Title: Dilated cardiomyopathy in a 16-year-old girl
Authors: A N. Okhotin 1, M A. Osipov 1
(1) Tarusa district hospital, Tarusa, Russian
Federation
Topic: 03.11 - Cardiomyopathies
Dilated cardiomyopathy in a tachycardia was diagnosed with occasional normal sinus
beats (one of each 15-20 beats, fig. 3).
16-years-old girl
What would be the most appropriate treatment?
Presentation
• Consider medical treatment
A 16-year-old girl was admitted to our hospital with
progressive exertional dyspnea. She was semi- • Referral to heart transplantation
professional sport dancer and was in good health until a • Referral to invasive electrophysiological
year ago when she began experiencing heartbeating and treatment
dyspnea on exertion. For near a month dyspnea worsens
and she sought for medical advice. No prior medical We referred the patient to electrophysiologycal
records or ECGs were available. laboratory, but electrophysiologists considered ablation
On admission she had regular heart rate of 150 beats per unsafe due to very poor left ventricular contractility.
minute, arterial blood pressure of 90/60 mm Hg, systolic Indeed, heart rate reduction to 60-70 beats per minute
murmur of 2/6 grade, her SpO2 was 98%. She had no with ejection fraction of 10% and LV volume of 354 ml
resting dyspnea, no rales and no peripheral edema. ECG would put the patient into a very low cardiac output state.
showed regular rhythm with P waves interpreted as sinus However, we believed that dilated cardiomyopathy was
or atrial tachycardia and signs of left ventricular due to tachycardia alone and that the only chance to cure
hypertrophy (fig. 1). Echocardiography was performed the patient was to slower her heart rate. Medical
and severe dilation and systolic dysfunction of left treatment with bisoprolol and digoxin was ineffective.
ventricle were revealed (fig. 2, loops #1, #2), with
marked eccentric left ventricular hypertrophy, mild-to-
moderate secondary mitral regurgitation and left atrial What else we can do to persuade
enlargement, moderate pulmonary hypertension. No electrophysiologists to treat patient invasively?
septal defects, valvular lesions or other signs of • Cardiac MRI to assess irreversible myocardium
congenital heart disease were found. Left ventricle end- fibrosis?
diastolic volume was 354 ml, ejection fraction was
approximately 10%, corresponding to cardiac output of • Stress-echocardiography with dobutamine to
5.31 l/min and cardiac index of 3.38 l/min/m2, dP/dT was assess contractility reserve?
533 mm Hg/s, left ventricle myocardium mass was 337 g
• Accurate analysis of post-sinus beats on
and left ventricle myocardium mass index was 215 g/m 2.
echocardiography?
Right ventricle was only moderately dilated, its end-
diastolic dimension being 4.6 mm. Standard evaluation Accurate analysis of post-sinus beats on
(CBC, ESR, creatine kinase, creatinine, liver function echocardiography showed much better contractility as
tests, iron, TSH, electrolytes) revealed no abnormalities. compared with regular tachycardic beats (loop #3). This
Therefore, our patient met criteria for dilated finding encouraged electrophysiologists to perform
cardiomyopathy. electrophysiological study. Automatic focus of abnormal
activity was found in right upper pulmonary vein and
Questions successfully ablated. Patient's symptoms improved
dramatically, in a few days left ventricular ejection
fraction raised up to 20% (fig. 4, loop #4, #5). Six
What should be the next step? months later ejection fraction raised to 48% and end-
• Coronary angiography? diastolic left ventricular volume decreased to 169 ml, left
ventricular mass decreased as well to 201 g (fig. 5, loops
• 24-hour ECG? #6, #7).
• Endomyocardial biopsy?
• Medical treatment without further evaluation? Conclusion
• Referral to heart transplantation without further Thus, we have the case of pure tachycardia-induced
evaluation? dilated cardiomyopathy, successfully treated with
radiofrequency ablation.
24-hour ECG was performed and incessant atrial
Figure 1. ECG at presentation Figure 2. Echocardiography at presentation. Short axis of left
ventricle, end-diastolic (A) and end-systolic (B). Apical four
chamber, end-diastolic (C) and end-systolic (D).
Figure 3. Strip from 24-hours ECG with 2 "pauses"
Figure 4. Echocardiography just after treatment. Short axis of left Figure 5. Echocardiography 6 months after treatment. Short axis of
ventricle, end-diastolic (A) and end-systolic (B). Apical four left ventricle, end-diastolic (A) and end-systolic (B). Apical four
chamber, end-diastolic (C) and end-systolic (D). chamber, end-diastolic (C) and end-systolic (D).