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PubMed1979

Nongenetically transmitted disproportionate ventricular septal thickening associated with left ventricular outflow obstruction.

Clinical, haemodynamic, and morphological features are described in 2 patients with disproportionate ventricular septal thickening, left ventricular outflow obstruction with systolic anterior motion of the anterior mitral leaflet, and either acquired or congenital heart disease. The disproportionate septal thickening in these patients appeared to be secondary to their underlying cardiac disease rather than a manifestation of genetically transmitted hypertrophic cardiomyopathy. One patient with combined aortic and mitral stenosis had severe systolic anterior motion of the anterior mitral leaflet and a residual large systolic pressure gradient between left ventricle and systemic artery after aortic valve replacement. In this patient the systolic anterior motion was evident in the presence of mitral valve stenosis. The other patient with mild aortic stenosis and a previously repaired coarctation of the aorta also had mild systolic anterior motion and a small subaortic systolic pressure gradient. Hence, these 2 patients demonstrate that disproportionate septal thickening secondary to acquired or congenital heart disease may be associated with left ventricular outflow obstruction and systolic anterior motion of the anterior mitral leaflet.

Adolescent↗

PubMed1980

Accessory flaplike tissue causing ventricular outflow obstruction.

In three cases, two in children and one in a young adult, ventricular outflow obstruction was caused by a valvelike flap of accessory endocardial tissue. The angiocardiographic feature was that of a narrow radiolucent, crescent-shaped or linear filling defect corresponding to the site of obstruction. Resection of the accessory tissue was performed in each case at the time of correction of associated conditions. In one case (a woman 20 years of age) the obstructing membrane was in the outflow tract of the right ventricle, and a ventricular septal defect was associated. In each of the other two cases the outflow tract of the left ventricle was the site of obstruction. In one (a 14-month-old boy), an ostium primum type of atrial septal defect and cleft mitral valve were associated; in the remaining case (a 7-year-old boy) complete transposition and ventricular septal defect were also present. In the latter case a Mustard procedure was performed and was followed by death. Successful results were obtained in the first two patients.

Adult↗

PubMed1990

Use of Doppler echocardiography and amyl nitrite inhalation to characterize left ventricular outflow obstruction in hypertrophic cardiomyopathy.

The presence of left ventricular outflow tract obstruction (LVOTO) of either a resting or dynamic nature may have important therapeutic and prognostic implications in patients with hypertrophic cardiomyopathy (HCM). Doppler echocardiograms combined with amyl nitrite (Amyl) inhalation were performed in 333 consecutive patients referred for suspected HCM to diagnose and categorize the nature and severity of LVOTO. Hypertrophic cardiomyopathy was present by 2-D and M-mode criteria in 145/333 (44 percent) patients. Normal limits of resting and post-Amyl continuous wave Doppler peak left ventricular outflow tract velocities were established in 15 subjects with completely normal 2-D and Doppler echocardiograms. Based on these criteria, of the 145 patients with HCM, 63 (43 percent) were classified as having resting LVOTO, peak velocity 4.2 +/- 1.3 m/s. Among 82 patients with HCM without resting LVOTO, 47 (57 percent) received Amyl. Latent LVOTO was provoked in 25/47 (53 percent), peak post-Amyl velocity 4.5 +/- 1.2 m/s. The remaining 22 (47 percent) had nonobstructive HCM, as indicated by no significant increase in post-Amyl velocity. Among a total 62 subjects receiving Amyl, none experienced serious morbidity or mortality. Doppler echocardiography, in conjunction with Amyl inhalation in selected patients, is a useful noninvasive method to diagnose and categorize patients with HCM according to the nature and severity of LVOTO.

Aged↗

PubMed1981

Right ventricular outflow obstruction from thrombus with small peripheral pulmonary emboli.

A patient is described with clinical features of massive pulmonary embolism and a normal pulmonary angiogram. At necropsy, a large thrombus obstructed right ventricular outflow. A right atrial or right ventricular angiogram is suggested in patients suspected of having pulmonary embolism when a pulmonary angiogram shows the pulmonary trunk and main right and left pulmonary arteries to be normal.

Adult↗

PubMed1998

Usefulness of preoperative echocardiography in predicting left ventricular outflow obstruction after primary repair of interrupted aortic arch with ventricular septal defect.

Residual left ventricular outflow tract (LVOT) obstruction is a significant problem after repair of interrupted aortic arch (IAA) and ventricular septal defect. Resection of subaortic tissue at the time of primary repair, however, is associated with increased morbidity and mortality. We reviewed the preoperative echocardiograms and the postoperative clinical course and echocardiograms of 23 consecutive patients who underwent primary repair of IAA without widening of the subaortic region. Nine patients (39%) developed significant LVOT obstruction (pressure gradient >40 mm Hg). LVOT obstruction was noted postoperatively in 7 of 9 patients by 1 month, 8 of 9 by 2 months, and 9 of 9 by 1 year. On retrospective analysis of the preoperative echocardiograms, the indexed cross-sectional area of the LVOT, the subaortic diameter index, and the subaortic diameter Z score were all significantly smaller in those requiring reintervention (p <0.04, p <0.05, p <0.05, respectively). Of these, indexed cross-sectional area had the least reproducibility and subaortic diameter index the most (coefficient of variation of 26.3% vs 11.2%). In conclusion, most patients who develop significant LVOT obstruction after repair of IAA do so within 1 month of operation. Although subaortic indexed cross-sectional area is the most sensitive predictor of LVOT obstruction after primary repair of IAA, other more simple standardized measurements of the subaortic diameter were comparably predictive and had better reproducibility.

Adolescent↗

PubMed1977

Coexisting right and left hypertrophic subvalvular stenosis and fixed left ventricular outflow obstruction due to aortic valve stenosis.

A case of fixed left ventricular outflow tract obstruction due to aortic valve stenosis coexisting with right- and left-sided subvalvular hypertrophic stenosis is documented with hemodynamic data, angiograms, echocardiograms and findings at surgery. Histologic examination of the septal muscle with light and electron microscopy revealed hypertrophy of the muscle but none of the characteristics of idiopathic hypertrophic subaortic stenosis. Septal hypertrophy with subvalvular obstruction can occur secondary to left ventricular pressure overload due to fixed left ventricular outflow tract obstruction and is not always the chance occurrence of two separate diseases.

Aged↗

PubMed1991

[Right ventricular outflow obstruction due to accessory tricuspid valve tissue in corrected transposition of the great arteries with ventricular septal defect].

A 27-year-old man known to have corrected transposition of the great arteries (CTGA) associated with a ventricular septal defect (VSD) was admitted to our hospital with a suspected increase of the shunt volume. The patient is a first class bodybuilder. Heart catheterization confirmed the diagnosis of a "right-ventricular" mass obstructing the "right-ventricular" outflow tract, which had already been seen at echocardiography. The intraoperative finding showed the tumor to be accessory tissue of the anterior tricuspid leaflet prolapsing through the VSD into the subvalvular outflow tract of the right ventricle. The accessory tricuspid valve tissue was removed by transatrial access. The VSD was closed by a dacron patch using the same route. The postoperative course was uneventful.

Adult↗

PubMedDate not supplied

Relationship between mitral regurgitation and left ventricular outflow obstruction in hypertrophic cardiomyopathy.

To clarify the relationship between mitral regurgitation and left ventricular outflow obstruction, Doppler and two-dimensional echocardiographic studies were performed in 62 patients with hypertrophic cardiomyopathy (22 with and 40 without obstruction caused by mitral systolic anterior motion with septal contact). Pulsed Doppler echocardiography with color Doppler flow imaging demostrated that in 20 of the 22 patients with obstruction, mitral regurgitation occurred mainly during midsystole from the onset to the end of mitral-septal contact. Such midsystolic mitral regurgitation was not observed in patients without obstruction, except in three of 25 patients with mild mitral systolic anterior motion without septal contact. Furthermore, that regurgitation developed or disappeared together with the obstruction during follow-up periods or pharmacologic interventions. Two-dimensional echocardiography showed that in 21 of the 22 patients with obstruction, a distal residual portion of the "anterior" mitral leaflet moved anteriorly in early systole and protruded into the outflow tract during midsystole to cause the obstruction. In the other patient with obstruction, who had only early systolic mitral regurgitation, a distal residual "posterior" leaflet moved similary. These results may indicate that the midsystolic mitral regurgitation is hydrodynamically induced by the midsystolic pressure gradient across the protruding distal residual anterior mitral leaflet.

Adult↗

PubMed1975

The association of fixed and dynamic left ventricular outflow obstruction.

Twelve patients were investigated echocardiographically and angiographically and were shown to have severe fixed obstruction to the left ventricular outflow tract. Eight had valvular stenosis, and four had discrete subvalvular membranes. Two of the patients had additional dynamic obstruction of the left ventricular outflow tract. This was recognized preoperatively by echocardiography because of abnormal systolic motion of the mitral leaflet. At the time of definitive surgery for relief of the fixed obstruction, the additional dynamic obstruction was identified and treated, since persistent residual obstruction may lead to death in the immediate postoperative period or to long-term symptoms. The dynamic left ventricular outflow obstruction is probably a result of the hypertrophy produced by the fixed obstruction.

Adolescent↗

PubMed1985

[Marked right ventricular outflow obstruction in two children with hypertrophic cardiomyopathy].

Two children, aged one year and nine months, and six years, both with hypertrophic cardiomyopathy, presented as severe right ventricular outflow obstruction were presented. Initially, both of them were suspected of having heart disease because of precordial systolic murmurs. Their tentative diagnosis was pulmonary stenosis. Their apex cardiograms and carotid pulse waves were not diagnostic. Asymmetrical septal hypertrophy with systolic anterior motion of the mitral valve was identified by echocardiography in both cases. Cardiac catheterization revealed left ventricular outflow tract pressure gradients of 5 mmHg and 30 mmHg, respectively. The right ventricular peak systolic gradient was 100 mmHg and 82 mmHg and left ventricular end-diastolic pressure was 26 mmHg and 18 mmHg, respectively. Selective right and left ventricular angiocardiograms demonstrated severe right ventricular outflow obstruction, and hypertrophy of the interventricular septum. One of them underwent resection of the right ventricular outflow tract muscle, which showed histological findings characteristic of hypertrophic cardiomyopathy. This patient died suddenly at the age of 10 years. Infants or young children with hypertrophic cardiomyopathy often have associated predominant right ventricular outflow tract obstruction, progressive congestive heart failure, and death. Therefore, correct diagnosis and careful treatment are mandatory for proper management.

Cardiac Output↗

PubMed2005

Subvalvular left ventricular outflow obstruction for patients undergoing aortic valve replacement for aortic stenosis: echocardiographic recognition and identification of patients at risk.

Persistently high gradients after aortic valve replacement (AVR), potentially caused by prosthesis-patient mismatch or superimposed but unrecognized nonvalvular obstruction, are associated with adverse clinical outcomes. Concomitant valvular and subvalvular left ventricular outflow obstruction was first hypothesized in 1957, and identified and further characterized in the 1960s, before the availability of echocardiography. Although obstruction as a result of subvalvular hypertrophy complicating valvular aortic stenosis has been subsequently described using echocardiography, it has largely fallen from consciousness in the preoperative and intraoperative echocardiographic assessment of patients undergoing AVR for aortic stenosis. As such, subvalvular left ventricular outflow obstruction complicating valvular aortic stenosis is a potentially preventable cause of persistently high gradients that remains relatively frequently encountered after AVR. This review is intended to draw attention to this phenomenon, to describe its mechanisms, and to provide guidance for its preoperative or intraoperative recognition using echocardiographic imaging techniques, with the goal that recognition and appropriate intervention at the time of AVR will decrease its clinical impact.

Aortic Stenosis, Subvalvular↗

PubMed1983

Diagnosis of right ventricular outflow obstruction in infants by cross sectional echocardiography.

Cross sectional echocardiographic studies were assessed prospectively in 58 infants in whom right ventricular outflow obstruction was subsequently shown angiographically. A subcostal cut was used to display simultaneously the short axis of the aortic root and the long axis of the right ventricular outflow tract. This facilitated the differentiation of the common right ventricular outflow tract obstructive lesions. Tetralogy of Fallot was diagnosed correctly in 22 of 26 infants; pulmonary atresia with intact septum in all of 14 neonates; isolated severe pulmonary valve stenosis in all of nine infants; and pulmonary atresia with ventricular septal defect in eight of nine infants. The subcostal approach is the technique of choice for evaluating right ventricular outflow tract obstruction as it is more reliable than the left parasternal approach.

Coronary Circulation↗

PubMed1998

Primary cardiac leiomyosarcoma growing rapidly and causing right ventricular outflow obstruction.

Leiomyosarcomas are extremely rare primary cardiac tumors. We report a rapidly growing primary leiomyosarcoma of the right ventricle, which obstructed the right ventricular outflow tract within one month after symptom onset in a 68-year-old man. Two-dimensional echocardiography was useful in diagnosing the extent and progression of the tumor. The tumor was surgically resected on an emergency basis, and the right ventricle and pulmonary artery were successfully reconstructed. Recurrence of the tumor on the right ventricle was observed, and the patient was overcome by sudden dyspnea and died three months after surgery.

Aged↗

PubMed1975

Relief of sever left ventricular outflow obstruction in a case of hypertrophic obstructive cardiomyopathy treated with practolol.

The clinical and haemodynamic findings in a patient with hypertrophic obstructive cardiomyopathy and severe left ventricular outflow obstruction are presented. Treatment with an increasing dose of orally administered practolol up to a maximum of 1200 mg a day, resulted in symptomatic improvement, and abolition of the resting gradient when the patient was recatheterized six months later.

Administration, Oral↗

PubMed2002

Midterm results of surgical treatment of systemic ventricular outflow obstruction in Fontan patients.

BACKGROUND: Achieving unobstructed blood flow from the systemic ventricle to the aorta is important during the Fontan procedure for complex cyanotic congenital heart disease when there is systemic ventricular outflow obstruction (SVOO). Because SVOO can progress after the Fontan procedure if there is morphologic obstruction, we have adopted a policy of relieving obstructions to systemic blood flow. METHODS: Twenty-five patients were treated by the Fontan procedure with SVOO. Twenty-one patients had undergone prior pulmonary artery banding and 10 patients had undergone prior arch repair. Systemic ventricular outflow obstruction progressed in 5 patients after the Fontan procedure. Main diagnosis was single ventricle in 12, tricuspid atresia in 5, transposition of the great arteries in 4, double-outlet right ventricle in 3, and common atrioventricular canal in 1. Mean age at operation was 6.5 years (range 1 to 15 years) and the average preoperative pressure gradient across the ascending aorta and systemic ventricle was 29 mm Hg (range 0 to 100 mm Hg). The Damus-Kaye-Stansel procedure was performed in 18 patients (double-barrel anastomosis in 13, end to side anastomosis in 5), and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in 7. Double-barrel anastomosis has been our first choice since 1994, if the pulmonary valve is intact. Follow-up has ranged from 4 months to 14 years (average 5.0 years). Twenty-three of the 25 patients have undergone recatheterization (average 21.4 months later). RESULTS: No early deaths were found; one late death was reported of a patient with single right ventricle (4.0%). The postoperative average pressure gradient was 1.1 mm Hg (0 to 10 mm Hg), and the average right atrial pressure was 14 mm Hg (9 to 20 mm Hg). In all patients who underwent ventricular septal defect or bulboventricular foramen enlargement, regular sinus rhythm was maintained postoperatively. Regarding the Damus-Kaye-Stansel procedure, there was minimal progression of semilunar valve insufficiency except in 1 patient who underwent end-to-side anastomosis with moderate pulmonary regurgitation postoperatively. CONCLUSIONS: The midterm results of the Fontan procedure with SVOO have been satisfactory. Because SVOO might progress after the Fontan procedure if there is morphologic obstruction, an appropriate strategy to relieve obstruction to systemic blood flow should therefore be performed concomitantly with the Fontan procedure.

Adolescent↗

PubMed1984

Apicoaortic valved conduits for complex left ventricular outflow obstruction: technical considerations and current status.

The surgical considerations, clinical follow-up, and hemodynamic data for 23 patients (age range, 4 months to 80 years) who underwent insertion of a porcinevalved apicoaortic conduit between December, 1976, and June, 1983, are reviewed. All patients had complex forms of left ventricular outflow obstruction. All were symptomatic, and 18 had had 23 prior attempts at surgical relief of the obstruction. There was a total of 6 deaths (26%); 2 were in small children with other complex cardiac malformations, and 2 others in patients who required emergency operation for acute cardiac decompensation. Sixteen patients have had cardiac catheterization 1 to 1.5 years postoperatively, and a reduction in the resting left ventricular-aortic gradient from 91 +/- 30 to 13 +/- 8 mm Hg was demonstrated (p less than 0.001). None of the patients is taking anticoagulants, and no thromboembolic events have occurred. Four of the 17 survivors have undergone a subsequent operation 1.5 to 4.0 years postoperatively with conduit removal, aortoventriculoplasty, and conduit valve replacement in 1, 1, and 2 patients, respectively. These data demonstrate that the apicoaortic conduit is effective in relieving complex left ventricular outflow obstruction and improving left ventricular performance with acceptable long-term results. Increasing the durability of the conduit valve will greatly improve the late results with this technique.

Adolescent↗

PubMedDate not supplied

Angiographic evidence of absent ductus arteriosus in severe right ventricular outflow obstruction.

The angiocardiograms of 5 newborn infants with autopsy and/or surgically-proven congenital absence of the ductus arteriosus (ADA) and right ventricular outflow obstruction (Group A), and of 14 neonates with pulmonary atresia complex and patent ductus arteriosus (Group B) were reviewed. Aortic size was similar in both groups; however, the diameters of the right and left pulmonary arteries were much smaller in Group A than in Group B (right pulmonary artery: 2.6 vs 4.5 mm, P less than 0.005; left pulmonary artery: 2.5 vs 4.3 mm, P less than 0.005). Extensive bronchial collaterals were observed in Group A but not in Group B. Tricuspid aortic valve stenosis was present in 2 patients in Group A but in none in Group B. The diagnosis of ADA may be made in newborn infants with severe right ventricular outflow obstruction if the angiocardiograms reveal hypoplasia of the pulmonary arteries, extensive bronchial collaterals, and nonvisualization of the ductus arteriosus. Other suggestive features include aortic valve stenosis and/or right aortic arch with aberrant left subclavian artery.

Angiocardiography↗

PubMed1992

[Closure of ventricular septal defect of interrupted aortic arch with left ventricular outflow obstruction].

Leftward shift of the infundibular septum (IS) in interrupted aortic arch (IAA) with ventricular septal defect (VSD) often develops significant left ventricular outflow obstruction (LVOTO). Seven-day-old boy with 2.6 kg body weight underwent the two-stage operation for this anomaly. The aortic arch was interrupted between the left common carotid and the left subclavian artery. At the first stage, a 5 mm GORE-TEX graft was used to connect the interrupted arch, and pulmonary artery banding was performed. In closure of VSD at the second stage, IS was penetrated by stitches for the VSD patch to left ventricular outflow tract. IS with leftward shift could be pulled toward right ventricular side with patch fixation and LVOTO was prevented by this method.

Abnormalities, Multiple↗