Nationwide Birth Prevalence of Crucial Congenital Heart Defects From 2014 to 2018 in Korea.

BACKGROUND AND OBJECTIVES: A comprehensive survey of congenital heart disease (CHD) prevalence has not yet been conducted in South Korea. This study aimed to investigate the prevalence of CHDs in Korean children and lay the foundation for national CHD epidemiology. METHODS: Target patients were infantile crucial CHDs, which include critical CHDs (requiring urgent procedures after birth with common hypoxemic defects) and diverse categorical defects excluding simple shunt defects. Data were obtained from the National Health Insurance Service over a 5-year period (2014-2018). Birth prevalence (new cases per 1,000 live births) of CHDs in Korea was analyzed and compared with that of other countries. RESULTS: The birth prevalences of right heart obstructive defects (pulmonary valve stenosis and pulmonary atresia), conus anomalies (tetralogy of Fallot and double outlet right ventricle), and total anomalous pulmonary venous return showed significant increases in the East Asian group (P < 0.001), whereas those of left heart obstructive defects (coarctation of aorta, aortic stenosis, and hypoplastic left heart syndrome), truncus anomalies (D-transposition of great artery and persistent truncus arteriosus), atrioventricular septal defect, and hypoplastic right heart syndrome were significantly decreased in the East Asian group (P < 0.001). CONCLUSIONS: The overall birth prevalence of crucial CHDs in Korea was similar to that of critical CHDs in previous studies from other countries. Some subtypes of right heart obstructive defects, left heart obstructive defects, and conotruncal anomalies showed significant differences between East Asian and Western populations. This study contributes to a foundation for national CHD epidemiology in Korean children.

Risk factors for delayed hemothorax in patients with rib fracture in the emergency department.

OBJECTIVES: Although rib fractures are a risk factor, not all rib fracture patients will develop delayed hemothorax. This study aimed to evaluate risk factors which can identify rib fracture patients in the emergency department who may develop delayed hemothorax. METHODS: Adult patients seen in the emergency room between January 2016 and February 2021 with rib fractures caused by blunt chest trauma were included in this retrospective observational study. Patients who underwent chest tube insertion within 2 days and those without follow-up chest radiographs within 2-30 days were excluded. We used a stepwise backward-elimination multivariable logistic regression model for analysis. RESULTS: A total of 202 patients were included in this study. The number of total (P < 0.001), lateral (P = 0.019), and displaced (P < 0.001) rib fractures were significantly associated with delayed hemothorax. Lung contusions (P = 0.002), and initial minimal hemothorax (P < 0.001) and pneumothorax (P < 0.001) were more frequently associated with delayed hemothorax. Age (adjusted odds ratio (aOR) 1.03, 95% confidence interval (CI) 1.00-1.06, P = 0.022), mechanical ventilator use (aOR 9.67, 95% CI 1.01-92.75, P = 0.049), initial hemothorax (aOR 2.21, 95% CI 1.05-4.65, P = 0.037) and pneumothorax (aOR 2.99, 95% CI 1.36-6.54, P = 0.006), and displaced rib fractures (aOR 3.51, 95% CI 1.64-7.53, P = 0.001) were independently associated with delayed hemothorax. CONCLUSIONS: Age, mechanical ventilation, initial hemo- or pneumothorax, and displaced rib fractures were risk factors for delayed hemothorax. Patients with these risk factors, and especially those with ≥2 displaced rib fractures, require close chest radiography follow-up of 2-30 days after the initial trauma.

Entelon150® (Vitis vinifera Seed Extract) Attenuates Degenerative Changes in Intravascular Valve Prostheses in Rabbits.

BACKGROUND AND OBJECTIVES: The therapeutic strategy for inflammation and degenerative calcification is of utmost importance for bioprosthetic heart valve (BHV) implanted patients. The purpose of this study was to compare the anti-inflammatory and anti-calcification effects of Entelon150® (grape seed extract), losartan, and rosuvastatin, in a rabbit model of intravascular BHV leaflet implantation in bovine pericardium. METHODS: A total of 28 rabbits were implanted with BHV leaflet in the external jugular veins. The Entelon150® group was administered 7.7 mg/kg Entelon150® twice daily for 6 weeks after surgery. The losartan and rosuvastatin groups received 5.14 mg/kg and 1 mg/kg, respectively, once per day. The control group received 1 ml of saline once daily. And then, calcium concentration was measured in the implanted BHV, and histological and molecular analyses were performed on the surrounding tissues. RESULTS: The calcium content of the implanted tissue in the Entelon150® group (0.013±0.004 mg/g) was lower than that in the control group (0.066±0.039 mg/g) (p=0.008). The losartan (0.024±0.016 mg/g, p=0.032) and rosuvastatin (0.022±0.011 mg/g, p=0.032) groups had lower calcium content than the control group, and higher tendency than the Entelon150® group. Immunohistochemistry revealed that the expressions of bone morphogenic protein 2 (BMP2), S-100, and angiotensin II type 1 receptor in the Entelon150® group showed lower tendency than those in the control group. The protein expression levels of BMP2 were reduced in the Entelon150® group compared with those in the control group. CONCLUSIONS: Entelon150® exhibited a significant effect, similar to other drugs, in reducing calcification and inflammation in the intravascular bovine pericardium.

Incidence and risk of stroke in Korean patients with congenital heart disease.

OBJECTIVES: The incidence and risk of ischemic stroke (IS) and hemorrhagic stroke (HS) in Korean patients with CHD have not been reported, therefore, we aimed to investigate this. MATERIALS AND METHODS: Participants were selected from the Korean National Health Insurance Service benefit records from 2006-2017. Cases were extracted using diagnosis codes related to CHD. Controls without CHD were selected through age- and sex-matched random sampling at a 1:10 ratio. RESULTS: The case and control groups included 232,203 and 3,024,633 participants, respectively. The median (interquartile range) follow-up period was 7.28 (3.59-8.73) years. The incidence rates of IS and HS per 100,000 person-years were much higher in cases than in controls (IS: 135 vs. 47; HS: 41.7 vs. 24.9). After adjusting for confounders, CHD was a risk factor for IS and HS (subdistribution HR; 1.96 and 1.71, respectively). In patients with CHD, the following risk factors were identified: diabetes, heart failure, and atrial fibrillation for any stroke; hypertension, atrial septal defects, and use of antiplatelet agents for IS only; and coronary artery bypass graft surgery for HS only. CONCLUSIONS: Korean patients with CHD have a high risk of stroke. A personalized preventive approach is needed to reduce the incidence of stroke in this population.

Prognosis of Unrepaired Ascending Aorta after the Surgical Replacement of Bicuspid Aortic Valves.

Background: The surgical threshold for bicuspid aortic valve (BAV)-related aortopathy is a matter of debate due to its uncertain etiology and prognosis. This study investigated the prognosis of unrepaired BAV aortopathy in patients undergoing surgical aortic valve replacement (SAVR). Methods: We retrospectively analyzed data from 720 patients (age, 60.8±11.5 years; 246 women) who underwent SAVR for BAV disease without aortic repair between 2005 and 2020 at Asan Medical Center. The clinical endpoints were defined as occurrences of sudden death, aortic dissection or rupture, and elective aortic repair. To estimate postoperative changes in the dimensions of the unrepaired aorta, the individual annual aortic expansion rate was calculated. Multiple linear regression models were used to evaluate the risk of aortic expansion. Results: The mean ascending aortic diameter was 39.5±4.6 mm, and 299 patients (41.5%) had a baseline ascending aorta diameter >40 mm. During 70.0±68.3 months of follow-up, the mean annual aortic expansion rate was 0.39±1.96 mm/yr, no aortic dissection or rupture was observed, and sudden deaths were reported in 12 patients (0.34% per person-year). Linear regression analysis revealed no significant correlation between the baseline ascending aortic diameter and postoperative aortic expansion (R2=0.004, ß=-0.84, p=0.082). Conclusion: In selected patients undergoing SAVR for a BAV (<55 mm), the risk of adverse aortic events was very low. As this observation contradicts current practice guidelines advocating for proactive aortic replacement in dilated ascending aortas measuring >45 mm, the study results need further validation by studies involving larger populations or randomized controlled trials.

Major Adverse Cardiovascular Events in Korean Congenital Heart Disease Patients: A Nationwide Age- and Sex-Matched Case-Control Study.

PURPOSE: Congenital heart disease (CHD) is a known risk factor for acquired cardiovascular and cerebrovascular diseases. However, available evidence on CHD is limited mostly to Western populations. This study aimed to evaluate the prevalence of vascular events and all-cause mortality in Korean patients with CHD and to further corroborate CHD as a predictor of vascular events and all-cause mortality. MATERIALS AND METHODS: The claims data of the Korean National Health Insurance Service (NHIS) were retrospectively reviewed. Information regarding diagnostic codes, comorbidities, medical services, income level, and residential area was also collected. Outcomes of interest included stroke, myocardial infarction (MI), all-cause mortality, and major adverse cardiovascular events (MACE). RESULTS: We included 232203 patients with CHD and 3024633 individuals without CHD as a control group through age- and sex-matched 1:10 random sampling. The prevalences of hypertension, congestive heart failure, ischemic heart disease, hyperlipidemia, and atrial fibrillation were significantly higher in the CHD group, which had a more than two-fold higher incidence of vascular events and all-cause mortality, than in the group without CHD. Multivariable models demonstrated that CHD was a significant risk factor for stroke, MI, all-cause mortality, and MACE. CONCLUSION: In conclusion, this nationwide study demonstrates that Korean patients with CHD have a high incidence of comorbidities, vascular events, and mortality. CHD has been established as an important predictor of cardiovascular events. Further studies are warranted to identify high-risk patients with CHD and related factors to prevent vascular events.

Left ventricular rehabilitation in an infant with transposition of the great arteries, intact ventricular septum, pulmonary stenosis and small left ventricle.

We report the case of successful biventricular repair after left ventricular rehabilitation in an infant with transposition of the great arteries with an intact ventricular septum, pulmonary stenosis, a large atrial septal defect and a borderline small left ventricle (mitral annulus z-score: -3.6). This baby presented to us at 2 months of age after having a modified Blalock-Taussig shunt at another hospital. We restricted the atrial septal defect with the child on cardiopulmonary bypass. Ten weeks later, the mitral annulus z-score increased to -1.5, and the transpulmonary peak pressure gradient increased to 87 mmHg. Subsequently, we performed the aortic root translocation. The patient is currently an active 4-year-old boy.

Satisfactory outcome with activated clotting time <160 seconds in extracorporeal cardiopulmonary resuscitation.

Patients undergoing cardiopulmonary resuscitation (CPR) prior to extracorporeal membrane oxygenation (ECMO) can have severely altered physiology, including that of the coagulation pathway. This could complicate the extracorporeal cardiopulmonary resuscitation (ECPR) management. We aimed to show that targeting an activated clotting time (ACT) < 160 seconds does not affect the complication rates in these patients. In this single-centered retrospective study, the medical records of 81 adult patients who were on ECMO support from March 2017 to March 2020 were reviewed. We compared the low ACT and conventional ACT groups, which were defined on the basis of the median of the ACT values of the included patients (160 seconds). The primary outcomes included bleeding or thromboembolic events. This study included 32 patients, who were divided into the low (n = 14) and conventional (n = 18) ACT groups. There were 2 cases of gastrointestinal bleeding (P = .183), one of intracranial hemorrhage (P = .437), and one of peripheral skin color change (P = .437) in the low ACT group. There was one case of prolonged bleeding at the cannulation site (P = 1.000) reported in the conventional ACT group. The successful weaning rate differed significantly between the low and conventional ACT groups (92.9% vs 50.0%; P = .019). Maintaining the ACT lower than the conventional ACT in patients requiring ECPR did not show a significant increase in the thromboembolic risk. Therefore, targeting a low ACT should be considered for this particular group of patients.

Midterm results of arch augmentation with autologous vascular patch in interrupted aortic arch.

OBJECTIVES: Aortic arch reconstruction of interrupted aortic arch remains challenging, and subsequent problems, including arch and airway stenosis, may occur. Thus, we investigated midterm results of an augmentation technique using autologous vascular patch. METHODS: This retrospective study included 24 patients who underwent arch reconstruction with an autologous vascular patch for interrupted aortic arch with biventricular physiology from 2006 to 2018. The median age and body weight at operation were 10 days (range 4-77 days) and 3 kg (range 2.5-5.1 kg), respectively. The reconstructed arch was supplemented in the lesser curvature with an autologous vascular patch that was harvested from main pulmonary artery (n = 19), left subclavian artery (n = 3) or aberrant right subclavian artery (n = 1). One patient used patches from both the main pulmonary and left subclavian artery. RESULTS: There was 1 early death due to right heart failure. All survivors were discharged 15 days (range 9-58 days) after surgery without residual arch stenosis. Late death occurred in 1 patient with Cri-du-chat syndrome and airway stenosis. Two reoperations and 1 intervention for arch stenosis were performed. The 1-, 5- and 10-year survival was 92%. Freedom from reoperation or intervention for arch stenosis was 86% 1, 5 and 10 years after surgery. No occurrence of arch aneurysm formation, left main bronchial stenosis and significant hypertension was found during a median follow-up period of 5.5 years (range 0.3-13.3 years). CONCLUSIONS: Augmenting the lesser curvature with an autologous vascular patch during arch reconstruction resulted in reasonable midterm outcomes.

Pericardial Versus Porcine Valves for Surgical Aortic Valve Replacement.

BACKGROUND AND OBJECTIVES: There still are controversies on which type between bovine pericardial and porcine valves is superior in the setting of aortic valve replacement (AVR). This study aims to compare clinical outcomes of AVR using between pericardial or porcine valves. METHODS: The study involved consecutive 636 patients underwent isolated AVR using stented bioprosthetic valves between January 2000 and May 2016. Of these, pericardial and porcine valves were implanted in 410 (pericardial group) and 226 patients (porcine group), respectively. Clinical outcomes including survival, structural valve deterioration (SVD) and trans-valvular pressure gradient were compared between the groups. To adjust for potential selection bias, inverse probability treatment weighting (IPTW) was conducted. RESULTS: The mean follow-up duration was 60.1±50.2 months. There were no significant differences in the rates of early mortality (3.1% vs. 3.1%; p=0.81) and SVD (0.3%/patient-year [PY] vs. 0.5%/PY; p=0.33) between groups. After adjustment using IPTW, however, landmark mortality analyses showed a significantly lower late (>8 years) mortality risk in pericardial group over porcine group (hazard ratio [HR], 0.61; 95% confidence interval, [CI] 0.41-0.90; p=0.01) while the risks of SVD were not significantly difference between groups (HR, 0.45; 95% CI, 0.12-1.70; p=0.24). Mean pressure gradient across prosthetic AV was lower in the Pericardial group than the Porcine group at both immediate postoperative point and latest follow-up (p values <0.001). CONCLUSIONS: In patients undergoing bioprosthetic surgical AVR, bovine pericardial valves showed superior results in terms of postoperative hemodynamic profiles and late survival rates over porcine valves.

Aortic Valve Repair in Young Patients: A Single Patch Design for Leaflet Extension.

The surgical treatment for young patients with aortic valve diseases has not been standardized because of differences in the patients' growth. We aimed to investigate short-term results of aortic valve repair with leaflet extension techniques. From October 2017 to June 2020, we performed aortic valve repair with leaflet extension technique in 15 consecutive patients with a median age of 13 years (range, 33 months-27 years). Among them, all had moderate or severe aortic regurgitation (AR), eight had concomitant aortic stenosis, and 12 underwent prior cardiac operations. The leaflet patch design has been modified from separate patch design to single patch design. No early and late deaths were reported, no re-operations were performed, and temporary ectopic atrial arrhythmia was the only early complication noted. The patients were discharged with less than moderate AR after a median postoperative period of 5 (range, 3-7) days. All the patients were New York Heart Association class I after a median follow-up period of 17.3 (range, 4.4-34.6) months. However, two patients progressed to moderate AR postoperatively at 6 and 30 months, respectively, after which one was treated with single patch technique. Among the remaining patients, six had trace, six had mild, and one had mild-to-moderate AR. Aortic valve repair with leaflet extension is acceptable in young patients. Our single patch design was easy to manipulate and showed good short-term results. Long-term follow-up is required to further confirm the efficacy of this technique.

Satisfactory outcome with low activated clotting time in extracorporeal membrane oxygenation.

Optimal anticoagulation is critical for successful extracorporeal membrane oxygenation (ECMO) to counterbalance the activation of the coagulation system initiated by the blood-biosurface reaction and mechanical stresses. Systemic anticoagulation is achieved mainly with unfractionated heparin (UFH). Activated clotting time (ACT) is a widely used laboratory parameter to monitor anticoagulation. The therapeutic range of ACT is 180-220 s. We investigated the effect of a lower target ACT (<150 s) during ECMO on safety and outcomes and compared it with those of a conventional target ACT (180-200 s). In this single-center, retrospective study, we reviewed 72 adult patients treated with ECMO from March 2017 to October 2019. We included 43 patients after applying the exclusion criteria and divided them into the low ACT group (<150 s, n = 14, 32.6%) and conventional ACT group (≥150 s, n = 29, 67.4%). There was no difference in the successful weaning from ECMO support (50% vs. 62.1%, p = 0.452) and discharge (50% vs. 41.4%, p = 0.594) rates between the groups. One patient in the conventional ACT group had intracranial hemorrhage. There was one thromboembolic complication case with an intra-circuit thrombus. To date, anticoagulation remains a challenge during ECMO. Our results suggest that a lower target ACT does not necessarily increase the thromboembolic risk during ECMO management. Clinicians may consider anticoagulation with lower ACT target for some patients with careful assessment and close monitoring. Further prospective trials are warranted to validate these results.

Effect of Pulmonary Valve Replacement in the Repaired Tetralogy of Fallot Patients with Trans-annular Incision: More than 20 Years of Follow-up.

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 follow-up, 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.

Early enteral nutrition and factors related to in-hospital mortality in people on extracorporeal membrane oxygenation.

OBJECTIVES: There are concerns about adverse events related to early enteral nutrition (EN) in people receiving extracorporeal membrane oxygenation (ECMO). This was a retrospective study evaluating. This nutritional support of people receiving ECMO, factors that may confer benefits in outcomes. METHODS: 60 adults on ECMO who survived for more than 48 h were enrolled in the study. We evaluated energy and protein intake and the associations of the timing, adequacy, and route of nutrition with in-hospital mortality. RESULTS: Thirty-three participants (55%) were successfully weaned off ECMO, and 30 (50%) survived. EN was initiated on day 2 of ECMO (interquartile range, 1-3), and the mean energy intake on day 7 of ECMO was 94.1% ± 41.8% of the energy requirement. Although early EN significantly decreased in-hospital mortality (hazard ratio, 0.413; 95% confidence interval, 0.174-0.984; P = 0.046), neither adequate energy intake (hazard ratio, 0.982; 95% confidence interval, 0.292-3.301; P = 0.977) nor EN-dominant nutritional support (hazard ratio, 0.394; 95% confidence interval, 0.138-1.128; P = 0.083) in the first week influenced survival. CONCLUSIONS: Although adequate nutritional support and EN-dominant nutritional support were not associated with changes in outcome, early EN was associated with reduced in-hospital mortality. Therefore, even when EN is not the dominant route of nutritional support, early EN may be recommended for better outcomes in people on ECMO.

Entelon (vitis vinifera seed extract) reduces degenerative changes in bovine pericardium valve leaflet in a dog intravascular implant model.

BACKGROUND AND AIMS: Inflammation and calcification are major factors responsible for degeneration of bioprosthetic valve and other substitute heart valve implantations. The objective of this study was to evaluate the anti-inflammatory and anti-calcification effects of Entelon150® (consisting of grape-seed extract) in a beagle dog model of intravascular bovine pericardium implantation. METHODS: In total, 8 healthy male beagle dogs were implanted with a bovine pericardium bilaterally in the external jugular veins and divided into two groups. Animals in the Entelon150® group (n = 4) were treated with 150 mg of Entelon150® twice daily for six weeks after surgery. The negative control (NC) group (n = 4) was treated with 5 ml of saline using the same method. After six weeks, we measured the calcium content, performed histological examination, and performed molecular analysis. RESULTS: The calcium content of implanted tissue in the Entelon150® group (0.56±0.14 mg/g) was significantly lower than that in the NC group (1.48±0.57 mg/g) (p < 0.05). Histopathological examination showed that infiltration of chronic inflammatory cells, such as fibroblasts and macrophages, occurred around the graft in all groups; however, the inflammation level of the implanted tissue in the Entelon150® group was s lower than that in the NC group. Both immunohistochemical and western blot analyses revealed that bone morphogenetic protein 2 expression was significantly attenuated in the Entelon150® group. CONCLUSIONS: Our results indicate that Entelon150® significantly attenuates post-implantation inflammation and degenerative calcification of the bovine pericardium in dogs. Therefore, Entelon150® may increase the longevity of the bovine pericardium after intravascular implantation.

Thoracomyoplasty for Chronic Empyema and Osteoradionecrosis of the Chest Wall

Herein, we report a case in which thoracomyoplasty was performed to manage chronic postlobectomy empyema (PLE). A 54-year-old male patient with a surgical history of right upper lobectomy and thymectomy 35 years previously who had undergone adjuvant radiotherapy presented with purulent discharge on the anterior chest wall. The patient was diagnosed with chronic PLE with ascending infection and concurrent osteonecrosis of the parasternum. Proper drainage was performed for local infection control and the dead spaces were successfully closed with muscle flaps. There have been no complications to date.

The Risk Prediction of Coronary Artery Lesions through the Novel Hematological Z-Values in 4 Chronological Age Subgroups of Kawasaki Disease.

Background and Objectives: Most cases of Kawasaki disease (KD) occur between the ages of 6 months and 5 years. Differences in immunological reaction and CAL (coronary artery lesion) by the age subgroups classified according to the prevalence of KD and those particularly in the earlier life of KD should be investigated. Materials and Methods: The laboratory data of 223 infantile and 681 non-infantile KD cases from 2003 to 2018 at Korea University Hospital were retrospectively analyzed. Patients with KD were divided into infants and non-infants and further subdivided into four subgroups by age. The age-adjusted Z-values were compared among the subgroups. Febrile controls were identified as patients with fever for >5 days and who showed some of the KD symptoms. Results: IVIG (intravenous immunoglobulin) resistance at the age of 6 months or less was significantly lower than that at the ages of 7-12 months and 13-60 months (respectively, p < 0.05). The significant risk factors for CAL in total KD patients were age, incomplete KD, post-IVIG fever, IVIG resistance, convalescent Z-eosinophil, and subacute platelet (p < 0.05). The significant risk factors for CAL at the age of 6 months or less were IVIG resistance, acute Z-neutrophil, subacute Z-neutrophil, subacute NLR (neutrophil to lymphocyte ratio), and subacute platelet (respectively, p < 0.05). Conclusion: Younger age and incomplete presentation in KD can be independent risk factors for CAL. The immune reactions of KD at a younger age are more tolerated compared with those at older ages during the acute phase. The immune response at the age of 6 months or less showed immune tolerance in terms of incomplete presentation and IVIG responsiveness. The risk factors such as IVIG resistance, subacute platelet, subacute NLR, and acute or subacute Z-neutrophil at the age of 6 months or less can be very useful parameters to predict CAL in young, incomplete KD.

Left Hemitruncus Treated Along with Ventricular Septal Defect in a Neonate.

Hemitruncus arteriosus is a rare cardiovascular malformation in which one of the pulmonary arteries anomalously originates from the aorta. Left hemitruncus arteriosus, defined as the origination of the left pulmonary artery from the aorta, is less common than right hemitruncus arteriosus. In this study, we report the case of a neonate diagnosed with left hemitruncus arteriosus, ventricular septal defect, and atrial septal defect who underwent successful surgical treatment.

Aortic Valve-Sparing Surgical Treatment of Supravalvar Aortic Stenosis in a 65-Year-Old Adult.

Supravalvar aortic stenosis (SVAS) is a rare congenital cardiac disease that usually co-occurs with Williams syndrome. In the adult population, a few SVAS cases have been reported in patients affected by homozygous familial hypercholesterolemia. However, because of the rarity of this disease entity, there is no standard surgical treatment for SVAS. Here, we present a case of successful surgical treatment using an autologous excised aortic patch in a 65-year-old patient with SVAS.