Detection of atrial septal defect with left-to-right shunt by inferior vena cava contrast echocardiography.

Contrast echocardiography was used before cardiac catheterisation in 37 patients with atrial septal defect and a left-to-right shunt and in 18 patients with a raised right atrial and ventricular pressure to assess the contrast echo effect in the inferior vena cava. Using two dimensional contrast apical echocardiography we found a negative contrast echo effect within the right atrium in many but not all patients with atrial defect. Contrast echoes entering the inferior vena cava during presystole or early to mid-diastole were detected in patients with heart disease causing raised right atrial and ventricular pressures and also in all patients with atrial septal defect. No contrast echo effect in the inferior vena cava was detected in 10 normal subjects. The sensitivity of this contrast pattern in the inferior vena cava in diagnosing atrial septal defect was 100%. When other conditions causing raised right atrial pressure were excluded, the specificity and predictive accuracy were 100% for both. The presystolic contrast echo effect in the inferior vena cava, semiquantitatively graded, correlated with the size of the shunt determined by oximetry. In 20 patients re-examined after the surgical correction of the atrial septal defect, no presystolic contrast echo effect was detected in the inferior vena cava. Contrast echocardiography of the inferior vena cava is a valuable and reliable method for diagnosing atrial septal defect with left-to-right shunt.     

Assessment of right heart hemodynamics by contrast echocardiography

We studied inferior vena cava contrast echocardiography after upper extremity injection in 70 subjects; 59 were patients and 11 were controls. Inferior vena cava contrast was seen in 35 patients and in 1 control. "A-wave synchronous pattern" of contrast appearance was observed in 13 patients and 1 normal subject. The pattern did not depend upon the height of right atrial a-wave pressure or the right ventricular (RV) end-diastolic pressure, but was related to the respiratory cycle. A "random pattern" of contrast appearance was seen in 3 patients with cardiac arrhythmia and normal right heart hemodynamics. One patient with ventricular premature beats showed both "a-wave synchronous" and "random" patterns. A "v-wave synchronous pattern" was found in 20 patients, of which 17 had tricuspid regurgitation. Persistence of inferior vena cava contrast correlated with the height of right atrial v-wave (r = 0.87, p less than 0.001) and the severity of tricuspid regurgitation estimated from RV cineangiography. The differences of RV systolic pressure and echocardiographic right ventricular dimension between the study patients with and without tricuspid regurgitation did not reach statistical significance. We conclude: the echocardiographic RV dimension and the degree of RV hypertension are not predictors for the presence of tricuspid regurgitation and its severity; inferior vena cava contrast echocardiography may be used to estimate the severity of tricuspid regurgitation.     

Right-to-left shunting with normal pulmonary pressure complicating atrial septal defect surgery

We report on a patient in whom a right-to-left shunt with normal pulmonary arterial pressures occurred after surgery of an inferior vena caval sinus venosus-type atrial septal defect. In addition to the inferior vena cava to left atrium anatomic defect, the exceptional occurrence of a post-operative right atrial inflow stenosis contributed to the right-to-left shunting.     

Assessment of tricuspid regurgitation by directional analysis of right atrial systolic linear reflux echoes with contrast M-mode echocardiography

Twenty-seven individuals were studied for diagnostic assessment of tricuspid regurgitation (TR) using directional analysis of echo contrast lines on M-mode echocardiograms (MME). Group I consisted of 12 patients with physical findings, as well as phonocardiogram and jugular venous pulse tracings, compatible with TR. Group II consisted of five normal volunteers and 10 control patients without any evidence of congestive heart failure or TR. Following peripheral venous injection of contrast material, all 12 patients in group I demonstrated linear reflux contrast echoes in the right atrium (RA) on MME consisting of multiple posteriorly directed echo lines throughout systole behind the tricuspid valve. In addition, linear reflux contrast echoes in the hepatic vein directed away from its entry into the inferior vena cava were noted in 8 of the 12 patients by a cursor-placed MME. The remaining 15 patients in group II showed none of the above findings on contrast echocardiography. These observations indicate that linear systolic reflux contrast echoes in the RA behind the tricuspid valve on MME may be a specific and sensitive sign of TR.     

Identification of atrial septal defects by cross-sectional contrast echocardiography.

Cross-sectional echocardiography, combined with injections of contrast into peripheral arm veins, has been used to study 15 patients with atrial septal defects and 10 patients with an intact interatrial septum. Of 11 patients with ostium secundum or sinus venosus atrial septal defects and left-to-right shunts a defect could be visualised in all, and in eight some degree of transfer of contrast from right atrium to left atrium was seen. In three of four patients with a dominant right-to-left shunt a defect was seen and in all there was free transfer of contrast from right atrium to left atrium. Though there may be variable loss of echoes in the septal image in patients with an intact interatrial septum, in general no fixed defect is seen an there is no transfer of contrast from right atrium to left atrium. This is a potentially valuable technique in the assessment of patients in whom an atrial septal defect is suspected.     

Atrial septal defect with right to left shunt despite normal pulmonary artery pressure

A 74 year old woman had right to left shunting through an atrial septal defect despite normal right heart pressures. Acute volume expansion temporarily reduced the shunt. Contrast echocardiography and angiography demonstrated that this shunting occurred almost exclusively from the inferior vena cava. At surgery a redundant flap of septum secundum was found that was adjacent to the inferior vena cava orifice, intercepting its blood return like a spinnaker and shunting it into the left atrium.     

Left Ventricular to Right Atrial Septal Defect Secondary to Blunt Thoracic Trauma Diagnosed by Transesophageal Echocardiography

A case report is presented of a 58-year-old man who developed a new holosystolic murmur 4 months after a high-speed motor vehicle accident. Cardiac catheterization demonstrated a left-to-right shunt at the right atrial level. Intraoperative transesophageal echocardiography (TEE) identified and localized a discrete atrioventricular septal defect associated with a shunt from the left ventricle to the right atrium without tricuspid regurgitation, findings that were confirmed after surgical exploration. TEE is recommended for patients with a heart murmur and a history of blunt cardiac trauma, may permit early diagnosis, and may allow surgical repair in selected patients without pre-operative catheterization. (ECHOCARDIOGRAPHY, Volume 8, May 1991)     

Left to right atrial shunting in tricuspid atresia.

In tricuspid atresia, an obligatory right to left shunt occurs at the atrial level. We have observed several patients with left to right interatrial shunts. Data from cardiac catheterisation in 40 consecutive patients were reviewed to determine the frequency and mechanism of left to right shunting in tricuspid atresia. An increase of 6% or more in oxygen saturation between the superior vena cava and the right atrium in two or more sets of saturations, representing a left to right shunt, was present in 29 out of 50 (58%) catheterisations in which the data were adequate. In most, the shunt was also seen cineangiographically in the laevophase. In only two catheterisations was an anatomical cause (ostium primum atrial septal defect in one and anomalous pulmonary venous return in the other) found. In the remaining 27 catheterisations, no anatomical cause was found. Age, Qp:Qs, and mean atrial pressure difference were similar between the shunt and non-shunt groups. In the shunt group right atrial "a" waves were equal to or higher than left atrial "a" waves and left atrial "v" waves were equal to or higher than right atrial "v" waves. Simultaneous pressure recordings (in one patient with left to right atrial shunt) from the left atrium and right atrium with isosensitised miniature pressure transducers mounted 5 cm apart showed (1) a higher pressure in the right atrium than in the left atrium during atrial systole and (2) a higher pressure in the left atrium than in the right atrium during atrial disatole. It is concluded that (a) left to right shunt across the atrial septum occurs frequently in tricuspid atresia and (b) the left to right shunt is the result of instantaneous pressure differences between the atria.     

Isolated tricuspid valve regurgitation resulting from severe annular dilatation: case report.

A rare case of isolated tricuspid regurgitation (TR) in a 65-year-old man is presented. Echocardiography revealed enlargement of the right atrium, dilatation of the tricuspid valve annulus without thickening or prolapse of the leaflets, and an intact atrial septum. No downward displacement of the tricuspid septal leaflet was observed by echocardiography. Mild mitral regurgitation and severe TR were detected on color flow Doppler studies. Cardiac catheterization indicated elevated right atrial pressure, with a pronounced V-wave. No left-to-right shunt was detected at the right atrium. At surgery, severe annular dilatation of the tricuspid valve (without organically diseased or deformed tricuspid leaflets) was observed, and tricuspid annuloplasty with a prosthetic ring performed. Postoperative echocardiography and right ventriculography showed trivial TR.     

Budd-Chiari syndrome as a late complication following closure of an atrial septal defect

A case of a Budd-Chiari syndrome in a 19-year-old female patient is reported who had undergone surgical closure of a secundum atrial septal defect 13 years before. 8 months before the development of the Budd-Chiari syndrome she started to take oral contraceptives. The clinical picture of the Budd-Chiari syndrome developed within several days. The inferior vena cava did not fill with contrast dye when an angiography was performed using the right vena iliaca approach. The contrast dye disappeared through collateral veins (vena azygos, vena hemiazygos). After 4 days of treatment with systemic streptokinase she underwent open-heart surgery. The orifice of the inferior vena cava was occluded to a diameter of 6 mm. No thrombi were found. The lesion was corrected with two patches, one in the right atrium and the other in the inferior vena cava. This case report demonstrates that a Budd-Chiari syndrome is a possible late complication after closure of an atrial septal defect which should be treated by surgery.     

Right atrial pressure-volume relationships in tricuspid regurgitation

Pressure-volume relationships in the right atrium were examined before and after the creation of acute experimental tricuspid regurgitation in pigs. A 1.3 kHz multielectrode impedance catheter with a measuring current of 4 mA was used to determine instantaneous right atrial pressure and relative blood volume; right atrial dimension was assessed simultaneously with ultrasonic crystals attached to the atrial walls. Impedance volume waveforms and ultrasonic crystal dimensions closely paralleled each other at baseline and after the induction of tricuspid regurgitation. The normal right atrial pressure-volume plot exhibited a figure-of-eight configuration, with an "a-loop" and a "v-loop" corresponding to the a-wave and v-wave of the right atrial pressure tracing. With severe tricuspid regurgitation, atrial pump function was abolished, and the pressure-volume plot exhibited a single clockwise loop, consistent with complete ventricularization of the right atrium. Intermediate degrees of tricuspid regurgitation preserved the figure-of-eight loop, but the size of both the a-loop and the v-loop were increased, consistent with a Starling-type load imposed on the atrium by the regurgitant blood volume. Increased right ventricular afterload mediated by constriction of the pulmonary artery and infusion of methoxamine reversibly converted the right atrial pressure-volume loop from that of mild to that of severe tricuspid regurgitation. Alternatively, constriction of the inferior vena cava and infusion of nitroprusside changed the right atrial pressure-volume loop from that of a severe pattern of tricuspid regurgitation to a less severe type of pattern. Infusion of dobutamine increased the size of the a-loop relative to the v-loop both at baseline and after induction of tricuspid regurgitation. We conclude that tricuspid regurgitation induces changes in right atrial mechanics that can be detected and quantified with an impedance catheter.     

Atrial septal hematogenous cyst with calcification mimicking myxoma: A rare case

A 61-year-old female presented with right atrial mass during physical examination. Contrast-enhanced left heart echocardiography revealed a mass with the size of 32*23 mm in the right atrium, attached to the atrial septum; there was a certain degree of activity and deformation. MRI showed a mass of about 35*22 mm in the right atrium adjacent to the atrial septum, which was diagnosed with right atrial myxoma. Intraoperative TEE showed that the mass was located in the atrial septum close to the inferior vena cava and spontaneous echo contrast with hyperechoic images within the mass. The lesion was resected under cardiopulmonary bypass. Pathological examination revealed that the filling defect was an atrial septal hematogenous cyst with calcification.     

Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve. The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.     

Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.     

Hypoxemia explained 36 years later.

A 37-year-old man who had an atrial septal defect (ASD) corrected as an infant was found to be hypoxemic with a 22% shunt. An MRI scan revealed that the patient's inferior vena cava drained into his left rather than his right atrium, a previously undetected complication of his ASD repair 36 years before.     

Masked abnormal drainage of the inferior vena cava into the left atrium

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Stroke after surgical treatment of sinus venosus type atrial septal defect: percutaneous treatment

Background and purposeSinus venosus-type atrial septal defect can be associated with anomalous drainage of the upper right pulmonary vein into the superior vena cava. Surgical correction provides reconstruction of superior vena cava and rerouting of the pulmonary vein into the left atrium.Summary of caseWe report the case of a young woman who experienced 2 strokes 11 years after surgical repair of sinus venosus-type atrial septal defect.ConclusionsInterventional treatment resolved the superior vena cava stenosis, rerouted the pulmonary vein into the left atrium and abolished the right to left shunt.     

Ebstein's anomaly: one and a half ventricular repair

Patients with Ebstein's anomaly can present after childhood or adolescence with cyanosis, arrhythmias, severe right ventricular dysfunction and frequently with left ventricular dysfunction secondary to the prolonged cyanosis and to the right ventricular interference. At this point conventional repair is accompanied by elevated mortality and morbidity and poor functional results. We report our experience with three patients (8, 16 and 35 years of age) with Ebstein's anomaly, very dilated right atrium, severe tricuspid valve regurgitation (4/4), bi-directional shunt through an atrial septal defect and reduced left ventricular function (mean ejection fraction = 58%, mean shortening fraction = 25%). All underwent one and a half ventricular repair consisting of closure of the atrial septal defect, tricuspid repair with reduction of the atrialised portion of the right ventricle and end-to-side anastomosis of the superior vena cava to the right pulmonary artery. All patients survived, with a mean follow-up of 33 months. In all there was complete regression of the cyanosis and of the signs of heart failure. Postoperative echocardiography showed reduced degree of tricuspid regurgitation (2/4) and improvement of the left ventricular function (mean ejection fraction = 77%, mean shortening fraction = 40%). In patients with Ebstein's anomaly referred late for surgery with severely compromised right ventricular function or even with reduced biventricular function, the presence of a relatively hypoplastic and/or malfunctioning right ventricular chamber inadequate to sustain the entire systemic venous return but capable of managing part of the systemic venous return, permits a one and a half ventricular repair with good functional results.     

Echocardiographic aspects of persistence of the right valve of the venous sinus

The aim of this work is to demonstrate the possibility to identify persistent right sinus venous valve and to differentiate it from other right sided heart pathology by two dimensional echocardiography. We report the echocardiographic findings observed in three out of 215 paediatric patients we examined for clinically suspected congenital heart disease in 12 months period. The first patient was a 24 hours old newborn with transient pulmonary hypertension; the second one was a 6 months old child with pulmonary atresia, severe right ventricular hypertrophy, atrial septal defect and patent ductus arteriosus; the third patient, aged 6 months, had atrial septal defect and mild pulmonary stenosis. In all three patients a particular linear structure was seen, as a membrane that crossed the right atrium from the orifice of the inferior vena cava towards the atrial septum. This structure was identified as persistent right sinus venosus valve according to its morphology, its position into the right atrium and its connection to the atrial septum. The incidence of this echocardiographic finding was 1.4% in our series. In the first case contrast echocardiography from the inferior vena cava demonstrated the deviation of blood flow caused by the persistent sinus valve, although no intra-atrial pressure gradient was shown at cardiac catheterization. In the second patient who died during the operative procedure for making a systemic-pulmonary anastomosis, post mortem examination showed a membranous structure connecting the orifice of the inferior vena cava to the atrial septum.(ABSTRACT TRUNCATED AT 250 WORDS)     

Edge-to-edge repair of congenital familiar tricuspid regurgitation: case report

We report a case of edge-to-edge (Alfieri's technique) repair of congenital familiar tricuspid regurgitation in a 49-year-old woman, who had severe tricuspid regurgitation, atrial septal defect with left-to-right shunt, and two stenoses in peripheral branches of the left pulmonary artery, of no clinical relevance. The repair was performed through a longitudinal inferior partial sternotomy. The atrial septal defect was closed by direct suture; the anterior and posterior leaflets of the tricuspid valve were sutured together. The chordae to the prolapsing medial part of the anterior leaflet were shortened by direct suture to the leaflet free edge. Annuloplasty was performed by means of a Carpentier ring. The final step was edge-to-edge approximation of the septal leaflet to the new antero-posterior position with two interrupted stitches. The hemodynamic result was excellent, and the patient eventually returned to full active life.