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Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery–pulmonary artery fistula. We present two cases with coronary artery–pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Background:Although percutaneous closure of patent ductus arteriosus is an established safe procedure, protrusion of the device to descending aorta may occur in various degrees during these procedures, especially in small infants. The aim of our study is to evaluate the benefits of balloon-assisted device releasing technique in the era of preventing device protrusion and conditions related to protrusion.Methods:One hundred and fifty-five infants, who underwent patent ductus arteriosus closure with Amplatzer duct occluder I device between January, 2012 and December, 2018, were retrospectively analysed. Balloon-assisted device releasing technique was used in 20 cases (group 1, 12.9%), between January, 2015 and December, 2018. Procedures in which the technique had been used were compared with the remaining ones (group 2, 87.1%, n = 135) with regard to device stabilisation, aortic disc protrusion to the aorta, iatrogenic coarctation, and device embolisation.Results:There was no significant difference by means of gender, age, weight, and the ductal diameter, whereas the average mean pulmonary artery pressure was significantly higher in group 1. Device protrusion and related complications were significantly higher in group 2; thus, additional catheterisations or surgical interventions were required, while no additional intervention was required in group 1.Conclusion:The balloon-assisted device releasing technique provides a good device stabilisation and prevents protrusion of the device and related complications during percutaneous patent ductus arteriosus closure in selected cases.

Aneurysms of the ascending aorta are frequently found in patients with a bicuspid aortic valve (BAV). This study assessed the risk factors of ascending aortic aneurysms and aortic elasticity in children with BAV. The study included 66 patients with no history of transcatheter intervention or surgical procedure who had been diagnosed with isolated BAV. Echocardiographic, blood pressure, and pulse measurements were obtained for all patients. The BAVs were classified as described by Sievers et al. (J Thorac Cardiovasc Surg 133:1226–1233, 2007), and aortic elasticity parameters were calculated using various formulas. The patients were divided into groups with and without cusp fusion, aortic stenosis (AS), aortic regurgitation (AR), or mixed lesions; the groups were then compared. The mean patient age was 10.43 ± 3.91 years; 15%he patients had no AS or AR, 33% had both AS and AR, 17% had AS alone, and 35% had AR alone. The most common type of BAV was type 5, and the ascending aorta z-scores were higher in children with mixed lesions and without a cusp fusion. Aortic distensibility (AD) was significantly higher, and the stiffness index was significantly lower, in patients with an ascending aorta z-score > 4. The ascending aortic z-scores were higher in the no-fusion and mixed lesion (AS + AR) groups, especially those originating from post-stenotic dilation due to AS. The AD was increased in patients with an ascending aorta z-score > 4. Patients should thus be monitored closely for dissection risk, and preventive medical treatment should be started early in those with AS without cusp fusion.

WOS: 000429628000003 PubMed ID: 29578201 Objective: To report clinical and procedural characteristics of twelve patients who received a covered stent for the treatment of aortic coarctation and concurrent patent ductus arteriosus (PDA). Methods: A single center database was retrospectively evaluated to obtain data of patients with combined aortic coarctation and PDA. We selected patients in whom a covered stent was used for the treatment of both pathologies. The stent length was chosen so as to cover the entire length of the lesion from healthy to healthy tissue and also cover the ampulla of PDA. Results: The median age of the patients was 15 (range, 6.5-35) years. The diameter of the coarctated segment increased from a median of 8.4 (range, 2.6-10.8) mm to 16 (range, 9-24) mm (p

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. Turkish Başlık: Aortopulmoner Pencereli Olguların Tanı ve Tedavi Sonuçlarının Değerlendirilmesi Anahtar Kelimeler: Aortopulmoner pencere, pulmoner hipertansiyon Arka Plan: Aortopulmoner pencere; iki ayrı semilunar kapak varlığnda asendan aorta ile pulmoner arter arasında bağlantı olmasıdır ve septal defektlerin en nadir görülen tipidir. Amaç: Bu çalışmada kliniğimizde tanı almış aortopulmoner pencereli olgularımızın tanı ve tedavi sonuçlarını değerlendirmeyi amaçladık. Çalışma Tasarımı: Retrospektif kohort çalışma Yöntemler: Haziran 2003 ile Ekim 2011 tarihleri arasında 13 olgu aortopulmoner pencere tanısı aldı. Bu olguların dosyalarından başvuru sırasındaki klinik özellikleri, ekokardiyografik ve kalp kateterizasyonu bulguları ile cerrahisonuçları geriye dönük olarak gözden geçirildi. Bulgular: Yaşları 10 gün ile 16 yaş arasında değişen 11 olguya aortopulmoner pencere tanısıyla cerrahi girişim uygulandı. 12 aylık bir olguda defekt transkateter yoldan kapatıldı. Cerrahi tamir sırasında 8 olguda yapay, 3 olguda perikardiyal yama kullanıldı. Aortopulmoner pencere tamiri sırasında 2 hastada VSD, 2 olguda da kesintili arkus aorta tamiri yapıldı. Cerrahi ya da transkateter yoldan tedavi edilmiş hiçbir olgu kaybedilmedi. Postoperatif dönemde 3 olguda önemli pulmoner hipertansiyon nedeniyle nitrik oksit ve iloprost kullanımı gerekti. İzlemde kesintili arkus aorta nedeniyle cerrahi tamir uygulanmışolguya rekoarktasyon nedeniyle balon angioplasti yapıldı. Median 40 aylık süredetakip edilen diğer olgular semptomsuz ve iyi klinik durumda izlenmektedir. Sonuç: Büyüme gelişme geriliği ve kalp yetersizliği bulguları olan infantlarda aortopulmoner pencere ayırıcı tanıda düşünülmelidir. Altı aydan küçük izole defekte sahip olgularda ekokardiyografi tanı için yeterli bir yöntemdir. Kompleks olgularda kardiyak anatominin değerlendirilmesi, altı aydan büyük olgularda operabilitenin değerlendirilmesi ve uygun olgularda defektin transkateter yoldan kapatılması için kalp kateterizasyonu gerekebilir.