[Show abstract][Hide abstract] ABSTRACT: Background and objectives:
Despite remarkable advances in pediatric cardiology, pulmonary arterial hypertension associated with congenital heart disease remains a major problem. In the past decade new vasodilators have been introduced and appear to be effective in reducing pulmonary vascular resistance (PVR).
Subjects and methods:
From 2000 to 2011, we retrospectively reviewed the records of 22 patients who had congenital septal defects and borderline pulmonary vascular disease (PVD). The PVR in these patients was from 6 to 16 wood units · m(2), and/or the systolic pulmonary arterial pressure was more than 2/3 of the systemic arterial pressure.
The median age was 16 years (range, 9 months-46 years). The median duration of follow-up was 7.4 years (range, 1.4-11.7 years). According to hemodynamic data and clinical symptoms, the initial management comprised targeted medical therapy in four (18%), complete closure in four (18%), and partial closure in 14 patients (63.6%). In the four patients who had a high PVR and negative vasoreactivity, the PVR decreased and vasoreactivity increased after targeted medical therapy; three of these patients underwent cardiac surgery later. Finally, 11 (50%) received targeted medical therapy and 21 patients (95.4%) underwent cardiac surgery. Complete closure resulted in six patients and partial closure in 17 patients. Mortality was observed in two patients. The other 19 patients (91%) had New York Heart Association functional class I.
Targeted medical therapy may be effective in reducing PVR in patients with congenital heart disease and borderline PVD. A stepwise approach may help to achieve improved outcomes in these patients.
Preview · Article · Sep 2015 · Korean Circulation Journal
[Show abstract][Hide abstract] ABSTRACT: Background
Atrial fibrillation (AF) in adult patients with atrial septal defect (ASD) accompanies an enlarged right atrium (RA) with a less enlarged left atrium (LA), which is the opposite situation in patients with AF and mitral valvular disease. This study was to compare the histopathological change in the atrium of patients with AF of two different etiologies: ASD and mitral disease.
Twenty-four patients were enrolled. Group 1 included patients with ASD (8), group 2 included patients with ASD with AF (6), and group 3 included patients with mitral disease with AF (10). Preoperative atrial volumes were measured. Atrial tissues were obtained during surgical procedures and stained with periodic acid-Schiff, smooth muscle actin, Sirius red, and Masson’s trichrome to detect histopathologic changes compatible with AF. The severity of histopathological changes was represented with “positivity” and “strong positivity” after analyzing digitalized images of the staining. We investigated the relationship between the degree of atrial dilatation and severity of histopathological changes according to the groups and tissues.
Group 2 and group 3 patients showed a tendency toward an enlarged RA volume and enlarged LA volume, respectively, compared with each others. However, in the histopathologic analysis, “positivity” and “strong positivity” showed no significant positive correlations with the degree of atrial volume in special staining.
A similar degree of histopathologic changes was observed in both atria in patients with AF (group 2 and 3) regardless of the degree of dilatation of atrial volume and disease entities.
No preview · Article · Jul 2014 · Cardiovascular pathology: the official journal of the Society for Cardiovascular Pathology
[Show abstract][Hide abstract] ABSTRACT: There has been a dramatic increase in the number and type of procedures performed in the field of cardiac intervention in the past decade. Percutaneous intervention is becoming an increasingly recognized modality for the management of prosthetic paravalvular leakages (PVLs) in severely symptomatic non-surgical candidates. Herein, we report our experience of percutaneous closure using the Amplatzer duct occluder for a PVL in a patient who underwent tricuspid valve replacement.
Preview · Article · Apr 2013 · Korean Circulation Journal
[Show abstract][Hide abstract] ABSTRACT: Aortopexy is a surgical procedure in which the aortic arch is fixed at other structures, thus widening the interaortic space. This study aimed to evaluate the outcome of aortopexy by means of chest computed tomography for patients with congenital heart disease. The study retrospectively reviewed the medical records of 16 patients with congenital heart disease who had undergone aortopexy by compressed airway. The severity of compressed bronchus before aortopexy, immediately after aortopexy (≤1 month), and after the patient had grown up were compared. To estimate the efficacy of the aortopexy, the interaortic distance index was calculated. Of the 16 patients, aortopexy was performed at the ascending aorta in 7, at the descending aorta in 7, and at the transverse arch in 2. The diameter ratio between the narrowest bronchus and the trachea was improved after aortopexy, as was the diameter ratio before aortopexy versus immediately after aortopexy (n = 9; p = 0.018) and the diameter ratio before aortopexy versus after the patient had grown up (n = 11; p = 0.006). Also, the interaortic distance index was increased after aortopexy, as was the diameter before aortopexy versus immediately after aortopexy (n = 9; p = 0.039) and the diameter before aortopexy versus after the patient had grown up (n = 11; p = 0.014). The study had one case of mortality due to sepsis. As shown by the results, aortopexy in patients with a compressed airway between arches is a useful surgical option. Such a compressed airway between arches should be considered for patients with an unusual clinical course before and after open heart surgery.
No preview · Article · Mar 2013 · Pediatric Cardiology
[Show abstract][Hide abstract] ABSTRACT: Atrial septal defect (ASD) is the one of most common congenital heart diseases detected in adults. Along with remarkable development of device technology, the first treatment strategy of secundum ASD has been transcatheter closure in feasible cases. However, there are only a few publications regarding the results of transcatheter closure of ASD in elderly patients, especially those over 60 years of age. We report our results of transcatheter closure of ASD in patients over 60 years old.
Between May 2006 and December 2011, 31 patients over 60 years old (25 female and 6 male; mean 66.7±5.25 years old, range 61-78 years old) were referred to our center.
A total of 23 patients underwent therapeutic catheterization to close secundum ASD, and the closure was successful in 22 patients (95.7%). All patients who underwent the procedure survived except for one patient who expired because of left ventricular dysfunction. A small residual shunt was observed in two (9%) of 21 patients before discharge but disappeared at follow-up. All patients eventually had complete closure. There were five patients who had coronary problems. One patient underwent percutaneous coronary intervention using a stent at the same time as transcatheter closure of ASD. Atrial arrhythmias were detected in 6 of 23 patients (26.1%) before the procedure. One patient was successfully treated by radiofrequency ablation before the procedure. No patients displayed new onset arrhythmia during the follow-up period. Follow-up echocardiographic evaluation showed a significantly improved right ventricular geometry.
We conclude that transcatheter closure of ASD is a safe and an effective treatment method for patients over 60 years old if the procedure is performed under a thorough evaluation of comorbidities and risk factors.
Preview · Article · Feb 2013 · Korean Circulation Journal
[Show abstract][Hide abstract] ABSTRACT: Routine use of intraoperative transesophageal echocardiography (TEE) is a safe monitoring and diagnostic method during pediatric congenital cardiac surgery. However, the question of whether intraoperative TEE is accurate and cost effective for patients with tetralogy of Fallot (TOF) has not been raised. This study aimed to analyze the cost-benefit of routine TEE during the repair of TOF. The medical records, including TEE results, for patients who underwent correction of TOF between January 1997 and June 2007 were reviewed and retrospectively analyzed. Intraoperative TEE was performed for 340 patients (85 %). Residual problems were detected in 17.9 % (61/340), and a return to bypass was needed for 10 % (34/340) of the patients. The degree of agreement between the intraoperative TEE and early postoperative transthoracic echocardiography (TTE) was relatively high. Surgeons with less surgical experience more frequently used intraoperative TEE (p = 0.007) and performed repeat bypass surgery at a higher rate (p = 0.00). Even relatively unskilled surgeons might be able to achieve surgical outcomes similar to those of experienced surgeons using intraoperative TEE. By avoiding late surgical revision, the possible cost savings were estimated to be 1,726,000 Korean won (US$1,489) per TEE examination. Intraoperative TEE can be used as a tool for surgeons in making decisions in the operating room. In addition, intraoperative TEE decreased the frequency of reoperations and postoperative interventions. The results of this study demonstrate that routine intraoperative TEE during repair of TOF was both clinically beneficial and cost effective.
No preview · Article · Jul 2012 · Pediatric Cardiology
[Show abstract][Hide abstract] ABSTRACT: 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.
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%).
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.
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.
No preview · Article · Dec 2011 · The Annals of thoracic surgery
[Show abstract][Hide abstract] ABSTRACT: A 17-year-old girl visited our hospital for an operation to correct a partial anomalous pulmonary venous return. All the right-side pulmonary veins returned to the azygos vein, which had no connection to the cardiac chamber through the great venous system, such as the superior or inferior vena cava. The azygos vein lacked a normal azygos arch and was connected by multiple connections only to the paravertebral plexus. For the surgical correction, a pericardiostomy was created in the right posterior pericardial wall and azygos vein which was divided at the diaphragm level and was anastomosed to the left atriotomy area through the pericardiostomy site.
No preview · Article · Sep 2011 · The Annals of thoracic surgery
[Show abstract][Hide abstract] ABSTRACT: 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.
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).
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.
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.
No preview · Article · Aug 2011 · The Journal of thoracic and cardiovascular surgery
[Show abstract][Hide abstract] ABSTRACT: 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.
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).
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.
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.
Preview · Article · Jul 2011 · European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery
[Show abstract][Hide abstract] ABSTRACT: Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. This broad term includes patients with a wide variety of very complex cardiac lesions. Patients with heterotaxy can be stratified into the subsets of asplenia syndrome and polysplenia syndrome, or the subsets of heterotaxy with isomerism of the right atrial appendages and heterotaxy with isomerism of the left atrial appendages. Treatment of patients with isomerism is determined by the nature and severity of the associated cardiac and extracardiac lesions. Most cardiac operations for patients with isomerism are palliative in nature, since normal anatomy is rarely achieved and mortality rates remain high for patients with heterotaxy syndrome. Patients with left isomerism in general have less severe cardiac malformations than those with right isomerism and, hence, more chance of biventricular repair. For almost all patients with right isomerism, and for many with left isomerism, biventricular repair will not be feasible, and all palliative protocols are then staging procedures prior to a Fontan-type repair. Recent advances in medical management, and improvements in surgical techniques have resulted in improved survival for these patients, and the surgical outcomes are comparable to those with Fontan circulation irrespective of the presence or absence of heterotaxy.
Preview · Article · May 2011 · Korean Circulation Journal
[Show abstract][Hide abstract] ABSTRACT: 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.
No preview · Article · Apr 2011 · Pediatric Cardiology
[Show abstract][Hide abstract] ABSTRACT: The purpose of this study is to evaluate the effectiveness of early surgical correction for atrioventricular valve regurgitation (AVVR) in single-ventricle patients.
The medical records of 39 single-ventricle patients who underwent atrioventricular surgery more than once between 1996 and 2008 were reviewed.
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).
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.
No preview · Article · Oct 2010 · The Annals of thoracic surgery
[Show abstract][Hide abstract] ABSTRACT: A fetal echocardiogram revealed a large right ventricular mass. After birth, the infant had pulmonary blood flow obstruction with cyanosis. The size and characteristics of the tumor prevented resection. The patient underwent placement of a systemic-to-pulmonary shunt as another alternative. This palliation allowed discharge from the hospital and provided good relief for the cyanosis until the tumor became resectable. The pathologic examination revealed a cardiac fibroma. (Ann Thorac Surg 2010; 90: 1011-4) (C) 2010 by The Society of Thoracic Surgeons
No preview · Article · Sep 2010 · The Annals of thoracic surgery
[Show abstract][Hide abstract] ABSTRACT: Abstract Isolation of the left innominate artery (IA) with right aortic arch is a rare congenital anomaly in which the IA loses its connection to the aorta and is connected to the pulmonary artery via a left ductus arteriosus. Here, we report a case of a 9-month-old girl with incomplete isolation of the IA and double outlet right ventricle. Along with repair of the intracardiac anomaly, the IA was reimplanted to aortic arch to ensure adequate blood flow from the aorta. (J Card Surg 2010;25:232-234)
No preview · Article · Feb 2010 · Journal of Cardiac Surgery
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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.
Despite improvement in operative results from TAPVR, surgery in univentricles is highly risky, especially with low weight and early age, with concomitant palliation.
Preview · Article · Jan 2010 · Korean Circulation Journal
[Show abstract][Hide abstract] ABSTRACT: We analysed the long-term results of mitral valve (MV) repair in children.
We reviewed clinical records of 139 children (<18 years) who underwent MV repair between 1988 and 2007. Patients with atrioventricular septal defect, single ventricle or atrioventricular discordance were excluded. Median age was 2.3 years (2 months to 17.6 years), and 47 children (34%) were infants. Mitral regurgitation (MR) was predominant in 125 patients (90%), and 91 (73%) of these showed MR grade > or = 3. Mitral stenosis (MS) was predominant in 14 patients (10%), and median mean pressure gradient across the MV was 9.0 mmHg (0-20 mmHg). Associated cardiac lesions were present in 111 patients (80%) and were addressed concurrently in 105 patients. Various surgical techniques were used according to the functional and pathologic findings of MV.
There was no early death. Median follow-up was 8 years (2 months to 20 years, 78% complete). Twenty-six patients required 29 MV re-operations, and 11 of these required MV replacements. At 15 years, freedom from MV re-operation and MV replacement was 77% and 90%, respectively. Diagnosis of MS and MV status on discharge (MR grade > or =3 or MS gradient > or =10 mmHg) were significant risk factors for re-operation. There were three late deaths, and the overall survival was 97% at 15 years. Among 122 survivors with MR, 102 patients (84%) underwent echocardiography during follow-up. The degree of MR decreased significantly and only five patients showed MR grade 3. Among 14 survivors with MS, eight patients (57%) underwent echocardiography during follow-up. The degree of MS decreased significantly and median MS gradient was 2.8 mmHg (0-10 mmHg). All survivors remain in the NYHA class I or II.
MV repair in children showed excellent survival, acceptable re-operation rate and satisfactory valve function at long-term follow-up. Residual MV dysfunction was a significant risk factor for re-operation, but re-repair was successful in more than half of the patients who underwent re-operation.
Preview · Article · Sep 2009 · European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery
[Show abstract][Hide abstract] ABSTRACT: It is well known that hepatic vein (HV) inclusion can ameliorate cyanosis in patients with pulmonary arteriovenous fistulas (PAVFs) during the sequence of Fontan type repair. Previously, we reported that most patients with bidirectional cavopulmonary shunt (BCPS) have clinical or subclinical evidence of a right to left shunt through PAVFs.
We studied 33 patients who already had clinical and subclinical PAVFs after BCPS. All patients have taken Fontan completion with HV inclusion. The state of PAVFs was reevaluated by pulmonary angiogram, contrast echocardiography, and lung scintigraphy 7.7 +/- 2.4 years after HV inclusion.
After Fontan completion, the mean oxygen saturation increased from 80.2 +/- 7.4% to 91.5 +/- 9.8% in the entire cohort. Moreover, the amount of right-to-left shunting through the PAVFs, measured by lung scintigraphy, was decreased from a mean of 23.8 +/- 15.1 to 13.0 +/- 8.2%. The degree of severity, for most patients, was decreased as demonstrated by contrast echocardiography. However, 5 patients (16.7%) showed persistent PAVFs, even after the HV inclusion. They all had left isomerism with azygous continuation of the IVC and the conduit was positioned on the contralateral side to the SVC with azygous drainage.
Most PAVFs regressed after Fontan completion. Left isomerism with azygous continuation of the IVC had risk for persistent PAVFs when the HV conduit was positioned at the contralateral side to the SVC receiving the azygous drainage. Therefore, appropriate design avoiding unilateral streaming of HV flow should be considered for HV inclusion surgery.
Preview · Article · Mar 2009 · The Annals of thoracic surgery