Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestati...Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestations of cyanosis, right-sided heart failure and arrhythmias. The leaflet tethering and dysplasia, together with dilatation of the tricuspid valve ring, constitute the anatomic cause of tricuspid regurgitation observed in this condition. Case Reports: The spectrum of leaflet tethering from mild to extreme with varying degrees of tricuspid regurgitation were documented by echocardiography in a 16-year-old cyanotic male with Ebstein’s anomaly associated with an atrial septal defect and mild low tricuspid regurgitation (TR), 22-year-old acyanotic male with right-sided heart failure due to severe tricuspid regurgitation and an intact atrial septum, 55-year-old asymptomatic female with moderate high tricuspid regurgitation. The extreme form was described as an “atretic” mem-brane in a 28-year-old cyanotic male and as a “blanket” of leaflet tissue in a 30-year-old cyanotic male. Conclusion: Ebstein’s anomaly has to be sus-pected clinically in presence of cyanosis with a WPW (Wolf-Parkinson-White) or atrioventricular (AV) block pattern of ECG and its management is complex and must be individualized. RV (right ventricular) exclusion procedures are preferred in advanced cases.展开更多
BACKGROUND Shone’s complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients.AIM To use echocardiography in the diagnosis of Shone’s complex and analyze the causes ...BACKGROUND Shone’s complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients.AIM To use echocardiography in the diagnosis of Shone’s complex and analyze the causes of missed diagnosis and misdiagnosis.METHODS This was a retrospective study of patients who underwent echocardiography and repair surgery from February 14,2008,to November 22,2019.The patients were followed once a year at the outpatient clinic after surgery.RESULTS Sixty-six patients were included.The patients were 2.7(0.8-5.6)years of age,and 54.5%were male.Ten(15.2%)had a history of heart surgery.The most common heart defect was the Annulo-Leaflet mitral ring(ALMR)(50/66,75.8%),followed by coarctation of the aorta(CoA)(43/66,65.2%).The patients had a variety of combinations of defects.Only two(3.0%)patients had all four defects.None of the patients had a family history of congenital heart disease.The preoperative echocardiographic findings were examined against the intraoperative findings.Echocardiography missed an ALMR in 31 patients(47.0%),a parachute mitral valve(PMV)in one patient(1.5%),subaortic stenosis in one patient(1.5%),and CoA in two patients(3.0%).CONCLUSION Echocardiography is an effective method to diagnose the Shone’s complex.Due to this disease’s complexity and interindividual variability, Improving the understanding of thedisease can reduce misdiagnosis and missed diagnosis.展开更多
文摘Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestations of cyanosis, right-sided heart failure and arrhythmias. The leaflet tethering and dysplasia, together with dilatation of the tricuspid valve ring, constitute the anatomic cause of tricuspid regurgitation observed in this condition. Case Reports: The spectrum of leaflet tethering from mild to extreme with varying degrees of tricuspid regurgitation were documented by echocardiography in a 16-year-old cyanotic male with Ebstein’s anomaly associated with an atrial septal defect and mild low tricuspid regurgitation (TR), 22-year-old acyanotic male with right-sided heart failure due to severe tricuspid regurgitation and an intact atrial septum, 55-year-old asymptomatic female with moderate high tricuspid regurgitation. The extreme form was described as an “atretic” mem-brane in a 28-year-old cyanotic male and as a “blanket” of leaflet tissue in a 30-year-old cyanotic male. Conclusion: Ebstein’s anomaly has to be sus-pected clinically in presence of cyanosis with a WPW (Wolf-Parkinson-White) or atrioventricular (AV) block pattern of ECG and its management is complex and must be individualized. RV (right ventricular) exclusion procedures are preferred in advanced cases.
文摘BACKGROUND Shone’s complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients.AIM To use echocardiography in the diagnosis of Shone’s complex and analyze the causes of missed diagnosis and misdiagnosis.METHODS This was a retrospective study of patients who underwent echocardiography and repair surgery from February 14,2008,to November 22,2019.The patients were followed once a year at the outpatient clinic after surgery.RESULTS Sixty-six patients were included.The patients were 2.7(0.8-5.6)years of age,and 54.5%were male.Ten(15.2%)had a history of heart surgery.The most common heart defect was the Annulo-Leaflet mitral ring(ALMR)(50/66,75.8%),followed by coarctation of the aorta(CoA)(43/66,65.2%).The patients had a variety of combinations of defects.Only two(3.0%)patients had all four defects.None of the patients had a family history of congenital heart disease.The preoperative echocardiographic findings were examined against the intraoperative findings.Echocardiography missed an ALMR in 31 patients(47.0%),a parachute mitral valve(PMV)in one patient(1.5%),subaortic stenosis in one patient(1.5%),and CoA in two patients(3.0%).CONCLUSION Echocardiography is an effective method to diagnose the Shone’s complex.Due to this disease’s complexity and interindividual variability, Improving the understanding of thedisease can reduce misdiagnosis and missed diagnosis.
