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PubMed1991

[Double subvalvular aortic stenosis (fixed and dynamic) diagnosed by ECHO-Doppler].

Fixed subvalvular aortic stenosis is an uncommon cause of left ventricular outflow obstruction which occasionally can be found in association with dynamic muscular obstruction. In the past, the diagnosis of this association has been difficult and relied upon cardiac catheterization and angiography. To our knowledge, no case of this pathology diagnosed by echo and Doppler has previously been reported. We present a case of double subvalvular aortic stenosis in a 35 years old patient which illustrates the feasibility of detection with echo and Doppler of both types of subvalvular aortic obstruction.

Adult↗
PubMedDate not supplied

Tetralogy of Fallot with complete atrioventricular canal: a report of successful surgical treatment.

A case of successful surgical repair of tetralogy of Fallot with complete atrioventricular canal in 4 year old female child is reported. Double patch technique and combined right atrial and right ventricular (RV) approach were used to repair this defect. The ventricular patch was tailored wide and redundant anteriorly in the form of a sail to prevent subaortic obstruction. Right ventricular outflow tract obstruction was relieved through an infundibular incision with subsequent use of subannular pericardial patch. The relevant literature on the management of this rare anomaly is briefly reviewed.

Child, Preschool↗
PubMed1990

[Primary hypertrophic cardiomyopathies. Diagnosis and treatment].

Hypertrophic cardiomyopathy (HCM) is currently considered to be a condition characterised by abnormal diastolic function, sometimes associated with left ventricular outflow obstruction which appears to be a secondary phenomenon. It is important to avoid overlooking this diagnosis especially in young subjects, because of the risk of sudden death, but conversely, false positive diagnosis can result in a serious social and psychological handicap. Echocardiography has assumed a major role in the diagnosis and follow-up of HCM over the last decade. It allows analysis of the localisation, extension and severity of the septal hypertrophy and of the mechanism of systolic anterior motion of the mitral valve. Cardiac doppler completes the non-invasive assessment of this condition by demonstrating the presence or absence of outflow tract obstruction, evaluating diastolic dysfunction, and documenting mitral and occasionally aortic valve regurgitation. Only severely symptomatic patients who do not respond to high dose medical therapy and for whom surgery might be an option need cardiac catheterization. Strenuous physical activity and sport are not recommended in asymptomatic forms. It is vital to identify those patients at high risk of sudden death (family history of sudden death, 48 to 72 hour Holter monitoring). In the absence of silent arrhythmias, preventive therapy should be more general in patients under 30 years of age. Despite a better understanding of the disease, the indications of medical and surgical treatment remain controversial and poorly defined. Treatment is mainly medical with Propranolol as the drug of choice. The dosage has to be tailored for each individual patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiomyopathy, Hypertrophic↗
PubMedDate not supplied

[Exercise 2D-Doppler echocardiography, for the evaluation of aortic valve prostheses].

STUDY OBJECTIVE: Exercise evaluation of eccentric monocuspid aortic prosthetic valves. DESIGN: Prospective study, using 2D Doppler echocardiography to analyse resting and exercise aortic flow patterns. SETTING: Laboratory of Echocardiography at Santa Marta Hospital. PATIENTS: Sequential sample of 28 patients with aortic prosthesis (19 Bjork-Shiley and 9 Hall-Kaster), without evidence of either cardiac failure, coronary artery disease, prosthesis dysfunction or stress test contraindication. INTERVENTIONS: Transaortic basal and exercise flow record, using Doppler echocardiography to analyse: instantaneous peak velocity (pv) and gradient (pg), systolic time intervals, heart rate (hr) and stress test time (stt). The patients were divided in two groups A (prosthesis valve size 19 and 21) and B (greater than 21). RESULTS: Technical success: 84%. basal pv ranged from 1.6 to 4.5 m/s in group A and 1.7 to 3 m/s in B. Rest pg was 35 +/- 1% mmHg--Group A--and 20 +/- 8 mmHg in B (p less than 0.01), increasing with stress test respectively to 62 +/- 26 (p less than 0.01) and 36 +/- 12 mmHg (p less than 0.001). 73% of group A patients had exercise pg greater than 50 mmHg. The correlation between basal and exercise pg was significant (r = 0.91 p less than 0.001). At rest the systolic time intervals revealed a significant difference between the two groups (p less than 0.05), while only the ejection time was significant with stress test (p less than 0.01). Exercise hr and stt were greater in group B-p = NS. CONCLUSIONS: These exercise Doppler echocardiography data suggest that mechanical moncuspid aortic prosthesis 19, 21 and same 23, result in left ventricular outflow obstruction mainly during exercise; these facts should be taken into account when aortic prosthesis insertion is considered. Given the good correlation between resting and exercise peak gradient, Doppler stress test is not routinely recommended in clinical practice.

Adult↗
PubMedDate not supplied

The role of beta blocker in the post operative low output state following adequate valvotomy in cases of pure valvar pulmonary stenosis (clinical experience).

The role of infundibular resection in cases of pure valvar pulmonary stenosis is much debated. Some advocate aggressive approach especially in cases of supra systemic right ventricular pressures. Some consider it unnecessary, and feel that it is secondary hypertrophy which will regress in due course following a successful valvotomy. But all agree that the dynamic right ventricular outflow obstruction plays a very crucial role in the immediate post operative period which can cause significant morbidity and mortality. We advocate the use of beta blocker to relieve the infundibular spasm in the immediate post operative period, thereby relieving the dynamic outflow obstruction. We herewith present two cases to exemplify this.

Adolescent↗
PubMed1989

[Possibilities of echocardiographic evaluation of hemodynamic disorders in hypertrophic cardiomyopathy].

In a group of 59 patients with hypertrophic cardiomyopathy relationship between echocardiographic parameters (interventricular septal thickness, left ventrical diastolic diameter, presence of SAM, distribution of hypertrophy) and certain hemodynamic measurements (diastolic compliance, left ventricular end-diastolic pressure, intraventricular pressure gradient, mean wall thickness and left ventricular mass index) were assessed. Substantial elevation of left ventricular end-diastolic pressure (LVEDP greater than 20 mmHg) was significantly more prevalent among patients with small left ventricular diameter and gross septal hypertrophy. Extensive ventricular hypertrophy (Maron type III) was not characterized by any distinctive hemodynamic pattern. Presence of SAM reaching interventricular septum was indicative of left ventricular outflow obstruction.

Adolescent↗
PubMed1987

Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options.

Anomalous origin of the left anterior descending coronary artery from the right coronary artery can interfere with the location of the usual ventriculotomy during repair of tetralogy of Fallot. The purpose of this study was to compare the results of two operative techniques: (1) a "tailored" right ventricular incision and outflow patch reconstruction and (2) placement of a conduit from the right ventricle to the main pulmonary artery. We reviewed the records of 416 patients who had complete repair of tetralogy of Fallot at the Mayo Clinic from 1973 through 1984. Twenty (5%) (median age 6.5 years) had anomalous origin of the left anterior descending coronary artery from the right coronary artery. Twelve of these patients had right ventricular outflow patch reconstruction, and eight had placement of a conduit from the right ventricle to the pulmonary artery. Three deaths occurred, all during hospitalization, two in the patch reconstruction group and one in the conduit group. The average reduction in right ventricular pressure postoperatively was slightly but not significantly greater for the conduit group. These data indicate that correction of tetralogy of Fallot with anomalous origin of the left anterior descending coronary artery can be done with either patch reconstruction or conduit placement. Selection of the more appropriate procedure depends on the exact location and degree of tortuosity of the anomalous artery and the level and severity of right ventricular outflow obstruction.

Blood Vessel Prosthesis↗
PubMed1986

Myectomy versus myotomy as an adjunct to membranectomy in the surgical repair of discrete and tunnel subaortic stenosis.

The results of membranectomy and deep myectomy in the left ventricular outflow tract were compared to those of membranectomy and myotomy in 42 patients who underwent surgical repair of discrete and tunnel subaortic stenosis. Fifteen consecutive patients (Group A) underwent membranectomy and myotomy, and 27 consecutive patients (Group B) underwent membranectomy and myectomy. Two patients of Group A and nine of Group B had tunnel subaortic stenosis. The preoperative mean (+/- standard deviation) peak systolic gradients across the left ventricular outflow tract in patients with discrete subaortic stenosis types I and II were 64 +/- 29 mm Hg in Group A and 52 +/- 3 mm Hg in Group B (p = not significant). In the patients with tunnel subaortic stenosis the preoperative mean gradients were 97 +/- 74 mm Hg in Group A and 73 +/- 26 mm Hg in Group B (p = not significant). In patients with discrete subaortic stenosis types I and II, postoperative catheterization at a mean follow-up of 21 months revealed residual mean gradients of 29 +/- 24 mm Hg in Group A and 10 +/- 13 mm Hg in Group B (p less than 0.01). In the patients with tunnel subaortic stenosis, the postoperative mean gradients were 25 +/- 7 and 30 +/- 30 mm Hg in Groups A and B, respectively (p = not significant). We conclude that in the surgical management of discrete subaortic stenosis types I and II, deep myectomy (in addition to membranectomy) produces better relief of the left ventricular outflow obstruction than do membranectomy and myotomy. In patients with tunnel subaortic stenosis myectomy is less effective than in the non-tunnel type but still produces acceptable results and may delay radical procedures to a later age.

Adolescent↗
PubMed1983

Verapamil: an effective calcium blocking agent for pediatric patients.

Verapamil is a slow-channel calcium-blocking agent that has been released recently by the Food and Drug Administration for treatment of cardiac dysrhythmias in all age groups. Its primary action is to slow conduction in the atrioventricular node, thereby abolishing those types of supraventricular tachycardia using the atrioventricular node as a part of the reentry circuit or slowing the ventricular rate in someone with atrial flutter. Other investigations have shown that it is likely to relieve left ventricular outflow obstruction in patients with hypertrophic obstructive cardiomyopathy. Because of its potential widespread usefulness in the pediatric population, all pediatricians should be more aware of how it is used and the potential hazards. The methods of administration and treatment of overdoses as well as indications for usage, contraindications, and adverse reactions will be explained.

Administration, Oral↗
PubMed1980

Primary cardiomyopathies: classification, pathophysiology, clinical recognition and management.

In recent years, the definition of cardiomyopathy has been restricted to the idiopathic forms of myocardial disease and has been grouped into three general categories: (1) congestive or dilated cardiomyopathy, (2) hypertrophic cardiomyopathy, and (3) restrictive cardiomyopathy. The history and physical examination and echocardiography appear to be the most helpful in the clinical recognition of these disorders. In general, the prognosis of patients with congestive cardiomyopathy is guarded. Their survival primarily depends on the natural history and progression of the disease as well as the response to treatment with anticongestive drugs. The recent advent of vasodilator therapy has provided additional help to the patient with refractory congestive heart failure. Hypertrophic cardiomyopathy appears to be much more frequently recognized since the advent of echocardiography. The young patient has a much higher incidence of serious complications (combined right and left ventricular outflow obstruction, more generalized hypertrophic cardiomyopathy, and sudden death), and the patient presenting in later life appears to have fewer associated complications. There is a familial association with hypertrophic cardiomyopathy, although the true incidence and full expression of the association is not well understood at present. The majority of adult patients acieve prolonged survival with medical and/or surgical treatment. The restrictive cardiomyopathies are probably the least well understood. All three subgroups (Löffler's endocarditis, primary restrictive cardiomyopathy, and endomyocardial fibrosis) have a guarded prognosis. The acute form (Löffler's endocarditis) is characterized by a debilitating illness with death usually within a couple of months; however, a small percentage of these patients can have long survival and may actually evolve into a more chronic form, i.e., primary restrictive cardiomyopathy or endomyocardial fibrosis. The latter two restrictive myopathies usually have a slow indolent course, and their prognosis is primarily determined by the amount of myocardial damage and endocardial fibrosis.

Adolescent↗
PubMedDate not supplied

[The surgical treatment of fixed aortic subvalvular stenosis].

Over the past 5 years, 45 patients (11 adults and 33 children) have undergone operations for discrete and fixed subaortic stenosis. The resection of the subvalvular membrane or the fibromuscular collar was the procedure of choice. 28 patients underwent myectomy and/or myotomy. None patients died during operations. No significant symptoms and gradients remained after operation. We conclude that in the surgical management of fixed discrete subaortic stenosis myectomy and myotomy in addition to membranectomy produces better relief of the left ventricular outflow obstruction than do membranectomy alone.

Adolescent↗
PubMed1994

[Successful aortic root replacement with pulmonary autograft in two infants].

Two infants with congenital aortic valve stenosis underwent successful aortic root replacement with pulmonary autograft. One of the patients was 9 months and weighted 4.2 kg, and the other was 10 weeks weighing 4.6 kg. The former had undergone balloon valvotomy with a resultant severe aortic regurgitation. Operative method was basically similar to that taught by Ross. In terms of the right ventricular outflow reconstruction, however, left atrial appendage was utilized as the posterior wall while equine pericardial monocusp patch formed the anterior wall in the former patient. In the latter, morphology of the left atrial appendage was not suitable for the same kind of reconstruction, and nonvalved equine pericardial tube was used. Both of the patients eventually gained satisfactory circulatory condition. Advantages of aortic root replacement with pulmonary autograft are good competence without residual stenosis, avoidance of anticoagulation, and the high potential of future growth. So this operation is especially indicated for neonates and infants with left ventricular outflow obstruction.

Aorta↗
PubMed1994

[Value of biplane transesophageal echocardiography in congenital abnormalities of the heart atrium and venous-atrial connection].

BACKGROUND: Transesophageal echocardiography with transversal planes offers many advantages in the evaluation of patients with congenital anomalies of atrium, allowing visualization of obscure areas, not visualized with traditional echocardiography, as appendages, venous connections, upper interatrial defects. The aim of this paper is to check what advantages the transesophageal imaging in longitudinal plane, recently insert in biplane probes, might confer over transversal plane imaging in the evaluation of patients with congenital heart disease of atrium and venous connections. METHODS: We carried out a prospective study on 70 patients (49 adults and 22 children) with congenital heart diseases of this area. Fifty-six studies out of 71 were preoperative diagnostic, 15 were carried out in the late postoperative period. Twenty-two studies were performed under general anaesthesia during concomitant cardiac catheterisation, 49 were carried out in outpatient clinic. All the transesophageal diagnoses were subsequently confirmed at either catheterization or surgery. Lesions studied included 36 atrial septal defects (ASD) ostium secundum, 6 interatrial septal aneurysms, 1 ASD coronary sinus, 5 ASD venous sinus, 5 partial and 2 complete atrioventricular septal defects, 1 double outlet right ventricle; in operated patients 2 ASD ostium secundum, 2 ASD sinus venosus, 11 post Mustard or Senning in TGA. RESULTS: Anomalies either better defined or that obtained important additional informations by long axis scanning (vs transverse scanning) included: ASD ostium secundum 36/36, ASD multiple 1/3, caval obstruction in Mustard or Senning 4/5, anterior mitral valve cleft 2/5, left superior vena cava to coronary sinus 3/3. Features visualized by longitudinal plane alone were: ASD coronary sinus 1/1, caval obstruction in sinus venosus operated 1/1, multiple ASD 2/3, coronary artery fistula to right atrium 1/1; for associated features anterior bringing leaflets 2/2, left ventricular outflow tract obstruction 2/2, right ventricular outflow tract obstruction 1/1. Anomalies better characterized by transversal plane versus longitudinal plane were: ASD ostium primum 2/7, ASD sinus venosus sinus 3/5; for associated anomalies VSD inlet 1/1. Features visualized by transversal plane alone were: right upper pulmonary vein in superior vena cava 4/4, right upper pulmonary vein to right atrium 1/1, DIA ostium primum 5/7, ASD sinus venosus 2/5, superior vena cava obstruction in Mustard 1/5, left juxtaposition of the atrial appendages 1/1. The transversal plane alone provided the correct morphologic and hemodynamic diagnosis in 74/88 (84%) (in this group longitudinal plane gave 68% additional informations), the longitudinal planes in 69/88 (78%). However in 11% of cases longitudinal plane was the only means for identifying the congenital lesion and transversal plane in 16%. It was the combination of longitudinal and transverse scanning which gave in 97% the correct complete morphologic diagnosis. CONCLUSIONS: Thus we conclude that both planes are required for optimal transesophageal evaluation of congenital disease of atrium and venous connection.

Adolescent↗
PubMed1993

Surgical treatment of infantile cardiac tumor: report of a case.

We herein report on a male infant who was diagnosed as having a cardiac tumor at the age of four days. This baby was quite well after birth, with the exception of a heat murmur heard by the pediatrician in a routine physical examination. Echocardiograms disclosed a tumor located in the muscular portion of the interventricular septum, almost occupying the whole right ventricular chamber. The patient underwent near total excision of the tumor to relieve the right ventricular outflow obstruction when he was 54 days old. The pathologic report revealed a rhabdomyoma.

Heart Neoplasms↗
PubMed1993

Isolated lung transplantation for patients with Eisenmenger's syndrome.

BACKGROUND: Eisenmenger's syndrome remains one of the greatest challenges in lung transplantation. METHODS AND RESULTS: Since October 1990, seven such patients with Eisenmenger's syndrome received isolated pulmonary grafts (six double lungs and one single lung). Mean patient age was 32 +/- 6 years (two men and five women). The preoperative mean pulmonary arterial pressure was 90.7 +/- 31.2 mm Hg, and the ventriculoscintigram showed markedly enlarged right ventricle and normal left ventricular function with ejection fraction of 0.660 +/- 0.115. Three atrial septal defects and four patent ducti arteriosus were repaired concomitantly. Excised lung histology showed plexogenic pulmonary arteriopathy with Heath-Edwards' grade 4 through 6. One double lung patient who had preexisting systemic vascular collapse died intraoperatively. The other six patients tolerated transplantation, and on the first operative day, mean pulmonary artery pressure decreased to 22.4 +/- 7.3 mm Hg (P < .002) and gas exchange was acceptable with an arterial/alveolar oxygen tension ratio of 0.47 +/- 0.15. Two patients died of mediastinal and pulmonary infection. The follow-up for the four survivors ranged from 13 to 25 months after transplantation. CONCLUSIONS: Our preliminary experience shows that concomitant isolated lung transplantation with cardiac repair could be a viable therapeutic option for patients with Eisenmenger's syndrome and normal left ventricular function. Dynamic right ventricular outflow obstruction is a potential hemodynamic problem in these pulmonary recipients.

Adult↗
PubMed1993

[Taussig-Bing malformation with straddling of both atrioventricular valves. Echocardiographic description of a case].

The authors report the case of an infant with the Taussig-Bing malformation and straddling of the two atrioventricular valves, the diagnosis of which was made by echocardiography and confirmed at surgery. Anatomic repair of the transposed great arteries was preceded by reconstruction of the interventricular septum which left the abnormal tricuspid subvalvular apparatus in the right ventricle and the abnormal mitral subvalvular apparatus in the left ventricle. Postoperative Doppler echocardiography confirmed the absence of left ventricular outflow obstruction.

Double Outlet Right Ventricle↗
PubMed1993

Congenital heart disease and Robinow syndrome.

We present a 6-month-old infant with Robinow syndrome and tricuspid atresia (type Ia). Up to now, at least 67 cases of Robinow syndrome have been published including nine cases with a congenital heart defect (CHD). As in our case, the majority of the CHD were right ventricular outflow obstruction. Robinow syndrome with CHD is not rare and in all cases of Robinow syndrome detailed cardiologic evaluation and echocardiography should be performed.

Abnormalities, Multiple↗
PubMed1977

Primary myocardial disease in the cat. A model for human cardiomyopathy.

Thirty-four cats with primary myocardial disease were studied. The cats were divided into two groups, depending on the clinical, hemodynamic, angiocardiographic, and pathologic findings. Group A consisted of those cats with hypertrophic cardiomyopathy and Group B consisted of those cats with congestive cardiomyopathy. Similarity in the characteristics of cardiomyopathy in the human cat was found. Both Group A and Group B consisted predominantly of mature adult male cats. The most common presenting signs were dyspnea and/or thromboembolism, systolic murmurs with gallop rhythms on auscultation, cardiomegaly with (Group A) or without (Group B) pulmonary edema, abnormal electrocardiograms, elevated left ventricular end diastolic pressures, and angiocardiographic evidence of mitral regurgitation with left ventricular concentric hypertrophy (Group A) or left ventricular dilatation (Group B). Some cats in Group A also had evidence of left ventricular outflow obstruction. The principal pathologic findings in these cats were left atrial dilatation, symmetric hypertrophy or asymmetric septal hypertrophy of the left ventricle (Group A), and dilatation of the four cardiac chambers (Group B). Aortic thromboembolism was commonly observed in both groups. These clinical and pathologic findings indicate that cardiomyopathy in the cat is similar to the two most common forms of cardiomyopathy in the human (hypertrophic cardiomyopathy, with and without obstruction, and congestive cardiomyopathy).

Age Factors↗