Cirurgias de Derivação do Ventrículo Pulmonar

Cavopulmonary connections have been extensively used in the palliation of complex forms of congenital heart disease requiring some form of right heart bypass. We examine the mid term outcomes of pulmonary ventricle bypass operations in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and April 2006, 62 patients underwent pulmonary ventricle bypass operations: bidirectional cavopulmonary anastomosis (Glenn procedure), total cavopulmonary connections (Fontan procedure) and one and a half ventricle correction in two cases. Age at operation averaged three years (range: 0.42-25 years) for the Glenn procedure and seven years (range: 3-14 years) for the Fontan procedure. There were 36 male patients (58%) and 26 female patients (42%). The most common indication for surgery was the single ventricle defect, present in 66% of patients. Associated lesions included: transposition of the great arteries in 16 patients (35.6%), bilateral superior vena cava in four patients (8.9%), situs ambigus in five patients (11%), situs inversus in another patient (2.2%), Ebstein disease in one patient (2.2) and coronary fistula in another patient (2.2%). Sub-aortic stenosis was present in one patient (2.2%). Palliative surgery was performed in all, but three patients (5%), before the Fontan procedure. RESULTS: Thirty two patients underwent bidirectional cavopulmonary anastomosis and thirty patients underwent cavopulmonary connections, total or 2nd stage. Mean cardiopulmonary bypass times were 50.6+/-21.9 minutes for the Glenn procedure and 88.5+/-26.3 minutes for the Fontan procedure. There was no intra-operative mortality, but two patients (3.2% (died in the first month after surgery; one due to failure of the Glenn circuit and sepsis and the other due to a low cardiac output syndrome and multi-organ dysfunction. Mean ventilation time was 5.2+/-1.7 hours for the Glenn operation and 6.2+/-3.2 hours for the Fontan operation. The mean length of stay in ICU was 3.4+/-2.8 days for patients undergoing the Glenn operation and 4.6+/-3.1 days for patients undergoing the Fontan operation and the mean length of hospital stay was 10.6+/-5.8 days for the Glenn operation and 19.1+/-12.6 days for the Fontan operation respectively. The mean follow up time was 4+/-2.1 years (minimum 0 years and maximum seven years), most patients being in NYHA class I. Epicardiac pacemakers were implanted in three patients due to arrhythmias. Two re-operations (6.7%) were needed, both in the same patient, after the Fontan procedure, this patient eventually died a few years after surgery. CONCLUSIONS: The immediate and mid term outcomes of pulmonary ventricle bypass operations can have excellent results. From our point of view there has been an improvement, namely in the use of the extracardiac conduit technique in the 2nd stage of the Fontan operation.

Os Factores Genéticos na Perda Gestacional

Pregnancy loss is the most common obstetric complication. Multiple factors have been associated with recurrent or sporadic pregnancy loss, and genetic factors, particularly at earlier gestational ages, are the most important ones. The proportion of miscarriages due to chromosomal factors decreases with increasing gestational age. The most common chromosomal abnormalities in early losses are autosomal trisomies, monosomy X and polyploidy. In later losses, aneuploidies are similar to those found in live newborns (trisomies 21,18 and 13, X monosomy and polysomy of sex chromosomes. In cases of recurrent miscarriage the most common cytogenetic changes are trisomies, polyploidy, monosomy X and unbalanced translocations. Identification of the causes of pregnancy loss facilitates the families’ grief and may indicate if there is the risk of repetition, in order to reduce recurrence. The investigation recommended in each case is far from consensual, and the cost/benefit analysis of diagnostic exams is essential. The determination of the karyotype of the products of conception is indicated in cases of fetal loss and recurrent miscarriage, while the parental karyotypes should be performed only in selected cases. Couples with identified genetic conditions should be counseled about reproductive options, including prenatal or pre-implantation diagnosis. Surveillance of a future pregnancy should be multidisciplinary and adjusted in each case. The cytogenetic factors, due to their high prevalence and complexity, have a fundamental, but still not completely clear, role in pregnancy loss.

Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

An infrequent but devastating late complication of Fontan circulation is protein-losing enteropathy (PLE), which results from unbalanced lymphatic homeostasis. Surgical decompression of the thoracic duct by redirecting its drainage to the pulmonary venous atrium has been introduced recently as a possible treatment. This report describes a single-institution experience with this innovative procedure in 2 patients with failing Fontan circulation with PLE refractory to optimized medical therapy.