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PubMed1993

Transatrial-transpulmonary repair of atrioventricular septal defect with right ventricular outflow tract obstruction.

Twenty patients had a repair of an atrioventricular septal defect with tetralogy of Fallot (n = 13) or double outlet right ventricle (n = 7). Mean age was 3.5 years. Surgical technique included transatrial-transpulmonary resection of right ventricular outflow tract obstruction and transatrial two patch repair of the atrioventricular septal defect. Ten patients required a transannular patch and one patient had a right ventricle-pulmonary artery conduit placed. There was no hospital mortality, and mean hospital stay was 15 days. One patient had late sudden death of unknown cause. Six patients have required reoperation because of residual ventricular septal defect (VSD), mitral incompetence, residual right ventricular outflow tract obstruction, and/or conduit stenosis. No patient was reoperated on because of left ventricular outflow tract obstruction. Fifteen patients are asymptomatic, one has exertional dyspnea, and two have intermittent occasional bronchospasm. The transatrial-transpulmonary two patch repair and extensive relief of right ventricular outflow tract obstruction have given good immediate results. Reoperation rate has been high mainly due to residual VSD and mitral incompetence.

Actuarial Analysis↗

PubMed1983

Two forms of hypertrophic cardiomyopathy distinguished by inheritance of HLA haplotypes and left ventricular outflow tract obstruction.

In this study we have performed human leukocyte antigen (HLA)-A and B typing on nine patients with hypertrophic cardiomyopathy (HCM) and their relatives. Four patients had relatives who also had the disease. HLA typing of the familial form of HCM revealed a very close association of a given HLA-A,B haplotype with the occurrence of the disease. All four patients who had affected relatives had obstruction of left ventricular outflow (LVOT), while four patients with unaffected relatives did not have obstruction. One additional patient with obstruction and without familial incidence was an only child and had few living relatives. Thus, HCM can be divided into two subtypes: a familial form linked to the HLA-A,B system, which may be related to obstructive type, and a sporadic form not linked to HLA antigens. These data confirm the existence of at least two separate forms of hypertrophic cardiomyopathy. The study also confirms their existence in the Japanese population, with a completely different gene pool than the population from the southeastern United States in whom the observation was initially described.

Adolescent↗

PubMed2001

Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction.

BACKGROUND: Management of newborns with interrupted aortic arch (IAA) remains challenging. Associated severe left ventricular outflow tract obstruction (LVOTO) have often led to increased mortality with neonatal biventricular repair. We review our experience with an alternative approach for this complex surgical problem. METHODS: From May 1991 to June 1999, 28 neonates were treated for IAA. Thirteen of 28 neonates (46%) had type B IAA, ventricular septal defect (VSD) and severe LVOTO (Z value -2 to -7; mean -5 +/- 1.7). Mean age was 8 days (3 to 23 days old) with average weight of 3.3 kg (2.4 to 4.2 kg). Eight of 13 (62%) had anomalous right subclavian artery. Ten of 13 (77%) had thymic aplasia and chromosome 22 region qll deletion. All 13 patients were treated initially with a modified Norwood procedure. RESULTS: There were no perioperative deaths. Complications included 2 patients with recurrent arch stenosis treated with balloon dilatation. Two patients had systemic arterial shunt revision. Follow-up ranged from 2 to 99 months old (mean 39 months). There were 2 late deaths unrelated to any operation. Nine of 12 patients had a second stage palliation consisting of a bidirectional Glenn shunt. Six patients went on to have biventricular repairs (3 Ross-Konno, 2 Rastelli, 1 VSD closure with LVOT resection). One patient had a modified Fontan operation and 5 patients are awaiting potential biventricular repair. CONCLUSIONS: Children with IAA and severe LVOTO may be managed by initial Norwood palliation with an excellent outcome likely. This initial "univentricular" approach has enabled eventual successful biventricular repair despite severe LVOTO.

Aortic Coarctation↗

PubMed2001

Left ventricular outflow tract obstruction in the transposition of great arteries defined by transthoracic three-dimensional echocardiography.

We assessed the feasibility of transthoracic three-dimensional reconstruction of the pulmonary valve and subpulmonary left ventricular outflow tract in two patients with transposition of great arteries, ventricular septal defect, and obstruction to the left ventricular outflow tract. Three-dimensional reconstruction of the pulmonary valve could be displayed as "en face" through a three-dimensional generated "pulmotomy view," allowing an overview of the pulmonary aspect of the valve from a surgeon's perspective. In similar fashion, reconstruction of the subpulmonary outflow tract could be displayed along its longitudinal axis as seen through a left ventriculotomy. Unique views could be obtained equivalent to surgical or autopsy dissections, allowing more complete understanding of the morphology and severity of left-sided obstructive lesions.

Echocardiography, Three-Dimensional↗

PubMed1991

Diagnosis, transport, and outcome in fetuses with left ventricular outflow tract obstruction.

Between March 1986 and April 1990, 22 consecutive fetuses (at gestational ages of 21 to 38 weeks) with a suspected diagnosis of critical (ductus-dependent) left ventricular outflow tract obstruction on fetal echocardiogram were referred to our center for delivery and surgical treatment. Diagnoses were hypoplastic left heart syndrome (n = 16), valvular aortic stenosis (n = 2), common atrioventricular canal with subaortic stenosis (n = 3), and single ventricle with subaortic stenosis (n = 1). Postnatal echocardiography revealed that fetal echocardiography was correct in predicting left ventricular outflow tract obstruction to be critical in all but one patient, for a positive predictive value of 96%. Of the 21 patients with true, critical left ventricular outflow tract obstruction, 17 patients underwent cardiac surgery as neonates (birth to 6 days of age, median 2 days); 13 (or 77%) survived and were discharged from the hospital. In addition, one patient underwent successful balloon aortic valvotomy for critical valvular aortic stenosis but later died of sepsis. Lethal chromosomal and congenital abnormalities should be sought and are contraindications for this approach. In utero transport of fetuses with suspected critical left ventricular outflow tract obstruction to a neonatal cardiac surgical center can result in improved neonatal condition and may improve overall survival.

Echocardiography, Doppler↗

PubMed2001

Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome.

OBJECTIVES: The goal of this study was to determine the prevalence and evolution of acquired right ventricular outflow tract obstruction (RVOTO) in the recipient twin in twin-twin transfusion syndrome (TTTS). BACKGROUND: Twin-twin transfusion syndrome complicates 4% to 26% of diamniotic monochorionic twin gestations and is associated with high fetal morbidity and mortality. Cardiac dysfunction and biventricular hypertrophy may develop in the recipient twin with the potential for RVOTO. METHODS: This was a retrospective review of a two-center experience of TTTS to describe the prevalence and evolution of acquired RVOTO in the recipient twin. Right ventricular outflow tract obstruction was diagnosed or excluded by fetal or postnatal echocardiography or clinical assessment. RESULTS: Of 73 twin pregnancies with TTTS identified between 1994 to 1998, a total of seven (9.6%) were complicated by RVOTO in the recipient twin: two subvalvar/muscular, four valvar and one combined. Of 44 pregnancies with fetal echo, six had in utero RVOTO with antegrade flow diagnosed at gestational ages ranging from 19 to 27 weeks. In utero progression occurred in four cases over a period of four to eight weeks, with the development of RVOT atresia by delivery. Postnatal progression of RVOTO occurred in two cases, one of which required pulmonary balloon valvuloplasty at age two years. Postnatal regression of subvalvar RVOTO occurred in two cases in early infancy. Death related directly or indirectly to the RVOTO occurred in all four patients who developed complete RVOT obliteration. CONCLUSIONS: Right ventricular outflow tract obstruction may occur in the recipient twin of at least 9% of pregnancies complicated by TTTS. Right ventricular outflow tract obstruction progression is common in utero and may worsen neonatal outcome.

Canada↗

PubMed2006

Dynamic left ventricular outflow tract obstruction induced by dobutamine stress echocardiography leading to myocardial ischemia and infarction.

Left ventricular outflow tract (LVOT) obstruction occurring during dobutamine stress echocardiography (DSE) occurs in approximately 15-20% of patients undergoing DSE. The clinical significance and mechanism of LVOT obstruction has been debated, but is now generally felt to result from the pharmacological effects of dobutamine in increasing inotropy and causing peripheral vasodilation. It must be realized that in rare instances, ischemia may occur and lead to wall motion abnormalities and eventually myocardial infarction. We present the case of a 70-year-old asymptomatic woman who underwent a routine pre-operative cardiac stress evaluation and was found to develop a dynamic outflow tract obstruction leading to myocardial ischemia and infarction.

Aged↗

PubMed2006

Modified Nikaidoh procedure for transposition of great arteries, ventricular septal defect and left ventricular outflow tract obstruction.

The surgical management of transposition of the great arteries with ventricular septal defect and left ventricle outflow tract obstruction is a real challenge in congenital heart surgery. The Rastelli operation has been used for many years with satisfactory early and late results. A newer operation described by Nikaidoh seems to take anatomy more into account and has been performed with promising outcomes. In this report, we present a patient with transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction who was successfully treated with a modification of the Nikaidoh procedure.

Cardiac Surgical Procedures↗

PubMed1983

[Generalized lentigo in a case of hypertrophic cardiomyopathy with right ventricular outflow tract obstruction].

A case was reported in which generalized lentigo was associated with hypertrophic cardiomyopathy and right ventricular outflow tract obstruction. A 34-year-old man was admitted to our hospital for cardiac evaluation. He had been noted to have a heart murmur since his childhood and had had a diagnosis of pulmonary stenosis in his high school age. Physical examination revealed precordial murmurs and numerous lentigines over his whole body surface including palms and soles. Two-dimensional and M-mode echocardiography disclosed asymmetric septal hypertrophy, systolic anterior motion of the mitral valve (SAM) and systolic semiclosure of both semilunar valves. A systolic turbulent flow was recorded at the outflow tracts of both ventricles by pulsed Doppler technique. Cardiac catheterization demonstrated a pressure gradient of 13 mmHg across the right ventricular outflow tract. We considered that the present case was an atypical LEOPARD syndrome with predominant right ventricular outflow tract obstruction which was precisely evaluated by noninvasive techniques.

Adult↗

PubMed1999

Rapid return of left ventricular outflow tract obstruction and symptoms following cessation of long-term atrioventricular synchronous pacing for obstructive hypertrophic cardiomyopathy.

Atrioventricular (AV) synchronous pacing reduces left ventricular (LV) outflow tract obstruction and symptoms in patients with obstructive hypertrophic cardiomyopathy (HC). The duration of gradient reduction, if pacing is discontinued for a prolonged period of time, is unknown. This question is addressed in the present randomized double-blind crossover study comparing continued with inactivated pacing. Ten patients, successfully paced for > or = 6 months, were randomized to continue pacing or to have their pacemakers inactivated after baseline examinations, including echo-Doppler imaging, exercise testing, and a quality-of-life questionnaire. When entering the study, the patients were in New York Heart Association functional classes I to II. After pacemaker programming, examinations were repeated at 1, 4, and 12 weeks. At the 12-week follow-up the alternate pacing mode was programmed, and the patient entered the second study arm. Premature pacemaker pacing occurred if severe clinical deterioration or a significant increase of the LV outflow tract obstruction were evident. Three patients started in the inactive mode and 7 patients in the active mode. All patients who started with the pacemaker inactivated required early reprogramming due to return of symptoms after 7, 10, and 13 days, respectively. All 7 patients who started in the active pacing mode completed the first period; however, after reprogramming to the inactive mode they required early activation after 1 to 20 days due to reappearance of intolerable subjective symptoms. The LV outflow tract gradient increased significantly after inactivation of pacing in all patients (22 +/- 21 mm Hg to 47 +/- 21 mm Hg). Thus, AV synchronous pacing effectively relieves symptoms and reduces the LV outflow tract gradient in patients with obstructive HC. This improvement, which is rapidly established with the initiation of cardiac pacing, is not persistent after cessation of pacing. Reinitialization of pacing promptly reduces the LV outflow tract obstruction and relieves symptoms to a preexisting extent.

Aged↗

PubMedDate not supplied

Right ventricular outflow tract obstruction by cardiac metastasis as the first manifestation of follicular thyroid carcinoma.

A 40-year-old man was sent to the echocardiographic laboratory because of a heart murmur. An intracardiac mass, causing obstruction of flow within right ventricle, was diagnosed and the patient was referred to surgery. Histologic examination classified the mass as a metastasis of highly differentiated follicular carcinoma of the thyroid gland. Thyroidectomy was performed and radioiodine treatment instituted. Thus echocardiographic identification of right ventricular outflow obstructing mass was the initial presentation of follicular carcinoma of the thyroid gland. Early detection of this moderately disseminated malignancy allowed for surgical excision and systemic radioactive iodine treatment.

Adenocarcinoma, Follicular↗

PubMed2002

Beta-blocker therapy for dynamic left-ventricular outflow tract obstruction.

BACKGROUND: In a small but significant group of elderly patients who present with breathlessness, dynamic left-ventricular outflow tract obstruction (DLVOTO) may be responsible for symptom generation. The aim of our study was to investigate the effect of beta-blockade on ventricular physiology and symptoms in patients with DLVOTO. METHODS: We performed a pilot study in 15 patients (age 76+/-10 years, mean+/-S.D., 14 female) with symptoms of exercise intolerance (New York Heart Association, NYHA, class 2.7+/-0.5). All patients had normal resting left ventricular (LV) systolic function together with DLVOTO based on the presence of basal septal hypertrophy and the development of high outflow tract velocities on stress echocardiography. All were commenced on oral atenolol (mean dose 45+/-19 mg), but this could not be tolerated in four patients due to a deterioration in clinical status. RESULTS: In the remaining 11 patients who could tolerate atenolol therapy, the rate pressure product was significantly lower (23%, P=0.028) and there was a marked reduction in LV outflow tract velocity (23%, P=0.001) following beta-blockade. Patient symptoms improved significantly following atenolol therapy, with a reduction in mean NYHA class from 2.8+/-0.4 to 1.5+/-0.5 (P<0.0001). CONCLUSIONS: Beta-blockade may represent a beneficial therapeutic approach in selected patients with DLVOTO as identified by stress echocardiography.

Adrenergic beta-Antagonists↗

PubMed1981

Hypertrophic obstructive cardiomyopathy and lentiginosis: a little known neural ectodermal syndrome.

Eleven patients, 10 male, with classic hypertrophic obstructive cardiomyopathy and lentiginosis are described. Physical examination showed differences from the few previously reported cases in that (1) this condition was not confined to children; (2) mental retardation, sensorineural deafness and gonadal and somatic infantilism were either rare or absent; and (3) detailed family studies provided no evidence that this condition was inherited. Nine patients underwent cardiac catheterization and left ventricular angiography; all had left ventricular outflow obstruction and three had concomitant right ventricular outflow obstruction with a pressure gradient in excess of 100 mm Hg. Ten of the 11 patients were severely symptomatic, and 7, each with a left ventricular pressure gradient of more than 70 mm Hg, underwent successful septal myotomy/myectomy that resulted in marked symptomatic improvement that was maintained after long-term follow-up.

Adolescent↗

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↗

PubMed1976

An operation for relief of severe left ventricular outflow tract obstruction.

The case history of a 30-year-old man with severe mitral and aortic stenosis and slight aortic regurgitation who underwent replacement of both valves is presented. At the initial surgical procedure, because of the small-size annuli, a small Beall mitral prosthesis and a No. 19 Björk-Shiley aortic prosthesis were used. The subsequent recurrence of severe symptoms and findings of an 80 mm. Hg gradient across the Björk-Shiley aortic valve led to repeat exploration and corrective surgery. The operative procedure to relieve left ventricular outflow obstruction is described. It is felt that this procedure can be used not only for the relief of severe aortic annular stenosis but also for other forms of severe left ventricular outflow tract obstruction such as idiopathic hypertrophic subaortic stenosis and particularly in the patients who have experienced poor results with one of the known surgical procedures for this clinical entity.

Adult↗

PubMed1996

Metastatic colonic carcinoma with intracavitary right ventricular outflow tract obstruction and cardiac tamponade: a case report.

Intracavitary metastasis causing right ventricular outflow tract (RVOT) obstruction is very rare. Combination of cardiac tamponade with RVOT obstruction due to intracavitary metastasis has never been reported in the literature. Herein, we report a case of colonic carcinoma with sudden onset of severe dyspnea. Echocardiography detected cardiac tamponade as well as a huge intracavitary mass obstructing the RVOT. Pericardiocentesis and surgical resection of the right ventricular mass were performed but the patient died during operation. Thus, the combination of intracavitary RVOT obstruction and cardiac tamponade in a patient with carcinoma suggests extensive myocardial and pericardial involvement with a poor prognosis.

Cardiac Tamponade↗

PubMed2004

Accessory mitral valve tissue causing severe left ventricular outflow tract obstruction in an adult.

Accessory mitral valve (AMV) is a rare cause of left ventricular outflow tract (LVOT) obstruction and is extremely rare in adults. We report a case of an older adult with an AMV that caused severe LVOT obstruction. A parachute-like piece of tissue (the AMV) protruding into the LVOT during systole was first detected in a 45-year-old woman by echocardiography. Because the pressure gradient and dyspnea gradually progressed, she finally underwent a successful operation for removal when she was 48 years old.

Adult↗

PubMed2004

Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: This study was undertaken to compare the outcomes of the Lecompte procedure and Rastelli repair in the transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) and to determine the risk factors associated with unfavorable events. METHODS: Over a 12-year period (April 1990-October 2002), 35 patients underwent complete repair for TGA, VSD, and LVOTO. Twenty-five patients (71%) underwent the Lecompte modification, and mean age and weight were 23.4+/-18.2 months and 10.2+/-3.0 kg. Ten patients (29%) underwent the Rastelli operation, and mean age and weight were 39.1+/-36.1 months and 13.8+/-6.8 kg. RESULTS: One early death (3%) occurred after the Lecompte procedure and no late death. The mean follow-up was 5.9+/-3.8 years. Eight patients in the Rastelli group (80%) underwent a late reoperation for obstruction of the extracardiac conduit, and in four of these patients, a reoperation for LVOTO was concomitantly required. Reoperation was also required in six patients of the Lecompte group (25%); five for right ventricular outflow tract obstruction (RVOTO) including one for LVOTO and two for VSD leakage, and one for mitral regurgitation and left pulmonary artery stenosis. The interval prior to reoperation ranged from 1.6 to 11.1 years, with a mean of 5.7+/-3.1 years. The actuarial figures for freedom from reoperation at 5 and 10 years were 40.0+/-15.5 and 26.7+/-15.0% after the Rastelli operation and 95.7+/-4.3 and 63.5+/-12.6% after the Lecompte procedure (P = 0.02). Multivariate analysis by Cox regression analysis revealed that the risk factors of RVOTO were a younger age at operation, the Rastelli operation, and ductus ligation during the operation. CONCLUSIONS: The Lecompte procedure and Rastelli repair provide satisfactory early and late results. However, substantial late morbidity is more associated with conduit obstruction, and LVOTO in Rastelli repair rather than Lecompte procedure.

Abnormalities, Multiple↗