The Lifesaving Impact of Transcatheter Interventions in the Early Post-Fontan Palliation Period.

Despite advancements in postoperative outcomes after Fontan surgery, there remains a risk of suboptimal outcomes and significant morbidity in the early postoperative period. Anatomical obstructions in the Fontan pathway can lead to prolonged pleural effusion or ascites, cyanosis, and low cardiac output syndrome (LCOS). Transcatheter interventions offer an alternative to early re-surgery for treating these complications. Over a 13-year period, early catheter angiography, performed within 30 days post-index procedure, was administered to 41 patients, identifying anatomical issues that necessitated re-intervention in 39 cases. This led to transcatheter interventions in 37 (10.4%) of the 344 Fontan surgery patients. The median age was 4.8 years (IQR: 4-9.4), and the median weight was 16.5 kg (IQR: 15-25.2), with females comprising 51.4% (19/37) of this group. The primary indications for the procedures were persistent pleural effusion or ascites in 27 patients (66%), LCOS in 8 patients (20%), and cyanosis in 6 patients (14%). Among the 37 undergoing transcatheter intervention, 30 were treated solely with this method and discharged, three died in ICU follow-up, and four required early re-surgery. No procedural mortality was observed. Our findings demonstrate that transcatheter interventions, including stent implantation, balloon angioplasty, and fenestration dilation, are safe and effective in the early post-Fontan period. Therefore, they should be considered an integral part of the management strategy for this patient group.

Utility of Balloon Occlusion Testing in Determining Fontan Suitability Among Patients with Elevated Pulmonary Artery Pressure and Additional Antegrade Pulmonary Blood Flow.

In individuals with a single ventricle undergoing evaluation before Fontan surgery, the presence of excessive pulmonary blood flow can contribute to increased pulmonary artery pressure, notably in those who had a Glenn procedure with antegrade pulmonary flow. 28 patients who had previously undergone Glenn anastomosis with antegrade pulmonary blood flow (APBF) and with elevated mean pulmonary artery (mPAP) pressure > 15 mmHg in diagnostic catheter angiography were included in the study. After addressing other anatomical factors that could affect pulmonary artery pressure, APBF was occluded with semi-compliant, Wedge or sizing balloons to measure pulmonary artery pressure accurately. 23 patients (82% of the cohort) advanced to Fontan completion. In this group, median mPAP dropped from 20.5 (IQR 19-22) mmHg to 13 (IQR 12-14) mmHg post-test (p < 0.001). Median PVR post-test was 1.8 (IQR 1.5-2.1) WU m2. SpO2 levels decreased from a median of 88% (IQR 86%-93%) pre-test to 80% (IQR 75%-84%) post-test (p < 0.001). In five patients, elevated mPAP post-test occlusion on diagnostic catheter angiography led to non-completion of Fontan circulation. In this group, median pre- and post-test mPAP were 23 mmHg (IQR 21.5-23.5) and 19 mmHg (IQR 18.5-20), respectively (p = 0.038). Median post-test PVR was 3.8 (IQR 3.6-4.5) WU m2. SpO2 levels decreased from a median of 79% (IQR 76%-81%) pre-test to 77% (IQR 73.5%-80%) post-test (p = 0.039). Our study presents a specialized approach for patients initially deemed unsuitable for Fontan due to elevated pulmonary artery pressures. We were able to successfully complete the Fontan procedure in the majority of these high-risk cases after temporary balloon occlusion test.

Patients with developmental dysplasia of the hip shows higher digit ratio (2D:4D).

BACKGROUND: Developmental dysplasia of the hip (DDH) encompasses a range of hip disorders, from dysplasia to dislocation. One key factor is sex, with women being four to eight times more susceptible to DDH than men. However, there have been no studies investigating the second-to-fourth digit ratio (2D:4D), a biomarker of prenatal sex steroid exposure, in DDH patients. This study aimed to explore whether there is an association between the 2D:4D ratio and DDH. METHODS: Digital calipers were used to measure the second and fourth finger lengths of 250 patients treated for DDH and 200 patients in the healthy control group between January 2020 and January 2023. The Mann-Whitney U test was used for non-normally distributed variables and two-way analysis of variance to assess differences in measurements between DDH patients based on sex, side, and the interaction of sex and side. The intraclass correlation coefficient (ICC) was used to assess measurement repeatability. RESULTS: The median (min-max) age was 10.00 (6-16) years in the DDH group, 12.00 (6-16) years in the control group, and 10 (6-16) years for the whole cohort. There was no significant difference in terms of age between the patient and control groups (p = 0.083). When comparing the DDH group with controls the former showed higher right 2D:4D and Δ2D:4D and these differences were significant in both males and females. Among the patients, controlling for side of dysplasia did not affect right 2D:4D, left 2D:4D or Δ2D:4D. CONCLUSION: There was a significant association between higher 2D:4D ratios (both right and left hands) and DDH in both men and women. This indicates that the 2D:4D ratio, a marker of intrauterine estrogen/testosterone balance, is associated with DDH.

A Challenging Interventional Procedure: Transcatheter Closure of Tubular Patent Ductus Arteriosus in Patients with Pulmonary Hypertension.

Transcatheter closure of the tubular ducts remains the most challenging procedure, with higher complication rates than other types. This study evaluates the characteristics of transcatheter closure of tubular ducts with pulmonary hypertension. 73 patients with tubular ducts who underwent cardiac catheterization for transcatheter PDA closure were analyzed. The mean age and weight were 1.93 ± 2.68 years and 8.83 ± 6.14 kg, respectively. Transcatheter closure was attempted in 72 patients. Four cases (5.5%) were referred to surgery, while the procedure was completed in the remaining (94.5%). Amplatzer duct occluder (ADO) I or Cardiofix duct occluder (CDO) was the most commonly used devices. However, the use of Amplatzer vascular plug (AVP) II raised in recent years. The most common concern was aortic protrusion/stenosis in ADO I/CDO devices, but most regressed during follow-up. Iatrogenic coarctation of the aorta was observed in two with ADO I/CDO. Embolization of the device to the pulmonary artery was observed in three with CDO, AVP II, and AVP I. Significant left pulmonary artery stenosis requiring stenting developed in one after closure with an MVSDO device. Tubular ducts are highly associated with pulmonary arterial hypertension, and transcatheter closure of them is still challenging despite the developing device armamentarium. Although ADO I or similar devices are widely used, off-label devices are usually needed at increasing rates. The AVP II device is unsuitable for short tubular ducts but seems the best option for long ones.

How does tranexamic acid affect blood transfusion and bleeding amount in pelvis-acetabulum fractures treated with open reduction and internal fixation?

BACKGROUND: This study aimed to investigate intravenous tranexamic acid's (TA) effect on blood loss and transfusion ratios in pelvis-acetabulum fractures treated with open reduction and internal fixation. METHODS: Patients who underwent open reduction and internal fixation due to pelvis-acetabulum fractures between January 2017 and January 2019 constituted this study's target population. After applying inclusion and exclusion criteria, patients were divided into two groups: Those who were perioperatively given 15 mg/kg TA (i.e., Group 1) and those who were not (i.e., Group 2). Data including age, gender, mechanism of injury, fracture type, presence or absence of additional injuries, the time interval between admission and surgery, incision site, pre-operative and post-operative hemoglobin levels, intraoperative estimated blood loss (EBL), number of blood units transfused, and complications were recorded. Two groups were compared regarding these parameters. RESULTS: The study cohort included 58 patients. There were 30 patients in Group 1 and 28 patients in Group 2. Our analysis revealed that the number of blood units transfused was significantly higher in Group 2 than Group 1 (p=0.016). However, there was no significant difference between the two groups regarding intraoperative EBL, pre-operative and post-operative hemoglobin levels, and the time interval between admission and surgery. CONCLUSION: Administration of intravenous TA reduces blood transfusion requirement in patients with pelvis-acetabulum fractures treated with open reduction and internal fixation. This approach can prevent potential blood transfusion-related complications.

Collagen/Nano-hydroxyapatite Composite Scaffold Application with Exchange Reamed Nailing Accelerates Bone Union and Improves Quality of Life in Atrophic Femoral Shaft Nonunions: A Retrospective Comparative Study.

BACKGROUND: Our aim in this study was to evaluate the effect of exchange intramedullary nailing in femoral shaft atrophic nonunions and the use of collagen/nano-hydroxyapatite composite scaffold applied in addition to the cancellous iliac crest autograft on the union, return to work, and quality of life. MATERIALS AND METHODS: Fifty-four patients with an atrophic nonunion in the isthmic region of the femoral shaft were included in the study. The patients were divided into two groups. Group A consisted of 24 patients who underwent collagen/nano-hydroxyapatite composite scaffold in addition to exchange intramedullary nailing and iliac autograft, while group B consisted of 30 patients without scaffold. Short Form-36 (SF-36) questionnaire scores, union rates, time to union, return to work were complications were compared. RESULTS: Mean age of patients was 47.5 ± 14.1. The mean follow-up period was 3.56 ± 1.88 years. There was no statistically significant difference between Group A and B in terms of age, gender, smoking and alcohol use, and trauma mechanism. Time to union and return to work were statistically significantly shorter in Group A than in Group B (p = 0.004, p = 0.001). All of the SF-36 survey scores at month six were better in Group A. In the first year, mental health and general health perception were still statistically better in group A (p = 0.009, p = 0.008). CONCLUSION: In the treatment of atrophic nonunions of the femoral shaft isthmic region, the use of collagen/nano-hydroxyapatite composite scaffolds together with exchange intramedullary nailing affects the union positively. This positive effect also brings about earlier return to work and better quality of life.

Fixation of open, unstable tuft fractures in toddlers and preschool-aged children using a 25-gauge hypodermic needle.

This study aimed to assess the treatment process of open, unstable tuft fractures occurring in toddlers and preschool-aged children treated using a 25-gauge hypodermic needle in the emergency department, and the cosmetic and functional results achieved by this treatment. This retrospective cohort study included a total of 72 patients. Children aged two to six years were included in the study. Van Beek classification was used for the evaluation of nail bed injuries. Cosmetic results were evaluated on the basis of Zook classification whereas functional results were evaluated according to the Buck-Gramko score simplified by Nietosvaara et al . The patient population consisted of 52 men and 20 women. The mean age was 4 ± 1.33 years. The operating time was 19.17 ± 3.66 minutes and the time to union was 46.68 ± 4.84 days. While excellent and good functional results were obtained in 65 (90.2%) of the patients, excellent and very good cosmetic results were obtained in 51 (70.8%) patients. Unless there is an accompanying extremity fracture, unstable pediatric tuft fractures can be successfully fixed with a hypodermic needle in the emergency department. Gentle and precise repair of the nail bed may provide cosmetically and functionally satisfactory results.

Comparison of different pulmonary valve reconstruction techniques during transannular repair of tetralogy of fallot.

BACKGROUND: Transannular patch, which results in pulmonary insufficiency (PI), is usually required during repair of tetralogy of fallot (TOF). In this study, we compared three types of pulmonary valve reconstruction techniques during transannular repair of TOF. METHODS: Between February 2014 and January 2018, 50 patients with TOF underwent primary repair with transannular patch. These patients were divided into three groups. In Group 1, (n = 15), a single gluteraldehyde-treated autologous pericardial patch (standard method) was reconstructed as monocusp. In Group 2, (n = 16) Nunn's bileaflet pulmonary valve reconstruction technique was used with pericardial patch. In Group 3, (n = 19), Nunn's bileaflet technique was performed with expanded polytetrafluoroethylene membrane. The outcomes of the patients and early and midterm competency of the pulmonary valves were analyzed. RESULTS: These techniques were significantly effective in early postoperative period. Freedom from moderate to severe PI were 73.3%; 100% and 89.4%, respectively. Mortality, duration of intensive care unit and hospital stay were similar between the groups. The mean follow-up period was 17.5 ± 13.0 (3-57) months. Freedom from moderate to severe PI decreased to 40%; 81.2% and 73.7%, respectively at the end of the follow-up period. Presence of moderate to severe PI was significantly higher in Group 1 (p = .018 between Groups 1 and 2, p = .048 between Groups 1 and 3). CONCLUSION: All three pulmonary valve reconstruction techniques provided competent pulmonary valves. Nunn's bileaflet technique had better outcome at midterm. It has a potential to delay right ventricular dysfunction at long-term.

The effectiveness of combined surgery and chemotherapy in primary hydatid cyst of thigh muscles, a rare localization and its management.

Hydatid cyst is a common infestation caused by helminths in humans and usually involves the liver and lungs. Primarily, cases of hydatid cysts in the thigh muscles are extremely rare and are generally in the form of case reports. It is an important disease due to its insidious course, its confusion with other soft tissue pathologies, delay in diagnosis and painstaking surgery. In our study, the largest case series in the literature, the clinical stages of the diagnosis, treatment and follow-up of 9 hydatid cysts that develop primarily in the thigh muscles were evaluated. The risk of recurrence in primary hydatid cyst of the thigh can be reduced with rapid diagnosis, careful preoperative planning, complete surgical resection by avoiding cyst rupture and concomitant anthelmintic chemotherapy.

Collateral dependent cerebral and coronary circulation in a newborn.

Association of interrupted aortic arch with aortic atresia as a variant of hypoplastic left heart syndrome (HLHS) is extremely rare and could not be compatible with life without a reliable source for cerebral and coronary blood flow. The patient with a large collateral artery between the descending aorta and the right subclavian artery is presented.

Early and midterm results of ductal stent implantation in neonates with ductal-dependent pulmonary circulation: a single-centre experience.

OBJECTIVE: We aimed to determine the early and midterm outcomes of ductal stenting in neonates with ductal-dependent pulmonary blood flow. METHODS: Between January, 2014 and July, 2018, 102 patients who underwent 115 cardiac catheterisation procedures for ductal stent implantation in our department were retrospectively reviewed. The age of the neonates ranged from 3 to 30 days (median: 11 days) and their weights ranged from 1.8 to 5.8 kg (mean, 2.8 ± 0.53 kg). Fifty-two patients had functional single ventricle and 50 had biventricular physiology. Thirty-one patients' weights were <2,500 g (30.3%). The patent ductus arteriosus was vertical in 60 patients (58.8%). The mean ductal length was 12.4 ± 4.1 mm (range, 7.8-23 mm), and the mean narrowest ductal diameter was 2.1 ± 0.7 mm (range, 1.2-3.4 mm). RESULTS: The technical success rate was 85.2%. Procedure-related mortality occurred in three patients (2.9%). After the procedure, the aortic oxygen saturation increased from a mean of 73.1 ± 6.2% to a mean of 90.4 ± 4.3% (p < 0.001), and the ductus diameter increased from a mean of 2.1 ± 0.7 mm to a mean of 4.2 ± 0.9 mm (p < 0.001). Either transcatheter or surgical reinterventions were required in 35 patients (34.3%) during the follow-up period after a median of 101 days (2-356 days). Thirty-three patients (32.3%) were bridged to surgical repair after a median of 288 days (163-650 days). The median duration of palliation with ductal stents was 210 days (range, 2-525 days). CONCLUSION: Ductus arteriosus stenting may be a reasonable and effective alternative to surgery for the initial palliation procedure in neonates with ductus-dependent pulmonary flow.

How do vertebroplasty and kyphoplasty affect the quality of life of patients with multiple myeloma spinal metastasis?

BACKGROUND: Metastatic spinal lesions are diseases that impair the quality of life and require early diagnosis and treatment. The count of spinal metastasis patients rises day by day. Increased life expectancy has increased the incidence of cancer, making metastases more observable. The aim of our study was to investigate how the quality of life is affected in the patient group with vertebral fracture due to spinal metastases of multiple myeloma and undergoing vertebroplasty and kyphoplasty. MATERIALS AND METHODS: Forty-two patients with fracture due to spinal metastasis and adequate follow-up between the years of 2011 and 2018 were included in the study. Patients were listed according to their primary malignancy and metastases. The effect of vertebroplasty and kyphoplasty was investigated by evaluating preoperative and postoperative VAS and Oswestry Disability Index scores. All patients' radiographic kyphosis angle, compression ratio and wedging index were calculated before operation and after operation. RESULTS: A total of 76 vertebrae were operated in 42 patients. Significant differences were found in the comparison of preoperative and postoperative quality of life according to VAS and Oswestry Disability Index scores in the patients undergoing vertebroplasty, kyphoplasty or both procedures after spinal metastases (VAS; p = 0.0001, ODI; p = 0.002/0.0001). There were statistically significant differences in preoperative local kyphosis angle, compression ratio and wedging index and post-op local kyphosis angle, compression ratio and wedging index (p = 0.001). CONCLUSION: Vertebroplasty and kyphoplasty, minimally invasive procedures performed after spinal metastases, improve the quality of life of the patients.

Staged Biventricular Repair After Hybrid Procedure in High-Risk Neonates and Infants.

BACKGROUND: Single-stage biventricular repair remains a challenging and difficult decision in high-risk newborns and early infants with the presence of left ventricular outflow tract obstruction (LVOTO) or borderline hypoplasia of the left ventricle (LV). METHODS: Six high-risk patients underwent the initial hybrid procedure (bilateral pulmonary banding + ductal stenting) for staged biventricular repair. Their median age was 17 days (range: 7-55 days). The diagnosis was interrupted aortic arch (IAA), ventricular septal defect (VSD), and LVOTO (n = 3); IAA and VSD (n = 1); and aortic annular hypoplasia, aortic arch hypoplasia, VSD, and LVOTO (n = 1). The last patient had borderline LV with large atrial septal defect (ASD) and aortic arch hypoplasia. The patient with borderline LV had also ASD closure with small fenestration. RESULTS: One patient died of sepsis after the hybrid procedure. Other patients underwent biventricular repair 8 to 13 months later. Three patients had conventional repair with conal septum resection. The other patient with IAA, in whom LVOTO was considered nonresectable, underwent Yasui operation. The last patient with borderline LV had enough development of left heart structures during follow-up and underwent aortic arch repair. One patient who had conal septum resection died after biventricular repair. One patient needed a tracheostomy; four patients were discharged uneventfully and their clinical conditions were good on postoperative year 1. CONCLUSION: Staged biventricular repair with the initial hybrid procedure may be a feasible and safe alternative in high-risk neonates and early infants. Hybrid intervention may provide the development of cardiac structures in time and a better evaluation for the possibility of biventricular repair in borderline patients.

Outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants.

BACKGROUND: In this study, we present the outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants. METHODS: In this study, a total of 97 pediatric patients who were operated due to hypoplastic left heart syndrome and its variants between March 2011 and October 2018 were retrospectively analyzed. Thirty-two of the patients (28 males, 4 females; median age 5 days; range, 1 to 25 days) underwent Norwood Stage I operation (Group N), while the remaining 65 patients (44 males, 21 females; median age 6 days; range, 1 to 55 days) underwent a hybrid procedure (Group H). Both treatment strategies were compared. RESULTS: The median body weight in Group H was significantly lower and the number of patients with a low birth weight (<2,500 g) was significantly higher than Group N (p=0.002 and 0.004, respectively). The postoperative early mortality rate was similar between the groups. Univariate and multivariate analyses revealed that the need for preoperative mechanical ventilation was a significant factor for mortality (p=0.004 and 0.003, respectively). Syndromic appearance was also a significant factor the multivariate analysis (p=0.03). There was a statistically significant difference between the groups in terms of the inter-stage mortality rates (p=0.0045). Second-stage procedure was performed in 32 patients. The early mortality rate after the Glenn operation was 7.6%. Six patients died after comprehensive Stage II operation. Five patients underwent biventricular repair and 8 patients had third-stage fenestrated extracardiac Fontan operation (Group N, n=7 and Group H, n=1). The Kaplan-Meier survival curve demonstrated that Group N had a higher survival rate at both one and five years than Group H, although the difference was not statistically significant (p=0.15). Subgroup analysis showed that the Norwood procedure with Sano modification had the highest survival rate with 40% at five years. CONCLUSION: Our study results show that patients undergoing the Norwood procedure have a more uneventful course of inter-stage period and Stage II and III, despite drawbacks early after Stage I procedure. Based on our experiences, we recommend performing the hybrid intervention in patients with a poor clinical condition and a body weight of <2,500 g.

Incidence and treatment of pulmonary vein stenosis after repair of total anomalous pulmonary venous connection.

BACKGROUND: In this study, we aimed to investigate the incidence and treatment of pulmonary vein stenosis after repair of total anomalous pulmonary venous connection. METHODS: Between December 2010 and December 2016, a total of 40 patients (25 males, 15 females; mean age 45±41 days; range, 2 days to 6 months) who underwent total anomalous pulmonary venous connection repair were retrospectively analyzed. RESULTS: Eighteen (45%) of the patients were supracardiac, seven (17.5%) were cardiac, eight (20%) were infracardiac, and seven (17.5%) were mixed-type anomaly. Twelve patients (30%) had obstruction of the pulmonary venous pathways. Hospital mortality was seen in three patients (7.5%). All of non-survivors received emergent surgery and the causes of death were sepsis and multiple organ failure. A total of 23 complications were seen in 20 patients (50%) mainly delayed sternal closure (n=11; 27.5%) and prolonged mechanical ventilation (n=6; 15%). The mean follow-up was 12.2±9.6 (range, 1 to 36) months in all patients, except one. Pulmonary vein stenosis developed in three patients (8.3%) after repair. All of them underwent sutureless repair. CONCLUSION: Total anomalous pulmonary venous connection can be repaired with low mortality and morbidity rates and favorable mid-term outcomes. Close follow-up is necessary due to the risk of development of pulmonary vein stenosis. Sutureless repair may provide relief in such cases.

Outcomes of truncus arteriosus repair with bovine jugular vein conduit.

BACKGROUND: In this study, we aimed to evaluate the outcomes of truncus arteriosus repair in patients undergoing Rastelli type truncus arteriosus. METHODS: A total of 13 patients (7 males, 6 females; median age 37 days; range, 16 to 60 days) underwent repair of truncus arteriosus using Contegra conduits between January 2011 and March 2 017. P reoperative d iagnosis w as t runcus a rteriosus type 1 (Edwards-Collett) in eight, type 2 in three, type 3 in one, and type 4 in one patient. Contegra conduits used for operations were 14 mm (n=5), 12 mm (n=7), and 16 mm (n=1). RESULTS: Early death was seen in two patients (15.4%). The median intensive care and hospital stays were 10 (range, 6 to 14) and 20 (range, 14 to 41) days, respectively. The median follow-up was 36 (range, 2 to 66) months. In four patients (31%), the conduit sizes severely increased during follow-up and reached 23 mm in two patients and 20 mm in one patient, and 18 mm in the other patient. Three patients had moderate distal conduit stenosis. Moderate pulmonary insufficiency was detected in four patients and severe pulmonary insufficiency in one patient. Two patients had moderate truncal valve insufficiency and one patient had moderate residual ventricular septal defect. None of the patients needed reoperation. CONCLUSION: Contegra conduit is a good alternative for repair of truncus arteriosus in infants; however close follow-up is necessary, as distal conduit stenosis and conduit dilatation may develop.

The impact of pulmonary valve-sparing techniques on postoperative early and midterm results in tetralogy of Fallot repair.

BACKGROUND: In this study, we analyzed the impact of pulmonary valve-sparing techniques on early and midterm postoperative results of tetralogy of Fallot repair. METHODS: A total of 64 patients diagnosed with tetralogy of Fallot, who underwent total correction operation by the same surgeon between November 2010 and September 2015 were included in this retrospective study. Mean age of the patients was 20.0±14.2 months (5.5-96 months). Forty patients (62.5%) were male. Thirty two of the patients (50%) were under one year of age. Pulmonary valve-sparing techniques were performed in 29 patients (Group 2), while transannular patch was applied in the remaining 35 patients (Group 1). Pericardial monocusp valve was constructed in 15 patients in Group 1. In Group 2, pulmonary valve-sparing techniques were transatrial repair in nine patients; transatrial-transpulmonary in eight, infundibular patch in eight, and infundibular-pulmonary patch in four patients. RESULTS: There was no early postoperative mortality in Group 2. Five patients (14.2%) in Group 1 died in the early postoperative period (p=0.058). Pulmonary monocusp insertion was performed in 2 (13.3%) of these patients (p=1). The causes of mortality were sudden cardiac arrest (n=2), multiorgan failure (n=1), low cardiac output (n=1), and neurological complications (n=1). Five patients in Group 1 required extracorporeal membrane oxygenation support (ECMO). Three of them were separated from ECMO and two of the patients that were separated were discharged uneventfully. Total postoperative morbidity rate was significantly higher in Group 1 (51.4% vs. 6.8%) (p=0.0001). Morbidity rate was significantly lower in patients with pulmonary monocusp insertion than patients in the same group without a monocusp (p=0.0176). Forty nine (83%) of the patients were followed up for a median of 6.5 (1-24) months. While free pulmonary regurgitation was detected in all non-monocusp patients in Group 1, pulmonary regurgitation was absent or mild in Group 2. Twelve (80%) of the patients in Group 1 who had monocusp insertion were followed up. Only two of these patients had free pulmonary regurgitation (16.6%). The rest of them had mild (n=6) or mildmoderate pulmonary regurgitation (n=4). CONCLUSION: Mortality and morbidity rates are lower when pulmonary valvesparing techniques are used in repair of tetralogy of Fallot. Monocusp pulmonary valve insertion may improve results in patients who require transannular patch repair. It is suggested that every effort should be made to achieve a competently working pulmonary valve during repair.

[A rare cause of cyanosis in childhood: Pulmonary arteriovenous malformation]. / Çocukluk çaginda siyanozun nadir bir sebebi: Pulmoner arteriyovenöz malformasyon.

Pulmonary arteriovenous malformation, which is defined as the presence of an ab-normal connection between the pulmonary artery and pulmonary vein, is rarely seen. Although it generally presents as a congenital condition, it may be accompanied by hereditary hemorrhagic telangiectasia. Clinical signs vary according to the amount of shunt in proportion to the number and size of the fistulae. Patients may present with cyanosis and respiratory trouble. If the disease remains untreated, it may result in cardiac failure and ineffective endocarditis, thereby leading to the rupture of the an-eurysmal fistula. Transcatheter embolization of abnormal vascular connection is the current treatment method in this disease. This article describes the case of an 8½-year-old child. He was presented with the symptom of getting tired quickly. Transcutaneous oxygen saturation of 75%, and pulmonary arteriovenous malfor-mation was detected in his examination. Successful transcatheter fistula embolization was performed.

[Closure of wide patent ductus arteriosus using a fenestrated muscular VSD occluder device in a pediatric patient with Down syndrome and pulmonary hypertension]. / Down sendromlu pulmoner hipertansiyonu olan bir çocuk hastada genis patent duktus arteriyozusun fenestrasyon uygulanmis muscular VSD occluder cihazi ile kapatilmasi.

Patients with wide patent ductus arteriosus and significant pulmonary hypertension not treated in time constitute a significant problem for cardiologists. For these patients, tests that could aid in decision-making for further planning include reversibility and balloon occlusion tests performed in the catheterization laboratory. Devices developed for the closure of ductus as well as different devices with off-label use may be employed in patients scheduled for transcatheter occlusion. When result of reversibility test is borderline positive, the use of fenestrated device may be applicable for selected patients. Presently described is case of a 10-year-old patient with Down syndrome who had a wide ductus and systemic pulmonary hypertension. Transcatheter closure procedure was performed with off-label use of a fenestrated muscular ventricular septal defect occluder device.

Extracorporeal cardiopulmonary resuscitation for refractory cardiac arrest in children after cardiac surgery.

OBJECTIVE: Extracorporeal membrane oxygenation (ECMO) is used to provide cardiorespiratory support during cardiopulmonary resuscitation (extracorporeal cardiopulmonary resuscitation; ECPR) unresponsive to conventional methods. In this study, the results of ECPR in a cardiac arrest setting after cardiac surgery in children were analyzed. METHODS: In this retrospective cohort study, between November 2010 and June 2014, 613 congenital heart operations were performed by the same surgical team. Medical records of all the patients who experienced cardiac arrest and ECPR in an early postoperative period (n=25; 4%) were analyzed. Their ages were between 2 days and 4.5 years (median: 3 months). Sixteen patients had palliative procedures. In 88% of the patients, cardiac arrest episodes occurred in the first 24 h after operation. Mechanical support was provided by cardiopulmonary bypass only (n=10) or by ECMO (n=15) during CPR. RESULTS: The CPR duration until commencing mechanical support was <20 min in two patients, 20-40 min in 11 patients, and >40 min in 12 patients. Eleven patients (44%) were weaned successfully from ECMO and survived more than 7 days. Five of them (20%) could be discharged. The CPR duration before ECMO (p=0.01) and biventricular physiology (p=0.022) was the key factor affecting survival. The follow-up duration was a mean of 15±11.9 months. While four patients were observed to have normal neuromotor development, one patient died of cerebral bleeding 6 months after discharge. CONCLUSION: Postoperative cardiac arrest usually occurs in the first 24 h after operation. ECPR provides a second chance for survival in children who have had cardiac arrest. Shortening the duration of CPR before ECMO might increase survival rates.