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At least 19 recordsLinked to original sources
PubMed1998

[Aneurysm of the membranous ventricular septum with ventricular septal defect, mitral and tricuspid insufficiency].

A seventy-year-old man was admitted at our hospital because of dyspnea. Echocardiogram and left ventriculogram showed an aneurysm formation of the membranous ventricular septum and small left-to-right shunt through ventricular septum defect and also severe mitral and tricuspid insufficiency. Operation was performed after medical therapy for congestive heart failure. During operation, mitral leaflets showed no organic lesions nor prolapse, but the annulus was dilated. The cause of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused to produce tricuspid insufficiency secondary. The ventricular septal communication became small (diameter; 5 mm) and was associated with aneurysm formation of the remaining portion of the membranous septum. And the aneurysm, protruding to the septal leaflet of tricuspid valves, enhanced tricuspid insufficiency. It was reported by many authors that the aneurysm formation was related to spontaneous closure of ventricular septal defect. Patients with small ventricular septal defect, without any symptoms, must be followed intensively, or they might get cardiac complications, such as arrhythmia, right ventricular outflow obstruction, tricuspid insufficiency, and so on.

Aged↗
PubMed1985

Standardized nomenclature of the ventricular septum and ventricular septal defects, with applications for two-dimensional echocardiography.

The anatomy of the ventricular septum is reviewed, and ventricular septal defects are classified on the basis of their position and the presence or absence of septal malalignment. The standardized nomenclature for the various types of ventricular septal defects is presented, and analysis of these defects by two-dimensional echocardiography is described.

Echocardiography↗
PubMed1991

Surgical management of pulmonary atresia with intact ventricular septum. Right ventricular size as a guideline for surgical intervention.

61 infants with critical valvular pulmonary stenosis (21 cases) or pulmonary atresia with intact ventricular septum (40 cases) were operated on between 1975-1989 in the Semmelweis University Medical School. Right ventricular volume, area, outflow tract dimension and tricuspid anulus diameter was measured by angiocardiography and echocardiography to evaluate right ventricular size, and to predict the operative outcome using these data. Our results suggested, that if the normalized right ventricular volume was less than 3 ml/m2, the normalized right ventricular area was less than 2.5 cm2/m2, and the normalized right ventricular area was less than 2.5 cm2/m2 and the normalized tricuspid anulus diameter was less than 1.2 cm2/m2/3 only a systemopulmonary shunt procedure is needed. In all other cases pulmonary valvotomy is necessary to decompress the right ventric and to help increase the right ventricular size. Where a different part of the right ventricle is hypoplastic a systemopulmonary shunt procedure is needed too. In the follow up period the right ventricular dimension and tricuspid valve diameter was measured by echocardiography. This noninvasive assessment can predict the preoperative diagnosis, postoperative outcome and demonstrates an adequate growth of the right ventricle after pulmonary valvotomy.

Congenital Abnormalities↗
PubMed1990

Repair of transposition of the great arteries with intact ventricular septum and left ventricular outflow tract obstruction.

Repair of transposition of the great arteries in patients with intact ventricular septum and fixed left ventricular outflow tract obstruction has been restricted to atrial baffle procedures, with or without attempts to relieve or bypass the left ventricular outflow obstruction. However, the suboptimal results of these procedures, coupled with excellent functional results with the arterial switch operation in patients without obstruction, has made anatomic correction the goal in repairing these anomalies. We report a technique for the anatomic correction of transposition of the great arteries, intact ventricular septum, and fixed left ventricular outflow tract obstruction. Its consideration in these difficult cases is advocated.

Child↗
PubMed1983

Roles of the right ventricular free wall and ventricular septum in right ventricular performance and influence of the parietal pericardium during right ventricular failure in dogs.

The roles of the right ventricular (RV) free wall and ventricular septum in RV performance were studied in the canine heart. The parietal pericardium was kept intact. Acute ischemia of the RV free wall from right coronary ligation decreased the RV stroke work index more than did that of the ventricular septum from the septal branch of the left coronary artery ligation (41 and 23%, respectively, p less than 0.01). The response of the RV stroke work index to acute volume loading was also decreased. Left ventricular dysfunction was detected only with ventricular septal ischemia. Combined RV free wall and ventricular septal ischemia produced more severe and predominant RV dysfunction with disproportionate elevation of RV end-diastolic pressure. After combined ischemia, pericardiotomy improved the RV stroke work index as well as the left ventricular stroke work index (40 and 27%, respectively, p less than 0.05), although the increase in RV stroke work index was greater than in left ventricular stroke work index (p less than 0.05). The results of this study suggest that (1) the RV free wall has a more important role than the ventricular septum in RV performance, (2) predominant RV failure can be induced experimentally after combined RV free wall and ventricular septal ischemia, and (3) the pericardium has a restrictive effect on the damaged and dilated right ventricle.

Animals↗
PubMed1979

Complete transposition of the great arteries with intact ventricular septum and left ventricular outflow tract obstruction. Surgical management and anatomic considerations.

Surgical management of patients with complete transposition and intact ventricular septum may become difficult in the presence of left ventricular outflow tract obstruction. A Mustard operation and direct resection of the obstruction through the pulmonary artery has been the treatment of choice for this combination. Our study of the structure of the left ventricular outflow tract in four specimens with the anatomic findings of complete transposition, intact ventricular septum, and subpulmonary stenosis suggests that direct resection of the stenosis through the pulmonary artery can seldom be adequate without major risk of damaging either the mitral valve or the conduction tissue. An alternative procedure, namely, a combined Mustard operation and insertion of an external conduit from the left ventricle to the main pulmonary artery, has been employed in the management of six patients with this combination of lesions. One early and one late death occurred. Postoperative cardiac catheterization performed in all of the survivors before discharge from the hospital showed good relief of the stenosis and no significant gradient across the conduit.

Angiocardiography↗
PubMed1982

Left ventricular function and compliance in pulmonary atresia with intact ventricular septum.

Left ventricular systolic function and compliance were assessed in 15 cases of pulmonary atresia with intact ventricular septum. The left ventricular ejection fraction measured from cineangiography was used for the evaluation of the systolic function. The end-diastolic pressure (EDP), left ventricular compliance index at end-diastole (dv/dp), and left ventricular stiffness constant K were used as compliance indices. The mean left ventricular ejection fraction was slightly diminished. The right ventricular end diastolic pressures (RVEDPs) correlated significantly (r = 0.90) with the left ventricular end diastolic pressure (LVEDP). On the basis of left ventricular compliance, two distinctive groups of patients could be identified: Group I consisted of Patients 1 to 7, all with impaired left ventricular compliance. A shunt operation was the basic palliation in this group. None of these patients survived infancy. Group II consisted of Patients 8 to 15, all with normal left ventricular compliance. Pulmonary valvotomy as well as aortopulmonary shunts were the basic palliative procedures in this group. Six of eight patients in this group are long-term survivors. No difference was found in right ventricular size, tricuspid valve size, presence of tricuspid insufficiency, presence of a patent ductus arteriosus, postoperative oxygen saturations, and atrial pressure gradients between the two groups. Evaluation of the left ventricular compliance in patients with pulmonary atresia and intact ventricular septum might be of significant prognostic valve for their early survival.

Blood Pressure↗
PubMed1996

[A case of total cavopulmonary connection with preceding tricuspid valve closure in pulmonary atresia and intact ventricular septum, right ventricular-coronary artery fistulae, and coronary artery stenoses].

More than half of the patients with pulmonary atresia and intact ventricular septum (PA/IVS) are known to complicate right ventricular-coronary artery fistula (fistulae) and particularly those with coronary artery stenoses bear a high mortality rate and remain in a surgical challenge. A 4-year-old girl was first admitted to our hospital at 5 days of age and right ventriculogram revealed markedly hypoplastic tripartite ventricle and multiple fistulae through which both coronary arteries and aortic root were retrogradely opacified. Echocardiographically measured diameter of the tricuspid valve was 5 mm (Z value: 4). She underwent pulmonary valvotomy and central aorto-pulmonary (AP) shunt at 16 days of age. Repeat right ventriculogram at 1.9 years of age disclosed multiple stenoses in left anterior descending coronary artery (LAD) with proximal dilatation and tortuosity. Additional findings of left ventricular dysfunction (LVEF of 61.5%) and depressed ST segment in left precordial leads prompted us to proceed to the second palliation which comprised take-down of central AP shunt, bidirectional cavopulmonary shunt and closure of tricuspid and pulmonary valves. Catheterization at 3.4 years of age disclosed antegradely filled LAD with apparent relief of stenoses and improvement of LVEF to 68.9%. She underwent definitive repair of total cavopulmonary connection at 4.0 years of age and is leading a normal life 2 years after surgery. This experience draws us to conclude that tricuspid valve closure is a meaningful palliative procedure for PA/IVS with fistulae and coronary artery stenoses, provided that proximal segments of both coronary arteries remain intact.

Child, Preschool↗
PubMed2004

Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure.

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.

Electrocardiography↗
PubMed1992

Changes of right ventricular size and function in neonates after valvotomy for pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Right ventricular end-diastolic and stroke volumes were calculated from orthogonal subcostal echocardiographic images in 24 neonates (mean weight +/- SD 3.4 +/- 0.4 kg) with pulmonary atresia (n = 18) or critical pulmonary stenosis (n = 6) and intact ventricular septum before and at an average of 5 days and then 19 days after pulmonary valvotomy. The preoperative echocardiographic volume determinations were compared with the respective angiographic determinations. In addition, the endocardial area outlines of the left and right ventricles were obtained by planimetry from an end-diastolic frame taken in the apical four-chamber view. End-diastolic and stroke volumes calculated by the echocardiographic method (y) correlated closely with those calculated by the angiographic method (x); the regression equations were y = 1.02 x -0.13 (r = 0.95, SEE +/- 0.45 ml) and y = 1.16 x -0.15 (r = 0.89, SEE +/- 0.38 ml), respectively. All except one infant had right ventricular hypoplasia before valvotomy with an end-diastolic volume of 16.6 +/- 6.4 ml/m2 (44.5 +/- 17.3% of normal). Right to left ventricular area ratio was 0.56 +/- 0.09 (normal 0.95). Five days after valvotomy, right ventricular end-diastolic volume decreased to 10.6 +/- 4.6 ml/m2 (p less than 0.05) and stroke volume decreased from 8.3 +/- 3.5 to 5.5 +/- 2.8 ml/m2 (p less than 0.05). Nineteen days after valvotomy, right ventricular end-diastolic volume and right to left ventricular area ratio had increased to their respective preoperative values; right ventricular stroke volume had increased further to 10.4 +/- 3.9 ml/m2 (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed2003

Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology.

OBJECTIVES: Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle-tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure. METHODS: Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10. RESULTS: There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years. CONCLUSIONS: We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.

Cardiac Catheterization↗
PubMed1993

Pulmonary atresia with intact ventricular septum. Surgical approach based on ventricular size and coronary anatomy.

Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality. This may relate to the nonuniformity of the anomaly. We have developed a management algorithm based on the right ventricular size and coronary anatomy. Patients with a well-developed ventricle and normal coronary arteries have undergone right ventricular outflow procedures. The adequacy of their right ventricles is subsequently evaluated; some patients are candidates for a four-chamber repair, whereas others are candidates for a Fontan procedure. Patients with severe hypoplasia of the right ventricle frequently have extensive ventriculo-coronary connections, and for these patients we have developed the technique of right ventricular obliteration. Over the past 7 years, we have treated 20 patients with pulmonary atresia with intact ventricular septum. Fourteen of the 20 patients underwent outflow tract procedures, with no operative mortality. There were two late deaths in this group. Six patients had hypoplasia of the ventricle with ventriculo-coronary connections and underwent right ventricular obliteration. There was one operative death and one late death in the group. Overall, 95% survived the neonatal period, and 80% were still alive at the time this article was written with an average follow-up of 32 months. We conclude that pulmonary atresia with intact ventricular septum can be successfully managed with the use of an algorithm based on ventricular size and coronary anatomy.

Cardiac Surgical Procedures↗
PubMed1980

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 less than 0.05). No significant differences were found in ventricular end-diastolic pressures.

Adolescent↗
PubMed1995

[Surgical treatment of transposition of the great arteries with intact ventricular septum associated with left ventricular outflow obstruction].

Thirteen patients have undergone surgical treatment for transposition of the great arteries (TGA) with intact ventricular septum (IVS) associated with left ventricular outflow obstruction (LVOTO) in our institute. Ages at operation ranged from three months to six years (mean 9.2 +/- 4.7 months). Of these patients, seven had dynamic type LVOTO (group I), and six had organic LVOTO (group II). Preoperative left ventricular end-diastolic volume was significantly smaller in group II (78 +/- 13% of normal) than control group (135 +/- 53% of normal). Preoperative pressure gradient between the pulmonary artery and left ventricle was significantly greater in group II (55.8 +/- 3.2 mmHg) than group I (35.2 +/- 3.9 mmHg). In group I, II patients underwent two-stage ASO, the other 5 patients underwent Senning operation. The reason for the Senning operation were era before introduction of ASO in our institute (1983) or unsuccessful training of the left ventricle. In group II, all but one patient underwent Senning operation, the other underwent a successful Fontan operation with Damus anastomosis because of too small left ventricle (LVEDV: 49% of normal). Techniques to correct LVOTO at the definitive operation included ventriculotomy (n = 4) and pulmonary valvotomy (n = 2). One patients in group II underwent a Fontan operation with Damus anastomosis due to an underdeveloped left ventricle (LVEDV: 49% of normal). There was no early or late death. The postoperative pressure gradients disappeared or reduced to trivial levels in all patients. At present no LVOTO has developed in any of the patients.

Cardiac Surgical Procedures↗
PubMed2000

Pulmonary atresia with intact ventricular septum, antegrade coronary-right ventricular sinusoidal communication, and Wolff-Parkinson-White syndrome.

A fenestrated Fontan operation was performed successfully in a patient with pulmonary atresia with intact ventricular septum, an antegrade sinusoidal communication, and Wolff-Parkinson-White syndrome. Unlike most cases, blood flow in the sinusoidal communication was antegrade, from the left anterior descending artery to the right ventricle. This is the first report of the combination of pulmonary atresia with intact ventricular septum and Wolff-Parkinson-White syndrome.

Child, Preschool↗