The relationship between systolic anterior motion of the mitral valve and the left ventricular outflow tract Doppler in hypertrophic cardiomyopathy

We report a case of a 73-year-old female patient, who was admitted to the coronary care unit due to chest pain, malaise and near syncope. During physical examination, the patient was hypotensive and there were signs of left-sided heart failure and a loud systolic murmur. Echocardiogram showed apical ballooning with dynamic left ventricular outflow tract obstruction, based on systolic anterior motion of the mitral valve with important mitral valve regurgitation. In the acute setting, the cardiogenic shock was treated cautiously with fluid resuscitation and intravenous metoprolol, resulting in direct stabilisation of her haemodynamic condition. As a codiagnosis, there was a significant stenosis of left anterior descending artery, which was treated successfully by percutaneous coronary intervention with drug eluting stents. During follow-up, left ventricular function normalised, and the left ventricular outflow tract obstruction, systolic anterior motion of mitral valve and related mitral regurgitation all resolved.

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Alcohol septal ablation for recurrent left ventricular outflow tract obstruction after combined myectomy and mitral valve repair in hypertrophic cardiomyopathy

International Journal of Cardiology ◽

10.1016/j.ijcard.2013.03.102 ◽

2013 ◽

Vol 167 (2) ◽

pp. e30-e32

Author(s):

Angelos G. Rigopoulos ◽

Barbara Pfeiffer ◽

Christoph Schimmer ◽

Jürgen Illnitzky ◽

Rainer Leyh ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Mitral Valve ◽

Mitral Valve Repair ◽

Left Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Outflow Tract ◽

Valve Repair ◽

Alcohol Septal Ablation ◽

Left Ventricular Outflow

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Membranous septal aneurysm in a child with Noonan syndrome and hypertrophic cardiomyopathy

10.22541/au.161496450.06346078/v1 ◽

2021 ◽

Author(s):

PRIYADARSHINI ARUNAKUMAR ◽

Usha MK ◽

RAMYA S ◽

Jayaranganath M

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Mitral Valve ◽

Noonan Syndrome ◽

Left Ventricular Outflow Tract ◽

Interventricular Septum ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Outflow Tract ◽

Mitral Leaflet ◽

Left Ventricular Outflow

A four year old boy was diagnosed with hypertrophic cardiomyopathy with moderate degree of obstruction of the left ventricular outflow tract, secondary to asymmetric septal hypertrophy and no features of congestive heart failure, at the age of the 7 months. He was also found to have myxomatous mitral valve, systolic anterior motion of anterior mitral leaflet and moderate mitral regurgitation. There was no evidence of ventricular septal defect at this point in time.In view of phenotypic features suggestive of Noonan syndrome, he underwent genetic evaluation with target gene sequencing and was detected to have a novel heterozygous mutation in exon 13 of LZTR 1 (Leucine- zipper-like transcriptional regulator 1) gene. Echocardiogram on follow up showed increase in left ventricular outflow tract obstruction and appearance of an aneurysm in the membranous portion of interventricular septum. There were no features of right ventricular outflow obstruction, tricuspid regurgitation or aortic regurgitation. The appearance of the membranous septal aneurysm may be related to the direction of jet arising from the point of contact of the anterior mitral leaflet with the hypertrophied basal septum and hitting this part of the interventricular septum. It is an unusual mechanism for formation of membranous septal aneurysm, given its absence in the earlier echocardiograms and absence of any left to right shunt across the aneurysm. He has been initiated on beta blockers due to severe left ventricular outflow tract obstruction and is planned for septal myomectomy, resection of membranous septal aneurysm and mitral valve repair.

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