Complex Presentation of Congenital Heart Block and Coexisting Congenital Heart Disease in a One-Year-Old Girl: A Case Report.

Congenital complete heart block (CCHB) is a rare and potentially life-threatening condition, often associated with maternal autoantibodies. We present the case of a one-year-old girl with recurrent respiratory symptoms, ultimately diagnosed with CCHB and congenital heart disease. She exhibited bradycardia and signs of congestive heart failure. A diagnostic workup revealed significant cardiac abnormalities, including dilated chambers, ventricular septal defect, and patent ductus arteriosus. Serological tests for maternal autoantibodies were negative. The child's parents opted for discharge without surgical intervention. This case underscores the importance of comprehensive evaluation and management strategies in patients with congenital heart block, particularly in resource-limited settings.

An Incidental Case of a Rare Ventricular Septal Defect (VSD): Does Infective Endocarditis Ger-Bode Well?

The Gerbode defect is a rare ventricular septal defect (VSD) between the left ventricle (LV) and right atrium (RA). We describe a challenging case of a rare acquired Gerbode defect from infective endocarditis. A 73-year-old male presented for left lower extremity edema and shortness of breath with exertion. He was discharged from the hospital one week prior after being diagnosed with right hip septic arthritis. A transthoracic echocardiogram (TTE) did not demonstrate an abscess or vegetation, but was significant for severely elevated tricuspid regurgitation velocity and pulmonary artery (PA) systolic pressure of 70 mm Hg without structural changes to the right ventricle or RA. A transesophageal echocardiogram (TEE) was performed due to these abnormal values and demonstrated a VSD between the LV and RA. This type of defect is known as a Gerbode defect, which is suggestive of an aortic root abscess. The patient ultimately was transferred to a tertiary care center, and the Gerbode defect with aortic root abscess was confirmed by direct visualization. This case reports a unique case of an acquired Gerbode defect secondary to infective endocarditis. Our patient's defect was noted to be above the tricuspid valve, which essentially confirmed the etiology as a VSD. Although the TEE did not demonstrate a clear aortic root abscess, direct visualization during the surgical intervention confirmed this suspicion. Prompt diagnosis of the Gerbode defect allowed the patient to receive urgent surgical intervention. Gerbode defects are rare but clinically important complications of infective endocarditis. This case highlights the importance of maintaining a high level of suspicion, especially if the values obtained during TTE do not fully explain a patient's clinical presentation. A high level of suspicion leading to a timely diagnosis of this condition is essential in preventing further valvular destruction and allowing prompt surgical intervention.

Simultaneous repair of diaphragmatic hernia and ventricular septal defect with postoperative complication in a Down syndrome child.

Morgagni hernia (MH) is a rare form of congenital diaphragmatic hernia, typically occurring predominantly on the right side and exhibiting a higher prevalence in females. Usually diagnosed incidentally, MH may coexist with congenital heart defects, chest wall abnormalities and certain genetic syndromes such as Down syndrome. A 4-year-old boy with Down syndrome underwent simultaneous repair of MH and closure of a ventricular septal defect (VSD). A vertical midline sternotomy was performed, and the VSD was repaired using the right atrium approach. Subsequently, MH repair was conducted. Three weeks after the surgery, this patient developed a complete heart block, which lead to the implantation of a VVI pacemaker.

Hemodynamics During Development and Postnatal Life.

A well-developed heart is essential for embryonic survival. There are constant interactions between cardiac tissue motion and blood flow, which determine the heart shape itself. Hemodynamic forces are a powerful stimulus for cardiac growth and differentiation. Therefore, it is particularly interesting to investigate how the blood flows through the heart and how hemodynamics is linked to a particular species and its development, including human. The appropriate patterns and magnitude of hemodynamic stresses are necessary for the proper formation of cardiac structures, and hemodynamic perturbations have been found to cause malformations via identifiable mechanobiological molecular pathways. There are significant differences in cardiac hemodynamics among vertebrate species, which go hand in hand with the presence of specific anatomical structures. However, strong similarities during development suggest a common pattern for cardiac hemodynamics in human adults. In the human fetal heart, hemodynamic abnormalities during gestation are known to progress to congenital heart malformations by birth. In this chapter, we discuss the current state of the knowledge of the prenatal cardiac hemodynamics, as discovered through small and large animal models, as well as from clinical investigations, with parallels gathered from the poikilotherm vertebrates that emulate some hemodynamically significant human congenital heart diseases.

Clinical Presentation and Therapy of Ventricular Septal Defect.

Ventricular septal defects (VSDs) occur in 1.5-3.5 of 1000 live births and constitutes 20 % of congenital cardiac defects. There is no gender predominance.

Ventricular Septal Defects: Molecular Pathways and Animal Models.

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.

Human Genetics of Ventricular Septal Defect.

Ventricular septal defects (VSDs) are recognized as one of the commonest congenital heart diseases (CHD), accounting for up to 40% of all cardiac malformations, and occur as isolated CHDs as well as together with other cardiac and extracardiac congenital malformations in individual patients and families. The genetic etiology of VSD is complex and extraordinarily heterogeneous. Chromosomal abnormalities such as aneuploidy and structural variations as well as rare point mutations in various genes have been reported to be associated with this cardiac defect. This includes both well-defined syndromes with known genetic cause (e.g., DiGeorge syndrome and Holt-Oram syndrome) and so far undefined syndromic forms characterized by unspecific symptoms. Mutations in genes encoding cardiac transcription factors (e.g., NKX2-5 and GATA4) and signaling molecules (e.g., CFC1) have been most frequently found in VSD cases. Moreover, new high-resolution methods such as comparative genomic hybridization enabled the discovery of a high number of different copy number variations, leading to gain or loss of chromosomal regions often containing multiple genes, in patients with VSD. In this chapter, we will describe the broad genetic heterogeneity observed in VSD patients considering recent advances in this field.

Human Genetics of Tricuspid Atresia and Univentricular Heart.

Tricuspid atresia (TA) is a rare congenital heart condition that presents with a complete absence of the right atrioventricular valve. Because of the rarity of familial and/or isolated cases of TA, little is known about the potential genetic abnormalities contributing to this condition. Potential responsible chromosomal abnormalities were identified in exploratory studies and include deletions in 22q11, 4q31, 8p23, and 3p as well as trisomies 13 and 18. In parallel, potential culprit genes include the ZFPM2, HEY2, NFATC1, NKX2-5, MYH6, and KLF13 genes. The aim of this chapter is to expose the genetic components that are potentially involved in the pathogenesis of TA in humans. The large variability in phenotypes and genotypes among cases of TA suggests a genetic network that involves many components yet to be unraveled.

Presentation of Complex Congenital Cardiac Anomalies in a Newborn Pediatric Patient: A Case Report.

Tricuspid atresia, a critical congenital heart defect (CHD), accounts for approximately 1% of all cases of CHDs. When tricuspid atresia is coupled with numerous other unexpected congenital cardiac anomalies, a patient's condition becomes more serious and more complex. We present a case that demonstrates the stepwise approach to the holistic treatment of congenital tricuspid atresia in the presence of normally related great vessels, a large ventricular septal defect (VSD), atrial septal defect (ASD), and trivial patent ductus arteriosus (PDA). While expanding upon the implementation of chest X-ray imaging, serial transthoracic echocardiogram (TTE) imaging, and the balloon atrial septostomy (BAS) procedure, we also provide insight into the multidisciplinary team-based approach utilized for this patient's case. This case illustrates a rare critical CHD coupled with other, more common congenital anomalies, and suggests that with multidisciplinary management and treatment, it is possible the mortality rates associated with this diagnosis could decline.

Ruptured Sinus of Valsalva Aneurysm: Three Case Reports and Literature Review.

Sinus of Valsalva aneurysm rupture (SOVAR) into the right cardiac chambers is an uncommon complication with unusual presentation, high morbidity and mortality, and unique hemodynamics as well as cardiac imaging findings. Here, we present three SOVAR cases (two with rupture into the right atrium and one with rupture into the right ventricle) that were initially confused for ventricular septal defects and describe their initial presentation, cardiac imaging studies, invasive hemodynamics, as well as treatment options. Some of the unique findings of SOVAR patients include an acute presentation, often with hemodynamic decompensation, the presence of a continuous murmur on examination, and also hemodynamics that include wide pulse pressure and right heart volume overload.

Atrial Septal Defect vs Ventricular Septal Defect: Getting the Right Mix in Transposition of the Great Arteries.

Transposition of the great arteries (TGA) is the second most common cyanotic congenital cardiac defect and affects around 4.7 in 10,000 live births. Patients present at birth with profound cyanosis due to inadequate oxygen delivery to the systemic circulation. Typical management after birth involves the administration of prostaglandins and oxygen while awaiting surgical repair. Balloon atrial septostomy may be performed depending on the adequacy of the interatrial communication. In this case report, we present a challenging case of TGA ventricular septal defect (VSD) and pulmonary stenosis (PS), demonstrating the importance of bedside clinical examination along with applying basic management principles. The patient underwent a right modified Blalock-Taussig-Thomas shunt (BTT) along with left pulmonary artery (LPA) reconstruction and main pulmonary artery band as an initial palliative procedure. The patient deteriorated post-operatively, with increasing desaturations and oxygen requirements. Though imaging suggested sufficient inter-circulatory mixing, the clinical picture of desaturation without respiratory distress and lack of response to oxygen and pulmonary vasodilatory therapy strongly suggested otherwise. The child therefore underwent a balloon atrial septostomy. Their clinical condition improved and they were discharged three days later. We describe this case's clinical course, medical and surgical management, and learning points.

Neural correlates of perceptual similarity masking in primate V1.

Visual detection is a fundamental natural task. Detection becomes more challenging as the similarity between the target and the background in which it is embedded increases, a phenomenon termed 'similarity masking'. To test the hypothesis that V1 contributes to similarity masking, we used voltage sensitive dye imaging (VSDI) to measure V1 population responses while macaque monkeys performed a detection task under varying levels of target-background similarity. Paradoxically, we find that during an initial transient phase, V1 responses to the target are enhanced, rather than suppressed, by target-background similarity. This effect reverses in the second phase of the response, so that in this phase V1 signals are positively correlated with the behavioral effect of similarity. Finally, we show that a simple model with delayed divisive normalization can qualitatively account for our findings. Overall, our results support the hypothesis that a nonlinear gain control mechanism in V1 contributes to perceptual similarity masking.

The paramount of three-dimensional echocardiography in percutaneous closure of large oval perimembranous ventricular septal defect: a case report.

Background: Ventricular septal defect (VSD) is the most common type of congenital heart abnormality with perimembranous VSD (pmVSD) accounting for ∼70% of all VSD. Nowadays, transcatheter closure is the first choice for suitable pmVSD. However, there was no report about closing the large oval-shaped VSD percutaneously. Case summary: A 34-year-old male with known VSD was referred for transcatheter closure after failed attempts in other hospital. Patient had been diagnosed with VSD at a young age, but he was lost to follow-up. He presented with shortness of breath due to heart failure and pulmonary hypertension. The initial measurement of the defect was 6-7 mm by transthoracic echocardiography (TTE), transoesophageal echocardiography (TEE), and LV angiography. However, re-measurement using TEE and 3D echocardiography revealed that the VSD is oval with diameters of 18 mm × 6 mm. Initially, device No. 12/14 was used, but it was dislodged on two attempts. The operator then decided to upsize the device size to No. 16/18 that was successful. The patient's condition was good, and 6 months follow-up after the procedure showed good outcomes without any residual defect or arrhythmia. Discussion: In this study, we would like to highlight the rarity of large oval pmVSD that almost failed to be closed with the conventional measurement with echocardiography and fluoroscopy. Transoesophageal echocardiography especially 3D can be the new modality of choice that might be superior to fluoroscopy to decide the right device size in some cases such as oval-shaped pmVSD.

A Comparative Study of Narrow/Ultra-Wideband Microwave Sensors for the Continuous Monitoring of Vital Signs and Lung Water Level.

This article presents an in-depth investigation of wearable microwave antenna sensors (MASs) used for vital sign detection (VSD) and lung water level (LWL) monitoring. The study looked at two different types of MASs, narrowband (NB) and ultra-wideband (UWB), to decide which one was better. Unlike recent wearable respiratory sensors, these antennas are simple in design, low-profile, and affordable. The narrowband sensor employs an offset-feed microstrip transmission line, which has a bandwidth of 240 MHz at -10 dB reflection coefficient for the textile substrate. The UWB microwave sensor uses a CPW-fed line to excite an unbalanced U-shaped radiator, offering an extended simulated operating bandwidth from 1.5 to 10 GHz with impedance matching ≤-10 dB. Both types of microwave sensors are designed on a flexible RO 3003 substrate and textile conductive fabric attached to a cotton substrate. The specific absorption rate (SAR) of the sensors is measured at different resonant frequencies on 1 g and 10 g of tissue, according to the IEEE C95.3 standard, and both sensors meet the standard limit of 1.6 W/kg and 2 W/kg, respectively. A simple peak-detection algorithm is used to demonstrate high accuracy in the detection of respiration, heartbeat, and lung water content. Based on the experimental results on a child and an adult volunteer, it can be concluded that UWB MASs offer superior performance when compared to NB sensors.

An unusual site of ablation for a ventricular tachycardia.

Scar-related ventricular tachycardia (VT) ablation involves localizing the critical isthmuses by overdrive pacing maneuvers and three-dimensional activation mapping. Implantable prosthetic devices have been known to complicate this by covering sites of potential isthmuses. We herein present a sentinel report of scar-VT ablation with a protected isthmus localized over an endothelialized post-myocardial infarction ventricular septal defect occluder device.

The Unrepaired Tetralogy of Fallot: A Tale of Delayed Presentation and Limited Access to Care.

Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease characterized by four distinct anatomical features. While surgical repair has significantly improved long-term outcomes, some individuals, particularly those from low socioeconomic backgrounds who lack access to medical care, may suffer from complications such as pulmonary hypertension (pHTN) and heart failure. We present a case report of a young female with unrepaired TOF who presented with acute-on-chronic hypoxic respiratory failure and heart failure, highlighting the complex nature and challenges associated with this condition.

Role of ECG in the Accidental Finding of an Atrioventricular Septal Defect in an Asymptomatic Patient Undergoing Cosmetic Surgery.

Electrocardiogram (ECG) is an important diagnostic tool in identifying congenital heart disease (CHD), as demonstrated by this case of a 48-year-old female who presented for a preoperative evaluation for cosmetic surgery. ECG showed a right bundle branch block (RBBB) and first-degree atrioventricular (AV) block, and further testing revealed a primum atrial septal defect (ASD) with mitral valve anterior leaflet cleft and a membranous ventricular septal defect (VSD). She underwent successful surgical repair and was discharged home without complications. This case highlights the importance of performing additional tests like echocardiography or other imaging modalities in cases of abnormal ECG findings to accurately diagnose the underlying heart condition and ensure proper treatment.

Investigating the efficacy of vacuum sealing drainage versus traditional negative pressure drainage in treating deep incision infections following posterior cervical internal fixation-a retrospective cohort study.

BACKGROUND: Assessing the efficacy and safety of Vacuum Sealing Drainage (VSD) in treating deep incision infections (DII) following posterior cervical internal fixation. METHODS: We retrospectively studied the clinical effects of VSD and Traditional Negative Pressure Drainage (TND) on 12 patients with deep incision infection after posterior cervical fixation surgery who were treated in our department from 2012 to 2020. A comparison of patient-related factors (age, gender, BMI, comorbidities, initial internal fixation surgery segment, preoperative laboratory inflammation indicators) and surgical-related factors (postoperative duration of fever, positive rate of drainage fluid bacterial culture, Visual Analogue Scale (VAS) score at 3 days after surgery, laboratory indicators at 3 days after surgery, debridement frequency and drainage time, hospital stay, internal fixation retention rate, and infection recurrence rate) between the VSD group and the TND group was conducted using independent sample t tests to draw experimental conclusions. RESULTS: This study included 12 patients, with six cases of VSD (5 males and 1 female) and six cases of TND (4 males and 2 females). The VSD group had significantly lower postoperative fever time (1.50 ± 0.46 days vs. 4.28 ± 0.97 days, P < 0.05), a higher positive rate of bacterial cultures in drainage fluid (5/6 vs. 2/6, P < 0.05), lower 3 day VAS scores (3.13 ± 0.83 vs. 3.44 ± 0.88, P < 0.05), lower 3 day CRP levels (66.89 ± 23.65 mg/L vs. 57.11 ± 18.18 mg/L, P < 0.05), a shorter total drainage time (14.50 ± 2.98 days vs. 22.56 ± 3.01 days, P < 0.05), and a higher total drainage flow rate (395.63 ± 60.97 ml vs. 155.56 ± 32.54 ml, P < 0.05) than the TND group (the total drainage volume throughout the entire treatment process). In addition, the frequency of debridement (2.67 ± 0.52 times vs. 3.17 ± 0.41 times, P < 0.05) and average hospital stay (23.13 ± 3.27 days vs. 34.33 ± 6.86 days, P < 0.05) were significantly lower in the VSD group, although both groups retained internal fixation. CONCLUSIONS: VSD is a secure and effective treatment for deep incision infections that results from cervical posterior internal fixation surgery.