Centre for Sports Cardiology - Internal General Medicine Department, AUSL della Romagna - District of Cesena, Italy
Corresponding author details:
Masimo Bolognesi
Centre for Sports Cardiology - Internal General Medicine Department
AUSL della Romagna -District of Cesena
Italy
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© 2020 Bolognesi M. This is an
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The Pickelhaube Sign is today recognized as a novel Echocardiographic Risk Marker for
Malignant Mitral Valve Prolapse Syndrome. In this short manuscript the author describes
the case of an asymptomatic and fit amateur 46-year old male cyclist who has a mitral valve
prolapse with mild to moderate mitral regurgitation. He also showed sporadic uncommon
Premature Ventricular Contractions (PVCs) at exercise stress test, and the Pickelhaube
sign during sports preparticipation screening. So, his eligibility for sports competition was
questionable.
Mitral Valve Prolapse (MVP) has long been recognized to be a relatively common valve abnormality in the general population [1,2]. Patients with relatively non-specific symptoms and asymptomatic athletes who have MVP still represent an important clinical conundrum for any physician involved in preventive medicine and sports screening[3].Although cardiac arrhythmias and/or cardiac death are an undesirable problem in MVP patients, when these subjects were studied with HolterElectrocardiogram (ECG) monitoring a prevalence of ventricular arrhythmias up to 34% was observed, with premature ventricular contractions as the most common pattern (66% of cases) [4]. At this regard a paper by Anders et al. described a series of cases that suggest that even clinically considered benign cases of MVP in young adults may cause sudden and unexpected death[5]. However, cardiac arrest and Sudden Arrhythmic Cardiac Death (SCD) resulted in rare events only in patients with MVP based on data from a community study[6].
For a long time the mysterious entity of the mitral valve prolapse has been the subject
of an always fruitful discussion among sports cardiologists in association with scientist and
experts of sudden cardiac death. This association between arrhythmogenic mitral valve
prolapse and sudden cardiac death of athletes carrying this congenital valve abnormality
has recently led many anatomopathologists in collaboration with cardiologists to report
some papers about malignant MVP.With this anedoctal case report the author gives
information about a typical situation that can occur in the setting of sport medicine and
sports preparticipation screening in everyday practice.
A middle-aged athletic male who has been practicing competitive cycling for about
20 years came to our Sports Medicine Centre to undergo screening of sports preparation
for competitive cycling and the related renewal of certification for participation in sports
competitions. This athlete was always considered suitable in previous competitive
fitness assessments performed in other sports medicine centers. His family history was
unremarkable, as well as his recent and remote pathological anamnesis. The physical
examination revealed a regurgitation heart murmur, 3/6 intensity, at the cardiac apex with
a click in the mid late systole. Previous echocardiographic examinations revealed a mitral
valve prolapse which was considered benign with mild mitral regurgitation hemodinamicly
not relevant. He did not complain of symptoms such as dyspnea or heart palpitations during
physical activity. The resting ECG (Figure 1) showed negative T waves in the inferior limb
leads, and the stress test showed sporadic premature ventricular beats (a couple) with right
bundle branch block morphology (Figure 2). An echocardiogram confirmed the presence of
a classic mitral valve prolapse with billowing of both mitral leaflets (Figure 3), associated
with a mild to moderate valve regurgitation. The TDI exam at the level of the lateral mitral
annulus showed a high-velocity mid-systolic spike (Figure 4) like a Pickelhaube sign, i.e.
spiked German military helmet morphology. Consequently, an in-depth diagnostic imaging
with cardiac magnetic resonance imaging was proposed, but the athlete refused it, both
because he was totally asymptomatic and above all because he would be forced to pay a
considerable amount of money as the examination is not guaranteed by the Italian National
Health Service. In conclusion, the athlete remained sub judice as for competitive suitability,
Figure 1: Resting ECG shows negative T waves in the inferior
limb leads