Multicenter Experience for Early and Mid-Term Outcome of MyVal Transcatheter Pulmonary Valve Implantation.

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12-19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31-63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10-53 mmHg) pre-procedure to 10 mmHg (IQR 5-16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5-44 min). The large sizes-mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120-300 mL). Patients were followed for a median period of 360 days (IQR 164-525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Giant pulmonary pseudoaneurysm following balloon dilatation of the pulmonary artery to relieve pulmonary band.

Pulmonary artery pseudoaneurysms are uncommon. They may occur secondary to trauma, infectious diseases, vasculitis syndromes, neoplasms, congenital diseases, and pulmonary hypertension. Due to increasing number of cardiac interventions, iatrogenic complications are among the major causes of pulmonary artery pseudoaneurysms.In this report, we present a 6-month-old patient with pulmonary pseudoaneurysm that occurred following pulmonary balloon angioplasty for the relief of a pulmonary band.

Experiences of Two Centers in Percutaneous Ventricular Septal Defect Closure Using Konar Multifunctional Occluder.

BACKGROUND: Transcatheter closure of perimembraneous ventricular septal defect still poses a challenge due to the adjacent structures of the tricuspid and aortic valves and the risk of atrioventricular block. We report our experience at 2 centers using the KONAR-MF (multifunctional occluder) ventricular septal defect device, which gained its CE mark in May 2018. METHODS: A retrospective study was carried out on all patients who underwent transcatheter ventricular septal defect closure with the KONAR-MF (multifunctional occluder) ventricular septal defect device at 2 centers. RESULTS: A total of 47 patients were identified. The median age and weight of the patients were 25.8 months and 11 kg. The ventricular septal defects that were closed in 5 cases were post-operative hemodynamically significant residual ventricular septal defects. Forty-eight devices were used in the 47 cases. As for the location of the ventricular septal defect, 40/48 (83.33%) ventricular septal defects were perimembranous and 8/48 (16.66%) were muscular. The percutaneous device closure was successful in 46 procedures (95.8%). Device embolization was observed in 2 patients, and a significant residual shunt was observed in 2 cases. In the follow-up, there was no enhancement in the residual shunt in the remaining cases. Temporary atrioventricular block occurred in 2 cases during the procedure and improved after long sheath withdrawal. CONCLUSION: Soft, flexible, and low-profile KONAR-MF (multifunctional occluder) occluders ensure easy and safe implantation, and small sheath sizes allow for their use in small infants. Although near and mid-term follow-ups did not indicate any permanent atrioventricular block, a larger sample of patients and a longer follow-up period is necessary to understand long-term outcomes.

Percutaneous Closure of Atrial Septal Defects with the MemoPart Atrial Septal Occluder: The Early-Term Results of a Multicenter Study.

Atrial septal defect (ASD) is a common congenital heart disease with left-to-right shunt that may lead to pulmonary hypertension over time. Secundum ASD closure with transcatheter technique is currently the preferred method. The aim of this study was to evaluate the clinical experience and early-term outcomes of patients treated with a MemoPart ASD occluder device between June 2013 and June 2019. Fifty-six patients (35 females) with a mean age of 9.4 ± 6.6 years (range: 2-44 years) were included in the study. The diameters of the devices used to close the ASDs were 7-28 mm. The ratio of the device/defect diameter was 1.14:1. Atrial septal defect closure was applied successfully in all patients. The MemoPart septal occluder is a safe and effective device for ASD closure. In wide ASDs and cases with more than one deficient rim, weak rims, or wide and complicated cases, it can be used carefully with sufficient experience.

The Use of Chronic Total Occlusion (CTO) Wires for Perforation of Atretic Pulmonary Valve; Two Centers Experience.

Pulmonary valve atresia with intact ventricular septum (PA-IVS) can be treated either surgically or transcatheterly for eligible patients. Perforation of pulmonary valves using chronic total occlusion (CTO) guidewires has been reported as an alternative to radiofrequency (RF) perforation. We sought to report our experience with CTO guidewires for perforation of atretic pulmonary valves and subsequent balloon dilatation (with or without patent ductus arteriosus stenting) in patients with PA-IVS from two centers. A retrospective study was carried out on PA-IVS patients who underwent intervention between March 2014 and September 2019, in which CTO guidewire was employed for pulmonary valve perforation. A total of 26 patients were identified. The median age and weight of the patients were 5.2 days (range 1-21 days) and 3.1 kg (range 2.2-3.8 kg), respectively. All patients were situs solitus, except one patient with left atrial isomerism. The right ventricle (RV) morphology was bipartite in 22/26 patients and tripartite in 4/26 patients. Before the procedure, the mean saturation was 76% (range 70-86%) while the patients were under prostaglandin infusion. The pulmonary valve perforation attempt was performed with the Asahi Conquest Pro 9 CTO wire (n = 6) or Asahi Pro 12 CTO wire (n = 18) and/or Asahi Miracle CTO wire (n = 2). The procedure was successful in 20/26 (77%) patients using CTO wires. We analyzed the efficiency of CTO wire based on the subtypes: Conquest Pro 9 in 6/6 (100%) patients, Conquest Pro 12 in 12/18 (67%) patients, and Miracle in 2/2 (100%) patients. Before CTO wire usage in 3 patients, radiofrequency (RF) perforation was unsuccessful. Among these 3 patients, pulmonary valve perforation was successful in 2 patients with CTO wire; hence, in the remaining patient, perforation was also unsuccessful with CTO wire. After CTO wire perforation was unsuccessful in 6 patients, RF perforation was attempted in 3 patients (2 successful attempts and 1 unsuccessful attempts), and one patient as referred to surgery. Desaturation was persistent in 19 cases, which necessitated ductus arteriosus stenting. Early procedural complication was observed in 3/26 (11%) patients. Two of these patients had vascular complications due to the sheath, which was treated with heparin infusion and streptokinase, and the remaining patient had sudden bradycardia and cardiac arrest during the procedure and did not respond to cardiac resuscitation. CTO wires should be keep in mind for atretic pulmonary valve perforation as a first choice or when RF perforation is unsuccessful.

Transcatheter closure of the aortopulmonary window in a three-month-old infant with a symmetric membranous ventricular septal defect occluder device.

Although most of aortopulmonary window cases are closed surgically, percutaneous closure can be also used in suitable patients. Defects which are far from the pulmonary and aortic valves, coronary artery, and pulmonary artery bifurcation, with adequate septal rims are considered suitable for percutaneous closure. A three-month-old male infant weighing 4 kg was referred to our pediatric cardiology department with the complaints of fatigue while breastfeeding, difficulty in weight gain, heart murmur, and respiratory distress. A large aortopulmonary window (5.3 mm) and left heart chamber dilatation were detected on echocardiography. The large aortopulmonary window was closed using a symmetric membranous ventricular septal defect occluder device. The closure procedure was performed via the antegrade route without forming an arteriovenous loop. In conclusion, the use of a symmetric membranous ventricular septal defect device for closure of large aortopulmonary window seems to be a safe and effective alternative to surgery in selected infants.

Current Strategies for the Management of Anomalous Origin of Coronary Arteries from the Pulmonary Artery.

INTRODUCTION: The coronary arteries, which have to originate from the aorta, may sometimes rise from the pulmonary artery. This study evaluated clinical and diagnostic findings, treatment methods, and follow up of cases with anomalous coronary arteries arising from the pulmonary artery. PATIENTS AND METHODS: Eight patients with the diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) (N = 6) and anomalous right coronary artery from the pulmonary artery (ARCAPA) (N = 2), between January 2014 and January 2020 from a single center university hospital, were included in the study. Data from patients' demographic characteristics, electrocardiography, echocardiography, angiographic findings, operation, hospitalization, and follow up were evaluated. RESULTS: The study included eight patients (six females and two males) - six patients with ALCAPA and two with ARCAPA. The ages of the patients ranged between 3-135 (average: 53.25) months. The median body weight was calculated as 17.4 kg. Severe mitral valve insufficiency was detected in two patients and two other patients had a moderate degree of mitral insufficiency on echocardiography. Ejection fractions ranged between 16-74%. One patient had perimembranous malalignment large ventricular septal defect with pulmonary stenosis. Operative techniques were Takeuchi procedure (three patients), direct implantation (four patients), and left internal thoracic artery to left main coronary artery bypass (one patient). Mechanical cardiac support was not required in the postoperative period. Mortality did not occur. Mitral insufficiency and ejection fractions improved following correction of the coronary anatomy. CONCLUSION: It is important to diagnose the ALCAPA or ARCAPA, where the coronary artery originates from the pulmonary artery. Patients should be treated before congestive heart failure and fatal complications occur. Surgical correction should be planned regardless of symptom status, even though some of patients reach adulthood with an increased number of collaterals.

Right ventricular outflow tract stenting during neonatal and infancy periods: A multi-center, retrospective study.

BACKGROUND: The aim of this study was to evaluate the outcomes of right ventricular outflow tract stenting for palliation during the newborn and infancy periods. METHODS: Between January 2013 and January 2018, a total of 38 patients (20 males, 18 females; median age 51 days; range, 3 days to 9 months) who underwent transcatheter right ventricular outflow tract stenting in three centers were retrospectively analyzed. Demographic characteristics, cardiac pathologies, angiographic procedural, and clinical follow-up data of the patients were recorded. RESULTS: The diagnoses of the cases were tetralogy of Fallot (n=27), double outlet right ventricle (n=8), complex congenital heart disease (n=2), and Ebstein"s anomaly (n=1). The median weight at the time of stent implantation was 3.5 (range, 2 to 10) kg. Five cases had genetic abnormalities. The median pre-procedural oxygen saturation was 63% (range, 44 to 80%), and the median procedural time was 60 (range, 25 to 120) min. Acute procedural success ratio was 87%. Reintervention was needed in seven of patients due to stent narrowing during follow-up. During follow-up period, seven cases died. Total correction surgery was performed in 26 patients without any mortality. While a transannular patch was used in 22 patients, valve protective surgery was implemented in two patients, and the bidirectional Glenn procedure was performed in two patients. CONCLUSION: Based on our study results, right ventricular outflow tract stenting is a form of palliation which should be considered particularly in cases in whom total correction surgery is unable to be performed due to morbidity.

Off-pump ascending aorta or aortic arch to descending aorta bypass with a pericardial roll for the treatment of critically ill infants with interrupted aortic arch.

AIM: Standard surgical treatment of the interrupted aortic arch with the use of cardiopulmonary bypass is risky especially in critically ill babies. In this manuscript, we present the results of off-pump pericardial roll bypass for the treatment of aortic interruption. MATERIAL AND METHODS: The technique was applied in nine critically ill infants between July 2011 and December 2019. Data were reviewed retrospectively. There were four girls and five boys. The types of the interruption were type B in six cases and type A in three babies. Additional cardiovascular anomalies were ventricular septal defect in all, atrial septal defect or patent foramen ovale in all, single-ventricle pathologies in two and bicuspid aortic valve in three cases. All the patients were in critical situations such as intubated, having symptoms of infection, congestive heart failure or ischaemia and malperfusion leading visceral organ dysfunction. RESULTS: All patients underwent off-pump ascending aorta or aortic arch to descending aorta bypass with a pericardial roll. Post-operative early mortality occurred in one patient with severe mitral regurgitation due to cardio-septic shock. One patient who had single-ventricle pathology underwent bidirectional Glenn and was lost on the post-operative 26th day due to sepsis 2 years after operation. Two patients presented with dilatation of the pericardial tube 18 and 24 months after the operations and one underwent reconstruction of the neo-arch. The remaining patients are asymptomatic, active and within normal limits of body and mental growth. CONCLUSION: Treatment of interrupted aortic arch with a bypass with an autologous pericardial roll treated with gluteraldehyde without cardiopulmonary bypass seems a safe and reliable technique especially for the treatment of critically ill infants.

Congenital cardiac interventions during the peak phase of COVID-19 pandemics in the country in a pandemics hospital in Istanbul.

INTRODUCTION: In this report, we aim to present our algorithm and results of patients with congenital cardiac disorders who underwent surgical or interventional procedures during the peak phase of the pandemics in our country. PATIENTS AND METHODS: The first COVID-19 case was diagnosed in Turkey on 11 March, 2020, and the peak phase seemed to end by the end of April. All the patients whom were referred, treated, or previously operated but still at the hospital during the peak phase of COVID-19 pandemics in the country were included into this retrospective study. Patient's diagnosis, interventions, adverse events, and early post-procedural courses were studied. RESULTS: Thirty-one patients with various diagnoses of congenital cardiovascular disorders were retrospectively reviewed. Ages of the patients ranged between 2 days and 16 years. Seventeen cases were males and 14 cases were females. Elective cases were postponed. Priority was given to interventional procedures, and five cases were treated percutaneously. Palliative procedures were preferred in patients whom presumably would require long hospital stay. Corrective procedures were not hesitated in prioritised stable patients. Mortality occurred in one patient. Eight patients out of 151 ICU admissions were diagnosed with COVID-19, and they were transferred to COVID-19 ICU immediately. Three nurses whom also took care of the paediatric cases became infected with SARS-CoV-2; however, the children did not catch the disease. CONCLUSION: Mandatory and emergent congenital cardiac percutaneous and surgical procedures may be performed with similar postoperative risks as there are no pandemics with meticulous care and preventive measures.

Left subclavian artery originating from left pulmonary artery in DiGeorge syndrome.

Left subclavian artery originating from the left pulmonary artery is a rare aortic arch anomaly. Herein, we, for the first time in Turkey, present a case of left subclavian artery originating from the left pulmonary artery via ductus arteriosus in DiGeorge syndrome and causing subclavian steal syndrome.

Use of covered stents in simultaneous management of coarctation of the aorta and patent ductus arteriosus.

OBJECTIVE: To report clinical and procedural characteristics of twelve patients who received a covered stent for the treatment of aortic coarctation and concurrent patent ductus arteriosus (PDA). METHODS: A single center database was retrospectively evaluated to obtain data of patients with combined aortic coarctation and PDA. We selected patients in whom a covered stent was used for the treatment of both pathologies. The stent length was chosen so as to cover the entire length of the lesion from healthy to healthy tissue and also cover the ampulla of PDA. RESULTS: The median age of the patients was 15 (range, 6.5-35) years. The diameter of the coarctated segment increased from a median of 8.4 (range, 2.6-10.8) mm to 16 (range, 9-24) mm (p<0.005), whereas the pressure gradient decreased from a median of 43 (range, 10-71) mm Hg to 0 (range, 0-8) mm Hg (p<0.005). Fourteen covered stents were used for 12 patients. Following deployment, seven stents were flared with larger and low-pressure balloons because of the gap between the distal end of the stent and the poststenotic dilated segment of the aorta, which caused residual PDA shunts and/or instability of the stent. After the procedure, no residual PDA shunt was present in any patient. CONCLUSION: To the best of our knowledge, this study includes the largest series of patients reported in literature in whom covered CP stents were used for simultaneous percutaneous treatment of coarctation and PDA. The procedure was successful and stable results were obtained during follow-up in all cases.

Comparison of Transcatheter Atrial Septal Defect Closure in Children, Adolescents and adults: Differences, Challenges and Short-, Mid- and Long-Term Results.

BACKGROUND AND OBJECTIVES: This study aims to compare the characteristics, effectiveness and results of transcatheter closure of atrial septal defect between children, adolescents, and adults. SUBJECTS AND METHODS: In this study, 683 patients who underwent atrial septal defect closure in the last 10 years were divided into three groups: children (age <12), adolescents (age 12 to 16), and adults (age >16) as group 1, group 2 and group 3, respectively. RESULTS: The average defect size and incidence of complex atrial septal defect were higher in group 3 (p=0.0001 and 0.03 respectively). While the average size of the devic was higher in adults (22.6±6.4 mm vs. 18.5±4.9 mm; p=0.0001), the ratio of the device size/total septum was higher in both children and adolescents (Group 1 and 2). In the child and adolescent groups and patients with only complex atrial septal defect, the use of techniques, other than standard deployment, was similar in all three groups (p=0.86 and 0.41, respectively). The ratio of the residual shunt was similar in all three groups. Major complications were seen in 5 cases (4 cases with migration, and 1 case with dislocation) in group 3 and 1 case (migration) in group 1. CONCLUSION: Depending on the complexity of the defect and age of the patient, transcatheter closure of atrial septal defect might have certain difficulties and complications. Patients must be evaluated in detail to avoid major complications and possible problems during the procedure.

Serum NGAL, cystatin C and urinary NAG measurements for early diagnosis of contrast-induced nephropathy in children.

AIM: The study investigated a number of biomarkers for the early diagnosis of contrast-induced nephropathy (CIN), which is an important cause of acute kidney injury (AKI). MATERIAL AND METHODS: The study included 91 children scheduled for elective cardiac angiography and 50 healthy controls. Biomarkers including serum (s) and urinary (u) sodium, serum and u-creatinine, s-cystatin-C, serum neutrophil gelatinase-associated lipocalin (NGAL) and urinary N-acetyl beta glucosaminidase (u-NAG)/creatinine ratio were measured 4 times sequentially in the patients and once in the controls. RESULTS: The patient group comprised 40 males (44%) and 51 females (56%) while the control group comprised 16 males (32%) and 34 females (68%). Age, gender, s-creatinine, estimated-glomerular filtration rate (eGFR), s-cystatin-C and fractional-excretion of sodium did not differ significantly between the groups. Serum sodium and s-NGAL were found to be lower in the patients than those of in the controls, while their u-NAG/creatinine ratio was found to be higher. Sequential data analysis revealed that s-NGAL and u-NAG/creatinine ratio increased in the first 6 h after radiocontrast media (RCM) administration and decreased at 12 and 24 h. Serum BUN and s-cystatin-C levels also showed a significant difference during the 24-h follow-up. eGFR, s-sodium and s-creatinine levels did not change in the following period. Serum cystatin-C levels revealed a significant negative correlation with eGFR. Administered RCM doses showed a positive correlation only with u-NAG/creatinine ratios. CONCLUSION: In the first 24 h, s-cystatin-C, s-NGAL and especially u-NAG/creatinine ratio showed promise as biomarkers, but eGFR is not adequate for early diagnosis of CIN. Sequential measurement of biomarkers may contribute to more accurate diagnosis of AKI.

A rare coronary anomaly with masked diagnosis: Anomalous left circumflex artery from right pulmonary artery.

Anomalous origin of the circumflex coronary artery from the pulmonary artery is a rare congenital coronary anomaly. While it generally follows an asymptomatic course, if undiagnosed it may lead to severe clinical outcomes, including sudden death. The condition can be masked by associated defects, so when it is clinically suspected, diagnosis must be confirmed by conventional and/or magnetic resonance angiography, even if echocardiography clearly shows coronary roots. This report describes a patient who underwent neonatal surgery for aortic coarctation and was diagnosed with coronary artery anomaly at 15 months old.

Cardiac Interventions in Pregnant Patients without Fluoroscopy.

INTRODUCTION: Cardiac interventions with fluoroscopy during pregnancy carry significant risks for the fetus. This report reviews three pregnant patients: two of them requiring pacemaker implantation and one underwent diagnostic cardiac catheterization without fluoroscopy. METHODS: The cases were performed using EnSite system (St. Jude Medical Inc., MN, USA) guidance. The necessary cardiac structure geometries were reconstructed with a deflectable quadripolar electrophysiology catheter without fluoroscopy. In two cases, pacemaker leads were connected to the EnSite system for navigation and fixation of leads. In the third case, long sheaths and electrophysiology catheters were used to access the right ventricle and pulmonary artery. Transthoracic echocardiography was also used in all three cases. RESULTS: A 31-year-old woman at 8-week pregnancy was admitted with ventricular septal defect and significant pulmonary hypertension. The patient underwent catheterization to assess for the risk of continuation of pregnancy. There was partial reactivity, it was decided not to terminate the pregnancy, and an uneventful delivery was succeeded at 35 weeks of gestation without complications. The rest of the two pregnant patients were a 28-year-old pregnant woman at 14 weeks of gestation and a 40-year-old woman at 12-week gestation. Both of them presented with symptomatic complete AV block. A single-chamber pacemaker was implanted in the first one, and a dual-chamber pacemaker was implanted in the latter. Pregnancy continued in both without complications. CONCLUSION: Successful cardiac catheterization and pacemaker implantation can be performed safely in selected pregnant patients using an electroanatomic mapping system guidance without fluoroscopy.

Efficacy of very low-dose prostaglandin E1 in duct-dependent congenital heart disease.

AIM: The present study aims to define the lowest effective prostaglandin E1 dose in patients with inadequacy of pulmonary blood flow and/or intracardiac blood mixing and those with inadequate systemic blood flow. METHODS: Patients with inadequacy of both pulmonary blood flow and/or blood mixing (Group 1) and those with inadequate systemic blood flow (Group 2) were retrospectively evaluated in two separate groups with regard to the prostaglandin E1 starting dose given in the referring facility, the lowest and the highest dose administered in our centre, treatment duration, adverse effects, and administered treatment. RESULTS: No difference between the groups could be detected with respect to sex or birth weight (p=0.95 and 0.42, respectively). Group 1 and Group 2 were statistically similar in aspect of prostaglandin treatment duration (9.73±0.81 days versus 11.6±1.05 days, p=0.064). When compared with Group 2, the initial, maintenance and lowest efficient doses of prostaglandin E1 treatment were significantly lower and the titrated dose of prostaglandin E1 was significantly higher in Group 1 (p=0.001 for each). CONCLUSION: Our findings indicate that the infusion of prostaglandin at a very low dose (0.003-0.005 mcg/kg/minute) is sufficient to maintain the patency of the ductus arteriosus. A higher dose of prostaglandin E1 may be necessary in patients with inadequate systemic blood flow.

Percutaneous closure of large VSD using a home-made fenestrated atrial septal occluder in 18-year-old with pulmonary hypertension.

BACKGROUND: Hemodynamically significant muscular ventricular septal defects in children after the infantile period are a rare occurrence and ideal for transcatheter closure. In cases of severe concomitant pulmonary hypertension, it may be necessary to fenestrate the device. In this report, we present an 18-year old patient with a large mid-trabecular ventricular septal defect and severe pulmonary hypertension that underwent percutaneous closure of the defect with a home-made fenestrated atrial septal occluder. CASE PRESENTATION: An 18-year-old female patient referred to us with complaints of dyspnea (NYHA score of 2-3). Physical examination revealed an apical rumble and a harsh second heart sound. Echocardiographic examination revealed a large mid-trabecular ventricular septal defect with bidirectional shunt and the widest diameter measuring 22 mm on 2D echocardiography. Left and right heart cavities were enlarged. Before and after the vasoreactivity test performed during cardiac catheterization, average aortic pressure was 65 → 86 mmHg, average pulmonary artery pressure: 58 → 73 mmHg, Qp/Qs: 1.6 → 3.2, PVR: 4.6 → 4.3 Wood/U/m2 and PVR/SVR: 0.5 → 0.2. On left-ventricular angiocardiogram, the largest end-diastolic defect diameter was 21 mm. The closure procedure was performed with transthoracic echocardiographic guidance, using a 24 mm Cera septal occluder and a 14 F sheath dilator to make a 4.5-5 mm opening. Measured immediately after the procedure and during cardiac catheterization one month later, average aortic pressure was 75 → 75 mmHg, average pulmonary artery pressure: 66 → 30 mmHg, Qp/Qs 1.5 → 1.4, PVR: 4.4 → 2.9 Wood/U/m2 and PVR/SVR: 0.4 → 0.2. Transthoracic echocardiographic examination performed 24 hours after the procedure showed a max 35-40 mmHg gradient between the left and right ventricles through the fenestration. After the procedure, we observed sporadic early ventricular systoles and a nodal rhythm disorder that started after approximately 12 hours and spontaneously reverted to normal 9 days later. CONCLUSION: In patients with large ventricular septal defects, large atrial septal occluders may be used. In cases with risk of pulmonary vascular disease, a safer option would be to close the defect using a manually fenestrated device.