Perimembranous Ventricular Septal Defect Research Articles

Ventricular septal defect characteristics influence presence and properties of septal anatomical isthmuses in patients with repaired tetralogy of Fallot

Abstract Background In repaired tetralogy of Fallot (rTOF), the septal anatomical isthmuses (AI), 3, between the ventricular septal defect (VSD) and pulmonary annulus, and 4, between the VSD and tricuspid annulus, are important ventricular tachycardia (VT) substrates when slow conducting. Patients with rTOF can have different VSD characteristics which may influence presence and properties of septal AIs. Methods This study included all consecutive patients with rTOF who underwent electroanatomical mapping (EAM) between 2005 and March 2023 for ventricular arrhythmias, risk stratification, or before pulmonary valve replacement and had a surgical report of initial repair available. Results In 130 of 154 (84%) patients a surgical report was available (35% female, median age at EAM 38 years [IQR 20-49]), with provided VSD details in 90 (69%). The majority of patients had an outlet perimembranous VSD (n=77, 86%), 7 (8%) a doubly-committed juxta-arterial (DC-JA) VSD, and 4 (4%) a muscular outlet VSD. All patients with a DC-JA VSD had a blocked/absent AI 3. Among patients with a DC-JA VSD with a muscular posteroinferior rim, 50% had AI 4, which was not observed in patients with a fibrous posteroinferior rim. In patients with a muscular VSD, AI 4 was present in 25%. In contrast, in patients with an outlet perimembranous VSD, an AI 4 or a blocked/absent AI 3 were rare findings (1% and 4%, respectively). Conclusions VSD characteristics influence presence and properties of septal AIs. In patients with DC-JA VSD septal VT substrates seem to be rare. Detailed surgical information is desirable for patient management.

Clinical Profile and Natural History of Children with Ventricular Septal Defects in Tripoli, Libya from 1996 through 2009

Introduction and Objectives: Ventricular septal defect (VSD) is the most common congenital heart diseases, it accounts 40%. This study carried out to describe the epidemiological characters and clinical presentation of patients with VSD in Western and Southern Libya, and to evaluate spontaneous versus surgical closure in different types.  Patients and Methods It is hospital case series study conducted by reviewing the medical records of 1092 patients followed at cardiology outpatient department (OPD) of Tripoli Children Hospital from 1996 through 2009, the data abstracted from medical records and filed in a worked sheet, Excel and SPSS software used to analyze the data.  Results We identified 1092 patients. The results revealed that 30.9% diagnosed during the first month, and 49.7% from1 to 12 months. From 1092 patients; 50.8% have peri- membranous VSD, 18.8% muscular, and 13% having an apical type. 97.2% of males have peri-membranous VSD where muscular and apical seen more in female, p value = 0.001. Cardiac murmur is the common presenting sign 61.3%, 9.3% have feature of Down syndrome. Positive Family history of CHD in 9.9% with significant relationship between family history and type of VSD (peri- membranous type), P value = 0.001. Small VSDs accounts 66.9%, large VSDs 16.1%, followed by medium size VSD 11.8% of cases. Patients with large VSDs more prone for hospital admission as 101 (57.4%) from 176 patients were admitted to hospital, followed by moderate size (45%), P value = 0.001. 70.3% of patients with large VSD need either medical or surgical treatment, P value = 0.001. Spontaneous closure seen in 17%, 0.6% died, while 41.9% still on follow up. Muscular VSD shows the highest tendency for spontaneous closer as 72% closed by the age of 2 years.  Conclusion We conclude that peri- membranous VSD is the most common type. Two thirds of patients had been diagnosed in the first year of life. Big defects and some of moderate VSDs need either medical or surgical treatment. Spontaneous closer is usually seen in the first 2 years of life.  Recommendations We highly recommend for further multi-center study all over the country to get reliable data on the prevalence of congenital heart diseases and the incidence of ventricular septal defect in our population.

A case report of Situs inversus totalis with Ventricular septal defect: Flipped Physiology

Dextrocardia with situs inversus (situs inversus totalis) is a rare congenital condition characterized by the abnormal positioning of the heart and other organs in the thoracic and abdominal cavities. When combined with a ventricular septal defect (VSD), it presents unique challenges in diagnosis and management. Here, we present a case report of a patient with dextrocardia, situs inversus, and a peri-membranous VSD, highlighting the diagnostic process and discussing available treatment options.

Characteristics of the Device and Conduction Disturbances in Transcatheter Device Closure of Perimembranous Ventricular Septal Defects.

Characteristics of the Device and Conduction Disturbances in Transcatheter Device Closure of Perimembranous Ventricular Septal Defects.

Transcatheter Closure of Perimembranous Ventricular Septal Defect Using KONAR-MF™: A Multicenter Experience.

Transcatheter closure of perimembranous ventricular septal defect (PmVSD) is an established procedure. However, the occurrence of complete heart block limits its scope. The newer KONAR-MF™ occluder has specific design characteristics that may improve the safety of PmVSD closure. The objective of the studywas to describe the efficacy and mid-term follow-up of transcatheter closure of PmVSD using KONAR-MF™. The study was conducted prospectively in 3 Indian centers (January 2018-December 2022). PmVSD closure was done by both antegrade and retrograde methods, and patients were followed up at 1, 3, 6, 12months, and annually after that. 121 out of 123 patients were included with the following characteristics: median age 4.4 (0.18-40) years; weight 15 (2.1-88) kg; mean Qp/Qs ratio 1.87 ± 0.52 and pulmonary artery mean pressure: 22 ± 6.9mmHg. The procedure was successful in all but 3; the device was removed due to significant residual shunt (n = 2) and new development of aortic regurgitation (AR) (≥ mild) in 1. The median defect size was 5.2 (2.5-12) mm. Device sizes from 6/4 to 14/12 were deployed (median fluoroscopy time 13.3min; range 3.6-47.8). Shunt occlusion rates were 90%-Immediate, 95%-pre-discharge, and 97%-1month, with no instances of complete heart block after the procedure and during follow-up. Six had new onset AR (mild: 2, trivial 4), and one had increased tricuspid regurgitation. All patients were well during follow-up (median: 20months; range: 6-46). The new KONAR-MF™ occluder appears to be a promising and safe alternative for the closure of the PmVSD; further long-term follow is merited.

Mid-term Results of Double Chamber Right Ventricle in Association with Genetic Syndromes

Double chamber right ventricle (DCRV) is a congenital disease in which a hypertrophied muscle band divides the right ventricle chamber into two. The early-mid-term follow-up of 6 patients with DCRV and distant genetic syndromes is reported in this paper. A retrospective analysis was performed of 6 DRCV patients with a mean age of 3.9±1.4 years. Concomitant cardiac anomalies were perimembranous ventricular septal defect (n=3), atrial septal defect (n=1), mild aortic regurgitation (n=1), discrete subaortic membrane (n=1). Associated genetic syndromes were Costello (n=1), Seckel (n=1) and Down syndromes (n=4). The mean follow-up period was 4.86±4.6 years. Mean systolic pressure gradient in the right ventricle in the postoperative was 18.5±11.5 mmHg. No mortality occurred and there was no requirement for reintervention. To the best of our knowledge, this is the first report in literature of concomitant DCRV with Costello and Seckel syndromes.

Streptococcus sinensis Causing Tricuspid Endocarditis in a Child: An Unusual Pathogen at an Unusual Site: A Case Report with Review of Literature

Background: Infective endocarditis (IE) involving tricuspid valve is rarely reported in children. Common etiological organisms of IE include Staphylococci, viridans group Streptococci, and Enterococci. Other less frequent organisms include Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, and Kingella (HACEK) group, and Candida, accounting for 1% of cases. We describe the rare case scenario of tricuspid valve endocarditis caused by an unusual pathogen. Clinical Description: An 8-year-old girl presented with complaints of cough, shortness of breath, and intermittent fever for the past 2 months. Since 10 days before presentation, the fever had become high grade, continuous, and associated with chills and rigors. As per parents, the child had incidentally been detected with some congenital heart disease (CHD) at 3 months of age, documents being unavailable, child remaining asymptomatic till this presentation, without any medications. On examination, the child was malnourished with pallor, Grade 3 clubbing, tachypnea, and tachycardia. Auscultation revealed bilateral basal crepitations and a pansystolic murmur of Grade 4 in tricuspid area. Management and Outcome: Along with routine investigations, a two-dimensional echocardiography was done which revealed perimembranous ventricular septal defect with left-to-right shunt, tricuspid valve vegetations with anterior tricuspid leaflet prolapse with ruptured chordea, severe tricuspid regurgitation, and thickened aortic valve with good biventricular function. Blood culture showed growth of Streptococcus sinensis. The child was treated with oxygen, diuretics, and intravenous ciprofloxacin and gentamicin according to antibiotic sensitivity pattern. The child became hemodynamically stable and symptomatically improved after 3 weeks of treatment. Conclusion: This case creates awareness regarding an usual pathogen – S. sinensis as an etiological agent of IE, involving an unusual site – the tricuspid valve, in a child with CHD.

Retracted: Study on Inflammatory Factors in Aneurysmal Perimembranous Ventricular Septal Defect in Congenital Heart Disease.

[This retracts the article DOI: 10.1155/2022/8282624.].

Predictive Scoring System for Spontaneous Closure of Infant Ventricular Septal Defect: The P-VSD Score.

Ventricular septal defect (VSD) is a common congenital heart disease. However, consensus on the utility of echocardiography in predicting spontaneous closure (SC) of VSD remains lacking. This study aimed to identify and validate significant predictors of SC through a predictive scoring system. This retrospective study included medical records of 712 echocardiography instances performed on 304 patients diagnosed with VSD from 2016 to 2020 in their first year of life. A novel scoring system for predicting the SC of VSD was developed and validated using another dataset from different hospitals. Of the 304 patients, 215 (70.7%) had perimembranous (PM) VSDs and 89 had muscular (29.3%) VSDs. The median follow-up periods were 36.2 (interquartile range [IQR], 13-59) months and 13.7 9 (IQR, 5-37.4) days for PM and muscular VSDs, respectively. The overall SC rate during follow-up was 29.3%. Pulmonary hypertension (HTN), concomitant left ventricle (LV)-right atrium (RA) shunt, VSD size to aortic valve (AV) annulus size ratio, and left ventricular end-diastolic dimension (LVEDD) z-score were significant risk factors affecting SC of VSD. The "P-VSD" score, a new scoring system, demonstrated an area under the curve for predictability of 0.769. Pulmonary HTN, concomitant LV-RA shunt, LVEDD z-score, and VSD size-to-AV annulus size ratio at diagnosis were significantly associated with non-SC VSD after infancy. The P-VSD score can predict the SC of VSD in clinical settings and simplify the identification and appropriate management of high-risk patients.

Ventricular Septal Defect And Maternal History Of Epilepsy: A Case Report

Pregnancy with epilepsy can cause major risks for both the mother and the offspring. It is known that the use of antiepileptic drugs (AEDs) during pregnancy can increase the risk of cardiac malformations. This journal reports a 19-day-old male infant who came with complaints of shortness of breath and a blue lip 2 days before admission. The mother had a history of epilepsy and had regularly taken epilepsy medication since before pregnancy. Physical examination revealed cyanotic lips, chest wall retraction, coarse crackles, and murmurs. The patient's symptoms and signs were associated with congenital heart disease, so he was further observed in the NICU. After bedside echocardiography, the results showed a patent foramen ovale and perimembranous ventricular septal defect. The teratogenic effect of AED is thought to be one of the major causative factors of congenital heart disease. A ventricular septal defect is the most common cardiac malformation found in population-based studies. The long-term effects of AED exposure during pregnancy require further investigation, both in terms of the type of AED (old or new) and the duration of AED consumption. It is important to educate mothers-to-be about this, especially to avoid the risk of congenital heart disease in their offspring. Keywords: epilepsy, ventricular septal defect, cardiac malformation.

Risk Factors of Long-Term Sequelae After Transcatheter Closure of Perimembranous Ventricular Septal Defect in Young Children.

Complications arising from transcatheter closure of perimembranous ventricular septal defects (pmVSD) in children, such as residual shunts and aortic regurgitation (AR), have been observed. However, the associated risk factors remain unclear. This study identified risk factors linked with residual shunts and AR following transcatheter closure of pmVSD in children aged 2-12 years.Methods and Results: The medical records of 63 children with pmVSD and a pulmonary-to-systemic blood flow ratio <2.0 who underwent transcatheter closure between 2011 and 2018 were analyzed with a minimum 3-year follow-up. The success rate of transcatheter closure was 98.4%, with no emergency surgery, permanent high-degree atrioventricular block, or mortality. Defects ≥4.5 mm had significantly higher odds of persistent residual shunt (odds ratio [OR] 6.85; P=0.03). The use of an oversize device (≥1.5 mm) showed a trend towards reducing residual shunts (OR 0.23; P=0.06). Age <4 years (OR 27.38; 95% confidence interval [CI] 2.33-321.68) and perimembranous outlet-type VSD (OR 11.94, 95% CI 1.10-129.81) were independent risk factors for AR progression after closure. Careful attention is crucial for pmVSDs ≥4.5 mm to prevent persistent residual shunts in transcatheter closure. Assessing AR risk, particularly in children aged <4 years, is essential while considering the benefits of pmVSD closure.

Procedural Efficiencies and Clinical Outcomes for Transcatheter Device Closure of Perimembranous Ventricular Septal Defects With Different Waist-Length Occluders.

Potential differences in complications and/or long-term outcomes of perimembranous ventricular septal defect (pmVSD) closures with 3-mm waist vs. 4-mm waist double-disk symmetrical occluders are not known.Methods and Results: A total of 395 consecutive pediatric patients with pmVSD recruited between January 2017 and March 2021 underwent successful transcatheter closure using symmetrical pmVSD devices. The final analysis involved 208×3-mm and 172×4-mm cases. The median follow-up was 42 months (range: 12-62 months). A total of 175 post-procedure adverse events (AEs) were observed. Most of these AEs were temporary, and there were only 8 major AEs. Compared to the 3-mm waist group, the incidence of residual shunts was significantly higher in the 4-mm waist group (13.4% vs. 6.7%; P=0.030), whereas other AEs showed similar incidences between the 2 groups. Multivariate Cox regression analysis revealed that larger defect, higher ratio between device size and body surface area, and longer procedure time can cause an increased likelihood of AEs, and smaller defect or left disk placement within aneurysmal tissue may reduce it. Transcatheter closure of pmVSD using a symmetrical double-disk occluder is safe and effective. Compared with a 3-mm waist symmetrical occluder, transcatheter closure with a 4-mm waist symmetrical occluder correlated with higher incidences of residual shunts.

Severe Pulmonic Valve Endocarditis with Septic Pulmonary Embolism in a Teenager: Case Report

A 14-year-old boy with an uneventful past history suffered from one week of high-grade fever and progressive dyspnea. The patient developed necrotizing pneumonia with massive loculated pleural effusion and septic shock. Echocardiogram revealed a small perimembranous VSD with multiple vegetations at pulmonic valve and both pulmonary arteries. Intravenous antibiotic therapy, mechanical ventilatory support, chest tube placement, and intrapleural fibrinolytic therapy were performed. He was clinically improved after eight weeks of antibiotic therapy. Keywords: Septic pulmonary embolism; Pulmonic valve endocarditis; Infective endocarditis

Right Ventricular Outflow Tract Obstruction by an Aneurysm of the Ventricular Membranous Septum: A Systematic Review of Case Reports.

We performed a literature search to identify the details of patients with right ventricular outflow tract obstruction caused by an aneurysm of the ventricular membranous septum in a perimembranous ventricular septal defect. Thirty-one cases with a median age of 29 years (range, 1-69 years) were studied. A right ventricle-pulmonary artery systolic pressure gradient ranged from 35 to 107 mm Hg (mean 69 mm Hg). An interventricular shunt was absent in eight patients: two children and six adults. It is necessary to monitor the size of an aneurysm of the ventricular membranous septum, whether or not an interventricular shunt is present.

Early Clinical Outcomes in Infants with Prenatally Diagnosed Perimembranous and Muscular Ventricular Septal Defects (VSDs).

Isolated ventricular septal defect (VSD) is often associated with good clinical outcomes. However, infants prenatally diagnosed with VSD are often recommended for delivery at tertiary care centers. The aim of our study was to determine the odds of neonatal intensive care unit (NICU) admission in infants with persistent isolated VSD and complicated VSD, where an infant is affected by VSD and other genetic/structural abnormalities. We performed a retrospective cohort study, with data collected from a single academic institution from June 2018 to March 2023. Individuals with prenatally diagnosed VSD, in the absence of any other heart defects, were included in this study. The primary outcome was admission to the NICU. Multivariable logistic regression was used to assess associations. The association between persistence of VSD and NICU admission was adjusted for maternal age, fetal genetic abnormalities, fetal extracardiac abnormalities, and gestational age at the time of delivery. The association between complicated VSD and NICU admission was adjusted for maternal age and gestational age of the infant at the time of delivery. The odds of NICU admission were similar in infants with persistent isolated VSD and VSD that closed in utero (adjusted OR 1.31, 95% CI 0.30-5.61). However, infants with complicated VSD were at increased risk of NICU admission (adjusted OR 15.52, 95% CI 2.90-82.92). The risk of NICU admission was only increased in infants whose VSD was complicated by another genetic/major structural abnormalities. Therefore, women whose infants are prenatally diagnosed with VSD alone may not require delivery at tertiary care centers.

The detailed profile of congenital heart diseases in 254 children with Down syndrome in Saudi Arabia

BackgroundDown syndrome is the most common chromosomal abnormality in humans. It is associated with several congenital anomalies, including a spectrum of congenital heart diseases. Understanding the true prevalence and distribution of congenital heart diseases is essential for health resource planning, outcomes, and family counseling.This study aimed to assess the prevalence and distribution of congenital heart disease in children with Down syndrome. It is a retrospective cohort review that included all patients treated at King Abdulaziz University Hospital. Frequencies were estimated using the SPSS software and comparisons were made using Student’s t test.ResultsThe ages of the 254 subjects ranged from less than 1 year to 53 years. Of these, 44.5% were female and 40.6% were Saudi nationals. Congenital heart disease was present in 66.5% with a significant difference between Saudi nationals 44.6%) and non-Saudi nationals 71.5% (P = 0.01). The atrioventricular septal defect was the most common pathology, representing 33.1% of all congenital heart diseases followed by perimembranous ventricular septal defect 18.9%, and right ventricular pathology 10.2%.ConclusionThe prevalence of congenital heart diseases in Saudi children with Down Syndrome is similar to that reported worldwide. Septal defects and right-sided pathologies are the dominant forms of congenital heart diseases, with atrioventricular septal defect and perimembranous ventricular septal defect representing the most common pathologies.

Word of caution: silent late device embolisation after perimembranous ventricular septal defect closure in a 6-Kg infant.

We report on a 6-month-old infant (6 Kg/ 64 cm) with perimembranous ventricular septal defect (absent sub-aortic rim, 10 mm left ventricular entry, and 4 and 6 mm right ventricular exists) and successful retrograde closure using an 8x6 mm KONAR-MF™ VSD occluder (Lifetech, China). Immediate and 48 hours post-procedure ultrasounds showed an accurately positioned device and two jets of mild-to-moderate residual shunts. At the 2-week follow-up, the device did not change position and the shunt was stable and intra-prosthetic. The scheduled 3-month follow-up was skipped for familial reasons. The patient came back without alarming symptoms for the regular 6-month follow-up, and the device was found embolised to the left pulmonary artery. The device was retrieved surgically, and the defect was patch-closed with excellent outcomes. There was a pseudoaneurysm involving the tricuspid valve chordae and the device was endothelialized partially on one edge suggesting that embolization occurred somewhere between 3 months and 6 months post-operative. Defects with compromised anatomies should be closed surgically to avoid suboptimal results, especially in small infants.

Echocardiographic Findings of Children with Down Syndrome

Objective: Down syndrome is the most common well-known chromosomal disorder. The incidence of congenital heart disease (CHD) in these patients is high and it is the most important cause of mortality. In this study, we reviewed the comorbid CHDs and their characteristics in patients with the diagnosis of Down syndrome. We compared our results with the results of studies conducted in different regions of our country in different years. Materials and Methods: Between January 2020 and December 2022, data from a total of 385 patients (175 females, 210 males) with Down syndrome were evaluated. The age of the mothers at the time of delivery and the availability of prenatal diagnosis information in accessible patients were determined. Results: Of 385 patients with Down syndrome, 38.2% (n=147) had CHD. Among patients with CHD, 57 patients had atrioventricular septal defect (AVSD) (38.7%), 35 patients had perimembranous ventricular septal defect (23.8%), 24 patients had secundum atrial septal defect (16.3%), 8 patients had patent ductus arteriosus (5.4%), and 10 patients without AVSD had CHD with multiple left-to-right shunts (6.8%). The most common cyanotic CHD was tetralogy of Fallot (n=7, 4.7%). Complete AVSD was present in 63.1% of AVSD patients. CHD was present in 36.2% of boys and 40.6% of girls. Among all female patients, 17.1% had AVSD, while this rate was 12.8% among all male patients (p&lt;0.05). The mean maternal age at delivery was 32.59±6.9 (17-48) years. It was found that children with Down syndrome whose mothers had a maternal age of 33.5 years and above had a lower rate of CHD. Of the 170 mothers for whom prenatal diagnosis information was available, 58.2% (n=99) had a prenatal diagnosis. Conclusion: Prenatal diagnosis opportunities have increased and become widespread over the years. However, no significant change was observed in the rates of CHD, which is most frequently responsible for mortality in children with Down syndrome. It is appropriate for every expectant mother with a prenatal diagnosis to be referred for fetal echocardiography or postnatal transthoracic echocardiography.

Congenital continuous retrograde basilar flow suggests type B interrupted aortic arch in a neonate: A case report

Reversed flow in the basilar artery can be acquired or congenital. Acquired reversed flow in the basilar artery can result from acute thrombosis of the basilar artery or retrograde vertebral artery flow. Congenital continuous retrograde basilar artery flow has not been described. We report a 2-day-old male presenting with hypocalcemic seizures which led us to obtain a Duplex echoencephalogram. An echocardiogram was subsequently ordered. In the coronal plane through the anterior fontanelle, retrograde flow was seen in the basilar artery and the right vertebral artery. In the axial plane through the temporal window, the flow was anteroposterior in both posterior communicating arteries. In the posterior cerebral arteries, the flow was retrograde in the P1 segment and anterograde in the P2 and P3 segments. An interrupted aortic arch was suspected. The echocardiogram showed a large perimembranous ventricular septal defect with bidirectional shunting, a hypoplastic and bicuspid aortic valve, an aortic arch interrupted between the left common carotid artery and the left subclavian artery (type B interrupted aortic arch), and a 5 mm patent ductus arteriosus with predominant right to left flow. Because of the patency of the large patent ductus arteriosus, our patient showed no sign of posterior circulation insufficiency. Prostaglandin E1 therapy was initiated immediately. Diagnosis of DiGeorge syndrome was proven. The infant underwent interrupted aortic arch repair and anterograde flow was established in the basilar artery. We conclude that congenital asymptomatic continuous retrograde flow in the basilar artery and left vertebral artery is a medical emergency as it implies the presence of type B interrupted aortic arch with large patent ductus arteriosus in a neonate.

Mechanical support for bridge to transplant in an infant with post-cardiotomy end-stage heart failure and complete heart block: report of a case.

The patient was diagnosed with perimembranous ventricular septal defect (VSD). She underwent VSD closure and muscle bundle resection across right ventricular outflow tract at the age of 3 months. Since then, she had suffered from severe heart failure and complete heart block. Permanent pacemaker generator was implanted in the left hypochondrium. She was depended on continuous catecholamine administration, so transferred to our hospital for further management. On arrival, her body weight was 5686g (- 2.7 SD). She underwent Excor pediatric left ventricular assist device implantation at the age of 9 months. Because the position of the left ventricular assist device cannula interfered with the pacemaker, herein, the pacemaker pocket was newly created in the left thoracic cavity. An 1mm in thickness of expanded polytetrafluoroethylene sheet was trimmed and sutured under the anterolateral wall of left thoracic cavity as a pacemaker pocket. Bipolar ventricular lead was sutured on left ventricular apex and basal wall to face each other, mimicking cardiac regeneration therapy. Even though she unfortunately required right diaphragmatic plication for iatrogenic phrenic nerve palsy, her respiratory function was well maintained; therefore, secondary right heart failure was not observed. Her cardiopulmonary function was quite stable until post-operative day 275 when the patient was transferred to another hospital for heart transplantation.