Interventional cardiac catheterization in infants weighing less than 2500 g.

The aim of our study was to share our clinical experience regarding cases of interventional cardiac catheterization in low-weight infants. We retrospectively reviewed all interventional catheterizations performed in infants weighing 2500 g or less between March 2001 and October 2012. Twenty patients were included in the study. The procedures included balloon atrial septostomy in 8 patients, balloon pulmonary valvuloplasty in 7 patients, balloon aortic valvuloplasty in 3 patients and balloon angioplasty for coarctation in 2 patients. The mean age at catheterization was 11.9 ± 9.6 days (range, 1 to 31 days) and the mean weight, 2038 ± 480 g (range, 1100 to 2500 g). The mean procedure time was 80.3 ± 32 minutes, and the mean fluoroscopy time was 17.5 ± 12 minutes. Only two patients had no benefit from the intervention. One patient died at day 4 after catheterization, due to sepsis. Complications occurred in 3 patients: one patient developed atrial flutter and respiratory arrest, one patient developed apnea episodes, and one patient developed anemia that required transfusion. In conclusion, although studies investigating complications related to invasive cardiac catheterization in infants weighing less than 2500 g have reported increased complication rates, such complications were less likely to result in permanent sequelae. We did not observe significant complications related to the procedure; therefore, we would like to suggest that invasive cardiac catheterization can appropriately be performed in low-weight infants.

Serum Bcl-2 values in children with pulmonary hypertension.

In this study, we aimed to assess levels of serum B cell lymphoma 2 (sBcl-2) in children, which has been implicated in the etiopathogenesis of pulmonary hypertension (PH), as well its association with tissue Doppler echocardiographic imaging (TDI) data and parameters used in the follow-up of PH. The sBcl-2 level was assessed in 35 children with PH (24 had eisenmenger syndrome, and 11 had idiopathic PH) and in 38 healthy children as controls. TDI was performed on 25 patients whose cardiac anatomy allowed the test. The respective sBcl-2 values in patients and controls were 35.69 ± 18.83 and 2.66 ± 7.95 ng/ml (p < 0.001). The sBcl-2 levels were significantly greater in the New York Heart Association (NYHA) functional class 3 patients than those in the NYHA class 2 patients (p = 0.033). The sBcl-2 value in patients who walked <475 m in the 6-min walk distance (6MWD) test was significantly greater than in those who walked ≥475 m (p = 0.038). The sBcl-2 level showed a negative correlation with ejection time measured at the septal anulus (p = 0.026) and a positive correlation with interventricular septum-Tei (p = 0.018). The results of this study showed for the first time that there is an increase in the levels of sBcl-2 as an inflammatory marker and that the sBcl-2 levels are associated with prognostic parameters in children with PH. Because sBcl-2 levels were greater in patients who walked <475 meters during the 6MWD test, we suggest 475 ms as the cut-off value for the 6MWD test to differentiate between a good and a bad prognosis.

Cardiac rhabdomyomas in childhood: six cases from a single institution.

Primary cardiac tumors are rare during childhood. The most frequently encountered tumors are rhabdomyomas. We reviewed the clinical characteristics, treatment results, and outcomes of six pediatric patients with primary cardiac rhabdomyomas. The mean age was 16.8 days. Only one patient was symptomatic. The tumors mostly originated from the left ventricle. The diagnosis was established by magnetic resonance imaging (MRI) plus echocardiography with or without histopathology. Total tumor resection was performed in two patients. After a median follow-up of 39 months, one patient had a stable tumor, two patients had marked tumor regression and one had complete tumor regression. Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up may be sufficient in hemodynamically stable cases. Although rhabdomyomas do not cause any symptoms at the time of diagnosis, they may lead to sudden death; thus, further studies may be required for the decision of surgery and/or followup. The localization and infiltrative characteristics of the tumor are critical factors for decision-making in children with symptomatic rhabdomyoma even if surgery is indicated in such cases.

Myocardial performance index by tissue Doppler in bronchopulmonary dysplasia survivors.

Bronchopulmonary dysplasia (BPD) survivors from the surfactant era were evaluated by echocardiography in a few studies and no significant differences were found between BPD and non-BPD children. In this study, we evaluated these children with myocardial performance index (MPI), which was obtained by tissue Doppler echocardiography (TDE) in addition to the conventional methods. Fifteen children with BPD who did not have any cardiopulmonary symptoms at the time of the study were examined. All children were studied with M-mode, two-dimensional and DE. Pulmonary artery systolic pressures (PAPs) were estimated from tricuspid regurgitant velocity, and MPI for both ventricles were obtained by TDE. Results were compared with those of term-born, age- and sex-matched control children. While the variables obtained by M-mode and DE did not differ between the groups, the right and left ventricular MPI were found to be significantly higher in the BPD group compared with the control group (mean right ventricular MPI 0.48 +/- 0.04 vs. 0.41 +/- 0.05; mean left ventricular MPI 0.47 +/- 0.05 vs. 0.39 +/- 0.06). In addition, mean PAPs values of the patients were found to be significantly higher than those of the controls (30.4 +/- 6.9 mmHg vs. 23.3 +/- 5.3 mmHg), and there was a positive correlation between PAPs and right ventricular MPI values in the BPD group (r = 0.5). While routine echocardiographic examinations revealed no difference between the groups, MPI measurements by TDE technique yielded significantly higher values in the BPD group. To this extent, our study is the first to show that survivors of BPD may, in fact, have a subclinical ventricular dysfunction.

Transthoracic echocardiography guidance during percutaneous closure of patent foramen ovale.

BACKGROUND: Percutaneous closure of patent foramen ovale (PFO) has been increasingly performed for several indications; mostly due to cryptogenic stroke. In this study we aimed to evaluate the safety and efficacy of transthoracic echocardiographic (TTE) guidance during percutaneous closure of PFO in using the Amplatzer and Occlutech Figulla PFO occluder devices. METHODS: Between October 2005 and March 2011, 139 patients (74 male, mean age: 40.4 ± 10.3) underwent transcatheter PFO closure. In all patients transesophageal echocardiography performed subsequently to diagnose, assess the size and evaluate for suitability of the defect for percutaneous closure. During the procedure fluoroscopy and TTE were used for guidance. RESULTS: Among 139 patients, Amplatzer PFO occluder was used in 74 patients and in 65 of them Occlutech Figulla device was selected for occlusion. The indications for PFO closure were ischemic stroke in 98 (70.5%), recurrent transient ischemic attacks (TIA) in 40 (28.7%), peripheral embolism in 1 (0.8%) of the patients. In all patients, percutaneous intervention was performed successfully under TTE guidance. There have been no neurologic (recurrent strokes or TIAs) and cardiovascular complications during the immediate and long-term follow-up period (2-67 months, median 29). There was significant difference between the mean fluoroscopic time from the beginning which is 8.6 ± 3.4 min in the former versus 3.4 ± 1.9 min in the latter (P < 0.05). CONCLUSION: Our study confirms the efficacy and safety of TTE guidance during percutaneous closure of PFO, which shortens the procedural time and obviates the need for general anesthesia or endotracheal intubation.

Zinc supplementation and TNF-α levels in vaccinated cardiac patients.

OBJECTIVE: To investigate whether zinc supplementation could affect serum tumor necrosis factor-alpha (TNF-α) levels in congenital and acquired cardiac patients attending for an influenza vaccine. METHODS: 9-18 years old, voluntary children with cardiac disease were weighed, measured height and blood samples for hemoglobin level, zinc level, IgA and IgG titers of influenza A and B serotypes and TNF-α levels were obtained. The children were randomly assigned to 1 of 2 groups: ZV group received both zinc supplementation and influenza vaccine; V group received influenza vaccine. Serum TNF-α levels were measured at 28 days after the immunization. RESULTS: Mean (±SD) serum zinc level was 100 (±20) µg/dL. Cases with congenital heart disease had lower serum zinc levels than acquired ones (93±17 µg/dL, 116±18 µg/dL; respectively, p<0.001). Median titers of serum TNF-α decreased after vaccination (78.9 pg/mL, 74.8 pg/mL; p<0.05) but this significant change occurred only in ZV group (77.1 pg/mL, 73.4 pg/mL; p=0.001). Vaccine associated adverse events were similar in ZV and V groups except malaise (0% in ZV and 23.8% in V group; p<0.05). Adverse events were similar in patients with congenital and acquired heart diseases. During the previous influenza season, 77.3% had more than two episodes of upper respiratory tract infection (URTI), however, after vaccination only 13.6% had more than two episode of URTI. CONCLUSION: Zinc supplementation has a beneficial effect on malaise, one of the influenza vaccine associated adverse events, and decrease serum TNF-α levels.

Midterm experience with implantable cardioverter-defibrillators in children and young adults.

AIMS: This single-centre study was undertaken to review our experience with implantable cardioverter-defibrillator (ICD) implantation in children with relatively different aetiologies. METHODS AND RESULTS: We retrospectively reviewed the records of the paediatric patients who underwent ICD implantation between October 2001 and December 2008. The data of these patients were collected by reviewing the patients' medical records and computerized departmental pacemaker databases. A total of 28 patients who underwent ICD implantation during this period were included in this study. The median age was 12 years and median weight was 32 kg. Most of the patients had ion-channel diseases (n = 13) or cardiomyopathy (n = 11). Devices were implanted for either secondary (n = 22) or primary (n = 6) prevention. The selected ICD generator type was single chamber in 22 patients, dual chamber in 5 patients, and biventricular in 1. Nineteen patients received 122 shocks. Fifteen of 22 patients (68.2%) from the secondary prevention group and 2 of 6 patients (33.3%) from the primary prevention group experienced at least one appropriate shock during a median period of 11.3 months (range: 4 days-6.5 years). Forty-two inappropriate shocks were delivered in seven (31.8%) patients from the secondary prophylaxis group during a median period of 11.3 months. The most important reason for inappropriate shocks was T-wave oversensing. In six patients, lead-related acute or chronic complications occurred. CONCLUSION: The ICD was safe and effective in interrupting malignant arrhythmias in children and adolescents with a high risk of sudden cardiac death. However, the occurrence of lead complications is significant. The incidence of therapies delivered by the device, with appropriate and inappropriate shocks, was high and interfered with the quality of life. The most important reason for inappropriate shocks was T-wave oversensing. Careful programming is mandatory to reduce the inappropriate shocks.

Nonstroke arterial thrombosis in children: Hacettepe experience.

Of the 488 pediatric patients with thrombosis in a single center, 50 (10.2%) had nonstroke arterial thrombosis. Half of the nonstroke arterial thrombosis patients had a preceding arterial catheterization history. Infection was a common contributing underlying disorder among both catheterized and overall groups (28 and 42%, respectively). The third most common underlying risk factor for development of arterial thrombosis was associated cardiac disease, and 70% of these patients had congenital heart disease and 30% had dilated cardiomyopathy. The most common site of arterial thrombosis was the heart, and 22% of the patients developed arterial thrombosis following cardiac surgery for congenital heart diseases. The prothrombotic risk factors were also analyzed in the study group and factor V G1691A and PT G20210A heterozygous mutations were detected to be higher (16 and 4%, respectively) than those in healthy controls (7.4 and 2.3%, respectively). In addition to factor V G1691A and PT G20210A mutations, other prothrombotic risk factors including protein C and protein S deficiencies and elevated factor VIII levels were commonly associated with pediatric nonstroke arterial thrombosis cases in the present study. This finding indicates the importance of these factors in arterial thrombosis development, similar to venous thrombosis. Another striking factor of the study is the higher number of prothrombotic risk factors (more than three) in patients who had persistent thrombosis, indicating the need for more intensive treatment in these patients.

Surgical management of infants with isolated supravalvular pulmonary stenosis: case reports.

Pulmonary stenosis (PS) can be seen from the right ventricular outflow tract to the peripheral pulmonary arteries. Most frequently, the obstruction occurs at the level of the pulmonary valve; however, it occurs less frequently at the infindibular level within the trabecular component of the right ventricle or within the pulmonary arterial pathways. Lesions at any of these levels can occur as part of more congenital cardiac malformations such as tetralogy of Fallot, complete transposition of great arteries, or atrial septal defect. Isolated supravalvular pulmonary stenosis (iSPS) is less common than other types of PS. In this study, we present our experience with 4 patients who underwent cardiopulmonary bypass operation for iSPS. In one patient, the circular stenotic area was noted on the touch point of the pulmonary valve. Right ventricular pressures ranged from 70 to 90 mmHg, and the pulmonary artery mean pressures ranged from 14 to 17 mmHg. In all patients, the left ventricular and aortic systolic, diastolic, and mean pressures were moderately increased. Pulmonary artery stenosis was treated successfully using a pericardial or Dacron patch on cardiopulmonary bypass. Various techniques such as balloon dilation have been proposed to deal with this problem, but these may often be unsuccessful because of the elasticity and recoil of the pulmonary artery constrictive ring. Even though endovascular stenting and/or balloon angioplasty have been recently proposed as an initial treatment strategy, they may be associated with some severe complications including pulmonary artery thrombosis or stent migration. Our study, even though it consists of a limited number of cases, suggests that open heart surgery using an oval-shaped patch may be a used as the other main choice for the treatment of iSPS.

Cardiac dysrhythmias after transcatheter closure of ASD with Amplatzer device.

Transcatheter closure of atrial septal defect (ASD) has been used as an alternative to open heart surgery. Although transcatheter closure of ASD with the Amplatzer septal occluder is a safe and feasible method in pediatric patients, there is little published data on arrhythmia analysis following transcatheter device closure of secundum ASD. We evaluated cardiac dysrhythmias with 24-hour ambulatory electrocardiographic (ECG) monitoring after transcatheter closure of ASD with Amplatzer device. A total of 85 consecutive patients with ASD underwent transcatheter closure of secundum ASD with Amplatzer device between October 1998 and December 2003. The study involved 65 of these patients assessed by 24-hour ambulatory ECG monitoring. Seven patients were evaluated a second time by 24-hour monitorization. During the procedure, transient complete atrioventricular (AV) block was seen in two patients. One of them returned to normal sinus rhythm in catheterization lab and the other returned to normal sinus rhythm in two hours. Transient junctional rhythm was observed in another patient during the device placement. Twenty-four hour ambulatory ECG monitoring was performed on all patients after a mean four-month period (1-12 months). Holter recordings demonstrated rare supraventricular extrasystole in two patients, rare ventricular premature beats in two patients, and intermittent sinus arrest with sinus pause lasting <1.5 seconds in one patient, for a total of five patients (7.6%). In conclusion, dysrhythmias after transcatheter device closure of secundum ASD with Amplatzer device are rare and benign. We need further long-term follow-up to evaluate late dysrhythmias after the transcatheter device closure of secundum ASD.

Diagnosis of perigraft seroma formation by use of echocardiography after modified Blalock-Taussig shunt.

Perigraft seroma is a collection of non-secretory fibrous pseudomembrane surrounding a vascular graft, which is a collection of clear, sterile fluid. Thoracal computed tomography, Magnetic Resonance Imaging, and thoracal ultrasonography have been proposed for diagnosis of this pathology. A consecutive modified Blalock-Taussig (m-BT) shunt complicated by perigraft seroma formation, which is diagnosed using a Doppler ultrasonography (US), is presented here.

Noncompaction of ventricular myocardium in a patient with congenitally corrected transposition of the great arteries treated surgically: case report.

Noncompaction of the ventricular myocardium is a rare disorder that represents numerous prominent trabeculations and intratrabecular recesses in the ventricles. It is believed to represent not only an arrest in endomyocardial morphogenesis but also an unclassified cardiomyopathy. The pathology has been almost invariably associated with other congenital cardiac malformations. A female patient with noncompaction of the myocardium of both ventricles and congenitally corrected transposition of the great arteries (cTGA), situs inversus totalis, and atrial and ventricular septal defects is described. When she was 7 days old a permanent pacemaker was implanted because of complete heart block. Prazosin (Minipress), an alpha-receptor blocker, was administered, and the cardiac ejection fraction showed a striking increase from 20% to 42%. Despite careful and regular follow-up evaluations, the general condition of the patient slowly worsened. Five months after surgery she died of hepatorenal failure and low cardiac output. This case report is thought to be the first description of congenital complete heart block, cTGA, and situs inversus totalis with noncompaction of the myocardium of both ventricles.

Non-valvular isolated pulmonary artery vegetations.

BACKGROUND: One of the serious potential complications of complex congenital heart defect (CHD) is bacterial endocarditis. Right sided endocarditis accounts for 5-10% of all cases of infective endocarditis, with the tricuspid valve more frequently affected than the pulmonary valve. Pulmonary artery involvement in bacterial endocarditis is very rare, but when it exists it is almost always associated with endocarditis of the pulmonary valve: isolated pulmonary artery vegetations have not previously been reported before. CASE REPORT: A 6-year-old boy with atrial septal defect (ASD), ventricular septal defect (VSD), coarctation of the aorta (CA), persistent ductus arteriosus (PDA) and pulmonary hypertension, who had been operated for closure of PDA, correction of CA and pulmonary artery banding, was readmitted with bacterial endocarditis. In surgery, the VSD was closed with a Dacron patch using interrupted pledget buttressed sutures, and the ASD was closed using primary sutures. After debanding, while pulmonary arteriotomy was performed for patch angioplasty, we observed that the entire pulmonary artery trunk, right and left pulmonary artery branches, were filled with a solid mass, which was successfully treated by surgical excision. CONCLUSIONS: With ongoing improvements in diagnostic tools, especially in echocardiography, antibiotic therapy and surgery, CHD can now be treated successfully in early infancy or childhood.