Apical 4-chamber echocardiogram in a patient with the apical variant of hypertrophic cardiomyopathy (HCM) Apical four-chamber view from a two-dimensional echocardiogram shows apical and midventricular hypertrophy. During systole, the left ventricular cavity is nearly obliterated. | Cardiovascular Education
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Apical 4-chamber echocardiogram in a patient with the apical variant …
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Apical H. Cardiomyopathy (ApHCM — a special variant of HCM, often called Yamaguchi syndrome. --- 1--Definition & Epidemiology A variant of hypertrophic cardiomyopathy characterized by predominant hypertrophy of the left ventricular (LV) apex, producing a "spade-like" configuration of the LV cavity on ventriculography/echo/MRI. More common in East Asian populations (esp. Japan), but also seen worldwide. Often diagnosed later in life compared to classic septal HCM. --- 2--Pathophysiology Genetic b
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🔴 YAMAGUCHI SYNDROME (Apical HCM) – ECHO CW DOPPLER PEARLS 🔴 🫀 Marked apical LV hypertrophy ♠️ “Ace-of-spades” LV cavity (end-diastole) ⚡ Normal / hyperdynamic EF 🚫 No significant LVOT obstruction ⏳ Diastolic dysfunction common ⚠️ Apical cavity obliteration ± aneurysm 📈 CW Doppler: 🔹 No dynamic LVOT gradient 🔹 Mid-cavity / apical systolic gradient (late-peaking, dagger-shaped) 🔹 Higher apical velocities due to cavity obliteration 📌 Classic ApHCM = apical thickening with mid-to-apical gr
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