Life expectancy without surgery in tetralogy of Fallot.

All published autopsy cases of patients with tetralogy of Fallot who died without surgical treatment were studied to determine the life expectancy of such persons. In addition, the data from a study of persons with tetralogy alive in Denmark in 1949 were reanalyzed. The survival data from these two sources were remarkably similar, indicating that 66 percent of persons with tetralogy of Fallot not treated surgically live to age 1 year, 49 percent to age 3 years and 24 percent to age 10 years; thereafter, the hazard function (or instantaneous risk of death) remains constant. The chance of survival is significantly less when pulmonary atresia, rather than stenosis, is present.

Cold cardioplegia versus hypothermia for myocardial protection. Randomized clinical study.

Seventeen of 34 consecutive patients undergoing coronary artery bypass grafting were randomly assigned to one of two methods of myocardial preservation. With the cold cardioplegic method (Group A), a 4 degrees C. asanguineous solution with 30 mEq. of potassium per liter was infused into the aortic root for about 2 minutes immediately after aortic cross-clamping and again after about 45 minutes or when myocardial temperature rose above 19 degrees C. External cardiac cooling was provided by constant infusion of 4 degrees C. Ringer's solution into the pericardium. Seventeen patients were assigned to simple cardiac cooling by hypothermic systemic perfusion before aortic cross-clamping plus external cardiac cooling (Group B). Electromechanical activity ceased within 1 to 2 minutes in Group A but continued throughout the ischemic period in 14 patients in Group B. Myocardial temperature (mean for all observations) during aortic cross-clamping was 17.2 +/- 0.44 degrees C. In Group A and 24.0 +/- 0.70 degrees C. in Group B. Operating conditions were better in Group A. Card-ac function early postoperatively was good in both groups clinically and according to measurements, but only in the cold cardioplegic group (A) was cardiac index not adversely affected by longer cross-clamp time. Myocardial necrosis occurred in both groups but was probably less in the cold cardioplegic group. Thirteen patients (76 percent) in Group A had no electrocardiographic evidence of myocardial injury, compared with eight (47 percent) in Group B (p = 0.08). Eleven (65 percent of Group A had no or short-lived appearance of ceatine phosphokinase isoenzyme (CK-MB), compared with six (35 percent) of Group B (p = 0.08). Time-related CK-MB and SGOT mean levels were consistently lower in Group A.

Preoperative prediction from cineangiograms of postrepair right ventricular pressure in tetralogy of Fallot.

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.

Short-term variations of the right ventricular/left ventricular pressure ratio following repair of tetralogy of fallot.

In 50 consecutive patients undergoing repair of tetralogy of Fallot, the peak systolic right ventricular/left ventricular pressure ratio (pRV/LV) was measured prospectively in the operating room and in the third postoperative week in order to assess its eventual short-term variations. Postoperatively, the ratio fell in 64% of the patients, remained unchanged in 6%, and increased in 30%. The mean postoperative pRV/LV ratio for the group as a whole showed a small but statistically significant fall with respect to the mean operating room pRV/LV ratio (0.47 [standard deviation] +/- 0.16 and 0.52 +/- 0.12, respectively; mean difference, -0.05; p value for the significance level of difference, 0.004). A separate analysis of patients in whom the right ventricular outflow tract was reconstructed with (N = 12) or without (N = 38) a transannular patch showed essentially the same results. Nevertheless, the difference between the pRV/LV ratio measured in the operating room and three weeks postoperatively is not significant in the group with a transannular patch because of the small number of patients. These data corroborate that the pRV/LV ratio measured in the operating room immediately after repair of tetralogy of Fallot reflects closely the postoperative ratio, being slightly higher by an average of 10%, is expected to fall shortly after operation in almost two-thirds of the patients, and is a useful variable in intraoperative decision making and in predicting the surgical result.

[Optimal age for the surgical treatment of aortic coarctation. Statistical study on long-term blood pressure values]. / Age optimum pour le traitement chirurgical de la coarctation de l'aorte. Etude statistique sur les chiffres tensionnels à long terme.

UNLABELLED: A retrospective statistical study on late blood pressure in patients operated for coarctation of the aorta have been done, in order to determine the optimum age for elective surgical treatment. Patients were considered hypertensive when their mean blood pressure exceeded the 90th percentile for their age and sex. The series included 126 isolated coarctation of the aortic isthmus operated at the CMC of the Porte de Choisy. The incidence of residual hypertension at rest in the series is as follows: age 1-5: 16,6 %;age 6-10: 34,3%; age 11-15: 47,5 %. In spite of these results there is no significative difference in the incidence by age group. A similar study by the Massachusetts General Hospital on 77 patients showed essentially the same results. A combination of both series (203 patients) which is possible because the study criteria are the same, gives the following results: age 1-5: 11,4 %;age 6-10: 31,9 %; age 11-20: 40,8 %. There is now a significative difference between age group 1-5 and the others (p Less Than 0,02). IN CONCLUSION: the optimum age for elective correction of coarctation of the aorta is 1-5 year. The incidence of residual hypertension is only 11,4 % in that group (70 % confidence limits: 6 to 20%).

[Adult progeria (Werner's syndrome)]. / Progeria del adulto (síndrome de Werner)

Two cases of Progeria of the adult (Werner's Syndrome) are presented. The outstanding characteristic of the mentioned syndrome is an early, progressive and fatal aging of the patient. The study of those two cases suggested, first: a literature search which has shown 140 references to the Werner's Syndrome up to 1971, and second: a clinical features review about general manifestations and specific particularities concerning the skin, cardiovascular system, eyes, glandular system, laboratory tests, etc. Our two patients, who respond to the signs required by Thannhauser for the diagnosis of the Werner's Syndrome, were very useful to clarify the clinical features review undestalren.

Protection against sudden coronary occlusion by induced mild chronic volume hypertrophy in dogs.

A model of mild chronic volume hypertrophy in the dog has been described which consists of establishing a small arteriovenous (A-V) shunt. The model was utilized to evaluate the protection afforded by mild chronic volume hypertrophy against sudden coronary occlusion. Eight animals were studied, 4 with a single shunt (mean estimated 25% increase in cardiac output) and 4 with a double shunt (mean estimated 57% increase in cardiac output). The A-V shunt resulted in the expected volume hypertrophy by roentgenographic and cardiac weight criteria, but was small enough to produce no signs of exercise intolerance, cardiac failure, or definite electrocardiographic changes. After 6 months, the A-V fistulae were surgically closed and 2 weeks later, under normal hemodynamic conditions, the heart was challenged by sudden, permanent ligation of the left circumflex coronary artery at its origin. The time from ligation to death was measured and compared with 10 control animals without such fistulae. Death occurred in 2 to 13 min in the control animals; in contrast, 5 of the 8 animals with mild chronic volume hypertrophy survived longer than any control animal, and 2 were long-term survivors. The actuarial estimate of the difference in survival was significant (p=0.007) and indicated that the fistulae reduced the hazard of coronary ligation by a factor of 6. A single small shunt afforded protection similar to a double one. Therefore, mild chronic volume hypertrophy induced by a small A-V fistula in the normal dog protects against sudden coronary occlusion. We speculate that the procedure may have a role in the multifactorial prophylactic management of coronary artery disease in asymptomatic high risk subjects, instead of an inapplicable exercise program.

Angiographic study of univentricular heart of right ventricular type.

An angiographic analysis of 10 cases of univentricular heart of the right ventricular type is reported. This congenital malformation is characterized by a large chamber with right ventricular morphology that receives both atrioventricular valves, and a second, smaller chamber, a trabecular pouch, with left ventricular morphology. These chambers are separated by a posterior septum but are connected by an inlet septal defect. The angiographic studies were done using the angled angiographic techniques in three patients and the standard frontal and lateral angiographic views in seven cases. The atrial situs in seven patients was solitus, in one inversus, and in two it was ambiguus with left isomerism. In seven patients the usually large right ventricular chamber received two atrioventricular valves and in four patients, one atrioventricular valve was straddling. Three patients had atresia of one atrioventricular valve. The trabecular pouch was small in seven patients but relatively large in three. In six patients the trabecular pouch was located posterior and to the left of the right ventricular chamber and in four anterior and to the right. Double outlet right ventricle was present in all cases. The aorta arose anteriorly to the pulmonary artery in nine patients and posteriorly in one. An autopsy was performed in one case and its correlation with the angiographic findings was remarkable. The angiographic demonstration of the anatomicaly details of this entity and its associated anomalies was facilitated by angled angiography.

Reproducibilidad del grado histológico en el carcinoma de mama / Reproducibility of histologic grading in breast carcinoma

Con el fin de determinar la reproducibilidad de la gradación del carcinoma de mama según los criterios de Bloom y Richardson se estudiaron prospectivamente 52 carcinomas ductales infiltrantes. Los tumores fueron incluidos en su totalidad y los cortes histológicos, 344 en total, examinados por tres patólogos en forma independiente. Cada caso fue examinado dos veces por los tres patólogos de tal forma que en ningún caso el observador sabía el grado asignado por los otros dos ni por él mismo en la oportunidad anterior. Para el estudio de la reproducibilidad entre patólogos ("interobservador") se comparó cada lectura de un patólogo con la primera y su segunda de los otros dos, obteniéndose un porcentaje de falta de reproducibilidad. Para el estudio de la reproducibilidad de cada patólogo consigo mismo ("intraobservador") se comparó para cada patólogo su primera y su segunda lecturas obteniéndose un porcentaje de falta de reproducibilidad. Para evaluar los resultados por grado se designó "grado verdadero" al promedio de las 6 lecturas de cada caso, obteniéndose de esa manera 4 casos G1, 36 casos G2 y 12 casos G3. Los resultados mostraron una falta de reproducibilidad global "inter" del 27% (335/1248 comparaciones posibles) y una falta de reproducibilidad "intra" global de 23% (36/156 comparaciones). La falta de reproducibilidad "inter" por grados fue: G1 52%, G2 19% y G3 42%, mientras que la "intra" también por grados fue: G1 25%, G2 17% y G3 42%. Analizando los resultados por patólogo se encontró la siguiente falta de reproducibilidad "inter": G1 44, 56 y 56%; G2 17, 19 y 21%; G3 38, 39 y 49%, y una falta de reproducibilidad "intra": G1 25, 0 y 50%; G2 8, 22 y 19%; G3 33, 50 y 42%. De estos resultados se destaca que el G2 presenta dos características que lo diferencian de los G1 y G3 agrupados: a) menor diferencia global "inter" (p<0,01) y b) menor diferencia global "intra" (p<0,01)...