Recurrent cardiogenic shock caused by dynamic mitral regurgitation in a patient with hypertrophic obstructive cardiomyopathy: a case report

A 68-year-old man with hypertrophic obstructive cardiomyopathy developed recurrent cardiogenic shock due to dynamic mitral regurgitation. The pressure gradient in the left ventricular outflow tract under medication was 30 mmHg, and he complained of no symptom. He developed shock with dyspnea suddenly after bathing and defecation. Echocardiography and left ventriculography revealed massive mitral regurgitation and mild increment of pressure gradient in the left ventricular outflow tract (50 mmHg). He underwent successful mitral valve replacement following treatment with beta-blocker under intraaortic balloon pumping support. This case illustrates that exacerbation of the systolic anterior motion of the mitral anterior leaflet can cause dynamic severe mitral regurgitation with 'mild' increment of pressure gradient in the left ventricular outflow tract, resulting in cardiogenic shock with severe lung edema.     

Evolution of dilated cardiomyopathy from hypertrophic obstructive cardiomyopathy: a case report

A 20-year-old woman whose echocardiograms showed a rare evolution from hypertrophic to dilated cardiomyopathy during a nine year observation period is described. This patient was initially diagnosed as having hypertrophic obstructive cardiomyopathy (HOCM) at the age of 12 years. Her echocardiogram showed marked thickening of the interventricular septum (IVS) and left ventricular posterior wall (LVPW), asymmetric septal hypertrophy (ASH) and systolic anterior motion of the mitral valve (SAM). Chest radiography revealed a prominent left ventricular border and a cardiothoracic ratio of 0.52. At 18 years of age she experienced onset of palpitation during the 16th week of pregnancy. Her echocardiogram recorded in June 1980 revealed a thickened IVS and LVPW with resolution of the SAM and of the narrow cavity. At 20 years of age she became pregnant again and was admitted to our hospital for the third time at 24 weeks gestation. On admission her blood pressure was 122/60 and her pulse was 56, and moist rales were audible over both lung fields. Peripheral edema was noted. Chest radiography revealed moderate cardiomegaly, a cardiothoracic ratio of 0.66, and congestion of the pulmonary vasculature. An echocardiogram showed thinning of the IVS and LVPW with hypokinesis and dilatation of the cavity compatible with dilated cardiomyopathy (DCM). She delivered a boy on July 15th 1982 at 32 weeks gestation following which she developed marked congestive heart failure. She expired one month later. A chest radiograph made one day before death revealed marked cardiomegaly, a cardiothoracic ratio of 0.76, prominent pulmonary vasculature and a pleural effusion. Autopsy was refused; therefore the terminal DCM-like features were not clarified pathologically. However, the slow progression from HOCM to HCM during eight years and then rapid progression from HCM to DCM during four months was most impressive.     

Possible acute and chronic synergistic effect of dual chamber pacing and disopyramide in obstructive hypertrophic cardiomyopathy: a case report

It remains unclear whether the combination of dual‐chamber (DDD) pacing and disopyramide can achieve prolonged left ventricular outflow tract (LVOT) gradient reduction and symptom relief in patients with obstructive hypertrophic cardiomyopathy (HCM). In an HCM patient with a severe LVOT gradient, the combination of DDD pacing and disopyramide achieved marked improvement of gradient in the catheter laboratory and also after medium‐term follow‐up. The patient's severe dyspnoea was alleviated during the follow‐up period. This combination might enable physicians to treat and manage elderly symptomatic obstructive HCM patients with a severe LVOT gradient more effectively and less invasively.     

A primer of disopyramide treatment of obstructive hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) occurs in 1 in 500 individuals. Treatment options for HCM differ from those administered in coronary disease, heart failure, and valvular disease patients that comprise the core of many cardiology practices. In this article, we offer a concise summary of the therapeutic use of disopyramide for reducing gradients and relieving symptoms in obstructive HCM.     

Effect of disopyramide on left ventricular pressure gradient in hypertrophic obstructive cardiomyopathy in comparison with propranolol--a case report

The effect of intravenous administration of disopyramide (total dose 100 mg, bolus 20 mg every 5 minutes) was compared with that of propranolol (total dose 10 mg, bolus 2 mg every 5 minutes) in a patient with hypertrophic obstructive cardiomyopathy. Left ventricular pressure gradient (LVPG) was assessed by continuous wave Doppler flowmetry. LVPG markedly decreased (97 to 16 mmHg), and preejection period (PEP) increased with an increase in heart rate (HR) during disopyramide injection. No changes were observed in LVPG and PEP, and a decrease occurred in HR during propranolol administration. These results indicate that disopyramide produced greater effects on the reduction of LVPG than propranolol, a negative inotropic agent, did.     

The recurrence of tako-tsubo cardiomyopathy complicated by cardiogenic shock: a case report

Tako-tsubo cardiomyopathy is a stress-related cardiomyopathy which occurs in postmenopausal women after severe emotional stress. Although no evidence supporting specific treatment with tako-tsubo cardiomyopathy has been established, the prognosis is considered favorable with normalization of wall motion abnormalities within weeks. In addition, recurrence of this syndrome seems to be rare. Now, we report a recurrent case of tako-tsubo cardiomyopathy complicated by cardiogenic shock after repeated emotional stress.     

Effective disopyramide treatment in a boy with mid-ventricular hypertrophic obstructive cardiomyopathy.

A 14-year-old boy with mid-ventricular hypertrophic obstructive cardiomyopathy (MVHOCM) first presented at the age of 10 years with severe chest pain. Two-dimensional echocardiography disclosed marked hypertrophy at the mid-portion of the ventricular septum, and left ventriculography showed an hourglass appearance at systole. He was initially treated with propranolol, but the chest pain and dyspnea on exertion worsened at the age of 12 years. After disopyramide was started, the chest pain disappeared and the degree of the pressure gradient at the mid-ventricular level was reduced. There was also significant improvement on a 123I beta-methyliodophenyl pentadecaonic acid (BMIPP) myocardial scintigram.     

Efficacy of cibenzoline for chest oppression and ventricular arrhythmia during effort and alcohol consumption in a patient with hypertrophic obstructive cardiomyopathy: a case report

A 67-year-old man presented with chest oppression and palpitation during effort and alcohol consumption. Echocardiography demonstrated asymmetric septal hypertrophy and systolic anterior motion of the anterior mitral leaflet with a pressure gradient of 80 mmHg across the left ventricular outflow tract (LVOT), leading to the diagnosis of hypertrophic obstructive cardiomyopathy. During the treadmill exercise test, blood pressure decreased with electrocardiographic ST-segment depression and subsequent frequent premature ventricular contractions. Holter-electrocardiographic monitoring also showed ST-segment depression with premature ventricular contractions during effort and alcohol consumption. Coronary angiography showed no abnormalities and cardiac catheterization at baseline showed a systolic pressure gradient of only 2 mmHg across the LVOT. However, the gradient increased to 33 mmHg after premature ventricular contraction, 27 mmHg at Valsalva maneuver and 75 mmHg with dobutamine infusion (5 micrograms/kg/min) and disappeared with 70 mg of intravenous cibenzoline. Medication with cibenzoline (300 mg/day) for one month reduced the LVOT gradient at rest to 53 mmHg and strikingly improved symptoms and exercise tolerance and also suppressed premature ventricular contractions during exercise and alcohol consumption. We conclude that cibenzoline was effective for reduction of LVOT gradient both at rest and during exercise and alcohol consumption.     

Junctional rhythm in hypertrophic cardiomyopathy: a case report

We observed a case of a 47-year-old male patient with hypertrophic cardiomyopathy and family history of sudden death. During cardiac catheterization, the patient presented spontaneous intermittent atrioventricular junctional rhythm without significant changes related to sinus heart rate. The gradient was absent with sinus beats, but the junctional rhythm induced the appearance of a left ventricular-aorta gradient, with parallel reduction of aortic pressure from 156 to 120 mm Hg. Subsequently, a couple of pre-mature ventricular beats induced an important post-extrasystolic potentiation of the gradient, followed in the last beats by sinus rhythm with disappearance of the gradient. Our case suggests that the decrease of left ventricular volume, due to the absence of an effective atrial systole, leads to left ventricular increased gradient, not imputable to an increased inotropic effect or heart rate modifications but to the decrease of pre-load.     

Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy

OBJECTIVES: In this study we assessed the long-term efficacy and safety of disopyramide for patients with obstructive hypertrophic cardiomyopathy (HCM). BACKGROUND: It has been reported that disopyramide may reduce left ventricular outflow gradient and improve symptoms in patients with HCM. However, long-term efficacy and safety of disopyramide has not been shown in a large cohort. METHODS: Clinical and echocardiographic data were evaluated in 118 obstructive HCM patients treated with disopyramide at 4 HCM treatment centers. Mortality in the disopyramide-treated patients was compared with 373 obstructive HCM patients not treated with disopyramide. RESULTS: Patients were followed with disopyramide for 3.1 +/- 2.6 years; dose 432 +/- 181 mg/day (97% also received beta-blockers). Seventy-eight patients (66%) were maintained with disopyramide without the necessity for major non-pharmacologic intervention with surgical myectomy, alcohol ablation, or pacing; outflow gradient at rest decreased from 75 +/- 33 to 40 +/- 32 mm Hg (p < 0.0001) and mean New York Heart Association functional class from 2.3 +/- 0.7 to 1.7 +/- 0.6 (p < 0.0001). Forty other patients (34%) could not be satisfactorily managed with disopyramide and required major invasive interventions because of inadequate symptom and gradient control or vagolytic side effects. All-cause annual cardiac death rate between disopyramide and non-disopyramide-treated patients did not differ significantly, 1.4% versus 2.6%/year (p = 0.07). There was also no difference in sudden death rate, 1.0%/year versus 1.8%/year (p = 0.08). CONCLUSIONS: Two-thirds of obstructed HCM patients treated with disopyramide could be managed medically with amelioration of symptoms and about 50% reduction in subaortic gradient over >/=3 years. Disopyramide therapy does not appear to be proarrhythmic in HCM and should be considered before proceeding to surgical myectomy or alternate strategies.     

Percutaneous transluminal septal ablation with ethanol in hypertrophic obstructive cardiomyopathy: a case report]

A 48-year-old man with hypertrophic obstructive cardiomyopathy was treated by nonsurgical septal ablation. He had previously received medical treatment using atenolol and verapamil, but severe symptoms of chest discomfort, dyspnea, and shortness of breath had persisted for 2 years. Under coronary angiography control, the first major septal branch of the anterior descending coronary artery was catheterized with a 2.0 mm coaxial percutaneous transluminal coronary angioplasty balloon catheter. After inflation of the balloon, 1.5 ml of absolute alcohol was slowly injected into the septal artery to induce a small septal infarction. The left ventricular outflow pressure gradient was decreased from 108 before to 30 mmHg after the procedure as measured by Doppler echocardiography. His chest symptom improved from New York Heart Association class III to II and left ventricular outflow gradient was maintained at 0 mmHg (at rest) at 3 months after treatment. This is a new, less invasive treatment using catheterization instead of surgical myectomy. The indication, complication, and long-term effect of the treatment must be carefully evaluated, but this is expected to become a useful method.     

Tako-Tsubo cardiomyopathy presenting with cardiogenic shock successfully treated with milrinone: A case report

We report the case of a middle age patient presenting with Tako-Tsubo cardiomyopathy (TTC) complicated by cardiogenic shock that was successfully handled with milrinone. A 64-year old man presented with cardiogenic shock after benzodiazepine and alcohol intoxication. A slight elevation of troponin and typical left ventricular ballooning without coronary lesions suggested TTC. Within a few hours milrinone infusion normalized the cardiac index. TTC is responsible for severe transient left ventricular dysfunction occurring after physical or psychological stress. The major pathophysiological mechanism involved is disproportionate catecholamine secretion, which may stun the myocardium. We considered if treatment of this unique physiopathology with catecholamines could be dangerous in these patients and if alternative inotropes such as milrinone should be preferred.     

Cibenzoline attenuates coronary systolic reversal flow in a patient with hypertrophic obstructive cardiomyopathy: a case report

Effects of cibenzoline on coronary hemodynamics, especially systolic reversal flow, were assessed in a 53-year-old man with hypertrophic obstructive cardiomyopathy by coronary flow study using a Doppler guidewire. Intravenous administration of cibenzoline resulted in decreases in the pressure gradient (60 to 0mmHg) and systolic left ventricular pressure (162 to 126 mmHg), and an increase in systolic arterial pressure (102 to 132mmHg). Furthermore, the systolic reversal flow observed at baseline was markedly diminished following administration of cibenzoline. Therefore, we speculate that cibenzoline reduces ventricular wall stress by diminishing the left ventricular obstruction that occurs with compression of the intramyocardial arteries. Cibenzoline-induced attenuation of coronary systolic reversal flow may be associated with the effectiveness of cibenzoline for diminishing the left ventricular obstruction.