scholarly journals A case of Behcet disease with pulmonary artery pseudoaneurysm: long term follow-up

2010 ◽  
Vol 16 (03) ◽  
pp. 346-349 ◽  
Author(s):  
M.H.S. Modaghegh ◽  
G.H. Kazemzadeh ◽  
M.H. Jokar
PEDIATRICS ◽  
1989 ◽  
Vol 83 (6) ◽  
pp. 986-992
Author(s):  
Yardena Rakover ◽  
Hanna Adar ◽  
Itamar Tal ◽  
Yaron Lang ◽  
Amos Kedar

Behcet disease is rare in children. There are only two reports of Behcet disease in childhood, describing seven patients. Three pediatric patients are described, in whom the age of onset ranged from 6 to 11 years. Aphthous stomatitis and arthritis were present in all of the patients; genital ulcers, iridocylitis, erythema nodosum, and CNS involvement were present in two patients. Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil. In two patients, follow-up of more than 10 years was done, with complete cure in one patient and benign course of illness in the other. Because of the rarity of the disease in childhood and the difficulty in making the diagnosis, there is not enough awareness by pediatricians concerning this disease.


2004 ◽  
Vol 127 (2) ◽  
pp. 590-592 ◽  
Author(s):  
Yong Han Yoon ◽  
Kwang Ho Kim ◽  
Wan Ki Baek ◽  
Jung Taek Kim ◽  
Kuk Hee Shon ◽  
...  

2009 ◽  
Vol 7 (3-4) ◽  
pp. 0-0
Author(s):  
Sigitas Čibiras ◽  
Eugenijus Kosinskas

Sigitas Čibiras, Eugenijus KosinskasVilniaus universiteto Širdies ir kraujagyslių ligų klinika, Vilniaus universiteto ligoninės Santariškių klinikos, Santariškių g. 2, LT-08661 VilniusE-mail: [email protected] Įvadas Darbo tikslas – apibendrinti 20 metų patirtį ir įvertinti įgimtos plaučių arterijos (PA) stenozės balioninės valvuloplastikos (BPV) tiesioginius, tarpinius ir vėlyvuosius rezultatus. Ligoniai ir metodai 1987–2007 metais Vilniaus širdies ligų klinikoje buvo atlikta 101 BPV, ligonių amžius nuo 1 paros iki – 39 metų. BPV atlikta esant spaudimo per PA vožtuvą skirtumui > 30 mm Hg. Ligoniai prieš BPV suskirstyti į dvi grupes pagal tai, ar pradinis spaudimo per PA vožtuvą skirtumas <50 mm Hg (1 gruoė), ar > 50 mm Hg (2 grupė). Analizuotas duomenų kitimas tiesiogiai po BPV, tarpiniu laikotarpiu (iki dvejų metų po BVP), vėlyvuoju laikotarpiu (praėjus daugiau kaip dvejiems metams). Ligoniai po BPV buvo suskirstyti į dvi grupes: turintys liekamąjį spaudimo skirtumą iki 36 mm Hg ir daugiau kaip 36 mm Hg. Rezultatai BPV atlikta 18 pacientų, kurių spaudimo per PA vožtuvą skirtumas < 50 mm Hg. Iškart po BVP spaudimo skirtumas per PA vožtuvą sumažėjo nuo 39,5 ± 5 iki 15,83 ± 8,37 mm Hg, tarpiniu laikotarpiu – iki 20 ± 6 mm Hg, vėlyvuoju – iki 21,5 ± 5 mm Hg. BPV atliktos 83 pacientams, kurių spaudimo per PA vožtuvą skirtumas > 50 mm Hg. Tiesiogiai po BVP vidutinis spaudimo skirtumas sumažėjo nuo 81,31 ± 21,28 iki 31,32 ± 13,82 mm Hg, tarpiniu laikotarpiu – iki 27,56 ± 12,71 mm Hg, vėlyvuoju – iki 19,89 ± 10,12 mm Hg. Esant liekamajam spaudimo skirtumui po BPV < 36 mm Hg (58 ligoniai), tarpiniu lakotarpiu vidutinis spaudimo skirtumas 23,66 ± 9,29 mm Hg, vėlyvuoju – 16,85 ± 7,98 mm Hg. Esant liekamajam spaudimo skirtumui po BPV > 36 mm Hg (21 ligonis), tarpiniu laikotarpiu vidutinis spaudimo skirtumas 51,99 ± 20,61 mm Hg, vėlyvuoju – 35,7 ± 16 mm Hg. Vėlyvuoju laikotarpiu spaudimo skirtumas mažėja, bet didėja PA nesandarumas. Tuoj po BPV nesandarumas nustatytas 7 %, tarpiniu laikotarpiu – 53 %, vėlyvuoju – 81,7 % ligonių. Išvados BPV yra gerai toleruojamas ir veiksmingas nechirurginis gydymo būdas. Daugumai ligonių išryškėja vėlyvasis nedidelis plaučių arterijos nesandarumas, kurį retai prireikia gydyti chirurginiu būdu. Mūsų studija rodo, kad tinkama ligonių atranka leidžia pasiekti gerų tiesioginių, tarpinius ir vėlyvųjų rezultatų. Reikšminiai žodžiai: įgimtos širdies ydos, įgimta plaučių arterijos vožtuvo stenozė, balioninė valvuloplastika. Balloon pulmonary artery valvuloplasty – immediate, mid-term and long-term follow-up results: 20-year experience Sigitas Čibiras, Eugenijus KosinskasVilnius University Clinic of Heart and Vascular Medicine, Vilnius University Hospital Santariškių Klinikos, Santariškių str. 2, LT-08661 Vilnius, LithuaniaE-mail: [email protected] Background To analyze immediate, mid-term and long-term follow-up results after percutaneous balloon pulmonary valvuloplasty (BPV) of congenital pulmonary artery (PA) stenosis for a 20-year period. Patients and methods During 1987–2007, in the Vilnius Clinic of Heart Diseases 101 BPV were performed, the patients’ age range being 1 day – 39 years. BPV was performed with the primary PA valvular pressure gradient > 30 mm Hg. Patients before BPV had been divided into two groups: (1) with primary PA pressure gradient < 50 mm Hg; (2) with primary PA pressure gradient > 50 mm Hg. Data were analyzed immediately after BPV, in a mid-term (2 years) and a long-term (more than 2 years) follow-up. The same patients after BPV were divided into two groups: with residual pressure gradient < 36 mmHg and > 36 mmHg. Results Eighteen BPV were performed with the primary PA pressure gradient < 50 mm Hg: the immediate mean pressure gradient decreased from 39.5  ±   5 to 15.83 ± 8.37 mm Hg, in the mid-term period to 20 ± 6 mm Hg, and in the long-term to 21.5 ± 5 mm Hg. Eighty-three BPV were performed with the primary PA pressure gradient > 50 mm Hg; the immediate mean pressure gradient decreased from 81.31 ± 21.28 mm Hg to 31.32 ± 13.82 mm Hg, in the mid-term period to 20 ± 6 mm Hg and in the long-term period to mm Hg. With the residual pressure gradient after BPV < 36 mm Hg (58 patients), in the mid-term period the pressure gradient decreased to 23.66 ± 9.29 mm Hg and in the long-term period to 16.85 ± 7.98 mm Hg. With the residual pressure gradient after BPV > 36 mm Hg (21 patients), in the mid-term period the pressure gradient decreased to 51.99 ± 20.61 mm Hg and in the long-term period to 35.7 ± 16 mm Hg. In the long-term follow-up, the pressure gradient decreased, but PA regurgitation (PAR) was progressive. Immediately after BPV, PAR was seen in 7%, in mid-term follow-up in 53 %, and in long-term follow-up in 81.7 % patients. Conclusions BPV is a well tolerated and effective non-surgical treatment method. Late trivial PAR develops in the majority of cases, but rarely requires surgical treatment. Our study has demonstrated that the appropriate patient selection enables achieving good immediate, mid-term and long-term follow-up results. Keywords: congenital heart defects, congenital pulmonary artery stenosis, balloon valvuloplasty.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
U Annone ◽  
P Omede' ◽  
F D'Ascenzo ◽  
A Montefusco ◽  
A Milan ◽  
...  

Abstract Introduction Prognosis in pulmonary hypertension (PH) is strictly linked to right ventricle (RV) failure, which results from uncoupling between RV and the superimposed pressure load; in first phases, coupling between these two actors still be preserved, at the price of augmented right ventricle wall tension (RVWT). Purpose We sought to describe how to estimate RVWT with echocardiography, how it correlates with RV hemodynamics and if it may predict prognosis. Methods A total of 190 patients without overt RV failure, with suspected pulmonary hypertension (PH) to a previous echocardiography, underwent to right heart catheterization (RHC) and nearly-simultaneous echocardiography. We estimated RVWT according to Laplace law (RV length × tricuspid regurgitation peak gradient [TRPG]), in order to predict initial RV stress, and was correlated with RV hemodynamic profile; its potential prognostic impact was tested along with canonical RV function parameters. Results In patients enrolled in our study, RVWT correlated significantly with invasive estimation of right ventricle end diastolic pressure (R 0.343, p<0.001); a significant relationship between RVWT and several hemodynamic variables was observed (mean pulmonary artery pressure, pulmonary artery compliance, transpulmonary gradient, pulmonary vascular resistance, RV telediastolic pressure, right atrial pressure, RV stroke work index; all p<0.001). At a mean follow up of five years and three months, only RVWT predicted all-cause mortality (p 0.036), while TAPSE, TAPSE/TRPG, RV fractional area change and RV S' wave did not. Correlation: RWVT and RV hemodynamic Hemodynamic variable R R2 p value Mean pulmonary artery pressure 0.742 0.550 <0.001 RV differential pressure 0.794 0.630 <0.001 Pulmonary artery pulsatory pressure 0.740 0.547 <0.001 Mean right atrium pressure 0.326 0.106 <0.001 Cardiac index/right atrial pressure 0.209 0.044 0.012 RV stroke work index 0.588 0.346 <0.001 Pulmonary artery compliance 0.449 0.202 <0.001 Pulmonary vascular resistance 0.531 0.282 <0.001 Prognosis: different RV variables Discussion We identified a novel bedside echocardiographic predictor of altered RV hemodynamic, which results precociously altered in patients without overt RV failure, and able to predict all cause mortality at a long term follow up. Further studies are needed to confirm its role in PH patients.


1983 ◽  
Vol 106 (2) ◽  
pp. 381-388 ◽  
Author(s):  
Douglas S. Moodie ◽  
Derek Fyfe ◽  
Carl C. Gill ◽  
Sebastian A. Cook ◽  
Bruce W. Lytle ◽  
...  

2012 ◽  
Vol 33 (5) ◽  
pp. 775-781 ◽  
Author(s):  
Shinichi Takatsuki ◽  
Jeffrey R. Darst ◽  
Bibhuti B. Das ◽  
Thomas E. Fagan ◽  
Robert Wolfe ◽  
...  

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