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Birth prevalence of congenital cardiovascular malformations in Hungary.

In a certain area of Szolnok county, 5,978 live births occurring in the period 1963-1965 were studied by the optimal epidemiological model. Of the study population, 5,433 children (91%), were identified. It was completed by 211 children, thus the total material involved 5,644 cases, and 67 congenital cardiovascular malformations were detected among them. Seven, being part of multiple congenital abnormalities, were excluded. Thus the birth prevalence of congenital cardiovascular malformations was 10.6 per 1000 live births.

Aortic Valve Stenosis↗

Reconstructive surgery of mitral valve incompetence: ten-year appraisal.

Between January, 1969, and January, 1978, 551 patients with mitral incompetence were treated by a system of reconstructive techniques. Mitral valve incompetence was classified into three types according to leaflet pliability; type I normal leaflet motion, 150 cases; type II, leaflet prolapse, 213 cases; and type III, restricted leaflet motion, 188 cases. Associated tricuspid valvular disease was present in 174 cases (31.5%) and was treated by prosthetic ring annuloplasty. The operative mortality rate was 4.2% (16/377) in the mitral group and 14% (25/174) in the mitral-tricuspid group. Follow-up data are available in 341 patients from 1 year to 10 years (average 4 1/2 years). The late mortality rate was 7% (24/341). Actuarial curves including hospital mortality rate show an 82% survival rate at 9 years in the mitral group and a 79% rate in the mitral-tricuspid group. Thirty-seven patients (11%) underwent reoperation mainly for residual (17) or recurrent (16) mitral incompetence. Thromboembolism occurred in 12 patients for an embolic rate of 0.6% per patient-year, even though 48% were not given anticoagulants. Acorrding to the New York Heart Association (N.Y.H.A.) classification, 76% (207/270) of the patients were in Class I, 19% (51/270) were in Class II, 4% (10/270) were in Class III, and 0.7% were in Class IV (2/270). Results of postoperative catheterization and angiocardiography are available in 52 patients. Comparison between the various groups shows that the best results were obtained in type II mitral incompetence, followed by type I and type III mitral incompetence. This experience demonstrates that predictable and stable long-term results have been achieved by techniques of valvular reconstruction with a low incidence of thromboembolism. Reproducibility of the techniques is a limiting factor which can be overcome by adequate training and progressive experience. Patient selection is based on the valvular disease rather than age, physical condition, or cause of valvular disease.

Adolescent↗

[Prenatal diagnosis of left cardiac abnormality].

We found out of 1500 prenatal cardiac ultrasound examinations very small left heart in 14 cases. Six of the 14 cases were hypoplastic left heart syndrome and eight functional-secondary left heart hypoplasia. The most important signs the echocardiography in cases of hypoplastic left heart are as follow: hypoplasia of mitral valve with or without measurable flow, absence of the anterograde flow through the aortic valve, retrograde flow in the isthmus. In cases of functional left heart hypoplasia the size of the left ventricle, aorta and mitral valves were under the 3rd percentile but there were measurable anterograde flow through the aorta. In eleven cases there were other malformations: hygroma colli, diaphragmatic hernia and omphalokele. The differentiation of the hypoplastic left heart from the secondary left heart hypoplasia has a great importance because of the therapic planning and prognosis.

Abnormalities, Multiple↗

The right pleuromediastinal reflection in children.

The authors review the anatomy of the pleural reflection of the right lung against the middle mediastinum in childhood. In 505 consecutive normal frontal radiographs of children, from birth to sixteen years, this pleural reflection (also known as the pleuro-esophageal line) was visible in 83%. The entire reflection from thoracic inlet to diaphragm was clearly seen in 13%, chiefly in upright, deep-inspiration, well penetrated radiographs. It is a radiographic landmark in the middle mediastinum, particularly well seen in childhood, and its use in assessment of mass lesions, cardiomegaly and lung collapse is described.

Adolescent↗

[Fetal supraventricular tachycardia associated with anasarca: poor prognosis despite treatment. Apropos of two cases].

Two cases of foetal supraventricular tachycardia with hydrops with fatal outcomes illustrate the poor general prognosis of this condition. The absence of therapeutic consensus, of large series in the existing literature, does not prevent logical and reasonable management based on rhythmological, pharmacological and prognostic criteria. A combined approach associating antiarrhythmic therapy by the transplacental and intrafunicular approaches seems acceptable now that funicular puncture can be undertaken easily, and certain antiarrhythmic molecules suggest encouraging results. It is important to try to assess the haemodynamic tolerance by foetal Doppler echocardiography because the foetal prognosis depends on ischaemic cerebral lesions induced by anoxia, difficult to diagnose in utero: when observed, aggressive and occasionally dangerous therapies to foetus and mother may be justified in these extreme situations of foetoplacental hydrops.

Adult↗

[Sudden death in a 4-month-old infant associated with anomalous origin of the left coronary artery].

We describe the autopsy findings of a 4 months-old boy, who died suddenly after an episode of high airway infection of 3 days time. Autopsy examination showed cardiomegaly (80 g) with widening of the left cavities and thick and white endocardial surface, besides a severe thinning of the cardiac apex at the level of the left ventricle. Left coronary origin was in the pulmonary artery trunk. Histologically, the myocardium shows endomyocardial fibroelastosis and also multiple and extensive areas of old and recent infarcts in the left ventricle. The collateral coronary arteries, were increased in number, and branches showed a marked intimal oedema and a reduction of the luminal diameter.

Autopsy↗

Etiology of valvular heart disease.

The etiology of valvular heart diseases (VHD) has changed in the last 50 years in the industrialized countries. A significant reduction in the incidence of rheumatic fever and its sequelae, increase in life expectancy, recognition of new causes of VHD and advancement in technology are responsible for the metamorphosis of the etiology of VHD. Heritable disorders of connective tissue (marfan syndrome, Ehlers-Danlos syndrome, adult polycystic kidney disease, floppy mitral valve/mitral valve prolapse); congenital heart disease (bicuspid aortic valve); inflammatory/immunologic disorders (rheumatic fever, AIDS, Kawasaki disease, syphilis, seronegative spondyloarthropathies, systemic lupus erythematosus, antiphospholipid syndrome); endocardial disorders (nonbacteremic thrombotic endocarditis, infective endocarditis, endomyocardial fibroelastosis); myocardial dysfunction (ischemic heart disease, dilated cardiomyopathy, hypertrophic cardiomyopathy); diseases and disorders of other organs (chronic renal failure, carcinoid heart disease); aging (calcific aortic stenosis, mitral annular calcification); postinterventional valvular disease; drugs and physical agents are all clinical entities associated with VHD. It should be emphasized that VHDs still constitute a major health problem which will increase with the aging population.

Heart Valve Diseases↗