Biomedical subjects
Publications and source records attributed to A Manetti.
Two patients, 35 and 36 years old respectively, had corrected transposition of the great vessels. They have been added to the nineteen cases above thirty years of age previously reported. The diagnosis and prognosis of the malformation in this age group have been discussed.
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Clinical signs of aortic regurgitation were noted after birth in an infant who died suddenly at the age of 18 months. Congenital malformation of the aortic root with an aneurysm of the right aortic sinus and disconnection of the corresponding cusp were found at necropsy.
A family in whom two brothers, a sister and the father presented a long QT interval is reported. The father died suddenly at the age of 37. The opportunity of a more careful research of the syndrome, now underdiagnosed, is underlined in view of the curability such disease.
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11,584 consecutive live-born babies in the Ostetric Clinic of Florence in the years 1975 and 1976 were examined for the presence of congenital heart disease. Diagnosis was made by necropsy, cardiac catheterization and operation in 20% of cases and by clinical observations in 80%. The 74% of babies who were diagnosed as affected by congenital heart disease at birth were eventually controlled. The incidence of congenital heart disease was 7.1% in 10,789 live-born of weight over 2500 g while it was 37% in 795 under such weight. Global incidence was 9.2%. The first essentials in epidemiological study of congenital heart malformations is to define the method which is used for diagnosis since this is the primary factor responsible for wide variations in incidence. It is suggested that the institution of a Regional Register of malformations would allow more accurate estimate of overall incidence of congenital cardiopathies.
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The development of a false aneurysm of the ascending aorta following arteriotomy and cannulation of the aorta performed during closure of a ventricular septal defect is described. The evidence suggest that the sutures of the arteriotomy led to rupture of the aortic wall and secondarily late formation of a false aneurysm.
Nine families affected by the mid-systolic click syndrome were studied. Of the one hundred and forty-four first-degree relatives, 117 of whom were living, eighty-four were examined. Thirty-five were found to be affected by the syndrome. Twenty-six were females and nine were males. Auscultatory and phonocardiographic findings consisted either of isolated mis-systolic clicks or systolic murmurs or a combination of the two. Electrocardiograms revealed changes of various types, most commonly of the ST-T segment. About seventy per cent of the patients were symptomatic. Nine members, not examined by the authors, had died suddenly; all had a previous history of "cardiopathy". Progressivity of mitral valve disease with age is not confirmed by the present study. It is suggested that the mode of inheritance of the defect might be that of an autosomal dominant form of Mendelian type with delayed expression of the defect. An alternatove hypothesis of a multifactorial inheritance mechanism, which is more stimulating for future studies on the cause of the syndrome, is also taken into consideration.
Infrahepatic interruption of the inferior vena cava with azygos continuation has been demonstrated by angiography in three patients; in a fourth case an anomalous venous channel connected the patent azygos vein and the inferior vena cava at the renal level. Cardiac defects were present in all cases.
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Two cases of left ventricular aneurysm occurring in young patients (23 and 29 years respectively) are reported. Coronary arteriograms were normal. Pertinent literature was reviewed. About 70 cases of left ventricular aneurysm in patients under 35 years of age have been reported: approximately half of them are congenital. They include both diverticuli of the apex, as a part of a syndrome of congenital defects, and those, much rarer, following myocardial infarction in children with anomalous origin of coronary arteries. A few cases may be attributed to trauma or myocarditis. In 26 patients, including the two reported here, etiology was uncertain (idiopathic aneurysm of the left ventricle) in as much as malformation or an infectious disease might have been the underlying cause.
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