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Abstract
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Report
CLEAR FILTERS
Not all left ventricular hypertrophy is hypertrophic cardiomyopathy: more than meets the eye
Session:
Casos Clínicos desafiantes 2
Speaker:
Pedro Brás
Congress:
CPC 2023
Topic:
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Theme:
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Subtheme:
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Session Type:
Speaker´s Corner
FP Number:
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Authors:
Pedro Garcia Brás; Isabel Cardoso; Sílvia Aguiar Rosa; José Viegas; Tânia Mano; Tiago Rito; Lídia Sousa; Rui Cruz Ferreira
Abstract
<p style="text-align:justify"><span style="font-family:Arial,Helvetica,sans-serif"><span style="font-size:11pt">A 39-year-old female patient was diagnosed with hypertrophic cardiomyopathy (HCM) at the age of 13 after diagnostic work-up for a systolic heart murmur. The patient lost regular medical follow-up, had two uncomplicated pregnancies and was asymptomatic, with no medication. There was no significant family history or previous genetic testing.</span></span></p> <p style="text-align:justify"><span style="font-family:Arial,Helvetica,sans-serif"><span style="font-size:11pt">She screened positive for SARS-CoV2 and a few days later developed intense palpitations and presyncope, presenting to the emergency department with new onset atrial fibrillation at 75 bpm (Figure 1). Rhythm control with amiodarone was successful, with a second ECG (Figure 2) showing sinus rhythm at 85 bpm, 1st degree AV block and QRS notching in leads V1-V2. A bedside echocardiogram (TTE) was performed revealing preserved pronounced hypertrabeculation of the mid and apical left ventricle (LV), and the patient was referred to our cardiomyopathies outpatient unit. The patient was discharged on rivaroxaban, amiodarone and bisoprolol, with no symptom recurrence.</span></span></p> <p style="text-align:justify"><span style="font-family:Arial,Helvetica,sans-serif"><span style="font-size:11pt"> The physical exam revealed an apical I/VI systolic murmur, with no increase in intensity after Valsalva maneuver or orthostatic position. A new TTE (Videos 1 to 4, Figure 3) showed a nondilated LV, with normal wall thickness and preserved global and segmental systolic function (LVEF 57%, GLS -15.2%). Notably, two intraventricular cavities were observed in the LV (the largest with 15.5 cm<sup>2 </sup>and the smallest with 7.9 cm<sup>2</sup>), divided by a prominent fibromuscular thickening, with a small (9 mm) orifice between the two LV chambers. The left atrium was moderately dilated and there was no RV dilation or mitral regurgitation. </span></span></p> <p style="text-align:justify"><span style="font-family:Arial,Helvetica,sans-serif"><span style="font-size:11pt">To understand this complex anatomy, a contrast/enhanced cardiac magnetic resonance (CMR) was performed (Videos 5-8), confirming the presence of a thick contractile fibromuscular band with accessory trabeculations dividing the LV in two distinct chambers, with a small communication creating an intercavitary gradient. Furthermore, there were mitral subvalvular abnormalities in the spectrum of parachute mitral valve with fusion of the papillary muscles. There was no LV outflow tract obstruction at rest or fibrosis (Figures 4-7). 24-hour Holter monitoring showed rare ventricular ectopics and stress testing did not reveal exercise-induced arrhythmias or repolarization abnormalities. Laboratory testing showed a slightly elevated NT-proBNP (972 pg/mL).</span></span></p> <p style="text-align:justify"><span style="font-family:Arial,Helvetica,sans-serif"><span style="font-size:11pt">Despite an initial suspected cardiomyopathy since childhood, cardiac imaging led to the final diagnosis of double-chambered left ventricle, an extremely rare congenital heart disease in which the LV is divided into two chambers by a septum or muscle fibre with abnormal proliferation. TTE and CMR were crucial for the correct diagnosis, allowing a comprehensive view of the patient’s cardiac anatomy and clarifying the origin of the systolic murmur (due to a communication between chambers, and not obstructive HCM as initially interpreted).</span></span></p>
Slides
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