Pulmonary Venous Abnormalities Research Articles

The role of multi-detector computed tomography in assessment of pulmonary venous abnormalities on PRE ablation mapping for atrial fibrillation

The role of multi-detector computed tomography in assessment of pulmonary venous abnormalities on PRE ablation mapping for atrial fibrillation

Computed tomography of pulmonary veins: review of congenital and acquired pathologies.

Newer-generation CT scanners with ultrawide detectors or dual sources offer millisecond image acquisition times and significantly decreased radiation doses compared to historical cardiac CT and CT angiography. This technology is capable of nearly freezing cardiac and respiratory motion. As a result, CT is increasingly used for diagnosing and monitoring cardiac and vascular abnormalities in the pediatric population. CT is particularly useful in the setting of pulmonary vein evaluation because it offers evaluation of the entire pulmonary venous system and lung parenchyma. In this article we review a spectrum of congenital and acquired pulmonary venous abnormalities, including potential etiologies, CT imaging findings and important factors of preoperative planning. In addition, we discuss optimization of CT techniques for evaluating the pulmonary veins.

Use of 3-dimensionally printed heart models in the planning and simulation of surgery in patients with Raghib syndrome (coronary sinus defect with left superior vena cava).

Use of 3-dimensionally printed heart models in the planning and simulation of surgery in patients with Raghib syndrome (coronary sinus defect with left superior vena cava).

Role of MDCT in evaluation of congenital and acquired anomalies of pulmonary venous drainage

ObjectiveThis work aimed for detection of different congenital and acquired abnormalities affecting the pulmonary veins using multislice computed tomography (MSCT). Patients and methodsThis study was carried on 74 patients. Cases were referred to Radiology department of Kasr Al-Ainy with different chest complaints for MSCT and only cases showing pulmonary venous abnormalities were included in this study. All patients underwent MSCT of the chest using different MSCT protocols according to the initial presentation of the patient. ResultsThis study included 74 patients with pulmonary veins abnormalities; congenital anomalies of the pulmonary veins were observed in 36.4% of cases and acquired abnormalities in 62.2% of cases, one case had both congenital and acquired pulmonary vein abnormalities (1.4%). The most common congenital anomaly was pulmonary vein varices while the most common acquired abnormality was pulmonary vein stenosis or obstruction. ConclusionThe presence of different pulmonary venous abnormalities can be easily delineated using MSCT, with its MPR capability, as it provides excellent resolution and a large field of view.

Noncompaction cardiomyopathy and heterotaxy syndrome

Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. This study sought to determine whether a non-random association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy.In a retrospective review of the Indiana Network for Patient Care, we identified 172 patients with heterotaxy (69 male, 103 female). Echocardiography and cardiac magnetic resonance imaging results were independently reviewed by two cardiologists to ensure reproducibility of LVNC. A total of 13/172 (7.5%) patients met imaging criteria for LVNC. The CHD identified in this subgroup included atrioventricular septal defects [11], dextrocardia [10], systemic and pulmonary venous return abnormalities [7], and transposition of the great arteries [5]. From this subgroup, 61% (n=8) of the patients developed arrhythmias; and 61% (n=8) required medical management for chronic heart failure.This study indicates that LVNC has increased prevalence among patients with heterotaxy when compared to the general population (0.014–1.3%) suggesting possible common genetic mechanisms. Interestingly, mice with a loss of function of Scrib or Vangl2 genes showed abnormal compaction of the ventricles, anomalies in cardiac looping, and septation defects in previous studies. Recognition of the association between LVNC and heterotaxy is important for various reasons. First, the increased risk of arrhythmias demonstrated in our population. Secondly, theoretical risk of thromboembolic events remains in any LVNC population. Finally, many patients with heterotaxy undergo cardiac surgery (corrective and palliative) and when this is associated with LVNC, patients should be presumed to incur a higher peri-operative morbidity based on previous studies. Further research will continue to determine long-term and to corroborate genetic pathways.

Pulmonary venous abnormalities encountered on pre-radiofrequency ablation mapping multidetector computed tomography

Multidetector computed tomography (MDCT) elegantly renders pulmonary venous anatomy. With increasing numbers of radiofrequency ablation procedures being performed, there is now a greater emphasis on pre-procedure imaging to delineate this anatomy. Pulmonary venous mapping studies can be performed with or without ECG-gating. However, ECG-gating improves both the quality of 3D images and the accuracy of pulmonary vein (PV) ostial diameter measurements. Including the superior thorax, and not just the left atrium and central PVs, allows visualization of aberrant pulmonary venous drainage to the brachiocephalic veins or superior vena cava. Normally, there are two superior PVs, one right and one left, and two inferior PVs, one right and one left. The right superior vein usually drains the right upper and middle lobe. The left superior vein drains the left upper lobe including the lingula. The inferior veins drain their respective lower lobe. PV anatomy is more variable than pulmonary arterial anatomy, and developmental anomalies are common. This article describes, illustrates and reviews the common anomalies of the PVs in our experience performing over 1000-pre-radiofrequency ablation cardiac MDCT studies. The commonest anomalies are supernumerary or accessory veins (on the right) and a (left) common trunk. More rarely, partial anomalous pulmonary venous return and Cor triatriatum are seen, and rarest of all is total anomalous pulmonary venous return, PV varix and single or multiple vein stenosis or atresia.

The concept of double inlet-double outlet right ventricle: a distinct congenital heart disease.

The aim of this study was to estimate the incidence and to analyze the anatomy of double inlet-double outlet right ventricle complex and its associated cardiac anomalies in our autopsy series. Among the 1640 hearts with congenital heart disease of our Anatomical Collection, we reviewed the specimens with double inlet-double outlet right ventricle, according to the sequential-segmental analysis, identifying associated cardiac anomalies and examining lung histology to assess the presence of pulmonary vascular disease. We identified 14 hearts with double inlet-double outlet right ventricle (0.85%). Right atrial isomerism was observed in 10 hearts, situs solitus in 3 and left atrial isomerism in one. Regarding the mode of atrioventricular connection, all hearts but one had a common atrioventricular valve. Systemic or pulmonary venous abnormalities were noted in all patients with atrial isomerism. In nine patients a valvular or subvalvular pulmonary stenosis was present. Among the functionally "univentricular hearts", double inlet- double outlet right ventricle represents a peculiar entity, mostly in association with right atrial isomerism. Multiple cardiac anomalies are associated and may complicate surgical repair.

Autopsy findings of 19 cases of pulmonary vein abnormalities associated with fetal cardiac anomalies

To improve the diagnostic accuracy of fetal pulmonary venous abnormalities through the analysis of the fetal pulmonary vein anatomy. 234 cases of congenital cardiac abnormalities were detected by echocardiography during pregnancy in An Zhen Hospital, Capital Medical University from May 2010 to August 2015. Autopsy was then performed. The type of fetal pulmonary venous malformation, cardiac abnormalities, systemic venous malformations, and other internal organs deformities were documented. There were ninteen cases of pulmonary venous malformations among the 234 cases of fetal congenital heart disease. These included two cases of congenital pulmonary venous hypoplasia (CPVH) or atresia, four cases of partial anomalous pulmonary venous drainage (PAPVD), seven cases of total anomalous pulmonary venous drainage (TAPVD), five cases of atresia of common pulmonary vein (CPV), one case of congenital pulmonary venous hypoplasia with total anomalous pulmonary venous drainage. There were eleven cases with single ventricle, eight cases with right aortic arch, seven cases with single atrium and six cases with pulmonary valve stenosis. Eleven cases had pulmonary hypoplasia and nine cases had abnormal spleen. There are many variations in pulmonary venous abnormalities associated with severe and complex cardiac abnormalities and internal organs malformation. Care should be exercised during autopsy examination to look for all branches of the pulmonary vein.

The Spectrum of Congenital Heart Disease and Outcomes After Surgical Repair Among Children With Turner Syndrome: A Single-Center Review

Turner syndrome (TS), a genetic abnormality affecting 1 in 2,500 people, is commonly associated with congenital heart disease (CHD). However, the surgical outcomes for TS patients have not been well described. This study reviewed the spectrum of CHD in TS at the authors' center. The authors report outcomes after coarctation of the aorta (CoA) repair or staged palliation of hypoplastic left heart syndrome (HLHS) and then compare the surgical outcomes with those of non-TS patients undergoing like repair. This retrospective chart review was conducted at the Children's Hospital of Wisconsin from 1999 to 2011. Of the 173 patients with TS, 77 (44.5%) were found to have CHD. Left-sided obstructive lesions were the most common. However, the spectrum of CHD was wide and included systemic and pulmonary venous abnormalities as well as abnormalities of the coronary arteries. In the comparative analysis of CoA repair, the TS patients younger than 60days had longer aortic cross-clamp times (24 vs. 16min; p=0.001) and longer hospital stays (12 vs. 6days; p≤0.0001) than the non-TS patients. At the follow-up assessment after 8.8±9.1years, 17% of the TS patients had hypertension, but no patient had required reintervention, and no deaths had occurred. Finally, three of the four TS patients with HLHS died within the first year. The spectrum of CHD within TS is wide and not limited to bicuspid aortic valve or CoA. Additionally, patients with TS undergoing CoA repair may have a more challenging early postoperative course but experience outcomes similar to those of non-TS patients. Finally, patients who have TS combined with HLHS remain a challenging population with generally poor survival.

Sağ Akciğer Hipoplazisi, Dekstrokardi ve Viseral Malpozisyonu Olan Nadir Bir Olgu Sunumu

Lung hypoplasia is often associated with pulmonary venous return abnormalities, referred to as the Scimitar syndrome, in pediatric patients. A two day-old male patient presented to our clinic with respiratory distress and mild cyanosis. Diagnostic studies revealed dextrocardia, right sided hypoplasia of upper and middle lung lobes and enlargement of the left lung due to the compensation, midline liver, right sided stomach and right sided spleen. No pulmonary venous return abnormalities were detected. This is the first report of lung hypoplasia associated with heterotaxy, visceral malposition and normal pulmonary venous return.

Echocardiographic parameters associated with pulmonary congestion in outpatients with Chagas' cardiomyopathy and non-chagasic cardiomyopathy

Despite significant left ventricular (LV) systolic dysfunction and cardiomegaly, pulmonary congestion does not seem to be a major finding in Chagas' cardiomyopathy (CC). This study sought to identify echocardiographic parameters associated with pulmonary congestion in CC and in dilated cardiomyopathy of other etiologies, such as non-CC (NCC), and to compare pulmonary venous hypertension between the two entities. A total of 130 consecutive patients with CC and NCC, with similar echocardiographic characteristics, were assessed using Doppler echocardiography and chest radiography. Pulmonary venous vessel abnormalities were graded using a previously described pulmonary congestion score, and this score was compared with Doppler echocardiographic parameters. NCC patients were older than CC patients (62.4 ± 13.5 × 47.8 ± 11.2, p = 0.00), and there were more male subjects in the CC group (66.2% × 58.5%, p = 0.4). Pulmonary venous hypertension was present in 41 patients in the CC group (63.1%) and in 63 (96.9%) in the NCC group (p = 0.0), the mean lung congestion score being 3.2 ± 2.3 and 5.9 ± 2.6 (p = 0.0), respectively. On linear regression multivariate analysis, the E/e' ratio (β = 0.13; p = 0.0), LV diastolic diameter (β = 0.06; p = 0.06), left atrial diameter (β = 0.51; p = 0.08), and right ventricular (RV) end-diastolic diameter (β = 0.02; p = 0.48) were the variables that correlated with pulmonary congestion in both groups. Pulmonary congestion was less significant in patients with CC. The degree of LV of systolic and diastolic dysfunction and the RV diameter correlated with pulmonary congestion in both groups. The E/e' ratio was the hallmark of pulmonary congestion in both groups.

Pseudo-Scimitar syndrome

The pulmonary venous abnormalities are very rare and are difficult to diagnose. We present a case of middle age male who presented with cough and chest pain. Chest radiograph showed abnormal tortuous vessel in right lung. Computed tomographic angiography of chest demonstrated meandering anomalous right superior pulmonary vein.

Mutations in ZIC3 and ACVR2B are a common cause of heterotaxy and associated cardiovascular anomalies

Heterotaxy syndrome is caused by left-right asymmetry disturbances and is associated with abnormal lateralisation of the abdominal and thoracic organs. The heart is frequently involved and the severity of the abnormality usually determines the outcome. We performed a direct sequence analysis of the coding sequence of genes including Zinc Finger Protein of the Cerebellum 3, Left-Right Determination Factor 2, Activin A Receptor Type IIB, and Cryptic in 47 patients with laterality defects and congenital cardiac disease. Of the 47 patients, 31 (66%) had atrioventricular septal defects, 34 (72%) had abnormal systemic venous return, 25 (53%) had transposed or malposed great arteries, and 20 (43%) had pulmonary venous abnormalities. We identified two novel genetic changes in Zinc Finger Protein of the Cerebellum 3, and these variants were not present in 100 ethnically matched control samples. One previously reported missense mutation in Activin A Receptor Type IIB was identified in two unrelated subjects. The genetic changes identified in this study are all located in conserved regions and are predicted to affect protein function in left-right axis formation and cardiovascular development. Mutations in Zinc Finger Protein of the Cerebellum 3 and Activin A Receptor Type IIB were identified in 4 of the 47 patients with heterotaxy syndrome for a yield of approximately 8.5%. Our results expand the mutation spectrum of monogenic heterotaxy syndrome with associated cardiac anomalies and suggest that there are other causes of heterotaxy yet to be identified.

Ellis–van Creveld Syndrome and Congenital Heart Defects: Presentation of an Additional 32 Cases

Ellis-van Creveld (EVC) syndrome is a rare genetic abnormality that has been linked to a mutation in the EVC or EVC2 genes. Common atrium (CA) is an uncommon cardiac malformation, and yet it is commonly found in patients with EVC. We performed a retrospective review of the cases submitted to the Pediatric Cardiac Care Consortium (PCCC) between 1982 and 2007. A review of the English-language literature for previously published cases, as well as current genetic research findings, was also performed. Thirty-two pediatric patients with congenital heart disease (CHD) and EVC syndrome were identified in the PCCC database. Twenty-eight (88%) had an endocardial cushion defect, with 15 of these having primary failure of atrial septation resulting in CA. Persistent left superior vena cava (LSVC) and pulmonary venous connection abnormalities were common. The incidence of persistent LSVC and pulmonary venous abnormalities were greater than previously reported for patients with EVC. Our study reviews the reported literature and adds 32 additional cases from the PCCC database. Review of the cardiac phenotype in patients with EVC syndrome reveals a characteristic pattern of atrioventricular canal defects with systemic and pulmonary venous abnormalities. The frequent association of these abnormalities is strongly reminiscent of the cardiac phenotype found in patients with heterotaxy syndromes. Emerging molecular and developmental studies suggest that EVC and EVC2 proteins may be important for cilia function, which is implicated in the pathogenesis of heterotaxy syndromes. It is speculated that coordinate function between the EVC proteins is required for a cilia-dependent cardiac morphogenesis.

Echocardiography in Pediatric and Adult Congenital Heart Disease

Benjamin W. Eidem, MD, FACC, FASE; Frank Cetta, MD, FACC, FASE; and Patrick W. O’Leary, MD, FACC, FASE, eds. 500 pages. Philadelphia, USA: Lippincott Williams and Wilkins, 2010. $229.00 ISBN 0-7817-8136-1 It has been nearly 8 years since a textbook on echocardiography specifically for pediatric congenital heart disease (CHD) has been published. A specific work on both pediatric and adult CHD emphasizing imaging is a new endeavor. Three expert senior clinicians from the Mayo Clinic are the editors and primary authors of this multiauthor textbook, which includes young investigators, midcareer investigators, and senior experts in pediatric and congenital heart echocardiography and imaging from around the United States and Canada. In their Introduction, the editors, citing the evolution of echocardiography, remind us of the major contributions of Drs James Seward and Jamil Tajik and Drs William Edwards and Donald Hagler to the field of 2-dimensional echocardiography and CHD in 1987, after the original investigations of M-mode echocardiography and 2-dimensional echocardiography by this reviewer (D.J.S.). Richard Meyer and Nils Lundstrom, the editors of this new text, also, in their Introduction, give special attention to Dr. Edwards, the renowned cardiac pathologist and a major resource. This textbook is well organized. The first chapter relates to principles of cardiovascular ultrasound, followed by practical issues, anatomic orientation, and segmental cardiovascular analysis; quantitative methods for echocardiography, basic and advanced; and then lesion-oriented chapters, including pulmonary venous abnormalities and abnormalities of the atrial septum, atrial ventricular septal defects, Ebstein malformation, mitral valve abnormalities, congenitally corrected transposition, ventricular septal defects, univentricular connections, …

Unusual Cardiac Associations with Tetralogy of Fallot—A Descriptive Study

We present a large single-center series (>2200 cases) operated for Tetralogy of Fallot (TOF). We analyzed the incidence of associated unusual and uncommonly described cardiac lesions and their diagnostic and therapeutic implications in TOF patients. This retrospective study was conducted by reviewing records of patients operated for TOF at a large tertiary care pediatric cardiac centre. From 2002 to 2008, a total of 2235 cases of TOF were evaluated with echocardiography, cardiac catheterization, and/or cardiac computed tomography followed by cardiac surgery. Known and well-described associations were excluded from the study. Unusual associations were tabulated. Several unusual associations having an incidence >0.1% were detected. These included subaortic membrane (1%), pulmonary venous abnormalities (0.5%), small left ventricle (0.5%), interrupted inferior vena cava (0.5%), mitral valve abnormalities (0.4%), hemitruncus (0.4%), tricuspid valve abnormalities (0.4%), biventricular dysfunction (0.3%), retroaortic innominate vein (0.3%), bicuspid aortic valve (0.2%), and pericardial effusion (0.2%). This series describes unusual, not previously routinely reported cardiac lesions associated with TOF that may affect management and should be sought on preoperative evaluation. We demonstrate that more unusual associations do exist in not infrequent numbers, i.e., a TET is not just a TET.

Functional state of patients with heterotaxy syndrome following the Fontan operation

Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation. In the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy. Patients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p=0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p=0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p=0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p=0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy. The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections

Pulmonary venous abnormalities are generally diagnosed by echocardiography and often confirmed by cardiac catheterization. Although angiography has been the gold standard for evaluation it carries certain inherent risks, especially in small and sick infants. In this study we retrospectively assessed the utility of magnetic resonance angiography (MRA) and computed tomography angiography (CTA) in the evaluation of pulmonary venous abnormalities in pediatric patients. The results were compared with operative findings. We reviewed nine patients with total APVC and four with partial APVC. Twelve patients were infant (age range: 1 day to 8 months) and the other was 16 years old. MRA and/or CTA clearly visualized the anomalous drainage of pulmonary veins in all patients. Cardiac catheterization was performed five patients with one total anomalous pulmonary venous connection and four with partial anomalous pulmonary venous connection. Ten patients were operated on. Diagnoses of patients were confirmed by operative findings. In Conclusions, MR and CT angiography allow detailed and comprehensive evaluation of the APVC and are good diagnostic modalities for use in the preoperative assessment of the anomaly in pediatric patients.

Fetal Cardiac Interventions

Team approach is essential to the success of an interventional cardiac program. The parents should be counseled jointly by the fetal medicine team and the cardiologist, and the risk of fetal demise during the procedure or after it made explicit. In cases in which pulmonary venous Doppler abnormalities exist, the parents need to understand that the risk of neonatal death is high as a result of the combination of severe pulmonary and cardiac pathology. Fetal therapy may prove beneficial in selected cases, minimizing secondary damage to the fetal myocardium and pulmonary vascular bed, and therefore may be more successful if performed early, rather than later, in pregnancy. Continued development of smaller and specialized equipment will help in this endeavor and is to be encouraged. Patient selection will best be rationalized by multicenter cooperation to establish an intention-to-treat database.

MRI of the Pulmonary Veins: Comparison Between 3D MR Angiography and T1-Weighted Spin Echo

The purpose was to determine the ability of three-dimensional (3D) magnetic resonance (MR) angiography to depict normal pulmonary veins in comparison with spin-echo MR imaging. MR imaging of 40 patients with cardiovascular disease were reviewed. Patients with known pulmonary venous abnormalities were excluded. Using a standard GE 1.5-T magnet, axial T1-weighted spin-echo 5-mm-thick contiguous slices and 3D MR angiography (contiguous slice thickness of 2.5-3.5 mm, 20-30 c.c. of gadolinium bolus at 1-1.5 c.c./sec, 32-43-second breath-hold, coronal and sagittal plane acquisition) were evaluated retrospectively on separate occasions by two experienced radiologists. Multiplanar imaging projection was used for the identification of pulmonary veins. Each lung was considered to have two drainage veins: a superior vein and an inferior vein. Identification of a pulmonary vein was made by visualizing a connection with the left atrium. 143 pulmonary veins (87.5% +/-5.2) were identified at the level of the left atrium on T1-weighted spin-echo images, and 157 (98.1% +/-1.9) were identified on 3D MR angiography (p<0.01). Overall we identified by T -weighted spin-echo imaging 36 right upper, 38 right lower, 27 left upper, and 38 left lower pulmonary veins. By 3D MR angiography, we identified 38 right upper, 40 right lower, 39 left upper, and 40 left lower pulmonary veins. All four pulmonary veins were detected in 22 patients on spin-echo imaging (55%) and in 37 patients (92.5%) on 3D MR angiography (chi = 3.81, p<0.05). A significant difference is demonstrated between 3D MR angiography and spin-echo MR imaging in identifying normal pulmonary veins. MR angiography provides a complete view of normal pulmonary venous anatomy and could be a valuable tool for the assessment of abnormal pulmonary venous drainage.