A 58-year-old male with a history of hypertension and tumor of suprarenal gland was referred to Dubrava University Hospital, Zagreb because of the chest pain and palpitation. There was no family history of sudden cardiac death, congestive heart failure or cardiomyopathy. We evaluated the patient with the MSCT of the kidney and suprarenal glands because of the previously suspected tumor of suprarenal gland that was characterized as adenoma.

The results of physical examination was normal, as well as the cardiac biomarkers and chest X-ray.

The dominant finding was an abnormal ECG, which was characterized primarily by deep negative T-waves in precordial leads (Figure 1) which were followed-up throughout the last 10 years’ period, which is why coronarography was performed showing normal values. Because of that finding, which was also noticed about ten years ago, the left-sided cardiac catheterization was done, with no evidence of coronary artery disease. Transthoracic echocardiography (TTE) showed concentric hypertrophic cardiomyopathy (HC) of the left ventricle with no valvulopathy.

TTE performed immediately after admission showed apical hypertrophy of the left ventricle with the systolic apex thickness of 43 mm, diastolic apex thickness of 35 mm, and the ratio of maximal apical to posterior wall thickness of 1.6 (Figure 2, 3). The follow-up 24-hour Holter ECG monitoring revealed no atrial or ventricular ectopy.

The patient was diagnosed with benign form of apical HC and received treatment with ACE inhibitors and beta-blockers.

Apical HC is frequently sporadic; however, a few families have been reported with autosomal dominant inheritance. (1) Positive family history is more common in patients with asymmetric septal hypertrophy than with apical HC.

The diagnostic criteria for apical HC include demonstration of asymmetric LV hypertrophy, confined predominantly to the LV apex, with apical thickness ≥15 mm and ratio of maximal apical to posterior wall thickness ≥1.5, based on an TTE or magnetic resonance imaging (MRI). The mean age of presentation of apical HC is 41.1±14.5 years and it is most commonly seen in males. About 54% of patients with apical HC are symptomatic and the most common presenting symptom is the chest pain, followed by palpitations, dyspnea and syncope. (2)

The most frequent ECG findings are negative T-waves in precordial leads which are found in 93% of patients, followed by LV hypertrophy in 65% of patients. TTE shows hypertrophy of LV apex and is the initial diagnostic tool for apical HC. Although the TTE is most commonly the initial diagnostic tool, if inconclusive, cardiac MRI is now emerging as “the gold standard” for diagnosis of apical HC. (3) Apical HC can mimic other conditions, including apical cardiac tumors, LV apical thrombus, endomyocardial fibrosis and coronary artery disease. (4-7)

Patients with apical HC can be symptomatic and asymptomatic. Verapamil, beta-blockers and anti-arrhythmic agents are used in symptomatic patients. (2) Verapamil and betablockers are found to be beneficial in improving symptoms, while amiodaron and procainamid are used in the treatment of atrial fibrillation and ventricular arrhythmias. (8, 9) Implantable cardioverter defibrilator is recommended for high risk HC patients with previous cardiac arrest or sustained episodes of ventricular tachycardia, syncope or a family history of sudden cardiac death. (10)

Unlike some other variants of hypertrophic cardiomyopathy, the prognosis of apical HC is relatively benign. The overall mortality rate is 10.5% and cardiovascular mortality is 1.9% after follow-up of 13.6±8.3 years. (2) Although sudden cardiac death and cardiovascular events occur more frequently in asymmetric septal hypertrophy than in the apical one, those patients may also develop sudden life-threatening complications.

In conclusion, it is important not to overlook the diagnosis of this form of HC; and although the prognosis in this form of HC is much better these patients are to be regularly followed-up and properly treated.