法洛氏四联症

法洛氏四联症 Related Articles, Links 1: J Pak Med Assoc. 2002 Feb;52(2):77-82. Abnormalities of conduction after total correction of Fallot's tetralogy: a prospective study. Hussain A, Malik A, Jalal A, Rehman M. Department of Cardiac Surgery, National Institute of Cardiovascular Diseases, Karachi. OBJECTIVE: To determine the frequency of post-operative conduction abnormalities in Pakistani patients undergoing total correction for Fallot's Tetralogy. PATIENTS AND METHODS: One hundred and fifteen patients of Fallot's Tetralogy underwent definitive repair between January, 1999 and April, 2000. Their mean age was 12.89 years (range 3-30 years). One hundred nine patients (94.78%) had severe cyanosis and 6 patients (5.21%) were moderately cyanotic due to mild right ventricular outflow tract (RVOT) obstruction. Thirty percent patients required frequent hospital admissions within 6 months before the time of operation due to -hyper-cyanotic spells. The mean haematocrit was 50.83 (range 28-71). The majority of patients were in NYHA class-III (57%) and 45% had previous palliative shunt procedures done. Surgical access was through the RVOT in 90% cases and trans-atrial in 10%. RVOT patch was used in 55.9%, Pulmonary artery patch in 13.5% and trans-annular patch in 17.1% of cases. RESULTS: The mean bypass time was 79.15 min and the mean cross clamp time 51.23 min. Average stay in the intensive care unit was 4.48 days. Twenty-three patients required re-exploration for bleeding. Sixty-nine patients required inotropic support. Fifteen patients had transient heart blocks and two had complete heart block requiring permanent pace-maker. Nineteen patients had various transient arrhythmias which were managed medically. Univariate analysis showed that higher age at operation, low preoperative heart rate, prolonged bypass time, prolonged cross clamp time and presence of patch on pulmonary artery were more common in patients who developed various heart blocks. However, none of these factors had statistical significance or definitive cause-effect relationship with heart blocks. CONCLUSION: With careful surgical technique, total correction of Fallot's tetralogy can be conducted in children and young adults, with a very low risk of conduction abnormalities. PMID: 12073716 [PubMed - indexed for MEDLINE] Related Articles, Links Pediatr Cardiol. 2001 May-Jun;22(3):233-7. Pressure-regulated volume control vs volume control ventilation in infants after surgery for congenital heart disease. Kocis KC, Dekeon MK, Rosen HK, Bandy KP, Crowley DC, Bove EL, Kulik T. Department of Pediatrics and Surgery, University of Southern California School of Medicine, Los Angeles, CA 90027, USA. The objective of this investigation was to compare how two modes of positive pressure ventilation affect cardiac output, airway pressures, oxygenation, and carbon dioxide removal in children with congenital heart disease in the immediate postoperative period. The investigation used a one group pretest-post-test study design and was performed in the pediatric cardiac intensive care unit in a university-affiliated children's hospital. Nine infants were enrolled immediately after repair of tetralogy of Fallot (2) or atrioventricular septal defects (7) with mean weight = 5.5 kg (4.2-7.3 kg). Children were admitted to the pediatric cardiothoracic intensive care unit after complete surgical repair of their cardiac defect and stabilized on a Siemen's Servo 300 ventilator in volume control mode (VCV1) (volume-targeted ventilation with a square flow wave pattern). Tidal volume was set at 15 cc/kg (total). Hemodynamic parameters, airway pressures and ventilator settings, and an arterial blood gas were measured. Patients were then changed to pressure-regulated volume control mode (PRVC) (volume-targeted ventilation with decelerating flow wave pattern) with the tidal volume set as before. Measurements were repeated after 30 minutes. Patients were then returned to volume control mode (VCV2) and final measurements made after 30 minutes. The measurements and results are as follows: After correction of congenital heart defects in infants, mechanical ventilation using a decelerating flow wave pattern resulted in a 19% decrease in peak inspiratory pressure without affecting hemodynamics, arterial oxygenation, or carbon dioxide removal. Publication Types: , Clinical Trial PMID: 11343150 [PubMed - indexed for MEDLINE] Related Articles, Links Kyobu Geka. 2004 Feb;57(2):100-6. [Palliative right ventricle outflow reconstruction in tetralogy of Fallot with pulmonary atresia and hypoplastic pulmonary artery] [Article in Japanese] Suzuki Y, Ikeda Y, Hisagi M, Nakayama S. Department of Cardiovascular Surgery, Tokyo Metropolitan Hachioji Children's Hospital, Tokyo, Japan. The management of small pulmonary artery in tetralogy of Fallot (TOF) with pulmonary atresia (PA) is complicated. Our strategy is palliative right ventricle outflow tract reconstruction (RVOTR). The aim of this study is to determine whether palliative RVOTR is useful as the first stage operation. Since 1994, 7 patients with TOF, pulmonary atresia and hypoplastic pulmonary arteries underwent palliative RVOTR. All patients survived operation without complication except for 1. He had pseudoaneurysm at right ventricular outflow patch requiring aneurysmectomy and re-RVOTR 1.5 months after the initial RVOTR. Nine months after RVOTR in the average, pulmonary artery index (PAI) increased from 139 +/- 87 to 306 +/- 156 (p < 0.05). No patient had pulmonary high flow or pulmonary hypertension. Two of them had pulmonary coractation due to ductal tissue in the pulmonary artery, necessitating additional modified Blalock-Taussig shunts at 8 and 10 months old. One patient with TOF, PA, major aorto-pulmonary collateral artery (MAPCA) had corrective surgery after 2 additional operations and interventional catheterization. Five patients of this series already have undergone corrective surgery, whereas 1 is waiting for it. RVOTR is a useful method for pulmonary artery growth in patients with TOF, PA and diminutive pulmonary artery. PMID: 14978901 [PubMed - indexed for MEDLINE] Related Articles, Links Ann Thorac Surg. 2003 Dec;76(6):1901-5. Risk factors for sudden death after repair of tetralogy of Fallot. Nollert GD, Dabritz SH, Schmoeckel M, Vicol C, Reichart B. Clinic of Cardiac Surgery, Klinikum Grosshadern, University of Munich, Munich, Germany. gnollert@hch.uni-muenche.de BACKGROUND: Sudden cardiac death remains the most common cause of death after repair of tetralogy of Fallot. It has been suggested that sudden cardiac death is related to right ventricular hypertrophy or dilation. However, it is uncertain whether the preoperative patient status or operative techniques predispose for sudden cardiac death. METHODS: From 1958 to 1977, 658 patients underwent repair of tetralogy of Fallot at our institution at a median age of 12.2 +/- 8.6 years. One third had at least one previous palliative operation 4.6 +/- 2.5 years earlier. A total of 490 patients survived the first postoperative year and were analyzed for sudden cardiac death. During a follow-up period of 25.3 +/- 5.8 years (range, 1.0 to 35.5 years), 42 patients died, and 15 (36%) of those deaths were as a result of sudden cardiac death. RESULTS: Actuarial 10-year, 20-year, and 30-year survival rates were 97%, 94%, and 89%. Freedom from sudden cardiac death was 99%, 98%, and 95% after 10, 20, and 30 years. The risk of sudden cardiac death increased after 10 years from 0.06%/y to 0.20%/y. Univariate predictors (p < 0.1) of sudden cardiac death were use of an outflow tract patch (p = 0.068), male sex (p = 0.048), no previous palliation (p = 0.013), and higher preoperative New York Heart Association status (p = 0.014). Multivariate analysis confirmed these risk factors except use of an outflow tract patch. CONCLUSIONS: The most important risk factors for sudden cardiac death were higher preoperative New York Heart Association class and no previous palliation. Thus, early surgical intervention is recommended. The risk of sudden cardiac death increases with time, suggesting that long-term follow-up by specialized cardiologists or pediatricians should be intensified. However, all patients who died suddenly had at least two risk factors at the time of surgery. PMID: 14667608 [PubMed - indexed for MEDLINE Related Articles, Links Masui. 2003 Nov;52(11):1227-32. [Postoperative pulmonary bleeding and reperfusion pulmonary edema in an adult patient after repair of tetralogy of Fallot with pulmonary atresia--percutaneous cardiopulmonary support (PCPS) and lung protective ventilatory strategy] [Article in Japanese] Takauchi Y, Imanaka H, Takeuchi M, Tachibana K. Department of Surgical Intensive Care Unit, National Cardiovascular Center, Suita 565-8565. After total correction of tetralogy of Fallot, pulmonary atresia and major aorto-pulmonary collateral arteries, a 31-year-old man developed life-threatening pulmonary hypertension and reperfusion pulmonary edema, leading to pulmonary hemorrhage, right heart failure and hypoxia. Because of difficulty in weaning from cardiopulmonary bypass and in controlling hemorrhage from pulmonary arteries, we applied percutaneous cardiopulmonary support (PCPS) for 281 hours with strategy of delayed sternal closure (17 days) and a large quantity of transfusion. We also applied lung protective ventilatory strategy for reperfusion pulmonary edema (high PEEP, limited peak inspiratory pressure and recruitment maneuver). As the result, he was discharged alive without any major complications. We report our postoperative managements, in terms of 1) lung protective ventilatory strategy, 2) surgical control of pulmonary blood flow, and 3) evaluation of the cardiopulmonary function during PCPS for early weaning from PCPS. Publication Types: , Case Reports PMID: 14661574 [PubMed - indexed for MEDLINE] Related Articles, Links Ann Thorac Surg. 2003 Aug;76(2):555-61; discussion 561. Does right ventricular outflow tract damage play a role in the genesis of late right ventricular dilatation after tetralogy of Fallot repair? d'Udekem d'Acoz Y, Pasquet A, Lebreux L, Ovaert C, Mascart F, Robert A, Rubay JE. Department of Cardiovascular and Thoracic Surgery, Cliniques Universitaires Saint-Luc, and the Public Health School, Universite Catholique de Louvain, Brussels, Belgium. dudekem@chir.ucl.ac.be BACKGROUND: The aim of this study was to determine the relative role of pulmonary insufficiency and right ventricular outflow tract damage in the genesis of late symptoms related to right ventricular dilatation. METHODS: In a retrospective study we compared the late outcomes of patients who had undergone operations known to generate pulmonary insufficiency, namely, transventricular repair of tetralogy of Fallot and pulmonary commissurotomy for isolated pulmonary stenosis. RESULTS: In our institution, between 1964 and 1984, a total of 44 patients were found to have had an isolated pulmonary commissurotomy and 189 survived a transventricular repair of tetralogy of Fallot. Of these patients, 134 had patching of the right ventricle and 55 direct closure of a right ventriculotomy. Follow-up was 94% complete after a mean of 22 +/- 7 years. On echocardiography, patients with isolated commissurotomy had similar degrees of moderate and severe pulmonary insufficiency as tetralogy of Fallot patients who had a right ventricular patch (p > 0.2). However, freedom from adverse events related to right ventricular dilatation was far better (log rank p < 0.001) in patients with isolated commisurotomy. CONCLUSIONS: Pulmonary insufficiency is not the only determinant of late symptomatic right ventricular dilatation after repair of tetralogy of Fallot. Pulmonary insufficiency seems much more deleterious in patients who have had right ventricular outflow tract patching. Long-term pulmonary insufficiency alone is responsible for a slight degree of right ventricular dilatation, but symptoms may develop much later if the contractility of the pulmonary infundibulum is preserved. The pulmonary infundibulum may be essential for right ventricular ejection, and for maintaining pulmonary valve competence. PMID: 12902102 [PubMed - indexed for MEDLINE] Related Articles, Links Croat Med J. 2003 Apr;44(2):234-8. Echocardiographic features of adult tetralogy of Fallot with natural palliative correction by patent ductus arteriosus. Sokol I, Vincelj J, Kirin M. Institute of Cardiovascular Diseases, Dubrava University Hospital, Avenija Gojka Suska 6, 10000 Zagreb, Croatia. isokol@kbd.hr A thirty-year-old man with the diagnosis of the tetralogy of Fallot and patent ductus arteriosus was admitted to our hospital because of a syncope. He reported no previous symptoms. We diagnosed adult tetralogy of Fallot, which included all four characteristic anomalies: ventricular septal defect, overriding aorta, pulmonary artery stenosis, and right ventricular hypertrophy. The associated persistent ductus arteriosus and the presence of compensatory arteriovenous communications produced a continuous flow load on the left ventricle, which resulted in moderate left ventricular hypertrophy, but without symptoms of pulmonary congestion or cardiac decompensation. Anatomic diagnosis and hemodynamic assessment were established by transthoracic and transesophageal echocardiography, with incidental finding of a quadricuspid aortic valve. To the best of our knowledge, our case of the adult form of Fallot's tetralogy associated with both patent ductus arteriosus and quadricuspid aortic valve is the first one ever described. It is well known that patients with tetralogy of Fallot who do not undergo operation in childhood have short survival, which depends predominantly on the degree of pulmonary artery stenosis and early development of collateral circulation to the lungs. Long-term persistence of natural aortopulmonary anastomosis with systemic collateral circulation to the lungs and remodeling of the heart, with better hemodynamic balance as well as the presence of mild pulmonary artery stenosis probably enhanced the survival of our patient. Publication Types: , Case Reports PMID: 12698517 [PubMed - indexed for MEDLINE] Arq Bras Cardiol. 2003 Feb;80(2):198-201, 194-7. Epub 2003 Feb Related Articles, Links 25. Right ventricular diastolic dysfunction in the postoperative period of tetralogy of Fallot. Cardoso SM, Miyague NI. Hospital Infantil Pequeno Principe e Hospital de Clinica da Universidade Federal do Parana. Brasil. OBJECTIVE: To assess right ventricular diastolic function in the intermediate postoperative period of repair of tetralogy of Fallot. METHODS: We carried out a case-control study with 60 patients divided into 2 groups as follows: 1) group I - 30 patients who had undergone repair of tetralogy of Fallot and 2) group II - 30 healthy children. The 2 groups were paired for age, sex, and body surface. The flows in the pulmonary and tricuspid valves were analyzed with Doppler echocardiography. The presence of anterograde flow at the end of diastole in the pulmonary artery defined restrictive right ventricular physiology. Surgical, radiological, electrocardiographic, and echocardiographic variables were analized in the group I. RESULTS: The velocity of the A wave and the E/A ratio for the tricuspid valve showed significant differences between the groups. Cases with E/A < 1.30 predominated in inspiration (group I - 19/30, and group II - 5/30). The duration of the QRS complex on the electrocardiogram was significantly increased in patients with E/A <1.30. Nineteen (63.3%) patients had restrictive right ventricular physiology, which had a longer postoperative period, longer duration of the QRS complex, and a lower E/A ratio in inspiration. The surgical and radiological variables showed no statistical difference. CONCLUSION: Restrictive right ventricular physiology was detected on the intermediate follow-up of most patients undergoing repair of tetralogy of Fallot. The postoperative period and QRS duration were increased in patients with impairment in diastolic function. PMID: 12640513 [PubMed - indexed for MEDLINE] Related Articles, Links Ann Thorac Surg. 2003 Feb;75(2):412-21. Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors. Al-Hay AA, MacNeill SJ, Yacoub M, Shore DF, Shinebourne EA. Department of Paediatric Cardiology, Royal Brompton and Harefield NHS Trust, NHLI, Imperial College, London, United Kingdom. amira_alhay@hotmail.com BACKGROUND: This study was conducted to evaluate surgical outcome and to identify risk factors for hospital mortality and reoperation after repair of complete atrioventricular septal defect. METHODS: A total of 147 consecutive children underwent repair between January 1986 and December 1998. Of those, 106 had Down syndrome, 37% had normal chromosomes, and 4 had other syndromes; 108 underwent primary repair, 19 had prior pulmonary artery banding, and 20 had additional tetralogy of Fallot. The median weight at primary repair was 4.5 kg. A two-patch technique was used in 88%. RESULTS: The 30-day mortality was 15% (70% confidence interval [CI] 12% to 19%). A double orifice atrioventricular valve was found to be a significant risk factor (p = 0.002), with 6 of 11 patients dying. If double orifice atrioventricular valve patients are excluded, the mortality rate falls to 12% (70% CI 9% to 15%). No difference in mortality was found between Down syndrome and chromosomally normal children but the latter more commonly required reoperation. Chromosomally normal children frequently have a dysplastic common atrioventricular valve (24% versus 3% in Down children, p < 0.001). In a multivariate Cox model including both variables, the presence of a dysplastic atrioventricular valve was a significant risk factor for reoperation. After controlling for the presence of a dysplastic atrioventricular valve, Down syndrome retained a significant protective effect but the upper limit of the confidence interval was close to 1. CONCLUSIONS: The presence of a double orifice atrioventricular valve emerged as an unforeseen risk factor for death. PMID: 12607648 [PubMed - indexed for MEDLINE] Related Articles, Links J Am Coll Cardiol. 2002 Dec 4;40(11):2044-52. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction. Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JC, Smith GC, Tat T, Pennell DJ, Gatzoulis MA. Department of Cardiology, Royal Brompton Hospital, London, United Kingdom. OBJECTIVES: We examined the relationship among biventricular hemodynamics, pulmonary regurgitant fraction (PRF), right ventricular outflow tract (RVOT) aneurysm or akinesia, and baseline and surgical characteristics in adults with repaired tetralogy of Fallot (rTOF). BACKGROUND: The precise relationship of pulmonary regurgitation with biventricular hemodynamics has been hampered by limitations of right ventricular (RV) imaging. METHODS: We assessed 85 consecutive adults with rTOF and 26 matched healthy controls using cardiovascular magnetic resonance imaging. RESULTS: Patients had higher right ventricular end-diastolic volume index (RVEDVi) (p < 0.001), right ventricular end-systolic volume index (RVESVi) (p < 0.001), right ventricular mass index (RVMi) (p < 0.001), and lower right ventricular ejection fraction (RVEF) (p < 0.001) and left ventricular ejection fraction (LVEF) (p = 0.002) compared to controls. The PRF (range 0% to 55%) independently predicted RVEDVi (p < 0.01) and the latter predicted RVESVi (p < 0.01) and RVMi (p < 0.01). The RVOT aneurysm/akinesia was present in 48/85 (56.9%) of patients and predicted RV volumes (RVEDVi, p = 0.01, and RVESVi, p = 0.03). There was a negative effect of RVOT aneurysm/akinesia and RVMi on RVEF (p < 0.01 and p = 0.02, respectively). There was only a tendency among patients with transannular or RVOT patching toward RVOT aneurysm/akinesia (p = 0.09). The LVEF correlated with RVEF (r = 0.67, p < 0.001). CONCLUSIONS: Pulmonary regurgitation and RVOT aneurysm/akinesia were independently associated with RV dilation and the latter with RV hypertrophy late after rTOF. The RVOT aneurysm/akinesia was common but related only in part to RVOT or transannular patching. Both RV hypertrophy and RVOT aneurysm/akinesia were associated with lower RVEF. Left ventricular systolic dysfunction correlated with RV dysfunction, suggesting an unfavorable ventricular-ventricular interaction. Measures to maintain or restore pulmonary valve function and avoid RVOT aneurysm/akinesia are mandatory for preserving biventricular function late after rTOF. PMID: 12475468 [PubMed - indexed for MEDLINE] Related Articles, Links Paediatr Anaesth. 2002 Oct;12(8):705-11. Perioperative management of tetralogy of Fallot with absent pulmonary valve. Stayer SA, Shetty S, Andropoulos DB. Division of Pediatric Cardiovascular Anesthesiology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX 77030, USA. sstayer@bcm.tmc.edu BACKGROUND: Tetralogy of Fallot with absent pulmonary valve is a rare form of congenital heart disease. The records of patients with this lesion were reviewed over a 6(1/2) year period, and the perioperative management of 13 patients is described. METHODS: We found that our patients could be divided into two groups by age. The younger group failed medical management and was referred for surgery at less than 1 year of age. This group of patients had considerable respiratory disease at the time of surgery, greater ventilatory problems during surgery and prolonged recovery. Most patients in this group have residual respiratory disease. RESULTS: There were significant differences in postoperative outcome between younger versus older patients: days of ventilation 37 +/- 41 versus 1 +/- 0.6 days, length of ICU stay 28 +/- 31 versus 3 +/- 1 days and length of hospital stay 64 +/- 48 versus 9 +/- 5 days. PMID: 12472708 [PubMed - indexed for MEDLINE] Related Articles, Links Zhonghua Wai Ke Za Zhi. 2002 Sep;40(9):685-8. [Lung protection by perfusion with hypothermic protective solution to pulmonary artery during total correction of tetralogy of Fallot] [Article in Chinese] Wei B, Liu Y, Wang Q, Chang Y, Li C. Department of Congenital Heart Defect, Fuwai Heart Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China. OBJECTIVE: To study lung protection by perfusion with hypothermic protective solution to the pulmonary artery during total correction of tetralogy of Fallot (TOF). METHODS: Sixty-four consecutive children with TOF were randomly divided into control group (n = 30) and lung protective group (n = 34). The way of lung protection in the lung protective group was to perfuse with hypothermic protective solution to the pulmonary artery. Patients in the control group were subjected to routine approach. Patients' hemodynamics and lung functions were monitored. Plasma malondialdehyde (MDA), TNF-alpha and IL-6, IL-8 in tracheal suction were measured. Lung biopsy specimens were obtained after operations to study histological changes. RESULTS: The oxygen index was higher in the lung protect group than in the control group at 6 h, 12 h and 24 h after operation (t = 2.400, P < 0.05; t = 3.898, P < 0.01; t = 3.339, P < 0.01, respectively). The time for ICU and mechanical ventilation was significantly less in the lung protective group than in the control group (t = -2.652, P < 0.05; t = -2.081, P < 0.05). The level of MDA was lower in the lung protective group than in the control group at 0 h and 6 h after operations (t = -4.255, P < 0.01; t = -2.372, P < 0.05 respectively). The level of TNF-alpha was lower in the lung protective group than in the control group at 0 h, 6 h and 24 h after operation (t = 3.112, P < 0.01; t = 3.072, P < 0.01; t = 2.306, P < 0.05, respectively). The levels of IL-6, IL-8 in tracheal suction were lower in the lung protective group (t = -2.419, P < 0.05; t = -2.613, P < 0.01). Tissue examination showed intraalveolar edema, capillary hyperemia, leukocytes accumulated, and mitochondria swelling in the control group, whereas no change in the lung protective group. CONCLUSION: Perfusion with hypothermic protective solution to the pulmonary artery in CPB could reduce lung injury during the total correction of TOF. Publication Types: , Clinical Trial , Randomized Controlled Trial PMID: 12411141 [PubMed - indexed for MEDLINE]