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This case report shows unique aneurysm at the left ventricular apex associated with a double-chambered left ventricle (DCLV) in asymptomatic 69-year-old female. The aneurysm was located at the apex of an accessory chamber, which was formed by hypertrophied muscle band and the interventricular septum, along with a pronounced jet flow directed towards the apex aneurysm. These finding suggests that the obstruction at the base of the accessory chamber caused a pressure overload at the apex, ultimately leading to the development of the aneurysm. To best of our knowledge, this is the first case to prove the underlying cause of aneurysm formation in DCLV.

Background: Myocardial infarction-related left ventricular pseudoaneurysm (LVP), covered by the adjacent pericardial or scar tissue, is a fatal sequela of left ventricular rupture. Whereas hypertrophic cardiomyopathy (HCM) may cause left ventricular true aneurysm. Differentiating LVP from left ventricular true aneurysm is crucial because their natural histories and treatment strategies are distinct. However, the incidence and management of HCM-related LVP remain unknown. Case presentation: An 88-year-old man was admitted to our hospital with sudden-onset chest pain. Upon initial examination, vital signs were stable, and a grade 4/6 systolic murmur was noted. An electrocardiogram revealed atrial fibrillation and poor R-wave progression without ST-T changes or negative T-waves. An echocardiography showed mild left ventricular hypertrophy, mid-ventricular obstruction with a significant intraventricular pressure gradient, left ventricular outflow tract obstruction, and a small left ventricular apical outpouching. Cardiac computed tomography angiography (CCTA) assisted in the diagnosis of LVP, and an accompanying pericardial effusion suggested impending cardiac rupture. Because the patient initially refused our proposed urgent surgery, medication was initiated with continuous hemodynamic monitoring in the intensive care unit; however, the patient's condition did not improve. During a semi-urgent surgical repair of the aneurysmal wall, LVP was observed and confirmed by pathology. Myocardial tissue adjacent to the pseudoaneurysm was consistent with that of HCM. Subsequently, a final diagnosis of HCM-related LVP was made. The postoperative course was notable for transient profound hypotension. Thereafter, the patient died of non-occlusive mesenteric ischemia on day 6. Conclusions: To our knowledge, this is the first reported case of HCM-related LVP mimicking impending cardiac rupture. Our case highlights the importance of considering HCM-related LVP in patients with left ventricular outpouching and CCTA in the LVP diagnosis. In further research, data on the appropriate management of HCM-related LVP should be accumulated.

Background: While there are a variety of surgical options for hypertrophic cardiomyopathy, there are small number of reports of transapical septal myectomy. Furthermore, the characteristics and incidence of anomalous structures in the left ventricle in hypertrophic cardiomyopathy patients which can be identified with imaging studies are not clear. Methods: We studied hypertrophic cardiomyopathy patients who underwent transapical septal myectomy from July 2013 to December 2019. We evaluated the frequency and characteristics of anomalous structures in the left ventricle which had been identified by preoperative examinations and studied their postoperative results. Results: A total of 59 patients was included. The median age was 40 years. Sixteen patients (27.4%) were in New York Heart Association Functional Classification III or IV. The median peak intraventricular gradient at rest was 65 mmHg. By preoperative imaging studies, anomalous structures were detected in 56 cases (94.9%), of which 88% were successfully resected with myectomy. There were two perioperative deaths, while one late death caused by acute myocardial infarction occurred. The estimated 5-year survival rate was 95%. The intraventricular gradient had significantly decreased at the time of discharge, and no reoperation for recurrent obstruction was conducted. The left ventricular ejection fraction had significantly decreased after the operation, was however within the normal range. Left atrium volume index and tricuspid regurgitant velocity significantly improved. Conclusions: Patients receiving transapical septal myectomy restored good hemodynamics from early postoperative period and showed improved subjective symptoms and good mid-term results. With multimodal imaging studies, we could accurately identify anomalous structures in hypertrophic cardiomyopathy patients and reliably treat them by transapical septal myectomy. [ABSTRACT FROM AUTHOR]

Background: Mid‐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. Hypothesis: The severity of MVO increases the incidence of NSVT in patients with hypertrophic obstructive cardiomyopathy (HOCM). Methods: Five 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. Results: Seventy‐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). Conclusions: The presence and severity of MVO was associated with higher incidence of NSVT in HOCM patients. [ABSTRACT FROM AUTHOR]

A 16‐year‐old healthy boy visited our department because of a heart murmur. A 12‐lead electrocardiogram showed left QRS axis deviation and repolarization abnormalities. Transthoracic echocardiography and a computed tomographic scan revealed a hypertrophied papillary muscle and a discrete ridge arising from the septal wall, causing mid‐ventricular obstruction. Doppler echocardiography revealed that the pressure gradient at the obstruction was mild. The patient will be followed up annually, without medication or physical restriction. [ABSTRACT FROM AUTHOR]

A diastolic murmur is informative in the diagnosis of valvular heart disease, such as mitral stenosis and aortic regurgitation. Patients with hypertrophic cardiomyopathy (HCM) could have diastolic murmurs, although this is not widely recognized. We describe an illustrative case of HCM which was found to have a diastolic murmur associated with mid-left ventricular obstruction. An asymptomatic 65-year-old male was referred because of an abnormal electrocardiogram. Cardiac auscultation showed a soft fourth heart sound, a systolic ejection murmur, and a third heart sound followed by a diastolic murmur at the apex. On phonocardiography, the mid-diastolic murmur was predominantly low-pitched with an onset of about 200 ms after the second heart sound and a duration of approximately 150 ms. The timing of the diastolic murmur was consistent with an increased blood inflow during diastole in the mid-ventricular obstruction (2.0 m/s), which was produced by narrowing between the hypertrophied ventricular septum and the protrusion of the anterior papillary muscle. < Learning objective: A diastolic murmur is common in valvular heart diseases, such as mitral stenosis and aortic regurgitation, and can also be heard in hypertrophic cardiomyopathy although not widely recognized. We report an illustrative case of hypertrophic cardiomyopathy with a diastolic murmur related to mid-left ventricular obstruction, findings that would highlight the importance of auscultation regarding not only the differential diagnosis but also risk stratification, given the possible association of diastolic murmurs with adverse outcomes, even in the era of advanced imaging techniques.> [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aims: Percutaneous transluminal septal myocardial ablation by alcohol-induced septal branch occlusion is a new treatment option in symptomatic patients with hypertrophic cardiomyopathy and subaortic, SAM-associated obstruction. We report on a patient with mid-ventricular obstruction and echocardiographic-guided reduction of septal hypertrophy.Methods and Results: A 52-year-old woman with NYHA class III and recurrent exercise-induced syncope suffered from hypertrophic cardiomyopathy with mid-ventricular obstruction. She had a systolic gradient of 71 mmHg at rest and 153 mmHg post-extrasystole, and diastolic inflow gradient of 20 mmHg. Echo-guided percutaneous transluminal septal myocardial ablation with occlusion of the fourth septal branch resulted in acute reduction and final elimination of systolic, as well as diastolic resting and provocable gradients. Complications were not seen. At 3 months’ follow-up the patient was asymptomatic and without further syncopes.Conclusions:Echocardiographic-guided percutaneous transluminal septal myocardial ablation is able to reduce gradients in hypertrophic cardiomyopathy and mid-ventricular obstruction with consecutive improvement of symptoms. [ABSTRACT FROM AUTHOR]

The effect of dual chamber atrioventricular sequential pacing on the intraventricular pressure gradient was tested using Doppler echocardiography in a patient with hypertrophic mid-ventricular obstruction. Atrioventricular sequential pacing with relatively short atrioventricular delays reduced in the left ventricular pressure gradient at the mid-ventricular level. Also, atrioventricular sequential pacing affected the degree and profile of the isovolumetric relaxation flow. These results suggest that atrioventricular sequential pacing affects both systolic and diastolic left ventricular flow dynamics in mid-ventricular obstruction. [ABSTRACT FROM AUTHOR]

We report the case of an 85-year-old woman with distal LV hypertrophy resulting in an intraventricular pressure gradient and incomplete systolic emptying who benefited from permanent DDD pacing. Our experience based on this case suggests that permanent dual chamber pacing might be a safe and effective therapy also in symptomatic patients with the rare form of hypertrophic cardiomyopathy with left mid-ventricular obstruction resulting in incomplete emptying of the apical portion of the LV and a significant intraventricular pressure gradient that was not responding to pharmacological therapy. [ABSTRACT FROM AUTHOR]

Catecholamines are entrenched in the management of shock states. A paradigm shift has pervaded the critical care arena in recent years acknowledging their propensity to cause harm and fuel a 'death-spiral'. We present the case of a 21-year-old male following a witnessed out-of-hospital cardiac arrest who received high-quality cardiopulmonary resuscitation and standard advanced life support for refractory ventricular fibrillation until return of spontaneous circulation after 70 min. Early post-admission echocardiography revealed severe diffuse sub-basal left ventricular hypertrophy with dynamic mid-cavity obstruction and akinetic apical pouching. Within this context, a decatecholaminised strategy comprising a beta-blocker was used to augment the left ventricular end-diastolic volume and attain cardiovascular stability.

A diastolic murmur is informative in the diagnosis of valvular heart disease, such as mitral stenosis and aortic regurgitation. Patients with hypertrophic cardiomyopathy (HCM) could have diastolic murmurs, although this is not widely recognized. We describe an illustrative case of HCM which was found to have a diastolic murmur associated with mid-left ventricular obstruction. An asymptomatic 65-year-old male was referred because of an abnormal electrocardiogram. Cardiac auscultation showed a soft fourth heart sound, a systolic ejection murmur, and a third heart sound followed by a diastolic murmur at the apex. On phonocardiography, the mid-diastolic murmur was predominantly low-pitched with an onset of about 200 ms after the second heart sound and a duration of approximately 150 ms. The timing of the diastolic murmur was consistent with an increased blood inflow during diastole in the mid-ventricular obstruction (2.0 m/s), which was produced by narrowing between the hypertrophied ventricular septum and the protrusion of the anterior papillary muscle. < Learning objective: A diastolic murmur is common in valvular heart diseases, such as mitral stenosis and aortic regurgitation, and can also be heard in hypertrophic cardiomyopathy although not widely recognized. We report an illustrative case of hypertrophic cardiomyopathy with a diastolic murmur related to mid-left ventricular obstruction, findings that would highlight the importance of auscultation regarding not only the differential diagnosis but also risk stratification, given the possible association of diastolic murmurs with adverse outcomes, even in the era of advanced imaging techniques.>.

Summary: A 54-year-old woman visited our hospital because of palpitation and dizziness. Systolic ejection murmur, Levine IV/VI, was heard at the left sternal border and atrial gallop was auscultated at apex. Echocardiogram demonstrated diffuse hypertrophy of the left ventricular walls extending to the apex. The left ventricular walls increased symmetrically; the septal and the posterior wall measured approximately 26mm and 30mm in thickness, respectively. Apical two-chamber views revealed a narrow outflow of the left ventricular cavity at apex with a distinct convergence zone, where color flow Doppler imaging showed turbulent jet flow from mid to late systole. The leukocyte α-galactosidase A activity was lower than normal. Percutaneous endomyocardial biopsy showed specific change for Fabry''s disease by electron microscopy. On cardiac catheterization, mid-ventricular resting pressure gradient of approximately 90mmHg was shown. This pressure gradient dramatically disappeared after intravenous administration of 70mg of cibenzoline. We describe a rare case of Fabry''s disease that revealed diffuse hypertrophy having dynamic obstruction at mid-left ventricle, like mid-ventricular obstruction, and the administration of cibenzoline had beneficial effects on pressure gradient of mid-left ventricle. [Copyright &y& Elsevier]

Abstract: Sigmoid-shaped ventricular septum (SSVS) is frequently observed in elderly patients, but association of mid-ventricular obstruction is rare. In this report, we describe 2 patients with SSVS who had mid-ventricular obstruction and intracavity pressure gradient detected by echocardiography and cine magnetic resonance imaging. Attention should be paid to SSVS because it may cause clinically significant hemodynamic derangements related to mid-ventricular obstruction. [Copyright &y& Elsevier]