Biomedical subjects
Publications and source records attributed to F Cetta.
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The authors report three siblings (two sisters and their aunt, aged 20, 22 and 36, respectively) with familial adenomatous polyposis (FAP) and papillary thyroid carcinoma. After diagnosis of FAP, a single, non palpable nodule was revealed in each patient by routine screening ultrasonography of the gland. The diagnosis of papillary carcinoma was made by fine-needle-aspiration biopsy of the nodules and confirmed by histologic examination of surgical specimens. A review of the literature reveals about 40 reports of such an association, that is considered not fortuitous. Nevertheless, in this family the association seems to be a distinctive, clinical feature of the syndrome, affecting three out of five members intensively screened for extracolonic lesions.
BACKGROUND: Arrhythmias, decreased exercise tolerance, or malabsorption will develop in a significant number of Fontan patients. Fontan revision consisting of creation of lateral atrial tunnel, reconnection of the Glenn shunt when present, or both appears to improve these patients. METHODS: Over a 34-month period, 9 patients underwent Fontan revision. The mean age was 11 +/- 5 years and the mean interval from Fontan operation to revision was 3 +/- 2 years. The reason for revision included marked impairment in exercise capacity, inability to go to school consistently, and chronic fatigue in 6 patients, 3 of whom also had serious atrial arrhythmias. Five of the 6 patients had a classic Glenn shunt. The mean right atrial pressure was greater than the pressure of the Glenn shunt (20 +/- 1.6 versus 17 +/- 0.8 mm Hg). Three of the 6 patients also showed a significant gradient between the right or left pulmonary artery wedge and ventricular end-diastolic pressure, indicating pulmonary vein obstruction from the bulging atrial septum or partitioning patch (13 +/- 3 versus 6.8 +/- 1 mm Hg). The remaining 3 patients had revision because of malabsorption (1), hepatomegaly and obstructed right pulmonary veins from bulging atrial septum (1), and tricuspid insufficiency (1). Fontan revision was accomplished with creation of a lateral atrial tunnel and Glenn reconnection in 6 patients, Glenn reconnection in 2, and creation of a lateral atrial tunnel in 1. Four patients had additional procedures. RESULTS: One patient died of Pseudomonas pneumonia. Early extubation, chest tube removal, and postoperative hospital discharge were accomplished in 8 patients (mean = 1.4 +/- 1, 2.8 +/- 1, and 8 +/- 3 days, respectively). One patient died 8 months postoperatively of brain damage after ventricular fibrillation from attempted cardioversion for atrial flutter. The remaining patients had marked improvement in exercise capacity with ability to consistently go to school, improvement in duration and tolerance to arrhythmias on less medication, and resolution of malabsorption up to 37 months postoperatively (mean, 20 +/- 12 months). CONCLUSIONS: We conclude that creation of lateral atrial tunnel with excision of a bulging atrial septum or atrial partitioning patch that causes pulmonary venous obstruction, reconnection of the Glenn shunt, which allows better distribution of flow based on the pulmonary vascular bed and resistance of each lung, or a combination of these procedures will improve Fontan patients.
OBJECTIVES: This study sought to evaluate changes in early morbidity and mortality as well as predictors of outcome in our most recent 339 patients undergoing modified Fontan operations. BACKGROUND: The Fontan operation is the preferred definitive palliation for patients with functional single ventricles. Previously reported early mortality rates after Fontan operation have been substantial. METHODS: Records of 339 consecutive patients who had a Fontan operation at the Mayo Clinic between 1987 and 1992 (recent cohort) were reviewed. This cohort was compared with the previous 500 patients who had Fontan operations performed between 1973 and 1986 (early cohort). RESULTS: Recently, overall early mortality after Fontan has decreased significantly compared with that for the early cohort (from 16% to 9%, p = 0.002). This decline occurred despite increased anatomic complexity of patients. Short-term posthospital survival has also improved significantly in recent patients. One-year survival improved to 88% from 79%, and 5-year survival to 81% from 73% (p = 0.006). Patients with common atrioventricular valves and those who took daily preoperative diuretic medication or had either postoperative renal failure or elevated postbypass right atrial pressure were at increased risk for early mortality. Young age was not found to be a risk factor for early mortality. Early mortality for patients with heterotaxia decreased dramatically: recent 30-day mortality was 15% compared with 41% in the early heterotaxy cohort. CONCLUSIONS: Many factors may have contributed to decreased early mortality after Fontan. Improved patient selection, younger age at time of operation, refinements in surgical techniques and postoperative management may all have had important roles. Proposed technical modifications of the Fontan operation must be evaluated in light of these improved results.
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Obstruction of the systemic venous pathway is a complication in patients who have undergone the Mustard operation for complete transposition of the great arteries. In this report, we discuss intravascular stent placement in three patients for relief of superior vena caval obstruction after the Mustard operation. The clinical history, cardiac catheterization data, and echocardiographic data before and after stent placement are presented for each patient. Overall, four stents were placed. The mean gradients from the superior vena cava to the right atrium before stent placement were 10, 8, and 6 mm Hg, and they decreased to 3, 0, and 1 mm Hg immediately after stent placement. No complications occurred. Doppler echocardiographic assessment of mean gradients before and after stent placement correlated with the mean gradients determined by cardiac catheterization. Intravascular stent placement is effective for relief of baffle obstruction after the Mustard operation. Serial Doppler assessments of the gradient across the stent offer a noninvasive method for follow-up of these patients.
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We studied 1312 consecutive patients who underwent surgery for gallstones in the biliary tract at one university hospital in Siena, Italy, with a systematic classification of gallstones found within the gallbladder. Of these patients, 1226 were found to have gallbladder stones; 94 of these had black pigment gallstones. Of these, 13 patients were found to have black pigment gallstones and cholesterol gallstones within their gallbladder. They all had multiple black pigment gallstones, usually very small (all < 6 mm diameter), in association with larger cholesterol stones in the gallbladder lumen. The cholesterol gallstones were single in seven cases, double in two cases, and multiple in four cases. All 13 of these patients with black pigment stones in association with cholesterol stones had histologic evidence of either adenomyomatosis or Rokitansky-Aschoff sinuses in the gallbladder wall. In nine of the 13 patients, the black pigment stones were located both in the gallbladder lumen and in close association with the gallbladder wall (in areas of adenomyomatosis or in Rokitanski-Aschoff sinuses). In the other four patients, the stones were found in close association with the gallbladder wall alone and not freely mobile within the gallbladder lumen. It is concluded that cholesterol stones and black pigment stones may be found in the same gallbladder. This association is infrequent with an incidence of 13 of 1226 (1.06%) in our series. There appears to be some relationship between the formation of the black pigment stones and the presence of adenomyomatosis or Rokitanski-Aschoff sinuses.(ABSTRACT TRUNCATED AT 250 WORDS)
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OBJECTIVE: To determine whether adults with congenital heart disease have adequate knowledge of infective endocarditis and endocarditis prophylaxis and to ascertain whether an educational program effectively improves patient knowledge and compliance. MATERIAL AND METHODS: We asked 102 consecutive patients to complete a 12-question survey to assess their knowledge of heart disease, infective endocarditis, and endocarditis prophylaxis. RESULTS: Of 102 patients, 100 (98%) completed the questionnaire. Sixty-eight patients knew the name of their heart disease. Fifty patients correctly defined endocarditis, but only 43 knew hygiene measures that could prevent endocarditis. Ninety-six patients knew that they needed to take "a medicine" before dental procedures, and 76 of those patients (79%) knew that an antibiotic was necessary. Patient use of cardiac medications and a history of endocarditis correlated significantly with knowledge of endocarditis. Patients who had been to the Adult Congenital Heart Disease Clinic at least once knew endocarditis prevention measures and the importance of regular dental and cardiology follow-up significantly more frequently than did first-time attendees. Despite educational counseling, however, patient recall of endocarditis and its prevention is disappointing. CONCLUSION: Many adults with congenital heart disease have inadequate knowledge of their cardiac lesion, endocarditis, and endocarditis prophylaxis. Educational efforts for adults with congenital heart disease need to be updated and reinforced regularly.
Idiopathic dilated cardiomyopathy (IDCM) is an often fatal heart disease characterized by ventricular dilation and reduced systolic function. Despite advances in diagnostic and therapeutic techniques that have enabled earlier identification of patients with IDCM, the etiology of the disease in most patients remains unknown. The current 5-year survival rate for patients with asymptomatic IDCM approaches 80%; this is a significant improvement compared with earlier reports of the natural history of IDCM. Men are more commonly afflicted with IDCM, but women with IDCM tend to present with more advanced disease. Recent analysis of 3-year transplant-free survival reveals no gender differences, however. Survival in children with IDCM is variable; 30% of infants die within 2 years of diagnosis, but 5-year survival for childhood IDCM is 60% to 84%. An association of IDCM with the peripartum period is well recognized. The etiology of peripartum cardiomyopathy remains unknown, and some cases are familial. This disease is reversible in approximately 50% of patients, and in patients with intractable congestive heart failure, cardiac transplantation is a viable treatment option. AIDS has been more recently associated with IDCM. Acute left ventricular dysfunction and consequent dilated cardiomyopathy occur with increased frequency in patients with advanced AIDS. The etiology of dilated cardiomyopathy in HIV-infected patients is presently poorly understood. Survival for patients with AIDS after development of left ventricular dysfunction is extremely poor.
We describe the successful diagnostic use of adenosine in two pediatric patients. In the first case, adenosine infusion enabled exclusion of accessory pathway conduction in a patient who had previous evidence of Wolff-Parkinson-White syndrome. In the second case, adenosine infusion aided in the diagnosis of a nonreciprocating reentrant atrial tachycardia in a postoperative Fontan patient. The transient atrioventricular nodal blocking effect of adenosine added diagnostic certainty which was not apparent from the surface 12-lead electrocardiograms or rhythm recording in both patients. As the therapeutic use of adenosine for reciprocating supraventricular tachycardia in children becomes more popular, clinicians should also recognize the situations in which its use may facilitate diagnosis of other supraventricular rhythms.
Dilated cardiomyopathy is a form of heart disease characterized by ventricular dilatation and reduced systolic function. In most patients, dilated cardiomyopathy is a sporadic disease. However, 20% of dilated cardiomyopathy patients may have a familial form of the disease. The aetiologies of both the sporadic and familial forms of dilated cardiomyopathy are unknown in most cases. Dilated cardiomyopathy has a spectrum of clinical and subclinical presentations. During the last 10 years, there have been many investigations concerning the possible aetiologic role of immune factors in dilated cardiomyopathy. It is plausible that an antecedent viral infection initiates an immunological cascade which in turn leads to production of autoimmune antibodies resulting in dilated cardiomyopathy. However, in most dilated cardiomyopathy patients, an antecedent viral infection cannot be identified. Similarly, the trail of immunological research has diverged as different groups have identified distinct autoantibodies or other immune factors in heterogeneous subsets of dilated cardiomyopathy and control patients. In this manuscript, we review the studies which have contributed supportive and confounding evidence to the theoretical autoimmune basis of dilated cardiomyopathy.
OBJECTIVE: To describe the clinical course and outcome of children with idiopathic restrictive cardiomyopathy (IRCM) and to present the Doppler echocardiographic features of this disease in childhood. DESIGN: We reviewed the Mayo Clinic patient database for the period from 1975 to 1993 to identify children who underwent assessment for IRCM. MATERIAL AND METHODS: Clinical records and diagnostic studies, including two-dimensional (2-D), M-mode, and Doppler echocardiograms, were reviewed for each patient. Characteristics were analyzed statistically to determine potential predictors of outcome. RESULTS: Eight children (five girls and three boys) were diagnosed with IRCM between 1975 and 1993 at our institution. The median age at diagnosis was 11 years, and the median duration of follow-up was 11.5 years. Of the eight patients, five died (the median time from initial examination to death was 1 year). All five of these patients had clinical and radiographic evidence of pulmonary venous congestion. In all patients, 2-D and M-mode echocardiography revealed atrial enlargement without ventricular dilatation or hypertrophy. The four patients who underwent detailed diastolic Doppler assessment had findings consistent with restrictive filling and increased left ventricular end-diastolic pressure: (1) short mitral deceleration time, (2) increased pulmonary vein atrial reversal velocity and duration, and (3) pulmonary vein atrial reversal duration greater than mitral A-wave duration. CONCLUSION: The prognosis for children with IRCM is poor. In this small group of patients, absence of pulmonary venous congestion most consistently predicted extended survival. A combined 2-D and Doppler echocardiographic examination provides a reliable noninvasive means of assessing the physiologic and morphologic features of IRCM in children.
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