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Artéria pulmonar proveniente de canal arterial patente
ORPHA:99049CID-10 · Q25.7CID-11 · LA8B.1DOENÇA RARA

O coração é um órgão muscular presente nos humanos e em outros animais que bombeia o sangue através dos vasos sanguíneos do sistema circulatório. O sangue fornece ao corpo oxigénio e nutrientes e ajuda a eliminar resíduos metabólicos. Nos humanos, o coração situa-se na cavidade torácica entre os pulmões, num espaço denominado mediastino.

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Introdução

O que você precisa saber de cara

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Vaso sanguíneo anômalo que leva sangue arterializado diretamente para a artéria pulmonar, em vez de para a aorta. Causa sobrecarga de fluxo pulmonar e pode levar a hipertensão arterial pulmonar.

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SUS: Cobertura mínimaScore: 15%
CID-10: Q25.7
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Últimos 10 anos4publicações
Pico20212 papers
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20202019Hoje · 2026📈 2021Ano de pico
Publicações por ano (últimos 10 anos)

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Transcatheter Approaches to Palliation for Tetralogy of Fallot.

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual2022

To this day, controversy still exists regarding the optimal method to treat symptomatic neonates and infants with Tetralogy of Fallot (TOF). Symptomatic (severely cyanotic or ductal dependent) infants with TOF can undergo either a staged repair approach (consisting of initial palliation followed by complete repair) or primary repair. Traditionally, initial palliative procedures have been surgical, for example placement of a Blalock-Taussig-Thomas (BTT) shunt. Recent advances in technology have facilitated the introduction of catheter-based procedures as palliative techniques, for example, patent ductus arteriosus (PDA) stenting and right ventricular outflow tract (RVOT) stenting as more durable solutions than balloon pulmonary valvuloplasty (BPV). In this article, we discuss the rationale for these procedures, technical aspects of these procedures and outcomes data compared to traditional surgical procedures. Recent data have suggested that RVOT and PDA stenting procedures offer many advantages over traditional surgical palliative procedures as palliative methods in this patient population. This comes at a cost of increased reintervention burden, which may be considered part of the overall treatment strategy in smaller neonates and can be minimized with a focus on technical aspects and overall treatment strategies. Advanced surgical techniques are required at the eventual complete repair to negotiate removal of stent material and pulmonary artery reconstruction in some instances. Further adoption of catheter based palliative procedures for infants with symptomatic TOF has the potential to tip the outcomes towards favoring a staged approach, particularly in high-risk infants.

#2

Expectant management of prolonged hemolysis following complete transcatheter coil closure of a patent ductus arteriosus after previous pulmonary artery banding: a case report.

BMC cardiovascular disorders2021 Nov 17

Transcatheter coil occlusion has been the treatment of choice for closure of small patent ductus arteriosus (PDA). In spite of its safety, complications such as hemolysis still occasionally occur. And the hemolysis almost always occurs following partial transcatheter closure of PDA; hence, it occurs extremely rarely following complete transcatheter closure of PDA without residual ductal flow. Here, we describe a male newborn who developed prolonged hemolysis following complete transcatheter coil closure of his PDA after previous palliative pulmonary artery banding. Over the following days, we corrected his refractory anemia by repeated blood transfusion with packed red blood cells and frequently monitored his hemoglobin, serum total bilirubin, and serum lactate dehydrogenase. We speculated that the high-velocity pulmonary blood flow jet coming into contact with the extruded part of the coil led to red blood cell mechanical injury, thereby resulting in the hemolysis. We adopted expectant management in expectation of the endothelialization of the coil with a resultant reduction in the hemolysis. The hemolysis, as expected, was reduced gradually until it spontaneously resolved 81 days after coil implantation. This case reminds us that hemolysis can still potentially occur following complete transcatheter coil closure of PDA. It also highlights the importance of preventing coils from extruding into the pulmonary artery in patients after previous pulmonary artery banding.

#3

Prolonged early antenatal indomethacin exposure is safe for fetus and neonate.

The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians2021 Jan

Objective: To evaluate fetal and neonatal safety of early-onset long-term antenatal indomethacin treatment (LIT) for short cervix.Methods: In this cohort study, women started LIT for short cervix (<25 mm) before completing 25 weeks. They followed a standardized regiment of oral indomethacin: 100 mg loading, 50 mg qid for 48 h, 25 mg qid until delivery or at 32 weeks gestational age (GA), whichever comes first. Weekly monitoring for oligohydramnios and ductus arteriosus (DA) constriction included confirmation of compliance with treatment/dose. This approach is established in our clinical practice. To identify LIT complications separate from prematurity, each neonate exposed to LIT were matched to two unexposed neonatal controls within ±3 days of GA of delivery and birth weight of ±10%. Odds ratios for neonatal variables included pulmonary hemorrhage, patent DA (PDA) requiring medical or surgical correction, necrotizing enterocolitis (NEC), spontaneous intestinal perforation (SIP), intraventricular hemorrhage (IVH) grade III-IV, other intracranial hemorrhage (ICH), neonatal mortality, calculated individually, and for total composite morbidity. Statistical determinants of neonatal morbidity were assessed using binary logistic regression. Exposure to LIT, maternal age, parity, BMI, GA at delivery, birth-weight (BW), neonatal gender, cord artery pH, and 5-min Apgar score were independent variables.Results: 166 LIT cases were matched with 332 controls. LIT median duration was 49 (3-108) days. Mean delivery GA was 34 weeks. LIT was stopped for 5 patients (2.9%) with oligohydramnios and 1 (0.6%) with DA constriction, without consequent morbidity. 71 cases (43%) completed LIT, stopping at 32 weeks. 95 stopped early for preterm premature ruptures of membranes (PPROM) (20%), active labor (11%) or patient choice (22%). Odds of any individual complication did not differ between treated cases and controls. LIT was not a statistical determinant of composite morbidity or any individual neonatal problem.Conclusion: Continuous early-onset indomethacin exposure, up to 15 weeks antenatally, did not increase fetal or neonatal complications. This level of safety is permissive to a randomized trial of indomethacin for the treatment of short cervix.

#4

Heart catheterization in adults in a sub-Saharan tertiary centre: 8 years' experience.

Cardiovascular diagnosis and therapy2019 Apr

The goal of the investigation was to analyze the results of heart catheterization in the Cardiac centre Shisong from December 2010 till December 2017. This retrospective study done in the Cardiac centre Shisong in adult patients that underwent a procedure in the catheterization laboratory from December 2010 till December 2017. Three hundred and sixty-five adult underwent a cardiac catheterization procedure during the study period. Among the patients, 126 were female while 239 were male. The mean age at presentation was 52.6±12.9 years old. Patients were coming from neighboring countries: Nigeria n=5 (1.3%), Tchad n=3 (0.8%), Equatorial Guinea n=4 (1%), Democratic republic of Congo n=2 (0.5%). Patients were also coming from all the ten regions of Cameroon: Littoral n=122 (33.2%), Centre n=127 (34.8%), North west n=47 (12.9%), South west n=17 (4.5%), West n=26 (7.1%), North n=7 (1.8%), Adamaoua n=8 (2.1%), Far North n=5 (1.3%), South n=2 (0.5%), East n=4 (1%). Depending on the type of procedures diagnostic coronarography in case with suspicion of coronary artery disease and presurgical coronarography were the main procedures done in 171 patients (46.8%) and in 146 patients (40%) respectively. Diagnostic coronarography was positive in 31 cases (8.4%). In patients with ischemic heart disease (IHD), percutaneous intervention with dilatation of the coronary arteries and implantation of stents was done in 19 cases (5.2%). The remaining 12 cases (3.2%) were having many lesions that could be managed only by coronary artery grafting surgery, done with success in all the cases. In grown up congenital heart disease patients, diagnostic catheterization was done in 48 cases meanwhile interventional catheterization: pulmonary artery valvuloplasty, patent ductus arteriosus (PDA) closure, atrial septal defect closure and decoarctation of the aorta were done in n=11 (3.4%), n=9 (2.4%), n=12 (3.2%), n=6 (1.6%) cases respectively. Coronary heart disease was confirmed by angiography in 8.4% cases, and among grown up congenital heart disease 'patients, atrial septal defect was the pathology the most managed. Heart catheterization is done in the Cardiac centre Shisong with good results.

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Transcatheter Approaches to Palliation for Tetralogy of Fallot.
    Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual· 2022· PMID 35835516mais citado
  2. Expectant management of prolonged hemolysis following complete transcatheter coil closure of a patent ductus arteriosus after previous pulmonary artery banding: a case report.
    BMC cardiovascular disorders· 2021· PMID 34789155mais citado
  3. Prolonged early antenatal indomethacin exposure is safe for fetus and neonate.
    The journal of maternal-fetal &amp; neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians· 2021· PMID 30905227mais citado
  4. Heart catheterization in adults in a sub-Saharan tertiary centre: 8 years' experience.
    Cardiovascular diagnosis and therapy· 2019· PMID 31143639mais citado
  5. Absent or occult pulmonary artery.
    Br Heart J· 1984· PMID 6743435recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:99049(Orphanet)
  2. MONDO:0020390(MONDO)
  3. GARD:19619(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55789324(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

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Artéria pulmonar proveniente de canal arterial patente
Compêndio · Raras BR

Artéria pulmonar proveniente de canal arterial patente

ORPHA:99049 · MONDO:0020390
CID-10
Q25.7 · Outras malformações congênitas da artéria pulmonar
CID-11
MedGen
UMLS
C3163916
Wikidata
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