Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.     

Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.     

Atrioventricular valve abnormalities in infancy: two-dimensional echocardiographic and angiocardiographic comparison

The results of two-dimensional echocardiography and biplane angiocardiography from 47 infants with congenital atrioventricular (AV) valve abnormalities were compared. Eleven patients had atresia of the right AV valve, 10 had atresia of the left AV valve, 4 had hypoplasia of the right AV valve and 5 had hypoplasia of the left AV valve. Twelve patients had endocardial cushion defect, three had single ventricle and two had straddling of the left AV valve. There was agreement between the two techniques as to the number of AV valves present in each patient. The echocardiographic estimate of valve anular diameter was below normal in seven of the eight patients thought to have a hypoplastic anulus by angiocardiography. In 10 of the 12 patients with endocardial cushion defect, there was agreement between the two techniques as to the presence or absence of atrial and ventricular septal defect. The chordal attachments of straddling valves were better visualized by echocardiography; flow patterns and effective orifice size were better demonstrated by angiocardiography. The subcostal four chamber echocardiographic views and cranially angulated oblique angiocardiographic views were comparable and provided the best images for determination of the size and number of AV valves and their relation to the atrial and ventricular septa.     

Diastolic ventricular septal motion in atrial septal defect: Analysis of M-mode echocardiograms in 31 patients

Previous echocardiographic studies suggest that diastolic motion of the ventricular septum reflects relative filling of the right and left ventricles. We studied 31 patients with atrial septal defect by M-mode echocardiography. Early diastolic posterior ventricular septal motion (DPSM) occurred in all patients. Measurement of DPSM correlated with pulmonary to systemic flow ratios (Qp:Qs) (r = 0.64, p < 0.001). All 15 patients with DPSM > 5 mm had a Qp:Qs > 2.5:1, whereas only 8 of 16 patients with DPSM < 5 mm had a shunt this large (p < 0.003). DPSM > 5 mm in patients with atrial septal defect is a specific but not sensitive echocardiographic sign of a large left-to-right shunt. Our findings substantiate the hypothesis that diastolic motion of the ventricular septum reflects relative filling of the ventricles.     

An echocardiographic study of perimembranous ventricular septal defect with left ventricular to right atrial shunting

Twenty nine patients with isolated perimembranous ventricular septal defects were investigated by M mode, cross sectional, and pulsed Doppler echocardiography. Tricuspid valve anomalies were present in all six patients with a left ventricular-right atrial shunt but in only six (26%) of 23 patients who had interventricular shunts only. Systolic flutter of the tricuspid valve was shown in five (83%) of the six patients with a ventriculoatrial shunt but not in the other patients. Systolic turbulence in both the right ventricle and right atrium was detected by Doppler echocardiography only in patients with ventriculoatrial shunting. A perimembranous ventricular septal defect with left ventricular to right atrial shunt can be diagnosed by its combined M mode, cross sectional, and pulsed Doppler echocardiographic features.     

An echocardiographic study of perimembranous ventricular septal defect with left ventricular to right atrial shunting.

Twenty nine patients with isolated perimembranous ventricular septal defects were investigated by M mode, cross sectional, and pulsed Doppler echocardiography. Tricuspid valve anomalies were present in all six patients with a left ventricular-right atrial shunt but in only six (26%) of 23 patients who had interventricular shunts only. Systolic flutter of the tricuspid valve was shown in five (83%) of the six patients with a ventriculoatrial shunt but not in the other patients. Systolic turbulence in both the right ventricle and right atrium was detected by Doppler echocardiography only in patients with ventriculoatrial shunting. A perimembranous ventricular septal defect with left ventricular to right atrial shunt can be diagnosed by its combined M mode, cross sectional, and pulsed Doppler echocardiographic features.     

The myocardial performance index in children with isolated left-to-right shunt lesions.

OBJECTIVE: The myocardial performance index (MPI) measures the ratio of isovolumic time intervals to ventricular ejection time. The effects of altered ventricular preload or afterload on MPI have yet to be determined. This study was designed to determine the impact of altered preload on left and right ventricular myocardial performance index in the clinical setting of left-to-right lesions. METHODS: The left and right ventricular myocardial performance indexes were measured in 17 patients with atrial septal defect (ages 6 to 148 months), 23 patients with ventricular septal defect (ages 2 to 160 months), and 24 healthy children (ages 3 to 160 months). A complete 2- dimensional and Doppler echocardiographic examination was performed in all study groups. RESULTS: In patients with atrial septal defect, ventricular septal defect, and control group subjects, the left ventricular MPI was 0.38, 0.37 and 0.32, respectively, and the right ventricular MPI was 0.24, 0.21, and 0.20, respectively. No significant differences in the left and right ventricular myocardial performance indexes were seen between patients with left-to-right shunt lesions and control subjects. CONCLUSION: This study documents that the myocardial performance index is a quantitative measure of ventricular function that appears to be relatively independent of changes in preload.     

Real-time evaluation of the hemodynamic effects of atrial septal defect closure in adults with left ventricular dysfunction.

Transcatheter closure of atrial septal defects with left-to-right shunt induces an abrupt overload of the left ventricle that may cause acute heart failure in patients with left ventricular dysfunction. We report two cases of ostium secundum atrial septal defects associated with left ventricular dysfunction of different etiology. The hemodynamic evaluation of left ventricular function during transient abolition of the shunt with the sizing balloon of the Amplatzer system helped to establish the most correct therapeutic strategy.     

Role of two-dimensional echocardiography, pulsed, continuous wave color flow Doppler techniques in the assessment of ventricular septal rupture after myocardial infarction

Two-dimensional echocardiography, pulsed and continuous wave Doppler techniques were used for the evaluation of 15 consecutive patients (9 men, 6 women; mean age 71 years, range 61 to 79) with ventricular septal rupture due to acute myocardial infarction (7 anterior, 8 posterior). Standard and modified off-axis 2-dimensional echocardiographic views from parasternal, apical and subcostal windows correctly identified this defect in 14 of the 15 patients. Pulsed Doppler echocardiography confirmed the presence of left-to-right-sided shunt by showing a high-velocity, aliased, systolic flow and a low-velocity diastolic flow in the right ventricle in 14 patients. Continuous wave Doppler echocardiography showed a high-velocity systolic and low-velocity diastolic flow signal of left-to-right shunt in 14 patients. Color flow Doppler imaging identified a left-to-right shunt in all 6 patients in whom it was performed. Doppler and 2-dimensional echocardiographic studies missed a small apical septal defect in 1 patient with anteroseptal myocardial infarction. Two-dimensional echocardiography correctly diagnosed right ventricular infarction in all 5 patients with posteroinferior infarction. Ventricular septal rupture and/or left-to-right-sided shunt was confirmed in all 15 patients by the following: surgical inspection in 11, necropsy in 3, left ventricular cineangiography in 5 and right-sided heart catheterization and oximetry data in 13 patients. Data indicate that 2-dimensional echocardiography correctly shows the precise location of septal rupture in most patients after acute myocardial infarction and allows assessment of left and right ventricular infarction and function.(ABSTRACT TRUNCATED AT 250 WORDS)     

Partial atrioventricular septal defect assessed by real-time three-dimensional echocardiography: a case report

A 48-year-old woman was admitted with a heart murmur and increased difficulty in breathing. Two-dimensional echocardiography revealed a defect in the lower part of the atrial septum [(primum atrial septal defect (ASD)] and a pouch at the interventricular septum. Color Doppler echocardiography detected grade 3/4 mitral regurgitation. Real-time three-dimensional echocardiography (RT-3DE) revealed a cleft in the anterior leaflet of the mitral valve toward the tricuspid valve, and the ASD located near the atrioventricular valves with 14mm in minor axis. Color Doppler three-dimensional echocardiography disclosed left-to-right ASD shunt toward the atrial posterior wall. No shunt through the pouch at the membranous part, left ventricular outflow obstruction, or partial anomalous pulmonary venous connection was observed. RT-3DE is quite useful to describe complicated congenital heart disease.     

Double-inlet single left ventricle: echocardiographic anatomy with emphasis on the morphology of the atrioventricular valves and ventricular septal defect.

The echocardiographic anatomy of double-inlet single left ventricle was studied in 57 patients, aged 1 day to 27 years (mean 6 years); the variables examined included morphology, size and function of the atrioventricular (AV) valves and ventricular septal defect and their relation to pulmonary stenosis, aortic stenosis and aortic arch obstruction. The visceroatrial situs was solitus and the heart was in the left side of the chest in all 57 patients. A d-loop ventricle was present in 21 patients and an l-loop ventricle in 36. The great arteries were normally related (Holmes heart) in 8 patients and transposed in 49. In all hearts, the right AV valve was anterior to the left AV valve. In 53 patients, the tricuspid valve (right valve in d-loop and left valve in l-loop) was closer to and had attachments on the septum. The tricuspid valve straddled the outflow chamber in eight patients. No significant difference was noted in the mean AV valve diameter when comparing mitral and tricuspid valves within the same group or between the groups with a d- or l-loop ventricle. The right AV valve diameter had a significant direct correlation with the aortic valve diameter and the size of the ventricular septal defect regardless of ventricular loop. Both AV valves were functionally normal in 34 patients. Among patients with AV valve dysfunction, the tricuspid valve tended to be stenotic in patients with an l-loop ventricle and regurgitant in patients with a d-loop ventricle. Mitral valve dysfunction was uncommon. The ventricular septal defect (46 patients) was separated from the semilunar valves in 24 patients (muscular defect) and adjacent to the anterior semilunar valve as a result of hypoplasia or malalignment, or both, of the infundibular septum (subaortic defect) in 19 patients. Multiple defects were present in three patients. The defect was unrestrictive in 26 patients, restrictive in 23 and could not be evaluated in 8. Pulmonary artery banding had been performed in 8 of the 26 patients with an unrestrictive defect and in 10 of the 23 patients with a restrictive defect. Only 4 of 19 subaortic defects compared with 16 of 24 muscular defects were restrictive. The size of the defect was significantly correlated with the measured pressure gradient. Among patients with transposition, only 2 of 13 with pulmonary stenosis had a restrictive ventricular septal defect compared with 15 of 30 without pulmonary stenosis. In patients with transposition, the defect size was significantly smaller when coarctation was present.     

Comparative value of transthoracic and transesophageal echocardiography in the assessment of congenital abnormalities of the atrioventricular junction

Information obtained from transthoracic and transesophageal echocardiography (two-dimensional echocardiography with spectral Doppler and color flow imaging) was compared in 17 patients with major congenital abnormalities of the atrioventricular (AV) junction (10 discordant AV connections, 1 criss-cross connection, 5 absent right connections and 1 absent left connection). The findings by either technique were correlated with findings at cardiac catheterization (12 patients) and at surgery (5 patients). In two of six patients with an absent AV connection as defined by transthoracic echocardiography, transesophageal imaging demonstrated an imperforate AV valve. In 11 of 11 patients with a discordant or criss-cross connection, assessment of AV valve and ventricular morphology (by defining the chordal attachments of both AV valves) was possible with transesophageal echocardiography (3 of 11 patients by transthoracic echocardiography); chordal straddling was detected in 1 patient and excluded in 3 others with an associated inlet ventricular septal defect. Anomalous pulmonary venous connection (one patient), atrial septal defect (three patients) and subpulmonary stenosis (five patients) were better assessed by transesophageal imaging, and atrial appendage morphology could be demonstrated in all. The transesophageal technique was less useful in demonstrating the anterior subaortic infundibulum or aortopulmonary shunt (two patients). Although systemic ventricular function could be assessed by either method with use of short-axis M-mode scans, transesophageal pulsed Doppler interrogation of AV valve and pulmonary venous flow patterns provided clues to diastolic dysfunction of the systemic ventricle.     

Left atrioventricular valve after surgical repair in atrioventricular septal defect with separate valve orifices ("ostium primum atrial septal defect"): an echo-Doppler study

Left atrioventricular (AV) valve dysfunction is the most frequent major postoperative hemodynamic complication in patients with AV septal defect. The anatomy and function of the left AV valve were investigated in 64 patients with separate valve orifices (ostium primum atrial septal defect) who had survived corrective surgery. M-mode and cross-sectional echocardiograms of the left AV valve were obtained. Doppler flow tracings were obtained at the left AV valve orifice to determine if regurgitation was present. The findings were related to the position of the commissures between the leaflets, the size of the 3 leaflets and the position of the papillary muscles. Left AV valve regurgitation was present in 29 of 51 patients (57%). These patients had a significantly different left AV valve leaflet configuration, characterized by a large mural leaflet and a small inferior bridging leaflet. The size of the superior bridging leaflet is not a determinant factor. Thus, the configuration of the left AV valve in AV septal defect is related to the postoperative functional result. Awareness of the echocardiographic anatomy may influence the surgical approach to this defect.     

Frequency and direction of interatrial shunting in valvular pulmonic stenosis with intact ventricular septum and without left ventricular inflow or outflow obstruction: An analysis of 127 patients treated by valvulotomy

This report summarizes observations in 127 patients who underwent pulmonic valvulotomy for valvular pulmonic stenosis with intact ventricular septum and without obstruction to left ventricular inflow or outflow. Of the 127 patients, 30 (24%) preoperatively by dye dilution curves had shunting at the atrial level: in 19 (63%), the shunt was right-to-left, and in the other 11 (27%), entirely left-to-right. The patients with right-to-left interatrial shunts had severe pulmonic valve stenosis (average peak systolic pressure gradient = 120 ± 11 mm. Hg) and small (average diameter 1.1 ± 0.1 cm.) sized defects in the atrial septum (patent foramen ovale). In contrast, the patients with left-to-right shunts had mild to moderate pulmonic valve stenosis (average peak systolic pressure gradient = 60 ± 5 mm. Hg) and relatively large (average diameter = 2.8 ± 0.1 cm.) defects in the atrial septum (true atrial septal defect). The patients with right-to-left interatrial shunts had no significant differences in right versus left atrial pressures. The patients with left-to-right interatrial shunts, however, had left atrial pressures significantly greater than right atrial pressures (7 ± 0.5 vs 5 ± 0.5, p < 0.05). No significant differences were found in ventricular end-diastolic pressures.     

Severe Right Ventricular Outflow Obstruction by Right Sinus of Valsalva Aneurysm

Aneurysms of the sinus of Valsalva are rarely diagnosed cardiac anomalies, occurring in 0.14%–0.96% of patients who have undergone open heart surgical procedures. The most common congenital anomalies accompanying sinus of Valsalva aneurysm (SVA) are ventricular septal defect, bicuspid aortic valve, atrial septal defect, and coarctation of aorta. We report a patient with an unruptured right SVA presenting with severe right ventricular outflow tract (RVOT) obstruction, and coexisting patent foramen ovale (PFO) with a right to left shunt. It could be assumed that the increase in right atrial pressure due to RVOT obstruction had led to a right to left shunt across the patent foramen ovale. (Echocardiography 2010;27:341‐343)     

Are routine preoperative cardiac catheterization and angiography necessary before repair of ostium primum atrial septal defect?

Two-dimensional and Doppler echocardiography were compared with cardiac catheterization and angiography in the preoperative evaluation of ostium primum atrial septal defect. Preoperative echocardiographic examinations as well as operative reports of all patients (33 patients aged 2 months to 23 years at surgery) with ostium primum atrial septal defect or transitional atrioventricular (AV) canal defect having had echocardiography and surgical repair at The Children's Hospital, Boston from July 1983 to January 1986 were retrospectively reviewed. Original cardiac catheterization and angiographic reports also were reviewed. Preoperative echocardiography resulted in no false positive or false negative primary diagnoses when compared with the diagnoses obtained at preoperative angiography or surgery. Doppler assessment of mitral regurgitation correlated well with angiographic (93% agreement) and intraoperative (85% agreement) assessments of mitral regurgitation to within two diagnostic categories on the six level scoring system used. There was reasonably good agreement between the two-dimensional echocardiographic estimate of right ventricular systolic pressure and that measured at catheterization when expressed as percent of the simultaneous left ventricular pressure. Seven of nine ventricular septal defects observed intraoperatively were noted on preoperative echocardiography; five of these defects were detected on preoperative angiography. A variety of other surgically confirmed associated cardiovascular defects were observed by both preoperative techniques. However, echocardiography appeared to be superior to angiography for evaluation of AV valve morphology and papillary muscle architecture. This study implies that in children with typical clinical and two-dimensional echocardiographic and Doppler findings for ostium primum atrial septal defect or transitional AV canal defect, routine preoperative cardiac catheterization and angiography are unnecessary.     

Appearance of Torrential Left to Right Intracardiac Shunt Following Erosion of Stenotic Pulmonary Valve

Summary: A 30-year-old patient had the complex of atrial septal defect, termination of left superior vena cava in left atrium and absence of coronary sinus together with ventricular septal defect and pulmonary valve stenosis. She was symptomatically well with balanced ventricular pressures and small bidirectional intracardiac shunt until erosion of the pulmonary valve occurred. This allowed large left to right shunt flow which induced intractable cardiac failure and led to her death.     

Abnormal right ventricular size and ventricular septal motion after atrial septal defect closure: etiology and functional significance.

Postoperative echocardiogram often demonstrate persistent right ventricular dilatation and paradoxic ventricular septal motion after repair of an atrial septal defect. To determine the prevalence, causes and significance of these echocardiographic abnormalities, 31 patients were studied with catheterization and echocardiography before and after repair of an atrial septal defect. Before operation, every patient manifested right ventricular dilatation, and all but one had abnormal septal motion. After operation, right ventricular dilatation was noted in 24 (77%) and abnormal septal motion in 21 (68%) patients despite the absence of residual left to right shunting in 30 (97%). These echocardiographic abnormalities could be correlated with age at operation and length of postoperative follow-up study but did not correlate with the degree of preoperative right ventricular enlargement or with shunt size or right ventricular pressure before or after operation. There was no associated functional deficit as demonstrated by the normal maximal oxygen consumption in all 13 patients who underwent treadmill exercise testing 5 to 38 months after operation; these patients included 9 with persistent right ventricular enlargement and abnormal septal motion.     

Usefulness of anatomic parameters derived from two-dimensional echocardiography for estimating magnitude of left to right shunt in patients with atrial septal defect

The ability of two-dimensional echocardiography (2DE) to quantitate the atrial septal defect size and left-to-right shunt magnitude was examined in 75 adult patients with simple ostium secundum atrial septal defect (ASD) with left-to-right shunts of 19-92% of systemic flow as determined by oximetry. The ASD was visualized in 71 of 75 (95%) patients utilizing subcostal 2DE, and the end-systolic atrial septal defect diameters in subcostal 2DE (ASDe) were measured. The maximal diameters of ASD measured during operation (ASDop) were obtained in 45 of these patients, who then underwent surgical ASD repair. The correlation between ASDe and ASDop was high (r = 0.91, p less than 0.001), indicating accuracy of quantitating defect size via subcostal 2DE approach. However, the correlation between the left-to-right shunt magnitude and ASDe was only fair (r = 0.76, p less than 0.01). In large ASDe the shunts varied greatly, while in small ASDe the shunts increased proportionally with increasing sizes of ASD. In addition, the ratio of left-to-right ventricular diameter (RVD/LVD) was determined. The RVD/LVD correlated relatively well with the shunt magnitudes (r = 0.83, p less than 0.001). Using the two new echocardiographic parameters of ASDe and RVD/LVD, a high percentage (85%) of patients with a large left-to-right shunt requiring surgical closure can be identified. All 43 patients with ASDe greater than 2.0 cm and RVD/LVD greater than 1.1 had a left-to-right shunt greater than 40%.(ABSTRACT TRUNCATED AT 250 WORDS)     

Echography in aneurysm of the interatrial septum

Atrial septal aneurysm can be detected by subcostal echocardiography as a bulge of the intermediate interatrial septum, ballooning toward the right atrium. We retrospectively revised 5412 echo examinations, consecutively performed in our laboratory, and we found 14 cases of atrial septal aneurysm (0.26%), mean age 36 +/- 15 years, 9 males and 5 females. In 7 patients atrial septal aneurysm was wide, including the whole atrial septum; in 5, only cranial two-thirds of the septum were involved and in 2, it regarded only the intermediate septum. No patients referred to arrhythmias, syncope, embolism, endocarditis or transient ischemic neurologic disorders. Cardiac abnormalities or defects were associated to atrial septal aneurysm in 12/14 patients: they consisted of atrial septal defect, mitral valve prolapse, false ventricular tendons or persistent Chiari network. Atrial left-to-right shunt was detected in all 6 cases with atrial communication. Considering each single associated cardiac abnormality, the prevalence of atrial septal aneurysm was 7% in patients with atrial septal defect, 1.7% in those with mitral valve prolapse, 6.6% in persistent Chiari network and 0.9% in false ventricular tendons. In conclusion, echocardiography is the first-choice technique to detect atrial septal aneurysm and other related cardiac defects.