ABSTRACT
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.
Subject(s)
Ectopia Cordis , Pentalogy of Cantrell/diagnosis , Heart Diseases , Magnetic Resonance Spectroscopy , Rare Diseases , Ultrasonography, PrenatalABSTRACT
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.