Anomalous origin of coronary arteries from the pulmonary artery: A single-center experience.

Background: The aim of the study was to evaluate the clinical and diagnostic findings, treatment, and follow-up of cases of anomalous origin of coronary arteries from the pulmonary artery. Methods: Between January 1998 and June 2021, a total of 14 patients (5 males, 9 females; median age: 15 months; range, 3 to 156 months) diagnosed with anomalous origin of coronary arteries from the pulmonary artery were retrospectively analyzed. Demographic and clinical data of the patients, electrocardiographic, echocardiographic, angiographic, surgical, and follow-up findings were evaluated. Results: The most common symptoms were respiratory distress (n=6) and murmur (n=3). With the exception of three cases, all other patients were diagnosed by echocardiography in the first examination. Severe mitral valve insufficiency was detected in four patients and four other patients had moderate mitral insufficiency on echocardiography. Ejection fraction values ranged between 38 and 79%. Eleven patients underwent direct implantation of the coronary artery into the aorta, and three underwent a Takeuchi procedure. Mortality occurred in only one case. After surgery, mitral insufficiency and ejection fraction values improved. Median follow-up was 62 (range, 5 to 170) months and all patients were asymptomatic, except one who required redo surgery. Conclusion: Anomalous origin of the coronary arteries from the pulmonary artery is an uncommon congenital anomaly. Echocardiography is the main diagnostic tool. If all echocardiographic findings of the coronary anomaly are not investigated thoroughly, the diagnosis may be overlooked.

Incidence and clinical characteristics of acute rheumatic fever in Turkey: Results of a nationwide multicentre study.

AIM: To evaluate the incidence and clinical features of acute rheumatic fever (ARF) in Turkey, following the revised Jones criteria in 2015. METHODS: This multicentre study was designed by the Acquired Heart Diseases Working Group of the Turkish Pediatric Cardiology and Pediatric Cardiac Surgery Association in 2016. The data during the first attack of 1103 ARF patients were collected from the paediatric cardiologists between 1 January 2016 and 31 December 2016. RESULTS: Turkey National Institute of Statistics records of 2016 were used for the determination of ARF incidence with regard to various cities and regions separately. The estimated incidence rate of ARF was 8.84/100 000 in Turkey. The ARF incidence varied considerably among different regions. The highest incidence was found in the Eastern Anatolia Region as 14.4/100 000, and the lowest incidence was found in the Black Sea Region as 3.3/100 000 (P < 0.05). Clinical carditis was the most common finding. The incidence of clinical carditis, subclinical carditis, polyarthritis, aseptic monoarthritis, polyarthralgia and Sydenham's Chorea was 53.5%, 29.1%, 52.8%, 10.3%, 18.6% and 7.9%, respectively. The incidences of clinical carditis, subclinical carditis, polyarthritis and polyarthralgia were found to be significantly different among different regions (P < 0.05). CONCLUSION: The findings of this nationwide screening of ARF suggest that Turkey should be included in the moderate-risk group.

What are the non-cardiac prognostic factors affecting mortality in neonates with aortopulmonary shunt.

Background/aim: Systemic to pulmonary shunts (SPS) have proven to be highly effective for the palliation of neonates with cyanotic congenital heart disease. Mortality after SPS surgery in neonates has multifactorial basis. We aimed to investigate the clinical results of the SPS in relation to the underlying cardiac disease and to identify the risk factors contributing to an adverse outcome.Material and method: All neonates who underwent first shunt insertion for cyanotic congenital heart disease during the study period from 1 January 2014 to 31 December 2017 were included. A retrospective review of patient records was done. Patients were grouped into two different categories: survived with or without any reintervention and death before or after any reintervention till discharge.Result: During the study period, 47 patients underwent SPS shunt placement. Patients who survived with or without any reintervention were in Group 1 and patients who died before or after any reintervention till discharge were in Group 2. Preoperative epinephrine requirement and mechanical ventilation and postoperative erythrocyte transfusion need were statistically significant.Conclusion: Although primary cardiac pathology is the most important prognostic factor, some other preoperative and postoperative factors like preoperative epinephrine requirement, and postoperative erythrocyte transfusion might also affect the prognosis. As there are very few centers in the region that specialize in pediatric cardiac surgery, a multicenter approach will be helpful in reaching reliable conclusions.

Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

Togetherness of Ebstein anomaly and giant hairy nevus in a neonate: first case in the literature.

Özlü F, Tülüce M, Satar M, Özbarlas N, Kozanoglu B, Yücel A. Togetherness of Ebstein anomaly and giant hairy nevus in a neonate: first case in the literature. Turk J Pediatr 2018; 60: 744-746. Ebstein anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced through the apex of the right ventricle. Giant congenital melanocytic nevi originate from melanocytes. It is usually seen after birth. Here we present the first neonate with prenatally diagnosed with Ebstein anomaly with giant hairy nevus in the literature.

Closure of patent ductus arteriosus in children, small infants, and premature babies with Amplatzer duct occluder II additional sizes: multicenter study.

OBJECTIVES: To evaluate safety and efficacy of closure of patent ductus arteriosus (PDA) with Amplatzer duct occluder II Additional Sizes (ADO II AS) and to report early and midterm results of the device in children and very young symptomatic infants. METHODS: Retrospective analysis of angiographic data of 60 children from four pediatric cardiology centers. RESULTS: The median patient age and weight were 6.5 (0.5-168) months and 6.8 (1.19-57) kg, respectively. In the study, 26 children had a body weight of ≤ 6 kg. Of these 26 children, 9 had a body weight of ≤ 3 kg. The median narrowest diameter of PDA was 2 (1.2-4) mm. Ductal anatomy was Type A in 29, Type B in 2, Type C in 11, Type D in 1, and Type E in 16 patients, and a residual PDA after surgery in 1 patient. Closure with ADO II AS was achieved in 58 (96.6%) of 60 attempted cases. In two infants, the device was not released because of significant residual shunt. ADO II was used in one, and the other was sent to surgery. Complete closure was observed in all ADO II AS deployed children by the next day on echocardiography. Median follow-up was 12 (1-18) months. Neither death nor any major complications occurred. CONCLUSIONS: Our study shows that closure of medium and small sized PDA by using ADO II AS device is effective and safe in children. The use of the device will expand the field of application of PDA closure in small infants.

Transjugular approach to device closure of atrial septal defect in a child with heterotaxia and interrupted inferior vena cava.

In heterotaxia syndrome with left atrial isomerism, the distinguishing feature is interrupted inferior vena cava with azygos continuation. We report using a transjugular approach to device closure of an atrial septal defect in an 8-year-old boy with heterotaxia syndrome. We found that device closure of the child's atrial septal defect through a jugular venous approach was safe when an inferior vena cava approach was not possible. To our knowledge, ours is the first report of the use of internal jugular vein access to close an atrial septal defect in a child.

[Long term follow-up of 799 children with isolated ventricular septal defects]. / Izole ventriküler septal defektli 799 çocugun uzun süreli takibi.

OBJECTIVES: We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD). STUDY DESIGN: The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months). RESULTS: The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure. CONCLUSION: Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.

A newborn infant with intrapericardial rhabdomyosarcoma: a case report.

Cardiac tumors are uncommon in neonates and most of them are histologically benign. The most common cardiac tumor in neonates and infants is rhabdomyoma. Malignant cardiac tumors are considerably rarer, and rhabdomyosarcoma (RMS) is the leading malignancy. To our knowledge, only one case of intrapericardial RMS was reported in the literature, in a seven-month-old baby. Here we present another newborn baby with intrapericardial RMS.

Systemic pulmonary shunt performed with Shelhigh internal mammary artery: early results.

OBJECTIVE: The purpose of this study was to evaluate early findings for aortopulmonary shunts using bovine internal mammary artery grafting. METHODS: Bovine internal mammary artery grafts biomodified with glutaraldehyde and the Shelhigh No-React process were used between May 2005 and April 2006 in our clinic for 20 cases of aorta-pulmonary artery shunts. We implanted 2 sizes of grafts, either a graft with 4-mm proximal and 5-mm distal diameters or a graft with 5-mm proximal and 6-mm distal diameters. Patients were between 20 days and 7 years of age, and the sex distribution was 55% female and 45% male. A Blalock-Taussig shunt with left thoracotomy was performed in patients 2 years of age and older, and a central shunt with sternotomy was performed for patients younger than 2 years. Eight patients underwent operation under emergency conditions. Nine patients had tetralogy of Fallot and/or pulmonary atresia (PA); 3 had transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (PS); 3 had tricuspid atresia; 3 had PS and double-inlet left ventricle; and 2 had PA. RESULTS: After the operation, immediate recovery of oxygen saturation and partial oxygen pressure was observed in all patients. Pulse oximetry measurements showed patient oxygen saturation to be between 84% and 100%. One patient underwent reoperation at the third postoperative hour because of bleeding. Two patients died from causes unrelated to the graft. The hospitalization period was between 7 and 29 days. Echocardiography evaluations showed no shunt obstruction for the early (first postoperative week) or middle (postoperative week 24) period. CONCLUSION: With this study, we assessed the use of the Shelhigh internal mammary artery graft instead of synthetic (polytetrafluoroethylene) tubular grafts in shunt operations for congenital heart diseases with decreased pulmonary blood flow and no evidence of calcification. Further investigation involving a larger number of cases and longer-term results for patency are needed to confirm our findings.

A novel approach for off-pump atrial septostomy applications.

Atrial septostomy or septectomy are required to enable atrial mixture in various congenital cardiac lesions. The aim of this article was to introduce a technique where atrial septostomy application could be employed off pump with the aid of a new device. To our knowledge this is the first report for this technique in the literature. We report the results of 7 patients for whom we employed our technique successfully using a new combined device as an alternative to traditional methods. The major advantage of our approach was avoiding detrimental effects of cardiopulmonary bypass applications.

Repair of aortic coarctation and aneurysm of ductus arteriosus in the presence of right-sided arch and descending aorta.

Association of right aortic arch and coarctation of the aorta is rare. A patient with aortic coarctation was presented to our clinic. Detailed radiological work-up revealed aortic coarctation, aneurysm of ductus arteriosus, mirror image brachiocephalic vessels, right-sided arch, and right-sided descending aorta. The patient underwent successful operative repair through a right thoracotomy. She is normotensive and doing well two years postoperatively.

Congenital aorto-left atrial tunnel--an unusual communication: a case report.

Congenital aortico-cameral communications are rarely seen. We present an asymptomatic patient in whom there was a congenital vascular communication rising from the aortic root and terminating in the left atrium. She had an atrial septal defect (ASD). The diagnosis was made with echocardiography and confirmed by aortography. She was treated by closing the tunnel and the ASD. The outcome was satisfactory in this extremely rare case of a congenital cardiac lesion. Coincidental diagnosis could be made during careful echocardiographic examination.

Right pulmonary artery--left atrial communication presenting with brain abscess: a case report.

Direct communication between the right pulmonary artery and left atrium is a very rare vascular malformation. We report a patient with this anomaly. She presented with unexplained cyanosis and brain abscesses. The diagnosis was made with contrast echocardiography and angiography. We treated this anomaly successfully with surgery. Complete cure for this anomaly can be achieved by ligation.

Mitral annular calcification and brown tumor of the rib in a child with chronic renal failure.

Mitral annular calcification and liquefaction necrosis of this lesion mimicking intracardiac tumor because of secondary hyperparathyroidism have been described in adult patients with chronic renal failure, but have not been reported in children. Chronic renal failure is one of the predisposing factors of this condition. We report the case of a 13-year-old patient with continuous ambulatory peritoneal dialysis with severe hyperparathyroidism who was found to have intracardiac and rib lesions considered to be brown tumors.