Partial Anomalous Pulmonary Venous Connection and the Nature of Associated Sinus Venosus Defect

Background:Partial anomalous pulmonary venous connection(PAPVC)is frequently associated with atrial septal defect(ASD),especially sinus venosus defect(SVD).Although Waggstaffe described the pathology of SVDs in 1868,the exact anatomic features and the nature of SVD remains controversial.SVDs with no posterior atrial rim were observed in recent years.However,no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD.The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD.Methods:From January 2011 to December 2019,256 children with PAPVC combined ASD and 878 children with isolated ASD who underwent corrective cardiac surgery were consecutively enrolled.Comprehensive review of preoperative transthoracic echocardiography,computed-tomography images and surgical findings were performed by experienced pediatric cardiologists.The subtypes of PAPVC,locations and types of ASD,and presence of posterior atrial rim of associated ASD were investigated.Results:PAPVC was right-sided in 244 children,left-sided in 6 children,and bilateral in 6 children.In PAPVC cases,ASD without posterior atrial rim existed in 226 SVD cases.ASD without posterior atrial septum only existed in cases with one or more right pulmonary veins returning to right atrium(RA)or to RA-superior vena cava junction.In cases with isolated ASD,there were 3 SVD,and the other 875 cases were secundum ASD.Conclusions:ASD without posterior atrial rims was associated with one or more right pulmonary veins returning to RA or RA-superior venous cava(SVC)junction.For SVD,the key feature is that the defect is in the posterior of the interatrial septum with no posterior septal rim,rather than adjacent to the SVC or to the inferior vena cava.

Intermediate and Long-Term Follow-Up of Transcatheter Closure of Congenital Coronary Cameral Fistulas in Infants and Children:Experience from a Single Center

Background:Limited data are available regarding intermediate and long-term outcomes of transcatheter closure(TCC)of coronary cameral fistulas(CCFs)in the pediatric patients.Methods:All pediatric patients diagnosed with CCFs who were scheduled to undergo TCC between 2005 and 2019 were retrospectively enrolled in the study.Results:A total of 66 patients(median age:3.93 years,median weight:15 kg)underwent attempted TCC of CCFs.Immediate successful device implantation was achieved in 62 patients,and immediate complete occlusion was achieved in 44 patients(44/62%,71.0%).The closure procedure was waived in 2 patients due to anatomical factors.A total of 6 periprocedural complications occurred in 5 patients,including acute myocardial infarction(n=3),procedure-related death(n=1),device embolization(n=1),and rupture of tricuspid chordae tendineae(n=1).The acute procedural success rate was 89.4%(59/66),while the acute complication rate was 9.1%(6/66).Follow-up data were collected for 58(93.5%)out of 62 patients at a median of 9.3 years(range:3.0–15.7 years).10 adverse events occurred in 9 patients,including 5 follow-up complications(1 aortic valve perforation,1 coronary thrombosis,1 progressive aneurysmal dilation after reintervention,and 2 cases of new-onset tricuspid valve prolapse with significant regurgitation),and 5 closure failure with large residual shunts.The intermediate and long-term adverse event rate was 17.2%(10/58).The anatomical features associated with both acute and follow-up adverse events were large CCFs(p=0.005),and giant coronary artery aneurysms(CAAs)(p=0.029).Conclusions:TCC of CCFs in infants and children appears to be effective and is associated with a relatively low complication rate.Large CCFs and giant CAAs represent a higher risk of both acute and intermediate and long-term adverse events after closure.

Morphology and Function of the Aortic Valve after Transcatheter Closure of Ventricular Septal Defect with Aortic Valve Prolapse

Objective:This study aims to evaluate the morphology and function of the aortic valve after transcatheter closure of ventricular septal defect(VSD)with aortic valve prolapse(AVP)abased on clinical and radiological outcomes.Methods:From January 2013 to November 2014,164 consecutive patients(97 males,59.1%)with VSD and AVP were treated by transcatheter closure.The patients were divided into the mild AVP group(n=63),moderate AVP group(n=89)and severe AVP group(n=12).The clinical and radiological outcomes of these patients were analyzed retrospectively.Results:In total,146(89.0%)patients were successfully treated with VSD occluders,including 59/63(93.7%)with mild AVP,80/89(89.9%)with moderate AVP and 7/12(58.3%)with severe AVP.The degree of AVP was ameliorated or disappeared in 39(26.7%)patients,and remained unchanged in 103(70.5%)patients after the intervention.In the 35 patients who initially had trivial-to-moderate aortic regurgitation(AR),the degree of AR was ameliorated or disappeared in 25(71.4%)patients,aggravated from trivial to mild AR in 1(2.9%)patient,and remained unchanged in 9(25.7%)patients.In 111 patients without AR,1(0.9%)patient had mild AR and 24(21.6%)patients had trivial AR after intervention.The depth and width of the prolapsed aortic valve decreased after transcatheter closure of VSD in all three groups.During the 70-month(range,54–77)follow-up period,no patients with AVP and AR needed an aortic valve intervention.Conclusions:Transcatheter closure of VSD with AVP is feasible.The morphology and function of the prolapsed aortic valve improved and remained stable for a long period after intervention.

Optical coherence tomography of the pulmonary arteries in children with congenital heart diseases:A systematic review

Importance:Optical coherence tomography(OCT)is a high-resolution intravascular imaging tool and has shown promise for providing real-time quantitative and qualitative descriptions of pulmonary vascular structures in vivo in adult pulmonary hypertension(PH),while not popular in pediatric patients with congenital heart diseases(CHD).Objective:The aim of this review is to summarize all the available evidence on the use of OCT for imaging pulmonary vascular remodeling in pediatric patients.Methods:We conducted the systematic literature resources(Cochran Library database,Medline via PubMed,EMBASE,and Web of Knowledge)from January 2010 to December 2021 and the search terms were“PH”,“child”,“children”,“pediatric”,“OCT”,“CHD”,“pulmonary vessels”,“pulmonary artery wall”.Studies in which OCT was used to image the pulmonary vessels in pediatric patients with CHD were considered for inclusion.Results:Five studies met the inclusion criteria.These five papers discussed the study of OCT in the pulmonary vasculature of different types of CHD,including common simple CHD,complex cyanotic CHD,and Williams-Beuren syndrome.In biventricular anatomy,pulmonary vascular remodeling was primarily reflected by pulmonary intima thickening from two-dimensional OCT.In single-ventricle anatomy,due to the state of hypoxia,the morphology of pulmonary vessels was indirectly reflected by the number and shape of nourishing vessels from three-dimensional OCT.Interpretation:OCT may be an adequate imaging procedure for the demonstration of pulmonary vascular structures and provide additional information in pediatric patients.