How the Real-Life Diagnostic and Therapeutic Approach Changed in the Last Two Decades in the Thrombocythemic Patients with Ph- Negative Myeloproliferative Neoplasm. Report on 2388 Subjects of the Registro Italiano Trombocitemie (RIT)

Abstract Background: the Registro Italiano Trombocitemie (RIT) was activated mainly to evaluate the diagnosis and therapy appropriateness in the thrombocythemic patients with Ph-negative myeloproliferative neoplasm (MPN) observed in the adhering centers. Objective: to evaluate how the diagnostic and therapeutic approach changed in the RIT patients diagnosed with thrombocythemic MPN in the last two decades. Methods: the RIT centers registered by a web-based system during the years 2005-2014 their thrombocythemic MPN patients, and semesterly updated their follow-up data. For patients diagnosed before 2005, the data on diagnosis and prior follow-up were retrospectively collected. The diagnostic process and the initial treatment (started into the first year after diagnosis) were comparatively analyzed in the patients diagnosed before and after 2005. Results: the RIT centers registered 2629 patients. 2388 of them, object of this analysis, were diagnosed between1995 and 2014: 1098 (46%) in the decade 1995-2004 (Group I), and 1290 (54%) in the decade 2005-2014 (Group II). The diagnostic process in the patients of Group II and Group I included bone marrow biopsy (BMB, performed into 1 year and before any cytoreduction): 85% vs 80%, p <0.001; clonality tests (JAK2 and/or others): 80% vs 9%, p<0.001; cytogenetic evaluation: 55% vs 57%, p 0.58; abdominal echography: 46% vs 41%, p 0.016. The patients of Group II, as compared with those of Group I, showed a similar gender distribution (M/F ratio 0.61 vs 0.65, p 0.452), and had at diagnosis: a higher age (median 60 vs 57 years, p <0.001; age >60 years in 50% vs 45% of cases, p <0.01); a similar rate of prior thrombosis (19% vs 19%), prior hemorrhage (3% vs 4%), CVRFs 69% vs 66%), splenomegaly (25% vs 25%), and hepatomegaly (22% vs 24%); a lower rate of disease-related symptoms (36% vs 40%, p 0.04); a higher rate of comorbidities (55% vs 51%, p 0.03). Moreover, they had: a lower platelet (PLT) count (median 737 vs 775 x 109/L, p <0.001; <600 x 109/L in 22% vs 14% of cases, p <0.001); a similar white blood cell (WBC) count (median 8.6 vs 8.5 x 109/L; >10 x 109/L in 28% vs 26% cases); a similar median levels of hematocrit (HCT %, in females 41.4 vs 41.0; in males 44.7 vs 44.6) and hemoglobin (Hb g/dL, in females 13.8 vs 13.6; in males 15.0 vs 14.9). The BMB, revised according to the WHO 2008 criteria, showed a not different distribution (p 0.21) of ET (64% vs 61%), ep-PMF (16% vs 17%), PMF (3% vs 2%), PV (4% vs 4%), and U-MPN (13% vs 16%. The JAK2 V617F mutation in patients of Group II (at diagnosis) and of Group I (after diagnosis) was found in 62% and in 58% of tested cases (p 0.054), respectively. The patients at high standard thrombotic risk were 58% vs 52%, p 0.004. In the patients of Group II and Group I the distribution of the treatment started into the first year was significantly different (p<0.001): AntiPLT 29% vs 24%, CYT 13% vs 16%, CYT+AntiPLT 49% vs 46%, CYT ± AntiPLT 62% vs 62%, AntiPLT ± CYT 78% vs 69%. The treatment CYT ± AntiPLT was started in the patients at high standard thrombotic risk with a rate of 81% vs 80%, respectively, and in the patients at low standard thrombotic risk in 37% vs 43% of cases, respectively. The initial treatment CYT±AntiPLT was related, in multivariate analysis, both in patients of Group II and Group I, with older age (>60 and 40-60 vs <40 years, p <0.001), higher PLT count (>1000 and 700-1000 vs <700 x 109/L, p <0.001), prior thrombosis (p <0.001), symptoms (p <0.001). Noteworthy, no relationship was found with JAK2 mutation, and WHO diagnosis. Conclusion: in the studied thrombocythemic MPN patients the real-life diagnostic approach was improved after 2005 not only due to the routine use of JAK2 tests, but also due to the higher rate of BMB done (85% vs 80%). The appropriateness of the cytoreductive treatment (CYT±AntiPLT started into one year from diagnosis) remained high in patients at high standard thrombotic risk (over 80% of cases were treated). Concomitantly, the inappropriate use of the cytoreductive drugs in patients at low standard thrombotic risk appreciably decreased (from 43% to 37% of cases). Moreover, it has to be remarked that the therapeutic approach was significantly influenced not only by older age and prior thrombosis, but also by thrombocytosis (PLT count >700 x 109/L), disease-related symptoms, and inconstantly by leukocytosis and CVRFs. Table Table. Disclosures Gugliotta: SHIRE Co.: Honoraria. Gugliotta:Bristol Myers Squibb: Consultancy, Honoraria; Novartis: Consultancy, Honoraria.

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Growth and development in girls with Turner's syndrome during early therapy with low doses of estradiol

Acta Endocrinologica ◽

10.1530/acta.0.112s157 ◽

1986 ◽

Vol 113 (4_Suppl) ◽

pp. S157-S163 ◽

Cited By ~ 8

Author(s):

K.W. KASTRUP ◽

_ _

Keyword(s):

Growth Velocity ◽

Final Height ◽

Bone Age ◽

Growth Spurt ◽

First Year ◽

Turner's Syndrome ◽

Turner’S Syndrome ◽

Early Therapy ◽

Group I ◽

Group Ii

Abstract Early therapy with a low dose of estrogen (estradiol-17β) was given to 33 girls with Turner's syndrome (T.s.) for a period of 4 years. The dose (0.25-2 mg/day) was adjusted every 3 months to maintain plasma estradiol in the normal concentration range for bone age. Growth velocity was compared with that of untreated girls with T.s. All girls were above age 10 years. Bone age was below 10 years in 11 girls (group I) and above 10 years in 22 girls (group II). Growth velocity in the first year of treatment in group I 7.5 ± 1.3 cm (SD) with mean SD score (SDS) of +4.3 and in group II 4.9 ± 1.3 with mean SDS of +3.5. Growth velocity decreased in the following years to 1.6 ± 1.0 cm, SDS -1.44 in group I and 0.9 ± 0.6cm, SDS -2.34 in group II during the fourth year. Withdrawal bleeding occurred in 16 girls of group II after the mean of 23 (range 15-33) months and in 3 girls of group I after 15 to 51 months of treatment. The treatment did not cause an inappropriate acceleration of pubertal development. Breast development appeared in most girls by 3 months of treatment. Pubic hair appeared by 12 months of treatment in group I; it was present in most girls in group II at start of treatment. Final height is known for 12 girls of group II; it was 144.2 ± 4.5 cm. The final height as predicted at the start of therapy was 142.2 ± 5.3 cm. Bone age advanced in the first year of treatment by 2 years. Early treatment with small doses of estrogens induces a growth spurt and normalizes the events of puberty. This will presumably decrease the psychological risks associated with abnormally delayed development.

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Comparative Assessment of Autogenous Cancellous Bone Graft and Bovine-Derived Demineralized Bone Matrix for Secondary Alveolar Bone Grafting in Patients With Unilateral Cleft Lip and Palate

The Cleft Palate-Craniofacial Journal ◽

10.1177/10556656211025197 ◽

2021 ◽

pp. 105566562110251

Author(s):

Vijay Kumar ◽

Vidya Rattan ◽

Sachin Rai ◽

Satinder Pal Singh ◽

Jai Kumar Mahajan

Keyword(s):

Bone Graft ◽

Iliac Crest ◽

Demineralized Bone Matrix ◽

Bone Matrix ◽

Volumetric Analysis ◽

Group I ◽

Alveolar Bone Grafting ◽

Group Ii ◽

Demineralized Bone

Objective: Comparison between bovine-derived demineralized bone matrix (DMBM) and iliac crest graft over long term for secondary alveolar bone grafting (SABG) in patients with unilateral cleft lip and palate (UCLP) in terms of radiological and clinical outcomes. Design: Prospective, randomized, parallel groups, double-blind, controlled trial. Setting: Unit of Oral and Maxillofacial Surgery, Oral Health Science Centre, Postgraduate Institute of Medical Education & Research, Chandigarh. Participants: Twenty patients with UCLP. Interventions: Patients were allocated into group I (Iliac crest bone graft) and group II (DMBM) for SABG. Outcomes were assessed at 2 weeks, 6 months, and then after mean follow-up period of 63 months. Outcomes Measures: Volumetric analysis of the grafted bone in the alveolar cleft site was done through cone beam computed tomography using Cavalieri principle and modified assessment tool. Clinical assessment was performed in terms of pain, swelling, duration of hospital stay, cost of surgery, alar base symmetry, and donor site morbidity associated with iliac crest harvesting. Results: Volumetric analysis through Cavalieri principle revealed comparable bone uptake at follow-up of 6 months between group I (70%) and group II (69%). Modified assessment tool showed no significant difference between horizontal and vertical bone scores over short- and long-term follow-up. In group II, there was higher cost of surgery, but no donor site morbidity unlike group I. Conclusions: Demineralized bone matrix proved analogous to iliac crest bone graft as per volumetric analysis over shorter period. However, although statistically insignificant, net bone volume achieved was lower than the iliac crest graft at longer follow-up.

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Platelet rich plasma versus extracorporeal shock wave therapy in patients with non-calcific supraspinatous tears

Egyptian Rheumatology and Rehabilitation ◽

10.1186/s43166-020-00020-7 ◽

2020 ◽

Vol 47 (1) ◽

Author(s):

Samah M. Alian ◽

Rabab S. Zaghlol ◽

Sahar S. Khalil

Keyword(s):

Shock Wave ◽

Outcome Measures ◽

Platelet Rich Plasma ◽

Extracorporeal Shock Wave Therapy ◽

Shock Wave Therapy ◽

Tendon Tear ◽

Extracorporeal Shock Wave ◽

Group I ◽

Group Ii

Abstract Background To compare the clinical efficacy of platelet rich plasma (PRP) subacromial injection and extracorporeal shock wave therapy (ESWT) on refractory non-calcific partial thickness supraspinatous tendon tear. Results Significant improvement in all outcome measures achieved in both groups at 4 weeks follow-up period (the end of treatment course) (P< 0.05). At 12 weeks follow-up period, group I, showed significant improvement in all outcome measures compared to baseline and group II; however, in group II, there was no significant difference compared to baseline; moreover, deterioration in the previous improvement was noticed. Conclusion Both PRP (group I) and ESWT (group II) are effective therapeutic methods in the management of refractory non-calcific partial thickness supraspinatous tendon tear; however, PRP (group I) has better long-term effects on both pain and function.

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RECURRENCE RATE AFTER REPEAT TRANSURETHRAL RESECTION AMONG PATIENTS WITH NEWLY DIAGNOSED T1 BLADDER CANCER

10.36106/ijar/7213765 ◽

2021 ◽

pp. 51-54

Author(s):

Md. Shafiqur Rahman ◽

Sanjeeb Bhakta Bista ◽

Md. Sayedul Islam ◽

A. S. M. Shafiul Azam ◽

Md. Shamim Hossain ◽

...

Keyword(s):

Bladder Cancer ◽

Recurrence Rate ◽

Transurethral Resection ◽

Tumor Recurrence ◽

Controlled Trial ◽

Intravesical Therapy ◽

Newly Diagnosed ◽

Group I ◽

Group Ii

Background: Transurethral resection of the bladder tumor (TURBT) is the cornerstone of diagnosis and TURBT followed by selective adjuvant intravesical chemotherapy or immunotherapy is the gold standard of treatment for the patients with non-muscle invasive bladder cancer (NMIBC). Even after complete resection of the tumor there is high risk of residual tumor and subsequent recurrence and progression of the disease. The recurrence of T1 tumor is found to be around 71 % within 5 years and High grade T1 lesions recur in more than 80% of the cases and progress in 50% of the patients within 3 years. Disease status at 3 months after initial resection is an important predictor of subsequent recurrence and progression. To assess the tumor recurrence rat Objective: e among patients with newly diagnosed T1 bladder cancer between repeat transurethral resection group and single TURBT group. Ra Type of study: ndomized controlled trial. Place of study: Department of urology, BSMMU, Dhaka, Bangladesh. This Randomi Materials And Methods: zed Controlled Trial was conducted in urology department of BSMMU, Dhaka, Bangladesh from February 2017 till September 2018. A total of 50 patients, diagnosed as a case of T1 bladder cancer and who fulll the selection criteria were randomly divided in two groups consisting of 25 patients in each group. After informed consent, repeat TUR was done after 4 weeks of the initial TURBT for only 22 patients in group I since 3 of the patients did not show up on the scheduled date of surgery. Whereas, repeat TUR was not done for other 25 patients in group II. All the patients in both the groups were further treated with intravesical therapy according to the histopathological report. The patients in both the groups were followed up at 3 months and 6 months of the initial intervention where detailed history was taken, relevant investigations were done and check cystoscopy was done. TURBT was done for recurrent tumors. Out of 22 patients in group I, 2 patients were excluded for follow up on the basis of repeat TUR ndings. Whereas in group II, 2 patients missed the follow up and one of them missed the normal scheduled dose of intravesical therapy. Hence, 22 patients completed the study in group II. The baseline variables like age and Results: sex were similar in both the groups with higher male predominance. There was no statistical signicant difference in tumor characteristics such as size, number and grade of the tumor between the two groups. Out of 22 patients in group I who underwent repeat TUR at 4 weeks of initial TUR, residual disease was found in 8 (36.36%) patients. One of the patient was found to have T2 disease and 1 patient had CIS during repeat TUR. These 2 patients were not followed up since the treatment strategy changed after the results of repeat TUR. After 6 months of follow up of all the patients, 2 (10%) out of 20 patients in group I were found to have tumor recurrence however, in group II, 9 (40.9%) patients had tumor recurrence. The tumor recurrence rate between the two groups was found to be statistically signicant (p=0.023). In the light of ndings of this Conclusion: study, it can be concluded that performing repeat transurethral resection in patients with newly diagnosed T1 bladder cancer at 4 weeks of initial TURBT, helps to detect signicant number of residual tumor and reduce early recurrence rate of the tumor.

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Good Operative Effects of Patellofemoral Arthroplasty Combined With the Tibial Tubercle Transfer for Isolated Patellofemoral Arthritis Patients With an Increased Tibial Tuberosity-Trochlear Groove Distance

10.21203/rs.3.rs-291693/v1 ◽

2021 ◽

Author(s):

Conglei Dong ◽

Chao Zhao ◽

Ming Li ◽

Huijun Kang ◽

Kang Piao ◽

...

Keyword(s):

Patellofemoral Joint ◽

Tibial Tuberosity ◽

Tibial Tubercle ◽

Trochlear Groove ◽

Patellofemoral Arthritis ◽

Patellofemoral Arthroplasty ◽

Group I ◽

Tibial Tubercle Transfer ◽

Group Ii

Abstract BackgroundThe objective of present study was to investigate the operative effect of patellofemoral arthroplasty combined with the tibial tubercle transfer for isolated patellofemoral arthritis patients with an increased tibial tuberosity-trochlear groove distance (>20mm). MethodsA prospective study was performed between November 2012 and December 2017. Finally, thirty-six cases, with a mean age of 61.1 ± 7.3 years, were admitted to our study. A total of 17 patients underwent patellofemoral arthroplasty combined with tibial tubercle transfer, and 19 patients underwent patellofemoral arthroplasty only. All eligible patients had CT scans preoperatively and at 12 months follow-up, to assess the stability of the patellofemoral joint on axial slices. In addition, the demographic and clinical features of all the patients were asked. Knee balance was assessed with the single leg stance test and timed get up and go, and functionality was evaluated with stair climbing test and the Western Ontario and McMaster Universities Osteoarthritis Index score. (P>0.05) ResultsPreoperatively, the data regarding the knee balance and functionality were not significantly different between the two Groups (P>0.05). Many measurements showed significant differences between the two groups at the last follow-up. Significant differences were seen in well-known measurements such as the SLST (Group I, 28.9 s (SD 7.5); Group II, 20.3 s (SD 5.9); p < 0.001), TGUG (Group I, 13.4 s (SD 3.2); Group II, 16.8 s (SD 3.1); p < 0.001), and SCT (Group I, 18.6 s (SD 6.8); Group II, 24.5 s (SD 8.7); p < 0.001). Additionally, the median WOMAC score was improved from 62.7 (SD 11.2) preoperatively to 25.7 (SD 8.2) one year postoperatively in Group I and from 64.1 (SD 10.7) to 36.2 (SD 9.7) in Group II, which were also significantly different between the groups. ConclusionsFor such special IPA patients with an increased TT-TG (>20mm), the combined operation of PFA combined with TTT can better restore the involutional relationship of patellofemoral joint and further improve the balance and function of knee joint.

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Do stone size and impaction influence therapeutic approach to proximal ureteral stones?

Vojnosanitetski pregled ◽

10.2298/vsp0902129r ◽

2009 ◽

Vol 66 (2) ◽

pp. 129-133 ◽

Cited By ~ 1

Author(s):

Slobodan Radulovic ◽

Aleksandra Vuksanovic ◽

Dragica Milenkovic-Petronic ◽

Bozo Vavic

Keyword(s):

Therapeutic Approach ◽

Ureteral Stone ◽

Treatment Success ◽

Ureteral Stones ◽

Stone Size ◽

Extracorporeal Shock Wave ◽

Group I ◽

Significant Difference ◽

Group Ii ◽

Proximal Ureteral Stone

Background/Aim. Primary therapeutic approach to lumbar ureteral stones is still contraversial. The aim of the study was to investigate the influence of stone impaction and size on the effectiveness of proximal ureteral stone lithotripsy. Methods. A total of 123 patients with proximal ureteral stones were investigated in this prospective study performed in a 10- month period. The patients were divided into the group I - 86 patients treated with extracorporeal shock wave lithotripsy (ESWL) and the group II - 37 patients treated with 'Swiss' Lithoclast. In the group I, 49 stones (57%) were classified as impacted, while 20 stones (23.3%) were larger than 100 mm2. In the group II, 26 stones (70.3%) were impacted, and 11 stones (29.7%) were larger than 100 mm2. Stones were defined as impacted by the radiographic, echosonographic as well as endoscopic findings in the group II of patients. Stone size was presented in mm2. Chemical composition of stones were almost the same in both groups of the patients. Results. Generally, there was no statistically significant difference in the treatment success between the groups. However, stones larger than 100 mm2 were statistically more successfully treated endoscopically, while there was no statistical difference in the treatment success of impacted stones between these two groups. Conclusion. ESWL can by considered as primary first therapeutic approach in treatment of all proximal ureteral stones except for stones larger than 100 mm2 that should primarily be treated endoscopically.

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A Seven-year Follow-up Study of Patient Satisfaction with Three-implant-retained Mandibular Overdentures

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2021.5896 ◽

2021 ◽

Vol 9 (D) ◽

pp. 103-107

Author(s):

Hisham Samir ElGabry

Keyword(s):

Long Term Results ◽

Complete Dentures ◽

Patients Satisfaction ◽

Follow Up Study ◽

Group I ◽

Significant Difference ◽

Male Patients ◽

Group Ii ◽

Anterior Mandible

PURPOSE: This study aimed to compare patients’ satisfaction with mandibular overdentures retained by three-splinted implants versus conventional complete denture wearers during a 7-year follow-up study period. MATERIALS AND METHODS: Thirty edentulous male patients (mean age: 60 years) were carefully selected and divided into two equal groups. All patients received a new set of complete dentures. Group I patients received three implants in the anterior mandible and were connected after 3 months with bars, clips, and loaded. Group II patients received conventional complete dentures. Patients’ satisfaction was recorded for both groups at 3 weeks (baseline) and after 1, 3, 5, and 7 years. Patients were then asked to grade their overdentures/dentures on a visual analog scale and written questionnaire to evaluate their overall satisfaction. RESULTS: Satisfaction scores of Group I patients were found to be statistically significantly higher than that of Group II patients (p < 0.05) at 3, 5, and 7 years follow-up, meanwhile, no statistically significant difference was found at baseline or after 12 months. CONCLUSION: The long-term results suggest that three-implant-retained mandibular overdenture with a clip-bar attachment appears to be a successful rehabilitation strategy which is superior to conventional dentures for patients with advanced ridge resorption.

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Microsurgical C-2 ganglionectomy for chronic intractable occipital pain

Journal of Neurosurgery ◽

10.3171/jns.1998.89.3.0359 ◽

1998 ◽

Vol 89 (3) ◽

pp. 359-365 ◽

Cited By ~ 57

Author(s):

Andres M. Lozano ◽

Graham Vanderlinden ◽

Robert Bachoo ◽

Peter Rothbart

Keyword(s):

Axonal Regeneration ◽

Excellent Result ◽

Spontaneous Pain ◽

Content Type ◽

Group I ◽

Experienced Pain ◽

Pain Outcome ◽

Group Ii ◽

Fine Print

Object. The authors evaluated the effectiveness of microsurgical C-2 ganglionectomy in 39 patients with medically refractory chronic occipital pain. In this procedure the neurons transmitting sensory inputs from the occiput are removed and, unlike peripheral nerve ablation, axonal regeneration is not possible. Methods. The patients in this series had symptoms for 1 to 43 years. In 22 patients the occipital pain was caused by trauma; in 17 patients the pain was spontaneous. Pain relief failed in 17 patients who had undergone a previous occipital neurectomy or C-2 rhizolysis. Twenty-three patients experienced pain that was described as shocklike, electric, shooting, jabbing, stabbing, sharp, or exploding (Group I). Eight patients described their pain as dull, pounding, aching, throbbing, or pressurelike (Group II). The patients underwent unilateral or bilateral C-2 open microsurgical ganglionectomies. The postoperative follow-up period ranged from 19 to 48 months. Nineteen patients experienced an excellent result (> 90% reduction in pain). Pain caused by trauma or that described using Group I terms responded best to ganglionectomy (80% good or excellent response). In contrast, the majority of the patients with nontraumatic pain or those described using Group II descriptors did not achieve favorable results. Conclusions. The authors conclude that: 1) patients who suffer from chronic occipital pain after having sustained injury obtain worthwhile benefit from microsurgical C-2 ganglionectomy; 2) patients suffering from migraine, tension, and vascular headaches involving the occipital area are most often not helped by this operation; and 3) terms such as “shock,” “electric,” “shooting,” “jabbing,” and “sharp” used to describe occipital pain predict a favorable pain outcome following a C-2 ganglionectomy.

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Abstract WP230: Real-life Detection Rate of Insertable Cardiac Monitors in Patients with ESUS

Stroke ◽

10.1161/str.48.suppl_1.wp230 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

Lars-Peder M Pallesen ◽

Julia Mayer ◽

Kristian Barlinn ◽

Jessica Barlinn ◽

Alexandra Prakapenia ◽

...

Keyword(s):

Detection Rate ◽

Real Life ◽

Treatment Modalities ◽

First Year ◽

Eligibility Criteria ◽

Strauss Syndrome ◽

Life Detection ◽

Baseline Characteristics ◽

Churg Strauss

Introduction: It has recently been shown that insertable cardiac monitors (ICM) reveal intermittent atrial fibrillation (AF) in up to 12.4% of patients with embolic stroke of undetermined source (ESUS) at 1 year follow-up. We sought to analyze the detection rate of intermittent AF with ICM in real-life clinical practice. Methods: We prospectively studied consecutive patients with ESUS who received an ICM (NeuroLinq®) from 01/2014 to 06/2015). Baseline characteristics, treatment modalities and AF detection rate in the first year following ICM placement was assessed. Results: We enrolled 75 ESUS patients (64% men, median age 61 years) who underwent ICM placement. Two patients were excluded due to diagnosis of prion disease and Churg-Strauss syndrome after placement of ICM. Sixty-nine of 73 (95%) patients complied with the eligibility criteria adopted by the CRYSTAL-AF study. Intermittent AF was detected in 14 (19%) patients with a median time from ICM placement to diagnosis of 57 (range, 312) days. Conclusions: Our data suggest that the real-life detection rate of intermittent AF with ICM placed in patients with ESUS is comparable to that one observed under clinical trial conditions.

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Evolving Solutions in the Surgical Treatment of Isolated Pulmonary Valve Stenosis

10.37191/mapsci-jccr-2(3)-044 ◽

2021 ◽

Author(s):

Sonia B. Albanese Albanese

Keyword(s):

Surgical Treatment ◽

Pressure Gradient ◽

Pulmonary Valve ◽

Balloon Valvuloplasty ◽

Pulmonary Valve Stenosis ◽

Group I ◽

Mean Pressure ◽

Valve Function ◽

Group Ii

Background: Surgical treatment of isolated pulmonary valve stenosis in infants and children has evolved over the years, shifting from the original exclusive aim at lowering right ventricle pressure to the current concomitant focus on preserving pulmonary valve function. In our study, we sought to analyze the effect of such evolving philosophy on long-term results. Methods: All consecutive patients treated in our center between July 1983 and March 2019 were included. Patients were categorized into 2 groups based on the introduction into treatment practice of sparing valve techniques (1995). Actuarial survival, freedom from reintervention on the right outflow tract, transvalvular mean pressure gradient decrease, and pulmonary valve function at follow-up were analyzed. Results: One hundred twenty-three patients operated before (Group I, n=81) or since (Group II, n=42) 1995 were enrolled in the study. Mean age and weight were 3.0 ± 0.36 years and 16.6 ± 1.7kg, respectively. Early mortality occurred exclusively in 3 patients of Group 1. Transvalvular mean pressure gradient decreased in the entire patient population (from 63.28 ± 12.9mmHg to 16.46 ± 7.9mmHg). At a mean follow-up interval of 4.9 ± 33 years, freedom from death was comparable, but freedom from right ventricular outflow tract reintervention was significantly greater in Group II. Although the transvalvular gradient remained stable over time in non-reoperated survivors (mean value of 16.46 ± 7.9mmHg), pulmonary valve function on 2D-Echo showed severe incompetence in 2 patients of Group I and just mild to moderate incompetence in 20 patients of Group II, with a significantly negative effect of unsuccessful preoperative pulmonary balloon valvuloplasty (14/20 vs 6/20, p=0.025) in the latter. Conclusion: Current pulmonary valve sparing techniques are associated with better results, particularly in terms of freedom from re-interventions and pulmonary valve function at follow-up. Balloon valvuloplasty prior to surgery may worsen operative results, promoting pulmonary insufficiency and therefore should probably be avoided in all patients in whom anatomical characteristics predict failure of percutaneous therapy.

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