Pentalogía de Cantrell: actualización y nuevas perspectivas / Pentalogy of Cantrell: update and new perspectives

La pentalogía de Cantrell es una enfermedad rara, con características clínicas, anatómicas y embriológicas peculiares. Esta entidad es una variedad toracoabdominal de la ectopia cordis en la que se asocian cinco anomalías: defecto epigástrico de la línea media abdominal supraumbilical, defecto del tercio inferior del esternón, deficiencia del segmento anterior del diafragma, defectos pericárdicos y malformaciones cardíacas congénitas. La comunicación interventricular e interauricular se encuentra en la mitad de los casos y puede asociarse a otras anomalías extracardíacas como espina bífida, diversas malformaciones del sistema nervioso central, divertículo de Meckel, poliesplenia, entre otros. El diagnóstico se hace mediante ultrasonografía fetal en el primer trimestre de la gestación; en casos incompletos la tomografía helicoidal y la resonancia magnética complementan el diagnóstico. El tratamiento es quirúrgico y en varias etapas, lo primordial es proteger las vísceras expuestas y corregir la cardiopatía congénita. Los resultados aún son controversiales. El pronóstico de la enfermedad depende de la extensión del defecto, el diagnóstico temprano y el manejo perioperatorio. Nosotros consideramos que la pentalogía de Cantrell es una enfermedad compleja en la que es necesaria la intervención multidisciplinaria del equipo de salud para llegar al diagnóstico preciso y decidir el mejor tratamiento.

The pentalogy of Cantrell is a rare disease, with uniqueclinical, anatomical and embryological characteristics. This disease is a thoraco-abdominal variety of ectopia cordis. Five anomalies are associated: epigastric defect of theover umbilical abdominal mean line, defect of inferiorbreastbone third, defect of the anterior segment of the diaphragm, pericardial defects and congenital cardiac malformations. Ventricular septal defect and atrial septal defects are in half of the cases and it could be associatedto other extra cardiac anomalies as bifid spine, several malformations of central nervous system, meckeldiverticulum and polispleenia, among others. The diagnosis is made by fetal ultrasonography in the first trimesterof pregnancy. In incomplete cases, helical tomography and magnetic resonance imaging complete the diagnosis.The treatment is surgical and is carried out in severalstages. It is fundamental to protect the exposed viscera and to correct the congenital heart disease. The results still are controversial. The prognosis of the disease depends on the defect extension, time of diagnosis and peri-operative handling. We considered that the pentalogyof Cantrell is a complex pathology. A multidisciplinary health team intervention is necessary to reacha precise diagnosis and to decide the best treatment.

Improvement of diastolic function after regression of left ventricular hypertrophy / Mejora de la función diastólica tras regresión de la hipertrofia ventricular izquierda

OBJECTIVE: To evaluate the diastolic function after regression of left ventricular hypertrophy, in mild to moderate hypertension treated with angiotensin converting enzyme(ACE) inhibitor and, if necessary, with a diuretic. METHODS: Ninety-eight hypertensive patients with left ventricular hypertrophy (LVH) and abnormal left ventricle diastolic function indexes received captopril (Capotena) 50 to 200 mg/day plus chlortalidone during 12 months to reach blood pressure control, defined as a diastolic blood pressure < or =90 and systolic blood pressure < or =140 mm Hg. Left ventricular (LV) mass index was calculated by M mode and two-dimensional echocardiography, and left ventricular diastolic function was assessed by transmitral pulsed Doppler ultrasound every 3 months. RESULTS: Sixty-three patients were women and 35 were men, mean age was 53.4 +/- 8.4 years (range 34-70). Thirty-six patients had mild (36.7%) and 62 (63.3%) moderate hypertension. Treatment reduced significantly both systolic pressure from 165 +/- 13 to 137 +/- 12.9 mm Hg (p<0.05) and diastolic pressure from 99 +/- 8.6 to 86 +/- 6.37 mm Hg (p<0.05). LV mass index decreased from 155.4 +/- 32.9 to 121.7 +/- 29.14 g/m2 (p<0.05). Late diastolic filling velocity (A wave) and the ratio of E/A waves improved (p<0.05), but early diastolic filling velocity (E wave) and isovolumetric relaxation time did not change with treatment. CONCLUSIONS: Some indexes of diastolic function improved after regression of left ventricular hypertrophy and good blood pressure control with captopril and chlortalidone.

Factores relacionados con hemorragia mayor durante la cirugía cardíaca bajo circulación extracorpórea / Factors associated to heavy bleeding during heart surgery with cardiopulmonary bypass

OBJECTIVE: To evaluate the risk factors related to bleeding in patients subjected to cardiac surgery using an extracorporeal circulation pump. MATERIAL AND METHODS: A study of cases and controls was designed performed in adults, matched by age and gender with indication of elective cardiac surgery using an extracorporeal circulation pump. Those patients with hepatic failure or blood coagulation disorders were excluded. Results were analyzed by Student t test, chi-square, and logistic regression analysis, considering p values significant at <0.05. RESULTS: The patients with functional class III or IV of the CCS presented more bleeding (p = 0.006), although there were no differences when comparing the ejection fraction in both. Obesity, dyslipidemia, and arterial hypertension were identified as risk factors. The coronary artery bypass graft, multivessels bypass surgery, and prolonged surgical times are associated to the complication (p < 0.05). According to the discriminant function analysis, the cardiopulmonary derivation time, weight, aortic clamp time, and heparin doses are the factors that better discriminate the possibility of hemorrhage. CONCLUSIONS: Heavy bleeding is related to ischemic patients, longer cardiac surgery time, overweight, and heparin doses during surgery.

Cardiopatía isquémica en mujeres mexicanas / Coronary artery disease in Mexican women

OBJECTIVE: To identify differences between both genders regarding coronary artery disease occurrence. PATIENTS AND METHODS: In referred patients for cardiac catheterization we investigated coronary risk factors, clinical diagnosis, associated diseases, left ventricle ejection fraction, and coronary lesions. Data were analyzed by X2 test, Student t test, odds ratio and confidence intervals, a p value < 0.05 was considered significant. RESULTS: We studied 586 patients, 409 were men. Women were older than men (59.43 +/- 9.93 vs 56.80 +/- 10.14 years old, p < 0.05). The frequency of coronary lesions in women was 56 vs 81% in men. The proportions of positive nuclear medicine studies (14 vs 16%) and exercise treadmill test (36 vs 28%) were similar. Acute myocardial infarction was the most frequent diagnosis in men (46%) whereas in women it was angor pectoris (57%). Smoking was observed more in men (72 vs 26%) and systemic arterial hypertension in women (65 vs 48%), There were no differences in diabetes mellitus and dyslipidemia frequencies. CONCLUSION: Systemic arterial hypertension was the risk factor more frequent in women, where as in men it was smoking. Ischemia induction tests are less specific to identify coronary atherosclerosis in women. In spite of the clinical data, image and laboratory results, we had a great proportion of women without coronary lesions.