Large Ventricular Septal Defect Research Articles

Echocardiographic detection of mesocardia, situs solitus associated with multiple congenital heart defects in a neonate: Rare case report

Mesocardia is a condition in which the heart is longitudinally oriented along its long axis in the midline. Cardiac position refers to the anatomical position of the left ventricular apex in the thorax. The heart has three positions: dextrocardia or right-sided heart, levocardia or left-sided heart, mesocardia or midline heart. Mesocardia is extremely rare. It represents only 0.2% of cardiac anomalies. Mesocardia is usually associated with cardiac structural abnormalities, heterotaxy syndrome, chromosomal disorders and genetic syndromes. Occasionally, it is an isolated finding. We are presenting an interesting case report of severely symptomatic neonate afflicted with mesocardia, situs solitus, moderate sized atrial septal defect (ASD) and large ventricular septal defect (VSD).

How do Age at the Surgery and Birth Weight Influence Post-Operative Anthropometric Parameters in Infants with Surgical Closure of Large Ventricular Septal Defects? A Prospective Cohort Study from a Lower-Middle-Income Country.

Closure of the large ventricular septal defects (VSD) in infancy can lead to normalization of growth, but data are limited. Our study is done to assess the growth pattern in different age groups of children and lower birth weight babies after shunt closure. This is a prospective observational study that included infants with isolated large VSD operated in infancy. Anthropometric data were collected at baseline and at follow-up, and growth patterns were analyzed. 99 infants were included in the study. The mean age and weight at the time of surgery were 6.97 ± 2.79months and 5.07 ± 1.16kg, respectively. The mean follow-up duration was 8.99 ± 2.31months. The weight for age (W/A) was the most adversely affected parameter preoperatively, and there was significant improvement noted in the mean Z score for W/A after shunt closure (-3.67 ± 1.18 vs. -1.76 ± 1.14, p = 0.0012). There was improvement in Z-scores for length for age (L/A) and weight for length (W/L), although it was not statistically significant. The infants from all the age groups had statistically significant growth in the anthropometric parameters. The rate of weight gain was maximum in the infants operated below 8months of age (2-4months = 3588g, 5-6months = 3592g, 7-8months = 3606g, 9-10months = 2590g, 11-12months = 2250g). Low birth weight and normal birth weight infants had similar Z-scores at the time of surgery and at follow-up in all 3 anthropometric parameters, and birth weight did not affect pre- as well as post-operative growth parameters. Suboptimal improvement in weight and length was seen in 40 and 20% of babies even after successful surgical repair, respectively. Growth failure in infants with a large VSD can be multifactorial. Early surgical closure of the shunt can lead to early normalization of growth parameters and faster catch-up growth. Few babies may fail to demonstrate a positive growth response even after timely surgical correction, and may be related to intrauterine and genetic factors or faulty feeding habits.

Double Intervention in a Patient with Large Patent Ductus Arteriosus (PDA) and Moderate Upper Muscular Ventricular Septal Defect (VSD) with Bidirectional Shunt with Severe Pulmonary Hypertension

Patent ductus arteriosus (PDA) and Ventricular septal defect (VSD) are commonly encountered problems and constitute about 40% of all congenital heart lesions. The association of these two conditions in a single patient is not very uncommon. Both these conditions can be treated by placing intracardiac devices. Double interventional closure of Patent Ductus Arteriosus (PDA) and Ventricular Septal Defect (VSD) was performed in a gravely ill infant at her 3 months and 5 months of age respectively, who was suffering from recurrent pneumonia and heart failure since birth. These were lifesaving procedure for that patient which led to writing this report.

Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining.

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart disease that has numerous subtypes. While most patients with CC-TGA have a large ventricular septal defect (VSD) and pulmonary stenosis, there are some patients who have either no VSD or a highly restrictive VSD. These patients will require left ventricular (LV) retraining prior to double switch. The purpose of this study was to review our experience with the double switch procedure in patients who had previously undergone LV retraining. This was a retrospective review of a single institution experience with the double switch procedure in patients who had undergone LV retraining (2002-present). Forty-five patients underwent double switch following LV retraining. Of these, 39 had an arterial switch with hemi-Mustard/bidirectional Glenn and six had a Senning. The median cross-clamp time was 135 min (range 71-272) and median bypass time was 202 min (range 140-430 min). Median hospital length of stay was eight days (range 4-108). There were no in-hospital deaths. Median duration of follow-up was 30 months (range 0-175). One patient subsequently underwent heart transplantation and died 65 months following double switch. At follow-up, 41 of the 44 survivors (93%) have normal or low normal LV function and 40 of the 44 survivors (91%) have no or trace mitral regurgitation. The data demonstrate early and mid-term survival of 100% and 97%. Ninety-three percent had preserved LV function. These results suggest that patients with CC-TGA who undergo LV retraining and double switch can have excellent clinical outcomes.

A case series of three patients of double-chambered right ventricle with distinctive features

Double-chambered right ventricle (DCRV) is an extremely rare condition caused by anomalous muscular bundle (AMB), dividing the RV into high- and low-pressure chambers. We are highlighting a case series of three rare patients of DCRV, presenting with diverse clinical and echocardiographic features. Case 1 is manifesting as isolated DCRV in an asymptomatic young female adult, with the AMB located in the subvalvular region of the right ventricular outflow tract. Case 2 is of a significantly symptomatic male infant, afflicted with DCRV, and associated with a large apical muscular ventricular septal defect (VSD). The AMB was situated near the RV apex, just proximal to the VSD. Case 3 represented an asymptomatic DCRV in an adolescent female along with a coexisting small hemodynamically insignificant VSD. Individually, all the three patients are exceedingly rare and their clinical and echocardiographic features were peculiarly distinctive, hence making this case series an interesting and exemplary manuscript.

Percutaneous Closure of Hemodynamically Significant Postoperative Residual Ventricular Septal Defects.

The experience with percutaneous closure of postoperative residual ventricular septal defects (VSDs) is expanding with improved device technology and techniques. To report our experience with percutaneous closure of residual VSDs after cardiac surgeries. Retrospective clinical data review of patients who had percutaneous closure of postoperative residual VSDs at our institution between 2010 and 2022. Patients' demographics, procedural, and follow-up data were looked at. Twelve patients (50% males) with a median age of 9.2years (range 0.9-22) were identified. Baseline surgeries were 8 tetralogy of Fallot corrections, 2 pulmonary bandings for large muscular VSD (mVSD) including 1 coarctation repair, 1 atrioventricular septal defect repair, 1 sub-aortic membrane resection-induced iatrogenic VSD, 1 isolated VSD closure, and 1 additional mVSD. Median duration between baseline surgery and percutaneous VSD closure was 2.2years (range 0.2-8.3). Residual VSD shunting was secondary to surgical patch leakage in 8/12 patients. The median angiographic defect diameter was 6.8mm (range 4.8-14). The defect was balloon-calibrated in 3/12 patients. Defects were tackled retrogradely in 3/9 patients. Amplatzer Membranous VSD occluder (n = 1), Lifetech Multifunctional (n = 5), Membranous (n = 1) and muscular VSD occluders (n = 2) and Occlutech Membranous (n = 1) and Muscular (n = 2) VSD occluders were used. The procedure was successful in 10/12 patients. Two devices embolized to the pulmonary artery and were snare-retrieved. Both patients were referred for surgery. The median follow-up was 1.3years (range 0.1-12). Six-month ultrasound showed one trivial residual shunt and one mild right ventricular outflow obstruction. One patient is receiving targeted therapy for pulmonary hypertension at 2years of follow-up. Transcatheter closure of postoperative residual VSDs is a feasible yet challenging intervention. Procedural complications can be encountered.

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.

Hybrid Perventricular Ventricular Septal Defect Device Closure in a 3-Months Old Infant with Severe Failure to Thrive

We report a case of successful hybrid device closure of large mid muscular Ventricular Septal Defect (VSD) in an infant for the very first time in Pakistan. Increasing collaboration between pediatric cardiac interventionalists and congenital cardiac surgeons has resulted in introduction of various hybrid procedures in the management of Congenital Heart Diseases (CHD). Advantages of hybrid procedures include complex interventions by using minimally invasive methods, avoidance of cardiopulmonary bypass, shorter procedure and recovery time leading to subsequent decrease in complications. A large VSD in an infant carries an increased risk of morbidity including failure to thrive, congestive cardiac failure, and recurrentrespiratory tract infections. Timely and safely the successful perventricular closure of large VSD using hybrid approach is a milestone in the management of VSDs in infants.

Abstract 14080: Broadening Indications for Neonatal Balloon Atrial Septostomy in Dextro-Transposition of the Great Arteries Reduce Predictive Value of Fetal Echocardiography

Introduction: Neonates with dextro-transposition of the great arteries (d-TGA) require adequate mixing at the atrial level. If not present, these infants experience severe cyanosis, necessitating an urgent balloon atrial septostomy (BAS). Multiple studies have identified various fetal echocardiography (FE) findings as risk factors for postnatal BAS. Anecdotally, the predictive value of these findings remains poor in clinical use. Hypothesis: We aimed to determine the relationship between inter-rater reliability (IRR) of FE BAS predictors and need for postnatal BAS, hypothesizing that IRR would be poor and perhaps contributing to limited predictive value of FE findings. Methods: We identified d-TGA pregnancies followed at our institution between 2012 and 2022, excluding fetuses with large ventricular septal defects and pregnancies that were either terminated or lost to follow up. Three independent readers blinded to BAS outcome assessed 7 FE predictors: pulmonary vein Doppler velocity > 41 cm/sec, reverse flow in patent ductus arteriosus (PDA), restrictive PDA, restrictive patent foramen ovale (PFO), bidirectional PFO flow, flat septum primum, and hypermobile septum primum. IRR was determined with the S metric. Clinical information immediately before BAS was collected to evaluate postnatal physiology. A multivariable logistic regression was used to determine significant associations between FE findings and postnatal BAS. Results: Fifty-six fetuses were included in this study, of whom 25 (45%) underwent BAS. IRR was lowest for PFO flow direction and highest for restrictive PDA, likely due to its rarity. The remainder of FE predictors had either a moderate or substantial level of IRR. The last pre-BAS range of PaO 2 (24-49 mmHg), O 2 saturation (56-86%) and pH (7.18-7.52) reflected variable physiology. Of the 5 FE characteristics that could be included in the regression analysis, none were found to be significantly associated with odds of a BAS. Conclusions: Our results demonstrate substantial but not excellent IRR for current FE BAS predictors, but no significant association between these predictors and postnatal BAS in d-TGA patients. Poor predictive value of FE for postnatal BAS could be impacted by broadening indications for BAS.

EP24.44: Double inlet ventricle with pulmonary atresia with large ventricular septal defect with right aortic arch

EP24.44: Double inlet ventricle with pulmonary atresia with large ventricular septal defect with right aortic arch

Surgical outcomes and long-term follow-up of patients with large ventricular septal defects with severe pulmonary arterial hypertension: single-center experience

Purpose: In this retrospective study, pediatric patients who underwent surgery for large ventricular septal defect (VSD) with severe pulmonary arterial hypertension (PAH) were evaluated in detail in terms of clinical characteristics, and long-term results, and reinterpreted with the current surgical criteria.
 Materials and Methods: Twenty-four patients who underwent surgery for large VSD due to PAH between 1994 and 2005 were included in the study. The patients were divided into two groups according to their pulmonary vascular resistance index (PVRI) and compared (group 1 with PVRI

Use of the Occlutech pmVSD Device for Hybrid Closure of a Large Mid-Muscular Ventricular Septal Defect in a Small Infant Born With Hypoplastic Aortic Arch.

We describe the perventricular device closure of a large mid-muscular ventricular septal defect (VSD) in a 2.9 kg infant born with hypoplastic aortic arch and VSD using an Occlutech perimembranous occluder. In this case, the anatomy required a short low-profile device and hence a perimembranous occluder was used. To our knowledge, this is the first described use of this device for hybrid closure of a muscular VSD and the application of this technique in a patient <3 kg.

Electrocardiographic characteristics of newborns with ventricular septal defects: a Copenhagen Baby Heart Study

Ventricular septal defects (VSD) represent the most common congenital heart defect in newborns. We assessed the electrocardiographic characteristics of newborns with VSDs in a general population sample. The Copenhagen Baby Heart Study is a prospective population–based cohort study offering cardiac evaluation of newborns. Echocardiograms and electrocardiograms were obtained within 30 days after birth and systematically analysed. A VSD was identified in 530 newborns (mean age 11 ± 7 days, 42% boys). Newborns with VSDs had a more left-shifted QRS axis (116 ± 34 vs. 120 ± 3°, p = 0.02), and a higher S-wave amplitude in V1 (721 ± 584 vs. 636 ± 549 µV, p = 0.001) than controls. The largest differences were found in newborns with large or perimembraneous VSDs with a higher frequency of left axis deviation, higher S-wave amplitudes in V1, and higher R- and S-wave amplitudes in V6 compared with controls. R-waves in V1 and V6 were significantly associated to left ventricular mass, whereas S-waves in V1 and V6 were dependent on left ventricular end-diastolic diameter on echocardiography. Conclusion: Newborns with VSDs showed significant differences in QRS axis, and R- and S-wave precordial amplitudes compared to matched controls. Perimembranous and large VSDs had the greatest effect on the neonatal ECG.What is Known:• Ventricular septal defects in newborns are prevalent and may affect cardiac function and structure.What is New:• The Copenhagen Baby Heart Study is the largest study including a cohort of unselected newborns undergoing postnatal cardiac examination.• We found that newborns with VSD showed significant electrocardiographic differences depending on size and type of VSD compared with healthy newborns.

Brain Abscess Drainage in a Case of Tetralogy of Fallot with Pulmonary Atresia with Major Aortopulmonary Collateral Arteries Under Scalp Block

AbstractTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. There are a few reports of uncorrected TOF patients surviving into adulthood requiring noncardiac surgery. They are commonly susceptible to developing brain abscesses due to paradoxical embolism and lack of pulmonary phagocytic clearance. A 32-year-old man, a known patient of TOF with pulmonary atresia and major aortopulmonary collateral arteries, presented with left side weakness and seizures. He was drowsy with a Glasgow Coma Scale (GCS) of 13/15, heart rate of 72/min, oxygen saturation of 78% with 6 L/min oxygen via face mask, and blood pressure of 90/60 mm Hg with noradrenaline at 0.5 mcg/kg/min. He had central cyanosis, clubbing, and icterus. The left upper and lower limb power was 2/5, whereas it was 5/5 for the right. Cardiovascular examination revealed a grade 3/6 continuous murmur at the base of the heart. No pulmonary abnormality was noted on auscultation. Echocardiography showed large subaortic ventricular septal defect (VSD) with overriding of the aorta, pulmonary atresia, right-sided aortic arch, and ejection fraction of 62%. Magnetic resonance imaging (MRI) of the brain revealed features suggestive of brain abscess. Brain abscess drainage was performed under scalp block with sedation to avoid major hemodynamic fluctuations. The procedure was uneventful, and the patient's power on the left side improved postprocedure. Scalp block with sedation is a suitable alternative to general anesthesia for drainage of brain abscess in TOF patients.

Late surgical ventricular septal defect closure in a low middle-income country setting: a case series

BackgroundVentricular septal defect (VSD) is the commonest type of congenital heart lesion accounting for up to 40% of congenital heart defects. Well timed VSD closures are reported to yield excellent long-term outcomes. Late surgical VSD closures, particularly from the developing countries, are infrequently reported.Case presentationWe report three cases of African children aged between 13 and 14 years who had late VSD presentations. They reported complaints of growth failure and recurrent respiratory infections since early infancy which necessitated frequent visits to primary health care facilities. They were found to have large ventricular septal defects by thoracic echocardiography. Diagnostic cardiac catheterization was done to all three patients to rule out irreversible pulmonary hypertension. After promising cardiac catheterization findings, they all underwent successful surgical VSD repair with good early outcomes.ConclusionVSD surgical closure is ideal in children below 2 years, however, it can be done in children who present at advanced age despite being considered high risk patients. All three of our patients who presented late had successful surgical VSD repairs with promising immediate outcome. The role of genetics in the protection against developing irreversible pulmonary vascular disease in these patients is a possible area for future studies.

Closure of transcatheter ventricular septal defect using LifetechTM Konar-MF Occluder in children weighing less than 10 kilograms: mid-term results, a tertiary single center experience.

Transcatheter closure of medium and large ventricular septal defects (VSDs) in young children is limited due to the use of over-sized devices that can cause hemodynamic instability and arrhythmia. In this study, we aimed to retrospectively evaluate the safety and efficacy of the device in the mid-term in children weighing less than 10 kg whose transcatheter VSD was closed only with the Konar-MFO device. Among 70 children whose transcatheter VSD was closed between January 2018 and January 2023, 23 patients weighing less than 10 kg were included in the study. Retrospectively, the medical records of all patients were reviewed. The mean age of the patients was 7.3 (4.5-26) months. 17 of the patients were females, 6 of them were males, F/M: 2.83. The average weight was 6.1 (3.7-9.9) kg. The mean the pulmonary blood flow/ systemic blood flow (Qp/Qs) was 3.3 (1.7-5.5). The mean defect diameter was 7.8 mm (5.7-11) for the left ventricle (LV) side, and 5.7 mm (3-9.3) for the right ventricle (RV) side. Based on the utilized device dimensions, the measurements on the LV side were recorded as 8.6 mm (range 6-12), while those on the RV side were recorded as 6.6 mm (range 4-10). Antegrade technique was applied to 15 (65.2%) patients and retrograde technique was applied to 8 (34.8%) patients in the closure procedure. The procedure success rate was 100%. The incidence of death, device embolization, hemolysis, or infective endocarditis was zero. Perimembranous and muscular VSDs in children under 10 kg can be successfully closed under the management of an experienced operator with the Lifetech Konar-MFO device. This is the first study in the literature to evaluate the efficacy and safety of the device in children under 10 kg in whom only Konar-MFO VSD occluder device is used for transcatheter VSD closure.

Case report of coexisting transposition of the great arteries with supracardiac total anomalous pulmonary venous connection: focus on computed tomography features

BackgroundThe coexisting of transposition of the great arteries (TGA) with total anomalous pulmonary venous connection (TAPVC) is one of the rare anomalies. The incidence of coexisting TAPVC and TGA is unknown with very few cases ever reported.Case presentationWe reported a case of a 13-month-old female toddler with history of cyanosis. Echocardiography revealed atrioventricular ambiguity with pulmonary atresia, all PVs drain into the innominate vein via vertical vein (VV), ostium secundum atrial septal defect (ASD) and ventricular septal defect (VSD) were observed. The CT scan confirmed co-occurrence of TGA and TAPVC. All four confluence PVs behind the small left atrium (LA), drains into an ascending lateral large VV, coursing to innominate vein without any PV access into LA. The superior vena cava, right atrium and right ventricle (RV) were dilated. The RV is the origin of the aortic root. The aorta continues on the right side, with an arterial connection to the right pulmonary aberrant artery at the level of the aortic arch. Main pulmonary artery originates from LV and appears atretic with only connection to the left pulmonary artery. Large ASD and VSD were identified.ConclusionsTGA and TAPVC are a rare combination and should be suspected in mild-cyanotic cases with levocardia with situs solitus. CT angiography is one of the modalities of choice to characterize the vasculature anomalies.

Myocardial infarction complicated by a large ventricular septal defect: a case report

Myocardial infarction complicated by a large ventricular septal defect: a case report

Anaesthesia for Caesarean Section in a Patient with Large Ventricular Septal Defect with Pulmonary Hypertension with Single Ventricle Physiology

Pregnancy in a patient with large ventricular septal defect (VSD) with severe pulmonary hypertension (PAH) increases the risk of mortality severalfold. This when combined with single ventricle physiology forms a rare and deadly combination. A 26-year-old female presented for elective LSCS at 35 weeks gestation. She had a large VSD with severe PAH with a single ventricle physiology. We report the successful conduct of a caesarean section in this patient under graded epidural anaesthesia.

Transcatheter closure of large postinfarct ventricular septal defect: Initial results of prototype Occlutech® device including the first-in-human.

Postinfarct ventricular septal rupture is a serious complication in delayed or failed reperfusion with a grim prognosis. The optimal timing and treatment option remain debatable in the absence of randomized controlled trials. Percutaneous device closure is a well-reported and less invasive treatment option but recent imaging studies indicate that majority of defects are too large to be adequately covered by the currently Conformite Europeenne and Food and Drug Administration approved occluder devices. Six patients presented with large and complex postinfarct ventricular septal ruptures, considered unsuitable for the Amplatzer post-infarct ventricular septal defect Occluder, sowere treated using the prototype Occlutech® 36 mm PI-VSD occluder, including the first-in-human use. The prototype device was successfully deployed in all patients with satisfactory immediate results and shunt reduction. Three patients (50%) in cardiogenic shock did not survive beyond discharge, of which two were complicated by device dislodgement or embolization. Percutaneous closure of large postinfarct ventricular septal ruptures is possible using newer device with a wider coverage. Further device refinement is necessary to improve treatment outcomes.