Partial Anomalous Venous Connection Research Articles

Partial anomalous venous connection with intrapulmonary dual drainage: Transcatheter treatment of a rare entity.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital condition, and dual-drainage connection PAPVR to the left atrium has been reported in a few cases in the literature; in which cases, percutaneous catheterization was successfully used in lieu of surgery. We, hereby, describe a 7-month-old boy with a functional single-ventricle physiology with dual drainage of the left upper pulmonary vein to the left atrium and the innominate vein. Appropriate recognition of this entity allowed safe occlusion of the anomalous draining vein.

Clinical Significance of Partial Anomalous Pulmonary Venous Connections (Isolated and Atrial Septal Defect Associated) Determined by Cardiovascular Magnetic Resonance.

Partial anomalous venous connections (PAPVC) are associated with left to right shunting and right heart dilatation. Identification of PAPVC has increased with widespread use of cross-sectional imaging modalities. However, management strategies are mostly based on expert opinion given the scarcity of data from large series. We aimed to define types and significance of isolated and atrial septal defect (ASD) associated PAPVC detected by cardiovascular magnetic resonance. We retrospectively reviewed our cardiovascular magnetic resonance database from 2002 to 2018 to identify isolated or ASD-associated PAPVC cases. A total of 215 patients (median age 46 years; range, 6-83) with isolated or ASD-associated PAPVC were identified among 102 135 clinical cardiovascular magnetic resonance studies. Of these, 104 were isolated and 111 were associated with an ASD. Anomalous connection of right upper pulmonary vein was the most common single venous anomaly (99/215), but in the isolated PAPVC group there were more anomalous left than right upper pulmonary veins (39 versus 34). The Qp/Qs was significantly higher for isolated anomalous single right upper pulmonary vein than left upper pulmonary vein (1.6 versus 1.4 respectively; P=0.01) as were right ventricular end-diastolic volumes (113.7±30.9 versus 90 [57-157] mL/m2, P=0.004). In the PAPVC with an ASD group, sinus venosus ASDs (82%) were associated with right-sided PAPVCs while both right and left-sided venous anomalies were seen in secundum ASDs (18%). In a substantial number of patients (30 out of 91) with sinus venosus ASDs, PAPVCs were more complex and involved more than a single anomalous right upper pulmonary vein; and in 5 patients with ASD, PAPVC was identified only after the ASD closure. This large series provides descriptive and hemodynamic features for isolated and ASD-associated PAPVCs. Anomalous isolated right upper pulmonary vein may cause a significant shunt (Qp/Qs >1.5). PAPVC associated with sinus venosus and secundum ASDs might be more complex than a single anomalous pulmonary vein and missed before ASD correction.

Unknown Partial Anomalous Pulmonary Venous Connection Causing Arterial Blood Gases During Central Venous Catheter Insertion

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly. We report about the case with an “arterial blood gas” analysis taken from a newly inserted central venous catheter (CVC). Thoracic computed tomography (CT) verified the catheter in the left internal jugular vein. The contrast was given through the catheter which revealed a partial anomalous venous connection of the left upper pulmonary vein to the left brachiocephalic vein. PAPVC should be taken into consideration in cases of “arterial blood gases” taken from a correctly inserted CVC. Most patients are asymptomatic. Adequate diagnostic tools to detect PAPVC are TEE, CT or MRI.

P20.07: Review of ultrasound markers in diagnosis of total and partial anomalous venous connection in mid and late gestation

P20.07: Review of ultrasound markers in diagnosis of total and partial anomalous venous connection in mid and late gestation

Minimally Invasive Robotically Assisted Repair of Partial Anomalous Venous Connection

Minimally Invasive Robotically Assisted Repair of Partial Anomalous Venous Connection

Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation.

There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33mL/m(2) vs. 118 ± 30mL/m(2)), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation.

An unusual cause of respiratory distress in an infant.

Partial anomalous venous connection (PAPVC) is a congenital cardiovascular defect where one or more (but not all) of the pulmonary veins return anomalously back to the right atrium, either via a direct or indirect connection. It often occurs with other cardiac defects, most commonly a secundum atrial septal defect. Individuals with a large degree of shunting will present with dyspnea, fatigue, and, in some cases, heart failure. Clinical associations and variants of PAPVC include scimitar syndrome, pseudo-scimitar or meandering right pulmonary vein, sinous venosus defects, malposition of the septum primum, and Turner syndrome. The patient in this case, a previously healthy, 6-month-old, full-term male, presented to the emergency department for evaluation of respiratory distress and wheezing. The infant was first seen in his pediatrician's office, where he was noted to be tachypneic and wheezing. He was feeding without difficulty, voiding well, and was active and playful. The patient had passed critical congenital heart disease screening after his birth and prior to discharge, and the family history was negative for any respiratory or cardiac conditions. Cardiac magnetic resonance imaging is becoming the mode of choice for diagnosis of PAPVC. The definitive treatment is surgical correction, but surgery is not indicated in all cases, especially if the patient is asymptomatic and the degree of shunting is small. Patients with isolated PAPVC who undergo surgical correction have good long-term outcomes. In this case, the patient underwent the Warden procedure, which causes an increased risk of superior vena cava stenosis or obstruction to the right atrium. This patient will require lifelong follow-up to assess for new onset pulmonary venous obstruction.

Prenatal diagnosis of isolated total anomalous pulmonary venous connection: a series of 10 cases

To report on a series of 10 fetuses with prenatally diagnosed isolated total anomalous pulmonary venous connection (TAPVC), focusing on echocardiographic features leading to diagnosis, assess accuracy of prenatal diagnosis and describe postnatal outcome. In this review of our experience of prenatal diagnosis of isolated TAPVC, we analyzed retrospectively medical records and fetal echocardiography findings in all cases with prenatal diagnosis of isolated TAPVC delivered between 1 January 2001 and 1 October 2011 at a tertiary referral center, paying special attention to echocardiographic signs that led to referral. During the study period, 95 infants with isolated TAPVC were seen at the center. Initially, expert fetal echocardiography identified 14 fetuses with isolated TAPVC. Prenatal diagnosis was made at a mean gestational age of 31 (range, 25-37) weeks. Ten true-positive cases of TAPVC were confirmed after birth. The remaining four were considered false-positive cases: two had normal heart with left superior vena cava to coronary sinus, one had partial anomalous venous connection and one was lost to follow-up. Of the 85 diagnosed postnatally with TAPVC, only one had been seen prenatally by an expert cardiac sonographer. Echocardiographic signs leading to referral were related to pulmonary venous connection in half of the cases. Other suspected defects which led to referral were ostium prium atrial defect (n = 3), left-right asymmetry (n = 1), abnormal mitral valve (n = 1) and hepatic vascular malformation (n = 1). All infants with TAPVC underwent surgery. There was one postoperative death and nine survivors, with a mean follow-up of 31 (range, 2-104) months. Fetal diagnosis of isolated TAPVC is challenging even for experts. Echocardiographic anomalies may appear late in gestation. New tools should be proposed to identify abnormal venous drainage at the screening level.

Repair of simple congenital cardiac defects via lower ministernotomy, using standard instruments

In the current scenario the costs of performing minimally invasive cardiac surgery are very high primarily due to the high cost of hardware used, limiting its widespread usage especially in a developing country like ours. The aim of our study was to test the feasibility and safety of performing minimally invasive surgery to repair simple congenital cardiac defects using standard equipment. From July 2011 to March 2012, 59 patients, weighing between 7 kg to 32 kg underwent closure of their defects via lower ministernotomy. Forty six cases were Ostium Secundum Atrial Septal Defect (OSASD), four cases had OSASD with Valvar Pulmonary Stenosis (PS), five cases of moderate Ventricular Septal Defect (VSD), three cases of Sinus Venosus Atrial Septal Defect (SVASD) and one case of Partial AtrioVentricular Canal Defect (PAVCD). All patients underwent central canulation of aorta and vena cava. Standard cannula and instruments were used during the procedure. The length of the postoperative scar measured ranged from 3.5 to 8 cm. One SVASD was converted to complete sternotomy because of high drainage of the Partial Anomalous Partial Venous Connection (PAPVC). None of the patients had any emergency conversion, re-exploration or neurological side effects. One patient had a tiny residual VSD and another patient had a pericardial effusion that required drainage. Repair of most simple congenital cardiac defects can be safely performed through a lower ministernotomy approach. The procedure resulted in a cosmetically appealing scar and decreased patient morbidity without using any special expensive instruments.

Association of Left Ventricular Diverticula and Sinus Venosus Atrial Septal Defect

Left ventricular diverticula are congenital anomalies and are not as rare as previously thought. In adults, cardiac diverticula are generally accidental findings during imaging modalities, but concomitant disorders might frequently coexist. The pathophysiology, management, prognosis, and natural history of cardiac diverticula remain poorly understood and controversial. Definite diagnosis is often challenging due to similarities in appearance to other more common anomalies such as aneurysms, pseudoaneurysms, endocarditis, cysts, and hypertrophied trabeculations. We herein report a rare case of an adolescent presenting with sinus venosus atrial septal defect, partial anomalous venous connection, and left ventricular diverticula.

Tc-99m MAA Findings in Dilated Cardiomyopathy With Partial Anomalous Venous Connections

Tc-99m MAA showed asymmetric uptake in the lung field in a 21-year-old man with dilated cardiomyopathy. CT revealed partial anomalous venous connections in the left upper lobe. Angiogram of the left pulmonary upper lobe showed all the contrast material drained into the left vertical vein. The possible cause of relative increase in the left upper lobe blood flow is that right pulmonary blood flow is slowed by the high pressure in the left atrium due to dilated cardiomyopathy, whereas the flow from the left upper lobe drains into the superior vena cava which has less pressure than left atrium.

Pulmonary vein and atrial wall pathology in human total anomalous pulmonary venous connection

Background Normally, the inside of the left atrial (LA) body and pulmonary veins (PVs) is lined by vessel wall tissue covered by myocardium. In total anomalous pulmonary venous connection (TAPVC), no connection of the PVs with the LA body exists. These veins have an increased incidence of PV stenosis. We describe the consequences of the absent connection for the histopathology of the wall of the LA body and the PVs, and hypothesize on a mechanism predisposing to PV stenosis. Methods and results In 10 human neonates with TAPVC, the wall of the LA body and PVs were studied using histological and immunohistochemical techniques. As controls, 2 normal neonatal and adult hearts and 5 neonatal hearts with partial anomalous venous connection (PAPVC) or situs inversus were studied. In hearts with TAPVC no vessel wall tissue was found in the LA body and its myocardial layer was hypoplastic. No myocardial sleeve was found around the abnormally draining PVs. In hearts with PAPVC, only the non-LA draining PV lacked myocardial covering, whereas in situs inversus PVs connecting to the right-sided LA, were normally myocardialized. Conclusion An open connection of the PVs with the morphological LA is necessary for the presence of vessel wall tissue in the LA and myocardialization of the PVs. Absence of myocardium covering the PVs is hypothesized to enhance susceptibility to PV stenosis and prevent onset of PV originating arrhythmias. The embryonic posterior heart field may be responsible for the abnormal myocardialization and smooth muscle cell formation in TAPVC.

Transesophageal echocardiographic diagnosis of sinus venosus-type atrial septal defect associated with partial anomalous venous connections during cardiac surgery in adults

T RANSESOPHAGEAL echocardiography (TEE) is useful for intraoperative monitoring of cardiac function 1,z and for evaluating valve repair. 3 Recently, TEE has been proposed as a useful intraoperative tool improving diagnostic accuracy and assisting in surgical repair of pediatric congenital heart malformations. 4 Its role in the diagnosis of congemtal cardiac malformations in adults has not been widely discussed. The sinus venosus variant of atrial septal defect (SVASD) occurs in up to 20% of adults with an atrial septal defect (ASD) and is the most difficult to Identify. 5 This defect is frequently associated with anomalous connection of the right pulmonary veins to the junction of the right atrium and the superior vena cava. 6 The authors describe two cases of adult cardmc surgery where intraoperative TEE helped to diagnose previously unsuspected SVASD associated with partial anomalous pulmonary venous connections (PAPVC) and discuss how the echocardiography results led to modification of the planned operation.

Horseshoe lung associated with anomalous pulmonary venous connection without pulmonary hypoplasia

SummaryHorseshoe lung is an exceedingly rare congenital malformation, characterized by unilateral pulmonary hypoplasia together with a midline isthmus producing fusion of the tissues of the lower lobes. It is frequently associated with other cardiac and extracardiac anomalies. We report an infant with a variant of the horseshoe lung with partial anomalous venous connection of intracardiac type, ventricular septal defect, and persistent left superior caval vein. There was no pulmonary hypoplasia.

Absent left pulmonary vein without anomalous connection: Diagnosis and management in the newborn

Unilateral atresia of the pulmonary vein is an uncommon condition and is often associated with partial anomalous venous connection. Various forms of alternative channels have been described.l-” While the term “atresia” is used to denote the existence of a variable size and length of cordlike pulmonary veins without any lumen, “absence” is used here to denote that no such connection exists between the pulmonary hilum and left atrium, as a result of either agenesis or involution. To our knowledge, only two cases of unilateral absence of the pulmonary vein without anomalous connection have been reported in the English literature.4 We report a case of unilateral pulmonary edema caused by an absent left pulmonary vein without anomalous connection, diagnosed and managed successfully by surgery in a newborn. A full-term male infant (birth weight 3011 gm) was born by emergency cesarean section because of fetal bradycardia to a 23-year-old gravida I, white female. The child was

Corrective Repair of Complete Atrioventricular Canal Defects and Major Associated Cardiac Anomalies

Twenty-nine patients with balanced forms of complete atrioventricular (AV) canal defects and tetralogy of Fallot in 12, double-outlet right ventricle (DORV) in 10, and additional anomalies associated with atrial isomerism in 7 underwent corrective repair. Age ranged from 39 days to 24 years (median, 3.7 years). Repair included reconstruction of the common AV valve in 25 patients and prosthetic valve replacement in 4. Construction of an intraventricular tunnel connecting the left ventricle with the aorta was accomplished in 23 of 25 patients with ventriculoarterial concordant or double-outlet connections. In 2 patients with situs inversus and associated DORV and in 4 patients in the isomerism group who had ventriculoarterial discordant connections, repair included intraatrial venous diversion by a modified Senning procedure in 2, a modified Mustard venous switch in 1, and a tailored atrial baffle in 3. A valved extracardiac conduit was required for repair in 4 patients. Partial anomalous systemic venous connection was repaired in 7 patients and total anomalous pulmonary venous connection, in 5. There was 1 hospital death (3%) and 3 late deaths (11%). There was 1 (3.6%) reoperation to replace an incompetent right (systemic) AV valve 17 months postoperatively, and each of the 25 surviving patients was in New York Heart Association Functional Class I (80%) or II (20%) at follow-up.

Coexistent mitral valve disease with left-to-right shunt at the atrial level: Clinical profile, hemodynamics, and surgical considerations in 67 consecutive patients

Between January, 1963 and June, 1985, 67 patients underwent corrective surgery for this complex anomaly. Symptoms and signs of atrial septal defect were dominant in the majority of subjects. History of rheumatic fever (46%), paroxysmal nocturnal dyspnea (33%), presence of an opening snap, mitral diastolic murmur, or pansystolic murmur provided clinical clues to document assiciated mitral valve disease. Open mitral valvotomy was accomplished in 39 subjects, while in the remainder (28 subjects) the valve required replacement. Partial anomalous venous connection was encountered in 12 subjects. Recognition and attention to the associated tricuspid incompetence is a high priority, and 21 subjects underwent concomitant annuloplasty. The overall hospital mortality was 13.4%, with no deaths in the last 22 consecutive patients. The period of follow-up ranged from 1 year to 22 years, with a mean ± SD of 9.34 ± 6.61 years. We belleve, with other authors, that since the hemodynamic and therapeutic considerations are very similar, both the stenotic and regurgitant lesions should be included in the same syndrome.

Scimitar syndrome in childhood

Scimitar syndrome is a rare but well-described constellation of cardiopulmonary anomalies: anomalous pulmonary venous connection of the right lung, hypoplasia of the right lung with dextroposition of the heart, hypoplasia and malformation of the right pulmonary artery (PA) and bronchial trees, and anomalous systemic arterial blood supply to the right lung, arising from the abdominal aorta or its branches. The partial or total anomalous venous connection of the right lung drains into the inferior vena cava or azygous system and is present in all cases. 1–5 Often, this anomalous vessel is visible on a chest radiograph as a curvilinear shadow just above the right diaphragm and resembles a Turkish sword or “scimitar.” This report summarizes our experience with scimitar syndrome in 15 children.

Two dimensional echocardiographic diagnosis of situs.

At present there is no reliable method of recognising atrial isomerism by two dimensional echocardiography. We therefore used two dimensional echocardiography to examine 158 patients including 25 with atrial isomerism and four with situs inversus. Particular attention was paid to the short and long axis subcostal scans of the abdomen. Using the position of the inferior vena cava and the aorta with respect to the spine it was possible to separate those with situs solitus from the others. Two false positives for abnormal situs had exomphalos. In situs solitus the aorta lay to the left of the spine and the inferior vena cava lay to the right. One patient with situs solitus and azygos continuation of the inferior vena cava also had inferior vena cava to right atrial connection. In the four patients with situs inversus the mirror image of the normal pattern was present. In nine patients with right isomerism the inferior vena cava and aorta ran together on one or other side of the spine. The inferior vena cava, anterior to the aorta at the level of the diaphragm, received at least the right hepatic veins (normal or partial anomalous hepatic venous connection). Of the 16 patients with left isomerism, 14 had azygos continuation of the inferior vena cava which was visualised posterior to the aorta in all but two. All patients with left isomerism had total anomalous hepatic venous connection to one or both atria via one or two separate veins. Two dimensional echocardiography therefore provides the means of detecting abnormal atrial situs and of diagnosing right or left isomerism in the great majority of patients, if not all.

Isolated Partial Anomalous Venous Connection: A Congenital Defect with Late and Serious Complications

The cases of 3 patients with partial anomalous pulmonary venous return with intact atrial septum are reported. The 2 adult patients had evidence of severe pulmonary artery hypertension with markedly increased pulmonary vascular resistance indicating advanced pulmonary vascular obstructive disease. This confirms earlier limited observations and emphasizes the potential for this congenital lesion to develop late, serious complications. In view of this potential, surgical repair of the anomaly should be considered whenever it is found. Descriptions of a successful operative approach are included.