Ventricular septation is a complex process which involves the major genes of cardiac development, acting on myocardial cells from first and second heart fields, and on mesenchymal cells from endocardial cushions. These genes, coding for transcription factors, interact with each other, and their differential expression conditions the severity of the phenotype. In this chapter, we will describe the formation of the ventricular septum in the normal heart, as well as the molecular mechanisms leading to the four main anatomic types of ventricular septal defects: outlet, inlet, muscular, and central perimembranous, resulting from failure of development of the different parts of the ventricular septum. Experiments on animal models, particularly transgenic mouse lines, have helped us to decipher the molecular determinants of ventricular septation. However, a precise description of the anatomic phenotypes found in these models is mandatory to achieve a better comprehension of the complex mechanisms responsible for the various types of VSDs.

Controversies continue regarding several aspects of the anatomy of the morphologically right ventricle. There is disagreement as to whether the ventricle should be assessed in bipartite or tripartite fashion, and the number of leaflets to be found in the tricuspid valve. In particular, there is no agreement as to whether a muscular outlet septum is present in the normally constructed heart, nor how many septal components are to be found during normal development. Resolving these issues is of potential significance to those investigating and treating children with congenitally malformed hearts. With all these issues in mind, we have revisited our own experience in investigating the development and morphology of the normal right ventricle. To assess development, we have examined a large number of datasets, prepared by both standard and episcopic microscopy, from human and murine embryos. In terms of gross anatomy, we have compared dissections of normal autopsied hearts with virtual dissections of datasets prepared using computed tomography. Our developmental and postnatal studies, taken together, confirm that the ventricle is best assessed in tripartite fashion, with the three parts representing its inlet, apical trabecular, and outlet components. The ventricular septum, however, has only muscular and membranous components. The muscular part incorporates a small component derived from the muscularised fused proximal outflow cushions, but this part cannot be distinguished from the much larger part that is incorporated within the free-standing muscular infundibular sleeve. We confirm that the tricuspid valve itself has three components, which are located inferiorly, septally, and antero-superiorly.

Heart rate variability (HRV) is a sign of the cardiac autonomic nervous system. Its evaluation in pediatric ventricular septal defect (VSD) cases before and after transcatheter closure contributes to an understanding of cardiac autonomic control.
Nineteen children with VSDs treated with transcatheter closure and 18 healthy children were enrolled in this study. A 24-h Holter rhythm monitor was applied to all patients before VSD closure and to those in the control group. Holter rhythm monitoring was repeated at three months in the patient group. HRV parameters were measured using the Cardio Scan Premier 12® program. Frequency-domain (total power; very-low-frequency, low-frequency (LF), and high-frequency (HF) indices; and the LF/HF ratio) and time-domain (standard deviation of all RR intervals (SDNN), standard deviation of 5-min averages of RR intervals (SDANN), the SDNN index, percentage of the difference between adjacent RR intervals, and the square root of the mean of the sum of square differences between adjacent filtered RR intervals) parameters were assessed.
Before the procedure, SDNN, SDANN, and total power values were lower in the patient group than in the control group; other parameters were similar in the two groups. No significant difference in the SDNN, SDANN, or total power was detected between the patient and control groups in the third month, indicating that autonomic control of patients\' hearts became normal during the third postoperative month. No correlation was detected between any hemodynamic parameters and any time-domain or frequency-domain parameters before closure.
This study showed that transcatheter closure of VSDs changed HRV parameters in pediatric patients.

Background Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. The surgical approach to TOF continues to evolve, with many centers now favoring early repair for TOF. This study aimed to determine the prevalence of postsurgical complications such as pulmonary insufficiency, residual pulmonary stenosis, and interventricular communication. Methodology We conducted a cross-sectional, observational study with a descriptive and analytic focus involving 61 patients who were operated on for TOF. Results In total, 39 (63.9%) patients had a regular TOF and 22 (36.1%) had an irregular TOF. In our study, 32 (56.14%) patients had a pulmonary insufficiency, of whom 20 had a minimal pulmonary insufficiency (62.5% of pulmonary insufficiency cases), and 79.4% of patients with right ventricular outflow tract enlargement had pulmonary insufficiency (p < 0.005). Among patients who did not have a right ventricular outflow tract enlargement, three cases of pulmonary insufficiency were identified with a prevalence of 16.6%. Six (10.52%) patients had residual pulmonary stenosis. In addition, two (3.2%) cases of minimal residual ventricular septal defects were identified in this study. Conclusions Postoperative complications of TOF treatment are frequent and require medical care throughout the lifetime of patients.

We report a 5-month-old infant who developed an unexpected acute ischaemia of the right lower limb following a surgical perimembranous interventricular communication closure. This rare case of ischaemia was due to an occlusive right common iliac artery dissection. It was early managed by angioplasty with two ABSORB® bioresorbable stents, resulted in complete revascularisation of the right leg.

Double-outlet right ventricle is a form of ventriculoarterial connection. The definition formulated by the International Society for Nomenclature of Paediatric and Congenital Heart Disease is based on hearts with both arterial trunks supported in their greater part by a morphologically right ventricle. Bilateral infundibula and ventricular septal defects are highly debated criteria. This study examines the anatomic controversies surrounding double-outlet right ventricle. We show that hearts with double-outlet right ventricle can have atrioventricular-to-arterial valvular continuity. We emphasize the difference between the interventricular communication and the zone of deficient ventricular septation.
The hearts examined were from the University of Florida in Gainesville; Johns Hopkins All Children\'s Hospital, St Petersburg, Fla; and Lurie Children\'s Hospital, Chicago, Ill. Each specimen had at least 75% of both arterial roots supported by the morphologically right ventricle, with a total of 100 hearts examined. The morphologic method was used to assess anatomic features, including arterial-atrioventricular valvular continuity, subarterial infundibular musculature, and the location of the hole between the ventricles.
Most hearts had fibrous continuity between one of the arterial valves and an atrioventricular valve, with bilateral infundibula in 23%, and intact ventricular septum in 5%.
Bilateral infundibula are not a defining feature of double-outlet right ventricle, representing only 23% of the specimens in our sample. The interventricular communication can have a posteroinferior muscular rim or extend to become perimembranous (58%). Double-outlet right ventricle can exist with an intact ventricular septum.

Many, if not most, of the controversies regarding the description of the congenitally malformed heart have been resolved over the turn of the 20th century. A group of lesions that remains contentious is the situation in which both arterial trunks, in their greater part, are supported by the morphologically right ventricle. It was considered, for many years, that presence of bilateral infundibulums, or conuses, was a necessity for such a diagnosis. It has now been appreciated that this suggestion founders on many counts. In the first instance, such bilateral infundibulums are to be found in patients with other ventriculo-arterial connections, including the otherwise normal heart. In the second instance, it is clear that such an approach abrogates the important principle now known as the morphological method. This states that entities should be defined in terms of their intrinsic morphology and not on the basis of other variable features. It is now also clear that, when assessed simply on the basis of the ventricular origin of the arterial trunks, a significant number of patients fulfil the criteria for so-called \"200%\" origin of the trunks from the right ventricle when there is fibrous continuity between the leaflets of the atrioventricular and arterial valves. In this review, we show how attention to the morphology of the channel between the ventricles now provides the key to accurately diagnose the ventriculo-arterial connection in patients with suspected double-outlet right ventricle. This is because, when both arterial trunks arise exclusively or predominantly from the morphologically right ventricle, the outlet septum, of necessity, is itself a right ventricular structure. The channel between the ventricles, therefore, is roofed by the inner heart curvature, whether that structure is fibrous or muscular. Our observations then confirm that it is the attachment of the outlet septum, which itself can be muscular or fibrous, which determines the commitment of the interventricular communication to the subarterial outlets. The interventricular communication itself, when directly committed to the ventricular outlets, opens between the limbs of the septomarginal trabeculation or septal band. The defect is subaortic when the outlet septum is attached to the cranial limb of the trabeculation, subpulmonary when attached to the caudal limb, and doubly committed when attached to the inner heart curvature in the roof of the defect. Non-committed defects are no longer positioned within the limbs of the septomarginal trabeculation. Although readily demonstrable by a skilled echocardiographer, we show how these anatomical features are more easily demonstrated with added accuracy when using CT data sets.

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Controversy still exists in the categorization of holes between the ventricles, although they are the most common congenital cardiac malformation. Advanced imaging techniques such as three-dimensional echocardiography and computed tomographic angiography offer superb anatomical details of these defects. In this review, we have sought to collate the features highlighted in different categorizations and identify their similarities, but also emphasize their differences. We hope that an analysis of this type, now achievable during life, using advanced imaging, might lead to the appearance of a unified system for diagnosis and description of holes between the ventricles.