Is the mechanics of the posterior mitral leaflet the trigger of malignant mitral valve prolapse?
Presented During:
Thursday, May 4, 2023: 6:30PM - Saturday, May 6, 2023: 2:29AM
New York Hilton Midtown
Posted Room Name:
Grand Ballroom Foyer
Abstract No:
MP027
Submission Type:
Abstract Submission
Authors:
Gheorghe CERIN (1), Diana BENEA (2), Diana Carmen BOTEZATU (3), Theodor CEBOTARU (4), MARCO DIENA (5)
Institutions:
(1) Cardioteam Foundation, Torino, Piemonte, (2) IRCCS San Donato, Milan, Lombardia, (3) IRCCS Multimedica, Milan, Lombardia, (4) The Cardiovascular Center, Monza Hospital, Bucharest, RI, (5) IRCCS Policlinico San Donato, SAN DONATO MILANESE, ITALY
Submitting Author:
Gheorghe CERIN
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Cardioteam Foundation
Cardioteam Foundation
Co-Author(s):
Diana BENEA
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IRCCS San Donato
IRCCS San Donato
Diana Carmen BOTEZATU
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IRCCS Multimedica
IRCCS Multimedica
Theodor CEBOTARU
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The Cardiovascular Center, Monza Hospital
The Cardiovascular Center, Monza Hospital
MARCO DIENA
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IRCCS Policlinico San Donato
IRCCS Policlinico San Donato
Presenting Author:
Gheorghe CERIN
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Cardioteam Foundation
Cardioteam Foundation
Abstract:
Objective: search for arrhythmias trigger and mechanism of myocardial fibrosis (MyF) in malignant mitral prolapse (MMVP).
Method: A 30 y.o. woman with negative T waves in D2, D3, recurrent prelipotymic crises and known trivial mitral regurgitation (tMR) is presented. In a fibroelastic deficiency MV, transthoracic echocardiography (TTE) reveals - Fig1: bileaflet prolapse (BiP), tMR, mitral annular disjunction (MAD of 9mm) and curling. Between the posterior mitral leaflet (PML) and inferior left ventricle wall (iLVW) a >90° angle was seen. TDI echo shows the pickelhaube helmet sign (PHS). ECG Holter shows complex ventricular arrhythmias (VA): a run of polymorphic ventricular tachycardia, premature ventricular complex (PVC) 25%, with bi and trigeminal beats. Exercise ECG test showed paired, bi, trigeminal PVC. Late Gadolinium Enhancement (LGE) was seen on the basal portion of the iLVW at CMR. In Heart Team the patient was judged to be MMVP at high risk of sudden death and surgery was proposed.
Results: In right minithoracotomy approach a thin MV was seen with BiP, more evident on P2. Surgery: P2 limited triangular resection + restoring of coaptation plan from left atrium inside of LV + PTFE chordae to stabilize P2 area + a complete prosthetic ring. TTE showed tMR, long coaptation (9mm), rebuilding of the coaptation triangle (CoT), loss of MAD, curling and of PHS.
CONCLUSIONS: MMVP can occur even in tMR uninfluenced by the degree of MR, if MyF is present and the key to analysis is the PML motion. Abnormal PML motion angle (>90°) releases kinetic energy to the MV hinge area creating cell damage, apoptosis and MyF. Since in a normal MV a sharp angle occurs between the PML and iLVW - creating a 3rd degree lever of forces, while in MMVP, due to the BiP, this angle increases >90° and so a lever of 1st degree rises. Loss of MAD, curling and PHS after surgery confirms that the PML jerk effect has been eliminated by rebuilding of a normal MV geometry. In fact, at 4y F-UP ECG is normal, the patient is asymptomatic with just 1,7% VA at Holter, prelipotyms disappeared and the ECG stress test showed only occasional bigeminal PVC. The only great difference from pre-Op and post-Op MV geometry at TTE was the restoring of CoT, initially absent, to a near normal one. Bringing back of the MV coaptation plane into the LV removes the mechanical triggers of PML in this arrhythmogenic prolapse. Further studies are needed for a better understanding of this life-threateni
Method: A 30 y.o. woman with negative T waves in D2, D3, recurrent prelipotymic crises and known trivial mitral regurgitation (tMR) is presented. In a fibroelastic deficiency MV, transthoracic echocardiography (TTE) reveals - Fig1: bileaflet prolapse (BiP), tMR, mitral annular disjunction (MAD of 9mm) and curling. Between the posterior mitral leaflet (PML) and inferior left ventricle wall (iLVW) a >90° angle was seen. TDI echo shows the pickelhaube helmet sign (PHS). ECG Holter shows complex ventricular arrhythmias (VA): a run of polymorphic ventricular tachycardia, premature ventricular complex (PVC) 25%, with bi and trigeminal beats. Exercise ECG test showed paired, bi, trigeminal PVC. Late Gadolinium Enhancement (LGE) was seen on the basal portion of the iLVW at CMR. In Heart Team the patient was judged to be MMVP at high risk of sudden death and surgery was proposed.
Results: In right minithoracotomy approach a thin MV was seen with BiP, more evident on P2. Surgery: P2 limited triangular resection + restoring of coaptation plan from left atrium inside of LV + PTFE chordae to stabilize P2 area + a complete prosthetic ring. TTE showed tMR, long coaptation (9mm), rebuilding of the coaptation triangle (CoT), loss of MAD, curling and of PHS.
CONCLUSIONS: MMVP can occur even in tMR uninfluenced by the degree of MR, if MyF is present and the key to analysis is the PML motion. Abnormal PML motion angle (>90°) releases kinetic energy to the MV hinge area creating cell damage, apoptosis and MyF. Since in a normal MV a sharp angle occurs between the PML and iLVW - creating a 3rd degree lever of forces, while in MMVP, due to the BiP, this angle increases >90° and so a lever of 1st degree rises. Loss of MAD, curling and PHS after surgery confirms that the PML jerk effect has been eliminated by rebuilding of a normal MV geometry. In fact, at 4y F-UP ECG is normal, the patient is asymptomatic with just 1,7% VA at Holter, prelipotyms disappeared and the ECG stress test showed only occasional bigeminal PVC. The only great difference from pre-Op and post-Op MV geometry at TTE was the restoring of CoT, initially absent, to a near normal one. Bringing back of the MV coaptation plane into the LV removes the mechanical triggers of PML in this arrhythmogenic prolapse. Further studies are needed for a better understanding of this life-threateni
Mitral Conclave:
Malignant Bileaflet Mitral Valve Prolapse Syndrome
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