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Ventricular septal defect.

Spicer DE, Hsu HH, Co-Vu J, Anderson RH, Fricker FJ - Orphanet J Rare Dis (2014)

Bottom Line: In infants with defects producing large shunts, surgical closure is now recommended in most instances as soon as symptoms manifest.Closure with devices inserted on catheters is now the preferred approach for many patients with muscular defects, often using a hybrid procedure.An approach on this basis permits recognition of the clinically significant phenotypic variants.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Cardiology, University of Florida, Gainesville, Florida, USA. spicerpath@hotmail.com.

ABSTRACT

Background: Ventricular septal defects are the commonest congenital cardiac malformations. They can exist in isolation, but are also found as integral components of other cardiac anomalies, such as tetralogy of Fallot, double outlet right ventricle, or common arterial trunk. As yet, there is no agreement on how best to classify such defects, nor even on the curved surface that is taken to represent the defect.

Methods: Based on our previous pathological and clinical experiences, we have reviewed the history of classification of holes between the ventricles. We proposed that the defects are best defined as representing the area of deficient ventricular septation. This then permits the recognition of clinically significant variants according to the anatomic borders, and the way the curved surface representing the area of deficient septation opens into the morphologically right ventricle.

Results: Clinical manifestation depends on the size of the defect, and on the relationship between systemic and pulmonary vascular resistances. Symptoms include failure to thrive, along with the manifestations of the increase in flow of blood to the lungs. Diagnosis can be made by physical examination, but is confirmed by echocardiographic interrogation, which delineates the precise anatomy, and also provides the physiologic information required for optimal clinical decision-making. Cardiac catheterization offers additional information regarding hemodynamics, particularly if there is a concern regarding an increase in pulmonary vascular resistance. Hemodynamic assessment is rarely necessary to make decisions regarding management, although it can be helpful if assessing symptomatic adults with hemodynamically restrictive defects. In infants with defects producing large shunts, surgical closure is now recommended in most instances as soon as symptoms manifest. Only in rare cases is palliative banding of the pulmonary trunk now recommended. Closure with devices inserted on catheters is now the preferred approach for many patients with muscular defects, often using a hybrid procedure. Therapeutic closure should now be anticipated with virtually zero mortality, and with excellent anticipated long-term survival.

Conclusion: Ventricular septal defects are best defined as representing the borders of the area of deficient ventricular septation. An approach on this basis permits recognition of the clinically significant phenotypic variants.

No MeSH data available.


Related in: MedlinePlus

The view of the opened right atrioventricular junction (A) shows a muscular ventricular septal defect opening to the inlet of the right ventricle beneath the septal leaflet of the tricuspid valve. In the specimen shown in Figure 4B, the free wall of the right ventricle has been lifted away to show a muscular ventricular septal defect opening into the outlet portion of the right ventricle. The ventricular septal defect lies within the arms of the septal band (red Y), with the caudal arm fusing with the inner heart curvature to produce a muscular bar (yellow dots) that interposes between the leaflets of the atrioventricular and arterial valves.
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Fig4: The view of the opened right atrioventricular junction (A) shows a muscular ventricular septal defect opening to the inlet of the right ventricle beneath the septal leaflet of the tricuspid valve. In the specimen shown in Figure 4B, the free wall of the right ventricle has been lifted away to show a muscular ventricular septal defect opening into the outlet portion of the right ventricle. The ventricular septal defect lies within the arms of the septal band (red Y), with the caudal arm fusing with the inner heart curvature to produce a muscular bar (yellow dots) that interposes between the leaflets of the atrioventricular and arterial valves.

Mentions: Although all holes between the ventricles produce the obvious potential for interventricular shunting of blood, not all defects are anatomically the same. The phenotypic differences are important, not only when determining the optimal approach to correction, but also for subsequent genetic counseling. This is because the morphogenesis of the various types is fundamentally different [9]. In terms of phenotypic features, all defects, whether they are isolated or part of a more complex lesion, and when they are viewed from the aspect of the cavity of the right ventricle, can be placed into one of three primary categories [3]. The first category is made up of the holes that have exclusively muscular borders. These holes can have gross malalignment between their caudal and cranial borders. Such malalignment can be described as an additional feature, while recognizing that all defects within this category, when viewed from the right ventricle, have exclusively muscular borders (Figure 4). The defects themselves are described as being muscular. Being encased within the components of the muscular septum, they can open into markedly different parts of the right ventricle. Hence, their geographical location must be described in addition to their anatomic borders as seen from the right ventricle, and in addition to acknowledging the presence of malalignment if present. Their geographical location can be achieved by describing whether they open centrally, apically, anteriorly, or to the right ventricular inlet or outlet components.Figure 4


Ventricular septal defect.

Spicer DE, Hsu HH, Co-Vu J, Anderson RH, Fricker FJ - Orphanet J Rare Dis (2014)

The view of the opened right atrioventricular junction (A) shows a muscular ventricular septal defect opening to the inlet of the right ventricle beneath the septal leaflet of the tricuspid valve. In the specimen shown in Figure 4B, the free wall of the right ventricle has been lifted away to show a muscular ventricular septal defect opening into the outlet portion of the right ventricle. The ventricular septal defect lies within the arms of the septal band (red Y), with the caudal arm fusing with the inner heart curvature to produce a muscular bar (yellow dots) that interposes between the leaflets of the atrioventricular and arterial valves.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4316658&req=5

Fig4: The view of the opened right atrioventricular junction (A) shows a muscular ventricular septal defect opening to the inlet of the right ventricle beneath the septal leaflet of the tricuspid valve. In the specimen shown in Figure 4B, the free wall of the right ventricle has been lifted away to show a muscular ventricular septal defect opening into the outlet portion of the right ventricle. The ventricular septal defect lies within the arms of the septal band (red Y), with the caudal arm fusing with the inner heart curvature to produce a muscular bar (yellow dots) that interposes between the leaflets of the atrioventricular and arterial valves.
Mentions: Although all holes between the ventricles produce the obvious potential for interventricular shunting of blood, not all defects are anatomically the same. The phenotypic differences are important, not only when determining the optimal approach to correction, but also for subsequent genetic counseling. This is because the morphogenesis of the various types is fundamentally different [9]. In terms of phenotypic features, all defects, whether they are isolated or part of a more complex lesion, and when they are viewed from the aspect of the cavity of the right ventricle, can be placed into one of three primary categories [3]. The first category is made up of the holes that have exclusively muscular borders. These holes can have gross malalignment between their caudal and cranial borders. Such malalignment can be described as an additional feature, while recognizing that all defects within this category, when viewed from the right ventricle, have exclusively muscular borders (Figure 4). The defects themselves are described as being muscular. Being encased within the components of the muscular septum, they can open into markedly different parts of the right ventricle. Hence, their geographical location must be described in addition to their anatomic borders as seen from the right ventricle, and in addition to acknowledging the presence of malalignment if present. Their geographical location can be achieved by describing whether they open centrally, apically, anteriorly, or to the right ventricular inlet or outlet components.Figure 4

Bottom Line: In infants with defects producing large shunts, surgical closure is now recommended in most instances as soon as symptoms manifest.Closure with devices inserted on catheters is now the preferred approach for many patients with muscular defects, often using a hybrid procedure.An approach on this basis permits recognition of the clinically significant phenotypic variants.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Cardiology, University of Florida, Gainesville, Florida, USA. spicerpath@hotmail.com.

ABSTRACT

Background: Ventricular septal defects are the commonest congenital cardiac malformations. They can exist in isolation, but are also found as integral components of other cardiac anomalies, such as tetralogy of Fallot, double outlet right ventricle, or common arterial trunk. As yet, there is no agreement on how best to classify such defects, nor even on the curved surface that is taken to represent the defect.

Methods: Based on our previous pathological and clinical experiences, we have reviewed the history of classification of holes between the ventricles. We proposed that the defects are best defined as representing the area of deficient ventricular septation. This then permits the recognition of clinically significant variants according to the anatomic borders, and the way the curved surface representing the area of deficient septation opens into the morphologically right ventricle.

Results: Clinical manifestation depends on the size of the defect, and on the relationship between systemic and pulmonary vascular resistances. Symptoms include failure to thrive, along with the manifestations of the increase in flow of blood to the lungs. Diagnosis can be made by physical examination, but is confirmed by echocardiographic interrogation, which delineates the precise anatomy, and also provides the physiologic information required for optimal clinical decision-making. Cardiac catheterization offers additional information regarding hemodynamics, particularly if there is a concern regarding an increase in pulmonary vascular resistance. Hemodynamic assessment is rarely necessary to make decisions regarding management, although it can be helpful if assessing symptomatic adults with hemodynamically restrictive defects. In infants with defects producing large shunts, surgical closure is now recommended in most instances as soon as symptoms manifest. Only in rare cases is palliative banding of the pulmonary trunk now recommended. Closure with devices inserted on catheters is now the preferred approach for many patients with muscular defects, often using a hybrid procedure. Therapeutic closure should now be anticipated with virtually zero mortality, and with excellent anticipated long-term survival.

Conclusion: Ventricular septal defects are best defined as representing the borders of the area of deficient ventricular septation. An approach on this basis permits recognition of the clinically significant phenotypic variants.

No MeSH data available.


Related in: MedlinePlus