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Improving the quality of life and reducing sudden cardiac death by limiting the impact of heart rhythm disturbances.
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A 40 -year-old woman was admitted due to palpitation during the third trimester of pregnancy. She had been previously healthy except for asthma and had no regular medication. On examination she was found to have sinus tachycardia (102 bpm), assumed to be caused by pregnancy. Two months later she gave birth to a healthy infant, her second child.
After few months she began to experience short tachyarrhythmias. She was admitted to the Emergency Department because of a sustained supraventricular tachycardia with a rate exceeding 200 beats per minute (Figure 1). There was no response to intravenous adenosine. DC cardioversion restored sinus rhythm, then SVT started again and sinus rhythm was restored with flecainide infusion (Fig.2). Except for slightly elevated troponin T level, standard laboratory analyses were normal. Echocardiography revealed left ventricular dysfunction with left ventricle end diastolic diameter of 61mm, normal wall thicknesses and LVEF was 42-48% (Fig 3 ).
Postpartum cardiomyopathy or cardiomyopathy caused by tachyarrhythmia were suspected. The patient was treated with bisoprolol. However, within the next three months the tachyarrhythmias recurred. They were treated with flecainide and bisoprolol. The ECG showed a first degree AV block while the QRS complex remained at 110 ms. Echocardiography was repeated several times. It showed that LVEF had decreased to 30-40% and an ACE-inhibitor was initiated.
The findings of endomyocardial biopsy (4 samples) were non-specific. Electrophysiologic study revealed a rare variant of intranodal re-entrant tachycardy and catheter ablation was attempted. After that, the patient still had tachyarrhythmias and as a new phenomenon, short ventricular tachycardias, at a speed of 170 bpm. Because the patient had additionally Wenckebach-type of atrioventricular conduction block and presyncope, she received a DDD pacemaker.
Eventually, one and a half years after the initial diagnosis, the patient was feeling relatively well. The serum pro-BNP level was normal, LVEDD was 64mm and LVEF 55%. The medical treatment consisted of a betablocker, an ACE-inhibitor and flecainide. The situation started to deteriorate again after three months. The patient complained fatigue. Correspondingly, the serum pro-BNP level was remarkably elevated, echocardiography showed enlarged left ventricle (LVEDD 70mm), significant mitral valve regurgitation and LVEF was 30%.
The atrioventricular conduction defect progressed to a third degree AV block. Later, almost three years after the initial diagnosis, the patient had a sustained ventricular tachyarrhythmia. Sinus rhythm was returned by DC cardioversion.
What is unusual in the clinical course of the disease? What clinical investigations would you suggest? What would be the appropriate management of this patient at this time?
Initially, the patient had left ventricular enlargement and mild systolic dysfunction compatible with beginning dilated cardiomyopathy (DCM) (Fig.1). The ECG showed sinusrhythm and first degree atrioventricular block (Fig. 2). The patient did not have hypertension or any valve disease, neither were there any signs of coronary artery disease. Her medical history or clinical examination did not suggest any other specific non-genetic etiology either. The family history was negative but because the first degree relatives were not examined, the possibility of a familial DCM could not be excluded. The timing of the symptoms was suggestive of postpartum cardiomyopathy, a quite rare disease in Finland.
On the other hand, the newly-appeared supraventricular tachycardia and the progression of electrocardiographic changes combined with ventricular tachyarrhythmias and decreasing systolic function were slightly atypical of postpartum cardiomyopathy (1). The
ordinary laboratory analyses remained uninformative. Since possible differential diagnoses included giant cell myocarditis or cardiac sarcoidosis, endomyocardial biopsy (EMB) from the right ventricle was performed (four samples), the results remaining nondiagnostic (2). Accordingly, the patient was given medical treatment for heart failure
and arrhythmias. Due to bradycardia and second degree AV-block, the patient received a dual-chamber pacemaker.
Because heart failure and AV conduction defect progressed and there were both atrial flutter and ventricular tachycardias, invasive evaluation was repeated. The coronary arteries were normal. LVEF was 25-30% in left ventricle cineangiography. More than four years after the initial symptoms and three years after the diagnosis of DCM, EMB was repeated (eight samples), showing this time granulomatotic inflammation with giant cells, epithelioid cells and lymphocytes, compatible with sarcoidosis (3) and the case was also discussed locally (4). The patient did not have any extracardiac manifestations of sarcoidosis. Cardiac positron emission tomography (PET) combined with computed tomography showed an enlarged left ventricle but no obvious inflammation (5). Despite antiarrhythmic and immunosuppressive therapy (azathioprine and prednisolone) the disease progressed and lead to cardiac transplantation.
1. Cooper LT, Baughman KL, Feldman AM, Frustaci A, Jessup M, Kuhl U, Levine GN, Narula J, Starling RC, Towbin J, Virmani R. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Circulation. 2007;116:2216-33.
2. Chow LH, Radio SJ, Sears TD, McManus BM. Insensitivity of right ventricular endomyocardial biopsy in the diagnosis of myocarditis. J Am Coll Cardiol. 1989;14:915-20.
3. Mahrholdt H, Goedecke C, Wagner A, Meinhardt G, Athanasiadis A, Vogelsberg H, Fritz P, Klingel K, Kandolf R, Sechtem U. Cardiovascular magnetic resonance assessment of human myocarditis: a comparison to histology and molecular pathology. Circulation. 2004;109:1250-8.
4. Skouri HN, Dec GW, Friedrich MG, Cooper LT. Noninvasive imaging in myocarditis. J Am Coll Cardiol. 2006;48:2085-93.
5. Assomull RG, Lyne JC, Keenan N, Gulati A, Bunce NH, Davies SW, Pennell DJ, Prasad SK. The role of cardiovascular magnetic resonance in patients presenting with chest pain, raised troponin, and unobstructed coronary arteries. Eur Heart J. 2007;28:1242-9.
6. McCarthy RE, 3rd, Boehmer JP, Hruban RH, Hutchins GM, Kasper EK, Hare JM, Baughman KL. Long-term outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis. N Engl J Med. 2000;342:690-5.
7. Mason JW, O'Connell JB, Herskowitz A, Rose NR, McManus BM, Billingham ME, Moon TE. A clinical trial of immunosuppressive therapy for myocarditis. The Myocarditis Treatment Trial Investigators. N Engl J Med. 1995;333:269-75.
8. Cooper LT, Jr., Berry GJ, Shabetai R. Idiopathic giant-cell myocarditis--natural history and treatment. Multicenter Giant Cell Myocarditis Study Group Investigators. N Engl J Med.1997;336:1860-6.
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