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Cases of Fontan failure with normal Fontan pressure have been reported. This study aimed to identify catheterisation-derived haemodynamic predictors of heart transplantation/death, other than Fontan pressure, in late post-Fontan patients.
Methods:
This retrospective study evaluated post-Fontan patients who underwent cardiac catheterisation at age ≥10 years between 1993 and 2018. The predictive effect of cardiac index-systemic vascular resistance index plot and perfusion pressure on freedom from the primary outcome (heart transplantation/death) was evaluated. Patients were categorised into haemodynamic categories A (cardiac index ≥ 3, systemic vascular resistance index ≥ 13), B (cardiac index < 3, systemic vascular resistance index ≥ 13), C (cardiac index ≥ 3, systemic vascular resistance index < 13), and D (cardiac index < 3, systemic vascular resistance index < 13).
Results:
In total, 79 patients (median age: 15.7 [range: 10.1–50.2] years) were included; of them, the primary endpoint occurred in 10 (13%; median follow-up: 1.9 [range: 0.1–18.8] years). Category C patients had significantly shorter freedom from the endpoint than categories A and B patients. Univariate analysis identified significant haemodynamic predictors, including Fontan pressure, pulmonary/systemic vascular resistance index, pulmonary/systemic flow, systemic arterial oxygen saturation, systemic venous oxygen saturation, systemic vascular resistance index, perfusion pressure, perfusion pressure < 53 mmHg, and category C. In multivariable analysis, perfusion pressure < 53 mmHg and category C emerged as predictors of heart transplantation/death alongside Fontan pressure.
Conclusion:
Haemodynamic profiling of late post-Fontan patients using the cardiac index-systemic vascular resistance index plot can aid to comprehend the post-Fontan status and predict clinical prognosis.
Discussing end-of-life (EOL) issues with patients remains challenging for health professionals. Physicians may use various expressions, including euphemistic ones, when disclosing the prognosis to their patients to reduce their psychological impact. However, the actual expressions of EOL disclosure in clinical practice are unclear. This study aims to investigate the expressions used in EOL disclosures and explore their associated factors.
Methods
A retrospective chart review was conducted enrolling all the patients who died in a university-affiliated hospital. Expressions used in the EOL disclosure were qualitatively analyzed. The patients’ participation rate and length from the discussion to death were investigated.
Results
EOL disclosures were observed in 341 of 358 patients. The expressions used by the physicians were categorized into 4 groups; Group 1: Clear presentation of life expectancy (n = 106; 31.1%), Group 2: Euphemistic presentation of life expectancy (n = 24; 7.0%), Group 3: Presentation of risk of sudden death (n = 147; 43.1%), Group 4: No mention on life expectancy (n = 64; 18.8%). The proportion of male patients was higher in Group 2 (79%) and lower in Group 4 (56%). Patients with cancer accounted for approximately 70% of Groups 1 and 4, but only approximately 30% of Group 3. The patient participation rate was highest in Group4 (84.4%), followed by Group 2 (50.0%). The median time from EOL disclosure to death was longer in Groups 1 and 4 (26 and 29.5 days, respectively), compared to Groups 2 and 3 (18.5 and 16 days, respectively).
Significance of results
A variety of expressions are used in EOL disclosure. Patterns of communication are influenced by patients’ gender and type of illness (cancer or noncancer). Euphemisms do not seem to facilitate timely disclosure of life expectancy or patient participation. For health professionals, not only devising the expressions to alleviate their patients’ distress when breaking bad news but also considering the communication process and patient background are essential.
Epidermal growth factor receptor (EGFR)-mutant non-small cell lung cancer (NSCLC) is a clinically important driver alteration affecting approximately one-third of lung cancer patients. Treatments for EGFR-exon 19 deletion and exon 21 L858R NSCLC have evolved over the last decade from first-generation reversible tyrosine kinase inhibitors (TKI) to third-generation irreversible TKIs, of which osimertinib has been the widely accepted as first-line therapy. Despite survival improvement seen with osimertinib and its efficacy against acquired T790M mutation, resistance through on-target and off-target pathways eventually develop. This Element describes the structural biology and pathophysiology of EGFR-mutant NSCLC and discusses past, current, and future treatment options in the metastatic, neoadjuvant, and adjuvant settings. It describes the biology and recently approved treatment for EGFR-exon 20 insertion mutation and the treatment for the uncommon exon 18 (G719X), 20 (S768I), and 21 (L861Q) mutations. It also outlines the promising clinical applications of circulating tumor DNA (ctDNA).
The Central Molecular Zone (CMZ) in the Galactic Center region shows outstanding non-circular motion unlike the Galactic disk. While several models describing this non-circular motion have been proposed, a uniform kinematic model of the CMZ orbit has not yet emerged. To uncover the dynamics of the Galactic center region, we conducted VLBI astrometric observations of 22GHz water maser sources towards the Galactic center using VERA. By measuring parallaxes and proper motions, we can determine whether each source is actually located in the CMZ or not, and identify the three-dimensional positions and velocities in the non-circular orbit if the source is indeed located in the CMZ. We present the results of our astrometric study for several maser sources associated with molecular clouds towards the Galactic center. The astrometric observations toward Sgr B2(M) indicated that Sgr B2 complex is moving toward the positive Galactic longitude relative to Sgr A*.
VERA has been regularly conducting astrometry of Galactic maser sources for ∼ 20 years, producing more than 100 measurements of parallaxes and proper motions of star-forming regions as well as AGB stars. By combining the observational results obtained by VLBA BeSSeL, EVN, and LBA, maser astrometry provides a unique opportunity to explore the fundamental structure of the Galaxy. Here we present the view of the Galaxy revealed by the maser astrometry, and also discuss the importance of maser astrometry in the era of GAIA by comparing the results obtained by VLBI and GAIA. We also present our view of “proper motions toward the future” of the relevant field, expected in the next decade based on global collaborations.
Transfemoral venous approach is the standard method for transcatheter patent ductus arteriosus closure using the Amplatzer Piccolo patent ductus arteriosus occluder in small infants. We report a 1.4 kg infant who underwent transcatheter Piccolo patent ductus arteriosus closure. Transjugular venous approach was taken due to the unexpected finding of interrupted inferior vena cava.
Neonatal aortic thrombus is a rare and critical condition that can present mimicking severe coarctation of the aorta or interrupted aortic arch. Transcatheter thrombectomy for this lesion has not been well described. We report a premature neonate with an occlusive proximal descending aorta thrombus, who underwent transcatheter mechanical thrombectomy using an Amplatzer Piccolo PDA occluder (Abbott, North Chicago, IL, USA). The procedure was successful with no subsequent distal thromboembolic events.
Pericardiocentesis is the invasive percutaneous procedure for acute and chronic excessive accumulation of pericardial fluid. There is a paucity of data on the effectiveness and safety of pericardiocentesis in children.
Objectives:
To evaluate the effectiveness and safety of pericardiocentesis and factors associated with acute procedural failure and adverse events.
Methods:
This was a single-centered retrospective study to describe all the children aged ≤20 years who underwent pericardiocentesis. Data on demographics, etiologies of pericardial effusion, and repeat intervention at follow-up were collected.
Results:
A total of 127 patients underwent 153 pericardiocentesis. The median age was 6.5 years (1 day–20 years) with weight of 17 kg (0.5–125). Most common etiology was post-pericardiotomy syndrome (n = 56, 44%), followed by infectious (12%), malignant (10%), and iatrogenic (9%). Pericardiocentesis was performed more commonly in the catheterisation laboratory (n = 86, 59%). Concurrent pericardial drain placement was performed in 67 patients (53%). Acute procedural success was 92% (141/153). Repeat intervention was performed in 33 patients (22%). The incidence of adverse events was 4.6% (7/153): hemopericardium requiring emergent surgery (n = 2); hemopericardium with hypotension (n = 2); seizure with anesthesia induction (n = 1); and right ventricle puncture with needle (n = 2). Pericardiocentesis at the bedside had a higher rate of acute procedural failure than that in the catheterisation lab (17 versus 1%, p < 0.01). No identifiable risk factors were associated with adverse events.
Conclusions:
Pericardiocentesis was life-saving in children with its high effectiveness and safety even in urgent situations. Although initial pericardiocentesis was effective, one of five patients required re-intervention for recurrent pericardial effusion.
Membranous ventricular septal aneurysm is a known entity but rarely causes severe right ventricular outflow obstruction. We report a 40-year-old female with trisomy 18 who developed severe right ventricular outflow obstruction caused by an enormous membranous septal aneurysm associated with unrepaired inlet ventricular septal defect with perimembranous extension.
Transcatheter device closure of an atrial septal defect (ASD) may require an additional supportive technique in challenging cases. We report a 15-year-old male with moderate-sized ASD and severe scoliosis. In spite of adequate positioning of the Gore Cardioform ASD occluder, the device was pulled into the right atrium by a retrieval cord due to the acute release of strong tension between the delivery catheter and its device upon locking. This phenomenon was prevented by the use of Mullins sheath, resulting in a successful release of the device. The use of a Mullins sheath may be considered to deliver a Gore Cardioform ASD device in selected cases.
Total anomalous pulmonary venous connection is a rare congenital heart defect. We report an infant with a mixed form of supracardiac TAPVC, in whom all pulmonary veins, except the right upper, entered a pulmonary venous confluence that is connected to a vertical vein and drained into the superior vena caval–right atrial junction. Several segmental right upper pulmonary veins entered the superior vena cava, superior to the entry of the vertical vein. Surgical repair consisted of the Warden procedure combined with direct anastomosis of the vertical vein to the left atrium. Separate pulmonary venous drainage pathways decreased the risk of post-operative pulmonary venous obstruction. Our patient had an uneventful post-operative course and encouraging 2-month follow-up echocardiography. Careful follow-up is warranted to detect post-operative complications, including obstruction of the pulmonary venous and cavoatrial anastomoses.
Percutaneous balloon pericardiotomy is a percutaneous procedure that creates a window in the parietal pericardium by balloon dilation. The use of percutaneous balloon pericardiotomy has not been reported well in children.
Objectives:
The objective of this study was to describe the single centre experience of percutaneous balloon pericardiotomy in children.
Methods:
This was a retrospective study to describe all the children aged <20 years undergoing percutaneous balloon pericardiotomy during an 18-year period (2001–2019). Patient characteristics, technical and ultimate procedural success, and repeat interventions were collected.
Results:
A total of 13 percutaneous balloon pericardiotomy’s were performed in 11 children at the median age of 12 years (range 1.8–19). The etiologies of pericardial effusion were post-pericardiotomy syndrome (n = 4), restrictive cardiomyopathy (n = 1), autoimmune diseases (n = 3), malignancy (n = 2), and idiopathic (n = 1). Two patients received two percutaneous balloon pericardiotomy. The technical success of percutaneous balloon pericardiotomy was 100% with no acute adverse events (balloon rupture or local bleeding). Five (45%) required re-intervention and ultimately three required a surgical pericardial window 6 to 35 days after the percutaneous balloon pericardiotomy. As a result, ultimate procedural success rate was 73% (8/11).
Conclusion:
Percutaneous balloon pericardiotomy was performed safely with high technical success in children. Percutaneous balloon pericardiotomy may be considered for recurrent and persistent pericardial effusion, before considering a surgical pericardial window.
Primary pediatric cardiac tumors are extremely rare. We report a 14-year-old girl with primary cardiac Hodgkin lymphoma. The large right atrial tumor extended upward and occluded the superior caval vein and left innominate vein.
Coronary-pulmonary artery fistula is a rare anomaly in which an aortopulmonary collateral artery arises from a coronary artery, often seen in patients with pulmonary atresia with ventricular septal defect. In the presented case, a coronary-pulmonary artery fistula arose from the left main coronary artery and supplied blood flow to a left upper lobe segment. The life-sized three-dimensional printed model was helpful in pre-surgical planning for unifocalisation of the aortopulmonary collateral arteries.
Acute pulmonary embolism is a life-threatening condition and rarely occurs in children. In adults, catheter-directed therapy emerges as a potentially safer and effective therapeutic option. However, there is a paucity of data on the safety and efficacy of catheter-directed therapy for pulmonary embolism in children. We report a single-centred experience of catheter-directed therapy for acute pulmonary embolism in children.
Methods
This is a retrospective study of children who had no CHD and underwent catheter-directed therapy at Detroit Medical Center during a 12-year period from 2005 to 2017. Demographic and clinical data associated with pulmonary embolism were collected along with the outcome.
Results
A total of nine patients of median age 16 years with the range from 12 to 20 received catheter-directed therapy for sub-massive (n = 6) and massive pulmonary embolism (n = 3). Among nine patients, one patient received Angiojet thrombectomy and balloon angioplasty, whereas eight patients received catheter-directed thrombolysis using tissue plasminogen activator through infusion catheters (n = 3) or EkoSonic ultrasound-accelerated thrombolysis system (n = 5). In four out of five patients treated with EkoSonic, significant clinical improvement was noticed within 24 hours. Among seven patients who survived, two patients had minor gastrointestinal bleeding with median hospital stay of 8 days with the range from 5 to 24 days, and two patients with massive pulmonary embolism died possibly due to delayed institution of catheter-directed therapy.
Conclusion
Catheter-directed therapy with/without EkoSonic is an emerging alternative therapy for sub-massive and massive pulmonary embolism in children. A timely institution of catheter-directed therapy appeared important to improve the outcome.
Anomalous drainage of the right superior caval vein into the left atrium is a rare congenital anomaly that causes cyanosis and occult infection owing to right-to-left shunting. Transcatheter management of this anomaly is unique and rarely reported. We report a 32-year-old man with a history of brain abscess, who was diagnosed with an anomalous right superior caval vein draining to the left atrium; right upper pulmonary vein and right middle pulmonary vein draining into the inferior portion of the right superior caval vein; and a left superior caval vein draining into the right atrium through the coronary sinus without a bridging vein. Pre-procedural planning was guided by three-dimensional printed model. The right superior caval vein was occluded with a 16-mm Amplatzer muscular Ventricular Septal Defect occluder inferior to the azygous vein, but superior to the entries of right upper and middle pulmonary veins. This diverted the right superior caval vein flow to the inferior caval vein system through the azygos vein in a retrograde manner and allowed the right upper pulmonary vein and right middle pulmonary vein flow to drain into the left atrium normally, achieving exclusion of right-to-left shunting and allowing normal drainage of pulmonary veins into the left atrium. At the 6-month follow-up, his saturation improved from 93 to 97% with no symptoms of superior caval vein syndrome.
Parents may experience anxiety and stress when their children undergo cardiac catheterisation. The goal of this study was to assess the level of anxiety in parents of children undergoing cardiac catheterisation and to identify factors that were associated with level of anxiety.
Methods
This was a cross-sectional survey of parents of children who underwent cardiac catheterisation. Anxiety levels were measured using a validated self-report questionnaire – State-Trait Anxiety Inventory, which generates state anxiety scores on the current state of anxiety and trait anxiety scores on the stable aspects of anxiety proneness. One sample t-test was used to compare the data with normative data. Multiple linear regression was used to assess the factors associated with the state score.
Results
A total of 113 parents completed the survey. The mean age of parents was 34.0±7.7 years and the mean age of children undergoing catheterisation was 6.7±5.7 years. Compared with normative data, mean state score was significantly higher in our cohort (p<0.05) despite no difference in the trait score. Final multivariate model showed that the state score was significantly associated with child age group (<1 year [coefficient β 7.2] and 10–18 years [6.3], compared to 1 to <10 years of age [reference]) and history of previous catheterisation (−5.2) (p<0.05).
Conclusions
Concurrent state anxiety level was high among parents of children undergoing cardiac catheterisation, whereas trait anxiety level was not. Higher anxiety was experienced by parents of infants and adolescents without a history of previous catheterisation.
In this proceeding paper, we introduce the recent results of Galactic maser astrometry by mainly focusing on those obtained with Japanese VLBI array VERA. So far we have obtained parallaxes for 86 sources including preliminary results, and combination with the data obtained with VLBA/BeSSeL provides astrometric results for 159 sources. With these most updated results we conduct preliminary determinations of Galactic fundamental parameters, obtaining R0 = 8.16 ± 0.26 kpc and Θ0 = 237 ± 8 km/s. We also derive the rotation curve of the Milky Way Galaxy and confirm the previous results that the rotation curve is fairly flat between 5 kpc and 16 kpc, while a remarkable deviation is seen toward the Galactic center region. In addition to the results on the Galactic structure, we also present brief overviews on other science topics related to masers conducted with VERA, and also discuss the future prospect of the project.
We have conducted astrometric observations toward a 22 GHz water maser source associated with the Sgr B2 complex in the Galactic center region with VERA (VLBI exploration of Radio Astrometry). We measured a trigonometric parallax and absolute proper motion of the Sgr B2 complex with respect to an extra-galactic source by observing the water maser source at 10 epochs from 2014 to 2017. The measured distance was 7.52+3.01−1.67 kpc for the Sgr B2M region.
We also succeeded to measure internal motions of maser spots in Sgr B2M, and N region. The number of spots which we could measure the internal motions is about 400. The distribution of the maser spots shows that the maser spots are associated with envelope of HII region seen in radio continuum image obtained with VLA and ALMA. We discuss relative motions between Sgr B2M, and N by using the internal motion.