Risk Stratification Models for Adults With Congenital Heart Disease

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Chylothorax after Surgery for Congenital Cardiac Disease: A Prevention and Management Protocol

Background: Chylothorax after congenital heart surgery is not an uncommon complication, and it is associated with significant morbidity. However, consensus treatment guidelines are lacking. To improve the treatment outcomes of patients with postoperative chylothorax, we implemented a standardized management protocol at Severance Hospital in September 2014. Methods: A retrospective review of patients treated at a single center was done. All corrective and palliative operations for congenital heart disease performed at our institution between January 2008 and April 2018 were reviewed. The incidence and treatment outcomes of postoperative chylothorax were analyzed. Results: The incidence of chylothorax was 1.9%. Sixty-one percent of the patients could be managed with a low-fat diet, while 28% of the patients required complete restriction of enteral feeding. Thoracic duct embolization was performed in 2 patients and chest tube drainage decreased immediately after the procedure. No patient required thoracic duct ligation or pleurodesis. After implementation of the institutional management protocol, the number of chest tube drainage days decreased (median, 24 vs. 14 days; p=0.45). Conclusion: Implementing a strategy to reduce postoperative chylothorax resulted in an acceptable incidence of postoperative chylothorax. Instituting a clinical practice protocol helped to curtail the treatment duration and to decrease the requirement for surgical treatment. Image-guided embolization of the thoracic duct is an effective treatment for postoperative chylothorax.ope

Improvement of fontan circulatory failure after conversion to total cavopulmonary connection

Background: By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. Material and Method: From October 1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0⁑7.0 years (range, 4.6∼26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4∼14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was III or IV in six patients. Paroxysmal atrial fibrillation (n=1), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). Result: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional tachyarrhythmia (n=1). Postoperative central venous pressure was lower than the preoperative value (17.9⁑3.5 vs. 14.9⁑1.0, p=0.049). Follow-up was complete in all patients and extended to 50.1 months (mean, 30.3⁑12.8 months). There was no late death. All patients were in NYHA class I or II. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure. PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. Conclusion: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.ope

Concomitant Right Ventricular Outflow Tract Cryoablation during Pulmonary Valve Replacement in a Patient with Tetralogy of Fallot

A 38-year-old female patient with a history of tetralogy of Fallot repair at 10 years of age underwent pulmonary valve replacement with a mechanical prosthesis, tricuspid annuloplasty, and right ventricular outflow tract cryoablation due to pulmonary regurgitation, tricuspid regurgitation, and multiple premature ventricular contractions with sustained ventricular tachycardia. After surgery, she had an uneventful postoperative course with arrhythmia monitoring. She was discharged without incident, and a follow-up Holter examination showed a decrease in the number of ventricular ectopic beats from 702 to 41.ope

Novel Therapeutic Effects of Pterosin B on Ang II-Induced Cardiomyocyte Hypertrophy

Pathological cardiac hypertrophy is characterized by an abnormal increase in cardiac muscle mass in the left ventricle, resulting in cardiac dysfunction. Although various therapeutic approaches are being continuously developed for heart failure, several studies have suggested natural compounds as novel potential strategies. Considering relevant compounds, we investigated a new role for Pterosin B for which the potential life-affecting biological and therapeutic effects on cardiomyocyte hypertrophy are not fully known. Thus, we investigated whether Pterosin B can regulate cardiomyocyte hypertrophy induced by angiotensin II (Ang II) using H9c2 cells. The antihypertrophic effect of Pterosin B was evaluated, and the results showed that it reduced hypertrophy-related gene expression, cell size, and protein synthesis. In addition, upon Ang II stimulation, Pterosin B attenuated the activation and expression of major receptors, Ang II type 1 receptor and a receptor for advanced glycation end products, by inhibiting the phosphorylation of PKC-ERK-NF-κB pathway signaling molecules. In addition, Pterosin B showed the ability to reduce excessive intracellular reactive oxygen species, critical mediators for cardiac hypertrophy upon Ang II exposure, by regulating the expression levels of NAD(P)H oxidase 2/4. Our results demonstrate the protective role of Pterosin B in cardiomyocyte hypertrophy, suggesting it is a potential therapeutic candidate.ope

The Pythagorean theorem reveals the inherent companion of cardiac ejection fraction

BACKGROUND: Quantification of ventricular performance requires a comprehensive metric which is manageable for patient care and clinical trials. Ejection fraction (EF) has been embraced as an attractive candidate. However, being a dimensionless ratio, EF has serious limitations. METHODS: We aim to identify what information is not recognized when limiting the volume-related analysis by exclusively relying on EF. This investigation applies the volume domain concept, relating end-systolic volume (ESV) to end-diastolic volume (EDV). This approach allows graphical identification of the information not covered by EF. Implications for atria, left ventricle (LV) and right ventricle (RV) are investigated in healthy individuals, and cardiac patient groups using various imaging modalities. RESULTS: The Pythagorean theorem indicates that the hypotenuse which relates any {EDV, ESV} combination to EF corresponds with the information not covered by the single metric EF. The impact of the recovered EF companion (EFC) is illustrated in healthy adults (N = 410, LV 2D echocardiography), heart transplant patients (N = 101, LV CT), individuals with heart failure (N = 197, biplane angiocardiography), for the RV with corrected Fallot (N = 124, MRI), diameters for left atrium (N = 49, MRI) and area for right atrium (N = 51, MRI). For any limited EF range we find a spectrum of EFC values, showing that the two metrics contain (partly) independent information, and emphasizing that the sole use of EF only partially conveys the full information available. CONCLUSIONS: The EFC is a neglected companion, containing information which is additive to EF. Analysis based on ESV and EDV is preferred over the use of EF.ope

Fate of Regurgitation of Left Atrioventricular Valve Following Repair of Atrioventricular Septal Defect

The purpose of this study was to evaluate the fate of left atrioventricular valve regurgitation(LAVVR) following repair of complete atrioventricular septal defects (AVSDs). Material and Method: Between July 1984 and March 2002, repair of complete AV defects were performed in 77 patients. Mean age at surgery was 30.23±69.11 months (range 1 to 456). Echocardiograms of all survivors after isolated AVSDs correction were reviewed. LAVVR were evaluated with color doppler echocardiography in 64 survival periodically. On each study, LAVVR severity was graded on a 1 to 4 scale, based upon the size of the regurgitated jet. Result: Mild deterioration of LAVV function was fairly common. LAVVR severity increased by >1 grade in 19 patients (30.2%) during the course of the study. However, the deterioration in LAVVR function occurred primarily between 12 and 24 months postoperatively. After the initial 24 postoperative months, LAVVR worsened on only 8 occasions and in each instance worsened by only 1 grade. Deterioration more than 3+ LAVVR occurred in only 3 patients. And deterioration to 4+ LAVVR was not observed after the initial 24 postoperative months but one. Survival curve analysis predicted a 88.2% of ten-year freedom rate from development of 4+ LAVVR after initial operation of complete AVSDs. Conclusion: Postoperative LAVVR remains fairly stable following AVSDs repair, Serious deterioration is rare after 24 postoperative months, especially after the initial 48 postoperative months. But serial follow-up study with echocariogram was need till 24 postoperative months after repair of complete AVSDs.ope

The Development of Medical Record Items: a User-centered, Bottom-up Approach

OBJECTIVES: Clinical documents (CDs) have evolved from traditional paper documents containing narrative text information into the electronic record sheets composed of itemized records, where each record is expressed as an item with a specific value. We defined medical record (MR) items to be information entities with a specific value. These entities were then used to compile form-based clinical documents as part of an electronic health record system (EHR-s). METHODS: We took a reusable bottom-up developmental approach for the MR items, which provided three things: efficient incorporation of the local needs and requirements of the medical professionals from various departments in the hospital, comprehensive inclusion of the essential concepts of the basic elements required in clinical documents, and the provision of a structured means for meaningful data entry and retrieval. This paper delineates our experiences in developing and managing medical records at a large tertiary university hospital in Korea. RESULTS: We collected 63,232 MR items from paper records scanned into 962 CDs. The MR item database was constructed using 13,287 MR items after removing redundant items. During the first year of service users requested changes to be made to 235 (1.8%) attributes of the MR items and also requested the additional 9,572 new MR items. In the second year, the attributes of 70 (0.5%) of the existing MR items were changed and 3,704 new items were added. The number of registered MR items increased by 72.0% in the first year and 27.9% in the second year. CONCLUSIONS: The MR item concept provides an easier and more structured means of data entry within an EHR-s. By using these MR items, various kinds of clinical documents can be easily constructed and allows for medical information to be reused and retrieved as data. The success of the use of MR items in a large tertiary university hospital system provides evidence that verifies our approach as being an efficient means of user-oriented and structured data entry, enabling the easy reuse of medical records.ope

Overexpression of transforming growth factor-beta 1 in the valvular fibrosis of chronic rheumatic heart disease

For the purpose of determining the pathogenic role of transforming growth factor-beta1 (TGF-beta 1) in the mechanism of chronic rheumatic heart disease, we evaluated the expression of TGF-beta 1, proliferation of myofibroblasts, and changes in extracellular matrix components including collagen and proteoglycan in 30 rheumatic mitral valves and in 15 control valves. High TGF-beta 1 expression was identified in 21 cases (70%) of rheumatic mitral valves, whereas only 3 cases (20%) of the control group showed high TGF-beta 1 expression (p<0.001). Additionally, increased proliferation of myofibroblasts was observed in the rheumatic valves. High TGF-beta1 expression positively correlated with the proliferation of myofibroblasts (p=0.004), valvular fibrosis (p<0.001), inflammatory cell infiltration (p=0.004), neovascularization (p=0.007), and calcification (p<0.001) in the valvular leaflets. The ratio of proteoglycan to collagen deposition inversely correlated with TGF-beta 1 expression in mitral valves (p=0.040). In conclusion, an ongoing inflammatory process, the expression of TGF-beta 1, and proliferation of myofibroblasts within the valves have a potential role in the valvular fibrosis, calcification, and changes in the extracellular matrix that lead to the scarring sequelae of rheumatic heart disease.ope

Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair

Repair of tetralogy of Fallot (TOF) has shown excellent outcomes. However it leaves varying degrees of residual hemodynamic impairment, with severe pulmonary stenosis (PS) and free pulmonary regurgitation (PR) at both ends of the spectrum. Since the 1980s, studies evaluating late outcomes after TOF repair revealed the adverse impacts of residual chronic PR on RV volume and function; thus, a turnaround of operational strategies has occurred from aggressive RV outflow tract (RVOT) reconstruction for complete relief of RVOT obstruction to conservative RVOT reconstruction for limiting PR. This transformation has raised the question of how much residual PS after conservative RVOT reconstruction is acceptable. Besides, as pulmonary valve replacement (PVR) increases in patients with RV deterioration from residual PR, there is concern regarding when it should be performed. Regarding residual PS, several studies revealed that PS in addition to PR was associated with less PR and a small RV volume. This suggests that PS combined with PR makes RV diastolic property to protect against dilatation through RV hypertrophy and supports conservative RVOT enlargement despite residual PS. Also, several studies have revealed the pre-PVR threshold of RV parameters for the normalization of RV volume and function after PVR, and based on these results, the indications for PVR have been revised. Although there is no established strategy, better understanding of RV mechanics, development of new surgical and interventional techniques, and evidence for the effect of PVR on RV reverse remodeling and its late outcome will aid us to optimize the management of TOF.ope