An unexpected outcome for an unlucky combination

Introduction: Hypertrophic Obstructive Cardiomyopathy (HOCM) is a challenging pathology, especially because of its multiple complications and the narrow therapeutic solutions. And when more than one potentially fatal condition occurs, the management becomes even more difficult.
Clinical case: A 63-year-old woman, presented in the emergency department with typical angina, accompanied by dyspnea. The electrocardiogram (ECG) revealed sinoatrial block with junctional escape rhythm, hypertrophy criteria with repolarization abnormalities and isolated ectopic ventricular beats. The TnI was elevated, following a pattern of rise and fall during hos-pitalization, typical for AMI Initial echocardiography revealed impressive concentric hypertrophy of the left ventricle (interventricular septum of 30 mm), intraventricular gradient up to 80 mmHg at rest with systolic anterior motion (SAM) of the mitral valve. Despite severe HOCM, which frequently associate high levels of TnI, we judged the case as an AMI. An emergency coronary angiography was performed, that revealed trivascular coronary disease – with proximal LAD subocclusive stenosis place where the main septal branch arise, with slow-flow in both main and septal branches, LCX critical stenosis of the marginal branch and chronic total occlusion of RCA. PCI with DES, one for each vessel was performed in LAD and LCX. However, the patient developed cardiogenic shock with acute liver and renal failure, having a particular and interesting mechanism, a combination of anterior AMI, sinoatrial block and HOCM. Beta blockers were administered but because of the sinoatrial block, she tolerated only very small doses, inefficient for the extreme intraventricular gra-dient. This new life-threatening scenario imposed not only surgical myomectomy, but also emergency car-diac stimulation. The patient was eligible for bicame-ral implantable cardioverter defibrillator (ICD) – due to the sudden death high risk and as antibradycardia treatment when appropriate beta blocker doses wo-uld be administered. After the procedure, there was a spectacular decrease in the intraventricular gradient (20 mmHg at rest, 50 mmHg with Valsalva) due to the right ventricle stimulation – that desynchronized the two ventricles. In this new context, the surgery was postponed. Other particular and interesting fact was the decrease of the interventricular septum width in time and the hipokinesis at this level, due to the myo-cardial necrosis secondary the main septal branch is-chemia. The patient had a slow, but favorable outcome, without signs and symptoms of cardiac failure and with improved effort tolerance enough to have intermittent claudication. Three months after AMI an peripheral ar-teriography was performed, showing critical stenosis of the left common iliac artery and chronic occlusion of left superficial femoral artery.
Conclusions: This complex case with three significant life-threatening conditions at the same patient – AMI, HOCM and sinoatrial block with junctional escape rhythm – demonstrates that even the worse scenarios can have unexpectedly favorable outcomes. Main sep-tal branch ischemia came along with beneficial septal necrosis – combination of sinoatrial block and HOCM led to ICD which desynchronized ventricles and ena-bled the high dose of beta-blocker, all together put the surgical myomectomy on a waiting list and revealed a new atherosclerotic condition: severe peripheral artery disease.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
ESC search engine
CODE: 379
CME Credits: 10 (Romanian College of Physicians)