Journal of Rare Cardiovascular Diseases

Apical hypertrophic cardiomyopathy (AHC) is a less common type of hypertrophic cardiomyopathy (HCM). It is characterized by typical changes in ECG (deeply inverted T waves that can mimic acute coronary syndrome), echocardiography (hypertrophy of apical segments) and ventriculography (spade-like shaped left ventricular cavity). Magnetic resonance is considered as a reference method for AHC diagnosis. We present a long follow-up period of a patient with this form of HCM. The management of patients with HCM is directed at decreasing left ventricle outflow tract narrowing (if present), decreasing of intraventricular gradient (if present), heart failure treatment and prevention of sudden cardiac death. In general treatment adequate hydration is essential and diuretics should be used with caution to prevent the increase of intraventricular gradient.

 

congenital heart disease; heart failure; rare disease; ventriculography; echocardiography; spade‑like shape

Elliott PM, Anastasakis A, Borger MA, et al. 2014 ESC Guidelines on Diagnosis and Management of Hypertrophic Cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014; 35:2733–2779.

Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet 2013; 381: 242–255.

Wiśniowska-Śmiałek S, Rubiś P, Biernacka-Fiałkowska B, et al. 45-year-old man with hypertrophic cardiomyopathy after alcohol ablation, progression to dilated cardiomyopathy. JRCD 2014; 2 (1): 23–26.

Sakamoto T, Tei C, Murayama M, et al. Giant T wave inversion as a manifestation of asymmetrical apical hypertrophy (AAH) of the left ventricle. Echocardiographic and ultrasono-cardiotomographic study. Jpn Heart J 1976; 17: 611–629.

Kuśnierz B, Tendera M, Kozielska E, et al. Apical hypertrophic cardiomyopathy – clinical, electrocardiographic, echocardiographic and hemodynamic evaluation. Pol Arch Med Wewn. 1988; 79 (5): 238–247.

Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients. J Am Coll Cardiol 1995; 26(7):1699–1708.

Grabysa R. Apical hypertrophic cardiomyopathy as a cause of cardiac insufficiency in an 84-year-old woman – a case report. Kardiol Pol. 2008; 66 (2): 179–182.

Olivotto I, Cecchi F, Casey SA, et al. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation 2001;104:2517–2524.

Petkow-Dimitrow P, Podolec P, Grodecki J, et al.Comparison of dual chamber pacing with nonsurgical septal reduction effect in patients with hypertrophic obstructive cardiomyopathy. International Journal of Cardiology 2004; 94: 31–34.

Elliott PM, Poloniecki J, Dickie S, et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 2000; 36: 2212–2218

O’Mahony C, Jichi F, Pavlou M, et al. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM Risk-SCD). Eur Heart J 2014; 35: 2010–2020.

Minami Y, Kajimoto K, Terajima Y, et al. Clinical implications of midventricular obstruction in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2011; 57: 2346–2355.

Efthimiadis GK, Pagourelias ED, Parcharidou D, et al. Clinical characteristics and natural history of hypertrophic cardiomyopathy with midventricular obstruction. Circ J 2013;77: 2366–2374

Said SM, Schaff HV, Abel MD, et al. Transapical approach for apical myectomy and relief of midventricular obstruction in hypertrophic cardiomyopathy. J Card Surg 2012; 27:443–448.

Kunkala MR, Schaff HV, Nishimura RA, et al. Transapical approach to myectomy for midventricular obstruction in hypertrophic cardiomyopathy. Ann Thorac Surg 2013; 96: 564–570.