BACKGROUND Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(G...BACKGROUND Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(GNTs)in left-sided chest leads are the hallmark electrocardiogram(ECG)change of AHCM.CASE SUMMARY The first patient was a 68-year-old woman complaining of recurrent chest tightness persisting for more than 3 years.The second was a 59-year-old man complaining of spasmodic chest tightness persisting for more than 2 years.The third was a 55-year-old woman complaining of recurrent chest pain persisting for 4 mo.In all three cases,GNTs were observed several years prior to apical cardiac hypertrophy after other causes of T-wave inversion were ruled out.CONCLUSION Electrophysiological abnormalities of AHCM appear earlier than structural abnormalities,confirming the early predictive value of ECG for AHCM.展开更多
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 thi...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.展开更多
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 other...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.展开更多
AIM To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy(HCM).METHODS We prospectively assessed 20 patient...AIM To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy(HCM).METHODS We prospectively assessed 20 patients(mean age 53 ± 16 years,range:18-81 years,10 were male),with apical HCM. We measured global longitudinal peak systolic strain(GLPSS) in the midwall and endocardium of the left ventricle. RESULTS The diastolic thickness of the 4 apical segments was 16.25 ± 2.75 mm. All patients had a normal global systolicfunction with a fractional shortening of 50% ± 8%. In spite of supernormal left ventricular(LV) systolic function,midwall GLPSS was decreased in all patients,more in the apical(-7.3% ±-8.8%) than in basal segments(-15.5% ±-6.93%),while endocardial GLPPS was significantly greater and reached normal values(apical:-22.8% ±-7.8%,basal:-17.9% ±-7.5%). CONCLUSION This study shows that two-dimensional strain was decreased mainly confined to the mesocardium,while endocardium myocardial deformation was preserved in HCM and allowed to identify subclinical LV dysfunction. This transmural heterogeneity in systolic strain had not been previously described in HCM and could be explained by the distribution of myofibrillar disarray in deep myocardial areas. The clinical application of this novel finding may help further understanding of the pathophysiology of HCM.展开更多
Objective:Late gadolinium enhancement(LGE) patterns of cardiovascular magnetic resonance (CMR) relying on PSIR(phase-sensitive inversion recovery sequence) techniques had been used to determine the characteristics of ...Objective:Late gadolinium enhancement(LGE) patterns of cardiovascular magnetic resonance (CMR) relying on PSIR(phase-sensitive inversion recovery sequence) techniques had been used to determine the characteristics of LGE in apical hypertrophic cardiomyopathy(ApHCM). Methods:Forty patients pure ApHCM[age,(60.2±10.4) years,31 men]were enrolled.LGE images were acquired using PSIR,and analyzed using a 17-segment model.Summing the LGE areas in all short axis slices yielded the total volume of late enhancement,which was subsequently presented as a proportion of total LV myocardium(%LGE).Results:Mean maximal apical wall thickness was(17.9±2.3) mm,and mean left ventricular(LV) ejection fraction was(67.7±8.0)%.LGE was detected in 130 segments of 30 patients(75.0%),occupying(4.9±5.5)% of LV myocardium.LGE was mainly detected at the junction between left and right ventricles in 12(30%) and at the apex in 28(70%),although LGE-positive areas were widely distributed,and not limited to the apex.Focal LGE at the non-hypertrophic LV segments was found in some ApHCM patients,even without LGE of hypertrophied apical segments.Conclusions:LGE was frequently observed not only in the thickened apex of the heart but also in other LV segments,irrespective of the presence or absence of hypertrophy.The simple presence of LGE on CMR was not representative of adverse prognosis in this population.展开更多
BACKGROUND Apical hypertrophic cardiomyopathy(HCM)is considered to have a benign prognosis in terms of cardiovascular mortality.This serial case report aimed to raise awareness of ventricular fibrillation(VF)and sudde...BACKGROUND Apical hypertrophic cardiomyopathy(HCM)is considered to have a benign prognosis in terms of cardiovascular mortality.This serial case report aimed to raise awareness of ventricular fibrillation(VF)and sudden cardiac death(SCD)in apical HCM.CASE SUMMARY Here we describe two rare cases of apical HCM that presented with documented VF and sudden cardiac collapse.These patients were previously not recommended for primary prevention using implantable cardioverter-defibrillator(ICD)therapy based on current guidelines.However,both received ICD therapy for the secondary prevention of SCD.CONCLUSION These cases illustrate serious complications including VF and aborted sudden cardiac arrest in apical HCM patients who are initially not candidates for primary prevention using ICD implantation based on current guidelines.展开更多
A 54-year-old female with Anderson-Fabry disease(AFD)-R342 Q missense mutation on exon 7 in alphagalactosidase A(GLA) gene- presented with sustained ventricular tachycardia. Imaging confirmed the presence of a new lef...A 54-year-old female with Anderson-Fabry disease(AFD)-R342 Q missense mutation on exon 7 in alphagalactosidase A(GLA) gene- presented with sustained ventricular tachycardia. Imaging confirmed the presence of a new left ventricular apical aneurysm(LVAA) and a significantly reduced intra-cavitary gradient compared to two years prior. AFDcv is an X-linked lysosomal storage disorder caused by GLA enzyme deficiency. The phenotypic expression of AFD in the heart is not well described. Cardiac involvement can include left ventricular hypertrophy(LVH), which is typically symmetric, but can also mimic hypertrophic cardiomyopathy(HCM). Left ventricular apical aneurysm is a rare finding in HCM. We suggest a shared mechanism of LVAA formation in AFD and HCM, independent of the underlying cardiomyopathy. Mechanisms of LVAA formation in HCM include genetic predisposition and long-standing left ventricular wall stress from elevated intra-cavitary systolic pressures due to mid-cavitary obstruction. Both mechanisms are supported in this patient(a brother with AFD also developed a small LVAA). Screening for AFD should be considered in cases of unexplained LVH, particularly in patients with the aneurysmal variant of HCM.展开更多
Background: We report the case of a 58-year-old hypertensive patient under treatment who presented with a ventricular tachycardia unveiling an obstructive cardiomyopathy complicated with an apical aneurysm. Aim: ...Background: We report the case of a 58-year-old hypertensive patient under treatment who presented with a ventricular tachycardia unveiling an obstructive cardiomyopathy complicated with an apical aneurysm. Aim: Highlight the rarity of the case and the difficulty of management. Case Presentation: This patient was transferred from Regional Hospital of Ziguinchor in southern Senegal for a brutal dizzy spell without loss of consciousness. The electrocardiogram showed a wide monomorphic QRS complex regular tachycardia with a rate of 215 beats/min. An external electrical cardio version at 300 joules was applied which led to the recovery of a sinus rhythm on the electrocardiogram. The Doppler echocardiography showed an asymetricalmedio-ventricular hypertrophy with a maximum left intraventricular gradient at 10 mm Hg at rest. The coronarography via the radial artery was normal. The evolution was labeled with a recurrence of the ventricular tachycardia. The patient was then put on amiodarone 200 mg, beta-blocker (bisoprolol 10 mg) and anti-vitamin K (acenocoumarol 4 mg). Facing rhythmic instability, an implantable automatic defibrillator was fitted. No complication has been reported after one year of evolution. Conclusion: HCM with medio ventricular obstruction and apical aneurysm complicated with ventricular arrhythmias is a rare entity. Its management is difficult and controversial.展开更多
文摘BACKGROUND Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(GNTs)in left-sided chest leads are the hallmark electrocardiogram(ECG)change of AHCM.CASE SUMMARY The first patient was a 68-year-old woman complaining of recurrent chest tightness persisting for more than 3 years.The second was a 59-year-old man complaining of spasmodic chest tightness persisting for more than 2 years.The third was a 55-year-old woman complaining of recurrent chest pain persisting for 4 mo.In all three cases,GNTs were observed several years prior to apical cardiac hypertrophy after other causes of T-wave inversion were ruled out.CONCLUSION Electrophysiological abnormalities of AHCM appear earlier than structural abnormalities,confirming the early predictive value of ECG for AHCM.
文摘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.
文摘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.
文摘AIM To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy(HCM).METHODS We prospectively assessed 20 patients(mean age 53 ± 16 years,range:18-81 years,10 were male),with apical HCM. We measured global longitudinal peak systolic strain(GLPSS) in the midwall and endocardium of the left ventricle. RESULTS The diastolic thickness of the 4 apical segments was 16.25 ± 2.75 mm. All patients had a normal global systolicfunction with a fractional shortening of 50% ± 8%. In spite of supernormal left ventricular(LV) systolic function,midwall GLPSS was decreased in all patients,more in the apical(-7.3% ±-8.8%) than in basal segments(-15.5% ±-6.93%),while endocardial GLPPS was significantly greater and reached normal values(apical:-22.8% ±-7.8%,basal:-17.9% ±-7.5%). CONCLUSION This study shows that two-dimensional strain was decreased mainly confined to the mesocardium,while endocardium myocardial deformation was preserved in HCM and allowed to identify subclinical LV dysfunction. This transmural heterogeneity in systolic strain had not been previously described in HCM and could be explained by the distribution of myofibrillar disarray in deep myocardial areas. The clinical application of this novel finding may help further understanding of the pathophysiology of HCM.
基金Supported by Haikou Key Science and Technology Project (2012-075)Haikou Science and Technology Planning Project(2009-049-1)Science and Technology Fund of Haikou Health Bureau(2010-SWY-13-058)
文摘Objective:Late gadolinium enhancement(LGE) patterns of cardiovascular magnetic resonance (CMR) relying on PSIR(phase-sensitive inversion recovery sequence) techniques had been used to determine the characteristics of LGE in apical hypertrophic cardiomyopathy(ApHCM). Methods:Forty patients pure ApHCM[age,(60.2±10.4) years,31 men]were enrolled.LGE images were acquired using PSIR,and analyzed using a 17-segment model.Summing the LGE areas in all short axis slices yielded the total volume of late enhancement,which was subsequently presented as a proportion of total LV myocardium(%LGE).Results:Mean maximal apical wall thickness was(17.9±2.3) mm,and mean left ventricular(LV) ejection fraction was(67.7±8.0)%.LGE was detected in 130 segments of 30 patients(75.0%),occupying(4.9±5.5)% of LV myocardium.LGE was mainly detected at the junction between left and right ventricles in 12(30%) and at the apex in 28(70%),although LGE-positive areas were widely distributed,and not limited to the apex.Focal LGE at the non-hypertrophic LV segments was found in some ApHCM patients,even without LGE of hypertrophied apical segments.Conclusions:LGE was frequently observed not only in the thickened apex of the heart but also in other LV segments,irrespective of the presence or absence of hypertrophy.The simple presence of LGE on CMR was not representative of adverse prognosis in this population.
文摘BACKGROUND Apical hypertrophic cardiomyopathy(HCM)is considered to have a benign prognosis in terms of cardiovascular mortality.This serial case report aimed to raise awareness of ventricular fibrillation(VF)and sudden cardiac death(SCD)in apical HCM.CASE SUMMARY Here we describe two rare cases of apical HCM that presented with documented VF and sudden cardiac collapse.These patients were previously not recommended for primary prevention using implantable cardioverter-defibrillator(ICD)therapy based on current guidelines.However,both received ICD therapy for the secondary prevention of SCD.CONCLUSION These cases illustrate serious complications including VF and aborted sudden cardiac arrest in apical HCM patients who are initially not candidates for primary prevention using ICD implantation based on current guidelines.
基金Supported by Rush University Medical Center,Chicago,IL,United States
文摘A 54-year-old female with Anderson-Fabry disease(AFD)-R342 Q missense mutation on exon 7 in alphagalactosidase A(GLA) gene- presented with sustained ventricular tachycardia. Imaging confirmed the presence of a new left ventricular apical aneurysm(LVAA) and a significantly reduced intra-cavitary gradient compared to two years prior. AFDcv is an X-linked lysosomal storage disorder caused by GLA enzyme deficiency. The phenotypic expression of AFD in the heart is not well described. Cardiac involvement can include left ventricular hypertrophy(LVH), which is typically symmetric, but can also mimic hypertrophic cardiomyopathy(HCM). Left ventricular apical aneurysm is a rare finding in HCM. We suggest a shared mechanism of LVAA formation in AFD and HCM, independent of the underlying cardiomyopathy. Mechanisms of LVAA formation in HCM include genetic predisposition and long-standing left ventricular wall stress from elevated intra-cavitary systolic pressures due to mid-cavitary obstruction. Both mechanisms are supported in this patient(a brother with AFD also developed a small LVAA). Screening for AFD should be considered in cases of unexplained LVH, particularly in patients with the aneurysmal variant of HCM.
文摘Background: We report the case of a 58-year-old hypertensive patient under treatment who presented with a ventricular tachycardia unveiling an obstructive cardiomyopathy complicated with an apical aneurysm. Aim: Highlight the rarity of the case and the difficulty of management. Case Presentation: This patient was transferred from Regional Hospital of Ziguinchor in southern Senegal for a brutal dizzy spell without loss of consciousness. The electrocardiogram showed a wide monomorphic QRS complex regular tachycardia with a rate of 215 beats/min. An external electrical cardio version at 300 joules was applied which led to the recovery of a sinus rhythm on the electrocardiogram. The Doppler echocardiography showed an asymetricalmedio-ventricular hypertrophy with a maximum left intraventricular gradient at 10 mm Hg at rest. The coronarography via the radial artery was normal. The evolution was labeled with a recurrence of the ventricular tachycardia. The patient was then put on amiodarone 200 mg, beta-blocker (bisoprolol 10 mg) and anti-vitamin K (acenocoumarol 4 mg). Facing rhythmic instability, an implantable automatic defibrillator was fitted. No complication has been reported after one year of evolution. Conclusion: HCM with medio ventricular obstruction and apical aneurysm complicated with ventricular arrhythmias is a rare entity. Its management is difficult and controversial.
