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Objective The aim of this study was to investigate fetal echocardiographic findings in predicting the need for surgical repair in fetuses with coarctation of the aorta (CoA) and to evaluate perinatal outcomes. Study Design In this retrospective study, fetuses diagnosed with CoA in a tertiary center between January 2015 and June 2021 were analyzed. Fetal echocardiographic measurements and quantitative findings, middle cerebral artery (MCA) and umbilical artery (UA) Doppler indices, and perinatal outcomes were recorded. Results A total of 57 fetuses with CoA were included in the study. In total, 51 (89.5%) pregnancies resulted in live births and 32 (62.8%) of the neonates underwent surgical repair. The left ventricle/right ventricle width ratio and aortic isthmus z -score were significantly lower in fetuses who underwent surgical repair (p = 0.004 0.001, respectively). Retrograde flow in the aortic isthmus (odds ratio [OR]:7.43; 95% confidence interval [CI]: 1.98–27.76), left-to-right foramen ovale shunt (OR: 8.50; 95% CI: 1.68–42.98), and ventricular septal defect (OR: 9.63; 95% CI: 1.90–48.74) were associated with the need for surgical repair. A new scoring system integrating these echocardiographic findings had 89% specificity and 54% sensitivity in predicting surgical repair. Fetal growth restriction rates, preterm birth rates, mean MCA pulsatility index (PI), and mean UA PI were similar in fetuses with and without surgical repair. Conclusion A scoring system integrating echocardiographic findings in fetuses with CoA may improve the prediction of surgical repair need. There is no evidence of an increased risk of FGR, preterm birth, and brain sparing effect in fetuses with CoA who require surgical repair. Key Points Coarctation of the aorta is one of the most difficult congenital heart defects to diagnose. A new scoring system may improve the prediction of surgical repair need. There is no increased risk of fetal growth restriction in fetuses requiring early surgical repair. [ABSTRACT FROM AUTHOR]

Purpose: Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. Methods: This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal–fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. Results: Ninety‐nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remote type, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra‐cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty‐nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). Conclusion: The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies. [ABSTRACT FROM AUTHOR]

Poland's syndrome is a rare congenital anomaly accompanied by the absence of the pectoralis major, and the ipsilateral upper-limb and chest wall deformities. Hypoplasia of the breast, agenesis of the ipsilateral rib cartilage, athelia, and ipsilateral developmental finger anomalies such as syndactyly can also be seen. In the literature, only 56 cases of dextrocardia and leftsided Poland's syndrome have been described. Herein, a case of left-sided Poland's syndrome coexisting with dextrocardia and nasal hemangioma was presented. [ABSTRACT FROM AUTHOR]

Background: Aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves and is the rarest of septal defects. Aims: To present our experience with the diagnosis and outcome of APW cases. Study Design: Retrospective cohort study. Methods: Between June 2003 and October 2011, thirteen patients were diagnosed with APW. Clinical features of patients, findings of echocardiographic and angiographic examination, results of surgical intervention and follow-up were reviewed retrospectively. Results: Eleven children (10 days to 16 years), underwent surgical correction of APW. In a 12-month-old boy, the defect was repaired by the transcatheter approach. In addition to APW repair, closure of VSD was performed in 2 patients. APW were associated with interruption in two patients; one also had a complex pathology. None of the patients died due to complications of surgical or transcatheter procedures. After a median follow-up period of 40 months, the patients were asymptomatic and none of them required additional medication, except for the patient with complex pathology including an interrupted aortic arch, who underwent balloon angioplasty for recoarctation. Conclusion: In any infant with the findings of congestive heart failure and failure to thrive, APW must be kept in mind as a differential diagnosis. In isolated APW cases before 6 months of age, echocardiography is often sufficient for diagnosis. In complex cases, cardiac catheterisation is performed for the comprehensive evaluation of associated defects. After 6 months, cardiac catheterisation could be utilised to perform vasoreactivity testing and, if possible, to close the defect. [ABSTRACT FROM AUTHOR]

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Purpose: We presented the experience of a tertiary care center for maternal and fetal diseases and assessed the findings fetuses with double-inlet left ventricle (DILV) regarding fetal echocardiography, prenatal course including fetal growth and death, and postnatal outcome., Methods: In this retrospective study, patients diagnosed with DILV via prenatal ultrasound in the maternal-fetal medicine department between 2015 and 2023 were included to evaluate important aspects of prenatal diagnosis and course, as well as postnatal management and outcome., Results: There were 33 DILV cases prenatally diagnosed and postnatally confirmed. Genetic abnormality was detected in two (8%) of 25 fetuses that had undergone genetic tests. Pulmonary artery obstruction was observed in 10 (30.3%) fetuses; pulmonary stenosis was found in nine, and pulmonary atresia in one. Aortic arc abnormalities were found in 11 (33.3%) fetuses. The great arteries were malpositioned in the 29 (87.8%) fetuses. The median duration of follow-up for the infants was 12 (2-96) months, and 22 (66.6%) of 33 cases survived. Among the 27 (81.8%) live births, 24 infants required univentricular palliation., Conclusions: Remarkable implications of this study were that unless extra-cardiac and genetic anomalies accompany DILV, the probability of a chromosomal anomaly is low; that univentricular repair could be performed in almost all cases; and that the major causes of poor prognosis may be related to the restrictive bulboventricular foramen and severe aortic obstruction. Although long-term survival rates after univentricular repair appear to be high, these patients require long-term follow-up and repeated surgical procedures., (© 2024 Wiley Periodicals LLC.)

Objective: To present our institutional experience of endovascular Cheatham-Platinum stent implantation in children and adults with native and recurrent aortic coarctation., Methods: Between August 2007 and November 2009, 45 patients had aortic coarctation treated with 47 stents implantation. We preferred primarily stent implantation in adult patient with coarctation, in children more than five years-old it is preferred in cases of aneurysm, subatretic or blind coarctation and coarctation with patent ductus arteriosus or in restenosis. Files of stent-implanted patients were retrospectively analyzed in terms of patients' demographic features, echocardiographic and angiographic findings both before and after procedure. Patients were grouped as Group 1: native coarctation and Group 2: recoarctation developed after either surgery or balloon angioplasty. Findings of the cases' were compared using paired and unpaired Student's t, Mann-Whitney U and Chi-square tests., Results: Sixteen covered and 31 bare totally 47 balloon expandable stents were implanted in 45 patients. The mean follow up duration was 12.1 ± 7.1, median 11 months (2-29 months). There was no procedure related death. In two patients two stents were implanted in tandem. While the coarctation of the aorta was native in 26 patients (functionally interrupted aortic arch in one), recoarctation was detected in 7 patients after surgery, in 8 patients after balloon angioplasty, in 4 patients both after surgery and balloon angioplasty. One patient had functionally interrupted aortic arch perforated with guide wire and then covered stent implanted. The mean age 12.2 ± 5.9 years (5-33 years) and mean body mass index was 21 ± 3.7 kg/m2 (14.8-31 kg/m2). Considering all cases, a statistically significant decrease in both the invasive and echocardiographic gradients (p<0.001 for both) and statistically significant increase in lesion diameter (p<0.001) were detected. The decrease in invasive and echocardiographic gradients and increase in lesion diameter is statistically significant in each group also (p<0.001, <0.001 and <0.001 for both groups, respectively). Before the procedure, the invasive gradient was significantly higher and the lesion diameter was significantly lower in group I than in group II (p=0.002 and p=0.005, respectively). Also the percentage of decrease in gradient and increase in diameter was statistically higher in group 1 than in group 2 (p=0.04 and p=0.04)., Conclusion: Our early and short- term follow-up results indicate that stent implantation is safe and very effective in reducing coarctation gradient and increasing lesion diameter both in native coarctation and recoarctation.

Arterial tortuosity syndrome (ATS) is a rare autosomal recessive connective tissue disease characterized by elongation, tortuosity, aneurysmatic formation, and stenotic lesions in large and medium-size arteries. We present two cases of ATS diagnosed during cardiac examination for murmurs. The first was an 11-year-old boy who had an atypical facial appearance and hyperelasticity. He had a prior operation for inguinal hernia. Echocardiography showed aneurysmatic dilatation in the main pulmonary artery and peripheral stenotic lesions. Angiography and computed tomography angiography confirmed aneurysmatic formation in the main pulmonary artery and multiple stenotic lesions in peripheral arteries and showed elongation and tortuosity of the major branches of the aorta. Surgical reconstruction of the pulmonary arterial system was performed. The second was a 3-month-old girl with an atypical facial appearance, hyperelasticity, and marked hypotonia. The aortic arch could not be visualized during echocardiography. Angiographic examination showed mild bilateral stenosis of distal pulmonary arteries, elongation and tortuosity of the aortic arch and its main branches.