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.

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.

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.

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.

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.

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.

Using a Cardiac Event Recorder in Children with Potentially Arrhythmia-Related Symptoms.

BACKGROUND: In this study, we reported our experience with the use of cardiac event recorders in pediatric patients. METHODS: We evaluated 583 patients fitted with an event recorder (15-30 days) between March 2010 and November 2014 at our clinic. Excluded from the study were 117 patients with no recorded events and six with records contaminated by electrocardiogram artifacts. All of the patients received electrocardiograms, Holter monitoring, and echocardiography before the cardiac event recording. RESULTS: The patient sample consisted of 460 patients (64% female). The mean age was 12.8 ± 4.1 years. The median number of recorded events was 7. The indications included palpitations in 336 (73%) patients, syncope in 27 (6%) patients, and chest pain and palpitations in 97 (21%) patients. Whereas 64 patients (14%) had structural heart disease according to echocardiographic examination, the remaining patients had normal echocardiographic examination results. The most frequent cardiac comorbidities were mitral valve prolapse (6%), operated tetralogy of Fallot (1.5%), and complicated congenital heart diseases with single ventricle physiology (1%). The recorded events were sinus tachycardia in 113 (25%) patients, supraventricular tachycardia in 35 (8%) patients, ventricular extrasystole in 20 (4%) patients, supraventricular extrasystole in nine (2%) patients, and ventricular tachycardia in two (0.4%) patients. Based on the event recorder and follow-up electrocardiogram findings, 46 patients received an electrophysiology study/ablation. The symptom-rhythm correlation was 39%. CONCLUSION: In the presence of possible arrhythmia-related symptoms in children, a cardiac event recorder can be considered a useful primary diagnostic method. More research on this topic is needed.

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.

Factors affecting perioperative mortality in tetralogy of Fallot.

BACKGROUND: We evaluated the preoperative, operative and postoperative risk factors affecting early mortality in patients who underwent total correction of tetralogy of Fallot (TOF). METHODS: One hundred and twenty-two TOF patients who underwent reparative surgery between January 2010 and November 2013 were enrolled in the study. RESULTS: Mean patient age and weight was 2.3 ± 2.5 years and 11.3 ± 6.4 kg, respectively. Cardiac catheterization was performed in 101 patients (83%),and coronary anomalies were found in 11 patients. Mean McGoon index, pulmonary annulus z-score, main pulmonary artery z-score, left pulmonary artery z-score and right pulmonary artery z-score were 2.0 ± 0.4, -1.85 ± 1.54, -2.84 ± 2.06, 1.17 ± 1.73, and 0.74 ± 1.57, respectively. Total reparative surgery with a transannular patch was performed in 97 patients (79.6%); the rest underwent valve-sparing surgery. Median duration of postoperative mechanical ventilation, intensive care and hospital stay were 19 h, 3 days and 9 days, respectively. Extracorporeal membrane oxygenation (ECMO) was required in 10 patients in the postoperative early period. Arrhythmias occurring in the early postoperative period were junctional ectopic tachycardia (n = 13), complete atrioventricular block(n = 10; permanent epicardial pacemaker implanted in four) and ventricular tachycardia (n = 4). Nine patients died in the early postoperative period (7.3%). Parameters found to be associated with increased mortality were low preoperative oxygen saturation; high right ventricular/aortic pressure ratio immediately after surgery; presence of coronary anomaly; requirement of postoperative ECMO; and pacemaker (P = 0.02, P = 0.04, P = 0.01, P = 0.0001, P = 0.03, respectively). CONCLUSIONS: Poor preoperative oxygenation, presence of coronary anomaly, complete AV block in the early postoperative period, high RV pressure and requirement of ECMO appear to be the most significant factors that affect early mortality in the surgical treatment of TOF. Appropriate preoperative assessment, correct surgical strategies and attentive intensive care monitoring are required in order to reduce mortality.

Combination of congenital left atrial appendage aneurysm in an infant with transposition of the great arteries: a previously unreported association.

A 35-day-old boy weighing 4.5 kg was referred to our department from a general paediatric hospital due to low oxygen saturation. His transcutaneous oxygen saturation was 75%. Transthoracic echocardiography showed discordant ventriculo-arterial connections, a non-restrictive atrial septal defect, and patent ductus arteriosus, in addition to a 23 mm×10-mm left atrial appendage aneurysm. As far as we know, this association has not been reported previously in the literature.

The use of flecainide in critical neonates and infants with incessant supraventricular tachycardias.

OBJECTIVE: This study aimed to evaluate the efficacy of flecainide therapy in neonates and infants with drug resistant incessant supraventricular tachycardia. METHODS: The study included 11 neonates and infants who received medical and/or ablation therapy between January 2010 and December 2013. Mean patient age and weight were 101.6 ± 96 days and 5.3 ± 1.9 kg respectively. Of the 12 patients, 5 underwent ablation between January 2010 and December 2011, and 6 were treated medically between January 2012 and December 2013. Mean follow-up time was 18 months (6 months-4 years). RESULTS: The antiarrhythmic agent flecainide only became available in Turkey in 2012, and the most noteworthy point was its addition to the therapy administered prior to ablation (adenosine, esmolol-propranolol, propafenone, amiodarone and cardioversion). In all 6 patients admitted between January 2012 and December 2013, refractory SVT was successfully treated with the administration of a triple therapy regimen of esmolol-propranolol, amiodarone and flecainide. One patient with myocarditis developed an atrial flutter complicated by a concealed accessory pathway and was put on extracorporeal membrane oxygenation (ECMO) support due to cardiopulmonary failure. The SVT was terminated, but the patient died on the fifteenth day of ECMO support. One patient with recurrent tachycardia, who had previously undergone ablation for a complex cardiac anomaly and Wolf-Parkinson-White syndrome, was treated with ablation again. No recurrence of tachycardia was observed in any of the other 9 patients. CONCLUSION: It appears that the use of propranolol-esmolol and amiodarone combined with flecainide in the medical treatment of drug-resistant SVT may reduce the need for ablation in critical neonates and infants.

Simultaneous transcatheter closure of intralobar pulmonary sequestration and patent ductus arteriosus in a patient with infantile Scimitar syndrome.

Scimitar syndrome is a rare disease associated with a right lung sequestration vascularised by arteries arising from the abdominal aorta and abnormal venous drainage into the inferior vena cava. The infantile form is generally presented with severe heart failure, pulmonary hypertension and respiratory distress. It may be associated with various intracardiac defects, including atrial septal defects, ventricular septal defects, patent ductus arteriosus or more complicated structural congenital heart defects. Here, we present a 2-month-old girl with Scimitar syndrome whose pulmonary arterial pressure decreased after transcatheter patent ductus arteriosus closure and embolization of the anomalous systemic arterial supply.

Assessment of atrioventricular conduction following cryoablation of atrioventricular nodal reentrant tachycardia in children.

BACKGROUND: Early-onset transient atrioventricular block (AVB) is a rare occurrence following cryoablation of atrioventricular nodal reentrant tachycardia (AVNRT), despite lack of any AVB at the end of the procedure. The purpose of this prospective study was to assess AVB shortly after successful cryoablation of AVNRT in children. METHODS: A 6-mm-tip cryocatheter was used in 39 procedures. An 8-mm-tip catheter was used in 11 procedures. Twelve-lead electrocardiograms (ECGs) and 24-hour ambulatory ECGs were performed 24 hours prior to the procedure and immediately following the procedure. All procedures were done using the EnSite system (St. Jude Medical, St. Paul, MN, USA) without fluoroscopy. RESULTS: Although nine (18%) patients developed variable degrees of transient AVB during the procedure, all of them had normal atrioventricular (AV) conduction at the end of the procedure and did not require any intervention. Four of these patients had variable degrees of transient AVB following the procedure despite having normal AV conduction at the end of the procedure. One developed Mobitz type I AVB, which lasted for 11.5 hours, and the other three experienced 2:1 AVB, which lasted for 2, 8, and 24 hours, respectively. All patients had complete resolution of the AVB, which was also documented with the 24-hour ambulatory ECGs after the procedure. CONCLUSION: Early transient AVB can develop following AVNRT cryoablation even if AV conduction is normal at the end of the procedure. Despite the transient AVB in the initial 24 hours after the procedure in some cases, there is no evidence for ongoing AV nodal dysfunction.

Holter electrocardiography findings and P-wave dispersion in pediatric patients with transcatheter closure of atrial septal defects.

BACKGROUND: This study aimed to determine the frequency of postintervention arrhythmia and factors associated with the development of arrhythmia, including the correlation between arrhythmia and P-wave dispersion, and the effects of the latter on transcatheter closure of atrial septal defects (ASDs). METHODS: Holter ECG recordings were performed before and after the intervention and 6 and 12 months later in 47 of the 59 patients who had undergone transcatheter ASD closure and once in the healthy control subjects. RESULTS: A statistically significant correlation was identified between the patients' arrhythmia grade according to Lown's system and each of the following: the number of defects, the size of the atrioventricular valve rim, the presence of an atrial septal aneurysm. CONCLUSION: The frequency of arrhythmia increases after transcatheter ASD closure, gradually decreases within the next year, and is most frequently of a benign nature. Lown's arrhythmia grading of patients occluded with either the Amplatzer septal occluder (ASO) or the Cardio-O-Fix septal occluder (CSO) were compared, and the arrhythmia frequency was higher with the latter. One day after the intervention, the P maximum (Pmax ) and the P dispersion(Pdis ) values were not increased but in fact slightly reduced in patients occluded with either ASO or CSO. An improvement in the electrical system resulting from early anatomical and mechanical healing following transcatheter ASD occlusion may explain the reduction in the Pmax and Pdis values.

Near infrared spectroscopy monitoring in the pediatric cardiac catheterization laboratory.

Near-infrared spectroscopy (NIRS) is a noninvasive method used to evaluate tissue oxygenation. We evaluated the relationship between cerebral and renal NIRS parameters during transcatheter intervention and adverse events in the catheterization room. Between January 1 and May 31, 2012, 123 of 163 pediatric patients undergoing cardiac catheterization were followed by NIRS. All were monitored by electrocardiography, noninvasive blood pressure measurement, pulse oxymetry, initial and final blood lactate level measurement. The number of interventional procedures was 73 (59%). During the procedures, 39 patients experienced a total of 41 adverse events: 18 (19.5%) had desaturation, 10 (8.1%) arrhythmia, three (2.4%) had respiratory difficulty, six (4.8%) had a situation calling for cardiopulmonary resuscitation, three (2.4%) had anemia necessitating transfusion, and one (0.8%) had a cyanotic spell. Cranial NIRS values worsened in 12 (9.8%) and renal measurements worsened in 13 (12.5%) patients. The sensitivity and specificity of a 9% impairment of cranial values were 90 and 61%, respectively, while the corresponding calculations for a 21% fall in renal measurements were 54% sensitivity and 90% specificity. When arrhythmia developed, NIRS values fell simultaneously, while the development of a desaturation problem was heralded by NIRS falling 10-15 s earlier than changes in pulse oxymetry; on improving saturation, NIRS returned to earlier values 10-15 s before pulse oxymetry readings. NIRS monitoring may provide an early warning with regard to complications likely to develop during a procedure. A fall of 9% in cranial NIRS values, or of 21% in renal measurements, should raise clinician awareness.