Is Pickelhaube Sign Really the Hallmark of Arrhythmogenic MVP in Athletes? And Does MVP Really Cause Sudden Death? A Case Report

Abstract

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.

Introduction

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.

Case Report

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, and was then lost in the follow up with filed practice not having delivered the required tests.