Prenatal diagnosis and pregnancy outcomes of 149 fetuses with tetralogy of Fallot accompanied by concomitant cardiac and extracardiac anomalies

Objective: We aimed to analyze the pregnancy outcomes of cases that were diagnosed prenatally with tetralogy of Fallot (TOF) and to investigate its prenatal diagnosis, treatment conditions, and postnatal outcomes. Methods: The clinical information data of a total of 149 fetuses diagnosed with TOF (145 cases were singleton and 4 cases were twins) were collected and reviewed retrospectively between January 2016 and January 2022 at İstanbul Kanuni Sultan Süleyman Training and Research Hospital. Results: Among completely 61,034 pregnancies between these years, 149 fetuses were diagnosed with TOF by fetal prenatal ultrasound imaging with the occurrence rate of about 0.24% (149/61,034), and the average gestational age was 26.13 weeks. Among these cases, 22 cases (14.76%, 22/149) opted for termination of pregnancy. Of these 149 fetuses, a total of 60 (46.30%, 60/149) accepted genetic testing and 11 (7.38%, 11/149) revealed chromosomal disorders with trisomy 21 in 5 cases, trisomy 18 in 1 case, 22q11.2 microdeletion syndrome in 4 cases and abnormality of the short arm of 8th chromosome in 1 case. After delivery, 4 cases were diagnosed with trisomy 21. Pregnancy was continued in a total of 127 fetuses, of which 114 cases resulted in delivery. While 36 cases were delivered by cesarean section, the other 78 cases were delivered vaginally. Thirteen fetuses died during the pregnancy period. Newborns who survived the postpartum period were followed up to 5 years of age for surgery. Among 114 cases, 28 cases died during the postnatal period at different times. Conclusion: The diagnosis of TOF is mainly established with the help of fetal ultrasound in the second trimester. A genetic examination is also necessary after prenatal diagnosis and multidisciplinary work is also important between departments. TOF without genetic disorder can be successfully corrected with surgery after birth

Prolonged P-Wave and QT Dispersion in Children with Inflammatory Bowel Disease in Remission

Objectives. Ulcerative colitis (UC) and Crohn’s disease (CD) are chronic inflammatory bowel diseases (IBD) with unclear underlying aetiologies. Severe cardiac arrhythmias have been emphasised in a few studies on adult IBD patients. This study aimed to investigate the alteration of the P-wave and QT interval dispersion parameters to assess the risk of atrial conduction and ventricular repolarisation abnormalities in pediatric IBD patients. Patients and Methods. Thirty-six IBD patients in remission (UC: 20, CD: 16) aged 3–18 years and 36 age- and sex-matched control patients were enrolled in the study. Twelve-lead electrocardiograms were used to determine durations of P-wave, QT, and corrected QT (QTc) interval dispersion. Transthoracic echocardiograms and 24-hour rhythm Holter recordings were obtained for both groups. Results. The P-wave dispersion, QT dispersion, and QTc interval dispersion (Pdisp, QTdisp, and QTcdisp) were significantly longer in the patient group. The mean values of Pminimum, Pmaximum, and QTcminimum were significantly different between the two groups. The echocardiography and Holter monitoring results were not significantly different between the groups. Furthermore, no differences in these parameters were detected between the CD and UC groups. Conclusion. Results suggest that paediatric IBD patients may carry potential risks for serious atrial and ventricular arrhythmias over time even during remission

Prenatal diagnosis of tetralogy of Fallot with an absent pulmonary valve: is this malformation still associated with a poor prognosis? A 5-year single-center experience

Objective: This study sought to assess the prenatal features and clinical outcomes of cases with a fetal diagnosis of tetralogy of Fallot (TOF) with an absent pulmonary valve (APV) at our maternal-fetal medicine unit. Methods: Twelve cases of TOF and APV prenatally diagnosed at Kanuni Sultan Süleyman Training and Research Hospital between 2015 and 2020 were retrospectively reviewed. Prenatal characteristics, additional cardiac and extracardiac anomalies, and postnatal outcomes of the cases were examined. Results: The median gestational age at diagnosis was 22 weeks (range: 18–24 weeks). The absence of ductus arteriosus was found in all cases (100%). Karyotype analysis was performed in 5 cases. A chromosomal abnormality was detected in 3 of these cases (60%); 2 cases with 22q11 microdeletion, and 1 case with trisomy 21. Parents opted for termination of pregnancy in two of these cases; 1 case with 22q11 microdeletion, and 1 case with trisomy 21. Two patients experienced spontaneous intrauterine fetal demise. Finally, 8 live-born fetuses underwent total correction surgery during the postnatal period. Four (33.3%) out of 12 cases survived at the end of the 4-years follow-up period. Conclusion: While TOF with APV cases were predominantly associated with poor prognoses in the past, more promising results have been obtained in recent years in parallel with the developments in surgery and postnatal care. For this reason, we think that the prognosis will be even better in the coming years as the developments in surgical technique and care continue

Fetal conotruncal heart anomalies: Is fourchamber view sufficient in the prenatal screening?

Amaç: Bu çalışmada hastanemiz perinatoloji ünitesinde konotrunkalkalp anomalisi tanısı alan olguların kayıtları incelenerek prenataldönemde konotrunkal kalp anomalisi tanısına yönlendiren faktörlerindeğerlendirilmesi ve prenatal konotrunkal kalp anomalisitanısı ile ilgili farkındalığın artırılması amaçlandı.Yöntem: Ocak 2015 – Aralık 2016 tarihleri arasında Kanuni SultanSüleyman Eğitim ve Araştırma Hastanesi Perinatoloji Ünitesindekonotrunkal kalp anomalisi tanısı alan olguların yönlendirilmenedenleri, eşlik eden kalp dışı anomali ve kromozom anomalisivarlığı, olguların perinatal sonuçları ve postnatal dönemde tanı-nın doğrulanma başarısı değerlendirildi.Bulgular: Tüm konjenital kalp anomalilerinin içinde konotrunkalkalp anomalisi sıklığı %20.4 idi. Çalışmaya dahil edilen 101 olgunun37’sinde (%36.6) gebelik terminasyonu gerçekleştirildi. ‹ntrauterinfetal ölüm 5 (%5) olguda gözlendi. Olguların %26.7’sindekromozom anomalisi ve %34.7’sinde kalp dışı ek yapısal anomalisaptandı. Canlı doğan 59 (%58.4) olgunun 52’sinde (%88.1) prenataltanı doğrulandı. Olguların yalnızca %27.7’sinde dört oda görünümününanormal olduğu saptandı.Sonuç: Dört oda görünümünün konotrunkal kalp anomalilerindesıklıkla normal olması nedeni ile bu anomalilerin prenatal tanı sıklığının artırılabilmesi için temel fetal kardiyak tarama programlarında üç damar ve üç damar trakea kesitleri rutin olarak görüntülenmelidir.Objective: In this study, we aimed to assess the factors leading to the diagnosis of conotruncal heart anomaly in the prenatal period by reviewing the records of the cases which were diagnosed with the conotruncal heart anomaly in our perinatology unit, and to raise the awareness of the diagnosis of prenatal conotruncal heart anomaly. Methods: The referral reasons, the presence of concomitant non-cardiac anomaly and chromosomal anomaly, perinatal outcomes of the cases and the confirmation success of the diagnosis at postnatal period of the cases which were diagnosed with the conotruncal hearth anomaly at the Perinatology Unit of Kanuni Sultan Süleyman Training and Research Hospital between January 2015 and December 2016 were evaluated. Results: Among all congenital cardiac anomalies, the incidence of conotruncal heart anomaly was 20.4%. The termination of pregnancy was performed in 37 (36.6%) of 101 cases included in the study. Intrauterine fetal death was observed in 5 (5%) cases. Chromosomal anomaly and non-cardiac additional structural anomaly were found in 26.7% and 34.7% of the cases, respectively. Prenatal diagnosis was confirmed in 52 (88.1%) of 59 (58.4%) cases which born alive. It was found that four-chamber view was abnormal in only 27.7% of the cases. Conclusion: Three vessels (3V) and three vessels trachea (3VT) views should be displayed routinely in basic fetal cardiac screening in order to increase the prenatal diagnosis frequency of these anomalies as four-chamber view is mostly normal in conotruncal heart anomaly

DiGeorge sendromunda sol pulmoner arterden köken alan sol subklavyen arter

Left subclavian artery originating from the left pulmonary artery is a rare aortic arch anomaly. Herein, we, for the first time in Turkey, present a case of left subclavian artery originating from the left pulmonary artery via ductus arteriosus in DiGeorge syndrome and causing subclavian steal syndrome.Sol pulmoner arterden köken alan sol subklavyen arter, nadir bir arkus aorta anomalisidir. Bu yazıda, Türkiye’de ilk kez, DiGeorge sendromunda duktus arteriyozus ile bağlantılı solpulmoner arterden köken alan ve subklavyen çalma sendromunaneden olan sol subklavyen arter olgusu sunuldu

Transcatheter correction of Scimitar syndrome: occlusion of abnormal pulmonary venous drainage and vascular supply in an infant

Treatment of Scimitar syndrome is usually surgical; however, if there is "dual drainage" - that is, one to the inferior caval vein and the other to the left atrium - it is possible to successfully treat this anomaly via a less-invasive transcatheter approach. We report a case of Scimitar syndrome in a 21-month-old, male infant successfully treated with transcatheter embolisation

Transcatheter correction of Scimitar syndrome: occlusion of abnormal pulmonary venous drainage and vascular supply in an infant

Treatment of Scimitar syndrome is usually surgical; however, if there is "dual drainage" - that is, one to the inferior caval vein and the other to the left atrium - it is possible to successfully treat this anomaly via a less-invasive transcatheter approach. We report a case of Scimitar syndrome in a 21-month-old, male infant successfully treated with transcatheter embolisation

Prenatal diagnosis and postnatal outcome of persistent right ductus arteriosus: a report of three cases

The ductus arteriosus is a fetal vascular connection between the main pulmonary artery and aorta that diverts blood away from the pulmonary bed. Left and right ductus arteriosi emerge from embryological aortic arches. In normal embryologic cardiac development, both right aortic and ductal arches regress and the left ones persist. Persistent right ductus arteriosus (rDA) is one of the congenital anomalies of the ductal arch. In this paper, we report three cases of persistent right ductus arteriosus with right aortic arch

An Exhaustive Echocardiographic Analysis of Left Ventricular Structure and Functions in Children with Duchenne Muscular Dystrophy

Objectives: We aim to characterize cardiac morphological and functional changes by echocardiography that are present in the early and late stages of Duchhenne type muscular dystrophy (DMD)

An incidentally detected anomalous origin of the right coronary artery from the pulmonary artery in an infant

Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications