scholarly journals Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin

2012 ◽  
Vol 33 (5) ◽  
pp. 775-781 ◽  
Author(s):  
Shinichi Takatsuki ◽  
Jeffrey R. Darst ◽  
Bibhuti B. Das ◽  
Thomas E. Fagan ◽  
Robert Wolfe ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Pediatric Patients ◽  
Clinical Manifestations ◽  
Long Term Follow Up ◽  
Isolated Pulmonary Artery

scholarly journals Mid- to long-term follow-up of pediatric patients with coronary artery fistula

2016 ◽  
Vol 115 (7) ◽  
pp. 571-576 ◽  
Author(s):  
Mao-Hung Lo ◽  
I-Chun Lin ◽  
Kai-Sheng Hsieh ◽  
Chien-Fu Huang ◽  
Shao-Ju Chien ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Pediatric Patients ◽  
Coronary Artery Fistula ◽  
Long Term Follow Up

scholarly journals Balioninė plaučių arterijos valvuloplastika – tiesioginiai, tarpiniai ir vėlyvieji rezultatai: 20 metų patirtis

2009 ◽  
Vol 7 (3-4) ◽  
pp. 0-0
Author(s):  
Sigitas Čibiras ◽  
Eugenijus Kosinskas
Keyword(s):  
Surgical Treatment ◽  
Pulmonary Artery ◽  
Pressure Gradient ◽  
Heart Diseases ◽  
Heart Defects ◽  
Residual Pressure ◽  
Mean Pressure ◽  
Long Term Follow Up

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.

Endoscopic Transventricular Transaqueductal Magendie and Luschka Foraminoplasty for Hydrocephalus

Neurosurgery ◽  
2013 ◽  
Vol 74 (4) ◽  
pp. 426-436 ◽  
Author(s):  
Jaime Torres-Corzo ◽  
Juan Sánchez-Rodríguez ◽  
Dominic Cervantes ◽  
Roberto Rodríguez-Della Vecchia ◽  
Fernando Muruato-Araiza ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Endoscopic Third Ventriculostomy ◽  
Outlet Obstruction ◽  
Third Ventriculostomy ◽  
Alternative Technique ◽  
Vp Shunt ◽  
Long Term Follow Up ◽  
Secondary Causes

ABSTRACT BACKGROUND: Routinely, hydrocephalus related to fourth ventricular outlet obstruction (FVOO) has been managed with ventriculoperitoneal (VP) shunting or endoscopic third ventriculostomy (ETV). Few reports on Magendie foraminoplasty exist, and Luschka foraminoplasty has not been described. OBJECTIVE: To present an alternative technique in the management of FVOO via an endoscopic transventricular transaqueductal Magendie and Luschka foraminoplasty and to discuss the indications, technique, findings, and outcomes. METHODS: Between 1994 and 2011, all patients who underwent endoscopic Magendie and Luschka foraminoplasty were analyzed. RESULTS: A total of 33 Magendie (28) and/or Luschka (5) foraminoplasties were performed in 30 patients. Twenty-three were adult and 7 were pediatric patients. The etiology of the FVOO was divided into primary etiologies (congenital membrane in 5 and atresia in 2) and secondary causes (neurocysticercosis in 14 patients, bacterial meningitis in 9). Fifteen (50%) had previously failed procedures. Intraoperative findings that led to Magendie/Luschka foraminoplasty were ETV not feasible to perform, nonpatent basal subarachnoid space, or primary FVOO. Minor postoperative complications were seen in 3 patients. Only 26 patients had long-term follow-up; 17 (65.3%) of these had clinical improvement and did not require further procedures. Nine (34.7%) did not improve. Eight required another procedure (7 shunts, and 1 endoscopic procedure). One patient died. CONCLUSION: Flexible neuroendoscopic transventricular transforaminal Magendie and Luschka foraminoplasty is feasible and safe. These procedures may prove to be viable alternatives to standard ETV and VP shunt in appropriate patients. Adequate intraoperative assessment of ETV success is necessary to identify patients who will benefit.

Long-Term Follow-Up of Pediatric Patients After Congenital Coronary Artery Fistula Closure

2020 ◽  
Vol 41 (7) ◽  
pp. 1346-1353
Author(s):  
Xiaoyong Li ◽  
Laichun Song ◽  
Ming Xu ◽  
Gangcheng Zhang ◽  
Jing Jin
Keyword(s):  
Coronary Artery ◽  
Pediatric Patients ◽  
Coronary Artery Fistula ◽  
Fistula Closure ◽  
Long Term Follow Up ◽  
Congenital Coronary Artery Fistula

P4355Echocardiographic estimation of right ventricle wall tension: hemodynamic comparison and long term follow up

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
U Annone ◽  
P Omede' ◽  
F D'Ascenzo ◽  
A Montefusco ◽  
A Milan ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Atrial ◽  
Stroke Work ◽  
Work Index ◽  
All Cause Mortality ◽  
Long Term Follow Up ◽  
Stroke Work Index

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.

scholarly journals Long Term Follow-Up on Pediatric Cases With Congenital Myasthenic Syndromes—A Retrospective Single Centre Cohort Study

2020 ◽  
Vol 14 ◽  
Author(s):  
Adela Della Marina ◽  
Eva Wibbeler ◽  
Angela Abicht ◽  
Heike Kölbel ◽  
Hanns Lochmüller ◽  
...  
Keyword(s):  
Treatment Response ◽  
Pediatric Patients ◽  
Muscular Weakness ◽  
Congenital Myasthenic Syndromes ◽  
Follow Up Studies ◽  
Bulbar Symptoms ◽  
Long Term Follow Up ◽  
Myasthenic Syndromes

Introduction: Congenital myasthenic syndromes (CMS) refer to a heterogenic group of neuromuscular transmission disorders. CMS-subtypes are diverse regarding exercise intolerance and muscular weakness, varying from mild symptoms to life-limiting forms with neonatal onset. Long-term follow-up studies on disease progression and treatment-response in pediatric patients are rare.Patients and Methods: We analyzed retrospective clinical and medication data in a cohort of 32 CMS-patients including the application of a standardized, not yet validated test (CMS-ST) to examine muscular strength and endurance in 21 patients at the last follow-up. Findings obtained in our cohort were compared with long-term follow-up studies of (adult) CMS-cohorts from the literature by considering the underlying molecular mechanisms. Outcomes of CMS-ST were compared to results of normal clinical assessment.Results: Thirty-two pediatric patients with defects in eight different CMS-genes were followed by a median time of 12.8 years. Fifty-nine percentage of patients manifested with first symptoms as neonates, 35% as infants. While 53% of patients presented a reduced walking distance, 34% were wheelchair-bound. Even under adequate therapy with pyridostigmine (PS) and 3,4-diaminopyridine, CHAT-mutations led to the progression of muscular weakness partly in combination with persistent respiratory and bulbar symptoms. RAPSN, CHRND, and CHRNB1 patients with neonatal manifestation, early respiratory problems, and bulbar symptoms showed a good and maintained treatment response. CHAT and CHRNE patients required higher PS dosages, whereas RAPSN patients needed a lower mean dosage at the last follow-up. The benefits of short-term medication and long-term progression of symptoms were highly dependent on the specific genetic defect. CMS-ST was carried out in 17/21 patients, determined affected muscle groups including bulbar and ocular symptoms, some of which were not reported by the patients.Conclusions: Our findings and comparison with the literature- suggest a better treatment-response and less severe progression of symptoms present in patients suffering from mutations in CMS-genes directly associated with receptor deficiency, while patients with defects leading to synaptopathy and presynaptic defects tend to have worse outcomes. Assessment of affected muscular groups and clinical symptoms by CMS-ST may be a useful tool for optimal therapeutic management of the patients, especially for future clinical studies.

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