The adaptability of the Pulsta valve to the diverse main pulmonary artery shape of native right ventricular outflow tract disease.

BACKGROUND: Pulsta valve is increasingly used for percutaneous pulmonary valve implantation (PPVI) in patients with a large native right ventricular outflow tract (RVOT). This study aims to elucidate the outcomes of Pulsta valve implantation within the native RVOT and assess its adaptability to various native main pulmonary artery (PA) anatomies. METHODS: A multicenter retrospective study included 182 patients with moderate to severe pulmonary regurgitation in the native RVOT who underwent PPVI with Pulsta valves® between February 2016 and August 2023 at five Korean and Taiwanese tertiary referral centers. RESULTS: Pulsta valve implantation was successful in 179 out of 182 patients (98.4%) with an average age of 26.7 ± 11.0 years. The median follow-up duration was 29 months. Baseline assessments revealed enlarged right ventricle (RV) volume (mean indexed RV end-diastolic volume: 163.1 (interquartile range, IQR: 152.0-180.3 mL/m²), which significantly decreased to 123.6(IQR: 106.6-137.5 mL/m2  after 1 year. The main PA types were classified as pyramidal (3.8%), straight (38.5%), reverse pyramidal (13.2%), convex (26.4%), and concave (18.1%) shapes. Pulsta valve placement was adapted, with distal main PA for pyramidal shapes and proximal or mid-PA for reverse pyramidal shapes. Two patients experienced Pulsta valve embolization to RV, requiring surgical removal, and one patient encountered valve migration to the distal main PA, necessitating surgical fixation. CONCLUSIONS: Customized valve insertion sites are pivotal in self-expandable PPVI considering diverse native RVOT shape. The rather soft and compact structure of the Pulsta valve has characteristics to are adaptable to diverse native RVOT geometries.

Long-term Outcome of Fontan-Associated Protein-Losing Enteropathy: Treatment Modality and Predictive Factor of Mortality.

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 11.6 years. 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.

Mid-term outcomes of the Pulsta transcatheter pulmonary valve for the native right ventricular outflow tract.

OBJECTIVES: The aim of this study is to present the mid-term outcomes of Pulsta valve. BACKGROUND: The Pulsta valve is a Self-expandable knitted nitinol-wire stent mounted with a treated tri-leaflet α-Gal-free porcine pericardial valve for percutaneous pulmonary valve implantation (PPVI) in patients with native right ventricular outflow tract (RVOT) lesions. METHODS: A multi-center clinical trial using Pulsta valve® was designed for patients with severe pulmonary regurgitation (PR) in the native RVOT in multiple centers in South Korea and 25 patients were enrolled. Before PPVI, severe PR (mean PR fraction: 45.5 ± 6.9%) and enlarged RV volume (mean indexed RV end-diastolic volume; 169.7 ± 13.0 ml/m2 ) was present. The mean age was 21.6 ± 6.6 years old. RESULTS: All patients were successfully implanted with 26, 28, or 32 mm diameter of Pulsta valve loaded on the 18 or 20 French delivery catheters. At 6 months follow up, indexed RV end-diastolic volume was decreased to 126.9 ± 16.9 ml/m2 . At mean 33.1 ± 14.3 months follow-up, the mean value of mean pressure gradient in Pulsta valve was 6.5 ± 3.0 mmhg without significant PR. There was no serious device-related adverse event. CONCLUSIONS: A multi-center clinical trial was completed successfully with planned Pulsta valve implantation and demonstrated good mid-term effectiveness without device-related serious adverse events.

Bilateral branch pulmonary artery Pulsta valve implantation for treatment of large right ventricular outflow tract in a high-risk patient.

Percutaneous pulmonary valve implantation (PPVI) has been implemented as a novel alternative strategy to surgical pulmonary valve replacement. However, PPVI has an inevitable limitation: the large right ventricular outflow tract (RVOT) lesions exhibit variable geometry and significant pulmonary regurgitation (PR). To overcome this limitation, bilateral branch pulmonary artery (PA) valve implantations using Melody or Sapien valves have been attempted and have shown a reduction in right ventricular volume with clinical benefits in the intermediate term. Nevertheless, these trials also have constraints of large branch PA size. Recently, a feasibility study using the Pulsta valve (Tae Woong Medical Co, Gyeonggi-do, South Korea) for native RVOTs was reported; the diameter of the Pulsta valve ranges from 18 to 32 mm. Herein, we present a successful percutaneous bilateral branch PA valve implantation using two 32 mm Pulsta valves in a 59-year-old man who showed right heart failure with severe pulmonary regurgitation despite several open heart surgeries for tetralogy of Fallot. The main PA was measured to be 49 mm, and both the right and left PAs were measured to be 30 mm.

Correlation Between Total Repair Timing and Late Aortic Root Dilatation in Repaired Tetralogy of Fallot.

Aortic root dilatation is frequently encountered in adults with repaired Tetralogy of Fallot (TOF). The timing of total repair is known to have a significant correlation with late aortic root dilatation, but it is not well established. The objective of this study was to investigate the risk factors and correlation with timing of total repair for aortic root dilatation in adults with repaired TOF. An observational retrospective cohort study was conducted in 314 adults (≥ 18 years) with repaired TOF. Aortic root dilatation was defined when the absolute diameter of Sinus of Valsalva (SoV) was over 40 mm. SoV was measured by echocardiography in 110 patients, computed tomography angiography in 168 patients, and magnetic resonance image in 36 patients. Aortic root dilatation was observed in 65 patients (20.7%). Among them, 3 patients underwent Bentall operation due to acute aortic dissection or aortic root aneurysm. On multivariate logistic regression analysis, age at total repair was a significant risk factor for late aortic root dilatation (OR = 3.14; 95% CI 1.62-6.08; p = 0.001) and the cutoff value of age was 1.9 years. However, late aortic root dilatation was also observed in 10% of patients (10/91) who operated before 1 year of age. Late total repair was a significant risk factor for late aortic root dilatation in repaired TOF. However, early total repair did not always prevent late aortic root dilatation. These results suggest that congenital aortic pathology may also be a possible cause of aortic root dilatation along with longstanding hemodynamic stress.

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single-secondary center experience.

BACKGROUND AND AIM OF THE STUDY: Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect. This study aimed to evaluate the outcome of isolated TAPVC repairs and the prognoses of affected patients in the last 12 years at a single center. METHODS: We retrospectively analyzed the medical records of 51 patients who underwent isolated TAPVC repair from 2007 to 2018. RESULTS: The median age at operation was 19 days, and the median body weight was 3.3 kg. Thirteen (25.5%) patients had emergency operations, and the median follow-up period was 29.54 ± 36.77 months. Early mortality was noted in five patients and late mortality was noted in one patient. Pulmonary vein stenosis was observed in 22 patients within 3 to 6 months after the operation, and six patients required reoperation or transcatheter interventions. Low birth weight, small left atrial volume, long operation time, and preoperative heart failure were identified as risk factors for mortality. CONCLUSIONS: Isolated TAPVC can rapidly lead to hemodynamic instability during the neonatal period and is associated with high mortality rates. Increasing the prenatal diagnosis rate and stabilizing the patients' condition before the operation are considered important for improving the surgical outcome.

Relation Between Exercise Capacity and Extracardiac Conduit Size in Patients with Fontan Circulation.

Because Fontan circulation does not have a subpulmonary ventricle, the preload is limited. In Fontan circulation with extracardiac conduit, the size of conduit could be an important factor in determining the preload. We compared exercise capacity with each conduit size and tried to search for optimal conduit size in Fontan circulation. We reviewed the medical record of 677 patients with Fontan circulation. Patients who had other type Fontan circulation (Kawashima, atriopulmonary, lateral tunnel), SpO2 < 85%, protein losing enteropathy, results of inappropriate exercise test were excluded. As a result, 150 patients were enrolled and classified according to conduit size. We compared with their exercise capacity and analyzed correlation between exercise capacity and conduit size per body surface area (BSA). 97 Males were included and mean age was 17.5 ± 5.1 years old. In cardiac catheterization, central venous pressure (CVP) was 12.4 ± 2.5 mmHg and pulmonary vascular resistance was 1.2 ± 0.5 wu m2. In cardiopulmonary exercise test, predictive peak VO2 was 59.1 ± 9.7% and VE/VCO2 was 36.2 ± 6.9. In analysis using quadratic model, impacts of gender, age at Fontan operation, ventricular morphology, isomerism, and fenestration on exercise capacity were excluded and conduit size per BSA had a significant curved correlation with predictive peak VO2 and VE/VCO2. Our results showed that patients with about 12.5 mm/m2 conduit per BSA have the best exercise capacity. Patients with larger than smaller-sized conduit were found to be more attenuated in their ability to exercise.

The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute.

PURPOSE: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. METHODS: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1-4 years). Medical records were reviewed retrospectively. RESULTS: The minimum lumen diameter (MLD) in PAS and CoA increased from 5.0±1.9 mm and 8.4±1.6 mm to 10.1±3.6 mm and 12.3±2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7±15.6 mmHg to 10.4±10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56±0.21 to 0.46±0.19. In the CoA group, the pressure gradient decreased from 50±33 mmHg to 17±8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%-70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29±12 months. CONCLUSION: Percutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.

Hepatic Stiffness Using Shear Wave Elastography and the Related Factors for a Fontan Circulation.

Hepatic problems related to a Fontan circulation have been highlighted and elastography using ultrasound is a non-invasive tool that can measure the severity of hepatic stiffness. We investigated the hepatic stiffness using shear wave elastography (SWE) and related factors in patients with a Fontan circulation. This study enrolled 64 patients with a Fontan circulation who underwent cardiac catheterization and abdominal ultrasound from 2011 to 2015. The correlation between the laboratory tests, hemodynamic factors by cardiac catheterization, and SWE was evaluated. The patients were classified into non-cirrhotic level (≥ 2.0 m/s) and cirrhotic level (< 2.0 m/s) groups by the SWE value. The mean age was 17.6 years and the mean duration after the Fontan operation was 12.1 years. The mean value of SWE in patients (1.95 m/s) was higher than the normal (< 1.3 m/s). The SWE was higher in patients without than those with a fenestration (2.03 vs. 1.75 m/s, P = 0.003). In a multiple regression analysis between SWE and other factors, the CVP, fenestration, and lipoprotein Apo B had a significant correlation. In a multivariate analysis of cirrhotic level group, the CVP was the only significant factor. The hepatic stiffness had significantly progressed in most patients with a Fontan circulation. A low CVP and Fontan circulation with a fenestration might reduce the progression of the hepatic stiffness.

Short-Term Change of Exercise Capacity in Patients with Pulmonary Valve Replacement after Tetralogy of Fallot Repair.

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate the effect of pulmonary valve replacement (PVR) on exercise capacity and determine cardiopulmonary exercise (CPEX) parameters associated with improvement in right ventricle (RV) function. SUBJECTS AND METHODS: We retrospectively analyzed CPEX and magnetic resonance imaging parameters in a total of 245 patients who underwent PVR from January 1998 to October 2015. In addition, we analyzed the characteristics of the patients who showed improved exercise capacity after PVR. RESULTS: Twenty-eight patients met the inclusion criteria for the study. CPEX parameters after PVR showed no significant changes in all patients. However, baseline predicted peak oxygen uptake (VO2peak) (%) value was significantly lower in patients with significant improvement in exercise capacity after PVR, as compared to patients who showed decreased exercise capacity after PVR (60.83±10.28 vs. 75.81±13.83) (p=0.003). In addition, patients with improved exercise capacity showed a positive correlation between the change of right ventricular ejection fraction (RVEF) (%) and the change of anaerobic threshold (r=0.733, p=0.007); whereas, patients with decreased exercise capacity showed a negative correlation between the change of RVEF (%) and the change of predicted VO2peak (%) (r=-0.575, p=0.020). CONCLUSION: The importance of predicted VO2peak (%) in evaluating exercise capacity differentiated from other CPEX variables. The change of anaerobic threshold and predicted VO2peak (%) might be a useful predictor of the change in RV function after PVR.

Impact of postoperative duration of Aspirin use on longevity of bioprosthetic pulmonary valve in patients who underwent congenital heart disease repair.

PURPOSE: Generally, aspirin is used as a protective agent against thrombogenic phenomenon after pulmonary valve replacement (PVR) using a bioprosthetic valve. However, the appropriate duration of aspirin use is unclear. We analyzed the impact of postoperative duration of aspirin use on the longevity of bioprosthetic pulmonary valves in patients who underwent repair for congenital heart diseases. METHODS: We retrospectively reviewed the clinical data of 137 patients who underwent PVR using a bioprosthetic valve between January 2000 and December 2003. Among these patients, 89 were included in our study and divided into groups I (≤12 months) and II (>12 months) according to duration of aspirin use. We analyzed echocardiographic data from 9 to 11 years after PVR. Pulmonary vale stenosis and regurgitation were classified as mild, moderate, or severe. RESULTS: The 89 patients consisted of 53 males and 36 females. Their mean age was 14.3±8.9 years (range, 2.6-48 years) and body weight was 37.6±14.7 kg (range, 14-72 kg). The postoperative duration of aspirin use was 7.3±2.9 months in group I and 32.8±28.4 months in group II. However, no significant difference in sex ratio, age, body weight, type of bioprosthetic valve, and number of early redo-PVRs. In the comparison of echocardiographic data about 10 years later, no significant difference in pulmonary valve function was found. The overall freedom rate from redo-PVR at 10 years showed no significant difference (P=0.498). CONCLUSION: Our results indicated no benefit from long-term aspirin medication (>6 months) in patients who underwent PVR with a bioprosthetic valve.

Does implantation of larger bioprosthetic pulmonary valves in young patients guarantee durability in adults? Durability analysis of stented bioprosthetic valves in the pulmonary position in patients with Tetralogy of Fallot†.

OBJECTIVES: In a previous study, we identified factors affecting the durability of bioprosthetic valves in the pulmonary position following total repair of Tetralogy of Fallot (TOF). In this study, we aimed to identify factors affecting the durability of the bioprosthetic valve with regard to patient age and implanted valve size in order to guide valve choice in adolescent patients. METHODS: We enrolled and analysed 108 cases of pulmonary valve replacement (PVR) with stented bioprosthetic valves in TOF patients between January 1998 and February 2014. Valvular dysfunction was defined as at least a moderate amount of pulmonary regurgitation or a peak pressure gradient of ≥40 mmHg on the most recent echocardiography. We analysed the effect of patient age and valve size on the durability of the bioprosthetic valve in the pulmonary position. RESULTS: There were 2 early deaths; no late deaths were observed. The follow-up duration was 92.8 ± 44.5 months. The mean age at PVR was 19.3 ± 9.1 years. The mean valve size was 24.7 ± 1.8 mm. Whereas patients ≥20 years old showed no valvular dysfunction (i.e. 100% freedom from valvular dysfunction at 10 and 14 years), patients who were adolescents and children (<20 years) showed worse durability, regardless of the z-score of valve size (68.2% at 10 years and 24.7% at 14 years). Although a larger valve with a z-score of ≥2 was implanted, patients <20 years old did not exhibit good valvular durability. The results were particularly worse in patients <10 years old, with 66.7% freedom from valvular dysfunction at 6 years and 33.3% at 8 years, compared with patients within the age range of 10 to <20 years (75.1% at 10 years, and 20.5% at 14 years). CONCLUSIONS: The durability of bioprosthetic valves in the pulmonary position was acceptable in patients aged ≥20 years, regardless of the z-score of valve size. However, patients who were children and adolescents did not show optimal durability of the bioprosthetic valve, irrespective of the z-score of valve size.

Factors associated with right ventricular dilatation and dysfunction in patients with chronic pulmonary regurgitation after repair of tetralogy of Fallot: analysis of magnetic resonance imaging data from 218 patients.

OBJECTIVE: The aim of the present study was to identify the factors associated with right ventricular (RV) dilatation and dysfunction in patients with chronic pulmonary regurgitation (PR) after repair of tetralogy of Fallot. METHODS: From April 2002 to June 2013, 218 patients with repaired tetralogy of Fallot underwent magnetic resonance imaging; 165 (76%) underwent transannular repair and 36 (17%) underwent nontransannular repair. Linear regression analyses were used to identify the predictors for RV end-diastolic volume index (EDVI), end-systolic volume index (ESVI), and ejection fraction. RESULTS: On univariable analysis, male sex, ventricular septal defect (VSD) closure through the right ventricle, larger pulmonary artery index, and greater PR fraction were associated with greater RV volume indexes. Multivariable analyses identified male sex (ß = 17.55, P < .001 for RV EDVI; ß = 14.08, P = .001 for RV ESVI), VSD closure through RV (ß = 8.49, P = .048 for RV ESVI), longer interval since repair (ß = 1.29, P = .014 for RV EDVI), and greater PR fraction (ß = 1.92, P < .001 for RV EDVI; ß = 1.38, P < .001 for RV ESVI) as independent predictors for greater RV volume indexes. On univariable analysis, male sex, VSD closure through the right ventricle, and greater PR fraction were associated with a lower RV ejection fraction. Multivariable analysis identified male sex (ß = -3.10, P = .018), VSD closure through the right ventricle (ß = -3.05, P = .020), and greater PR fraction (ß = -0.27, P < .001) as independent predictors for a lower RV ejection fraction. CONCLUSIONS: Male sex, VSD closure through the right ventricle, longer interval since repair, and greater PR fraction were independent predictors of RV dilatation after tetralogy of Fallot repair. Male sex, VSD closure through the right ventricle, and greater PR fraction were also independent predictors of RV dysfunction.

The echocardiographic paradox index in patients with a repaired tetralogy of Fallot.

OBJECTIVES: Right ventricular (RV) volume is very important for pulmonary valve replacement after the total correction of tetralogy of Fallot (TOF), and we attempted to identify a convenient echocardiographic index that is well correlated with the volumetric data obtained through magnetic resonance imaging (MRI). DESIGN: All patients who underwent cardiac MRI and echocardiography at Sejong General Hospital for evaluating pulmonary regurgitation after TOF total correction were included. The paradox index is the amount of paradoxical motion of the interventricular septum on the short-axis echocardiographic view. The paradox index was compared to several cardiac MRI indices. RESULTS: Fifty-four patients were included. The paradox index for all patients was 1.22 ± 0.12 (1.06-1.67), and the index of the operation group was significantly higher than that of the non-operation group (1.26 ± 1.12 vs 1.16 ± 1.12, P = 0.009). The paradox index was well correlated with the RV systolic and diastolic volumes, as measured by cardiac MRI (P = 0.002 and 0.003). Using a simple linear regression analysis, a paradox index of 1.24 corresponded to a RV diastolic volume of 160 ml/m(2). CONCLUSIONS: The paradox index could help to indicate the time for an MRI analysis of the RV volume in patients after TOF total correction.

Does limited right ventriculotomy prevent right ventricular dilatation and dysfunction in patients who undergo transannular repair of tetralogy of Fallot? Matched comparison of magnetic resonance imaging parameters with conventional right ventriculotomy long-term after repair.

OBJECTIVE: The objective of this study was to test the hypothesis that limited (<1 cm) right ventriculotomy (RV-tomy) in the setting of transannular tetralogy of Fallot (TOF) repair might result in less right ventricular (RV) dilatation and dysfunction compared with conventional RV-tomy. METHODS: Between June 2002 and April 2012, 113 patients with transannular repair of TOF underwent magnetic resonance imaging (MRI). Patients were divided into a limited RV-tomy group (n = 39) and a conventional RV-tomy group (n = 74). Thirty-nine patients from each group were matched for comparison using propensity scores. The MRI parameters of the 2 groups were compared. RESULTS: The interval between TOF repair and MRI examination was shorter in the limited RV-tomy group (limited, 12.7 ± 3.8 years; conventional, 17.2 ± 4.7 years; P < .001). Indexed RV volumes were similar between the groups (RV end-diastolic volume index: 149 ± 31 mL/m(2) vs 152 ± 42 mL/m(2); P = .704. RV end-systolic volume index: 70 ± 24 mL/m(2) vs 77 ± 38 mL/m(2); P = .313). There was no difference in the RV ejection fraction between the groups (54% ± 9% vs 51% ± 9%; P = .160). Propensity score-matched comparison also revealed no differences in RV volume and function. CONCLUSIONS: No long-term benefits of limited RV-tomy were demonstrated compared with conventional RV-tomy in patients who underwent transannular TOF repair, at least in terms of RV volume and function. Further studies are necessary to define the role of limited RV-tomy in patients who undergo transannular TOF repair.

The results of cardiopulmonary exercise test in healthy Korean children and adolescents: single center study.

PURPOSE: The cardiopulmonary exercise test (CPET) is an important clinical tool for evaluating exercise capacity and is frequently used to evaluate chronic conditions including congenital heart disease. However, data on the normal CPET values for Korean children and adolescents are lacking. The aim of this study was to provide reference data for CPET variables in children and adolescents. METHODS: From August 2006 to April 2009, 76 healthy children and adolescents underwent the CPET performed using the modified Bruce protocol. Here, we performed a medical record review to obtain data regarding patient' demographics, medical history, and clinical status. RESULTS: The peak oxygen uptake (VO2Peak) and metabolic equivalent (METMax) were higher in boys than girls. The respiratory minute volume (VE)/CO2 production (VCO2) slope did not significantly differ between boys and girls. The cardiopulmonary exercise test data did not significantly differ between the boys and girls in younger age group (age, 10 to 14 years). However, in older age group (age, 15 to 19 years), the boys had higher VO2Peak and METMax values and lower VE/VCO2 values than the girls. CONCLUSION: This study provides reference data for CPET variables in case of children and adolescents and will make it easier to use the CPET for clinical decision-making.