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PubMedDate not supplied

Coarctation of the aorta, aortic valvar stenosis, and supravalvar aortic stenosis with left coronary artery ostial stenosis: management using a staged hybrid approach.

In this report, we describe a case of coarctation of the aorta, congenital aortic stenosis with bicuspid valve, and supravalvar aortic stenosis with left coronary artery ostial stenosis. The child underwent staged treatment using a hybrid approach employing both surgical and interventional cardiology methods. A single pericardial patch repair technique for supravalvar aortic stenosis associated with left coronary ostial stenosis is also described.

Angioplasty, Balloon↗
PubMedDate not supplied

Elongation of the aorta and multiple cardiovascular abnormalities associated with larsen syndrome.

Larsen syndrome is a genetically heterogeneous group of disorders characterized by multiple joint dislocations and a characteristic face. We describe a girl with the typical features of Larsen syndrome. She also had associated multiple cardiovascular anomalies. The anomalies included elongation of the aorta, bicuspid aortic valve, subaortic stenosis, mitral valve prolapse with mitral regurgitation, atrial septal defect of the secundum type, and a patent ductus arteriosus.

Abnormalities, Multiple↗
PubMedDate not supplied

Turner's syndrome: cardiologic profile according to the different chromosomal patterns and long-term clinical follow-Up of 136 nonpreselected patients.

The preferential association between Turner's syndrome and congenital heart defects (CHD) have been well known since the first description by Morgagni. There are few studies about the different cardiologic problems stemming from different chromosomal patterns of X monosomies. We reviewed a large series of 136 patients with Turner syndrome without cardiologic preselection, 29 of whom had some kind of CHD (21.5%). Partial anomalous pulmonary venous drainage (PAPVD; 2.9%), aortic valve disease (stenosis and/or incompetence) (AoVD; 5. 1%), aortic coarctation (AoCo; 4.4%), and bicuspid aortic valve (BicAo; 14.7%) are much more frequent in Turner's syndrome than in the normal population, with the difference being statistically highly significant. In our cases, only the 45, X subjects showed severe CHD and multiple lesions, whereas the X-ring pattern was associated with an elevated prevalence of BicAo. Patients with X-deletion showed no signs of congenital heart malformations. Eleven patients, all with 45, X pattern, and significant CHD, underwent cardiac surgery at a mean age of 7.7 +/- 5.3 years (range 7 days-18 years) without complications. At follow-up of 3-18 years (8.6 +/- 5. 2), we were unable to observe any type of evolution of the remaining untreated cardiovascular anomalies.

Adolescent↗
PubMed1999

[Histological biomorphosis of human heart valve. II. Morphometric studies].

Previous polarization-microscopical measurements combined with silver-staining methods at histological sections of the bicuspid valve and the tricuspid valve in the region of fibrosa showed (similar to that of the corresponding tendinous cords) for middle-aged persons a distinct difference in the collagen composition in comparison between high pressure vs. low pressure system. The aim of the current study was the morphometric investigation of the collagen composition, of the relative content of total collagen (measured as relative number of total collagen fibers per measuring area), and of the cell-collagen relation in dependence of age and possibly on sex in the high and low pressure system, respectively. Tissue samples of atrioventricular valves of 67 probands of both sexes were available. The probands were healthy with regard to heart and circulation and distributed in 3 age groups (1st to 2nd decade, 3rd to 5th decade, and 6th to 9th decade). After corresponding preliminary treatment, the histological sections of tissue samples were evaluated by a suitable combination of polarization-microscopical, immuno-histochemical, and morphometrical methodology. The thickness of the heart valves was measured by microscopic image analysis. A rise of the number of fibers per measuring area (ma) for both sexes and both valves was observed with increasing age. The slopes of the linear regression curves and the mean values of the numbers of collagenous fibers/ma (for each age group) were different between both sexes. They depend upon the valve considered. Analogous to these results, the differentiated statements were possible to the number of fibers/ma and to the percentages of the fiber species by the distinction between the collagen types I and III in the fibrosa of the heart valves. Whereas the relative numbers of fibers/ma rose with increasing age, the number of cell (fibroblasts, fibrocytes) nuclei for both valves and sexes slightly decreased. For the latter, there was no difference between high and low pressure system in contrast to the collagenous fibers. The opposite age-dependent behavior of the collagenous fibers and of their producing cells during the biomorphosis of the heart valves could be explained with the collagen turn-over.

Adolescent↗
PubMed2001

[Changes in the collagen structures of human heart valves in relation to age and hypertension].

Using polarization microscopy and morphometrical methods, the influence of hypertension on the collagenous structures in the Lamina fibrosa of bicuspid valves of 14 middle-aged persons (30 to 50 years) were examined. The measurements were performed on histological sections. A group of 14 middle-aged subjects free from heart disease served as the control group. Furthermore, the results were compared to earlier findings on the histological biomorphosis of the atrioventricular valves. The following changes were observed and quantitatively determined: a) an enhancement of the percentage of the mechanically more resistant collagenous fibers (unsilvered, type I collagen), b) a stronger lateral aggregation of the collagenous fibrils of both types I and III collagen, c) a significant decrease of the total numbers of collagenous fibers per measuring area, and d) a significant decrease of the portion of silvered fibers (type III collagen) per measuring area for both sexes. The fiber density distributions confirm the observed changes: in the hypertension group the collagenous fibers are less densely distributed than in the control group. The observed regression of the content of collagenous fibers in the hypertension group is probably due to the anti-hypertensive treatment. Following the findings the turn-over of type III collagen is stronger influenced than that of type I collagen. The initial results determined in human heart valves confirm findings in animal models and characterize them with regard to the collagen types mentioned.

Adult↗
PubMed2004

[Piercing and tattoos in patients with congenital heart disease -- is it a problem?].

Piercing and tattooing enjoy widespread popularity in modern society. Patients with congenital heart disease are at elevated risk for infective endocarditis. However, it is not yet known whether piercing and tattooing are dangerous for these patients.A search of the literature provided 10 published cases of infective endocarditis after piercing or tattooing. Affected patients were adolescents or young adults ranging in age from 13 to 30 years (5 male, 5 female). Four of the patients had a known cardiac risk factor for endocarditis (bicuspid aortic valve, postoperative trans-position of the great arteries, postoperative coarctation, postoperative aortic valve stenosis). Piercing preceded endocarditis in 9 cases (4 times mouth, 2 ear, 1 nose, 1 breast, 1 navel), one tattoo. The following agents were isolated: S. aureus in 4 cases, 2 S. epidermidis, 1 Str. viridans, 1 Neisseria mucosa, 1 Haemophilus aphrophilus, 1 Haemophilus parainfluenzae. All patients were treated with antibiotics. Six patients underwent cardiac surgery (5 of them valve replacement). Patients with congenital heart disease constitute less than 1% of the population. Thus, they are clearly overrepresented in the published literature. Epidemiologic conclusions are not possible from these data. However, patients with congenital heart disease and their parents should be strongly advised against piercing and tattooing with regard to the risk of infective endocarditis.

Adolescent↗
PubMed1999

Autogenous valve reconstruction technique for post-thrombotic reflux.

Post-thrombotic reflux in deep veins of the lower extremities cannot be treated by in situ valvuloplasty because of valve degeneration. The outcome of transplantation and transposition of segments with valves is controversial. From feasibility tests in animals and fresh human cadavers we have developed an autogenous valve reconstruction technique. The valve is fashioned from the proximal end of the greater saphenous vein that is left attached to the femoral vein, invaginated, and fixed to the venous wall. This technique provides a competent bicuspid valve. In a series of 19 patients operated on in 1995 we performed 20 valve reconstruction procedures at the level of the femorosaphenous junction by invagination of a fragment from the proximal end of the greater saphenous vein in the common femoral vein. Mean follow-up time was 10 months. No complications were observed. All femoral veins were patent and competent except one in which mild reflux was observed because of insufficient valve size. Further follow-up is needed to confirm the efficacy of this simple, new technique.

Animals↗
PubMed2005

Dysfunction of an ATS valve in the aortic position: the first reported case caused by pannus formation.

A 64-year-old woman underwent aortic valve replacement with a 21-mm Advancing The Standard (ATS) open-pivot mechanical heart valve for bicuspid aortic valve stenosis. In addition to the appearance of a new cardiac murmur, echocardiography performed 3 years after surgery showed a high pressure gradient across the ATS valve and a reduction in the valve orifice area. Cineradiography of the valve revealed restricted leaflet opening. Subsequent multidetector-row computed tomography clearly demonstrated pannus overgrowth on the inflow aspect of the ATS valve. During a repeat operation, subvalvular overgrown pannus was confirmed and the ATS valve was replaced with a bioprosthetic valve. This is the first reported case of prosthetic valve dysfunction resulting from pannus formation in a patient with an ATS valve in the aortic position.

Aortic Valve Stenosis↗
PubMed2002

First definite case of aortic valve endocarditis due to Moraxella phenylpyruvica.

Described here is the first definite case of endocarditis due to Moraxella phenylpyruvica, which occurred in a 50-year-old male with a bicuspid aortic valve. The diagnosis was delayed because of the confounding positivity of the Widal and Wright tests. The patient was cured with surgical valve replacement and antibiotic treatment.

Aortic Valve↗
PubMed2003

Percutaneous transluminal balloon aortic valvuloplasty with a small balloon as a bridge to surgery for severe aortic stenosis in an 83-year-old patient.

An 83-y-old man was admitted to our hospital with pneumonia and critical cardiac failure. He had severe calcific stenosis of a bicuspid aortic valve, with a pressure gradient of about 100 mmHg, poor left ventricular function and an ascending aortic aneurysm. His family requested the surgery, but there was an unacceptably high surgical risk because of the presence of serious infection. We tried percutaneous transluminal balloon aortic valvuloplasty (PTAV) with a small sized (12 mm) peripheral percutaneous transluminal angioplasty balloon as a bridge to surgery. We managed to control his critical hemodynamics and infection after the procedure. Thereafter, he underwent successful aortic valve replacement and ascending aorta replacement. PTAV may be a useful palliative therapy, but there are various procedure-related complications. To avoid such complications, we used a small sized balloon. Actually we could not completely normalize the pressure gradient, but it was enough to control his critical cardiac failure until he could undergo surgery.

Aged↗
PubMed1975

Calcific aortic insufficiency--a review of 26 patients.

Twenty-six patients, 8.3 per cent of all patients with aortic valve disease, and 10.7 per cent of all patients with any degree of aortic insufficiency detected in our catheterization laboratory, had pure calcific aortic insufficiency (no associated stenosis). Nineteen (73 per cent) males and seven (27 per cent) females ranged in age from 25 to 75 years of age (mean 51). Twenty-three per cent were younger than 40. Sixteen (62 per cent) had rheumatic heart disease, one had luetic aortic valve disease, one had congenital bicuspid valve, and eight (31 per cent) had aortic insufficiency of undetermined etiology. Twenty-three patients (89 per cent) had an aortic systolic ejection murmur, and seven (28 per cent) had an aortic ejection click. Aortic valve calcification was detected by plain chest films in only four patients (16 per cent), and by routine image intensification fluoroscopy (before catheterization) in fifteen patients (68 per cent). The reamining 32 per cent had the calcification of the aortic valve detected during catheterization. Aortic valve calcification was severe in nine patients (35 per cent), moderate in eleven patients (42 per cent), and minimal in six patients (22 per cent). Aortic insufficiency was severe in twenty patients (77 per cent), moderate in five patients (19 per cent), and minimal in one patient (4 per cent). Nineteen patients (77 per cent) had reduced left ventricular contractility. Sixteen patients (67 per cent) had low cardiac index. Eighteen patients had obstructive coronary artery disease. Aortic stenosis was misdiagnosed as the predominant lesion in fourteen patients (54 per cent)--prior to catheterization. This series demonstrates that all patients with calcified aortic valve disease and with ejection murmurs do not necessarily have aortic stenosis. Pure calcific aortic insufficiency is a distinct entity, more common than previously suspected.

Adult↗
PubMed1979

Incidence of mitral valve prolapse in one hundred clinically stable newborn baby girls: an echocardiographic study.

Clinical and echocardiographic examinations were performed on 100 clinically stable, newborn baby girls. Mitral valve prolapse was noted on the echocardiograms of seven babies. Three subjects had systolic clicks, two of whom had systolic murmurs following the click. The four other babies who had echocardiographic evidence of mitral valve prolapse had no abnormal auscultatory signs. Of the 93 babies without evidence of mitral prolapse, 91 had normal echocardiograms and auscultatory features; one was noted to have a murmur consistent with a ventricular septal defect, and another had an eccentric aortic valve on the echocardiogram which was suggestive of a bicuspid aortic valve. Serial studies on our group of subjects will yield useful information regarding the natural history of mitral valve prolapse.

Echocardiography↗
PubMed1987

Coarctation repair without cardiac catheterization in infants.

Of 35 infants who underwent an operation for coarctation of the aorta, 15 did not undergo cardiac catheterization before surgery. The diagnoses in all uncatheterized patients were made by clinical examination, chest radiography, ECG, and two-dimensional echocardiography combined with pulsed Doppler echocardiography. Associated anomalies diagnosed by two-dimensional/Doppler echocardiography included a patent ductus arteriosus in all patients, bicuspid aortic valve in six, small ventricular septal defect in four, and complete common atrioventricular canal in one. In no instance was the diagnosis of coarctation in error. In addition, the clinical significance of the ventricular septal defect was judged correctly by two-dimensional echocardiography, and no patient required an early reoperation because of significant left-to-right shunt. Two significant lesions were missed in one patient each: mitral stenosis and aortic stenosis. These diagnoses were missed in patients who were first seen with either profound congestive heart failure or shock. Coarctation of the aorta and associated lesions can be diagnosed accurately by two-dimensional echocardiography. This permits proper patient management without the added risk of cardiac catheterization. Although mitral and aortic lesions may be missed because of low cardiac output, this does not result in management errors.

Aortic Coarctation↗
PubMed1991

Intraoperative valvuloplasty in calcific aortic stenosis: a study comparing the mechanism of a novel expandable device with conventional balloon dilatation.

In selected patients with calcific aortic stenosis, balloon valvuloplasty is an intermediate alternative to surgery. The effect of balloon valvuloplasty to increase valve area, however, is limited and the restenosis rate is high during follow-up. To improve the results and reduce the complication rate, a new device for valvuloplasty of calcific aortic stenosis was developed. This system consists of three expandable prongs mounted on a freely movable catheter tip. To evaluate the efficacy of this new device, valvuloplasty was performed in 10 patients with severe aortic stenosis intraoperatively just prior to valve replacement. Comparison was made with the results of conventional balloon dilatation performed in an additional 20 patients during surgery. Using the new device, the relative orifice area increased from 10 +/- 3% before to 20 +/- 6% following intervention. However, in only one patient was a considerable increase of static valve area (greater than 15%) found. The results were comparable to the effect of conventional balloon dilatation, which led to an increase of orifice area from 12 +/- 7% to 24 +/- 10%. With both systems, the best results were achieved in patients with aortic stenosis and significant commissural fusion. In contrast, in bicuspid or tricuspid valves without fused commissures the effect of the intervention was limited. Because complete obstruction of the aortic valve does not occur during dilatation, this new device might be superior to conventional balloon dilatation. Preselection of patients according to the morphology of the valve seems mandatory to improve the success and reduce the complication rate of valvuloplasty in aortic stenosis.

Aged↗
PubMed1993

The role of transesophageal echocardiography in cardiac donor screening.

Transthoracic echocardiography has played a useful role in the screening of cardiac transplant donors. However, transthoracic echocardiograms may be suboptimal in many patients on ventilators. The role of transesophageal echocardiography in cardiac donor screening is unknown. Therefore we compared the potential benefit of transesophageal echocardiography combined with transthoracic echocardiography in 24 (16 men and 8 women) consecutive brain-dead patients with a mean age of 29 +/- 9 years (range 16 to 44 years), who were being considered as cardiac transplant donors. Transthoracic echocardiography was performed immediately before or after transesophageal echocardiography. Transthoracic echocardiography was technically difficult in 7 of 24 (29%) patients. Results of transesophageal echocardiography were abnormal in five of the seven patients and demonstrated left (n = 4) and right (n = 3) ventricular wall motion abnormalities and concentric left ventricular hypertrophy (n = 2). The four patients with wall motion abnormalities were eliminated as potential donors. In 16 of 17 patients with technically adequate transthoracic echocardiograms, transesophageal and transthoracic echocardiographic findings agreed and demonstrated normal hearts in 13 patients, left (n = 2) and right (n = 1) ventricular wall motion abnormalities in two patients, and isolated concentric left ventricular hypertrophy in one patient. In 1 of the 17 patients with a technically adequate transthoracic echocardiographic study, a bicuspid aortic valve was demonstrated by transesophageal echocardiography but not diagnosed by transthoracic echocardiography. Overall seven patients were eliminated as cardiac donors on the basis of transesophageal echocardiograms (n = 7), transthoracic echocardiograms (n = 2), or both.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1981

Relation between aortic arch hypoplasia of variable severity and central muscular ventricular septal defects: emphasis on associated left ventricular abnormalities.

Among 25 heart specimens with a central muscular ventricular septal defect (including 3 with the architecture of a spontaneously closed defect), only 6 (24 percent) had an aortic arch of normal size and configuration. Isthmus atresia was present in one case, isthmus hypoplasia in eight cases, local coarctation in six and a combination of isthmus hypoplasia and local coarctation in another four. A study of the left ventricular morphologic features of the 25 hearts revealed the presence of a spectrum of additional anomalies. The aortic valve was bicuspid in 16 and mitral valve anomalies were present in 13 specimens. Mitral valve deformities present in the 13 specimens included congenital stenosis (5), deficient chordae tendineae (4), parachute valve (2), adherent valve leaflets (1) and a hypoplastic posteromedial papillary muscle (1). Furthermore, there was a large incidence of abnormal left ventricular muscular structures that could be classified into three basic types: (1) the anteroseptal "twist": thickening and deviation of the anterobasal septum and anterior wall, resulting in a relatively posterior position of the aortic ostium (16 cases); (2) a prominent anterolateral muscle (9 cases); and (3) a posteromedial muscle (9 cases). It is concluded that a central muscular ventricular septal defect is not an isolated malformation but is commonly part of a complex developmental anomaly. This complex of associated inflow and outflow tract lesions presumably may cause reduced aortic flow during morphogenesis, resulting in aortic arch hypoplasia of various degrees of severity.

Adult↗
PubMed1983

Left ventricular false tendons in children: prevalence as detected by 2-dimensional echocardiography and clinical significance.

During a 28-month period, consecutive 2-dimensional echocardiograms were reviewed to determine the prevalence of left ventricular (LV) false tendons, their associated anomalies and clinical significance. LV false tendons were found in 31 (0.8%) of 3,847 consecutive 2-dimensional echocardiograms. Of 31 LV false tendons, 30 passed longitudinally from papillary muscle to septum and 1 went from free wall to free wall. The 31 patients were aged 1 day to 15 years. Associated heart disease, most often ventricular septal defect, bicuspid aortic valve and coarctation of the aorta, was present in 48%, of whom 73% were girls. Of those without heart disease, 69% were boys. In patients with heart disease, precordial murmurs were due to the underlying cardiac anomaly. Of those without heart disease, 15 of 16 (94%) had a precordial murmur, usually of the Still's type over the lower left sternal border. Four of 31 (13%), 1 with and 3 without heart disease, had unifocal premature ventricular contractions that were rate-dependent in the 2 patients undergoing stress testing. LV false tendons appear to occur in 0.8% of pediatric patients and usually are accompanied by a Still's type innocent murmur if unassociated with heart disease. Some LV false tendons are associated with rate-dependent premature ventricular contractions.

Adolescent↗
PubMed1987

Treatment of calcific aortic stenosis by balloon valvuloplasty.

Recent reports have established the feasibility of using balloon valvuloplasty to reduce left ventricular outflow tract obstruction due to a calcified aortic valve. The present study summarizes experiences with this technique in 9 patients (7 women, 2 men, mean age 78 years) in whom balloon valvuloplasty was used to treat calcific aortic stenosis. Peak aortic valve gradient (mm Hg) decreased from 68 +/- 8 (mean +/- standard error of the mean) before valvuloplasty to 35 +/- 5 after valvuloplasty (p = 0.003). Mean aortic valve gradient decreased from 57 +/- 7 before valvuloplasty to 30 +/- 5 after valvuloplasty (p = 0.006). Calculated aortic valve area increased from 0.42 +/- 0.04 to 0.81 +/- 0.06 cm2 (p = 0.005). Balloon valvuloplasty failed to diminish aortic valve obstruction in only 1 patient who, at subsequent surgery, had a congenitally bicuspid aortic valve. Significant aortic regurgitation was not observed in any of the 9 patients after valvuloplasty. One patient did have a highly focal, presumably embolic, brain stem infarct during the procedure. Femoral arterial blood loss, related to wire-guided exchange of balloon catheters too large for a 12Fr introducer sheath, was minimized by direct arterial exposure in 8 of the 9 patients. Thus, these findings confirm the efficacy of balloon valvuloplasty for the treatment of calcific aortic stenosis. The procedure, however, is not without hazard.

Aged↗