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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The equation that we proposed in this JOURNAL (1977), relating the postrepair ratio between peak right ventricular pressure and peak left ventricular pressure (PRV/LV) to the intraoperatively measured internal diameter of the narrowest point in the pulmonary arterial outflow tract (formerly called pulmonary "anulus"), performed well when prospectively tested in 25 patients undergoing repair of the tetralogy of Fallot. The observed PRV/LV in all 21 patients in whom no transannular patch was used fell within the 70 percent confidence limits (1 standard deviation) of that predicted from the measured diameter. A trivial difference (-0.03 +/- 0.069 PRV/LV units, P = 0.08) was found between predicted PRV/LV and that observed after repair, with body surface area (BSA) used for normalization. When the four additional patients who had secondary transannular patching are included, or where the normalization was with weight, the differences were not significant (p greater than 0.2). A slightly revised equation has been devised using the combined data from our original group and the patients used for prospective testing. This equation predicts with reasonable accuracy (r = -0.69, p less than 0.0001) postrepair PRV/LV without transannular patching from the intraoperatively measured diameter of the pulmonary arterial outflow tract. We believe it is therefore helpful in the operating room in making the important decision for or against using transannular patching.
J Thorac Cardiovasc Surg 1979 Apr
PMID:Decision-making in repair of tetralogy of Fallot based on intraoperative measurements of pulmonary arterial outflow tract. 42 86

To aid preoperative decision-making, we have related the ratio of postrepair peak pressure in the right and left ventricles (PRV/LV) to preoperative cineangiographic measurements in a retrospective study of 135 patients undergoing complete repair of tetralogy of Fallot or tetralogy of Fallot with pulmonary atresia. Postrepair PRV/LV was related to the preoperative diameter of right (DRPA) and left (DLPA) pulmonary arteries normalized to the descending thoracic aorta (DescThAo) in patients undergoing repair with transannular patching or a valved external conduit by the dquation: PRV/LV = 0.4840/(DRPA/DescThAO + DLPA/DesThAo) + 0.2007. Stenosis of the right pulmonary artery orifice and pulmonary artery arborization abnormalities incrementally increased postrepair PRV/LV. When a transannular patch was not used in classical tetralogy of Fallot, an increment of postrepair PRV/LV usually resulted, depending upon the size of the "anulus" measured intraoperatively: Incremental PRV/LV = 0.09437 . exp(-0.6344 . Z) where Z is a normalized expression in circumference terms of the diameter of the pulmonary arterial outflow tract (DPAOT) measured intraoperatively after infundibular dissection and valvotomy. DPAOT is itself related to the cineangiographically measured pulmonary valve anulus diameter (DPVA): DPAOT = 3.357 . DPVA0.5789 . BSA0.1551. In toto, these relations allow postrepair PRV/LV without transannular patching to be estimated from preoperative cineangiographic measurements. This allows preoperative predictiom in classical tetralogy of Fallot of the need for transannular patching, and in infants this can determine the choice between primary one-stage repair and two-stage repair. Prediction of postrepair PRV/LV when transannular patching or an external conduit is planned allows identification of patients in whom right and left pulmonary arteries are too small for safe complete repair, and in them an initial palliative operation should be done to enlarge the arteries.
J Thorac Cardiovasc Surg 1979 Oct
PMID:Preoperative prediction from cineangiograms of postrepair right ventricular pressure in tetralogy of Fallot. 48 Sep 61

Thirteen (16 percent) of 80 patients with tetralogy of Fallot and pulmonary atresia undergoing corrective operations between Jan. 1, 1967, and Jan. 1, 1978, died in the hospital. The hospital mortality rate was 13 percent (10 deaths) among the 77 patients with confluent right and left pulmonary arteries. The risk of operation was not significantly affected by age at operation or by use of a valved external conduit versus a transannular outflow patch. It was affected (p = 0.008) by the ratio of peak right ventricular to left ventricular pressure (PRV/LV) immediately after repair. This (PRV/LV) was determined primarily by size of left and right pulmonary arteries. An equation was developed relating postrepair PRV/LV to diameter of right and left pulmonary artery (normalized by dividing by size of descending thoracic aorta), body surface area, and possible arborization abnormalities and stenoses of the right and left pulmonary arteries. Cardiac performance after repair was better in those in whom a transannular patch was used rather than a valved external conduit. Important pulmonary dysfunction postoperatively occurred more often in patients with large "bronchial" arteries than in those without them, but was less when these were not ligated. Four (8 percent) of 48 traced hospital survivors died late postoperatively. Reoperations late postoperatively were required only in patients receiving valved external conduits. Forty (91 percent) of 44 living traced patients are asymptomatic.
J Thorac Cardiovasc Surg 1978 Sep
PMID:Surgical treatment of tetralogy of Fallot with pulmonary atresia. 68 64

Fifty-five consecutive patients with the tetralogy of Fallot underwent intracardiac repair in a 12 month period. A standardized protocol for outflow tract enlargement was used. When the measured diameter of the pulmonary valve ring was at least the "minimum acceptable pulmonary valve ring diameter," primary patch-graft enlargement across the valve ring was not done (Group I, 34 patients); when it was less, primary patch-graft enlargement was done (Group II, 21 patients). The right ventricular-left ventricular systolic pressure ratio (PRV/LV) after repair without patch-graft enlargement across the ring was related to the size of the ring. Sixteen (94 percent) of 17 such patients with normal-sized or large pulmonary valve rings (according to the criteria of Rowlatt, Rimoldi, and Lev) had PRV/LV equal to or less than 0.65; four (80 percent) of five patients with smaller rings but within the 50 percent confidence limits had PRV/LV equal to or less than 0.65; five (56 percent) of nine with still smaller rings but within the 72.5 percent confidence limits had PRV/LV equal to or less than 0.65. In Group II, 15 patients had abnormally small rings, but after patch-graft enlargement the mean PRV/LV was 0.44 +/- 0.140. A surgical protocol based on these data has been developed.
J Thorac Cardiovasc Surg 1977 Sep
PMID:Surgical management of pulmonary stenosis in tetralogy of Fallot. 89 72

Coronary thrombosis complicating polycythaemia rubra vera in a 42-year-old woman was treated with coronary artery bypass grafting. Nonatherosclerotic causes of coronary obstruction, though rare, should be considered, especially in younger patients.
Scand J Thorac Cardiovasc Surg 1991
PMID:Coronary artery bypass grafting for coronary thrombosis complicating polycythaemia rubra vera. Case report. 194 12

We present a case of acute total aortic occlusion at the time of cardiac catheterization in a 62-yr-old female with an acute myocardial infarction and newly diagnosed polycythemia vera. Despite normalization of serum viscosity and red cell mass by phlebotomy, her predisposition to thrombosis persisted. Caution is advised when considering cardiac catheterization in patients with this disease.
Cathet Cardiovasc Diagn 1988
PMID:Acute total aortic occlusion during cardiac catheterization associated with polycythemia vera. 336 59

Based on our experience and the data presented here the following general approach may be used in the management of patients with tetralogy of Fallot: 1. Symptomatic infants under 6 months of age should be considered for an initial palliative shunt, preferably a Blalock-Taussig shunt or a Gore-tex modification of that procedure. 2. Symptomatic patients older than 6 months of age should be considered candidates for total correction, unless there are anatomic contraindications (anomalous left anterior descending artery, severe hypoplasia of the main pulmonary artery, multiple branch stenoses, multiple ventricular septal defects, or pulmonary atresia), or the predicted PRV/LV is greater than 0.7. 3. Total correction with closure of shunt should be undertaken in all patients prior to 5 years of age.
Cardiovasc Clin 1981
PMID:Tetralogy of Fallot. 616 37

Between 1975 and 1982, valve xenograft conduits were used to establish continuity between the right ventricle and the pulmonary arteries in 28 patients between the ages of 3 to 39 years (mean 14.7 years) with 4 hospital deaths (14%). The indications for operation were pulmonary atresia types I and II in 7, extreme tetralogy of Fallot with hypoplastic pulmonary artery and valvular ring in 10, secondary obliteration of the infundibulum following Waterston shunt in 4, pulmonary valve insufficiency after transannular right ventricular outflow tract patch in 5 and tetralogy of Fallot with anomalous coronary artery in 2. Twenty-one patients (87%) between 9 and 41 years of age (mean 17.4 years) were available for follow-up 1/2 to 8 years after operation. The late death incidence during the follow-up period was 8% (2/24). Postoperative cardiac catheterization, which included right and left ventriculogram and measurements of gradients, was performed in 14 patients 4 months to 6 years after operation. Four patients were in New York Heart Association (NYHA) class 1, 6 in class II and 4 in class III. The other 7 non-catheterized patients were in class II. There were resting peak systolic gradients of 15 to 35 mmHg in 4, 36 to 55 mmHg and more than 55 mmHg across the xenograft valve and the proximal anastomosis in 4 other patients. The right and left ventricular end-diastolic pressures (RVEDP, LVEDP) averaged 18 and 17.5 mmHg, respectively, in 3 patients. The mean ratio of PRV/PLV quotient in NYHA class I group was 0.3, in class II 0.45 to 0.7 and in class III greater than 0.7 (including 2 with residual VSD and pulmonary hypertension). Late densitometric studies for assessing pulmonary valve competence revealed regurgitant fraction of up to 40% of the total stroke volume in the absence of a residual shunt 2 to 4 years after conduit implantation. Three children underwent uneventful surgical replacement of calcified xenograft conduit 1 1/2 to 4 1/2 years after surgery with antibiotic-sterilized valve allograft. Four other patients have residual ventricular septal defects (VSD), 2 of them underwent surgical reclosure while the other 2 patients with pulmonary hypertension still have their residual VSD open.(ABSTRACT TRUNCATED AT 400 WORDS)
Thorac Cardiovasc Surg 1984 Aug
PMID:Late results of valve xenograft conduits between the right ventricle and the pulmonary arteries in patients with pulmonary atresia and extreme tetralogy of Fallot. 620 17

The hemodynamics, contractility and compliance of the right ventricle were examined during the early postoperative phase in 9 children operated for correction of tetralogy of Fallot. The same assessments were made in 5 patients after the transventricular closure of ventricular septal defects (control group). A further reduction of the PRV/LV quotient was observed in the Fallot group during the first 3 hours postoperatively. The contractility of the right ventricle (dp/dt max) was greatly reduced in all cases. The pressure/volume relationship of the right ventricle showed severe disturbance in compliance. The type of correction (with/without outflow tract patch or monocusp) did not appreciably affect the results. The hemodynamic changes observed in the control group (VSD) were considerably less pronounced. Apparently it is not the ventriculotomy but the infundibulectomy which is the traumatic factor of corrections in the area of the right ventricular outflow tract.
Thorac Cardiovasc Surg 1984 Aug
PMID:The hemodynamics and contractility of the right ventricle in the early postoperative phase following correction of tetralogy of Fallot. 620 18

Retrospective analysis was undertaken to determine the influence of residual pulmonary stenosis and surgically induced pulmonary insufficiency on the operative mortality rate in 104 patients with tetralogy of Fallot who underwent total correction between 1967 to 1970 at First Department of Surgery, Osaka University Hospital. This study revealed that, in order to improve the operative outcome in this anomaly, it is necessary to correct pulmonary stenosis to the point of the right-to-left ventricular peak pressure ratio (PRV/LV) less than 0.8 as well as preventing severe pulmonary insufficiency. Through this study, the criteria for enlargement of the right ventricular outflow tract (RVOT) for each given body size which will produce a PRV/LV of less than 0.8 were derived in 1971. If the size of the RVOT after infundibulectomy and valvotomy is smaller than that prescribed by the criteria, an outflow patch must be placed on the pulmonary outflow tract. Since 1971, these criteria have been used in total correction of this anomaly in our affiliated hospital without any problem and have been yielding good operative results. Postoperative hemodynamic studies have shown that our criteria are suitable.
J Thorac Cardiovasc Surg 1980 Oct
PMID:The criteria for reconstruction of right ventricular outflow tract in total correction of tetralogy of Fallot. 742 Dec 90


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