Permanent pacemaker implantation in post one-and-a-half ventricle repair with tricuspid valve replacement in Ebstein anomaly: An innovative technique.

INTRODUCTION: Transvenous pacemaker implantation in patients post bidirectional Glenn anastomosis in one-and-a-half ventricle repair is usually not feasible. However, with a modified surgical technique for Glenn anastomosis and a combined interventional and electrophysiologic approach, the transvenous pacemaker was successfully implanted. FINDINGS AND CONCLUSIONS: We reported a novel technique of pacemaker implantation in a 27-year-old woman, underlying Ebstein anomaly of the tricuspid valve, who developed intermittent complete atrioventricular block at 5 years after surgical repair. The patient had a tricuspid valve replacement and a novel modified bidirectional Glenn anastomosis for one-and-a-half ventricle repair. The Glenn circuit was conducted by opening a window between the posterior wall of the superior vena cava (SVC) and the anterior wall of the right pulmonary artery (RPA), combined with putting a Goretex membrane in the SVC below the SVC-RPA window without disconnecting the SVC from the right atrium. The transvenous pacemaker was implanted by perforating the Goretex membrane, then passing the leads from the axillary vein through the perforated membrane and placing them in the coronary sinus and right atrium.

Effect of Balloon Pulmonary Valvuloplasty on Growth of Pulmonary Annulus in Infants with Tetralogy of Fallot.

Percutaneous balloon pulmonary valvuloplasty (PBPV) is an alternative intervention in infants with Tetralogy of Fallot (TOF). It can not only improve hypoxia but also promote pulmonary annulus (PA) growth. In this study, we evaluated the effect of PBPV on PA growth in infants with TOF. To eliminate the effect of the systemic to pulmonary shunt (SPS) that may promote PA growth, we divided TOF infants into 2 groups: group A, patients who underwent PBPV with or without other SPS, and group B, patients who attempted SPS but without PBPV. Sixty patients were included, 28 patients in group A and 32 patients in group B. Age at the time of intervention in group A (range, 0.4-5.4; median 1.4 months) was lower than that in group B (range, 2.3-7.7; median 4.8 months), p-value 0.02. The body weight in group A (range, 3-5.5; median 3.7 kg) was also lower than that in group B (range 4.1-6.4; median 5.9 kg), p-value 0.02. Echocardiographic data at the mean follow-up period of 37.2 months (3-88 months) in group A and 39.6 months (6-95 months) in group B demonstrated an increase in mean PA diameter from 5.0 ± 1.3 mm to 10.2 ± 2.9 mm, p-value < 0.001 in group A; and from 6.2 ± 2 mm to 9.5 ± 2.9 mm, p-value < 0.001 in group B. The median PA z-score increased from - 3.4SD (- 3.9 to - 2.6SD) to - 1.8SD (- 2.5 to - 0.8SD), with the p-value of 0.002 in group A; and increased from - 2.9SD (- 4.5 to - 1.3SD) to - 2.7SD (- 3.6 to - 1.4SD), with the p-value of 0.73 in group B. By using the PA z-score as the absolute value, there was a statistically significant increase in the PA z-score during follow-up in group A, but not in group B. Balloon pulmonary valvuloplasty in infants with TOF can facilitate the growth of the pulmonic annulus even after eliminating the effect of the systemic to pulmonary shunt.

Outcomes of duct stenting and modified Blalock-Taussig shunt in cyanotic congenital heart disease with duct-dependent pulmonary circulation.

BACKGROUND: Transcatheter ductus arteriosus stenting (DS) is emerging as an alternative method to modified Blalock-Taussig shunt (MBTS) in providing pulmonary blood flow in cyanotic congenital heart disease (CCHD) with duct-dependent pulmonary circulation. OBJECTIVE: To evaluate post-procedural outcomes and survival between patients undergoing DS and MBTS. METHODS: All infants ≤ 60 days of age having CCHD with diminished pulmonary blood flow who underwent palliative procedure either with MBTS or DS at King Chulalongkorn Memorial Hospital during January 1st, 2013 and December 31th, 2017 were retrospectively reviewed. RESULTS: 98 patients were included; 34 patients underwent a transcatheter DS and 64 patients underwent MBTS. There was no significant difference in post-procedural outcomes and overall mortality rate between two groups (17.6% in MBTS group and 6.1% in DS group, p = 0.09). Single ventricle morphology was the major risk factor associated with increased mortality compared with biventricular morphology (aHR 3.9, 95% CI 1.49-10.2, p = 0.01). There was similar number of early and pre-repair additional interventions focusing on MBTS/DS patency between two groups. The MBTS group had a greater number of early interventions on PA branch stenosis related to baseline diagnosis. Risk factors associated with additional intervention were pre-existing pulmonary branch stenosis (aHR 2.54, 95% CI 1.3-4.97, p = 0.006) and body weight less than 2.5 kg (aHR 3.33, 95% CI 1.57-7.08, p = 0.003). Having pulmonic valve perforation or balloon pulmonary valvuloplasty to promote antegrade pulmonary blood flow could result in a lower number of additional interventions required before definitive repair. CONCLUSION: Duct stenting is a feasible and safe alternative to MBTS in cyanotic infants with duct-dependent pulmonary circulation. However, mortality rate was significantly higher in patients with single ventricle that required careful follow-up after procedure.

Pulmonary cysticercosis: A case report of abnormal lung nodule based on chest computerized tomography.

Pulmonary cysticercosis is one of the rare complications of cysticercosis that occurs worldwide, especially in developing countries. Its clinical presentation can range from asymptomatic, pleural effusion, and constitutional symptoms to incidental pulmonary nodule. Here, we report pulmonary cysticercosis detected as a pulmonary nodule in computed tomography. Positron emission tomography revealed that the nodule was non-FDG avidity. However, the slow-growing pattern of the nodule raised a concern to the clinician to achieve definite diagnosis by surgical biopsy. Histopathological examination revealed cysticercosis. The patient was treated with praziquantel.

Prognostic factors in completely resected lymph-node-negative pulmonary adenocarcinoma.

Background: Lymph node involvement is one of the important prognostic factors for early-stage lung cancer. However, in lymph node-negative (N0) lung cancer the recurrent rate may be as high as 30%. We aimed to study potential prognostic factors including clinicopathological factors and epidermal growth factor receptor (EGFR) mutation status in this lung cancer population. Methods: We retrospectively reviewed the medical records and pathological examinations of patients with completely resected N0 pulmonary adenocarcinoma treated in our institute between 2009 and 2016. We used Cobas® test to determine EGFR mutation status. Recurrence-free survival (RFS) was analyzed by univariable and multivariable Cox regression analyses. Results: We recruited 220 patients with median duration of follow up 5 years. Majority of these patients were in stage I (80%) and did not receive adjuvant therapy (86%). There were 53% with EGFR mutations which comprised of exon 19 deletion 51% and L858R 43%. Recurrence occurred in 64 out of 220 patients (29%). The median time to recurrence was 2.1 years. Statistically significant prognostic factors in both univariate and multivariate analyses included tumor size ≥4 centimeter (cm) (HR: 1.94; 95% CI: 1.03-3.67), visceral pleural invasion (HR: 2.53; 95% CI: 1.34-4.79), tumor necrosis (HR: 2.45; 95% CI: 1.13-5.31) and bronchial resection margin <2 cm (HR: 1.96; 95% CI: 1.10-3.51). However, presence of sensitizing EGFR mutation was not found to be a significant prognostic factor (HR: 1.20; 95% CI: 0.66-2.18; P=0.56). Conclusions: In N0 surgically resected lung adenocarcinoma, there were significant pathological prognostic factors including tumor 4 cm or more, visceral pleural invasion, tumor necrosis and bronchial resection margin less than 2 cm. Mutation of EGFR is not a significant prognostic factor to determine the risk of recurrence in this population and their risks shall be determined by the other poor prognostic factors.

Long-term course after pediatric right ventricular outflow tract reconstruction.

OBJECTIVES: Valved homografts are commonly used for right ventricular outflow tract reconstruction. However, despite good early results, they lack durability. This study was designed to compare single-center results of implantation of 3 types of right ventricular outflow tract conduit, in terms of patient survival, graft failure, reoperation, and risk factors for reoperation. METHODS: One hundred and forty-three pediatric patients who underwent right ventricular outflow tract conduit implantation between January 2006 and December 2018 were reviewed. We stratified conduits by aortic, pulmonic homograft, and Contegra; 74 aortic homografts, 61 pulmonic homografts, and 8 Contegra conduits were implanted. Median age at implantation was 3 years. The primary diagnosis was truncus arteriosus in 41.3%. We analyzed the role of sex, age, diagnosis, and graft size. Endpoints included freedom from graft failure, freedom from reoperation, and survival. RESULTS: The survival rate was 83.2% at 10 years. Freedom from graft failure at 2, 5, and 10 years was 100%, 97.9%, and 63.4%, respectively. Freedom from reoperation was 85.8% for pulmonic homografts and 74.9% for aortic homografts at 10 years, and 100% for Contegra at 6 years. Multivariable analysis identified conduit diameter <18 mm as a risk factor for reoperation (hazard ratio: 3.16, 95% confidence interval: 1.38-7.23, p = 0.007). CONCLUSION: Homograft valves used for right ventricular outflow tract reconstruction provided excellent long-term durability and late survival. The only factor that adversely affected graft longevity was small graft size (diameter <18 mm). Reoperation for conduit failure was not significantly different among the groups.

Endomyocardial Biopsy and Prevalence of Acute Cellular Rejection in Heart Transplantation.

BACKGROUND: Percutaneous endomyocardial biopsy (EMB) remains the criterion standard method for surveillance of allograft rejection after heart transplant (HT). However, data regarding utility of EMBs and prevalence of acute cellular rejection (ACR) in Asian populations are still limited. We aimed to report our experience in the use of EMBs and prevalence of ACR in HT recipients. METHODS: We retrospectively evaluated all EMBs from consecutive HT recipients between January 2008 and December 2018. EMB pathology results were according to International Society for Heart and Lung Transplantation 2004 revision of biopsy grading. We also divided patients into previous era and current era group (underwent HT before and after 2015) to compare prevalence of ACR and survival outcome. RESULTS: A total of 832 EMBs from 81 HT recipients were included. Pathologic reports revealed ACR grade 1R 22.8%, 2R 4.2%, and 3R 0.6%. At patient level, at least 1 episode of ACR grade 1R, 2R, and 3R were found in 70.6%, 24.7%, and 3.5% of the patients, respectively. When compared between era, frequency of EMB during the first year after HT in current era was significantly higher (9.74 ± 3.38 vs 4.93 ± 3.29, P < .001), but lower frequency of rejection grade ≥ 2R were found (2.3% vs 8.1%, P < .001). However, 1-year survival was not statistically different (76% in previous era vs 80% in current era, P = .37). CONCLUSIONS: From our study, prevalence of grade ≥ 2R rejection was approximately 5%, which is comparable with previous studies. Further studies are needed to evaluate proper interval and number of EMBs in HT recipients.

Balloon pulmonary valvuloplasty in tetralogy of Fallot: effects on growth of pulmonary annulus and transannular patch.

BACKGROUND: Balloon pulmonary valvuloplasty and its benefit on the growth of pulmonary annulus and pulmonary artery in tetralogy of Fallot (TOF) patients remains controversial. The purpose ofthe present study was to determine the growth of pulmonary valve annulus and pulmonary artery and to evaluate the need of transannular patch during total surgical correction in patients with tetralogy of Fallot (TOF) after balloon pulmonary valvuloplasty. MATERIAL AND METHOD: Fifty-one severely hypoxic TOF patients, who underwent balloon dilatation ofpulmonary valve, were included in the present study. The clinical outcomes, oxygen saturation, and echocardiographic parameters before and after balloon dilatation were analyzed. RESULTS: There were 33 males and 18females. The mean age was 3 years 5 months old (range, I month-15 years 8 months old). The mean oxygen saturation increasedfrom 73.9 +/- 9.1 to 84.8 +/- 6.7% immediately after the procedure (p-value < 0.05). There was no serious procedural-related complication. At the mean follow-up period of 2 years and 4 months, the mean Z-score of pulmonary annulus size increased from -2.56 SD to -1.87 SD (p-value < 0.05) and the right pulmonary artery size from -0.29 SD to + 0.46 SD (p-value < 0.05). Thirty-seven patients (66.1%) underwent corrective surgery. Pulmonary transannular patch was performed in 11 of37 patients (29.7%). CONCLUSION: Pulmonary valve dilatation in patients with TOF is safe. It promotes the growth ofthe pulmonary valve annulus and pulmonary artery and may decrease the need of transannular patch at the time of surgical correction.

Structurally Abnormal Myocardium Underlies Ventricular Fibrillation Storms in a Patient Diagnosed With the Early Repolarization Pattern.

OBJECTIVES: The aim of this study was to investigate the mechanism underlying QRS-slurring in a patient with the early repolarization pattern in the electrocardiogram (ECG) and ventricular fibrillation (VF) storms. BACKGROUND: The early repolarization pattern refers to abnormal ending of the QRS complex in subjects with structurally normal hearts and has been associated with VF. METHODS: We studied a patient with slurring of the QRS complex in leads II, III, and aVF of the ECG and recurrent episodes of VF. Echocardiographic and imaging studies did not reveal any abnormalities. Endocardial mapping was normal but subxyphoidal epicardial access was not possible. Open chest epicardial mapping was performed. RESULTS: Mapping showed that the inferior right ventricular free wall activated the latest with local J-waves in unipolar electrograms. The last moment of epicardial activation concurred with QRS-slurring in the ECG whereas the J-waves in the local unipolar electrograms occurred in the ST-segment of the ECG. Myocardial biopsies obtained from the late activated tissue showed severe fibrofatty alterations in the inferior right ventricular wall where fractionation and local J-waves were present. After ablation, the early repolarization pattern in the ECG disappeared and arrhythmias have been absent since (follow-up 18 months). CONCLUSIONS: In this patient, the electrocardiographic early repolarization pattern was caused by late activation due to structurally abnormal myocardium. The late activated areas were marked by J-waves in local electrograms. Ablation of these regions prevented arrhythmia recurrence and normalized the ECG.

Recurrent Cardiac Myxoma Treated by Orthotopic Heart Transplantation: A Case Report and Literature Review of Heart Transplantation for Primary Cardiac Tumor.

Primary cardiac myxoma is the most common primary cardiac tumor. Tumor resection is the treatment of choice and overall long-term prognosis is good and recurrence is rare. This report presents a case of a young girl who presented with multiple recurrent cardiac myxoma. She underwent 3 sternotomy surgeries of 3 separated episodes of cardiac myxoma resection. On the fourth recurrence, the patient underwent orthotopic heart transplant. The patient tolerated the procedure well and is alive 6 months after the procedure with NYHA class I. We reviewed evidences and summarized reported cases of orthotopic heart transplant operation for primary cardiac tumor in the literature.

Thrombocytopenia in Moderate- to High-Risk Sutureless Aortic Valve Replacement.

BACKGROUND: This study aimed to compare preliminary data on the outcomes of sutureless aortic valve replacement (SU-AVR) with those of aortic valve replacement (AVR). METHODS: We conducted a retrospective study of SU-AVR in moderate- to high-risk patients from 2013 to 2016. Matching was performed at a 1:1 ratio using the Society of Thoracic Surgeons predicted risk of mortality score with sex and age. The primary outcome was 30-day mortality. The secondary outcomes were operative outcomes and complications. RESULTS: A total of 277 patients were studied. Ten patients (50% males; median age, 81.5 years) underwent SU-AVR. Postoperative echocardiography showed impressive outcomes in the SU-AVR group. The 30-day mortality was 10% in both groups. In our study, the patients in the SU-AVR group developed postoperative thrombocytopenia. Platelet counts decreased from 225×103/µL preoperatively to 94.5, 54.5, and 50.1×103/µL on postoperative days 1, 2, and 3, respectively, showing significant differences compared with the AVR group (p=0.04, p=0.16, and p=0.20, respectively). The median amount of platelet transfusion was higher in the AVR group (12.5 vs. 0 units, p=0.052). CONCLUSION: There was no difference in the 30-day mortality of moderate- to high-risk patients depending on whether they underwent SU-AVR or AVR. Although SU-AVR is associated with favorable cardiopulmonary bypass and cross-clamp times, it may be associated with postoperative thrombocytopenia.

Clinical course and outcome of children with single ventricle physiology at King Chulalongkorn Memorial Hospital.

BACKGROUND: Children with single ventricle physiology comprise 10% of all children with congenital heart disease (CHD) and one-third of children with cyanotic CHD seen at King Chulalongkorn Memorial Hospital. The prognosis of these children is generally thought to be poor but no study of the outcome has previously been done in this hospital and in Thailand. OBJECTIVES: To study the clinical course and outcome of children with single ventricle physiology at King Chulalongkorn Memorial Hospital during the current era. MATERIAL AND METHOD: One hundred and seventeen patients with single ventricle physiology were initially seen at this hospital during the year 1999-2001. Retrospective chart reviews were carried out in 90 children with available medical records. The status of the patients was determined in 2003 at clinic visits, by phone calls and mail. RESULTS: The main diagnoses were tricuspid atresia (TA, n = 10), pulmonary atresia with intact ventricular septum (PA/IVS, n = 16), single ventricle associated with cardiac malposition or heterotaxy syndrome (malposition, n = 35), hypoplastic left heart syndrome (HLHS, n = 11) and other complex single ventricle (others, n = 18). Most children did not have other major anomalies. Survival of patients with TA and PA/IVS was approximately 92 and 87% at 1 and 4 years, respectively. For patients in the other three groups, 1 and 4 year survival was 69 and 42%, respectively. Patients with HLHS had the worse survival, partly because of decisions not to pursue further treatments by the parents. Among HLHS patients who underwent Norwood procedure, the 1 and 4 year survival were 83% and 42%, respectively. Approximately 40 and 90% of all patients underwent heart surgery at 1 and 4 years follow-up, respectively. CONCLUSION: Despite limited resource, the short-term outcome of Thai children with single morphologic left ventricle (TA and PA/IVS) is reasonably good. Timely evaluation and treatment of these children toward Fontan procedure should be ensured. After proper discussion with the parents, palliative care is still acceptable for patients with single morphologic right ventricle or other complex single ventricle due to poor survival in the current era. As access to health care improves, re-evaluation of these outcomes is necessary to find the best strategy for the management of these patients.

Myocardial revascularization with the posterior tibial artery.

A large number of coronary artery bypass grafts are performed in Thailand. Some patients develop restenosed coronary arteries or stenosed graft conduits. Great saphenous veins, internal thoracic arteries, radial arteries, and right gastroepiploic arteries are used for redo coronary artery bypass grafting. But even with many conduits to choose from, sometimes graft conduits are not available. We report a case of redo coronary artery bypass grafting where the posterior tibial artery was harvested for the graft conduit. Clinical outcome and angiographic results are reported at 1 year postoperation.

Single midline approach for permanent pacemaker implantation in children.

Permanent pacemaker implantation was carried out in 12 children with a mean age of 1.4 years (range, 2 days to 4 years) and a mean weight of 8.6 kg (range, 2.4-13.6 kg), using a single midline approach for placement of both the epicardial lead and the pacemaker generator (intermuscular abdominal implantation). Platinized porous-tipped steroid-eluting epicardial leads were used in all patients. The pacemakers worked well, and there was no early postoperative complication. This technique was found to be rapid, simple, and safe in children, especially neonates and infants.

Bicuspidized pulmonary homograft for truncus arteriosus repair.

Primary repair is preferable to palliation in infants with truncus arteriosus. At our institute, an appropriately small homograft valved conduit is not available for every patient; a bicuspidized pulmonary valve homograft is an alternative. Between December 1996 and August 2005, 24 patients aged 28 days to 21 months with truncus arteriosus underwent primary repair with a homograft valved conduit; bicuspidized homografts were used in 15 of them. In the 18 (75%) patients who survived to hospital discharge, 5-year survival was 94% (75% for tricuspid homografts and 100% for bicuspidized homografts, which was not significantly different). Freedom from reoperation or balloon angioplasty in all 18 survivors was 89% at 5 years. Freedom from reoperation in tricuspid and bicuspidized homograft groups at 5 years was 67% and 100%, respectively; the difference was not statistically significant. Bicuspidized homografts worked as well as tricuspid conduits in the intermediate term. The remodeled homografts showed excellent hemodynamic characteristics and appear to be a reasonable alternative when an appropriate size of valved homograft is unavailable.

Roxithromycin induced torsade de pointes in a patient with complex congenital heart disease and complete atrioventricular block.

We reported a 6-year-old girl who developed torsade de pointes (TdP) after taking roxithromycin. The patient had congenital complete heart block and complex cyanotic heart disease. Before taking roxithromycin, her corrected QT interval (QTc) was 0.39 second with a ventricular rate of 55-60 beats/min. Repetitive bursts of TdP with prolonged QT interval (QTc = 0.55 s) developed after taking roxithromycin. After stopping the medication, her QT interval normalized (QTc = 0.38 s). This case demonstrated the potential of roxithromycin in causing TdP especially in a patient with other risk factors for prolong QT interval and TdP.