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Left Stellectomy in the Long QT Syndrome
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
F.tCUBB 1. Standard lead 2 rhythm sbip prior to surgery. Note sinus bradycardia characteristic of this syndrome: QT interval is prolonged to 0.54 sec.
.
- , - r-·
,.
I
~
- :.J
I
I
l
l.
k.
1' -
I- r-r-
FIGUI\E 2. Intraoperative rhythm sbip during manipulation of left stellate ganglion showing numerous premature venbicular beats of varied origin and run of ventricuJar tachycardia; ectopy ceased when manipulation ended.
FIGURE 3. Standard lead 2 rhythm sbip after surgery. Note persistence of sinus bradycardia, but normalization of QT interval. QT has remained normal one year postoperatively.
attacks persisted. In October, 1974, whUe playing football, he experienced his first episode of prolonged collapse requiring cudiopuJmonary resuscitation. Afterward he had two subsequent attacks, both requiring cardiopulmonary resuscitation. Following his last attack, surgical intervention was deemed advisable. Figure 1 represents his ECG prior to surgery: note the QT prolongation to 0.54 sec. On Feb 10, 1977, the left cervical sympathetic ganglia cham was exposed. Physical manipulation of the steDate ganglia (traction. clamping, etc) produced dramatic wntricular Irritability and arrhythmias. One of these arrhythmias is shown in Figure 2. The stellate ganglia in its entirety and the first four thoracic sympathetic ganglia were removed. Figure 3 demonstrates the patient's QT interval after surgery. His QT interval normalized and has remained normal since surgery one year ago. More importantly, he has remained totally free of syncopal attaclcs. He remains on pblockade therapy. No LQTS was found in any family members. Concurring with the experience of Frogatt and James,• this patient demonstrates that this seemingly congenital and usually familial syndrome may occur as an isolated case. DISCUSSION
Therapy for the LQTS has taken many forms. Varying drug regimens including oxygen, potassium, diphenylhydantoin, digitalis preparations, hyoscyamine, phenobarbital and even quinidine have all been used alone and in combination with disappointing results. Cardiac pacing has been tried without significant effect. p-blocking agents have been highly effective. The only large series ( 300 Q&Ses) revealed a 78 percent untreated mortality, a 60 percent mortality in those treated with non-p blockade, but a tenfold reduction in mortality to 6
CHEST, 74: 5, NOVEMBER, 1978
percent in those treated with p blockade. (Personal communication, Peter J. Schwartz, 1977) . Despite this dramatic improvement, 6 percent of the patients are unresponsive to p-blockade, leaving them vulnerable to repeated syncopal attacks and sudden death. Even among responders, the possible untoward effects of lifelong p-blockade are not known. Also, pblockade may result in many side-effects requiring lifestyle alterations. In several cases, patients who inadvertently missed medication were exposed to potentially fatal recurrent attacks." The etiology of the LQTS is not fully understood, but recent research implicates the role of sympathetic imbalance in the innervation of the heart as the cause. The present case offers further evidence for this etiology. The autonomic innervation of the heart includes both sympathetic and parasympathetic fibers, but only sympathetic fibers terminate in the ventricular musculature. These sympathetic fibers originate from the superior, middle and inferior (stellate) cervical ganglia in conjunction with the first four or five thoracic sympathetic ganglia. Much investigative work has been accomplished manipulating various segments of this innervation. Reports" reveal a prolongation of the QT interval with blockade or removal of the right stellate ganglion, whfle shortening of the QT interval has occurred with blockade or removal (LSGR) of the left stellate ganglion. Although not without some contradictory evidence, a congenital "'underactivity" of the right-half or an •overactivity.. of the left-half of this system seem to be plausible explanations. Further substantiation of these possible_ etiologies has recently been reported by Schwartz et al,'
LEFT STELLECTOMY IN LONG QT SYNDROME 585
in animal models. Their work has demonstrated a lowering of the ventricular fibrillation threshold by stellate ganglion manipulations in dogs, possibly implying a similar situation in these patients. To date, ten patients have undergone LSGR, ours being the tenth. Although followup has been relatively short (one to six years), cessation of syncopal attacks occurred in an patients, although QT interval shortening on ECG has not occurred in all cases. Some patients also remain on {Jblockade therapy and therefore possibly benefit from both forms of treatment. To date, problems with denervation hypersensitivity or with nerve regeneration have not been reported. The LQTS may be more common than suspected and currently is often overlooked. There are many ramifications: sudden death in infants, the crib deaths, may be one example. Whether the LQTS is responsible remains to be fully investigated. The possible fatal complications associated with the LQTS demand recognition and therapy early in life. This early recognition and therapy is often needed to prevent sudden death, carrying the patient into adulthood where spontaneous improvement has been noted. Further implications of this syndrome have been pointed out by Schwartz and co-workers• in experiments with ventricular fibrillation threshold and arrhythmias in the setting of coronary occlusion, perhaps pointing to new considerations in coronary artery disease. The in vivo production of life-threatening arrhythmias demonstrated here is the first such evidence in a human subject. Limited clinical experience indicates that left stellectomy decreases the potential for the fatal arrhythmias associated with this syndrome. Therapy should, however, follow certain guidelines. {Jblockade is the first treatment of choice, but left stellectomy is recommended if propranolol fails to control symptoms.
Correlation of Phono- and Apexcardiographic Findings with Tumor Motion in Left Atrial Myxoma Fttlflclsco G. COIIo, M.D .;• Emilio Marin, M.D., F.C.C.P .;•
Juan Tascon, M.D.;• Antonio Sanchez, M.D.;• tmd ]08e M. AgUDdo, M.D. ••
We bave analyzed die genesis of pbyakal sips Ia a CMe of prolapsiag left atrial myxoma by slmuleaaeoas pboao-, apex- and M-mode eebocardiognpby. Oar 8adlap eoa6rm a direct relationship of tumor movements with aotdalng in the upstroke of die apexeardlopam and with the protodlastolk "tumor plop."
eae••
From tbe Cardiolo_gy• and Intensive Services Ciadad Sanitaria, 1 o de Octubre, Madrid, Spain. ' &rmnt reqfllllll: Dt. GGrcltJ Corio, CludiJtl SanltariiJ 1• Ootubre, Madrld26, Spain
REFERENCES 1 Jervell A, Lange-Nielsen F: Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death. Am Heart J 54:59, 1957 2 Romano C, Gemme G, Pongiglione R: Aritmie cardiache rare dell'eta' pediatrica. Clin Ped 45:656, 1963 3 Ward OC: New familial cardiac syndrome in children. J lr Med Assoc54 :103, 1964 4 Frogatt P, James TN: Sudden unexpected deaths in infants. Evidence of a lethal cardiac arrhythmia. Ulster Med J 42:136, 1973 5 Schwartz PJ, Periti M, Malliani A: The long Q-T syndrome. Am Heart J 89:378, 1975 6 Yanowitz F, Preston JB, Abildskov JA: Functional distribution of right and left stellate innervation to the ventricles: Production of neurogenic electrocardJographic changes by unilateral alteration of sympathetic tone. Circ Res 18:146, 1966 7 Schwartz PJ, Snebold NG, Brown AM: Effects of unilateral cardiac sympathetic denervation on the ventricular &hfillation threshold. Am J Cardiol37: 1034, 1976 8 Schwartz PJ, Stone HL, Brown AM: EfFects of unilateral stellate ganglion blockade 011 tbe arrhythmia aaociated with coronary occlusion. Am Heart J 92:589, 1976
•
COSIO ET Al
I . ..
: t -;:~ i.-ti ~Jir" '"'~
.
_±- . - ~~
FrotJBB 1. Simultaneous electrocardiogram, phonocardiogruu. apexcardiogram and mitral echocardiogram. Firat DOtch OD upstroke of apexcardiogram coincides with tumor motioD away &om left ventricle at Olll8t of systole ( STM) and with first component of first heart IOUJ1Cl ( S1 ). ·Secoud notch occura at time of mitral valve closure and seCoud component of 8nt heart IOUDd. Protodiastolfc eound (TS) coincides with mulmum diadolfc tumor prolapse ( DTM) and precedes 0 poiDt of apexcardfogram.
CHEST, 74: 5, NOVEMBER, 1918
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
F.tCUBB 1. Standard lead 2 rhythm sbip prior to surgery. Note sinus bradycardia characteristic of this syndrome: QT interval is prolonged to 0.54 sec.
.
- , - r-·
,.
I
~
- :.J
I
I
l
l.
k.
1' -
I- r-r-
FIGUI\E 2. Intraoperative rhythm sbip during manipulation of left stellate ganglion showing numerous premature venbicular beats of varied origin and run of ventricuJar tachycardia; ectopy ceased when manipulation ended.
FIGURE 3. Standard lead 2 rhythm sbip after surgery. Note persistence of sinus bradycardia, but normalization of QT interval. QT has remained normal one year postoperatively.
attacks persisted. In October, 1974, whUe playing football, he experienced his first episode of prolonged collapse requiring cudiopuJmonary resuscitation. Afterward he had two subsequent attacks, both requiring cardiopulmonary resuscitation. Following his last attack, surgical intervention was deemed advisable. Figure 1 represents his ECG prior to surgery: note the QT prolongation to 0.54 sec. On Feb 10, 1977, the left cervical sympathetic ganglia cham was exposed. Physical manipulation of the steDate ganglia (traction. clamping, etc) produced dramatic wntricular Irritability and arrhythmias. One of these arrhythmias is shown in Figure 2. The stellate ganglia in its entirety and the first four thoracic sympathetic ganglia were removed. Figure 3 demonstrates the patient's QT interval after surgery. His QT interval normalized and has remained normal since surgery one year ago. More importantly, he has remained totally free of syncopal attaclcs. He remains on pblockade therapy. No LQTS was found in any family members. Concurring with the experience of Frogatt and James,• this patient demonstrates that this seemingly congenital and usually familial syndrome may occur as an isolated case. DISCUSSION
Therapy for the LQTS has taken many forms. Varying drug regimens including oxygen, potassium, diphenylhydantoin, digitalis preparations, hyoscyamine, phenobarbital and even quinidine have all been used alone and in combination with disappointing results. Cardiac pacing has been tried without significant effect. p-blocking agents have been highly effective. The only large series ( 300 Q&Ses) revealed a 78 percent untreated mortality, a 60 percent mortality in those treated with non-p blockade, but a tenfold reduction in mortality to 6
CHEST, 74: 5, NOVEMBER, 1978
percent in those treated with p blockade. (Personal communication, Peter J. Schwartz, 1977) . Despite this dramatic improvement, 6 percent of the patients are unresponsive to p-blockade, leaving them vulnerable to repeated syncopal attacks and sudden death. Even among responders, the possible untoward effects of lifelong p-blockade are not known. Also, pblockade may result in many side-effects requiring lifestyle alterations. In several cases, patients who inadvertently missed medication were exposed to potentially fatal recurrent attacks." The etiology of the LQTS is not fully understood, but recent research implicates the role of sympathetic imbalance in the innervation of the heart as the cause. The present case offers further evidence for this etiology. The autonomic innervation of the heart includes both sympathetic and parasympathetic fibers, but only sympathetic fibers terminate in the ventricular musculature. These sympathetic fibers originate from the superior, middle and inferior (stellate) cervical ganglia in conjunction with the first four or five thoracic sympathetic ganglia. Much investigative work has been accomplished manipulating various segments of this innervation. Reports" reveal a prolongation of the QT interval with blockade or removal of the right stellate ganglion, whfle shortening of the QT interval has occurred with blockade or removal (LSGR) of the left stellate ganglion. Although not without some contradictory evidence, a congenital "'underactivity" of the right-half or an •overactivity.. of the left-half of this system seem to be plausible explanations. Further substantiation of these possible_ etiologies has recently been reported by Schwartz et al,'
LEFT STELLECTOMY IN LONG QT SYNDROME 585
in animal models. Their work has demonstrated a lowering of the ventricular fibrillation threshold by stellate ganglion manipulations in dogs, possibly implying a similar situation in these patients. To date, ten patients have undergone LSGR, ours being the tenth. Although followup has been relatively short (one to six years), cessation of syncopal attacks occurred in an patients, although QT interval shortening on ECG has not occurred in all cases. Some patients also remain on {Jblockade therapy and therefore possibly benefit from both forms of treatment. To date, problems with denervation hypersensitivity or with nerve regeneration have not been reported. The LQTS may be more common than suspected and currently is often overlooked. There are many ramifications: sudden death in infants, the crib deaths, may be one example. Whether the LQTS is responsible remains to be fully investigated. The possible fatal complications associated with the LQTS demand recognition and therapy early in life. This early recognition and therapy is often needed to prevent sudden death, carrying the patient into adulthood where spontaneous improvement has been noted. Further implications of this syndrome have been pointed out by Schwartz and co-workers• in experiments with ventricular fibrillation threshold and arrhythmias in the setting of coronary occlusion, perhaps pointing to new considerations in coronary artery disease. The in vivo production of life-threatening arrhythmias demonstrated here is the first such evidence in a human subject. Limited clinical experience indicates that left stellectomy decreases the potential for the fatal arrhythmias associated with this syndrome. Therapy should, however, follow certain guidelines. {Jblockade is the first treatment of choice, but left stellectomy is recommended if propranolol fails to control symptoms.
Correlation of Phono- and Apexcardiographic Findings with Tumor Motion in Left Atrial Myxoma Fttlflclsco G. COIIo, M.D .;• Emilio Marin, M.D., F.C.C.P .;•
Juan Tascon, M.D.;• Antonio Sanchez, M.D.;• tmd ]08e M. AgUDdo, M.D. ••
We bave analyzed die genesis of pbyakal sips Ia a CMe of prolapsiag left atrial myxoma by slmuleaaeoas pboao-, apex- and M-mode eebocardiognpby. Oar 8adlap eoa6rm a direct relationship of tumor movements with aotdalng in the upstroke of die apexeardlopam and with the protodlastolk "tumor plop."
eae••
From tbe Cardiolo_gy• and Intensive Services Ciadad Sanitaria, 1 o de Octubre, Madrid, Spain. ' &rmnt reqfllllll: Dt. GGrcltJ Corio, CludiJtl SanltariiJ 1• Ootubre, Madrld26, Spain
REFERENCES 1 Jervell A, Lange-Nielsen F: Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death. Am Heart J 54:59, 1957 2 Romano C, Gemme G, Pongiglione R: Aritmie cardiache rare dell'eta' pediatrica. Clin Ped 45:656, 1963 3 Ward OC: New familial cardiac syndrome in children. J lr Med Assoc54 :103, 1964 4 Frogatt P, James TN: Sudden unexpected deaths in infants. Evidence of a lethal cardiac arrhythmia. Ulster Med J 42:136, 1973 5 Schwartz PJ, Periti M, Malliani A: The long Q-T syndrome. Am Heart J 89:378, 1975 6 Yanowitz F, Preston JB, Abildskov JA: Functional distribution of right and left stellate innervation to the ventricles: Production of neurogenic electrocardJographic changes by unilateral alteration of sympathetic tone. Circ Res 18:146, 1966 7 Schwartz PJ, Snebold NG, Brown AM: Effects of unilateral cardiac sympathetic denervation on the ventricular &hfillation threshold. Am J Cardiol37: 1034, 1976 8 Schwartz PJ, Stone HL, Brown AM: EfFects of unilateral stellate ganglion blockade 011 tbe arrhythmia aaociated with coronary occlusion. Am Heart J 92:589, 1976
•
COSIO ET Al
I . ..
: t -;:~ i.-ti ~Jir" '"'~
.
_±- . - ~~
FrotJBB 1. Simultaneous electrocardiogram, phonocardiogruu. apexcardiogram and mitral echocardiogram. Firat DOtch OD upstroke of apexcardiogram coincides with tumor motioD away &om left ventricle at Olll8t of systole ( STM) and with first component of first heart IOUJ1Cl ( S1 ). ·Secoud notch occura at time of mitral valve closure and seCoud component of 8nt heart IOUDd. Protodiastolfc eound (TS) coincides with mulmum diadolfc tumor prolapse ( DTM) and precedes 0 poiDt of apexcardfogram.
CHEST, 74: 5, NOVEMBER, 1918