scholarly journals Rapid Regression of Obstructive Cardiac Rhabdomyoma in a Preterm Neonate after Sirolimus Therapy

2017 ◽  
Vol 2 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Seung Jae Lee ◽  
Eun Song Song ◽  
Hwa Jin Cho ◽  
Young Youn Choi ◽  
Jae Sook Ma ◽  
...  
Keyword(s):  
Mtor Inhibitor ◽  
Mammalian Target Of Rapamycin ◽  
Mtor Inhibitors ◽  
Ventricular Outflow Tract ◽  
Hemodynamic Instability ◽  
Premature Neonate ◽  
Left Ventricular ◽  
Cardiac Rhabdomyoma ◽  
Left Ventricular Outflow ◽  
Rapid Regression

Cardiac rhabdomyoma can be subclinical or fatal depending on the onset age, involving site, and the size and degree of invasion. Although most rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction induces hemodynamic instability. Mammalian target of rapamycin (mTOR) inhibitors have been used to treat neonates and children with hemodynamically obstructive cardiac rhabdomyoma. Herein, we report a premature neonate at the gestational age of 30 + 4 weeks with severe left ventricular outflow tract obstructive cardiac rhabdomyoma who was successfully treated with the mTOR inhibitor sirolimus. To the best of our knowledge, this is the first recorded case of a premature neonate with obstructive cardiac rhabdomyoma who was successfully treated with an mTOR inhibitor. Therefore, sirolimus could be considered as an alternative medical option for managing premature neonates with obstructive cardiac rhabdomyoma.

scholarly journals Role of Perioperative TEE in Diagnosing and Management of a Case of Dynamic LVOT Obstruction/SAM

2015 ◽  
Vol 3 (2) ◽  
pp. 58-61
Author(s):  
GD Puri ◽  
V Rajkumar
Keyword(s):  
Transesophageal Echocardiography ◽  
Crucial Role ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Hemodynamic Instability ◽  
Left Ventricular ◽  
Systolic Anterior Motion ◽  
Left Ventricular Outflow ◽  
Perioperative Transesophageal Echocardiography

ABSTRACT Systolic anterior motion (SAM) of the mitral leaflets can lead to hemodynamic instability in post bypass period. Perioperative transesophageal echocardiography (TEE) plays a crucial role in the management of SAM. Perioperative echocardiography helps to (a) identify cause of left ventricular outflow tract obstruction, (b) assess SAM and diagnose severity and (c) management of SAM. A case is presented illustrating identification and management of SAM in post bypass period. How to cite this article Kumar A, Rajkumar V, Kumar A, Puri GD. Role of Perioperative TEE in Diagnosing and Management of a Case of Dynamic LVOT Obstruction/SAM. J Perioper Echocardiogr 2015;3(2):58-61.

Rapid Regression of Left Ventricular Outflow Tract Rhabdomyoma After Sirolimus Therapy

PEDIATRICS ◽  
2014 ◽  
Vol 134 (4) ◽  
pp. e1199-e1202 ◽  
Author(s):  
C. Breathnach ◽  
J. Pears ◽  
O. Franklin ◽  
D. Webb ◽  
C. J. McMahon
Keyword(s):  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Left Ventricular Outflow ◽  
Rapid Regression

scholarly journals Excision of Congenital Rhabdomyoma of the Left Outflow Tract through the Left Atrium and Mitral Valve

2018 ◽  
Vol 7 (3) ◽  
pp. 35
Author(s):  
Lucio Careddu ◽  
Francesco Dimitri Petridis ◽  
Emanuela Angeli ◽  
Giorgio Romano ◽  
Valentina Agostini ◽  
...  
Keyword(s):  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Perinatal Period ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Cardiac Murmur ◽  
Cardiac Rhabdomyoma ◽  
Left Ventricular Outflow ◽  
Myocardial Involvement ◽  
The Right

Primary rhabdomyomas obstructing the right or left outflow tract are uncommon findings in the perinatal period. The presenting symptom may be arrhythmia, cardiac murmur, complete or variable atrioventricular block, pericardial effusion, cardiomegaly, cardiac failure, or sudden death. The variety of symptoms can be explained on the basis of obstruction of blood flow, myocardial involvement, and disturbance of the cardiac rhythm. Commonly, rhabdomyoma spontaneously regresses in the majority of cases, and neonatal surgery is advocated only in case of severe left ventricular outflow tract (LVOT) obstruction or the development of arrhythmias. Herein, we describe the pathologic and clinical characteristics of neonatal presentation of a cardiac rhabdomyoma with a nearly obstructive mass in the LVOT, discovered during pregnancy and operated 4 months after delivery.

scholarly journals Transesophageal Echocardiography in the Intensive Care Unit

2013 ◽  
Vol 1 (1) ◽  
pp. 4-15
Author(s):  
Sundar Krishnan ◽  
Dallen Mill
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Transesophageal Echocardiography ◽  
Diagnostic Technique ◽  
Ventricular Outflow Tract ◽  
Hemodynamic Instability ◽  
Pericardial Tamponade ◽  
Left Ventricular ◽  
Hemodynamic Assessment ◽  
Left Ventricular Outflow

ABSTRACT Management of shock in the intensive care unit involves advanced hemodynamic monitoring. Invasive monitoring with central venous lines and pulmonary artery catheters may be inadequate in guiding therapy and improving outcomes. Echocardiography is a reasonably-safe, minimally-invasive diagnostic technique that provides rapid bedside evaluation of ventricular filling and function. While transthoracic echocardiography is the method of choice initially, images can be suboptimal in up to a third of intensive care patients. Transesophageal echocardiography is then required to better evaluate the cause of hemodynamic instability. In addition, transesophageal echocardiography can be used to diagnose other causes of hemodynamic failure (for e.g., pericardial tamponade, pulmonary embolism and left ventricular outflow tract obstruction) and to diagnose intracardiac shunt. Echocardiography is also vital in diagnosing the cause and guiding management in patients with cardiac arrest. Specific training is required for physicians in order to achieve competence in probe insertion, completion of a comprehensive examination and interpretation of the images. In this article, we provide an overview of the indications and complications of the technique and training pathways for the intensivist, followed by transesophageal echocardiography-guided hemodynamic assessment and diagnosis of specific cardiac disorders commonly encountered in the intensive care unit. How to cite this article Krishnan S, Mill D. Transesophageal Echocardiography in the Intensive Care Unit. J Perioper Echocardiogr 2013;1(1):4-15.

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