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The influence of exercise on left ventricular outflow tract obstruction, left ventricular performance and electrocardiogram in hypertrophic cardiomyopathy.

To investigate exertional changes in hypertrophic cardiomyopathy (HCM), 42 patients with HCM were studied by echocardiography and ECG at a supine ergometer exercise. In 12 cases with left ventricular (LV) outflow tract obstruction at rest (Group I), systolic anterior movement (SAM) of mitral valve was intensified during exercise; and among 30 cases without obstruction at rest (Group II), in 6 cases (Group IIA) SAM appeared during exercise. SAM might be further intensified 1 minute after exercise. Inverted T-wave was seen at rest especially in Group IIB (no SAM both at rest and during exercise), and tended to become less deep during exercise with an increase of LV wall motion. In conclusion, in HCM, LV outflow tract obstruction may be produced or intensified during exercise. Inverted T-wave in HCM may become less deep during exercise, with accelerated LV function.

Adolescent↗

A case of idiopathic hypertrophic obstructive cardiomyopathy causing severe right ventricular outflow tract obstruction in infancy.

An infant with idiopathic hypertrophic obstructive cardiomyopathy, presenting as severe right ventricular outflow obstruction, is described. The nonspecific clinical features and an unexpected angiocardiographic appearance made determining the correct diagnosis difficult. This infant was mistakenly diagnosed as having severe pulmonary stenosis. Attempted surgical correction was unsuccessful and at necropsy this diagnosis was confirmed. It is suggested that cross-sectional echocardiography may be diagnostic for infants with idiopathic hypertrophic obstructive cardiomyopathy.

Cardiac Catheterization↗

Unruptured sinus of Valsalva aneurysm with right ventricular outflow tract obstruction associated with ventricular septal defect.

We describe an aneurysm originating from the right sinus of Valsalva, with right ventricular outflow tract obstruction associated with ventricular septal defect, in a 9-year-old boy. Diagnosis was made by echocardiography and cardiac catheterization. Successful surgical repair of ventricular septal defect and aortic valvuloplasty resulted in symptomatic improvement.

Aortic Aneurysm↗

Surgical treatment of non-critical right ventricular outflow tract obstruction with intact ventricular septum in patients over one year of age.

Between March 1966 and May 1982, 257 patients with clinically noncritical right ventricular outflow tract obstruction with intact ventricular septum (RVOTO/IVS) underwent elective open-heart correction, with an overall early mortality of 3.9%. Age ranged from 13 months to 48 years (mean 6.6 +/- 6.5 - median 5). We describe three different anatomical types, for which a standardized surgical approach is suggested. When one or more of the following features--small right ventricular cavity; rigid infundibular stenosis; hypoplastic pulmonary annulus and trunk; dysplastic cusps--were present, the mortality (10.6%) was significantly higher than in isolated valvular stenosis (2.2%) or in valvular plus dynamic infundibular stenosis or isolated low-lying stenosis (2.5%). In a series of 159 consecutive patients operated upon since September 1974 the cumulative early mortality was 1.3%. Late results on 202 patients (mean follow-up period of 25.6 +/- 22.6 months) are good in 90.6% and fair in 8.9% of the cases. One patient died of encephalitis 2 years after the operation. We believe that a more precise characterization of the underlying lesions and a more liberal use of different surgical techniques to minimize residual gradients, iatrogenic pulmonary incompetence and impairment of right ventricular contractility, may decrease early mortality and improve overall results.

Heart Defects, Congenital↗

Left ventricular outflow tract obstruction due to systolic anterior motion of the anterior mitral leaflet in patients with concentric left ventricular hypertrophy.

Patients with hypertrophic cardiomyopathy (i.e., asymmetric septal hypertrophy) may show obstruction to left ventricular outflow under basal conditions or with provocative maneuvers. The presence of dynamic left ventricular outflow tract obstruction in patients with concentric ventricular wall thickening (but without abnormalities of the aortic valve) has been less well appreciated. Clinical and morphologic features of five patients with nondilated left ventricles and with left ventricular outflow obstruction are presented. In each patient peak systolic pressure gradients between left ventricle and systematic artery were measured at cardiac catheterization and ranged from 60-140 mm Hg under basal conditions or with provocation. Each patient had echocardiographically documented systolic anterior motion of the anterior mitral leaflet, which was apparently responsible for the outflow obstruction, and concentric left ventricular wall thickening (septal-free wall thickness ratio of less than 1.3). Two of the five patients had evidence of genetically transmitted hypertrophic cardiomyopathy, as evidenced by disorganized muscle cells in the ventricular septum or asymmetric septal hypertrophy in first degree relatives. Hence, left ventricular outflow tract obstruction associated with systolic anterior motion of the anterior mitral leaflet may occur in some patients with concentric left ventricular hypertrophy who do not have typical hypertrophic cardiomyopathy.

Adult↗

Surgical treatment of critical right ventricular outflow tract obstruction with intact ventricular septum in infancy.

Between December 1965 and August 1981, a total of 104 operations were performed on 101 infants for treatment of critical right ventricular outflow tract obstruction with intact ventricular septum (RVOTO/IVS). Twenty-three patients had pulmonary atresia (PA/IVS) and 78 critical pulmonary stenosis (CPS/IVS). One of those had an emergency reoperation within the first year of life because of acquired atresia after valvotomy. The cumulative early 30 days mortality was 35.6% (58.3% for PA/IVS and 28.7% for CPS/IVS). Analysis of this series indicates that the surgical risk does not significantly differ in PA/IVS and CPS/IVS within the same age group. Patients with small right ventricular cavity, dictating surgery within the first 30 days of life, present significantly higher surgical risk in both groups. The higher overall mortality in PA/IVS may be influenced by the lower median age at operation. Our present surgical indications are the following. Patients with normal sized right ventricle are treated with pulmonary valvotomy at any age. Patients with small sized right ventricle undergo early total correction over 6 months of age. Under 30 days of life balloon atrial septostomy, valvotomy and systemic-to-pulmonary artery shunt are performed. Between one and 6 months the surgical technique depends upon the clinical presentation and the right ventricular anatomy in the single patient. Since this policy of treatment has been pursued, in the last 12 consecutive cases the 30 days mortality was 8.3%.

Follow-Up Studies↗

Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study.

Right ventricular outflow tract obstruction can complicate the natural history of patients with isolated ventricular septal defect. Although numerous pathologic mechanisms may be responsible for this phenomenon, it is less clear what the frequency is of each of these mechanisms in any cohort of patients studied in a single institution. We identified 20 patients with isolated ventricular septal defect from 1970 to 1981 who acquired a right ventricular outflow tract gradient of 25 mm Hg or more. They ranged in age at the initial catheterization investigation from 4 days to 23 months and at the subsequent study from 13 months to 8 years. Angiographically, 2 mechanisms were implicated in the 20 patients. Hypertrophy of right ventricular anomalous muscle bundles was identified in 19 of the 20 while hypertrophy of a malaligned infundibular septum (that is, classic tetralogy type) was identified in only 1 patient. These data indicate that acquired right ventricular outflow tract obstruction is rarely of the classic tetralogy type.

Aortic Valve↗

[Left ventricular outflow tract obstruction without left ventricular hypertrophy treated with ACE inhibitors].

We describe a very unusual case of sudden, severe worsening of congestive heart failure which was caused by ACE inhibitors. Diagnosis was made by echocardiogram showing a typical picture of dynamic, left ventricular outflow tract obstruction without left ventricular hypertrophy, which disappeared on discontinuing ACE inhibitors. This phenomenon has already been described as a complication of other drugs such as nitrates, commonly used as provocative tests for latent obstructive cardiomyopathy. To our knowledge ACE inhibitors have not been described as a causative factor.

Aged↗

Ross procedure for complex left ventricular outflow tract obstruction.

BACKGROUND: Complex left ventricular outflow tract (LVOT) obstruction in children continues to pose a significant therapeutic challenge to cardiac surgeons. The Ross procedure, in combination with resection of subaortic stenosis or a Konno type septal incision, is an important option for these difficult patients. METHODS: Recently two children aged 14 and 5 years with LVOT obstruction involving combined subaortic and valvar stenosis underwent surgical correction using the pulmonary autograft. Clinical presentation, operative technique, outcome and intermediate follow-up are detailed. RESULTS: One patient had resection of an isolated subaortic membrane in combination with a pulmonary autograft and the second a Ross Konno procedure. Postoperative hospital stays were without complication. Both patients were discharged at 5 days and have no significant obstruction nor semilunar valve insufficiency at 3 years' follow-up. CONCLUSIONS: Pulmonary autografts can be used in combination with resection of subaortic tissue or a septal incision for reconstruction of complex left ventricular outflow tract obstruction. This technique renders excellent short term valve function, relief of obstruction, avoids anticoagulation and provides potential for future growth.

Adolescent↗

Dynamic left ventricular outflow tract obstruction due to concentric left ventricular hypertrophy: effect of pacing with a short AV interval.

Dual chamber pacing with a short atrioventricular (AV) interval has emerged as a novel therapeutic approach in dynamic left ventricular outflow tract obstruction. A morbidly obese 65-year-old man with previous borderline hypertension and documented normal coronary arteries and concentric left ventricular hypertrophy who underwent uneventful elective hip replacement is reported. Eight hours postoperatively the patient developed junctional tachycardiac and hypotension. Echocardiogram revealed concentric left ventricular hypertrophy with dynamic left ventricular outflow tract obstruction and peak gradient of 262 mmHg. The patient improved with intravenous fluid replacement. The gradient fell to 112 mmHg 14 days later. Twelve days later the patient developed symptomatic sinus pauses and a dual chamber pacemaker was implanted. After testing various AV intervals, the lowest gradient of 138 mmHg was associated with an AV interval of 100 ms. One year later the gradient was 37 mmHg at the same AV interval, with a higher gradient at shorter and longer AV intervals. Dual chamber pacing with a short AV interval has been associated with improvement in hypertrophic obstructive cardiomyopathy. This case suggests the benefit of this therapy may extend to acquired forms of dynamic left ventricular outflow tract obstruction such as concentric left ventricular hypertrophy.

Aged↗

Left ventricular outflow tract obstruction in children.

Left ventricular outflow tract obstruction is classified by site of presentation into valvular, subvalvular, and supravalvular forms. This disorder affects multiple age groups, and success rates of operative therapies vary dramatically. Abnormalities in any of the three principal anatomic sites of the LV outflow tract can lead to obstruction. Valvular stenosis is the most common, and when present in infants, carries a high mortality rate. Subvalvular stenosis is much less common and may present as a discrete ring or a diffuse tunnel. Supravalvular stenosis is the least common and likewise has discrete and diffuse forms. These various forms of obstruction require surgical relief. Each operation must be tailored to the individual patient and may comprise simple valvotomy for bicuspid aortic valve or complex intraventricular manipulations to ameliorate diffuse subaortic tunnel. The mortality for valvotomy alone can range from 0 to 70%, depending on the age of the patient at the time of surgery. Additionally, the possibility for recurrent stenoses after operation is always present. For these reasons, therapy and interventions directed toward relief of LVOTO must be considered palliative, and lifelong patient follow-up is necessary.

Cardiac Surgical Procedures↗

Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections.

OBJECTIVES: To determine the accuracy of prenatal diagnosis of pulmonary atresia and intact ventricular septum (PAIVS), and pulmonary stenosis, including prenatal detection of ventriculocoronary connections, to evaluate heart size during the prenatal period, and to evaluate the outcome. DESIGN AND PATIENTS: Medical records of 20 cases with prenatally diagnosed PAIVS and pulmonary stenosis were reviewed retrospectively. Prenatal and postnatal echocardiography were also reviewed and dimensions of the ventricles and vessels were measured retrespectively. RESULTS: Of 20 prenatal diagnoses (15 PAIVS and five pulmonary stenosis), 16 were confirmed as correct. One critical pulmonary stenosis case had been diagnosed as PAIVS prenatally; three had no confirmation. Eight pregnancies were terminated, three had no active treatment, and nine were treated; all survived. Of 13 assessed with ventriculocoronary connections prenatally, seven were diagnosed correctly (four with, three without ventriculocoronary connections), but one was falsely positive; five had no confirmation. The more prominent hypoplasia of the main pulmonary artery and the tricuspid valve annulus, and the sigmoid shape of the ductus arteriosus, seemed to be associated with the presence of ventriculocoronary connections. CONCLUSIONS: Current prenatal echocardiography can accurately diagnose right ventricular outflow tract obstruction and ventriculocoronary connections. Prenatal detection of this constellation of abnormalities aids in family counselling and decisions on postnatal management.

Abnormalities, Multiple↗

Relief of left ventricular outflow tract obstruction following inadvertent left ventricular apical pacing in a patient with hypertrophic cardiomyopathy.

Dual chamber (DDD) pacing improves symptoms and relieves left ventricular (LV) outflow obstruction in hypertrophic cardiomyopathy. The ventricular lead is usually positioned at the right ventricular apex (RVA). We report a patient in whom the ventricular lead had inadvertently penetrated the septum, resulting in DDD pacing from the LV apex. However, after 3 months, obstruction was reduced and symptoms were improved. Pacing from LV apex and RVA resulted in comparable hemodynamic improvement. This case suggests that the asynchronous wave of septal contraction, originating from the apex, irrespective of ventricular site, accounts for the reduction in LV outflow obstruction.

Adult↗

Dynamic right ventricular outflow tract obstruction in cardiac surgery.

BACKGROUND: Right ventricular outflow tract obstruction can be a cause of hemodynamic instability but it has not been described in non-congenital cardiac surgery. METHODS: The prevalence of right ventricular outflow tract obstruction was retrospectively studied in 670 consecutive patients undergoing cardiac surgery. Significant right ventricular outflow tract obstruction was diagnosed if the right ventricular systolic to pulmonary artery peak gradient was more than 25 mm Hg. The diagnosis was based on measurement of the right ventricular and pulmonary artery systolic pressure through the paceport and distal opening of the pulmonary artery catheter. To further validate the prevalence and the importance of right ventricular outflow tract obstruction, 130 patients were prospectively studied over a 12-month period. RESULTS: In the retrospective cohort, 6 patients (1%) undergoing various types of cardiac surgical procedures were found to have significant dynamic right ventricular outflow tract obstruction with a mean gradient of 31 +/- 4 mm Hg (26 to 35 mm Hg). In the prospective study significant dynamic right ventricular outflow tract obstruction was identified in 5 patients (4%) (average peak: 37 +/- 15 mm Hg; range: 27 to 60 mm Hg). The typical transesophageal echocardiography finding was end-systolic obliteration of the right ventricular outflow tract. In patients with significant dynamic right ventricular outflow tract obstruction, hemodynamic instability was present in 10/11 patients (91%). CONCLUSIONS: Right ventricular outflow tract obstruction is easily diagnosed using the paceport of the pulmonary artery catheter and should be considered as a potential cause of hemodynamic instability especially when transesophageal echocardiography reveals systolic right ventricular cavity obliteration.

Aged↗

The modified Konno procedure for complex left ventricular outflow tract obstruction.

BACKGROUND: Complex left ventricular outflow tract (LVOT) obstruction with normal aortic valve function requires aggressive resection in the subaortic region and preservation of the aortic valve. The modified Konno procedure allows generous exposure of the LVOT from the left ventricular apex to the inter leaflet trigones of the aortic valve. Widespread use of this procedure has been limited by concern over injury to the aortic valve, the conduction system, and possibility of residual ventricular septal defect (VSD). METHODS: Retrospective analysis of pertinent data for all patients undergoing the modified Konno procedure (1994 to 2001) at the University of Iowa were reviewed. RESULTS: The modified Konno procedure was used in 18 patients (age 1 to 31) for LVOT obstruction associated with diffuse narrowing of the LVOT (n = 7), a discrete fibrous ring (n = 7), or a fibrous ring associated with abnormal mitral attachments (n = 4). Eight patients had previously undergone LVOT resection. There were no perioperative deaths. Estimated LVOT peak gradients by echocardiogram were 70.4 +/- 24.2 mm Hg (preoperative) and 19.2 +/- 20.4 mm Hg (postoperative) at most recent followup (p < 0.001 vs preop). Aortic insufficiency was moderate in one patient (present preop) and mild or less in all other patients. There were no cases of permanent heart block. Small residual VSDs were present in five patients (28%). Median follow-up is 3.1 years. CONCLUSIONS: The modified Konno procedure can effectively relieve complex LVOT obstruction and preserve aortic valve function. Extension of this procedure for use in the initial presentation of LVOT may be appropriate in cases at increased risk of recurrent LVOT obstruction.

Adolescent↗

Double outlet right ventricle with severe left ventricular outflow tract obstruction due to small ventricular septal defect and anomalous adherence of the mitral valve to the ventricular septum.

A 1-month-old male infant with a double outlet right ventricle complicated by left ventricular outflow tract obstruction due to a very small ventricular septal defect is described. The atrial septum was intact, but mitral stenosis was present. The characteristic finding in this patient was that the inherently small ventricular septal defect was further narrowed by abnormal adherence of the anterior mitral leaflet to the left ventricular septal surface below the defect. Intracardiac anatomical features other than the adherent mitral valve could be evaluated accurately by two-dimensional echocardiography. Because the atrial septum was intact, balloon atrioseptostomy was not possible. An attempt at surgical creation of an atrial septal defect was unsuccessful, and the patient died immediately after the operation. The autopsy findings of the heart are described, and diagnostic problems and the possibility of radical operation are discussed.

Autopsy↗

Anomalous papillary muscle as a cause of left ventricular outflow tract obstruction in an adult.

Left ventricular outflow tract obstruction may be caused by abnormalities of the various structures comprised by the outflow tract. Hypertrophic cardiomyopathy is one of the more common causes, but many are rare anomalies, a collection of which we have compiled. We present a case of left ventricular outflow tract obstruction mimicking aortic stenosis in an adult. This was found to be due to abnormal insertion of a hypertrophied papillary muscle, successfully corrected by mitral valve replacement.

Aged↗