Right lower lung midline herniation as a rare complication in an infant with heart-lung transplantation: A case report.

BACKGROUND: Lung herniation is a rare complication of heart-lung transplantation that can be fatal owing to vascular compromise and airway obstruction. To date, only five cases of lung herniation related to heart-lung transplantation have been reported in the literature; however, to the best of our knowledge, this is the first worldwide report of heart-lung transplantation-related lung herniation in an infant. METHODS: We describe the case of lung herniation as a rare heart-lung transplantation-related complication in an infant. A 12-month-old female baby developed severe bronchopulmonary dysplasia with severe pulmonary hypertension, and she underwent extracorporeal membrane oxygenation for cardiac collapse and lung support. Then, we performed heart-lung transplantation to manage the irreversible deterioration of her lung function. After the heart-lung transplantation, we found the radiological abnormalities persisted on follow-up chest radiographs until the 13th postoperative day diagnosed as lung herniation of the right lower lobe on chest computed tomography. RESULTS: After the relocation of the herniated lung, the clinical condition of the patient improved, and the patient is currently growing without any respiratory symptoms. CONCLUSIONS: In this case report, we emphasize that clinical awareness and high suspicion of this rare complication are needed for early diagnosis and proper treatment to prevent post-transplantation morbidity and mortality related to potential ischemic injury.

Outcomes of surgery for coarctation of the aorta based on a new classification system.

OBJECTIVES: To evaluate early- and long-term outcomes of the surgical treatment for coarctation of the aorta based on a new classification system. METHODS: A retrospective clinical review of 111 patients with coarctation of the aorta who underwent surgery (March 2011 to August 2020) was performed. We categorised coarctation of the aorta into type I, with all three head vessels tightly packed; type II, with the left subclavian artery separated from the two other head vessels; and type III, with all three head vessels separated from one another. Each type included subtype a, with a short isthmic portion, and subtype b, with a long isthmic portion. RESULTS: The median patient age and weight at operation were 8 (range, 1-1490) days and 3.2 (range, 1.9-18.5) kg, respectively. Extended end-to-end anastomosis was performed via sternotomy in 54, via thoracotomy in 12, end-to-side anastomosis in 31, autologous main pulmonary artery patch augmentation in 12, and modified end-to-end anastomosis combined with subclavian artery flap aortoplasty in two patients. There was one (0.9%) case of early mortality and 12 (10.8%) cases of post-operative complications. Two (1.8%) late deaths occurred during follow-up. Five (4.5%) patients underwent balloon dilatation and three (2.7%) underwent reoperation for restenosis of coarctation of the aorta. All patients with type Ia (21 patients, 18.9%) underwent extended end-to-end anastomosis via sternotomy or thoracotomy. CONCLUSIONS: According to the early and late outcomes observed in this study, surgical treatment of coarctation of the aorta using the new classification system could be safe and low risk.

Effectiveness of posterior aortopexy for the left pulmonary vein obstruction between the left atrium and the descending aorta.

BACKGROUND: Left pulmonary vein (PV) obstruction can occur due to compression between the left atrium (LA) and the descending aorta (DA). One of the effective solutions for this problem is posterior aortopexy. In this study, we have reported five cases of posterior aortopexy to relieve left PV obstruction between the LA and the DA. METHODS: Since August 2012, five patients have undergone posterior aortopexy for compression of the left PV between the LA and the DA. The median age and weight of the patients at the time of operation were 5.5 months (range, 1-131 months) and 5.2 kg (range, 4.2-29.5 kg), respectively. The left PV obstruction was initially diagnosed on echocardiography in four patients and computed tomography angiography in one patient. The median peak pressure gradient across the obstructed left PV was 7.3 mmHg (range, 4-20 mmHg). Concomitant procedures were ventricular septal defect closure in one patient and patent ductus arteriosus ligation in one patient. RESULTS: There was no PV obstruction on echocardiography in any of the patients after the operation except in the case of one patient who had diffuse pulmonary vein stenosis. The median follow-up duration was 34 months (range, 14-89 months), and during follow-up no incidence of the left PV obstruction was observed in any of the surviving patients. CONCLUSIONS: The posterior aortopexy technique could be a good surgical option for the left PV obstruction caused by compression between the LA and the anteriorly positioned DA.

A Case of Prenatally Diagnosed Uhl's Anomaly with Absent Pulmonary Valve Leaflets and Dysplastic Tricuspid Valve.

Uhl's anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood. Previously, diagnosis was made at post-mortem evaluation, but advances in cardiac imaging now permit diagnosis during fetal life. We report a case of Uhl's anomaly in a newborn baby imaged at 23 + 3 weeks of gestation by fetal echocardiography. There was an aneurysmally dilated thin-walled right ventricle with hypertrophy of the right ventricular apical muscles, the tricuspid valve was dysplastic, and the pulmonary valve leaflets were absent.

[Application of Three-Dimensional Printed Models in Congenital Heart Surgery: Surgeon's Perspective]. / ì„ ì²œì„± 심기형의 ìˆ˜ìˆ ì— 있어서 삼차원 í”„ë¦°íŒ ëª¨ë¸ì˜ ì ìš©: 심장외과의사의 ê´€ì .

To treat congenital heart disease, it is important to understand the anatomical structure correctly. Three-dimensional (3D) printed models of the heart effectively demonstrate the structural features of congenital heart disease. Occasionally, the exact characteristics of complex cardiac malformations are difficult to identify on conventional computed tomography, magnetic resonance imaging, and echocardiography, and the use of 3D printed models can help overcome their limitations. Recently, 3D printed models have been used for congenital heart disease education, preoperative simulation, and decision-making processes. In addition, we will pave the way for the development of this technology in the future and discuss various aspects of its use, such as the development of surgical techniques and training of cardiac surgeons.

Erratum: Application of Three Dimensional Printed Models in Congenital Heart Surgery: Surgeon's Perspective.

[This corrects the article on p. 310 in vol. 81.].

The coronary reimplantation after neoaortic reconstruction technique can make a difference in arterial switch operation.

BACKGROUND: The aim of this study was to determine if there was a difference between coronary reimplantation after neoaortic reconstruction and open coronary reimplantation technique in arterial switch operation (ASO). METHODS: A total of 236 patients who underwent ASO from March 1994 to August 2018 were enrolled in this study. Multivariate analysis was performed for postoperative early mortality. Patients were divided into the open coronary reimplantation and coronary reimplantation after neoaortic reconstruction groups. The 30-day mortality, intraoperative and postoperative coronary artery (CA) revisions, CA-related late morbidity and mortality, and early and late neoaortic valve regurgitations after ASO were compared between the two groups. RESULTS: Overall postoperative early mortality was 7.2% (17/236). Patients who underwent open coronary reimplantation had higher early mortality as compared with those who underwent coronary reimplantation after neoaortic reconstruction. Risk factors for postoperative early mortality from multivariate analysis were cardiopulmonary bypass time and open coronary reimplantation. There was a higher incidence of CA-related late mortality or morbidity in the open coronary reimplantation group. The open coronary reimplantation group had a higher incidence of intraoperative or postoperative CA revision. There were no differences in the incidence of mild or more neoaortic valve regurgitation at discharge or in the 5-year freedom from mild or more neoaortic valve regurgitation. CONCLUSIONS: CA reimplantation after neoaortic reconstruction yields better results in mortality and intraoperative or postoperative CA-related problems in ASO without increasing postoperative neoaortic valve regurgitation.

Risk Factors for Prolonged Pleural Effusion After Extracardiac Fontan Operation.

Prolonged pleural effusion after Fontan operation is a significant morbidity that leads to long hospital stays. We investigated the association of multiple risk factors, including clinical characteristics, hemodynamic parameters, and preoperative, operative, and postoperative factors, with prolonged pleural effusion after Fontan operation. Eighty-five patients who underwent a Fontan operation between January 2005 and June 2018 in our center were included in this retrospective study. Patients were divided into two groups: group 1 (n = 36, 42.4%) included those with prolonged pleural effusion, defined as lasting > 14 days after the Fontan operation, and group 2 included patients without prolonged pleural effusion. Patients with hypoplastic left heart syndrome (HLHS) were more prevalent in group 1 (n = 15, P = 0.006). No differences in age at Fontan operation, central venous pressure at Fontan operation, or hemodynamic parameters during the pre-Fontan evaluation were found between the two groups. In multivariable analysis, HLHS (P = 0.002), non-fenestration (P = 0.018), and high central venous pressure at bidirectional cavopulmonary shunt (BCPS) operation (P = 0.043) were independent risk factors for prolonged pleural effusion after Fontan operation. Adverse outcomes such as death, need for heart transplantation, and Fontan failure were not associated with prolonged pleural effusion. In conclusion, patients with HLHS and higher central venous pressure at BCPS were more likely to have a prolonged pleural effusion after Fontan operation, but fenestration was more likely to decrease prolonged effusion. We should consider closer management of fluid status before, during, and after surgery in patients with these risk factors after Fontan operation.

Simplified Tricuspid Polytetrafluoroethylene Valved Conduit: Midterm Results of Multicenter Study.

BACKGROUND: Conduit survival without significant dysfunction is important when selecting the right ventricular outflow tract conduit. We made an expanded polytetrafluoroethylene tricuspid valved conduit using a simplified technique. We aimed to investigate the midterm functional results and longevity of this conduit. METHODS: Between November 2008 and December 2016, four hospitals in Korea implanted 145 valved conduits. We retrospectively analyzed their functional results and longevity. RESULTS: The patients' median age at operation was 36.6 months; the median body weight was 11.3 kg. The mean follow-up duration was 32.3 ± 24.5 months. There were four inhospital deaths and three late deaths, but there were no conduit-related deaths. The mean peak systolic pressure gradient across the conduit was 14.7 ± 8.3 mm Hg and 31.6 ± 17.7 mm Hg at discharge and last follow-up, respectively. Six patients (4.4%) had moderate or more conduit valve regurgitation at last follow-up. Conduit dysfunction was observed in 30 patients (21.9%), mainly caused by increased pressure gradient (24 of 30, 80%). Freedom from conduit dysfunction was 88.1% and 58.5% at 3 and 5 years, respectively. Lower freedom from conduit dysfunction was observed in small conduits. Eleven patients (7.8%) underwent conduit explantation, and freedom from explantation was 94.8% and 81.7% at 3 and 5 years, respectively. The main cause of explantation was conduit stenosis. Small conduits tended to have lower freedom from explantation. CONCLUSIONS: Functional results and longevity of our expanded polytetrafluoroethylene tricuspid valved conduit are acceptable. Although our conduits tend to have increasing pressure gradient over time, especially in small conduits, they have low incidence of moderate or more regurgitation.

Three children of meningoencephalitis with Kikuchi necrotizing lymphadenitis.

BACKGROUND: Kikuchi necrotizing lymphadenitis (KNL) is a rare and benign cause of lymphadenopathy, most often cervical. The etiology of KNL remains unknown. Central nervous system (CNS) involvement, such as in meningoencephalitis, is a very rare clinical manifestation of KNL, especially in children. CASE REPORTS: A 12-year-old boy presented with unilateral cervical lymphadenopathy and fever. Histopathological findings led to the diagnosis of KNL. He revisited due to severe headache and vomiting one week later. Cerebrospinal fluid (CSF) analysis demonstrated pleocytosis (lymphocytic 57%), high protein (312 mg/dL) and low CSF/serum glucose ratio (52/121 mg/dL.) The next day, he had a seizure. Brain MRI revealed increased signal involving posterior area of both hemisphere. Another 17-year-old boy presented with headache for 7 days and behavioral and personality changes. He had a history of cervical lymphadenopathy two weeks ago. CSF analysis demonstrated lymphocytosis, high protein and low glucose ratio. MRI revealed the involvement of right cerebellum and posterior brainstem. A biopsy of one cervical lymphadenopathy demonstrated the findings of KNL. A 15-year-old girl presented with fever, headache, and cervical pain lasting 10 days. CSF analysis demonstrated pleocytosis (lymphocytic 95%), high protein and low CSF/serum glucose ratio. Histopathological findings of lymph node were suggestive of KNL. Above three patients were undertaken the steroid therapy and recovered fully without neurological dysfunction. CONCLUSIONS: Recognition of CNS involvement in KNL may help evaluate the patients of acute meningitis/encephalitis with regional lymphadenopathy, thereby avoiding unnecessary treatment.