Paternal peri-conceptional physical activity and the risk of congenital heart disease in offspring: A case-control study.

BACKGROUND: Genetic and environmental factors have been shown to contribute to the development of congenital heart disease (CHD). To date, the focus of scientific articles has primarily centered on genetics and maternal environmental factors, with comparatively less attention given to paternal risk factors. OBJECTIVES: This study aims to investigate the potential association between paternal pre-conceptional physical activity levels (PA), along with paternal peri-conceptional smoking and alcohol consumption, and the risk of CHD in offspring. MATERIALS AND METHODS: An observational case-control study was conducted in Lebanon, with 279 participants, aiming to investigate potential risk factors for CHD. We included children with confirmed CHD, born between 2012 and 2022. Controls born in the same timeframe were selected randomly from the general population using online questionnaire forms. Mean age of children included was 6 years old (0-10). The pre-conceptional PA was assessed using the Global Physical Activity Questionnaire validated in Arabic. In addition, paternal smoking, alcohol consumption, and maternal risk factors were collected. RESULTS: The study included 128 CHD cases (45.9%) and their parents, as well as 151 healthy infants (54.1%) and their parents. There were no statistically significant variations in the alcohol consumption noted between the fathers in the case and control groups (p = 0.18). The paternal involvement in recreational-related PA during the peri-conception period was associated with a reduced risk of the CHD development in offspring by 46.9% (OR = 0.531, 95% CI: 0.301-0.936, p = 0.029). Additionally, increasing paternal total sitting time by 1 h above the average, which was approximately 260 min (4 h), increased the risk of CHD in offspring by 0.4% (p = 0.001). Moreover, paternal smoking exhibited an apparent association with a 56% increased risk of offspring developing CHD, notwithstanding that the confidence intervals included the null (OR = 1.56, 95% CI: 0.86-2.8, p = 0.136). DISCUSSION AND CONCLUSION: This observational study is the first to report a potential association between paternal PA, and CHD in offspring. This study aligns with previous reports, advocating for the paternal engagement in PA and the adoption of healthy lifestyle habits, especially during the critical stages of conception. Such practices are strongly recommended to enhance fertility and promote optimal health for offspring. However, due to the subjectivity in reporting PA and lack of molecular proof, additional prospective and molecular studies are required to validate these findings.

Word of caution: silent late device embolisation after perimembranous ventricular septal defect closure in a 6-Kg infant.

We report on a 6-month-old infant (6 Kg/ 64 cm) with perimembranous ventricular septal defect (absent sub-aortic rim, 10 mm left ventricular entry, and 4 and 6 mm right ventricular exists) and successful retrograde closure using an 8x6 mm KONAR-MF™ VSD occluder (Lifetech, China). Immediate and 48 hours post-procedure ultrasounds showed an accurately positioned device and two jets of mild-to-moderate residual shunts. At the 2-week follow-up, the device did not change position and the shunt was stable and intra-prosthetic. The scheduled 3-month follow-up was skipped for familial reasons. The patient came back without alarming symptoms for the regular 6-month follow-up, and the device was found embolised to the left pulmonary artery. The device was retrieved surgically, and the defect was patch-closed with excellent outcomes. There was a pseudoaneurysm involving the tricuspid valve chordae and the device was endothelialized partially on one edge suggesting that embolization occurred somewhere between 3 months and 6 months post-operative. Defects with compromised anatomies should be closed surgically to avoid suboptimal results, especially in small infants.

Comparative outcomes of two competitive devices for retrograde closure of perimembranous ventricular septal defects.

Background: Retrograde closure of perimembranous ventricular septal defects (pmVSDs) is a well-established procedure. However, interventionists are still looking for the best closure device. Methods: We performed a single-center retrospective review of 5-year-experience (from July 2015 to July 2020) with retrograde closure of pmVSDs using AmplatzerTM Duct Occluder II (ADOII) and KONAR-MF™ VSD occluder (MFO). Deficient sub-aortic rim (SAR) (≤2.5 mm for MFO and ≤3 mm for ADOII) was an exclusion criterion in defects with a diameter ratio (right-side exit/left-side entry) > 0.5. Results: We identified 77 patients (57.1% males) with a median age of 4.3 years (IQR, 2.2-8.3) and a median weight of 16 kg (IQR, 11.2-24.5). 44 (57.1%) defects (22.7% with deficient SARs) with a median left-side defect diameter of 8.7 mm (IQR, 5.7-10) were closed with ADOIIs. 33 (42.9%) defects (51.5% with deficient SARs) with a median left-side defect diameter of 10.8 mm (IQR, 8.8-13.5) were closed with MFOs. One 7/5 MFO was removed before release and upsized to a 12/10 MFO. Implantation success rate was 100% with ADOII and 90.9% with MFO devices. Two MFOs were snare-recaptured after embolization, and one 9/7 MFO was snare-retrieved for a new onset of grade-2 aortic regurgitation that persisted afterward. Median follow-up was 3.3 years (IQR, 2.1-4.2) for ADOII and 2.3 years (IQR, 1.7-2.5) for MFO. No permanent heart block or death occurred. Freedom from left ventricular dilation was 94.62% at 36 months of follow-up. Freedom from residual shunt was 90.62% for MFO and 89.61% for ADOII at 24 months of follow-up. One 2.6-year-old patient with baseline mild aortic valve prolapse and trivial aortic regurgitation developed a grade-2 aortic regurgitation after 9/7 MFO implantation. He was treated surgically after two years without device extraction. One new grade-2 asymptomatic tricuspid regurgitation persisted at the last follow-up in the ADOII group. Conclusions: ADOII and MFO are complementary devices for effective retrograde closure of pmVSDs in children, including defects with absent or deficient SAR. ADOII is limited to smaller defects but offers a lower profile and a flexible left-side disk for better maneuverability over the aortic valve during retrograde implantation.

Three centers experience with device closure of congenital Gerbode-type perimembranous ventricular septal defects.

OBJECTIVES: We aim to evaluate our experience with interventional closure of Gerbode-type perimembranous ventricular septal defects (pmVSDs). METHODS: We performed three-center retrospective data review of patients with congenital indirect Gerbode-type pmVSDs treated percutaneously between August 2017 and May 2021. Standard safety and latest follow-up outcomes were assessed. RESULTS: Ten patients (six females) were identified with a median age of 6.8 years (range: 2.5-54) and a median weight of 26.5 kg (range: 12-88). The median left ventricular defect size was 10 mm (range: 3-15.5). On baseline ultrasound, 6 patients had absent subaortic rim , 6 patients had trivial aortic regurgitation, and 3 patients had tear-drop-type (small) aortic cusp prolapse. The tricuspid regurgitation was graded II (n = 5) and III (n = 5). Five Lifetech Konar-Multifunctional occluders, four Amplatzer duct occluders II and one Amplatzer duct occluder I were implanted. The median fluoroscopy time was 10.4 min (range: 4.3-20.2). Pre-existing aortic regurgitations remained identical. One new aortic regurgitation was identified before discharge and remained trivial after 48 months of follow-up. No heart block or tricuspid stenosis was observed on a median follow-up of 17 months (range: 3-48). All patients are symptom-free with complete shunt closure and significant regression or resolution of tricuspid regurgitation. CONCLUSIONS: Despite anatomical challenges, interventional closure of congenital indirect Gerbode-type pmVSD appears to be feasible, safe, and most importantly clinically effective using different commercially available devices. Amplatzer duct occluder II and Lifetech Konar-Multifunctional occluder offer interesting specifications to retrogradely target this specific defect with success.

Word of caution: Severe aortic valve injury linked to retrograde closure of perimembranous ventricular septal defects.

Retrograde device closure of perimembranous ventricular septal defects (pmVSDs) is an interesting procedure with several technical advantages and encouraging follow-up results. Nevertheless, aortic valve injuries may occur and require sufficient attention. Herein, we report the first two cases of severe aortic regurgitation that we have linked to the retrograde pmVSD closure with the KONAR-MF™ VSD occluder. We detail the technical aspects, comprehensively discuss procedural errors and conclude with important learning points.

Off-label use limitation of the Super Arrow-Flex® sheath introducer in congenital heart disease interventions.

Many interventionists are infatuated by the recent resurgence of the coilwire design with the Super Arrow-Flex® sheath (Teleflex, Inc., NC, United States of America). This exclusive sheath is a highly flexible, durable, conduit intended for use in diagnostic and interventional procedures with several advantages and maximum effectiveness in challenging cases. We report failure to easily advance memory shape occluders through Super Arrow-Flex® sheaths larger than the recommended implant French size. We detail the technical reasons behind this non-previously reported drawback and describe benchside tests as possible solutions.

Unusual Findings in Down's Syndrome: Hand Agenesis and Hypospadias.

A baby with Down syndrome presented initial findings at the first-trimester ultrasound of increased nuchal thickness and unilateral hand agenesis. During follow-up, other elements were found mainly hypospadias. This report emphasizes through prenatal and postnatal imaging the phenotypic variability of Down syndrome babies.

Transcatheter closure of congenital Gerbode-type ventricular septal defect using the new multifunctional occluder device.

The KONAR-Multifunctional™ VSD Occluder (MFO) (Lifetech) is one of the most recent additions to the armamentarium of transcatheter therapies. The device offers superior technical features and experienced interventionists having been using it in risky and complex anatomies. Herein, we report the first use of the MFO device to close a congenital Gerbode-type perimembranous ventricular septal defect in an 18-year-old girl. The device was retrogradely implanted into proper position under ultrasound and fluoroscopic guidance. Immediate and 12-month-follow-up confirmed successful outcomes with complete shunt closure and resolution of tricuspid regurgitation.

Optimal management of pulmonary atresia with intact ventricular septum in a developing country: the art of pulmonary valve mechanical perforation in the era of CTO hardware.

BACKGROUND: Transcatheter valve mechanical perforation (TVMP) in pulmonary atresia with intact ventricular septum (PAIVS) is an acceptable yet challenging alternative to radiofrequency. AIMS: To evaluate and compare safety, feasibility, and efficiency of two TVMP techniques. METHODS: Clinical data of neonates with PAIVS who underwent an attempt for TVMP between 2009 and 2019 were retrospectively reviewed. Patients were divided into two groups according to perforation technique: using the stiff end of a percutaneous transluminal coronary angioplasty (PTCA) ordinary 0.014" wire (group A) and subsequently with the floppy tip of a chronic total occlusion (CTO) guidewire (group B). The technical aspects, procedural and discharge outcomes of both groups were compared. RESULTS: A total of 19 antegrade TVMP procedures (Group A, n=10, and Group B, n=9) were attempted in 18 neonates with an overall success rate of 73.7% and no procedure-related mortality. Groups' analysis showed that the introduction of CTO hardware maximized procedure success rates (P=0.002) with zero failure and misperforations (P=0.022). The significant drop in perforation time (P < 0.001) and irradiation exposure (P=0.006) allowed additional ductal stenting during the same procedure, optimizing patients' clinical outcomes and shortening overall hospital stay. Discharged patients had room air mean oxygen saturation of 91.4% (± 5.5) with no evidence of heart failure. CONCLUSIONS: In selected cases of PAIVS, TVMP using CTO wires is a safer, highly efficient, and simplified alternative to other mechanical perforation techniques. It substantially revolutionized the management of PAIVS in our center optimizing short-term prognosis.

Percutaneous closure of ventricular septal defects in children: key parameters affecting patient radiation exposure.

BACKGROUND: Ventricular septal defect (VSD) transcatheter closure is gaining worldwide popularity despite its complexity. Reports on key factors affecting radiation exposure in children are scarce. AIMS: This clinical study is the first to comprehensively analyze the impact of all relevant parameters on children's radiation exposure during VSD interventional closures. METHODS: Between March 2016 and August 2019, all pediatric VSD cases percutaneously treated at a reference center for interventional congenital cardiology and equipped with a single-plane Innova 2100 X-ray unit were retrospectively reviewed. Multiple linear regression was performed to investigate the impact of clinical, technical, and procedural parameters on patients' radiation exposure assessed using total air kerma area product (PKA,T). RESULTS: A total of 85 patients were included in this study and 82.4% had perimembranous defects. Device implantation was successful in 96.5% of cases. The procedure lasted for a median of 60 min with a median PKA,T of 19.6 Gy.cm2 (range, 1.1 to 244.8 Gy.cm2). Patients' weight (B = 1.679, P = 0.01), number of operators (B = 1.561, P = 0.02), device positioning complexity (B = 2.381, P = 0.002), and procedural incidents (B = 2.096, P = 0.008) significantly increased PKA,T. Patients' age (B = 1.053, P = 0.784), device design (B = -1.216, P = 0.780) and approach of delivery (B = -1.119, P = 0.511) did not significantly affect PKA,T. CONCLUSIONS: Radiation exposure in children undergoing VSD percutaneous closure was highly variable. A higher patient's weight, numbers of operators, complexity in device positioning, and procedural incidents, were identified as key factors increasing patient dose for this kind of intervention.

Not just another large atrial septal defect: complex anatomy, challenging procedure, and an unusual complication.

We report the case of a 59-year-old patient with a complex atrial septal defect in whom a 40-mm Amplatzer™ septal occluder was surgically extracted 50 days following implantation. Deployment manoeuvres were challenging leading to an immediate pericardial effusion that was closely monitored and uneventfully drained after 11 days. A dry pericardium was documented until 4 weeks of outpatient routine follow-up. However, the device was surgically explanted 2 weeks later, when an urgent chest computed tomography performed for worrisome symptoms showed pericardial effusion recurrence with peripheral contrast enhancement. Surprisingly, the surgical view showed a well-positioned device and an intact pericardium. We discuss the atypical sequence of clinical findings misleading our clinical judgement and precipitating surgery.

Endovascular stent implantation for aortic coarctation: parameters affecting clinical outcomes.

OBJECTIVE: To evaluate safety and efficacy of endovascular stenting for aortic coarctation (AC) and to explore the effect of clinical parameters and stent characteristics on outcomes. MATERIAL AND METHODS: Clinical data of all patients with AC who had attempted transcatheter stenting between 2004 and 2019 were retrospectively reviewed. Eligible patients had native or recurrent AC with systemic arterial hypertension and resting arm-leg pressure gradient > 20 mmHg. Exclusions included distance between takeoff of cervical arteries and stenotic aortic lesion < 10 mm, contraindication to antithrombotic therapy, bodyweight < 25 kg, and secondary hypertension. RESULTS: A total of 20 patients (75.0% with native lesions) were included with a mean age of 18.4 years and a mean bodyweight of 59.2 kg. Procedure was successful in 90.0% of cases with an immediate drop in the invasive pressure gradient across lesions. On a median follow-up of 12 months (range, 8 to 144.9 months), coarctation reoccurred in five patients, but four of them required intervention after a median of 104.4 months with successful outcomes. Cheatham Platinum stents were significantly associated with lower rates of recoarctations and reinterventions. At the latest follow-up, three out of six patients with persistent hypertension had no recoarctation. Analysis showed that the need for antihypertensive therapy was not influenced by clinical parameters, aortic arch geometry, or stent characteristics. CONCLUSION: Treating AC with stent implantation is a safe and successful procedure. Using Cheatham Platinum stents appears to be associated with better outcomes. The persistence of arterial hypertension despite successful stenting remains a complex and challenging phenomenon.

Percutaneous closure of restrictive-type perimembranous ventricular septal defect using the new KONAR multifunctional occluder: Midterm outcomes of the first middle-eastern experience.

OBJECTIVES: To evaluate the safety, efficiency, and midterm outcomes of transcatheter perimembranous ventricular septal defect (pmVSD) closure using the new KONAR-MF™ VSD occluder. BACKGROUND: Off-label percutaneous pmVSD closure is a well-established procedure with promising results. Yet, interventionists are still searching for the ideal device. METHODS: Between June 2018 and March 2019, 20 patients with hemodynamically significant but restrictive-type pmVSD underwent an attempted transcatheter closure using the new KONAR-MF™. All implantations were performed retrogradely under general anesthesia, transoesophageal echocardiography, and fluoroscopic guidance. Prospective follow-up using transthoracic echocardiography and electrocardiogram was done until August 2019. RESULTS: The median age was 6.4 years (8 months to 43.4 years), and the median body weight was 17.3 (9-74) kg. The mean defect size on the left ventricular aspect was 11.7 ± 2.8 mm. All devices were successfully and rapidly implanted. One device embolized in the pulmonary artery, 24 hr after implantation and was percutaneously retrieved with no complication. Over a mean follow-up period of 8.2 ± 3.0 months, complete closure was achieved in 84.2% of patients. One new onset of mild aortic regurgitation was detected before discharge and remained stable. Tricuspid valve regurgitation, complete heart block, major complication, and death were not observed. CONCLUSIONS: Transcatheter pmVSD closure using the KONAR-MF™ can be successfully performed in adult and pediatric patients. It is a safe and promising device, designed to provide high conformability to septal defects with a lower risk of heart block. Retrograde implantation allows procedural flexibility, efficient deliverability, and control of valvular interferences.

Diagnostic Reference Levels, Deterministic and Stochastic Risks in Pediatric Interventional Cardiology Procedures.

To establish diagnostic reference levels (DRLs) and investigate deterministic and stochastic risks in pediatric interventional cardiology (IC) procedures. Exposure parameters were retrospectively reviewed for 373 patients treated between May 2016 and November 2018 at a single specialized hospital. Weight specific DRLs were derived for pediatric IC procedures. Additionally, peak skin dose (Dskin,max) was measured using thermoluminescent dosimeters for a sample of 7 diagnostic and 43 therapeutic procedures. Finally, using PCXMC software, organ doses were computed and the risk of exposure-induced cancer death (REID) was estimated using the risk models of the Biological Effects of Ionizing Radiation VII committee. DRLs for ventricular septal defect (VSD) occlusions, lacking in the literature, in terms of air kerma at patient entrance reference point (388 and 629 mGy) and total air kerma-area product (28 and 61 Gycm) were proposed for patients weight-groups 5 - < 15 kg and 15 - < 30 kg, respectively. The mean (range) Dskin,max was 15 (1-30) mGy and 94 (1-491) mGy for diagnostic and therapeutic procedures, respectively. Meanwhile, VSD occlusion involved the highest organ doses where the lungs, liver, stomach, and breasts mean doses were 57, 37, 6, and 10 mGy, respectively, and the associated REID were 0.5% and 0.3% in female and male patients, respectively. DRLs were proposed for pediatric IC procedures; these will help optimize patient exposure. Dskin,max values were lower than the 2 Gy threshold for skin injuries. Pediatric organ doses and the REID were the highest during VSD occlusion and may be critical for repetitive procedures.

Device Closure of Perimembranous Ventricular Septal Defect: Choosing Between Amplatzer Occluders.

Background: Off-label device closure of perimembranous ventricular septal defect (pmVSD) is well reported in the literature with encouraging results. However, technical challenges may be encountered. Objectives: To evaluate and compare feasibility, technical aspects, procedural outcomes, and mid-term follow-up of pmVSD closure using Amplatzer™ occluders. Patients and Methods: From July 2015 to July 2018, patients in whom pmVSD closure was attempted using an Amplatzer occluder were retrospectively identified from our institution's database. Device selection was made according to the defect anatomy that was obtained via ventriculography and trans-esophageal echocardiography. Follow-up evaluations were done at discharge, then at 1, 3, 6, and 12 months and yearly thereafter with transthoracic echocardiography and electrocardiogram. Results: In total, 8 Amplatzer Duct Occluder (ADO), 27 ADO II, and 17 Amplatzer Muscular VSD Occluder (AMO) were used in 51 patients with a mean age of 7.4 ± 6.9 years and a mean weight of 25.4 ± 19.8 kg. Implantation was successful in 50/51 patients (98.0%). There was no procedure related mortality. One ADO accidentally embolized to the aorta after release and was surgically recaptured from the iliac artery. All ADO II were delivered retrogradely with the least amount of time (p = 0.002) and the lowest radiation exposure (p < 0.001). Minor valvular disturbances occurred in 8/49 patients (16.3%), including five tricuspid regurgitation (three with ADOII and two with AMO) and three trivial aortic regurgitations (two with ADO and one with ADOII). On a median follow-up of 194 days (range, 60-895 days), no surgical device removal was necessary. At 6 months of follow-up, trivial residual shunt was present in 5/49 patients (10.2%), among which none occurred with ADO. One complete atrioventricular block was detected 18 months after ADO implantation and required permanent pacing. Conclusions: Transcatheter closure of PmVSD using Amplatzer occluders is feasible, safe and efficacious in properly selected patients. The major key factor behind high procedural success rate is proper device selection. ADOII is remarkably superior in terms of device softness, flexibility and faster implantation process. Yet, its use is limited to small defects with particular anatomy.

Ductal stenting to improve pulmonary blood flow in pulmonary atresia with intact ventricular septum and critical pulmonary stenosis after balloon valvuloplasty.

OBJECTIVE: To assess the feasibility, safety, and efficiency of ductal stenting in pulmonary atresia with intact ventricular septum or critical pulmonary stenosis after balloon pulmonary valvuloplasty. BACKGROUND: Ductal stenting in pulmonary atresia with intact ventricular septum is a re-emerging and promising technique. There is little data available on its outcomes after establishing prograde pulmonary blood flow. METHODS: We retrospectively reviewed all neonates with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis who underwent ductal stenting after balloon valvuloplasty. Ductal stenting was performed either in the same setting (group A) or a few days later after balloon valvuloplasty (group B). We compared the two groups. RESULTS: Eighteen coronary stents were transvenously delivered and successfully deployed in 18 newborns. There was no procedure-related mortality. The median hospital stay post-intervention was 6 days with a mean discharge oxygen saturation of 94%. Group A had a shorter overall hospital stay with a shorter overall time of irradiation but with a longer overall procedural time. On a follow-up of 18 months, no re-intervention for stent failure or overflow was undertaken. The median stent patency based on echocardiography was 12 months. CONCLUSION: Stenting the arterial duct in pulmonary atresia with intact ventricular septum or critical pulmonary stenosis is a feasible, safe, and efficient technique. It avoids surgery or long hospital stay with prostaglandin infusion. The minimal 6 months stent longevity provides a period of time long enough to decide whether the right ventricular diastolic function is normalised or Glenn surgery is still needed.

Catheter closure of a recanalized vertical vein after repair of total anomalous pulmonary venous connection.

The vertical vein is sometimes left open in repair of total anomalous pulmonary venous connection. It usually closes later but can remain patent leading to a significant shunt. We describe a recanalized vertical vein in a 7-year-old having undergone repair in infancy. It was closed using an Amplatzer device.

PULMONARY ARTERY ACCELERATED FLOW REVEALING HODGKIN'S LYMPHOMA.

We present a case in which transthoracic echocardiography was the first diagnostic tool to suspect mediastinal Hodgkin's lymphoma by revealing a change in the hemodynamic of left pulmonary artery flow, and it was used as a follow-up method for monitoring treatment efficacy by demonstrating a normalization of pulmonary artery hemodynamics.

[Supravalvular aortic stenosis with and without coronary lesions in pediatrics. A Lebanese study]. / STÉNOSE SUPRAVALVULAIRE AORTIQUE AVEC OU SANS LÉSIONS CORONAIRES EN PÉDIATRIE--ÉTUDE LIBANAISE.

OBJECTIVE: This study aims to analyze several parameters concerning the supravalvular aortic stenosis (SVAS) in children such as age of diagnosis, place of residence, the existence of a metabolic disorder or dysmorphic syndrome and possible damage to the coronary ostia, the means of diagnosis and outcome of these patients. MATERIALS AND METHODS: A large group of patients (2868) with congenital heart disease enrolled between 1 May 1999 and 30 April 2010 at the National Register of Pediatric and Congenital Heart Disease, Lebanese Society of Cardiology. RESULTS: SVAS were found in 14 patients (0.5%) aged 8.5 months to 15 years. The Williams Beuren syndrome was the most common etiology (6 cases) without ostial stenosis, asymptomatic and not treated, followed by forms without dysmorphic syndrome (5 cases) and without ostial stenosis, one patient was operated because of severe SVAS; finally, 3 cases of homozygous familial hypercholesterolemia treated differently: a patient had a successful liver transplantation at age of 4.5 years but has developed, despite the normalization of cholesterol level, a SVAS associated with severe ostial lesions 10 years after transplantation, another treated by coronary artery bypass graft surgery and the latter treated medically. CONCLUSION: SVAS is a very rare disease, but its discovery must lead to search for coronary lesions especially in presence of homozygous familial hypercholesterolemia. The ultrasound monitoring is mandatory and is designed to detect this anomaly and early coronary lesions. The slightest suspicion should carry out more extensive explorations to detect ostial stenosis.

FALSE AND TRUE ACUTE RHEUMATIC FEVER: A Lebanese Experience.

OBJECTIVES: The main aim of this study is to evaluate the significance of several clinical and laboratory findings presented by a large number of pediatric patients who were suspected to have acute rheumatic fever (ARF) by physicians who ordered echocardiogram for confirmation and for avoiding overuse of unnecessary antibiotics. These suspecting reasons include elevated ASO titers and recurrent tonsillitis. Another aim is to stress the role of Doppler echocardiogram in confirming the diagnosis of ARF by detecting rheumatic heart disease (RHD) in suspected cases. MATERIALS AND METHODS: 401 patients were registered in the National Registry of the Pediatric and Congenital Heart Disease (RNCPC) from April 1, 2002 until the end of March 2013 as suspected cases of ARF. The clinical reasons that brought suspicion are discussed and evaluated in accordance to modified Jones criteria. The patients are distributed into two groups: 1) confirmed ARF and 2) different diagnosis. All patients underwent echocardiogram. Patients came from different geographic areas and were referred by a variety of physicians. RESULTS: 48 patients out of 401 suspected cases turned to have confirmed ARF (12%). In this group, the predominant suspecting reasons are reported as heart murmur in 29/48 (60.4%), arthritis in 28/48 (58.3%), arthralgia with reduced physical activity in 15/48 (31%) and chorea in 5/48 (10%). In the different diagnosis group with a high ASO titer 174/353 (49.3%), isolated arthralgia 91/353 (25.8%) and combined elevated ASO with arthralgia in 44/353 (12.5%) were the predominant suspecting reasons. Doppler echocardiography could detect 38/48 cases of active carditis and subclinical carditis (SCC). CONCLUSION: Heart murmur, arthritis, arthralgia with reduced physical activity and chorea are the main clinical reasons that lead to suspicion in the ARF group. High ASO titers, arthralgia, and combination of high ASO titers with arthralgia are the main presenting symptoms in the group with a different diagnosis. We concluded that elevated ASO titer and normal ESR with arthralgia not reducing physical activity can exclude ARF. Doppler echocardiography is found to be a gold standard key to confirm acute rheumatic heart disease and ARF.