Aged ; Cardiomyopathy, Hypertrophic ; diagnosis ; Diagnostic Errors ; Female ; Humans ; Hypertrophy, Left Ventricular ; complications ; diagnosis

Asymmetric septal hypertrophy with systolic anterior motion of the mitral valve is frequently a phenotypic, but not pathognomonic, expression of genetic hypertrophic cardiomyopathy (HCM) with or without obstruction. It can, however, be associated nonspecifically with other forms of increased left ventricular (LV) afterload. We herein report the case of a young man with obesity cardiomyopathy and heart failure who presented with asymmetric septal hypertrophy and marked LV hypertrophy, and endomyocardial biopsy ruled out genetic HCM.
Adult ; Cardiomyopathy, Hypertrophic, Familial ; Diagnosis, Differential ; Echocardiography ; Humans ; Hypertrophy, Left Ventricular ; complications ; diagnosis ; Male ; Obesity, Morbid ; complications

Medically refractory ventricular tachycardia (VT) storm can be controlled with radiofrequency catheter ablation (RFCA), however, it may be difficult to control in some patients with hemodynamic overload. We experienced a patient with intractable VT storm controlled by hemodynamic unloading. The patient had mid-septal hypertrophic cardiomyopathy with an implantable cardioverter defibrillator (ICD) back-up. Because of the severe mid-septal hypertrophy, his left ventricle (LV) had an hourglass-like morphology and showed apical ballooning; the focus of VT was at the border of apical ballooning. Although we performed VT ablation because of electrical storm with multiple ICD shocks, VT recurred 1 hour after procedure. As the post-RFCA monomorphic VT was refractory to anti-tachycardia pacing or ICD shock, we reduced the hemodynamic overload of LV with beta-blockade, hydration, and sedation. VT spontaneously stopped 1.5 hours later and the patient has remained free of VT for 24 months with beta-blockade alone. In patients with VT storm refractory to antiarrhythmic drugs or RFCA, the mechanism of mechano-electrical feedback should be considered and hemodynamic unloading may be an essential component of treatment.
Cardiomyopathy, Hypertrophic/complications/diagnosis/*physiopathology/therapy ; Catheter Ablation ; Electrocardiography ; Gated Blood-Pool Imaging ; Heart Catheterization ; Humans ; Male ; Middle Aged ; Tachycardia, Ventricular/diagnosis/etiology/*physiopathology/therapy ; Takotsubo Cardiomyopathy/complications/diagnosis/*physiopathology/therapy

Among the congenital coronary artery fistulae, multiple coronary artery microfistulae arising from the left and right coronary artery and emptying into the left ventricle are very rare and little is known of their anatomic and clinical features, especially in apical hypertrophic cardiomyopathy. A 67-year- old woman was referred for the evaluation of chest pain at exertion, and shortness of breath. Electrocardiographic and echocardiographic findings were typical of apical hypertrophic cardiomyopathy. Coronary arteriography showed normal epicardial coronary arteries, but multiple coronary artery-left ventricular microfistulae arising from the left and right coronary arteries. Transthoracic color Doppler echocardiography, using a high frequency transducer with a low Nyquist limit, demonstrated multiple coronary artery-left ventricular microfistulae just beneath the apical impulse window.
Aged ; Cardiomyopathy, Hypertrophic/*complications/diagnosis ; Coronary Angiography ; Coronary Vessel Anomalies/*complications/diagnosis/*ultrasonography ; Echocardiography ; Electrocardiography ; Female ; Heart Defects, Congenital/*complications/diagnosis/*ultrasonography ; Heart Ventricles ; Human ; Hypertrophy, Left Ventricular/complications/diagnosis

Mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHCM) is a rare type of cardiomyopathy, associated with apical aneurysm formation in some cases. We report a patient presenting with ventricular fibrillation, an ECG with an above normal ST segment, and elevated levels of cardiac enzymes but normal coronary arteries. Left ventriculography revealed a left ventricular obstruction without apical aneurysm. There was a significant pressure gradient between the apical and basal sites of the left ventricle. Cine magnetic resonance imaging (MRI), performed on the 10th hospital day, showed asymmetric septal hypertrophy, mid-ventricular obstruction, and an apical aneurysm with a thrombus. The first evaluation by contrast-enhanced imaging showed a subendocardial perfusion defect and delayed enhancement. It was speculated that the intraventricular pressure gradient, due to mid- ventricular obstruction, triggered myocardial infarction, which subsequently resulted in apical aneurysm formation.
Cardiomyopathy, Hypertrophic/complications/*diagnosis ; Coronary Angiography ; Echocardiography, Doppler ; Heart Aneurysm/*diagnosis/etiology ; Humans ; Hypertrophy, Left Ventricular/complications/*diagnosis ; Magnetic Resonance Imaging, Cine ; Male ; Middle Aged ; Myocardial Ischemia/complications/diagnosis

A 29-year old male was transferred to our hospital with an abnormal chest X-ray finding diagnosed as hypertrophic cardiomyopathy with apical necrosis and aneurysm formation. Four years after the initial hospitalization, we confirmed the aneurysm and necrosis using both integrated positron emission tomography (PET) and computed tomography (CT) scanning. The F-18 2-fluoro-2-deoxy-D-glucose (FDG) PET/CT enabled precise localization of the aneurysm, which was found to be composed of semi-lunar calcification of non-metabolic myocardium. A contrast-enhanced CT angiography showed an hour-glass appearance of the left ventricular cavity. The integrated PET/CT fusion scanner is a novel multimodality technology that allows for a comprehensive analysis of the anatomical and functional status of complex heart disease. Based on these findings, long standing mechanical and physiologic abnormalities may have led to chronic ischemia in the hypertrophied myocardium, induced necrosis and calcification at the cardiac apex.
Tomography, X-Ray Computed ; Positron-Emission Tomography ; Necrosis/complications/*diagnosis ; Male ; Humans ; Heart Ventricles/*pathology ; Heart Aneurysm/complications/*diagnosis ; Fluorodeoxyglucose F18/diagnostic use ; Contrast Media ; Cardiomyopathy, Hypertrophic/complications/*diagnosis ; Angiography, Digital Subtraction ; Adult

OBJECTIVETo analyze the cardiac manifestations of mitochondriopathy patients.

METHODSWe retrospectively analyzed the clinical (Electrocardiogram, Holter monitoring, echocardiogram and laboratory examinations) and pathological data of 90 mitochondriopathy patients diagnosed within recent 20 years. The cardiac involvement data from these patients were summarized.

RESULTSHypertrophic cardiomyopathy was found in 2 patients and dilated cardiomyopathy in 3 patients Mitochondriopathy diagnosis was made in 1 patient two years after heart transplantation due to heart failure resulting from previously diagnosed hypertrophic cardiomyopathy with noncompaction. The prevalence of cardiomyopathy is 5.6% (5/90). The prevalence of various arrhythmias was 22.2% (20/90). Four patients received permanent pacemaker because of Adams-Stokes attack or bradyarrhythmias (mitochondriopathy diagnosis was made 1-3 years post pacemaker implantation in 3 cases). History of syncope, respiratory failure, RBBB, atrial fibrillation and episodic ventricular tachyarrhythmias were presented in 1 patient with mitochondriopathy, another mitochondriopathy patient developed atrial tachyarrhythmias. Arrhythmia were present in 14 mitochondriopathy patients including RBBB, bifascicular block, intraventricular block, Wolff-Parkinson-White syndrome and short PR interval syndrome. The mtDNA 3243A-G mutation was detected in 8 patients.

CONCLUSIONSIncidence of cardiomyopathy, heart failure and severe arrhythmias is high in patients with mitochondriopathy. Therefore, young cardiomyopathy patients with severe conduction block disorders should undergo relevant etiologic and genetic screening for mitochondriopathy and patients with diagnosed mitochondriopathy should regularly receive electrocardiogram and echocardiography examinations for possible cardiac involvement.

Adolescent ; Adult ; Aged ; Cardiomyopathy, Hypertrophic ; diagnosis ; etiology ; Cardiovascular Abnormalities ; diagnosis ; etiology ; Child ; Child, Preschool ; Female ; Heart Block ; diagnosis ; etiology ; Humans ; Infant ; Male ; Middle Aged ; Mitochondrial Diseases ; complications ; diagnosis ; pathology ; Prognosis ; Retrospective Studies ; Young Adult

Anticoagulants ; therapeutic use ; Cardiomyopathy, Hypertrophic ; complications ; diagnostic imaging ; therapy ; Coronary Angiography ; Death, Sudden, Cardiac ; prevention & control ; Defibrillators, Implantable ; Echocardiography ; Electric Countershock ; Electrocardiography ; Heart Aneurysm ; complications ; diagnostic imaging ; therapy ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tachycardia, Ventricular ; diagnosis ; etiology ; therapy

Cases of iatrogenic coronary artery fistulas draining into the left ventricle after surgical myectomy for hypertrophic obstructive cardiomyopathy have been published as sporadic reports. However, its management scheme and prognosis are not clear because of the low incidence. A 46-yr-old woman was hospitalized for evaluation of chest pain and shortness of breath for 3 months. Transthoracic echocardiographic examination showed typical hypertrophic obstructive cardiomyopathy with a peak pressure gradient of 71 mmHg across the left ventricular outflow tract. The patient underwent surgical septal myectomy. Postoperative color Doppler imaging revealed a diastolic blood flow from the interventricular septal myocardium to the left ventricular cavity, i.e. iatrogenic coronary artery fistula to the left ventricle. Ten days later, the fistula closed spontaneously which was diagnosed by transthoracic echocardiography and confirmed by coronary angiography.
Vascular Fistula/diagnosis/*etiology ; Middle Aged ; *Iatrogenic Disease ; Humans ; Heart Ventricles/*abnormalities ; Heart Septum/*surgery ; Female ; Coronary Vessel Anomalies/diagnosis/*etiology ; Cardiovascular Surgical Procedures/*adverse effects ; Cardiomyopathy, Hypertrophic/complications/*surgery