Background: and Objective: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein’s anomaly. Methods: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein’s anomaly at a single tertiary center between January 2005 and June 2021. Results: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein’s anomaly; the median age was 5.8 years (range, 0.01–16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. −1.15, p<0.001).However, from 1 year to 7 years after surgery, the z values were maintained between −1 and +1.Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. Conclusions Ebstein’s anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.

Tegoprazan is a novel potassium-competitive acid blocker that treats gastric acid-related diseases. Clarithromycin was widely used as one of various regimens for eradicating Helicobacter pylori. This study compared the pharmacokinetic and safety profile of tegoprazan and clarithromycin between combination therapy and monotherapy to evaluate the potential drug-drug interaction. An open-label, randomized, 6-sequence, 3-period crossover study was conducted in 24 healthy subjects. According to the assigned sequence, the subject was administered the assigned treatment during 5 days in each period. PK parameters of tegoprazan and clarithromycin administered in combination were compared with those of the respective monotherapies. The co-administration of tegoprazan with clarithromycin increased maximum steady-state plasma concentration (C ss,max ) and area under the plasma concentration-time curve in dosing interval at steady-state (AUC ss,tau ) of tegoprazan (1.6-fold in C ss,max and 2.5-fold in AUC ss,tau ) and M1 (2.0-fold in C ss,max , 2.5-fold in AUC ss,tau ) than tegoprazan alone. The C ss,max and AUC sss,tau of 14-hydroxyclarithromycin increased 1.8- and 2.0-fold in co-administration, respectively. The AUC ss.tau of clarithromycin was slightly increased in co-administration, but C ss,max was not changed. Combination of tegoprazan and clarithromycin and those of the respective monotherapies were tolerated in 24 healthy subjects. There may exist drug interaction that lead to reciprocal increase in plasma drug concentrations when tegoprazan and clarithromycin were administrated in combination and no safety concerns were raised. It is suggested that an in-depth analysis of the concentrationresponse relationship is necessary to determine whether these concentration changes warrant clinical action.

Here we present a rare case of pulmonary arterial thrombosis associated with a ductus arteriosus aneurysm that caused severe pulmonary stenosis. A 5-day-old newborn was admitted to our hospital for the evaluation of an intracardiac mass-like lesion found after the detection of a cardiac murmur. Echocardiography and heart computed tomography revealed a mass-like lesion measuring 8.1 mm in diameter across the distal main pulmonary artery to the proximal left pulmonary artery resulting in localized severe stenosis of the left pulmonary artery. Left pulmonary artery angioplasty for surgical resection of the thrombus revealed that the mass was adherent to the proximal part of the left pulmonary artery anterior wall and extended to the ductus arteriosus. Histological examination of the mass showed an old thrombus with dystrophic calcification. Five months after surgery, follow-up echocardiography showed that the left pulmonary artery peak pressure gradient had decreased but the proximal left pulmonary artery stenosis remained. Cardiac catheterization and balloon angioplasty suc cessfully relieved the pulmonary stenosis.

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.
Aorta, Thoracic ; Aortic Coarctation ; Arrhythmias, Cardiac ; Arteries ; Birth Rate ; Echocardiography ; Fetus ; Heart Defects, Congenital ; Heart Diseases ; Hypoplastic Left Heart Syndrome ; Parturition ; Prenatal Diagnosis ; Pulmonary Atresia ; Surgeons ; Tetralogy of Fallot

It is challenging to achieve sufficient hearing gain in patients with mixed hearing loss. In chronic middle ear diseases, conventional passive reconstructive surgeries often result in suboptimal hearing gain and additional hearing aids may have limitations due to insufficient sound amplification, occlusion effect, acoustic feedback, and skin irritation. Middle ear implantation (MEI) using Vibrant Soundbridge (VSB) is another option for auditory rehabilitation in mixed hearing loss as well as sensorineural hearing loss. The floating mass transducer of VSB can be placed on various middle ear structures either directly or using different types of couplers in order to deliver vibratory mechanical energy to the cochlea. We report a patient who presented with bilateral mixed hearing loss due to chronic otitis media and had limitations using conventional hearing aids in the worse hearing ear. The patient was successfully treated with MEI using the Bell coupler together with middle ear surgery in a single step.
Acoustics ; Cochlea ; Ear ; Ear, Middle* ; Hearing ; Hearing Aids ; Hearing Loss, Mixed Conductive-Sensorineural* ; Hearing Loss, Sensorineural ; Humans ; Ossicular Prosthesis* ; Otitis Media ; Rehabilitation ; Skin ; Transducers

BACKGROUND AND OBJECTIVES: The early assessment of treatment is not done for benign paroxysmal positional vertigo (BPPV) since the well-known phenomenon of fatigability after a repeated positional test can mimic successful treatment. The aim of this study is to evaluate the clinical implication of ‘fatigability’ after Epley maneuver and to identify the therapeutic efficacy of Epley maneuver in posterior canal BPPV (PC-BPPV). SUBJECTS AND METHOD: This study was prospectively conducted by two dizziness clinics on 51 consecutive patients diagnosed with PC-BPPV. All patients included in the study received Epley maneuver treatment. The therapeutic results were reassessed immediately after a single trial of Epley maneuver. After 30 minutes, results were reassessed repeatedly to confirm the fatigability of diagnostic procedure immediately after treatment. If the treatment was not successful after 30 minutes, Epley maneuver was repeatedly performed until complete resolution. RESULTS: Immediately after the first maneuver, 45 of 51 (88.2%) patients had neither vertigo nor nystagmus during the positional test. All patients demonstrated complete resolution after receiving one to three Epley maneuvers on the day of diagnosis. ‘Fatigability (false negative result)’ was confirmed for only one case (1 of 6 patients, 16.7%), in which nystagmus was observed after 30 minutes but not identified immediately after the first Epley maneuver. CONCLUSION: The therapeutic efficacy of Epley maneuver is very high in PC-BPPV. Considering the possibility of fatigability when reassessment is performed immediately after therapeutic maneuver, clinicians should avoid assessing the outcome immediately after treatment in patients with PC-BPPV.
Benign Paroxysmal Positional Vertigo* ; Diagnosis ; Dizziness ; Fatigue ; Humans ; Methods ; Prospective Studies ; Vertigo

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient, the right subclavian artery originated from the pulmonary artery, which is why he did not show reversed differential cyanosis. We conclude that the presence of an aortic arch anomaly should be considered in patients with d-TGA who do not present with reversed differential cyanosis. A further imaging work-up, including computed tomography or magnetic resonance imaging, might be helpful.
Aorta, Thoracic ; Arteries* ; Cyanosis ; Humans ; Magnetic Resonance Imaging ; Pulmonary Artery ; Subclavian Artery*

Cholesterol embolization syndrome (CES) usually occurs after endovascular procedures, it may also occurs after using anticoagulants and thrombolytics. We report a case of 66-year-old man with sudden elevation of creatinine after using warfarin due to cortical infarction. Histologic examinations revealed a cholesterol cleft on the arcuate artery. We concluded it as warfarin induced atheroembolic renal disease. Careful observation of kidneys is necessary in the case of renal abnormalities after using anticoagulation, considering the possibility of cholesterol embolism due to anticoagulant therapy.
Aged ; Anticoagulants ; Arteries ; Cholesterol ; Creatinine ; Embolism ; Embolism, Cholesterol ; Endovascular Procedures ; Humans ; Infarction ; Kidney ; Warfarin

BACKGROUND AND OBJECTIVES: We investigated the effectiveness of balloon dilatation of homograft conduits in the pulmonary position in delaying surgical replacement. SUBJECTS AND METHODS: We reviewed the medical records of patients who underwent balloon dilatation of their homograft in the pulmonary position from 2001 to 2015. The pressure gradient and ratio of right ventricular pressure were measured before and after the procedure. The primary goal of this study was to evaluate the parameters associated with the interval to next surgical or catheter intervention. RESULTS: Twenty-eight balloon dilations were performed in 26 patients. The median ages of patients with homograft insertion and balloon dilatation were 20.3 months and 4.5 years, respectively. The origins of the homografts were the aorta (53.6%), pulmonary artery (32.1%), and femoral vein (14.3%). The median interval after conduit implantation was 26.7 months. The mean ratio of balloon to graft size was 0.87. The pressure gradient through the homograft and the ratio of right ventricle to aorta pressure were significantly improved after balloon dilatation (p<0.001). There were no adverse events during the procedure with the exception of one case of balloon rupture. The median interval to next intervention was 12.9 months. The median interval of freedom from re-intervention was 16.6 months. Cox proportional hazards analysis revealed that the interval of freedom from re-intervention differed only according to origin of the homograft (p=0.032), with the pulmonary artery having the longest interval of freedom from re-intervention (p=0.043). CONCLUSION: Balloon dilatation of homografts in the pulmonary position can be safely performed, and homografts of the pulmonary artery are associated with a longer interval to re-intervention.
Allografts* ; Angioplasty, Balloon ; Aorta ; Catheters ; Dilatation* ; Femoral Vein ; Freedom ; Heart Ventricles ; Humans ; Medical Records ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Rupture ; Transplants ; Ventricular Pressure

BACKGROUND AND OBJECTIVES: Recently, minimally invasive surgical (MIS) techniques including robot-assisted operations have been widely applied in cardiac surgery. The thoracoscopic technique is a favorable MIS option for patients with atrial septal defects (ASDs). Accordingly, we report the mid-term results of thoracoscopic ASD closure without robotic assistance. SUBJECTS AND METHODS: We included 66 patients who underwent thoracoscopic ASD closure between June 2006 and July 2014. Mean age was 27±9 years. The mean size of the ASD was 25.9±6.3 mm. Eleven patients (16.7%) had greater than mild tricuspid regurgitation (TR). The TR pressure gradient was 32.4±8.6 mmHg. RESULTS: Fifty-two (78.8%) patients underwent closure with a pericardial patch and 14 (21.2%) underwent direct suture closure. Concomitant procedures included tricuspid valve repair in 8 patients (12.1%), mitral valve repair in 4 patients (6.1%), and right isthmus block in 1 patient (1.5%). The mean length of the right thoracotomy incision was 4.5±0.9 cm. The mean cardiopulmonary bypass time was 159±43 minutes, and the mean aortic cross clamp time was 79±29 minutes. The mean hospital stay lasted 6.1±2.6 days. There were no early deaths. There were 2 reoperations. One was due to ASD patch detachment and the other was due to residual mitral regurgitation after concomitant mitral valve repair. However, there have been no reoperations since July 2010. There were 2 pneumothoraxes requiring chest tube re-insertion. There was one wound dehiscence in an endoscopic port. The mean follow-up duration was 33±31 months. There were no deaths, residual shunts, or reoperations during follow-up. CONCLUSION: Thoracoscopic ASD closure without robotic assistance is feasible, suggesting that this method is a reliable MIS option for patients with ASDs.
Cardiopulmonary Bypass ; Chest Tubes ; Follow-Up Studies ; Heart Septal Defects, Atrial* ; Humans ; Length of Stay ; Methods ; Minimally Invasive Surgical Procedures ; Mitral Valve ; Mitral Valve Insufficiency ; Pneumothorax ; Sutures ; Thoracic Surgery ; Thoracic Surgery, Video-Assisted ; Thoracoscopes ; Thoracotomy ; Tricuspid Valve ; Tricuspid Valve Insufficiency ; Wounds and Injuries