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Background: The aim of this study was to evaluate the efficacy and feasibility of a novel method of narrowing pulmonary arteries with catheter angiography using radiofrequency energy in rabbits. Methods: A total of nine New Zealand white rabbits weighing 3.0 to 4.2 kg each were utilized in this experimental study. After the location of pulmonary artery was confirmed by echocardiography and angiography, radiofrequency energy was applied by starting with 5 W energy for the first time and increasing by 5 W, if there was no rupture to a maximum of 20 W. Multiple applications of radiofrequency energy with different durations were performed at different levels of the pulmonary artery. Eight weeks later, surviving rabbits were sacrificed. The pulmonary arteries were removed for histological investigation. Results: Five rabbits remained alive after the experiment. There was a significant correlation between radiofrequency power and the degree of vessel thickness change in the pulmonary artery. The endothelial integrity of the tunica intima was impaired in all groups and the diameter of vessel was thickened by an average of 3.5 times. Conclusion: The narrowing of the pulmonary artery using radiofrequency energy was successfully performed in a small sample size of experimental animals in this study.

Coronavirus disease 2019 (COVID-19) is the greatest pandemic in a century. In this study, children with mild COVID-19 infections were evaluated at least 8 weeks after the polymerase chain reaction (PCR) test positivity, and the frequency of hypertension (HT) in these children was investigated. These results were compared to those of the control group, which consisted of 32 healthy children in the same age range as the trial group but who did not have COVID-19. This prospective multicenter study was conducted including children aged 10 to 18 years. There were 16 patients (16%) who had elevated systolic blood pressure, while 11 patients (11%) had stage-1 HT and 1 patient (1%) had stage-2 HT. It was revealed that children with COVID-19 had significantly higher systolic and diastolic blood pressures than normal healthy children. The findings of this study contribute toward increasing the understanding of the sequelae of mild COVID-19 in pediatric patients.

Danon disease is a rare and fatal disease caused by a mutation in the lysosome-associated membrane protein 2 gene. Impaired intracellular autophagy causes lysosomal vacuoles to accumulate mainly in myocardial and skeletal muscle cells, leading to hypertrophic cardiomyopathy, skeletal myopathy, and varying degrees of intellectual disability. Two distinct childhood presentations of Danon disease are described in this report.

In this study, we report our experience with the use of external loop recorders (ELRs), in terms of diagnostic efficiency according to symptoms and symptom-rhythm correlation in pediatric patients. We evaluated ELRs applied to 178 patients between April 2017 and November 2020 at our center. The mean age of 172 patients included in the study was 13.6 ± 3.8 years, and 69.8% were female. ELR indications were palpitations in 98 (56.9%) cases, chest pain and palpitations in 43 (25%) cases, presyncope/syncope in 28 (16.2%) cases, and pacemaker/ implantable cardioverter-defibrillator (ICD) problems in 3 (0.2%) cases. ELR recording times were 14.2 ± 9.7 days on average, ranging from 2 to 67 days. While the symptom-rhythm correlation was 29.1% in total, when the indications were evaluated one by one, this correlation was found to be 30.2% in palpitations, 34.7% in chest pain and palpitations, and 10.7% in presyncope/syncope. The total diagnostic efficiency was 68.1%. In the follow-up of ELR cases, a total of 139 (80.8%) patients received clinical follow-up without medication, 15 (8.8%) patients received medical treatment, and 18 (10.4%) patients underwent EPS. The cardiac ELR system is useful in detecting underlying arrhythmias. Demonstrating sinus tachycardia at the time of the symptom may be seen as negative finding, but while experiencing symptoms, it is diagnostically valuable and may help avoid further investigation with costly and invasive diagnostic procedures. For diagnostic efficiency and cost effectiveness, the optimal recording time is 2 weeks, but it should be extended to 4 weeks in cases such as of presyncope/syncope that cannot be explained with a 2-week ELR use.

IntroductionDiagnostic and interventional catheter angiography of the heart is frequently used in paediatric cardiology. It is also possible to detect urinary system anomalies with cineurography images that may be obtained during angiocardiography. In this study, the aim was to determine the frequency, distribution, and properties of urinary system anomalies accompanying heart diseases, and to find out the effectiveness of cineurographic images in detecting the urinary system anomalies.MethodsThe cineurographic images of 2022 children who had undergone angiocardiography between 1995 and 2015 were retrospectively examined.ResultsUrinary system anomalies were detected in 261 of the 2022 cases (12.9%). Of these 261 cases, 148 were males (56.7%), whereas 113 were females (43.3%). Among the heart diseases, the group most accompanied by urinary system anomalies was the non-cyanotic left-to-right shunted heart diseases, which was detected in 120 (39.1%) patients. Pelvicalyceal ectasia was the most common urinary system anomaly encountered and was detected in 89 patients (34.1%). Of the urinary system anomalies cases, 94 detected by cineurography were determined to be clinically severe. When the effectiveness of the cineurography was evaluated using the ultrasonography records of the patients, it was found to have 63.8% accuracy.ConclusionMany of the patients with CHDs concomitantly have urinary system anomalies. It is possible to determine asymptomatic urinary system anomalies using cineurographic imaging during angiocardiography. This may lead to earlier treatments and improved prognosis for the patients, thus making it possible to prevent potential future problems.

Background: Jervell and Lange-Nielsen syndrome (JLNS) isa recessive model of long QT syndrome which might also be related to possible hearing loss. Although the syndrome has been demonstrated to be originated from homozygous or compound heterozygous mutations in either the KCNQ1 or KCNE1 genes, additional mutations in other genetic loci should be considered, particularly in malignant course patients. Case presentations: Three patients were admitted into hospital due to recurrent seizures/syncope, intrauterine and postnatal bradycardia respectively; moreover all three patients had congenital sensorineural hearing-loss. Their electrocardiograms showed markedly prolonged QT interval. Implantable defibrillator was implanted and left cardiac sympathetic denervation was performed due to the progressive disease in case 1. She had countless ventricular fibrillation and appropriate shock while using an implantable defibrillator. The DNA sequencing analysis of the KCNQ1 gene disclosed a homozygous c.728G > A (p.Arg243His) missense mutation in case1. Further targeted next generation sequencing of cardiac panel comprising 68 gene revealed a heterozygous c.1346 T > G (p.Ile449Arg) variant in RYR2 gene and a heterozygous c.809G > A (p.Cys270Tyr) variant in NKX2-5 gene in the same patient. Additional gene alterations in RYR2 and NKX2-5 genes were thought to be responsible for progressive and malignant course of the disease. As a result of DNA sequencing analysis of KCNQ1 and KCNE1 genes, a compound heterozygosity for two mutations had been detected in KCNQ1 gene in case 2: a maternally derived c.477 + 1G > A splice site mutation and a paternally derived c.520C > T (p.Arg174Cys) missense mutation. Sanger sequencing of KCNQ1 and KCNE1 genes displayed a homozygous c.1097G > A (p.Arg366Gln) mutation in KCNQ1 gene in case 3. beta-blocker therapy was initiated to all the index subjects. Conclusions: Three families of JLNS who presented with long QT and deafness and who carry homozygous, or compound heterozygous mutation in KCNQ1 gene were presented in this report. It was emphasized that broad targeted cardiac panels may be useful to predict the outcome especially in patients with unexplained phenotype-genotype correlation. Clinical presentations and molecular findings will be discussed further to clarify the phenotype genotype associations., Ministry of Science, Industry and Technology

Known as a benign arrhythmia and normally requiring no specific treatment, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. Here, we present a child with left coronary cusp-originating accelerated idioventricular rhythm, degenerating into torsades de pointes and resulting in cardiac arrest, which was ablated with a cryocatheter. An 11-year-old boy, followed due to asymptomatic accelerated idioventricular rhythm before, was referred to our department because he had experienced an aborted cardiac arrest during sleep. He had been resuscitated for 5 minutes. Twenty-four-hour Holter-ECG revealed incessant accelerated idioventricular rhythm, consisting up to 90% of the whole record and two torsades de pointes attacks, triggered by accelerated idioventricular rhythm-induced “R on T” phenomenon, and resulting in syncope and cardiac arrest. Transthoracic echocardiography revealed no structural cardiac defect but mild left ventricular systolic dysfunction with an ejection fraction of 45% and shortening fraction 23%. An electrophysiologic study was conducted, and accelerated idioventricular rhythm focus was mapped to left aortic coronary cusp. A cryocatheter with an 8-mm tip was preferred for successful ablation of the accelerated idioventricular rhythm focus, due to close neighbourhood to coronary ostium. The patient was discharged in 3 days without any premature ventricular contractions or accelerated idioventricular rhythm and with normalised cardiac functions. After 9 months on follow-up, he was still asymptomatic, without any premature ventricular contractions or accelerated idioventricular rhythm and with normal cardiac functions. Although the clinical course of accelerated idioventricular rhythm is known as benign, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. In such cases, electrophysiologic study and catheter ablation are a good option in such cases with accelerated idioventricular rhythm for an ultimate cure.

In the published version of this paper the author Neus Baena's name was incorrectly given as Neus Baena Diez. This has now been corrected in both the HTML and PDF versions of the paper.

Giris: Senkop geciren hastalarda anamnez, fizik muayene ve elektrokardiyografi EKG ile etiyoloji aciklanamiyorsa genellikle Holter monitorizasyonu HM yapilir. Bu calismada senkoplu cocuklarda HM’nin tanisal degerinin belirlenmesi amaclanmistir.Gerec ve Yontem: Bu calismada 2010-2014 yillari arasinda hastalara uygulanan toplam 3,122 HM retrospektif olarak incelendi. Hastalarin cinsiyet, tani yasi, ayrintili anamnez, fizik muayene, 12-derivasyon EKG ve ekokardiyografik sonuclari standart form kullanilarak kayit edildi.Bulgular: Calismaya yas ortalamasi 13,21±3,67 olan 323 hasta dahil edildi. Hastalarin 199’u kiz, 124’u erkek idi. Tum hastalarin 284’unde %87,9 Holter sonuclari normal saptanirken 11 hastada %3,4 senkopu aciklayan anormal Holter sonuclari tespit edildi. Anormal Holter sonuclari olan 11 hastanin ucunde HM oncesinde uygulanan EKG ile tani kondugu icin HM’nin tanisal degeri %2,4 olarak hesaplandi. Aile oykusu pozitif olan cocuklarda ise HM’nin tanisal degeri %16,6 olarak bulundu. Calismada EKG’si normal olan yedi hastada ise HM sonucu ile uzun QT sendromu dusunuldu

PurposeWe delineate the clinical spectrum and describe the histology in arterial tortuosity syndrome (ATS), a rare connective tissue disorder characterized by tortuosity of the large and medium-sized arteries, caused by mutations in SLC2A10.MethodsWe retrospectively characterized 40 novel ATS families (50 patients) and reviewed the 52 previously reported patients. We performed histology and electron microscopy (EM) on skin and vascular biopsies and evaluated TGF-β signaling with immunohistochemistry for pSMAD2 and CTGF.ResultsStenoses, tortuosity, and aneurysm formation are widespread occurrences. Severe but rare vascular complications include early and aggressive aortic root aneurysms, neonatal intracranial bleeding, ischemic stroke, and gastric perforation. Thus far, no reports unequivocally document vascular dissections or ruptures. Of note, diaphragmatic hernia and infant respiratory distress syndrome (IRDS) are frequently observed. Skin and vascular biopsies show fragmented elastic fibers (EF) and increased collagen deposition. EM of skin EF shows a fragmented elastin core and a peripheral mantle of microfibrils of random directionality. Skin and end-stage diseased vascular tissue do not indicate increased TGF-β signaling.ConclusionOur findings warrant attention for IRDS and diaphragmatic hernia, close monitoring of the aortic root early in life, and extensive vascular imaging afterwards. EM on skin biopsies shows disease-specific abnormalities.GENETICS in MEDICINE advance online publication, 11 January 2018; doi:10.1038/gim.2017.253. ispartof: Genetics in Medicine vol:20 issue:10 pages:1236-1245 ispartof: location:United States status: published

Cardiac fibroma is a rare primary tumor of the heart. Patients can be asymptomatic or present with palpitations, cardiac murmur, syncope, arrhythmias, symptoms of congestive heart failure, or sudden death. We report a case of successful surgical excision of a giant cardiac fibroma in an asymptomatic child.

Ya z›fl ma Ad re si/Ad dress for Cor res pon den ce Dr. Ipek Guney Varal, Uludag Universitesi Tip Fakultesi, Cocuk Sagligi ve Hastaliklari Anabilim Dali, Neonatoloji Bilim Dali, Bursa, Turkiye Tel.: +90 532 341 33 89 E-posta: ibettyg@yahoo.com Ge lis ta ri hi/Re cei ved: 07.01.2014 Ka bul ta ri hi/ Ac cep ted: 22.03.2014 1Uludag Universitesi Tip Fakultesi, Cocuk Sagligi ve Hastaliklari Anabilim Dali, Neonatoloji Bilim Dali, Bursa, Turkiye 2Uludag Universitesi Tip Fakultesi, Cocuk Sagligi ve Hastaliklari Anabilim Dali, Pediatrik Kardiyoloji Bilim Dali, Bursa, Turkiye 3Uludag Universitesi Tip Fakultesi, Radyoloji Anabilim Dali, Pediatrik Radyoloji Bilim Dali, Bursa, Turkiye Ipek Guney Varal1, Nilgun Koksal1, Hilal Ozkan1, Ozlem Bostan2, Onur Bagci1, Fahrettin Uysal2, Zeynep Yazici3, Pelin Dogan1

Objectives: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. Coronary artery lesions (CAL) develop in 15% to 20% of untreated cases. Our objective was to evaluate demographic, clinical, and laboratory features and short-intermediate coronary artery outcomes of children with KD. Patients and Methods: Medical records of patients with KD were retrospectively identified. Clinical information and echocardiography, laboratory, and angiographic results were noted using a standardized form. Results: The study included 44 patients with a mean age of the 29.72 ± 21 months (ranging from 1 month to 9.5 years). There were 28 male and 16 female patients; 20 patients were diagnosed as having had incomplete KD. Four cases with atypical presentation were significantly older than children with complete and incomplete KD; 17 patients (38.6 %) had coronary artery aneurysm (CAA), which declined to 6.8% after intravenous immunoglobulin (IVIG) treatment. Time between fever and diagnosis and abnormal levels of hemoglobin and platelets were all associated with CAA. The children were followed up for a mean of 36.39 ± 19 months (with a maximum of 16 years). Angiographic evolution and regression of CALs have been observed in 14 (82.3%) patients. Three patients in whom CALs persisted did not receive IVIG therapy because of delayed diagnosis. Conclusions: Awareness of KD in children has led to an increase in the number of cases. Utility of IVIG treatment to reduce the coronary artery involvement in patients with delayed diagnoses should be discussed and considered. Long-term results are required to assess whether the KD represents a risk factor for coronary artery diseases seen during adulthood.

Background The aim of this study was to identify the sensitivity of electrocardiogram (ECG) in early diagnosis of cardiac autonomic function disorder in children with type 1 diabetes mellitus. Methods A total of 150 children and adolescents with type 1 diabetes mellitus were enrolled between June 2009 and June 2010, as well as 100 age- and sex-matched healthy control children. Twelve-lead ECG was done in all cases and heart rate, QT and QTc interval, dispersion of P wave (Pd), and of QT (QTd) and QTc interval (QTcd) were measured. The clinical and demographic features such as age, gender, duration of follow up and level of HbA1c and fasting glucose were obtained and the effects of these parameters on ECG measurements were investigated. Results The mean age of the patients and controls was 11.61 ± 3.72 years and 10.92 ± 3.2 years, respectively. QT and QTc interval and QTcd interval were significantly higher in diabetic children compared to healthy controls but these ECG findings were not associated with the duration of diabetes or glycemic state. Pd was significantly higher in the diabetic patients with HbA1c >7.5% compared to control, and this was also found in patients that were followed up >1 year. Conclusions Cardiac autonomic function disorder, which is one of the most important causes of morbidity and mortality, may emerge in the course of type 1 diabetes mellitus. It can be diagnosed on ECG even when the patients are asymptomatic.

Congenital nephrotic syndrome (CNS) is a rare disease inherited as an autosomally recessive trait and defined as proteinuria manifesting at birth or in the first 3 months of life. The classical form is the Finnish type of CNS (CNF), which is caused by mutations in the nephrin gene (NPHS1). The classical findings include prematurity, large placenta and massive proteinuria. Minor cardiac findings have been reported as a minor functional disorder but CNS with major cardiac malformation is rare. Here we report the case of a Turkish child with CNS with small indel mutation (c.614_621delCACCCCGGinsTT) in exon 6 of NPHS1 and also major cardiac malformation who did not develop end-stage renal disease until the age of 5 years.

Background: In this study, we present our five-year extracorporeal membrane oxygenation experiences in patients requiring mechanical support after pediatric open heart surgery. Methods: We retrospectively reviewed the medical records of 29 children (16 males, 13 females; mean age 21.6 months; range 5 days to 162 months) who underwent open heart surgery and required extracorporeal membrane oxygenation support due to postcardiotomy circulatory failure between February 2010 and March 2015. Results: The most common diagnosis was tetralogy of Fallot in eight patients (27.5%). The most common extracorporeal membrane oxygenation indication was failure to wean from cardiopulmonary bypass in 12 (41%) patients. The mean duration of extracorporeal membrane oxygenation support was 6.9 days (range 14 hours to 32 days). The most common complication related to extracorporeal membrane oxygenation support was renal insufficiency in 14 patients (48.3%). Fourteen patients (48%) were able to be successfully weaned from extracorporeal membrane oxygenation support, while six patients (20.7%) were discharged without any neurological sequelae. No significant predictor of mortality was found. Failure to wean from cardiopulmonary bypass resulted improved outcomes than other extracorporeal membrane oxygenation indications. Conclusion: Extracorporeal membrane oxygenation provides an effective cardiopulmonary support for cardiopulmonary failure after pediatric open heart surgery. Careful patient selection, and correct timing and appropriate management of extracorporeal membrane oxygenation are crucial for optimal outcomes.

The Amplatzer septal occluder (ASO) has become the device of choice for interventional closure of atrial septal defects (ASDs) in many institutions during the last decades. Although excellent results have been reported for the device, concerns have arisen about the long-term complications (1). Of these complications, thrombus formation was rarely seen after 1 year in patients (2–4). This is the first report of a pediatric patient of a huge thrombus developing on an ASO device detected by transthoracic echocardiography on a routine examination after 1 year of implantation without a risk factor.

Objective: Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement used for evaluating right ventricular systolic function. While established reference values of TAPSE exist for the adult population, only a limited number of studies have attempted to evaluate reference values for the pediatric population. The aim of the present study was to determine the reference values for TAPSE in healthy children in Turkey. Methods: A total of 765 healthy children aged between 0 and 18 years, all of whom were referred to our clinic with cardiac murmurs, were evaluated prospectively. Patients with no cardiac pathologies or other disorders were excluded from the study. The measurement of TAPSE was obtained using a 2D-guided M-mode technique with echocardiography, and the relationship between age and surface area with TAPSE was investigated. The statistical analysis was carried out using the SPSS 20.0 software package (SPSS Inc., Chicago, IL, USA, 2012). Results: The mean TAPSE value was found to be 19.56 +/- 5.54 mm, and no significant difference was identified between male and female children. TAPSE values showed a positive correlation with increasing age and surface area. The mean TAPSE value was 9.09 +/- 1.36 mm in newborns and 25.91 +/- 3.60 mm in the 13-18 years age group. A negative correlation was seen between TAPSE and heart rate. Conclusion: In the present study, the reference values for TAPSE in healthy Turkish children were presented in percentile tables and the corresponding z-scores were determined. These reference values may be useful in daily practice for the evaluation of right ventricular systolic function in children.

Congenital ventricular diverticulum is a rare cardiac anomaly defined as a localized protrusion of the ventricular free wall. Although, it is usually asymptomatic, complications such as embolism, infective endocarditis, and arrhythmias can occur. The diagnosis can be made by echocardiography, cardiac magnetic resonance imaging, or catheter angiography. Surgical resection is the treatment of choice in symptomatic patients, whereas the management of asymptomatic patients often represents a therapeutic dilemma. We report here, a 9-month-old patient with asymptomatic congenital left ventricular (LV) diverticulum associated with epigastric hernia.

Congenital nephrotic syndrome (CNS) is a rare disease inherited as an autosomally recessive trait and defined as proteinuria manifesting at birth or in the first 3 months of life. The classical form is the Finnish type of CNS (CNF), which is caused by mutations in the nephrin gene (NPHS1). The classical findings include prematurity, large placenta and massive proteinuria. Minor cardiac findings have been reported as a minor functional disorder but CNS with major cardiac malformation is rare. Here we report the case of a Turkish child with CNS with small indel mutation (c.614_621delCACCCCGGinsTT) in exon 6 of NPHS1 and also major cardiac malformation who did not develop end-stage renal disease until the age of 5 years.

Although congenital coronary artery anomalies are seen in 0.6-1 % of adult patients undergoing coronary angiography, the data for the pediatric population are few. This study of 22 children with coronary artery anomalies evaluated them in terms of demographic and clinical features and analyzed their angiographic findings and surgical results. Databases in the Department of Pediatric Cardiology at the University of Uludag were searched for all the patients with a diagnosis of congenital coronary artery anomaly who underwent coronary angiography between 1993 and 2013. Patients with coexistent congenital heart disease were excluded from the study. The study noted 22 patients (0.9 %; 10 boys and 11 girls) with coronary artery anomalies. The mean age of these patients was 58.77 +/- A 52.04 months (range, 1 month-16 years). Coronary arteriovenous fistula (50 %) and anomalous left coronary artery from the pulmonary artery (ALCAPA) (36 %) were the most common anomalies. In addition, the study included one patient with diffuse coronary artery hypoplasia, one patient with muscular bridge, and one patient with left main coronary artery originating from the right aortic sinus valsalva. Of the 11 patients who had coronary atrioventricular fistula, 7 were asymptomatic, whereas 75 % of the patients with ALCAPA syndrome were admitted because of heart failure. Although 13 patients had an exact diagnosis by echocardiography, 50 % of the patients with ALCAPA syndrome had their diagnosis determined by catheter angiography performed because of severe mitral regurgitation or dilated cardiomyopathy. The mortality rate for all the patients was found to be 18.1 %. Eight patients with coronary arteriovenous fistula have been followed up without surgery to the present. In contrast, seven patients with ALCAPA syndrome have undergone surgery, and three have died. Two of these patients died during the postoperative period, and the remaining patient died suddenly during the preoperative period at home. Isolated congenital coronary artery anomalies are very rare in the pediatric population. Although most congenital coronary artery anomalies are clinically silent, they may be associated with severe symptoms in children. Recognition of potentially serious anomalies such as ALCAPA syndrome is mandatory so that early surgical treatment can be prescribed.

The aim of this study was to identify the sensitivity of electrocardiogram (ECG) in early diagnosis of cardiac autonomic function disorder in children with type 1 diabetes mellitus.A total of 150 children and adolescents with type 1 diabetes mellitus were enrolled between June 2009 and June 2010, as well as 100 age- and sex-matched healthy control children. Twelve-lead ECG was done in all cases and heart rate, QT and QTc interval, dispersion of P wave (Pd), and of QT (QTd) and QTc interval (QTcd) were measured. The clinical and demographic features such as age, gender, duration of follow up and level of HbA1c and fasting glucose were obtained and the effects of these parameters on ECG measurements were investigated.The mean age of the patients and controls was 11.61 ± 3.72 years and 10.92 ± 3.2 years, respectively. QT and QTc interval and QTcd interval were significantly higher in diabetic children compared to healthy controls but these ECG findings were not associated with the duration of diabetes or glycemic state. Pd was significantly higher in the diabetic patients with HbA1c7.5% compared to control, and this was also found in patients that were followed up1 year.Cardiac autonomic function disorder, which is one of the most important causes of morbidity and mortality, may emerge in the course of type 1 diabetes mellitus. It can be diagnosed on ECG even when the patients are asymptomatic.

Subvalvular aortic stenosis accounts for 1-2 % of all congenital heart disease and for 8-20 % of cases of left-ventricular outflow tract (LVOT) obstruction in children. Recurrence of subaortic stenosis (SAS) is not uncommon after surgical management. This study was performed to investigate the clinical and surgical outcomes and to estimate the predictability of recurrences of SAS. Seventy-nine patients age 3-21 years with SAS between 1994 and 2010 were reviewed. Fifty-one patients had discrete SAS, whereas the remaining 15 patients had fibromuscular ridge-type SAS. Mean follow-up time without surgery was 22 months (range of 1-94). Forty-one patients with a diagnosis of SAS underwent surgery. Recurrence rates were 22.7 % (15 patients), and these patients developed SAS at a mean of 4.7 years follow-up. We performed second surgical membrane resection in only 1 patient. The risk of recurrence of SAS was only linked to higher preoperative LVOT gradient. Twenty-three patients had no aortic regurgitation (AR) at preoperative echocardiography. Of these, 39.1 % had trivial, 8.7 % had mild, and 8.7 % had moderate AR after surgery; there was no significant AR. We conclude that surgical intervention was required most of the time in patients with SAS, and surgical outcomes was excellent even if there were associated cardiac defects. The risk of recurrences was higher, especially in patients with higher initial LVOT gradients, although a second surgery was rarely necessary in these patients.