3: 346, 1944.. 14. LYMBURNEK, R. M. Tumors of the heart: histopathologica and clinical study. Canad. M. A. J.,
3654, ‘939. I 1. WOLFF, L. Diagnostic implicaticns cf pericardial, pIeurn and puImonary invc1vemcr.t in cardio-
30: 368, 1934.
264
Diseases Secondary Diseases
to Heart
DIAGNOSIS AND TREATMENT NATHANIEL
E. REICH, M.D.,Brooklyn,
ULMONARYcompIications are a frequent ocduring the course of manv heart diseases and are- of vita1 concern “to the thoracic surgeon as we11 as to the internist and chest specialist. Our rapidIy expanding knowIedge in both fields makes it imperative to estabIish the correct diagnosis if treatment is to be effective. SeveraI congenita1 cardiac defects, acute and chronic inflammations of the heart and aorta, degenerative Iesions of the coronary arteries and aorta and primary neoplasms of the heart are capabIe of producing a variety of puImonary compIications.
P currence
CONGENITAL CARDIAC LESIONS Interest in this field has been reawakened since it is now possible to correct a number of congenita1 cardiovascular Iesions by surgical means. These incIude patent ductus arteriosus, coarctation, tetraIogy of Fallot, “vascuIar ring” anomaIies, aberrant subcIavian artery, septa1 defects, puImonary stenosis and various transpositions. The onIy congenita1 vascuIar Iesions which directly affect the trachea or bronchi by compression are the vascular ring anoma1ies.l Other defects, such as tetraIogy of FaIIot, produce dyspnea, cyanosis, cIubbing, etc., but these are due to Iack of proper oxygenation of bIood because of the existence of abnorma1 shunts. However, a11 infants with cyanosis or dyspnea do not have congenita1 heart disease. A large diaphragmatic hernia, congenita1 air cyst, Iung anomaIy or puImonary hemorrhage may simuIate the signs and symptoms of congenita1 heart disease. In addition to insuffIcient puImonary bIood flow and congestive failure induced by some congenita1 Iesions, subacute bacteria1 infections may resuIt in embolic infarctions, multipIe abscesses and bronchopneumonia. Right Aoytic Arch. This congenita1 anomaIy may sometimes form a vascuIar ring by the constricting effect of a patent ductus arteriosus or American
Journal OJ Surgery,
Volume 89, January,
,995
252
New York
Iigamentum arteriosus. (Fig. I.) It produces symptoms in the newborn by pressure on the right upper Iobe bronchus thus producing ateIectasis, obstructive emphysema or recurrent infections (trabeobronchitis or pneumonitis). It may also cause the pulmonary artery to compress the anterior surface of the trachea. Symptoms may be initiated Iater in life owing to traction caused by eIongation and posterior displacement of the arteriosclerotic aorta. Pulmonary symptoms incIude dyspnea, cyanosis, cough and chest pain. Head retraction and stridor may occur in infants. X-ray studies of the barium-fiIIed esophagus reveal an indentation at the IeveI of the third intervertebra space. 2 Lipiodol studies in the IateraI position deIineate anterior trachea1 compression just above the carina. Angiocardiography (7 second fiIm) usuaIIy reveals the exact Iocation of the vascuIar deformity. When the obstruction produces symptoms, the Iigamentum arteriosus is divided, aIIowing the pulmonary artery to faI1 forward.3 Double Aortic Arch. This anomaIy occurs when both fourth branchia1 arteries persist. The Ieft (anterior) arch is usuaIIy much smaIIer than the right. The vascuIar ring produces encroachment upon the trachea and esophagus, resuIting in symptoms and diagnostic studies similar to those Iisted under the right aortic arch. Complete reIief is afforded by the Iigation and extirpation of the Ieft (anterior) portion of the doubIe arch. (Fig. I.) Pulmonary Stenosis. PuImonary tubercutosis has been found to occur with greater frequency in the presence of severa congenita1 heart conditions. This is especiaIIy true of puImonary stenosis and tetraIogy of FaIIot. Abbott4 found an incidence of 36 per cent and attributed this to the insuffIcient puImonary bIood flow. This expIanation appears IikeIy in view of the fact that tubercuIosis seIdom occurs in the chronicaIIy engorged Iungs of
Pulmonary
Diseases
Secondary
to Heart
Diseases
Double Aortic Arch Right Aortic Arch FIG;. I. Vascular ring anomalies.
with mitral stenosis. Superimposed patients subacute bacterial infections with pulmonary embolization may also occur. This results in infarction, pneumonitis or lung abscess. The BlaIock shunt operation has been employed with success. A direct attack on the stenosing tissue by incision or punch operation produces equally satisfactory results in other cases, especiaiIy in those with an infundibulum. Patent Ductus Arteriosus. Ordinarily the higher pressure in the aorta allows a return ilow to the pulmonary artery through this abnormal communication. Pulmonary congestion may occur eventually if the shunt is of considerable size. The most frequent complications are congestive heart failure and subacute bacteria1 endocarditis. The Iatter results in embolic infarctions of the lung. Multiple smaIl abscesses or patchy pneumonitis may ensue. Ligation and section of the ductus and the use of antibiotics are producing remarkabIe cures. The operation is contraindicated if evidences of puImonary hypertension appear during the course of this disease. ACUTE
Rheumatic
monia
RHEUMATIC
Pneumonia.
is a rare
but
FEVER
Rheumatic pneuintegra1 phase of acute
rheumatic fever since the collagen tissue and its ground substance may be affected in any part of the body. An incidence of pulmonary involvement has been reported by numerous observers as varying from z to 13 per cent of all cases. The occurrence would be noted in a much larger percentage if aTI patients with acute rheumatic fever had chest x-rays at frequent intervals but especialIy at the height of an attack of acute rheumatic fever. The pathologic changes consist essentially of irregularly scattered areas of hemorrhagic consolidation. Cough and sputum are not prominent features. The diagnosis rests on finding5 signs of transitory or migratory pulmonary exudates. This is demonstrable on serial x-ray studies. (Fig. 2.) Part of one lobe may be involved or the inAammatory process may extend to ail the lobes. The lesions cIear rapidly FolIowing the use of ACTH or cortisone, but this is di&ult to evaluate because of the evanescent character of the lesions. Pleu7i.g~. Pleurisy is probably another manifestation of inflammation of the subendothelia1 connective tissue. Although acute fibrinous or serofihrinous pleuritis is recognized clinically in only 5 to 15 per cent of a11 cases of acute rheumatic fever, it is found in half of the cases that
Pulmonary
Diseases
Secondary
to Heart
Diseases
FIG. 2. Rheumatic pneumonia. A, irreguIarIy scattered areas of soft infiItrations throughout both lower lung fields, more marked on the left side. Onset of pneumonic involvement occurred the previous day. B, marked diminution in extent and intensity of the infiltrations the foIlowing day iIIustrating the transient nature of the exudates.
come to postmortem examination. It may occur on either or both sides and usuaIIy foIlows puImonary or pericardial invoIvement.5 CharacteristicaIIy, the initial severe inspiratory pain and friction rub disappear within several days as effusion accumulates. Routine roentgenograms demonstrate tIuid Ievels in a high percentage of cases. Aspiration is seIdom required but shows a clear or cloudy serofibrinous exudate. On rarer occasions it may appear hemorrhagic, possibly due to the presence of pulmonary infarction. Although the fluid is rapidly absorbed, adhesions deveIop occasionaIIy. These may be pIeuropericardia1 adhesions which give the cardiac silhouette a scaIIoped appearance or partiaIIy obliterate the pleura1 space. MITRAL
VALVULAR
thickening of the capillary basement membrane. These changes thicken the aIveoIar wall and interfere with gaseous exchange. They eventuaIIy produce an ineIasticity of the Iung tissue which seriously interferes with ventilation. Dyspnea, orthopnea and cyanosis are major symptoms. Hemoptysis occurs in IO per cent of cases of mitra1 stenosis due to vascuIar hypertrophy. AIthough it may appear at any time during the course of the disease, it usuaIIy denotes an advanced degree of stenosis with increasing pulmonary congestion. The blood loss is seIdom massive but augurs a dire prognosis. Cough is commonIy present, and when productive the sputum may revea1 numerous “heart faiIure” ceIIs. The sputum is mucoid but may be bIood-streaked or frankIy purulent due to secondary bronchopuImonary infection. Acute Pulmonary Edema. This may occur in mitraI stenosis and other vaIvuIar lesions even when reguIar sinus rhythm exists. It usuaIIy foIIows severe exertion, operation, rapid digitalization or paroxyspregnancy, ma1 tachycardia. Attacks are precipitated by the added strain on the right heart in the presence of puImonary congestion. Treatment incIudes digitaIization, morphine and atropine, and antifoaming agents.6 oxygen, mercurials
DISEASE
Several pulmonary complications commonIy occur folIowing distortion of the mitra1 vaIve. On rare instances mitral stenosis may be associated with an interauricuIar septa1 defect (Lutembacher’s syndrome). Chronic Congestive Failure. MitraI vaIvuIar lesions, especiaIIy stenosis, may produce earIy puImonary congestion. As a resuIt of chronic congestive heart faiIure the Iungs become firm, dense, dry and red-brown in appearance. There is interstitia1 edema, increased coIIagen and 254
PuImonary
Diseases
Secondary
Subsequently, surgery may be indicated. Remarkable results are constantly being reported by numerous surgeons. Commissurotomy or finger fracture of the stenosed valve, or anastomosis bet\yeen the aorta and pulmonary arter! or between the right inferior pulmonary vein to the azygos veins will relieve puImonar> hypertension. Rupture. Rupture of a papillary muscle of chordae tendineae results in an unusually rapid form of pulmonary congestion. This may be caused by trauma, superimposed subacute bacterial endocarditis or coronary thrombosis. It produces an extremely loud murmur which is frequently audible at a distance from the chest. Pulmonan, Infarction. The effects of pulinfarction are discussed elsewhere. monary Suffice it to sa\- that this serious compIication may result from the discharge of clots from the right auricle during auricular fibrillation. It is important not to overlook other major causes such as peripheral or pelvic \.enous thrombosis. Early treattnent of the primar? heart condition and the use of anticoagulants have greatI\- reduced morbidity and mortality. Amputation or ligation of the auricular appcndages is presently employed with success in cases with recurrent embolization thladden). HtCTERl.iL
ENDOChRDITIS
The acute and subacute forms of bacterial infections ot‘ the valvular and mural endocardium may be caused by manv microorganisms. Howver, the non-hemoly& streptococcus accounts for 95 per cent of all cases. In order for emboli to reach the lungs, the right heart must obviously he affected. Septal defects, pulmonary or tricuspid stenosis, patent ductus arteriosus, tetralogy of Fallot or other combined congenital lesions may also provide a nidus. Pulmonary emboli and infarctions, congestion, bronchopneumonia or lung abscesses ma! fx encountered. The infarctions usually are too small to be visuahzed on the x-ray. PIeural effusions or hetnorrhages are not uncommonly found. Emboli in pulmonary arteria1 branches may also result in arteritis or mycotic aneurysm. Symptoms include cough, chest pain, dypsnea or bloody expectoration. Antibiotics are effective in most cases when properly selected and administered in adequate dosage.
to Heart -\CCTE
Diseases
X0&-SPECIFIC
PERICARDITIS
Acute pericarditis is usually regarded as evidence of serious underIying disease such as rheumatic fever, mvocardia1 infarction, uremia, tubercuIosia and pneumonia. There is a rarer group of disorders which may also produce acute pericarditis. However, \ve are primarily concerned with acute non-specific pericarditis. This type occurs in j-oung males as a rule and has an increasing incidence. Frequency ot recurrences has been emphasized lately. In\-ariably there is involvement of the pleura in \-iral inflammation of the pericardium. A pleural lesion must be suspected n-hen the clinical ichest pain, dyspnea, fever, rub) and laboratory findings ieIectrocardiogram and y-ray) of acute non-specific pericarditis are accompanied or followed shortly by the onset of a pleural friction rub which may last for da?-s or weeks. This ma>- progress to pleural effusion of \.arying degree (usualI\- small in amount) and, finally, progressive pleurai thickening. The aspirated fIuid is esudativc ancl non-blood\and ma>- be bilateral.; Pulmon:ir>- involvement is uncommon. Pleural fluid is demonstrable on the roentgenogram. Persistence of lever, following improvement of the pericarditis and invariable recovery further suggest this possibility. Result; with aureom!-tin, chloromvcetin’~ and terramycin@’ have been equivocal thus far. The disease is self-limited but aspiration of fluid is indicated \vhen its presence embarrasses cardiac or pulmonar>function. t Fig. 3.) CHRONIC
CONSTRICTIVE
PERICr\RDITIS
Chronic constrictive pericarditis is a dense fibrous thickening of the pericardium (Fig. 4) \vhich ma)- cause compression of the heart ancl interfere \vith normal filling. It may completeI! envelop the heart or produce a band-like or other localized strictures. It is characterized clinically by the triad of a small quiet heart, elevated venous pressure and ascites. The cause is often obscure. AIthough tuberculosis is the most common cause in cases \yith proven etiology, this disease accounts for onI\ 13 per cent of al1 cases. Rheumatic fever, pleuropulmonary disease, myocardial infarction and neoplastic involvement make up the buIk of all cases. (Fig. 4.) The Iungs usuaIIv do not revea1 significant congestion or fihros’is unIess tuberculosis is the 255
PuImonary
Diseases
Secondary
to Heart
Diseases
FIG. 3. Marked pericardia1 effusion. Reduction of pulmonary function by very marked effusion. A, note waterbottle configuration of pericardial effusion. B, in Ieft anterior obIique view of angiocardiogram the pericardia1 effusion is more sharpIy defined. Note posterior compression of Iung space.
FIG. 4. Types of chronic intrapericardial pressure. A, compIete enveloping scar. B, ventricuIar scar; note compression with bulge above. C, band over ventricIes. D, band around superior vena cava, resuIting in superior vena cava1 syndrome. E, band over auricIes. F, tamponade (bIood or effusion); note compression of auricular appendages and intrapericardia1 portions of venae cavae and puImonary veins. G, adhesive pericarditis. H, band around inferior vena cava, resuIting in inferior vena cava1 syndrome. (From REICH, N. E. The Uncommon Heart Diseases. SpringfieId, III., 1954. Charles C Thomas.)
256
PuImonary
Diseases
Secondary
to Heart
Diseases
5A
FIG. 5. Chronic constrictive pericarditis. A, eIectrocardiogram reveals negative T waves and decreased voltacre comnlrses. B. x-ravs reveal chronic tuberculous pericnrditis with biIatcra1 apical lung involve__ of ORS ment. Arrows point to calcification. 9
cause.
(Fig. 5B.)
Yet pIeura1 adhesions
sions are fairly common. pericardial auricle,
may be seriousIy around
interfered
pulmonary
congestion
owing to marked by ascites.
over the left
of this chamber
with.
Constriction
vein orifices engorgement.’
may be due to extensive monary
mainly
and filling
the pulmonary
in marked
When the constricting
scar is Iocalized
reIaxation
or effu-
pIeural
or decreased eIevation
The vita1 capacity
to 68 per cent of normal
may result Dyspnea
effusions,
signs of cardiac appear.
or puImonary
Dramatic
ing decortication practiced
by
Streptomycin, atives
of the Cutler
are of value
tuberculous
as
heart. far
embarrassment occurs This back
PAS and isonicotinic in the treatment
follow-
was first as
1924.
acid derivof active
lesions.
puISYPHILIS
vital capacity
of the diaphragm was reduced
improvement
up
in the cases reviewed
b?- Stewart and Heuer.8 Signs of cardiac fixation and evidences of the right heart faiIure sh-ndrome comprise the cardiac findings. The electrocardiogram is characteristic. (Fig. 5A.) ;IledicaI treatment is of little avai1 when
It
has been
estimated
miliion Americans yet only
2
that
a quarter
have cardiovascular
of a
syphilis,
per cent of these develop aneurysm.g
The unfavorable prognosis of cardiovascular syphiIis is we11 known. Aortic vaIvuIar lesions are generally considered to be more serious than mitra1 Iesions because they are often caused by negIected syphilis and because of
PuImonary
Diseases
Secondary
to Heart
Diseases
FIG. 6. SyphiIis.
the poor prognosis. When Ieft ventricuIar faiIure ensues, puImonary edema is rapid and reIativeIy unresponsive to therapy. SyphiIitic myocarditis is equaIIy diffIcuIt to treat in the stage of congestive faiIure. Saccular aneurysms may reach aIarming proportions and may produce symptoms and signs of compression of the Iungs, bronchi and other structures depending on the direction and extent of their diIation. (Fig. 6.) Aneurysms of the arch are most important because of their greater proximity to respiratory structures. PuImonary signs of ateIectasis, bronchiectasis and secondary infection may resuIt. Trachea1 tug may be elicited and Ieft recurrent IaryngeaI nerve compression may resuIt in hoarseness or aphonia. Phrenic nerve invoIvement may produce paraIysis of the diaphragm, thereby interfering with oxygenation. The most important of these compIications is atelectasis. UnIess the pathoIogic condition is su&cientIy extensive, tactiIe fremitus may be non-reveaIing.10 Diminished breath sounds and aItered resonance on percussion may be noted. Bronchiectasis may ensue from Iack of drainage and infection induced by bronchia compression. EndobronchiaI tumors and other Iesions must be differentiated occasionaIIy. Bronchography may revea1 the true cause of IocaIized obstructions or distortions of the bronchia tree. Tomography may excIude endobronchia1 tumor
by delineating cavitation within the aneurysmal mass. This is unreIiabIe when the aneurysm is cIotted. Kymography may show typica expansiIe puIsations when the aneurysm is uncIotted. Further differentiation of adenoma, carcinoma and inspissated mucous or granuIomatous tissue rareIy requires cautious bronchoscopy, biopsy or PapanicoIaou staining of sputum for tumor cells. SeroIogy is positive in approximateIy 85 per cent of cases. Calcification in the waI1 of the ascending aorta (Ieft anterior obIique view) is characteristically found in approximately haIf of a11 cases of Iuetic aortitis. Antibiotics now have a definiteIy estabIished position in the treatment of cardiovascular syphiIis. Aneurysms have been wired and eIectrocoaguIated, or surrounded by various ceIIophanes in order to produce a fibrotic reaction which wiI1 shrink or prevent further expansion with rupture. MYOCARDIAL
INFARCTION
This condition has aIready received the direct attention of surgeons. Not onIy are a number of surgica1 procedures avaiIabIe pertaining to revascuIarization of the heart, but aIso excision of Iarge myocardial scars and aneurysms is being practiced presentIy. A number of puImonary compIications is possibIe in patients suffering from myocardial
258
PuImonary
Diseases
Secondary
to Heart
Diseases
tion is commonly confused with pneumonia or In addition to duIIness puImonary infarction. or ffatness and bronchia breathing, a shower of fine inspiratory rales is frequentIy heard. The clinical course is usuahy severe with frank hemoptysis, marked pyrexia, and Ieukocytosis. Signs of congestive failure may ensue in some patients. The x-ray picture is diagnostic.” Pulmonary Infarction. Emboli to the Iung may be due to auricular fibrillation, peripheral and peIvic venous thrombosis, bacteria1 endocarditis and right-sided mural thrombosis owing to myocardial infarction. Emboli may enter any part of the pulmonary arteria1 system although the right base is commonIy affected. The sudden onset of chest pain, cough and bloody expectoration usually heralds this complication. Signs incIude a patch of crepitant rales, dullness and bronchia breathing. Pleural pain is present in half of the cases but a rub occurs in only IO per cent of the cases.‘l Effusions are usuahy minimal and may be clear or sanguineous. On rare occasions large recurrent effusions may dominate the cIinica1 picture. These effusions seldom require aspiration. The icterus index may be slightly eIevated on the second or third day due to absorption. The electrocardiogram is diagnostic in a large percentage of the cases, but x-ray investigation is usually disappointing. Immediate anticoagulant therapy is indicated. High venous ligation must be considered when embolization from the peripheral or pelvic veins is suspected. Inferior \‘ena caval ligation produces peripheral edema which may be quite disabhng for long periods,
infarction. They are mistakenly referred to as in most instances. Five condi“pneumonia” tions must be considered when lung findings appear following this condition. This dire pulmonary Pulmonary Edema. complication is due to acute left ventricmar faiIure, since the left ventricuIar musculature is most commonly affected by infarction. It may be the dominant manifestation of an acute myocardial infarct appearing suddenIy within hours to days folIowing an occlusion. Symptoms include thoracic oppression, dyspnea, orthopnea and cough. The attack may be characterized by episodes of paroxysmal nocturna dyspnea with wheezing respirations and rhonchi. MiId puImonary congestion is common when the dominant manifestation is pain, shock or congestive heart faiIure. BasaI raIes are heard in such cases, especiaIIy on the right side. It may become more severe with noisy respirations when copious white or pink foam appears. In advanced cases it may pour out of the oronasa1 passages and bubbling raIes may become audibIe up to the apices. DigitaIis, oxygen, morphine and atrophine, diuretics and antifoaming agents are empIoyed.6 Anticoagulants are aIso employed since they affect the ultimate prognosis. Atelectasis. It is very common to find a Iocalized patch or patches of crepitant rales at the Ieft Iung base, coinciding with x-ray, demonstration of plate-like areas of ateIectasis. However, this is found so frequently early in the attack that it is usuaIIg considered norma by WoIff and not necessaray indicative of congestive failure, pulmonary infarction or infection.” An area of dullness and bronchia breathing without temperature elevation usually indicates the presence of atelectasis. Oxy-gen and expectorants may be of vaIue in such cases. Rebreathing is obviousIy contraindicated following myocardial infarction. Pneumonia. Pneumonia is rarely associated with acute myocardia1 infarction and yet this diagnosis is made with aIarming frequency and treated as such. The possibility of other Iesions such as localized pulmonary edema, puImonary infarction, ateIectasis or chronic bronchiectasis must be investigated. When there is leukocgtosis, hyperpyrexia and mucopuruIent sputum, a therapeutic trial with an antibiotic is indicated. Localized Pu1monar.y Edema. This compIication is not uncommon in acute myocardial infarction and is usually biIatera1. The condi-
DISSECTING
ANEURYSM
There has been a marked improvement in the recognition of dissecting aneurysms during the last decade. (Fig. 7.) This has been occasioned by an awareness of the disease on the part of most clinicians. Efforts at Iimiting the pathoIogic condition to a single factor have been generahy unsuccessful although medial necrosis is recognized as the most important pathoIogic factor. 1 Pulmonary symptoms are rare in the absence of rupture ahhough cough and hemoptysis may occur. When not instantly fatal, various cIinica1 syndromes may appear depending on the site and extent of dissection or rupture. Twenty per cent of al1 dissections rupture into the left pleural cavity and present the 259
PuImonary
Diseases
FIG. 7. Dissecting aneurysm. EventuaI rupture gram prior to rupture. 6, injected specimen.
Secondary
into the left pIeuraI
characteristic signs of chest fIuid.13 However, fluid in the left pleura1 cavity must be differentiated from tubercuIosis circuIatory faiIure (which seIdom affects the left side aIone), maIignancy of the pIeura and Meig’s syndrome. The aspiration of pure bloody fluid is highIy significant for dissection. Maiignancy may revea1 characteristic ceIIs in addition, and tubercuIosis may show organisms on smear or cuIture. On rare occasions ruptures into the right pIeura1 and retroperitonea1 space, mediastinum and other structures have been described. Massive hemoptysis may indicate rupture directIy into the trachea, bronchi or Iung. Blood repIaCement and pleura1 aspiration are indicated. Enzymatic agents (tryptar,@ or streptokinase and streptodornase) are capabIe of Iiquefying bIood cIots prior to aspiration. SurgicaI repair of the site of rupture may be attempted by empIoying gelatin sponge cuffs or by applying poIythene ceIIophane (PT 300). Occasiona cases are known to hea spontaneousIy.
to Heart
Diseases
space with massive
hemothorax.
A, roentgeno-
any tissue in the body, the most common sources being the trachea, breast, Iung and stomach. On rare occasions they may produce the syndrome of chronic constrictive pericarditis. Primary neopIasms occur approximately once in every 2,000 cases that come to autopsy.14 The frequency of types has been reported in the foIlowing order: myxoma, sarcoma, rhabdomyoma, fibroma, Iipoma, angioma, cystoma, papilloma, teratoma and epicardia1 epitheIioma. Any heart chamber may be the site of neopIasm, but the right side is more commonIy invoIved. Therefore, pulmonary metastases from primary cardiac malignancies are frequently found. Diagnosis of a primary Iesion shouId be suspected in cases of heart disease of undetermined etioIogy when: (I) roentgenogram shows an unexpected or bizarre heart size or shape, (2) there are progressive eIectrocardiographic (3) pericardia1 changes otherwise unexpIained, aspiration reveaIs red ceIIs and tumor ceIIs, changing arrhythmias and and (4) frequentIy murmurs appear. PeduncuIated intracardiac tumors may produce intermittent obstruction of valve orifices and simuIate vaIvuIar lesions. The subsequent appearance of metastatic puImonary Iesions with or without pIeura1 invoIvement shouId increase the index of suspicion.
NEOPLASMS
AIthough primary tumors of the heart are reIativeIy rare, metastatic Iesions are not uncommon. Metastatic lesions may arise from 260
Pulmonary
Diseases
Secondary
to Heart
Diseases
This is especialIy true when pIeura1 transudates aIso contain tumor ceIIs or when stained bronchial secretions reveaI tumor celIs of noncarcinomatous origin. (Fig. 8.) Treatment mav be effective when the tumor is benign and pedunculated. Such tumors have Y
TABLE 1 DlSEASES OF TllE LUNG PAKEYCHYMA"
1. ln,fections Acute pneumonitis, chronic bronchitis, bronchiectasis, chronic bronchopulmonary suppuration; various virus and rickettsial diseases 2. Infectious Granulomus Tuberculosis, sarcoidosis, fungus infections 3. Parasitic Diseases are excellent Isease and schistosomiasis :~y$,“’ 4. Allergies Bronchial asthma and aIlergic bronchitis (with secondary emphysema) 5. Chemical and Physical Irritants Dusts, fumes, gases, x-ray and radium fibrosis. (!ncluded are the various pneumoconioses of which silicosis, anthracosis and berylIosis are the most important.) 6. Malignancies malignancies. Extensive primary or metastatic (Metastatic endoIymphatic carcinoma is the most common cause.) 7. ,l/liscellaneous Diseases amyIoidosis (primary systemic and Pulmonary secondary forms), pulmonary fibrosis and emphysema due to various causes, congenital cystic Iung disease, massive puImonary coIIapse, extensive pleural adhesions, chronic passive congestion (secondary sclerosis) *Tables I to IV from REICH, N. E. The Uncommon Heart Diseases, SpringfieId, III., 1954. CharIes C Thomas.
FIG. 8. Syphilis with malignancy in a sixty-one year 01~1man. Note aneurysma diIation of thoracic aorta and large bronchogenic carcinoma invading mediastinum with metastases to right upper Iung field. Autopsy corroboration.
Since we are considering the l>asic mechanism.’ pulmonary compIications of primary heart disease, the other factors wilI not be considered in any detail. However, it is significant that TABLE II I'HI\IAHY HEAHT DISE4SES I. JIitraI
2.
been removed from within the heart (Beck, Bailey). The deveIopment of a good mechanical heart will permit more extensive surgery. Radiotherapy and chemotherapy have produced no known cures thus far, aIthough life may be prolonged in some cases of malignant Iymphoma. Urethane, nitrogen mustard and certain radioactive isotopes have proven of some benefit in appropriate cases.
3. 4.
5. 6.
CHKONIC
COR
PCLMOKALE
Chronic car puImonale is a term applied to hypertrophy of the right ventricle, with or without congestive heart faiIure. Despite a varied etiology which includes a number of puImonary (TabIe I), cardiac (TabIe II) and vascular disturbances (TabIe III), and bony cage deformities (TabIe IV), increased resistance and pressure in the pulmonary circuit is the
valve involvement a. .\litral stenosis b. Lutembacher’s syndromr (mitral stenosis \\ith intcrauricular septal defect) c. Subacute bacterial endocarditis Left ventricular failure, due to myocardial infarction, amyloid disease, beriberi, etc., but most frequent1.y due to aortic disease or hypertension. (None of these wiII show right axis deviation but may have pulmanic hypertension, right ventricular hypertrophy and failure.) Auricular fibrillation (with right auricular thrombus producing pulmonary emboli) Chronic constrictive pericarditis affecting the Icft auricle Tumor of heart causing mural thrombi and pulmonarv embolism Congenital defects (patent ductus, srptal defects, aberrant pulmonary veins)
when congcXive failure supervenes, other puImonary findings become superimposed on the underlying lung disease process (e.g., pulmonarq’ emph~-scma and other bronchopulmonar) diseases). Kyphoscoliosis, funnel chest and other chest deformities are interesting in that they mav also cause hvpertrophp and dilation of the
261
PuImonary
Diseases
Secondary
to Heart
Diseases
tasis or secondary emphysema in addition to bronchopuImonary infections. The diagnosis of chronic car puImonaIe is based upon the recognition of the underIying causative factor (TabIes I to IV) and the discovery of right ventricuIar enlargement with
I.
2.
TABLE IV THORACIC BONY CAGE DEFORMITIES CongenitaI deformities of the chest (funne1 or pigeon breast) KyphoscoIiosis due to neuromuscular diseases (e.g., poIiomyeIitis) and bone diseases (e.g., rickets) 3. ThoracopIasty 4. Ankylosing SpondyIarthritis
or without evidences of faiIure. Right-sided faiIure is recognizabIe by the presence of hepatomegaly, dependent edema and ascites, eIevated venous pressure and proIonged armto-Iung circuIation time. It is important to remember that dyspnea and cyanosis are usuaIIy due to the underIying Iung or heart disease, but may be aggravated in the presence of congestive faiIure. RoentgenoIogic studies show: (I) an exaggeration of the hiIar markings due to enlargement of the puImonary artery and its major branches; (2) enIargement of the right ventricuIar outflow tract in the Ieft anterior obIique view as indicated by the posterior dispIacement ofthe interventricular groove, and in the right IateraI position by the encroachment of the right ventricIe on the retrosterna1 space; and (3) prominence of the pulmonary segment in the postero-anterior view. (Fig. IoA.) When decompensation intervenes, (I) the right auricIe may (2) there is enIargement of become enlarged, the right ventricuIar inflow tract as weI1, (3) the transverse diameter of the heart is further increased in size, (4) the Iower Iung fieIds revea1 congestive changes or fIuid. EIectrocardiograms show a progressive right ventricuIar strain pattern. (Fig. IoB.) PuImonary function tests are empIoyed in the evaIuation of the Iung status. The vita1 capacity is markedIy diminished. Dyspnea is out of a11 proportion to the cardiac state and is mainIy due to the associated pulmonary disease. Treatment must be directed at the causative lesion as we11 as myocardia1 insuffrciency. In many disorders, such as kyphoscoIiosis, it is unsatisfactory. fn others, such as patent ductus arteriosus, earIy surgica1 intervention resuIts in compIete reIief. When dyspnea and cyanosis occur due to either cardiac or puImonary fac-
FIG. g. Kyphoscohotic heart disease. Marked rachitic spina deformity in fifty-eight year old woman. Severe exertiona dyspnea. Note spinat curvature toward right eventually producing chronic car pulmonaIe. Note prominent puImonary vesseIs, atelectasis at bases with compensatory emphysema.
right vent&e. It is the consensus that the syndrome occurs in about 75 per cent of cases of severe kyphoscoIiosis. (Fig. 9.) These deformities may aIso produce compression ateIecTABLE III DISEASES OF THE PULMONARY VASCULAR SYSTE’M Arteries I. Pulmonary a. EmboIism (I) PhIebothrombosis and thrombophIebitis of the peripherat, abdominal and peIvic veins and the venae cavae. (Thromboembolic complications due to myocardial infarction are considered under primary heart disease.) (2) EmboIism due to air, fat and foreign bodies (bullets, etc.) b. Endarteritis obIiterans (Ayerza’s disease) c. Aneurysm (syphilis, congenita1, post-stenotic) d. Thrombosis due to blood dyscrasias e. Persistent truncus arteriosus f. Thromboangiitis obliterans, Iupus erythematosus disseminatus, poIyarteritis nodosa g. Congenital and acquired intravascuIar bands h. Primary neoplasms of the puImonary vascuIar system i. Neoplastic invasion or compression of Iarger branches 2. Pulmonary Veins a. Massive infectious endophIebitis b. ThrombophIebitis migrans c. NeopIastic invasion or compression of large branches d. Thrombosis due to bIood dyscrasias e. Aberrant pulmonary veins
262
Pulmonary Diseases Secondary to Heart Diseases
FIG. IO. Chronic car pulmonale. A, postero-anterior and right B, electrocardiogram views showing chamber enlargements. acteristic changes of right ventricuIar strain p:\ttern.
obIique anterior reveals char-
and aorta, degenerative lesions of the coronary arteries and aorta, primary neoplasms of the heart and chronic car pulmonale.
tors, oxygen therapy is indicated. Digitalis produces equivocal results in this type of failure but should be given a therapeutic trial. Venesection may prove benetkia1 in the presence of high pIasma and cd1 volumes. Antibit&s are indicated in the presence of underIging or intercurrent bronchopulmonar? infections. Morphine is definitely contraindrcated since it depresses respiratory activity. New t_vpes of surgica1 anastomoses are being advocated to reheve pulmonary engorgement in some cases; puImonary to azygos veins (Swan) and various operations for mitral stenosis. Orthopedic conditions require correction.
REFERENCES I.
2.
3.
4. 5.
S~LM~IARY
I. The appearance of puImonary findings during the course of many heart diseases must be evaluated carefully for proper diagnosis, prognosis and therapy. 2. Pulmonary complications ma.y appear following certain congenital cardiac defects, acute and chronic inffammations of the heart
6.
7”
263
N. E. Diseases of the Aorta, Springfield, 1949. Charles C Thomas. diagnosis of NEUHALJSEK, E. B. D. Roentgen doubk aortic arch and other anomalies of the great vessels. Am. J. Roentgenok, 56: I, 1946. GROSS, R. E. Surgical Treatment for Abnormalities of the Heart and Great Vessels. SpringfieId, III., ~947. Charles C Thomas. AISBOI1, M. E. Atlas of Congenital Cardiac Disease. New York, 1936. American Heart Association. REICH, N. E. Protean manifestations of acute rhcumatic fever. Am. Pratt., 12: 645, ~947; Acute rheumatic fwer in urlcommon sites. Am. Pratt., 2: 328, 1951. :a) REICH, N. E. The use of 2-ethylhexanol in acute pulmonary edema. Dis. of Cbesl, 23: 43. 1953. (b) REICH, N. E., ROSENBERG, B. A. and METZ, M. A new therapy for acute pubnonary edema. Neuj l’ork &ate .J. Med., fz: 2647, 1952. TOMLIN, C. E. et at. Recurrent nature of acute benign pericarditis. J. A. M. A., 149: I 215, 1952. REICH,
PuImonary
Diseases Secondary to Heart Diseases vascular
8. REICH, N. E. et aI. The Uncommon Heart Diseases. SpringfieId, III., 1954. Charles C Thomas. g. STEWART, H. J. and HEUER, G. J. Chronic constrictive pericarditis: dynamics of circulation and resuIts of surgica1 treatment. Arch. Int. Med., 63:
disease. Neu: England
J. Med., 244: 965,
‘94’. 12. SAGALL, E. L. et al. Clinica syndrome in patients with puImonary embolism. Arch. Int. Med., 76: 234, ‘945. 13. REICH, N. E. Dissecting aneurysm of the aorta: a chnico-pathoIogic correIation of rg cases. Clinics,
504. 1939. 10. REICH, N. E. Method of appIying flask to chest in tactile fremitus. New York State J. Med., 39:
3: 346, 1944.. 14. LYMBURNEK, R. M. Tumors of the heart: histopathologica and clinical study. Canad. M. A. J.,
3654, ‘939. I 1. WOLFF, L. Diagnostic implicaticns cf pericardial, pIeurn and puImonary invc1vemcr.t in cardio-
30: 368, 1934.
264