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Background and Objectives@#Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE. @*Methods@#We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea. @*Results@#PLE occurred in 4.6% of the total 832 patients after the Fontan operation. After a mean period of 7.7 years after Fontan operation, PLE was diagnosed at a mean age of 11.6years. The mean follow-up period was 8.9 years. The survival rates were 81.6% at 5 years and 76.5% at 10 years. In the multivariate analysis, New York Heart Association Functional classification III or IV (p=0.002), low aortic oxygen saturation (<90%) (p=0.003), and ventricular dysfunction (p=0.032) at the time of PLE diagnosis were found as predictors of mortality. PLE was resolved in 10 of the 38 patients after treatment. Among medical managements, an initial heparin response was associated with survival (p=0.043). Heparin treatment resulted in resolution in 4 patients. We found no evidence on pulmonary vasodilator therapy alone. PLE was also resolved after surgical Fontan fenestration (2/6), aortopulmonary collateral ligation (1/1), and transplantation (1/1). @*Conclusions@#The survival rate of patients with Fontan-associated PLE has improved with the advancement of conservative care. Although there is no definitive method, some treatments led to the resolution of PLE in one-fourth of the patients. Further investigations are needed to develop the best prevention and therapeutic strategies for PLE.
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Background and Objectives@#We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes. @*Methods@#This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99). @*Results@#The median age of the patients was 14.0 months (interquartile range [IQR], 10.7–19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the followup, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2–17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group. @*Conclusions@#Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age <1 year at the time of TOF repair and shunt implantation prior to TOF repair were predictive factors for requiring PVR.
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Background and Objectives@#We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes. @*Methods@#This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99). @*Results@#The median age of the patients was 14.0 months (interquartile range [IQR], 10.7–19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the followup, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2–17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group. @*Conclusions@#Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age <1 year at the time of TOF repair and shunt implantation prior to TOF repair were predictive factors for requiring PVR.
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Background@#The double-lumen cannula (DLC) has begun to be used worldwide for venovenous (VV) extracorporeal membrane oxygenation (ECMO). We aimed to examine whether the DLC could be an effective tool in the treatment of pediatric respiratory failure in Korea. @*Methods@#We reviewed the records of patients weighing under 15 kg who underwent ECMO due to respiratory failure between January 2017 and December 2018. Outcomes of ECMO using a DLC and conventional ECMO using central method or 2 peripheral cannulas were compared. @*Results@#Twelve patients were treated with ECMO for respiratory failure. Among them, a DLC was used in 5 patients, the median age of whom was 3.8 months (interquartile range, 0.1–49.7 months). In these patients, the median values of pH, partial pressure of carbon dioxide, and partial pressure of oxygen were 7.09, 74 mm Hg, and 37 mm Hg before ECMO and corrected to 7.31, 44 mm Hg, and 85 mm Hg, respectively, after ECMO cannulation. Median blood flow rate in the patients treated with ECMO using a DLC was slightly higher than that in the conventional ECMO group, but this difference was not statistically significant (86.1 mL/kg/min and 74.3 mL/kg/min, respectively; p=1.00). One patient from the DLC group and 3 patients from the conventional group were weaned off ECMO. @*Conclusion@#VV ECMO using a DLC provided adequate oxygenation, ventilation, and blood flow rate in Korean pediatric patients with respiratory failure. Further prospective and randomized studies are warranted.
ABSTRACT
Insertion of an epicardial pacemaker is a useful treatment for pediatric patients with an abnormal heart rhythm. However, there are limitations and concerns when implanting epicardial pacemakers in infants and neonates due to their small body size. We report a patient who experienced rare complications after implantation of a permanent pacemaker.
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Purpose@#The aim of this study was to evaluate the feasibility and satisfaction of an online global health education course for medical students in comparison with an in-person of the course and to assess students’ preferences regarding online methods of delivery. @*Methods@#Second-year medical students enrolled in this course in 2019 (in-person) and 2020 (online). The attendance rate, satisfaction in the course evaluation survey, and academic achievement on the written final examination were utilized to compare the two different methods of course delivery. The medical students who took the online course were also asked about their preferences regarding the method of course delivery and the advantages and drawbacks of each method of online lectures. @*Results@#There was no significant difference in the attendance rate and overall satisfaction between the two groups. The mean score on the written examination of the online course (84.1±19.6) showed comparable effects to the in-person course (78.0±18.3). The percentages of students who achieved high performance (55.5%) and the achieved minimum requirement (95.9%) were also maintained compared to the in-person course (14.6% and 93.6%, respectively). Medical students preferred the online course to the in-person course; in particular, they preferred prerecorded videos over live streaming online lectures. @*Conclusion@#The participation, satisfaction, and the academic achievement of the online course were comparable to those of the in-person course. However, the greatest drawback of the online course was the lack of interaction between peer learners. Therefore, diverse methods for online education should be considered to increase students’ sense of belonging to a learning community.
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Purpose@#The aim of this study was to evaluate the feasibility and satisfaction of an online global health education course for medical students in comparison with an in-person of the course and to assess students’ preferences regarding online methods of delivery. @*Methods@#Second-year medical students enrolled in this course in 2019 (in-person) and 2020 (online). The attendance rate, satisfaction in the course evaluation survey, and academic achievement on the written final examination were utilized to compare the two different methods of course delivery. The medical students who took the online course were also asked about their preferences regarding the method of course delivery and the advantages and drawbacks of each method of online lectures. @*Results@#There was no significant difference in the attendance rate and overall satisfaction between the two groups. The mean score on the written examination of the online course (84.1±19.6) showed comparable effects to the in-person course (78.0±18.3). The percentages of students who achieved high performance (55.5%) and the achieved minimum requirement (95.9%) were also maintained compared to the in-person course (14.6% and 93.6%, respectively). Medical students preferred the online course to the in-person course; in particular, they preferred prerecorded videos over live streaming online lectures. @*Conclusion@#The participation, satisfaction, and the academic achievement of the online course were comparable to those of the in-person course. However, the greatest drawback of the online course was the lack of interaction between peer learners. Therefore, diverse methods for online education should be considered to increase students’ sense of belonging to a learning community.
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Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.
Subject(s)
Adult , Female , Humans , Aortic Coarctation , Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Constriction, Pathologic , Diagnosis , Follow-Up Studies , Heart , Heart Defects, Congenital , Mitral ValveABSTRACT
Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.
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BACKGROUND: Early extubation after cardiovascular surgery has some clinical advantages, including reduced hospitalization costs. Herein, we review the results of ultra-fast-track (UFT) extubation, which refers to extubation performed on the operating table just after the operation, or within 1–2 hours after surgery, in patients with congenital cardiac disease. METHODS: We performed UFT extubation in patients (n=72) with a relatively simple congenital cardiac defect or who underwent a simple operation starting in September 2016. To evaluate the feasibility and effectiveness of our recently introduced UFT extubation strategy, we retrospectively reviewed 195 patients who underwent similar operations for similar diseases from September 2015 to September 2017, including the 1-year periods immediately before and after the introduction of the UFT extubation protocol. Propensity scores were used to assess the effects of UFT extubation on length of stay (LOS) in the intensive care unit (ICU), hospital LOS, and medical costs. RESULTS: After propensity-score matching using logistic regression analysis, 47 patients were matched in each group. The mean ICU LOS (16.3±28.6 [UFT] vs. 28.0±16.8 [non-UFT] hours, p=0.018) was significantly shorter in the UFT group. The total medical costs (182.6±3.5 [UFT] vs. 187.1±55.6 [non-UFT] ×100,000 Korean won [KRW], p=0.639) and hospital stay expenses (48.3±13.6 [UFT] vs. 54.8±29.0 [non-UFT] ×100,000 KRW, p=0.164) did not significantly differ between the groups. CONCLUSION: UFT extubation decreased the ICU LOS and mechanical ventilation time, but was not associated with postoperative hospital LOS or medical expenses in patients with simple congenital cardiac disease.
Subject(s)
Humans , Heart Defects, Congenital , Heart Diseases , Hospitalization , Intensive Care Units , Length of Stay , Logistic Models , Operating Tables , Propensity Score , Respiration, Artificial , Retrospective StudiesABSTRACT
One of the complications of permanent pacemaker implantation is unintended phrenic nerve stimulation. A 15-year-old boy with a permanent pacemaker presented with chest discomfort due to synchronous chest wall contraction with pacing beats. Even after reprogramming of the pacemaker, diaphragmatic stimulation persisted. Therefore, we performed thoracoscopic phrenic nerve insulation using a Gore-Tex patch to insulate the phrenic nerve from the wire. A minimally invasive approach using a thoracoscope is a feasible option for retractable phrenic nerve stimulation after pacemaker implantation.
Subject(s)
Adolescent , Humans , Male , Minimally Invasive Surgical Procedures , Pacemaker, Artificial , Phrenic Nerve , Polytetrafluoroethylene , Thoracic Wall , Thoracoscopes , Thoracoscopy , ThoraxABSTRACT
Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adjacent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.
ABSTRACT
Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adjacent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.
Subject(s)
Humans , Allografts , Aneurysm , Aorta , Aortic Valve Insufficiency , Arteries , Dilatation , Fontan Procedure , Heart Ventricles , Norwood Procedures , Pulmonary ArteryABSTRACT
A 6-year-old boy with underlying hemolytic anemia of unknown etiology, atopic dermatitis, and recurrent urticaria visited our hospital because of acute respiratory failure induced by influenza A. Despite mechanical ventilation after endotracheal intubation along with inhalation of nitric oxide, respiratory acidosis and hypoxemia persisted. Veno-venous extracorporeal membrane oxygenation (VV ECMO) insertion was performed to provide respiratory support. After performing flexible bronchoscopy, we found that thick mucus plugs were obstructing the right bronchus intermedius and the upper lobe orifice. After bronchial washing and removal of the plugs, we were able to wean the patient off VV ECMO and transfer him to the general ward. He was discharged without any neurologic or pulmonary sequelae.
Subject(s)
Child , Humans , Male , Acidosis, Respiratory , Anemia, Hemolytic , Hypoxia , Bronchi , Bronchitis , Bronchoscopy , Dermatitis, Atopic , Extracorporeal Membrane Oxygenation , Influenza A virus , Influenza, Human , Inhalation , Intubation, Intratracheal , Mucus , Nitric Oxide , Patients' Rooms , Plastics , Pneumonia , Respiration, Artificial , Respiratory Insufficiency , UrticariaABSTRACT
Pulmonary arteriovenous fistula (PAVF) is a complication of the Glenn shunt. A 57-year-old tetralogy of Fallot (TOF) patient, who had undergone a Glenn shunt and TOF total correction, complained of dyspnea and cyanosis. PAVFs were present in the right lung, and right lung perfusion was nearly absent. After coil embolization, takedown of the Glenn shunt, and reconstruction of the right pulmonary artery, the patient's symptoms were relieved. Extrapulmonary radioisotope uptake caused by the PAVFs shown in lung perfusion scans decreased, and right lung perfusion increased gradually. Although the development and resolution of PAVFs after a Glenn shunt have been reported in the pediatric population, this may be the first report on this change in old age.
Subject(s)
Humans , Middle Aged , Arteriovenous Fistula , Cyanosis , Dyspnea , Embolization, Therapeutic , Fontan Procedure , Hepatopulmonary Syndrome , Lung , Perfusion , Pulmonary Artery , Tetralogy of FallotABSTRACT
BACKGROUND AND OBJECTIVES: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit. Of these, 9 grown-up congenital heart disease patients were over 18 years of age, and the median age of the subject group was 5.82 years (range: 1 day to 41.6 years). A review of all demographic, clinical, and surgical data and survival analysis were performed. RESULTS: A total of 45 (52.3%) patients were successfully weaned from the mechanical assist device, and 25 (29.1%) survivors were able to be discharged. There was no significant difference in results between patients over 18 years and under 18 years of age. Risk factors for mortality were younger age (<30 days), functional single ventricle anatomy, support after cardiac operations, longer support duration, and deteriorated pre-ECMO status (severe metabolic acidosis and increased levels of lactate, creatinine, bilirubin, or liver enzyme). The survival rate has improved since 2010 (from 25% before 2010 to 35% after 2010), when we introduced an upgraded oxygenator, activated heart transplantation, and also began to apply ECMO before the end-stage of cardiac dysfunction, even though we could not reveal significant correlations between survival rate and changed strategies associated with ECMO. CONCLUSION: Mechanical circulatory support has played a critical role and has had a dramatic effect on survival in patients with medically intractable heart failure, particularly in recent years. Meticulous monitoring of acid-base status, laboratory findings, and early and liberal applications are recommended to improve outcomes without critical complication rates, particularly in neonates with single ventricle physiology.
Subject(s)
Humans , Infant, Newborn , Acidosis , Bilirubin , Cardiac Output, Low , Creatinine , Critical Care , Extracorporeal Membrane Oxygenation , Heart Defects, Congenital , Heart Failure , Heart Transplantation , Heart-Assist Devices , Intensive Care Units , Lactic Acid , Liver , Mortality , Oxygen , Oxygenators , Physiology , Resuscitation , Retrospective Studies , Risk Factors , Survival Rate , SurvivorsABSTRACT
BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.
Subject(s)
Humans , Arrhythmias, Cardiac , Arteriovenous Fistula , Biliary Atresia , Follow-Up Studies , Heart , Heart Diseases , Heterotaxy Syndrome , Isomerism , Medical Records , Mortality , Prognosis , Survival RateABSTRACT
Management of severely dilated pulmonary artery (PA) associated with severe pulmonary hypertension from congenital heart disease remains controversial, primarily due to its rare nature and concern for perioperative unpredictable complications. Herein, we report a 25 year-old female with a severely dilated PA (up to 73 mm), who was successfully treated by a PA graft replacement by creating a Y-shaped conduit using a 28 mm hemashield tube in the main PA and a 20 mm hemashield tube in both proximal parts of the branch PA.
Subject(s)
Female , Humans , Heart Defects, Congenital , Hypertension, Pulmonary , Pulmonary Artery , Pulmonary Surgical Procedures , TransplantsABSTRACT
Persistent fifth aortic arch (PFAA) is a rare congenital anomaly of the aortic arch frequently associated with other cardiovascular anomalies, such as tetralogy of Fallot and aortic arch coarctation or interruption. We report the case of a neonate with PFAA with coarctation who successfully underwent surgical repair.
Subject(s)
Humans , Infant, Newborn , Aorta, Thoracic , Tetralogy of FallotABSTRACT
BACKGROUND AND OBJECTIVES: The aim of the study is to evaluate the long-term results after a surgical repair of Ebstein's anomaly. SUBJECTS AND METHODS: Forty-eight patients with Ebstein's anomaly who underwent open heart surgery between 1982 and 2013 were included. Median age at operation was 5.6 years (1 day-42.1 years). Forty-five patients (93.7%) demonstrated tricuspid valve (TV) regurgitation of less than moderate degree. When the patients were divided according to Carpentier's classification, types A, B, C, and D were 11, 21, 12, and 4 patients, respectively. Regarding the type of surgical treatment, bi-ventricular repair (n=38), one-and-a half ventricular repair (n=5), and single ventricle palliation (n=5) were performed. Of 38 patients who underwent a bi-ventricular repair, TV repairs were performed by Danielson's technique (n=20), Carpentier's technique (n=11), Cone repair (n=4), and TV annuloplasty (n=1). Two patients underwent TV replacement. Surgical treatment strategies were different according to Carpentier's types (p<0.001) and patient's age (p=0.022). RESULTS: There were 2 in-hospital mortalities (4.2%; 1 neonate and 1 infant) and 2 late mortalities during follow-up. Freedom from recurrent TV regurgitation rates at 5, 10, and 15 years were 88.6%, 66.3%, 52.7%, respectively. TV regurgitation recurrence did not differ according to surgical method (p=0.800). Survival rates at 5, 10, and 20 years were 95.8%, 95.8%, and 85.6%, respectively, and freedom from reoperation rates at 5, 10, and 15 years were 85.9%, 68.0%, and 55.8%, respectively. CONCLUSION: Surgical treatment strategies were decided according to Carpentier's type and patient's age. Overall survival and freedom from reoperation rates at 10 years were 95.8% and 68.0%, respectively. Approximately 25% of patients required a second operation for TV during the follow-up.