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      Truncus arteriosus: A major cause of proteinuria in children

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          Abstract

          Background:

          There are many studies about the association of nephropathy with congenital heart diseases (CHD), and the risk factors such as cyanosis and pulmonary hypertension have been evaluated. In our study, we have considered the relation of CHD associated nephropathy with other newer factors and the type of the structural heart defect.

          Materials and Methods:

          A prospective cross sectional study was carried out. 48 children were selected on the basis of specific inclusion criteria, and reviewed over a period of 9 months. Nine different simple and complex structural heart defects were evaluated and compared after obtaining the imaging, blood and urine test results.

          Results:

          Significant proteinuria occurred in 8 patients included in the study. More severe forms of pulmonary hypertension were observed in patients suffering from truncus arteriosus (TA); while the least values were detected in cases of pulmonary stenosis (PS) and tetralogy of fallot (TOF). The highest values of protein excretion were seen in patients of TA; and, the lowest values were observed in patients of PS and aortic stenosis (AS). Renal insufficiency was uncommon in infants and children with CHD.

          Conclusion:

          TA is an important cause of proteinuria among the infants and children suffering from CHD, probably because of the associated severe pulmonary hypertension (PH) and cyanosis. Also, proteinuria occurred at an earlier age in patients of TA as compared to other conditions, and was also found to be more severe if the TA was associated with moderate to severe tricuspid regurgitation.

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          Most cited references11

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          Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart.

          Morbidity and mortality patterns were characterized in adults with the Eisenmenger syndrome when two ventricles with a ventricular septal defect (VSD) joined two great arteries or one great artery, or when one ventricle joined two great arteries. Although afterload in these disorders differs, clinical differences have not been defined. Seventy-seven patients were studied. Group A comprised 47 patients with VSD, aged 23 to 69 years (mean 39.5+/-10.2), follow-up 5 to 18 years (mean 7.2+/-4.9); group B, 14 patients with truncus arteriosus, aged 27 to 50 years (mean 33.7+/-7.3), follow-up 6 to 18 years (mean 7.7+/-5.1), and group C, 16 patients with univentricular heart, aged 18 to 44 years (mean 30.6+/-8.4), follow-up 5 to 15 years (mean 4.4+/-4.2). Echocardiography established the diagnoses and anatomic and hemodynamic features. Data were compiled on tachyarrhythmias, pregnancy, infective endocarditis, noncardiac surgery and the multisystem disorders of cyanotic adults. Thirty-five percent of the patients died. Sixty-three percent of deaths were sudden, and resulted from intrapulmonary hemorrhage, rupture of either the pulmonary trunk, ascending aorta or a bronchial artery, or vasospastic cerebral infarction, or the cause was unestablished. There were no documented tachyarrhythmic sudden deaths. Medical management of coexisting cardiac disease, multisystem systemic disorders, noncardiac surgery and pregnancy has reduced morbidity. Increased longevity exposed patients to proximal pulmonary arterial aneurysms, thromboses and calcification; to truncal valve stenosis and regurgitation; to semilunar and atrioventricular valve regurgitation, and to major risks of nontachyarrhythmic sudden death.
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            Chromosome 22q11 deletion in patients with truncus arteriosus.

            The association between truncus arteriosus and chromosome 22q11 deletion is well recognized, but the frequency of a chromosome 22q11 deletion has not been characterized in a large series of patients with truncus arteriosus, and little is known about cardiovascular morphologic features associated with a chromosome 22q11 deletion in this group of patients. We prospectively enrolled 50 consecutive patients with truncus arteriosus who were admitted to The Children's Hospital of Philadelphia between November 1991 and December 2001. Patients were studied for chromosome 22q11 deletion using fluorescence in situ hybridization. Correlations between anatomic features and chromosome 22q11 deletion were assessed. A chromosome 22q11 deletion was detected in 20 of the 50 patients (40%). Anatomic features that were significantly associated with a chromosome 22q11 deletion included a right-sided aortic arch, an abnormal aortic arch branching pattern, both abnormal sidedness and branching of the aortic arch, and the combined category of either abnormal sidedness or branching of the aortic arch. There was a trend toward the association of discontinuous pulmonary arteries with a chromosome 22q11 deletion. Interruption of the aortic arch and truncal valve morphology and function did not correlate significantly with the presence of a chromosome 22q11 deletion. In conclusion, a chromosome 22q11 deletion is common in patients with truncus arteriosus, and those with abnormal sidedness and/or branching of the aortic arch are significantly more likely to have a deletion. Clinically important anatomic variables, such as abnormalities of the truncal valve and interrupted aortic arch, were not associated with a chromosome 22q11 deletion in this series.
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              Renal involvement in patients with congenital cyanotic heart disease.

              Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3 +/- 1.3 vs 5.6 +/- 1.4 10(12)/l p less than 0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.
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                Author and article information

                Journal
                J Cardiovasc Dis Res
                JCDR
                Journal of Cardiovascular Disease Research
                Medknow Publications Pvt Ltd (India )
                0975-3583
                0976-2833
                Oct-Dec 2011
                : 2
                : 4
                : 237-240
                Affiliations
                [1 ] Pediatric Cardiologist, Department of Pediatric Nephrology, Tabriz Medical University, Tabriz, Iran
                [2]
                Author notes
                Address for correspondence: Dr. Majid Malaki, Department of Pediatric Nephrology, 1-Tabriz Children Hospital, Pediatric Health Research Center, Tabriz - 5136735886, Iran. E-mail: madjidmalaki@ 123456gmail.com
                Article
                JCDR-2-237
                10.4103/0975-3583.89809
                3224445
                22135483
                d7f9b49f-70a5-4dc0-b441-54fe37166849
                Copyright: © Journal of Cardiovascular Disease Research

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Original Article

                Cardiovascular Medicine
                congenital heart disease,proteinuria,truncous arteriosus
                Cardiovascular Medicine
                congenital heart disease, proteinuria, truncous arteriosus

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