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PubMed2004

Congenitally corrected transposition of the great arteries: clues for prenatal diagnosis.

Congenitally corrected transposition of the great arteries (ccTGA) is an uncommon cardiac defect characterized by the atria connecting with the anatomically discordant ventricles and the ventricles connecting with discordant and transposed great arteries. Parallel vessels are evident in corrected TGA, but as this sign is also present in complete TGA, a heart anomaly requiring major cardiac surgery in the postnatal period, it is important to differentiate between the entities prenatally. Most cases of ccTGA have associated anomalies but isolated forms or those with a mild associated cardiac anomaly are infrequently detected prenatally. We report on three cases detected between 21 and 25 weeks' gestation on screening ultrasound with associated mild findings. One fetus had an isolated ventricular septal defect (VSD) first detected at 34 weeks. The child developed heart block at 4 years of age. The second case was associated with a small VSD, a tiny pulmonary trunk and a persistent right umbilical vein. After birth, mild pulmonary stenosis was found as an additional cardiac finding at 4 months of age. The third fetus had no additional cardiac anomalies prenatally, but after birth a bicuspid aortic valve was detected. The first case needed pacemaker implantation but the other two children required no cardiac surgery. Two of the cases were referred because abnormal vessel anatomy was detected on screening ultrasound. As prenatal detection of TGA is becoming a more frequent occurrence, this paper aims to present clues aiding in the prenatal diagnosis of atrioventricular and ventriculoarterial discordance, especially in its differentiation from complete transposition. These details are crucial for counseling and perinatal management.

Adolescent↗
PubMed1998

Body proportions of Australopithecus afarensis and A. africanus and the origin of the genus Homo.

New discoveries of A. africanus fossils from Member 4 Sterkfontein reveal a body form quite unlike earlier Australopithecus species. The new adult material consists of over 48 fore- and hindlimb specimens and includes an associated partial skeleton, Stw 431. The forelimbs and relatively large: the average size of their joints corresponds to a modern human with body mass of 53 kg. The hindlimbs are much smaller with an average size matching a modern human of only 33 kg. Analyses of the Stw 431 partial skeleton confirm these results. In contrast, A. afarensis and anamensis more closely approximate a human pattern of forelimb joint size. This is an unanticipated complication in our understanding of early human evolution. In general, craniodental morphology tracks time in species of Australopithecus: A. anamensis (3.5-4.1 Ma) is the the most primitive with a strongly sloping symphysis, large canine roots, etc., A. afarensis (3.0-3.6 Ma) is less primitive, and A. africanus (2.6-3.0 Ma) shares many derived characteristics with early Homo (e.g., expanded brain, reduced canine, bicuspid lower third premolar, reduced prognathism, greater flexion of the cranial base, deeper TMJ). the new postcranial material, however, reveals an apparently primitive morphology of relatively large forelimb and small hindlimb joints resembling more the pongid than the human pattern. More pongid-like proportions are also present in the two known associated partial skeletons of H. habilis (OH 62 KNM-ER 3735). This may imply either (1) that A. africanus and H. habilis evolved craniodental characters in parallel with the lineage leading to later Homo, or (2) that fore- to hindlimb proportions of A. afarensis (and perhaps A. anamensis) evolved independent of the lineage leading to Homo and does not imply a close phylogenetic link with Homo. Both of these explanations or any other phylogeny imply homoplasy.

Adult↗
PubMed2002

Ultrastructure of absorbing peripheral lymphatic vessel (ALPA) in guinea pig Peyer's patches.

Corrosion casts from neoprene direct injection of lymphatic and blood vessels in guinea pig gut-associated lymphoid tissue, that is, solitary lymphoid follicles and Peyer's patches, have shown both their numerical density and their topographical arrangement in physiological conditions, after starvation and lymphatic stasis. The absorbing peripheral lymphatic vessel (ALPA) begins with the lacteal vessel, which continues in the mucosal lymphatic network. The latter is formed by subepithelial and interfollicular vessels wrapping single lymphoid follicles like a basket. Interfollicular vessels drain in the submucosal network, which flows into muscular tunica vessels with nonsegmentary bicuspid valves. They in turn drain lymph in subserosal precollectors and then in prelymphonodal collectors with conduction function. The follicles' germinal center and dome are completely devoid of ALPA vessels, while they are rich in blood vessels. Ultrastructurally, the ALPA vessel wall consists of a monolayer of endothelial cells devoid of pores, fenestrations, and open junctions and lacking a continuous basal lamina. Endothelial cells are joined by overlapping and interdigitating intercellular contacts, while end-to-end contacts are rare. They have a sizeable cell body, containing the nucleus and the common endocytoplasmic organelles, and a peripheral cytoplasm with actin-like filament bundles, free microvesicles or forming channels and a few rough-surfaced encloplasmic reticulum (RER) canaliculi. The presence of intraendothelial channels crossed by lymphocytes can often be detected within the endothelial wall during the different phases of cell transendothelial migration from lymphoid tissue to lymphatic vessel lumen. These channels undergo a numerical increase during starvation, while they are scarce during lymphatic stasis. We have quantitatively evaluated the prevalence of T lymphocytes in the lymph of interfollicular ALPA vessels and of prelymphonodal collectors draining the small intestine tract with or without Peyer's patches, under physiological and experimental conditions (starvation, lymphatic stasis).

Animals↗
PubMed1991

Functional anatomy of the temporomandibular joint. A morphologic study on human autopsy material.

A study of 72 temporomandibular joints of adults without any gross signs of degenerative changes was made, in order to analyse the stress on individual regions of the joint, and to determine the path of the split line pattern in the articular cartilage. The cancellous architecture and the material distribution in the juxta-articular bone were also investigated. Zones of high-density compact substance in the lateral region of the joint, and the presence of attractive singular points in the split line orientation in this localization, suggest great stress on the lateral part of the temporomandibular joint. This increased stress in the lateral region is confirmed by the fact that arthrotic changes in the temporomandibular joint first almost always appear laterally. Degenerative changes usually originate at the articular disc, affect the articular tubercle after a delay, and finally spread to the condyle. Besides the greater physiological, mechanical stress on the lateral region of the joint, the force acting on every individual temporomandibular joint is influenced by the dental condition. The molars and bicuspids serving as a support zone play a decisive role in this context.

Adult↗
PubMed1986

Anatomy of the veins of the dorsum pedis with regard to their suitability as vascular grafts in reconstructive microsurgery.

Attempts to locate veins suitable as micro-surgical vascular grafts were carried out in 16 preparations of the subcutaneous venous network of the dorsum pedis. For this reason the length, diameter, valve distances and frequency of longitudinal veins were especially examined. Four types of veins were defined as being suitable for transplantation and it was established that they are situated in the medial half of the dorsum pedis in the so-called microsurgical area in all cases. Collecting and conducting veins can be distinguished by means of the location of the valves, which are generally bicuspidal. In most cases, the level of the opening spaces of both the segment and the opening valves lies parallel to the surface of the skin.

Humans↗
PubMed1989

Mitral valve prolapse in Turner syndrome.

We have evaluated 46 patients with Turner syndrome by clinical examination, M-mode and two-dimensional echocardiography, dynamic exercise testing and 24 h Holter monitoring. Twelve patients (26.1%) had mitral valve prolapse and 7 patients (15.2%) had isolated non stenotic bicuspid aortic valve. Aortic root dilation was present in 2 patients (4.3%). Our data indicate that incidence of mitral valve prolapse is significantly higher in Turner syndrome than in the general population (P less than 0.025).

Adolescent↗
PubMed1975

Studies of malformation syndromes in man XXXVI: the Pfeiffer syndrome, association with Kleeblattschädel and multiple visceral anomalies. Case report and review.

This paper reports sporadic occurrence of the Pfeiffer syndrome with Kleeblattschädel (KS) in a male infant who died at 6 months of pneumonia with signs of increased intracranial pressure and who was found to have hydrocephalus, polymicrogyria, cerebellar herniation, bicuspid aortic valve, a common mesentery, absence of lesser omentum, hypoplasia of gallbladder, a single umbilical artery, and multiple eye defects. This case is presumed to represent a new mutation: in other families the Pfeiffer syndrome has been dominantly inherited. The Pfeiffer syndrome is a form of acrocephalosyndactyly and impresses clinically as a mild form of the Apert syndrome. The Kleeblattschädel is an etiologically non-specific developmental field defect (DFC); about two fifths of 51 known cases have apparent thanatophoric dwarfism and about one fifth are probable or possible examples of the Pfeiffer syndrome. The KS-DFC has also been seen in the syndromes of Carpenter, Apert and Crouzon.

Abnormalities, Multiple↗
PubMed1992

Bacterial endocarditis in children: trends in its diagnosis, course, and prognosis.

In a population-based study of 41 children with bacterial endocarditis (BE), diagnosed in the period 1970 through 1989 in eastern Denmark, we analyzed trends in the diagnosis of BE and in mortality, and searched for possible prognostic factors. During this period the delay in diagnosis from first symptom to treatment did not change, but the delay from admission to treatment was significantly prolonged from 0 to 3 days, despite the introduction of echocardiography (ECHO). There was a significant improvement in the prognosis, the mortality rate having decreased from 40 to 0% [95% confidence limits: 12-74 vs. 0-26 (0.01 less than p less than 0.02)]. The improved prognosis was not explained by changes in the etiology or pattern of antibiotic resistance and may reflect a milder course of BE in children. Children with "mild anomalies"--such as bicuspid aortic valve (n = 5), coarctation of the aorta (n = 2), and prolapse of the mitral valve (n = 2)--had a significantly poorer prognosis than children with other forms of congenital heart disease (CHD) (p = 0.004), a reminder of the importance of suspecting BE in all children with unexplained long-lasting or intermittent fever, because some may have unrecognized "mild" CHD.

Child↗
PubMed1998

The force module for the bending art system. Preliminary results.

The force module for the bending art system (BAS) is used to calculate the initial forces and moments expected to act on a tooth after changing arch wires. The present study analyses the accuracy of the force module on the basis of 10 patients treated with the BAS (with an average observation period of 10 months). An average of 6 arch wires (0.16" x 0.016" steel) were used on each jaw. The approximate pressure in the periodontium was determined and statistically evaluated from the force module readings. The resulting average pressure values for the molars ranged between 0.26 N/cm2 and 0.54 N/cm2. The value in the area of the incisors, cuspids and bicuspids ranged between 1.03 N/cm2 and 2.83 N/cm2. Maximum pressure was 8.02 N/cm2. The results are discussed from a clinical point of view on a case-to-case basis. They are plausible in and for themselves. The more severe the initial state of misalignment, the higher the pressure values. These forces can be reduced by increasing the number of arch wires or by altering the form of the archwire correspondingly. The computer-generated values should be confirmed by taking a direct measurement on the tooth. The force module is just the first step in providing a reproducible estimation of the forces acting on a tooth. Even if the calculated absolute values should still be judged with reservation, they are nevertheless suitable for providing a comparative evaluation of various treatment concepts with reference to the forces acting on the periodontium and can serve clinicians as a rapidly available decision-making aid.

Adolescent↗
PubMed1994

Congenital aortic regurgitation caused by a rudimentary noncoronary cusp: report of a case.

A 14-year-old asymptomatic boy was admitted to our department for investigation of a diastolic murmur which had been discovered by his family doctor during a routine examination. Echocardiography showed aortic regurgitation with dilatation of the left ventricle. Inspection of the aortic valve at the time of operation revealed normal left and right cusps with a rudimentary noncoronary cusp. An aortic commissuro-plication was performed and a new bicuspid aortic valve successfully reconstructed. His postoperative course was uneventful and he has been well and leading an active life since his discharge from hospital.

Adolescent↗
PubMed1987

Anatomic features of congenital pulmonary valvar stenosis.

A total of 31 specimens of hearts with congenital pulmonary valvar stenosis were studied. To define the anatomy of the normal pulmonary valve, 210 specimens of hearts considered to have a normal pulmonary valve were also reviewed. On the basis of gross morphology of the valve leaflets and annulus, the stenotic valves were subgrouped into domed, unicommissural, bicuspid, tricuspid, hypoplastic annulus, and dysplastic. The valve leaflets in all subgroups were thickened. The thickness varied in degree, but involved the entire length of the leaflet. Microscopically, the thickness in most cases was due to an increase in myxomatous tissue. In a few cases, the elastic and collagen components of the leaflet were increased. The valve annulus was abnormal in most cases. The abnormalities included replacement of the fibrous backbone of the annulus by myxomatous tissue and partial or complete absence of the annulus. The impact on valvar anatomy by direct surgical valvotomy (14 patients) and closed Brock valvotomy (two patients) was reviewed. Precise knowledge of pulmonary valve anatomy is an aid to successful balloon pulmonary valvuloplasty.

Adolescent↗
PubMed1989

[Periodontal development after tooth germ transplantation within the framework of orthodontic treatment].

The normal development of the periodontal structures was confirmed histologically on young beagle dogs following tooth germ transplants. In humans, clinical and radiographic examinations revealed no pathology of the periodontium following transplantation of tooth germs. The grafted teeth could be moved orthodontically in the same way as any other teeth. No precautions need to be taken. This paper describes the most common indications for transplanting the tooth germs of canines, bicuspids and molars.

Adolescent↗
PubMedDate not supplied

Group C beta-hemolytic streptococcal endocarditis: report of a pediatric case.

A 9-year-old boy with a ventricular septal defect and a bicuspid aortic valve developed bacterial endocarditis due to group C streptococci. He responded to an initial antibiotic regimen of nafcillin plus gentamicin and was cured by the use of penicillin G following the isolation of the organism. The unusual nature of this case is discussed and physicians are cautioned to recognize this organism as a potential cause of infectious endocarditis in the pediatric population.

Aortic Valve↗
PubMed1982

Left-sided anomalies in Ebstein's malformation of the tricuspid valve.

A review of 34 autopsied cases with classical Ebstein's malformation of the tricuspid valves revealed 8 cases with left-sided anomalies. Among these, appearing in 1 case each, were aortic atresia and persistent common atrioventricular canal. The latter 2 conditions were dominant clinically. In the remaining 6 cases the left-sided anomalies were not apparent clinically and probably of no functional significance. These conditions were parachute mitral valve, bicuspid aortic valve, cor triatriatum and pulmonary stenosis, cleft mitral valve, stenosis of individual pulmonary veins, and prolapse of mitral valve.

Adolescent↗
PubMed1986

Persistent truncus arteriosus: pathologic anatomy in 54 cases.

Fifty-four specimens of heart with persistent truncus arteriosus (PTA) were reviewed anatomically. According to the Collett-Edwards classification there were 28 examples of type I and 26 type II. The sex distribution was equal. The number of the truncal cusps ranged from one to four (42% tricuspid, 30% bicuspid, 24% quadricuspid, and 4% unicommissural). A unicommissural truncal valve has not been previously reported. In 72% of cases, the truncal valve leaflets were thickened or dysplastic. Two valves were stenotic. The truncus arteriosus originated from both ventricles equally in 42% of the cases, predominantly from the right ventricle in 42%, and predominantly from the left ventricle in 16% of the cases. In unoperated cases of PTA originating predominantly from the right ventricle, it appeared to us that usual operative correction might result in left ventricular outflow obstruction. Variations in coronary arterial origins and patterns were present in nearly half of the cases. A single coronary artery was observed in ten cases (18.5%). Stenosis of the ostium of one coronary artery was seen in each of four cases (7%). High posterior origin of the left coronary artery was observed in ten cases (18.5%). Among the associated cardiovascular anomalies, the most common were right aortic arch (36%) and interruption of the aortic arch (11%). Three cases with the latter condition exhibited crossed pulmonary arteries. Isolated cases with tricuspid atresia, vascular sling (left pulmonary artery arising from right pulmonary artery), and persistent common atrioventricular canal were encountered.

Adolescent↗
PubMed1980

Mycotic aneurysm of the aorta.

The radiographic and pathologic features of 13 cases of mycotic aneurysm of the aorta were reviewed retrospectively. In four cases the mycotic aneurysm was associated with bacterial endocarditis following aortic valve replacement, and in nine cases it was associated with spontaneous bacterial endocarditis. Postmortem examination revealed that the mycotic aneurysm was most frequently found in cases with bicuspid aortic valves. In all cases the chest radiograph revealed cardiomegally, usually with pulmonary vascular congestion. In the six patients in whom the diagnosis was established angiographically, the aneurysm was manifested by an irregular saccular collection of contrast medium under one of the coronary arteries. Mycotic aneurysms must be differentiated from other lesions with similar angiographic findings, such as a congenital aneurysm of the sinus of Valsalva, prolapse of an aortic cusp through a membranous ventricular septal defect, and a congenital aortic-left ventricular tunnel.

Adult↗
PubMedDate not supplied

Management of asymptomatic chronic aortic regurgitation with left ventricular dysfunction: a decision analysis.

STUDY OBJECTIVE: To determine the optimal strategy for the timing of aortic valve replacement in patients with chronic, severe aortic regurgitation with left ventricular dysfunction. DESIGN: Decision analysis comparing early surgery (timed at the onset of left ventricular dysfunction) with delayed surgery (timed at the onset of symptoms) using data from the literature and expert opinion for variables in a representative case scenario (40-year-old man with bicuspid aortic valve disease). SETTING: Tertiary care center doing valve replacement surgery. MEASUREMENTS AND MAIN RESULTS: The early-surgery approach was preferred based on quality-adjusted life years. Sensitivity analysis showed that the result was not affected by the following variables within their derived ranges: rate of symptom development after onset of left ventricular dysfunction for the delayed-surgery approach, perioperative mortality for both approaches, and occurrence of major nonfatal stroke or congestive heart failure for both approaches. Although the decision was sensitive to the yearly postoperative mortality rates, the delayed-surgery operative mortality rate had to be almost as low as the early-surgery rate to change the preference to the delayed-surgery approach. The preference could also change if survival were much more important to the patient in the first five years than after five years or if the patient disliked living on anticoagulants enough to value a year on anticoagulants as worth only 80% of a year not on anticoagulants. CONCLUSION: This decision analysis provides quantitative support for the impression that patients similar to the case scenario do better with surgery timed at the onset of ventricular dysfunction than with surgery delayed until symptoms develop. It thus supports the practice of following these patients noninvasively in order to time surgery.

Adult↗
PubMed2002

Aortic stenosis.

Valvular aortic stenosis in pediatric age group is mostly congenital in origin. The aortic valve may be unicuspid, bicuspid, tricuspid or rarely quadricuspid. Left ventricle undergoes concentric hypertrophy secondary to obstruction to its outflow tract. In neonatal aortic stenosis, left ventricle may be hypoplastic. The pathophysiology and clinical presentation vary with the age of onset and severity of obstruction. Echocardiography and Doppler are indispensible for the diagnosis of aortic stenosis and its severity. Cardiac catheterization is not necessary for the diagnosis, however it is performed as a part of balloon aortic valvuloplasty in severe cases. Balloon valvuloplasty is an effective alternative to surgery in pediatric age group. Some of these patients require surgical valve repair or replacement on follow-up. Neonates and young infants with critical aortic stenosis present with cardiogenic shock and need aggressive treatment with prostaglandin E1 infusion along with inotropic support. In experienced hands, balloon valvuloplasty is a safe procedure in neonates and infants with critical stenosis. Patients with mild and moderate aortic stenosis may be left on medical follow-up.

Adolescent↗