Combined Surgical and Endoscopic Approach for Ring–Sling Complex

2019 ◽  
Vol 68 (01) ◽  
pp. 051-058
Author(s):  
Giulio Porcedda ◽  
Alice Brambilla ◽  
Silvia Favilli ◽  
Bruno Murzi ◽  
Lorenzo Mirabile ◽  
...  

Background Left pulmonary artery (LPA) sling (PAS) is a vascular ring, which is frequently associated with long-segment tracheal stenosis (TS). Mortality rate in operated children is still high, especially in cases of severe tracheal hypoplasia and/or associated congenital heart defects (CHDs). We report our experience of treatment and follow-up in a pediatric cohort of patients affected by PAS with severe tracheobronchial involvement. Methods From 2005 to 2017, we enrolled 11 children diagnosed with PAS and congenital TS requiring surgical intervention. Echocardiography, computed tomography, and bronchoscopy were performed in all patients. Associated CHD were present in 5 (45%) patients. Tracheal reconstruction techniques included slide tracheoplasty (7/11; 63%), slide tracheoplasty and costal cartilage graft (2/11; 18%), and Hazekamp technique (2/11; 18%).Nine patients underwent LPA direct reimplantation and concomitant tracheoplasty; concomitant surgical repair for CHD was performed in three children. Results Over a mean follow-up of 30 months (range: 3–75 months), a late mortality of 18% was registered; no early death occurred. Good flow through LPA could be documented in all patients. Ten children required operative bronchoscopies (mean: 16/patients) aimed at stent positioning/removal, treatment of granulomas, and tracheobronchial dilatation. Conclusions Severe tracheobronchial stenosis and associated CHD were the main determinants for hospitalization time, intensive assistance, and repeated endoscopic procedures.Patients affected by PAS/TS complex require a careful management at high-specialized centers providing multidisciplinary team.Respiratory endoscopy may play a central role both in preoperatory assessment and in postoperative management of patients showing severe tracheobronchial involvement.

2021 ◽  
pp. 000348942098742
Author(s):  
Stephen R. Chorney ◽  
Joanne Stow ◽  
Luv R. Javia ◽  
Karen B. Zur ◽  
Ian N. Jacobs ◽  
...  

Objectives: Tracheocutaneous fistula (TCF) is a common occurrence after pediatric tracheostomy decannulation. However, the persistence of TCF after staged reconstruction of the pediatric airway is not well-described. The primary objective was to determine the rate of persistent TCF after successful decannulation in children with staged open airway reconstruction. Methods: A case series with chart review of children who underwent decannulation after double-stage laryngotracheal reconstruction between 2017 and 2019. Results: A total of 26 children were included. The most common open airway procedure was anterior and posterior costal cartilage grafting (84.6%, 22/26). Median age at decannulation was 3.4 years (IQR: 2.8-4.3) and occurred 7.0 months (IQR: 4.3-10.4) after airway reconstruction. TCF persisted in 84.6% (22/26) of children while 15.4% (4/26) of stomas closed spontaneously. All closures were identified by the one-month follow-up visit. There was no difference in age at tracheostomy ( P = .86), age at decannulation ( P = .97), duration of tracheostomy ( P = .43), or gestational age ( P = .23) between stomas that persisted or closed. Median diameter of stent used at reconstruction was larger in TCFs that persisted (7.0 mm vs 6.5 mm, P = .03). Tracheostomy tube diameter ( P = .02) and stent size ( P < .01) correlated with persistence of TCF on multivariable logistic regression analysis. There were 16 surgical closure procedures, which occurred at a median of 14.4 months (IQR: 11.4-15.4) after decannulation. Techniques included 56.3% (9/16) by primary closure, 18.8% (3/16) by secondary intention and 25% (4/16) by cartilage tracheoplasty. The overall success of closure was 93.8% (15/16) at latest follow-up. Conclusions: Persistent TCF occurs in 85% of children who are successfully decannulated after staged open airway reconstruction. Spontaneous closure could be identified by 1 month after decannulation and was more likely when smaller stents and tracheostomy tubes were utilized. Surgeons should counsel families on the frequency of TCF and the potential for additional procedures needed for closure.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Amir ◽  
N Soffair ◽  
G Frenkel ◽  
E Bruckheimer ◽  
E Nachum ◽  
...  

Abstract Background Vascular anomalies of the Aortic arch can cause respiratory symptoms in children due to tracheal compression. Treatment consists of division of the vascular rings, nevertheless data regarding mid- term results is scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. Methods Between2007–2014, 85 children underwent vascular ring surgery. 51 had Double Aortic Arch (DAA, 60%), 31 Right Arch with Aberrant Subclavian Artery (RAA & ALSA, 36.5%) and 3 had RAA, mirror image branching & left ductus arteriosus (3.5%). Mean age and weight at operation were 12.4±13months and 8.6±4.1 kg respectively. Mid-term follow-up included clinical follow up by a pulmonologist (38 patients, 44.7%) and a telephone questionnaire (71 patients, 83%) performed 57±25.7 months after surgery. Results In most patients, symptomatic relief occurred in less than 6 months (table 1). Mid –term follow up revealed that although most parents described a significant improvement in their child's respiratory symptoms (95%), a significant number of patients described some residual respiratory symptoms (table 2). We did not find any significant association between age at surgery (under 6 month), or vascular ring anatomy (DAA vs. RAA&ASA) and the presence of residual symptoms at follow up Conclusions Surgical division of vascular rings results in a significant clinical improvement within one year, nevertheless many patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms in mid- term follow up. Further evaluation whether a more aggressive surgical approach is warranted in order to decrease the incidence long-term symptoms. Funding Acknowledgement Type of funding source: None


2015 ◽  
Vol 26 (1) ◽  
pp. 127-138 ◽  
Author(s):  
Daniela Laux ◽  
Lucile Houyel ◽  
Fanny Bajolle ◽  
Francesca Raimondi ◽  
Younes Boudjemline ◽  
...  

AbstractBackgroundDiscordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach.ObjectiveTo report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up.MethodsA retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections.ResultsSegmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors.DiscussionDiagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.


2021 ◽  
pp. 1-3
Author(s):  
Mariana Lemos ◽  
Miguel Fogaça da Mata ◽  
Ana Coutinho Santos

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.


2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Wojciech Ścierski ◽  
Grażyna Lisowska ◽  
Grzegorz Namysłowski ◽  
Maciej Misiołek ◽  
Jan Pilch ◽  
...  

The aim of this study was to evaluate a novel composite material for tracheal reconstruction in an ovine model. A polymer containing various forms of carbon fibers (roving, woven, and nonwoven fabric) impregnated with polysulfone (PSU) was used to create cylindrical tracheal implants, 3 cm in length and 2.5 cm in diameter. Each implant, reinforced with five rings made of PSU-impregnated carbon-fiber roving, had three external layers made of carbon-fiber woven fabric and the inner layer formed of carbon-fiber nonwoven fabric. The inner surface of five implants was additionally coated with polyurethane (PU), to promote migration of respiratory epithelium. The implants were used to repair tracheal defects (involving four tracheal rings) in 10 sheep (9-12 months of age; 40-50 kg body weight). Macroscopic and microscopic characteristics of the implants and tracheal anastomoses were examined 4 and 24 weeks after implantation. At the end of the follow-up period, outer surfaces of the implants were covered with the tissue which to various degree resembled histological structure of normal tracheal wall. In turn, inner surfaces of the prostheses were covered only with vascularized connective tissue. Inner polyurethane coating did not improve the outcomes of tracheal reconstruction and promoted excessive granulation, which contributed to moderate to severe stenosis at the tracheal anastomoses. The hereby presented preliminary findings constitute a valuable source of data for future research on a tracheal implant being optimally adjusted for medical needs.


2014 ◽  
Vol 142 (9-10) ◽  
pp. 542-546
Author(s):  
Vojislav Parezanovic ◽  
Milan Djukic ◽  
Sanja Dzelebdzic ◽  
Tamara Ilisic ◽  
Igor Stefanovic ◽  
...  

Introduction. Pulmonary artery stenosis (PS) is a congenital heart defect which occurs in 10% of all congenital heart defects. Pulmonary balloon valvuloplasty (BVP) has been the treatment of choice of PS over the last 30 years. Objective. The purpose of this study was to evaluate the efficacy of this method based on middle-term hospital follow-up, and safety of BVP based on our experience. Methods. The study included 88 patients diagnosed with PS. The patients were divided into three groups based on the severity of the disease. Also, they were divided into two age groups in order to analyze the frequency of complications. Hemodynamic measurements and echocardiography results were recorded before, 24-36 hours after BVP and at the end of follow-up. Results. The studied group involved patients of average age 3.75?4.3 years (20 days to 17 years). Immediately after BVP a significant decrease of pressure gradient across the pulmonary valve (PV) was recorded in all patients; this result was similar in all 3 groups of patients regardless of the severity of stenosis (p<0.001). Complications of BVP occurred most commonly in children up to 12 months of age (ventricular tachycardia 4.5% and supraventricular tachycardia 6.8%). Pulmonary valve insufficiency after dilatation occurred in 6.6% of cases, and was most common in children aged up to 12 months. In 87 (98.9%) patients BVP was a definitive solution, and a significant residual stenosis was not recorded during follow-up. Conclusion. BVP is a safe and effective procedure in the treatment of isolated PS in children, regardless of the severity of stenosis but also regardless of patients? age.


Author(s):  
Philip Moons ◽  
Sandra Skogby ◽  
Ewa‐Lena Bratt ◽  
Liesl Zühlke ◽  
Ariane Marelli ◽  
...  

Background The majority of people born with congenital heart disease require lifelong cardiac follow‐up. However, discontinuity of care is a recognized problem and appears to increase around the transition to adulthood. We performed a systematic review and meta‐analysis to estimate the proportion of adolescents and emerging adults with congenital heart disease discontinuing cardiac follow‐up. In pooled data, we investigated regional differences, disparities by disease complexity, and the impact of transition programs on the discontinuity of care. Methods and Results Searches were performed in PubMed, Embase, Cinahl, and Web of Science. We identified 17 studies, which enrolled 6847 patients. A random effects meta‐analysis of single proportions was performed according to the DerSimonian‐Laird method. Moderator effects were computed to explore sources for heterogeneity. Discontinuity proportions ranged from 3.6% to 62.7%, with a pooled estimated proportion of 26.1% (95% CI, 19.2%–34.6%). A trend toward more discontinuity was observed in simple heart defects (33.7%; 95% CI, 15.6%–58.3%), compared with moderate (25.7%; 95% CI, 15.2%–40.1%) or complex congenital heart disease (22.3%; 95% CI, 16.5%–29.4%) ( P =0.2372). Studies from the United States (34.0%; 95% CI, 24.3%–45.4%), Canada (25.7%; 95% CI, 17.0%–36.7%), and Europe (6.5%; 95% CI, 5.3%–7.9%) differed significantly ( P =0.0004). Transition programs were shown to have the potential to reduce discontinuity of care (12.7%; 95% CI, 2.8%–42.3%) compared with usual care (36.2%; 95% CI, 22.8%–52.2%) ( P =0.1119). Conclusions This meta‐analysis showed that there is a high proportion of discontinuity of care in young people with congenital heart disease. The highest discontinuity proportions were observed in studies from the United States and in patients with simple heart defects. It is suggested that transition programs have a protective effect. Registration URL: www.crd.york.ac.uk/prospero . Unique identifier: CRD42020182413.


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.


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