Subaortic and midventricular obstructive hypertrophic cardiomyopathy with extreme segmental hypertrophy

BACKGROUND: Subaortic and midventricular hypertrophic cardiomyopathy in a patient with extreme segmental hypertrophy exceeding the usual maximum wall thickness reported in the literature is a rare phenomenon. CASE PRESENTATION: A 19-year-old man with recently diagnosed hypertrophic cardiomyopathy (HCM) was referred for sudden death risk assessment. The patient had mild exertional dyspnea (New York Heart Association functional class II), but without syncope or chest pain. There was no family history of HCM or sudden death. A two dimensional echocardiogram revealed an asymmetric type of LV hypertrophy; anterior ventricular septum = 49 mm; posterior ventricular septum = 20 mm; anterolateral free wall = 12 mm; and posterior free wall = 6 mm. The patient had 2 types of obstruction; a LV outflow obstruction due to systolic anterior motion of both mitral leaflets (Doppler-estimated 38 mm Hg gradient at rest); and a midventricular obstruction (Doppler-estimated 43 mm Hg gradient), but without apical aneurysm or dyskinesia. The patient had a normal blood pressure response on exercise test and no episodes of non-sustained ventricular tachycardia in 24-h ECG recording. Cardiac MRI showed a gross late enhancement at the hypertrophied septum. Based on the extreme degree of LV hypertrophy and the myocardial hyperenhancement, an implantation of a cardioverter-defibrillator was recommended prophylactically for primary prevention of sudden death. CONCLUSION: Midventricular HCM is an infrequent phenotype, but may be associated with an apical aneurysm and progression to systolic dysfunction (end-stage HCM)

Risk factors for sudden cardiac death in hypertrophic cardiomyopathy

Aim of this study was the evaluation of six non invasive clinical indices as risk factors for sudden death (SD)in hypertrophic cardiomyopathy (HCM). Previous syncope, family history of SD, non sustained ventricular tachycardia, abnormalblood pressure response during exercise, excessive hypertrophy ≥3 cm and left ventricular outflow tract obstructionwith a peak gradient ≥30 mmHg were evaluated in a cohort of 166 patients(112 males, 51.8 ± 15.6 years), followed up for amedian of 32.4 months (range 1 to 209 months). During follow up 13 patients reached study’s endpoints: SD, cardiac arrest,documented sustained ventricular tachycardia and/or Implantable Cardioverter Defibrillator (ICD)-discharge. Patients havingexperienced syncope or presenting with a Maximum Wall Thickness ≥3cm in echocardiography were more sensitive to SDemergence since they had a 13.07 (95%CI: 4.00-46.95, p < 0.0001) and a 10.07 (95%CI: 2.92-34.79, p = 0.003) greater relativerisk respectively. In our cohort of patients only two of the six ‘recognised’ potential risk factors for SD were found sensitive,a result causing scepticism about the validity of criteria used for ICD implantation in HCM patients for SD prevention

Pseudoinfarction pattern in a patient with hyperkalemia, diabetic ketoacidosis and normal coronary vessels: a case report

<p>Abstract</p> <p>Introduction</p> <p>A rare electrocardiographic finding of hyperkalemia is ST segment elevation or the so called 'pseudoinfarction' pattern. It has been suggested that hyperkalemia causes the 'pseudoinfarction' pattern not only through its direct myocardial effects, but also through other mechanisms, such as anoxia, acidosis, and coronary artery spasm.</p> <p>Case presentation</p> <p>A 33-year-old Caucasian woman with insulin-treated diabetes presented with continuous epigastric pain of four hours duration. Her coronary heart disease risk factors apart from diabetes included hypercholesterolemia and smoking. Her initial electrocardiogram revealed ST segment elevation in the anteroseptal leads consistent with anterior myocardial infarction. Blood tests revealed hyperglycemia, hyperkalemia, metabolic acidosis and urine ketones, while a bed-side cardiac echocardiogram showed no segmental wall motion abnormality. We provisionally diagnosed diabetic ketoacidosis that was possibly precipitated by acute myocardial infarction, as there were findings in favor of (epigastric pain, electrocardiogram pattern, presence of 3 coronary heart disease risk factors) and against (young age, normal echocardiogram) the diagnosis of acute myocardial infarction. We performed cardiac angiography in order to exclude an anterior acute myocardial infarction, which could lead to myocardial damage and possible severe complications should there be a delay in treatment. Angiography revealed normal coronary arteries. During the procedure, ST segment elevation in the anteroseptal leads was still present in our patient's electrocardiogram results.</p> <p>Conclusion</p> <p>ST segment elevation is a rare manifestation of hyperkalemia. In our patient, coronary spasm did not contribute to such an electrocardiography finding.</p

Spontaneous Dissection of Right Coronary Artery Manifested with Acute Myocardial Infarction

Spontaneous coronary artery dissection is a rare cause of acute ischemic coronary events and sudden cardiac death. It usually occurs in young women without traditional risk factors for coronary artery disease during pregnancy or postpartum period. However, it has also been reported in patients with atherosclerotic coronary disease. We present a case of spontaneous right coronary artery dissection in a 48-year male with recent myocardial infarction and previous percutaneous coronary intervention