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.

A 59-year-old man presented for possible durable ventricular assist device (VAD) implantation. He had previously been diagnosed with congenitally corrected transposition of the great arteries, a ventricular septal defect, an atrial septal defect, pulmonary valve stenosis, and aortic valve regurgitation. In the previous 22 years, he had undergone palliative cardiac surgery 3 times. VAD implantation as a bridge to transplantation was planned. Owing to severe adhesions, mesocardia, a left ascending aorta, and moderate aortic regurgitation, we performed VAD implantation and aortic valve closure via a dual left thoracotomy and partial sternotomy.

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*

BACKGROUND: The Ross/Ross-Konno procedure is considered a good option for irreparable aortic valve disease in pediatric patients because of its hemodynamic performance and potential for growth of the pulmonary autograft. This study is a review of the long-term results of our 20-year experience with the Ross and Ross-Konno operations in a single institution. METHODS: Between June 1995 and January 2016, 16 consecutive patients (mean age, 6.0±5.9 years; range, 16 days to 17.4 years) underwent either a Ross operation (n=9) or a Ross-Konno operation (n=7). The study included 12 males and 4 females, with a median follow-up period of 47 months (range, 6 to 256 months). RESULTS: There were no cases of in-hospital or late mortality. Six reoperations were performed in 5 patients. Four patients underwent right ventricular-pulmonary artery (RV-PA) conduit replacement. Two patients underwent concomitant replacement of the pulmonary autograft and RV-PA conduit 10 years and 8 years after the Ross operation, respectively. The rate of freedom from adverse outcomes of the pulmonary autograft was 88% and 70% at 5 and 10 years, respectively. The rate of freedom from valve-related reoperations was 79% and 63% at 5 and 10 years, respectively. CONCLUSION: Pulmonary autografts demonstrated good durability with low mortality. The Ross/Ross-Konno procedure is a good option that can be performed safely in pediatric patients with aortic valve disease, even in a small-volume center.
Allografts ; Aortic Valve ; Arteries ; Autografts ; Female ; Follow-Up Studies ; Freedom ; Hemodynamics ; Humans ; Male ; Mortality

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

BACKGROUND AND OBJECTIVES: The aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure. SUBJECTS AND METHODS: We conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study. The median age at the time of the operation was 3.5 years (range: 0.5-29.7), and the median body weight was 13.3 kg (range: 5.8-54). The median follow-up duration was 7.7 years (range: 0.2-17.4). RESULTS: Atrial switch was achieved using the Mustard procedure in 12 patients (hemi-Mustard procedure in 11) or the Senning procedure in four patients. The ventriculoarterial procedure was performed using the Rastelli procedure in 11 patients and arterial switch in five patients. Six patients underwent tricuspid valvuloplasty. The survival rate was 93.8±6.1%. The rate of freedom from reoperation at 5 years was 92.3±7.4% in the Rastelli group. All patients except one were New York Heart Association class I. All patients except one had mild tricuspid regurgitation. CONCLUSION: Anatomic repair can be performed with a low risk of in-hospital mortality. The hemi-Mustard strategy for selected patients is one solution for reducing early mortality and morbidity, and long-term complications such as venous pathway stenosis or sinus node dysfunction.
Arterial Switch Operation ; Arteries* ; Body Weight ; Constriction, Pathologic ; Follow-Up Studies ; Freedom ; Heart ; Heart Defects, Congenital ; Hospital Mortality ; Humans ; Mortality ; Reoperation ; Retrospective Studies ; Sick Sinus Syndrome ; Survival Rate ; Tricuspid Valve Insufficiency

The objectives of this study were to assess the clinical outcomes of adults with Ebstein Anomaly (EA) according to their treatment modalities. All adult EA patients diagnosed between October 1994 and October 2014 were retrospectively evaluated by medical record review. Total 60 patients were categorized into 3 groups according to their treatment strategy, i.e. non-operative treatment (Group I, n = 23), immediate operative treatment (Group II, n = 27), and delayed operative treatment (Group III, n = 10). A composite of major adverse cardiac and cerebrovascular events (MACCE) and factors associated with MACCE were assessed in each treatment group. MACCE occurred in 13.0% patients in Group I, 55.6% patients in Group II and 50% in Group III (P = 0.006). Event free survivals at 5 years were 90% in Group I, 52.7% in Group II, 50.0% in Group III (P = 0.036). Post-operatively, most patients showed improvement on clinical symptoms. However, event free survival rate was lower in patients with operation compared to those with non-operative treatment (58.7% vs. 90.9%; P = 0.007). Major arrhythmic event occurred more frequently even after surgical ablation (50.0% vs. 20.0%; P = 0.034). Re-operation was more frequent in patients underwent delayed surgery compared to those with immediate surgery (50.0% vs. 18.5%; P = 0.001). Current guideline to decide patient's treatment strategy appeared to be appropriate in adult patients with EA. However, surgical ablation for arrhythmia was not enough so that concomitant medical treatment should be considered. Therefore, attentive risk stratification and cautious decision of treatment strategy by experienced cardiac surgeon are believed to improve clinical outcome.
Adult* ; Arrhythmias, Cardiac ; Disease-Free Survival ; Ebstein Anomaly* ; Humans ; Medical Records ; Retrospective Studies

BACKGROUND AND OBJECTIVES: The objective of this study was to analyze cardiovascular risk factors in adults with congenital heart disease (ACHD). SUBJECTS AND METHODS: The subjects for this study comprised 135 patients, aged 18 years and above, who visited the ACHD clinic at the Samsung Medical Center and 135 adults with a structurally normal heart who were randomly selected from the Center for Health Promotion during the same period. For the analysis, the ACHD group was further divided into an ACHD group that underwent correction by cardiac surgery and a cyanotic group. RESULTS: The mean (standard diviation) age (years) of patients in the surgically corrected group was 48.4 (10.9) years, while that of patients in the cyanotic group was 43.1 (9.0) years and that of patients in the control group was 47.1 (10.3) years (p=0.042). The adjusted odds ratios (ORs) for past smoking, hypertension, diabetes mellitus, hypercholesterolemia, obesity, and metabolic syndrome were significantly higher in the surgically corrected patients than in the controls. However, the ORs for all variables excluding past smoking were significantly lower in the cyanotic group compared with the control group. After adjustment for age, gender, smoking, alcohol use, and exercise, the ORs for metabolic syndrome were 0.46 (0.35-0.57, p<0.001) and 1.48 (1.14-1.92, p=0.003) in the cyanotic and surgically corrected groups, respectively. CONCLUSION: Cardiovascular risk factors need to be considered in surgically corrected ACHD patients as well as in adults with a structurally normal heart. A further study with a long-term follow-up is needed for developing guidelines for prevention.
Adult* ; Diabetes Mellitus ; Follow-Up Studies ; Health Promotion ; Heart ; Heart Defects, Congenital* ; Humans ; Hypercholesterolemia ; Hypertension ; Metabolic Syndrome X ; Obesity ; Odds Ratio ; Risk Factors* ; Smoke ; Smoking ; Thoracic Surgery

Tracheal agenesis is an extremely rare and typically lethal congenital disorder. Approximately 150 cases have been described since 1900, and very few cases of survival have been reported. We describe tracheal reconstruction with external esophageal stenting in a patient with Floyd's type II tracheal agenesis. Neither long-term survival nor survival without mechanical ventilation for even a single day has previously been reported in patients with Floyd's type II tracheal agenesis. The infant in the present case survived for almost a year and breathed without a ventilator for approximately 50 days after airway reconstruction using external supportive stents.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Humans ; Infant ; Respiration, Artificial ; Stents* ; Trachea ; Ventilators, Mechanical

Vasculitis is a heterogeneous group of diseases that destroy blood vessel walls by inflammation. Approximately half of vasculitis cases are idiopathic, but sometimes associated with genetic factors, medicines, chronic infection, autoimmune diseases, and malignancies. Although the mechanism remains unclear, vasculitis secondary to malignancy, also known as paraneoplastic vasculitis, has been reported. It is generally associated with hematologic malignancies rather than solid malignancies and commonly presents as leukocytoclastic vasculitis or polyarteritis nodosa. We experienced a case of leukocytoclastic vasculitis in a patient with hepatocellular carcinoma and membranous obstruction of the inferior vena cava. Here, we report this case with a brief review of literature.
Autoimmune Diseases ; Blood Vessels ; Carcinoma, Hepatocellular* ; Hematologic Neoplasms ; Humans ; Inflammation ; Polyarteritis Nodosa ; Vasculitis* ; Vena Cava, Inferior*