Echogenic Mass Lesion within the Main Pulmonary Artery in a Neonate

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.

Left Ventricular Assist Device Implantation via Dual Left Thoracotomy in an Adult Patient with Congenitally Corrected Transposition of the Great Arteries

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.

Outcomes of Coronary Artery Bypass Grafting after Extracorporeal Life Support in Patients with Cardiac Arrest or Cardiogenic Shock / 대한흉부외과학회지

BACKGROUND: Extracorporeal life support (ECLS) is used as a bridge to revascularization in high-risk patients with ischemic heart disease. We reviewed our experiences of coronary artery bypass grafting (CABG) after ECLS in patients with cardiac arrest or refractory cardiogenic shock. METHODS: We retrospectively reviewed 4,616 patients who underwent CABG at our institution between May 2006 and February 2017. We identified patients who underwent CABG following ECLS for cardiogenic shock or cardiac arrest. Twenty-three patients (0.5% of all CABG cases) were enrolled in the analysis. Their median age was 65 years (Q1–Q3, 58–77 years). Nine patients (39.1%) were diagnosed with ST-elevation myocardial infarction. Mechanical complications after acute myocardial infarction, including acute mitral regurgitation, left ventricular rupture, and ventricular septal defect, occurred in 9 patients (39.1%). RESULTS: The median time from cardiopulmonary resuscitation to ECLS initiation was 25 minutes (Q1–Q3, 18.5–28.5 minutes). Conventional CABG was performed in 10 patients (43.5%) who underwent concomitant intracardiac procedures. Postoperative ECLS was required in 16 patients (69.5%). The rate of successful ECLS weaning was 91.3% (n=21). There were 6 early mortalities (26.1%). CONCLUSION: CABG after ECLS was very rare in real-world circumstances. Although the early mortality rate was high, the risk of mortality may be acceptable under such devastating circumstances.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease

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.

Planned Transfusion of D-Positive Blood Components in an Asia Type DEL Patient: Proposed Modification of the Korean National Guidelines for Blood Transfusion

No abstract available.

Outcomes of Coronary Artery Bypass Grafting after Extracorporeal Life Support in Patients with Cardiac Arrest or Cardiogenic Shock

BACKGROUND@#Extracorporeal life support (ECLS) is used as a bridge to revascularization in high-risk patients with ischemic heart disease. We reviewed our experiences of coronary artery bypass grafting (CABG) after ECLS in patients with cardiac arrest or refractory cardiogenic shock.@*METHODS@#We retrospectively reviewed 4,616 patients who underwent CABG at our institution between May 2006 and February 2017. We identified patients who underwent CABG following ECLS for cardiogenic shock or cardiac arrest. Twenty-three patients (0.5% of all CABG cases) were enrolled in the analysis. Their median age was 65 years (Q1–Q3, 58–77 years). Nine patients (39.1%) were diagnosed with ST-elevation myocardial infarction. Mechanical complications after acute myocardial infarction, including acute mitral regurgitation, left ventricular rupture, and ventricular septal defect, occurred in 9 patients (39.1%).@*RESULTS@#The median time from cardiopulmonary resuscitation to ECLS initiation was 25 minutes (Q1–Q3, 18.5–28.5 minutes). Conventional CABG was performed in 10 patients (43.5%) who underwent concomitant intracardiac procedures. Postoperative ECLS was required in 16 patients (69.5%). The rate of successful ECLS weaning was 91.3% (n=21). There were 6 early mortalities (26.1%).@*CONCLUSION@#CABG after ECLS was very rare in real-world circumstances. Although the early mortality rate was high, the risk of mortality may be acceptable under such devastating circumstances.

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries

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.

The Ross Procedure in Pediatric Patients: A 20-Year Experience of Ross Procedure in a Single Institution

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.

Erratum: Inter-Facility Transport on Extracorporeal Life Support: Clinical Outcomes and Comparative Analysis with In-house Patients

We would like to correct the affiliation number to match the author and affiliation name.

Outcomes of Extracorporeal Membrane Oxygenation in Children: An 11-Year Single-Center Experience in Korea

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) has become an important treatment modality in pediatric patients with cardiopulmonary failure, but few studies have been conducted in Korea. METHODS: We conducted a retrospective review of pediatric patients younger than 18 years who were placed on ECMO between January 2004 and December 2014 at Samsung Medical Center. RESULTS: We identified 116 children on ECMO support. The overall rate of successful weaning was 51.7%, and the survival to discharge rate was 37.1%. There were 39, 61, and 16 patients on ECMO for respiratory, cardiac, and extracorporeal cardiopulmonary resuscitation, respectively. The weaning rate in each group was 48.7%, 55.7%, and 43.8%, respectively. The survival rate was 43.6%, 36.1%, and 25.0%, respectively. Sixteen patients on ECMO had functional single ventricle physiology; in this group, the weaning rate was 43.8% and the survival rate was 31.3%. Ten patients were on ECMO as a bridge to transplantation (8 for heart and 2 for lung). In patients with heart transplantation, the rate of survival to transplantation was 50.0%, and the overall rate of survival to discharge was 37.5%. CONCLUSION: An increasing trend in pediatric ECMO utilization was observed. The outcomes were favorable considering the early experiences that were included in this study and the limited supply of specialized equipment for pediatric patients.

Inter-Facility Transport on Extracorporeal Life Support: Clinical Outcomes and Comparative Analysis with In-house Patients

BACKGROUND: Extracorporeal life support (ECLS) is widely used in refractory heart or lung failure, and the demand for inter-facility transportation on ECLS is expanding. However, little is known about post-transportation outcomes, the clinical safety of such transportation, or the characteristics of the transported patients. METHODS: This was a retrospective review of a 3-year, single-institution experience with inter-facility ECLS transport, as well as a comparative analysis of clinical outcomes with those of in-house patients. We also analyzed the risk factors for hospital mortality in the entire ECLS population using univariate and multivariate analyses to investigate the effects of transport. RESULTS: All 44 patients were safely transported without adverse events. The average travel distance was 178.7 km, with an average travel time of 74.0 minutes. Early survival of the transported group seemed to be better than that of the in-house group, but the difference was not statistically significant (70.5% vs. 56.6%, p=0.096). The incidence of complications was similar between the 2 groups, except for critical limb ischemia, which was significantly more common in the transported group than in the in-house group (25.0% vs. 8.1%, p=0.017). After adjusting for confounders, being part of the transported group was not a predictor of early death (adjusted odds ratio, 0.689; p=0.397). CONCLUSION: Transportation of patients on ECLS is relatively safe, and the clinical outcomes of transported patients are comparable to those of in-house ECLS patients. Although matched studies are required, our study demonstrates that transporting patients on ECLS did not increase their risk of hospital mortality after adjustment for other factors.

Right Aortic Arch with a Retroesophageal Left Subclavian Artery and an Anomalous Origin of the Pulmonary Artery from the Aorta

We report the case of a newborn with a rare anatomic variation: a right aortic arch with a retroesophageal left subclavian artery and an anomalous origin of the pulmonary artery from the aorta. This variation was diagnosed using echocardiography and computed tomography, and we treated the condition surgically.

The Mid-term Results of Thoracoscopic Closure of Atrial Septal Defects

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.

How Long Can the Next Intervention Be Delayed after Balloon Dilatation of Homograft in the Pulmonary Position?

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.

Clinical Outcomes after Anatomic Repair Including Hemi-Mustard Operation in Patients with Congenitally Corrected Transposition of the Great Arteries

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.

Hemodynamic Follow-Up in Adult Patients with Pulmonary Hypertension Associated with Atrial Septal Defect after Partial Closure

PURPOSE: We evaluated the hemodynamic statuses of patients after partial closure of atrial septal defects with fenestration due to pulmonary hypertension. MATERIALS AND METHODS: Seventeen adult patients underwent partial atrial septal defect closure and follow-up cardiac catheterization. We analyzed hemodynamic data and clinical parameters before and after closure. RESULTS: The median age at closure was 29 years old. The baseline Qp/Qs was 1.9+/-0.6. The median interval from the operation to the cardiac catheterization was 27 months. The CT ratio decreased from 0.55+/-0.07 to 0.48+/-0.06 (p<0.05). The mean pulmonary arterial pressure decreased from 50.0+/-11.5 mm Hg to 32.5+/-14.4 mm Hg (p<0.05), and the pulmonary resistance index decreased from 9.2+/-3.6 Wood units*m2 to 6.3+/-3.8 Wood units*m2 (p<0.05). Eleven patients (64.7%) continued to exhibit high pulmonary resistance (over 3.0 Wood units*m2) after closure. These patients had significantly higher pulmonary resistance indices and mean pulmonary arterial pressures based on oxygen testing before the partial closures (p<0.05). However, no significant predictors of post-closure pulmonary hypertension were identified. CONCLUSION: Despite improvement in symptoms and hemodynamics after partial closure of an atrial septal defect, pulmonary hypertension should be monitored carefully.

Natural Course of Adult Ebstein Anomaly When Treated according to Current Recommendation

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.

Complicated Azygos Vein Aneurysm in an Infant Presenting with Acute Pulmonary Thromboembolism

Azygos vein aneurysm is a rare cause of mediastinal mass. Most cases present as an incidental finding on imaging modalities, but in few cases the thrombosis in the aneurysm leads to pulmonary thromboembolism, which may require surgical resection. We present a case where, for the first time, a case of a complicated azygos vein aneurysm was diagnosed in infancy, which required surgical resection.

Diagnosis of Right Ventricular Vegetation on Late Gadolinium-Enhanced MR Imaging in a Pediatric Patient after Repair of a Ventricular Septal Defect

We report a case of vegetation in a 4-year-old female with infective endocarditis, diagnosed by late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) imaging. The patient had a history of primary closure for ventricular septal defect and presented with mild febrile sensation. No remarkable clinical symptoms or laboratory findings were noted; however, transthoracic echocardiography demonstrated a 14 mm highly mobile homogeneous mass in the right ventricle. On LGE CMR imaging, the mass showed marginal rim enhancement, which suggested the diagnosis of vegetation rather than thrombus. The extracellular volume fraction (≥ 42%) of the lesion was higher than that of normal myocardium. Based on the patient's clinical history of congenital heart disease and pathologic confirmation of the lesion, a diagnosis of infective endocarditis with vegetation was made.

Tracheal Agenesis Reconstruction with External Esophageal Stenting: Postoperative Results and Complications

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.