Coronary artery-left ventricular fistula with apical hypertrophic cardiomyopathy

We describe two patients with coronary artery-left ventricularfistulae in association with apical hypertrophic cardiomyopathywho presented with chest pain.     

Left ventricular aneurysm associated with apical hypertrophic cardiomyopathy.

A 47-year-old man with apical hypertrophic cardiomyopathy and an apical left ventricular aneurysm with palpitation as the initial manifestation is described. There was no intraventricular pressure gradient. The aneurysm is suggested to be a part of the myocardial disease or to be caused by myocardial bridging of the left anterior descending coronary artery demonstrated by angiography. The 24-hour ambulatory ECG recording showed only isolated ventricular ectopic beats and the clinical course has been favorable during 20 months without therapy.     

心尖肥厚型心肌病的研究进展

心尖肥厚型心肌病(AHCM)是肥厚型心肌病(HCM)的一种较为罕见的表型,其肥厚部位主要限于左室心尖部,主要以V4~V6巨大倒置T波及左室造影呈“锹状”改变为特征。本文对该病发病情况、病因、病理、病理生理及诊治情况的最新进展作一综述。     

Apical hypertrophic cardiomyopathy and ventricular tachycardia

Apical hypertrophic cardiomyopathy was originally reported fromJapan as one of the subsets of hypertrophic cardiomyopathy withoutgradient, usually associated with deep T-wave inversion anda spade-like appearance of the left ventricle. Although manyreports from Japan have indicated that these patients with apicalhypertrophic cardiomyopathy are mostly asymptomatic and haverelatively good prognosis, data on serious ventricular arrhythmiasare not yet available. Present documentation of ventriculartachycardia in a patient with this disease suggests that atleast some of such patients are also at risk of sudden deathand that a systematic study for life-threatening arrhythmiaby ambulatory electrocardiographic monitoring may be necessaryin these cases.     

Apical hypertrophic cardiomyopathy

We describe a patient with asymptomatic apical hypertrophic cardiomyopathy(AHCM)who later developed cardiac arrhythmias,and briefly discuss the diagnostic modalities,differential diagnosis and treatment option for this condition.AHCM is a rare form of hypertrophic cardiomyopathy which classically involves the apex of the left ventricle.AHCM can be an incidental finding,or patients may present with chest pain,palpitations,dyspnea,syncope,atrial fibrillation,myocardial infarction,embolic events,ventricular fibrillation and congestive heart failure.AHCM is frequently sporadic,but autosomal dominant inheritance has been reported in few families.The most frequent and classic electrocardiogram findings are giant negative T-waves in the precordial leads which are found in the majority of the patients followed by left ventricular(LV)hypertrophy.A transthoracic echocardiogram is the initial diagnostic tool in the evaluation of ACHM and shows hypertrophy of the LV apex.AHCM may mimic other conditions such as LV apical cardiac tumors,LV apical thrombus,isolated ventricular non-compaction,endomyocardial fibrosis and coronary artery disease.Other modalities,including left ventriculography,multislice spiral computed tomography,and cardiac magnetic resonance imagings are also valuable tools and are frequently used to differentiate AHCH from other conditions.Medications used to treat symptomatic patients with AHCM include verapamil,beta-blockers and antiarrhythmic agents such as amiodarone and procainamide.An implantable cardioverter defibrillator is recommended for high risk patients.     

Detection of myocardial ischaemia caused by coronary artery-left ventricular fistulae using myocardial contrast echocardiography

A coronary artery-left ventricular fistula (CAF) is an extremelyrare anatomical abnormality in which blood drains directly froma coronary artery into the left ventricle. CAF may cause myocardialischaemia and angina. Myocardial contrast echocardiography (MCE)is a non-invasive technique which assesses myocardial perfusion.We describe a patient with CAF in whom transmural myocardialischaemia was demonstrated using MCE.     

Multimodality imaging in apical hypertrophic cardiomyopathy

Apical hypertrophic cardiomyopathy(AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the firstline imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease.     

心尖肥厚型心肌病的心电图特征

目的探讨心尖肥厚型心肌病的心电图特点。方法对29例心尖肥厚型心肌病的常规12导联心电图进行分析。结果患者V3～V5导联R波电压增高、ST段压低，均表现为V4〉V5〉V3，T波对称性倒置，呈V4〉V5〉V6。V3～V6导联同导联R波高度与T波倒置深度、ST段压低深度呈负相关（P均〈0．05），T波倒置深度与ST段压低深度呈正相关（P〈均0．01）。结论常规心电图显示胸导联R波电压增高伴ST-T特征性改变，要高度考虑心尖肌肥厚性心肌病。     

Mid‐ventricular obstruction is associated with non‐sustained ventricular tachycardia in patients with hypertrophic obstructive cardiomyopathy

BackgroundMid‐ventricular obstruction (MVO) is a rare subtype of hypertrophic cardiomyopathy (HCM) but it is associated with ventricular arrhythmia. The relationship between MVO and non‐sustained ventricular tachycardia (NSVT) in HCM patients is unknown.HypothesisThe severity of MVO increases the incidence of NSVT in patients with hypertrophic obstructive cardiomyopathy (HOCM).MethodsFive hundred and seventy‐two consecutive patients diagnosed with HOCM in Fuwai Hospital between January 2015 and December 2017 were enrolled in this study. Holter electrocardiographic and clinical parameters were compared between HOCM patients with and without MVO.ResultsSeventy‐six (13.3%) of 572 patients were diagnosed with MVO. Compared to patients without MVO, those with MVO were much younger, and had a higher incidence of syncope, greater left ventricular (LV) posterior wall thickness, a higher percentage of LV late gadolinium enhancement, and higher prevalence of NSVT. Furthermore, the prevalence of NSVT increased with the severity of MVO (without, mild, moderate or severe: 11.1%, 18.2%, 25.6%, respectively, p for trend < .01). Similarly, the prevalence of NSVT differed among patients with isolated LV outflow tract (LVOTO), both MVO and LVOTO, and isolated MVO (11.1%, 21.3%, 26.6%, respectively, p for trend = .018). In addition to age, diabetes, left atrial diameter, and maximal wall thickness, multivariate analysis revealed the presence of MVO as an independent risk factor for NSVT (Odds ratio 2.69; 95% confidence interval 1.41 to 5.13, p = .003).ConclusionsThe presence and severity of MVO was associated with higher incidence of NSVT in HOCM patients.     

Apical hypertrophic cardiomyopathy presenting with sustained monomorphic ventricular tachycardia and electrocardiographic changes simulating coronary artery disease and left ventricular aneurysm

A 52-year-old male presented with sustained monomorphic ventricular tachycardia as the initial manifestation of apical hypertrophic cardiomyopathy. The electrocardiogram during normal sinus rhythm showed a pattern of an old anterior wall myocardial infarction with aneurysm formation. Cardiac catheterization documented angiographically normal coronary arteries. Apical hypertrophic cardiomyopathy was documented at cardiac catheterization and by echocardiogram and Doppler studies. Monomorphic ventricular tachycardia was reproducibly initiated and terminated during electrophysiological studies and antiarrhythmic drugs failed to control the tachycardia. At the time of implantation of a cardioverter defibrillator, left ventricular apical biopsy revealed pathologic findings characteristic of hypertrophic cardiomyopathy.     

Transient left ventricular aneurysm in a patient with hypertrophic cardiomyopathy and myocarditis

Left ventricular wall motion abnormalities, aneurysm formation, and progression to global hypokinesis have been described in patients with myocarditis and in patients with hypertrophic cardiomyopathy. We document a case of reversible aneurysm formation, cardiogenic shock, and complete recovery in a patient with myocarditis and hypertrophic cardiomyopathy. Pathophysiologic mechanisms of myocardial injury and recovery are discussed.     

Longitudinal left ventricular strain in hypertrophic cardiomyopathy: correlation with nonsustained ventricular tachycardia

Aims: Stratifying risk of sudden death is a major issue in the management of hypertrophic cardiomyopathy (HCM). Existing risk factors have low positive predictive value and new parameters are needed. Determination of myocardial deformation (strain) by 2D Speckle tracking is a new methodology for determining LV regional function and could correlate with myocite disarray and fibrosis. The aim of this study was to assess the relationship between strain analysis and nonsustained ventricular tachycardia (NSVT) in patients with HCM. Methods: Thirty‐two consecutive patients with HCM (mean age 55, 17–78) were studied. All underwent standard echocardiographic and two‐dimensional strain examination. Twenty‐four‐hour Holter monitoring was performed and echocardiographic parameters were correlated with NSVT. Results: Nine patients (28%) had one or more episodes of NSVT. Patients with NSVT had a higher value of maximal LV thickness (23.6 mm vs. 19.4 mm, P = 0.027). There were no significant associations between NSVT on Holter monitoring and LV outflow gradient left atrial diameter, E/Em or left ventricle ejection fraction. Patients with HCM and NSVT had significant reductions in mid septal, apical‐septal, apical‐lateral strain, and in mean longitudinal strain. Midseptal strain >–10.5% had a sensitivity of 89% and a specificity of 74% (area under the curve, 0.787; P < 0.0013) for predicting NSVT independently of age or maximum wall thickness. Conclusion: Lower end‐systolic peak longitudinal strain obtained by 2D speckle tracking was a predictor of NSVT in HCM patients. This parameter could become a useful tool in stratifying SCD risk in this population. (Echocardiography 2011;28:709‐714)     

Sustained ventricular tachycardia as a first manifestation of hypertrophic cardiomyopathy with mid‐ventricular obstruction and apical aneurysm in an elderly female patient

Sustained ventricular tachycardia complicating left ventricular apical aneurysms has been reported previously solely in middle‐aged patients with hypertrophic cardiomyopathy and mid‐cavity obstruction. We report a case of an elderly female patient who presented with incessant ventricular tachycardia as the first clinical manifestation of hypertrophic cardiomyopathy with mid‐ventricular obstruction and apical aneurysm.     

Non-obstructive apical hypertrophic cardiomyopathy—Emergence of clinical features within three years in a Chinese

A Chinese engineer presented with non-specific palpitation, atypical chest pain and normal electrocardiogram both at rest and during exercise, was documented to develop electrocardiographic evidence of left ventricular hypertrophy and abnormal isotopic cardiac scintigraphy during stress test within a period of three years. His echocardiogram remained unremarkable. Cardiac catheterisation and cineventriculogram confirmed the presence of apical hypertrophic cardiomyopathy, without any outflow tract obstruction or coronary artery disease. This case, together with 14 others detected recently in Hong Kong, will arouse the existence of this form of hypertrophic cardiomyopathy in the Chinese. The emergence of all diagnostic features in this patient within a short time will support an aetiological hypothesis that apical hypertrophic cardiomyopathy can be acquired in patients with genetic predisposition.