Disease relevance of Tricuspid Atresia

• Homozygotes for the Hey2 mutant allele display a spectrum of cardiac malformations including ventricular septal defects, tetralogy of Fallot, and tricuspid atresia, defects that resemble those associated with mutations of human JAG1 [1].
• Pulmonary artery banding was also used in patients with d-transposition of the great vessels with ventricular septal defect, double-outlet right ventricle, univentricular heart, tricuspid atresia, and truncus arteriosus [2].
• The diagnoses were tricuspid atresia in 21, single ventricle with low pulmonary vascular resistance in seven, and one case of dextro-transposition of the great arteries with ventricular septal defect and pulmonary stenosis [3].
• When transposition of the great arteries accompanies tricuspid atresia, narrowing of the ventricular septal defect (VSD) leads to subaortic stenosis [4].
• A 9-month-old boy with left juxtaposition of the atrial appendages, tricuspid atresia, pulmonary atresia, and ventriculoarterial discordance underwent anastomosis between the atrial appendages after failure of balloon/blade atrial septostomy because of restrictive atrial septal defect [5].

High impact information on Tricuspid Atresia

• Our findings indicate the importance of Zfpm2 in the normal looping and septation of the heart and suggest a genetic basis for the syndrome of tricuspid atresia [6].
• Subaortic obstruction caused by either a restrictive bulboventricular foramen in single left ventricle with an outflow chamber or by a restrictive ventricular septal defect in tricuspid atresia with transposition of the great arteries can lead to a hypertrophied, noncompliant ventricle and excessive pulmonary blood flow [7].
• Management of tricuspid atresia with orally administered prostaglandin E2 [8].
• A neonate with VACTERL association including tricuspid atresia was scheduled for thoracoscopic esophageal atresia with tracheoesophageal fistula (EA/TEF) repair and laparoscopic gastrostomy tube placement [9].
• OBJECTIVES: This study was undertaken to assess the pattern of left ventricular hypertrophy, the myocardial capillary network, and the extracellular matrix in hearts with tricuspid atresia [10].

Chemical compound and disease context of Tricuspid Atresia

• Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia [11].

Biological context of Tricuspid Atresia

• However, both invasive and noninvasive laboratory assessments of ventricular function have revealed abnormalities in some subsets of patients after repair of atrial septal defect, ventricular septal defect, aortic or pulmonary stenosis, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia [12].

Anatomical context of 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 [13].
• Bicaval pulmonary connection in tricuspid atresia using an extracardiac tube of autologous pediculated pericardium to bridge inferior vena cava [14].
• The right auricle ir-ANP level was high in patients with heart failure combined with pressure or volume overload of the right atrium, as observed in tricuspid atresia, complete endocardial cushion defect, and total anomalous pulmonary venous drainage [15].

Gene context of Tricuspid Atresia

• ZFPM2/FOG2 and HEY2 genes analysis in nonsyndromic tricuspid atresia [16].
• In two thirds of the cases with classic tricuspid atresia, the size of the orifice of the juxtaposed appendage was larger than the atrial septal defect, and an anastomosis of the appendage with the pulmonary trunk was morphologically feasible [17].
• Diagnoses were 27 cases of transposition of the great arteries (TGA), 1 mitral atresia, 9 tricuspid atresia, 7 critical pulmonary stenosis with hypoplastic right ventricle, 3 pulmonary atresia with intact interventricular septum, and 1 total anomalous pulmonary venous return [18].

Analytical, diagnostic and therapeutic context of Tricuspid Atresia

• We investigated the acute changes in ventricular geometry that occur after the Fontan operation and hemi-Fontan operation for tricuspid atresia, and compared them with closure of a ventricular septal defect in a two-ventricle heart [19].
• We reviewed the results of echocardiography performed before and 8 +/- 7 days after (1) Fontan operation for tricuspid atresia (n = 9), (2) hemi-Fontan operation for tricuspid atresia (n = 10), and (3) closure of a ventricular septal defect (n = 13) [19].
• First pass radionuclide angiography was used to demonstrate a leaking Glenn anastomosis at the superior vena cava--right atrial junction as well as tricuspid atresia and a ligated persistent left superior vena cava [20].

References