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.

BACKGROUND: The Gerbode defect is an infrequent cardiac anomaly, with an incidence below 1 % in current worldwide literature. It consists of a communication between the left ventricle and right atrium in the membranous septum with consequential hemodynamical and structural heart changes and can present as either congenital or acquired. The concomitant affectation of the tricuspid valve poses its difficulty as a diagnostic and therapeutical challenge.
METHODS: This case report presents a 27-year-old patient with an incidental finding of congenital Gerbode defect with hemodynamical repercussion during surgical treatment for multidisciplinary management in the context of open thoracic trauma.
CONCLUSIONS: This defect has been infrequently described in the literature, and although several classification methods have been proposed, its diagnosis is challenging and must be considered when faced with nonspecific cardiac systems.
CONCLUSIONS: It reports an infrequent congenital heart defect posing as traumatic or postoperative, generating a challenging diagnosis and successful surgical treatment given to the patient using a multidisciplinary approach to further broaden scientific literature on such an underrepresented pathology.

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.

Left ventricular diverticulum is a rare cardiac congenital anomaly. It is believed to be caused by the impaired development of the endocardial tube during the fourth week of embryologic development. It contains endocardium, myocardium, and pericardium and displays normal contraction. We hereby report a case of a patient with a left ventricular apical diverticulum who presented with unstable angina and was initially misdiagnosed as a ventricular septal defect. Since there were no complications or symptoms associated with the anomaly, the management was conservative. This manuscript also highlights the importance of using multiple diagnostic imaging modalities to exclude differential diagnoses.

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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.

BACKGROUND: Intrapericardial diaphragmatic hernia is an abdominal organ prolapse inside the pericardium. It is one of the less frequent instances within the diaphragmatic hernia group.
METHODS: This is the case of a 4-month-old infant undergoing surgery for interventricular communication (IVC). Postoperatively, pulmonary auscultation detected air-fluid sounds, and thoracic radiological control showed an atypical pneumopericardium. Given clinical and radiological findings, and in the absence of additional abdominal symptoms, gastrointestinal transit (GIT) was performed, demonstrating intrapericardial herniation of the intestinal loops. The patient was discharged following abdominal surgical repair, with no further complications.
CONCLUSIONS: Intrapericardial diaphragmatic hernia is an infrequent instance within the diaphragmatic hernia group, with cardiac surgery being a rare potential iatrogenic factor. Clinical suspicion and imaging findings are key to perform early diagnosis and surgical treatment.
BACKGROUND: La hernia diafragmática intrapericárdica consiste en un prolapso de las estructuras abdominales dentro del pericardio, representando una de las entidades menos frecuentes dentro del conjunto de las hernias diafragmáticas.
METHODS: Se presenta el caso de un lactante de 4 meses intervenido de comunicación interventricular (CIV) que, en el postoperatorio, la auscultación cardiopulmonar detecta ruidos hidroaéreos y el control radiológico de tórax muestra un neumopericardio atípico. Ante los hallazgos clínico-radiológicos, y sin sintomatología abdominal sobreañadida objetivable, se solicita un tránsito gastrointestinal (TGI) donde se pone de manifiesto la herniación intrapericárdica de asas intestinales. Tras la intervención quirúrgica abdominal reparadora, el paciente fue dado de alta, sin otras complicaciones.
UNASSIGNED: Se realiza un breve recordatorio de las hernias diafragmáticas intrapericárdicas como una entidad de escasa incidencia dentro del conjunto de hernias diafragmáticas, siendo la cirugía cardiaca un posible factor iatrogénico poco frecuente. La sospecha clínica, junto con los hallazgos de imagen, son fundamentales para llegar a un diagnóstico y tratamiento quirúrgico precoz.

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.