[Reliability of digital coronary quantification in a hemodynamic laboratory. Comparison with a film-based system]. / Fiabilidad de la cuantificación coronaria digital realizada en el laboratorio de hemodinámica. Comparación con un sistema basado en película.

BACKGROUND AND OBJECTIVES: Quantitative coronary angiography can be performed in two ways: on-line during catheterism, and off-line once the procedure is finished. Consequently, several studies have been published comparing both systems. Nevertheless, none of them has compared the measurements made off-line with those acquired on-line by the hemodynamist in charge of procedure. The objective of this study was to compare the measurements made on-line by the hemodynamist involved in the procedure with a digital system (DCI) with those obtained off-line by an independent and alien observer to the procedure by using film-based system (CMS). MATERIAL AND METHODS: Forty coronary lesions suitable for quantification were measured in a prospective fashion. They came from follow-up angiograms. Either balloon or stent were used in the previous angioplasty. Stenoses were assessed on-line and off-line by using the most severe view as judged by the hemodynamist. RESULTS: No significant differences were found for obstruction diameter, reference diameter nor percent diameter stenosis. Pearson's correlation coefficient values (r), intraclass correlation coefficient (ri), regression line equation and mean of signed differences with their standard deviations are showed: a) obstruction diameter: r = 0.83, ri = 0.83, DCI = 0.42 + 0.76 x CMS, -0.01 +/- 0.42 mm; b) reference diameter: r = 0.72, ri = 0.69, DCI = 1.29 + 0.61 x CMS, 0.003 +/- 0.38 mm, y c) percent diameter stenosis: r = 0.86, ri = 0.86, DCI = 10.05 + 0.77 x CMS, 1.19 +/- 10.75%. CONCLUSIONS: We attained good concordance between both quantification systems under clinical conditions. In our opinion these results support the use of on-line quantification as a reliable tool for clinical decision making in the catheterization laboratory.

[Outcome of anatomical correction of transposition of the great vessels in the neonatal period]. / Resultados de la corrección anatómica en la transposición de las grandes arterias en el periodo neonatal.

Between february 1992 to January 1994, anatomic correction was performed on 15 patients with transposition of the great arteries and intact ventricular septum. The mean age was 8.3 +/- 2.9 days and the mean weight 3.39 +/- 0.39 Kg. Before the operation, 13 patients (86.6%) received prostaglandin El infusion and 13 patients (86.6%) underwent Rashkind septostomy. Mean aortic cross-clamps was 56 +/- 11 minutes and mean cardiopulmonary bypass was 108 +/- 91 minutes. Hospital mortality rate was 13%. Mean extubation period was mean discharge from ICU was 13.1 +/- 12.3 days and discharge of hospital was 17.8 +/- 7.5 days. The mean followup period was 11.4 months. All the patients remained asymptomatic with adequate psychomotor and ponderal development. All patients remained in sinus rhythm. Pulmonary suture gradient was over 60 mm Hg in five patients (33%). One patient needed reintervention and four pulmonary artery angioplasty (PAA) that was successful. In the midterm follow-up pulmonary stenosis suture was the most common complication. If the stenosis is severe and PAA should be the initial approach.

[The myxoma syndrome: "cardiac myxoma, cutaneous pigmented lesions and peripheral and endocrine neoplasms". Apropos 2 cases]. / Síndrome mixoma: "Mixoma cardíaco, lesiones pigmentadas cutáneas y tumores periféricos y endocrinos". A propósito de dos casos.

We present two cases, mother and son, with "syndrome myxoma". Both had cardiac myxoma and cutaneous pigmented lesions. The son had a cutaneous myxoma and the mother had been diagnosed as having Cushing's syndrome caused by adrenal adenoma. The "syndrome myxoma" is a systemic disease which causes cardiac, cutaneous, and breast myxomas, adrenal disease, testicle and pituitary tumours. Whenever it is diagnosed all first relatives should be tested for the syndrome. The patient should be re-examined every six-twelve months, due to frequent recurrence of cardiac myxoma.

[Pulmonary valvuloplasty in children with cyanotic cardiopathy]. / Valvuloplastia pulmonar en niños con cardiopatías cianóticas.

We present 5 patients with cyanotic congenital heart disease in whom a pulmonary valvuloplasty was performed as palliative therapy. The patients symptoms were hypoxemic spells, very low oxygen saturation and no weight gain. The oxygenation improved and symptoms disappeared. We believe that in properly selected patients this technique represents a good therapeutic modality.

[Rheumatic fever: clinical and therapeutic aspects]. / Fiebre reumática: aspectos clínicos y terapéuticos.

From 1974 to 1987 we have diagnosed thirty three patients with rheumatic fever. The age of onset ranged from three to sixteen years with a mean age of nine years and six months. Carditis was the most frequent major criteria (27/33) followed by arthritis (14/33) and Sydenham's chorea (6/33). All the patients with carditis had the mitral valve affected. The usual treatment in the acute phase was penicillin and aspirin. The prophylaxis recommended to the patients was penicillin G benzatine every 21 days if they had carditis and every 28 days if they didn't. Two patients allergic to penicillin are receiving erythromycin. We have observed that the incidence of rheumatic fever has remained unchanged that the age of onset increased and that the antiinflammatory drug of choice in most patients with carditis is aspirin.

[Symptomatic pericardial effusions in childhood]. / Derrames pericárdicos sintomáticos en la edad pediátrica.

We present the clinical data and the management of twelve patients with symptomatic pericardial effusion (PE). The etiology of PE was: Chronic renal failure, viral infection, cardiac surgery, juvenile rheumatoid arthritis and chronic myelocytic leukemia. Four cases were diagnosed as idiophatic. PE in childhood is usually asymptomatic. When symptoms are present they are non-specific and don't help to know the size of the effusion; therefore, it's necessary to practice an echocardiography to demostrate the presence of PE. The hemodynamic findings permit to diferentiate patients with and without cardiac tamponade. The treatment of first choice is aspirin. In patients with cardiac tamponade the treatment should be pericardiocentesis.

[Congenital long QT-interval syndrome]. / Síndrome del intervalo QT largo congénito.

Authors report one pediatric patient with prolonged QT-interval syndrome. Importance of this syndrome is based on the possibility of syncopal episodes, due to serious arrhythmias, and sudden death. Therapeutics protocols and prophylactic treatment's indications. They are reviewed conclude that familial study is necessary in congenital type and that better therapeutical results are achieved with propranolol than with others drugs.

[Congenital unilateral pulmonary hypoplasia]. / Hipoplasia pulmonar unilateral congénita.

Authors report twelve patients who presented unilateral diminished lung volume on chest x-ray and analyze ECG, bronchography, lung scan and angiography performed in this patients. Based on these data and following classification proposed by CURRARINO they divide patients in groups: 1) Simple lung hypoplasia, seven cases, four of these were of the left side. 2) Absence of one pulmonary artery, two cases. 3) Scimitar syndrome, three cases. Eleven patients have remained free of significant symptoms. Authors believe that diagnostic work-up of a patient with unilateral diminished lung volume on chest x-ray should depend on clinical symptoms and possibility of surgical treatment. Therapeutic approach will depend on clinical and anatomic findings.

[Reiter's syndrome: considerations on the frequency and mid-term course of its juvenile form]. / Síndrome de Reiter: consideraciones sobre la frecuencia y la evolución a medio plazo de su forma juvenil.

Reiter's Syndrome (RS) is not common in children and adolescents. From 1982 to 1987 we have seen 13 pediatric patients (8 male and 5 female) with clinical manifestations that characterized RS. Age of onset was 13 years. Clinical findings have been arthritis (100%) involving the knee and ankle joints, heel pain (84%), lumbosacral pain (38%). One to three weeks before joint involvement, six patients (46%) complained of urethritis, 5 (38%) had enteritis and two patients had both. 46% of the patients had HLA-B27 antigen. Radiologic findings were sacroileitis in five patients and calcaneal spurs. All patients were treated with non-steroidal antiinflammatory therapy and, in general, there was a clinical remission in 1-3 months. Nevertheless, after 4 years of follow up, 2 patients had acute relapsing arthritis and 4 had chronic arthropathy. Three patients have developed secondary ankylosing spondylitis. RS is not all that infrequent in pediatric patients with joint involvements if one looks for urethritis, enteritis, eye involvement and cutaneous manifestations. The clinical course is favorable, but there might be some chronic or relapsing cases. Some patients can developed secondary ankylosing spondylitis. For that reason a prolonged follow up of these patients is necessary.

[Clinical evolution of rheumatic carditis treated with aspirin]. / Evolución clínica de las carditis reumáticas tratadas con aspirina.

We have analyzed the clinical, laboratory, electrocardiographic and echocardiographic findings of six patients with rheumatic carditis whose antiinflammatory treatment was aspirin. We include one case who was initially treated with steroids. She relapsed when the steroid treatment was discontinued and she was then treated with aspirin. There were 2 males and four females. The age range was 9 to 14 years. This was the first rheumatic attack for all the patients. They were also treated with penicillin G during ten days. The prophylaxis was accomplished with benzatine penicillin 1.200.000 IU at 28 days intervals. The aspirin was given at doses ranging from 60 to 100 mg/Kg/day for eight to twelve weeks. All the patients had auscultatory findings consistent with mitral insufficiency accompanied in two cases with findings of aortic regurgitation. In three cases, there was a transient mild diastolic murmur during the first five days. In one case the rheumatic carditis was complicated with acute renal failure due to acute rheumatic nephritis that required peritoneal dialysis. The follow-up period ranged from nine to twenty six months. There have been no relapses. All the patients remain asymptomatic and leading a normal life. At the last visit three patients had mild mitral insufficiency and in three patients the clinical findings, electrocardiogram and echocardiogram were normal. If we accept that most of the cases of rheumatic carditis are mild or moderate, is our believe that the aspirin is the antiinflammatory treatment of choice at the present time.