Successful use of oral prostaglandin E1 derivative for maintaining ductus-dependent systemic circulation in a neonate with trisomy 18

2019 ◽  
Vol 29 (09) ◽  
pp. 1222-1224
Author(s):  
Akiko Miyake ◽  
Seigo Okada ◽  
Yuichi Ishikawa

AbstractA Japanese female infant with trisomy 18 was diagnosed with hypoplastic left heart syndrome variant. She was administered oral prostaglandin E1 every 6 hours through a feeding tube as an alternative drug for lipo-prostaglandin E1. Oral prostaglandin E1 was effective for maintenance of the ductus arteriosus and may serve as a palliative treatment approach.

2021 ◽  
pp. 1-3
Author(s):  
Ryohei Matsuoka ◽  
Jun Muneuchi ◽  
Yoshie Ochiai

Abstract A newborn with supracardiac total anomalous pulmonary venous connection vein presented the small left ventricle with z score of −7.5, retrograde blood supply in the transverse arch, and the dutcus-dependent systemic circulation. The patient underwent the repair of the anomalous pulmonary vein and bilateral pulmonary arterial banding soon after the birth and then transcatheter pulmonary arterial debanding at the age of 10 months because of an appropriate growth of the left ventricle.


2012 ◽  
Vol 34 (3) ◽  
pp. 656-660 ◽  
Author(s):  
Holly A. Nadorlik ◽  
Matthew J. Egan ◽  
Sharon L. Hill ◽  
John P. Cheatham ◽  
Mark Galantowicz ◽  
...  

Author(s):  
Giovanni Biglino ◽  
Silvia Schievano ◽  
Catriona Baker ◽  
Alessandro Giardini ◽  
Richard Figliola ◽  
...  

The Stage I of Fontan palliation for neonates with hypoplastic left heart syndrome, namely the Norwood procedure, aims to improve the flow of oxygenated blood in the systemic circulation while at the same time providing blood flow to the pulmonary circulation1. This surgical operation usually involves enlargement of the hypoplastic aorta by means of a patch, reconstruction of aortic coarctation and increase pulmonary flow. The latter point, at present, is achieved in three different ways: i) a Blalock-Taussig (BT) shunt from the innominate artery to the pulmonary artery, ii) an atrio-pulmonary shunt, referred to as Sano modification2 and iii) stenting the ductus arteriosus and banding the pulmonary arteries, referred to as “hybrid” Norwood3. In general, it is clear that the circulation following the Norwood procedure presents a very specific and complex arrangement.


2021 ◽  
Vol 8 ◽  
Author(s):  
Hannah Bellsham-Revell

Hypoplastic left heart syndrome (HLHS) is a spectrum of left heart underdevelopment leaving the left side unable to support the systemic circulation. If active management is pursued, then the condition is managed with staged palliation to the Fontan circulation, leaving a systemic right ventricle. Through all surgical stages, and even after completion of Fontan, there are multiple areas that may require intervention, most frequently the branch pulmonary arteries which are essential to a successful Fontan circulation. Echocardiography is the mainstay of assessment, but there is an increasing use of magnetic resonance imaging (MRI) and computed tomography (CT) particularly in relation to extracardiac structures which can be more challenging with echocardiography. Both MRI and CT require set-up, experience and training, and usually sedation or anesthetic in smaller children, but can provide excellent imaging to guide interventions. Cardiac MRI is also able to quantify right ventricular (RV) function which can be challenging on echocardiography. This article describes the modalities available and their use in assessing patients with HLHS prior to catheter interventions.


2013 ◽  
Vol 2 ◽  
pp. 187-189
Author(s):  
Maciej Chojnicki ◽  
Ireneusz Haponiuk ◽  
Mariusz Steffens ◽  
Radoslaw Jaworski ◽  
Aneta Szofer-Sendrowska ◽  
...  

2021 ◽  
Vol 18 (5) ◽  
pp. 416-422
Author(s):  
Nodira M. Normuradova ◽  
Аkram A. Fazilov ◽  
Munisa M. Rasulova

The article discusses the possible diagnostic errors in the hypoplastic left heart syndrome (HLHS) with mitral atresia and intact interventricular septum. “Atrioventricular canal defects” can be commonly and mistakenly diagnosed the prenatal period in such cases. The aspects and possibilities of differential diagnosis of these defects in the prenatal period are discussed. Ultrasound sign is presented in the article, we have named it “hockey stick with puck” that characterizes the retrograde blood flow in the aortic arch. This sign is diagnosed by color Doppler mapping of the sagittal section of the ductus arteriosus and can serve as a marker of mitral/aortic atresia.


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