Differences in clinical, morphological and laboratory parameters between hypertrophic cardiomyopathy patients with and without heart failure
S Wisniowska-Smialek, A Karabinowska Malocha, E Wypasek, J Szachowicz Jaworska, E Dziewiecka, K Graczyk, M Winiarczyk, A Stepien, P RubisAbstract
Background
Hypertrophic cardiomyopathy (HCM) is a heterogeneous group of genetic diseases characterized by structural and functional changes in the heart. A certain group of HCM patients met the criteria and can be classified as a heart failure with preserved ejection fraction as the most common phenotype of HCM, while 5-10 % developed "burned- out phase" with reduced ejection fraction of left ventricle.
Aim
We sought to determine the clinical, echocardiographic, cardiac magnetic resonance and laboratory factors that can characterize the HCM with heart failure.
Methods
A total of 72 consecutive ambulatory HCM patients, with mean age 52,8 +/- 16,2, including 47 (65,3%) men, have been enclosed to the analysis. They were divided into two groups with (35 patients) and without (37patients) heart failure according to ESC diagnostic algorithm for Heart Failure with Preserved Ejection Fraction (HFpEF) and with reduced ejection fraction (HFrEF). Each patients had clinical, echocardiographic and laboratory assessment including serum levels of fibrosis markers: PICP, PIIINP, Galectin 3, TGF-ß1, ANP, INF, IL-1R, TGF, Galectin, CTX, GDF 15, and also IL-6 and sCD 146 as a novel marker of heart failure or body overhydration markers. Additionally, 52 HCM patients had CMR with late gadolinium enhancement (LGE) evaluation.
Results
HCM patients with HF were more often in NYHA class II (19 [54,3%] vs 9 [24,3%], and III (8 [22,9%] vs 1 [2,7%], p=0,0001), had greater value of NT- proBNP level (1175 [669-2307] vs 194 [71-481], p <0,0001) and troponin T (hsT), greater thickness of intraventricular septum (19,34 +/- 3,85 vs 16,82 +/-2,91) and left atrium diameter, more often had ventricular arrhythmia and bundle branch blocs than HCM without HF. According to CMR HCM with HF had greater LGE extent (6,48 +/- 5,69 vs 3,28 +/- 3,95, p 0,029). There were no differences in maximal LVOT obstruction gradient (24,5 [10-60] vs 14 [7-45], p-0,15) between groups. HCM with HF had greater risk of sudden cardiac death in ESC Risk Score calculator (3,8 [2,38-7,0] vs 2,58 [2,11-3,85], p – 0,0037). There were no differences in serum markers of fibrosis between groups however IL-6 and soluble CD 146 were significantly higher in HCM with HF (2,29 [1,45-4,31] vs 1,56 [1,0-2,13], p -0,014 and 8,02 [2,61-18,82] vs 0,45 [0,00-12,34], p- 0,022) respectively.
Conclusion
There are many clinical, morphological and laboratory differences between HCM patient with and without HF. HCM patients with heart failure had greater prevalence of AF, pronounced left atrium remodeling (greater LA diameter and area), left ventricular hypertrophy and diastolic dysfunction. HF group has greater LGE extent, higher burden of ventricular arrhythmias ( nsVT episodes, VES and LBBB/RBBB) and higher risk of sudden cardiac death according to HCM risk calculator. Finally, a serum level of soluble Cd 146 and IL-6 were higher in HF group, while there were no differences in serum level of fibrosis markers.For image description, please refer to the figure legend and surrounding text.For image description, please refer to the figure legend and surrounding text.