Echocardiographic predictors of aortopulmonary collaterals in infants with tetralogy of fallot and pulmonary atresia.
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OBJECTIVES: This study was designed to identify echocardiographic predictors of aortopulmonary collaterals (APCs) in infants with tetralogy of Fallot and pulmonary atresia (TOF/PA) and to prospectively validate these predictors. BACKGROUND: In patients with TOF/PA, routine preoperative diagnostic cardiac catheterization is usually performed to determine the presence and distribution of APCs and the anatomy of the branch pulmonary arteries. METHODS: The clinical and diagnostic imaging data of infants with TOF/PA treated between 1990 and 2000 were reviewed. Patients were classified into two groups based on cineangiographic findings: 1) no APCs (n = 34), and 2) >or=1 APCs (n = 59). Echocardiographic variables were examined for their ability to identify patients having >or=1 APCs. RESULTS: Median branch pulmonary artery diameter Z scores were significantly larger in patients without APCs compared with those having >or=1 APCs: -0.56 versus -3.24 for the left pulmonary artery and -0.76 versus -3.46 for the right pulmonary artery (p < 0.001). The presence of a branch pulmonary artery diameter Z score or=1 APCs. Detection of APCs by color Doppler was 93% sensitive and 91% specific. A combination of branch pulmonary artery diameter Z score or=1 APCs. The diagnostic accuracy of these echocardiographic variables was subsequently validated in a prospective study of 11 infants. CONCLUSIONS: Echocardiography is a sensitive and specific test for the detection of >or=1 APCs in infants with TOF/PA. These data can be used to select patients who can undergo complete repair of TOF/PA without further preoperative diagnostic imaging. (+info)
Right ventricular diastolic dysfunction in the postoperative period of tetralogy of Fallot.
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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. (+info)
Tetralogy of Fallot and hypertrophic cardiomyopathy: a rare association.
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Tetralogy of Fallot is known as the most common cyanotic congenital heart disease and has a prevalence of 10% of all congenital heart diseases. Although many other heart anomalies may coexist, the association of tetralogy of Fallot and hypertrophic cardiomyopathy is extremely rare. We report this association in a 15-month-old female, cyanotic since birth, in her first hospital admission for diagnosis and treatment of recurring cyanotic crises. In addition, a review of the literature and of the problems related to the treatment is provided. (+info)
Echocardiographic features of adult tetralogy of Fallot with natural palliative correction by patent ductus arteriosus.
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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. (+info)
Tetralogy of Fallot in a 2-year-old Holstein heifer.
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A 2-year-old, purebred Holstein heifer with exercise intolerance and cardiovascular compromise was diagnosed at postmortem with tetralogy of Fallot, which typically results in death within a few months of life. Survival past the age of 2 was unexpected. The concurrent endocarditis of the pulmonic valve is discussed. (+info)
Genome-wide array analysis of normal and malformed human hearts.
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BACKGROUND: We present the first genome-wide cDNA array analysis of human congenitally malformed hearts and attempted to partially elucidate these complex phenotypes. Most congenital heart defects, which account for the largest number of birth defects in humans, represent complex genetic disorders. As a consequence of the malformation, abnormal hemodynamic features occur and cause an adaptation process of the heart. METHODS AND RESULTS: The statistical analysis of our data suggests distinct gene expression profiles associated with tetralogy of Fallot, ventricular septal defect, and right ventricular hypertrophy. Applying correspondence analysis, we could associate specific gene functions to specific phenotypes. Furthermore, our study design allows the suggestion that alterations associated with primary genetic abnormalities can be distinguished from those associated with the adaptive response of the heart to the malformation (right ventricular pressure overload hypertrophy). We provide evidence for the molecular transition of the hypertrophic right ventricle to normal left ventricular characteristics. Furthermore, we present data on chamber-specific gene expression. CONCLUSIONS: Our findings propose that array analysis of malformed human hearts opens a new window to understand the complex genetic network of cardiac development and adaptation. For detailed access, see the online-only Data Supplement. (+info)
Differential regurgitation in branch pulmonary arteries after repair of tetralogy of Fallot: a phase-contrast cine magnetic resonance study.
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BACKGROUND: The importance of pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) is increasingly recognized, but little is known regarding its underlying mechanisms. This phase-contrast cine magnetic resonance (PC MR) study was performed to evaluate the relative contribution of each lung to total regurgitant volume. METHODS AND RESULTS: Twenty-two patients with significant PR underwent a PC MR 3 to 16 years after repair of TOF. Regurgitant fraction of the main pulmonary artery was 39+/-10%. Regurgitant fraction of the left pulmonary artery (LPA; 46+/-18%) was greater than that of the right pulmonary artery (34+/-16%; P=0.002). The average contribution of the LPA to the total regurgitant flow volume was 54+/-19%, whereas its average contribution to the total forward flow volume was 44+/-13% (P=0.002). In 4 patients, regurgitant flow in the LPA accounted for 75% to 100% of the total regurgitant flow. There was a linear relationship between regurgitant fraction and fraction of the regurgitant flow duration in the main pulmonary artery (P<0.001) and right pulmonary artery (P=0.001) but not in the LPA (P=0.129). CONCLUSIONS: PR after repair of TOF is commonly associated with differential regurgitation in the branch pulmonary arteries, which is usually greater in the LPA. Although the cause of this disparity requires further investigation, those patients with a significant unilateral contribution to total PR may be amenable to localized techniques to reduce regurgitation. (+info)
Reoperation in a Jehovah's Witness 22 years after aortic allograft reconstruction of the right ventricular outflow tract.
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We report the case of a 29-year-old male Jehovah's Witness who presented with hemolysis and right heart failure as a consequence of extreme calcification of an aortic valve-containing allograft and Dacron prosthesis that had been placed 22 years earlier to repair tetralogy of Fallot. Reoperation had been denied by several other centers, because of the patient's refusal to undergo blood transfusion. For 2 weeks preoperatively, we raised the patient's hemoglobin level by treating him with recombinant erythropoietin, oral iron, and folic acid. At surgery, under normothermic cardiopulmonary bypass, we replaced the aortic allograft in the right ventricular outflow tract with a cryopreserved pulmonary allograft, also containing a valve. The postoperative course was uneventful, and the patient was released from the hospital on the 13th postoperative day in excellent condition. (+info)