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[Mid-systolic ejection murmur with thrill caused by right ventricular outflow tract obstruction secondary to septal aneurysm following myocardial infarction: a case report].

A 71-year-old woman with a history of previous myocardial infarction was transferred to our hospital for evaluation of chest pain and ventricular tachycardia. On admission, a loud mid-systolic ejection murmur accompanied by a thrill was found at the left sternal border in the third intercostal space, and it was significantly accentuated in the post-extrasystolic beat. Abnormal Q waves and ST elevations were noted in leads I, aVL and V5,6 on electrocardiograms. Echocardiograms, confirmed a septal-to-apical aneurysm, and a thin interventricular septum (IVS) with paradoxical motion. Right ventricular (RV) catheterization showed a pressure gradient of 21 mmHg between the outflow tract (RVOT) and the apex, and a mid-systolic ejection murmur was recorded in the RVOT on an intracardiac phonocardiogram. Coronary arteriograms revealed total occlusion of the left anterior descending artery in its proximal portion, and a 90% stenosis of the circumflex artery. A left ventriculogram demonstrated a septal-to-apical aneurysm with a markedly reduced ejection fraction of 0.16. A right ventriculogram showed obstruction to RVOT caused by systolic ballooning of the IVS. In this patient, the mid-systolic ejection murmur was probably caused by the obstruction of the outflow tract secondary to septal aneurysm following old myocardial infarction.

Aged↗

Identification of two subtypes of infantile acid maltase deficiency.

Infantile patients with acid maltase deficiency have severe hypertrophic cardiomyopathy, left ventricular outflow obstruction, and generalized muscle weakness and die before 1 year of age. We identified 12 infants with acid maltase deficiency who had a similar clinical presentation but less severe cardiomyopathy and absence of left ventricular outflow obstruction, and 9 of 12 had longer survival with assisted ventilation and supplemental intubation.

Age of Onset↗

Hemodynamic function after the Mustard operation for transposition of the great arteries.

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The post-operative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6%), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.

Adolescent↗

Surgical correction of subaortic stenosis via right ventriculotomy and septal resection in a dog.

After 2 years of medical management with a beta-adrenoreceptor blocking agent, a 30-month-old castrated male Golden Retriever with subaortic stenosis was treated surgically because of progression of its condition. In an attempt to achieve complete relief of the left ventricular outflow obstruction, a modified Konno procedure consisting of right ventriculotomy and septal myectomy from the infundibular portion of the right ventricle was performed; this combination of procedures allowed wide resection of the septal portion of the left ventricular outflow obstruction. Two years after surgery, the mass of the dog's left ventricle had decreased and the peak calculated pressure gradient across the aortic valve had decreased to 40 mm Hg, compared with a preoperative value of 240 mm Hg; at that evaluation, the dog had gained weight and was able to play normally. It is suggested that use of this modified approach to the outflow tract may have a positive effect on long-term survival time in dogs with subaortic stenosis.

Animals↗

Detection of mechanisms of immediate failure by transesophageal echocardiography in quadrangular resection mitral valve repair technique for severe mitral regurgitation.

Residual mitral regurgitation (MR) after repair is a risk factor for late reoperation. The use of intraoperative transesophageal echocardiography (IOTEE) decreases the incidence of immediate repair failure. This study identifies the mechanisms of immediate failure by IOTEE in the quadrangular resection technique, a well-standardized mitral valve repair procedure to guide further repair procedures. Two hundred five consecutive patients underwent quadrangular resection due to prolapse or flail posterior leaflet. Twenty-four patients (11%) had immediate failure. Immediate reinstitution of cardiopulmonary bypass ("second pump run") was needed in 21 patients (10%) for further repair. The identified mechanisms of failure were residual cleft provoking interscallop malcoaptation into the posterior leaflet in 8 patients, residual prolapse of the anterior or posterior leaflets in 1 and 4 patients, respectively, residual annular dilation in 3, left ventricular outflow obstruction in 2, suture dehiscence in 2, and other mechanisms in another 2 patients. In 20 patients (95%), IOTEE guided further repair with resolution of the residual MR, whereas 1 patient underwent valve replacement due to pharmacologically untreatable left ventricular outflow obstruction. In conclusion, even if this type of valve repair technique is well standardized, the incidence of immediate failure is not negligible. IOTEE identified the mechanisms of the immediate failure and guided further repair procedures, thus reducing the incidence of valve replacement (0.5%) without increasing perioperative mortality and morbility.

Adult↗

Hemangioma of the right ventricle causing outflow tract obstruction.

A 3 1/2-year-old child had a murmur of pulmonary stenosis. Echocardiography and cardiac catheterization revealed a pulmonary infundibular obstruction. Magnetic resonance imaging of the heart demonstrated a mass in the interventricular septum. The mass was successfully resected and a pathologic diagnosis of capillary hemangioma was made. Only two previous cases of hemangioma causing right ventricular outflow obstruction have been reported; both of these cases involved adults. This case represents the first report of a hemangioma causing right ventricular outflow tract obstruction in a child. An exploratory operation with resection is the treatment of choice.

Child, Preschool↗

Auscultatory features of hypertrophic obstructive cardiomyopathy. A study of 90 patients.

The auscultatory signs in 90 subjects with hypertrophic obstructive cardiomyopathy are described. The late-onset ejection systolic murmur and its responses to vaso-active manoeuvres reflect a volume-dependent outflow tract obstruction. Late vibrations of the systolic murmur, not uncommonly recorded at the apex, are due to associated mitral incompetence. Non-ejection systolic clicks may occur, and the likely explanation is inequality of the functional length of the mitral chordae tendineae secondary to asymmetrical myocardial hypertrophy. The second heart sound is often abnormal, usually with delay in the aortic component. Some correlation was demonstrated between the relative degrees of left and right ventricular outflow obstruction and the pattern of splitting of the second heart sound. Reversed or partially reversed splitting is usually associated with a more severe left ventricular outflow obstruction. Ejection systolic clicks and early diastolic murmurs occur infrequently, but are not incompatible with the diagnosis of hypertrophic obstructive cardiomyopathy.

Adolescent↗

Interrupted aortic arch--anatomical features of surgical significance.

Analysis of 26 autopsy specimens with interruption of the aortic arch has led to a clearer understanding of the vital importance of associated cardiovascular abnormalities. In all cases there was an associated patent ductus arteriosus "supporting" the distal systemic circulation. "Proximal" septal defects, 22 ventricular septal defects and 3 aorto-pulmonary windows, were found in all but one specimen. The infundibular component of the ventricular septum was displaced posteriorly and leftward in 9 hearts resulting in significant left ventricular outflow obstruction in 8. In one specimen the infundibular septum was displaced anteriorly giving the effect of a "Fallot type" obstruction to the right ventricular outflow tract. Abnormal ventriculo-arterial connections were found in 7 hearts; 2 with discordant connection, 2 with double outlet, and 3 with single outlet of the heart. In 6 specimens there was an aberrant origin of the right subclavian from the distal aorta passing behind the esophagus to the right side. Various other arterial abnormalities were identified including a truncus arteriosus with separate but "transposed" origins of the pulmonary arteries. In short, in these hearts there were no examples of the rare situation where aortic arch interruption exists in isolation. This study therefore makes it very clear that a detailed understanding of associated anatomical abnormalities must accompany any attempt at surgical correction. Certainly in many of these cases management of the associated lesion will present more of a challenge than the difficult problem of dealing with the interrupted arch itself.

Abnormalities, Multiple↗

Unlocking the mystery of systolic anterior motion: the key is timing.

Though a generation has elapsed since the recognition of hypertrophic cardiomyopathy, controversy continues to rage regarding its pathophysiology. Central to the controversy is the mechanism and significance of the pressure gradient. Systolic anterior motion (SAM) of the mitral valve, as demonstrated by angiography and echocardiography, is accepted as the cause of the pressure gradient by some groups but not others. The latter authors suggest that: SAM is an incidental effect of vigorous contraction which is itself the cause of the pressure gradient and that the pressure gradient does not represent left ventricular outflow obstruction. These views are examined in light of recent studies that have assessed the temporal and quantitative relationship between SAM and the pressure gradient. It is concluded that SAM with early mitral septal contact is the cause of the pressure gradient, and represents true left ventricular outflow obstruction.

Blood Flow Velocity↗

Severe right ventricular outflow tract obstruction caused by an intracavitary cardiac neurilemoma: succesful surgical removal and postoperative diagnosis.

A 32-year-old woman with a one-year history of progressive shortness of breath and chest pain was found to have a grade 4/6 systolic murmur at the base of the heart and left sternal border. Right ventricular enlargement was found by physical examination, ECG, and chest roentgenogram. Cardiac catheterization showed elevated right ventricular pressure, an intracavitary pressure gradient, and inability to enter the pulmonary artery. Angiography revealed a mass in the right ventricular outflow tract. Successful surgical removal of a large, well-encapsulated tumor mass was accomplished, and the tumor was interpreted as a benign neurilemoma. Postoperatively, the patient improved remarkably.

Adult↗

Development of the ductus arteriosus in right ventricular outflow tract obstruction.

We studied the morphology of the ductus arteriosus in 14 infants, ages 2--90 days. Eight (group 1) had pulmonary atresia (structural and functional) with an intact interventricular septum; six (group 2) had pulmonary atresia with a ventricular septal defect. The inferior angle of the ductus arteriosus at the aortic junction was measured in each patient. In group 1, this angle was obtuse in all but one patient. In group 2, the angle was acute in all. Further study of intracardiac anatomy suggested that in group 1, the obtuse inferior angle of the ductus arteriosus was the result of a late and progressive obstructive phenomenon that allowed normal right-to-left flow through the ductus arteriosus during much of fetal life. In group 2, the direction of ductus arteriosus flow (normally from the pulmonary trunk to the aorta) was reversed, and flowed from the aorta to the pulmonary trunk. This reversal of flow was probably of early onset in the fetus, the aorta receiving the total combined ventricular output, and produced a small ductus arteriosus with an acute inferior angle. It is extremely important not to rule out pulmonary atresia with an intact interventricular septum when aortography in the newborn shows a normal-sized ductus arteriosus with an obtuse inferior angle. Despite existing pulmonary atresia, these patients have neither a hypoplastic right ventricle nor discontinuity of the right ventricle with the pulmonary artery.

Ductus Arteriosus↗

[Reproducible (latent) obstruction of the left ventricular outflow tract].

BACKGROUND AND OBJECTIVE: Left ventricular outflow tract obstruction (LVOTO) with flow acceleration in late systole (Vmax > or = 2.0 m/s and acceleration/ejection time [AT/ET] > or = 0.5) is often present in a latent form in left ventricular hypertrophy; it can be unmasked by administration of glyceryl trinitrate or with the Valsalva manoeuvre (preload reduction). It was investigated prospectively whether latent LVOTO also occurs on ergometric exercise. PATIENTS AND METHODS: 61 patients (19 female, 42 male, mean age 55.5 +/- 12.9 years) with left ventricular hypertrophy at rest were examined by Doppler echocardiography at rest, under provocation with glyceryl trinitrate and the Valsalva manoeuvre, and during bicycle ergometry. Manifest LVOTO was already present before the test in 3 patients. RESULTS: In 24 patients LVOTO occurred during the provocation (Vmax rose from 1.38 +/- 0.25 to 2.93 +/- 0.53 m/s; AT/ET rose from 0.47 +/- 0.16 to 0.68 +/- 0.09). In 19 of these 24 patients LVOTO also occurred during the ergometry. In four additional patients changes were marginal (AT/ET > or = 0.5; Vmax = 1.8 and 1.9 m/s, respectively). There was a highly significant correlation between provocation and ergometry with regard to Vmax, as well as AT/ET (with r = 0.84 and 0.76, respectively). CONCLUSIONS: These findings show that provokable LVOT gradients are likely to occur during exercise to a similar degree as those on preload reduction. Thus, course of disease could possibly be influenced by early treatment in patients with left ventricular hypertrophy.

Adult↗

Reconstruction of the right ventricular outflow tract in patients with tetralogy of Fallot: early and late results.

Total correction of tetralogy of Fallot was performed on 161 consecutive patients between 1966 and 1979. Forty-four per cent had undergone a previous palliative operation. A right ventricular outflow patch was used in 58% of the patients. In about half of these cases, the patch extended across the pulmonary annulus onto the main pulmonary artery and in five per cent it extended beyond the pulmonary bifurcation. The overall operative mortality was 13%, for patients less than three years of age it was 38% and for patients three years and older 11%. Operative mortality was chiefly related to an unrelieved right ventricular hypertension. The use of an outflow patch did not influence the operative mortality. Eight survivors (6%) were re-operated upon, six due to residual outflow obstruction, one due to residual ventricular septal defect and in one patient re-operation was indicated because of neo-intimal thickening in a tubular dacron graft. Inadequate infundibulectomy (one patient), narrow pulmonary annulus (two patients), calcified valve remnants (two patients) and a ridge in the posterior wall of the main pulmonary artery (two patients, one of whom underwent two re-operations) were the anatomical bases for the obstructions. It is concluded that at total correction of tetralogy of Fallot every effort should be made to relieve the right ventricular outflow obstruction, even if the pulmonary valves have to be sacrificed. If the pulmonary artery is hypoplastic or has localized stenosis, the pulmonary annulus should be incised and an outflow patch carried all the way to the pulmonary artery branches.

Adolescent↗

Tetralogy of Fallot with subarterial ventricular septal defect.

BACKGROUND: Tetralogy of Fallot with subarterial ventricular septal defect is frequently seen among Asians. Compared with infracristal ventricular septal defect, postoperative right ventricular outflow obstruction is more likely because of subpulmonary extension of the defect. Moreover the incidence of aortic regurgitation is a concern because of the absence of a supporting infundibulum. METHODS: Four hundred cases of classic tetralogy were reviewed, 61 of which had subarterial ventricular septal defect. RESULTS: Aortic regurgitation (of more than mild degree) was identified in 7 cases with subarterial and 7 with infracristal ventricular septal defects. The mechanism of infracristal defect was predominantly an annular dilation before surgery. In contrast 5 cases with subarterial defect had progression of aortic regurgitation after operation yielding an actuarial incidence of 29.7% at 20 years. In 2 patients the cause seemed to be fixation or plication of the aortic valve annulus by the ventricular septal patch. Compared with infracristal defect, subarterial defect was associated with increased incidence of reoperation (12.0% versus 1.9% at 10 years, p = 0.01), frequent use of transannular patch (70.5% versus 45.7%, p = 0.0004), and worse New York Heart Association (NYHA) functional class (p = 0.007). Right ventricular outflow obstruction was the reason for reoperation in 3 patients with subarterial defect and was associated with worse NYHA classification in the long-term, on multivariate analysis (p = 0.0002). CONCLUSIONS: Tetralogy with subarterial ventricular septal defect was associated with worse functional outcome. To prevent adverse outcomes, precise suturing of the distal ventricular septal patch, extensive infundibulectomy, lower threshold for transannular incision, and smaller-sized ventricular septal patch placement are warranted.

Aortic Valve↗