Partial Anomalous Pulmonary Vein Research Articles

Diagnosis of Congenital Heart Disease in Adulthood: How Often, How Relevant?

Congenital heart disease (CHD) is typically detected during fetal life, infancy, or early childhood. However, there is no published data regarding the proportion of congenital heart defects that are diagnosed in adulthood or the impact of these defects. Retrospective analyses of all consecutive patients (n = 1,010) referred to an adult CHD unit between 2018 and 2023. We analyzed the proportion of cases diagnosed in adulthood, defining the type of defect, reasons for diagnosis, complications, and need for intervention. In total, 26.5% of patients were diagnosed in adulthood (mean age 47 ± 16 years). Overall, 75% were in New York Heart Association class I/IV. Most were mild complexity lesions (57.5%). The most common diagnoses were pre-tricuspid shunts, including ostium secundum atrial septal defect (ASD, 23.9%), partial anomalous pulmonary vein drainage (18.3%), and other types of ASD (5.9%). Bicuspid aortic valve (16.8%) and aortic coarctation (8.2%) were common. Other diagnoses included Ebstein's anomaly (5.6%), ventricular septal defect (4.5%), patent ductus arteriosus (2.6%), or congenitally corrected transposition of the great arteries (2.6%). The main reason for diagnostic work-up was cardiac symptoms (28.4%) such as dyspnea (19%) and palpitations (7.1%), followed by incidental findings on imaging (25.4%). A total of 47.4% had some complications, the most common being pulmonary hypertension (24.3%). Surgical repair was required in 27.2% and 25.4% underwent percutaneous intervention. About one-fourth of patients with CHD were diagnosed in adulthood, and up to 42.5% had moderate or severe complexity lesions. A significant proportion had developed complications at the time of diagnosis and half of them required intervention.

The Ottoman empire sword: a variant of partial anomalous pulmonary veins connections.

The Ottoman empire sword: a variant of partial anomalous pulmonary veins connections.

Video assisted thorascopic assisted correction of left partial anomalous pulmonary venous connection: one case report

IntroductionThe left partial anomalous pulmonary vein connection is a rare congenital heart disease, especially with intact atrial septum. Now we reported a case of the left superior pulmonary vein drainage to left innominate vein through a vertical vein, and corrected with video assisted thoracoscopy.Case presentationA-59-years old man diagnosed left anomalous partial pulmonary vein connection with presentation of short breathiness and palpation, and diagnosed with computer tomography pulmonary angiography. The operation was carried out under video assisted thoracoscopy with one manipulation incision and one observational incision, the vertical vein was dissected and anastomosis with left atrial appendage. The patients recovered smoothly and postoperative CTPA showed anastomosis ostium was unobstructed.ConclusionThe left lateral thoracotomy and video assisted thoracoscopic surgery is a feasible for correction of left PAPVC with intact interatrial septum without using CPB.

Analysis of risk factors and outcomes of acute kidney injury in young children after cardiac surgery

The aim of this study was to analysis of risk factors and outcomes of acute kidney injury (AKI) in young children with congenital heart disease (CHD) after cardiac surgery. The study included 137 young children with CHD after various types of cardiac surgery. The stages of AKI and indications for peritoneal dialysis (PD) were determined based of Kidney Disease: Improving Global Outcomes (KDIGO) guidelines. The incidence of AKI in young children in the general group was 40.9% (n=56). Stage 1 AKI was diagnosed in 21.9% (n=29) of patients, stage 2 AKI in 12.4% (n=17), and stage 3 AKI in 7.3% (n=10) of patients. Peritoneal dialysis was performed in 11.7% (n=16) of children. The incidence of AKI development after radical correct transposition of the great vessels (TGV) was 55.5% (n=5), truncus arteriosus (TA) was 100%, pulmonary atresia (PA)=25%, tetralogy of Fallot (TF)=38.1%, total anomalous pulmonary vein drainage (TAPVD)=60%, partial anomalous pulmonary vein drainage (PAPVD)=37.5%, atrioventricular canal (AVC)=44.4%, double outlet of main vessels from the right ventricle (DOMV from the RV)=60% , interventricular septal defect with high pulmonary hypertension (VSD)=21.6%, and combined operations was 46.6%. The need for PD after TGV correction was 22.2% (n=2), after TA=100%, after TF=33.3%, after TAPVD=20%, after AVC=11.1%, after VSD=1.9%, and after combined operations was 13.3%. Risk factors for AKI in young children were: younger age, initial heart failure, type of operation, prolonged cardiopulmonary bypass (CPB) and aortic clamping (AC), low cardiac output syndrome, inotropic and vasopressor therapy, hyperlactatemia. The development of AKI led to increased length of stay in the intensive care unit, overall hospitalization and infant mortality.

Management of donor partial anomalous pulmonary veins during lung transplantation

Management of donor partial anomalous pulmonary veins during lung transplantation

Coronary artery and congenital heart diseases in adult patients: a case series

Background: The relevance of coronary artery disease treatment in congenital heart disease is related to the increasing number of older patients with congenital conditions, predisposing factors and continuous silent ischemia.Objective: To analyze clinical data and results of surgery in patients with a combination of congenital heart disease and hemodynamically significant coronary artery lesions.Methods: 17 patients (7 women (41.2%), 10 men (58.8%)) were included in the study. All of them underwent myocardial revascularization and congenital heart disease corrective surgery from 2003 to 2020. The mean age at the time of surgery was 57.2 years. The congenital diagnosis was an atrial septal defect (14 patients), partial anomalous pulmonary vein connection (1 patient), recanalization of atrial septal defect (1 patient), recanalization of ventricular septal defect (1 patient). In 7 cases congenital and coronary pathology correction were performed percutaneously — coronary stenting as the first stage, defect closure at an interval of 4 to 10 days as the second. One patient underwent stenting 7 months prior to an open-heart surgery. In 9 cases one-staged open-heart surgery was performed simultaneously. Results: At the hospital 1 patient died after surgery due to multiple organ dysfunction syndrome. In all other cases there were no symptoms of ischemia on discharge, the patients were in NYHA class I–II (New York Heart Association).Conclusion: The tactics of coronary revascularization is determined by the necessity of percutaneous or open-heart intervention. Myocardial revascularization may be preferable as the first stage, or a one-stage correction may be performed and proved by good results. Received 14 October 2022. Revised 25 November 2022. Accepted 27 November 2022. Informed consent: The patient’s informed consent to use the records for medical purposes is obtained. Funding: The study did not have sponsorship. Conflict of interest: The authors declare no conflict of interest. Contribution of the authorsLiterature review: A.V. MinaevDrafting the article: A.V. Minaev, A.V. Sobolev, I.V. ZemlyanskayaCritical revision of the article: V.P. PodzolkovSurgical treatment: M.R. Chiaureli, V.N. Cheban, E.P. Golubev, K.V. PetrosyanFinal approval of the version to be published: V.P. Podzolkov, A.V. Minaev, M.R. Chiaureli, V.N. Cheban, E.P. Golubev, K.V. Petrosyan, A.V. Sobolev, I.V. Zemlyanskaya

Abstract 15712: Imaging Conundrum: The Diagnosis of Unusual Partial Anomalous Pulmonary Venous Return With Dual Connections

Case: A 68 year old male with a past medical history of hypertension, hyperlipidemia and chronic kidney disease presented for progressive dyspnea on exertion and bilateral lower extremity edema over three weeks. He was found to be in decompensated heart failure and new onset Atrial Flutter with rapid ventricular rate. Transthoracic echocardiography revealed severely impaired left ventricular systolic function with ejection fraction <20%, enlarged right ventricle, moderate right ventricular systolic dysfunction, biatrial enlargement, mild pulmonary hypertension. He was started on guideline directed medical therapy and referred for transesophageal echocardiogram (TEE) with cardioversion for new onset Atrial Flutter and possible tachycardia induced cardiomyopathy. Imaging findings: During TEE, routine doppler of right upper pulmonary vein showed markedly dilated superior vena cava (SVC) (3.6 cm) and unusual predominant outflow with brief mid late systolic inflow (Fig A and B), suggestive of partial anomalous pulmonary venous return (PAPVR). All three other pulmonary veins appeared normal. Also noted to have positive bubble study likely related to PAPVR. Subsequent right heart catheterization left to right shunt with Qp/Qs 1.6, step up noted between SVC and right atrium. CTA revealed partial anomalous right upper lobe pulmonary vein (RUPV) had dual connection to both SVC via window like communication and left atrium (Fig C), no sinus venosus defect seen. Conclusion: This case illustrates an unusual example of PAPVR with bidirectional flow, as well as the importance of thorough evaluation even on routine transesophageal echocardiograms especially for patients with right heart enlargement.

CLINICAL CASE OF BIPLASTIC CORRECTION OF ABNORMAL PULMONARY VEIN DRAINAGE WITH SINUS VENOSUS DEFECT IN A DOMINANT INNOMINATE VEIN IN ADOLESCENCE

Sinus venosus defectis an unusual type of interatrial communication and is almost always combined with partial abnormal pulmonary vein drainage (PAPVD) into the superior venacava (SVC) or right atrium (RA). Different types of venous outflow through the vena cava occur: the presence of an additional left vena cava with different drainage loci to both right and left atriaor, as in our case, the dominant unpaired vein directly draining into the right atrium with outprior communication with the superior right vena cava. This paper describes a clinical case of radical correction of partial anomalous pulmonary vein drainage combined with sinus venosus atrial septal defect (ASD) in a 14-year-old child with dominant venous outflow from the upper half of the body via innominate vein, by combined biplasty from auto pericardial patches, showing adequate upper-venous outflow under cardiopulmonary bypass. It is necessary to provide clear information about congenital heart disease and optimal timing of its surgical correction to a wide audience, not only to the professional medical community, but importantly, to the entire population of the Republic. The provision of publicly available professional information should be highlighted on the official websites of clinics and all available media opportunities, where parents and patients could be fully acquainted with the specific soft entire treatment processing an accessible and convenient information platform.

Sinus venosus atrial septal defect with partial anomalous pulmonary vein return, diagnostic approach of this rare entity.

Sinus venosus atrial septal defect with partial anomalous pulmonary vein return, diagnostic approach of this rare entity.

Late Repairs of Right-Sided Partial Anomalous Pulmonary Veins

In this issue of The Annals of Thoracic Surgery, Griffeth and colleagues1 at the Mayo Clinic describe their results of the surgical repair of right-sided partial anomalous pulmonary veins. In contrast to the typical patient seen in pediatric heart practices, their patients were older children and adults. Unlike school age children undergoing early repair, for the most part, these patients have been subjected to decades of chronic volume loading with resultant adverse atrial and ventricular remodeling.

Non diagnosed PAPVC induce large reverse venovenous shunt after modified Fontan surgery: A case report of a rare anomaly and embolization therapy

Fontan operation is a reliable palliative surgery for patients with single ventricle physiology. Still, the development of complication is common; one of these complications that need to interventional approach is veno-venous collaterals between systemic and pulmonary veins. A 16-yearoldgirl with a history of modified Fontan operation at 9 years ago was referred with progressive cyanosis and dyspnea on exertion. In contrast trans-thoracic echocardiography (TTE), no fenestration was seen in Fontan circulation. Cardiac magnetic resonance revealed partial anomalous pulmonary vein connection (PAPVC) from left upper pulmonary vein to vertical vein and then into the in nominate vein and SVC with the reverse flow from superior vena cava (SVC) to left upper pulmonary vein(LUPV). This anomalous vein became severe engorged and tortuous. Possibly, LUPV and the verticalvein was dilated gradually as a result of increased pressure in the Fontan circuit. Finally, she underwent successful coil embolization in the midpart of the vertical vein. The oxygen saturation increased from80% to 93%.

Sinus septal defect closure with correction of partial anomalous pulmonary vein drainage through mini-thoracotomy

Mini-thoracotomy is one of the most popular minimally invasive approaches. This approach is used in the treatment of congenital and acquired heart diseases and characterized less surgical trauma, intraoperative blood loss, pain syndrome severity, risk of infectious complications and better cosmetic results. Successful correction of atrial septal defect (ASD) through right-sided mini-thoracotomy is reported in the article.

P648 Right heart volume overload after surgical correction of atrial septal defect sinus venosus type with partial anomalous pulmonary veins drainage

Abstract 40 year-old male with history of congenital heart disease. Atrial septal defect (ASD) sinus venosus type associated with partial anomalous pulmonary venous drainage (PAPVD) was diagnosed during childhood and surgically repaired at the age of three. Since then the patient was asymptomatic and he was lost to follow up when reached adulthood. A transthorathic echocardiogram was performed during a hospitalization because of a complicated pneumonia. A severe dilatation of right chambers and main pulmonary artery was observed. The estimated Qp:Qs by this technique was 2.0 but the atrial septum seemed to be intact. Agitated saline was administered in this procedure and no passage of microbubbles was observed. A cardiac MRI was performed because of suspicion of anomalous pulmonary venous drainage. The findings observed in the echocardiogram were confirmed and an uncommon PAPVD was demonstrated by this technique: the superior left pulmonary vein drainaged into a dilated innominated vein. Also, a small pulmonary vein from the right upper lobe emptied into a "venous conduct" (yellow circle) located posterior to the superior cava vein (SCV) that later drainaged into the right atrium under the mouth of the SVC, posterior to the interatrial septum. Surgically correctioin of the PAPVD was performed. Discussion PAPVD is a congenital heart disease characterized by the drainage of one or several pulmonary veins –but not all of them- into the right atrium or systemic veins, which leads to left-to-right shunting. The estimated incidence of this disease ranges between 0.1 and 0.2% of the general population. The right superior pulmonary vein is the most frequently involved and is commonly associated to ASD sinus venosus type. Bilateral anomalous drainage, as occurred in this case, is exceptional. Furhermore, this case highlights the importance of long-term follow-up in patients with congenital heart disease due to the fact that, even when the disease is considered to be cured, long term complications could appear compromising the prognosis of the patient. Abstract P648 Figure. Unusual PAPVD

Does Echocardiography Have a Role in the Cardiologist’s Diagnosis of Innocent Murmurs in Childhood?

Innocent murmurs are common in childhood. Echocardiography may diagnose almost all congenital cardiac abnormalities. Earlier studies have suggested that it may be unnecessary when a clinical diagnosis of an innocent murmur is made by an experienced cardiologist. Yet, echocardiography is commonly performed despite such a diagnosis. Is that justifiable? Patients referred to a paediatric cardiologist for evaluation of a murmur were assessed. If an innocent murmur was clinically diagnosed, an echocardiogram and an electrocardiogram were ordered when requested. The cardiologist completed a questionnaire documenting the reasons for the investigations and his degree of confidence in the clinical diagnosis. The parents and patients were informed of the diagnosis and the results of the investigations. In almost all 62 cases whose murmurs were clinically considered to be innocent, the referring doctor expected an echocardiogram to be performed. Following echocardiography, one patient was found to have a small muscular ventricular septal defect. On review, the murmur was consistent with that diagnosis. Three (3) others had abnormal echocardiograms namely congenitally corrected transposition of great arteries, partial anomalous right upper lobe pulmonary vein, and left ventricular non-compaction. All on review were still considered clinically to have innocent murmurs. Despite the competency of the cardiologist, echocardiography diagnosed important but relatively uncommon cardiac abnormalities with prognostic implications that would have been missed if only the clinical examination was performed. Over and above the referring doctor's expectations for an echocardiogram to be performed is the need for the cardiologist to provide a complete and definitive diagnosis.

Радикальная коррекция частичного аномального дренажа левых легочных вен в коронарный синус с интактной межпредсердной перегородкой

Радикальная коррекция частичного аномального дренажа левых легочных вен в коронарный синус с интактной межпредсердной перегородкой

UNUSUAL COMPLICATION OF PERMANENT PACEMAKER (PPM)

Permanent pacemaker implantation is a common procedure with a low complication risk. However, congenital heart anomalies can lead to devastating and unusual complications. Partial anomalous pulmonary vein return (PAPVR) is rare anomaly in which a pulmonary vein (PV) drains into right atrium or

P1631Results of correction of partial anomalous right upper pulmonary veins connection in the superior vena cava

P1631Results of correction of partial anomalous right upper pulmonary veins connection in the superior vena cava

A clinical study of normothermic open heart surgery

Background: Normothermic open heart surgery is a new approach which is envisaged for better myocardial protection during cardiac surgery based on the concept that chemically arrested heart is perfused continuously with blood and maintained at 37ºC.Methods: This study was conducted in 50 patients at Super Specialty Hospital, Jammu in the Department of CTVS for a period of one year from 2015 to 2016. The patients of all age groups and both sexes were taken up for surgery which included patients of congenital and acquired heart diseases. All patients were undergone thorough history taking, investigations like cardiac enzymes estimation, LFT, RFT, echocardiography, ECG. Cardioplegia used during surgery consisted of warm oxygenated blood, KCL, sodium bicarbonate, hydrocortisone, Mannitol.Results: Majority of patients were in age group of 11-20 years which comprised 30% of all patients. the male and female patients were equal and ratio was 1:1. Per-operative diagnosis was same as pre-operative echocardiographic diagnosis except that one (2%) patient had ASD with partial anomalous pulmonary veins opening into right atrium. One patient (2%) had single ventricle with pulmonary stenosis. Maximum number of patients underwent mitral valve replacement followed by ASD repair. Mean aortic cross-clamp time was 62 patients and mean total CPB was 71.5 minutes. Mean value of volume of blood cardioplegia used was 1100 ml and for priming solution it was 1500 ml. Cardiac enzyme level showed peak rise above normal after 24 hours after surgery in majority of patients.Conclusions: Normothermic open heart surgery can be used in any diverse surgical procedure safely and effectively.

Pulmonary vein obstruction after catheter ablation in a patient with partial anomalous pulmonary vein connection

A 75-year-old man with symptomatic atrial fibrillation was referred to our hospital for catheter ablation. Preprocedural echocardiogram showed mild-moderate degree mitral regurgitation (MR) and mild tricuspid regurgitation (TR) with transvalvular peak gradient (TVPG) of 27mmHg. The complete isolation of the pulmonary veins was performed by radiofrequency ablation, and the patient was kept in sinus rhythm after the procedure. However, the exertional dyspnea had gradually developed one year after the procedure. Echocardiogram showed severe TR with TVPG 60mmHg. MDCT showed partial anomalous pulmonary vein connection (PAPVC) of the left upper pulmonary vein and the occlusion of left lower pulmonary vein ostium: pulmonary venous flow of the left upper lung was drained into the brachiocephalic vein. The patient underwent surgical correction of PAPVC and annuloplasty of the mitral and tricuspid valve. Although it is unclear whether there is direct causal relationship between PAPVC and PV obstruction, anatomical evaluation of PVs is important in patients undergoing catheter ablation.<Learning objective: Partial anomalous pulmonary vein connection (PAPVC) is rare and often asymptomatic in adults. In the present case, pulmonary vein obstruction occurred after radiofrequency catheter ablation in a patient with PAPVC. Although pulmonary venous flow from only left upper lobe drained into the brachiocephalic vein before ablation, occlusion of the left lower pulmonary vein resulted in right heart failure one year after ablation.>

A case of common atrial flutter after a surgical repair of partial anomalous pulmonary vein return– silent change to uncommon flutter during cavo-tricuspid isthmus ablation

A case of common atrial flutter after a surgical repair of partial anomalous pulmonary vein return– silent change to uncommon flutter during cavo-tricuspid isthmus ablation