Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment.

Background and objective: Unilateral agenesis of pulmonary arteries (UAPA) is a rare disease, with approximately 400 cases reported to date. UAPA is often associated with congenital heart disease, and the uncomplicated form is isolated UAPA, which accounts for approximately 30% of all cases of UAPA. The incidence of pulmonary hypertension due to UAPA has been reported to range from 19 to 44%. There is no consensus treatment for pulmonary hypertension associated with UAPA. We present the first reported case in which a three-drug combination, comprising of iloprost inhalation, riociguat, and ambrisentan, was administered to a patient with UAPA, and was followed-up for 3 years post-diagnosis. Case presentation: A 68-year-old Japanese woman presented to our hospital with dyspnea and chest discomfort. She underwent chest radiography, blood tests, and echocardiography; however, the cause of the patient's symptoms could not be identified. During regular follow-up, an echocardiography 21 months after the initial visit revealed elevated right ventricular pressure (peak tricuspid regurgitation velocity: 5.2 m/s and right ventricular systolic pressure: 120 mmHg) and a diagnosis of pulmonary hypertension was made. Contrast-enhanced computed tomography (CT) of the chest and a pulmonary blood flow scintigram were performed to investigate the cause of pulmonary hypertension, and isolated UAPA was diagnosed. The patient was treated with a three-drug combination of iloprost inhalation, riociguat, and ambrisentan and followed up for 3 years with good therapeutic outcomes. Conclusions: We present a case of pulmonary hypertension caused by isolated UAPA. Although rare, this disease can lead to pulmonary hypertension and should be treated cautiously. While there is no consensus regarding the treatment of this disease, a three-drug combination of iloprost inhalation, riociguat, and oral ambrisentan proved effective.

Trithiazolyl-1,3,5-triazines bearing decyloxybenzene moieties: synthesis, photophysical and electrochemical properties, and self-assembly behavior.

We report the synthesis, photophysical properties, redox characteristics, and self-assembly behavior of disk-shaped trithiazolyl-1,3,5-triazines that bear decyloxybenzene moieties. These compounds were synthesized by a Migita-Kosugi-Stille coupling reaction of 1,3,5-trichlorotriazine with three different tributyltin(thiazoles) as the key step. The structure-property relationships, namely the effects of the incorporation of thiazole units into the triazine unit, the conjugation connectivity between the thiazole and triazine units, and the insertion of ethynylene spacers between the thiazole and decyloxybenzene moieties on the properties of the trithiazolyl-1,3,5-triazines were comprehensively investigated. Binding of the triazine core at the 5-position of the thiazole moieties effectively extended the π-conjugation and afforded high fluorescence quantum yields. The ethynylene spacers substantially lowered the LUMO level relative to the HOMO level. The prepared trithiazolyl-1,3,5-triazines self-assembled in solution, and the introduction of thiazole units at the 5-position enhanced this behavior. Detailed thermodynamic studies on the self-association behavior were conducted, and the formation of self-assembled 1D clusters is disclosed.

Tetraalkoxyphenanthrene-Fused Thiadiazoloquinoxalines: Synthesis, Electronic, Optical, and Electrochemical Properties, and Self-Assembly.

π-Extended thiadiazoloquinoxaline (TQ) derivatives 1a,b-3a,b, in which a tetraalkoxyphenanthrene moiety is annulated with the TQ core and benzene rings are incorporated via the ethynylene spacer, were synthesized. They display absorption bands reaching into 750 nm and possess the electron-affinity comparable to [60]fullerene. The CF3- and OMe-substituents on the benzene rings have moderate effects on modulation of the HOMO and LUMO levels. Tetraalkoxyphenanthrene-fused TQs 1a,b-3a,b aggregate in the solid state and assemble in solution through π-π stacking interactions. The self-assembly of 1a,b-3a,b into 1D superstructures was confirmed, and the difference in the alkoxy groups and the solvents for self-assembly proved to change their morphology. Comparison of the properties of 1a and those of reference compounds 4 and 5 clarified the effects of both the fusion of the phenanthrene moiety and the introduction of ethynylene spacers on the properties.

Tetraalkoxyphenanthrene-Fused Hexadecadehydro[20]- and Tetracosadehydro[30]annulenes: Syntheses, Aromaticity/Antiaromaticity, Electronic Properties, and Self-Assembly.

Tetraalkoxyphenanthrene-fused hexadecadehydro[20]- and tetracosadehydro[30]annulenes possessing octatetrayne linkages were synthesized and their properties together with those of phenanthrene-fused octadehydro[12]- and dodecadehydro[18]annulenes have been investigated. Various spectroscopic and electrochemical measurements as well as quantum chemical calculations support that planar [20]- and [30]annulenes are weakly antiaromatic and nonaromatic, respectively. The detailed concentration- and temperature-dependent 1H NMR and UV-vis data of present dehydroannulenes provided evidence for the enhancement of π-π stacking interactions by extension of the acetylenic linkages. Dehydroannulenes formed self-assembled clusters and their morphology and crystallinity proved to depend on the length of acetylenic linkages, the shape of dehydroannulene core, and the bulkiness of alkoxy groups appended to the phenanthrene moieties.

Comparison of neointimal coverage and extra-stent lumen between sirolimus and everolimus-eluting stent using optical coherence tomography.

The external lumen of a stent [defined as extra-stent lumen (ESL)] assessed by optical coherence tomography (OCT) may be related to the risk of thrombus formation after sirolimus-eluting stent (SES) implantation. An everolimus-eluting stent (EES) might provide relatively minimal inflammatory reaction and appropriate neointimal coverage. The purpose of this study was to compare the neointimal thickness and ESL between SES and EES. Patients who underwent OCT examination more than 7 months after either SES or EES implantation were enrolled. Stent area (SA), lumen area (LA), neointimal area (NIA) and neointimal thickness (NIT) of each strut were measured at 1-mm intervals between stented segments. The area, angle (summation per cross-section) and depth (maximum distance from adjacent vessel surface to the outline of stent) of ESL were analyzed. A total of 49 lesions were included (SES n = 20, EES n = 29). Mean follow-up period was 11 months. A total of 998 cross-sections and 9874 struts were analyzed. There were no differences in stent area, lumen area and neointimal area (SA: 6.01 ± 1.60 vs. 6.02 ± 1.40 mm(2), p = 0.572, LA: 5.37 ± 1.52 vs. 5.29 ± 1.34 mm(2), p = 0.692, NIA: 0.64 ± 0.49 vs. 0.72 ± 0.37 mm(2), p = 0.493). Mean NIT of SES and EES were 0.11 ± 0.05 and 0.10 ± 0.05 mm, respectively (p = 0.367). Conversely, area, angle and depth of ESL in SES group were significantly greater than those in EES group (0.20 ± 0.39 vs. 0.03 ± 0.09 mm(2), p < 0.001, 56.2 ± 59.1° vs. 20.1 ± 41.9°, p < 0.001, 0.10 ± 0.09 vs. 0.03 ± 0.03 mm, p < 0.001). OCT showed that the efficacy of neointimal growth suppression is similar between SES and EES, whereas the adverse vascular response after EES implantation is smaller than that after SES implantation.

Modification of physical properties of poly(L-lactic acid) by addition of methyl-ß-cyclodextrin.

Poly(L-lactic acid) (PLLA) is a biodegradable plastic and one of the most famous plastics made from biobased materials. However, its physical strength is insufficient compared to general-purpose plastics. In this study, the effect of methylcyclodextrin (MeCD) addition on the structure and physical properties, especially the drawing behavior, of PLLA was investigated. Through thermal analysis, it was found that MeCD addition lowers the crystallinity and enhances the mobility of PLLA. The sample containing approximately 17% MeCD was drawn to more than 1000% at 60 °C, although PLLA fractured at a strain of less than 100%. Differential scanning calorimetry (DSC)-Raman in situ measurements also revealed decreases in the glass transition temperature (T g), cold crystallization temperature (T c), and melting point (T m), and improvement in structural distribution with temperature. DSC-Raman measurements simultaneously supplied information about crystallinity and thermal properties. Thus, it was concluded that MeCD had high affinity for PLLA, and the addition of MeCD increased the amorphous component of PLLA and enhanced the drawability.

Impact of stentablation with rotational atherectomy in coronary microcirculatory function.

Key Clinical Message: Stentablation (SA) has been used as a bailout method for undilated, under-expanded stents, but one reason that SA is associated with a high rate of major adverse cardiac events may be its adverse effect on microcirculation. Consequently, appropriate lesion preparation should always be considered for heavily calcified lesions to avoid such complications. Abstract: Under-expansion of the coronary stent is associated with increased rates of in-stent restenosis and thrombosis. Stentablation (SA) with rotational atherectomy is used in the treatment of undilatable, under-expanded coronary stents; however, its effect on coronary microcirculatory function remains unclear. This novel report compares microcirculation indices before and after SA.

Bailout Strategy for Paclitaxel-Coated Nitinol Self-Expanding Stent Deployment Failure: The Cut and Peel Technique.

The patient received endovascular therapy for a superficial femoral artery occlusion. Placement of a SMART stent distal to the lesion was successful, but deployment issues occurred with the Innova stent, requiring forceful retraction and causing elongation. The "cut and peel technique" was developed as a bailout strategy for such cases.

Early and short-term use of proprotein convertase anti-subtilisin-kexin type 9 inhibitors on coronary plaque stability in acute coronary syndrome.

Aims: Proprotein convertase anti-subtilisin-kexin type 9 inhibitors (PCSK9Is) improve plaque volume and composition and reduce major adverse coronary events in chronic coronary artery disease. We evaluated the effects of the short-term use of PCSK9Is on coronary plaque stability in patients with acute coronary syndrome (ACS) using optical coherence tomography (OCT). Methods and results: This is a multicentre, open-label randomized controlled trial. The enrolled 80 subjects met the inclusion criteria. Of these, 52 patients (age 60 ± 11 years, 38 men, 14 women) with ST-elevated ACS had undergone successful primary percutaneous coronary intervention with LDL-cholesterol (LDL-C) levels > 70 mg/dL while receiving high-intensity statins. Participants were randomly assigned to the PCSK9I group (evolocumab 420 mg for 3 months, n = 29) or the standard of care (SoC) group (n = 23). Optical coherence tomography was performed at baseline (BL) and 3 and 9 months after randomization to assess lipid-rich plaques in non-culprit lesions. The change in the minimum fibrous cap thickness (MFCT) from BL to 9 months was the primary endpoint. The percentage change in LDL-C levels from BL to 3 months was significantly greater in the PCSK9I group (-67.8 ± 21.5% in the PCSK9I group vs. -16.3 ± 21.8% in the SoC group; P < 0.0001), and the difference between the two groups disappeared from BL to 9 months (-20.0 ± 37.8% in the PCSK9I group vs. -6.7 ± 34.2% in the SoC group; P = 0.20). The changes in MFCT from BL to 9 months were significantly greater in the PCSK9I group, even after PCSK9I discontinuation {100 µm [interquartile range (IQR): 45-180 µm] vs. 50 µm [IQR: 0-110 µm]; P = 0.032}. Conclusion: Combination treatment with PCSK9Is and statins resulted in more marked plaque stabilization after ACS than SoC alone, and this effect persisted for 6 months after PCSK9I discontinuation. Registration: Adage-Joto study, UMIN ID No. 26516.

Implementation of the kissing balloon technique using a perfusion balloon in acute myocardial infarction: a case report.

Background: Prolonged antiplatelet-agent administration associated with stenting for acute myocardial infarction is not ideal in young patients. We successfully performed a perfusion balloon-based kissing balloon technique in the left anterior descending artery ostium in a young patient with acute myocardial infarction. Case summary: A 34-year-old female presenting with recurrent chest pain was diagnosed with ST-segment elevation acute myocardial infarction. Emergency coronary angiography revealed 90% stenosis of the left anterior descending artery ostium. Considering her age, lesion dilation using a perfusion balloon was preferred. This inadvertently impinged the left circumflex artery, causing chest pain and ischaemia-related hypotension with only a 10 s perfusion dilation balloon. We transitioned to an alternative 7 Fr guiding catheter and performed the kissing balloon technique using a perfusion balloon. This allowed prolonged inflation for 120 s, without complications. Optical coherence tomography of the lesion revealed layered plaques. Based on the patient's age and medical history, coronary spasm may be the aetiological factor underlying acute ST-segment elevation myocardial infarction. Coronary angiography 3 months post-discharge revealed no restenosis. Discussion: Regarding acute myocardial infarction, prolonged inflation with a perfusion balloon had higher frequency of non-stent-related interventions than that of semi-compliant or non-compliant balloons. In this case, the perfusion balloon resulted in chest pain and hypotension. Perfusion balloon-based kissing balloon technique was useful in this setting. Optical coherence tomography revealing the responsible lesions can help determine the cause of acute myocardial infarction in young patients; in our case, a layered plaque was observed. Layered plaques are associated with coronary spasm; therefore, initiating treatment of coronary spasm was considered herein.

Acute Cholecystitis Caused by Campylobacter jejuni Mimicking Acute Coronary Syndrome.

Campylobacter spp. is a widely recognized pathogen accountable for acute enteritis, frequently linked to sepsis, primarily attributed to C. jejuni. Instances of Campylobacter-induced cholecystitis are infrequent, with only a limited number of documented case reports. Acute cholecystitis has been sporadically documented to induce electrocardiographic alterations, occasionally simulating an acute coronary syndrome (ACS). Herein, we present an instance of cholecystitis induced by C. jejuni, posing a challenge in its differentiation from ACS due to electrocardiographic modifications. An 85-year-old Japanese male presented to our hospital with a complaint of chest discomfort lasting one hour. His medical history included hypertension, dyslipidemia, and effort angina pectoris, with a prior percutaneous coronary intervention. The chest discomfort, accompanied by pain and pressure, raised uncertainty about its similarity to a previous angina episode. Vital signs were in the normal range. Physical examination revealed no abnormal heart or lung sounds. Electrocardiography indicated a right bundle branch block and new ST-segment elevation in V2-3. Echocardiography, chest X-rays, and blood tests showed no abnormalities. Emergency coronary angiography revealed no stenosis. Post-angiography, chest discomfort persisted, and the patient developed fever and chills. Contrast-enhanced CT revealed gallbladder lithiasis, prompting suspicion of sepsis. C. jejuni was detected, and antimicrobial therapy resolved symptoms.

Naphazoline intoxication with transient QT prolongation and acute myocardial injury.

A 27-year-old Japanese woman with a history of depression and an eating disorder presented to our emergency department with a chief complaint of generalized weakness. Electrocardiography showed prominent QT prolongation with multiple ventricular contractions. Chest X-ray plain computed tomography revealed pulmonary edema. Echocardiography showed decreased left ventricular systolic function. Suspecting acute myocarditis, we performed a myocardial biopsy from the right ventricular septum. The biopsy histology revealed extensive myocardial fibrosis and a very mild inflammatory cell infiltrate. In an additional detailed medical interview, the patient admitted that she had consumed three bottles of a first-aid liquid containing naphazoline approximately ~12 h before her presentation, in a suicide attempt. Her QTc and left ventricular ejection fraction improved during hospitalization. Learning objective: Acute drug intoxication can cause QT prolongation and ventricular arrhythmias, cardiomyopathy, and pulmonary edema. When acute QT prolongation, myocardial damage, and pulmonary edema are seen (suggesting acute myocarditis), naphazoline intoxication should be investigated in the differential diagnosis.

Long-term effects of ipragliflozin on blood pressure in patients with type 2 diabetes: insights from the randomized PROTECT trial.

Although previous reports have shown that sodium-glucose cotransporter-2 (SGLT2) inhibitors have a blood pressure (BP) lowering effect, relevant long-term data is limited. This study aimed to evaluate the effect of the SGLT2 inhibitor ipragliflozin on BP, and associations between BP reduction and changes in cardiometabolic variables in diabetic patients. This was a sub-analysis of the PROTECT trial, a multicenter, randomized, open-label study to assess if ipragliflozin delays carotid atherosclerosis in patients with type 2 diabetes. Participants were randomized to ipragliflozin and control groups. The primary endpoint of the present sub-analysis was the trajectory of systolic BP over 24 months. Correlations between systolic BP changes and cardiometabolic variables were also evaluated. A total of 232 eligible participants with well-balanced baseline characteristics were included in each study group. Throughout the 24-month study period, mean systolic BP was lower in the ipragliflozin group. At 24 months, a between-group difference (ipragliflozin minus control) in mean systolic BP change from baseline was -3.6 mmHg (95% confidence interval, -6.2 to -1.0 mmHg), and the reduction in systolic BP in the ipragliflozin group was consistent across subgroups examined. Changes in systolic BP significantly correlated with those in body mass index in the ipragliflozin group, while no significant correlations with other cardiometabolic variables tested were observed. In conclusion, ipragliflozin treatment was associated with BP reduction throughout the 24-month follow-up period as compared to control treatment. BP reduction correlated with weight loss, which might be one of the mechanisms for the BP lowering effect of SGLT2 inhibitors.

Tetraalkoxyphenanthrene-fused dehydroannulenes: synthesis, self-assembly, and electronic, optical, and electrochemical properties.

Novel tetraalkoxyphenanthrene-fused dehydro[12]-, [18]-, and [24]annulenes 1-3 were synthesized by using Cu-mediated or Pd-catalyzed oxidative macrocyclization reactions as key steps, and their electronic, optical, and electrochemical properties have been investigated in detail. X-ray crystallographic analysis of a single crystal of 1 a demonstrated that the molecules were arranged longitudinally in a slipped π-stacked fashion to form a 1D column. (1)H NMR and UV/Vis spectroscopic and cyclic voltammetric analysis in conjugation with nucleus-independent chemical shift (NICS) calculations for 1-3 support that the annulation at the 9,10-positions of phenanthrene to the dehydroannulene ring enhances the tropicity and decreases the HOMO-LUMO gaps of the molecules relative to the benzannulation and that 1 possesses an antiaromatic character. Self-association behavior due to π-π stacking in CDCl3 was observed for 1 and 2 and was quantified by concentration-dependent (1)H NMR spectroscopic measurements. The self-assembly of 1 and 2 into well-defined 1D superstructures with high aspect ratios were obtained, and the morphology and crystallinity of these compounds were investigated by means of SEM and wide-angle X-ray diffraction (WAXD) measurements. Furthermore, it was shown that 1 b and 2 b display liquid-crystalline phases by means of differential scanning calorimetry, polarizing optical microscopy, and variable-temperature WAXD measurements.

Transcoronary mapping using a guidewire during transcoronary ethanol ablation for ventricular tachycardia with a deep intramural substrate: a case report.

Background: Transcoronary ethanol ablation is effective in treating ventricular tachycardia (VT) in the deep myocardium. The selection of the target coronary artery plays an important role in the success of transcoronary ethanol ablation. Transcoronary mapping, using a guidewire, may be effective for identifying the target coronary artery. Case summary: A 72-year-old man, who had undergone thrombolytic therapy for acute myocardial infarction 40 years ago, was admitted to the emergency department with a chief complaint of syncope. Five years ago, a cardiac resynchronization therapy defibrillator was implanted for a left bundle branch block (QRS duration 153 ms), with New York Heart Association Class Ⅲ and a left ventricular ejection fraction of 30%.Due to VT, he experienced a critical deterioration in his vital parameters, leading to shock. The first VT ablation was performed on the 3rd day of hospitalization. Activation mapping showed that the earliest activation site was located in the mid-anterior septum of the left ventricle. Mapping from the endocardial surface showed no mid-diastolic potential around the VT. Radiofrequency catheter ablation therapy was performed at the targeted site, resulting in transient termination of VT. However, the VT showed recurrence the next day. A transcoronary ethanol ablation was performed on the 10th day of hospitalization. A 0.014 inch guidewire and microcatheter were advanced into the target coronary septal branch, and the myocardial septum was mapped. The guidewire-assisted transcoronary mapping showed a potential 43 ms ahead of QRS onset during VT. The coronary septal artery branch was considered the target artery, and 0.5 mL of ethanol was injected. No further VT was observed for 12 months after the transcoronary ethanol ablation. Discussion: Transcoronary ethanol ablation is considered in cases where a deep intramural substrate is suspected or when early activation at the interventricular septum is identified. Guidewire-assisted transcoronary mapping allows mapping of VT with deep intramural substrates and may be useful in selecting target coronary arteries while performing transcoronary ethanol ablation.

Sequential Multiple Visceral Artery Dissection within a Short Time, without Aortic Dissection.

A 65-year-old Japanese man without medical history presented with sudden onset lower abdominal pain to our emergency department. Contrast-enhanced computed tomography (CT) revealed dissections of the inferior mesenteric artery and left renal artery with false lumen thrombosis without aortic dissection. He was immediately hospitalized, and conservative treatment was administered. However, on the third-day post-onset, the patient reported severe upper abdominal pain and contrast-enhanced CT showed a new superior mesenteric artery dissection. He continued to receive conservative treatment, and his symptoms improved. He was discharged after ten days of hospitalization.

Tertiary Cardiovascular Syphilis Presenting as Aortic Regurgitation, Aortitis, Thrombus, and Coronary Artery Occlusion, Requiring Percutaneous Coronary Intervention.

BACKGROUND Heart failure is caused by coronary artery disease, valvular disease, and arrhythmias and is highly treatable with recent technology. However, the incidence of syphilis is increasing worldwide. This case report describes tertiary cardiovascular syphilis, accompanied by aortic regurgitation, syphilitic aortitis complicated by thrombus of the ascending aorta, and coronary artery occlusion, requiring percutaneous coronary artery intervention. CASE REPORT A 51-year-old Japanese man with no significant medical history was admitted to the hospital for worsening shortness of breath on exertion. On physical examination, there was no edema in either lower leg. Chest X-rays showed an enlarged heart and pulmonary congestion, and echocardiography showed a left ventricular ejection fraction of 18%, with full circumferential wall motion impairment. Heart failure was diagnosed, and the patient was found to have severe coronary artery disease and aortic regurgitation. He underwent percutaneous coronary intervention (PCI) for his coronary artery occlusion and was treated with medications for heart failure. Two months later, his condition improved, and PCI was performed for the revascularization of the remaining coronary artery. After PCI was completed, the patient was evaluated for vasculitis. The aortic wall lesion was likely a result of non-active syphilitic aortitis, and the results of serological tests of syphilis were positive. Therefore, we concluded that the diagnosis was cardiovascular syphilis. CONCLUSIONS This case report has highlighted the need for clinicians to be aware of the cardiovascular findings in syphilis, including syphilitic aortitis, particularly at this time, when the global incidence of syphilis is increasing.

Glucocorticoid-Induced Cardiomyopathy Caused by Uncontrollable Asthma.

Hypercortisolism is a risk factor for adverse cardiovascular and cerebrovascular outcomes, including hypertension, hyperglycemia, and dyslipidemia. It has been suggested that cardiovascular risk increases with increasing steroid use in patients taking oral steroids as immunosuppressive drugs. Cardiomyopathy is often reported to occur concomitantly in patients with Cushing's syndrome. Reports of cases of long-term high-dose glucocorticoid ingestion and concomitant cardiomyopathy are rare. We report a case of cardiomyopathy in a 63-year-old Japanese man. He had refractory bronchial asthma and had been on prednisolone ≥15 mg/day equivalent for >20 years. Echocardiography showed severe left ventricular dilatation, left ventricular systolic dysfunction, and mitral regurgitation. Since other secondary cardiomyopathies were excluded, a diagnosis of glucocorticoid cardiomyopathy was made, cardioprotective drugs were introduced, and the steroid dose was reduced during hospitalization. Four months after the patient's discharge, echocardiography showed normalization of left ventricular systolic function.

Acute aortic dissection with left coronary artery obstruction.

If the electrocardiogram shows ST-segment elevation in lead aVR, the complication of aortic dissection must always be assumed.