Ann Thorac Surg 2000;69:628-630
© 2000 The Society of Thoracic Surgeons
Case Reports
Weaning from mechanical support in a patient with acute heart failure and multiple sclerosis
Ulf W. Kjellman, MDa,
Per Hallgren, MDb,
Claes-Håkan Bergh, MDb,
Jan Lycke, MDc,
Anders Oldfors, MDd,
Lars Wiklund, MDa
a Department of Cardiothoracic Surgery, Sahlgrenska University Hospital, Göteborg, Sweden
b Department of Cardiology, Sahlgrenska University Hospital, Göteborg, Sweden
c Department of Neurology, Sahlgrenska University Hospital, Göteborg, Sweden
d Department of Pathology, Sahlgrenska University Hospital, Göteborg, Sweden
Address reprint requests to Dr Wiklund, Department of Cardiothoracic Surgery, Sahlgrenska University Hospital, S-413 45 Göteborg, Sweden
e-mail: lars.wiklund{at}medfak.gu.se
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Abstract
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We describe a 19-year-old woman developing acute left ventricular heart failure during her first exacerbation of multiple sclerosis. Histopathologic examination of myocardial tissue showed extensive myocytolysis. A left ventricular assist device was implanted. Three months later the cardiac function was restored and the left ventricular assist device was explanted. After 1 year the patient still remains well and her cardiac function is normal.
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Introduction
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We describe acute left ventricular heart failure developing in a young woman during her first exacerbation of multiple sclerosis that was successfully treated with a left ventricular assist device (HeartMate; Thermo Cardiosystems, Inc, Woburn, MA).
A 19-year-old previously healthy woman, with a normal delivery 9 months earlier, was admitted with findings of acute heart failure. During the 3 preceding weeks the patient had developed neurologic symptoms and signs of a progressive brainstem syndrome with headache, vertigo, impaired balance, nausea, fatigue, and left-sided sensory disturbances. The neurologic examination revealed dysesthesia of the left side of the face and the left arm, a right-sided pharyngeal paresis, and left side deviation of the tongue. Magnetic resonance imaging (MRI) demonstrated multiple periventricular and subcortical lesions of the brain as well as one lesion of the right side of the medulla oblongata. The neuroophthalmologic findings and examination with visual-evoked potentials revealed signs of left optic neuritis.
The patient deteriorated with clinical signs of autonomic dysfunction and progressive heart failure. By the time of admission the patient had developed severe left ventricular heart failure. Chest roentgenogram showed severe pulmonary edema with a normal heart size. Echocardiography demonstrated a slightly enlarged dysfunctional left ventricle with a left ventricular ejection fraction less than 0.10 (Table 1). The right ventricular pressure was normal. Further invasive hemodynamic data on admission are shown in Table 2.
Histopathologic examination of endomyocardial biopsies revealed no abnormality. Immunostaining for cytomegalovirus was negative as well as various tests for viruses and bacteria.
Cardiogenic shock with signs of multiorgan failure developed in the patient, and an left ventricular assist device (HeartMate) was successfully implanted. Histologic examination of the excised myocardial specimen from the left ventricular apex showed an extensive myocytolysis but few inflammatory cells. After the implantation the cardiac function improved and the neurologic symptoms slowly resolved. Liver and kidney function tests were normalized. Three weeks after left ventricular assist device implantation a drive-line infection (Staphylococcus aureus) was diagnosed. Eight weeks after implantation the patient experienced a relapse of neurologic symptoms and the diagnosis of multiple sclerosis [1] was confirmed. This diagnosis was based on findings of intrathecal immunoglobulin synthesis in the cerebrospinal fluid.
After the recovery phase of her multiple sclerosis relapse, about 2.5 months after implantation, an invasive hemodynamic evaluation was made. The patient was heparinized (300 U/kg) and the investigation was performed with the left ventricular assist device in the on and off position, both at rest and during supine bicycle exercise with a slight workload. The overall results showed in summary recovery of left ventricular function (Table 2). Because of increasing infectious problems with repeated periods of sepsis despite antibiotic treatment, the device was explanted 83 days after implantation. The cardiac function was normalized and remains so today, 1 year after explantation. The patient is now in excellent clinical condition and in New York Heart Association functional class I. She is not taking any pharmacologic therapy for heart failure, and so far she has not experienced any more relapses of multiple sclerosis.
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Comment
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This case report illustrates a successful temporary treatment with an implantable mechanical assist device in a young woman with severe acute heart failure, at the onset of multiple sclerosis, unresponsive to conventional inotropic support. In the literature multiple sclerosis is rarely associated with acute heart failure [2]. More common causes of acute heart failure in a young woman similar to this case are myocarditis and peripartum cardiomyopathy [3]. However, the sparse number of inflammatory cells in the endomyocardial biopsies does not support the diagnosis of acute myocarditis, and the time of onset of symptoms in relation to the time of delivery 9 months earlier does not support the diagnosis of peripartum cardiomyopathy [4]. There was no history of hypertension or endocrine disorders, which could be of etiologic importance.
Implantation of left ventricular assist device has frequently saved the lives of patients waiting for heart transplantation [5, 6]. The devices are mainly used as bridge to transplantation. However, in a number of patients, mainly with dilated cardiomyopathy or myocarditis, the devices have been used for temporary support as a bridge to recovery for a shorter or longer period [7]. In this context, a critical issue is how to assess the improvement in myocardial function to find the optimal time point for explantation of the device. The duration of support must not be too short because of the risk of relapsing heart failure and not too long, as complications and the durability of the device are time dependent [6]. Recording of myocardial function for short periods with the device turned off is one way to evaluate recovery of myocardial function, but it may be associated with increased risk of thromboembolic events. However, it is of utmost importance to establish reliable tests of native cardiac function to be able to decide whether weaning or to continue with heart transplantation. In selected patients with idiopathic dilated cardiomyopathy the levels of anti-ß1 adrenoreceptor autoantibodies has been found to monitor recovery [8]. However, this could not be used in this patient as the levels of autoantibodies were normal and we found no other marker for recovery.
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References
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Melin J., Usenius J.-P., Fogelholm R. Left ventricular failure and pulmonary edema in acute multiple sclerosis. Acta Neurol Scand 1996;93:315-317.[Medline]
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Accepted for publication June 26, 1999.
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Abstract
Introduction
