Two-dimensional echocardiography in the pre- and postoperative management of totally anomalous pulmonary venous connection

The records of 23 infants who underwent surgical repair of isolated totally anomalous pulmonary venous connection were reviewed to assess the accuracy of pre- and postoperative echocardiographic diagnoses. Preoperative echocardiographic diagnoses were accurate in 22 of 23 patients, including the sites of connection of the individual pulmonary veins. Cardiac catheterization in 13 patients confirmed the echocardiographic findings. Analysis of multiple pre- and postoperative variables revealed no statistically significant difference between the infants with and without catheterization, although there was a tendency toward a higher mortality rate in the catheterized group. Postoperative echocardiographic examination revealed obstruction to pulmonary venous return in 7 of 19 patients. Catheterization confirmed the echocardiographic findings, localizing the obstruction in one patient. The size of the venoatrial anastomosis was measured on postoperative echocardiograms performed on 14 patients. The cross-sectional area of the anastomosis was less than 0.3 cm2/m2 of body surface area in the four patients with obstruction of the anastomosis, and greater than 0.95 cm2/m2 in all long-term survivors examined. Two-dimensional echocardiography with pulsed Doppler examination and Doppler color flow mapping is an excellent means of diagnosing totally anomalous pulmonary venous connection. The connections of the individual pulmonary veins can be identified in nearly all cases. Surgical repair can usually be undertaken on the basis of echocardiographic diagnosis alone. Echocardiography also provides an extremely accurate method of evaluating surgical repair and of identifying and localizing postoperative obstruction to pulmonary venous return.     

Ultrafast three-dimensional contrast-enhanced magnetic resonance angiography and imaging in the diagnosis of partial anomalous pulmonary venous drainage

OBJECTIVES: The purpose of our study was to evaluate patients with suspected anomalous pulmonary veins (APVs) and atrial septal defects (ASDs) using fast cine magnetic resonance imaging (MRI) and ultrafast three-dimensional magnetic resonance angiography (MRA). BACKGROUND: Precise anatomic definition of anomalous pulmonary and systemic veins, and the atrial septum are prerequisites for surgical correction of ASDs. Cardiac catheterization and transesophageal echocardiography (TEE) are currently used to diagnose APVs, but did not provide complete information in our patients. METHODS: Twenty consecutive patients with suspected APVs were studied by MRA after inconclusive assessment by catheterization, TEE or both. The MRI images were acquired with a fast cine sequence and a novel ultrafast three-dimensional sequence before and after contrast injection. RESULTS: Partial anomalous pulmonary venous drainage was demonstrated in 16 of 20 patients and was excluded in four patients. Magnetic resonance imaging correctly diagnosed APVs and ASDs in all patients (100%) who underwent surgery. For the diagnosis of APVs, the MRI and catheterization results agreed in 74% of patients and the MRI and TEE agreed in 75% of patients. For ASDs, MRI agreed with catheterization and TEE in 53% and 83% of patients, respectively. CONCLUSIONS: Fast cine MRI with three-dimensional contrast-enhanced MRA provides rapid and comprehensive anatomic definition of APVs and ASDs in patients with adult congenital heart disease in a single examination.     

Gadolinium-enhanced three-dimensional magnetic resonance angiography of pulmonary and systemic venous anomalies.

OBJECTIVE: The goal of this study was to evaluate the diagnostic value of gadolinium (Gd)-enhanced three-dimensional (3D) magnetic resonance angiography (MRA) in patients with congenital and acquired anomalies of the pulmonary and systemic veins. BACKGROUND: Gadolinium-enhanced 3D MRA is a fast magnetic resonance imaging technique that has shown great promise in the evaluation of large and medium-sized arteries. However, its application to venous anomalies has not been studied in detail. METHODS: The study retrospectively analyzed all patients who underwent Gd-enhanced 3D MRA examination from January 1998 through January 2001, were diagnosed with anomalies of the pulmonary or systemic veins and had additional diagnostic data available for comparison with the MRA findings. RESULTS: Sixty-one patients (age 1 day to 60 years) were included. Image acquisition was completed in 29 +/- 6.9 s. Pulmonary venous anomalies were found in 37 patients, systemic venous anomalies in 17 patients and both pulmonary and systemic venous anomalies in 7 patients. Compared with available diagnostic information by other modalities, all known or suspected venous anomalies were imaged by 3D MRA. In three patients, catheterization did not detect anomalies of the pulmonary veins that were subsequently diagnosed by MRA. The 3D MRA diagnoses were followed by 10 interventional catheterization procedures and 15 operations. In 74% of patients, 3D MRA either diagnosed previously unsuspected venous anomalies (28%) or added new clinically important information (46%). The mechanism of pulmonary vein compression in eight patients was determined by MRA but not by other imaging modalities. Using a five-level grading system for MRA image quality (1 = nondiagnostic; 5 = excellent), the average grade was 4.6 +/- 0.6, with a 0.28 +/- 0.6 mean grade difference between two independent observers. CONCLUSIONS: Gadolinium-enhanced 3D MRA is capable of rapidly and accurately diagnosing a wide spectrum of pulmonary and systemic venous anomalies and is a useful noninvasive alternative to diagnostic catheterization.     

Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined two-dimensional and Doppler echocardiography

Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascular abnormalities occurring both before and after surgery. Hemodynamic assessment by cardiac catheterization is often difficult. A noninvasive method for detection and quantitation of obstruction to systemic ventricular inflow would be clinically useful. Two-dimensionally directed pulsed and continuous wave Doppler echocardiography was performed before cardiac catheterization in 31 patients thought clinically to have possible obstruction to left ventricular inflow or pulmonary venous return. Primary diagnoses included transposition of the great arteries after the Mustard or Senning procedure in nine patients, total anomalous pulmonary venous connection in nine (in two after surgical repair), cor triatriatum in eight (in four after surgical repair), congenital mitral stenosis in four (in one after surgical repair) and mitral atresia in one. Severe obstruction was defined as a mean pressure gradient at catheterization of greater than or equal to 16 mm Hg at any level of the pulmonary venous return or of the systemic ventricular inflow. Severe obstruction was predicted if Doppler examination measured a flow velocity of greater than or equal to 2 m/s across any area of inflow obstruction. At catheterization, 12 patients (39%) had severe obstruction to left ventricular inflow or pulmonary venous return and all obstructions were correctly detected by Doppler echocardiography. The site of pulmonary venous obstruction was localized by two-dimensionally directed pulsed Doppler study. Patients with a lesser degree of obstruction had a lower Doppler velocity, but none had a maximal Doppler velocity of greater than or equal to 2 ms/s.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cor Triatriatum Sinister with and without Left Ventricular Inflow Obstruction: Visualization of the Entire Supravalvular Membrane by Real‐time Three‐dimensional Echocardiography. Impact on Clinical Management of Individual Patient

We present 4 cases of cor triatriatum in whom the diagnosis was correctly made by 2‐dimensional transthoracic echocardiography, which showed the supravalvular left atrial membrane that divides the left atrium into 2 chambers. The pulmonary veins were connected normally to the proximal left atrial chamber and the left atrial appendage was connected to the distal left atrial chamber. In 1 patient there was evidence of severe pulmonary venous obstruction to the mitral valve by Doppler examination, while in the other three, there was no venous obstruction. Patients were then examined by real‐time 3‐dimensional echocardiography (RT3DE, using ×4 matrix array transducer connected to Sonos 7500 echocardiographic system Phillips, Andover, Mass, USA). This showed the exact morphology of the membrane and led to cancellation of planed surgical intervention in 1 case in which the membrane was only a broad band crossing the left atrial cavity. In addition to delineating the exact morphology of the intracavitary anomaly, this novel echocardiographic imaging modality should be an additive tool to better understand the natural history of these nonobstructive left atrial membranes via longitudinal follow‐up of these patients.     

Two‐ and four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation in the diagnosis of fetal isolated partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare malformation. We describe a case of PAPVC, in which the left pulmonary veins coursed to the left innominate vein through a vertical vein and finally drained into the right superior vena cava; the right pulmonary veins were connected to the left atrium. Tracing the origin and destination of abnormal vessels presented at the three‐vessel and trachea view is useful for the diagnosis. Four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation facilitates the identification of the drainage of fetal pulmonary veins, which should be considered as a complementary modality in obstetric ultrasonic examination when cardiac abnormalities are suspected.     

18‐year experience with transseptal procedures through baffles,conduits, and other intra‐atrial patches

The presence of an intra‐atrial patch (IAP) has been considered a relative contraindication to transseptal puncture (TSP). The purpose of this study is to determine the efficacy and safety of the TSP through baffles, conduits, pericardial patches and other prosthetic materials in the intra‐atrial septum. We reviewed the records of all pediatric patients with IAP who underwent TSP at Texas Children's Hospital from November 1979 through February 1998. The review included the cardiac diagnoses, indications for TSP, technical difficulties and follow up echocardiograms specifically addressing residual atrial shunts A total of 1958 TSP were performed. Thirty‐nine patients had IAP. Cardiac diagnoses in those 39 patients included D‐transposition of the great arteries after Mustard (10) or Senning procedure (6), single ventricle variant post‐Fontan operation (4), total anomalous venous return repair (4), atrioventricular canal repair (9) and atrial septal defect with patch repair (6). Patients' age ranged from 1–31 years (median 7 years). The duration from the time of surgical repair to TSP ranged from 0.1–21 years (median 5 years). Indications for TSP included diagnostic and therapeutic intervention for pulmonary venous obstruction (12), creation of a baffle fenestration (2), prosthetic mitral valve evaluation (1), left ventricular outflow tract evaluation (1), access the left heart for hemodynamic evaluation (23). The IAP was traversed in 38/39 patients (97.5%), followed by diagnostic or therapeutic prograde left‐heart catheterization. No complications were encountered. Follow up echocardiography in 30/38 PTS demonstrated no residual shunting across the atrial septum except for two cases in which the atrial baffle had been intentionally fenestrated. Transseptal puncture through an intra‐atrial patch is a safe procedure. This technique is effective in permitting diagnostic and therapeutic left heart catheterization and does not result in residual shunting through the patch. Cathet. Cardiovasc. Intervent. 50:434–439, 2000. © 2000 Wiley‐Liss, Inc.     

Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?

Between January 1976 and July 1983, 217 patients with atrial septal defect underwent surgical repair at Children's Hospital. Thirty with a primum atrial septal defect and 26 who underwent cardiac catheterization elsewhere before being seen were excluded from analysis. Of the 161 remaining patients, 52 (31%) underwent preoperative cardiac catheterization, 38 because the physical examination was considered atypical for a secundum atrial septal defect and 14 because of a preexisting routine indication. One hundred nine (69%) underwent surgery without catheterization, with the attending cardiologist relying on clinical examination alone in 5, additional technetium radionuclide angiocardiography in 5, M-mode echocardiography in 13 and two-dimensional echocardiography in 43; both M-mode echocardiography and radionuclide angiography were performed in 24 and two-dimensional echocardiography and radionuclide angiography in 19. Since 1976, there has been a trend toward a reduction in the use of catheterization and use of one rather than two noninvasive or semiinvasive techniques for the detection of atrial defects. Of the 52 patients who underwent catheterization, the correct anatomic diagnosis was made before catheterization in 47 (90%). Two patients with a sinus venosus defect and one each with a sinus venosus defect plus partial anomalous pulmonary venous connection, partial anomalous pulmonary venous connection without an atrial septal defect and a sinoseptal defect were missed. Of 109 patients without catheterization, a correct morphologic diagnosis was made before surgery in 92 (84%). Nine patients with a sinus venosus defect, three with sinus venous defect and partial anomolous pulmonary venous connection, four with partial anomalous pulmonary venous return without an atrial septal defect and one with a secundum defect were incorrectly diagnosed.(ABSTRACT TRUNCATED AT 250 WORDS)     

Usefulness of magnetic resonance angiography in the evaluation of complex congenital heart disease in newborns and infants

This study evaluated the quality of the visualization of extracardiac thoracic vessels by magnetic resonance angiography (MRA) in young infants with congenital heart disease. Echocardiography is often sufficient in evaluating CHD in young infants. Cardiac catheterization is needed in some instances to evaluate extracardiac thoracic vessels. Extracardiac thoracic vessels can be accurately evaluated using MRA in adults and older children, but image quality in small infants may be limited. Twenty-nine magnetic resonance angiographic scans were performed at a single institution on 28 infants aged <3 months (median 6 days, range 1 to 90 days) with complex CHD in whom imaging was inconclusive by echocardiography. A blinded observer at a different institution graded (from 0 to 3) the quality of the visualization of the main, branch, lobar, and second-generation pulmonary arteries; lobar pulmonary veins; aortopulmonary collaterals; vena cavae; thoracic aorta and its branches; patent ductus arteriosus; and visceral sidedness. The results of MRA were compared with those of x-ray angiography and surgical inspection, when available. The mean image quality grade was >2 for all structures except the second-generation pulmonary arterial branches, for which it was 2. The median total scan duration was 9 minutes (range 3 to 46). Findings were concordant with surgical inspection (n = 25) and cardiac catheterization (n = 8) in all subjects. There were no complications. In conclusion, MRA is excellent for the visualization of extracardiac thoracic vessels in young infants with CHD and can be used as an alternative to cardiac catheterization when echocardiography is inconclusive.     

Pulmonary atresia with ventricular septal defect. Selection of patients for systemic-to-pulmonary artery shunt based on echocardiography.

From January 1987 to December 1988, in 22 infants with PAVSD, the diagnostic results obtained with echocardiography (two-dimensional, Doppler, and color) were prospectively compared to the angiocardiographic findings. We classified into group 1 patients with confluent and good-sized pulmonary (greater than or equal to 3 mm) arteries, single ductus arteriosus, and normal pulmonary venous connections ("favorable pattern"). The other patients with PAVSD were classified into group 2 ("unfavorable pattern"). The intracardiac anatomy, the morphology of the pulmonary arteries, and the pattern of pulmonary blood supply and pulmonary venous connection were correctly identified with echocardiography in all but one patient, who was erroneously considered to be in group 2. No false-positive of the "favorable pattern" (group 1) was detected. Echocardiography is an effective tool in infants with PAVSD, in order to discriminate cases with "favorable" and "unfavorable" patterns of pulmonary arteries, pulmonary blood supply, and pulmonary veins. The first group with the "favorable pattern" may be considered for systemic-to-pulmonary shunt surgery without angiocardiography. Based on this experience from January to December 1989, four patients with this "favorable pattern" underwent a successful systemic-to-pulmonary artery shunt with an echocardiographic diagnosis alone.     

Double‐Chambered Right Ventricle with Ventricular Septal Defect and Subaortic Membrane— Three‐Dimensional Echocardiographic Evaluation

Double‐chambered right ventricle (DCRV) is a rare congenital anomaly in which the right ventricle is divided into two compartments with varying pressures due to an anomalous muscle bundle. Here, we describe a case of an adolescent male with DCRV with associated ventricular septal defect and subaortic membrane. Two‐dimensional and three‐dimensional transthoracic echocardiography with color flow clearly outlined all the three cardiac anomalies as well as their relationship with each other. The diagnosis was confirmed by cardiac catheterization. The patient underwent successful surgical resection of the anomalous muscle bundle along with repair of the associated anomalies.     

Accuracy of two-dimensional echocardiography in the diagnosis of congenital heart disease

To assess the accuracy of 2-dimensional (2-D) echocardiography in the evaluation of cardiac anatomy in patients with congenital heart disease, 2-D echocardiograms were performed in 126 infants and children before cardiac catheterization and angiocardiography. The segmental echocardiographic analysis included determination of intracardiac, great artery, systemic venous and pulmonary venous anatomy. The 126 patients had 259 separate cardiovascular abnormalities, of which 226 (87%) were prospectively identified by 2-D echocardiography. There were 8 false-positive diagnoses. The most common lesions and the sensitivity and specificity of echocardiography were: patent ductus arteriosus, 41 patients (83% and 100%, respectively), ventricular septal defect, 35 patients (86% and 100%); atrial septal defect, 26 patients (85% and 99%); pulmonary valve stenosis, 25 patients (77% and 97%), transposition of the great arteries, 16 patients (100% and 100%); and total anomalous pulmonary venous connection, 14 patients (85% and 100%). Less common defects and their rate of detection included coarctation of the aorta, 10 of 12 patients; atrioventricular canal, 10 of 10 patients; tetralogy of Fallot, 10 of 10 patients; aortic valve stenosis 8 of 8 patients; right aortic arch, 8 of 8 patients; interrupted aortic arch, 4 of 4 patients; and unilateral pulmonary vein atresia, 0 of 1 patient. In 33 patients (26%), the errors in echocardiographic analysis were judged to have surgical importance. Most errors were the result of overlooking or misinterpreting data that had been appropriately recorded on videotape. Pulmonary valve stenosis and patent ductus arteriosus are the lesions most likely to be misdiagnosed by ultrasound studies relying on imaging alone.     

Management and outcome of infants and children with right atrial isomerism.

OBJECTIVES: To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease. SETTING: Tertiary referral centre. METHODS: 20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures. RESULTS: Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine). CONCLUSIONS: Surgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.     

Diagnosis and treatment of acute pulmonary embolism in 2010

Diagnostic approaches in acute pulmonary embolism include evaluation of clinical likelihood, D-dimers, echocardiography and spiral CT angiography and pulmonary scintigraphy. Determination of D-dimers is only meaningful in patients with low or intermediate clinical likelihood. It is safe not to initiate anticoagulation treatment (or to discontinue such treatment) in patients with low clinical likelihood of acute pulmonary embolism and negative D-dimer test (only if methods with 99-100% sensitivity are used). Duplex sonography and pulmonary scintigraphy are only necessary at the centres with a first generation spiral CT and not those with multidetector devices. Investigations in normotensive patients should include echocardiography that should also include assessment of the right ventricular function using echocardiography and determination of biomarkers of pulmonary embolism. Right ventricular dysfunction together with elevated troponins identifies a normotensive group at an increases risk. Highly sensitive troponin T (hsTnT) appears to be particularly valuable. Echocardiography reading might the decisive factor for treatment initiation in patients with massive acute pulmonary embolism. Negative or unclear echocardiography finding warrants spiral CT angiography (CTA). Ventilation/perfusion scan or pulmonary arteriography are recommendable in patients with unclear CTA finding and patients with high clinical likelihood of pulmonary embolism and negative CTA finding. A combination of CTA and CTV also appears useful as it increases the overall sensitivity of the investigation and enables imaging of pelvic veins. Thrombolytic treatment is indicated in haemodynamically unstable patients, patients with a high risk of a massive pulmonary embolism associated with cardiogenic shock or hypotension (systolic pressure below 90 mmHg or a decrease in systolic pressure by > 40 mmHg) or symptoms of acute right-sided heart failure. Thrombolytic treatment is also indicated in pulmonary embolism not receding following heparin treatment, in recurring or expanding pulmonary embolism, in the presence of thrombi in the right heart and in patients with right-to-left shunting through patent foramen ovale. This treatment should also be considered in patients with submassive pulmonary embolism associated with a dysfunction of the right ventricle and increased troponins, and particularly in patients lacking even a relative contraindication of thrombolytic treatment. A thrombolytic of choice is alteplase. Embolectomy or catheterization should be used if thrombolytic treatment is contraindicated or ineffective. Long-term monitoring of massive and submassive acute pulmonary embolism is highly recommended. Low molecular weight heparins or unfractioned heparin or fondaparinux are used in haemodynamically stable patients.     

Total anomalous pulmonary venous drainage: is a surgical correction possible on the sole basis of echocardiography?

Between April '83 and August '89, 33 patients with total anomalous pulmonary venous drainage were studied at the department of Cardiology in Bergamo. There were 18 males and 15 females, aged between three days and 8 months (average: two months). In this study only cases of isolated total anomalous pulmonary venous drainage were taken into consideration. The patients underwent echocardiographic examination with ATL MK 600, Vingmed 700, ATL Ultramark 9 with 3.5; 5; 7.5; MHz transducers; in the last three years the echocardiographic examination was integrated by continuous and pulsed wave Doppler and, in the last year, by color Doppler. The morphologic diagnosis was routinely established by means of the sequential approach method. A common feature in all types of total anomalous pulmonary venous drainage was the impossibility of defining the connections of the pulmonary veins with the left atrium. Furthermore, patients had a volume overload of the right heart, and atrial septal defects of various sizes. The site of anomalous drainage of the pulmonary veins was assessed by means of multiple cuts from subcostal, precordial and suprasternal windows. The echocardiographic diagnosis was exact and complete in 29 cases (87.9%), and in four cases it was incomplete but basically correct (12.1%). The anatomical findings were confirmed during cardiac surgery in 32 cases and by autopsy in one case of supracardiac total anomalous pulmonary venous drainage in a critically ill patient, who died before surgery. Of the 32 patients who underwent surgical correction, 20 (62.5%) had only an echocardiographic diagnosis, which resulted correct in all cases. The majority of patients with isolated TAPVD can be confidently diagnosed by means of echocardiography, thus, avoiding preoperative catheterization.     

Echocardiographic and color flow Doppler assessment of systemic and pulmonary venous connection and drainage in the neonate with congenital heart disease

Systemic and pulmonary venous anomalies are frequently encountered either as isolated lesions or as a significant component of a more complex lesion in the newborn infant with congenital heart disease. Two-dimensional echocardiography and Doppler techniques (conventional and color flow) have become the primary diagnostic imaging modality in this setting. Precise pre-operative definition of these variable venous connection and drainage patterns is critical as the required surgical procedure may solely be based on exact understanding of the veins' anatomy and physiology. On the systemic venous site, anomalies of superior and inferior venae cavae, innominate vein, and coronary sinus can be equally well imaged with either echocardiography or angiography. However, on the pulmonary venous site, echocardiography and Doppler techniques including color flow mapping are superior to angiography for precise definition of the connection and drainage sites of the individual pulmonary veins.     

Concealed left atrial membrane: Pitfalls in the diagnosis of cor triatriatum and supravalve mitral ring

Cor triatriatum and supravalve mitral ring are forms of congenital left ventricular inflow obstruction produced by membranes within the left atrium. Typically, these defects occur as isolated anomalies with manifestations of pulmonary venous obstruction. Four children are presented whose left atrial membrane was associated with other significant cardiac defects, including, in one patient each, simple coarctation of the aorta, sinus venosus atrial septal defect, tricuspid atresia and complex coarctation of the aorta syndrome. The patient with the latter defect had undergone previous pulmonary arterial banding. None of these patients demonstrated significant pulmonary venous obstruction at cardiac catheterization. All patients had a normal value for either pulmonary arterial diastolic or pulmonary arterial wedge pressure. Three mechanisms explained the lack of pulmonary venous obstruction: (1) a large cross-sectional area of membrane openings, (2) an atrial septal defect that was confined to the pulmonary venous chamber and decompressed it by allowing blood to escape into the right atrium, and (3) decreased pulmonary blood flow. The diagnosis was facilitated by two dimensional echocardiography. Accurate diagnosis of left atrial membrane in the setting of other cardiac defects is of practical significance because pulmonary venous obstruction may occur after surgery for the associated defects.     

Absence of the inferior vena cava in a patient with omphalocele: Two-dimensional echocardiographic and cineangiographic findings

Summary We report an unusual form of absence of the inferior vena cava (IVC) in a patient with a repaired omphalocele. Two sets of bilateral paravertebral veins served as the channels of systemic venous return from the lower half of the body. These veins were narrower than typical azygos or hemiazygos continuation in the absence of the IVC, with the result that a catheter from the femoral vein could not reach the right atrium (RA). Other associated venous-side anomalies were present, including a chamber between the hepatic vein (HV) and RA, narrowing and angulation at the junction between the HV and the chamber, and a subaortic innominate vein. All these anomalies were demonstrated by two-dimensional and color Doppler echocardiography. The recognition of these venous anomalies is important for cardiac catheterization or IVC cannulation for cardiopulmonary bypass in patients with omphalocele.     

Echocardiographic versus cardiac catheterization diagnosis of infants with congenital heart disease requiring cardiac surgery

The success of noninvasive preoperative evaluation of infants with congenital heart disease using cardiac ultrasound depends not only on diagnostic accuracy, but also on risk of morbidity and mortality as compared with infants who undergo cardiac catheterization. Fifty-six infants (age 10 weeks or younger) with coarctation of the aorta (n = 16), coarctation with ventricular septal defect (n = 12), valvar aortic stenosis (n = 10) or total anomalous pulmonary venous connection (n = 18) were examined. Thirty-one underwent noninvasive preoperative assessment and 25 underwent evaluation including cardiac catheterization. Age, level and duration of support, pH, renal function, mortality, complications of cardiac catheterization and errors of diagnosis were compared. Significant differences between the 2 groups were more frequent preoperative use of prostaglandin E1 and shorter hospital stay in the noninvasively evaluated coarctation group. Of the infants with coarctation and ventricular septal defect, 1 who had cardiac catheterization required renal transplantation and 1 evaluated noninvasively required surgery at age 3 months for mitral stenosis not discovered on preoperative evaluation. One noninvasively evaluated infant with total anomalous pulmonary venous connection had a stenotic communication between the pulmonary venous confluence and the left atrium not detected by ultrasound. Surgery was successful in the latter 2 infants. Noninvasive preoperative diagnosis of some infants with congenital heart disease can be performed without increasing the risk of operative morbidity and mortality. Eliminating cardiac catheterization reduces hospital costs, decreases total numbers of catheterizations performed and influences the structure of training programs.     

Repair of total anomalous pulmonary venous connection in early infancy

From May 1995 through October 2001, 19 infants less than 90 days old underwent surgical correction of total anomalous pulmonary venous connection. In 15 babies with isolated total anomalous pulmonary venous connection, there was one operative death. In 4 with complex anomalies, there were 2 operative deaths. The vertical vein was not ligated in 6 cases for various reasons. Two patients died during reoperation for early pulmonary venous obstruction. In the late follow-up, 2 babies required reoperation for late anastomotic stricture; one needed additional balloon dilatation. Of the 14 surviving patients, one had a small residual gradient and infrequent supraventricular tachycardia, the others were asymptomatic and without gradients. Surgical correction of total anomalous pulmonary venous connection can be carried out in early infancy with low mortality and morbidity. However, associated complex cardiac anomalies and small caliber pulmonary arteries and veins carry higher risks. Recurrent pulmonary venous obstruction and diffuse pulmonary vein stenosis are causes of early reoperation and poor surgical outcome.