Surgical procedures for coronary arteries in pediatric cardiac surgery: Risk factors and outcomes.

BACKGROUND: Limited data exist regarding the coronary revascularization procedures needed during the repair of several congenital and pediatric cardiac malformations. We aimed to determine risk factors for in-hospital mortality and long-term outcomes of various pediatric coronary revascularization procedures. METHODS: We retrospectively reviewed the records of 32 consecutive pediatric patients who underwent coronary revascularization procedures at our institution between May 1995 and June 2020. In-hospital mortality, risk factors, surgical indications, revascularization patency, and mid- and long-term follow-up data were investigated. Patients were categorized into the coronary artery bypass grafting (n = 11) and other coronary artery procedure (n = 21) groups. RESULTS: The median age and weight of patients at the time of surgery were 9 months and 4.8 kg, respectively. There were five in-hospital deaths (5/32, 15.6%). The mortality rates were 27.2% (3/11) in the coronary artery bypass grafting group and 9.5% (2/21) in the other coronary artery procedure group (p = .206; 95% confidence interval: 0.496-25.563). The mortality rates for planned and rescue procedures were 8.3% (2/24) and 37.5% (3/8) (p = .06), respectively. The median follow-up time was 12.5 years. Control imaging studies for coronary patency were performed in 70.3% (19/27) of surviving patients. The overall coronary patency rate was 94.7% (18/19). CONCLUSIONS: Pediatric coronary revascularization procedures with elective-planned indications can be performed with good outcomes. Young age and rescue and emergency procedures may carry an increased risk of in-hospital mortality, although not found to be statistically significant. Surviving patients require lifelong follow-up regarding the patency of reperfused coronary arteries.

Transcatheter Closure of Left Ventricular Pseudoaneurysm After Mitral Valve Replacement.

Left ventricular pseudoaneurysm (LVPA) is a rare but lethal complication of mitral valve replacement (MVR) or myocardial infarction. Early correction is necessary for patients with extensive and expanding LVPA. We report a transcatheter closure of LVPA after MVR. A 63-year-old woman presented with an LVPA 2 months after MVR. The repeated computed tomographic scan and transthoracic echocardiography showed enhancement of pseudoaneurysm. The LVPA was closed successfully with Amplatzer Vascular Plug using a transcatheter approach.

Valve-sparing repair with skeletonization of the pulmonary annulus for tetralogy of Fallot.

Pulmonary valve preservation in tetralogy of Fallot (TOF) repair is one of the most challenging issues. Herein, we describe a novel valve-sparing technique for TOF repair that primarily consists of skeletonization of the anterior part of the pulmonary annulus and gentle dilatation by preserving the pulmonary valve and annulus integrity. With encouraging early results, this technique is suggested to prevent severe pulmonary regurgitation and provide acceptable relief of pulmonary stenosis in patients with TOF.

Pulmonary valve replacement in patients with repaired tetralogy of Fallot: early results for recovery of right ventricular dilatation and QRS duration

Background/aim: Although pulmonary valve replacement (PVR) improves ventricular function and symptoms, the benefit and optimal timing of PVR are controversial. This study aimed to evaluate early response to PVR for right ventricle (RV) dilatation and QRS duration. Materials and methods: Retrospective analysis was performed for 32 patients with repaired tetralogy of Fallot (TOF) between March 2005 and October 2017. The differences between preoperative and postoperative changes in echocardiographic parameters, clinical symptoms, and QRS duration were evaluated. Results: There were no in-hospital or late deaths. Mean age at the time of PVR was 16.57 ± 7.97 years. The interval between TOF repair and PVR was 12.99 ± 7.06 years. Postoperative echocardiographic findings showed significant reduction in indexed RV end-diastolic diameter (RV-EDDI) and the ratio of RV/LV-EDDI (P = 0.001 and P = 0.001, respectively). Higher preoperative RV-EDDI was associated with decreased change in RV-EDDI after PVR (r = 0.63; P = 0.001). Normalization of RV diameters was found to be independent of age at PVR, interval between TOF repair and PVR, preoperative QRS duration, and preoperative RV-EDDI. Conclusion: Significant improvement in RV diameter and symptoms could be obtained with PVR in patients with severe pulmonary regurgitation.

Coronary artery bypass grafting in children.

BACKGROUND: We present our clinical experience with coronary artery bypass grafting (CABG) in children. METHODS: Ten children who underwent CABG between July 1995 and August 2017 were retrospectively analyzed. Data including congenital cardiac malformations, previous surgical procedures, age and sex, type of coronary complications, ischemic events preceding surgery, and ventricular function before and after CABG were recorded. RESULTS: The study population consisted of five males and five females with a median age of 2.5 years (range, 88 days to 15 years). Eight internal mammary arteries (IMAs) and two saphenous veins were used for grafting. Indications for bypass grafting were coronary artery (CA) complications related to the post-arterial switch operation in six, CA complications during the Ross procedure in two, and an iatrogenic CA injury during complete repair of tetralogy of Fallot with abnormal CA, crossing the right ventricular outflow tract in two patients. Six of the grafts were performed as rescue procedures. Three patients died during hospitalization. The mean follow-up time was 6.8 years (range, 3 months to 18 years). Anastomoses were evaluated by coronary angiography in four patients, and were all patent. Echocardiography revealed normal myocardial function in all patients. CONCLUSION: Our study suggests that the IMA should be the graft of choice in children due to its growth potential and long-term patency.

Late Cardiac Rupture after Amplatzer Septal Occluder Implantation.

Transcatheter device implantation has become an attractive alternative to surgery in the closure of atrial septal defects in selected patients. However, it can lead to early and late sequelae, some of them life threatening. For example, 79 days before her admission to our emergency department with sudden-onset respiratory distress and respiratory arrest (leading to cardiac tamponade and rupture), a 22-year-old woman had undergone percutaneous closure of an atrial septal defect. We describe the damage and its treatment. Although the adverse effects of transcatheter device implantation are rare, physicians should know that these events can be life threatening. Further data are needed to prevent such sequelae and to design new devices. It is of utmost importance that patients and their family members be informed both of possible sequelae and of life-saving interventions to be administered at early diagnosis.

Successful percutaneous removal of broken umbilical vein catheter in a very low-birth-weight preterm infant.

A 28-week-old preterm infant with a birth weight of 1250 g had a broken segment of umbilical vein catheter (UVC) lodged in the right atrium. It was observed that a 7-cm fragment of catheter had migrated to the right atrium through the inferior vena cava. The catheter was successfully retrieved by fluoroscopically guided percutaneous cardiac catheterization, using a cobra catheter and snare wire, without complication. Fracture and embolization of UVC is a rare but serious complication. Presently described was a case of fractured and embolized UVC in a very low-birth-weight preterm infant, and its successful retrieval via percutaneous endovascular approach.

An Easy Modification of Tube Graft Interposition During Fenestration in Borderline Fontan Circulation.

One of the options for the management of borderline/failing extracardiac Fontan circulation is surgical creation of an atrial fenestration to decompress the systemic venous compartment and improve cardiac output. Depending on the body surface area of the patient, a 5- to 10-mm polytetrafluoroethylene (PTFE) tube graft can be used. When fenestration is required in a patient with failing Fontan circulation, particularly in redo cases, application of a side-biting clamp may be challenging because of adhesions and a thickened atrial wall. In this article, we present our off-pump technique of atrial-side anastomosis of PTFE graft interposition between an extracardiac Fontan conduit and the atrium without using a side-biting clamp.

Successful stenting of systemic venous pathway stenosis after double switch repair for congenitally corrected transposition of great arteries in a child.

An 8-year-old boy with previous shunt operation for corrected transposition of great arteries, ventricular septal defect, pulmonary stenosis and multiple aortopulmonary collateral arteries underwent corrective surgery. In the early post-operative period, there were clinical findings of superior vena cava obstruction. Cardiac catheterization at 72 h following surgery showed a systemic venous baffle stenosis between the vena cava and right atrium. A stent was successfully implanted in the vena cava percutaneously, and the stenosis was relieved. Her symptoms resolved in a short time period, and she was extubated rapidly. During the follow-up, excellent maintenance and patency of systemic venous baffle were observed.

Balloon valvuloplasty for critical aortic stenosis in a fetus: a case report.

The mortality and morbidity of fetal aortic stenosis (AS) depend on the degree of the hemodynamic effects of the stenosis, and left ventricular (LV) adaptation, development and function during fetal life. In the case of critical AS, the development of hydrops and death in utero are well recognized entities. A 23-week gestation fetus was diagnosed with critical severe AS, cardiomegaly, a dilated LV with very poor contractility, and mitral regurgitation. There was a reversal of flow in the aortic arch through the ductus arteriosis and a reversed a-wave in the ductus venosus on Doppler examination. The fetus had hydrops with ascites, and massive scalp, face and skin edema. Fetal amniocentesis was normal. Aortic valvuloplasty was performed under general anesthesia and echocardiographic guidance. Pericardial effusion was not observed after the procedure. However, LV function could not be ameliorated and continued to diminish. There was no cardiac activity in the fetus two hours after the intervention. Aortic valvuloplasty in utero for AS is technically feasible. Mortality is mainly associated with technical errors, LV function, and the degree of endofibroelastosis in the effected fetuses.

Aorto-left ventricular tunnel originating from the left coronary sinus with aortic aneurysm in an 11-year-old boy.

Aorto-left ventricular tunnel, characterized by extracardiac communication between the ascending aorta and left ventricle, is a very rare congenital condition. Although some affected infants remain asymptomatic until adulthood, most present with symptoms of heart failure during their first year of life. We report the case of an 11-year-old boy who had coexisting abnormalities: an aorto-left ventricular tunnel originating from the left coronary sinus, and an ascending aortic aneurysm. The patient underwent on-pump surgical correction and was in New York Heart Association functional class I status a year later. We also review the nature of this rare condition.

Indications and risks of delayed sternal closure after open heart surgery in neonates and early infants.

BACKGROUND: Delayed sternal closure (DSC) has been an essential part of neonatal and infant heart surgery. Here, we report our single institution experience of DSC for eight years. METHODS: The successive 188 patients were analyzed retrospectively. Sternum was closed at the end of the operation in 97 (51.6%) patients (primary sternal closure [PSC] group). Sternum was left open in 91 (48.4%) patients. Among them, 45 (23.9%) had only skin closure (DSCs group) and 46 (24.4%) had membrane patch closure (DSC membrane [DSCm] group). Median age was higher in PSC group (90 days) than DSCs (11 days) and DSCm groups (9.5 days). RESULTS: Mortality was 1%, 11.1%, and 28.2% in PSC, DSCs, and DSCm groups, respectively (P < .05). Univariate analysis recognized the neonatal age (odds ratio [OR] = 4.2), preoperative critical condition (OR = 5.3), cardiopulmonary bypass time >180 minutes (OR = 4), and cross clamp time >99 minutes (OR = 3.9) as risk factors for mortality. Total morbidity rate was higher in DSCm group (73.9%) than DSCs group (51.1%) and PSC group (23.7%; P < .001). Mechanical ventilation time, intensive care unit stay, and hospital stay were longer in DSCs and DSCm groups than PSC group (P < .001). The incidence of hospital infection was also higher in DSCs (43.5%) and DSCm (33.3%) groups than PSC group (20.6%; P < .05). But there was no difference in the incidence of sternal wound complications, including both deep and superficial (4.1%, 8.8%, and 4.4%, respectively). CONCLUSION: Although the risk of sternal wound complications is not different, patients who necessitate DSC (using both skin and membrane closure techniques) have more complicated postoperative course than patients with PSC.

Peritoneal dialysis requirements following open-heart surgery in children with congenital heart disease.

This article reviews our experience with 111 pediatric patients following open-heart surgery over 1-year period. Peritoneal dialysis was required in 34 of 111 children (30.6%). We randomly selected 33 patients who did not require peritoneal dialysis as control group. The indications of dialysis were oligoanuria and/or elevated serum creatinine level (19/34, 55.8%), fluid overload and/or hemodynamic alterations (10/34, 29.5%), and hyperkalemia and/or acidosis (5/34, 14.7%). Among the 34 dialyzed patients, 19 (55.6%) had acute renal failure (ARF). Cyanotic congenital heart disease was significantly higher in patients who required dialysis than the patients who did not require dialysis (67.6% and 22.6%, respectively, p < 0.001). Cardiopulmonary bypass time was significantly longer in patients with ARF than those without ARF (p < 0.05). Overall mortality rate was significantly higher in patients who required dialysis than control group (42.1% and 18.2%, respectively, p < 0.05). However, in the dialyzed group the mortality for patients who developed ARF was 68.4% and 6.7% for those who did not develop ARF [odds ratio (OR): 30.3, confidence interval (CI) 95%: 3.2-28.7, p < 0.001]. In conclusion in children high mortality rate following open-heart surgery was associated with ARF. Patients with cyanotic congenital heart disease and prolonged cardiopulmonary bypass time are at risk for ARF. The presence of these factors can be predicted in the early institution of peritoneal dialysis after cardiac surgery.

Peripheric stem cell transplantation in children with dilated cardiomyopathy: preliminary report of first two cases.

We report two pediatric patients with IDC who underwent autologous PSCT. Both cases were referred to our clinic for cardiac transplantation because of end-stage heart failure resistant to conventional therapy with digoxin, diuretics, ACE inhibitors, and sympathomimetics. They had ejection fractions below 35%. In each case, autologous stem cell transplantation was performed via the coronary arteries, and five wk after the procedure transthoracic echocardiography showed a striking gain in their ejection fractions and an improvement in the left ventricular dimensions compared with the initial measurements. Although heart transplantation is the only option for children with IDC, stem cell transplantation can lessen the waiting list mortality and prolong the time for a patient to wait for a suitable donor.

Impact of peritoneal transport characteristics on cardiac function in paediatric peritoneal dialysis patients: a Turkish Pediatric Peritoneal Dialysis Study Group (TUPEPD) report.

BACKGROUND: The peritoneal equilibration test (PET) is recommended in paediatric peritoneal dialysis (PD) patients to assist prescription management. Despite contradictory reports, high transporter status is associated with reduced survival rate in adults. Since cardiac disease is one of the main causes of mortality in paediatric PD patients, we aimed to evaluate whether transport features have any effect on biochemical data and cardiac function in this group. METHODS: One hundred and ten PD patients (13 +/- 5 years, PD vintage: 31 +/- 27 months) were enrolled into the study. Four-hour dialysate/plasma creatinine ratio was used for differentiating PET groups. Thirty-eight patients were high transporters, 29 were high-average transporters and 43 were low-average/low transporters. Echocardiography was performed in all subjects. RESULTS: Age, PD vintage, dialysate glucose concentration, ultrafiltration volume, urine volume and blood pressure levels were similar in all PET groups. No biochemical or echocardiographic data (ejection fraction, fractional shortening, left ventricular mass index, myocardial performance index, power Doppler E/tissue Doppler E ratio reflecting diastolic function) were different among PET groups except lower albumin (P = 0.025) levels in high transporters and higher high-sensitivity C-reactive protein (P = 0.026) levels in high and high-average transporters compared to other transport groups. CONCLUSIONS: Cardiac structural and functional abnormalities are highly prevalent among paediatric PD patients. Transport rates did not have a significant effect on biochemical parameters or cardiac structural/functional parameters. It might be suggested that being a high transporter does not provide a disadvantage in terms of atherogenic tendency and cardiac disease in paediatric PD patients. Oligoanuria, anaemia and hypertension were independent predictors of cardiac disease.

Kearns-Sayre syndrome presenting as somatomedin C deficiency and complete heart block.

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease in which neuromuscular structures, endocrine glands, and cardiac conduction systems are most commonly involved. An 11-year-old boy was admitted with blurred consciousness, respiratory instability, and bradycardia of two-hour onset. He was immediately intubated. His medical history included growth retardation and myopic refractive defect for six years, therapy for somatomedin C deficiency for 15 months, and bilateral ptosis for three months. On physical examination, he was unconscious, had a peak heart rate of 40/min, blood pressure of 60/20 mmHg, and a weak pulse. Laboratory findings showed elevated blood lactate and blood pyruvate levels and an increased lactate/pyruvate ratio. The electrocardiogram showed complete atrioventricular block and echocardiography showed mitral valve prolapse. Following implantation of a temporary transvenous cardiac pacemaker, his heart rate and clinical condition improved. Further analysis with cranial magnetic resonance (MR) imaging demonstrated hyperintense signal changes in the subcortical white matter of the two cerebral hemispheres, bilateral thalamus, putamen, cerebral peduncles, dorsal medulla, and midbrain. The typical clinical and MR findings confirmed the initial diagnosis of KSS. A permanent cardiac pacemaker was implanted into the right ventricle.

[Pulmonary artery banding operation and results of terminal biventricular and univentricular repair]. / Pulmoner arter bant ameliyati ve sonrasinda biventriküler ve üniventriküler tamir sonuçlari.

OBJECTIVE: In this study, we report the current indications, early-midterm results and the outcome of pulmonary artery banding (PAB) operation after definitive repair. METHODS: Between 2000 and 2007, 28 infants underwent PAB operation. Ages were between 21 days and 6 months (mean 3.0+/-1.7 months). All patients had pulmonary hypertension at systemic level. Fourteen patients were candidates for terminal biventricular repair [atrioventricular septal defect (AVSD) (n=6), double outlet right ventricle (DORV) +ventricular septal defect (VSD) (n=4), Swiss cheese VSD (n=2), transposition of the great arteries (TGA) +Swiss cheese VSD (n=1), ASD+VSD+aortic coarctation (n=1)], and the remaining 14 were candidates for terminal univentricular repair [double inlet left ventricle (n=5), double inlet right ventricle (n=2), AVSD+left ventricular (LV) hypoplasia (n=2), DORV+LV hypoplasia (n=2); tricuspid atresia (n=1), left AV valve atresia (n=1), TGA+Swiss cheese VSD+LV hypoplasia (n=1). RESULTS: Three patients (1 in biventricular group; 2 in univentricular group) died in the early postoperative period (10.7%). Three patients needed long duration of mechanical ventilatory support. Twenty- three of the surviving patients (92%) were followed -up between 1 month to 7 years. Currently 7 patients underwent successful biventricular repair and five patients underwent univentricular repair (extracardiac Fontan 3; Glenn 2). Two patients died early and 1 patient died 1 year after extracardiac Fontan operation. Survival for biventricular group was 92.8% at 1 and 4 years and 85.7% and 58.4% respectively for univentricular group (p<0.05). CONCLUSION: Pulmonary artery banding operation has still a significant role in the palliation of certain congenital cardiac anomalies. Outcome of patients who are candidates for biventricular repair is better than the univentricular repairs.