SPC365

Recognition of Malignant Arrhythmic Valve Prolapse (Mitral and/or Tricuspid): still a long way to go

Session: Prémio Melhor Caso Clínico
Speaker: Gonçalo José Lopes Da Cunha

Congress: CPC 2022
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Session Type: Prémios
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Authors: Gonçalo Lopes da Cunha; Gonçalo Cardoso; Rita Carvalheira Santos; Pedro Freitas; Sara Guerreiro; João Abecasis; António Ferreira; Regina Ribeiras; Maria João Andrade; Miguel Mendes
<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">73-year-old man referred to outpatient consultation for light-headedness. </span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">His personal and familiar cardiovascular history was solely remarkable for arterial hypertension, for which he was not compliant with the prescribed medication (ramipril+hydrochlorothiazide). </span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">In the previous month, the patient complained of light-headedness and near-syncope with associated with diaphoresis both with exercise and at rest. The vital signs and physical exam were unremarkable.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:&quot;Arial&quot;,sans-serif">An urgent 24h-holter monitoring during which the patient complained of several episodes of light-headedness, demonstrated multiple bursts of non-sustained VT, the longest lasting for 15 seconds at a rate of 220bpm, hastening his hospitalization </span>for investigation of symptomatic VT.</span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">The blood tests, electrolytes and thyroid function included, showed no abnormalities. ECG was in sinus rhythm, 77bpm, cQT 412ms and with one premature ventricular contraction positive in V1-V3.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">Transthoracic echocardiography revealed non-dilated LV with moderate hypertrophy and preserved ejection fraction without WMA. Mitral valve had bileaflet billowing, mild (7mm). annular disjunction and trivial regurgitation. Non dilated LA but dilation of RV and RA with TAPSE 30mm, S&rsquo;RV 23cm/s. The tricuspid valve had also myxomatous changes and annular dilation.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">Coronary angiography excluded coronary artery lesions. Cardiac MRI confirmed the bivalvular billowing and annuli disjunction as well as the presence of subepicardial late gadolinium enhancement at the basal inferolateral segment.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Lucida Sans Unicode&quot;,sans-serif"><span style="color:black"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">After initiation of amiodarone and bisoprolol, a treadmill stress test was done for risk stratification and showed occasional premature ventricular contractions, 2 couplets and 1 triplet.</span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">Due to the presence of mitral annular disjunction, ventricular tachycardia and LGE in LV posterior wall in CMR, the diagnosis of arrhythmic mitral valve prolapse was made and, since the patient had non sustained VT and near-syncope, a subcutaneous ICD was implanted as secondary prevention.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">The patient was discharged with amiodarone 200mg, bisoprolol 2.5mg, edoxaban 60mg and ramipril+hydrochlorothiazide.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="font-family:&quot;Arial&quot;,sans-serif">Two years after the diagnosis, the patient had and an episode of sustained VT, eliciting an appropriate ICD shock.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-size:11.0pt"><span style="background-color:white"><span style="font-family:&quot;Arial&quot;,sans-serif"><span style="color:black">This case depicts one more example of malignant arrhythmic mitral and tricuspid valve billowing with annuli disjunction, and illustrates the pressing need for the definition of tools for risk stratification of sudden arrhythmic death in this recently recognized valvular patient group. </span></span></span></span></span></span></p>