scholarly journals Long-term outcome of patients with Marfan syndrome with previous aortic surgery but native aortic roots

2020 ◽  
Vol 58 (6) ◽  
pp. 1289-1295
Author(s):  
Selim Mosbahi ◽  
Murat Yildiz ◽  
Paul-Philipp Heinisch ◽  
Bettina Langhammer ◽  
Silvan Jungi ◽  
...  

Abstract OBJECTIVES The goal of this study was to report the long-term outcomes of patients with Marfan syndrome who had aortic surgery on any aortic segment except for the replacement of the aortic root itself. METHODS An observational retrospective single-centre study was conducted with 115 Marfan syndrome patients who underwent 189 major aortic interventions from 1995 until 2018. Patients without aortic root replacement were identified and aortic root growth was analysed over time. RESULTS Eleven of 115 patients (9.5%) did not have aortic root replacement during a follow-up of 10.5 [standard deviation (SD) 5.7] years and a mean age at last follow-up of 53.9 (SD 13.4) years. Patients without root replacement did not suffer less frequently from any type of acute aortic dissection (type A 27% vs 25%, P = 0.999; type B 36% vs 25%, P = 0.474). Patients with native aortic roots did not undergo fewer aortic interventions than those with aortic root replacement [12/11, mean 1.09 (SD 0.54) operations/patient vs 177/104, mean 1.7 (SD 1.3); P = 0.128]. Progression of the aortic root dimension was 0.5 (SD 0.3) mm/year in the group of patients with native aortic roots. CONCLUSIONS Current data suggest that 10% of patients with Marfan syndrome with previous aortic surgery will be free from aortic root replacement until the sixth decade of life.

2019 ◽  
Vol 68 (04) ◽  
pp. 301-308
Author(s):  
Thomas Puehler ◽  
Sandra Freitag-Wolf ◽  
Christine Friedrich ◽  
Mohamed Salem ◽  
Jochen Renner ◽  
...  

Objective Therapy of complex aortic root pathologies remains a great surgical challenge. Though different graft materials are available to replace the often-destroyed aortic root, long durability and freedom from reoperation of the latter are still under debate. The aim of our study was to investigate patients' postoperative outcome after implantation of the BioIntegral conduit in complex aortic root pathologies. Methods From February 2014 to May 2017, 33 consecutive patients (69.7% male) with a median age of 73 (57.5; 76.2) years underwent aortic root replacement with the BioIntegral conduit at our institution. Severe aortic valve endocarditis (78.8%) was the predominant indication for surgery. In 28 patients (84.9%), implantation was performed as redo or in 87.8% as urgent and emergent surgery. Primary end-point was the 30-day survival time, evaluated by Cox regression analysis. Secondary midterm outcome and graft-related reoperation were analyzed. Results Median follow-up for all patients was 178 (8; 659) days. Median EuroScore II was 19.9% (13.4; 29.9). Freedom from reoperation was 97%. The overall 30-day mortality rate was 33% mainly caused by multiorgan failure in six (18.2%) patients and cardiac failure in five (15.1%) patients. One further death occurred during follow-up at day 156. None was directly conduit-related. Graft reinfection after the 4th surgery with basal abscess formation occurred in only one patient (3%). Early echocardiographic assessment of the valve revealed good functional results. A higher EuroScore II was significantly associated with a poorer 30-day survival time (hazard ratio, 1.039; 95% confidence interval, 1.015–1.063, p = 0.001). Conclusion Aortic root replacement for complex pathologies is associated with substantial 30-day mortality, but survival of patients after discharge from hospital was stable. Early functional status of the BioIntegral valve was good. Though freedom from re-operation was low, long-term outcome and long-term functional status have to be further evaluated.


VASA ◽  
2002 ◽  
Vol 31 (1) ◽  
pp. 36-42 ◽  
Author(s):  
. Bucek ◽  
Hudak ◽  
Schnürer ◽  
Ahmadi ◽  
Wolfram ◽  
...  

Background: We investigated the long-term clinical results of percutaneous transluminal angioplasty (PTA) in patients with peripheral arterial occlusive disease (PAOD) and the influence of different parameters on the primary success rate, the rate of complications and the long-term outcome. Patients and methods: We reviewed clinical and hemodynamic follow-up data of 166 consecutive patients treated with PTA in 1987 in our department. Results: PTA improved the clinical situation in 79.4% of patients with iliac lesions and in 88.3% of patients with femoro-popliteal lesions. The clinical stage and ankle brachial index (ABI) post-interventional could be improved significantly (each P < 0,001), the same results were observed at the end of follow-up (each P < 0,001). Major complications occurred in 11 patients (6.6%). The rate of primary clinical long-term success for suprainguinal lesions was 55% and 38% after 5 and 10 years (femoro-popliteal 44% and 33%), respectively, the corresponding data for secondary clinical long-term success were 63% and 56% (60% and 55%). Older age (P = 0,017) and lower ABI pre-interventional (P = 0,019) significantly deteriorated primary clinical long-term success for suprainguinal lesions, while no factor could be identified influencing the outcome of femoro-popliteal lesions significantly. Conclusion: Besides an acceptable success rate with a low rate of severe complications, our results demonstrate favourable long-term clinical results of PTA in patients with PAOD.


Crisis ◽  
1999 ◽  
Vol 20 (3) ◽  
pp. 115-120 ◽  
Author(s):  
Stephen Curran ◽  
Michael Fitzgerald ◽  
Vincent T Greene

There are few long-term follow-up studies of parasuicides incorporating face-to-face interviews. To date no study has evaluated the prevalence of psychiatric morbidity at long-term follow-up of parasuicides using diagnostic rating scales, nor has any study examined parental bonding issues in this population. We attempted a prospective follow-up of 85 parasuicide cases an average of 8½ years later. Psychiatric morbidity, social functioning, and recollections of the parenting style of their parents were assessed using the Clinical Interview Schedule, the Social Maladjustment Scale, and the Parental Bonding Instrument, respectively. Thirty-nine persons in total were interviewed, 19 of whom were well and 20 of whom had psychiatric morbidity. Five had died during the follow-up period, 3 by suicide. Migration, refusals, and untraceability were common. Parasuicide was associated with parental overprotection during childhood. Long-term outcome is poor, especially among those who engaged in repeated parasuicides.


2019 ◽  
Vol 24 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Bianca K. den Ottelander ◽  
Robbin de Goederen ◽  
Marie-Lise C. van Veelen ◽  
Stephanie D. C. van de Beeten ◽  
Maarten H. Lequin ◽  
...  

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.


Author(s):  
Sandeep Mohindra ◽  
Manjul Tripathi ◽  
Aman Batish ◽  
Ankur Kapoor ◽  
Ninad Ramesh Patil ◽  
...  

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Keskin ◽  
H.C Tokgoz ◽  
O.Y Akbal ◽  
A Hakgor ◽  
S Tanyeri ◽  
...  

Abstract Background and aims Although syncope (S) has been reported as one of the presenting findings in patients (pts) with acute pulmonary embolism (APE), its clinical and haemodynamic correlates and impacts on the long-term outcome in this setting remains to be determined. In this single-centre study we evaluated the clinical and haemodynamic significance of S in APE in initial asessment, and during short- and long-term follow-up period. Methods Our study was based on the retrospective and prospective analysis of the overall 641 pts (age 65 (51–74 IQR) yrs, 56.2% female) with diagnosis of documented APE who underwent anticoagulant (n=207), thrombolytic (n=164), utrasound-facilitated thrombolysis (UFT) (n=218) or rheolytic thrombectomy (RT) (n=52). The systematic work- up including multidetector computed tomography (MDCT), Echo, biomarkers, and PE severity indexes were performed in all pts, and Qanadli score (QS) was used as the measure of the thrombotic burden in the pulmonary arteries (PA). Results The S as the presenting symptom In 30.2% of pts with APE. At baseline assessment, S(+) vs S(−) APE subgroups had a significantly shorter symptom-diagnosis interval, a higher risk status according to the significant elevations in troponin T, D-dimer, the higher PE severity indexes, a more deteriorated right ventricle/left ventricle ratio (RV/LV r), right atrial/left atrial ratio (LA/RAr) and RV longitudinal function indexes including tricuspid annular planary excursion (TAPSE) and tissue velocity (St), a significantly higher PA obstructive burden as assessed by QS and PA pressures. Thrombolytic therapy (36.2% vs 21%, p&lt;0.001) and RT (11.9% vs 6.47%, p=0.037) were more frequently utilized S(+) as compared to S(−) group. However, all these differences between two subgroups were found to disappear after evidence-based APE treatments. In-hospital mortality (IHM) (12.95% vs 6%, p=0.007) and minor bleeding (10.36% vs 2.9%, p&lt;0.001) were significantly higher in S(+) pts as compared to those in S(−) subgroup. Binominal logistic regression analysis revealed that PESI score and RV/LVr independently associated with S while IHM was only predicted by age and heart rate. The COX proportional hazard method showed that RV/LVr at discharge and malignancy were independently associated with cumulative mortality during follow-up duration of 620 (200–1170 IQ) days. Conclusions The presence of S in pts with APE was found to be asociated with a higher PA obstructive burden, a more deteriorated RV function and haemodynamics and higher risk status which may need more agressive reperfusion treatments. However, in the presence of the optimal treatments, S did not predict neither in-hospital outcome, nor long-term mortality. Funding Acknowledgement Type of funding source: None


Author(s):  
Loay Shoubash ◽  
Jörg Baldauf ◽  
Marc Matthes ◽  
Michael Kirsch ◽  
Matthias Rath ◽  
...  

AbstractThe aim of this study is to analyze the long-term quality of life after surgery of cavernoma. A monocentric retrospective study was conducted on 69 patients with cavernoma treated microsurgically between 2000 and 2016. The eloquence was adopted from Spetzler-Martin definition. A most recent follow-up was elicited between 2017 and 2019, in which the quality of life (QoL) was evaluated with the Short Form-12 questionnaire (SF12). Forty-one lesions were in eloquent group (EG), 22 in non-eloquent group (NEG), 3 in orbit, and 3 in the spinal cord. Postoperative worsening of the modified Rankin scale (mRS) occurred in 19.5% of cases in EG versus 4.5% in NEG. After a mean follow-up of 6.5 years (SD 4.6), the neurological status was better or unchanged compared to baseline in 85.4% of EG and 100% of NEG. Regarding QoL assessment of 44 patients (EG n = 27, NEG n = 14) attended the last follow-up. Patients after eloquent cavernoma resection reported a non-inferior QoL in most SF12 domains (except for physical role) compared to NEG. However, they reported general health perception inferior to norms, which was affected by the limited physical and emotional roles. At a late follow-up, the surgical morbidity was transient in the NEG and mostly recovered in the EG. The QoL comparison between eloquent and non-eloquent cavernomas created interesting and new data after prolonged follow-up. These results add value for decision-making as well as patient counseling for future encountered cases. Preoperative evaluation of QoL is recommended for future studies to assess QoL dynamics.


Sign in / Sign up

Export Citation Format

Share Document