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Myocardial Infarction Due to Tumor Embolization Following Pulmonary Resection
Myocardial lnfardion Due to Tumor Embolization Following Pulmonary Resedion* Ronald P. Karl8b~g. Thomas B. F~guson,
M.D.;•• Stuart S. Sagel, M.D.;t and M.D., F.C.C.P.t
A fatal case of IIUIIIive systemk tumor embollzaUon, with Involvement of the eoronary arteries, wlllela oeenrred foDowlng resedlon of • ..aastatle pabaonary 8broUposareoma, Is preeentecl. Elevadon of the eardlae speclftc creatine ldn8le lloenzyme (MII-CK.) doeamellted myocardial damage due to oiJstrnctlon of the COI"'OI88')' arteries by tumor emboli. The preoperative compntecl tomopapby IC8D deiDOII8tnltecl eontlplty between the pulmonary ....., the Inferior pnlmoaary vela and left atrium. In fntnre cases this 8ndln& sbonld alert the IUJ'Ieon that c:ardiopnlmonary .,.... and reJDOVII of the left atrial e:dellllon of the tumor under direct vlsloD may be reqnlnd to prevent embolization dnrlna pulmonary resection. Embolization of neoplastic tissue resulting in major systemic artery obstruction is a very rare complication of pulmonary resection. 1 Equally uncommon is myocardial infarction caused by embolization of any sort, although a recent review suggests that this condition is frequently unrecognized. 2 We present an unusual case of major arterial embolization, including the coronary arteries, caused by liberation of tumor at the time of bilobectomy.
The patient, a 70-year-old woman, in August, 1974, had a wide resection of a flbroliposarcoma of the left shoulder followed by radiation therapy to a dose of 6,000 rad. Two years later a 3-cm right hilar mass was noted on a chest radiograph. Laminagrams showed a noncalcified mass adjacent to the bronchus intermedius and the right inferior pulmonary vein (Fig 1 ). Computed tomographic scans demoustrated a 3-cm lobulated mass fmpreaing the postedor aspect of the bronchus intermedius, in intimate contact with the right inferior pulmonary vein and the lateral aspect of the left atrium (Fig 2). (The potential significance of this association was not appreciated at the time.) There was 110 evidence of local recurrence. Cardiopulmonary examination and tests were normal. Fiberoptic bronchoseopy findings were negative. At operation on Dec. 11, 1976, a mass was palpable within the superior segment of the right lower lobe, which extended across to involve the middle lobe. A bilobectomy was performed without incident The patient suddenly became hypo-
1. Anteroposterior laminagram demoustrating DOD· calcified mass medial and posterior to bronchus intermedius.
FIGURE
tensive; however, as she was turned to a supine position in preparation for transfer to her bed. ECG showed an acute inferior infarction with periods of Mobitz I block. All peripheral pu1ses were unobtainable in spite of a good apical cardiac impulse. Acute pericardial tamponade was ruled out, and then an intra-aortic balloon pump was inserted. Central aortic pressure was 100 mm Hg and still no peripheral pu1ses were obtainable. The patient was transferred to the Cardiathoracic Intensive Care Unit She never regained consciousness, and died 18 hours after the initial episode. The working diagnosis was cardiogenic shock with intense peripheral vuoCODStriction. Total creatine ldnue wu 5,870 W/liter and the cardiac isoeDZ)'IDe ( MB-CK) was predornfn•nt on electrophoresis 6 and u hours postoperatively. At autopsy, luge flbroliposarcoma tumor emboli were present in all branches of the aorta, with occlusion of the arch vessels and branches of the hepatic, renal and mesenteric arteries. Microscopic tumor emboli were pdlellt throughout
• From the Washington University School of Medicine, St
Louis. ..Presently Chief, Cardiac Ph)'lio]ogy ~. Veterans Administration Hospital, Long Bi8ch, and Aaistant Professor of Medicine1 University Of Califoinia, Irvine. Professor of Radiowgy. Clinical Professor of Surgery, Division of Cardiothoracic Surgery. ll6rlrlm requem: Dr. Karllberg" Canlltlc Pltuftololzu LtJb. oraloty, VA HosplltJI, Long BfUJCn. Califoml4 9080r
!
582 URLSBERG, SAGEL, FERGUSON
FIGOlUl !. Computed tomogram showbig somewhat lobulated 3-cm mass ( m) posterior to bronchus intermedius. Mass is in dUect contiguity with right inferior pulmonary vein and lateral aspect of left atrium ( Z.).
CHEST, 74: 5, NOVEMBER, 1978
FrotJU 3. Microscopic tumor embolus in corouary arteriole. Pleomorphic cells were identical to those of primary tumor ( 600X ). the heart (Fig 3). The Dative corouary arteries were normal Because of the large volume of metastatic tumor ( estimated at 75 ml) found in all parts of the body, it is postulated that the tumor in the superior segment of the right lower lobe had invaded the inferior pulmouary vein and grown centrally into the cavity of the left atrium in a dumbbeD configuration. Separation and fragmentation of the very soft tumor then occurred when the inferior pulmouary vein was ligated. That such a postulate is correct is suggested by the finding at autopsy of tumor remDaDts in the left atrium at the point of entrance of the inferior pulmouary vein.
the left atrium. Intraoperative major artery tumor embolization from a pulmonary source is rare, and only sporadically reported (Table 1). The overall mortality was 75 percent (9/12 patients). Smaller emboli to the aortic bifurcation alone were removed successfully.•·• There is no other reported case of tumor embolization occurring after pulmonary resection presenting as acute myocardial infarction, although myocardial infarction due to spontaneous tumor embolization bas been previously reported. 11 Myocardial infaretion was substantiated by serial electrocardiograms and positive MB-CIC isoenzymes.• Previous communications have suggested that the avoidance of manipulation of the lung and 1igation of the pulmonary vein draining the tumor-bearing area as the initial step in the resection wiD decrease the incl-
DiscuSSION
Death in this patient was the consequence of massive · tumor embolization from a metastatic pulmonary BbroJiposarcoma which had invaded the pulmonary vein and
Senior Author, Yr
Reference No. Age
Primary Embolization Location Pulmonary
Sex
Tumor Type
EuonEH
(1900)
10
61
M
Bronchial CA
AylwinJA
(1951)
11
37
F
Fibrosarcoma
AylwinJA
(1951)
11
52
M
Bronchial CA
Probert WR
(1956)
12
56
F
Metastatic Adrenal CA
Taber R
(1961)
8
57
M
Highly ADaplastic
Christianson TW (1966)
13
69
M
Undiirerentiated
(1969)
3
64
M
Undiirerentiated
Balaa p
(1971)
7
64
M
Undiirerentiated
MacMahonH
(1974)
4
56
F
Giant Cell
MacMahonH
(1974)
4
63
M
Undiirerentiated
Miranda A
(1975)
14
67
M
Epidermoid
KarJsberg R
(1978)
70
F
Fibrolipoearcoma
DeBoerHHM
(Preeent oaae)
CHEST, 74:
~
NOVSMBER, 1978
Vein
Aorta Cerebral Other
...;
...;
v
v v v
v
...;
...;
v
...;
v v v v
Ligation No No
Died
No
Died
No
Died
No
Died
Probably Died No Succeesful Embolectomy No
Died
Yes
Succesaful Embolectomy
v v
v v
v
Outcome Died
Succeesful Embolectomy Yes
Died
Yes
Died
MYOCARDIAL INFARcnON DUE TO TUMOR mBOUZAnGI Ill
dence of tumor embolization. 7•8 This case demonstrates that embolization is not prevented by these precautions when direct invasion of! the left atrial cavity by the tumor has occurred. The key to the management of such a case lies in the preoperative recognition that pulmonary vein and left atrial invasion may be present, so that an appropriate surgical approach may be planned. Computed tomography (CT) provides a potential method of resolving this problem.8 The location and extent of neoplastic disease may be determined more precisely than with conventional radiologic techniques. In this patient, computed tomography demonstrated the intimate association of the lung neoplasm with the pulmonary vein and left atrium. Because this case occurred very early in our experience with CT scanning, the clinical significance of this finding was not fully appreciated. Today, recognition of such an abnormality would prompt the radiologist to repeat the computed tomographic scans after contrast media infusion to determine if definite invasion by tumor of the pulmonary veins and left atrium is present. Pulmonary angiography might also help in this assessment. Certainly, if CT scans suggest pulmonary venous and intracardiac extension of tumor, the surgeon should consider utilizing the cardiopulmonary bypass with aortic cross-clamping so that the left atrium can be opened and the tumor with its mural attachments removed under direct vision, the technique now preferred for the management of left atrial myxoma. Concomitant lobectomy or pneumonectomy through the median sternotomy approach poses no technical problems, and has been utilized at our institution in a number of cases. ACKNOWLEDGMENTS: We thank Dr. Fredric Askin who assisted with the photomicrogra~hs. Dr. Robert Roberts and his laboratory for performing the MB-CK assay, and Dr. Scott M. Noidlicht and Dr. Alan N. Weiss who participated fn the management of this case.
ltEFEBENCES 1 Kirsh MM, Rotman H, Behrendt DM, et al: ComplicatioDI of pu1moJW'Y resection. Ann Thorac Surg 20:i15-S36, 1975 2 Prizel KR, HutcheDI GM, Bulkley BH: CoroJW'Y artery embolism and myocardial infarction: A clinicopathologic study of 55 patients. Ann Int Med 88:155-161, 1978 3 De Boer HHM, Prillevitz HW: Massive tumor embolfml. Arch Chir Neerl21 :ii3-234, 1969 . 4 MacMahon H, Forrest JV, Weisz D, et al: Massive tumor embolism occurring during pneumonectomy. Ann Thorac Surg 17:395-397, 1974 5 Cera LJ, Karlinsky W, Rodin AE: Tumor embolism of the left coroJW'Y artery. Am Heart J 53:472-478, 19157 6 Klein MS, Shell WE, Sobel BE: Serum creatine phosphokinase ( CPK) isoenzymes after intramuscular injections, surgery, and myocardfal infarction. Cardiovasc Res 7:412418, 1973 7 Balas P, Katsaras E, Zoitopoub M: Peripheral arterial embolism by malignant tumor. Vase Surg 5:i7-29, 1971 8 Taber RE: Massive systemic tumor embolization during pneumonectomy: a case report with comments on routine primary pWmOJW'Y vein ligation. Ann Surg 154:!63-188, 1961 9 Jost RG, Sagel SS, Stanley RJ, et al: Compatlld tomography of the thorax. Radiology 128: 125-138, 1978
584 COYER ET AL
10 Eason EH: A case of cerebral infarction due to neoplastic embolism. J Path Bact 62:454-457, 1950 11 Aylwin JA: Avoidable vascular spread in resection for bronchial carcinoma. 'l11oru 6:250-267, 1951 H Probert WR: Sudden operative death. due to massive tumour embolism. Br Med J 1:435-436, 1956 13 Christiansen TW, Morgan S: Tumor embolfml fn a peripheral artery. Ann Thorac Surg 1::311-313, 1965 14 Miranda AL, Bnfl}ancbas JJ, Juffe A, et al: Direct enension of bronchogenic carcinoma through the pWmOJW'Y veins: Surgical implicatioDJ. Chest 68: 1!3-114, 19'71S
Left Stellectomy 1n the Long QT Syndrome* Btucs H. COfiM, M.D.;•• &, Prvor, M.D.;t Wolf/ M. Kitsch, M.D.;t and S. Gilbert Blounl, ]r., M.D.§
Reeent Investigations have rea8irmed the role of antoHmie innervation of the heart In the genesis of eea1llln ardlac: a'l'hytbmias. Tbe long QT syndro111e (LQTS) hal been deserlbed for yean, bat only recently hal evidence of Its Unk to autonomle lmbaiii'M:e been estUUshed. A ease of LQTS Is pn~ented with lntrMperatift evidence at the tbne of left stelleetomy of llfe-tbreatelllq arrhythmias trlgered by stimulation of tbls neunl body. Removal of the left stellate pnallon normalized the eleetroeardlopaphie (ECG) abnonnalftle8 aad ... rendered the patient asymptomatle since IIII'J'UY• Left lteJ. leetomy may beeome definitive therapy for !leleeted patients with the LQTS. LQTS was first described by .Jervall and Lange• Nielsen1 in 1957. They identified patients with this ECG abnormality and associated congenital deafness, pointing out an autosomal recessive mode of inheritance. Later, Romanol and Ward~ reported patients with an autosomal dominant inheritance pattern without congenital deafness. Although this syndrome has varying degrees of expression, clinical importance is found in its association with syncopal attacks and sudden death secondary to episodes of ventricular flbrillation. These episodes are often associated with, if not precipitated by, emotional or physical stress. ~e
CASE REPoRT The patient, now 20 years old, began experiencing monthly episodes of syncope which were self-terminating, lasting a few seconds to a few minutes, at eight years of age. When identified as LQTS, varying drug regimens including quinidine, phenobarbital and propranolol therapy ( 40 mg QID) were initiated. Despite propranolol therapy, his syncopal
•From the J)eparbJ!ents of Medicine and Surgery_, DivisioDI of Cardiology and Neu101UJ'881'Y, Univeralty or Colorado Medical Center, Denver. ••Fellow fn CardiOlogy. tProfessor of Mediclile (Cardiology). tprofeaor of N~ and Division Head. §Professor of Medicine, CUdioloiiY Divf.don Bead. reqtiB8fl: Dr. Prvor, 4JOO ""!Gat Ninth A.,._, n.no.r
='
CHES~
74: 5, NOVEMBER, 1978
M.D.;•• Stuart S. Sagel, M.D.;t and M.D., F.C.C.P.t
A fatal case of IIUIIIive systemk tumor embollzaUon, with Involvement of the eoronary arteries, wlllela oeenrred foDowlng resedlon of • ..aastatle pabaonary 8broUposareoma, Is preeentecl. Elevadon of the eardlae speclftc creatine ldn8le lloenzyme (MII-CK.) doeamellted myocardial damage due to oiJstrnctlon of the COI"'OI88')' arteries by tumor emboli. The preoperative compntecl tomopapby IC8D deiDOII8tnltecl eontlplty between the pulmonary ....., the Inferior pnlmoaary vela and left atrium. In fntnre cases this 8ndln& sbonld alert the IUJ'Ieon that c:ardiopnlmonary .,.... and reJDOVII of the left atrial e:dellllon of the tumor under direct vlsloD may be reqnlnd to prevent embolization dnrlna pulmonary resection. Embolization of neoplastic tissue resulting in major systemic artery obstruction is a very rare complication of pulmonary resection. 1 Equally uncommon is myocardial infarction caused by embolization of any sort, although a recent review suggests that this condition is frequently unrecognized. 2 We present an unusual case of major arterial embolization, including the coronary arteries, caused by liberation of tumor at the time of bilobectomy.
The patient, a 70-year-old woman, in August, 1974, had a wide resection of a flbroliposarcoma of the left shoulder followed by radiation therapy to a dose of 6,000 rad. Two years later a 3-cm right hilar mass was noted on a chest radiograph. Laminagrams showed a noncalcified mass adjacent to the bronchus intermedius and the right inferior pulmonary vein (Fig 1 ). Computed tomographic scans demoustrated a 3-cm lobulated mass fmpreaing the postedor aspect of the bronchus intermedius, in intimate contact with the right inferior pulmonary vein and the lateral aspect of the left atrium (Fig 2). (The potential significance of this association was not appreciated at the time.) There was 110 evidence of local recurrence. Cardiopulmonary examination and tests were normal. Fiberoptic bronchoseopy findings were negative. At operation on Dec. 11, 1976, a mass was palpable within the superior segment of the right lower lobe, which extended across to involve the middle lobe. A bilobectomy was performed without incident The patient suddenly became hypo-
1. Anteroposterior laminagram demoustrating DOD· calcified mass medial and posterior to bronchus intermedius.
FIGURE
tensive; however, as she was turned to a supine position in preparation for transfer to her bed. ECG showed an acute inferior infarction with periods of Mobitz I block. All peripheral pu1ses were unobtainable in spite of a good apical cardiac impulse. Acute pericardial tamponade was ruled out, and then an intra-aortic balloon pump was inserted. Central aortic pressure was 100 mm Hg and still no peripheral pu1ses were obtainable. The patient was transferred to the Cardiathoracic Intensive Care Unit She never regained consciousness, and died 18 hours after the initial episode. The working diagnosis was cardiogenic shock with intense peripheral vuoCODStriction. Total creatine ldnue wu 5,870 W/liter and the cardiac isoeDZ)'IDe ( MB-CK) was predornfn•nt on electrophoresis 6 and u hours postoperatively. At autopsy, luge flbroliposarcoma tumor emboli were present in all branches of the aorta, with occlusion of the arch vessels and branches of the hepatic, renal and mesenteric arteries. Microscopic tumor emboli were pdlellt throughout
• From the Washington University School of Medicine, St
Louis. ..Presently Chief, Cardiac Ph)'lio]ogy ~. Veterans Administration Hospital, Long Bi8ch, and Aaistant Professor of Medicine1 University Of Califoinia, Irvine. Professor of Radiowgy. Clinical Professor of Surgery, Division of Cardiothoracic Surgery. ll6rlrlm requem: Dr. Karllberg" Canlltlc Pltuftololzu LtJb. oraloty, VA HosplltJI, Long BfUJCn. Califoml4 9080r
!
582 URLSBERG, SAGEL, FERGUSON
FIGOlUl !. Computed tomogram showbig somewhat lobulated 3-cm mass ( m) posterior to bronchus intermedius. Mass is in dUect contiguity with right inferior pulmonary vein and lateral aspect of left atrium ( Z.).
CHEST, 74: 5, NOVEMBER, 1978
FrotJU 3. Microscopic tumor embolus in corouary arteriole. Pleomorphic cells were identical to those of primary tumor ( 600X ). the heart (Fig 3). The Dative corouary arteries were normal Because of the large volume of metastatic tumor ( estimated at 75 ml) found in all parts of the body, it is postulated that the tumor in the superior segment of the right lower lobe had invaded the inferior pulmouary vein and grown centrally into the cavity of the left atrium in a dumbbeD configuration. Separation and fragmentation of the very soft tumor then occurred when the inferior pulmouary vein was ligated. That such a postulate is correct is suggested by the finding at autopsy of tumor remDaDts in the left atrium at the point of entrance of the inferior pulmouary vein.
the left atrium. Intraoperative major artery tumor embolization from a pulmonary source is rare, and only sporadically reported (Table 1). The overall mortality was 75 percent (9/12 patients). Smaller emboli to the aortic bifurcation alone were removed successfully.•·• There is no other reported case of tumor embolization occurring after pulmonary resection presenting as acute myocardial infarction, although myocardial infarction due to spontaneous tumor embolization bas been previously reported. 11 Myocardial infaretion was substantiated by serial electrocardiograms and positive MB-CIC isoenzymes.• Previous communications have suggested that the avoidance of manipulation of the lung and 1igation of the pulmonary vein draining the tumor-bearing area as the initial step in the resection wiD decrease the incl-
DiscuSSION
Death in this patient was the consequence of massive · tumor embolization from a metastatic pulmonary BbroJiposarcoma which had invaded the pulmonary vein and
Senior Author, Yr
Reference No. Age
Primary Embolization Location Pulmonary
Sex
Tumor Type
EuonEH
(1900)
10
61
M
Bronchial CA
AylwinJA
(1951)
11
37
F
Fibrosarcoma
AylwinJA
(1951)
11
52
M
Bronchial CA
Probert WR
(1956)
12
56
F
Metastatic Adrenal CA
Taber R
(1961)
8
57
M
Highly ADaplastic
Christianson TW (1966)
13
69
M
Undiirerentiated
(1969)
3
64
M
Undiirerentiated
Balaa p
(1971)
7
64
M
Undiirerentiated
MacMahonH
(1974)
4
56
F
Giant Cell
MacMahonH
(1974)
4
63
M
Undiirerentiated
Miranda A
(1975)
14
67
M
Epidermoid
KarJsberg R
(1978)
70
F
Fibrolipoearcoma
DeBoerHHM
(Preeent oaae)
CHEST, 74:
~
NOVSMBER, 1978
Vein
Aorta Cerebral Other
...;
...;
v
v v v
v
...;
...;
v
...;
v v v v
Ligation No No
Died
No
Died
No
Died
No
Died
Probably Died No Succeesful Embolectomy No
Died
Yes
Succesaful Embolectomy
v v
v v
v
Outcome Died
Succeesful Embolectomy Yes
Died
Yes
Died
MYOCARDIAL INFARcnON DUE TO TUMOR mBOUZAnGI Ill
dence of tumor embolization. 7•8 This case demonstrates that embolization is not prevented by these precautions when direct invasion of! the left atrial cavity by the tumor has occurred. The key to the management of such a case lies in the preoperative recognition that pulmonary vein and left atrial invasion may be present, so that an appropriate surgical approach may be planned. Computed tomography (CT) provides a potential method of resolving this problem.8 The location and extent of neoplastic disease may be determined more precisely than with conventional radiologic techniques. In this patient, computed tomography demonstrated the intimate association of the lung neoplasm with the pulmonary vein and left atrium. Because this case occurred very early in our experience with CT scanning, the clinical significance of this finding was not fully appreciated. Today, recognition of such an abnormality would prompt the radiologist to repeat the computed tomographic scans after contrast media infusion to determine if definite invasion by tumor of the pulmonary veins and left atrium is present. Pulmonary angiography might also help in this assessment. Certainly, if CT scans suggest pulmonary venous and intracardiac extension of tumor, the surgeon should consider utilizing the cardiopulmonary bypass with aortic cross-clamping so that the left atrium can be opened and the tumor with its mural attachments removed under direct vision, the technique now preferred for the management of left atrial myxoma. Concomitant lobectomy or pneumonectomy through the median sternotomy approach poses no technical problems, and has been utilized at our institution in a number of cases. ACKNOWLEDGMENTS: We thank Dr. Fredric Askin who assisted with the photomicrogra~hs. Dr. Robert Roberts and his laboratory for performing the MB-CK assay, and Dr. Scott M. Noidlicht and Dr. Alan N. Weiss who participated fn the management of this case.
ltEFEBENCES 1 Kirsh MM, Rotman H, Behrendt DM, et al: ComplicatioDI of pu1moJW'Y resection. Ann Thorac Surg 20:i15-S36, 1975 2 Prizel KR, HutcheDI GM, Bulkley BH: CoroJW'Y artery embolism and myocardial infarction: A clinicopathologic study of 55 patients. Ann Int Med 88:155-161, 1978 3 De Boer HHM, Prillevitz HW: Massive tumor embolfml. Arch Chir Neerl21 :ii3-234, 1969 . 4 MacMahon H, Forrest JV, Weisz D, et al: Massive tumor embolism occurring during pneumonectomy. Ann Thorac Surg 17:395-397, 1974 5 Cera LJ, Karlinsky W, Rodin AE: Tumor embolism of the left coroJW'Y artery. Am Heart J 53:472-478, 19157 6 Klein MS, Shell WE, Sobel BE: Serum creatine phosphokinase ( CPK) isoenzymes after intramuscular injections, surgery, and myocardfal infarction. Cardiovasc Res 7:412418, 1973 7 Balas P, Katsaras E, Zoitopoub M: Peripheral arterial embolism by malignant tumor. Vase Surg 5:i7-29, 1971 8 Taber RE: Massive systemic tumor embolization during pneumonectomy: a case report with comments on routine primary pWmOJW'Y vein ligation. Ann Surg 154:!63-188, 1961 9 Jost RG, Sagel SS, Stanley RJ, et al: Compatlld tomography of the thorax. Radiology 128: 125-138, 1978
584 COYER ET AL
10 Eason EH: A case of cerebral infarction due to neoplastic embolism. J Path Bact 62:454-457, 1950 11 Aylwin JA: Avoidable vascular spread in resection for bronchial carcinoma. 'l11oru 6:250-267, 1951 H Probert WR: Sudden operative death. due to massive tumour embolism. Br Med J 1:435-436, 1956 13 Christiansen TW, Morgan S: Tumor embolfml fn a peripheral artery. Ann Thorac Surg 1::311-313, 1965 14 Miranda AL, Bnfl}ancbas JJ, Juffe A, et al: Direct enension of bronchogenic carcinoma through the pWmOJW'Y veins: Surgical implicatioDJ. Chest 68: 1!3-114, 19'71S
Left Stellectomy 1n the Long QT Syndrome* Btucs H. COfiM, M.D.;•• &, Prvor, M.D.;t Wolf/ M. Kitsch, M.D.;t and S. Gilbert Blounl, ]r., M.D.§
Reeent Investigations have rea8irmed the role of antoHmie innervation of the heart In the genesis of eea1llln ardlac: a'l'hytbmias. Tbe long QT syndro111e (LQTS) hal been deserlbed for yean, bat only recently hal evidence of Its Unk to autonomle lmbaiii'M:e been estUUshed. A ease of LQTS Is pn~ented with lntrMperatift evidence at the tbne of left stelleetomy of llfe-tbreatelllq arrhythmias trlgered by stimulation of tbls neunl body. Removal of the left stellate pnallon normalized the eleetroeardlopaphie (ECG) abnonnalftle8 aad ... rendered the patient asymptomatle since IIII'J'UY• Left lteJ. leetomy may beeome definitive therapy for !leleeted patients with the LQTS. LQTS was first described by .Jervall and Lange• Nielsen1 in 1957. They identified patients with this ECG abnormality and associated congenital deafness, pointing out an autosomal recessive mode of inheritance. Later, Romanol and Ward~ reported patients with an autosomal dominant inheritance pattern without congenital deafness. Although this syndrome has varying degrees of expression, clinical importance is found in its association with syncopal attacks and sudden death secondary to episodes of ventricular flbrillation. These episodes are often associated with, if not precipitated by, emotional or physical stress. ~e
CASE REPoRT The patient, now 20 years old, began experiencing monthly episodes of syncope which were self-terminating, lasting a few seconds to a few minutes, at eight years of age. When identified as LQTS, varying drug regimens including quinidine, phenobarbital and propranolol therapy ( 40 mg QID) were initiated. Despite propranolol therapy, his syncopal
•From the J)eparbJ!ents of Medicine and Surgery_, DivisioDI of Cardiology and Neu101UJ'881'Y, Univeralty or Colorado Medical Center, Denver. ••Fellow fn CardiOlogy. tProfessor of Mediclile (Cardiology). tprofeaor of N~ and Division Head. §Professor of Medicine, CUdioloiiY Divf.don Bead. reqtiB8fl: Dr. Prvor, 4JOO ""!Gat Ninth A.,._, n.no.r
='
CHES~
74: 5, NOVEMBER, 1978