Large Perimembranous Ventricular Septal Defect Research Articles

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.

Transcatheter closure of large perimembranous ventricular septal defects with inlet to outlet extension with the Amplatzer Vascular Plug-II.

Transcatheter closure of perimembranous ventricular septal defect is an alternative to surgery in selected small to medium defects with a reasonable distance between the defect and the aortic or tricuspid valves. Surgical closure is generally preferred for large defects with an inlet to outlet extension, aortic malalignment, or deficient aortic rim. We report two successful cases of percutaneous closure of large perimembranous ventricular septal defects with inlet to outlet extension using an Amplatzer Vascular Plug-II via a retrograde approach. Transcatheter closure of large perimembranous ventricular septal defect with inlet to outlet extension is feasible and facilitated by the characteristics of the Amplatzer Vascular Plug-II device and the specific implantation technique.

Case report of a Gore-Tex TCPC conduit dissection causing severe stenosis

BackgroundThe patient is a 15-year-old male with situs inversus, dextrocardia, bilateral superior caval veins, atrioventricular discordance with a single outlet, large perimembranous ventricular septal defect, aortic override, pulmonary atresia, and right aortic arch. The complex anatomy with a Ventricular Septal Defect (VSD) distant from the aorta (unsuitable for baffling to the aorta) meant he was unsuitable for biventricular repair and proceeded down a univentricular palliation pathway.Case summaryPost-total cavopulmonary connection his clinical course was uneventful until the age of 5 when he developed fatigability with desaturation. An accessory hepatic vein was surgically banded with improved saturations and exercise tolerance. At the age of 15, cardiovascular magnetic resonance (CMR) was performed to investigate borderline saturations and as workup for transition to adult services. Cardiovascular magnetic resonance and cardiac computed tomography (CT) imaging demonstrated an eccentric thrombus causing stenosis of the extracardiac conduit and a thrombus outside of the lumen contained by the thin outer membrane of the Gore-Tex conduit. Collateralization suggested this was longstanding. Cardiac catheterization demonstrated a 4 mm × 6 mm stenosis at the junction of the conduit with the pulmonary arteries. The region was successfully balloon dilated and stented with a 34 mm-long Cheatham Platinum stent, with no complications.DiscussionTo date, this is the first documented case of a dissecting thrombus of a Gore-Tex graft in the literature. This case emphasizes the need for anticoagulation and serial cross-sectional imaging (CT or CMR) in Fontan patients with prosthetic grafts throughout a patients’ lifetime.

Prenatal diagnosis of isolated perimembranous ventricular septal defects undergoing primary surgical repair in infancy

Objective We retrospectively analyzed our center’s experience with the prenatal diagnosis of isolated perimembranous ventricular septal defects that underwent primary surgical repair in infancy. Methods We identified patients born in Southern Nevada, between October 2012 and October 2020, with prenatal care that underwent surgical closure of an isolated large perimembranous ventricular septal defect between 1 and 12 months of age. The description at surgery defined ventricular septal defect morphology. We included only those with situs solitus, levocardia without dextroposition, and without any other cardiovascular abnormality. We analyzed prenatal detection rates for each of the eight years. Results We identified 81 patients. Of the 81, 35 (43%) had trisomy 21. We identified no other aneuploidies in those that underwent surgical repair; however, 1 had a 15q13.3 deletion syndrome, and 1 had a 22 q11.2 deletion syndrome. Of the 81, 27 (33%) overall were prenatally diagnosed. Increasing prenatal detection rates strongly correlated with time (R = 0.92, p = .002). Conclusions Trisomy 21 is common in isolated perimembranous ventricular septal defects undergoing primary repair in infancy. Further, prenatal detection rates significantly improved over time, up to 65% detection in the current years.

Left Ventricular Function Assessment of Patients with Postoperative Ventricular Septal Defect Closure by Longitudinal Strain on Two-Dimensional Echocardiography 1 Year after Surgery

Background: Patients with large perimembranous ventricular septal defects (pm-VSDs) undergo surgical repair with prosthetic material during infancy. However, the effect of the presence of akinetic patch in the ventricular septum on the left ventricular (LV) mechanical synchrony and ventricular function has not been studied. In our study, we assessed LV function in postoperative pm-VSD closure patients on two-dimensional (2D) echocardiography (echo) after 1 year of surgery using biplane Simspon's method, fractional shortening (FS), and global longitudinal strain (GLS) to assess the regional strain pattern. Methods: This was a a single-institution observational study. Patients following up 1 year after surgical ventricular septal defect closure at our institute were enrolled. On follow-up, detailed 2D echo was done with special emphasis on left atrial size, LV size (by M-mode), and presence of any residual shunt. LV function was assessed using biplane Simpson's method and longitudinal strain on 2D echo. GLS was measured in three standard apical views. Results: Of the 121 patients enrolled, 101 patients fulfilling the inclusion and exclusion criteria were analyzed. Of these 101 patients, 75.2% were males. The median age at follow-up after surgery was 3.67 ± 2.7 years. About 92.1% of patients did not have residual VSD lesion. No patient had pulmonary arterial hypertension. All except one patient had normal ventricular function. Patients with ventricular dysfunction had LV ejection fraction (LVEF) of 47%, GLS –17.4%, and FS −20.5%. The mean values of FS, LVEF, and GLS were 32.0 ± 5.28%, 62.2% ± 4.2%, and −26.53 ± 2.93%, respectively, in pm-VSD closure patients. Regional strain pattern analysis showed significantly lower strain values in basal anteroseptal segment with a mean of −21.4 ± 2.7%. FS and GLS were noted to have significant correlation with each other (P = 0.01) and LVEF by biplane Simpson's method and GLS had significant correlation with each other (P = 0.0001). Conclusions: GLS has strong correlation with LVEF by Simson's method and FS. Basal anteroseptal segment in pm-VSD patients had significantly lower strain values compared to other segments. There is no significant correlation between age at surgery and GLS in pm-VSD closure patients who were operated within 1 year of age. The type of VSD closure does not have significant influence on GLS.

Percutaneous closure of moderate to large perimembranous ventricular septal defect in small children using left ventricular mid-cavity approach

Aim & objectiveTo report mid-term follow-up result of transcatheter closure of perimembranous Ventricular septal defect (VSD) in children weighing less than 10 kg using Amplatzer Duct Occlude-I (ADO-I) by left ventricular (LV) mid-cavity approach. Material & methodThis is retrospective review of 35 children weighing less than 10 kg with moderate to large perimembranous VSD who were selected for transcatheter closure of VSD using ADO-I in between October 2016 to September 2018. Mean age was 2.08 ± 0.67 years (mean ± SD) and mean weight was 7.2 ± 1.2 kg (mean ± SD). Procedure was done by crossing the VSD from right ventricular side instead of using the standard approach by forming arterio-venous loop. Average fluoroscopic time was 9.2 ± 2.9 min (mean ± SD) and mean procedure time was 34.1 ± 13.1 min (mean ± SD). Mean follow-up period was 8.7 months (3–12 months) ResultDevice closure was successfully done in 32 patients. There was device induced aortic regurgitation (AR) in one case who was sent for surgery. One child was found to have complete heart block on first post-procedure day requiring permanent pace-maker implantation. During follow up none of them had any residual VSD, rhythm disturbance, AR and left or right ventricular outflow obstruction. ConclusionDevice closure can be successfully done in moderate to large perimembranous VSD using left ventricular mid cavity approach in small children. LV mid-cavity approach has advantages in terms of lesser hemodynamic instability, lesser fluoroscopy and lesser chance of device induced AR than standard technique particularly in smaller children.

An unusual cause of changing QRS morphology

A 7-day old term neonate with D-malposition of great arteries, large perimembranous ventricular septal defect (almost single ventricle), without pulmonary stenosis was admitted with cyanosis and congestive heart failure. ECG revealed supraventricular tachycardia with alternating QRS axis with every beat. We discuss our approach to this scenario, which leads us to a rather rare cause of changing QRS morphology.

Tetralogy of Fallot with absent pulmonary valve: main differences with classic Fallot are crucial for an accurate prenatal diagnosis and counselling

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Classic features are a large perimembranous ventricular septal defect; aorta overriding the interventricular septum; right ventricular hypertrophy and...

P270 Complex cyanotic heart disease in adult patient with down syndrome accompanied by mild pulmonary hypertension

Abstract Funding Acknowledgements No funding Introduction Patients with Down syndrome and complex unrepaired cyanotic heart disease have impaired life expectancy. The clinical status of each patient depends both on the primary lesion type as well as the cummulative haemodynamic burden in the cardiovascular system. Assessment of an adult patient with complex congenital heart disease that has not been surgically repaired presents several challenges since initial haemodynamic parameters and compensatory mechanisms have to be taken into account. Case presentation A 43 year old male patient with known history of Down syndrome and no regular follow up presented to the Outpatient Department of our hospital to undergo pre-operative control for minor soft tissue surgery. The patient exhibited typical morphological features of Down syndrome as well as central cyanosis and digital clubbing. On auscultation a holosystolic type murmur 3/6 on the 3rd left parasternal border was audible. His blood gases revealed hypoxia and mild metabolic acidosis. Electrocardiogram displayed right axis deviation and signs of right ventricular hypertrophy. Transthoracic echocardiography demonstrated a large perimembranous ventricular septal defect (VSD) with normal anatomy and function of the aortic valve and no overiding aorta. Of note was excessive infundibular subpulmonary stenosis which resulted in a functionally double chambered right ventricle. Left ventricle was of normal diamater with good overall systolic function while the right ventricle was non-dilated with significant hypertrophy. Intracavitary gradient was estimated at 60 mmHg. Continuous Doppler showed mild mitral regurgitaion and mild tricuspid regurgitaion with estimated systolic pulmonary pressure of 40 mmHg. Bidirectional flow with low velocities at the level of the defect was recorded. Inferior vena cava was mildly dilated with normal respiratory variation. Discussion/ Conclusion: The natural history of large VSDs results in progressive dilatation of the right ventricle, the left ventricle and the left atrium with gradually increasing pylmonary vascular resistance and development of pulmonary hypertension, followed by right ventricular hypertrophy, flow reversal and Eisenmenger syndrome. However when RVOT obstruction at any level coexists, significant right ventricular hypertrophy develops and the incresed right ventricular systolic pressures acts protectively by minimizing the shunt at the VSD level. Thus a restrictive VSD due to a functionally double chambered right ventricle created a very fragile balance; a cyanotic heart disease with mild bidirectional flow depending on preload and afterload conditions that survived to adulthood. Abstract P270 Figure. Complex unrepaired CHD

Ventricular Septal Defect and Bicuspidity of Both Semilunar Valves with Obstruction Creating Complex Hemodynamics

Aortic and pulmonary valves share a common developmental origin from the embryonic arterial trunk, so there are chances of involvement of both semilunar valves in the process of congenital malformation involving one valve. However, in reported literature, this is not a common association. Here, we report an infant diagnosed to have bicuspid morphology of both semilunar valves with a large perimembranous ventricular septal defect.

Transcatheter Closure of Moderate to Large Perimembranous Ventricular Septal Defects in Children Weighing 10 kilograms or less.

Transcatheter device closure of ventricular septal defect (VSD) is an alternative to conventional surgical closure. Device closure of moderate to large perimembranous VSD (pmVSD) is considered technically challenging in smaller children weighing ≤10 kg. Very few studies are published on the outcomes of the same. Descriptive single-center retrospective study. Data of 49 children ≤10 kg with moderate to large pmVSDs taken up for transcatheter device closure in our institute were analyzed and their follow-up details were reviewed. Of the 87 patients referred for VSD closure, 49 patients qualified for the inclusion criteria. Median age was 18 months (interquartile range: 13-22). Successful device deployment was achieved in 42 (85.7%) patients. Mean VSD size by transthoracic echocardiography was 5.98 mm (range: 4-12 mm). Mean waist size of the device used was 8.26 mm (range: 4-14 mm). There was one device embolization, requiring catheter-directed retrieval from the left ventricle and subsequent surgical referral for VSD closure. Minor complications such as device-related persistent new aortic regurgitation was noted in one patient and mild tricuspid regurgitation and transient heart block occurred in two patients each. There was no mortality or complete heart block requiring permanent pacemaker implantation immediately or during midterm follow-up (mean follow-up: 20 months; range: 6-72.5 months). Device closure of moderate to large pmVSDs in children weighing ≤10 kg is feasible and safe with a success rate of 85.7%. Careful selection of patients and avoidance of oversizing the defect makes the immediate and midterm results acceptable.

Symptomatic improvement using everolimus in infants with cardiac rhabdomyoma.

Background:Cardiac rhabdomyoma (CR) often shows spontaneous regression and needs close follow-up. These tumors may be associated with tuberous sclerosis complex (TSC), caused by the disinhibition of m-TOR protein.Objective:The aim of the study is to observe the efficacy of everolimus in infants with significant CR.Materials and Methods:This is a single-center prospective observational study including infants with significant CR causing either clinical symptoms or obstruction to the blood flow. Everolimus was administered at a dose of 4.5 mg/M2/wk till the symptomatic improvement. Liver and renal function tests were monitored during treatment.Results:There were six cases of suspected CR included in the study. Median age and weight of patients were 5 days (range: 1–90 days) and 3.2 kg (range: 2.2–4.5 kg), respectively. One patient was excluded after surgical excision biopsy during concomitant closure of associated large perimembranous ventricular septal defect confirmed it as a fibroma. The remaining all five cases showed regression of tumor during mean follow-up of 6.1 ± 5.1 months. One child developed varicella infection necessitating temporary discontinuation of medicine during follow-up. One case had sudden death at 4 months of age.Conclusion:Everolimus appears to be useful in selected cases of symptomatic CR. Multicentric studies are needed to determine its safety and efficacy in larger population.

Successful surgical management of combined presence of congenital diaphragmatic hernia, ventricular septal defect, and patent ductus arteriosus in a child with allergy to frusemide—a case report

A 6-week-old male child presented with a right-sided congenital diaphragmatic hernia (CDH), large perimembranous (PM) ventricular septal defect (VSD) and multiple muscular VSDs, and a patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PAH). The child was found to be allergic to frusemide. The VSDs were closed, the PDA was ligated, and the CDH was repaired through a right subcostal incision. Postoperatively, there were recurrent episodes of right lung lobar collapse, but the child responded to intravenous antibiotics and nasal continuous positive airway pressure (CPAP). The child is medically stable and on outpatient follow-up. We present this rare combination of diseases in view of a successful simultaneous surgical repair.

Recent advances in managing septal defects: ventricular septal defects and atrioventricular septal defects.

This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD) is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe and effective and prevent the development of PVOD and cardiac dysfunction.

A Unique Case of Facial Dysmorphism in an Infant

A preterm infant with a ventricular septal defect and facial dysmorphisms. ### Prenatal and Birth Histories ### Presentation After birth, the infant was admitted to the NICU because of prematurity. He required supplemental oxygen through a nasal cannula for a brief period (<1 week) and was successfully weaned to room air. Echocardiography revealed a large perimembranous ventricular septal defect (VSD) with 2 small ventricular muscular defects. The team identified facial dysmorphisms and ordered a microarray analysis. ### Physical Examination (After Birth)

Two-stage surgery for a large ventricular septal defect and patent ductus arteriosus associated with severe pulmonary arterial hypertension in an adult patient

Introduction. Adult patients with pulmonary arterial hypertension (PAH) associated with congenital left-to-right shunting are often considered inoperable. Case outline. A 26-year-old man presented with effort intolerance and palpitations. The diagnosis of PAH in the presence of a large perimembranous ventricular septal defect (VSD) and patent ductus arteriosus (PDA) was established. The patient was managed with a two-stage surgical approach involving an initial ligation of PDA, followed by VSD closure using unidirectional valved patch. Treatment decisions were based on the results of both invasive and non-invasive investigations. At follow-up, the patient was asymptomatic with pulmonary arterial pressure and vascular resistance returning to normal levels. Conclusion. There is a possibility for an adult patient to have a congenital heart disease associated with marked pulmonary overcirculation that is still amenable to surgical repair. This implies that there is an individual response to a long-standing left-to-right shunt, and that the therapy should be considered on a case-by-case basis.

Ventricular Septal Defect with Pulmonary Valve Endocarditis with Vegetation - Successful Surgical Treatment in Apollo Hospitals Dhaka

Background: Pulmonary valve vegetation with ventricular septal defect needs early surgical intervention.Case presentation: A 21 years old man got admitted in Apollo Hospitals Dhaka, with the complaints of dyspnoea on exertion since childhood. Echocardiogram revealed a large perimembranous ventricular septal defect, moderate pulmonary stenosis, vegetation attached with pulmonary valve leaflet moving during systole &amp; diastole. During operation ventricular septal defect was closed by dacron patch. Large vegetation was excised from anterior cusp of pulmonary valve. A perforation in right cusp of pulmonary valve was repaired with pericardial patch. Transesophageal Echocardiogram revealed well functioning ventricles and no leakage through the repaired Ventricular Septal Defect and pericardial patch in pulmonary valve leaflet.Result: Culture Sensitivity from vegetation showed no growth and histopathological examination revealed tissue consistent with vegetation of Pulmonary Valve. Post- operative echocardiogram showed no residual shunt or vegetation. The patient was discharged on 8th post operative day in good general condition and is doing well till now.Conclusion: Pulmonary valve endocarditis with vegetation with ventricular septal defect is a rare association. Early surgical intervention gives good outcome.Pulse Vol.9 January-December 2016 p.69-72

Intracardiac Lateral Tunnel Fontan by using Right Atrial Wall

The Fontan procedure is a palliative surgical procedure used in children with complex congenital heart defects. It involves diverting the venous blood from the right atrium to the pulmonary arteries without passing through the morphologic right ventricle. A 23 years old adult male from Noakhali admitted with the diagnosis of transposition of great arteries (D-TGA) with large perimembranous ventricular septal defect (PM VSD) with severe pulmonary stenosis (PS) and single ventricle morphology in National Institute of Cardiovascular Disease (NICVD), Dhaka .He underwent Intracardiac lateral tunnel Fontan by using right atrial wall. Patient recovered uneventfully in his early postoperative period. The lateral tunnel Fontan procedure results in excellent long-term outcome even when used in patients with diverse anatomic diagnoses. The incidence of atrial tachyarrhythmia is low and mainly depends on the underlying cardiac morphology and preoperative arrhythmia. The good long-term outcome after an intracardiac lateral tunnel Fontan procedure should serve as a basis for comparison with other surgical alternatives. But using right atrial wall as a buffle is rare and done first time in Bangladesh.Cardiovasc. j. 2016; 9(1): 68-72

Closure of a large perimembranous ventricular septal defect in a 4.8 kg baby with Down syndrome using a duct occluder

A 9.5-month-old boy with Down syndrome, weighing 4.8kg, presented with history of failure to thrive. Clinically, he had symptoms and signs of congestive heart failure. His echocardiogram showed a large perimembranous ventricular septal defect (pmVSD) with some inlet extension covered by a large aneurysmal tissue with multiple right ventricular (RV) exits. Additionally, he had hypothyroidism and Hirschsprung disease. Instead of closing the VSD surgically, the VSD was successfully closed utilizing an 8×6mm duct occluder. The baby remained in the intensive care unit for one night. The day after the procedure, the infant was stable and showed clinical improvement. Electrocardiogram (ECG) showed normal sinus rhythm with no evidence of heart block. Twenty-four hours later, echocardiography showed the device was in an excellent position, with a small residual leak. There was normal tricuspid valve inflow and normal aortic valve outflow with no significant valvar insufficiency. The baby was discharged after 3days in stable condition. We believe infants with such co-morbidities which might complicate their post-operative course and prolong the intensive care unit admission, might benefit from such alternative management.

Permanent complete heart block following surgical closure of isolated ventricular septal defect

A serious complication after surgical closure of ventricular septal defect (VSD) is complete heart block (CHB). It continues to be a leading cause of long-term postoperative cardiac morbidity despite all surgical technical improvements, especially with performance of more surgical procedures in increasingly younger patients. ObjectiveThis study was undertaken to determine the incidence of early postoperative CHB requiring pacemaker implantation following surgical repair of isolated ventricular septal defect, and try to identify possible procedural risk factors. DesignProspective study design. SettingMulticenter study: Ain-Shams University Hospital, Aboul Reesh Student Hospital and The National Heart Institute. MethodWe reviewed four hundred patients who had a surgical repair of isolated VSD from 2009 to 2011. Results14 out of 400 patients (3.5%) developed permanent post-operative CHB. All; but one; underwent closure of large perimembranous VSD. CHB patients had a significant lower body weight (8.36 vs. 12.68kg, p<0.01), longer ACC time (42.6 vs. 36.4min, p<0.01), longer CPB time (75.4 vs. 67.4min, p<0.01) and longer hospital stay (19 vs. 8.3days, p<0.01). Tricuspid valve detachment was performed in 28 patients (7%) and was associated with a higher incidence of CHB (7% vs. 3.75%, p=0.6). ConclusionLarge perimembranous VSD and lower body weight appear to be independent risk factors for permanent CHB following the surgical closure of isolated VSD.