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A Case of Symptomatic Hypertrophic Cardiomyopathy with Severe Dynamic Gradient

Andrea Piccioni*, Marinella Mazzone, Fabrizio Lupparelli, Claudia Mandolini, Giulia Ciocci, Manfredi de Marle Giusti, Cacciotti Luca, Gerardo Ansalone and Cinzia Sighieri

Hypertrophic cardiomyopathy is a disease of the myocardium characterized by the thickening of the heart walls. It is a genetic disorder transmitted by first-degree relatives, caused by mutations in genes that code for sarcomere protein. A 77-year-old woman presented to the emergency department and complained of continuous vertigos and presyncope with subsequent fall to the floor with a concussive traumatic brain injury. The ECG observed signs of left ventricular hypertrophy. Blood exams highlighted a slight increase of troponin T (25 ng/L). The ultrosonography instead demonstrated a pronounced left ventricular hypertrophy with a 240 mmHg dynamic gradient, a moderate mitral regurgitation secondary to SAM.

Lastly, ECG Holter excluded significant arrhythmias (1 couple of ventricular contractions). In the ward, the echocardiographic pattern was repeated and confirmed the clinical picture of a pronounced ventricular hypertrophy at the expense of the IVS (VTD 45 ml), a diastolic dysfunction Grade I with anterior systolic movement of the mitral flap valves with a dynamic obstruction to the left ventricular outflow (DP max 260 mmHg). Moreover, a moderatesevere mitral insufficiency was reported. Vital signs were stable.

The antihypertensive therapy was enhanced with added ACE inhibitors, loop diuretics, beta blockers and the disopyramide administration started. The patient was discharged from the hospital and was scheduled a cardiologic echocardiographic follow up in order to evaluate the effectiveness of the therapy. This study has demonstrated the association between the clinical symptom, the clinical objectivity and the echocardiographic anomalies typical of the obstructive hypertrophic cardiomyopathy. Severe blood pressure values of dynamic obstruction to the left ventricular outflow have been recorded and which are not currently known by the scientific literature. Our purpose will be to evaluate the clinical and echocardiographic follow up and the potential response to the pharmacological therapy prescribed.