Posterior pericardial window: a simple and reproducible technique in order to prevent pericardial tamponade in paediatric cardiac surgery.

OBJECTIVE: Pericardial tamponade, which increases postoperative mortality and morbidity, is still not uncommon after paediatric cardiac surgery. We considered that posterior pericardiotomy may be a useful and safe technique in order to reduce the incidence of early and late pericardial tamponade. Herein, we present our experience with creation of posterior pericardial window following congenital cardiac surgical procedures. METHODS: This retrospective study evaluated 229 patients who underwent paediatric cardiac surgical procedures between June 2021 and January 2023. A posterior pericardial window was created in all of the patients. In neonates and infants, pericardial window was performed at a size of 2x2 cm, whereas a 3x3 cm connection was established in elder children and young adults. A curved chest tube was placed and positioned at the posterolateral pericardiophrenic sinus. An additional straight anterior mediastinal chest tube was also inserted in every patient. Transthoracic echocardiographic evaluations were performed daily to assess postoperative pericardial effusion. RESULTS: A total of 229 (135 male, 94 female) patients were operated. Mean age and body weight were 24.2 ± 26.7 months and 10.2 ± 6.7 kg, respectively. Eight (3.5%) of the patients were neonates where 109 (47.6%) were infants and 112 (48.9%) were in childhood. Fifty-two (22.7%) re-do operations were performed. Six (2.6%) patients underwent postoperative surgical re-exploration due to surgical site bleeding. Any early or late pericardial tamponade was not encountered in the study group. CONCLUSIONS: Posterior pericardial window is an effective and safe technique in order to prevent both the early and late pericardial tamponade after congenital cardiac surgery.

Revisiting the central aortopulmonary shunt procedure.

Background: In this study, we present our experience with the central aortopulmonary shunt technique with interposing a polytetrafluoroethylene graft between main pulmonary artery (end-to-end) and the ascending aorta (side-to-side) in a variety of cyanotic congenital heart defects. Methods: Between January 2019 and June 2022, a total of 10 patients (6 males, 4 females; mean age: 4.3±2.8 months; range, 5 days to 10 months) with hypoplastic central pulmonary arteries who underwent central aortopulmonary shunt procedure were retrospectively analyzed. Demographic characteristics, preoperative, operative, and postoperative data of the patients were recorded. The Nakata indices of the patients were also noted before the procedure, as well as before the second stage of palliation or definitive repair. Results: Four (40%) patients were operated as the first-step palliation for univentricular circulation. Six (60%) patients had well-developed ventricles and were palliated to be treated with total correction. The median follow-up after the procedure was 12 (range, 8 to 16) months. The mean systemic arterial saturation level at room air was 89.3±2.9% during follow-up. No mortality was observed in any patient. Conclusion: A central aortopulmonary shunt procedure provides a reliable antegrade blood flow with a relatively non-challenging surgical technique that offers sufficient growth for the hypoplastic and confluent central pulmonary arteries with a very low risk of shunt thrombosis and overflow.

A rare coexistence: Hammock mitral valve and aortopulmonary window.

Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral valve, also known as anomalous mitral arcade, is a rare congenital anomaly particularly in infants and children. Hammock mitral valve may not be suitable for repair regarding the advanced dysplastic mitral valve structure. Aortopulmonary window is an unusual cardiac anomaly which is defined as a communication between the main pulmonary artery and the ascending aorta. As a result of the excessive left-to-right shunt, early intervention and surgical closure deemed mandatory to avoid development of severe pulmonary hypertension and its consequences. All patients with an aortopulmonary window necessitates prompt repair immediately. In this brief report, mitral valve replacement with a mechanical valve and repair of aortopulmonary window with a Dacron patch were performed simultaneously in a 5-month-old patient with a hammock mitral valve and accompanying aortopulmonary window.

Plication for diaphragm paralysis after paediatric cardiac surgery: a single-centre experience.

OBJECTIVE: Diaphragm paralysis is a well-known complication following surgery for CHDs, which increases morbidity, mortality, and length of hospital stay as well as costs. Herein, we present our experience with diaphragm plication following paralysis of the phrenic nerve encountered after paediatric cardiac surgery. METHODS: This study retrospectively reviewed the medical records of 23 diaphragm plications in 20 patients who underwent paediatric cardiac surgery between January 2012 and January 2022. The patients were carefully selected based on aetiology and a combination of clinical manifestation and chest imaging characteristics including chest X-ray, ultrasonography, and fluoroscopy. RESULTS: Twenty-three successful plications were performed in 20 patients (15 males and 5 females) out of a total of 1938 operations performed in our centre. Mean age and body weight were 18.2 ± 17.1 months and 8.3 ± 3.7 kg, respectively. The period between the cardiac surgery and diaphragmatic plication was 18.7 ± 15.1 days. The highest incidence of diaphragm paralysis was encountered in systemic to pulmonary artery shunt patients with 7 out of 152 patients (4.6%). Any mortality was not encountered during a mean follow-up period of 4.3 ± 2.6 years. CONCLUSIONS: Early results of plication of the diaphragm following phrenic nerve palsy in symptomatic patients who underwent paediatric cardiac surgery are encouraging. Evaluation of the diaphragmatic function should be a routine part of post-operative echocardiography. Diaphragm paralysis may be a consequence of dissection, contusion, stretching, and thermal injury both in terms of hypothermia and hyperthermia.

Transcatheter management of life-threatening pulmonary arteriovenous fistula with extracorporeal membrane oxygenation support in an infant.

Pulmonary arteriovenous malformation is a rare disease leading to cyanosis, where there is a direct relation between the pulmonary artery and pulmonary vein without a capillary structure. Arteriovenous fistulae may be single or multiple. Clinical signs emerge depending on the size of the fistulae and amount of shunt. Due to the advancements in transcatheter devices and increased experience render enable the fistula embolisation procedure as an alternative to surgical treatment. Extracorporeal membrane oxygenation is used to support the patient haemodynamically and respirationally in cases of treatment-resistant, severe and revocable cardiac or pulmonary sufficiency. This paper presents an infant patient with pulmonary arteriovenous malformation, who had haemodynamic instability due to severe hypoxia and received successful transcatheter fistula embolisation via extracorporeal membrane oxygenation under emergency conditions.

Rare combination in an infant patient: trisomy 7p and tetralogy of fallot.

This case report presents an infant patient with the association of trisomy 7p and tetralogy of fallot (TEF). Patients diagnosed with trisomy 7p should certainly be scheduled for an echocardiographic exam and be scanned for any CHDs that may accompany it. The CHDs that most frequently accompany this syndrome include atrial septal defect, ventricular septal defect, and patent ductus arteriosis. Yet, it should be known that TEF may also be present, albeit rarely.

Rare combination in an infant patient: trisomy 7p and tetralogy of Fallot.

This case report presents an infant patient with the association of trisomy 7p and tetralogy of Fallot(ToF). Patients diagnosed with trisomy 7p should certainly be scheduled for an echocardiographic exam and be scanned for any CHD that may accompany it. The CHD that most frequently accompany this syndrome include atrial septal defect, ventricular septal defect, and patent ductus arteriosis. Yet, it should be known that ToF may also be present, albeit rarely.

Early outcomes of fenestrated intra-extracardiac Fontan procedure: Insights, experiences, and expectations.

BACKGROUND: Intraextracardiac Fontan procedure (FP) aimed to combine the advantages of lateral tunnel and extracardiac conduit modifications of the original technique. Herein, we present our early outcomes in patients with intraextracardiac fenestrated FP. METHODS: A retrospective analysis was performed to evaluate intraextracardiac fenestrated Fontan patients between 2014 and 2021. Seventeen patients were operated on with a mean age and body weight of 9.1 ± 5.5 years and 28.6 ± 14.6 kg. RESULTS: Sixteen patients (94%) were palliated as univentricular physiology with hypoplasia of one of the ventricles. One patient (6%) with well-developed two ventricles with double outlet right ventricle and complete atrioventricular septal defect had straddling of the chordae prohibiting a biventricular repair. All of the patients had cavopulmonary anastomosis before Fontan completion, except one case. Fenestration was performed in all cases. Postoperative mean pulmonary artery pressures and arterial oxygen saturation levels at follow-up were 10 ± 2.4 mmHg and 91.3 ± 2.7%, respectively. Mean duration of pleural drainage was 5.4 ± 2.3 days. All of the fenestrations are patent at a mean follow-up period of 4.8 ± 7.7 years, except one case. Any morbidity and mortality were not encountered. CONCLUSIONS: Early outcomes of intraextracardiac fenestrated FP are encouraging. This procedure may improve the results in a patient population who should be palliated as univentricular physiology, especially in cases with complex cardiac anatomy.

Does a prenatal diagnosis affect mortality and morbidity for neonatal arterial switch operation.

BACKGROUND: The effect of prenatal diagnosis on prognosis in patients with transposition of the great arteries is not clear. In this study, we compared the outcomes after arterial switch operation. METHODS: Outcome of 112 patients who had arterial switch operation in the neonatal period were analysed. The patients were divided into two groups: those who had prenatal diagnosis (Group 1; n = 34) and those who did not (Group 2; n = 78). The patients were also classified based on their diagnosis: simple transposition, transposition with ventricular septal defect and/or aortic arch hypoplasia, and Taussig-Bing anomaly. RESULTS: In Group 1, the C-section delivery rate was higher (82% vs. 44%; p = 0.004), and it was observed that patients in Group 1 were more often intubated upon admission to the neonatal ICU (38% vs. 9%; p = 0.005). No differences were found between the two groups in terms of operation time, cardiopulmonary bypass time, post-operative invasive respiratory support duration, or extracorporeal membrane oxygenation support. It was observed that those who had Taussig-Bing anomaly had a higher mortality. CONCLUSIONS: Timely treatment have a positive effect on neonatal mortality and morbidity. That's why all families with prenatal diagnosis of critical CHD should be recommended to have the delivery in a tertiary care hospital. Although it could not be demonstrated in this study, prenatal diagnosis has a potential to improve surgical results especially in countries or cities, which does not have enough resources for transfer and surgical units. Further efforts are needed to improve prenatal screening programmes.

Fontan procedure in patients with preoperative mean pulmonary artery pressure over 15 mmHg.

BACKGROUND: Several factors affect the long-term outcome of Fontan procedure, but a high pulmonary artery pressure is still one of the most important limitation for proceeding to a Fontan circulation. Herein, we present our experience in Fontan patients with high preoperative pulmonary artery pressures. METHODS: A retrospective analysis was performed to evaluate Fontan patients with a preoperative pulmonary artery pressure >15 mmHg between 2009 and 2020. Sixteen patients were operated on with a mean preoperative pulmonary artery pressure of 17.5 ± 2.1 mmHg. RESULTS: Mean age at the time of Fontan procedure was 7.8 ± 5.6 years. All the patients had stage 2 cavopulmonary anastomosis before Fontan completion, with a mean interstage period of 4 ± 2.6 years. Fontan completion was achieved with a polytetrafluorethylene tubular conduit, two of which were intra-extracardiac. Fenestration was performed in five (31%) cases. Postoperative pulmonary artery pressures and arterial oxygen saturation levels were 11.2 ± 2.8 and 97.8 ± 2 mmHg, respectively. Mean duration of pleural drainage was 3.9 ± 5.3 days. Any morbidity and mortality were not encountered during a mean follow-up period of 4.8 ± 7.7 years. CONCLUSIONS: The midterm results of stage 3 Fontan completion in patients with pulmonary artery >15 mmHg are encouraging. Not only the mean pulmonary artery pressure but also the pulmonary vascular resistance may be helpful in order to identify the high risk patients before Fontan completion.

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.

Use of Lifetech™ Konar-MF, a device for both perimembranous and muscular ventricular septal defects: A multicentre study.

BACKGROUND: The objective of this study was to evaluate the safety and efficacy of transcatheter closure of ventricular septal defects (VSD) using the LifeTech™ multifunctional occluder device (MF-Konar). METHODS: Clinical features and demographic characteristics and follow-up findings were evaluated retrospectively from three centers. RESULTS: MF-Konar was used in 98 patients. The median age and weight of the patients were 3.8 years (range 5.4 months-50 years) and 15.3 kg (range 5.5-80 kg), respectively. The mean fluoroscopy time was 13.7 ± 8.2 min (range 3.4-42.6 min). Procedural success was obtained for 96 out of 98 patients (98%). In 54 out of 98 patients, closure was performed via the antegrade route. Major complications occurred in four patients (embolization in two, complete heart block in one, and device dislocation needing surgical treatment in one). All of the complications were treated successfully, and there was no mortality. Mild residual flow in eight patients (8%), new onset tricuspid valve insufficiency in one (moderate), and new onset aortic valve insufficiency in one (mild) were observed during a mean follow-up duration of 224 ± 149 (10-515) days. Minor rhythm disturbances were observed in eight patients. CONCLUSIONS: Transcatheter closure of VSDs in selected patients using the LifeTech MF-Konar device seems effective. Its advantages are softer design, use of both an antegrade and retrograde approach, and an advanced smaller delivery system. Increasing the number of usage and the experience will provide more accurate data and low complication rates.

[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.

Spontaneous Remission of Congenital Complete Atrioventricular Block in Anti-Ro/La Antibody-Negative Monozygotic Twins: Case Report.

BACKGROUND: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins. CASE REPORT: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin's physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute.The patient was confirmed to have congenital complete atrioventricular block. CONCLUSION: Despite this case appears to be an isolated one, a discordant complete atrioventricular block regression without any autoimmune evidence should be included in the differential diagnosis of bradycardia in infants.

Rare combination and transcatheter treatment during single session in an infant: Patent ductus arteriosus and major aortopulmonary collateral artery concordance.

Major aortopulmonary collateral arteries are abnormal vascular structures that may be seen in cyanotic diseases that progress with reduced pulmonary flow. They occur rather rarely in the absence of cyanotic congenital heart disease. Presently described is the case of an infant who underwent patent ductus arteriosus (PDA) and major aortopulmonary collateral artery occlusion in a single session, without presence of cyanotic congenital heart disease. To the best of our knowledge, this is the first case of congenital aortopulmonary collateral artery to be reported in a symptomatic infant with PDA.

Major Intrahepatic Veno-Venous Fistula after Fontan Operation Treated by Transcatheter Implantation of Amplatzer Septal Occluder through Internal Jugular Vein.

Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay. Three weeks after the first intervention, a second procedure was performed to retrieve the first device and to close the fistula distally. Multiple attempts with different types of gooseneck snares and a bioptome catheter failed to retrieve the first device, so a telescopic method was used to re-screw it. Using a Mullins long sheath and delivery sheath, the delivery cable was manipulated to fit into the slot of the end screw, and the cable was rotated gently in a clockwise direction to re-screw the device. Then, another Amplatzer septal occluder was placed at the distal end of the fistula. In conclusion, distal transcatheter occlusion of intrahepatic veno-venous fistulas might lead to better clinical outcomes in selected patients. Amplatzer septal occluder device can be retrieve without any complication within three weeks.

A rare pathology: Levoatriocardinal vein.

OBJECTIVE: Levoatriocardinal vein (LACV) is a rare cardiac pathology that represents a connection between the pulmonary venous and cardinal systems. The aim of the present study was to discuss morphological and clinical characteristics, as well as diagnostic methods, of experience with LACV. METHODS: Records of 11 patients (4 male, 7 female; mean age 79±1.83 days; range 1-390 days) diagnosed with LACV between 2010 and 2014 were retrospectively reviewed. Presence of LACV was confirmed with echocardiography. The primary obstructive lesion associated with cardiac defects and the integrity of the interatrial septum was identified in each patient with left-sided obstruction. RESULTS: Mean weight was 4.4±0.4 kg (range: 2-8). Age at presentation was under 1 year in 82% of patients. Nine patients had left-sided obstruction, and 2 had normal intracardiac anatomy and pulmonary venous return. In patients with left-sided obstruction, LACV was initially demonstrated with echocardiographic evaluation, performed in apical 4-chamber, high parasternal, and subcostal views. Atrial septum was restrictive or intact in patients with left-sided obstructions. LACV originated directly from the left atrium in all patients. CONCLUSION: Levoatriocardinal vein is an extremely rare cardiac pathology, presenting almost exclusively in patients with left-sided obstructive lesions. In patients with left-sided obstructions, LACV must be kept in mind. It may also present in patients with normal intracardiac anatomy and pulmonary venous return.

Closure of aortopulmonary window using Nit-Occlud® PDA-R device in a 3-month-old infant.

Aortopulmonary window (APW) is a rare abnormality in which a pulmonary defect exists between the ascending aorta and the main pulmonary artery. Given that it may result in cardiac failure and pulmonary vascular disease in the early period, treatment needs to be performed without delay. In addition to surgical treatment, transcatheter closure may also be performed for selected patients. This study describes the case of an infant diagnosed with APW and who underwent successful transcatheter closure using a Nit-Occlud® PDA-R device.

Double inlet left ventricle-ventriculoarterial discordance without surgical treatment.

Univentricular heart is a rare congenital defect that consists, in nearly 80% of cases, of double inlet left ventricle, to which both atrioventricular valves connect. A 31-year-old male patient was diagnosed with single ventricle at the age of 9. He had not received any surgical intervention and was not on any medication. Functional capacity was assessed as New York Heart Association class III. Both atrioventricular valves were found to open into the morphological left ventricle, which was located on the right; the hypoplasic right ventricle was on the left side of the left ventricle, the posteriorly located aorta emerged from the right ventricle, and the anteriorly located pulmonary artery emerged from the left ventricle. There was significant subvalvular and valvular stenosis in the pulmonary valve site. The disease is complex and it is very rare that untreated patients reach adulthood.