Atypical pompholyx presentation of secondary Staphyloccoccus and Klebsiella infections in a patient with premorbid Ebstein anomaly: A case report

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.

Giant-Size Main Pulmonary Artery Aneurysm in an Adult Patient with Ebstein Anomaly and Dextrocardia

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.

Ebstein Anomaly: A Clinical Case Series among Adolescents in Guinea

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/m2, 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.

Strategy and technique for surgical treatment of Ebstein’s anomaly

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.

Clinical analysis of arrhythmia in 297 Ebstein＇s anomaly patients

Ebstein＇s anomaly is a kind of congenital heart disease ＇with part or all leaflets of tricuspid valve notnormally attaching to the tricuspid annulus in the normal position, but spirally shifting downward and abnormally attaching to the right ventricular wall. Ebstein＇s anomaly patients often suffer from arrhythmias, featured by paroxysmal fibrillation and supraventricular tachycardia, atrial atrial flutter, etc.

Anesthetic considerations of an emergency decompressive craniotomy complicated with Ebstein＇s anomaly and atrial septal defect

Despite considerable published papers regarding Ebstein＇s anomaly （EA） patients receiving open-heart tricuspid valve replacement, non-cardiac emergency surgeries were rarely reported. We report a case of emergency decormpressive craniotormy in a patient with EA. Anesthesiologists should pay special attention to the complications and anesthetic management during the non-cardiac surgeries performed in EA patients.hile papers regarding Ebstein＇s anomaly （EA）patients receiving open-heart tricuspid valve replacement are numerous, the reports of non-cardiac emergency surgeries have not been to the same degree.We report a case of emergency decompressive craniotomy in a patient with EA. This case report describes the symptoms and anesthetic management during the surgery.

Intraoperative Transesophageal Echocardiography in the Operation of Ebstein＇s Anomaly： A Retrospective Study

Background： Ebstein＇s anomaly （EA） has various spectrums in clinical and anatomic features. This study aimed to report the experience of two-dimensional intraoperative transesophageal echocardiography （2D-ITEE） during the EA surgery and to analyze the characteristics of the tricuspid valve （TV） by comparing the data from 2D-ITEE with the results from the surgery. Methods： 2D-ITEE data of 164 patients with EA who were operated in the First Hospital of Tsinghua University between July 2004 and April 2014 were retrospectively analyzed in this study. 2D-ITEE was applied in all patients. Downward displacement distances were measured, and the numbers of downward displacement or absent leaflets were compared with that of the surgery and with that of the two-dimensional-transthoracic echocardiogram （2D-TTE）. Data comparison was peribrmed using the Chi-square test. Results： The anterior leaflet partial or total downward displacement was 37.76 ±17.50 mm in 54 cases, absent in one patient; septal leaflet downward displacement was 29.07 ± 12.34 mm in 134 cases, absent in 17 cases; and posterior leaflet downward displacement was 43.18 ± 19.16 mm in 115 cases, absent in 34 cases. Statistically, there was no significant difference between the results from 2D-ITEE and that of 2D-TTE. The consistency rates of 2D-ITEE with operation for septal and posterior leaflets were 93.2% and 96.1%, respectively, while the rate for anterior was only 40.1%o, which was significantly different. Color Doppler flow image showed severe regurgitation in 150 cases and moderate in 14 cases. After surgical correction, moderate regurgitation of TV was found in 58 cases with 2D-ITEE, repair was performed again until the effect was satisfied. No complication occurred relating to the use of 2D-ITEE. Conclusions： 2D-ITEE could help diagnose anterior leaflet, evaluate the effect of TV repair, increase operational success rate, and reduce complication.

Managements of Ebstein's anomaly with Hetzer technique

Background Ebstein’s anomaly is a rare and complex congenital malformation of the tricuspid valve and the right ventricle with variable pathological anatomy. The surgical treatment of this anomaly has long been controversial. We reviewed cases corrected with Hetzer technique to analyze the uni/biventricular strategy,timing of surgery and results. Methods Between October 2009 and December 2013, 21 patients with Ebstein’s anomaly were admitted to Guangdong General Hospital and underwent surgical correction. There were 11 males and 10 females. Type A, 3 cases; Type B, 16 cases; Type C, 1 case, Type D, 1 case. The 2 patients of Type C and Type D undergoing Starnes procedure were excluded, all other 19 patients who underwent surgical repair with Hetzer technique in this time frame were included in the analysis. In this “Hetzer group”, the age at the time of the repair ranged from 13 months to 42 years（median： 8 years） and weight ranged from 11.3 to 55kg（median： 19.5 kg）. Combined anomalies include： 2 of 3 Type A combined with secondum atrial septal defect（ASD）, 14 of 16 cases of Type B combined with secondum ASD, both of 2 cases of Type C and D combined with secondum ASD. 2 of 21（9.5%） of these patients have Wolff-Parkinson-White（WPW）syndrome. Results There was no early, or late mortality. One 13-month infant boy, after valvuloplasty, ASD closure and bidirectional Glenn（BD-Glenn） procedure, presented with low cardiac output, depression of the left ventricle, severe tricuspid regurgitation（TR） with massively dilated right ventricle, the patient underwent emergent redo procedure, however, even after re-valvuloplasty, Transesophageal echocardiography（TEE）showed moderate to severe TR, we decided to reopen and enlarge the ASD, sewed a 5 mm fenestrated pericardial patch to close the tricuspid orifice partially, after 7 days, the patient weaned successfully from the ventilator. Another two 1-year old infants after valvuloplasty, BD Glenn and partial ASD closure showed no improvement of TR, but postoperative oxygen saturation increased, symptoms diminished and exercise tolerance improved significantly. Other 16 patients, whose age ranged from 5-42 years, had satisfactory results.Echocardiography showed： 14 of the 16 patients’ TR improved from severe to mild, 2 of them improved from moderate to trace or mild. Except the redo case, the pulse oxygen saturation at rest goes up to over 95%without cyanosis; 15 of 18 patients shows 85%-90% O2 saturation after exercise in room air. Conclusions Hetzer technique is a duplicable surgical approach to Ebstein’s anomaly with satisfactory results for older patients, especially for adults. However, we notice a trend that this technique may be not suitable for small infants, and further evaluation will be needed.