Pulmonary Venous Connection Research Articles

Caval vein obstruction resulting in right to left shunt and desaturation post endocardial pacing implantation in a 3 year old: a case report

Abstract Background Superior caval vein obstruction is a rare complication of endocardial pacing lead implantation that can result in a right to left shunt. Case summary A 3-year-old child with type 2 Brugada syndrome presented with mild cyanosis post endocardial pacing implantation due to evolutionary right superior caval vein obstruction. This obstruction resulted in a right to left shunt across a previously unrecognized patent levo-atrial cardinal vein associated with partial anomalous pulmonary venous drainage. The patient underwent endocardial pacing explantation, balloon dilation and stenting of the right superior caval vein, banding of the levo-atrial cardinal vein to left upper pulmonary vein venous channel and implantation of an epicardial pacing system. Discussion Levo-atrial cardinal vein and partial anomalous pulmonary venous drainage with dual drainage can go undetected on cardiac imaging and may not ever cause symptoms (high left-to-right shunt or cyanosis). The levo-atrial cardinal vein and associated partial anomalous venous drainage with dual drainage was missed on multiple occasions and with multiple imaging modalities in our patient. Blood flow may not be detected in a small calibre or collapsed levo-atrial cardinal vein with pulmonary venous connection when the pulmonary vein remains widely patent and connected to the left atrium. Detailed comprehensive echocardiography of any child referred for cardiac intervention should include pulsed wave Doppler and colour flow mapping interrogation of the innominate vein from the suprasternal approach.

EP01.12: Developmental malformations of the fetal systemic and pulmonary venous connections: a systematic ultrasound approach and clinical implications

EP01.12: Developmental malformations of the fetal systemic and pulmonary venous connections: a systematic ultrasound approach and clinical implications

3D Modeling and Printing in Congenital Heart Surgery: Entering the Stage of Maturation.

3D printing allows the most realistic perception of the surgical anatomy of congenital heart diseases without the requirement of physical devices such as a computer screen or virtual headset. It is useful for surgical decision making and simulation, hands-on surgical training (HOST) and cardiovascular morphology teaching. 3D-printed models allow easy understanding of surgical morphology and preoperative surgical simulation. The most common indications for its clinical use include complex forms of double outlet right ventricle and transposition of the great arteries, anomalous systemic and pulmonary venous connections, and heterotaxy. Its utility in congenital heart surgery is indisputable, although it is hard to “scientifically” prove the impact of its use in surgery because of many confounding factors that contribute to the surgical outcome. 3D-printed models are valuable resources for morphology teaching. Educational models can be produced for almost all different variations of congenital heart diseases, and replicated in any number. HOST using 3D-printed models enables efficient education of surgeons in-training. Implementation of the HOST courses in congenital heart surgical training programs is not an option but an absolute necessity. In conclusion, 3D printing is entering the stage of maturation in its use for congenital heart surgery. It is now time for imagers and surgeons to find how to effectively utilize 3D printing and how to improve the quality of the products for improved patient outcomes and impact of education and training.

A Rare Associated Anomaly in Tetralogy of Fallot: Scimitar Syndrome in an Infant.

Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.

Perinodular Vascularity Distinguishes Benign Intrapulmonary Lymph Nodes From Lung Cancer on Computed Tomography.

A common diagnostic dilemma in the assessment of small pulmonary nodules on computed tomography (CT) is in distinguishing benign intrapulmonary lymph nodes (IPLNs) from small primary pulmonary malignancies. Several CT features have been described of IPLNs, including attachment to a pleural surface. We had observed that IPLNs were often connected to a pulmonary vein and sought to evaluate the utility of this sign in discriminating IPLNs from lung adenocarcinomas. The frequency of other previously described CT signs of IPLNs was also compared with lung adenocarcinomas. We retrospectively identified histopathologically proven benign IPLNs (n=62) and small (<15 mm) adenocarcinomas (n=61). CTs were reviewed to assess the number and type of pulmonary blood vessels arising from, or terminating within, these nodules, as well as other CT features of IPLNs (shape, location, and outline). The termination of a pulmonary artery within a nodule was strongly associated with primary lung adenocarcinoma (55.7%), and this was never seen in isolation in IPLNs (0%) (P<0.001). IPLNs were more frequently associated with pulmonary venous connections (93.5%) compared with lung adenocarcinomas (21.3%) (P<0.001). The connection to a pleural surface was observed in both IPLNs (38.7%) and lung adenocarcinomas (37.7%) (P=1.0). We describe a novel imaging marker that can help to differentiate between benign and malignant pulmonary nodules. However, attachment of a nodule to a pleural surface should not be used in isolation to distinguish IPLNs from lung malignancy.

Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report.

BackgroundScimitar syndrome consists of anomalous pulmonary vein drainage to the inferior vena cava. Its name derives from the image this anomalous pulmonary vein creates on a chest radiograph. We describe a case of normal venous pulmonary vein drainage that also presented the scimitar sign due to an aorto-collateral vessel.Case summaryA 15-month-old girl presented with mild dyspnoea and fever. Control chest X-ray showed an image of cardiac dextroposition, hypoplastic right lung, and the ‘scimitar sign’. Although the transthoracic echocardiogram confirmed the initial suspicion of anomalous pulmonary venous drainage, the computed tomography (CT) scan showed normal right pulmonary veins connected to the left atrium and revealed that an aorto-collateral vessel caused the scimitar sign.DiscussionAlthough the patient had several typical alterations of the scimitar syndrome, the pulmonary venous connection was normal, and the scimitar sign was due to an aorto-collateral vessel. It might be difficult to describe venous pulmonary connections on the basis of echocardiography, so an angio CT scan proved to be a valuable tool in this scenario.

Atrioventricular septal defect with anomalous systemic venous and pulmonary venous connection

An anomalous pulmonary venous and systemic connection associated with a partial atrioventricular septal defect (AVSD) is rare. A 23-year-old patient, who presented with dyspnea and cyanosis on exertion, was found to have an anomalous pulmonary and systemic venous drainage with separate drainage of the left superior caval vein and hepatic veins into the left atrium and a partial AVSD. A patch diversion was successfully performed using two patches of glutaraldehyde-treated pericardium to create a nonobstructive pulmonary and systemic venous drainage.

Pulse oximetry used in newborn screening for congenital heart diseases

Congenital heart diseases are the most frequent congenital malformations in newborns, and the critical ones, which are defined as requiring surgery or catheter-based intervention in the first year of life, count for 25%. Pulse oximetry scre­ening test is a non-invasive test that can detect these mal­for­ma­tions, some of them asymptomatic initially, lowering infatile mor­ta­li­ty and morbidity. The screening procedure is quite sim­ple: the preductal (right hand) and postductal (either foot) oxi­gen saturations are measured, then using the American’s Aca­de­my of Pediatrics algorithm as we describe below, the in­fant can test positive or negative. Targeted malformations are: ductal dependent lesions (transpositions of the great ar­teries, severe aortic stenosis, coarctation of the aorta, in­ter­rupted aortic arch, hipoplastic left heart syndrome, pul­mo­na­ry valve atresia with intact ventriculum septum, severe pulmonary stenosis, severe tetralogy of Fallot), total ano­molous pulmonary venous connection, truncus arteriosus, tri­cu­spid atreisa, Ebstein’s anomaly, single ventricle, double outlet right ventricle. We encourage and support pulse oxi­me­try screening test in all infants before discharge from ma­ter­ni­ty, because it is not an expensive procedure, it can be easily done, reducing the surgical complications due to a late diag­no­sis of these critical malformations.

Identification of fetal cardiac anatomy and hemodynamics: a novel enhanced screening protocol.

BackgroundPrenatal cardiac screening is of great importance as it contributes to appropriate neonatal management and helps parents to make a decision regarding their pregnancy. The aim of our study was to evaluate the efficiency of a newly proposed screening protocol in the detection of fetal congenital heart disease (CHD).MethodsThis was a prospective study. A total of 52 cases of confirmed CHD fetuses and 248 cases of randomly selected normal fetuses were included in the study. Two sonographers with similar experience performed the cardiac screenings under two different protocols independently. The conventional protocol (Protocol A) paid greater attention to the four-chamber view and the outflow tract views. A 6-month training program was provided to sonographers performing scans under the new protocol (Protocol B), which emphasized systematically evaluating fetal cardiac anatomy and hemodynamics. Color Doppler was mandatory and some ultrasonic signs for special cardiac anomalies were also introduced into this protocol.ResultsProtocol B detected more cardiac anomalies than did Protocol A (96.2 % vs. 61.5 %, P < 0.01). Specifically, Protocol B was superior to Protocol A in detecting cardiac malpositions, abnormal systemic and pulmonary venous connection, right aortic arch, transposition of the great arteries, and congenital corrected transposition of the great arteries. By visualizing flow disturbance and retrograde flow with color Doppler, Protocol B was better than Protocol A in screening valvular associated malformations, such as pulmonary atresia, pulmonary stenosis, tricuspid dysplasia, etc. For the normal fetuses, Protocol B was better than Protocol A in reducing the false-positive detection of septal defects.ConclusionsThe current study introduces an enhanced protocol for fetal cardiac screening, under which the obstetric screening sonographers systematically identify fetal cardiac anatomy and hemodynamics. A short-term training program makes it possible for the screening sonographers to become familiar with the new protocol, and its value has been confirmed due to improvements made in screening efficiency.Electronic supplementary materialThe online version of this article (doi:10.1186/s12884-016-0933-9) contains supplementary material, which is available to authorized users.

Catheter Ablation for Persistent Atrial Fibrillation in a Patient With Previous Repair of Total Anomalous Pulmonary Venous Connection

25-year-old male with a history of repaired total anomalous pulmonary venous (PV) connection and persistent atrial fibrillation (AF) refractory to antiarrhythmic medication was referred for percutaneous catheter ablation. At 10 weeks of age, he underwent surgical repair for total anomalous PV connection to the coronary sinus (cardiac variant) involving deroofing of the coronary sinus and closure of the atrial septal defect. At 19 years of age, he developed symptoms of breathlessness and palpitations and was found to be in persistent AF. He underwent a cardioversion and maintained sinus rhythm for 3 months before undergoing a second cardioversion for recurrent symptomatic persistent AF. He maintained sinus rhythm for 18 months on sotalol 120 mg BID before representing with increased breathlessness on exertion. Exercise echocardiography demonstrated an increase in pulmonary arterial pressures from 34 at rest to 60 mm Hg at peak exercise together with the initiation of AF. In view of this, he underwent a cardiac MRI, which demonstrated all 4 PVs draining into a dilated PV confluence (unroofed coronary sinus), which was connected to the left atrium by a small orifice measuring 13 by 15 mm. This was associated with restricted and turbulent flow between the 2 chambers, thought to be responsible for the significant dilatation of the PV confluence, and may have provided the substrate for the initiation and maintenance of AF. He remained in AF and was rate controlled with β-blockers. At 23 years of age, he underwent an operation to relieve the obstruction between the 2 chambers. Access to the right atrium was made through the original incision from the superior to the inferior vena cava and to the left atrium through the interatrial septum. The connection between the PV confluence and left atrium was confirmed to be 15 mm in both directions. The coronary sinus was cut back further to enlarge the opening between the 2 chambers. Although initial plans were for a MAZE procedure, the surgeon felt the anatomy was difficult and was complicated further by extensive adhesions and, therefore, this was not undertaken. After his operation, the patient was commenced on amiodarone, cardioverted, and maintained sinus rhythm for 1 year before developing symptomatic persistent AF when he was referred for catheter ablation. Under general anesthesia, a single transseptal puncture facilitated access to the left atrium but only limited access to the circumference of the PV confluence could be achieved because of difficulty in catheter manipulation. A circumfer ential mapping catheter confirmed the presence of rapid atrial activity within the venous confluence (Figure); however, electric isolation could not be achieved. Therefore, he was commenced on amiodarone, and a further procedure was planned after a preprocedural computed tomographic scan for integration with Carto 3 (CARTO-MERGE, Biosense Webster). The computed tomography image demonstrated all 4 PVs draining into the dilated venous confluence, which was connected to a

Can a Complete Fetal Echocardiogram Be Performed at 12 to 16 Weeks' Gestation?

The aim of this study was to determine the feasibility of performing complete early fetal echocardiography (FE) at <17 weeks of gestation with comparison with standard FE in the midtrimester (17-23 weeks). Fetal echocardiograms obtained in pregnancies studied at <17 weeks at the University of California, San Francisco, over a 5-year period were retrospectively reviewed. FE was considered complete if anatomic details could be assessed (systemic and pulmonary venous connections and atrial, ventricular, and septal [four-chamber sweeps], outflow and great artery, branch pulmonary artery, and arch anatomy) and if color and pulsed Doppler evaluations of the inferior vena cava, pulmonary veins, ventricular inflows and outflows, umbilical artery and vein, and ductus venosus were demonstrated. One hundred thirty-nine pregnancies were assessed by early FE at <17 weeks transabdominally during the study period (median gestational age, 14.0 weeks; range, 12-0/7-16-6/7 weeks). Additional transvaginal imaging was performed in 14 of 139 (10%) of early fetal echocardiographic studies. One hundred thirteen pregnancies were assessed using both early and later, standard (>17 weeks) FE. Of these, complete fetal echocardiograms were obtained in 27 early (24%; 95% confidence interval [CI], 17%-33%) and 76 later (67%; 95% CI, 58%-75%) exams. In most early exams, color and pulsed Doppler interrogation of the pulmonary veins was unsuccessful. If pulmonary vein Doppler assessment was excluded, complete studies were performed in 80 early exams (71%; 95% CI, 62%-78%) and 97 standard midtrimester exams (86%; 95% CI, 78%-91%). On early FE, heart disease was suspected in 20 pregnancies, and although no major congenital heart disease was missed, in four pregnancies, ventricular septal defects were found only on later FE or after birth. Early FE yields nearly complete information (exclusive of pulmonary venous interrogation) in the majority of patients.

Divided Left Atrium (Cor Triatriatum) in the Setting of Common Atrium

Cor triatriatum is commonly used to describe the congenital malformation in which an anomalous fibromuscular shelf divides the left atrium. We report the successful surgical repair of an extremely rare combination of divided left atrium, virtual absence of the interatrial septum, and anomalous right pulmonary venous connection in a 7 year-old girl. Preoperative transthoracic echocardiography could not detect the dividing left atrial shelf. In this report, we point out the limitations of transthoracic echocardiography in the diagnosis of atypical cases of so-called cor triatriatum and highlight errors in the scientific literature resulting from the variance in terminology ascribed to congenital division of the left atrium.

Outcome of Caval Division Techniques for Partial Anomalous Pulmonary Venous Connections to the Superior Vena Cava

Correction of a high partial anomalous pulmonary venous (PV) connection to the superior vena cava (SVC) may be complicated by sinus node dysfunction, or SVC or PV obstruction. We have used the caval division technique when the anomalous PV insert high above the cavoatrial junction and report our long-term outcome. Between 1990 and 2009, 178 consecutive patients underwent operations for a partial anomalous PV connection. Caval division was used in 41 patients (23%). Median age was 34 years (range, 1.3 to 76 years). Thirty patients (73%) had an atrial septal defect. Eighteen patients (44%) underwent Warden procedure in which the SVC was divided, the upper end was connected to the right atrial appendage, and the remaining SVC served as a conduit for PV drainage to the left atrium through the atrial septal defect. A modified Warden was performed in 17 patients (41%) using a short ringed interposition graft between the SVC and the right atrial appendage. Six patients (15%) with persistent left SVC underwent right SVC division without reimplantation. No early deaths occurred. One Warden group patient (5%) required early reoperation for SVC obstruction. One patient (2%) with atrial fibrillation underwent a concomitant Maze procedure and permanent pacemaker implantation for tachybrady syndrome. All other patients remained in sinus rhythm at follow-up. Follow-up was complete in all patients (mean, 2±2.6 years; maximum, 14 years). One noncardiac-related death occurred at 14 years. SVC obstruction developed in 3 Warden group patients (17%) and was managed with angioplasty and stenting. No patient had pulmonary vein stenosis on follow-up. Caval division techniques appear to be effective, with low morbidity and mortality. The modified Warden procedure may decrease the risk of SVC obstruction. Late arrhythmias or PV obstruction are uncommon.

Perioperative Management of the Pediatric Cardiac Transplantation Patient

The major diagnoses carried by children undergoing cardiac transplantation worldwide are congenital heart defects, cardiomyopathies, and retransplantation. The leading diagnosis in infancy is congenital heart disease, whereas cardiomyopathy predominates in older children. In view of this wide spectrum of diagnoses, the perioperative management of these children requires medical, interventional, and surgical expertise in treatment of complex congenital heart defects, end-stage heart failure, and cardiac transplantation. According to the Pediatric Heart Transplantation Survey database, the majority of children listed for cardiac transplantation eventually require higher levels of cardiac support before transplantation. The team caring for these children should be prepared to escalate support in a timely fashion in order to avoid end-organ dysfunction or a catastrophic event that will remove the patient from the cardiac transplantation list. The first step is advanced hemodynamic monitoring in a specialized pediatric cardiac intensive care unit and initiation of inotropic support. Further escalation of care should be based on careful analysis of the hemodynamic profile, end-organ function, and biochemical markers of perfusion and myocardial stress. A patient who continues to deteriorate in spite of inotropic support requires positive pressure ventilation, and if deterioration continues, mechanical circulatory support is initiated. Cardiac transplantation is a challenging operation, and even more so in children with complex congenital heart defects. The abnormal cardiovascular anatomy requires planning and anticipation of possible pitfalls as hypoplasia of the aortic arch, abnormal pulmonary arteries, and abnormal systemic and pulmonary venous connections. The time required to remove adhesions in children with prior cardiac operations increases the ischemic time of the graft and the risk of primary graft dysfunction. Assessment of pulmonary vascular resistance in children with congenital heart defects is problematic, and even children with a normal transpulmonary gradient and pulmonary vascular resistance are at increased risk of postoperative pulmonary hypertension and right ventricular graft failure. The postoperative course is directly linked to the patient's preoperative physical condition and perioperative course. The induction of immunosuppression and the use of plasmapheresis in children with a positive cross-match may lead to further hemodynamic compromise. If severe primary graft dysfunction evolves, early initiation of extracorporeal membranous oxygenator is indicated to avoid irreversible end-organ dysfunction.

Does a New Ultrasound Flow Modality Improve Visualization of Neonatal Pulmonary Veins?

Blood flow imaging is a new ultrasound modality that supplements color Doppler imaging with angle-independent information of flow direction that is not influenced by velocity aliasing. This is done by visualizing the blood speckle movement superimposed on the color Doppler images. This study aimed to investigate whether this method improves the visualization of the pulmonary veins in neonates. Twenty-six neonates with suspected congenital heart disease were prospectively examined with echocardiography and blood flow imaging of the pulmonary veins after parental consent. For each patient, blood flow imaging and color Doppler imaging cine loops were presented to four observers (pediatric cardiologist/cardiologists) in a random order. Questions regarding the pulmonary venous connections and the overall quality of the pulmonary vein imaging were evaluated on a visual analogue scale from 0 (worst) to 100 (best). The methods were compared within each observer using the Wilcoxon's exact signed-rank test. Blood flow imaging (color Doppler imaging combined with the blood speckle movement) was consistently ranked as better than conventional color Doppler imaging for visualization of the pulmonary veins for all observers (all P-values < 0.002). Blood flow imaging may improve the visualization of the pulmonary veins in neonates.

Infracardiac total anomalous pulmonary venous drainage with unusual presentation

Total anomalous pulmonary venous drainage (TAPVD) is a rare entity which forms approximately 0.4 to 2% of all congenital heart disease. The infracardiac type usually involve obstructions on pulmonary venous connections and comprising a quarter of all TAPVD cases. The clinical findings in patients with obstructed infracardiac TAPVD could mimic respiratuary distress of several different etiologies during first hours of life. In this article, we present a case of a neonate with infracardiac type of TAPVD presented with only distinct subcutaneous veins of abdominal and thoracic wall.

Novel Modification of Total Cavopulmonary Connection for Isolated Hepatic Vein

A surgeon needs an innovative technique to establish a Fontan circulation for a patient who has a widely separated hepatic vein from the inferior vena cava. The inferior vena cava was redirected by placing a trimmed GoreTex baffle (W. L. Gore and Associates, Flagstaff, AZ) on the internal side of the atrium connecting the hepatic venous flow, and then directing it to the extra-atrium. Another extracardiac half-circumferential GoreTex baffle was sutured to the epicardial atrial wall and to the pulmonary artery opening. This procedure is efficacious for patients with an isolated hepatic vein and has advantages in terms of using less synthetic material in the conduit.

Spectrum of Cardiovascular Disease, Accuracy of Diagnosis, and Outcome in Fetal Heterotaxy Syndrome

Because there is a paucity of information regarding the diagnosis and outcomes of fetal heterotaxy syndrome (HS), this study sought to determine the spectrum of cardiac pathology, accuracy of diagnosis, and outcome of fetal HS. All cases of fetal HS encountered in the investigators' institution over a 10-year period through 2002 were identified. Prenatal and postnatal echocardiograms and medical records were reviewed. Seventy-one fetuses were diagnosed with HS, including 48 with left atrial isomerism (LAI) and 23 with right atrial isomerism (RAI). For LAI and RAI, most intracardiac lesions, the pulmonary venous connections, and superior vena caval anatomy were correctly diagnosed in utero (93%, 86%, and 77% accuracy, respectively), whereas hepatic venous connections and inferior vena caval-atrial connections in RAI were difficult to define (65% and 56% accuracy, respectively). Of 32 continued and followed pregnancies with LAI, 22 are currently alive at 48 +/- 30 months. Heart block and associated major extracardiac pathology were significantly more common in nonsurvivors with fetal LAI (p = 0.007 and 0.024, respectively). Outcomes were even worse for prenatally diagnosed RAI: of 14 continued pregnancies, only 3 are currently alive. In conclusion, fetal HS is associated with a broad spectrum of cardiac pathology, which can be diagnosed accurately in utero. Fetal LAI is associated with a mortality of 31%, with heart block and extracardiac pathology as primary risk factors for perinatal mortality. The outcome of prenatally diagnosed RAI is poor.

Cardiac screening examination of the fetus: guidelines for performing the ‘basic’ and ‘extended basic’ cardiac scan

Cardiac screening examination of the fetus: guidelines for performing the ‘basic’ and ‘extended basic’ cardiac scan

Modified Fontan Operation in Patients with Anomalies of Systemic and Pulmonary Venous Connection

The presence of anomalies of the systemic and pulmonary venous connection associated with single ventricle anomalies has been considered a contraindication for the Fontan operation (FO). The aim of this study is to outline the technical considerations associated with the presence of anomalies of systemic and/or pulmonary venous connections and to identify the risk factors for mortality in this group of patients undergoing the modified FO. Between 1989 and 2004, 63 patients (median age, 3.2 years) with anomalous systemic or pulmonary venous connection underwent a Fontan procedure at our institution. Nine patients had a combination of anomalous systemic and pulmonary venous connection, 49 patients had anomalous drainage only from the systemic circulation, and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 25 patients. Previous palliative operations had been performed in 51 patients (81%). There was 1 early death, and 2 patients required take down of Fontan procedures. Two patients required reoperation for revision of the atrial baffle. At a mean follow-up of 4.6 +/- 3.4 years, there have been 5 late deaths (8%) and 45 patients (71%) have undergone Fontan completion. Actuarial survival was 92% at 1 year and 91% at 5 and 10 years-not significantly different from the overall survival of the Fontan patients. We conclude that the modified FO can be successfully performed in patients with anomalous systemic or pulmonary venous connections, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients with normal connections.