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.

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.

Development of Myocardial Infarction in a 12-Year-Old Female after the Use of Inhaled Salbutamol.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) accounts for 0.023% of all cases reported in pediatric patients. According to literature, only a handful of ALCAPA patients are able to reach adulthood. Clinical manifestations of ALCAPA range from fatigue during exercise to sudden death in adulthood. Herein, we described a 12-year-old symptomatic patient with ALCAPA who had severe chest pain after using salbutamol treatment for presumed asthma. ALCAPA is one of the curable versions of myocardial ischemia and infarction in childhood. Due to clinical findings in conjunction with electrocardiogram and echocardiography, a computed tomography scan with coronary angiography was performed and the diagnosis of ALCAPA was confirmed. We presented this case because ALCAPA-related myocardial ischemia and infarction in children are rare with only sporadic cases reported. This case illustrated the need for close monitoring and surgery as the best treatment for ALCAPA associated with myocardial infarction.

Short-term efficacy of ORS formulation and propranolol regimen in children with POTS.

BACKGROUND: To evaluate the short-term effectiveness of reduced-osmolarity oral rehydration salt formulation (ORS) and propranolol in children diagnosed with postural orthostatic tachycardia syndrome (POTS) in head-up tilt testing (HUTT). METHODS: Children were admitted with symptoms of orthostatic intolerance (OI) occurring in a standing position and disappearing in the supine position. Patients with heart rate increments of ≥40bpm and symptoms of OI constituted the pediatric POTS group in HUTT. A total of 70 pediatric patients with POTS were included in the study. POTS patients were divided into two groups based on whether they were prescribed reduced-osmolarity ORS and propranolol or not. The study group comprised patients on a regimen of reduced-osmolarity ORS and propranolol (n=34), while the control group comprised patients who were not prescribed any medication (n=36). The frequency of symptoms and standardized symptom scores were analyzed before and after 3 months of treatment in both groups. RESULTS: The post-treatment frequency of syncopal attacks was significantly reduced in both groups (P<0.01 for both groups), but the post-treatment standardized symptom scores were significantly reduced in the pediatric study group compared with the control group (P<0.01). CONCLUSION: The frequency of syncopal attacks was significantly reduced and the symptom scores for OI were improved in the study group. The improvement in OI symptom scores was better in the treatment group than in the control group. The control group symptoms persisted and caused extreme difficulty in their daily activities. In view of its clinical efficacy, we strongly advocate the use of combined treatment of reduced-osmolarity ORS and low-dose propranolol in pediatric patients with POTS.

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.

A surgical technique for ascending aorta, aortic arch and descending aorta replacement without cross-clamp, circulatory arrest or hypothermia.

Treatment of the aneurysms comprising the aortic arch is challenging. Surgical reconstruction usually requires aortic cross-clamping, cardiac arrest, and even deep hypothermia for a bloodless field. In this report, we present our surgical technique providing normothermic ascending aorta, aortic arch, and proximal descending aorta replacement with selective cannulation and perfusion of the whole body.

Echocardiographic evaluation of cardiac functions in newborns of mildly preeclamptic pregnant women within postnatal 24-48 hours.

The aim of this study is to detect preeclampsia-related cardiac dysfunction within 24-48 hours of delivery in newborns born from preeclamptic mothers. Forty newborns from mildly preeclamptic mothers formed the study group and the control group was formed by 40 healthy newborns. Cardiac function for the groups were evaluated using conventional echocardiography and myocardial performance index (MPI) within the first 24-48 hours of their lifetime and the results of both groups were compared. A significant difference between the groups was observed especially in the PW Doppler MPI measurements (the left ventricle MPI 0.37 ± 0.09 and 0.26 ± 0.11, p < .001; the right ventricle MPI 0.29 ± 0.08 and 0.26 ± 0.07, p < .035) for the control group and the study group. Elongation in the left and right ventricle MPI was detected to be more significant in terms of comparing systolic and diastolic functions to determine preeclampsia-related cardiac injury in newborns from preeclamptic mothers within the first 24-48 hours of their lifetime. Impact statement Today, the methods which may detect cardiac injury earlier than conventional echocardiographic methods are used for evaluating cardiac functions. Among them, myocardial performance index (MPI) measurement with PW Doppler is the most common ones. While studies are available in the literature evaluating foetal cardiac functions with MPI in foetuses of preeclamptic women, studies evaluating cardiac functions with MPI index within the first 24-48 hours in postnatal period are not available. This is the first study to detect cardiac injury by measuring cardiac functions of the newborns of preeclamptic babies using conventional echocardiography (EF, SF, mitral and tricuspid E/A) and myocardial performance index within the first 24-48 hours of life and compare these values with those of a control group composed of healthy newborns with similar demographic characteristics. According to the results of the study, elongation in right and left ventricle MPI was detected to be more significant compared to systolic and diastolic functions for determining preeclampsia-related cardiac injury in newborns of preeclamptic mothers within 24-48 hours of delivery. Ventricle functions of the newborns of preeclamptic mothers should also be evaluated with MPI measurement besides conventional echocardiographic measurements.

Global Deformation Parameters Response to Exercise in Adolescents with Repaired Tetralogy of Fallot.

Exercise stress echocardiography is rarely used in the follow-up of children with congenital heart disease following surgical repair. There are no data on global longitudinal strain (GLS) and global circumferential strain (GCS) during exercise in adolescents with repaired tetralogy of Fallot (TOF). The purpose of this prospective study was to investigate GLS and GCS using speckle tracking two-dimensional echocardiography (2D-STE) at rest and during exercise in adolescents with repaired TOF. Twenty patients with repaired TOF and 20 controls were included. The subjects exercised on a semi-supine cycle ergometer using a two-staged 3-min step protocol of 20-40 W at 60-75 rpm. Exercise images with 2D-STE measurements were available for all 40 subjects. In the study group, the GLS values changed significantly between the baseline and third and sixth minute of exercise (-18.10 ± 2.20, -16.23 ± 4.47, -14.22 ± 2.63, respectively, p < 001). GCS did not vary significantly from baseline to end of the exercise testing (-17.49 ± 5.14, -17.15 ± 9.20, -15.66 ± 3.59, respectively, p > 0.05). There was a statistically significant difference between the Stage I longitudinal strain values of the study and control groups (-14.26 ± 9.13 and -20.22 ± 2.88, respectively, p = 0.02) but no significant difference between the Stage II values of these groups (-14.22 ± 2.63 and -15.11 ± 2.45, respectively, p > 0.05). The results revealed significant changes in GLS during exercise in patients with repaired TOF. Data gathered from deformation analysis of 2D-STE during exercise testing can enhance the ability to detect subtle ventricular dysfunction in the follow-up of children with repaired TOF.

Prolonged Tp-e Interval and Tp-e/QT Ratio in Children with Mitral Valve Prolapse.

Although it is considered to be a benign condition, previous studies have shown that a subset of patients with mitral valve prolapse (MVP) may be at risk of ventricular arrhythmia and sudden cardiac death (SCD). Previous studies have suggested that the interval between the peak and the end of the T wave (Tp-e) can be used as a marker for the transmural dispersion of repolarization. Increased Tp-e interval and Tp-e/QT ratio are associated with ventricular arrhythmias and SCD. The aim of this study was to assess alterations in ventricular repolarization by using the Tp-e interval and Tp-e/QT ratio in children with MVP and to investigate their relationships with the degree of valvular regurgitation. This study prospectively investigated 110 children with MVP and 107 age- and sex-matched healthy control subjects. Tp-e interval, Tp-e/QT ratio, and QT and QTc dispersions were measured from a 12-lead electrocardiogram and compared between groups. QT and QTc dispersions, Tp-e interval, and Tp-e/QTc ratio were found to be significantly higher in patients with MVP. A positive correlation was found between Tp-e/QTc ratio and increase in the degree of mitral regurgitation (MR) (p < 0.05; r = 0.2). However, the degree of MR was not associated with QT, QTc, or Tp-e intervals; QT, QTc, or Tp-e dispersions; or Tp-e/QT ratio (all p values >0.05). Individuals with MVP may be more prone to ventricular arrhythmias due to prolonged QTd, QTcd, and Tp-e interval and increased Tp-e/QT and Tp-e/QTc ratios. Therefore, due to their longer life expectancy, children with MVP should be followed up on regarding life-threatening arrhythmias.