1/9. Circumscribed intestinal protein loss with deficiency in CD4 lymphocytes after the fontan procedure.Protein-losing enteropathy is an important complication after right heart bypass operations (fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4 lymphocytes but normal proportion of CD8 lymphocytes (CD4 14%, CD8 23%) and decreased serum levels of immunoglobulin g. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4 to CD8 lymphocytes <1 due to an almost selective loss of CD4 lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4 lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4 lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the fontan procedure.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/9. Absorbable pulmonary artery banding in tricuspid atresia.A child with tricuspid atresia, concordant ventriculoarterial connections, large ventricular septal defect, and elevated pulmonary artery pressure underwent pulmonary artery banding with a polydioxanone ribbon. This procedure was successful in this patient as the ventricular septal defect became restrictive while the banding was fully absorbed after 5 months. This technique could be included in the panel of surgical strategies for patients with single ventricle physiology and potential but insufficient subpulmonary stenosis in early infancy.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/9. pregnancy after fontan repair of complex congenital heart disease.We describe four successful pregnancies in three women who had previously had a Fontan repair for congenital heart disease. Each pregnancy resulted in a live birth and there was no maternal mortality The infants were premature, being delivered at 26, 30 and 35 weeks, and weighing 1,020, 1,333 and 1,930 g respectively The fourth infant was born at 32 weeks and no birthweight is available. Maternal complications occurred and were those anticipated after a Fontan repair. Two mothers required treatment for supraventricular arrhythmias (atrial flutter and fibrillation). Ventricular failure was present in two mothers and required ongoing drug treatment. Raised systemic venous pressures caused peripheral oedema in two mothers and hepatomegaly and ascites in one mother. The physiology, potential complications, anaesthetic concerns and drug treatment in pregnancy after Fontan repair are discussed.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/9. Altered dystrophin expression in the right atrium of a patient after fontan procedure with atrial flutter.Underlying mechanisms in the development of atrial flutter or intra-atrial re-entry tachycardia in patients with structural cardiac abnormalities remain poorly defined. The right atrial myocardium from two patients with congenital heart disease was evaluated, of whom one presented with severe right atrial dilation and arrhythmia and the other with a normal right atrium, to assess whether increased right atrial pressure and volume overload give rise to sarcolemmal alteration. N-terminus dystrophin staining in the atrium from the patient who had undergone a fontan procedure showed a normally distributed but significantly reduced staining signal compared with the second patient. This is the first report that patients with severe right atrial dilation and atrial flutter have marked reduction in atrial dystrophin expression.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/9. Reversal of fenestration flow during ventricular systole in Fontan patients in junctional or ventricular paced rhythm.Sinus node dysfunction is relatively common in patients with Fontan palliation for single ventricle congenital heart disease, and such patients often are in junctional rhythm or may have pacemaker systems for bradycardia. Because the physiologic determinants of left atrial pressure play a major role in determining pulmonary blood flow and therefore cardiac output in Fontan patients, the loss of atrioventricular (AV) synchrony in junctional rhythm or demand ventricular pacing in these patients might be expected to influence cardiac output. We report two cases of Fontan patients with the absence of AV synchrony that resulted in reversal of flow through the Fontan fenestration during ventricular systole. In both cases, restoration of AV synchrony by atrial pacing resulted in the elimination of retrograde fenestration flow, increased cardiac output, and improved clinical status.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/9. A Gore-Tex 'new-innominate' vein: a surgical option for complicated bilateral cavopulmonary shunts.A bilateral bidirectional cavopulmonary shunt was performed in a cyanotic 14-month-old girl who had tricuspid and pulmonary valve atresia, with right pulmonary artery (RPA) hypoplasia (3 mm), bilateral superior vena cavae and a ductus arteriosus-dependent pulmonary blood flow. Because of 62% postoperative arterial oxygen saturation and a right superior vena cava (RSVC) pressure of 30 mmHg, a 5 mm Gore-Tex tube was interposed to connect the two superior venae cavae. The creation of a 'new-innominate' vein allowed decompression of the right superior vena cava and an increase in arterial oxygen saturation to 86%.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/9. Percutaneously adjustable pulmonary artery band.BACKGROUND. pulmonary artery banding, originally introduced to palliate increased pulmonary blood flow, is now primarily used for complex congenital cardiac defects with a prohibitive risk in the early repair, such as tricuspid atresia, or occasionally to prepare the left ventricle for eventual arterial switch. Conventional banding is, however, often poorly tolerated, and the precise adjustment required to reduced pulmonary blood flow or sufficiently challenge the left ventricle is difficult and can cause life-threatening hemodynamic changes. methods. We designed a band that allows accurate tightening and is reversible during the operation and at the bedside with the chest closed, allowing precise gradual adjustment over days, thereby minimizing cardiovascular instability. This design is the extension of previous work done by one of us on an adjustable annuloplasty ring. RESULTS. The band has been used for a 16-month-old child with tricuspid atresia type 1C who had undergone two failed attempts at banding. Hemodynamic measurements after 3 months confirmed achievement of the desired pulmonary pressure. CONCLUSIONS. This design, the simple insertion, and the ability to finely readjust the banding over days, combined with excellent patient tolerance, encourage further use of our adjustable band.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/9. The fontan procedure in adults.BACKGROUND: A retrospective clinical study was performed to document the course of adult patients undergoing the fontan procedure. methods: Between 1982 and 1994, 21 adults aged 18 to 40 years (mean age, 27 /- 7 years) underwent a fontan procedure. Anatomic diagnosis was tricuspid atresia in 9, double-inlet left ventricle in 4, and various single ventricles in 8. Four underwent a right atria-right ventricle connection, 13 had a right atria-pulmonary artery connection, and 4 had a lateral-tunnel Fontan. Three of these 4 had a snare-adjustable atrial septal defect. Preoperative risk factors assessed were left ventricular end-diastolic pressure greater than 10 mm Hg, ejection fraction lower than 0.45, mean pulmonary artery pressure higher than 15 mm Hg, transpulmonary gradient greater than 10 mm Hg, pulmonary artery abnormalities, and atrioventricular valve regurgitation. Mean preoperative risk score was 1.6 /-1.1. Mean new york heart association class was 2.6 /- 0.5. RESULTS: The operative mortality rate was 5% (1/21). Six patients (30%) had a major complication, four being prolonged effusions. One patient was lost to follow-up; the remaining 20 have been followed for a mean of 7.4 /- 3.8 years. At follow-up, mean new york heart association class was 1.7 /- 0.5. There has been one late death (5%) at 9 2/3 years, which was probably due to ventricular arrhythmia. Three patients (16%) have required and survived reoperation. During follow-up, 7 patients (37%) have had development of atrial arrhythmias requiring medication, and 2 have been treated for ventricular arrhythmias. CONCLUSIONS: These results indicate that properly selected adults can undergo the fontan procedure with low morbidity and mortality. However, late-developing arrhythmias, need for reoperation, and decreasing ventricular function are serious problems that mandate careful follow-up.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
9/9. Preoperative ECMO in congenital cyanotic heart disease using the AREC system.BACKGROUND: In cyanotic congenital heart disease, oxygen delivery is impaired either by reduced pulmonary perfusion or by limited entry of oxygenated blood into the systemic circulation. Additional impairment of oxygen delivery (eg, in pulmonary hypertension) leads to hypoxic cerebral damage. Preoperative extracorporeal membrane oxygenation enables oxygenation in otherwise untreatable cases. methods: In 3 neonates suffering from cyanotic congenital heart disease (1 with tricuspid atresia and 2 with transposition of the great arteries) with arterial desaturation despite application of prostaglandins, balloon atrioseptostomy, and eventually inhaled nitric oxide during intermittent positive-pressure ventilation with an inspired oxygen fraction of 1, oxygenation could only be established by means of preoperative extracorporeal membrane oxygenation. We used a venovenous single-lumen cannula tidal-flow extracorporeal membrane oxygenation system described by Chevalier and associates that has previously been used for extracorporeal lung support. In this system, called AREC (assistence respiratoire extra-corporelle), alternating clamps and a nonocclusive roller pump were used. RESULTS: All 3 survived. CONCLUSIONS: We conclude that the AREC system enables sufficient preoperative oxygenation in patients with cyanotic congenital heart disease and hypoxia in spite of all conventional therapeutic means. This provides a stable preoperative condition for elective palliation or correction.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |