Myocardial tuberculosis presenting as restrictive cardiomyopathy

Volume Number

Table

120 3

Brief Communications 703

I. Cardiac catheterization Pressure (mm &?I (mean)

Site Superior vena cava Inferior vena cava Right atrium Right ventricle Pulmonary artery Right pulmonary artery Left pulmonary artery Left atrium Left ventricle Right lower pulmonary vein Left upper pulmonary vein Aorta

(12) 30112 30/14 (18)

02) 110/12 110/70

Oxygen saturation* (%) 64 52 60 59 56 55 62 80 19 96 96 80

*Obtained while patient was breathing 100% oxygen.

of the interatrial communication, the degreeof right ventricular hypoplasia, and the maintenance of adequate blood flow to the lungs.The age of onset of symptoms varies; however, prolonged survival into adult life is rare. Sackner et a1.3describeda patient who survived to the age of 30, dying from refractory right heart failure. The clinical course of our patient is unique due to his prolonged survival into adult life. Furthermore, despite marked cyanosishe has remained relatively asymptomatic. We assumethat this wasdue to multiple physiologic responsesthat preserved tissue oxygenation despite severe cyanosis,such assecondarypolycythemia and increasesin cellular 2,3-DPG (2,3-diphosphoglycerate). However, the greatly increasedhematocrit can lead to undesirableconsequences.In this patient depressederythropoiesis from chronic renal failure led to attenuation of the polycythemic response.In addition, the hypoplastic right ventricle protected the pulmonary vascularbed from increasedflow that occursin a simple atria1 septal defect, but fortuitously the degree of hypoplasia allowed sufficient flow, permitting adequate oxygenation and the prevention of right-sided heart failure. Moreover, it is possiblethat long-standing hypertension in our patient may have played a role in decreasingthe right-to-left shunt, thereby augmentingblood flow through the pulmonary artery. These mechanisms may explain the prolonged survival in our patient. The presenceof cyanosisin an adult with an atrial septal defect usually heralds the development of suprasystemic

pulmonary

hypertension.

Only rarely

has cyanosis

with an atrial septal defect been reported in the presence of normal pulmonary artery pressures.In the present case, cyanosisoccurred despite normal pulmonary artery pressures becauseof restriction of venous inflow by the hypoplastic right ventricle, causingan obligatory right-to-left shunt at the atria1 level. The tricuspid valve, though small, wasnot a significant abnormality; its size simply reflected hypoplasia of the right ventricle. Other anatomic anomalies may favor right-to-left shunting with normal pulmonary artery pressures.Gallaher et a1.4describedthree patients

with

abnormally

large

eustachian

valves

that

extended from the origin of the inferior vena cava to the

margin of the atria1 septal defect, selectively channeling blood into the left atrium. Similarly, congenital anomalous systemic venous connections that terminate in the atria1 wall at the site of the atria1 septal defect result in the return of venous blood into the left atrium.5 After pneumonectomy, cyanosisfrom shunt reversal acrossan atria1 septal defect may occur despite normal pulmonary artery pressures,possibly due to mechanical alterations in right atria1 and ventricular configurations.6 In conclusion,severalpoints in this caserequire specific attention. First, although this malformation is often corrected surgically, prolonged asymptomatic survival despite severe cyanosis may permit a conservative treatment strategy in some patients. Second, not all right-to-left shunting acrossan atria1 septal defect is associated-with severepulmonary hypertension. Although this congenital malformation is rare in an adult population, it and other anatomic anomaliesshould be consideredin the differential diagnosisof a cyanotic adult with an atria1 septal defect and normal pulmonary artery pressures.Finally, in evaluating a patient with congenital heart diseasein whom the transthoracic echocardiogramis not adequate, transesophagealechocardiography should be performed. This is especially important in a sick adult with a potentially complex anomaly prior to cardiac catheterization, which may be difficult, prolonged, and of increased risk. REFERENCES

1. Okin JT, Vogel JHK, Pryor R, Blount SG Jr. Isolated right ventricular hypoplasia. Am J Cardiol 1969;24:135-40. 2. Enthoven R, Dunst M, Richman B. Congenital hypoplasia of the right ventricle and tricuspid valve with survival into adult life. Am J Cardiol 1963;11:532-6. 3. Sackner MA, Robinson MJ, Jamison WL, Lewis DH. Isolated right ventricular hypoplasia with atria1 septal defect or patent foramen ovale. Circulation 1961;24:1388-402. 4. Gallaher ME, Sperling DR, Gwinn JL, Meyer BW, Fyler DC. Functional drainage of the inferior vena cava into the left atrium-three cases. Am J Cardiol 1963:12:561-6. 5. Raghib G, Ruttenberg HD, Anderson RC, Amplatz K, Adams P, Edwards JE. Termination of left superior vena cava in left atrium, atria1 septal defect, and absence of coronary sinus. Circulation 1965;31:906-18. 6. Holtzman H, Lippman M, Nakhjavan F, Kimbel P. Postpneumonectomy interatrial right-to-left shunt. Thorax 1980; 35:307-g.

Myocardial tuberculosis presenting restrictive cardiomyopathy

as

H. K. Bali, MD, DM, S. Wahi, MD, B. K. Sharma, MD, I. S. Anand, DPhihOXON), B. N. Datta, MD, and P. L. Wahi, MD. Chandigarh, India From the Departments of Cardiology, Medicine and Pathology, Postgraduate Institute of Medical Education and Research. Reprint requests: Professor P. L. Wshi, Department of Cardiology, Postgraduate Institute of Medical Education and Research,Chandigarh 160 012, India. 414122043

704

Brief Communications

American

September 1990 Heart Journal

1. Two-dimensional echocardiographicstudy. A, Apical four-chamber view showing nodular filling defect at right ventricular apex. 6, Parasternal short-axis view at baseof heart showingnodular shadows at right ventricular outflow tract and interatrial septum. C, Parasternal long-axis view showing nodular echodensity in left atrium in relation to posterior mitral leaflet. Note thickened left ventricular posterior wall and interventricular septum. Fig.

Table

Before After

1. Hemodynamic data

treatment treatment

RA

RV

MPA

LV

A0

v15 ii a3v2 2

32 ED14 18 ED5

33/14 ?i?s 1716 is

99 ED14 100 ED12

100/65 @ 104167 84

RA, Right atrium; RV, right ventricle; MPA, main pulmonary left ventricle; AO, aorta; ED, end-diastolic pressure.

artery;

LV,

We describea caseof tuberculous involvement of the myocardium diagnosedby endomyocardial biopsy, which responded to antitubercular therapy. Our patient initially had fever of undetermined origin and featuresof restrictive cardiomyopathy and supraventricular rhythm disorder. The clinical, echocardiographic,angiographic, and histopathologic features are described in a short review of the literature. Patient R. K., a 21-year-old man was admitted to the Nehru Hospital of the Postgraduate Institute of Medical Education and Research, Chandigarh, with moderategrade,remittent fever of 7 months’ duration. He had a his-

tory of three episodesof sudden-onset,self-limiting palpitations. He also had progressiveanorexia and weight loss. During the examination, the pulsewas54/min and irregular, and blood pressurewas 110/70 mm Hg. He had mild pallor. Jugular venouspressurewasraised 10cm abovethe sternal anglewith a prominent vy collapse.Firm hepatomegaly of 5 cm and splenomegalyof 2 cm were present. The apex beat wasin the fifth intercostal space10 cm from the midline. There wasa mild left parasternal heave, and pulmonary valve closure was palpable. The first heart sound wasvariable, and the pulmonary component of the second sound was loud. Ss gallop was audible, but no murmurs could be appreciated. During his hospital stay the patient continued to be febrile. He had recurrent episodesof short-lasting atria1 fibrillation and paroxysmal supraventricular tachycardia. He wasvery sensitiveto digoxin. Even a small dosecausednausea,vomiting, and excessiveslowing of the ventricular rate. During clinical investigation, blood counts and routine biochemistry findings were normal except for mild normocytic normochromic anemiaand an elevated erythrocyte sedimentation rate. Results of a Mantoux test werenegative. Sputum examination findings on six occasionswerenegative for acid-fast bacillus. Results of chest x-ray examination showedmoderatecardiomegaly

Volume

120

Number

3

Brief Communications

705

2. Right ventricular angiogramin anteroposterior (left) and lateral (right) projections showingfilling defect at right ventricular apex.

Fig.

with prominent upper lobar veins and an enlarged left atrium. ECG showed varying rhythms at different times including normal sinus rhythm, paroxysmal supraventricular tachycardia, occasionally with aberrant conduction, and atrial fibrillation. Nonspecific ST and T changeswere alsoseen.Both M-mode and two-dimensional echocardiographic studies were done. Left ventricular contractility was normal. Interventricular septum and left ventricular posterior wall thicknesses were increased. There were localized echodenseshadowsin the left atrium near the posterior mitral leaflet, the posterior wall of the aorta, and the interatrial septum. Similar nodular shadowswere also seenin the right ventricular outflow tract, and a large nodule wasseenat the right ventricular apex (Fig. 1). The pulmonary valve showedevidence of mild pulmonary hypertension. Results of thoracic computerized tomographic scanningshoweda calcified right mediastinal lymph node. There was no evidence of hilar lymphadenopathy or parenchymal lung lesions.Results of cardiac catheterization showedabnormal pressuressuggestiveof restrictive cardiomyopathy (Table I). A right ventricular angiogramin anteroposterior and lateral projections showed a large, well-defined filling defect at the right ventricular apex (Fig. 2). A left ventricular angiogram showedgood left ventricular contractility and mild mitral regurgitation. An endomyocardial biopsy specimen(Fig. 3) taken from the right ventricle showedinfiltrative tissue, which included foci of necrosis and poorly formed granulomas with occasional Langhan’s giant cells. The infiltrate was rich in mononuclear and plasmacells. The myocardium showedevidence of myocytolysis and mild edema.A few arterioles showed endothelial proliferation. No acid-fast bacillus was seen. The patient wasstarted on a regimenof isoniazid, rifampitin, and ethambutol. He becameafebrile within 1 week. Hepatosplenomegaly regressedand heart failure cleared completely within 3 weeks.He was readmitted 3 months

later. Resultsof clinical examination were normal, and the ECG showed symmetric T wave inversion in Vi-Vs. Echocardiographic findings included marked regressionof the nodules at the right ventricular apex, interatrial septum, and right ventricular outflow tract. Repeat cardiac catheterization showednormal hemodynamics(Table I). A right ventricular angiogramshowedthat the filling defect at the apical region had regressedmarkedly. Resultsof repeat endomyocardial biopsy showed interstitial and endocardial fibrosis and occasionalfoci of infiltrate but no myococytolysis-findings consistent with healed myocarditis. Tuberculous involvement of the myocardium is exceedingly rare.1*2Since the introduction of effective antitubercular drugs, the incidence has decreasedeven further.3 Three types of myocardial involvement are recognised4:(1) localized mass(tuberculoma), (2) miliary tubercles, and (3) diffuse infiltration. Thesetypes may occur in various combinations in the sameperson.The myocardium is involved either by a hematogenousroute, by lymphatic spread, or contiguously from the pericardium. Casesof myocardial tuberculosisalmostalwaysshowevidenceof tuberculosisat other sitesaswell. There is no consensuson the most common site of involvement. The right atrium,l right ventricle,2 and left ventricle3 have been found to be involved most frequently in different series.Myocardial tuberculosismay causea variety of manifestations.Rhythm disturbances include supraventricular arrhythmias,2 ventricular tachycardia! and varying degreesof heart block.6 Right ventricular outflow obstruction,‘, 2 ventricular aneurysm,3 ventricular pseudoaneurysm,* aortic insufficiency,g and coronary artery obstruction6 have alsobeen described.Diagnosisis usually madeat autopsy, although four casesof antemortem diagnosisand successfultreatment have been reported.‘-lo Kirshnaswami and CherianlO describeda caseof right atrial tuberculoma initially seenas

706

September 1990 American Heart Journal

Brief Communications

3. Endomyocardial biopsy. A, Two fragments of endomyocardium showing excess of lymphomononuclear cellsin interstitium (Hematoxylin & eosin;~44). B, Higher magnification showingepithelioid cell granuloma with Langhan’s original magnification giant cell (arrowi). Hematoxylin & eosin; original magnification X220.) Fig.

restriction to filling of the right ventricle. The diagnosiswas suspectedbased on endomyocardial biopsy findings and was confirmed only at open biopsy. Our patient initially had fever of undetermined origin with features of restrictive cardiomyopathy and varying supraventricular arrhythmias. Resultsof echocardiography showed nodular infiltration of the left atrium and right ventricle, and the diagnosiswasmade by endomyocardial biopsy. Although acid-fast bacillus could not be seen,our diagnosiswasbasedon the history of prolonged fever, calcified right mediastinal lymph node, suggestivehistopathology, and the dramatic response to antitubercular drugs.Tuberculosis of the myocardium, although rare, is a treatable causeof restrictive cardiomyopathy. The diagnosiscan be madein life by endomyocardial biopsy if clinical suspicionis strong and echocardiographicfindings are suggestive. Antitubercular drugsalone may be “curative” and in uncomplicated cases surgical intervention may not be necessary. REFERENCES

1. Anders JM. Tuberculosis of the myocardium. JAMA 1902; 39:1081-6. 2. Kapoor OP, Marcarenhas E, Rananaware MM, Gadgil RK. Tuberculoma of the heart. Report of 9 cases. AM HEART J 1973;86:334-40. 3. Rose AG. Cardiac tuberculosis. A study of 19 patients. Arch Path01 Lab Med 1987;111:422-6. 4. Horn H, Saphir 0. the involvement of the myocardium in tuberculosis. A review of the literature and report of three cases. Am Rev Respir Dis 1935;32:492-506. 5. Behr G, Palm HC, Temperley JM. Myocardial tuberculosis Br Med J 1977;951.

Kinare SG. Interesting facts of cardiovascular tuberculosis. Indian J Surg 1975;37:144-51. Rawls WJ, Shuford WH, Logan WD, Hurst JW, Schlant RC. Right ventricular outflow tract obstruction produced by a myocardial abscess in a patient with tuberculosis. Am J Cardiol 1968; 21:738-45. Halim MA, Mercer EN, Guinn GA. Myocardial tuberculoma with rupture and pseudoaneurysm formation: successful treatment. Br Heart J 1985;54:603-4. Soyer R, Brunet A, Chavalier B, Leroy J, Morere M, Redonnet M. Tuberculous aortic insufficiency. Report of a case with successful surgical treatment. J Thorac Cardiovasc Surg 1981;82:254-6. 10. Krishnaswami H, Cherian G. Right atria1 tuberculoma: report of a case with complete recovery. Thorax 1984;39: 550-l.

Diagnosis aneurysm imaging

of a false left ventricular with magnetic resonance

H. Pollak, MD, H. Mayr, MD, T. Binder, MD, H. Imhof, MD, T. Miihlbauer, MD, and D. Glogar, MD. Vienna,

Austria

From the Ludwig Boltzmann Institut fiir Herzinfarktforschung, the Department of Cardiology, and the MRI Institute, University Clinic Vienna, Austria, Medical School, and the Institute of Pathology, Krankenhaus der Stadt Wien-Lainz. Reprint requests: H. Pollak, forschung, Wolkersbergenstr. 414122051

Ludwig Boltzmann 1, Vienna, Austria

Institut A-1130.

fiir

Herzinfarkt-