- Email: [email protected]
Complete Heart Block and Severe Tricuspid Regurgitation After Radiotherapy
evaluation of the mass was consistent v.~th a large organized hematoma. Postope ratively, the patient improved. and th e abnormalities evidenced by Dopple r echocardiography resolved. The patient was late r able to recall an episode occurring 3 years hefore the onset of ascites in which he had sustained blunt chest trauma to the ante rior thorax after a fall.
DISCUSSION
This case demonstrates an unusual example of intrapericardial hematoma manifesting as constrictive pericarditis and supports the notion that traumatic hemopericardium may be unrecognized for prolonged periods of time. In one case, the patient presented 17 years after the suspected initial trauma; this same report discussed the association of a hematoma with fibrous pseudomembranes. 3 Several modalities have been used to characterize intrapericardia! masses, including CT and two-dimensional echocardiography. However, the clinical experience of using gated MRI to assess cardiac and pericardiac masses is limited. Although the capability of MRI to assess intracardiac thrombus is well documented, to our knowledge this is the first case that demonstrates MRI of an intrapericardial hematoma causing hemodynamically significant cardiac compression. Alarge organized h ematoma was suspected b efore surgical exploration, which aided in planning the operative approach. Finally, this case illustrates the complimentary use of Doppler echocardiography to define the hemodynamic effects of a pericardia! mass. Respiratmy vmiation of transvalvular flows and expiratory flow reversals in the hepatic vein suggested constrictive physiology5 which was confirmed at catheterization. In conclusion, we believe that echocardiography (imaging and Doppler) should be the initial diagnostic modality for assessing suspected pericardia! pathologic abnormalities owing to its ability to characterize structural and hemodynamic abnormalities in a cost-effective manner. In certain instances, transesophageal approaches may be necessary. CT or gated MRI are used to furth er assess pericardia! thickness and paracardiac masses; tissue boundaries are better characterized, and in some cases, the attenuation coefficients and signal intensities are diagnostic. We recommend a formal catheterization study if definitive surgical therapy is dependent upon hemodynamic findings.
REFERENCES
1 Liedtke JA, De Muth \NE. Nonpenetrating cardiac injuries: a collective review. Am Herut J 1973; 86:687-97 2 Dunlap TE, Sorkin RP, Mori K\V, et al. Massive organized intrapericardial he matoma mimicking constrictive pericarditis. Am Heart J 1982; 104:1373-75 3 Hartl WI-I , Kreuzer E, Reuschei-Janetschek E, et al. Pe1icardial mass mimicking constrictive pericarditis. Ann Thorac Surg 1991; 52:557-59 4 Wolfende n I-1, Newman DC. Constrictive pericarditis associated ~th trauma and pectus excavatum. Aust NZ J Surg 1992; 62:750-52 5 Oh JK, Hatle LK, James SB, et al. Diagnostic role o f Dopple r echocardiography in constrictive pericarditis. J Am Coil Carcliol 1994; 23:154-61
1748
Complete Heart Block and Severe Tricuspid Regurgitation After Radiotherapy* Case Report and Review of the Literature C.
f.
Knight , MA ; and G. C. Sutton , MD
Cardiac complications can occur long after chest radiotherapy. We describe a patient who developed both valve disease and complete heart block at different intervals following radiotherapy for Hodgkin's disease. The combined presentation of these two very rare cardiac complications and surgery for radiationinduced tricuspid valve disease have not been described before. (CHEST 1995; 108:1748-51) Key words: heart block; radiotherapy; valve disease with radiotherapy to the chest, particularly for T reatment Hodgl..in's disease, has resulted in increased survival of
patients , some of whom may develop complications of this treatment many years after presentation. The heart has long been considered relatively resistant to the effects of radiation although an increasing variety of cardiac complications has been described. The most common cardiac complication is involvement of the pericardium. Radiation damage to the valves and conducting system of the heart is rare and although both have been described in isolation, the case we describe is unique in that both complications have occurred in the same patient. The valvular involvement followed the heart block by a number of years, suggesting that after irradiation a sequence of cardiac complications may occur since different cardiac structures degenerate at different speeds. The case also is the first description of surgery for isolated, radiation-induced tricuspid valve disease. CASE REPORT
Th e patie nt developed a carcinoma of the •ight breast in 1974 at the age of 24 years. She was treated \vith a right mastectomy and postope rative radiothe rapy to the chest wall , right axilla, and supraclavicular fossa with an incident dose of 5,500 rad. On regular yearly revi ew, there had bee n no evidence of recurre nce. In 1983, a diagnos is of hypothyroidism was made, and she was treated \vith thyroxin e, 100 pgld. An ECG performed in 1984 was normal. In October 1986, 12 years after radioth e rapy, she was admitted to the hospital \vith episodes of syncope. She was found to be in complete heart block (Fig 1). Cardiovascular examination was within normal lim its, as was an echocardiogram (but ~thout Doppler). On September 10, 1986, a single chambe r (v entricular) permane nt pacemake r was implanted. Atrial pacing was attempted but could not b e obtained. No furth e r investigations were carried out to dete rmine the cause of heart block. Follo~ngthis procedme, she remained well. She did not present lor cardiologic follow-up until Decembe r 1990. DUling the early months of 1990. she developed dvspn ea and was *From Ilillingdon Hospital , Uxb1idge, Middlesex, United Kingdom. Selected Reports
o L
The patient experienced only minimal improvement in her symptoms in the months following the operation and still required considerable diuretic support. Although she still had tricuspid regurgitation, it was thought that a physiologic pacing mode might make a significant difference to her hemodynamic state. In June 1992, she was successfully changed from the single to a dual chamber pacemaker mode. There was a marked clinical improvement following this, with clearance of peripheral edema and a marked reduction in heart size seen on the chest x-ray film (Fig 3). A second echocardiogram showed normal left ventricular function, a normal-sized right ventricle, and no evidence of tricuspid regurgitation. The patient is currently well and able to lead an active life, taking only 80 mg of furosemide a day.
FIGUHE 1. ECG showing complete heart block.
admitted 4 times to another hospital with heart failure. Her chest x-ray film showed cardiomegaly. A CT scan of her thorax showed evidence of pericardia! thickening. Echocardiography (July 1990) showed good left ventricular systolic function with trivial aortic and mitral regurgitation. In December 1990, she presented to this hospital, for the first occasion after pacemaker implantation, with abdominal distension. Examination revealed a grossly elevated jugular venous pressure with a dominant systolic wave and steep "y" descent (Fig 2). There also was a midsystolic murmur at the left sternal edge, hepatomegaly, and ascites. Doppler echocardiography was performed. There was Doppler evidence of severe tricuspid regurgitation with a very active right ventricle and reversed septal movement. Apart from trivial mitral regurgitation, no left-sided abnormality could be demonstrated. It was concluded that the hemodynamic picture was dominated by severe tricuspid regurgitation rather than by pericardia! constriction or restrictive cardiomyopathy. An attempt was made to manage her medically, awaiting an operation on the tricuspid valve. The patient remained stable on a regimen oflarge doses of diuretics until July 1991 when she developed worsening fluid retention. She was therefore referred for surgical correction of her tricuspid valve lesion. In February 1992, she had a De Vega tricuspid annuloplasty and limited pericardiectomy. At the time of operation, thickened, non rheumatic tricuspid valve leaflets were observed. The valve cusps were white and shrunken. A biopsy of the right atrial wall showed no evidence of radiation damage to the endomyocardium. There was some epicardial thickening, possibly related to radiation.
FIGUHF: 2. Recording of jugular venous pulse showing dominant systolic wave and steep "y" descent.
FIGURE 3. Chest x-ray film before (top) and after (bottom) dual chamber pacing showing reduction in heart size. CHEST / 108 / 6 / DECEMBER, 1995
1749
DISCUSSION
Radiation in significant doses can affect any part of the heart, probably as a result of microcirculatory injury causing ischemia and progressive fibrosis. 1 Pericardia! effusion, acute pericarditis, and constrictive pericarditis are the most common manifestations of radiation-induced heart disease, although cardiomyopathy and coronary artery stenoses are well recognized. Radiation-associated valve disease and conducting-tissue disease are both much rarer. There seems to be a spectrum of valve involvement from asymptomatic valvular thickening to symptomatic valvular dysfunction. In one necropsy series of 16 patients, 4 to 5 years after radiotherapy, 2 80% had valvular endothelial thickening, but none had had clinical evidence of valve dysfunction. In a review of radiation-associated valve disease, Carlson et al3 identified 38 patients described in the world medical literature. Of these, 66% were asymptomatic. These patients were first seen on an average of 11.5 years afi:er their radiotherapy, as compared with the 13 symptomatic patients who were seen on an average of 16.5 years after radiotherapy. It was postulated that valvular thickening is the early manifestation of a radiation-induced degenerative process which results in valve dysfunction in some patients after a number of years. In this review, 93% of valve lesions were left-sided and predominantly regurgitant. There was one case of tricuspid regurgitation in a symptomatic patient with coexisting aortic and mitral disease. There was no patient with a predominantly right-sided abnormality. An echocardiographic review of 25 patients 10 to 20 years after radiotherapy for Hodgkin's disease4 showed Doppler evidence of tricuspid regurgitation in 88%. This was mild in all but one case when moderate tricuspid regurgitation and enlargement of the right ventricle were demonstrated. None of the patients was symptomatic. The presence of Doppler evidence of mild tricuspid regurgitation may be of little pathologic significance as a regurgitant flow pattern may be found across the tricuspid valve in normal subjects. An incidence of 78% has been reported. 5 Ten patients in the literature have re~uired surgery for valve defects associated \vith radiation. 4 • All these operations involved the aortic or mitral valves or both. In one case, a tricuspid valve repair was performed together with mitral valve replacement. 6 No case has been reported before involving surgery for an isolated right-sided lesion. Transient complete heart block following radiation treatment for thyrotoxicosis was first described by Kremer and Laplace 7 in 1936, but atrioventricular block requiring pacemaker implantation following radiotherapy remains rare. Pathologic studies have shown marked fibrosis of the atrioventricular node and arteriolar narrowing. 8 A review of the literature provides details of 30 cases (including the current one) in which symptomatic atrioventricular block has occurred at an interval after thoracic radiotherapy.6·8-26 Details of age of the patient and interval after radiotherapy are available in 27: the patients were aged between 25 and 73 years at the onset of atrioventricular block with a mean age of 42 years. The interval between treatment and the development of atrioventricular block varied from l month to 23 years with a mean of 12 years. Most cases are clustered between 10 and 14 years. Hodgkin's disease was the original pathologic finding in 15 patients (56%). In only 1750
one other case was heart block associated with the treatment of carcinoma of the right breast.12 There was associated pericardia! disease in ten patients, and right ventricular outflow tract stenosis was seen in 2.1 9 ·24 None of these papers reported coexistent valvular dysfunction. In summary, we have presented a patient in whom two extremely rare complications of thoracic radiotherapy have occurred together. The latency betv•een treatment and complication appears typical from review of the limited number of reported cases. In patients that have survived for long periods after radiotherapy to the chest, it is important to remember that cardiac complications can occur. ACKNOWLEDGMENTS: We would like to thank Dr. D. Gibson for his help with the diagnostic studies and management of this patient and Professor Sir M. Yacoub who carried out the surgery. REFERENCES
Stewart JR, Fajardo LF. Radiation-induced heart disease: an update . Prog Cardiovasc Dis 1984; 27:173-94 2 Brosius FC, Waller BF, Roberts WC. Radiation heart disease. Am J Med 1981; 70:519-30 3 Carlson RG, Mavfield WR, Normann S, eta!. H.adiation associated valvular dis~ase. Chest 1991; 99:539-45 4 Gustavasson A, Eskilsson J, Landberg T, e ta!. Late cardiac effects after mantle radiotherapy in patients with Hodgkin's disease. Ann Oncol 1990; 1:355-63 5 Suzuki Y, Kambara H, Kadota K, eta!. Detection and evaluation of tricuspid regurgitation using real-time, two-dimensional, colour coded, doppler flow imaging system: comparison with contrast two-dimensional echocardiography and right ventriculography. Am J Cardiol1986; 57:811-15 6 Orzan F, Brusca A, Conte MH., et a!. Severe coronary artery disease after radiation therapy of the chest and mediastinum: clinical presentation and treatment. Br Heart J 1993; 69:496-500 7 Kremer DM, Laplace LB. Heart block following x-ray treatment for thyrotoxicosis. Am Heart J 1936; 11:227 8 Cohen SI, Bharati S, Glass J, et al. H.adiotherapy as a cause of c.'Omplete atrioventricular block in Hodgkin's disease: an electrophysiological-pathological correlation. Arch Intern Med 1981; 141:676-79 9 Rubin E, Camara J, Grayzel DM, et al. H.adiation induced cardiac fibrosis. Am J Med 1963; 34:71-5 lO Tzivoni D, Ratzkowski E, Biran S, et a!. Complete heart block following therapeutic irradiation of the left side of the chest. Chest 1977; 71:231-34 11 Kereiakes DJ, Morady F, Ports TA. High-degree atrioventricular block after radiation therapy. Am J Cardiol1983; 51:1233-34 12 Patri B, Patri N. Bloc auriculo-ventriculaire succedant a Ia radiotherapie thoracique: a propos de deux cas. Sem Hop Paris 1977; 53:2141-42 13 Nakao T, Kanaya H, Namura M, et al. Complete atrioventricular block following radiation therapy for malignant thymoma. Japan J Med 1990; 29:104-10 14 Nishizawa T, Suzuki I, Kobayashi J. A case report of complete atrioventricular block following therapeutic irradiation for breast cancer. Rinsyo-Geka 1987; 42:1993 15 Ali MK, Khalil KG, Fuller LM. Radiation-related myocardial injury: management of two cases. Cancer 1976; 38:1941 16 Ladero-Quesada JM, Amarillas-Sanchez L, Furio-Bacete V, eta!. Peritoneal mesothelioma and complete auriculo-ventricular block as late complications in Hodgkin's disease. Rev Clin Esp 1991; 189:221-23 17 Slama MS, Le Guludec D, Sebag C, eta!. Complete atrioventricular block following mediastinal irradiation: a report of six cases. Pacing Clin Electrophysiol1991; 14:1112-18 Selected Reports
18 Borre! E, Wolf JE, Page E, et al. A rare complication of thoracic radiotherapy: auriculoventricular block. Ann Cardiol Angeiol Paris 1990; 39:351-55 19 Ebagosti A, Gueunoun M, Favre R, et al. Atrioventricular block, a complication of radiotherapy of the mediastinum. Arch Mal Coeur Vaiss 1989; 82:935-39 20 Thombansen E, Menges M, Thimme W. Glossopharyngeal neuralgia with syncope. Dtsch Med Wochenschr 1992; 117:941-44 21 Prez M, Degheldere CH. Une observation de bloc auriculo-ventriculaire post radique. Stimucoeur 1988; 15:269-70 22 Mary-Rabine L, Waleffe A, Kulbertus ME. Severe conduction disturbances and ventricular arrhythmias complicating mediastinal irradiation for Hodgkin's disease: a case report. Pacing Clin Electrophysiol 1980; 3:612-17 23 Fischbein L, Valtier B, Sachs RN, et al. Bloc auriculo-ventriculaire post radique. Arch Mal Coeur 1987; 12:1823-25 24 Velebit V, von Segesser L, Gabathuler J, e t al . Right ventricular outflow tract obstruction after radiation therapy. J Thorac Cardiovasc Surg 1986; 92:153-55 25 Baylac-Domengetroy F, Gallimard JF, Elbaz-Rostykus C, et al. Complications rythmologiques de Ia radiotherapie: a propos d'un cas. Ann Cardiol Angeiol 1989; 38:219-23 26 Lamaud M, Duranton B, Reymond S, et al. Bloc auriculoventriculaire complet apres radiotherapie. Lyon Med 1985; 253: 335-37
Digital Clubbing Associated With Polymyositis and Interstitial Lung Disease* Capt Kurt W. Grathwohl, MC, USA; Capt James W. Thompson, MC, USA; Maj Kathym K Riordan, MC, USA; Maj Bemard]. Roth, MC, USA, FCCP; and Lt Col Thonws A. Dillard, MC, USA, FCCP
Clubbing of the fmgers is commonly associated with interstitial lung diseases (ILDs). Although ILD occurs in as many as 40% of patients with polymyositis/ dermatomyositis (PMIDM), clubbing of the digits has never been reported to occur in patients with PM/DM and ILD. We report the first case of clubbing associated with PM/DM and ILD. (CHEST 1995; 108:1751-52)
ILD =interstitial lung disease; PM/D M =polymyositis/dermatomyositis
Key words: bronchoalveolar lavage; clubbing; dermatomyositis; interstitial lung disease; polymyositis *From the Pulmonary/Critical Care Service (Drs. Grathwohl, Thompson, Roth, and Dillard) and Rheumatology Service (Dr. Riordan), Madigan Army Medical Center, Tacoma, Wash. The opinions or assertions herein are the private views of the authors and are not to be construed as reflecting the views of the Department of Defense.
FIGUHE l. Finger clubbing with PM/DM associated with ILD. Objective findings of clubbing-5 include increased hyponychial angles and phalangeal depth ratios. The hyponychial angle is formed by a line connecting the dorsal surface of the distal interphalangeal joint and the cuticle and a line joining the cuticle and the hyponychium (normal, <195 degrees). The phalangeal depth ratio consists of the thickness of the fingertip at the cuticle divided by the thickness of the finger at the distal inte1phalangeal joint (nom1al, <1.0).
and idiopathic inflammatory myopathy are Polymyositis very uncommon with 5 to 10 new cases per million per-
sons per year in the Urn ted States. 1 However, interstitial lung disease (ILD) occurs in as many as 40% of patients with polymyositis/dermatomyositis (PM/DM). 2 ILDs are frequently associated witl1 tl1e physical finding of digital clubbing.3 Despite tl1e significant association of ILD witl1 polymyositis, digital clubbing has never been reported to occur in association with polymyositis. 4 We report tl1e first case of digital clubbing in association witl1 polymyositis and ILD. CASE REPORT
A 33-year-old black woman presented to Madigan Army Medical Center for evaluation of persistent shortness of breath associated with an established diagnosis of PM/DM and ILD. Physical examination was notable for the new finding of prominent clubbing of the fingers (Fig 1) and toes in addition to bibasilar crackles which had been noted previously. The physical examination was otherwise unremarkable, and the patient had no rash or lymphadenopathy. Muscle examination revealed normal bulk and strength bilaterally without tenderness or fatigability. Her medical history disclosed sickle cell trait, uterine fibroid tumors treated with Ieuprolide acetate, and removal of a cyst in Bartholin's gland. She denied alcohol use or medications other than oral prednisone. She had smoked one pack of cigarettes a week for 10 years but quit at the onset of her symptoms. The patient had worked as a dental assistant but denied any significant exposures. Her present illness dated to 14 months earlier when she presented with a history of progressive dyspnea, fever, and nonproductive cough. Despite treatment 'vith oral antibiotics, her symptoms worsened, prompting the physician to order a chest x-ray film that demonstrated bilateral basilar interstitial infiltrates. She was hospitalized and treated with intravenous antibiotics for presumed atypical pneumonia. Absence of clubbing was noted at that time by three observers. Further evaluation was done when her condition failed to improve. Laboratory examination included a normal CBC count with erythrocyte sedimentation rate of 48 mm/h and normal urinalysis, screening electrolyte levels, thyroid function , and complement CHEST I 108 I 6 I DECEMBER, 1995
1751
DISCUSSION
This case demonstrates an unusual example of intrapericardial hematoma manifesting as constrictive pericarditis and supports the notion that traumatic hemopericardium may be unrecognized for prolonged periods of time. In one case, the patient presented 17 years after the suspected initial trauma; this same report discussed the association of a hematoma with fibrous pseudomembranes. 3 Several modalities have been used to characterize intrapericardia! masses, including CT and two-dimensional echocardiography. However, the clinical experience of using gated MRI to assess cardiac and pericardiac masses is limited. Although the capability of MRI to assess intracardiac thrombus is well documented, to our knowledge this is the first case that demonstrates MRI of an intrapericardial hematoma causing hemodynamically significant cardiac compression. Alarge organized h ematoma was suspected b efore surgical exploration, which aided in planning the operative approach. Finally, this case illustrates the complimentary use of Doppler echocardiography to define the hemodynamic effects of a pericardia! mass. Respiratmy vmiation of transvalvular flows and expiratory flow reversals in the hepatic vein suggested constrictive physiology5 which was confirmed at catheterization. In conclusion, we believe that echocardiography (imaging and Doppler) should be the initial diagnostic modality for assessing suspected pericardia! pathologic abnormalities owing to its ability to characterize structural and hemodynamic abnormalities in a cost-effective manner. In certain instances, transesophageal approaches may be necessary. CT or gated MRI are used to furth er assess pericardia! thickness and paracardiac masses; tissue boundaries are better characterized, and in some cases, the attenuation coefficients and signal intensities are diagnostic. We recommend a formal catheterization study if definitive surgical therapy is dependent upon hemodynamic findings.
REFERENCES
1 Liedtke JA, De Muth \NE. Nonpenetrating cardiac injuries: a collective review. Am Herut J 1973; 86:687-97 2 Dunlap TE, Sorkin RP, Mori K\V, et al. Massive organized intrapericardial he matoma mimicking constrictive pericarditis. Am Heart J 1982; 104:1373-75 3 Hartl WI-I , Kreuzer E, Reuschei-Janetschek E, et al. Pe1icardial mass mimicking constrictive pericarditis. Ann Thorac Surg 1991; 52:557-59 4 Wolfende n I-1, Newman DC. Constrictive pericarditis associated ~th trauma and pectus excavatum. Aust NZ J Surg 1992; 62:750-52 5 Oh JK, Hatle LK, James SB, et al. Diagnostic role o f Dopple r echocardiography in constrictive pericarditis. J Am Coil Carcliol 1994; 23:154-61
1748
Complete Heart Block and Severe Tricuspid Regurgitation After Radiotherapy* Case Report and Review of the Literature C.
f.
Knight , MA ; and G. C. Sutton , MD
Cardiac complications can occur long after chest radiotherapy. We describe a patient who developed both valve disease and complete heart block at different intervals following radiotherapy for Hodgkin's disease. The combined presentation of these two very rare cardiac complications and surgery for radiationinduced tricuspid valve disease have not been described before. (CHEST 1995; 108:1748-51) Key words: heart block; radiotherapy; valve disease with radiotherapy to the chest, particularly for T reatment Hodgl..in's disease, has resulted in increased survival of
patients , some of whom may develop complications of this treatment many years after presentation. The heart has long been considered relatively resistant to the effects of radiation although an increasing variety of cardiac complications has been described. The most common cardiac complication is involvement of the pericardium. Radiation damage to the valves and conducting system of the heart is rare and although both have been described in isolation, the case we describe is unique in that both complications have occurred in the same patient. The valvular involvement followed the heart block by a number of years, suggesting that after irradiation a sequence of cardiac complications may occur since different cardiac structures degenerate at different speeds. The case also is the first description of surgery for isolated, radiation-induced tricuspid valve disease. CASE REPORT
Th e patie nt developed a carcinoma of the •ight breast in 1974 at the age of 24 years. She was treated \vith a right mastectomy and postope rative radiothe rapy to the chest wall , right axilla, and supraclavicular fossa with an incident dose of 5,500 rad. On regular yearly revi ew, there had bee n no evidence of recurre nce. In 1983, a diagnos is of hypothyroidism was made, and she was treated \vith thyroxin e, 100 pgld. An ECG performed in 1984 was normal. In October 1986, 12 years after radioth e rapy, she was admitted to the hospital \vith episodes of syncope. She was found to be in complete heart block (Fig 1). Cardiovascular examination was within normal lim its, as was an echocardiogram (but ~thout Doppler). On September 10, 1986, a single chambe r (v entricular) permane nt pacemake r was implanted. Atrial pacing was attempted but could not b e obtained. No furth e r investigations were carried out to dete rmine the cause of heart block. Follo~ngthis procedme, she remained well. She did not present lor cardiologic follow-up until Decembe r 1990. DUling the early months of 1990. she developed dvspn ea and was *From Ilillingdon Hospital , Uxb1idge, Middlesex, United Kingdom. Selected Reports
o L
The patient experienced only minimal improvement in her symptoms in the months following the operation and still required considerable diuretic support. Although she still had tricuspid regurgitation, it was thought that a physiologic pacing mode might make a significant difference to her hemodynamic state. In June 1992, she was successfully changed from the single to a dual chamber pacemaker mode. There was a marked clinical improvement following this, with clearance of peripheral edema and a marked reduction in heart size seen on the chest x-ray film (Fig 3). A second echocardiogram showed normal left ventricular function, a normal-sized right ventricle, and no evidence of tricuspid regurgitation. The patient is currently well and able to lead an active life, taking only 80 mg of furosemide a day.
FIGUHE 1. ECG showing complete heart block.
admitted 4 times to another hospital with heart failure. Her chest x-ray film showed cardiomegaly. A CT scan of her thorax showed evidence of pericardia! thickening. Echocardiography (July 1990) showed good left ventricular systolic function with trivial aortic and mitral regurgitation. In December 1990, she presented to this hospital, for the first occasion after pacemaker implantation, with abdominal distension. Examination revealed a grossly elevated jugular venous pressure with a dominant systolic wave and steep "y" descent (Fig 2). There also was a midsystolic murmur at the left sternal edge, hepatomegaly, and ascites. Doppler echocardiography was performed. There was Doppler evidence of severe tricuspid regurgitation with a very active right ventricle and reversed septal movement. Apart from trivial mitral regurgitation, no left-sided abnormality could be demonstrated. It was concluded that the hemodynamic picture was dominated by severe tricuspid regurgitation rather than by pericardia! constriction or restrictive cardiomyopathy. An attempt was made to manage her medically, awaiting an operation on the tricuspid valve. The patient remained stable on a regimen oflarge doses of diuretics until July 1991 when she developed worsening fluid retention. She was therefore referred for surgical correction of her tricuspid valve lesion. In February 1992, she had a De Vega tricuspid annuloplasty and limited pericardiectomy. At the time of operation, thickened, non rheumatic tricuspid valve leaflets were observed. The valve cusps were white and shrunken. A biopsy of the right atrial wall showed no evidence of radiation damage to the endomyocardium. There was some epicardial thickening, possibly related to radiation.
FIGUHF: 2. Recording of jugular venous pulse showing dominant systolic wave and steep "y" descent.
FIGURE 3. Chest x-ray film before (top) and after (bottom) dual chamber pacing showing reduction in heart size. CHEST / 108 / 6 / DECEMBER, 1995
1749
DISCUSSION
Radiation in significant doses can affect any part of the heart, probably as a result of microcirculatory injury causing ischemia and progressive fibrosis. 1 Pericardia! effusion, acute pericarditis, and constrictive pericarditis are the most common manifestations of radiation-induced heart disease, although cardiomyopathy and coronary artery stenoses are well recognized. Radiation-associated valve disease and conducting-tissue disease are both much rarer. There seems to be a spectrum of valve involvement from asymptomatic valvular thickening to symptomatic valvular dysfunction. In one necropsy series of 16 patients, 4 to 5 years after radiotherapy, 2 80% had valvular endothelial thickening, but none had had clinical evidence of valve dysfunction. In a review of radiation-associated valve disease, Carlson et al3 identified 38 patients described in the world medical literature. Of these, 66% were asymptomatic. These patients were first seen on an average of 11.5 years afi:er their radiotherapy, as compared with the 13 symptomatic patients who were seen on an average of 16.5 years after radiotherapy. It was postulated that valvular thickening is the early manifestation of a radiation-induced degenerative process which results in valve dysfunction in some patients after a number of years. In this review, 93% of valve lesions were left-sided and predominantly regurgitant. There was one case of tricuspid regurgitation in a symptomatic patient with coexisting aortic and mitral disease. There was no patient with a predominantly right-sided abnormality. An echocardiographic review of 25 patients 10 to 20 years after radiotherapy for Hodgkin's disease4 showed Doppler evidence of tricuspid regurgitation in 88%. This was mild in all but one case when moderate tricuspid regurgitation and enlargement of the right ventricle were demonstrated. None of the patients was symptomatic. The presence of Doppler evidence of mild tricuspid regurgitation may be of little pathologic significance as a regurgitant flow pattern may be found across the tricuspid valve in normal subjects. An incidence of 78% has been reported. 5 Ten patients in the literature have re~uired surgery for valve defects associated \vith radiation. 4 • All these operations involved the aortic or mitral valves or both. In one case, a tricuspid valve repair was performed together with mitral valve replacement. 6 No case has been reported before involving surgery for an isolated right-sided lesion. Transient complete heart block following radiation treatment for thyrotoxicosis was first described by Kremer and Laplace 7 in 1936, but atrioventricular block requiring pacemaker implantation following radiotherapy remains rare. Pathologic studies have shown marked fibrosis of the atrioventricular node and arteriolar narrowing. 8 A review of the literature provides details of 30 cases (including the current one) in which symptomatic atrioventricular block has occurred at an interval after thoracic radiotherapy.6·8-26 Details of age of the patient and interval after radiotherapy are available in 27: the patients were aged between 25 and 73 years at the onset of atrioventricular block with a mean age of 42 years. The interval between treatment and the development of atrioventricular block varied from l month to 23 years with a mean of 12 years. Most cases are clustered between 10 and 14 years. Hodgkin's disease was the original pathologic finding in 15 patients (56%). In only 1750
one other case was heart block associated with the treatment of carcinoma of the right breast.12 There was associated pericardia! disease in ten patients, and right ventricular outflow tract stenosis was seen in 2.1 9 ·24 None of these papers reported coexistent valvular dysfunction. In summary, we have presented a patient in whom two extremely rare complications of thoracic radiotherapy have occurred together. The latency betv•een treatment and complication appears typical from review of the limited number of reported cases. In patients that have survived for long periods after radiotherapy to the chest, it is important to remember that cardiac complications can occur. ACKNOWLEDGMENTS: We would like to thank Dr. D. Gibson for his help with the diagnostic studies and management of this patient and Professor Sir M. Yacoub who carried out the surgery. REFERENCES
Stewart JR, Fajardo LF. Radiation-induced heart disease: an update . Prog Cardiovasc Dis 1984; 27:173-94 2 Brosius FC, Waller BF, Roberts WC. Radiation heart disease. Am J Med 1981; 70:519-30 3 Carlson RG, Mavfield WR, Normann S, eta!. H.adiation associated valvular dis~ase. Chest 1991; 99:539-45 4 Gustavasson A, Eskilsson J, Landberg T, e ta!. Late cardiac effects after mantle radiotherapy in patients with Hodgkin's disease. Ann Oncol 1990; 1:355-63 5 Suzuki Y, Kambara H, Kadota K, eta!. Detection and evaluation of tricuspid regurgitation using real-time, two-dimensional, colour coded, doppler flow imaging system: comparison with contrast two-dimensional echocardiography and right ventriculography. Am J Cardiol1986; 57:811-15 6 Orzan F, Brusca A, Conte MH., et a!. Severe coronary artery disease after radiation therapy of the chest and mediastinum: clinical presentation and treatment. Br Heart J 1993; 69:496-500 7 Kremer DM, Laplace LB. Heart block following x-ray treatment for thyrotoxicosis. Am Heart J 1936; 11:227 8 Cohen SI, Bharati S, Glass J, et al. H.adiotherapy as a cause of c.'Omplete atrioventricular block in Hodgkin's disease: an electrophysiological-pathological correlation. Arch Intern Med 1981; 141:676-79 9 Rubin E, Camara J, Grayzel DM, et al. H.adiation induced cardiac fibrosis. Am J Med 1963; 34:71-5 lO Tzivoni D, Ratzkowski E, Biran S, et a!. Complete heart block following therapeutic irradiation of the left side of the chest. Chest 1977; 71:231-34 11 Kereiakes DJ, Morady F, Ports TA. High-degree atrioventricular block after radiation therapy. Am J Cardiol1983; 51:1233-34 12 Patri B, Patri N. Bloc auriculo-ventriculaire succedant a Ia radiotherapie thoracique: a propos de deux cas. Sem Hop Paris 1977; 53:2141-42 13 Nakao T, Kanaya H, Namura M, et al. Complete atrioventricular block following radiation therapy for malignant thymoma. Japan J Med 1990; 29:104-10 14 Nishizawa T, Suzuki I, Kobayashi J. A case report of complete atrioventricular block following therapeutic irradiation for breast cancer. Rinsyo-Geka 1987; 42:1993 15 Ali MK, Khalil KG, Fuller LM. Radiation-related myocardial injury: management of two cases. Cancer 1976; 38:1941 16 Ladero-Quesada JM, Amarillas-Sanchez L, Furio-Bacete V, eta!. Peritoneal mesothelioma and complete auriculo-ventricular block as late complications in Hodgkin's disease. Rev Clin Esp 1991; 189:221-23 17 Slama MS, Le Guludec D, Sebag C, eta!. Complete atrioventricular block following mediastinal irradiation: a report of six cases. Pacing Clin Electrophysiol1991; 14:1112-18 Selected Reports
18 Borre! E, Wolf JE, Page E, et al. A rare complication of thoracic radiotherapy: auriculoventricular block. Ann Cardiol Angeiol Paris 1990; 39:351-55 19 Ebagosti A, Gueunoun M, Favre R, et al. Atrioventricular block, a complication of radiotherapy of the mediastinum. Arch Mal Coeur Vaiss 1989; 82:935-39 20 Thombansen E, Menges M, Thimme W. Glossopharyngeal neuralgia with syncope. Dtsch Med Wochenschr 1992; 117:941-44 21 Prez M, Degheldere CH. Une observation de bloc auriculo-ventriculaire post radique. Stimucoeur 1988; 15:269-70 22 Mary-Rabine L, Waleffe A, Kulbertus ME. Severe conduction disturbances and ventricular arrhythmias complicating mediastinal irradiation for Hodgkin's disease: a case report. Pacing Clin Electrophysiol 1980; 3:612-17 23 Fischbein L, Valtier B, Sachs RN, et al. Bloc auriculo-ventriculaire post radique. Arch Mal Coeur 1987; 12:1823-25 24 Velebit V, von Segesser L, Gabathuler J, e t al . Right ventricular outflow tract obstruction after radiation therapy. J Thorac Cardiovasc Surg 1986; 92:153-55 25 Baylac-Domengetroy F, Gallimard JF, Elbaz-Rostykus C, et al. Complications rythmologiques de Ia radiotherapie: a propos d'un cas. Ann Cardiol Angeiol 1989; 38:219-23 26 Lamaud M, Duranton B, Reymond S, et al. Bloc auriculoventriculaire complet apres radiotherapie. Lyon Med 1985; 253: 335-37
Digital Clubbing Associated With Polymyositis and Interstitial Lung Disease* Capt Kurt W. Grathwohl, MC, USA; Capt James W. Thompson, MC, USA; Maj Kathym K Riordan, MC, USA; Maj Bemard]. Roth, MC, USA, FCCP; and Lt Col Thonws A. Dillard, MC, USA, FCCP
Clubbing of the fmgers is commonly associated with interstitial lung diseases (ILDs). Although ILD occurs in as many as 40% of patients with polymyositis/ dermatomyositis (PMIDM), clubbing of the digits has never been reported to occur in patients with PM/DM and ILD. We report the first case of clubbing associated with PM/DM and ILD. (CHEST 1995; 108:1751-52)
ILD =interstitial lung disease; PM/D M =polymyositis/dermatomyositis
Key words: bronchoalveolar lavage; clubbing; dermatomyositis; interstitial lung disease; polymyositis *From the Pulmonary/Critical Care Service (Drs. Grathwohl, Thompson, Roth, and Dillard) and Rheumatology Service (Dr. Riordan), Madigan Army Medical Center, Tacoma, Wash. The opinions or assertions herein are the private views of the authors and are not to be construed as reflecting the views of the Department of Defense.
FIGUHE l. Finger clubbing with PM/DM associated with ILD. Objective findings of clubbing-5 include increased hyponychial angles and phalangeal depth ratios. The hyponychial angle is formed by a line connecting the dorsal surface of the distal interphalangeal joint and the cuticle and a line joining the cuticle and the hyponychium (normal, <195 degrees). The phalangeal depth ratio consists of the thickness of the fingertip at the cuticle divided by the thickness of the finger at the distal inte1phalangeal joint (nom1al, <1.0).
and idiopathic inflammatory myopathy are Polymyositis very uncommon with 5 to 10 new cases per million per-
sons per year in the Urn ted States. 1 However, interstitial lung disease (ILD) occurs in as many as 40% of patients with polymyositis/dermatomyositis (PM/DM). 2 ILDs are frequently associated witl1 tl1e physical finding of digital clubbing.3 Despite tl1e significant association of ILD witl1 polymyositis, digital clubbing has never been reported to occur in association with polymyositis. 4 We report tl1e first case of digital clubbing in association witl1 polymyositis and ILD. CASE REPORT
A 33-year-old black woman presented to Madigan Army Medical Center for evaluation of persistent shortness of breath associated with an established diagnosis of PM/DM and ILD. Physical examination was notable for the new finding of prominent clubbing of the fingers (Fig 1) and toes in addition to bibasilar crackles which had been noted previously. The physical examination was otherwise unremarkable, and the patient had no rash or lymphadenopathy. Muscle examination revealed normal bulk and strength bilaterally without tenderness or fatigability. Her medical history disclosed sickle cell trait, uterine fibroid tumors treated with Ieuprolide acetate, and removal of a cyst in Bartholin's gland. She denied alcohol use or medications other than oral prednisone. She had smoked one pack of cigarettes a week for 10 years but quit at the onset of her symptoms. The patient had worked as a dental assistant but denied any significant exposures. Her present illness dated to 14 months earlier when she presented with a history of progressive dyspnea, fever, and nonproductive cough. Despite treatment 'vith oral antibiotics, her symptoms worsened, prompting the physician to order a chest x-ray film that demonstrated bilateral basilar interstitial infiltrates. She was hospitalized and treated with intravenous antibiotics for presumed atypical pneumonia. Absence of clubbing was noted at that time by three observers. Further evaluation was done when her condition failed to improve. Laboratory examination included a normal CBC count with erythrocyte sedimentation rate of 48 mm/h and normal urinalysis, screening electrolyte levels, thyroid function , and complement CHEST I 108 I 6 I DECEMBER, 1995
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