Percutaneous dilatation angioplasty in congenital heart disease.
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Angioplasty, Balloon↗
Biomedical subjects
U Squarcia
Publications and source records attributed to U Squarcia.
[Percutaneous dilatation of pulmonary valve stenosis].
We present 11 cases of pulmonary valve stenosis who underwent percutaneous dilatation angioplasty. Patients were between 2 and 15 years old (mean age = 6.5 yrs) and all of them except 3 had moderate or severe degree of valve stenosis. Pulmonary anulus diameter ranged between 11 and 23 mm. at ECHO and between 10 and 21 mm. at angio. The catheter was chosen to be 20-40% larger than pulmonary anulus. The mean pressure gradient between RV and PA was 65 mmHg. before dilatation and 21 mmHg. after the procedure. The procedures were without complications, except one in which we observed an intermittent idioventricular rhythm for few days after the procedure. Percutaneous dilatation angioplasty has to be considered the treatment of choice for pulmonary valve stenosis.
Adolescent↗
[Persistent supraventricular tachycardia in childhood].
Persistent or chronic supraventricular tachycardia is defined as the one occurring in more than 10% of cardiac rhythm during 24 hours. It may begin at any age, persisting months or years, and frequently it is discovered occasionally because it can be asymptomatic. The persistent supraventricular tachycardia (PSVT) represents 20-30% of all supraventricular tachycardias in pediatric age. The two main forms of PSVT are: atrial ectopic tachycardia, and persistent junctional reciprocating tachycardia (PJRT). The authors present 9 cases of children affected by PJRT seen from 1972 to 1985. They discuss the clinical course (mean follow up = 9.5 years), the pharmacologic treatment, the results obtained. They underline the particular resistance to conventional antiarrhythmic therapy of this form. On the other hand an antiarrhythmic treatment is recommended to avoid the risk of congestive heart failure. Nevertheless the prognosis is considered benign because in the majority of cases this type of arrhythmia disappears later on.
Child↗
[Dilated cardiomyopathy due to primary carnitine deficiency].
A case of a 3 and a half years old girl with severe congestive heart failure, and typical picture of dilated cardiomyopathy is presented. The serum level of carnitine (17.2 micromoles/l, versus 44.1 +/- 12.2 micromoles/l, normal value for age) and the histologic and biochemical evaluation of quadriceps muscle tissue confirmed the diagnosis of primary deficit of carnitine. L-carnitine (2 gr. three times a day p.o.) was added to anti-congestive therapy. After 8 weeks of therapy, the general and cardiocirculatory conditions are much improved. The physiopathology of dilated cardiomyopathy due to deficit of carnitine are discussed. An early diagnosis, and an early substitutive therapy with L-carnitine dramatically improve the outcome of the disease.
Biopsy↗
[Cardiomyopathy in Friedreich's ataxia].
Friedreich's ataxia (FA) is an hereditary degenerative disease involving the spino-cerebellar via which in 10-15% of the cases is associated with symptomatic cardiac disease. Abnormal ECG or ECHO finding are present even in absence of cardiac symptoms in 100% of the patients. A 7 y.o. girl with the clinical picture of FA with cardiac involvement is presented. The features of the cardiomyopathy present in FA studied with ECHO, myocardial perfusion with Thallium 201 and with histologic examination and its relationship with the hypertrophic cardiomyopathy are discussed. Different theories aiming to discover a unique biochemical factor responsible of both the neurological and cardiac disorders are presented.
Cardiomyopathy, Hypertrophic↗
[Hemodynamic study of pulmonary vascular resistances in subjects with Down's syndrome with atrioventricular canal].
Down patients frequently present congenital heart disease (CHD). Among these the most common is complete atrioventricular canal (CAV). The Authors studied by cardiac catheterization 2 groups of subjects with CAV:21 Down-patients, aged 5-48 months (mean = 24.7) and 17 non-Down patients aged 6-50 months (mean = 23.1). Hemodynamic data were compared and in particular pulmonary vascular resistances (PVR) were analysed. Mean pulmonary resistance was 11.1 UM2 for Down children and 4.6 UM2 for non-Down children. Considering the subjects under 2 years of age, PVR greater than or equal to 10 UM2 was present in 5 of 14 (35.7%). Down children, while none in the non-Down group. The cause of this behaviour is unknown; some Authors, in pathological studies, found in Down-patients primitive structural anomalies of the vascular bed with decrease of total vascular surface area. This fact might explain higher values of PVR in Down-patients than in non-Down patients.
Child, Preschool↗
[Kawasaki's syndrome: description of 4 cases].
In this study we present four cases of Kawasaki syndrome; in two of them were present some particular aspects, besides the characteristic signs of the lymphonodal-mucocutaneous syndrome. One little girl showed, during the acute phase of the disease, paralysis of facial nerve, without other signs of neurological involvement; in the second patient, the outcome of the syndrome was quite atypical, because of an osteoarticular involvement; we also observed the presence of immature white blood cells in the peripheral blood. Three of the four cases were treated with acetyl-salicylic acid for almost one year, two of them were also treated with dypiridamol. A four years follow-up, after the acute phase, demonstrated a complete recovery, a normal clinical status and no damage of the cardiocirculatory system.
Blood Cells↗
[Patency of ductus arteriosus in the premature newborn infant: diagnosis, complications, treatment].
The authors consider the problem of patency of ductus arteriosus (P.D.A.) in pre-term infants. On the basis of their own experience and reviewing international literature they discuss about mechanisms maintaining open ductus, incidence, methods of diagnosis, clinical findings and indomethacin therapy. Finally current indications for indomethacin use are discussed.
Contrast Media↗
[Abnormal left pulmonary artery: a cause of severe newborn cardiorespiratory insufficiency].
Aberrant left pulmonary artery (vascular sling) indicates the vascular anomaly in where the left pulmonary artery arises from the right pulmonary artery, passes over the right mainstem bronchus, loops to the left and courses between the trachea and esophagus to the left hilus. Almost invariably respiratory symptoms result from compression of the trachea and right mainstem bronchus by the anomalous vessel as well as by associated tracheobronchial defects. A case is reported. The distinctive radiologic features include anterior indentation of the barium esophagogram and a characteristic pulmonary angiogram. Surgery is the only resolutive treatment for symptomatic patients, but results are often poor owing to associated intracardiac defects and intrinsic tracheobronchial anomalies.
Female↗
[Hepatic hemangiomatosis with congestive cardiac failure and development into a cholostatic hepatopathy].
The authors describe a case of hepatic hemangiomathosis in a three months old infant with a clinical picture of multiple cutaneous hemangioma, epatomegaly and severe cardiac failure. The cardiac catheterization with selective angiography showed extensive communication between the mammary artery and the hepatic circulation with the presence of large bloody areas into the markedly enlarged liver. The medical treatment with digitalis, diuretics, and corticosteroids obtained rapid resolution of the cardiac failure and of the A-V fistula signs. A clinical picture of severe liver cholostatic disease developed few months later which was confirmed by histological examination. The subsequent course has been favorable with complete clinical resolution of the liver disease.
Bile Duct Diseases↗
[Bronchiolitis: new pathogenetic aspects and personal cases].
The etiological, epidemiological and pathogenetical features of bronchiolitis and some clinical and biochemical data concerning seventy nine cases were discussed. It has been underlined the possible pathogenetical role of immunoallergic mechanism (immunoreactions type II, III, IV and VI), and of alterations of humoral immunocompetence (rise of IgM and deficit of IgA). Moreover it has been pointed out that hereditary, socioeconomical and environmental factors could play an important role in determining the seriousness of the clinical picture. The relationship between bronchiolitis and the respiratory diseases of the following years have been examinated.
Bronchiolitis, Viral↗
[Congestive cardiomyopathy in infancy: pathogenetic role of carnitine and selenium deficiency (author's transl)].
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Cardiomyopathies↗
[Atrio ventricular canal defect. Two dimensional echocardiographic features (author's transl)].
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Echocardiography↗
[Epidemiological study of congenital heart diseases in the province of Parma during the years 1972-80 (author's transl)].
We have carried out an epidemiological-statistical study about congenital heart disease on 33.245 live-born infants in Parma from 1972 to 1980. 327 newborns with congenital heart disease were found; the incidence was 9,8/1000 live-born. The lowest value was 5,8/1000 live-born in 1972, the highest was 14,5/1000 live-born in 1977. In the majority of cases diagnosis was only clinical VSD, PDA, ecc.), whereas in the more complex cardiac malformations and in patients with severe congestive heart failure diagnosis was confirmed at cardiac catheterization and at surgery. In 60 cases diagnosis was confirmed also at autopsy. The congenital heart disease most frequently observed was VSD (33%), followed by PDA (13,4%) and TGA (8,8%), ecc. Clinical status at 1 year of age in all patients was evaluated. The Authors think that infant cardiac program is necessary to improve the results on congenital heart disease control.
Europe↗
[Primary pulmonary hypertension in childhood: familial aspects].
Primary pulmonary hypertension (PPH) is a rare, progressive and usually fatal disease. Its pathogenesis remains obscure. Probably multiple genetic and environmental factors play a role in the occurrence of PPH. PPH sometimes runs in families. We have studied two families in which some members were affected: in family A two sibs, their father and probably their paternal grandmother, in family B two sibs. When PPH is present in more than one generation (family A), an autosomal dominant mode of inheritance would be likely, whereas, when the disease is present in only one generation (family B), an autosomal recessive mode of inheritance is more probable. Treatment of PPH is based on pulmonary vasodilator drugs, because one of the most important mechanisms is thought to be an inappropriate vasoconstriction. Some drugs like oxygen, tolazoline, acetylcholine, isoproterenol seem to get poor results, while phentolamine, nifedipine, hydralazine and diazoxide show promise. Diazoxide appears to have some advantages because it reduces not only pulmonary arteriolar resistance, but also pulmonary artery pressure, without producing tachycardia.
Child↗
[Scientific research, clinical activity, human development: what interactions?].
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Adult↗
[Heart transplant: an emotional storm for the child and his family].
The purpose of the present study was a psychological analysis of the emotional state of a family awaiting heart transplantation. Current practice suggests that such emotional reaction is characterized by mixed and contradictory feelings which require further investigation. Subjects were 5 children waiting for transplant and 5 with previous transplant. Methods were: 1) open interview with children and parents; 2) projective drawing test for children (Corman family test, H.T.P., L.A.D.S., M.U.C.T., K.F.D.); 3) "palinomas" questionnaire which investigates: awareness of the illness, child relation with the food, feelings of competence and control, self-esteem and perception of the self. "Palinomas" was created in Italy by a hospital psychological team in order to give a chance to the child to talk about himself and to maintain control over the situation. The information we obtained by "palinomas" revealed to be very useful to understand the personality of the child through the main areas reported above. Within the limits of an introductory study, results indicate that family needs an active psychological support in order to develop a more positive attitude.
Adolescent↗
[Defense and promotion of life of children with heart diseases. Nobody's monopoly, everybody's goal and responsibility].
The suffering of children with congenital heart disease, or with other clinical conditions, inquires people and urges us to face and solve many problems. It makes us to reflect on the life's secrets and bring us to consider that every condition can be or became negative if accepted with indifference, inertia, fear or convenience. The clinicians must always fight the disease, not passing by with indifference to those who live and suffer. The physicians, the researchers should not surrender to what is unknown. They must always be looking to the not-yet of the human possibilities, to the not-yet of research. Human problems become difficult to solve it we do not enter in one echosystem of solidarity. The ABC (Association of families with children suffering of congenital heart disease) represents an answer to many problems related to the presence of a heart condition in infants and children.
Age Factors↗