Results of additional pulsatile pulmonary blood flow with bidirectional glenn cavopulmonary anastomosis: positive effect on main pulmonary artery growth and less need for fontan conversion.

BACKGROUND: Additional antegrade pulsatile pulmonary blood flow obtained by leaving the main pulmonary artery patent during bidirectional cavopulmonary shunt has been shown to give additional benefits to the bidirectional Glenn cavopulmonary anastomosis. We retrospectively evaluated our 20-patient pulsatile Glenn series in order to find out whether these salutary effects were valid or not. METHODS: Between June 2007 and November 2011, 20 patients (11 girls and 9 boys) with single-ventricle physiology underwent bidirectional cavopulmonary anastomosis. The additional source of blood flow was through the unligated main pulmonary artery in all patients. A retrospective review of our surgical experience was performed focusing on the role of additional pulmonary flow. Medical records and perioperational and postoperative follow-up data including clinical outcomes were retrospectively retrieved and analyzed. RESULTS: Two patients died in the early postoperative period. One patient died in the follow-up period. Mean follow-up time was 23.9 ± 15.7 months. No superior vena cava syndrome and no increase in pulmonary vascular resistance were observed. Improvement of partial oxygen pressure after pulsatile Glenn has been shown in all patients (P = .00). At a mean interval of 22.9 months, main pulmonary artery size continued to increase after pulsatile Glenn cavopulmonary anastomosis (P = .028). Only 1 patient was converted to Fontan type circulation after pulsatile Glenn cavopulmonary anastomosis. CONCLUSIONS: The pulsatile cavopulmonary shunt is a useful procedure in the early and intermediate term management of patients with a functional univentricular heart. It improves partial oxygen pressure and the impact of pulsatility on the main pulmonary artery.

Absent right superior vena cava with persistent left superior vena cava: two case reports.

Persistent left and absent right superior vena cava is a rare congenital anomaly, which is usually asymptomatic. Persistent left superior vena cava (PLSVC) is generally coexistent with right superior vena cava (RSVC), but rarely associated with absent RSVC. Herein, we report two children referred to our department because of ventricular septal defect. We determined PLSVC with absent RSVC during the angiography.

[Stent implantation into a totally occluded Blalock-Taussig shunt: a case report]. / Tam kapali Blalock-Taussig santa stent uygulamasi: Olgu sunumu.

Stent implantation into a ductus arteriosus or systemic-pulmonary shunt is a relatively new but infrequent approach for palliation. A 22-month-old boy with a modified Blalock-Taussig shunt for tetralogy of Fallot was admitted with severe cyanosis. Echocardiographic examination showed complete occlusion of the shunt. Initial balloon angioplasty resulted in reocclusion of the shunt by acute thrombosis. Finally, recanalization was achieved by stent implantation. Oxygen saturation of the patient increased from 32% to 92% following stenting. He was discharged on aspirin therapy.

Cardiac involvement in an adolescent with acute lymphoblastic leukemia.

Cardiac complications of the pediatric patients with acute leukemia are common. Most of the cardiac complications may be due to chemotherapeutics such as antracyclins, besides anemia, infections, or direct leukemic infiltrations of the heart. It is reported that leukemic infiltration is frequent in the postmortem examination of the myocardium and pericardium. However, at the antemortem examination, pericardial involvement is rare and there is no myocardial involvement reported at the time of diagnosis in patients with acute leukemia in the English literature. Here, the authors report an adolescent with acute lymphoblastic leukemia who had myocardial infiltration at the time of diagnosis.

Propranolol for infantile hemangiomas: a preliminary report on efficacy and safety in very low birth weight infants.

Despite the relatively recent introduction of propranolol in the treatment of infantile hemangiomas, there can be little doubt of its efficacy. With regard to safety issues, there are no prior data for very low weight infants. In this study, we used propranolol in preterm and very low weight infants. We used clinical criteria to assess the response to the therapy. We noted all side effects expected from beta-adrenergic blocking drugs, and followed the patients' weight gain during propranolol treatment. Objective, clinical evidence of hemangioma regression was seen after two months in all patients. None of the patients required treatment discontinuation due to adverse side effects. During the propranolol treatment, weight gain was normal in all patients. To the best of our knowledge, this is the first report on the use of propranolol in preterm and very low weight infants, and also the first report from Turkey on the use of propranolol in infantile hemangiomas.

Quadricuspid aortic valve: a rare cause of aortic regurgitation and stenosis.

Quadricuspid aortic valve is a rare congenital condition that occurs not only as an isolated anomaly, but also with other cardiac defects. We describe a 10-year-old boy whose aortic stenosis was diagnosed during infancy. Transthoracic echocardiography revealed dilation of the left ventricle, valvular and subvalvular aortic stenosis, bicuspid aortic valve, aortic regurgitation, and mitral valve prolapse. The results of cardiac catheterization and aortography showed severe aortic regurgitation, an aortic valve gradient of 76 mmHg, a bicuspid aortic valve, a subaortic membrane, and an ascending aortic aneurysm. The patient underwent elective valve replacement with a mechanical prosthesis, and during surgery, the valve was noted to be quadricuspid. The patient was diagnosed as having a quadricuspid aortic valve associated with aortic regurgitation, severe aortic stenosis, and an ascending aortic aneurysm.

The incidence and risk factors of arrhythmias in the early period after cardiac surgery in pediatric patients.

Rhythm disturbances that develop after pediatric surgery for heart disease significantly increase mortality and morbidity risk. The aim of this study was to determine incidence rates of different arrhythmias and identify risk factors for these disturbances in this patient group. The study involved 580 children in the pediatric cardiovascular intensive care unit who had undergone cardiac surgery between May 2001 and December 2002. Each was followed until discharge. The patient who sustained arrhythmia was recorded. Fifty-one of the patients (8.8%, mean age 1.7+/-2.3 years) developed arrhythmias. Twenty-one (41.1%) had supraventricular tachycardia, 12 (23.5%) had junctional ectopic tachycardia, 10 (19.6%) had complete atrioventricular block, 3 (5.8%) had ventricular arrhythmias, and 5 (9.8%) had atrial fibrillation and atrioventricular dissociation. There was a trend toward higher incidence of arrhythmia (rate, 43.1%) in the 0-6 months age group. The incidence rates of arrhythmia after certain procedures were as follows: 75% after Rastelli operation, 16.7% after total anomalous pulmonary venous return repair, 13.8% after ventricular septal defect repair, 12.8% after the arterial switch operation or arterial switch with ventricular septal defect closure for transposition of the great arteries, 12.5% after atrioventricular septal defect repair, 12.1% after total correction of tetralogy of Fallot, 9.1% after bidirectional cava-pulmonary connection and Fontan procedure, and 6.6% after other miscellaneous procedures. The mean cardiopulmonary bypass time was 105.4+/-54.1 min. At the time of arrhythmia appearance, the mean values for electrolyte (sodium 144+/-5 mEq/L, potassium 3.78+/-0.91 mEq/L, ionized calcium 1.15+/-0.33 mmol/L) and arterial blood gas parameters (pH 7.40+/-0.12 and HCO3 24.7+/-6.3 mmol/L) were all in the normal range. Fifteen (29.4%) of the patients with arrhythmias died and in 7 of these cases, the death was directly linked to resistant arrhythmia. Arrhythmias can be life-threatening especially in the early period after pediatric heart surgery. The incidence of arrhythmia in this series was 8.8%. The results identified type of operation as a major risk factor for arrhythmia after pediatric heart surgery and they also suggest that age may be important as well.

Tetralogy of Fallot and hypertrophic cardiomyopathy in a case of cardiofaciocutaneous syndrome.

Cardiofaciocutaneous (CFC) syndrome is a rare disorder characterized by psychomotor and growth retardation, a typical facial dysmorphism, congenital heart defects, and ectodermal abnormalities. Pulmonic stenosis, atrial and ventricular septal defects, patent ductus arteriosus, and hypertrophic cardiomyopathy are cardiac findings identified in patients with this syndrome; however, tetralogy of Fallot has never been associated with CFC syndrome. CFC should be considered in patients with skin abnormalities in addition to phenotypic features and a congenital heart defect, including tetralogy of Fallot.

Mid-term results of Solysafe® septal occluder for percutaneous closure of secundum atrial septal defects.

BACKGROUND: In this article, we report mid-term follow-up results of the Solysafe® septal occluder for percutaneous closure of secundum atrial septal defects. METHODS: A total of 25 patients (8 males, 17 females; mean age 8.4±3.6 years; range 5 to 12 years) who underwent percutaneous closure of secundum atrial septal defect between July 2008 and June 2010 were included in this study. RESULTS: The mean follow-up was 6.1±0.5 (range, 5.2 to 7.2) years. The device was successfully implanted in 22 of 25 patients. The mean stretched diameter of the atrial septal defect as assessed by balloon sizing was 13.6±4.4 (range, 8 to 26) mm. Nine 15-mm devices, eight 20-mm devices, six 25-mm devices, and two 35-mm devices were used. A 20-mm and two 35-mm devices were used in three patients and the procedure failed in these patients. Among the remaining 22 patients, no pericardial effusion, endocarditis, hemolysis, electrocardiographic changes, valvular problems, or suspicious echocardiographic findings were observed during or after the procedure. Only in one patient, a wire fraction was seen at six years, while another patient had a residual shunt during a six-year follow-up. Device embolization (n=1) and hemiparesis (n=1) were the early major complications related to the procedure. CONCLUSION: Although percutaneous closure of secundum atrial septal defects is successful, it would be wiser to check the device regularly, at least once a year, as the manufacturing of the device has been discontinued due to wire fractions.

Case report: seizures in a child caused by a large venous angioma.

Cerebral venous angioma is a congenital anomaly of the medullary vein, the vessel that drains into the transparenchymal venous stem. This lesion is also referred to as a developmental venous anomaly. A few reports in the literature have documented developmental venous anomaly-related epilepsy, neurologic deficits, and intracranial hemorrhage. A 3-year-old boy was referred to our hospital after he sustained an afebrile, tonic-clonic, focal seizure of 15 minutes' duration that affected his right arm, leg, and eyebrow. Cerebral digital subtraction angiography showed a bilateral, large periventricular developmental venous anomaly. This report describes the clinical and radiologic findings for this large venous angioma that caused seizures in a child.

Truncus arteriosus with double aortic arch: A rare association.

Yildirim SV, Yildirim A. Truncus arteriosus with double aortic arch: A rare association. Turk J Pediatr 2017; 59: 221-223. Truncus arteriosus (TA) is a congenital heart defect often diagnosed in neonatal period; it represents 0.7% of all congenital heart lesions. The pulmonary arteries originate generally above the coronary ostium. Aorto-pulmonary and interventricular defects are believed to represent an abnormality of conotruncal septation. TA is classified into four types, according to Van Praagh and Colette Edwards. Some of congenital heart defects may be associated with TA, such as aortic interruption. In literature, TA with a double aortic arch is observed as a very rare condition. We present here a newborn diagnosed with a combination of TA type 1 and double aortic arch.

The effect of obesity on echocardiographic and metabolic parameters in childhood.

BACKGROUND: Obese children are predisposed to left ventricular (LV) hypertrophy and cardiac dysfunction. We evaluated ventricular function of obese children with conventional echocardiography and tissue Doppler imaging (TDI) and correlated these with fasting serum glucose, lipid and insulin levels. METHODS: Thirty obese children were examined by conventional echocardiography and TDI and compared with an age-matched control group. In the obese children, fasting serum glucose, lipid, and insulin levels were obtained. RESULTS: Systolic and diastolic function of the LV was normal in obese children. LVM/ht2.7 (LVM normalized for height), relative wall thickness (RWT), and LV end-diastolic diameter were significantly greater in obese children. The early to late relaxation velocity ratio (E/A) determined by TDI for the right ventricle and interventricular septum (IVS) were significantly lower in the obese group, LVM/ht2.7 and RWT correlated with body mass index (BMI). In the obese group, IVS and LV posterior wall thickness correlated with insulin, very low density lipoprotein, and triglyceride levels. However, we failed to show this correlation when these measurements were indexed to height. CONCLUSION: Left ventricular hypertrophy, as evidenced by increased LV mass, was present in obese children. Higher BMI, insulin, very low density lipoprotein, and triglyceride levels were associated with LV hypertrophy. TDI revealed subclinical changes in diastolic function of the right ventricle and IVS.

Oral versus intranasal midazolam premedication for infants during echocardiographic study.

Movement and anxiety during echocardiographic study may reduce the reliability and affect the quality of echocardiographic images. Thus, sedation is an essential component when it is performed in infants. This randomized, single-blinded, placebo-controlled study was undertaken to evaluate the acceptability and effectiveness of intranasal midazolam (INM) versus oral midazolam (OM) in infants during transthoracic echocardiography. Eighty patients between the ages of 6 mo and 3 y who presented for elective echocardiographic study were divided into 3 groups: the OM group received 0.4 mg/kg of injectable midazolam mixed with an equal volume of cherry juice, the INM group received 0.2 mg/kg as drops,and the control group was given oral cherry juice or intranasal serum physiologic. A blinded clinician assessed and scored the level of sedation and comfort during the procedure for each child, and a score for ease of administration was recorded by the nurse. The intranasal route was more acceptable to infants than the oral route (P<.001). No significant difference in the effects of sedation was observed between the OM group and the INM group (P=.583), but significant differences were observed between the sedated groups and the control group (P<.001). The procedure was significantly more comfortable in groups given OM and INM than in the control group (P<.001). Although no difference in sedation score was seen between the oral and nasal routes, INM was better accepted by infants than OM. Echocardiography was performed more reliably and comfortably in those given midazolam than in those in the control group.

[Assessment of atrial pathologies in children using transesophageal echocardiography]. / Cocuklarda Atriyuma Ait Patolojilerin Transözofajiyal Ekokardiyografi ile Degerlendirilmesi.

OBJECTIVE: Transesophageal echocardiography (TEE) is indicated for suspected atrial septal pathology and for monitoring of interventional procedures such as an atrial septal defect (ASD) closure during cardiac catheterization. Transesophageal echocardiography also helps to demonstrate postoperative complications and residual defects of complex congenital cardiac anomalies. METHODS: Transesophageal echocardiography was performed in 112 pediatric patients with or suspected atrial pathology at our institution between 1999-2002, using the standard techniques. The mean age was 8.7+/-4.2 years. RESULTS: In 45 of 112 children the suspected atrial defects were confirmed with the TEE. Patent foramen ovale was correctly predicted in 13.4% of patients by TEE, but only in 8.7% of patients by echocardiography. Multiple ASD's were correctly defined in 4.1%, and high venosus defects were documented in 6.1% of children by the TEE. We used TEE in 13% of patients for detecting atrial vegetations in patients with possible endocarditis, and evaluation of the postoperative care of atrial surgery such as Fontan or Senning operations and total correction of abnormal pulmonary venous return. Successful transcatheter closure of 7 ASD's was accomplished under TEE guidance. CONCLUSION: Transesophageal echocardiography allows a much more detailed evaluation of atrial morphology than transthoracic echocardiography even in infants. Transesophageal echocardiography is also indicated during interventional procedures and postoperative evaluation of the atrial pathology.

Frequency, origins and courses of anomalous coronary arteries in 607 Turkish children with tetralogy of Fallot.

BACKGROUND: The aim of this study was to investigate the incidence, origins and courses of coronary artery anomalies using a combination of angiographic and surgical methods in Turkish children with tetralogy of Fallot (ToF). METHODS: Seventy-seven patients in whom coronary artery anomalies had been identified by angiography and/or at operation out of 549 ToF and 58 Fallot-type double outlet right ventricle (total 607) patients, were enrolled in the study. RESULTS: Coronary artery anomalies were identified in 12.7% of the patients. The incidence was 12.2% (67/549) in patients with aortic overriding 50%, and 17.2% (10/58) with aortic overriding 〉 50% (p 〉 0.05). The incidence of anomalous coronary arteries crossing the right ventricular outflow tract (RVOT) was 7.91%. The commonest anomaly was the left anterior descending artery (LAD) or accessory LAD arising from the right coronary artery (RCA; n = 25). Other frequent anomalies were single coronary ostium (n = 21) and enlarged conal branch of RCA (n = 18). In 62.3% (48/77) of the patients with a coronary anomaly, the anomalous vessels were crossing the RVOT. The ratio of crossing the RVOT was 92.0% for LAD arising from the RCA, 66.7% for conal branch, and 42.9% for single coronary ostium. CONCLUSIONS: Two thirds of the anomalous coronary arteries were crossing the RVOT, and had surgical importance. The most frequent coronary artery anomaly that crossed the RVOT was the LAD or the accessory LAD arising from the RCA. Also, an enlarged conus artery should be considered as an anomaly because of its surgical importance, given its high rate of crossing the RVOT.