Bilateral pulmonary artery banding facilitates the systemic ventricular outflow tract growth for biventricular and univentricular repair candidates of complex arch anomaly.

Various surgical approaches address complex heart disease with arch anomalies. Bilateral pulmonary artery banding (bPAB) is a strategy for critically ill patients with complex arch anomalies. Some reports argued the potential effect of bPAB on the growth of the left ventricular outflow tract (LVOT) during inter-stage after bPAB. This study aimed to analyze the LVOT growth for biventricular repair candidates with arch anomaly and systemic ventricular outflow tract (SVOT) for univentricular repair candidates with arch anomaly. This retrospective study analyzed 17 patients undergoing initial bPAB followed by arch repair. The Z-scores of LVOT and SVOT were compared between pre-bPAB and pre-arch repair. Patient characteristics, transthoracic echocardiogram data, and PAB circumferences were reviewed. The diameter of the minimum LVOT for biventricular repair (BVR) candidates, the pulmonary valve (neo-aortic valve, neo-AoV) and the pulmonary trunk (the neo-ascending aorta, neo-AAo) for univentricular repair (UVR) candidates, and the degree of aortic or neo-aortic insufficiency in each candidate was statistically analyzed. 17 patients were divided into the UVR candidates (group U) with 9 patients and the BVR candidates (group B) with 8 patients. In group B, the median value of the Z-score of the minimum LVOT increased from -3.2 (range: - 4.1 ~ - 1.0) at pre-PAB to -2.8 (range: - 3.6 ~ - 0.3) at pre-arch repair with a significant difference (p = 0.012). In group U, the median value of the Z-score of the neo-AoV increased from 0.5 (range: - 1.0 ~ 1.7) at pre-bPAB to 1.2 (range: 0.2 ~ 1.9) at pre-arch repair with a significant difference (p < 0.01). The median value of the Z-score of the neo-AAo was also increased from 3.1 (range: 1.5 ~ 4.6) to 4.3 (range: 3.1 ~ 5.9) with a significant difference (p = 0.028). The growth of the LVOT for BVR candidates and SVOT for UVR candidates during the inter-stage between bPAB and arch repair was observed. These results suggest the potential advantage of bPAB in surgical strategies. Further research is needed to validate these findings and refine surgical approaches.

Long-term results of the frozen elephant trunk technique in primary chronic type B aortic dissection.

OBJECTIVES: The aim of this study is to analyze long-term results of the total aortic arch replacement with the frozen elephant trunk technique for primary chronic type B aortic dissection. METHODS: Among 322 patients who had the frozen elephant trunk technique, 43 (13.4%) patients (median age, 64.0 years) with primary chronic type B aortic dissection were analyzed. The patients underwent surgery at a median of 27.0 months after the onset of aortic dissection. The false lumen was patent in 30 (69.8%) patients preoperatively. RESULTS: Two patients suffered paraplegia and another died of cerebral infarction. Complete thrombosis of the peri-stent false lumen was achieved in 36 (83.7%) patients. Overall survival, freedom from aortic events, and freedom from aortic reintervention were 83.5%, 37.2%, and 42.2% at 5 years, respectively. Survival rates of the patients with or without aortic events were 75.0% and 95.8% at 5 years (Log-rank, p = 0.22). Late aortic events occurred in 19 (44.2%) patients including distal stent graft-induced new entry, type Ib endoleak, and false lumen dilation. The ratio of the stent graft diameter to the preoperative short axis diameter of the true lumen (hazard ratio, 1.90; 95% confidence interval, 1.01-3.59), and the maximal diameter of a postdissection aneurysm (1.07; 1.01-1.12) were the significant multivariate risk predictors of late aortic events. CONCLUSIONS: Late aortic events occurr fairly frequently, and new strategies need to be explored to enhance the effectiveness of this procedure in the future.

Hemolytic Anemia Caused by Graft Kinking Following Ascending Aortic Replacement: Endovascular Treatment With a Palmaz XL Stent.

A 66-year-old man presented with hemolytic anemia, which required frequent blood transfusion, 6 months after surgical repair of an ascending aortic pseudoaneurysm. Hemolysis was attributed to luminal stenosis caused by graft kinking by laboratory test, CT and four-dimensional magnetic resonance imaging. First, an Excluder cuff was placed at the stenotic site under rapid pacing, but it migrated distally. Thereafter a Palmaz XL stent was placed at the stenotic site, which led to resolution of anemia. In this case, a Palmaz XL stent was successfully used to treat hemolytic anemia caused by graft kinking following ascending aortic surgery.

Severe leukocytopenia due to copper deficiency induced by zinc supplementation in a patient on peritoneal dialysis: a case report.

Zinc deficiency is one cause of anemia. However, it has been reported that some patients who were treated with zinc supplementation to resolve this anemia subsequently experienced copper deficiency, which lead to continued anemia, as well as leukocytopenia and other symptoms. However, only two patients with copper deficiency induced by zinc supplementation undergoing peritoneal dialysis have been reported. Here, we report the case of a 59 year-old man with copper deficiency after zinc supplementation undergoing peritoneal dialysis (PD). He took meals only once a day and drank about 750 mL/day of wine every day. He had been receiving zinc supplementation for 4 months. He was diagnosed with severe leukocytopenia and worsening anemia at a planned outpatient visit; in addition, his copper levels had markedly decreased. Thus, zinc supplementation was discontinued, and the patient was instructed to take cocoa for copper supplementation. Because of severe leukocytopenia, he was admitted to our hospital, and granulocyte colony-stimulating factor was administered. Red blood cell transfusions were performed for anemia. After discontinuing zinc supplementation, his white blood cell count and hemoglobin levels improved.To avoid Cu deficiency, patients' dietary history should be checked in detail and Cu should be monitored carefully when Zn is supplemented in patients undergoing PD.

Transmitral extraction of a huge left ventricular mural thrombus using a novel mitral leaflet retractor.

A 52-year-old woman with a past history of anterior myocardial infarction 15 years previously was found to have a pedunculated mobile thrombus with a narrow stalk originating from the left ventricle, and a huge laminated mural thrombus. Surgical extraction of the two organized thrombi was successfully performed with transmitral approach using a novel, flexible, self-retained, and reusable leaflet retractor, which was originally developed for exposure of the subvalvular apparatus during mitral valve repair. Excellent access, exposure, and visualization of the left ventricle were achieved by this heart valve retractor and an endoscope for removal of a huge mural thrombus.

Tolvaptan Reduces Extracellular Fluid per Amount of Body Fluid Reduction Less Markedly than Conventional Diuretics.

Objective Tolvaptan, a vasopressin V2 receptor antagonist, is a water diuretic, removing electrolyte-free water from the kidneys and affecting the water balance between the intracellular and extracellular fluid. We previously reported that tolvaptan efficiently reduced the intracellular fluid volume, suggesting its utility for treating cellular edema. Furthermore, tolvaptan is known for its low incidence of worsening the renal function, with conventional diuretics use associated with worsening of the renal function Methods In this retrospective observational study, five chronic kidney disease (CKD) patients with fluid retention were assessed by the bioelectrical impedance (BIA) method twice (before and after tolvaptan therapy). Tolvaptan was used with conventional diuretics. The post/pre ratio of extracellular water (ECW)/total body water (TBW) in the tolvaptan group was compared with that in 18 CKD patients undergoing body fluid reduction with conventional diuretics alone (conventional diuretics groups), taking the reduced amount of body fluid into consideration. Results Removing body fluid, either by tolvaptan or by conventional diuretics alone, decreased the ECW/TBW ratio. Of note, the reduction in extracellular fluid was milder in the tolvaptan group than in the conventional diuretics group. Conclusion Tolvaptan reduces the extracellular fluid per amount of body fluid reduction less markedly than conventional diuretics.

Snake Technique in Osler's Disease-Associated Thoracoabdominal Aortic Aneurysm.

Osler's disease is an autosomal dominant disorder characterized by epistaxis, telangiectases, and arteriovenous malformations of the internal organs. Herein, we report the first published case of dissecting thoracoabdominal aortic aneurysm repair in a 66-year-old woman with hereditary hemorrhagic telangiectasia associated with ACVRL1 gene mutations. We maintained the activated clotting time around 400 seconds during cardiopulmonary bypass to avoid lethal hemorrhage from telangiectases or arteriovenous malformations. The Adamkiewicz artery could not be identified on imaging studies preoperatively. Therefore, we used the snake technique for intercostal revascularization, which was effective in preventing paraplegia.

A Formula for the Estimation of 24-Hour Urinary Creatinine Excretion: A Derivation and Validation Study.

OBJECTIVE: Twenty-four-hour urinary creatinine (Cr) excretion (24h-uCr) is the basis of Cr clearance and urinary protein-Cr ratio, and it is related to frailty, worsening kidney function, and mortality in patients with chronic kidney disease. Although subjects with lower estimated glomerular filtration rate (eGFR) tend to have lower 24h-uCr, previous formulae for the estimation of 24h-uCr did not include Cr as a predictor. METHODS: This retrospective study included patients admitted to the Department of Nephrology at our hospital (derivation cohort and validation cohort: patients admitted between April 2016 and March 2020). The prediction formula of 24h-uCr was calculated using a multivariate linear regression model with the bootstrap method. Age, height, weight, sex, Cr, and cystatin C were used as predictors. RESULTS: The derivation and validation cohorts included 187 and 63 patients, respectively. The characteristics of the derivation and validation cohorts were as follows: age 73 (61-79.5) years and 70 (58.5-79) years; males, 61.5% and 60.3%; eGFRCr 27.0 (13.7-48.6) mL/min/1.73 m2 and 26.3 (14.0-51.5) mL/min/1.73 m2; and 24-hour urinary protein excretion 0.79 (0.17-2.12) g/day and 1.08 (0.26-2.55) g/day, respectively. Seven prediction formulae were derived. In all models, the Pearson's correlation coefficient was relatively high and statistically significant. However, previous models tended to overestimate the 24h-uCr. Furthermore, the predicted 24h-uCr calculated by the models that do not include Cr as a predictor fluctuates depending on the eGFRCr. CONCLUSION: The best formula for predicting 24h-uCr (mg/day) in a wide range of eGFR populations is a Cr-containing formula: [-9.04 × age (years) + 8.03 × weight (kg) + 0.66 × height (cm) + 188.59 (if male) - 32.11 × Cr (mg/dL) + 779.14].

Gitelman syndrome with a novel frameshift variant in SLC12A3 gene accompanied by chronic kidney disease and type 2 diabetes mellitus.

Gitelman syndrome is an autosomal recessive genetic disease caused by pathogenic variants in SLC12A3 resulting in the loss of function of the Na-Cl co-transporter (NCC) in the distal tubules. Hypokalemia and diuretic effects can cause secondary type 2 diabetes and renal function decline. Here, we present the case of a 49-year-old male patient with chronic persistent treatment-resistant hypokalemia for the past 13 years who had been receiving treatment for type 2 diabetes mellitus for 6 years. He was referred to our department due to the presence of urinary protein, impaired renal function, high renin activity, and hyperaldosteronism. Laboratory test results showed hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. Using next-generation and Sanger sequencing, we identified a novel stop-gain variant (NM_000339.3:c.137del [p.His47fs]) and a missense variant (NM_000339.3:c.2927C > T [p.Ser976Phe]) in the SLC12A3 gene. This novel pathogenic variant was located at the intracellular N-terminus of the NCC. Based on these findings, the patient was diagnosed with Gitelman syndrome. The use of next-generation sequencing facilitated the exclusion of diseases with similar clinical symptoms.

Identification of a small pericardial effusion on contrast-enhanced computed tomography indicating cardiac perforation and pericardial injury following blunt trauma: A case report.

Blunt trauma may cause cardiac perforation requiring emergency surgical repair. Cardiac perforations are usually diagnosed by the presence of a pericardial effusion on echocardiography. However, cardiac perforations and pericardial effusions are sometimes too small to detect, resulting in underdiagnosis. In this case report, we describe a 22-year-old man who was involved in a traffic accident, admitted in a state of shock, and was initially treated for tension pneumothorax and liver and spleen injuries. His initial computed tomography scans revealed a small region of enhancement, corresponding to a small pericardial effusion, indicative of a cardiac perforation. Thus, an emergency median sternotomy was performed. He was diagnosed with perforation of the left atrial ear and right atrium, which were repaired surgically. His liver and spleen injuries were also treated, and the patient was discharged 44 days after admission. The detection of a small pericardial effusion on enhanced computed tomography enabled rapid diagnosis of a cardiac perforation and ensured emergency surgical repair could be performed as soon as possible. LEARNING OBJECTIVES: •To acknowledge the difficulty of diagnosing cardiac perforation in patients with pericardial injury, based on conventional signs of blunt cardiac injury, such as sternal fracture, serum cardiac enzymes, and hemothorax.•To recognize that a small pericardial effusion on enhanced computed tomography scans is an important finding that should raise suspicion of cardiac perforation and pericardial injury.

Venopulmonary artery extracorporeal lung assist in repair of traumatic aortic injury via left thoracotomy.

An urgent surgery was performed for a 28-year-old man who sustained a traumatic descending thoracic aortic injury in an automobile collision. Severe respiratory failure was encountered during surgery, which did not allow for single-lung ventilation for adequate exposure of the descending aorta. We used venopulmonary artery extracorporeal lung assist by main pulmonary artery cannulation with concurrent distal aortic perfusion using a single centrifugal pump. Cannulating the easily accessible main pulmonary artery for venopulmonary artery extracorporeal lung assist is a safe and feasible technique in patients complicated with profound respiratory failure undergoing aortic surgery via left thoracotomy.

[Coronary-pulmonary Artery Fistula Manifested by Cardiac Tamponade;Report of a Case].

Coronary-pulmonary artery fistula( CPAF) is a relatively rare congenital malformation. We successfully treated a patient who presented with cardiac tamponade due to ruptured CPAF. A 58-year-old woman was admitted to our hospital due to consciousness disorder. Enhanced computed tomography revealed hemopericardium, and she was diagnosed with cardiac tamponade due to a ruptured coronary artery aneurysm with fistula arising from the right coronary and entering the main pulmonary artery. Therefore, emergency operation was performed. Under cardiopulmonary bypass, the aneurysm was opened and the ostium of the fistula was closed with pledgetted mattress sutures. After ligating the tortuous CPAF, the aneurysmal wall was sutured. Postoperative course was uneventful, and she was discharged on postoperative day 16.

Calcified amorphous tumor with caseous calcification of mitral annulus in hemodialysis patients.

Mitral annular calcification (MAC) is a common finding, especially among the elderly. Caseous calcification of the mitral annulus (CCMA) is a rare variant of MAC. Calcified amorphous tumor (CAT) is an extremely rare non-neoplastic cardiac tumor accompanied by calcification and is often associated with MAC. We encountered two patients with end-stage renal failure in whom a cardiac CAT developed on the mitral annulus. In both patients, preoperative examination showed MAC of the posterior mitral annulus and a mobile mass attached to the MAC. CAT and CCMA were considered, and surgical resection was performed because of a high risk of systemic embolization. Intraoperatively, the mobile mass was associated with the CCMA. The CCMA with the mass was entirely removed, and the diagnosis of CAT was histologically confirmed. We herein report these two cases of CAT arising from CCMA in patients undergoing hemodialysis and propose the characteristic imaging features and operative findings.