Comparison of Outcomes after Device Closure with Transseptal Puncture and Standard Technique in Patients with Patent Foramen Ovale and Ischemic Events.

OBJECTIVES: The purpose of this study was to compare the effectiveness of device closure with the transseptal puncture and standard technique in patients with patent foramen ovale (PFO) and ischemic events. METHODS: Eighty-two consecutive patients (men: 60 patients, mean age: 45.2 years) who underwent PFO closure with the Amplatzer PFO Occluder were enrolled. PFO closure with the transseptal puncture was performed in 22 patients (transseptal puncture technique, group I). In the remaining patients (n = 60), PFO closure was performed with the standard technique (group II). The co-primary end points were the incidence of significant residual shunt on follow-up transesophageal echocardiography (TEE) and a composite of death, stroke, transient ischemic attack (TIA), and peripheral embolism. RESULTS: Baseline characteristics were similar between the two groups. On TEE, despite similar grade of interatrial right-to-left shunt, shunt at rest/septal hypermobility was less common in group I than in group II (40.9% vs. 72.9%, P < 0.010). The device was successfully implanted in all patients. On follow-up TEE, significant residual shunt was more common in group I than in group II (28.6% vs. 4.3%, P = 0.021). In addition, composite of death, stroke, TIA, or peripheral embolism was more common in group I than in group II (13.6% vs. 0%, P = 0.017) during the follow-up period (mean 25.4 months). CONCLUSION: Compared to the standard technique, PFO closure with the transseptal puncture technique showed higher incidence of residual shunt and ischemic events. Therefore, this technique might be considered in only highly selected patients as the last option.

Migraine with aura: a predictor of patent foramen ovale in children and adolescents.

BACKGROUND: The prevalence of patent foramen ovale (PFO) is higher among adult migraine patients. The purpose of this study was to determine the frequency of PFO in children and adolescent migraine patients. METHODS AND RESULTS: A total of 32 patients with migraine (divided into two subgroups, the migraine with aura subgroup and the migraine without aura subgroup) and 31 normal control subjects were enrolled in this study. All of the participants underwent transthoracic echocardiography with an agitated saline test. We compared the prevalence of PFO and the severity of right-to-left shunt (RLS) in each group. No statistical difference in age and sex ratio was observed in either group. The prevalence of PFO was higher in the migraine group than in the control group, but without statistical significance (46.9% vs. 25.8%, P = 0.084). The prevalence of PFO was significantly higher in the migraine with aura subgroup than in the migraine without aura subgroup ( P = 0.031) and the normal control group ( P = 0.0074). Migraine with aura was the only significant factor showing an association with PFO (<0.01). RLS size did not have an influence on migraine. CONCLUSIONS: Considering the significantly high prevalence of PFO in pediatric migraine with aura patients, migraine with aura is a clear predictor of PFO among children and adolescents.

The effects of perioperative inhaled iloprost on pulmonary hypertension with congenital heart disease.

OBJECTIVES: The treatment of choice for congenital heart disease (CHD) with pulmonary arterial hypertension (PAH) is still controversial. We assessed the efficacy and safety of perioperative inhaled iloprost therapy in CHD with PAH. METHODS: Among 45 patients with a ventricular septal defect and/or an atrial septal defect with PAH, 28 patients were treated with inhaled iloprost before and after surgery. Perioperative clinical parameters and plasma B-type natriuretic peptide (BNP) were evaluated. RESULTS: No statistical difference in the estimated right ventricular systolic pressure (e-RVP), the e-RVP-to-systemic pressure ratio, and preoperative BNP levels between the iloprost group and the control group were found. Among the iloprost group, oxygen saturation was increased significantly after iloprost inhalation therapy (p = 0.0052). The iloprost group was also significantly correlated with less use of inhaled nitric oxide in the immediate postoperative period compared to the control group (p = 0.021). The durations of mechanical ventilation (p = 0.018), ICU stay (p = 0.005), and chest tube use (p = 0.039) were significantly shorter in the iloprost group compared to the control group. The plasma BNP, checked on 7th day of postoperatively, was lower in the iloprost group than in the control group (p = 0.008). CONCLUSION: Perioperative inhaled iloprost therapy showed the benefit of cardiac functional improvement and early weaning of postoperative supportive care in the management of CHD with PAH.

Effects of inhaled iloprost on congenital heart disease with Eisenmenger syndrome.

Identification of the pathophysiology associated with Eisenmenger syndrome has led to the evaluation of targeted therapies. Iloprost is one such targeted therapy used for patients with Eisenmenger syndrome. This study aimed to assess the efficacy and safety of iloprost used for patients with Eisenmenger syndrome. In this study, 12 patients with Eisenmenger syndrome (mean age, 33.2 ± 12.1 years; 75% female) started receiving iloprost 10 µg/dose administered six times a day. Of the 12 patients, 9 were classified as New York Heart Association (NYHA) functional class 3, and three were categorized as functional class 4. Changes in 6-min walk distance, NYHA functional class, oxygen saturation at resting, and results after the 6-min walk test were checked, as well as changes in right ventricle diameter and pulmonary arterial pressure shown by echocardiography. The distance during a 6-min walk increased from 255.8 ± 120.4 to 349.4 ± 134.7 m (p = 0.013), and 10 patients improved their NYHA functional class by one grade (p = 0.007). The mean resting oxygen saturation (SpO(2)) increased from 80.6 ± 14.2 to 84.9 ± 13.0% (p = 0.040), and after the 6-min walk test, it increased from 63.8 ± 22.9 to 68.8 ± 21.5% (p = 0.007). The mean right ventricle diameter during the diastolic phase changed from 53.7 ± 4.8 to 51.4 ± 3.9 mm (p = 0.068), and the mean pulmonary arterial pressure changed from 62.8 ± 13.7 to 58.9 ± 11.7 mmHg (p = 0.059). Neither death nor critical adverse effects occurred for any patients. Mild headache and dyspnea were common reports during the iloprost treatments. No patients stopped the therapy due to these adverse effects. Iloprost is well tolerated and appears to be beneficial in the management of patients with Eisenmenger syndrome.

Respiratory variation in aortic blood flow velocity as a predictor of fluid responsiveness in children after repair of ventricular septal defect.

This study aimed to compare respiratory variation in transthoracic echo-derived aortic blood flow velocity (∆Vpeak) and inferior vena cava diameter (∆IVCD) with central venous pressure (CVP) as predictors of fluid responsiveness in children after repair of ventricular septal defect (VSD). A prospective study conducted in pediatric intensive care unit investigated 21 mechanically ventilated children who had undergone repair of VSD. Standardized volume replacement (VR) was the intervention used. Hemodynamic measurements including CVP, heart rate, mean arterial pressure, transthoracic echo-derived stroke volume (SV), cardiac output, ∆Vpeak, and ∆IVCD were performed 1 h after patient arrival in the intensive care unit. Hemodynamic measurements were repeated 10 min after VR by an infusion of 6% hydroxyethyl starch 130/0.4 (10 ml/kg) over 20 min. The volume-induced increase in the SV was 15% or more in 11 patients (responders) and less than 15% in 10 patients (nonresponders). Before volume replacement, the ∆Vpeak (23.1 ± 5.7% vs. 14.0 ± 7.7%; p = 0.006) and ∆IVCD (26.5 ± 16.2% vs. 9.2 ± 9.1%; p = 0.008) was higher in the responders than in the nonresponders, whereas CVP did not significantly differ between the two groups. The prediction of fluid responsiveness was higher with the ΔVpeak, as shown by a receiver operating characteristic curve area of 0.83 (95% confidence interval [CI], 0.61-1.00; p = 0.01), a ΔIVCD of 0.85 (95% CI, 0.69-1.00; p = 0.01), and a CVP of 0.48 (95% CI, 0.22-0.73; nonsignificant difference). The ∆Vpeak and ∆IVCD measured by transthoracic echocardiography can predict the response of SV after volume expansion in mechanically ventilated children at completion of VSD repair.

Non-Responders to Intravenous Immunoglobulin and Coronary Artery Dilatation in Kawasaki Disease: Predictive Parameters in Korean Children.

BACKGROUND AND OBJECTIVES: In Kawasaki disease (KD), high dose intravenous immunoglobulin (IVIG) significantly lowers the coronary complications. However, some patients either do not respond to initial therapy or develop coronary complications. We aimed to identify the predictive factors for unresponsiveness to initial IVIG therapy and coronary artery dilatation (CAD; defined by Z-score≥2.5) in the acute phase and convalescent phase. SUBJECTS AND METHODS: A retrospective review was conducted of 703 patients with KD, admitted to Gachon University Gil Medical Center between January 2005 and June 2013. The patients were divided into two groups-IVIG responders vs. non-responders-based on the IVIG treatments, and presence of fever after treatment. Further, these groups were divided into two subgroups based on their CAD. RESULTS: Among the 703 patients with KD, the rate of non-responders to initial IVIG was 16.8%. Serum total bilirubin, platelet count, and neutrophil proportion were independent predictive parameters of unresponsiveness (p<0.05). CAD was found in 234 patients (33.3%) in the acute phase, and in 32 patients (4.6%) in the convalescent phase. Male gender, fever duration, serum C-reactive protein, and white blood cell count were related to CAD (p<0.05). CAD was detected more frequently in non-responders than in the responders (47.5% vs. 31.5%, p=0.001). Kobayashi, Egami, and Sano scoring systems applied to our study population reflected low sensitivities (28.0-33.9%). CONCLUSION: Several independent parameters were related to unresponsiveness to the initial IVIG or CAD. These parameters might be helpful in establishing more focused and careful monitoring of high-risk KD patients in Korea.

Comparison of Outcomes after Device Closure and Medication Alone in Patients with Patent Foramen Ovale and Cryptogenic Stroke in Korean Population.

PURPOSE: To compare the effectiveness of device closure and medical therapy in prevention of recurrent embolic event in the Korean population with cryptogenic stroke and patent foramen ovale (PFO). MATERIALS AND METHODS: Consecutive 164 patients (men: 126 patients, mean age: 48.1 years, closure group: 72 patients, medical group: 92 patients) were enrolled. The primary end point was a composite of death, stroke, transient ischemic attack (TIA), or peripheral embolism. RESULTS: Baseline characteristics were similar in the two groups, except age, which was higher in the medical group (45.3±9.8 vs. 50.2±6.1, p<0.0001), and risk of paradoxical embolism score, which was higher in the closure group (6.2±1.6 vs. 5.7±1.3, p=0.026). On echocardiography, large right-to-left shunt (81.9% vs. 63.0%, p=0.009) and shunt at rest/septal hypermobility (61.1% vs. 23.9%, p<0.0001) were more common in the closure group. The device was successfully implanted in 71 (98.6%) patients. The primary end point occurred in 2 patients (2 TIA, 2.8%) in the closure group and in 2 (1 death, 1 stroke, 2.2%) in the medical group. Event-free survival rate did not differ between the two groups. CONCLUSION: Compared to medical therapy, device closure of PFO in patients with cryptogenic stroke did not show difference in reduction of recurrent embolic events in the real world's setting. However, considering high risk of echocardiographic findings in the closure group, further investigation of the role of PFO closure in the Asian population is needed.

Inhaled iloprost for the treatment of patient with Fontan circulation.

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

Functional class and targeted therapy are related to the survival in patients with pulmonary arterial hypertension.

PURPOSE: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. MATERIALS AND METHODS: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9±14.5 years, 80% of female) were enrolled. RESULTS: Causes were congenital heart disease in 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6±45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, median survival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). CONCLUSION: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore, early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.

Efficacy of inhaled iloprost in cor pulmonale and severe pulmonary hypertension associated with tuberculous destroyed lung.

Chronic obstructive pulmonary disease (COPD) is one of the causes of cor pulmonale. Cor pulmonale patients with pulmonary hypertension have a significant lower survival rate than patients without. However, there is no conclusive treatment options in cor pulmonale and pulmonary hypertension associated with COPD until now. We report a patient with cor pulmonale and pulmonary hypertension associated with severe form of COPD and tuberculous destroyed lung who achieved marked clinical, functional and echocardiographic hemodynamic improvements with inhaled iloprost for six months.

Clinical Features of Symptomatic Meckel's Diverticulum in Children: Comparison of Scintigraphic and Non-scintigraphic Diagnosis.

PURPOSE: Meckel's diverticulum (MD) has various clinical manifestations, and diagnosis or selectection of proper diagnostic tools is not easy. This study was conducted in order to assess the clinical differences of MD diagnosed by scintigraphic and non-scintigraphic methods and to find the proper diagnostic tools. METHODS: We conducted a retrospective review ofthe clinical, surgical, radiologic, and pathologic findings of 34 children with symptomatic MD, who were admitted to Gachon University Gil Medical Center, Inha University Hospital, and The Catholic University of Korea, Incheon St. Mary's Hospital between January 2000 and December 2012. The patients were evaluated according to scintigraphic (12 cases; group 1) and non-scintigraphic (22 cases; group 2) diagnosis. RESULTS: The male to female ratio was 7.5: 1. The most frequent chief complaint was lower gastrointestinal (GI) bleeding in group 1 and nonspecific abdominal pain in group 2, respectively. The most frequent pre-operative diagnosis was MD in both groups. Red blood cell (RBC) index was significantly lower in group 1. MD was located at 7 cm to 85cm from the ileocecal valve. Four patients in group 1 had ectopic gastric tissues causing lower GI bleeding. The most frequent treatment modality was diverticulectomy in group 1 and ileal resection in group 2, respectively. CONCLUSION: To diagnose MD might be delayed unless proper diagnostic tools are considered. It is important to understand indications of scintigraphic and non-scintigraphic methods according to clinical and hematologic features of MD. Scintigraphy would be weighed in patients with anemia as well as GI symptoms.

The results of transcatheter occlusion of patent ductus arteriosus: success rate and complications over 12 years in a single center.

BACKGROUND AND OBJECTIVES: Percutaneous occlusion of patent ductus arteriosus (PDA) has become increasingly attractive with the evolution of devices and techniques. We reviewed results for percutaneous occlusion of PDA using various devices in a single center. SUBJECTS AND METHODS: A retrospective review was done for 118 consecutive procedures performed in 111 patients with PDA between January 1996 and December 2007. RESULTS: The median age of the patients was 4.5 years (0.9 to 60.3 years); body weight was 16.9 kg (6.8 to 74.7 kg). The median PDA diameter at the pulmonic end was 3.8 mm (0.7 to 10 mm); mean pulmonary artery pressure was 21.0 mmHg (7 to 60 mmHg). Complete occlusion occurred in 76/111 (68.4%) immediately after implantation and in 100/111 (90.0%) at one year of follow-up. Second procedures for residual shunts were done in 7 patients. After the year 2001, the complete closure rate was 95.2% compared to 71.4% before 2001. Complications associated with the procedure were left pulmonary artery narrowing (all <20 mmHg) in 14, arrhythmia in 2, and death in 1. CONCLUSION: Evolution of devices, cumulative experience, and health insurance covering the cost of devices have contributed to good outcomes in our center for percutaneous occlusion of PDA. Our results have improved over the years, particularly with the use of the Amplatzer duct occluder.

A case of refractory Kawasaki disease complicated by peripheral ischemia.

Kawasaki disease (KD), an acute systemic vasculitis that primarily affects infants and young children, is occasionally refractory to initial high-dose intravenous immunoglobulin (IVIG). Some patients with KD develop peripheral gangrene as a fatal complication, though this is rare. There has been no case reported in the literature of complicated peripheral gangrene that completely resolved without sequelae. Recently, we encountered a patient with KD which was refractory to IVIG, later developing peripheral gangrene. For the treatment of a peripheral gangrene dexamethasone, methotrexate, and an anticoagulant were administered, resulting in resolution of the gangrenous lesions without sequelae. Early diagnosis and treatment can reduce permanent sequelae and mortality from refractory KD complicated by peripheral ischemia.