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Unilateral Neck Pulsations in a Patient with a Permanent Pacemaker
An asymptomatic patient with a permanent pacemaker presented with undateral ac
I FIGURE 3. 'IWenty-seven clays later, the anterior mediastinal mass had recurred. The bilateral pleural effusions had increased in size. cell count between the time of initial evaluation of the lymphoma mass and the time of disappearance of the mass. This presumably was associated with heightened activity of the immune system which could have resulted in increased host immune response against the tumor. Surgery at a site other than a given tumor mass has been associated with spontaneous regression of the mass.1 This proposed mechanism could also have played a role in the case presented in this report. Weingarten et al1 recently published a series describing fOur patients who had transient spontaneous regression of intracerebral lymphoma. Their paper stressed that spontaneous regression of an intracerebral neoplasm should not be construed as a good prognostic sign nor as an indication of benignancy. It appears that the same admonitions apply to transient spontaneous regression of mediastinal lymphoblastic lymphoma. This disease carries a dismal prognosis, and transient spontaneous regression of a mediastinal lesion cannot be viewed as a reliable favorable indicator. REFERENCES 1 Cole WH. "Spontaneous regression of cancer: the metabolic triumph of the host? Ann NY Acad Sci 1974; 230:111-41 2 Gattkier HH, Wiltshaw E, Galton DAG. Spontaneous regression in non-Hodgkins lymphoma. Cancer 1980; 45:2627-32 3 Krikorian JG, Portlock CS, Cooney DP, Rosenberg SA. Spontaneous regression of non-Hodgkin's lymphoma: a report of nine cases. Cancer, 1980; 46:2093-99 4 Rees GLC. Abscopal regression in lymphoma: a mechanism in common with total body irradiation? Clio Radio 1981; 32:475-80 5 Weingarten KL, Zimmerman RD, Leeds NE. Spontaneous regression of intercerebral lymphoma. Radiology 1983; 149:721-24
·Unilateral Neck Pulsations Patient with a Permanent Pacemaker*
In a
Nell L. Coplan, M.D.; John A. AmbroH, M.D.; and johnothon L. Halperin , M.D .
•From the Division ofCardiolottv, Department of Medicine, Mount Sinai Medical Center, New York.
Reprint requem: Dr. Coplan, Annmberg 8-ZOO, One Gwtave L.
Levy Place, New York 10029
nternal jugular vein occlusion, especially in combination with tricuspid regurgitation, may result in asymmetrical neck pulsations. The fOllowing patient demonstrates the importance of considering these factors in difterential diagnosis. CASE REPORr
A 64-ye&M>ld man bad pulsations of the right side of the neck, which he bad noticed about tOur months earlier. He denied trauma, local dUcomtOrt, or neurologic symptoms. The pulsations were continuous and increasingly prominent. Four years previously, he sustained an inferior wall myocardial infarction complicated by complete heart block, and temporary pacing was achieved by right subclavian venipuncture. A permanent ventricular demand (VVI-mode) pacemaker was then installed via the left subclavian vein because of penistent high grade atrioventricular block. · Physical eumination revealed obvious pulsation of the right side of the neck. The heart rate was regular at 52 beats per minute; blood pressure was 140180 mm Hg In each arm, without pulsus paradorus. Discontinuous scratchy sounds were heard in both systole and diastole over the pulsatile area. A strong systolic wave could be palpated beneath the right sternocleidomastoid muscle; no discrete mass or lymphadenopathy was appreciated. The left side of the neck bad no acessive pulsatile waves. The left carotid pulse was normal, but the right carotid pulse could not be readily distingl,lished &om the diffi.ue pulsation. The external jugular veins were distended to the level ofthe mandibuJar angle bilaterally with the patient upright The chest was clear. There was no displacement of the apical impulse of the heart, and no precordial lift or thrill was fell Auscultation revealed a grade 216 coarse left parasternal systolic murmur without radiation, along with grade J/6 short high-pitched left parasternal decrescendo diastolic murmur. These did not change with Valsalva strain, standing, or squatting, but the systolic murmur intensified during inlpiration. No third or fOurth heart sound was heard. The abdomen displayed fAint hepatic pulsations, and minimal hepatomegaly. The venous pressure was elevated in both upper atremities, but no distended collateral venous pattern was apparent over the thorax, abdomen, or lower extremities. Edema was absenl Chest roentgenograms showed mild cardiomegaly, with prominence of the right atrial border and the right ventricle; the pacemaker generator was seen in the left pectorali.Ossa, with Its electrode terminating in the apex of the right ventricle. The lung fields appeared normal. The ECG revealed a predominant ventricular paced rhythm, without evidence ofventriculoatrial activation. Other laboratory data were remarlcable '?r an elevated alkaline phosphatase (300 units, with normal below 120), while serum bilirubin, transaminases and electrolytes, the hemogram, and coagulation profile were normal. Serum thyroxine and tri-iodothyramine resin uptake values were also within normal limits. Contrast-enhanced digital subtraction images of the neck vasculature are shown in Figure L The carotid arteries appeared normal bilaterally, except that the right carotid vessel seemed medially
FICURE 1. Digital subtraction angiogram of the neck. (A) Arterial phase: no carotid artery pathology or arteriovenous flstuJa Is evident. The right carotid artery Is displaced laterally. (B) Venous phase: markedly dilated right internal jugular vein is demonstrated, but the left internal jugular vein Is not opacified.
displaced. There was no recirculation of contrast to suggest arteri
The concurrence of bicuspid regurgitation and unilateral internal jugular vein occlusion has not previously been described, but this combination provides the best explanation for our patient's presenting complaint. Severe tricuspid regurgitation caused exaggerated pulsatile venous waves and signs of volume overload of the right side of the heart, while the concomitant left internal jugular occlusion led to diminished pulsations on the side of occlusion and increased pulsations on the right side of the neck. The result was the appearance of marked unilateral neck pulsations, the etiology of which was difficult to distinguish from arterial origin (fe, from aneurysm or fistula) on physical examination. The tricuspid regurgitation in this patient was probably related either to the pacemaker electrode or to right ventricular dysfunction associated with the prior inferior myocardial infarction. u The pulmonary artery pressure was
not sufficiently elevated to account for the development of secondary bicuspid regurgitation. There was no diastolic gradient across the bicuspid valve or echocardiographic findings consistent with bicuspid stenosis, making bicuspid regurgitation secondary to rheumatic heart disease unlikely. There was also no echocardiographic evidence to support tricuspic valve prolapse as the primary problem. In addition, there was no clinical or laboratory evidence of other factorS~· which have been associated with the development of tricuspid regurgitation (fe, carcinoid syndrome, hyperthyroid-· ism, 3 endocarditis). Permanent pacemaker electrodes have been associated with thrombosis of the superior vena cava, axillary, innominate, internal jugular, and subclavian veins, ..7 and thrombosis secondary to the permanent pacemaker electrode installation seems the most likely explanation for the lack of visualization of the left internal jugular vein during phlebography in this patient. Venous thrombosis due to a permanent pacemaker is probably not infrequent. Storey et al• performed upper extremity phlebograms on 32 patients admitted for elective replacement of permanent pacemakers in order to determine the incidence of thrombosis associated with permanent pacing systems, and found no evidence of obstruction in 21 percent, mild obstruction (less than 50 percent) in 15 percent, 50 percent to 90 percent obstruction in 44 percent, and complete obstruction in 21 percent, with collateral venous drainage in all cases. Thrombosis is seldom clinically significant, although potential problems reported include the superior vena cava syndrome, 7 pulmonary embolism, 8 and large right atrial thrombi causing obstruction ofthe bicuspid orifice. • Most patients have no symptoms, but reported signs include arm edema, elevated venous pressure, and a prominent collateral venous pattern over the shoulder. Our patient demonstrates that internal jugular vein CHEST I 87 I 4 I APRIL, 1985
548
occlUIIoD, especll)ly in combination with tricuspid regUJ'Iitation, can result in marbdly asymmetrical neclc pu)satlollland abould be COIIIidered in the d6ratial cit....... of this llDdiq in patients with permanent pacemakers. BI:PBBBNCES
Rflht ,_tricular ID&rctloa with triculpid IDaullcleDcy mel cbraatc rllht bell't &dlure. Am J Cudiol1878;
1 Zoae DD, Botti BE. 37~
I McADfater RC, FrleliDpr CC, SbacJab..Smith BC. 'IHculpAd
iirlllllwllllblferlarm)'UCII'Cital bdiln:ltlaD. Arch IDterD Med 1878; 138:811-118 3 DouibertY MJ, Cnlie E. Apltbetlc hyperthynl6cllam pre~~~atlq ~
• trlcuapld J'eiUI'IItltlaD. Cbest 18'73; 83:781-11 4 StiDDey WS, Addlellaae RB, Alllrcl WC, 81111U1 CB, Prist RA, 'l1lamls CS. '1'he iDcldeDoe WWDDUI tbrombolfa i»>lowiq laq-
-
t8rm traD1WDoU1 J*llq. ADD 'l1lanc 5111'11878; 11:118-70 S Friedmao SA, Berpr N, Cematl MM, KGIIDOIIr:l J. 'VeDoul tbramboala aad penDaDeDt canltlc J*11D1. Am Helrt J 18'13; 8S:S31-33 I SethiCK, Bhaya&JN, ScattSM.IJU:vnnlnateWDDUitbrombolia: • l'll'e ooaaplbliaD w triDiwJDoul piCeiJI&Irer eleatrodel. Am Helrt J 18'74; 87:770-'11 7 'Wirtbelmer M, Huahel BE, c.tle CR. Superior ,_. cava qDdrome: ClOIDplicllfoa wpeniiUiellt traDIWDoUI eadoclnfill piCeiJI&Irer elecbuclet. JAMA 18'13; IIM:ll'IJ-73 8 Lulbeek MC, ErulroDil BE. Plcemaker electrade-iaduced tbramboala ID the IUperiar waa cava with pulmoaary embolaatlaa. Am J Cardfoll870; I8:IIJ5.07 8 LaadaD AR, Ruap PJ, ........ BF. .... rllht ltrlal thrombi IUI'IOIIIIdiDc penDaDeDt traDIY8DOUI ........... CUculltlao 1818; 40:811-M
I FIGURE 3. 'IWenty-seven clays later, the anterior mediastinal mass had recurred. The bilateral pleural effusions had increased in size. cell count between the time of initial evaluation of the lymphoma mass and the time of disappearance of the mass. This presumably was associated with heightened activity of the immune system which could have resulted in increased host immune response against the tumor. Surgery at a site other than a given tumor mass has been associated with spontaneous regression of the mass.1 This proposed mechanism could also have played a role in the case presented in this report. Weingarten et al1 recently published a series describing fOur patients who had transient spontaneous regression of intracerebral lymphoma. Their paper stressed that spontaneous regression of an intracerebral neoplasm should not be construed as a good prognostic sign nor as an indication of benignancy. It appears that the same admonitions apply to transient spontaneous regression of mediastinal lymphoblastic lymphoma. This disease carries a dismal prognosis, and transient spontaneous regression of a mediastinal lesion cannot be viewed as a reliable favorable indicator. REFERENCES 1 Cole WH. "Spontaneous regression of cancer: the metabolic triumph of the host? Ann NY Acad Sci 1974; 230:111-41 2 Gattkier HH, Wiltshaw E, Galton DAG. Spontaneous regression in non-Hodgkins lymphoma. Cancer 1980; 45:2627-32 3 Krikorian JG, Portlock CS, Cooney DP, Rosenberg SA. Spontaneous regression of non-Hodgkin's lymphoma: a report of nine cases. Cancer, 1980; 46:2093-99 4 Rees GLC. Abscopal regression in lymphoma: a mechanism in common with total body irradiation? Clio Radio 1981; 32:475-80 5 Weingarten KL, Zimmerman RD, Leeds NE. Spontaneous regression of intercerebral lymphoma. Radiology 1983; 149:721-24
·Unilateral Neck Pulsations Patient with a Permanent Pacemaker*
In a
Nell L. Coplan, M.D.; John A. AmbroH, M.D.; and johnothon L. Halperin , M.D .
•From the Division ofCardiolottv, Department of Medicine, Mount Sinai Medical Center, New York.
Reprint requem: Dr. Coplan, Annmberg 8-ZOO, One Gwtave L.
Levy Place, New York 10029
nternal jugular vein occlusion, especially in combination with tricuspid regurgitation, may result in asymmetrical neck pulsations. The fOllowing patient demonstrates the importance of considering these factors in difterential diagnosis. CASE REPORr
A 64-ye&M>ld man bad pulsations of the right side of the neck, which he bad noticed about tOur months earlier. He denied trauma, local dUcomtOrt, or neurologic symptoms. The pulsations were continuous and increasingly prominent. Four years previously, he sustained an inferior wall myocardial infarction complicated by complete heart block, and temporary pacing was achieved by right subclavian venipuncture. A permanent ventricular demand (VVI-mode) pacemaker was then installed via the left subclavian vein because of penistent high grade atrioventricular block. · Physical eumination revealed obvious pulsation of the right side of the neck. The heart rate was regular at 52 beats per minute; blood pressure was 140180 mm Hg In each arm, without pulsus paradorus. Discontinuous scratchy sounds were heard in both systole and diastole over the pulsatile area. A strong systolic wave could be palpated beneath the right sternocleidomastoid muscle; no discrete mass or lymphadenopathy was appreciated. The left side of the neck bad no acessive pulsatile waves. The left carotid pulse was normal, but the right carotid pulse could not be readily distingl,lished &om the diffi.ue pulsation. The external jugular veins were distended to the level ofthe mandibuJar angle bilaterally with the patient upright The chest was clear. There was no displacement of the apical impulse of the heart, and no precordial lift or thrill was fell Auscultation revealed a grade 216 coarse left parasternal systolic murmur without radiation, along with grade J/6 short high-pitched left parasternal decrescendo diastolic murmur. These did not change with Valsalva strain, standing, or squatting, but the systolic murmur intensified during inlpiration. No third or fOurth heart sound was heard. The abdomen displayed fAint hepatic pulsations, and minimal hepatomegaly. The venous pressure was elevated in both upper atremities, but no distended collateral venous pattern was apparent over the thorax, abdomen, or lower extremities. Edema was absenl Chest roentgenograms showed mild cardiomegaly, with prominence of the right atrial border and the right ventricle; the pacemaker generator was seen in the left pectorali.Ossa, with Its electrode terminating in the apex of the right ventricle. The lung fields appeared normal. The ECG revealed a predominant ventricular paced rhythm, without evidence ofventriculoatrial activation. Other laboratory data were remarlcable '?r an elevated alkaline phosphatase (300 units, with normal below 120), while serum bilirubin, transaminases and electrolytes, the hemogram, and coagulation profile were normal. Serum thyroxine and tri-iodothyramine resin uptake values were also within normal limits. Contrast-enhanced digital subtraction images of the neck vasculature are shown in Figure L The carotid arteries appeared normal bilaterally, except that the right carotid vessel seemed medially
FICURE 1. Digital subtraction angiogram of the neck. (A) Arterial phase: no carotid artery pathology or arteriovenous flstuJa Is evident. The right carotid artery Is displaced laterally. (B) Venous phase: markedly dilated right internal jugular vein is demonstrated, but the left internal jugular vein Is not opacified.
displaced. There was no recirculation of contrast to suggest arteri
The concurrence of bicuspid regurgitation and unilateral internal jugular vein occlusion has not previously been described, but this combination provides the best explanation for our patient's presenting complaint. Severe tricuspid regurgitation caused exaggerated pulsatile venous waves and signs of volume overload of the right side of the heart, while the concomitant left internal jugular occlusion led to diminished pulsations on the side of occlusion and increased pulsations on the right side of the neck. The result was the appearance of marked unilateral neck pulsations, the etiology of which was difficult to distinguish from arterial origin (fe, from aneurysm or fistula) on physical examination. The tricuspid regurgitation in this patient was probably related either to the pacemaker electrode or to right ventricular dysfunction associated with the prior inferior myocardial infarction. u The pulmonary artery pressure was
not sufficiently elevated to account for the development of secondary bicuspid regurgitation. There was no diastolic gradient across the bicuspid valve or echocardiographic findings consistent with bicuspid stenosis, making bicuspid regurgitation secondary to rheumatic heart disease unlikely. There was also no echocardiographic evidence to support tricuspic valve prolapse as the primary problem. In addition, there was no clinical or laboratory evidence of other factorS~· which have been associated with the development of tricuspid regurgitation (fe, carcinoid syndrome, hyperthyroid-· ism, 3 endocarditis). Permanent pacemaker electrodes have been associated with thrombosis of the superior vena cava, axillary, innominate, internal jugular, and subclavian veins, ..7 and thrombosis secondary to the permanent pacemaker electrode installation seems the most likely explanation for the lack of visualization of the left internal jugular vein during phlebography in this patient. Venous thrombosis due to a permanent pacemaker is probably not infrequent. Storey et al• performed upper extremity phlebograms on 32 patients admitted for elective replacement of permanent pacemakers in order to determine the incidence of thrombosis associated with permanent pacing systems, and found no evidence of obstruction in 21 percent, mild obstruction (less than 50 percent) in 15 percent, 50 percent to 90 percent obstruction in 44 percent, and complete obstruction in 21 percent, with collateral venous drainage in all cases. Thrombosis is seldom clinically significant, although potential problems reported include the superior vena cava syndrome, 7 pulmonary embolism, 8 and large right atrial thrombi causing obstruction ofthe bicuspid orifice. • Most patients have no symptoms, but reported signs include arm edema, elevated venous pressure, and a prominent collateral venous pattern over the shoulder. Our patient demonstrates that internal jugular vein CHEST I 87 I 4 I APRIL, 1985
548
occlUIIoD, especll)ly in combination with tricuspid regUJ'Iitation, can result in marbdly asymmetrical neclc pu)satlollland abould be COIIIidered in the d6ratial cit....... of this llDdiq in patients with permanent pacemakers. BI:PBBBNCES
Rflht ,_tricular ID&rctloa with triculpid IDaullcleDcy mel cbraatc rllht bell't &dlure. Am J Cudiol1878;
1 Zoae DD, Botti BE. 37~
I McADfater RC, FrleliDpr CC, SbacJab..Smith BC. 'IHculpAd
iirlllllwllllblferlarm)'UCII'Cital bdiln:ltlaD. Arch IDterD Med 1878; 138:811-118 3 DouibertY MJ, Cnlie E. Apltbetlc hyperthynl6cllam pre~~~atlq ~
• trlcuapld J'eiUI'IItltlaD. Cbest 18'73; 83:781-11 4 StiDDey WS, Addlellaae RB, Alllrcl WC, 81111U1 CB, Prist RA, 'l1lamls CS. '1'he iDcldeDoe WWDDUI tbrombolfa i»>lowiq laq-
-
t8rm traD1WDoU1 J*llq. ADD 'l1lanc 5111'11878; 11:118-70 S Friedmao SA, Berpr N, Cematl MM, KGIIDOIIr:l J. 'VeDoul tbramboala aad penDaDeDt canltlc J*11D1. Am Helrt J 18'13; 8S:S31-33 I SethiCK, Bhaya&JN, ScattSM.IJU:vnnlnateWDDUitbrombolia: • l'll'e ooaaplbliaD w triDiwJDoul piCeiJI&Irer eleatrodel. Am Helrt J 18'74; 87:770-'11 7 'Wirtbelmer M, Huahel BE, c.tle CR. Superior ,_. cava qDdrome: ClOIDplicllfoa wpeniiUiellt traDIWDoUI eadoclnfill piCeiJI&Irer elecbuclet. JAMA 18'13; IIM:ll'IJ-73 8 Lulbeek MC, ErulroDil BE. Plcemaker electrade-iaduced tbramboala ID the IUperiar waa cava with pulmoaary embolaatlaa. Am J Cardfoll870; I8:IIJ5.07 8 LaadaD AR, Ruap PJ, ........ BF. .... rllht ltrlal thrombi IUI'IOIIIIdiDc penDaDeDt traDIY8DOUI ........... CUculltlao 1818; 40:811-M