675 A rare case of tako-tsubo syndrome in a patient with hypertrophic cardiomyopathy
Authors: Parato Vito Maurizio , Gizzi Germana , D'Agostino Simone 
Published: December 2021

Abstract

Aims
We know that basal septal hypertrophy is a rare and unique anatomical finding associated with hypertrophic cardiomyopathy (HCM). Tako-Tsubo cardiomyopathy (TTC) is a transient left ventricular systolic dysfunction induced by high physical or emotional stress. Its occurrence with HCM is unusual. However, this presentation occurs more often with the classic asymmetrical septal hypertrophy compared with the apical variant. This case demonstrates that the coexistence of TTC with septal HCM in an elderly patient may lead to a severe haemodynamic instability picture.

Methods and results
A 81-year-old female presented to the emergency department (ED) complaining of dyspnoea and chest pain lasting for 1 day. She had hypertension and dyslipidemia associated with a familial history of sudden death. On physical exam, we found a severe hypotension (systolic blood pressure of 80 mmHg) associated with bilateral rales at chest auscultation. Cardiac auscultation revealed a harsh systolic murmur, best heard over the left sternal border. Heart rate was 60 b.p.m. in sinus rhythm. Labs were significant for HS-I troponin of 6.035 ng/L (NV: ≤ 12) and NT-proBNP of 7.640 pg/ml (NV: ≤1800). A 12-leads electrocardiogram (ECG) at admission revealed a STEMI-like ST segment elevation from V2 to V6 (Figure 1A). For this reason she was urgently taken to the cath-lab where she was found to have tortuous but normal coronary arteries. After coronary angiography, a trans-thoracic echocardiogram (TTE) revealed a pathological LV hypertrophy with a septal diastolic thickness of 19 mm, depressed LV ejection fraction (LVEF) due to a severe apical ballooning. At continuous wave (CW)-Doppler there was a dynamic obstruction across the LV outflow tract (LVOT), with a late peak velocity of 4.9 m/s and an estimated peak gradient of 98 mmHg. The gradient was increased by a systolic anterior motion (SAM) of anterior mitral leaflet causing a moderate mitral regurgitation (MR). All these findings were consistent with obstructive septal HCM associated with Takotsubo cardiomyopathy. After treatment with intravenous diuretics and metoprolol (5 + 5 mg i.v. bolus followed by oral dose of 100 mg daily), her clinical condition markedly improved. One week later, ECG demonstrated deeply inverted T waves on antero-lateral leads and QT prolongation (501 ms). Three weeks later, after a complete resolution of the LV apical dyskinesia, LVEF normalized. LVOT gradient decreased to 20 mmHg, with a dynamic increase to 70 mmHg during Valsalva manoeuvre.

Conclusions
It is well known that TTC may be complicated by a reversible LVOT obstruction by itself but the combination with obstructive HCM can lead to low cardiac output and acute heart failure. This combination has been found to be not common and the correct treatment of this unusual type of cardiogenic shock is still unclear. Careful initial evaluation and continuous monitoring must be warranted in such rare cases. Supportive care afterward with beta blockers, along with echocardiogram surveillance, are the mainstay of management. Cardiologists, intensivisits, and clinicians alike need to recognize and comprehend the pathophysiology behind this unique clinical manifestation so that they may adjust their management and treatment accordingly.