Volume 129, Number 2 American Heart.Journal Wells et al. 4 0 9

Fig. 5. Histologic findings revealed diffuse lymphocytic infiltration with red blood cells in tumor tissues (Hematoxillin-eosin, original magnification ×20).

trative lesions and that recurrence is possible if the resec- tion is incomplete. 2, 4 In our case, the patient 's history of an axillary tumor raises the possibility that the mediastinal tumor might have existed as a slowly growing residual le- sion. Another unique feature of our case is the sudden in- tracavitary hemorrhage. 5 We speculate that the inflamma- tory process may have played a role in triggerring hemor- rhage. Although Pasaoglu et al. 6 reported a successfully resected case of cardiac lymphangioma, our pat ient is the first reported to undergo emergency surgery because of the development of symptoms caused by the rapid growth of tumor after the intracavitary hemorrhage. To prevent these serious complications and recurrence, complete sur- gical resection is recommended when the diagnosis of lym- phangioma is made.

REFERENCES

1. Wychulis AR, Payne WS, Clagett OT, Woolner LB. Surgical treatment of mediastmal tumors: a 40-year experience. J Thorac Cardlovasc Surg 1971;62:379-92.

2. Bill AH, Sumner DS. A unified concept of lymphangioma and cystic hygroma. Surg Gynecol Obstet 1965;120:79-86

3. Grosfeld JL, Weber TR, Vane DW. One-stage resection for massive cervicomediastinal hygroma. Surgery 1982;92:693-9.

4. Grenadier E, Margulis T, Palant A, Safadi T, Merin G. Huge cavernous hemangioma of the heart: a completely evaluated case report and review of the literature. AM HEART J 1989;117:479-81.

5. Brown LR, Reiman HM, Rosenow III EC, Gloviczki PM, Divertie MB. Intrathoracic lymphangioma. Mayo Clin Proc 1986,61:882-92.

6. Pasaoglu I, Dogan R, Ozme S, Kale G, Bozer AY. Cardiac lymphangi- oma. AM HEART J 1991;121:1821-4.

Reversibility of severe left ventricular dysfunction in patients with subarachnoid hemorrhage

Calvin Wells, MD, a Bibiana Cujec, MD, a David Johnson, MD, b and Gary Goplen, MD c Saskatoon, Saskatchewan, Canada

Sympathetic nervous system and adrenal medullary acti- vation in patients with subarachnoid hemorrhage 1 may re- sult in catecholamine-mediated myocardial injury. Patho- logic changes in the myocardium of patients dying of sub- arachnoid hemorrhage include focal myocytolysis and myofibrillar degeneration. 2, 3 Left ventricular wall motion abnormalities are found by echocardiography in 10 % to 30 % 4-6 of patients with subarachnoid hemorrhage and tend to occur more commonly in patients with severe neurologic deficits. 4, 5 There is, however, little documentation in the English l i terature of the reversibility of left ventricular

From the Divisions of aCardiology and bCritical Care Medicine, Department of Medicine, and the CDivision of Neurosurgery, Department of Surgery, Royal University Hospital, University of Saskatchewan. Reprint requests: Bibiana Cujec, MD, Division of Cardiology, Royal University Hospital, Saskatoon, Saskatchewan, Canada S7N 0X0. AM HEART J 1995;129:409-12. Copyright ® 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 4/4/59831

February 1995 410 We/L~ et aL American Heart Journal

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Fig. 1. Patient 1. Chest radiograph shows bilateral peri- hilar opacification consistent with pulmonary edema.

systolic dysfunction in patients with subarachnoid hemor- rhage. This has important implications for prognosis and for cardiac donation should brain death ensue. We present two patients with pulmonary edema caused by subarach- noid hemorrhage who had resolution of severe left ventric- ular systolic dysfunction over a period of I to 2 weeks.

Case 1. A 40-year-old native American Indian woman had acute dyspnea after a generalized seizure. Physical ex- amination showed her to be drowsy and disoriented with- out any focal neurologic deficits. She was in severe respira- tory distress with audible wheezing. Her blood pressure was 120/80 mm Hg, heart rate 110 beats/min, respiratory rate 46 breaths/min. The jugular venous pressure was elevated. Heart sounds were faint. Chest examination revealed bibasilar inspiratory crackles and diffuse wheezes. Elec- trocardiogram revealed sinus tachycardia with abnormal left-axis deviation and T-wave inversion in leads I and aVL. Chest radiograph showed perihilar alveolar infiltrates con- sistent with pulmonary edema (Fig. 1). The patient was mechanically ventilated. The right atrial and pulmonary capillary wedge pressures were elevated at 17 mm Hg and 19 mm Hg, respectively, and the cardiac index by ther- modilution was severely decreased at 1.5 L/min/m 2. She was treated with dobutamine and furosemide. Computed tomography of the brain showed hyperdense material in all cortical sulci and basilar cisterns consistent with subarach- noid hemorrhage. Cerebral angiography revealed two an- eurysms of the left internal carotid artery. Two-dimen- sional echocardiogram (Hewlett Packard 77027A, An- dover, Mass.) performed on the day of admission revealed severe global left ventricular hypokinesia. Only the basal segments of the posterior and lateral walls contracted nor- mally. Left ventricular fractional shortening was 10 %, and estimated ejection fraction was 20 %. Repeat echocardio- gram on the third hospital day showed marked improve-

merit in left ventricular contraction. The left ventricular fractional shortening was 26 %, and the estimated ejection fraction was 50 %. The basal segments of the left ventricle and the apex contracted normally with residual hypokine- sia of the mid septum, mid lateral, and mid posterior wall segments. On the fifth hospital day, echocardiography re- vealed further improvement in left ventricular systolic function with resolution of septal hypokinesia and an esti- mated ejection fraction of 57 %. The peak total serum cre- atine kinase level was 232 IU/L (normal <180 IU/L) on the second hospital day with absence of myocardial isoenzyme. Clipping of the cerebral aneurysms on the thirteenth hos- pital day was complicated by intraoperative rupture and left frontoparietal infarction. After prolonged rehabilita- tion, the patient was discharged with residual aphasia and right hemiplegia.

Case 2. A 25-year-old native American Indian woman had acute onset of severe headache and vomiting. Physical examination revealed normal vital signs. She was drowsy and disoriented to time and place. Cardiac examination was normal. She had nuchal rigidity and mild right spastic hemiparesis. Electrocardiogram and chest radiograph were normal. Computed tomography of the brain revealed a left frontal parenchymal hematoma with blood in all four ven- tricles. Cerebral angiography revealed an 8 mm aneurysm at the bifurcation of the left internal carotid artery. The aneurysm was clipped on the tenth hospital day. Postop- eratively, she had diabetes insipidus with decreased level of consciousness from severe spasm of the left internal ca- rotid artery. She was treated with nimodipine, intravascu- lar volume expansion, induced systemic arterial hyperten- sion, and mechanical ventilation. Right atrial pressure ranged from 1 to 4 mm Hg, and her arterial systolic pres- sure was 150 to 160 mm Hg while receiving intravenous in- fusions of phenylephrine and norepinephrine. She had se- vere pulmonary edema on the second postoperative day. The right atrial and pulmonary capillary wedge pressures were elevated at 14 mm Hg and 19 mm Hg, respectively, with a depressed cardiac index of 1.94 L/min/m 2. She was treated with fluid restriction, amrinone, dopamine, and norepinephrine. The serum creatine kinase level was ele- vated at 442 IU/L on the second postoperative day with a myocardial isoenzyme of 10 IU/L. An echocardiogram (Aloka 870, Tokyo, Japan) on the second postoperative day revealed severe global left ventricular hypokinesia (Fig. 2, A and B). The left ventricular ejection fraction by single- plane area-length method was 26%. Echocardiogram on the fifth postoperative day did not show change in left ventricular function despite radiologic resolution of the pulmonary edema. A third echocardiogram on the ninth postoperative day, revealed marked improvement in left ventricular function with an ejection fraction of 43 % and fractional shortening of 27 %. A final echocardiogram on the sixteenth postoperative day (2 weeks after the devel- opment of pulmonary edema) showed normalization of global left ventricular systolic function with an ejection fraction of 58 % (Fig. 2, C and D). There was minimal re- sidual hypokinesia of the inferior and lateral walls. The patient was discharged with resolving cognitive deficits.

Volume 129, Number 2 American Heart Journal ~tTeIL~ et aL 411

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Fig. 2. Patient 2. Echocardiographic parasternal long-axis views. A and B, There is severe left ventricular systolic dysfunction with minimal change in left ventricular internal dimensions between end diastole (A) and end systole (B). C and D, 2 weeks later, left ventricular systolic function is normal. C, End-diastolic frame; D, end-systolic frame.

There is a paucity of information on the natural history of left ventricular systolic dysfunction in patients who sur- vive a subarachnoid hemorrhage. This is probably the re- sult of the more severe neurologic involvement and higher mortality rate in these patients. In an echocardiographic study of 13 patients with subarachnoid hemorrhage, Pol- lick et al. 4 found left ventricular wall motion abnormalities in four patients. Two of these four patients had improved left ventricular function over the course of hospitalization. In a Japanese study, 9 of 99 patients with subarachnoid hemorrhage and abnormal electrocardiograms had wall motion abnormalities detected by echocardiography. 6 Seven of these nine patients had pulmonary edema associ- ated with increased pulmonary capillary wedge pressures. Left ventricular dysfunction improved markedly over the course of hospitalization. There is also echocardiographic evidence of reversibility of diffuse left ventricular hypo- kinesia in transplanted hearts from brain-dead donors with subarachnoid hemorrhage. 7

Our cases illustrate rapid and complete resolution of se- vere left ventricular systolic dysfunction in two patients with subarachnoid hemorrhage. In both cases, the myocar-

dial dysfunction resulted in pulmonary edema and respi- ratory failure. In the first case, the respiratory failure masked the symptoms of the subarachnoid hemorrhage and led to an initial diagnosis of asthma. In the second case, severe vasospasm of the left internal carotid artery after aneurysm clipping likely resulted in hypothalamic ische- mia, which precipitated an intense sympathetic nervous system and catecholamine discharge, which caused myo- cardial injury. The lack of significant rise of the creatine kinase myocardial isoenzyme in both patients is consistent with minimal myocardial necrosis. Neither patient was treated with ~-blockers, which may prevent catechola- mine-induced myocardial injury. The severity of the left ventricular systolic dysfunction and the time course of re- covery is suggestive of myocardial stunning. Although cor- onary angiography was not performed in either patient, the absence of risk factors for atherosclerosis in these two pre- menopausal women makes myocardial ischemia very un- likely. There was no significant increase in myocardial loading conditions that could account for the depressed systolic function. Left ventricular systolic dysfunction should be suspected when hemodynamic instability or

February 1995 41 2 Pinamonti et aI. American Heart Journal

pulmonary edema develops in a pat ient with subarachnoid hemorrhage. Even severe left ventricular systolic dysfunc- tion in this setting may be reversible.

REFERENCES

1. Neil-Dwyer G, Cruickshank JM, Stott A, Brice J. The urinary cate- cholamine and plasma cortisol levels in patients with subarachnoid hemorrhage. J Neurol Sci 1974;22:375-82.

2. Connor RCR. Heart damage associated with intracranial lesions. B M J 1968;3:29-31.

3. Doshi R, Nefl-Dwyer G. A clinicopathological study of patients follow- ing a subarachnoid hemorrhage. J Neurosurg 1980;52:295-301.

4. Pollick C, Cujec B, Parker S, Tator C. Left ventricular wall motion ab- normalities in subarachnoid hemorrhage: an echocardiographic study. J Am Coll Cardiol 1988;12:600-5.

5. Davies KR, Gelb AW, Manninen PH, Boughner DR, Bisnane D. Car- diac function in aneurysmal subarachnoid haemorrhage: a study of electrocardiographic and echocardiographic abnormalities. Br J Anaesth 1991;67:58-63.

6. Sato K, Masuda T, Kikuno T, Kobayashi A, Ikeda Y, Ohwada T, Kikawada R. Left ventricular asynergy and myocardial necrosis accom- panied by subarachnoid hemorrhage: contribution of neurogenic pul- monary edema [in Japanese]. J Cardiol 1990;20:359-67.

7. Sefler C, Laske A, Gallino A, Turina M, Jenni R, Echocardiographic evaluation of left ventricular wall motion before and after heart trans- plantation. J Heart Lung Transplant 1992;11:867-74.

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Long-term evolution of right ventricular dysplasia-cardiomyopathy

Bruno Pinamonti , MD, a Andrea Di Lenarda, MD, a Gianfranco Sinagra, MD, a Furio Silvestri, MD, b Rossana Bussani, MD, b Fulvio Camerini, MD, a and The Hear t Muscle Disease Study Group Trieste, Italy

Right ventricular dysplasia-cardiomyopathy (RVD) is a myocardial disorder of unknown cause that mainly involves the right ventricle. It is usually first seen clinically with ventricular arrhythmias of left bundle branch block mor- phologic changes, changes of repolarization on right pre- cordial leads, and localized or diffuse right ventricular contraction abnormalities. The pathologic findings are characterized by a progressive atrophy of right ventricular myocardium with adipose or fibroadipose substitution. 1

From the aDepartment of Cardiology and bInstitute of Pathology, Ospedale Maggiore and University. The Heart Muscle Disease Study Group: Department of Cardiology, Ospedale Maggiore and University: Ezio Alberti, MD, Andrea DiLenarda, MD, Gerardina Lardieri, MD, Tullio Morgera, MD, Andrea Perkan, MD, Bruno Pinamonti, MD, Serena Rakar, MD, Alessandro Salvi, MD, Gian- franco Sinagra, MD, Massimo Zecchin, MD, and Fulvio Camerini, MD. In- ternational Center for Genetic Engineering and Biotechnologies: Mauro Giacca, MD, Luisa Mestroni, MD, Giammaria Severini, MSc, and Arturo Falaschi, MD. Department of Morbid Anatomy, University of Trieste and Siena, Italy: Rossana Bussani, MD, Piero Tanganelli, MD, and Furio Silvestri, MD. Reprint requests: Bruno Pinamonti, MD, Divisione di Cardiologia, Ospe- dale Maggiore, 34129 Trieste, Italy. AM HEART J 1995;129:412-5. Copyright ® 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 4/4/59830

Fig . 1. Pathologic findings of explanted heart of patient with RVD transplanted for refractory heart failure at age of 57 years. She was first seen clinically 17 years earlier with sustained ventricular tachycardia. At macroscopic exami- nation (A) massive fatty infiltration of right ventricular free wall (RV) is evident. Fat ty infiltration is also present at level of interventricular septum (right side), apex, and free wall of left ventricle (arrows). Histologic specimen of right ventricular free wall (B) shows transmural severe fatty in- filtration, subendocardial fibrosis (arrow), and very few myocardial cells (hematoxylin-eosin, original magnifica- tion ×10).

Although the clinical, electrocardiographic (ECG), echocar- diographic, angiographic, and pathologic characteristics of RVD were already described, 1, 2 few data are presently available about long-term evolution of the disease. 35

To assess the evolution and prognosis, we studied the follow-up of a series of 45 consecutive patients with RVD. Twenty-seven of them were males and 18 were females, with a mean age at clinical presentation of 32 years (range 4 months to 59 years). In 43 cases the diagnosis was made clinically and by means of two-dimensional echocardiogra- phy and/or angiography according to current criteria in the presence of localized (akinetic/dyskinetic areas or discrete wall bulges) and/or diffuse (global dilatation and hypo- kinesia) right ventricular abnormalities and in the absence of any other identifiable structural heart or pulmonary disease. 2 Patients with minor changes of left ventricular function (ejection fraction 45 % to 51%) in association to