Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Parachute deformity of the tricuspid valve was observed with a severe form of Fallot's Tetralogy with pulmonary atresia in a newborn. Signs of tricuspid stenosis (peripheral oedema, right atrial dilatation, hepatomegaly and conduction defects) were associated with the clinical signs of the principal malformation (severe hypoxaemia). To our knowledge, this is the first reported case of parachute deformity of the tricuspid valve.
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PMID:[Parachute tricuspid valve associated with Fallot's tetralogy]. 11 42

From Jan. 1, 1961, through Dec. 31, 1987, 530 patients underwent an intracardiac operation that included a tricuspid valve procedure. The tricuspid valve was repaired in 351 patients (66%) and replaced in 179 (34%). Mean age was 56.9 years. Risk factors associated with tricuspid valve replacement included tricuspid stenosis (p = 0.02), jugular venous distention (p = 0.04), previous operation (p = 0.05), and angiographic severity of tricuspid valve incompetence (p less than 0.001). There were 78 hospital deaths (15%). Risk factors for hospital death included previous operation (p = 0.03), male gender (p = 0.03), hepatomegaly (p = 0.03), De Vega or Carpentier annuloplasty (repair group only), (p = 0.01), and older age at operation (p = 0.06). Ninety-eight percent of the patients were followed up. There were 185 late deaths (41%). The actuarial survival rate was 20% at 180 months. Risk factors for late death included male gender (p = 0.03), hepatomegaly (p = 0.04), and lack of postoperative warfarin therapy (p less than 0.001). Actuarial freedom from reoperation was 25.5% at 180 months. There was no difference in reoperation rates (p = 0.10) or survival (p = 0.42) whether the tricuspid valve had been repaired or replaced. We conclude that the requirement for surgical treatment of tricuspid valve insufficiency in patients with multivalvular disease constitutes a high risk group for cardiac surgery. Preoperative variables may predict the result of tricuspid valve replacement. Tricuspid valve replacement may be performed with the expectation of a low risk of valve-related events.
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PMID:Tricuspid valve operations in 530 patients. Twenty-five-year assessment of early and late phase events. 229 45

The right ventricle in patients with severe outflow obstruction or atresia and a small tricuspid valve often remains too hypoplastic even after optimal palliation to tolerate biventricular repair with closure of the atrial septal defect. In these patients, nonpulsatile cavopulmonary (Glenn) anastomosis has traditionally facilitated biventricular repair. In 1989, Billingsley and associates reported the addition of a bidirectional cavopulmonary anastomosis to the definitive biventricular repair in patients with hypoplastic right ventricle, pulmonary atresia, and intact ventricular septum. The atrial septal defect was left open with an adjustable snare for later closure. We report five patients with hypoplastic right ventricle (mean diastolic volume 48.4%, mean stroke volume 40.2% of predicted value) who had the atrial septal defect closed at the time of the biventricular repair. Four patients, who had the bidirectional cavopulmonary anastomosis supplementing the biventricular repair, had no evidence of excessive right atrial or superior vena cava hypertension postoperatively. One patient, who had atypical tetralogy of Fallot with tricuspid stenosis, developed recurrent pericardial tamponade and marked hepatomegaly following conventional tetralogy repair with closure of the atrial septal defect. These complications were controlled with the addition of bidirectional cavopulmonary anastomosis 2 months later. Postoperative hemodynamic or Doppler studies in these patients revealed pulsatile flow in the entire pulmonary artery system, including the artery distal to the Glenn anastomosis. This modification of biventricular repair allows primary closure of the atrial septal defect and provides pulsatile arterial flow in the entire pulmonary artery, even when the right ventricle is significantly hypoplastic.
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PMID:Biventricular repair of hypoplastic right ventricle assisted by pulsatile bidirectional cavopulmonary anastomosis. 841 91

Tricuspid valve stenosis and occlusion of superior vena cava are severe complications to Port-a-cath. In a child with SLE, symptoms started to develop about five to seven years after Port-a-cath insertion and cyclophosphamid injections. The patient developed hepatomegaly with abdominal and venous distension. Open heart surgery was necessary to remove the catheter. At operation it was found that the catheter was placed adjacent and through the tricuspid valve. The valve was severely stenosed with thrombus formation. The catheter and thrombus were removed, commissurotomy and bicuspidization of the valve and chordeal replacement performed to achieve an acceptable functional result. The superior vena cava was repaired with a pericardial patch. Retrospective analyses of the echocardiograms and chest x-rays show that the catheter was nearly related to the tricuspid valve and with the tip in the right ventricle. In such circumstances it is recommended with early withdrawal of the catheter, and in patients with immunological disease the indwelling time should be considered and limited.
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PMID:[Tricuspid valve stenosis--an serious complication to Port-a-cath]. 1634 47