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Background: PTPN11 is ubiquitously expressed and has a variety of phenotypes even in a single heart. We examined LEOPARD syndrome (LS) in a patient with PTPN11 variants through pathological, electrophysiological, and anatomical studies. Case summary: A 49-year-old man with no previous medical history was brought to our emergency department because of syncope. An electrocardiogram (ECG) revealed alternating bundle branch block, and echocardiography revealed hypertrophic cardiomyopathy-like morphology with systolic anterior motion of the posterior mitral valve. Atrioventricular block, left ventricular outflow tract (LVOT) obstruction, and ventricular tachycardia were considered the differential diagnoses; however, the treatment plan was difficult to determine. An electrophysiological study revealed the cause of the ECG abnormality to be accelerated idioventricular rhythm, and the programmed ventricular stimulation was negative. Genetic testing revealed LS with PTPN11 variant, which was speculated to be the cause of these various unique cardiac features. The cause of syncope was considered to be exacerbation of LVOT obstruction due to dehydration, and the patient was treated with oral beta-blockers. Implantable loop recorder observation for 1 year revealed no arrhythmia causing syncope, and an implantable cardioverter-defibrillator and pacemaker were deemed unnecessary for primary prevention of syncope. During 2.5 years of follow-up, the LVOT peak velocity fluctuated between 2.5 and 3.5 m/s, but the patient remained stable with no recurrent syncope. Conclusion: We confirmed that LS is distinct from other cardiomyopathies using characterization, physiological, electrophysiological, and pathological examinations. Evidence supporting a specific treatment strategy for LS is limited, and understanding the pathogenesis may help establish effective treatment strategies.
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Introduction: An arteriovenous fistula (AVF) is a potential complication of endovascular therapy (EVT). Arteriovenous fistula steal syndrome sometimes leads to severe limb ischaemia; however, assessment of peripheral perfusion in AVF has not yet been established. Report: A 90 year old woman diagnosed with chronic limb threatening ischaemia underwent EVT. However, subintimal angioplasty of infrapopliteal lesions resulted in AVF formation in the posterior tibial artery (PTA). Revascularisation of the anterior tibial artery and PTA was performed, but severe AVF steal syndrome persisted and wound healing was delayed. An attempt to physiologically assess the effects of AVF closure and perform an AVF closing manoeuvre, if necessary, was performed. The physiological assessment was performed by laser Doppler flowmetry (LDF) and blood flow was temporarily blocked via the AVF at the distal PTA using a 6 F guiding extension catheter. A significant increase in blood flow was observed in the perfused area of the plantar artery. Coil embolisation and covered stent implantation in the PTA completely closed the AVF. During the procedure, peripheral perfusion with LDF gradually increased in the heel and fifth toe. After AVF closure, the skin perfusion pressure values increased significantly, wound healing was accelerated, and complete healing was achieved. Discussion: Laser Doppler flowmetry measurements under simulated AVF closure using a guiding extension catheter may be useful for the physiological assessment of peripheral perfusion before percutaneous AVF closure.
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BACKGROUND: The trans femoral ipsilateral approach is often adopted for endovascular treatment (EVT) for better steerability of guidewires or better device deliverability. However, contrary to the trans femoral contralateral approach, ipsilateral antegrade puncture sometimes causes peculiar bleeding complications. CASE PRESENTATION: A 76-year-old female underwent EVT for chronic occlusion of the left superficial femoral artery (SFA) via the ipsilateral antegrade approach. After guidewire passage, we inflated the drug-coated balloons, but angiography showed blood flow stasis at the mid segment of the SFA. We also ensured prolonged balloon inflation, which resulted in favorable blood flow. While trying to ensure hemostasis, the blood pressure remained decreased, but neither bleeding nor superficial hematoma were observed at the puncture site. After hemostasis was achieved, we removed the surgical drape and noticed a swelling in the mid-portion of the thigh, distant from the puncture point. We then approached the left common femoral artery (CFA) contralaterally. Angiography showed continuous bleeding from a little bit distally to the sheath insertion point that was spreading through an intramuscular space. We stopped the bleeding with balloon tamponade inside the CFA. Angiography after hemostasis demonstrated blood flow stasis at the mid-segment of the SFA, similarly as that seen before. We confirmed compression of the SFA by a large hematoma using both intra- and extra- vascular ultrasound. Therefore, we deployed a self-expandable stent at the compressed SFA position. Finally, we achieved favorable blood flow on angiography. CONCLUSION: We encountered a case that latent bleeding unrecognized in the surgical field persisted while prolonged inflation of DCB was conducted at just proximal SFA. We could have avoided bailout stenting by noticing the bleeding incident in a timely manner. Prediction and prevention are essential for all kinds of procedural complications in EVT.
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Diagnóstico Tardío , Arteria Femoral , Hemorragia , Punciones , Humanos , Femenino , Anciano , Arteria Femoral/diagnóstico por imagen , Hemorragia/etiología , Hemorragia/terapia , Resultado del Tratamiento , Enfermedad Arterial Periférica/terapia , Enfermedad Arterial Periférica/diagnóstico por imagen , Enfermedad Arterial Periférica/fisiopatología , Valor Predictivo de las Pruebas , Cateterismo Periférico/efectos adversos , Cateterismo Periférico/instrumentación , Procedimientos Endovasculares/instrumentación , Procedimientos Endovasculares/efectos adversos , Técnicas Hemostáticas/instrumentación , Técnicas Hemostáticas/efectos adversosRESUMEN
PURPOSE: This study aimed to evaluate the relationship between nutritional status and hospital outcomes in patients with chronic limb-threatening ischemia (CLTI) in a local area of contemporary super-aged society of Japan. MATERIALS AND METHODS: We analyzed 131 consecutive patients with 179 lower limb diseases admitted to our hospital for the treatment of CLTI between April 2018 and March 2023. These 131 patients were divided into 3 groups according to hospital outcomes: home discharge (HD), out-of-home discharge (OD), and in-hospital death (ID). Patient and lesion backgrounds were compared among the 3 groups, and a multivariable regression analysis was used to analyze the interaction between malnutrition and composite hard endpoints. RESULTS: The median age was 82.8 years, and non-ambulatory patients comprised 61.8% of the study population. The HD group included more ambulatory and fewer patients with higher CONUT score or inflammation than OD or ID group. The Rutherford classification and Wound, Ischemia, and foot Infection stage were significantly more severe in the ID group than in the HD group. Endovascular treatment (EVT) was more often implemented in the HD (94.9%) and OD (81.7%) groups than in the ID group (60.0%). However, all EVT procedures in the ID group were performed until as distally as possible to achieve the target arterial path success contrary to some EVT procedures in the HD or ID group that targeted lesions only above the knee. Multivariate analysis showed that a non-ambulatory state (hazard ratio [HR]=3.65, 95% confidence interval [CI]=1.48-9.02) and a higher controlling nutritional status (CONUT) score (≥5) (HR=7.46, 95% CI=1.66-33.6) were significant predictors for composite endpoints (major amputation or ID). Patients with lower CONUT scores (≤4) showed better outcomes in all indices including overall survival, major amputation-free survival, and wound healing. CONCLUSION: Condition of the CLTI patients represented by higher CONUT score emerged as the most influential predictor of major amputation or ID. Furthermore, non-ambulatory status or condition of higher CONUT score affects the destination after discharge. Implementing multidisciplinary approaches to address patients' nutritional state and physical disability, in addition to revascularization, may enhance comprehensive prognoses in patients with CLTI. CLINICAL IMPACT: In this single-center retrospective study, we analyzed prognoses of 131 consecutive patients with 179 lower limb diseases admitted for the treatment of chronic limb-threatening ischemia (CLTI) between April 2018 and March 2023. Our main finding was that condition of the CLTI patients represented by higher controlling nutritional status (CONUT) score was the most significant predictor of either major amputation or in-hospital death. Furthermore, condition of higher CONUT score or non-ambulatory status affects the destination after discharge. This suggests that multidisciplinary approaches to address patients' nutritional state and physical disability, in addition to revascularization, may enhance the prognosis in patients with CLTI. This is the first report to evaluate nutritional status associated with comprehensive hospital outcomes in addition to previously reported hard endpoints, such as major amputation or overall survival, and will be of great help in future clinical practice.
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BACKGROUND: Infectious aortic disease is a rare and fatal disease, that requires the appropriate intervention. An accurate diagnosis should be promptly established. However, this is difficult because the clinical manifestations of this disease vary and are non-specific. CASE PRESENTATION: (CASE 1) An 87-year-old male, presenting with generalized malaise and weight loss, was admitted for further examination. A chest computed tomography (CT) showed mediastinal emphysema. Empirical intravenous antibiotics were administered to address the non-specific infectious findings in the laboratory data. The treatment was effective, and the patient fully recovered. However, he was in shock due to aortic rupture and marked pseudo aneurysmal formation around the aortic arch day 25 of hospitalization. An emergency total aortic arch replacement was performed, and the patient was discharged. (CASE 2) An 82-year-old male who had undergone Y-graft replacement in the abdominal aorta 15 years previously was admitted due to general malaise and anorexia. Abdominal CT revealed emphysematous changes adjacent to the abdominal aorta. The patient responded favorably to empirical treatment with intravenous antibiotics and was discharged 19 days after admission. Four days after discharge, the patient went into cardiac arrest after an episode of hematemesis. Abdominal CT revealed an enlarged stomach and duodenum, filled with massive high-density contents proximal to the abdominal aorta. He died of hemorrhagic shock despite cardiopulmonary resuscitation. CONCLUSIONS: Although emphysematous changes are rare, they are red flag signs during the early stage of infectious aortic disease. Thus, physicians should remain vigilant for this kind of critical sign.
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Aneurisma de la Aorta Abdominal , Enfermedades Transmisibles , Masculino , Humanos , Anciano de 80 o más Años , Aorta Abdominal , Aorta Torácica , Procedimientos Quirúrgicos Vasculares , Antibacterianos/uso terapéuticoRESUMEN
Tako-tsubo syndrome (TTS) can be triggered by emotional or physical stress and is characterized by transient left ventricular dysfunction with apical ballooning. Some neurologic disorders and pheochromocytoma serve as triggers for TTS, however, its association with primary aldosteronism (PA) is not well known. Pulmonary vein isolation (PVI) with catheter ablation for atrial fibrillation (AF) has been performed worldwide, and TTS following PVI has been reported as a rare complication. Sympathetic stimulation can play an important role in TTS development, however, its mechanism and risk factors are not yet understood.We describe a 72-year-old woman with PA who developed TTS after PVI with radiofrequency catheter ablation (RFCA) for symptomatic paroxysmal AF. Complete isolation of the pulmonary vein was carried out without any complications, however, she complained of epigastric discomfort 7 hours after the procedure. An electrocardiogram showed recurrent AF with a new negative-T wave and prolonged QT interval. Transthoracic echocardiography revealed apical ballooning and basal hypercontraction, characteristic of TTS, and coronary angiography showed no significant stenosis. She was diagnosed with TTS following RFCA for AF and managed well with conservative therapy.The present case suggests that TTS should be recognized as a complication associated with AF ablation. Moreover, PA may be involved in TTS development by increasing sympathetic activity. Further studies on the mechanism and characteristics of TTS are required.
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Fibrilación Atrial , Ablación por Catéter , Hiperaldosteronismo , Venas Pulmonares , Cardiomiopatía de Takotsubo , Femenino , Humanos , Anciano , Fibrilación Atrial/complicaciones , Cardiomiopatía de Takotsubo/etiología , Cardiomiopatía de Takotsubo/complicaciones , Venas Pulmonares/diagnóstico por imagen , Ecocardiografía , Ablación por Catéter/efectos adversos , Ablación por Catéter/métodos , Hiperaldosteronismo/diagnóstico , Hiperaldosteronismo/etiología , Hiperaldosteronismo/cirugía , Resultado del Tratamiento , RecurrenciaAsunto(s)
Vena Axilar , Enfermedades Vasculares , Humanos , Axila , Músculo Esquelético , Edema/diagnóstico , Edema/etiología , Extremidad SuperiorRESUMEN
Coronary debulking devices are essential in obtaining optimal results in percutaneous coronary intervention (PCI) for severely calcified lesions. However, since the introduction of these devices in Japan, the presence of full-time cardiovascular surgeons in their own facilities has been an essential condition (on-site surgical back-up) as the facility criteria for their use. The criteria were revised in April 2020, making their implementation possible at our hospital. Between May 2020 and January 2022, we administered PCIs using rotational atherectomy (RA) for 33 lesions in 28 patients and orbital atherectomy system (OAS) for 36 lesions in 27 patients. The most preferred strategy in our hospital is OAS via the distal radial approach using a 6Fr Glide sheath or RA via the femoral approach using a 7Fr sheath. The percentages of usable imaging modality as an initial device without lesion modification were 57.1 and 66.7% in the RA and OAS groups, respectively. In the RA procedure, 1.5- and 2.0-mm Rota burrs were more frequently adopted for the initial and second sessions, respectively. In the OAS procedure, the debulking was always initiated at a low speed. Nineteen of the 27 patients underwent additional high-speed debulking. Pre-procedural quantitative coronary angiographic analysis revealed that the minimal lumen diameter was significantly smaller in the RA than in the OAS group. Debulking procedures were successful in all patients excluding two instances of procedure-related complications in the RA group, one of which was coronary perforation safely treated via covered stent deployment without any resulting hemodynamic instability. Our early experience with coronary debulking devices with off-site surgical back-up clearly reveals the safety and feasibility of this procedure in a newcomer facility.
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Aterectomía Coronaria , Enfermedad de la Arteria Coronaria , Intervención Coronaria Percutánea , Calcificación Vascular , Humanos , Enfermedad de la Arteria Coronaria/cirugía , Intervención Coronaria Percutánea/efectos adversos , Japón , Procedimientos Quirúrgicos de Citorreducción , Resultado del Tratamiento , Factores de Tiempo , Índice de Severidad de la Enfermedad , Aterectomía Coronaria/efectos adversos , Angiografía CoronariaAsunto(s)
Aneurisma Coronario , Infarto del Miocardio , Aneurisma Coronario/diagnóstico , Aneurisma Coronario/etiología , Aneurisma Coronario/cirugía , Angiografía Coronaria , Vasos Coronarios/diagnóstico por imagen , Vasos Coronarios/cirugía , Humanos , Inmunoglobulina G , Infarto del Miocardio/diagnóstico , Infarto del Miocardio/etiologíaAsunto(s)
Síndrome Coronario Agudo , Enfermedad de la Arteria Coronaria , Síndrome Coronario Agudo/diagnóstico , Síndrome Coronario Agudo/etiología , Síndrome Coronario Agudo/cirugía , Angiografía Coronaria/métodos , Vasos Coronarios/diagnóstico por imagen , Vasos Coronarios/cirugía , Humanos , Diálisis Renal/efectos adversos , Stents/efectos adversos , Resultado del Tratamiento , Ultrasonografía Intervencional/métodosAsunto(s)
Disección Aórtica , Arteria Ilíaca , Disección Aórtica/complicaciones , Disección Aórtica/diagnóstico , Disección Aórtica/cirugía , Aorta Abdominal , Isquemia Crónica que Amenaza las Extremidades , Humanos , Arteria Ilíaca/diagnóstico por imagen , Isquemia/etiología , Resultado del TratamientoRESUMEN
BACKGROUND Vascular access (VA) venous hypertension is a major complication for patients with long-term arteriovenous access in the upper extremities. Endovascular treatment (EVT) is the first option for treating it. A possible cause of VA venous hypertension is stenosis at a site downstream of the arteriovenous fistula. We report a case of VA venous hypertension with complex venous drainage routes. CASE REPORT A 68-year-old woman had worsening VA venous hypertension that led to difficulties in the venous blood return during hemodialysis. The cephalic vein distal to the arteriovenous fistula branched into 3 routes. The most proximal branch was occluded just before the junction to the subclavian vein at the level of the first rib. The pressure gradient between the brachial artery and the VA vein was 30 mmHg. Therefore, we performed an EVT for the occlusion and deployed a 3.0-mm balloon-expandable bare-metal stent, achieving good vascular patency with favorable blood flow. When the outside of the implanted stent was stained with contrast media, the appearance suggested the formation of varices that could have lowered the pressure at that lesion. The pressure gradient between the brachial artery and the VA vein had increased to 80 mmHg, which indicated an improvement of the VA venous hypertension. CONCLUSIONS EVT was effective for an occluded cephalic arch in a hemodialysis patient showing VA venous hypertension, despite the presence of collateral venous routes. VA venous hypertension can be life-threatening for hemodialysis patients. Therefore, it is essential that physicians who use vascular access interventional therapy should determine the cause of the VA venous hypertension and resolve it.
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Derivación Arteriovenosa Quirúrgica , Hipertensión , Anciano , Derivación Arteriovenosa Quirúrgica/efectos adversos , Drenaje , Femenino , Humanos , Diálisis Renal/efectos adversos , Resultado del Tratamiento , Grado de Desobstrucción VascularRESUMEN
BACKGROUND In practical settings of endovascular treatment (EVT) for below-the-knee arteries, we often encounter cases of severe calcification. To overcome problems regarding device uncrossing due to severe calcifications, a bidirectional approach and subsequent guidewire externalization is one of critical methods. CASE REPORT A 74-year-old female with refractory skin ulcers on the lower frontal thigh and necrotic toes on the left side showed occlusion in both the anterior tibial artery (ATA) and tibio-peroneal trunk. Both occluded vessels were accompanied with dense calcification. In the process of EVT targeting the occluded ATA, the retrograde guidewire successfully passed the occlusion and was advanced into the antegrade guide sheath. Next, we attempted guidewire externalization, but severe calcification of the ATA hampered the procedure. Therefore, we introduced a guide extension catheter and a balloon catheter in an antegrade fashion to establish a system of trapping the retrograde guidewire between these devices. Then, we pulled the system back outside the guide sheath, which completed guidewire externalization. We performed prolonged balloon dilatation and finally achieved favorable revascularization of the ATA. CONCLUSIONS Our novel method led to successful retrograde guidewire externalization, overcoming severely calcified lesions. It is generally essential for clinicians to increase their expertise regarding EVT procedures to attain better outcomes.
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Arteriosclerosis Obliterante/cirugía , Procedimientos Endovasculares/métodos , Calcificación Vascular/cirugía , Anciano , Arteriosclerosis Obliterante/complicaciones , Procedimientos Endovasculares/instrumentación , Femenino , Humanos , Arteria Poplítea , Úlcera Cutánea/etiología , Arterias Tibiales , Calcificación Vascular/complicacionesRESUMEN
BACKGROUND: Although timely coronary intervention can result in markedly improved clinical outcomes of patients with acute coronary syndrome (ACS), non-ST-elevation (NSTE)-ACS is sometimes difficult to accurately diagnose. CASE PRESENTATION: A 52-year-old woman complained of acute chest pain with sudden onset. Both electrocardiography (ECG) and echocardiography showed normal results, and we urgently needed to make a differential diagnosis among critical illnesses such as acute coronary syndrome and nonischemic cardiovascular life-threatening diseases. Contrast-enhanced computed tomography (CT) without ECG synchronization showed evidence of neither aortic dissection nor pulmonary embolism, but regionally reduced contrast enhancement in the posterior myocardium, which were suggestive of myocardial ischemia. Emergency coronary angiography demonstrated severe stenosis of the left circumflex artery, and we achieved favorable revascularization with drug-eluting stent deployment. CONCLUSIONS: We diagnosed a patient with NSTE-ACS in whom contrast-enhanced CT without ECG synchronization was effective for visualization of reduced myocardial perfusion, suggesting ischemic heart disease.
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Síndrome Coronario Agudo/diagnóstico por imagen , Imagen de Perfusión Miocárdica/métodos , Infarto del Miocardio con Elevación del ST/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Síndrome Coronario Agudo/fisiopatología , Síndrome Coronario Agudo/terapia , Circulación Coronaria , Stents Liberadores de Fármacos , Femenino , Humanos , Persona de Mediana Edad , Intervención Coronaria Percutánea/instrumentación , Valor Predictivo de las Pruebas , Infarto del Miocardio con Elevación del ST/fisiopatología , Infarto del Miocardio con Elevación del ST/terapia , Resultado del TratamientoRESUMEN
Recently, there have been increasing opportunities to treat patients with peripheral arterial disease. Of those patients, both pathological conditions, such as acute limb ischemia (ALI) and chronic critical limb ischemia (CLI), are closely associated with high risks of major amputation, disability and death. We encountered a very rare case of CLI subsequent to ALI. An 83-year-old male showed the sudden onset of ALI, probably due to thromboembolism from an abdominal aortic aneurysm during an operation for gastric cancer. The patient was referred to another hospital for Fogarty thrombectomy. About 1 month after ALI onset, necrosis of the left first toe gradually progressed. On angiography of the left lower limb, we noted occlusions of both anterior and posterior tibial arteries. Then, we successfully conducted balloon angioplasty for the below-the-knee arteries. Thereby, favorable blood flow was achieved, which led to successful wound healing without amputations.
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BACKGROUND Ventricular rupture is a complication of acute myocardial infarction (AMI) that results in hemopericardium and cardiac tamponade and has a high mortality rate. Most cases involve the left ventricular free wall, and there have been few previous reports of solitary right ventricular free wall rupture. This report is of a case of fatal right ventricular free wall rupture during percutaneous coronary intervention (PCI) for inferior acute myocardial infarction (AMI). CASE REPORT A 76-year-old woman underwent emergency coronary angiography following inferior AMI. During angiography and attempted percutaneous coronary intervention (PCI), sudden onset of cardiac arrest occurred due to cardiac tamponade. Blood was drained from the pericardium by pericardiocentesis. Despite of advanced cardiac support, the patient died. The post mortem findings showed a solitary right ventricular free wall rupture due to inferior myocardial infarction. CONCLUSIONS A rare case is presented of right ventricular free wall rupture following AMI that occurred during PCI. This case demonstrates that early diagnosis and management are required to prevent patient mortality.