Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult population, especially if it is associated with other abnormalities such as ...Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult population, especially if it is associated with other abnormalities such as Ebstein’s anomaly and abnormal origin of coronary arteries. Case presentation: Twenty-two-year-old woman admitted to intensive care unit with acute decompensated heart failure. The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein’s anomaly. The computed tomography corroborated these findings and additionally showed a left superior vena cava and an abnormal origin of the coronary arteries. The patient is in NYHA functional class II, receiving treatment with diuretic, digitalis, beta-blockers and angiotensin-converting enzyme (ACE) inhibitor. Conclusion: This is a very interesting case of an adult in the third decade of life with cc-TGA associated with abnormalities not described in the literature that had reached adulthood. Noninvasive images play an important role in its diagnosis.展开更多
Background:Ebstein’s anomaly(EA)is a rare and complex congenital heart anomaly,and the effect of surgical treatment is not ideal.This study aims to introduce our experience in management strategies,surgical technique...Background:Ebstein’s anomaly(EA)is a rare and complex congenital heart anomaly,and the effect of surgical treatment is not ideal.This study aims to introduce our experience in management strategies,surgical techniques,and operative indications for patients with Ebstein’s anomaly.Methods:A retrospective study of 258 operations was performed in 253 patients by the same cardiac surgeon in The First Hospital of Tsinghua University between March 2004 and January 2020.32 patients had previously received cardiac surgery in other hospitals.The clinical data including diagnosis,operative indications,techniques,pathological changes,and survival rates were collected and analyzed.Results:Anatomical correction was performed in 203(78.7%)operations,1½ventricle repair in 38(14.7%)operations,tricuspid valve repair only in four operations(1.6%),tricuspid valve replacement in ten(3.9%),total cavopulmonary connection(TCPC)in two(0.8%),and Glenn operation in one operation(0.4%).Reoperation was performed in five patients(2.0%)during hospitalization.Among them,tricuspid valve replacement was performed in one patient,1½ventricle repair in two patients,and tricuspid valve annulus reinforcement in two patients.Five patients died with an early mortality rate of 2.0%.Complete atrioventricular conduction block was complicated in one patient(0.4%).A total of 244 patients was followed up(four in the 253 patients lost)with a duration of 3.0-168.0(87.6±38.4)months.Cardiac function of 244 patients improved significantly with mean New York Heart Association(NYHA)functional class recovery from 3.5 to 1.1.The mean grade of tricuspid valve regurgitation improved from 3.6 to 1.5.Three late deaths(1.2%)occurred.The survival rates at five and ten years after surgery were 98.6%and 98.2%,respectively.Reoperation was performed in five patients(2.0%)during the follow-up period.Conclusion:Based on our management strategies and operative principles and techniques,anatomical correction of EA is capable of achieving excellent long-term results,and low rates of TCPC,1½ventricle repair and valvular replacement.展开更多
Ebstein's anomaly(EA) is a rare cardiac congenital malformation with displacement of septal and posterior tricuspid leaflets,resulting in atrialization of the right ventricle.We report a case of EA in which the et...Ebstein's anomaly(EA) is a rare cardiac congenital malformation with displacement of septal and posterior tricuspid leaflets,resulting in atrialization of the right ventricle.We report a case of EA in which the etiology of a malfunctioning prosthetic tricuspid valve is depicted on cardiac computed tomography to be as a result of thrombus lodged in the valve.展开更多
Rationale: Pompholyx refers to pruritic vesicles or bullous rash that mainly distribute on the palms and lateral surfaces of the fingers. It is less common among Asians, and in a severe condition, secondary bacterial ...Rationale: Pompholyx refers to pruritic vesicles or bullous rash that mainly distribute on the palms and lateral surfaces of the fingers. It is less common among Asians, and in a severe condition, secondary bacterial infection of pompholyx can happen and result in pain, swelling and pustules.Patient concerns: A 15-year-old girl complained of progressive wound and small bumps containing yellowish pus and crusts on her hands and feet for over 6 months and worsened in the last month before admission. She also had Ebstein anomaly.Diagnosis: Atypical pompholyx with secondary Staphylococcus and Klebsiella infections. Interventions: Wound care with wet dressing and applying moisturizer on crusts, application of antibiotics for Gram positive and negative bacteria and giving nutritional support with reckoning of proper calories. Outcomes: Skin lesions were completely healed and the patient was discharged after 10 days of hospitalization.Lessons: Atypical manifestation of pompholyx makes it hard to diagnose. The diagnosis can be confirmed with meticulous historytaking and physical examination. Wound caring and controlling of the infection should be done to earn an optimal outcome.展开更多
Among all congenital heart disease,Ebstein anomaly is the lesion with the widest spectrum of presentation.It can present as early as the neonatal period where immediate intervention is often needed to the adult with n...Among all congenital heart disease,Ebstein anomaly is the lesion with the widest spectrum of presentation.It can present as early as the neonatal period where immediate intervention is often needed to the adult with no discernable symptoms.It is also one where overall outcome greatly relates to age at presentation.Generally,presentation in the neonatal period is associated with poor outcomes while presentation beyond the neonatal period has excellent outcomes with low operative mortality.It is thus apparent that understanding every aspect of the anatomy and physiology of Ebstein anomaly and how it manifests clinically is paramount to successful treatment.The purpose of this review is to outline the current concepts and strategies in the surgical management of Ebstein anomaly,and to discuss the current expert consensus on how best to tackle this congenital heart disease at different ages of diagnosis.展开更多
Ebstein’s anomaly is a rare congenital heart disease. We report a series of 4 cases of Ebstein’s anomaly discovered in adolescents. The mean age was 13.75 ± 0.96, with 3 boys and one girl. Three ...Ebstein’s anomaly is a rare congenital heart disease. We report a series of 4 cases of Ebstein’s anomaly discovered in adolescents. The mean age was 13.75 ± 0.96, with 3 boys and one girl. Three were in heart failure. The EKG showed atrial hypertrophy (1 case), right bundle branch block (1 case), Wolff-Parkinson-White (1 case), and atrial fibrillation (1 case). Cardiomegaly was present in all patients with a mean cardiothoracic index of 0.77 ± 0.03. On echocardiography, the mean apical displacement of the tricuspid septal leaflet was 22.5 ± 3.30 mm/m<sup>2</sup>, the Celermajer index was Grade 2 in 2 patients and Grade 4 in 2 patients. Depletive medical treatment was prescribed to 3 patients. Anticoagulant therapy was initiated in one patient. Two are awaiting surgery. One death was recorded, it was at Grade 4 of the Celermajer index.展开更多
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
Main pulmonary artery aneurysms are rare,mostly asymptomatic and discovered accidentally.The main pulmonary artery aneurysms may be idiopathic or secondary to underlying diseases such as pulmonary hypertension,Behcet...Main pulmonary artery aneurysms are rare,mostly asymptomatic and discovered accidentally.The main pulmonary artery aneurysms may be idiopathic or secondary to underlying diseases such as pulmonary hypertension,Behcet’s disease,connective tissue disorders,congenital heart disease,vasculitis,syphilis,tuberculosis and endocarditis.There are some indices that dextrocardia is associated with anomalies of the pulmonary arteries and pulmonary valve.A rare occurrence of main pulmonary artery aneurysms results in a lack of recommendations,so the remaining challenges are whether main pulmonary artery aneurysms should be treated,how,and when.The decision on surgical intervention or conservative treatment of the main pulmonary artery aneurysms depends on its size,etiology and accompanying diseases and includes a multidisciplinary heart team.Our case of the main pulmonary artery aneurysm and pulmonary valve abnormality associated with Ebstein anomaly and dextrocardia in a 67-year-old male patient brings causality considerations and treatment options in such a unique condition.展开更多
文摘Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult population, especially if it is associated with other abnormalities such as Ebstein’s anomaly and abnormal origin of coronary arteries. Case presentation: Twenty-two-year-old woman admitted to intensive care unit with acute decompensated heart failure. The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein’s anomaly. The computed tomography corroborated these findings and additionally showed a left superior vena cava and an abnormal origin of the coronary arteries. The patient is in NYHA functional class II, receiving treatment with diuretic, digitalis, beta-blockers and angiotensin-converting enzyme (ACE) inhibitor. Conclusion: This is a very interesting case of an adult in the third decade of life with cc-TGA associated with abnormalities not described in the literature that had reached adulthood. Noninvasive images play an important role in its diagnosis.
文摘Background:Ebstein’s anomaly(EA)is a rare and complex congenital heart anomaly,and the effect of surgical treatment is not ideal.This study aims to introduce our experience in management strategies,surgical techniques,and operative indications for patients with Ebstein’s anomaly.Methods:A retrospective study of 258 operations was performed in 253 patients by the same cardiac surgeon in The First Hospital of Tsinghua University between March 2004 and January 2020.32 patients had previously received cardiac surgery in other hospitals.The clinical data including diagnosis,operative indications,techniques,pathological changes,and survival rates were collected and analyzed.Results:Anatomical correction was performed in 203(78.7%)operations,1½ventricle repair in 38(14.7%)operations,tricuspid valve repair only in four operations(1.6%),tricuspid valve replacement in ten(3.9%),total cavopulmonary connection(TCPC)in two(0.8%),and Glenn operation in one operation(0.4%).Reoperation was performed in five patients(2.0%)during hospitalization.Among them,tricuspid valve replacement was performed in one patient,1½ventricle repair in two patients,and tricuspid valve annulus reinforcement in two patients.Five patients died with an early mortality rate of 2.0%.Complete atrioventricular conduction block was complicated in one patient(0.4%).A total of 244 patients was followed up(four in the 253 patients lost)with a duration of 3.0-168.0(87.6±38.4)months.Cardiac function of 244 patients improved significantly with mean New York Heart Association(NYHA)functional class recovery from 3.5 to 1.1.The mean grade of tricuspid valve regurgitation improved from 3.6 to 1.5.Three late deaths(1.2%)occurred.The survival rates at five and ten years after surgery were 98.6%and 98.2%,respectively.Reoperation was performed in five patients(2.0%)during the follow-up period.Conclusion:Based on our management strategies and operative principles and techniques,anatomical correction of EA is capable of achieving excellent long-term results,and low rates of TCPC,1½ventricle repair and valvular replacement.
文摘Ebstein's anomaly(EA) is a rare cardiac congenital malformation with displacement of septal and posterior tricuspid leaflets,resulting in atrialization of the right ventricle.We report a case of EA in which the etiology of a malfunctioning prosthetic tricuspid valve is depicted on cardiac computed tomography to be as a result of thrombus lodged in the valve.
文摘Rationale: Pompholyx refers to pruritic vesicles or bullous rash that mainly distribute on the palms and lateral surfaces of the fingers. It is less common among Asians, and in a severe condition, secondary bacterial infection of pompholyx can happen and result in pain, swelling and pustules.Patient concerns: A 15-year-old girl complained of progressive wound and small bumps containing yellowish pus and crusts on her hands and feet for over 6 months and worsened in the last month before admission. She also had Ebstein anomaly.Diagnosis: Atypical pompholyx with secondary Staphylococcus and Klebsiella infections. Interventions: Wound care with wet dressing and applying moisturizer on crusts, application of antibiotics for Gram positive and negative bacteria and giving nutritional support with reckoning of proper calories. Outcomes: Skin lesions were completely healed and the patient was discharged after 10 days of hospitalization.Lessons: Atypical manifestation of pompholyx makes it hard to diagnose. The diagnosis can be confirmed with meticulous historytaking and physical examination. Wound caring and controlling of the infection should be done to earn an optimal outcome.
文摘Among all congenital heart disease,Ebstein anomaly is the lesion with the widest spectrum of presentation.It can present as early as the neonatal period where immediate intervention is often needed to the adult with no discernable symptoms.It is also one where overall outcome greatly relates to age at presentation.Generally,presentation in the neonatal period is associated with poor outcomes while presentation beyond the neonatal period has excellent outcomes with low operative mortality.It is thus apparent that understanding every aspect of the anatomy and physiology of Ebstein anomaly and how it manifests clinically is paramount to successful treatment.The purpose of this review is to outline the current concepts and strategies in the surgical management of Ebstein anomaly,and to discuss the current expert consensus on how best to tackle this congenital heart disease at different ages of diagnosis.
文摘Ebstein’s anomaly is a rare congenital heart disease. We report a series of 4 cases of Ebstein’s anomaly discovered in adolescents. The mean age was 13.75 ± 0.96, with 3 boys and one girl. Three were in heart failure. The EKG showed atrial hypertrophy (1 case), right bundle branch block (1 case), Wolff-Parkinson-White (1 case), and atrial fibrillation (1 case). Cardiomegaly was present in all patients with a mean cardiothoracic index of 0.77 ± 0.03. On echocardiography, the mean apical displacement of the tricuspid septal leaflet was 22.5 ± 3.30 mm/m<sup>2</sup>, the Celermajer index was Grade 2 in 2 patients and Grade 4 in 2 patients. Depletive medical treatment was prescribed to 3 patients. Anticoagulant therapy was initiated in one patient. Two are awaiting surgery. One death was recorded, it was at Grade 4 of the Celermajer index.
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Main pulmonary artery aneurysms are rare,mostly asymptomatic and discovered accidentally.The main pulmonary artery aneurysms may be idiopathic or secondary to underlying diseases such as pulmonary hypertension,Behcet’s disease,connective tissue disorders,congenital heart disease,vasculitis,syphilis,tuberculosis and endocarditis.There are some indices that dextrocardia is associated with anomalies of the pulmonary arteries and pulmonary valve.A rare occurrence of main pulmonary artery aneurysms results in a lack of recommendations,so the remaining challenges are whether main pulmonary artery aneurysms should be treated,how,and when.The decision on surgical intervention or conservative treatment of the main pulmonary artery aneurysms depends on its size,etiology and accompanying diseases and includes a multidisciplinary heart team.Our case of the main pulmonary artery aneurysm and pulmonary valve abnormality associated with Ebstein anomaly and dextrocardia in a 67-year-old male patient brings causality considerations and treatment options in such a unique condition.
