Idiopathic left ventricular outflow tract pseudoaneurysm.

Left ventricular outflow tract (LVOT) pseudoaneurysm is a rare occurrence and may produce clinically unpredictable symptoms. A very few cases of LVOT pseudoaneurysm are reported and there has always been a predisposing factor in these reported cases such as history of infective endocarditis, myocardial infarction, prosthetic aortic valve replacement or chest trauma. Our patient did not have the above predisposing conditions. Intra operative transesophageal echocardiography helped patient management and guided the surgical team in securing and isolation of the aneurysmal sac from the LVOT.

O diagnóstico da obstrução da via de saída do ventrículo esquerdo na cardiomiopatia hipertrófica / The diagnosis of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

A cardiomiopatia hipertrófica é uma doença genética prevalente caracterizada por hipertrofia ventricular esquerda, em que obstrução dinâmica da via de saída com geração de gradiente subaórtico incide em repouso em 30% dos casos. A obstrução é atribuida complexa interação entre o folheto anterior mitral, o septo interventricular e vetores anômalos de fluxo gerados no ventrículo esquerdo aliada a modificações na geometria da via de saída. Regurgitação mitral em grau variável é detectada associada ou não a deformidades estruturais do aparelho valvar. O ecocardiograma de esforço demonstra obstrução latente facilmente induzida por exercício em 60 a 75% das formas não obstrutivas. A determinação do gradiente nessas condições impõe-se na investigação de rotina dos pacientes com obstrução leve ou ausente em repouso. A avaliação da cardiomiopatia hipertrófica incorpora métodos de imagem baseados no ultrassom, os quais, adicionados ressonância magnética, possibilitam o reconhecimento de mecanismos geradores de obstrução ventricular, de modo a favorecer o diagnóstico e o manejo das formas obstrutivas e obstrutivas latentes.

Hypertrophic cardiomyopathy is a prevalent genetic disease characterized by left ventricular hypertrophy, presenting dynamic obstruction of outflow tract with subaortic gradient happening at rest in 30% of the cases. It is attributed to the intricate interaction between the anterior mitral leaflet, the interventricular septum and altered flow vectors generated in left ventricle along with changes in outflow tract geometry. Mitral regurgitation in varying degrees is found with or without association with structural deformities of the valve apparatus. The exercise echocardiogram evidences latent obstruction easily induced by exercise in 60 to 75% of non-obstructive forms. The determination of the gradient under this condition must be considered in routine investigation of patients with mild or no obstruction at rest. The evaluation of hypertrophic cardiomyopathy incorporates methods based on the ultrasound image, which, along with MRI, allow recognizing ventricular obstruction generating mechanisms, thus facilitating the diagnosis and management of obstructive and latent obstructive forms.

Tunnel type left ventricular outflow tract obstruction: an unusual complex congenital cardiac anomaly in adult

A 24-year-old man, referred to our center with generalized body edema and hypotension. He had a complex congenital heart disease that finally discharged after konno operation

Isolated thrombus-producing right ventricular outflow tract obstruction: an unusual presentation of primary antiphospholipid antibody syndrome.

Right ventricular outflow tract obstruction secondary to an isolated organized thrombus is a rare entity. Proper diagnosis of this condition is essential for the survival of such patients. We describe the case of a middle aged woman who presented with dyspnea and decreased effort tolerance, and was managed with surgical excision of a firm mass which was identified as organized thrombus.

Discrete subvalvular aortic stenosis.

Discrete Subaortic Stenosis is one of the many lesions responsible for left ventricular outflow tract (LVOT) obstruction. It may present as in an isolated from as membranous or fibromuscular ring below the aortic valve or in association with other congenital anamolies such as VSD, PDA, coarctation of aorta, hypoplastic aortic annulus, double chamber right ventricle among others. The condition is rarely diagnosed antenataly or in infancy but often manifests in the first decade of life with features of progressive LVOT obstruction, LV hypertrophy and dysfunction aortic regurgitation due to damage to the aortic cusps because of the jet from the subaortic narrowing which may also render the aortic valve prone to infective endocarditis. Interaction of genetic predisposition and morphologically deformed long and narrow LVOT cause rheological abnormalities and increased shear stress in the region of subaortic stenosis and seem to be the main etiological factor alongwith poorly defined role of more extensive but subtle changes in the LV endocardium. Condition can be easily diagnosed by cross-sectional and Doppler echocardiography and confirmed by demonstrating a pressure gradient below aortic valve on cardiac catheterisation and LV angiography. Surgical membranectomy alongwith myotomy or myomectomy remain the mainstay of treatment but long term results are not satisfactory as there is a high rate of recurrences requiring reoperations. A close follow up with serial echocardiographic examinations is very helpful in early detection of subaortic obstruction in patients who have so called functional murmurs in the childhood.

Correlation of Doppler and cardiac catheterization gradients in children with ventricular outflow obstruction

Los gradientes de presión calculados por el método de Eco Doppler en 23 pacientes pediátricos con obstrucción a la salida del ventrículo derecho (13) o del ventrículo izquierdo (10) fueron comparados con los gradientes de presión obtenidos por cateterismo cardíaco. Los gradientes obtenidos por método de Doppler variaron desde 15 a 148mm Hg y de 15 a 143mm Hg por cateterismo. El coeficiente de correlación linear fue excelente,. 94 en las obstrucciones del ventrículo derecho y .95 en las obstruciones del ventrículo izquierdo, tanto en pacientes con lesiones aisladas com en aquellos con defectos congénitos asociados