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      Recent advances in managing septal defects: ventricular septal defects and atrioventricular septal defects

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          Abstract

          This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD) is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe and effective and prevent the development of PVOD and cardiac dysfunction.

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          Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience.

          To understand better the contribution of a right atrium in a valveless atriopulmonary connection, we performed some basic hydrodynamic studies. Pulsation of a valveless chamber in a simple continuous flow circuit was found to generate turbulence and thereby to increase resistance to net forward flow. Visualization of flow through cavities and around corners and measurements of energy losses across nonpulsatile cavities, corners, and stenoses indicated the importance of streamlining. These studies suggested ways in which hydrodynamic designs of the Fontan circulation might be improved. In parallel with these in vitro studies, we have developed a modified approach to Fontan reconstruction that entails exclusion of most or all of the right atrium (total cavopulmonary connection). The operation consists of three parts: (1) end-to-side anastomosis of the superior vena cava to the undivided right pulmonary artery; (2) construction of a composite intraatrial tunnel with the use of the posterior wall of the right atrium; and (3) use of a prosthetic patch to channel the inferior vena cava to the enlarged orifice of the transected superior vena cava that is anastomosed to the main pulmonary artery. The operation was performed in 20 patients between March 1987 and March 1988. The diagnoses were double-inlet ventricle (11 patients), hypoplastic systemic or pulmonary ventricle (seven patients), and absent right atrioventricular connection (two patients). There were two early deaths and one late death. None of the deaths was related to the actual procedure but rather to increased pulmonary vascular resistance (two patients) or systemic ventricular failure (one patient). Total cavopulmonary connections have the following advantages: (1) They are technically simple and reproducible in any atrioventricular arrangement and are away from the atrioventricular node; (2) most of the right atrial chamber remains at low pressure, which reduces the risk of early or late arrhythmias; (3) reduction of turbulence prevents energy losses and should minimize the risk of atrial thrombosis; (4) postoperative cardiac catheterization performed in 10 patients confirmed these favorable flow patterns with minimal gradients throughout the connections. These encouraging early results support the continuing use of total cavopulmonary connection, at least for patients with a nonhypertrophied right atrium.
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            Transcatheter closure of perimembranous ventricular septal defects: early and long-term results.

            We sought to analyze safety, efficacy, and follow-up results of percutaneous closure of perimembranous ventricular septal defects (pmVSD). Results of pmVSD transcatheter closure have been reported in the literature; however, follow-up data are still limited. Between January 1999 and June 2006, 104 patients underwent percutaneous closure of a pmVSD at our institution. An Amplatzer VSD device (muscular or eccentric) (AGA Medical Corp., Golden Valley, Minnesota) was used in all subjects. The mean age at closure was 14 years (range 0.6 to 63 years). The attempt to place a device was successful in 100 patients (96.2%). The median device size used was 8 mm (range 4 to 16 mm). No deaths occurred. Total occlusion rate was 47% at completion of the procedure, rising to 84% at discharge and 99% during the follow-up. A total of 13 early complications occurred (11.5%), but in all but 2 subjects (1.9%) these were transient. The median follow-up was 38.5 months. The most significant complication was complete atrioventricular block (cAVB), which required pacemaker implantation in 6 subjects (5.7%; 2 in the early phase and 4 during the follow-up). Cox proportional hazards regression analysis showed that the only variable significantly associated with the occurrence of this complication was age at the time of the procedure (p = 0.028; relative risk 0.25). All subjects experiencing this problem were <6 years old. In the current era and in experienced hands, pmVSD closure can be performed safely and successfully. The major concern is the occurrence of cAVB; therefore, very careful monitoring of rhythm is mandatory during follow-up.
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              Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.

              The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more (p < 0.001), younger age at operation (p = 0.001), heterotaxy syndrome (p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve (p = 0.001), pulmonary artery distortion (p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage (p = 0.001), the absence of a baffle fenestration (p = 0.002), and longer cardiopulmonary bypass time (p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation (p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure (p = 0.003). These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Formal AnalysisRole: InvestigationRole: MethodologyRole: Project AdministrationRole: SupervisionRole: Writing – Review & Editing
                Role: Writing – Original Draft Preparation
                Journal
                F1000Res
                F1000Res
                F1000Research
                F1000Research
                F1000 Research Limited (London, UK )
                2046-1402
                26 April 2018
                2018
                : 7
                : F1000 Faculty Rev-498
                Affiliations
                [1 ]University of Texas-Houston McGovern Medical School, Children Memorial Hermann Hospital, Houston, USA
                [2 ]Pediatrix Cardiology Associates of New Mexico, Albuquerque, USA
                Author notes

                PSR provided conceptualization, methodology, project administration, and supervision and contributed to the writing of the article by reviewing and editing the manuscript. ADH contributed to the writing of the article by preparing the original draft.

                No competing interests were disclosed.

                Author information
                https://orcid.org/0000-0002-6382-407X
                Article
                10.12688/f1000research.14102.1
                5931264
                b32f8c9b-af6d-40f9-b628-87b847a7fbb9
                Copyright: © 2018 Rao PS and Harris AD

                This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 20 April 2018
                Funding
                The author(s) declared that no grants were involved in supporting this work.
                Categories
                Review
                Articles

                ventricular septal defect,atrioventricular septal defect,surgery,percutaneous treatment,hybrid procedure

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