Abstract 063: Diagnostic and Treatment Patterns of Cardiac Sarcoidosis: a Single Center Experience

Author(s):  
Anna Grodzinsky ◽  
Timothy Fendler ◽  
Bhaskar Bhardwaj ◽  
Akshit Sharma ◽  
Stephanie Lawhorn ◽  
...  

Background: Clinically manifest cardiac involvement occurs in approximately 5% of patients with sarcoidosis. The principal manifestations of cardiac sarcoidosis (CS) are conduction abnormalities, ventricular arrhythmias, and heart failure. In 2014, the first international guideline for the diagnosis and management of CS was published. There are emerging data to support cardiac MRI and PET imaging in the diagnosis of CS. Additionally, despite a paucity of data, immunosuppression therapy (primarily with corticosteroids) has been advocated for the treatment of clinically manifest CS. Device therapy, primarily with implantable cardioverter-defibrillators, is often recommended for patients with clinically manifest disease. There are few contemporary descriptions of treatment and outcomes related to CS. In this case series, we describe diagnostic and treatment patterns of thirty patients with CS. Methods: We performed a retrospective chart review of 30 patients with diagnosed CS. We used a spreadsheet with deidentified patient information to describe baseline patient characteristics including age, presenting symptoms, diagnostic modality, duration of follow up, medication regimen and follow up imaging, as well as the variables listed above concerning those who are deceased. Results: Of thirty patients included in this case series, age at diagnosis ranged from 28-69, the diagnostic modalities included autopsy of explanted heart, cardiac MRI in combination with mediastinal lymph node biopsy, endomyocardial biopsy, cardiac MRI, and cardiac PET. For the majority of patients, dyspnea was the presenting symptom. Most patient presented with NYHA class I or II symptoms. Half of patients underwent ICD implantation. Eleven patients had documented arrhythmias, including ventricular tachycardia, complete heart block, and atrial fibrillation. Of our cohort, eight patients have concomitant pulmonary sarcoidosis. Follow up duration has ranged between 1 and 12 years. Five patients proceeded to cardiac transplantation. Two patients are deceased, including one patient who suffered recurrence of cardiac sarcoidosis following orthotropic heart transplantation. Conclusions: In this case series of thirty patients with cardiac sarcoidosis, we describe that patients are commonly undergoing cardiac MR and PET imaging to support the diagnosis of CS. Tissue diagnosis remains a standard. Immunomodulators and cardiac device are frequently used, and further follow up data would be helpful in describing outcomes related to these therapies. This descriptions may prompt longer term observational studies that document diagnostic and therapeutic modalities, as well as cardiovascular outcomes, related to cardiac sarcoidosis.

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.


2019 ◽  
Vol 24 (3) ◽  
pp. 246-257 ◽  
Author(s):  
Jennifer L. Quon ◽  
Lily H. Kim ◽  
Peter H. Hwang ◽  
Zara M. Patel ◽  
Gerald A. Grant ◽  
...  

OBJECTIVETransnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors.METHODSThe authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon.RESULTSThe series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1–120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8–120 months). Most patients (76%) were offered adjuvant therapy.CONCLUSIONSIn this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Dusi ◽  
L Pugliese ◽  
I Passarelli ◽  
R Camporotondo ◽  
M Driussi ◽  
...  

Abstract Background Left cardiac sympathetic denervation (LCSD) is an established therapy for refractory ventricular arrhythmias (VAs) in channelopathies. A multicentric American and Indian case series suggested a greater efficacy of bilateral denervation (BCSD) in patients with structural heart disease (SHD). Purpose To describe our single-center experience with BCSD in SHD. Methods Nine patients (78% male, mean 55±18 yrs, mean LVEF 31±14%) with SHD and refractory VAs underwent BCSD. All had a Video-Assisted Thoracoscopic Surgery (VATS), in 2 cases associated with the robotic technique. The underlying cardiomyopathy (CMP) was non-ischemic (NICMP) in most cases (n=5, 55%), ischemic in 2 cases, arrhythmogenic right ventricular (ARVC) in one and related to lamin A/C deficiency in one. All patients had an ICD, 44% (n=4) a CRT-D. NYHA functional class I was present in 4 patients, the rest were in NYHA class II (n=3) or III (n=2). Three patients were candidates to heart transplant/LV assistance device. The arrhythmic burden pre BCSD included in 7 pts (78%) a history of electrical storm (ES); the median number of shocks/patient in the 12 months before BCSD was 5 (IQ range 3–18). Except for 2 patients with previous thyrotoxicosis, the remaining were either on amiodarone (n=6) or on sotalol (n=1) before BCSD. Main BCSD indications were represented by drug refractory fast VT in 7 pts (cycle <250 msec) and by recurrent monomorphic VT episodes (mean cycle 351 msec) after endocardial VT ablation in 2 patients. Results No major complication occurred. One patient (NICMP, NYHA II), has an uneventful follow up (FU) of less than 1 month and was excluded from the efficacy analysis. The median FU in the remaining 8 patients is 10 months (IQ range 6–19), during which the median number of shocks/patients was 0.5 (IQ range 0–3). Overall, 4 patients (50%) had ICD shock recurrences. Two cases (mean LVEF 17.5%, NYHA class III) had an ES during severe hemodynamic instability and subsequently died because of cardiogenic shock respectively 1 and 7 months after BCSD. One case had three, not consecutive ICD shocks 20 months after BCSD in the setting of severe amiodarone-induced thyrotoxicosis. Finally, one patient received a single intra-hospital ICD shock 5 days after BCSD before reintroduction of full-dose beta-blockers. The figure summarizes ICD shocks burden in the 6 months before and after BCSD. Among the 5 patients with NICMP/ARVC (4 in NYHA class I), only 1 had a single ICD shock recurrence. ICD shocks pre versus post BCSD, n=8 Conclusions Our case series, although numerically small, has a good follow-up and is the first reported in Europe. The results are in agreement with the suggested remarkable efficacy of BCSD in patients with good functional capacity and fast VAs. Therefore, cardiac sympathetic denervation should always be considered in patients with SHD and refractory ventricular tachyarrhythmias, especially in case VT ablation is either not indicated or fails.


2019 ◽  
Vol 50 (03) ◽  
pp. 188-192
Author(s):  
Saša Ilovar ◽  
Mirjana Benedik ◽  
Tina Vesnaver ◽  
Damjan Osredkar

Background Brain aneurysms are rare in the pediatric population. The diagnosis of a brain aneurysm in a child may be difficult because of its infrequency and often subtle or nonspecific clinical presentation. Endovascular therapy and microsurgical treatment are increasingly used approaches in treating children, possibly contributing to favorable outcomes if patients are treated in a timely manner. Objective We were interested in the clinical presentation, symptoms, diagnostics, treatment, and follow-up of pediatric patients with brain aneurysms in Slovenia. Methods This was a retrospective review of medical documentation of children with intracranial aneurysms treated at the University Children's Hospital in Ljubljana, Slovenia, from January 1998 to December 2017. Results We identified a cohort of eight children (median age: 14.9 years; range: 2.8–17.7). The estimated incidence of pediatric brain aneurysms in Slovenia is 0.12/100,000 children per year. We observed a male predominance (1.7:1). Half of the patients presented with acute onset of neurologic symptoms and three with subarachnoid hemorrhage. One of the patients had a related stroke. The presenting symptoms were tonic–clonic seizures, hemiparesis, paresthesias, speech disturbance, and cranial nerve palsy. The other half of aneurysms were identified incidentally. Five patients had anterior circulation aneurysms; the most prevalent location was the internal carotid artery. One patient was treated with surgical procedures, four patients were treated with endovascular procedures, and three patients were treated conservatively. Outcome was excellent in all patients. Conclusion Endovascular interventions and microsurgical procedures appear to be safe and effective in the treatment of brain aneurysms in the pediatric population. Asymptomatic patients with brain aneurysms need close follow-up.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Yoshimori An ◽  
Kenji Ando ◽  
Michio Nagashima ◽  
Masato Fukunaga ◽  
Kenichi Hiroshima ◽  
...  

Background: There are still limited data on the mortality for a long-term follow-up and the clinical factors influencing appropriate therapies in Japanese patients with implantable cardioverter-defibrillator (ICD) for primary prevention, who satisfied the criteria in Multicenter Automatic Defibrillator Implantation Trial 2 (MADIT2). Methods: Between January 2000 and December 2012, a total of 436 patients without prior ventricular arrhythmic event underwent ICD implantation for primary prevention at our institution. Among these patients, we enrolled consecutive 122 patients (69±10 years, male: 84%, biventricular-pacing: 54%, median follow-up: 1390 days) who met the MADIT2 criteria; left ventricular ejection fraction (LVEF) ≤30% with ischemic heart disease, more than 4 weeks after myocardial infarction. Results: At the 3 years of follow-up, the mortality rate (21%) was comparable with that of the original MADIT2 ICD group (20%). The Kaplan-Meier event rate for appropriate ICD therapy (shock and anti-tachycardia pacing therapy) (35%) was also similar to that of the original MADIT2 ICD group (32%). Multivariate analysis by Cox regression model revealed that left ventricular diastolic diameter (LVDd) ≥60mm (Hazard Ratio [HR]: 1.65, 95% Confidence Interval [CI]: 1.16-2.14, P=0.004) and non-sustained ventricular tachycardia (NSVT) (HR: 1.55, 95%CI: 1.13-2.15, P=0.007) were independent predictors for appropriate ICD therapy. On the other hand, LVEF, NYHA class, biventricular-pacing, amiodarone or inducibility of ventricular arrhythmia was not associated with appropriate ICD therapy. Conclusion: Appropriate ICD therapy was delivered in Japanese primary prevention patients as often as in the original MADIT2 ICD group and strongly predicted by dilated left ventricle and NSVT.


Author(s):  
Pedro Brandão ◽  
Juliana Silva-Rocha ◽  
Ana Rita Pinto ◽  
Fernanda Costa

Gestational trophoblastic diseases are abnormalities of the trophoblastic tissue development. They have Β-hCG as tumour marker, similar clinical manifestations but different pathological features, management and prognosis. Gestational trophoblastic neoplasia includes all forms of gestational trophoblastic disease that are invasive and/or metastasize and correspond to 10% of this group of diseases. The authors performed a retrospective review of all patients referred to our department between 1st January 2011 and 30th June 2016. Presenting symptoms, Β-hCG levels, obstetric and personal history and microscopic features were reviewed. 4 cases were identified - 2 invasive complete and 1 partial hydatidiform moles and 1 placental site trophoblastic tumour. Management and follow-up were individualized. Neither persistence nor recurrences were diagnosed after primary treatment. These case series highlights the importance of an accurate diagnosis and a long-term follow-up considering the risk of persistency or malignancy, as gestational trophoblastic neoplasia usually have good response to the adequate therapy.


2020 ◽  
Vol 8 (2) ◽  
pp. 232596712090372 ◽  
Author(s):  
Travis J. Dekker ◽  
Matthew D. Crawford ◽  
Nicholas N. DePhillipo ◽  
Mitchell I. Kennedy ◽  
W. Jeffrey Grantham ◽  
...  

Background: Clinical outcomes pertaining to isolated lateral fabellectomy in the setting of fabella syndrome are limited to small case reports at this time. Purpose: To assess the most common presenting symptoms, clinical outcomes, and satisfaction after fabella excision in the setting of fabella syndrome. Study Design: Case series; Level of evidence, 4. Methods: Consecutive patients with a minimum of 21-month follow-up after isolated fabellectomy for fabella syndrome were reviewed retrospectively. Clinical outcome scores of the following domains were collected: Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) score and Lysholm knee survey, along with a simple numeric patient satisfaction score (range, 1-10; 10 = “very satisfied”). Statistical analysis was performed using paired t tests for all clinical outcome data. Results: A total of 11 isolated fabella excisions were included in 10 patients with isolated lateral-sided knee pain in the setting of fabella syndrome (8 males, 2 females), with a mean age of 36.9 years (range, 23-58 years) and a mean follow-up of 2.4 years (range, 21-47 months). A total of 8 patients (80%) were able to return to full desired activities, including sports. Only 5 of 11 (45%) excisions had concomitant lateral femoral condyle cartilage pathology. There were significant improvements across multiple WOMAC domains, and the WOMAC total score improved from 28.5 ± 17.6 preoperatively to 11.6 ± 10.2 postoperatively ( P < .05). Lysholm scores significantly improved from 66.6 ± 23.1 preoperatively to 80.2 ± 13.9 postoperatively ( P = .044). Overall patient-reported satisfaction was 8.8 ± 1.6. Conclusion: Fabella excision in the setting of fabella syndrome demonstrated improvements in clinical outcome scores, high rate of returning to preinjury level of activities, and low risk of complications or need for additional surgical procedures.


Neurosurgery ◽  
2010 ◽  
Vol 67 (2) ◽  
pp. 278-285 ◽  
Author(s):  
Manu S. Goyal ◽  
Christopher L. Hallemeier ◽  
Gregory J. Zipfel ◽  
Keith M. Rich ◽  
Robert L. Grubb ◽  
...  

Abstract OBJECTIVE To define the clinical characteristics and outcome of patients with idiopathic stenosis or occlusion of the basal arteries, without moyamoya collateral vessel formation. METHODS We identified patients who presented to our institution from 1996 to 2005 with occlusive disease of the distal internal carotid artery or the proximal middle or anterior cerebral arteries demonstrated by digital subtraction cerebral angiography. We excluded those with evidence of atherosclerotic disease, systemic vasculitis, moyamoya phenomenon, or any other condition that could otherwise explain their arterial occlusive disease. Medical records were reviewed for presenting symptoms and clinical characteristics. Outcome was determined from chart review and phone interviews. RESULTS Twelve patients were identified. All presented with transient ischemic attack or stroke. Eleven were women. Age at presentation ranged from 34 to 71 years. Nine had a history of hypertension; 5 had unilateral intracranial disease. Recurrent stroke on medical therapy occurred in none of the 5 during an average follow-up of 29 months. Seven had bilateral disease. Ischemic stroke occurred between 2 and 107 months after the initial event in 5 of 8 medically treated hemispheres. Moyamoya collateral vessels developed in 1 patient as shown on follow-up angiography. CONCLUSION The clinical features and outcome of these patients are similar to those reported in large case series of North American patients with moyamoya phenomenon. These data suggest a common etiology for the basal arterial occlusive process and a variable ability to form moyamoya collateral vessels.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Kamada ◽  
K Ishibashi ◽  
K Nakajima ◽  
N Ueda ◽  
T Kamakura ◽  
...  

Abstract Background Prior ventricular tachycardia (VT) and low left ventricular ejection fraction (LVEF) are the most important prognostic factors in cardiac sarcoidosis (CS). Recently diagnosis of CS was renewed according to Japanese new guidelines. Patients with preserved cardiac function often have VT events, thus new guidelines recommends to assess the implantable cardioverter defibrillator (ICD) implantation for CS patients with preserved LVEF (35%≤LVEF&lt;50%). However, the long-term prognosis of CS patients with preserved LVEF is unclear. Objective In CS patients with preserved LVEF, we evaluated the prognosis between VT manifestation and non-VT manifestation groups at CS diagnosis from Japanese nationwide questionnaire survey. Methods Total of 757 Japanese patients from 57 hospitals who diagnosed CS were examined. Patients who unsatisfied the criteria of the Japanese new guidelines, who had LVEF≤35%, LVEF&gt;50%, or who underwent cardiac transplantations were excluded. 104 patients with LVEF 35–50% (67 females, mean age 60±15 years old, median follow-up periods 2134 days [interquartile range: 758–2935 days]) were analyzed. The prognosis between VT manifestation and non-VT manifestation groups at CS diagnosis were evaluated. Results 30 patients had VT manifestation at CS diagnosis and 24 patients (80%) received ICDs. 74 patients had no VT manifestation at CS diagnosis and 19 patients (44%) received ICDs during follow up period. All-cause mortality was not different between two groups (Figure). Appropriate ICD therapy of non-VT manifestation group was significantly lower compared with that of VT manifestation group (log-rank p=0.001), however considerable number (n=7, 15%) of non-VT manifestation group had appropriate ICD therapy event during follow-up period. Cox hazard analysis revealed that concomitant non-sustained VT (NSVT) with atrioventricular block (AVB) was a predictor of appropriate ICD therapy in non-VT manifestation group. Conclusion This nationwide survey showed that considerable number of CS patients with preserved LVEF had VT events, independent of VT manifestation. Concomitant NSVT with AVB was a predictor of VT events, and ICD implantation should be assessed. Funding Acknowledgement Type of funding source: None


2021 ◽  
pp. 112067212110043
Author(s):  
Hetvi Bhatt ◽  
Linda Okafor ◽  
Rina Bhatt

Purpose: Exposure of orbital implant post enucleation or evisceration remains one of the common complications irrespective of the type of implant used. Dermis fat graft and temporalis fascia have been used to repair the implant exposure in anophthalmic sockets. Tutoplast® pericardium, gamma sterilised dehydrated human pericardium, has been used as a scleral patch graft for glaucoma drainage device exposure and scleral thinning post squint surgery. We report the novel use of Tutoplast® patch graft to repair orbital implant exposure in this case series. Methods: The case notes of three patients who received Tutoplast® pericardium patch graft to repair implant exposure were reviewed. Data regarding presenting symptoms, implant type, time to exposure from primary surgery, post-operative complications and length of follow up post Tutoplast® pericardium patch graft were collected. Results: Three patients presented with implant exposure following evisceration. The presenting symptoms were recurrent infection, discharging socket and discomfort in all three patients. One patient had a Medpore implant, two had silicone implants. Total follow-up ranged from 9 to 22 months. In all three cases, there was relief from symptoms and the graft had incorporated fully into the surrounding orbital tissue with no recurrent exposure noted during the follow-up period. Conclusion: Tutoplast® pericardium has demonstrated a good safety profile and is a suitable material to use as a patch graft for orbital implant exposure.


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