Hypertrophic obstructive cardiomyopathy and the mitral valve

Background: The purpose of this study was to summarize our experience of extended ventricular septal myectomy in patients with hypertrophic obstructive cardiomyopathy (HOCM).Methods: Thirty-eight patients (26 men, 12 women) with HOCM underwent extended ventricular septal myectomy. The mean age was 36.3 years (range, 18-64 years). Diagnosis was made by echocardiography. The mean (mean � SE) systolic gradient between the left ventricle (LV) and the aorta was 89.3 � 31.1 mm Hg (range, 50-184 mm Hg) according to echocardiographic assessments before the operations. Moderate or severe systolic anterior motion (SAM) of the anterior leaflet of the mitral valve was found in 38 cases, and mitral regurgitation was present in 29 cases. Extended ventricular septal myectomy was performed in all 38 cases. The results of the surgical procedures were evaluated intraoperatively with transesophageal echocardiography (TEE) and with transthoracic echocardiography (TTE) at 1 to 2 weeks after the operation. All patients were followed up with TTE after their operation.Results: All patients were discharged without complications. The TEE evaluations showed that the mean systolic gradient between the LV and the aorta decreased from 94.8 � 35.6 mm Hg preoperatively to 13.6 � 10.8 mm Hg postoperatively (P = .0000) and that the mean thickness of the ventricular septum decreased from 28.3 � 7.9 mm to 11.8 � 3.2 mm (P = .0000). Mitral regurgitation and SAM were significantly reduced or eliminated. During the follow-up, all patients promptly became completely asymptomatic or complained of mild effort dyspnea only, and syncope was abolished. TTE examinations showed that the postoperative pressure gradient either remained the same or diminished.Conclusions: Extended ventricular septal myectomy is mostly an effective method for patients with HOCM, and good surgical exposure and thorough excision of the hypertrophic septum are of paramount importance for a successful surgery.

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Tri-leaflet mitral valve variant of hypertrophic obstructive cardiomyopathy: Comprehensive assessment with 3-D transesophageal echocardiography

International Journal of Cardiology ◽

10.1016/j.ijcard.2015.08.141 ◽

2015 ◽

Vol 201 ◽

pp. 549-551 ◽

Cited By ~ 4

Author(s):

Thomas Butler ◽

Catherina Tjahjadi ◽

Dong Kang ◽

Darryl J. Burstow ◽

John F. Sedgwick

Keyword(s):

Mitral Valve ◽

Transesophageal Echocardiography ◽

Hypertrophic Obstructive Cardiomyopathy ◽

Comprehensive Assessment

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A Case of Mitral Valve Replacement for Hypertrophic Obstructive Cardiomyopathy

Japanese Journal of Cardiovascular Surgery ◽

10.4326/jjcvs.33.295 ◽

2004 ◽

Vol 33 (4) ◽

pp. 295-298

Author(s):

Junji Yunoki ◽

Hitoshi Ohteki ◽

Kozo Naito ◽

Kazuhiro Hisajima

Keyword(s):

Mitral Valve ◽

Valve Replacement ◽

Mitral Valve Replacement ◽

Hypertrophic Obstructive Cardiomyopathy

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New Repair Technique of Systolic Anterior Motion (SAM) of Mitral Valve in Patient with Hypertrophic Obstructive Cardiomyopathy (HOCM): A Case Report

10.1055/s-0039-1678957 ◽

2019 ◽

Author(s):

I. Subbotina ◽

M. Patten ◽

T. Thottakara ◽

H. Reichenspurner ◽

C. Detter

Keyword(s):

Case Report ◽

Mitral Valve ◽

Hypertrophic Obstructive Cardiomyopathy ◽

Systolic Anterior Motion ◽

Repair Technique

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Abstract 15934: Refractory Cardiogenic Shock in a Patient With Hypertrophic Obstructive Cardiomyopathy and Mitral Regurgitation Necessitating Intra-Aortic Balloon Pump Use

Circulation ◽

10.1161/circ.130.suppl_2.15934 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Indrajeet Mahata ◽

Michael Faulx ◽

Snigdha kola ◽

Sweta Singh

Keyword(s):

Mitral Valve ◽

Mitral Regurgitation ◽

Cardiogenic Shock ◽

Pulmonary Edema ◽

Muscle Protein ◽

Hypertrophic Obstructive Cardiomyopathy ◽

Left Ventricular ◽

Afterload Reduction ◽

Intra Aortic Balloon Pump ◽

Refractory Cardiogenic Shock

Introduction: Hypertrophic obstructive cardiomyopathy (HOCM) is a genetic disease due to a mutation in cardiac muscle protein resulting in left ventricular wall and septal hypertrophy. The presence of systolic anterior motion (SAM) of the mitral valve leads to dynamic left ventricular outflow tract (LVOT) obstruction. With increasing SAM of the anterior mitral leaflet there is resultant loss of coaptation leading to mitral regurgitation (MR). MR has been associated with HOCM but severe MR physiology causing refractory cardiogenic shock and requiring the use of afterload reduction through intra-aortic balloon pump (IABP) is rare and seems paradoxical to the conventional therapy for HOCM. Case summary: This is a case of 71year old female with HOCM, presenting with worsening shortness of breath. She had pulmonary vascular congestion on Chest X-ray and her Transthoracic Echocardiography demonstrated significant LVOT obstruction with moderate MR. She was being evaluated for myomectomy while being treated medically with beta blocker therapy for HOCM. She decompensated with acute respiratory failure from pulmonary edema, her blood pressure and oxygen saturation dropped. She was intubated. Swan- ganz catheter reading suggested wedge pressures of 22 and elevated pulmonary pressures. MvO2 was 32% and this was suggestive of cardiogenic shock. The Trans-esophageal echocardiogram (TEE) showed normal EF with severe concentric LVH and a moderate to severe (3+) MR due to restricted leaflet motion with regurgitant orifice area being 2.5cm2. At that point her MR was the dominant physiology behind her acute decompensation and cardiogenic shock and hence an IABP was placed for reducing afterload that helped in stabilizing her. Subsequently her wedge pressure and MvO2 improved, she was weaned off the IABP and extubated. The patient is being evaluated for myomectomy and mitral valve repair. Conclusion: This case illustrates complex hemodynamics and a challenging management due to competing MR and HOCM physiologies, too much central volume to offset HOCM may worsen MR and pulmonary edema while too much afterload reduction might worsen the HOCM. The use of IABP in a HOCM patient though seems paradoxical but was necessary in this setting to deal with complex physiologies.

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