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PubMed1991

Pulmonary valve-moderator band distance and association with development of double-chambered right ventricle.

Double-chambered right ventricle (DCRV), a form of right ventricular outflow obstruction that sometimes accompanies a ventricular septal defect (VSD), is associated with superior and rightward displacement of the septal insertion of the moderator band. It was hypothesized that this superior displacement is present and identifiable by echocardiography in patients with a VSD even before right ventricular outflow tract obstruction develops. Eight patients who had a previous echocardiographic study showing a VSD alone were echocardiographically diagnosed as having DCRV. Their initial echocardiographic studies were reviewed, and superior displacement of the moderator band was quantified by measuring the distance between the pulmonary valve and moderator band, normalized to tricuspid anulus diameter. These measurements were compared with those from the initial studies of the following 3 other groups: (1) an age-matched group of 10 patients with no structural heart disease; (2) an age-matched group of 10 patients with a VSD who did not develop DCRV; and (3) a group (not age-matched) of 10 patients with VSD and DCRV in whom subpulmonary obstruction was present on the initial study. The 8 patients who eventually developed subpulmonary obstruction had significant superior displacement of the moderator band at the time of their initial echocardiogram compared with that of the 2 age-matched control groups (p less than 0.01). In contrast, there was no significant difference in moderator band displacement between these patients and the 10 with DCRV who already had right ventricular outflow obstruction at their initial study (p = not significant).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1999

Echocardiographic diagnosis of left ventricular outflow tract obstruction caused by an acquired subaortic membrane after mitral valve replacement.

Although an acquired subaortic membrane has been reported as a cause of left ventricular outflow tract (LVOT) obstruction in various clinical settings, it previously has not been reported after mitral valve surgery. We describe 3 cases of acquired LVOT obstruction that resulted from development of a subaortic membrane after mitral valve replacement. This report emphasizes the role of an acquired subaortic membrane in LVOT obstruction after mitral valve replacement, the use of echocardiography in diagnosing this condition, and the importance of early intervention.

Adult↗
PubMed2001

Long-term results after early primary repair of tetralogy of Fallot.

OBJECTIVE: Early primary repair of tetralogy of Fallot has been routinely performed at Children's Hospital, Boston, since 1972. We evaluated the long-term outcome of this treatment strategy including the influence of a transannular patch. METHODS: Fifty-seven patients less than 24 months of age (median 8 months) underwent primary repair of tetralogy of Fallot between January 1972 and December 1977. Thirty-one patients had a transannular patch. Survival and freedom from reintervention were determined by the Kaplan-Meier method with 95% confidence intervals. RESULTS: There were 8 early deaths, and 1 patient died 24 years after initial repair. Recent follow-up was obtained for 45 of the 49 long-term survivors (92%). Median follow-up was 23.5 years. Ten patients underwent reintervention, 8 of whom underwent relief of right ventricular outflow tract obstruction. Right ventricular outflow tract obstruction occurred in 6 patients without a transannular patch and 2 with a transannular patch (33% vs 6%, P =.04). One pulmonary valve replacement was performed at another institution 20 years after the repair. Forty-one long-term survivors were in New York Heart Association class I and 4 were in class II. Actuarial survival was 86% at 20 years (95% confidence intervals = 80%-92%). Freedom from reintervention was 93% at 5 years (95% confidence intervals = 87%-99%) and 79% at 20 years (95% confidence intervals = 70%-86%). No significant differences were found between patients with and without a transannular patch (survival, P =.34; freedom from reintervention, P =.09, log-rank tests). CONCLUSIONS: Long-term survival is excellent and the freedom from reintervention is satisfactory after early primary repair of tetralogy of Fallot in the 1970s. Use of a transannular patch does not reduce late survival and is associated with a lower incidence of right ventricular outflow tract obstruction.

Exercise Tolerance↗
PubMed2005

Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999-2001.

BACKGROUND: The left ventricular outflow tract (LVOT) malformations aortic valve stenosis (AVS), coarctation of the aorta (CoA), and hypoplastic left heart syndrome (HLHS) contribute significantly to infant mortality due to birth defects. Previous epidemiology data showed rate differences between male and female and white and black ethnic groups. The Texas Birth Defects Registry, an active surveillance program, enables study in a large, diverse population including Hispanics. METHODS: Records of children up to 1 year old with AVS, CoA, and HLHS born in Texas from 1999 to 2001, were collected from the registry. Those including additional heart defects or a chromosomal anomaly were excluded. Multivariate analysis included: infant sex; United States-Mexico border county residence; and maternal age, race/ethnicity, birthplace, and education. RESULTS: There were 910 cases among 1.08 million live births, of which 499 met inclusion criteria. Multivariate modeling of all LVOT malformations combined demonstrated lower prevalence rate ratios (PRRs) for black males (0.26) and Hispanic males (0.70). Similar results were found for CoA but not AVS or HLHS. Higher PRRs were noted for increased maternal age for LVOT (1.3 for 24-34 years; 1.7 for >34 years), AVS, and HLHS, but not CoA, and higher PRRs across all diagnoses for males (LVOT PRR, 2.4) were noted. CoA PRRs were higher in border county vs. non-border county residents (PRR, 2.1). Maternal education and birthplace were not significant factors. CONCLUSIONS: There are rate differences for males among all 3 ethnic groups. Sex and ethnic differences suggest genetic etiologies, where the ethnic differences could be used to find susceptibility loci with mapping by admixture linkage disequilibrium. Increased CoA rates along the U.S.-Mexico border suggest environmental causes that will require further monitoring.

Aortic Coarctation↗
PubMed2001

Myectomy for hypertrophic obstructive cardiomyopathy after failed alcohol septal ablation: clinicopathological correlations.

Hypertrophic cardiomyopathy - a genetically transmitted cardiac disorder - has diverse clinical, pathological and molecular manifestations. Echocardiography is the most reliable tool for clinical diagnosis of hypertrophic cardiomyopathy. Reduction of the intraventricular pressure gradient and improvement of symptoms are major objectives of all therapeutic strategies. The recently introduced concept of catheter-based interventional treatment involves selective coronary perforator branch injection of 96% ethanol to reduce septal thickness, left ventricular outflow obstruction, left ventricular filling pressure and symptoms. The long term morphological features after medical ablation are presented for the first time and compared with both the echocardiographic findings and the findings reported in the English-language literature. The findings show that injection of ethanol into the perforator branch is associated with a fairly localized area of myocardial scarring.

Cardiac Surgical Procedures↗
PubMed2004

Transcoronary ablation of septal hypertrophy for hypertrophic obstructive cardiomyopathy: feasibility, clinical benefit, and short term results in elderly patients.

OBJECTIVE: To evaluate symptomatic and haemodynamic results of transcoronary ablation of septal hypertrophy for hypertrophic obstructive cardiomyopathy in elderly patients. SETTING: Tertiary referral centre for patients with hypertrophic obstructive cardiomyopathy. DESIGN: Retrospective study of two groups of consecutive patients divided at a median age (59 years). PATIENTS: Transcoronary ablation of septal hypertrophy was compared for 80 patients (group 1) < 60 years of age and 77 patients (group 2) > or = 60 years of age. At baseline both groups were similar concerning the proportion of familial hypertrophic cardiomyopathy, concomitant moderate hypertension, prior syncope, left ventricular outflow obstruction, left ventricular end diastolic pressure, and left ventricular ejection fraction. Patients in group 2 had a lower interventricular septal thickness and more severe disease as measured by New York Heart Association (NYHA) functional class, exercise capacity, pulmonary artery mean pressure at workload, and cardiac index at peak exercise. RESULTS: Median follow up was seven months after transcoronary ablation of septal hypertrophy. Both groups had a significant and similar improvement in basal and provokable obstruction, septal thickness, NYHA functional class, exercise tolerance, peak oxygen consumption, and pulmonary artery mean pressure at workload. Significant differences, compared with the younger group, were a higher proportion of persistent total atrioventricular block (5% v 17%, p = 0.015) and a slight decrease in left ventricular ejection fraction (3 (12) v -6 (11)%, p = 0.001) in the elderly, despite a trend to a lower induced peak creatine kinase activity (596 (339) v 491 (331) U/l, p = 0.051). CONCLUSIONS: Short term results with transcoronary ablation of septal hypertrophy suggest that independent of a patient's age similar treatment strategies are justified in hypertrophic obstructive cardiomyopathy.

Adult↗
PubMed2005

Autosomal dominant inheritance of left ventricular outflow tract obstruction.

Most nonsyndromic congenital heart malformations (CHMs) in humans are multifactorial in origin, although an increasing number of monogenic cases have been reported recently. We describe here four new families with presumed autosomal dominant inheritance of left ventricular outflow tract obstruction (LVOTO), consisting of hypoplastic left heart (HLHS) or left ventricle (HLV), aortic valve stenosis (AS) and bicuspid aortic valve (BAV), hypoplastic aortic arch (HAA), and coarctation of the aorta (CoA). LVOTO in these families shows a wide clinical spectrum with some family members having severe anomalies such as hypoplastic left heart, and others only minor anomalies such as mild aortic valve stenosis. This supports the suggestion that all anomalies of the LVOTO spectrum are developmentally related and can be caused by a single gene defect.

Animals↗
PubMed1976

Surgical treatment of left ventricular outflow tract obstruction with apicoaortic valved conduit.

From Aug. 13, 1975, through May, 1976, nine patients underwent creation of a left ventricular "vent" for relief of severe left ventricular outflow tract obstruction. A Dacron fabric graft containing a heterograft valve was used to establish a conduit from the left ventricle to the abdominal aorta. There were five male and four female patients in this group; their ages ranged from 4 to 72 years. All had valvular, subvalvular, or supravalvular aortic stenosis, and all but two had undergone previous surgical procedures for relief of the stenosis. All patients survived the operation and none are receiving anticoagulant therapy. All are asymptomatic at present and follow-up is approaching one year. Postoperative cardiac catheterization studies revealed gradients across the aortic valve to be reduced by approximately 90% and mean ventricular systolic pressures by 45%. Although this concept is not new, it has not been used widely and we believe its effectiveness warrants further application.

Adolescent↗
PubMed1984

A morphometrical study of myocardial disarray associated with right ventricular outflow tract obstruction.

In order to define quantitatively the histological characteristics of the hypertrophied myocardium of the right ventricular outflow tract in patients with congenital heart disease, a light-microscopic study of biopsy specimens obtained from 20 cases with tetralogy of Fallot (TOF) and 14 cases with muscular obstruction of the right ventricular outflow tract ( RVOTO ) was performed using a semi-automatic sampling counter. As the controls, the crista supraventricularis of 11 autopsied cases without cardiac disease were used. The TOF patients were divided into two groups: those with (9 cases) or without (11) a history of hypoxic spells. The myocyte diameter was calculated according to Chalkley 's and Arai 's method. The areas of myocardial fibrosis and myocyte disarray were determined quantitatively by the point count method. Myocardial fibrosis was classified into five types: focal, mild perimysial, severe perimysial, perivascular and plexiform. In the control group, the myocyte diameter and area of fibrosis and disarray tended to increase with age. In the obstructive groups, the myocyte diameter and area of disarray were greater than those of the control in any age group. Myocyte diameter tended to increase with age in all groups, except for the TOF cases with a history of hypoxic spells. The latter also had the largest area of myocardial disarray (25.3%) (p less than 0.01), and a largest area of fibrosis (20.5%) than the TOF cases without spell (14.4%), but severe perimysial fibrosis was observed in only five out of the nine patients with spells. The lack of a relation between the pressure and the presence of an increased area of the myocardial disarray in TOF with spells implies that this myocardial change is brought about by myocardial abnormality accompanied by cardiac malformation.

Adolescent↗
PubMed2000

Intra-aortic balloon pump associated with dynamic left ventricular outflow tract obstruction after valve replacement for aortic stenosis.

An unstable patient with critical aortic stenosis had an intra-aortic balloon pump placed preoperatively for hemodynamic support and alleviation of symptoms. After separation from cardiopulmonary bypass following aortic valve replacement, the patient was hypotensive with increased pulmonary artery pressures. Transesophageal echocardiography revealed left ventricular outflow tract obstruction associated with systolic anterior motion of the mitral valve and severe mitral regurgitation. This pathophysiology was present when ventricular systole was preceded by balloon counterpulsation, but was absent during unassisted systole. This case report demonstrates a potentially significant untoward effect of intra-aortic balloon pump augmentation after aortic valve replacement for aortic stenosis. The timely diagnosis of this iatrogenic condition in the operating room permitted the prompt implementation of appropriate management strategies and avoided unnecessary surgical intervention.

Aged↗
PubMed1986

Results of total correction in complete atrioventricular septal defects with congenital or surgically induced right ventricular outflow tract obstruction.

The outcome of total repair in 29 children who had complete atrioventricular septal defect (AVSD) and congenital or surgically induced right ventricular outflow tract obstruction (RVOTO) is reviewed. All 11 patients with congenital RVOTO had normal pulmonary artery (PA) pressure before the complete repair. Of the 18 children who had undergone PA banding, seven had PA pressure above 30 mm Hg (mean, 53.5). Two had elevated pulmonary vascular resistance (greater than 3 units). Early mortality was 18.2% for the patients with congenital RVOTO and 44.4% for those who had undergone PA banding (p not significant). After a mean follow-up of 5 years, the results are good in the survivors of both groups. Analysis of multiple-risk factors indicate that, for the total group of patients, death was significantly more common in children less than 5 years of age (p less than 0.01) or less than 15 kg (p less than 0.02) than in older or larger patients.

Adolescent↗
PubMed2002

Recurrence of left ventricular outflow tract obstruction after left anterior descending coronary artery revascularization in a patient with hypertrophic obstructive cardiomyopathy.

A patient with known hypertrophic obstructive cardiomyopathy presented with an anteroseptal myocardial infarction which resulted in the disappearance of his subaortic pressure gradient. Surgical revascularization of his left anterior descending coronary artery after the viability of his myocardium had been documented led to the recurrence of his left ventricular outflow tract obstruction and subaortic pressure gradient.

Cardiomyopathy, Hypertrophic↗
PubMed1981

Accessory mitral valve tissue causing left ventricular outflow tract obstruction in D-transposition of the great arteries.

Two patients with D-transposition of the great arteries (D-TGA) were found to have an unusual form of left ventricular outflow tract obstruction. Both had ball-like accessory mitral valve tissue that partially occluded the outflow tract. In one patient there was an intact septum, while in the other there were ventricular septal defects. The appearances have been described previously, although not in D-TGA. Recognition of accessory mitral valve tissue may allow resection of the tissue at the time of repair of the transposition complex.

Female↗
PubMed1981

Rastelli procedure for transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. Early and late results in 41 patients (1971 to 1978).

Forty-one children with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction underwent a Rastelli operation between 1971 and 1978. A homograft valve preserved in an antibiotic solution and extended with A dacron tube was the conduit of choice. Alternatively, conduits with porcine heterografts or valves constructed from calf pericardium were used. They were positioned to the left of the aorta whenever possible. The intraventricular tunnel from the left ventricle to the aorta was constructed from Dacron velour. There were four early and seven late deaths. The last 13 consecutive patients have survived. Early deaths were related to unfavourable anatomy, conduit compression, and sepsis. Residual ventricular septal defects and postoperative infection were the main factors contributing to the late deaths.

Adolescent↗
PubMed2002

Painless acute aortic dissection presenting as echocardiographically diagnosed left ventricular outflow tract obstruction.

We present an unusual case of painless acute aortic dissection with an atypical echocardiographic presentation that could be confused with an aortic stenosis. Transesophageal echocardiography excluded the presence of aortic stenosis and disclosed a systolic anterior movement of the mitral valve as the cause of left ventricular outflow tract obstruction. It also revealed a type A aortic dissection.

Acute Disease↗
PubMed1997

Patch repair of subsemilunar conal septal defect resulting in severe left ventricular outflow tract obstruction.

A subsemilunar conal septal defect was closed at 8 months of age with a redundant Dacron patch that bowed into the left ventricular outflow tract, resulting in severe subaortic stenosis and massive left ventricular hypertrophy. Mistaken for cardiomyopathy or myocarditis, this rare complication of subsemilunar ventricular septal defect patch closure led to orthotopic cardiac transplantation followed by death.

Abnormalities, Multiple↗