Status of survivors after atrial redirection for transposition of the great arteries: a complete long-term follow-up

All 32 survivors with transposition of the great arteries, born in 1964-83 and operated on at our institution using atrial redirection, were evaluated by cardiac catheterization, echocardiography and Holter monitoring. There were 17 Mustard patients, age 17.1 years (±3.5, 12.0-22.0) and 15 Senning patients, age 9.4 years (±1.6, 7.2-12.1). All but one had simple transposition. Six had caval obstruction, one had pulmonary venous obstruction, three had large atrial shunts, four had considerable pulmonary hypertension, seven had mild ventricular outflow tract obstruction, four had significant tricuspid regurgitation, 11 had systemic ventricle dysfunction (one severe), 14 had sinus node dysfunction (three symptomatic) and two had atrioventricular block (one with pacemaker). Eight Mustard patients (47%) and one Senning patient (7%) had symptomatic cardiac sequelae, and only one patient (Senning) was free from sequelae, illustrating that these patient groups will need continuing medical attention.     

Color flow mapping to document normal pulmonary venous return in neonates with persistent pulmonary hypertension being considered for extracorporeal membrane oxygenation

This study investigated the value of color flow mapping in documenting normal pulmonary venous return in neonates with persistent pulmonary hypertension who were candidates for extracorporeal membrane oxygenation (ECMO). Forty newborn infants with persistent pulmonary hypertension underwent conventional (two-dimensional and Doppler) echocardiography and color flow mapping. Of 25 candidates for ECMO therapy, 18 subsequently received it. Conventional echocardiography demonstrated normal pulmonary venous return in only 21 of the 40 patients. In all 40, however, color flow mapping demonstrated normal right and left pulmonary venous drainage entering the left atrium. In three other patients with total anomalous pulmonary venous return, conventional echocardiography demonstrated the anomalous pulmonary venous pathways, and color flow mapping did not show jets emanating from the left atrial wall; the left atrium was shown to fill exclusively from right to left shunting through the foramen ovale. We conclude that color flow mapping is superior to conventional echocardiography for verifying normal pulmonary venous return in neonates with persistent pulmonary hypertension.     

Resolution of Acquired Pulmonary Arteriovenous Malformations in a Patient with Total Anomalous Systemic Venous Return

An 11-year-old male with total anomalous systemic venous return had surgical repair except for the hepatic venous return, which drained to the left atrium. He developed progressive cyanosis and fatigue and was diagnosed with large pulmonary arteriovenous malformations (PAVMs) during cardiac catheterization with the use of bubble contrast echocardiography. After surgical redirection of hepatic venous flow to the right heart and pulmonary arterial system, resolution of these PAVMs was demonstrated clinically and by contrast echocardiography. This unique case report demonstrates the development of PAVMs with exclusion of hepatic venous return through the pulmonary vascular bed while pulsatile pulmonary blood flow remains intact. It reinforces the likelihood of the absence of an as yet unidentified hepatic vasoactive substance as the source for development of PAVMs.     

Peripheral vein contrast echocardiography in atrial septal defect

This study was carried out on 233 children suspected clinically of having atrial septal defect with the aim of investigating the diagnostic capability of peripheral venous contrast echocardiography. The transfer of contrast material from the right atrium into the left atrium was evaluated as "positive contrast", while noncontrast blood, passing from the left atrium into the right atrium was termed "negative contrast". Positive contrasts were quantitated in four grades. A significant negative contrast effect was graded 3- or 4-. Three positive, 4+ and/or 3-, 4- contrast effects were considered definite evidence of an atrial septal defect. Among the cases with the above findings 92 underwent surgical closure of atrial septal defect. The procedure was successful in all patients operated; the size of the defect was large. This result demonstrates that the method applied is a safe and reliable one. However, in a group of cases without the above echocardiographic findings the presence of an atrial septal defect was detected by cardiac catheterization and angiocardiography. Therefore, we can conclude that the method applied is not a sensitive, but a specific one, for definite detection of atrial septal defect.     

Morphological Features of the Levoatriocardinal (or Pulmonary-to-Systemic Collateral) Vein

Three cases with an anomalous pulmonary-to-systemic collateral vein (levoatriocardinal vein) connecting the left atrium or one of the pulmonary veins to a systemic vein are described. In two of these cases the atrial septum is intact, the left atrioventricular connection is absent (mitral atresia), and the anomalous vein is the escape channel for pulmonary venous return. In the remaining case, a muscular membrane divides the left atrium, separating the pulmonary venous component from the distal component. The collateral vein may be mistaken for the vertical vein that is associated with totally anomalous pulmonary venous connections, but in all our cases the pulmonary veins inserted normally into the left atrium. Cross-sectional echocardiography, including conventional and color flow Doppler mapping, should overcome potential difficulties in diagnosis.     

Mid-term follow-up of patients with transposition of the great arteries after atrial inversion operation using two- and three-dimensional magnetic resonance imaging

BACKGROUND: Older patients with transposition of the great arteries who have undergone an atrial inversion procedure (ATRIAL-INV) are difficult to image by echocardiography. The surgical baffles are spatially complex. OBJECTIVE: To test the hypothesis that two- and three-dimensional MRI can elucidate the spatially complex anatomy in this patient population. MATERIALS AND METHODS: Twelve patients with ATRIAL-INV, ages 16+/-4.5 years, underwent routine T1-weighted spin-echo axial imaging to obtain a full cardiac volumetric data set. Postprocessing created three-dimensional shaded surface displays and allowed for multiplanar reconstruction. Routine transthoracic echocardiography was available on all patients. RESULTS: Three-dimensional reconstruction enabled complete spatial conceptualization of the venous pathways, and allowed for precise localization of a narrowed region in the upper limb of the systemic venous pathway found in two patients. This was subsequently confirmed on angiography. Routine MRI was able to image the full extent of the venous pathways in all 12 patients. Routine transthoracic echocardiography was able to visualize proximal portions of the venous pathways in eight (67%), the distal upper limb in five (42%), and the distal lower limb in four (33%) patients, and it was able to visualize the outflow tracts in all patients. CONCLUSION: Three-dimensional reconstruction adds important spatial information, which can be especially important in stenotic regions. Routine MRI is superior to transthoracic echocardiography in delineation of the systemic and pulmonary venous pathway anatomy of ATRIAL-INV patients at mid-term follow-up. Although transesophageal echocardiography is an option, it is more invasive.     

Use of echocardiography in newborns with patent ductus arteriosus: A review

Summary Assessment of the contribution of left-to-right shunted ductal flow to the clinical state of a newborn with respiratory distress syndrome is difficult. Echocardiography has aided in the bedside diagnosis and management of these infants. M-mode echocardiography allows evaluation of left atrial dimension in both precordial and suprasternal planes, left ventricular dimension, and indices of function. M-mode echocardiography combined with saline contrast aortic injections allows a very sensitive demonstration of the presence of left-to-right shunting patent ductus arteriosus even if no murmur is present. Two-dimensional echocardiography demonstrates the physical appearance of the ductus and helps rule out associated congenital cardiac lesions. Incorporation of venous and arterial contrast injections help demonstrate flow direction as well. Range gated pulsed Doppler echocardiography helps evaluate flow characteristics and aids in differential diagnosis. In the future, ultrasonic evaluation will probably combine Doppler echocardiography with two-dimensional echocardiography allowing clearer noninvasive definition of anatomy and flow direction. None of these techniques should be used by itself. Careful clinical assessment by standard techniques along with judicious use of ultrasonic methodology should result in improved diagnosis and management.This paper was part of the Ray C. Anderson Symposium.     

Isolated Total Anomalous Systemic Venous Drainage in an Adult: Case Report

Total anomalous systemic venous drainage is a rare form of congenital heart disease. All the systemic venous flow from the body (i.e., from the superior vena cava, inferior vena cava, and coronary sinus) drains abnormally into the left atrium. This condition requires the presence of a left-to-right shunt (atrial septal defect, patent ductus arteriosus, or ventricular septal defect) to allow the systemic venous return to reach the pulmonary circulation. This disorder may be associated with heterotaxy syndromes. This report describes a patient with unique total anomalous systemic venous drainage that was successfully corrected surgically.     

Diagnostic features and pitfalls in the two-dimensional echocardiographic evaluation of a child with cor triatriatum

Summary Two-dimensional echocardiography has been successfully used to diagnose cor triatriatum in adults and children, and surgical referral of these patients has been undertaken without preceding cardiac catheterization and angiography. A child with cor triatriatum demonstrated by two-dimensional echocardiography was directly referred for surgical resection. Despite thorough preoperative echocardiographic examination and direct intraoperative inspection of the posterior left atrial chamber, partial anomalous right pulmonary venous drainage was not diagnosed until the postoperative period. The failure to detect this uncommon associated anomaly underscores the limitations of relying on two-dimensional echocardiography as the sole preoperative method of evaluation.     

Regression of pulmonary arteriovenous malformations following heart transplantation

Pulmonary arteriovenous malformations (PAVMs) can occur following caval to pulmonary artery connection, Glenn and/or Fontan procedure, leading to severe cyanosis and exercise intolerance. It is unknown whether these abnormalities regress or persist following heart transplantation (HTx). Twenty patients with failed Fontan or Glenn procedures were screened for PAVMs prior to HTx by contrast echocardiography, selective pulmonary angiography, and pulmonary venous desaturation. Age at transplant, diagnosis, previous operations, time from Glenn to transplant, systemic oxygenation, hemoglobin level, and ventricular function were determined. The clinical course after HTx was characterized in three patients with significant PAVMs. Indications for HTx were exercise intolerance and severe cyanosis in one patient, and cyanosis and ventricular dysfunction in two. Pre-HTx, mean systemic saturation was 67%; mean pulmonary venous wedge saturation was 81%. Post-HTx, oxygen saturations were normal (> 96%) at 14, 40, and 180 days. Contrast echocardiography, performed 1 month to 3.3 yrs after HTx, showed no intrapulmonary shunting in two patients and minimal shunting in one. One patient suffered an embolic stroke from right-to-left shunting post-HTx. All patients are alive and well 35, 71, and 73 months post-HTx. In patients with single ventricle physiology, PAVMs are not an absolute contraindication to HTx. Heart-lung transplant may not be required for these patients.     

Site of Coronary Sinus Drainage Does Not Significantly Affect Coronary Flow Reserve in Patients Long Term After Fontan Operation

This study was designed to investigate the impact of postoperative coronary sinus drainage pressure on coronary flow reserve (CFR) assessed by Doppler guidewire in patients long term after Fontan operation. Twenty-nine patients (median age, 17.4 years female, 11) at a median of 10.6 years after Fontan operation were examined with intracoronary Doppler guidewire during cardiac catheterization. Fourteen patients had coronary sinus (CS) drainage to the systemic venous atrium and 15 patients had CS drainage to the pulmonary venous atrium after Fontan operation. Median CS drainage pressure was significantly higher in systemic venous CS drainage compared to pulmonary venous CS drainage (11 vs 5 mmHg, p < 0.0001). Median CFR values for the right and left coronary artery did not differ significantly with respect to CS drainage. There was a positive correlation between coronary flow reserve and pulmonary arteriolar resistance (p < 0.05) in multivariate regression analysis. The site of coronary drainage into the systemic atrium or the pulmonary venous atrium did not significantly affect CFR. Our data do not support a surgical strategy of elective redirection of coronary sinus blood to a low-pressure compartment but support an early staged approach. The positive correlation between CFR and pulmonary resistance demands further evaluation.     

Double total anomalous pulmonary venous connection

We present a newborn infant with right atrial isomerism, complex congenital heart malformation and anomalous pulmonary venous connection, reliably diagnosed by 2-D Doppler color echocardiography. The infant had no significant obstetric antecedents. The neonatal clinical picture included cyanosis, heart murmur and respiratory distress. The infant was treated with prostaglandin from the age of 24 hours until his death after surgery. The 2-D echo Doppler color flow mapping showed findings that suggested right atrial isomerism, severe left ventricular hypoplasia, pulmonary atresia and ductus arteriosus. The pulmonary veins flowed together to a posterior cardiac chamber from which an emissary vertical venous vessel connected with a left superior aneurysmal sack. Two venous channels emerged from this sack: one connecting to the innominate vein and the other to the atrium. The malformations were confirmed by cardiac catheterization. On the sixth day of life, the patient underwent anastomosis between the posterior venous chamber with the atrium, a modified Blalock-Taussig shunt implant, and ductus closure but died during surgery. The association between complex cardiac anomalies and uncommon obstructive total anomalous pulmonary venous connection in the context of right atrial isomerism is lethal and few neonates survive surgical repair. Two-dimensional echo color flow Doppler is a reliable diagnostic technique and an indispensable guide in angiography.     

“Superimposition” digital subtraction angiography: Evaluation of total anomalous pulmonary venous connection

Summary Total anomalous pulmonary venous connection (TAPVC) has a relatively high surgical mortality, especially the infracardiac variety. A small left atrium may limit postoperative cardiac output. Superimposition of digitally subtracted angiographic (DSA) images may be used to define pulmonary venous anatomy, left atrial size, and its spatial relationship to the common pulmonary vein. The technique for acquiring superimposed digitally subtracted images and the results of two cases with infracardiac TAPVC are presented. We have found that this superimposition technique is easily performed and may provide important preoperative information in infants with TAPVC.     

Adverse Hemodynamic Effects Observed with Inhaled Nitric Oxide After Surgical Repair of Total Anomalous Pulmonary Venous Return

The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. At the time of diagnosis, the patient had evidence of systemic right ventricular pressures estimated by continuous-wave Doppler. He was sedated and paralyzed for hyperventilation in preparation for surgery and underwent pulmonary vein confluence to left atrial anastomosis. Postoperative pulmonary hypertension was managed by hyperventilation, sedation, and paralysis until a sudden onset of systemic-level pulmonary pressure required NO therapy. Satisfactory results were obtained in minutes, but a rebound pulmonary hypertension occurred with concomitant systemic hypertension and no radiographic changes. We suspected left atrial hypertension secondary to a sudden increase in pulmonary blood flow to an noncompliant left ventricle. Discontinuation of NO resulted in stabilization of the hemodynamic profile of the patient and he continued to be managed with paralysis, hyperventilation, and sedation. Based on this experience we suggest that NO should be used with caution in patients with obstructive lesions at the atrial level prior to surgery (mitral valve stenosis and cor triatriatum) or in patients with a poorly compliant left ventricle (cardiomyopathy and left ventricular dysfunction). These entities are unable to tolerate a sudden increase in pulmonary blood return thus creating paradoxical pulmonary hypertension.     

Unusual form of total anomalous pulmonary venous connection with double drainage

A patient with complex congenital heart disease was diagnosed by two-dimensional echocardiography. Total anomalous pulmonary venous connection (TAPVC) was suspected because of the results of two-dimensional echocardiography, but the exact anatomy was delineated by cineangiocardiography as an unusual form of TAPVC with double drainage or connections to the left superior vena cava at nearly the same level. The clinical implications and possible embryogenesis for such a condition are discussed.     

Total anomalous infra-cardiac pulmonary venous connection. Value of the color Doppler echocardiography

A case of total anomalous pulmonary venous connection is described in a newborn. The pulmonary veins join a common trunk entering portal vein below the diaphragm. The pattern of pulmonary venous connections was identified by two-dimensional Doppler color flow-mapping echocardiography. The common pulmonary venous trunk was anastomosed to the left atrium at the age of 3 weeks but the infant died because of pulmonary hypertension and acute renal insufficiency. The Doppler color echocardiography allows a good preoperative evaluation. The spontaneous prognosis being poor, surgical correction is indicated and should be performed early, even for critically ill neonates. Patients who survive to childhood lead active and full lives.     

Functional total anomalous pulmonary venous connection via levoatriocardinal vein

We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the “dead‐end” left atrium, the pulmonary venous flow had a to‐and‐fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left‐side heart obstruction.     

Echocardiography diagnosis of total anomalous pulmonary venous drainage in the neonatal period

A 20-days-old female newborn was referred to our hospital because of persistent respiratory distress and a mild central cyanosis. On echocardiography from the suprasternal notch all 4 pulmonary veins could be visualized entering a common chamber posterior to the left atrium. This chamber communicated with a left vertical vein which drained through a large innominate vein into the right superior vena cava. The diagnosis of supracardiac type of TAPVD was confirmed the following day by angiography. The child was successfully operated and the postoperative course was uneventful. Cardiac catheterization carries an increased risk in patients with TAPVD in case of pulmonary hypertension due to pulmonary-venous obstruction. In experienced centers echocardiography has become the definite diagnostic method with a high level of sensitivity and specificity in patients with atrial sitlus solitus, unifocal pulmonary venous connection, and no evidence of other major congenital cardiac defects. With continuously improving knowledge an increasing number of newborns will benefit from improved ability to perform surgical repair without prior cardiac catheterization.     

Atypical atrial septal defects in children: noninvasive evaluation by cardiac MRI

Background  Atypical left-to-right shunts at the level of the atrium in children such as sinus venosus atrial septal defects (ASDs) and partial anomalous pulmonary venous return (PAPVR) may be difficult to assess by transthoracic or transoesophageal echocardiography. Free-breathing cardiac MRI may be a powerful alternative. Objective  To assess the value of free-breathing cardiac MRI in the delineation of atypical ASDs in children. Materials and methods  A total of 82 children (mean age 5.9 years, range 1.1–15.7 years) with suspected ASD and inconclusive transthoracic echocardiography underwent cardiac MRI under free-breathing, mostly sedated conditions. Phase-contrast MRI was used for defect visualization and shunt quantification, and multiphase inflow MR angiography for delineation of pulmonary/systemic venous connections. Results  Of the 82 patients, 34 (41%) were diagnosed with atypical shunt lesions at the level of the atrium and 48 (59%) with simple secundum ASDs. No false-negative or false-positive findings were reported by MRI compared to cardiac catheterization and intraoperative findings. Superior sinus venosus ASD with partial anomalous PAPVR was present in 10 of the 82 children (12.2%), whereas 2 (2.4%) had a large posterior-inferior defect, 5 (6.1%) had isolated PAPVR, and 17 (20.7%) had multiple ASDs and/or associated vascular anomalies. Q_p/Q_s by phase-contrast MRI agreed well with oximetry values (mean difference 3%, limits of agreement ±21–25%; Bland/Altman analysis). Conclusion  Free-breathing cardiac MRI under sedation allows reliable identification of atypical left-to-right shunt defects at the level of the atrium in children in whom transcatheter ASD closure is unsuitable, including delineation of pulmonary or systemic venous anomalies and shunt quantification.     

Tissue plasminogen activator for a left atrial thrombus after Senning repair.

OBJECTIVE: To avoid the surgical removal of an obstructive thrombus in a Senning baffle by the administration of recombinant tissue-type plasminogen activator. SETTING: A pediatric intensive care unit in a children's university hospital. PATIENTS: A 3-yr-old male was diagnosed with a large left atrial thrombus 2 wks after Senning repair for D-transposition of the great arteries. The child presented with massive chylous pleural, pericardial effusions, and cardiac tamponade, secondary to partial obstruction of the pulmonary venous channel. INTERVENTION: Thrombolysis with recombinant tissue-type plasminogen activator was instituted. RESULTS: We observed a resolution of the thrombus in <48 hrs. Minor local bleeding was the only noted side effect. No signs of systemic thromboembolization were detected. CONCLUSION: Early thrombolysis with recombinant tissue-type plasminogen activator could be considered a possible alternative to surgical thrombectomy in selected postoperative pediatric cases, although there may be a potential risk of serious bleeding.