Extensive cardiac lymphomatous infiltration manifesting as pericarditis and right ventricular outflow obstruction in a patient with multiorgan transplants.
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Adult↗
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Results for “Ventricular Outflow Obstruction”
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Doppler evaluation of normalized peak filling rate in normal children and children with left ventricular outflow obstruction.
To evaluate the early diastolic peak filling rate of the left ventricle, three groups of children (normal children, patients with aortic valvular stenosis, and patients with aortic coarctation) were examined with the peak filling rate normalized to stroke volume calculated from the mitral valve inflow Doppler recording as the peak E velocity divided by the velocity time integral. The normal value for this index in children was 6.78 +/- 0.99 SV/sec and did not vary with age, weight, body surface area, or heart rate. Compared with normal subjects, both patients with aortic stenosis and patients with coarctation had increased left ventricular mass, but patients with aortic stenosis had decreased normalized peak filling rates (5.3 +/- 0.84 SV/sec, p < 0.01), while patients with coarctation had normal rates (6.79 +/- 0.98 SV/sec, p = 0.97). Compared with patients with aortic coarctation, patients with aortic stenosis had higher Doppler gradients. Thus the Doppler index of peak filling rate normalized to stroke volume is particularly useful in children because it is independent of heart rate, age, weight, and body surface area. Patients with coarctation may have normal peak filling rates normalized to stroke volume despite increased left ventricular mass because of milder obstruction or better coronary artery perfusion compared with that of patients with aortic stenosis.
Adolescent↗
Ventricular outflow obstruction, valve aplasia, bradyarrhythmia, pulmonary hypoplasia and non-immune fetal hydrops because of a large rhabdomyoma in a case of unknown tuberous sclerosis: a prenatal diagnosed cardiac rhabdomyoma with multiple symptoms.
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Adult↗
Surgical treatment for primary cardiac leiomyosarcoma causing right ventricular outflow obstruction.
A 55-year-old man was admitted to another hospital because of dry cough and dyspnea that rapidly worsened before admission. Chest computed tomography revealed a low-density mass that nearly obstructed the main pulmonary arterial trunk. Pulmonary thromboembolism was suspected and treated with catheter-directed thrombolysis therapy. Despite optimal thrombolysis and anticoagulant therapy, his symptoms persisted. He was referred for further examination and possible surgery for presumed pulmonary thromboembolism. The mass appeared more likely to be a tumor than a thrombus on careful analysis of the magnetic resonance imaging. At surgery, the anterior wall of the main pulmonary arterial trunk, the pulmonary valve, annulus, and the right ventricular outflow tract were all invaded by what was found to be a tumor and were resected under conventional cardiopulmonary bypass. The resected area was reconstructed with a 25-mm-diameter bioprosthetic valve and Xenomedica patch. Final pathological diagnosis was primary cardiac leiomyosarcoma involving the pulmonary valve. Postoperative course was uneventful, and he was discharged 11 days after surgery without adjuvant therapy because he refused it. Ten months later, the patient was well, but a chest X-ray revealed some coin lesions in the bilateral lung fields that were thought to be metastatic tumor.
Heart Neoplasms↗
[Functional maneuvers for the diagnosis of hypertrophic cardiomyopathies with and without left ventricular outflow obstruction].
PURPOSE OF THE STUDY: to assess the diagnostic value of the commonly adopted haemodynamic criteria for the diagnosis of obstructive and non-obstructive hypertrophic cardiomyopathy. PATIENTS: 16 cases of hypertrophic cardiomyopathy, 7 cases of the non-obstructive form, 16 fixed subaortic stenosis, 8 valvular aortic stenosis, and 7 cases of hypertensive heart disease. METHODS: left and right heart catheterisation, with particular attention to the pressure gradient and to the aortic pulse pressure by means of simultaneous pressure recording from the left ventricle and aorta under basal conditions, after a ventricular premature beat, during the Valsalva manoeuvre, during amyl nitrite inhalation, and isoproterenol administration. RESULTS AND CONCLUSION: the Valsalva manoeuvre increases significantly (p < 0.001) the pressure gradient, and the aortic pulse pressure in the post-extrasystolic beat decreases significantly (p < 0.001) in patients with hypertrophic obstructive cardiomyopathy. This response is significantly different from that of the other 3 disease groups. If the non-obstructive form is defined by strict criteria (i.e. no gradient above 30 mmHg even after provocative manoeuvres), then it cannot be separated from other forms of secondary left ventricular hypertrophy.
Adult↗
Total relief of severe left ventricular outflow obstruction after spontaneous rupture of chordae tendineae in a patient with hypertrophic cardiomyopathy.
In hypertrophic cardiomyopathy (HCM), rupture of mitral chordae tendineae is infrequent and causes acute haemodynamic deterioration. A 38 year old male patient had chordae rupture leading to prolapse of both mitral leaflets and severe regurgitation, without change in symptomatic status. One year before, he had had mild mitral regurgitation and a resting left ventricle outflow tract of 105 mm Hg that disappeared in the present evaluation. In this unique case, worsening of mitral regurgitation was counterbalanced by total relief of the severe obstruction. This case report highlights the role of the mitral valve apparatus in the genesis of obstruction in HCM, further stimulating surgical techniques in which mitral repair can be the main procedure.
Adult↗
Left ventricular outflow obstruction.
Patients with LVOT obstruction require lifelong follow-up because the obstruction may be progressive or recurrent. Several procedures are usually required, either by surgery or by interventional cardiac catheterization, to repair or palliate the obstructive lesion. The treatment of these patients continues to evolve, and, despite the complexity of these patients' lesions, the morbidity and mortality rates have decreased and are expected to decrease further in the future.
Aftercare↗
Congenital papillary tumor of the tricuspid valve. An unusual cause of right ventricular outflow obstruction in a neonate with trisomy E.
A trisomy E neonate had congenital cardiac anomalies that included a ventricular septal defect, a bicuspid aortic valve, and a congenital papillary tumor of the tricuspid valve. The large papillary tumor was responsible for severe intermittent pulmonary outflow obstruction. The cause and pathogenesis of these rare papillary tumors are unknown, but they are probably related to the more sessile varieties of congenital valvular dysplasia. The life-threatening obstruction to blood flow caused by the congenital valvular tumor probably can be alleviated by simple surgical resection. This type of tumor should be considered in the differential diagnosis of atypical forms of outflow tract obstruction.
Abnormalities, Multiple↗
Surgical treatment of left ventricular outflow obstruction in the neonate: profound hypothermia and circulatory arrest.
In the 1950s, Sealy and his co-workers developed an improved technique for inducing cardiac and total body hypothermia using a pump oxygenator and heat exchanger. These techniques have been gradually adopted, and now markedly facilitate the repair of some congenital cardiac malformations presenting in infancy. In this paper, I discuss two forms of congenital cardiac lesions: an interrupted aortic arch and critical aortic stenosis. For both of these congenital malformations, operation in infancy using closed techniques has resulted in a very poor outcome. Using cardiopulmonary bypass, induced hypothermia, and circulatory arrest, accurate arch repair and aortic valvotomy can be performed together with repair of associated lesions. Improved results can be expected from the use of the more precise repair techniques afforded by a dry and bloodless field.
Aorta↗
Intravascular stents for management of pulmonary artery and right ventricular outflow obstruction.
This study was performed to determine the efficacy of balloon-expandable stents in the treatment of branch pulmonary artery-stenoses and conduit stenosis in children. A total of eight stainless steel stents were implanted in seven patients. Three patients had tetralogy of Fallot with pulmonary artery stenosis following total correction, one patient had conduit stenosis following correction of transposition of the great arteries, one patient had intra-cardiac conduit stenosis after septation for single left ventricle, and two patients had pulmonary artery stenosis after Fontan operation. Six stents were placed in the branch pulmonary arteries, one in the extracardiac conduit, and one in the intracardiac conduit. The mean age at implantation was 13 +/- 3 years and the mean weight 37 +/- 12 kg. Follow-up time ranged from 0.3-2 years. The diameter of pulmonary arteries with stenoses increased from 5.6 +/- 2.2 mm to 10.6 +/- 1.8 mm (n = 7). The systolic pressure gradient decreased from 56 +/- 26 mmHg to 22 +/- 16 mmHg (n = 5). No embolization or thrombotic event has been noted. One stent placed in the intracardiac conduit was compressed and fractured. These data indicate that balloon-expandable stents are useful in the treatment of pulmonary artery branch stenoses and extracardiac conduit stenosis in children. The use of stents for intracardiac stenosis may result in stent fracture.
Adolescent↗
Left ventricular outflow tract obstruction.
Left ventricular outflow tract obstruction can occur at the supravalvar, valvar, or subvalvar level. Each level of obstruction is associated with distinct symptomatology, natural history, and operative approach. Reconstructive techniques can usually be used with low operative risk and excellent immediate and longer-term outcomes. Valve replacement for valvar obstruction is advised when reconstruction is not possible. The Ross procedure has greatly improved the results of valve replacement in children.
Aortic Stenosis, Subvalvular↗
[Noninvasive studies of dynamic left ventricular outflow obstruction in patients with sigmoid septum].
In order to evaluate the clinical significance of the markedly protruding interventricular septum into the left ventricular (LV) cavity (sigmoid septum), we performed non-invasive studies including amyl nitrite (AN) inhalation in 21 patients (pts) with two-dimensional echocardiographic (2DE) documentations. LV outflow tract (LVOT) obstruction was determined by the presence at least three of the following findings at rest or during AN inhalation: 1) a loud apical ejection systolic murmur (ESM), 2) a midsystolic dip in the carotid pulse, 3) systolic anterior motion (SAM) of the mitral valve (MV) or chordae tendineae, and 4) systolic semiclosure of the aortic valve (AV). The 21 pts were subdivided into six pts (group I) with resting (two pts) or provocative (four) obstruction, and 15 pts (group II) without obstruction. Their ages ranged from 40 to 85 years with an average of 65. No pt had evidence of hypertrophic cardiomyopathy. Results were as follows: In five pts of group I a long ESM with a mid-systolic peak was recorded near the apex. After AN inhalation, this murmur was markedly intensified. On the contrary, all pts of group II had a short and early systolic murmur, which was not markedly intensified by AN. In contrast to group II, group I pts had a significantly smaller LV end-diastolic dimension, a smaller LVOT dimension, higher percent thickening of the LV posterior wall, higher fractional shortening and decreased aorto septal angle (the angle between the anterior aortic wall and the interventricular septum by 2DE). On 2DE, each pt of group I showed significant narrowing between the protruded septum and the hypercontractile LV posterior wall with the papillary muscle. Anteriorly shifted chordae tendineae noted as the SAM on the M-mode echocardiogram might also play an important role on the genesis of obstruction. The signs of LVOT obstruction at rest disappeared following oral administration of propranolol in two pts of group I. These observations suggested that LVOT obstruction might occur in some pts with sigmoid septum and the hypercontractile state, and that a systolic murmur observed in this condition should be differentiated from a functional murmur in the aged or a systolic murmur in hypertrophic obstructive cardiomyopathy.
Adult↗
Balloon-induced right ventricular outflow obstruction: a new approach to control of acute interventricular shunting after myocardial infarction in canines and swine.
Current management of ventricular septal defect (VSD) after myocardial infarction (MI) is aimed at improving left ventricular (LV) performance by afterload reduction as a means of hemodynamic stabilization or shunt control. The current investigation was undertaken to determine whether primary manipulation of right venticular (RV) performance by afterload enhancement was an effective means of reducing MI-VSD shunting. In five open-chest dogs an external LV-RV shunt was created with pulmonary-systemic flow ratios (Qp/Qs) averaging 2.26:1. Inflation of a balloon-tipped catheter in the main pulmonary artery (PA) reduced average QP/Qs to 1.28:1 and shunt flow from 783 to 343 ml/min. However, this increase in RV afterload caused further significant increases in RV systolic and end-diastolic pressure and suggested that deterioration of RV function might be limiting the usefulness of this technique. To investigate whether inotropic support for the RV would overcome this limitation, a similar shunt was created in 11 open-chest swine. We then investigated the effects of dopamine, infused at 24, 60, and 120 mug/min, on QP/QS and other hemodynamic variables both with and without PA balloon inflation. Optimal shunt control was obtained when effects of dopamine were added to those of PA balloon inflation. Shunt flow that had been 1,633 ml/min was reduced to 892 ml/min with the PA balloon and reduced further to 757 ml/min with dopamine, which also lowered RV and LV end-diastolic pressure and reduced total systemic vascular resistance. In four other swine with left anterior descending ligations, PA balloon inflation and dopamine infusion again favorably affected hemodynamics. Epicardial mapping in these swine showed an increase in S-T segment displacement, suggesting that the cumulative effect of these interventions allowed hemodynamic improvement at the expense of enhanced ischemic injury. These data indicate that acute RV outflow obstruction with a balloon catheter is an effective means of temporarily reducing acute ventricular shunting and that dopamine may be a useful pharmacological agent to use with RV afterload manipulation to stabilize a patient prior to transfer to a medical center for more definitive therapy.
Animals↗
Valve placement in the ventricular apex for complicated left ventricular outflow obstruction.
Successful correction of severe valvular calcific aortic stenosis is described in which a stented porcine aortic heterograft was placed in the ventricular apex. Creation of a double outlet is a valid alternative approach to relieve left ventricular hypertension, and indication for such a procedure may be encountered unexpectedly. Intramyocardial placement of a durable tissue valve provides a simple and effective option for the cardiac surgeon.
Aged↗
The transmitral approach to left ventricular outflow tract obstruction.
Left ventricular outflow tract obstruction is a particularly vexing problem in patients with complete transposition (atrioventricular concordance and ventriculoarterial discordance) and intact ventricular septum. Previous techniques, such as resecting the obstructive lesion through the pulmonary valve or bypassing the obstruction, have not given entirely satisfactory results. An alternative approach of resection through the left atrium and the retracted mitral valve has recently been proposed. We have used this new technique to achieve excellent relief of this condition in three patients with different types of obstruction.
Cardiomyopathy, Hypertrophic↗
Diagnosis of right ventricular outflow obstruction in infants by cross sectional echocardiography.
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Echocardiography↗
Creation of double-outlet left ventricles for left ventricular outflow obstructions: initial clinical results in six patients.
Operations for supravalvular, valvular, and subvalvular stenoses are frequently complicated by aortic insufficiency, recurrence, or aortic valve prosthetic malfunction. Without surgery, increasing left ventricular pressures, myocardial hypertrophy, congestive failure, and sudden death are common. Following an extended series of bovine experiments, we have created double-outlet left ventricles in 6 patients. All but one had been operated on previously for combined supravalvular, valvular, or subvalvular aortic stenosis or idiopathic hypertrophic subaortic stenosis. All left ventricular-ascending aortic gradients were markedly decreased and the deranged left ventricular physiologic indices returned toward normal following operation and discharge from the hospital. None of these patients is receiving Coumadin, and none has exhibited any deleterious effects from the fractionation of left ventricular output, cephalad and caudad. Our current prosthesis is a composite consisting of a special LTI carbon inlet tube with a left ventricular apical sewing ring and graft extension anastomosed to a Hancock prosthesis of appropriate size. The procedure is simple, reproducible, effective, and has much to recommend. Special conduits are now being fabricated for future use. In certain instances, this approach may have advantages over conventional primary aortic valvotomy in infants or valve replacement in adults.
Adolescent↗
Membranous right ventricular outflow obstruction.
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Adult↗