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PubMed1989

The outcome of surgical treatment of hypertrophic obstructive cardiomyopathy. Experience over 15 years.

From 1972 through 1987, 115 patients between the ages of 1 and 83 years (mean, 44.5 years) underwent operation for hypertrophic obstructive cardiomyopathy. Methods of relief of left ventricular outflow obstruction were septal myectomy/myotomy (n = 109), mitral valve replacement (n = 4), and myectomy/myotomy plus mitral valve replacement (n = 2); concomitant procedures included coronary artery bypass (n = 19) and aortic valve replacement (n = 9). Systolic gradient (peak-to-peak) from the left ventricle to the aorta decreased from 70 +/- 38 mm Hg (mean +/- standard deviation) to 9 +/- 11 mm Hg. There were six hospital deaths, for an overall operative risk of 5.2%; one death occurred among 83 patients less than age 65 years (operative risk, 1.2%), and five deaths occurred in 32 older patients (operative risk, 15.6%; p = 0.008 for difference between age groups). Four (22.2%) of 18 patients with a residual gradient greater than 15 mm Hg died, compared with two (2.1%) of 97 patients with a lower gradient (p = 0.003). Follow-up ranged from 0.5 to 16 years (mean, 5.1 years), and 5-year actuarial survival rate, including hospital deaths, was 84% +/- 4%. The 5-year survival rate was decreased in patients who had operative procedures other than myectomy/myotomy (69% versus 91%, p less than 0.005) and in patients aged 65 years or older (54% versus 93%, p less than 0.005). No correlation was found between preoperative symptoms, functional class, left ventricle-aorta pressure gradient, or mitral valve insufficiency and operative or late mortality. Preoperative symptoms were relieved in 57 (76%) of 75 patients with dyspnea, 49 (83%) of 59 patients with angina, and 22 (96%) of 23 patients with syncope. This experience confirms the effectiveness of operation for relief of symptoms in patients with the obstructive form of hypertrophic cardiomyopathy. The current operative mortality rate is low, especially in patients less than 65 years of age (1.2%). Our experience suggests that incomplete relief of left ventricular outflow obstruction may increase the risk of early postoperative death.

Adolescent↗
PubMed1993

Left ventricular outflow tract obstruction following repair of pneumococcal mitral annular abscess.

An unusual case of a mitral annular abscess caused by Streptococcus pneumoniae was diagnosed by transesophageal echocardiography. The patient underwent surgical resection of the abscess and developed outflow tract obstruction. This is an unusual complication of the surgical procedure. The outflow tract obstruction may have been due to anterior displacement of the mitral valve by the abscess.

Abscess↗
PubMed2001

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

Aneurysm of the sinus of Valsalva (ASV) is a rare anomaly. It may be congenital or acquired basis. In pediatric patients, most are congenital in origin. It is frequently associated with other congenital disease, most frequently ventricular septal defect (VSD). Pathologically, this is due to the total absence of normal elastic fibers. Patients with ASV are generally asymptomatic unless aneurysm rupture occurs. Unruptured ASV with right ventricular outflow tract (RVOT) obstruction is very rare. Herein we describe an unusual ASV with RVOT obstruction associated with VSD in a 14-year-old boy. He had experienced heart failure and exertional dyspnea for 2 months. The diagnosis was made by echocardiography, angiography and surgery. After surgical repair of the VSD and resection of the ASV, dyspnea and heart failure were relieved. The patient remained well during the 3 years of follow-up.

Adolescent↗
PubMed1975

Pathologic anatomy of the cardiomyopathies. Idiopathic dilated and hypertrophic types, infiltrative types, and endomyocardial disease with and without eosinophilia.

This presentation summarizes necropsy observations in patients with three types of cardiomyopathy: idiopathic, infiltrative, and endomyocardial disease. The idiopathic variety is subdivided into two types depending on the size of the ventricular cavity. In the dilated ventricular type the left ventricular wall is frequently less than 1.5 cm. thick, intracardiac thrombi are common, the atrioventricular valve rings usually are mildly dilated, and focal myocardial and endocardial scars are common. In the nondilated type (hypertrophic cardiomyopathy), the ventricular septum is usually thicker than the left ventricular free wall, which also is thick (greater than 1.5 cm.). When the septum is similar in thickness to the left ventricular free wall (symmetric), left ventricular outflow obstruction does not occur. When the septum is thicker than the left ventricular free wall (asymmetric), left or right ventricular outflow obstruction may or may not be present. The orientation of myocardial fibers one to another in the ventricular septum in the nondilated (hypertrophic) type is abnormal, whereas it is normal in the dilated ventricular type. Intracardiac thrombi are rare and atrioventricular valve rings are never dilated in the nondilated type of idiopathic cardiomegaly. The infiltrative types of cardiomyopathies include iron, calcium, lipids, mucopolysaccharides, granulomas, amyloid, and neoplasms. The first four usually are located within myocardial cells and the latter three, between myocardial cells. It is probable that all these myocardial infiltrates are capable of producing cardiac dysfunction, primarily on a restrictive basis. Endomyocardial disease may or may not be associated with eosinophilia. When the latter occurs, the eosinophils are structurally normal. Death is related to congestive cardiac failure. This category is actuality also in idiopathic.

Adolescent↗
PubMed1989

[Surgical mitral valvuloplasty and dynamic left ventricular obstruction syndrome].

The "dynamic" left ventricular outflow obstruction syndrome, which is found in 4.5 to 11 p. 100 of patients who underwent mitral valve repair by Carpentier's technique, seems to be due to the association of two different sets of circumstances. The first set is anatomical; the syndrome is thought to result from the implantation of a semi-rigid valve ring, from the excessive length of the mitral valve system in relation to the size of the ventricular cavity, and from excessive correction of mitral regurgitation with subsequent excessive reduction in caliber of the left ventricular outflow tract, change in the interrelation between outflow tract and ventricular filling and strongly anterior position of the mitral valve. The second set is functional; it is due to a more than 20 p. 100 reduction in caliber of the left ventricular outflow tract on the one hand, and to a more than 2.5 m/s increase in blood flow velocity on the other hand. These two phenomena may be produced by a decrease in left ventricular volume as a result of hypovolaemia, tachycardia or more rapid circulation. Treatment of the syndrome consists of correcting the associated abnormalities by prescribing a diet with a normal sodium content, together with an appropriate anti-arrhythmic agent and a beta-blocker.

Adult↗
PubMed1980

Left ventricular outflow tract obstruction due to an aneurysm of the membranous septum in a patient with pulmonary atresia and ventricular septum defect - case report.

Although the occurrence of subpulmonary stenosis resulting from displacement of an aneurysm of the membranous septum is well known, resultant subaortic stenosis has not been well documented. We present an unusual case of acquired pulmonary atresia with restrictive ventricular septal defect and associated severe subaortic resulting from an aneurysm of the membranous septum.

Abnormalities, Multiple↗
PubMed2006

"Isolated" diastolic dysfunction in left ventricular outflow tract obstruction.

We sought to investigate the prescence of "isolated" diastolic disease, defined as reduced long-axis early diastolic velocity with normal systolic velocity, in 21 young patients with left outflow tract obstruction. Most patients had depressed systolic velocities despite normal ejection fractions. The close relations amond long-axis velocities suggest isolated diastolic disease is unlikely.

Adult↗
PubMed1996

Acute myocardial infarction with dynamic outflow obstruction precipitated by intra-aortic balloon counterpulsation.

Dynamic left ventricular outflow obstruction is associated with structural findings of asymmetric septal hypertrophy (less commonly concentric left ventricular hypertrophy) and systolic anterior motion of the anterior mitral valve leaflet. A patient who did not have this usual substrate for outflow obstruction presented with an acute anterior wall myocardial infarction complicated by congestive heart failure and cardiogenic shock. When an intra-aortic balloon pump was placed, the patient rapidly deteriorated and a dynamic outflow gradient was detected.

Electrocardiography↗
PubMed2004

Left ventricular outflow tract obstruction due to anomalous insertion of papillary muscle.

A 56-year-old man who complained of quadrantic hemianopsia was admitted to determine its etiology. Cerebral angiography revealed no organic stenosis. Echocardiography showed clear direct continuity between a hypertrophied anterolateral papillary muscle and the anterior mitral leaflet, and the left ventricular (LV) outflow tract (LVOT) was narrowed by the presence of an accessory papillary muscle. The LVOT obstruction caused an intra-LV pressure overload that resulted in LV concentric hypertrophy. Arrhythmia, such as paroxysmal atrial fibrillation (PAF), was thought to have caused a cerebral embolism. Mitral valve replacement (MVR), septal myectomy, and myectomy of the abnormal papillary muscle were performed, and complete release of the LVOT obstruction was accomplished. Anomalous insertion of papillary muscle is a rare cause of LVOT obstruction. Echocardiography was useful in identifying the papillary muscle malformation, and surgery was completely curative.

Cerebral Infarction↗
PubMedDate not supplied

Giant pulmonary artery aneurysm with right ventricular outflow tract obstruction.

Aneurysm of the main pulmonary artery is rare. Its natural history is not well understood and there are no clear guidelines regarding its optimal treatment. We present a case of a huge saccular aneurysm of the main pulmonary artery which was associated with infundibular and valvular pulmonary stenosis. It was repaired using a pericardial patch with concomitant pulmonary valvotomy and infundibular resection. Postoperative recovery was uneventful and the patient is doing well. Follow-up echocardiogram revealed good repair.

Aneurysm↗
PubMed1981

Conduit repair for complex congenital heart disease with pulmonary atresia or right ventricular outflow tract obstruction. part I: surgical results.

From 1972 to 1981, 40 valved extracardiac conduits have been implanted to bridge the right ventricular outflow tract (RVOT) in 37 patients for different complex congenital cardiac anomalies. The patients ranged in age from 2 to 23 years (mean 8.5 years). Two Dacron prostheses with incorporated Lillehei-Kaster valves, 1 valveless Dacron conduit, 27 Hancock conduits, and 9 lonescu-Shiley conduits were chosen according to availability and the progress in conduit technology. Thirty-three patients had had up to 4 previous palliative procedure before conduit repair. Although the complication rate appears considerable, the mortality rate seemed to be unaffected by the high percentage of prior surgery. Exchange of the conduit became necessary in 2 children, because of conduit stenosis with calcification of the Hancock valve 5 and 7 years respectively after implantation, and in another patient an outgrown Lillehei-Kaster valve Dacron conduit had to be replaced 9 years after repair. Five children died in the early postoperative course: one in pulmonary failure and kinking of the conduit; one with right heart failure and pulmonary hypertension stage IV; 2 in myocardial failure, and one due to severe bleeding from myocardial failure, and one due to severe bleeding from the prosthesis. Three patients died in the late postoperative course 11 weeks, 4 years and 4 years respectively after conduit repair. Conduit surgery offers new possibilities for repair of complex cyanotic cardiac malformations. At present, however, none of the commercially available conduits is an ideal substitute. Our limited experience with the hemodynamic results of lonescu Shiley conduits is promising although longer observation periods are needed for a definitive judgement. At present, the largest possible conduit should be used whenever possible; problems of compression or kinking can be avoided with proper positioning of the conduit.

Adolescent↗
PubMed2000

[Modified extended septoplasty for left ventricular outflow tract obstruction after repair of double-outlet right ventricle report of a case].

A three-year-old boy with residual VSD and LVOTO after repair of double outlet right ventricle underwent modified extended septoplasty successfully. Preoperative left ventriculography showed a residual VSD and LVOTO. The peak systolic pressure gradient between the left ventricle and aorta was 38 mmHg. Operation aimd not only at relief of the obstruction, but also at streamlining of the LVOT by removal of protrusions. We modified the extended septoplasty reported by Belli et al in 1996. The postoperative course was uneventful and patient was discharged at 15 postoperative day. The most recent echocargiogram revealed no residual LVOTO.

Cardiac Surgical Procedures↗
PubMed1994

Left ventricular outflow tract obstruction defined by active three-dimensional echocardiography using rotational transthoracic acquisition.

A rotational data acquisition system was used to create three-dimensional images from thoracic and subxiphoid echocardiographic windows in children with various types of subaortic stenosis. Thirteen patients, ranging in age from 2 days to 17 years, were examined. Subaortic obstruction was caused by a discrete fibrous ridge in six patients, hypertrophic cardiomyopathy in two patients, subaortic tunnel in two patients, and septal malalignment, restrictive VSD, and abnormal suture placement each in one patient. Unique views could be obtained equivalent to surgical or autopsy dissections, and allowed more complete understanding of morphology than conventional imaging techniques.

Adolescent↗
PubMed1987

Transposition of the great arteries with aortic arch obstruction. Anatomical review and report of surgical management.

Transposition of the great arteries (S,D,D) is a common congenital cardiovascular malformation that is occasionally associated with ventricular septal defect and left ventricular outflow tract obstruction. Recently right ventricular outflow tract obstruction associated with an aortic arch anomaly has been recognized as an infrequent but important variant of transposition of the great arteries, and this constellation presents a unique surgical challenge. Five infants with this constellation whose systemic circulation was dependent on flow through the ductus arteriosus have undergone definitive surgical treatment with four survivors. An anatomical review of 129 specimens with transposition of the great arteries revealed that 17% had right ventricular outflow tract obstruction and 7% had associated aortic arch obstruction as well. All specimens with aortic arch obstruction and ventricular septal defect except one had a malalignment type ventricular septal defect. Although several treatment options may be considered, recent experience with arterial switch repair in the neonate with transposition of the great arteries (S,D,D) prompted repair in these patients by arterial switch, ventricular septal defect closure, repair of aortic arch obstruction, and augmentation of the right ventricular outflow tract.

Aorta, Thoracic↗
PubMed1978

Electrocardiographic findings in patients with obstructive and nonobstructive hypertrophic cardiomyopathy.

One hundred and thiry-four patients with hypertrophic cardiomyopathy were evaluated by standard 12-lead electrocardiography. Normal electrocardiograms were extremely uncommon, occurring in less than 7% of each subgroup of patients (i.e., those with or without either symptoms or obstruction to left ventricular outflow), with the exception of those who were both asymptomatic and had no left ventricular outflow obstruction. Even in this subgroup, however, normal electrocardiograms occurred in only 27% of patients. Repolarization abnormalities and left ventricular hypertrophy were the most common abnormalities, occurring in 81% and 62%, respectively, of the total population. A broad spectrum of other electrocardiographic abnormalities was found, but none was unique to hypertrophic cardiomyopathy. Patients with vs those without electrocardiographic left ventricular hypertrophy or left atrial abnormality had significantly (P less than 0.005) greater mean ventricular septal thickness (22 +/- 0.6 vs 19 +/- 0.6 mm) and left atrial dimension (48 +/- 1 vs 40 +/- 1 mm) measured by echocardiography, and signficantly (P less than 0.01) higher mean pulmonary capillary wedge pressure (16 +/- 1 vs 10 +/- 1 mm Hg) and left ventricular end-diastolic pressure (20 +/- 1 vs 15 +/- 1 mm Hg). The high prevalence and diverse nature of electrocardiographic abnormalities suggest that any patient with an unusual and unexplained electrocardiogram should be suspected of having hypertrophic cardiomyopathy even if the physical examination is normal, as is often the case in patients without obstruction.

Cardiomyopathy, Hypertrophic↗
PubMedDate not supplied

Repair of tetralogy of Fallot with anomalous coronary arteries coursing across the obstructed right ventricular outflow tract.

Anomalous coronary artery (ACA) anatomy occurs in 2-9% of patients with tetralogy of Fallot (TOF), in which the left anterior descending coronary artery (LAD) originates from the right coronary artery (RCA) crossing the right ventricular outflow tract. The purpose of this study was to review our results of repair for TOF with ACA. Between 1978 and 2001, 43 ACA patients (mean age, 4.8 years; range, 5 months-41 years) underwent repair for TC. The ACA anatomy was classified as the single LAD from the RCA (n = 20), a significant conal branch (dual LAD) from the RCA (n = 13), paired anterior descending arteries originating from the left and right coronary arteries (n = 7), and single RCA from the LAD (n = 3). In cases in which the anomalous LAD crossed the obstructed infundibulum, thinning or coring of the endocardium was done. Patch infundibuloplasty was performed in 39 patients, with 10 needing separate RV and pulmonary artery patches, and the pulmonary valve was preserved. Nine patients had the addition of a monocusp to a transannular incision. Two patients had a main pulmonary arterioplasty alone. There was one early (2.3%) and no late deaths. Mean early and late postoperative gradients were 21.5 +/- 10.5 mmHg (4 patients had > or =30 mmHg) and 27.1 +/- 13.7 mmHg (5 patients had >30 mmHg; p = 0.12), respectively. There were four reoperations during a mean follow-up of 4.8 years (range, 6 months to 18 years). Actuarial freedom from reoperation was 90% at 5, 10, and 15 years. At the latest follow-up, all patients were in NYHA functional class I. TOF repair for an ACA can be performed without disturbing the native coronary anatomy and without the use of conduits in most cases. Outcomes are similar to those of other patients with TOF. The presence of ACA does not impose increased risk after this surgical strategy.

Adolescent↗
PubMed2001

Ventricular outflow tract obstruction secondary to leiomyosarcoma of the right ventricle.

Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. We report the case of a 70-year-old Belgian woman presenting with the symptoms of progressive exertional dyspnea and left-sided pleuritic pain. A leiomyosarcoma which originated from the right lateral ventricle wall, causing pulmonary outflow obstruction, was diagnosed. Pathology revealed a neoplasm with a myxoid stroma, high mitotic activity and nuclei expressing atypia. Immunohistochemical staining was positive for vimentine and desmin. Seven months after complete surgical resection the tumor relapsed. This case demonstrates the poor outcome, the high relapse rate and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding the incidence, diagnostic techniques, treatment strategies and survival rates of this rare but terminal disease is reviewed.

Aged↗