Multiple ventricular septal defects associated to anomalous venous returns, mitral valve disease, myocardium hypertrophy, and right outflow obstruction: a multimodality imaging assessment.

We report a rare congenital heart disease characterized by multiple ventricular septal defects associated to anomalous systemic and pulmonary venous returns, marked apical myocardial hypertrophy of both ventricles and of right outflow, and hypoplastic mitral anulus. Multimodality imaging is mandatory to assess anatomical details.

Surgical and Cardiac Catheterization Outcomes of Scimitar Syndrome Patients: A Three Decade Single-Center Experience.

Scimitar syndrome (SS) is a rare congenital condition which includes partial anomalous pulmonary venous return (PAPVR) and a variable degree of pulmonary hypoplasia. We describe the clinical features, therapeutic approach and outcomes of patients who underwent cardiac catheterization and/or surgical repair of the scimitar vein at a single institution in the United States. This retrospective cohort study included all patients with SS who underwent scimitar vein surgical repair or cardiac catheterization from October 1989 through August 2021 in a tertiary care center. A total of 84 patients with SS were included and median follow-up time was 74 months. Patients diagnosed with SS under the age of one year had a significantly greater incidence of congenital heart defects (CHD) (p < 0.001), non-cardiac anomalies (p = 0.02), pulmonary hypertension (p = 0.02), and mortality (p = 0.04) compared to those diagnosed over the age of 1 year. Twenty-eight patients underwent surgical repair of the scimitar vein. Overall, eight (10%) patients died. Compared to surviving patients, deceased patients had a significantly higher incidence of pulmonary hypertension (PH), neonatal SS diagnosis, and extracorporeal membrane oxygenation (ECMO) support. Median scimitar vein pressure (20 mmHg) of deceased patients was significantly higher compared to pressures in surviving patients (11 mmHg; p = 0.02). PH, CHD, neonatal SS diagnosis, ECMO support, and markedly elevated scimitar vein pressure are associated with mortality. Scimitar vein surgical repair during infancy is commonly associated with PH and restenosis that requires re-intervention.

Midline crossing right pulmonary veins with dual drainage to the left atrium.

We report the exceptional case of transcatheter treatment of a partial anomalous pulmonary venous drainage of the right lung to the innominate vein and dual drainage to the left atrium.

Superior sinus venosus defect with partial anomalous pulmonary venous return in a patient with tetralogy of Fallot.

We report a case of a 2-year-old boy with tetralogy of Fallot-pulmonary atresia where CT angiography additionally demonstrated the presence of superior sinus venosus defect with partial anomalous pulmonary venous return. Our case highlights the role of CT angiography in the preoperative evaluation in patients with tetralogy of Fallot in identifying such uncommon anomalies, which would help avoid postoperative complications and re-exploration in these patients.

Partial anomalous pulmonary venous return after orthotopic heart transplantation case report.

BACKGROUND: Partial anomalous pulmonary venous return (PAPVR) is a congenital heart defect. Reports of repair and treatment in pediatric cases have been published, but incidence of PAPVR in adults is not common. To our knowledge, there has not been a diagnosis of left-sided PAPVR after a heart transplant an in adult patient. CASE PRESENTATION: A 62-year-old patient with ischemic cardiomyopathy and systolic heart failure underwent orthotopic heart transplantation. The immediate post-operative course was remarkable for an elevated cardiac index and pulmonary artery pressures as well as decreased systemic vascular resistance. The post-operative echocardiogram did not reveal an intra-cardiac shunt. However, computed tomographic angiography (CTA) showed a left superior pulmonary vein draining into the innominate vein. Operative repair of the left superior pulmonary venous connection to the left atrial appendage was completed under cardiopulmonary bypass with beating heart. Her hemodynamics improved immediately, and she had an unremarkable postoperative course. CONCLUSIONS: While uncommon, any patient with a high cardiac output and abnormal hemodynamics after heart transplant should be evaluated for the existence of a shunt. While not a part of all traditional preoperative imaging protocols, a chest CTA should be considered if PAPVR is suspected as it can both diagnose the condition and enable a plot of the corrective course of surgical action.

Recurrent fibrovascular granulation on PhotoFix® bovine pericardium causing systemic and pulmonary venous obstruction after repair of scimitar syndrome.

A young adult with late diagnosis of scimitar syndrome underwent infradiaphragmatic baffling of the scimitar vein to left atrium through an intra-atrial tunnel using PhotoFix® bovine pericardium with recurrent extensive fibrovascular granulation of the patch causing pulmonary and systemic venous obstruction leading to eventual explantation of the bovine pericardium.

Quantification of pulmonary/systemic shunt ratio by single-acquisition phase-contrast cardiovascular magnetic resonance.

PURPOSE: Phase-contrast cardiovascular magnetic resonance (PC-CMR) quantification of intracardiac shunt (measuring the pulmonary to systemic flow ratio, Qp/Qs) is typically determined by measuring flow through planes perpendicular the pulmonary trunk (PA) and ascending aorta (Ao). This method is subject to error from presence of background velocity offsets and requires two scan acquisitions. We evaluated an alternate PC-CMR technique for quantifying Qp/Qs using a single modified plane that encompasses both the PA and Ao. MATERIAL AND METHODS: In 53 patients evaluated for intracardiac shunting, PC-CMR measurement in the individual Ao and PA planes and also in a single-acquisition plane was obtained and Qp/Qs calculated by each method. Bland-Altman analysis was performed to evaluate the agreement between the two methods. RESULTS: The 95% confidence limits of agreement ranged from -0.52 to +0.34 indicating good agreement between the two methods. There was excellent agreement on the clinically relevant threshold value of Qp/Qs ratio of 1.5 (representing criteria for surgical correction of shunt). CONCLUSIONS: Qp/Qs determined from the single-acquisition approach agrees well with that of the individual PA and Ao method and offers potential improved accuracy (due to background velocity offset).

Steroids as a possible effective therapy in the management of large isolated chylopericardium following open heart surgery.

BACKGROUND: Chylopericardium is the collection of lymph fluid inside the pericardial cavity. The incidence of chylopericardium is very low, as this diagnosis is rarely reported following cardiac procedures in children. While some reports were published worldwide on isolated chylopericardium after cardiac surgeries for diverse reasons, it has never been reported after repair for partial anomalous pulmonary venous return. In addition, management of this diagnosis ends up being surgical with minimal concentration on medical treatment which proved unsuccessful. We present a medical approach with corticosteroids as an effective method to treat isolated chylopericardium. CASE PRESENTATION: In this manuscript, we present an approach to treat isolated post-operative chylopericardium in a child following repair of partial anomalous pulmonary venous return. Chylous drainage responded to corticosteroids and completely ceased. There was no need for surgical intervention. CONCLUSION: Until now, isolated chylopericardium has never been reported to occur with partial anomalous pulmonary venous return repair. A review of the literature showed that most patients follow a conservative approach consisting of diuretics and non-steroidal anti-inflammatory agents with some of them undergoing surgical re-intervention. With future research on the topic still needed, we hope that this will encourage physicians worldwide to consider administering a trial of corticosteroids as an option to treat chylopericardium.

Biatrial drainage of right superior caval vein with preferential streaming into the left atrium.

Biatrial drainage of the right superior caval vein is an extremely rare cardiac anomaly that generally presents in childhood. We present a case of anomalous connection of the right superior caval vein with superior sinus venosus atrial septal defect and partial anomalous pulmonary venous return in a 5-month-old male presenting with unexplained cyanosis and hypoxia.

Multimodality imaging of scimitar syndrome in adults: A report of four cases.

Partial anomalous pulmonary venous return (PAPVR) comprises a group of congenital cardiovascular anomalies associated with pulmonary venous flow directly or indirectly into the right atrium. Scimitar syndrome is a variant of PAPVR in which the right lung is drained by right pulmonary veins connected anomalously to the inferior vena cava. Surgery is the definitive treatment for scimitar syndrome. However, it is not always necessary as many patients are asymptomatic, have small left-to-right shunts, and enjoy a normal life expectancy without surgery. We report multimodality imaging in four adults with scimitar syndrome and the implications for management of this rare syndrome.

A rare case of a patient with aortic root aneurysm, bicuspid aortic valve, and Scimitar syndrome with anomalous venous return to the right superior pulmonary vein.

A rare case of Scimitar Syndrome with an abnormal drainage of the right lower pulmonary vein into the right superior pulmonary vein associated with aortic root aneurism and bicuspid aortic valve is reported.

Acute Type A Aortic Dissection with Iliac Malperfusion in a Patient with Scimitar Syndrome.

We encountered a 35-year-old male patient with scimitar syndrome, acute type A aortic dissection, and severe iliac malperfusion. He presented with pain and weakness of the left lower extremity and underwent ascending aorta replacement through sternotomy and right anterior thoracotomy. Left iliac artery flow was recovered after the operation and the patient was discharged on the tenth day without any complication. The purpose of this report is to discuss the considerations in aortic surgery in patients with scimitar syndrome, and the surgical principles for treatment of aortic dissection with critical limb ischemia.

Anomalous Pulmonary Venous Return: Insights Into Prenatal Detection.

OBJECTIVES: To review all cases of total anomalous pulmonary venous return (TAPVR) or partial anomalous pulmonary venous return (PAPVR) identified prenatally or postnatally at a single institution and to identify factors that may lead to a correct or missed diagnosis in both high- and low-risk fetuses on screening examinations. METHODS: Fetal images from 16 cases of prenatally or postnatally diagnosed T/PAPVR were retrospectively reviewed to analyze factors that influenced interpretations and diagnoses. RESULTS: Sixteen diagnoses of T/PAPVR were made, with a final number of 10 confirmed cases, 1 of which was PAPVR. Ten fetuses with a presumptive diagnosis of T/PAPVR before delivery were at an average gestational age of 24.7 weeks, with 5 cases diagnosed postnatally. None of the diagnoses of isolated TAPVR were made during a screening examination. Twelve of the pregnancies were complicated by complex cardiac defects, including 6 with heterotaxy syndromes. Of the 5 abnormal cases identified in the postpartum period, 3 had isolated TAPVR. In the 3 patients with isolated defects, prenatal echocardiography was not performed; the anatomy scan interpretations were confounded by multiple factors. In retrospect, there was no obvious sonographic evidence of TAPVR in these patients; however, color flow Doppler imaging of the pulmonary veins was not performed on any of them. CONCLUSIONS: Although fetal echocardiography has improved the overall detection of TAPVR or PAPVR, this abnormality continues to elude prenatal diagnosis during screening in both low- and high-risk patients. We hypothesize that the use of color flow Doppler imaging in the 4-chamber view may assist in diagnosing TAPVR in screening low-risk patients, especially in those with difficult scans.

Persistent primitive hepatic venous plexus with Scimitar syndrome: description of a case and review of the literature.

Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.

Right S3 segmentectomy for lung cancer with partial anomalous pulmonary venous return in the right upper pulmonary vein: A case report.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital malformation where the pulmonary vein partially refluxes into the venous system. Here, we present the first robotic-assisted right S3 segmentectomy in a 70-year-old male with early-stage lung cancer and PAPVR in the right upper pulmonary vein. The patient, with suspected primary lung cancer (11 mm diameter, pure solid appearance in right S3 segment), exhibited clinical stage T1bN0M0 stage IA2. Preoperative computed tomography revealed severe lung emphysema, and right V1-3 returned directly to the superior vena cava. However, no signs of right-sided heart failure were observed, and echocardiogram was normal with a pulmonary-to-systemic blood flow ratio of 1.4. Successful robot-assisted right S3 segmentectomy with hilar nodal dissection was performed, and the patient was discharged on the sixth postoperative day without complications. One year postoperatively, there has been no recurrence of lung cancer or respiratory/right-sided heart failure symptoms.

Sinus Venosus Atrial Septal Defect as an Overlooked Source of Shortness of Breath Among Patients With Pulmonary Arterial Hypertension.

Sinus venosus atrial septal defects (SVASD) associated with partial anomalous pulmonary venous return (PAPVR) can be overlooked as a source of dyspnea in adult patients with pulmonary hypertension. We present the case of a 61-year-old male with exertional dyspnea initially attributed to pulmonary hypertension, who was subsequently diagnosed with SVASD and right superior PAPVR. This case underscores the critical importance of maintaining high clinical awareness and utilizing multimodal imaging techniques in cardiology to accurately diagnose and manage pulmonary hypertension secondary to congenital heart disease. Timely surgical correction can significantly improve morbidity and mortality outcomes.

An Unexpected Diagnostic Twist in an Elderly Patient: No Heart Failure With Preserved Ejection Fraction.

Heart failure with preserved ejection fraction (HFpEF) is considered to be the dominant cause of dyspnea and pulmonary hypertension (PH) in elderly patients with preserved left ventricular systolic function and cardiovascular comorbidities. However, it is important to keep in mind that left ventricular diastolic dysfunction is not the only possible cause of PH in cases of late-onset clinical manifestation. A multiparametric approach is essential for accurate diagnosis and therapeutic decision-making. A 74-year-old patient was admitted due to progressive dyspnea and suspicion of PH. Given the patient's risk profile, HFpEF and concomitant post-capillary PH were anticipated. Despite negative findings on CT angiography and transesophageal echocardiography, right heart catheterization was performed, revealing discrepant oxygen saturations in the superior vena cava and right atrium. A partial anomalous pulmonary venous return and an atrial septal defect were identified through cardiac magnetic resonance imaging.

Three-Dimensional-Enabled Surgical Planning for the Correction of Right Partial Anomalous Pulmonary Venous Return.

Objectives: The surgical technique for right partial anomalous pulmonary venous return (PAPVR) depends on the location of the anomalous pulmonary veins (PVs). With this in mind, we sought to evaluate the impact of 3D heart segmentation and reconstruction on preoperative surgical planning. Methods: A retrospective study was conducted on all patients who underwent PAPVR repair at our institution between January 2018 and October 2021; three-dimensional segmentations and reconstructions of all the heart anatomies were performed. A score (the PAPVR score) was established and calculated using two anatomical parameters (the distance between the most cranial anomalous PV and the superior rim of the sinus venosus defect/the sum of the latter and the distance between the PV and the azygos vein) to predict the type of correction. Results: A total of 30 patients were included in the study. The PAPVR score was found to be a good predictor of the type of surgery performed. A value > 0.68 was significantly associated with a Warden procedure (p < 0.001) versus single/double patch repair. Conclusions: Three-dimensional heart segmentations and reconstructions improve the quality of surgical planning in the case of PAPVR and allow for the introduction of a score that may facilitate surgical decisions on the type of repair required.

Atrial Fibrillation Ablation in a Patient With SV ASD and PAPVR Preceding Transcatheter Septal Closure.

Atrial fibrillation (AF) is common in adults with unrepaired atrial septal defects (ASDs). Sinus venosus (SV) ASDs associated with partial anomalous pulmonary venous return (PAPVR) are traditionally managed surgically. We report the first AF catheter ablation in a patient with SV ASD and PAPVR preceding transcatheter ASD repair with a covered stent. (Level of Difficulty: Advanced.).