A dual-targeting approach using a human bispecific antibody against the receptor-binding domain of the Middle East Respiratory Syndrome Coronavirus.

The emergence of the Middle East Respiratory Syndrome Coronavirus (MERS-CoV) has posed a significant global health concern due to its severe respiratory illness and high fatality rate. Currently, despite the potential for resurgence, there are no specific treatments for MERS-CoV, and only supportive care is available. Our study aimed to address this therapeutic gap by developing a potent neutralizing bispecific antibody (bsAb) against MERS-CoV. Initially, we isolated four human monoclonal antibodies (mAbs) that specifically target the MERS-CoV receptor-binding domain (RBD) using phage display technology and an established human antibody library. Among these four selected mAbs, our intensive in vitro functional analyses showed that the MERS-CoV RBD-specific mAb K111.3 exhibited the most potent neutralizing activity against MERS-CoV pseudoviral infection and the molecular interaction between MERS-CoV RBD and human dipeptidyl peptidase 4. Consequently, we engineered a novel bsAb, K207.C, by utilizing K111.3 as the IgG base and fusing it with the single-chain variable fragment of its non-competing pair, K111.1. This engineered bsAb showed significantly enhanced neutralization potential against MERS-CoV compared to its parental mAb. These findings suggest that K207.C may serve as a potential candidate for effective MERS-CoV neutralization, further highlighting the promise of the bsAb dual-targeting approach in MERS-CoV neutralization.

Empowering SARS-CoV-2 variant neutralization with a bifunctional antibody engineered with tandem heptad repeat 2 peptides.

With the global pandemic and the continuous mutations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the need for effective and broadly neutralizing treatments has become increasingly urgent. This study introduces a novel strategy that targets two aspects simultaneously, using bifunctional antibodies to inhibit both the attachment of SARS-CoV-2 to host cell membranes and viral fusion. We developed pioneering IgG4-(HR2)4 bifunctional antibodies by creating immunoglobulin G4-based and phage display-derived human monoclonal antibodies (mAbs) that specifically bind to the SARS-CoV-2 receptor-binding domain, engineered with four heptad repeat 2 (HR2) peptides. Our in vitro experiments demonstrate the superior neutralization efficacy of these engineered antibodies against various SARS-CoV-2 variants, ranging from original SARS-CoV-2 strain to the recently emerged Omicron variants, as well as SARS-CoV, outperforming the parental mAb. Notably, intravenous monotherapy with the bifunctional antibody neutralizes a SARS-CoV-2 variant in a murine model without causing significant toxicity. In summary, this study unveils the significant potential of HR2 peptide-driven bifunctional antibodies as a potent and versatile strategy for mitigating SARS-CoV-2 infections. This approach offers a promising avenue for rapid development and management in the face of the continuously evolving SARS-CoV-2 variants, holding substantial promise for pandemic control.

Valve-sparing root replacement in children with connective tissue disease: Long-term risk of aortic events.

BACKGROUND: Cardiovascular involvement in pediatric patients with connective tissue disease (CTD) is life-threatening, with aortic root dilatation being the most prevalent cardiovascular abnormality. We attempted to determine long-term outcomes of valve-sparing root replacement (VSRR) in this group, including cardiovascular reoperations for aortic aneurysm and dissection. METHODS: We conducted a retrospective analysis of pediatric patients with CTD who received VSRR in a single center from 2002 to 2021. The primary end point was a composite event of all-cause death and cardiovascular reoperations. The median follow-up duration was 8.3 years, with a maximum of 20.7 years. RESULTS: The median age of 24 pediatric patients who had VSRR was 14.4 years. Marfan syndrome and Loeys-Dietz syndrome affected 19 (79.2%) and 5 (20.8%) patients, respectively. There was no early death. The 15-year survival rate was 91.7%. At 10 years after VSRR, the cumulative incidence of reoperation for aortic regurgitation was 15.6%, and for aortic aneurysm or dissection, it was 29.1%. The 10-year rate of freedom from the primary end point was 53.1%. The Cox multivariable analysis revealed younger age at surgery (hazard ratio, 1.279; 95% confidence interval, 1.086-1.505; P = .003) and VSRR before 13 years of age (hazard ratio, 5.005; 95% confidence interval, 1.146-21.850; P = .032) as independent prognostic factors for the primary endpoint. CONCLUSIONS: VSRR for aortic root dilatation in pediatric patients with CTD demonstrated good long-term survival and low reoperation rates for aortic regurgitation. However, several patients developed later aortic aneurysm or dissection, and careful surveillance may be required, particularly in those who received VSRR at younger age.

Dynamic thermal imaging on actinic keratosis patients: A preliminary study.

BACKGROUND: Diagnosis of actinic keratosis (AK) based only on clinical findings can be misleading, and histopathological diagnosis results in scars. Dynamic thermal imaging is a potential non-invasive tool for the diagnosis of AK. This imaging technique quantifies the infrared (IR) radiation emitted by a subject after exposure to external thermal stimuli, such as heat or cold. METHODS: Twenty-six histopathologically confirmed AK patients participated in the study. We compared the dynamic thermal images of AK lesions and normal skin (control sites). Temperature changes were plotted as a thermal response graph. After fitting exponential curves to the thermal response graph, the curve was converted to a logarithmic form. RESULTS: Comparison of the early thermal response graphs of lesions and control sites showed faster thermal recovery of AK lesions. There was a significant difference in the gradient component of the calculated logarithmic equation between the AK lesions and control sites (P < 0.001). CONCLUSION: Dynamic thermal imaging can be used as an auxiliary diagnostic tool for AK.

Nonsyndromic Peripheral Pulmonary Artery Stenosis Is Associated With Homozygosity of RNF213 p.Arg4810Lys Regardless of Co-occurrence of Moyamoya Disease.

BACKGROUND: Peripheral pulmonary arterial stenosis (PPAS) in childhood is frequently associated with other syndromes; however, PPAS in adolescents and adults is rare and its etiology is not well understood. We report the clinical characteristics of adult-onset nonsyndromic PPAS associated with the p.Arg4810Lys variant of the RNF213 gene. METHODS: We recently encountered an index case of severe pulmonary hypertension with multiple PPAS and intra- and extracranial arteriopathy. Because of a family history of Moyamoya disease (MMD), genetic analysis was performed, and revealed that this patient was homozygous for RNF213 p.Arg4810Lys. We searched for PPAS by reviewing the pulmonary hypertension registry and the MMD registry, and found four more cases of PPAS. Clinical features of the five patients and their families were analyzed. RESULTS: Mean age at diagnosis of pulmonary hypertension was 26 years, and the male to female ratio was 4:1. Genetic analysis of four patients revealed that all these patients were homozygous for the RNF213 p.Arg4810Lys variant. Pulmonary angiograms showed a string of beads pattern and/or diffuse stenosis of peripheral pulmonary arteries. Notably, three patients had MMD, whereas two patients did not. The three MMD patients had multiple stenoses of extracranial arteries other than the pulmonary artery. CONCLUSIONS: PPAS in segmental or subsegmental arteries in adulthood with multiple extracranial vasculopathies was found to be associated with homozygosity for RNF213 p.Arg4810Lys. RNF213 variant-associated vasculopathy should be categorized as a discrete disease entity of adulthood-onset PPAS regardless of the presence of MMD.

Outdoor artificial light at night, obesity, and sleep health: Cross-sectional analysis in the KoGES study.

Obesity is a common disorder with many complications. Although chronodisruption plays a role in obesity, few epidemiological studies have investigated the association between artificial light at night (ALAN) and obesity. Since sleep health is related to both obesity and ALAN, we investigated the association between outdoor ALAN and obesity after adjusting for sleep health. We also investigated the association between outdoor ALAN and sleep health. This cross-sectional survey included 8526 adults, 39-70 years of age, who participated in the Korean Genome and Epidemiology Study. Outdoor ALAN data were obtained from satellite images provided by the US Defense Meteorological Satellite Program. We obtained individual data regarding outdoor ALAN; body mass index; depression; and sleep health including sleep duration, mid-sleep time, and insomnia; and other demographic data including age, sex, educational level, type of residential building, monthly household income, alcohol consumption, smoking status and consumption of caffeine or alcohol before sleep. A logistic regression model was used to investigate the association between outdoor ALAN and obesity. The prevalence of obesity differed significantly according to sex (women 47% versus men 39%, p < 0.001) and outdoor ALAN (high 55% versus low 40%, p < 0.001). Univariate logistic regression analysis revealed a significant association between high outdoor ALAN and obesity (odds ratio [OR] 1.24, 95% confidence interval [CI] 1.14-1.35, p < 0.001). Furthermore, multivariate logistic regression analyses showed that high outdoor ALAN was significantly associated with obesity after adjusting for age and sex (OR 1.25, 95% CI 1.14-1.37, p < 0.001) and even after controlling for various other confounding factors including age, sex, educational level, type of residential building, monthly household income, alcohol consumption, smoking, consumption of caffeine or alcohol before sleep, delayed sleep pattern, short sleep duration and habitual snoring (OR 1.20, 95% CI 1.06-1.36, p = 0.003). The findings of our study provide epidemiological evidence that outdoor ALAN is significantly related to obesity.

Current expectations of the arterial switch operation in a small volume center: a 20-year, single-center experience.

BACKGROUND: We reviewed our 20-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) or double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly) to assess the early and long-term outcomes. METHODS: Between January 1995 and December 2014, 139 consecutive patients who underwent ASO for TGA or Taussig-Bing anomaly were included in this retrospective study. The median age at the operation was 9 (0-485) days, and 97 patients (70 %) underwent ASO less than 2 weeks. The median weight was 3.3 (2.1-10.3) kg. The patients were divided into three groups; simple TGA (n = 78) included patients with TGA with intact ventricular septum, complex TGA (n = 46) included those who had TGA with ventricular septal defect or other anomalies, and Taussig-Bing anomaly (n = 15). Median follow-up duration was 72.5 (0.4-230) months. RESULTS: There were 3(2.2 %) in-hospital deaths. One patient (0.7 %) underwent early reoperation due to coronary insufficiency. Late deaths occurred in 3 (2.2 %) of 136 survivors. The Kaplan-Meier's survival rate was 97.6 ± 1.4 % at 15 years. Twenty-three patients (16.9 %) required 26 reintervention. The freedom from reintervention rates were 82.5 ± 3.7 % at 5 years and 75.8 ± 4.7 % at 10 years, respectively. Median interval between ASO and first reintervention was 22.8 (6.4-89.2) months. The multivariate analysis showed that diagnosis of Taussig-Bing anomaly (hazard ratio, 7.09; P < 0.001) and side by side great artery relationship (hazard ratio, 7.98; P = 0.001) were independent risk factors for reoperation. Five patients (3.9 %) had developed at least moderate neo-aortic regurgitation during the follow-up and one patient underwent reoperation mainly for neo-aortic regurgitation. By multivariate analysis, Taussig-Bing anomaly was the risk factor for at least moderate neo-aortic regurgitation (P = 0.035). CONCLUSIONS: ASO can be performed with a low risk of early mortality and satisfactory long-term outcomes even in a small volume center. Close long-term surveillance is mandatory to detect structural or hemodynamic changes.

Clinical outcomes of slide tracheoplasty in congenital tracheal stenosis.

OBJECTIVES: Treatment of long-segment congenital tracheal stenosis (CTS) remains challenging. Recently, slide tracheoplasty has become the standard approach in many centres. The aim of this study was to evaluate the clinical outcomes of slide tracheoplasty. METHODS: Between 2004 and 2011, 18 patients underwent slide tracheoplasty in our centre. The median patient age was 2.5 months (range, 18 days-4 years) and the median body weight was 4.2 (range, 2.2-17.7) kg at operation. Eleven (61%) patients were on a mechanical ventilator prior to surgery. The median stenotic segment estimated by a computed tomography scan was 52% of the length of total trachea (range, 18-84%). Five (28%) patients had proximal bronchial stenosis, 3 (17%) had tracheal bronchus, 2 (11%) had tracheobronchomalacia and 1 (6%) had agenesis of the right lung. Thirteen (72%) patients had a combined cardiac anomaly, including 8 patients with a pulmonary artery sling. Ten (56%) patients had associated extracardiac anomalies. Slide tracheoplasty was performed on cardiopulmonary bypass in all patients, and cardiac lesions were corrected. RESULTS: There was no early death. The patient with agenesis of the right lung died of left bronchial stenosis 3 months after the surgery. Two (11%) patients were reoperated on for tracheal restenosis. In the other 15 patients, the median duration of ventilator support was 8 (range, 5-34) days and the median duration of hospitalization was 31 (range, 12-79) days. During the follow-up (median duration of 17 months; range, 2-77 months), 13 (72%) patients were symptom-free and 2 (11%) underwent tracheostomy for tracheomalacia. CONCLUSIONS: Based on this study, slide tracheoplasty seems to be an effective technique for CTS. However, shortening of the trachea after reconstruction may give rise to recurrent obstruction.

Surgery for partial anomalous pulmonary venous connections: modification of the warden procedure with a right atrial appendage flap.

BACKGROUND: Surgical repair of a partial anomalous pulmonary venous connection (PAPVC) to the superior vena cava (SVC) may be complicated by sinus node dysfunction or SVC obstruction. We modified the Warden procedure by using a right atrial auricular flap to decrease the occurrence of these complications. METHODS: Between February 2005 and July 2012, 10 consecutive patients underwent a modified Warden procedure to correct PAPVC. The median patient age was 5.7 years. Eight patients (80%) had an atrial septal defect. To surgically correct the PAPVC, we made a U-shaped incision on the right atrial appendage and sutured the flap to the posterior wall of the SVC. The anterior wall was reconstructed with various patch materials. RESULTS: No early or late deaths occurred, nor did any patient require early or late reoperation for SVC or pulmonary venous obstruction. No new arrhythmias appeared during follow-up, which was complete in all patients (mean, 29.5 months). CONCLUSION: Our modification of the Warden procedure seems to be effective and safe. This technique may lower the risk of SVC obstruction, pulmonary venous obstruction, and sinus dysfunction.

Transcatheter closure of perimembranous ventricular septal defect using Amplatzer ductal occluder.

OBJECTIVE: To show that transcatheter closure of perimembranous ventricular septal defect (PMVSD) with the Amplatzer ductal occluder (ADO, AGA Medical Corp, Plymouth, Minnesota) is a safe and effective treatment option. BACKGROUND: Transcatheter closure of PMVSD is a challenging procedure. Recently, the Amplatzer PMVSD occluder (APMVSDO, AGA Medical Corp, Plymouth, Minnesota) has been shown to be effective in closing hemodynamically significant PMVSDs. However, the high incidence of complete atrioventricular block (CAVB) after device occlusion of a PMVSD has been a hot issue as well. METHODS: Among several devices used in closing PMVSD percutaneously, we prefer the ADO because of the anatomic resemblance between PMVSD with aneurysm and patent ductus arteriosus, and it has no right ventricular disc which may contribute to the occurrence of CAVB. RESULTS: Between August 2009 and May 2012, 21 patients (5 males and 16 females) underwent percutaneous PMVSD closure using ADO. The patients' ages ranged from 3 to 42 years (median: 7 years), and their weights ranged from 18 kg to 60 kg (median: 27 kg). All patients showed echocardiographic signs of left ventricular volume overload and trivial to small mitral regurgitation (Qp/Qs = 1.7 ± 0.4). The mean defect size of the right ventricular side was 4.3 ± 1.0 mm. Devices 2 mm larger than the measured narrowest VSD diameter were selected in most patients. The ADOs were successfully implanted in all patients without any significant complications except one transient CAVB, one case of delivery wire fracture, and one case of surgery due to significant residual leak. Small residual shunts were observed immediately after the device implantation, but they disappeared during follow-up for 18 of 20 patients. The mean follow-up period was 20 ± 9 months, and CAVB or aortic regurgitation was not observed in all patients. CONCLUSION: Transcatheter closure of PMVSD with the ADO is a safe and promising treatment option, but long-term follow-up in a large number of patients would be warranted.

Bicuspid pulmonary valve implantation using polytetrafluoroethylene membrane: early results and assessment of the valve function by magnetic resonance imaging.

OBJECTIVES: The durability of bioprosthetic valves in the pulmonary position is suboptimal. The objectives of this study were to evaluate the early results of polytetrafluoroethylene (PTFE) bicuspid pulmonary valve (PV) implantation and to better define the function of this valve by magnetic resonance imaging (MRI). METHODS: Fifty-six patients who underwent PTFE bicuspid PV implantation between June 2009 and August 2011 were retrospectively analysed. The median age was 17.5 years and median valve size was 26 mm. Fundamental diagnoses were tetralogy of Fallot (n = 38), pulmonary atresia with ventricular septal defect (n = 8), double outlet right ventricle (n = 7) and absent PV syndrome (n = 3). Thirty-two patients with pulmonary regurgitation (PR) underwent MRI preoperatively and 22 of them underwent follow-up MRI at a median of 6.7 months postoperatively. RESULTS: There was one early death. Postoperative echocardiography (n = 53) showed no or trivial PR in 49 patients and mild PR in 4. Median follow-up duration was 15.2 months. There was no late death or reoperation. Follow-up echocardiography (n = 41) performed at a median of 7.5 months postoperatively showed no or trivial PR in 33 patients and mild PR in 8 patients. Follow-up MRI showed a significant reduction in right ventricular volumes and improvement in biventricular function. The median PR fraction of this valve was 10%. CONCLUSIONS: Early results of bicuspid PV implantation using PTFE membrane were satisfactory. PTFE bicuspid PV demonstrated excellent performance for the short term as evidenced by echocardiography and MRI. Long-term follow-up is mandatory to determine the durability of this valve.

Dilated unroofed coronary sinus mimicking cor triatriatum in cardiac-type total anomalous pulmonary venous connection.

We present a rare case of a patient with a dilated unroofed coronary sinus mimicking cor triatriatum that altered mitral inflow in a cardiac-type total anomalous pulmonary venous connection. The membranous tissue in the left atrium, thought to be cor triatriatum tissue preoperatively, was actually a remnant of a dilated coronary sinus tissue. Although this tissue did not create a significant pressure gradient and merely induced a turbulent mitral inflow, we resected it to avoid future problems with mitral leaflet function.

Palliation using a self-expandable stent in a patient with obstructive right-ventricular outflow tract after total correction of tetralogy of Fallot: self-expandable stent in tetralogy of Fallot.

Total correction of tetralogy of Fallot (TOF) is typically performed within the first year of life. However, subsequently, right-ventricular dysfunction often progresses by pulmonary stenosis or regurgitation. Although most patients with corrected TOF require additional surgery for right-ventricular outflow tract, intervention could be an alternative to palliative surgery and associated morbidities, especially in children. We describe a palliative application of the self-expandable stent in a patient with pulmonary stenosis after total correction of TOF who lacked a conduit between the right ventricle and pulmonary artery.

Partial anomalous pulmonary venous connection to the superior vena cava: the outcome after the Warden procedure.

OBJECTIVE: Partial anomalous pulmonary venous connection (PAPVC) draining into the superior vena cava (SVC) has been repaired with various techniques. We investigated the outcome of the Warden procedure for repair of this anomaly. METHODS: From December 1994 to January 2011, 30 patients underwent a Warden procedure for repair of PAPVC to the SVC in our center. Their median age at the time of the operation was 4.9 years (range, 1 month to 55 years). Follow-up data were obtained through a review of medical records, correspondence with the patients' cardiologists, and direct telephone contact. The mean follow-up duration was 5.3 ± 5.1 years (range, 1 month to 16 years). RESULTS: One patient died of an underlying cardiac condition and cerebral complication unrelated to the Warden procedure. One patient had transient postoperative sinus node dysfunction. During follow-up, pulmonary venous pathway obstruction occurred in one patient, and systemic venous pathway obstruction occurred in three patients. Re-operation or re-intervention for systemic venous pathway obstruction was required in younger (<2 years) and smaller (<7 kg) patients within 1 year after the Warden procedure. All patients were in regular sinus rhythm in the latest electrocardiogram. CONCLUSIONS: The Warden procedure is a safe and effective surgical option for repair of PAPVC to the SVC in terms of preserving the sinus node function and non-obstructive pulmonary venous pathway. However, more attention must be paid to the reconstruction of non-obstructive systemic venous pathway, especially in younger and smaller children. Patch augmentation could be considered and effectively performed, if there is any doubt regarding tension-free anastomosis.

Permanent epicardial pacing in pediatric patients: 12-year experience at a single center.

BACKGROUND: Permanent cardiac pacing is not often done in children, and when done is usually accomplished through epicardial pacing. We reviewed a 12-year experience with the implantation of epicardial pacemakers by our clinical group. METHODS: Fifty-three patients who underwent their first implantation of an epicardial pacemaker before the age of 18 years and between 1997 and 2009 were included in our study. The mean age of the patients at the time of first pacemaker implantation was 5.7±4.8 years. Indications for pacemaker implantation included postoperative or congenital atrioventricular block and sinus node dysfunction. The patients underwent 105 operations for the replacement of pacemaker pulse generators and 75 operations for the replacement of pacemaker leads. The most commonly used generator mode was the rate-responsive accelerometer-based (DDDR) mode, which was used in 40.9% of the patients. We used more non-steroid-eluting leads (70.1%) than steroid-eluting leads (29.1%). RESULTS: The overall duration of follow-up in the study was 8.0±4.5 years (range, 2.1 months to approximately 17.0 years). Freedom from the need for generator replacement was 98.0%, 60.7%, and 11.1% at 1, 5, and 8 years, respectively. A tendency toward early generator exhaustion was observed among younger patients (p=0.058). The generator mode used for pacing did not significantly affect generator longevity. Freedom from the need for lead replacement was 98.3%, 83.8%, and 63.6% at 1, 5, and 10 years, respectively. The mean longevity of the leads used in the study was 10.8±0.8 years. Neither patient age at the time of lead implantation nor type of lead significantly affected lead longevity. CONCLUSIONS: Lead longevity was sufficiently long and did not vary significantly according to type of lead. Generator longevity was not affected by lead type, generator mode, or patient age at the time of pacemaker implantation.

Sirolimus-eluting stent fracture and aneurysm formation in a young child with a coronary artery bypass graft.

Clinical experience with coronary stent implantation in children is very limited. In-stent restenosis, thrombosis, and aneurysm formation are known complications. Recently, fracture of a drug-eluting stent was reported to be a cause of in-stent restenosis, but the natural course of stent fracture and proper management options remain uncertain. This report describes the case of a 7-year-old boy with a sirolimus-eluting stent implanted to treat stenosis of a coronary artery bypass graft who showed complete stent fracture and aneurysm formation. During the 2-year follow-up period, the boy experienced complete regression of the aneurysm without in-stent restenosis.

Durability of bioprosthetic valves in the pulmonary position: long-term follow-up of 181 implants in patients with congenital heart disease.

OBJECTIVES: Durability of bioprosthetic valves in the pulmonary position is not well defined. We examined the durability of bioprosthetic valves in the pulmonary position and risk factors associated with bioprosthetic pulmonary valve failure. METHODS: Between 1993 and 2004, 181 patients underwent pulmonary valve replacement using bioprostheses. Patients who underwent valved conduit or homograft implantation were excluded. Mean age was 14.2 ± 9.8 years and median valve size was 23 mm (range, 19-27 mm). Types of bioprosthesis used were Hancock II (n = 83), Perimount (n = 53), Freestyle (n = 23), Carpentier-Edwards porcine valve (n = 18), and others (n = 4). RESULTS: There were 3 early and 7 late deaths. Follow-up completeness was 88.6% and mean follow-up duration was 7.3 ± 2.9 years. Forty-three patients underwent redo pulmonary valve replacement. Overall freedom from redo pulmonary valve replacement at 5 and 10 years was 93.9% ± 1.9% and 51.7% ± 8.6%, respectively. Overall freedom from both valve failure and valve dysfunction at 5 and 10 years was 92.2% ± 2.1% and 20.2% ± 6.7%, respectively. In multivariable analysis, younger age at operation, diagnosis of pulmonary atresia with ventricular septal defect, and use of stentless valve were identified as risk factors for redo pulmonary valve replacement. CONCLUSIONS: Durability of bioprosthetic valves in the pulmonary position was suboptimal. Valve function was maintained stable until 5 years after operation. By 10 years, however, about 80% will require reoperation or manifest valve dysfunction. In our experience, the stentless valve was less durable than stented valves.

Early surgical correction of atrioventricular valvular regurgitation in single-ventricle patients.

BACKGROUND: The purpose of this study is to evaluate the effectiveness of early surgical correction for atrioventricular valve regurgitation (AVVR) in single-ventricle patients. METHODS: The medical records of 39 single-ventricle patients who underwent atrioventricular surgery more than once between 1996 and 2008 were reviewed. RESULTS: The mean preoperative grade of AVVR was 2.6 ± 0.7. Four patients underwent valvular operations at first palliative surgery, 3 patients before bidirectional cavopulmonary connection, 13 at bidirectional cavopulmonary connection, 6 in the interstage between bidirectional cavopulmonary connection and Fontan, 10 at Fontan, and 3 after Fontan procedure. Surgical techniques for valve were edge-to-edge sutures for bridging leaflets, leaflet cleft repair, partial or complete annuloplasty with strip, or artificial valve implantation. Although there was no statistical significance, the patients who underwent early operation (AVVR grade less than 2) showed a tendency toward better atrioventricular valvular function during the postoperative follow-up (57.1 months, range: 2 ∼ 129 months). None of the patients whose preoperative AVVR grade less than 2 showed an AVVR more than 2 at the final echocardiography. There were 4 deaths overall due to sepsis, Fontan failure, and sudden cardiac arrest. Final echocardiographic findings showed a mean AVVR of 1.6 ± 0.8 and acceptable cardiac function. All living patients were in a good New York Heart Association functional class (1.07 ± 0.2). CONCLUSIONS: Even though we could not find statistically significant evidence of benefit for early correction of AVVR in single-ventricle patients, the patients undergoing early valvular operation for regurgitation showed a tendency toward better atrioventricular valvular function at midterm.

The recent surgical result of total anomalous pulmonary venous return.

BACKGROUND AND OBJECTIVES: Recent surgical results from total anomalous pulmonary venous return (TAPVR) are reported to have improved; however, mortality and morbidity are still high in the univentricles. This review was performed in order to demonstrate surgical results from TAPVR for recent years in a single institute. SUBJECTS AND METHODS: A total of 53 patients with TAPVR underwent surgery between January 2000 and December 2008. Preoperative anatomy and hemodynamics were evaluated by 2-dimensional echocardiogram. We reviewed medical records on preoperative management, age, and body weight at time of surgery, postoperative mortality, and pulmonary venous obstruction. RESULTS: The study included 36 biventricle patients and 17 univentricle patients. Median age and weight at time of surgery were 26 days and 3.5 kg, respectively. During the mean follow up period of 48.2 months, a total of 11 deaths and 8 early deaths occurred. Postoperative pulmonary venous stenosis occurred in 8 patients. Among 36 biventricle patients, mortality occurred in 1 patient and postoperative pulmonary venous stenosis occurred in 6 patients. No statistical significances were observed between postoperative pulmonary venous stenosis and other factors. Among 17 univentricule patients, mortality was 10, including 7 early deaths, and postoperative pulmonary venous stenosis occurred in 2 patients. Combined palliations were modified Blalock-Taussig (BT) shunts in 7, pulmonary artery banding (PAB) in 3, bidirectional cavopulmonary shunt in 5, and the Fontan procedure in 1. The highest mortality was recorded in patients with a modified BT shunt (85.7%) and next in patients with PAB (66.6%). Mortality was not related to anatomy of TAPVR, nor preoperative obstruction, but with significant age and weight at time of surgery. CONCLUSION: Despite improvement in operative results from TAPVR, surgery in univentricles is highly risky, especially with low weight and early age, with concomitant palliation.