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PubMed2002

Late outcome after arterial switch operation for complete transposition of great arteries with left ventricular outflow tract obstruction.

BACKGROUND: Long-term follow-up of patients who underwent arterial switch operation for complete transposition of great arteries with anatomic left ventricular outflow tract obstruction (LVOTO) has rarely been brought into the focus. METHODS: Of 299 patients who underwent an arterial switch operation between January 1991 and January 2001, 23 patients had anatomic LVOTO. Age ranged from 4 days to 18 years (median 90 days) and weight ranged from 2.6 to 35 kg (median 4.3 kg). Surgical management included arterial switch operation, closure of ventricular septal defect wherever indicated, and excision of LVOTO. RESULTS: Among patients with preoperative LVOTO there were 2 early deaths and 8 patients had mild neoaortic regurgitation at the time of discharge. Follow-up ranged from 8 months to 9 years (mean 60 +/- 12 months). In 4 patients who had mild neoaortic regurgitation at discharge, the regurgitation progressed to moderate or severe degree after a follow-up of 22 to 72 months. In 1 patient mild mitral regurgitation present at the time of discharge progressed to severe mitral regurgitation. This patient subsequently underwent double valve replacement. CONCLUSIONS: Presence of preoperative anatomical LVOTO in patients undergoing arterial switch operation predicts high incidence of postoperative neoaortic regurgitation.

Adolescent↗
PubMed2001

Left ventricular outflow tract obstruction unmasked by nifedipine: a therapeutic pitfall in the management of chronic aortic regurgitation.

This case report illustrates the aggravation of a clinically silent left ventricular outflow tract obstruction by maintenance use of nifedipine in a patient with chronic severe aortic regurgitation, and demonstrates a potential limitation of vasodilator therapy in the management of this patient population. Recognition of this clinical scenario is imperative, as decision making in patients with chronic severe aortic regurgitation rests on the development of symptoms and/or left ventricular dysfunction in relation to the regurgitant volume. The importance of echocardiography in the detection of this valvular finding and in the follow up of these patients is emphasized.

Aortic Valve Insufficiency↗
PubMed1977

Left ventricular apical-abdominal aortic conduits for left ventricular outflow tract obstructions. Clinical results in eleven patients with a special composite prosthesis.

Cardiologists and surgeons are occasionally faced with therapeutic dilemmas regarding the management of recurrent left ventricular outflow tract obstructions, such as supravalvular, valvular, subvalvular or "tunnel" stenoses and obstructive myopathies. Following an extended series of biomaterial, hemodynamic, reliability and configuration studies in calves, we have created double-outlet left ventricles in 11 patients. All gradients were markedly reduced. All patients were discharged without anticoagulants, and the longest follow-up period is in excess of 1 year. In eight cases, the percent of cardiac output flowing antegrade and retrograde was quantitated with velocity-tipped catheters. An average ratio of 2:1 (antegrade [ascending aorta]: retrograde [composite conduit]) was found. The procedure is simple and effective in relieving primary or recurrent left ventricular outflow tract obstructions.

Adolescent↗
PubMed1996

Combined Rastelli and atrial switch procedure: anatomic and physiologic correction of discordant atrioventricular connection associated with ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVE: To evaluate our early experience with the combined Rastelli and atrial switch operation for repair of discordant atrioventricular connection associated with ventricular septal defect and left ventricular outflow tract obstruction. METHODS: Detailed study of the first three cases including preoperative assessment, operative procedure, postoperative progress and follow-up. RESULTS: All three patients survived and remained in sinus rhythm. Postoperative ITU/hospital stay was 2/8, 5/10 and 33/45 days. The third patient developed a venous pathway obstruction and required balloon dilatation with stenting on the 25th postoperative day. All three patients were well at follow-up examination 21, 6 and 5 months after operation. CONCLUSIONS: We believe that this operation is likely to become the operation of choice for this combination of lesions. More experience and longer follow-up is needed.

Adult↗
PubMed1984

Solitary tumour metastasis: a rare cause of right ventricular outflow tract obstruction and sudden death.

The clinical features of a patient presenting with recent breathlessness indicated right ventricular outflow tract obstruction, while echocardiography suggested the presence of tumour in the right ventricle. Before further investigations could be instituted, she died suddenly. Autopsy revealed a haemopericardium secondary to rupture of the right atrium while the right ventricle was found to contain a tumour arising from most of its endocardial surface. Histology showed this to be a poorly differentiated mucous secreting carcinoma similar in appearance to her colonic carcinoma excised 8 years previously. There was no other evidence of metastatic disease.

Adenocarcinoma, Mucinous↗
PubMed2003

Aortoventriculoplasty in a patient with complex left ventricular outflow tract obstruction, mitral valve stenosis and aneurysm of the ascending aorta.

We describe a new method of aortoventriculoplasty in a patient with calcified mitral stenosis, aortic valvular stenosis, severe left ventricular outflow tract obstruction, and aneurysm of the ascending aorta. This complex pathology was successfully treated with replacement of both the valves and a tubular dacron graft. The proximal end of the dacron tube was tailored as a patch for the repair of the ventricular septum and the aortic root, and the distal end was anastomosed to the distal ascending aorta. The patient had an uneventful recovery and postoperative echocardiography showed no significant residual gradient on the left ventricular outflow tract.

Aortic Aneurysm, Thoracic↗
PubMed2001

Biventricular hypertrophic cardiomyopathy with right ventricular outflow tract obstruction associated with Noonan syndrome in an adult.

This report describes an adult patient with Noonan syndrome accompanied by biventricular hypertrophic cardiomyopathy causing isolated right ventricular outflow tract obstruction. Biventricular hypertrophic cardiomyopathy causing right- and/or left-side outflow tract obstruction, as well as valvular pulmonary stenosis, is relatively common in infants with Noonan syndrome. However, this condition without a dysplastic pulmonary valve, or indeed any polyvalvular dysplasia, is rare in adults with Noonan syndrome. Treatment with a beta-adrenergic receptor blocking agent improved the patient's symptoms. Because neither the etiologic and prognostic relationship nor the genetic linkage between hypertrophic cardiomyopathy associated with Noonan syndrome and non-syndromic hypertrophic cardiomyopathy is clearly defined, clinicopathological findings and further follow-up may provide important evidence for the pathogenesis of hypertrophic cardiomyopathy.

Adult↗
PubMed1997

Pericardial cyst causing right ventricular outflow tract obstruction.

Pleuropericardial cysts are rare. Rarer still are cardiopulmonary complications caused by their presence. We report the case of a pericardial cyst producing high-grade right ventricular outflow tract obstruction and its subsequent management. The clinical importance of transesophageal echocardiography is highlighted.

Aged↗
PubMed1995

Hypotension during dobutamine stress echocardiography: is it related to dynamic intraventricular obstruction?

Although it has been shown that a hypotensive response during dobutamine stress echocardiography is not a marker of coronary artery disease, the mechanism of this response remains unclear. We hypothesize that hypotension during dobutamine stress echocardiography is not related to the development of dynamic intraventricular obstruction. The development of left ventricular outflow obstruction was defined as a late-peaking Doppler velocity profile that exceeded baseline outflow velocity by at least 1 m/sec in 104 consecutive patients undergoing dobutamine stress echocardiography. Left ventricular outflow obstruction was seen in 13% of 15 patients with a hypotensive response (group 1) and in 13% of 89 patients without a hypotensive response (group 2). The mean baseline systolic blood pressure was 157 +/- 21 mm Hg in group 1 compared to 139 +/- 25 mm Hg in group 2 (p = 0.008). An ischemic response to dobutamine infusion as manifested by the development of new or worsening wall motion abnormalities was seen in 40% of group 1 patients and 34% of group 2 patients (p = 0.77). These data demonstrate that a hypotensive response is not related to the development of dynamic intraventricular obstruction during dobutamine stress echocardiography. Rather, there is a significant association between a higher baseline systolic blood pressure and a hypotensive response during dobutamine infusion.

Adult↗
PubMed2006

[Case in which landiolol hydrochloride improved left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve following mitral valve plasty].

We report a case of left ventricular outflow tract (LVOT) obstruction caused by systolic anterior motion of the mitral valve (SAM) following mitral valve plasity (MVP). A 65-year-old man underwent mitral valve plasty for grade III mitral valve regurgitation. The plasty was done smoothly and the patient was weaned from cardiopulmonary bypass successfully with continuous dobutamine infusion. However, about 30 minutes after the weaning, severe cardiovascular collapse developed. Inotropic agent, such as dobutamine, ephedrine, or calcium hydrochloride was not effective. Trans-esophageal echocardiography (TEE) showed severe mitral valve regurgitation with LVOT obstruction due to SAM. The collapse was successfully treated with volume loading and a small amount of a beta1-adrenergic antagonist, landiolol hydrochloride. We conclude that acute LVOT obstruction with SAM could develop following MVP. TEE was a much useful tool for early diagnosis and landiolol hydrochloride would be a notable agent for nonsurgical treatment of LVOT obstruction with SAM.

Adrenergic beta-Antagonists↗
PubMed1984

The surgical management of left ventricular outflow tract obstruction due to tricuspid valve pouch in complete transposition of the great arteries.

Subpulmonary stenosis in transposition of the great arteries, resulting from a tricuspid valve pouch bulging into the left ventricular outflow tract through a ventricular septal defect, can be missed at the time of operation in the flaccid, nonbeating heart unless preoperative diagnosis has been established. In our experience, six patients were found to have this lesion. In four patients the tricuspid valve pouch was recognized preoperatively. At operation, retraction of the tricuspid valve pouch into the right ventricle, patch closure of the ventricular septal defect, and a Mustard procedure were performed in three patients; the fourth is awaiting correction following initial palliation with a subclavian-pulmonary shunt. In the other two, the ventricular septal defect was partially or completely obliterated by a tricuspid valve pouch that was missed preoperatively and during exploration at the time of the Mustard procedure. Residual left ventricular outflow tract obstruction was subsequently corrected with a left ventricle-pulmonary artery valved conduit. Echocardiographic and angiocardiographic examinations offer helpful signs for the diagnosis of tricuspid valve pouch. Transatrial retraction of the redundant tricuspid valve tissue into the right ventricle, patch closure of the ventricular septal defect, and Mustard operation are the procedures of choice. A left ventricle-pulmonary artery valved conduit may be required for residual unresectable left ventricular outflow tract obstruction.

Cardiomyopathy, Hypertrophic↗
PubMed2005

Massive posterior mitral annular calcification causing dynamic left ventricular outflow tract obstruction: mechanism and management implications.

We report a case of massive posterior mitral annular calcification causing severe systolic anterior motion of the anterior mitral leaflet and dynamic left ventricular outflow tract obstruction. Mechanism of genesis of systolic anterior motion by this unusual mechanism is illustrated. Importance of recognizing this mechanism and its implications for surgical therapy are discussed. Our patient also had liquefaction necrosis of mitral annular calcification causing its extension into left ventricular myocardium mimicking a tumor.

Calcinosis↗
PubMedDate not supplied

Cavo-pulmonary shunt for left ventricular outflow tract obstruction after senning.

We report a case of Senning procedure in a 32-month-old girl with transposition of the great arteries and dynamic left ventricular outflow tract obstruction (LVOTO). After discontinuation of cardio-pulmonary bypass, the persisting, worsened LVOTO caused hemodynamical instability. A cavo-pulmonary shunt (CPS) was performed, with considerable subsequent improvement. We consider the use of CPS as a valuable option in patients with persisting, significant dynamic LVOTO after atrial switch.

Child, Preschool↗
PubMed1991

Metastatic cardiac malignant fibrous histiocytoma presenting as right ventricular outflow tract obstruction.

An unusual case of metastatic disease of the right ventricle is described in a 74-year-old woman who had presented with a malignant fibrous histiocytoma of the right thigh previously treated with surgery and radiotherapy. Two years later she presented with signs and symptoms of tricuspid regurgitation and right ventricular outflow tract obstruction. The diagnosis of right ventricular tumor was made by echocardiographic and angiographic studies and confirmed on postmortem examination.

Aged↗
PubMed1995

Reversible steroid-induced hypertrophic cardiomyopathy with left ventricular outflow tract obstruction in two newborns.

Dexamethasone is now frequently used in the treatment of bronchopulmonary dysplasia. We report on two premature babies receiving dexamethasone who developed symptomatic myocardial hypertrophy and left ventricular outflow tract obstruction, as documented by M-mode echocardiography and Doppler studies. A normal heart was recorded on echocardiography before and after dexamethasone treatment. The patients had no known risk factors associated with the development of obstructive hypertrophic cardiomyopathy.

Bronchopulmonary Dysplasia↗
PubMed2005

Dynamic left ventricular outflow tract obstruction secondary to catecholamine excess in a normal ventricle.

Hypertrophic cardiomyopathy (HCM) is the most common cause of left ventricular outflow tract (LVOT) obstruction. The LVOT obstruction is a consequence of the asymmetric septal hypertrophy and the mitral systolic anterior motion (SAM), causing both of them a dynamic gradient in LVOT. LVOT obstruction has been observed also in other conditions like hypertensive hypertrophy, dehydration, sepsis, vasodilatation, excessive sympathetic stimulation, pericardial tamponade, and after mitral valve repair and aortic valve replacement for aortic stenosis. We report in this document the case of two patients who developed a significant gradient at LVOT in the context of amine treatment during their admission into the intensive unit care. In both, cases there were no gradient, significant hypertrophy or SAM at baseline cardiac evaluation. We have met only one case reported in the literature matching those conditions. In order to treat this type of patients properly, it is essential to take in consideration this pathology.

Aged↗
PubMed2000

Comparison of left ventricular outflow tract obstruction in interruption of the aortic arch and in coarctation of the aorta, with diagnostic, developmental, and surgical implications.

A morphometric comparison of the anatomic causes of left ventricular (LV) outflow obstruction in interruption of the aortic arch and in coarctation of the aorta with ventricular septal defect (VSD), based on 30 postmortem cases of each, revealed that posterior malalignment of the conal septum with a conoventricular VSD was significantly more prevalent with interruption (93%) than with coarctation (47%) (p <0.001). The ratio of the aortic valve diameter-to-the pulmonary valve diameter, which provided a quantitative index of the degree of posterior conal septal malalignment and of the consequent LV outflow tract obstruction at and immediately below the level of the aortic valve, was significantly smaller with interruption (</=0.50 in 67%) than with coarctation (</=0.50 in 17%) (p <0.001). A bicuspid or unicuspid aortic valve, both with interruption and with coarctation, was more prevalent with posterior conal septal malalignment (74%) than with normal conal septal alignment (42%) (p <0.05). Posterior conal septal malalignment was associated with LV outflow tract obstruction at 3 different sites: subvalvar, annular, and leaflet. The anatomic findings explain the incidence of postoperative LV outflow tract obstruction in patients with interrupted aortic arch after simple VSD closure, and may support a surgical strategy of elevating or otherwise removing the posteriorly malaligned conal septum from the LV outflow tract at the time of VSD closure.

Aorta, Thoracic↗
PubMed2005

Dynamic left ventricular outflow tract obstruction in an orthotopic liver transplant recipient.

A 53 year-old man with Laennec's and hepatitis C-related cirrhosis was found to have dynamic left ventricular outflow tract obstruction during routine evaluation for orthotopic liver transplantation. The outflow tract obstruction gradient was quantified as being 155 to 189 mmHg maximally during dobutamine stress echocardiography. The patient subsequently underwent successful orthotopic liver transplantation at our institution. We discuss here the use of intraoperative transesophageal echocardiography to detect early signs of dynamic outflow tract obstruction and provide a rational guide for fluid and hemodynamic management. We conclude that the measured pressure across the left ventricular outflow tract during dobutamine stress testing does not necessarily predict either intraoperative hemodynamic perturbations such as obstruction or outcome in these patients.

Echocardiography, Stress↗