Surgical closure of multiple large apical ventricular septal defects: how we do it

AbstractThe management of apical ventricular septal defects continues to be challenging because of the difficulty in achieving a complete closure without a left apical ventriculotomy. In this study, we present our innovative technique of closing multiple and/or large muscular apical ventricular septal defects through a right atriotomy. We operated three patients with multiple apical muscular trabecular ventricular septal defects (“Swiss cheese”) using a technique that involved exclusion of the right ventricular apex. Their ages ranged between 2 months and 13 years. The VSDs were approached through right atriotomy. The trans right atrial approach using a 5–0 polypropylene purse-string suture or a two-patch procedure is a novel method of closing large apical ventricular septal defects. It was found to be effective with no persistent residual defects and did not have the disadvantages of a ventriculotomy.

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Gain-of-function mutation in Gli3 causes ventricular septal defects

10.1101/2020.02.10.942144 ◽

2020 ◽

Author(s):

Antonia Wiegering ◽

Paniz Adibi ◽

Ulrich Rüther ◽

Christoph Gerhardt

Keyword(s):

Cell Proliferation ◽

Developmental Disorders ◽

Current Knowledge ◽

Heart Defects ◽

Ventricular Septal Defects ◽

Gain Of Function ◽

Surgical Closure ◽

Septal Defects ◽

Life Threatening ◽

The Right

ABSTRACTVentricular septal defects (VSDs) are developmental disorders, characterised by a gap in the septum between the right and the left ventricle, that lead to life-threatening heart defects. At present, the only curative treatment of VSDs is surgical closure. Since these surgeries comprise several severe risks, the development of alternative therapies against VSDs is urgently needed. To develop such therapies, the current knowledge of the molecular factors and mechanisms underlying VSDs has to be increased. Based on our previous data, we analysed the relevance of the HH signalling pathway mediator GLI3 in ventricular septum (VS) formation. GLI3 functions as both a transcriptional activator (GLI3-A) and repressor (GLI3-R). By analysing two different mouse Gli3 mutants, we revealed that the lack of GLI3-A with simultaneous presence of GLI3-R impairs cilia-mediated PDGFRα signalling causing reduced cell proliferation and in consequence the development of VSDs. Moreover, we showed that the rescue of PDGFRα signalling restores cell proliferation. Since VSDs are also appear in humans with comparable gain-of-function mutations in GLI3, our findings propose activators of PDGFRα signalling as potential agents against the development of VSDs.SUMMARYThe article reports how a gain-of-function mutation of Gli3 causes ventricular septal defects and paves the way for therapies tackling these congenital heart defects.

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Delayed Closure of Multiple Muscular Ventricular Septal Defects in an Infant after Coarctation Repair and a Hybrid Procedure�A Case Report

The Heart Surgery Forum ◽

10.1532/hsf98.20101081 ◽

2011 ◽

Vol 14 (1) ◽

pp. 67 ◽

Cited By ~ 2

Author(s):

Ireneusz Haponiuk ◽

Maciej Chojnicki ◽

Radosaw Jaworski ◽

Jacek Juciski ◽

Mariusz Steffek ◽

...

Keyword(s):

Septal Defect ◽

Color Doppler ◽

Hybrid Approach ◽

Ventricular Septal Defects ◽

Color Doppler Echocardiography ◽

Septal Defects ◽

Coarctation Repair ◽

Step Procedure ◽

The Right ◽

Delayed Closure

There are several strategies of surgical approach for the repair of multiple muscular ventricular septal defects (mVSDs), but none leads to a fully predictable, satisfactory therapeutic outcome in infants. We followed a concept of treating multiple mVSDs consisting of a hybrid approach based on intraoperative perventricular implantation of occluding devices. In this report, we describe a 2-step procedure consisting of a final hybrid approach for multiple mVSDs in the infant following initial coarctation repair with pulmonary artery banding in the newborn. At 7 months, sternotomy and debanding were performed, the right ventricle was punctured under transesophageal echocardiographic guidance, and the 8-mm device was implanted into the septal defect. Color Doppler echocardiography results showed complete closure of all VSDs by 11 months after surgery, probably via a mechanism of a localized inflammatory response reaction, ventricular septum growth, and implant endothelization.

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Faculty Opinions recommendation of Transcatheter versus surgical closure of perimembranous ventricular septal defects in children: a randomized controlled trial.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.718270614.793492370 ◽

2014 ◽

Author(s):

Larry Latson ◽

Athar Qureshi

Keyword(s):

Randomized Controlled Trial ◽

Controlled Trial ◽

Ventricular Septal Defects ◽

Surgical Closure ◽

Randomized Controlled ◽

Septal Defects

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Surgical closure of muscular ventricular septal defects using double umbrella devices (intraoperative VSD device closure)

European Journal of Cardio-Thoracic Surgery ◽

10.1016/s1010-7940(97)00086-9 ◽

1997 ◽

Vol 12 (3) ◽

pp. 450-455 ◽

Cited By ~ 21

Author(s):

B Murzi

Keyword(s):

Ventricular Septal Defects ◽

Device Closure ◽

Surgical Closure ◽

Septal Defects

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Initial Design and Evaluation of a Pediatric Intra-Cardiac Camera System for Ventricular Septal Defects

Journal of Medical Devices ◽

10.1115/1.4005778 ◽

2012 ◽

Vol 6 (1) ◽

Author(s):

Mark E. Rentschler ◽

Keir D. Hart ◽

Max B. Mitchell

Keyword(s):

Left Ventricular ◽

Primary Objective ◽

Preliminary Evaluation ◽

Design Development ◽

Ventricular Septal Defects ◽

Camera System ◽

Surgical Closure ◽

Septal Defects ◽

Commercial Device

The primary objective of this project is to design, fabricate, and test a small, integrated camera system for aiding in the visualization and surgical repair of certain types of ventricular septal defects (VSD), in pediatric patients. Currently, no purpose-designed commercial device to view VSDs from the left ventricle of the heart exists. The left ventricular perspective is ideal for obtaining an unobstructed view of the VSD. This VSD camera device would also provide a platform for passing a suture through the hole in the ventricular septum, with future work implementing additional tools capable of more advanced tasks. This camera device will help solve some of the major issues currently associated with cardiac imaging and surgical closure of VSDs in newborns and young children This paper examines the design development and preliminary evaluation of a proof of concept device. Included are preliminary results of image quality comparisons, design details of a pediatric-specific VSD camera device, and initial outcomes from in vitro testing.

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