Author + information
- Published online December 21, 2016.
- S1936878X16307999-eb5fe10f385df6255542f57d96d65aa8Lakshmi Muthukumar, MD,
- S1936878X16307999-169729b3d7cab37a02a512864c0256ecFaraaz Rahman, MD,
- S1936878X16307999-4857b6e756c83861e82e4d63e85aea53M. Fuad Jan, MBBS, MD,
- S1936878X16307999-9bab451e957fe4631b6a6279f0ce8300Armaan Shaikh, DO,
- S1936878X16307999-15a643c51608fa23c813a24aa4c0c6b5Lindsey Kalvin, RCDS,
- S1936878X16307999-f271bdbcb7ed716f82634e6df1483af7Anwer Dhala, MD,
- S1936878X16307999-c8f878b80aba3fca63a8529967249178Arshad Jahangir, MD and
- S1936878X16307999-7bcb354099ef1689030a04a10aba7cc6A. Jamil Tajik, MD∗ ()
- ↵∗Aurora Cardiovascular Services, Aurora St. Luke’s Medical Center, 2801 West Kinnickinnic River Parkway, Suite 840, Milwaukee, Wisconsin 53215
Natural history of mitral valve prolapse (MVP) in the community is widely heterogeneous (1). Prognosis in MVP ranges from normal life expectancy to subsets with high morbidity/excess mortality, including sudden cardiac death (2). Although MVP is identified in a significant number of patients with sudden cardiac death, especially young women (3), and clinical and echocardiographic markers for adverse outcomes have been described (1–3), debate about MVP leading to sudden cardiac death survives in contemporary cardiology. To this end, we report our observation of a novel echocardiographic marker of high risk in patients with myxomatous mitral valve disease (MMVD) and bileaflet MVP (BMVP).
Of the 278 patients at our institution who fulfilled myxomatous BMVP published criteria (3), 21 were referred to our specialized valve clinic for further characterization (quantification) of the severity of mitral regurgitation (MR) and further management options. These referrals were generated by the primary cardiologists who had initially evaluated the patient. All underwent careful interrogation of lateral and medial annuli by pulsed-wave tissue Doppler from the apical window. Extent of mitral prolapse, leaflet thickness, medial and lateral wall Doppler velocity, MR severity, left ventricular ejection fraction, left atrial size, and 12-lead electrocardiograms were analyzed. Cardiac magnetic resonance (CMR) imaging studies were reviewed with particular focus on delayed gadolinium enhancement of posterobasal myocardium and of the papillary muscles.
Patients were divided into 2 groups based on tissue Doppler: those with a spiked systolic high-velocity signal ≥16 cm/s (Group 1) and those <16 cm/s (Group 2). Among 21 patients with MMVD and BMVP (median age, 51.6 ± 12.3 years; 71% female), 12 patients were in Group 1 (47.4 ± 9.5 years; 75% female) and 9 in Group 2 (57.1 ± 14.0 years; 67% female). Group 1 patients with distinctive spiked signal (≥16 cm/s) had 8 malignant (combined ventricular tachycardia/ventricular fibrillation) events compared with 2 malignant events in Group 2 patients (67% vs. 22%; p < 0.08 [Fisher exact test]). Of the 12 patients in Group 1, a total of 5 patients had an implantable cardioverter defibrillator implanted versus none in Group 2. Delayed gadolinium enhancement was noted only in Group 1 (2 of 6; 33%), out of the 11 patients who underwent CMR. Inverted or biphasic T waves in the inferior leads were observed in 6 patients in Group 1 and 2 patients in Group 2 (p = NS). There was no difference between the 2 groups in severity of prolapse, leaflet thickness, medial annulus Doppler velocity, MR severity, or left ventricular ejection fraction. This observation of high-velocity systolic signal with tissue Doppler imaging resembling the “Pickelhaube,” a spiked helmet, was thus an indicator of a malignant phenotype of MMVD and BMVP (Figure 1).
We hypothesize that the tugging of the posteromedial papillary muscle in mid-systole by the myxomatous prolapsing leaflets causes the adjacent posterobasal left ventricular wall to be pulled sharply toward the apex, resulting in the observed spiked configuration of the lateral annular velocities (Pickelhaube sign). It has been suggested that this mechanical traction of the papillary muscles and posterolateral left ventricular wall is arrhythmogenic with early electrical dysfunction being recognized during electrophysiological studies even in the absence of gadolinium enhancement on CMR (4). Endocardial friction lesions in the inferolateral mural endocardium are additional mechanisms for ventricular arrhythmias in MMVD and MVP (2,4). Recent CMR studies (5) have shown delayed gadolinium enhancement in these areas, suggesting fibrosis. Our novel finding adds to the emerging risk markers of arrhythmogenic MVP syndrome (2–5), suggesting the possibility that this spiked tissue Doppler velocity profile may be a risk marker for malignant arrhythmias in patients with MMVD and BMVP.
Future research with a large cohort of patients will help elucidate the clinical implications of this observation.
Please note: The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
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