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.

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.

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.

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.

Randomized comparison between mild and moderate hypothermic cardiopulmonary bypass for neonatal arterial switch operation.

OBJECTIVES: To compare neonates receiving arterial switch operation (ASO) either with mild or moderate hypothermic cardiopulmonary bypass. METHODS: Forty neonates undergoing ASO were randomized to receive either mild (Mi > 32 °C, n = 20) or moderate (Mo > 26 °C, n = 20) hypothermic cardiopulmonary bypass (CPB) between April 2007 and June 2010. All patients were diagnosed with simple transposition of the great arteries. Mean age (Mi: 8.32 ± 4.5 days, Mo: 7.54 ± 5.0 days, P = 0.21) and body weight were similar in both groups (Mi: 3.64 ± 0.91 kg, Mo: 3.73 ± 0.84 kg, P = 0.14). Follow-up was 3.1 ± 2.5 years for all patients. RESULTS: Lowest perioperative rectal temperature was 33.5 ± 1.4 °C (Mi) versus 28.2 ± 2.1 °C (Mo) (P < 0.001). All patients safely weaned from CPB required lower doses of dopamine (Mi: 5.1 ± 2.4 µg/kg min, Mo: 6.5 ± 2.1 µ/kg min, P = 0.04), dobutamine (Mi: 7.2 ± 2.5 µg/kg min, Mo: 8.6 ± 2.4 µ/kg min, P = 0.04) and adrenalin (Mi: 0.02 ± 0.02 µg/kg min, Mo: 0.05 ± 0.03 µ/kg min, P = 0.03) in mild hypothermia group. Intraoperative blood transfusion (Mi: 190 ± 58 ml, Mo: 230 ± 24 ml, P = 0.03) and postoperative lactate levels (Mi: 2.7 ± 0.9 mmol/l, Mo: 3.1 ± 2.2 mmol/l, P = 0.02) were lower under mild hypothermia. Secondary chest closure was performed in 30% (Mi) versus 35% (Mo) (P = 0.65). Duration of inotropic support (Mi: 7 (4-11) days, Mo: 11 (7-15) days, P = 0.03), time to extubation (Mi: 108 (88-128) h, Mo: 128 (102-210) h, P = 0.04), lengths of intensive care unit (ICU) stay (Mi: 9 (5-14) days, Mo: 12 (10-18) days, P = 0.04) and hospital stay (Mi: 19 (10-29) days, Mo: 23 (15-37) days, P = 0.04) were significantly shorter under mild hypothermia. Two-year freedom from reoperation was 100% for both the groups. CONCLUSIONS: The ASO under mild hypothermia seemed to be beneficial for pulmonary recovery, need for inotropic support and length of ICU and hospital stay. No worse early- or intermediate-term effects of mild hypothermia were found.

A different therapeutic strategy for severe tetralogy of Fallot with origin of the left pulmonary artery from the ascending aorta: stenting of the right ventricular outflow tract before complete repair.

The origin of pulmonary artery branches (particularly the left pulmonary artery) from the ascending aorta is a rare condition. We detected prominent hypoplasia of the main and right pulmonary arteries in a 3.5-month-old 3.7 kg female infant who had tetralogy of Fallot with origin of the left pulmonary artery in the ascending aorta. In order to ensure the development of the right pulmonary artery, a stent was put in that extended from the right ventricular outflow tract to the right pulmonary artery. During follow-up, after the patient's right pulmonary artery had developed sufficiently, a complete repair surgery was done. It is common practice for patients with abnormal origin of the left pulmonary artery to perform the complete repair using the direct reimplantation technique. However, we think that another possibility is to implant the stent in patients with hypoplastic pulmonary artery and branches in the early stages, wait for a short period of time and perform the complete repair surgery before permanent pulmonary hypertension develops.

Cheatham-Platinum stent for native and recurrent aortic coarctation in children and adults: immediate and early follow-up results.

OBJECTIVE: To present our institutional experience of endovascular Cheatham-Platinum stent implantation in children and adults with native and recurrent aortic coarctation. METHODS: Between August 2007 and November 2009, 45 patients had aortic coarctation treated with 47 stents implantation. We preferred primarily stent implantation in adult patient with coarctation, in children more than five years-old it is preferred in cases of aneurysm, subatretic or blind coarctation and coarctation with patent ductus arteriosus or in restenosis. Files of stent-implanted patients were retrospectively analyzed in terms of patients' demographic features, echocardiographic and angiographic findings both before and after procedure. Patients were grouped as Group 1: native coarctation and Group 2: recoarctation developed after either surgery or balloon angioplasty. Findings of the cases' were compared using paired and unpaired Student's t, Mann-Whitney U and Chi-square tests. RESULTS: Sixteen covered and 31 bare totally 47 balloon expandable stents were implanted in 45 patients. The mean follow up duration was 12.1 ± 7.1, median 11 months (2-29 months). There was no procedure related death. In two patients two stents were implanted in tandem. While the coarctation of the aorta was native in 26 patients (functionally interrupted aortic arch in one), recoarctation was detected in 7 patients after surgery, in 8 patients after balloon angioplasty, in 4 patients both after surgery and balloon angioplasty. One patient had functionally interrupted aortic arch perforated with guide wire and then covered stent implanted. The mean age 12.2 ± 5.9 years (5-33 years) and mean body mass index was 21 ± 3.7 kg/m2 (14.8-31 kg/m2). Considering all cases, a statistically significant decrease in both the invasive and echocardiographic gradients (p<0.001 for both) and statistically significant increase in lesion diameter (p<0.001) were detected. The decrease in invasive and echocardiographic gradients and increase in lesion diameter is statistically significant in each group also (p<0.001, <0.001 and <0.001 for both groups, respectively). Before the procedure, the invasive gradient was significantly higher and the lesion diameter was significantly lower in group I than in group II (p=0.002 and p=0.005, respectively). Also the percentage of decrease in gradient and increase in diameter was statistically higher in group 1 than in group 2 (p=0.04 and p=0.04). CONCLUSION: Our early and short- term follow-up results indicate that stent implantation is safe and very effective in reducing coarctation gradient and increasing lesion diameter both in native coarctation and recoarctation.

Aortico-left ventricular tunnel experience on three different ages.

Aortico-left ventricular tunnel is extremely rare congenital paravalvar communication between the aorta and the left ventricle. Usually it is treated surgically. In addition to the surgery the tunnel can be closed by percutaneous transcatheter intervention in appropriate patients. We present in this paper 7 months, 10 years, and 1,5 months old three male cases with aortico-left ventricular tunnel that were surgically treated and followed up within 7 years in our clinic.

Anomalous origin of one pulmonary artery branch from the ascending aorta: experience of our center.

OBJECTIVES: Anomalous origin of one pulmonary artery branch from the aorta in the presence of separate aortic and pulmonary valves is a rare but important entity necessitating early diagnosis and surgery to prevent irreversible vascular pulmonary disease. We evaluated our experience with seven infants having this anomaly. STUDY DESIGN: Between December 2003 and 2009, a total of seven infants (2 girls, 5 boys, age range 4 days to 84 days) were diagnosed as having anomalous origin of one pulmonary artery branch from the aorta. Clinical records were reviewed for clinical features, operative procedures, and postoperative follow-up. RESULTS: Common symptoms were dyspnea, tachypnea, and poor feeding. All the cases were diagnosed by echocardiography. The right pulmonary artery in six cases and the left pulmonary artery in one case originated from the ascending aorta. In addition, three patients had patent ductus arteriosus (PDA), five patients had patent foramen ovale, and one patient had interruption of the aortic arch and aortopulmonary window. All patients underwent surgical re-implantation of the anomalous pulmonary artery branch to the pulmonary trunk. Associated surgical procedures included PDA ligation in three patients, and total repair of interrupted aortic arch and aortopulmonary window in one patient. There were no in-hospital deaths. Two patients had prolonged intubation (10 and 16 days). All patients were discharged in good clinical condition. During a follow-up period ranging from two months to six years, only one patient developed stenosis at the site of anastomosis. CONCLUSION: Prompt diagnosis at infancy, improved surgical technique, and good patient care decrease mortality and morbidity associated with anomalous origin of the pulmonary artery from the aorta.

Association of cardiac valve calcification and inflammation in patients on hemodialysis.

BACKGROUND/AIMS: This study investigates the possible relationship between inflammation and cardiac valve calcification (VC) in patients on hemodialysis (HD), and identifies risk factors for VC in this patient group. METHODS: Seventy-nine patients on HD (mean age, 52.2 +/- 13.6 years; mean HD duration, 46.8 +/- 34.3 months) were assessed echocardiographically for the presence of VC. Systolic and diastolic blood pressure (BP) values were determined. The blood parameters studied in each case were hemoglobin, blood urea nitrogen, creatinine, calcium, phosphate, calcium-phosphorous (Ca x P) product, albumin, alkaline phosphatase, intact parathyroid hormone, total cholesterol, high-density lipoprotein, low-density lipoprotein, triglyceride, lipoprotein(a), fibrinogen, and C-reactive protein (CRP). The number of patients receiving vitamin D and calcium-containing phosphate binder was determined from records, and presence of diabetes mellitus was noted. RESULTS: Cardiac VC was detected in 36 patients (46%). Five of these patients (6%) had mitral VC, 11 (14%) had aortic VC, and 20 (25%) had calcification of both valves. The patients with VC were significantly older than those without VC (60 +/- 11 vs. 43 +/- 15 years, respectively; P=.001). Compared with the group without VC, the group with calcification had significantly higher systolic (145.1 +/- 14.7 vs. 124.3 +/- 20.7 mmHg, P=.001) and diastolic BP (91.3 +/- 10.3 vs. 75.09 +/- 14.9 mmHg, P=.001); significantly higher phosphate (5.1 +/- 1.4 vs. 4.5 +/- 1.4 mg/dL, P=0.04), CaxP product (48.6 +/- 16.2 vs. 39.8 +/- 11.8, P=.01), lipoprotein(a) [28 (15, 45) vs. 16 (5,42) mg/dL, P=.04], fibrinogen (4.2 +/- 1.2 vs. 3.5-0.9, P=.005), and CRP levels [9 (4, 19) vs. 5 (3, 11) mg/L, P=.05]; and significantly longer HD duration [49 (27, 99) vs. 26 (17, 52) month, P=.01). Apart from age, duration of HD, systolic and diastolic BP, and Ca x P product, VC was associated with CRP (odds ratio, 1.151; P=.007) and fibrinogen (odds ratio, 1.119; P=.005). CONCLUSIONS: The results confirm well-known risk factors for cardiac VC in HD patients, such as older age, longer HD duration, elevated BP, and high Ca x P product. In addition, they suggest that elevated levels of CRP and fibrinogen were associated with VC in the HD population.