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1.
World J Pediatr Congenit Heart Surg ; 12(2): 230-233, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33684007

RESUMO

BACKGROUND: During unifocalization procedures for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, collateral arteries are either ligated or detached. Not much is known of the fate of the remaining arterial origins in the long term. Available computed tomography (CT) or magnetic resonance (MR) imaging of the intrathoracic arteries was examined to investigate possible abnormalities at the former position of the collateral arteries as well as ascending aortic diameters. METHODS: From 1989 to 2018, we performed 66 unifocalization procedures in 39 patients. One hundred and twenty-nine collateral arteries were ligated or detached. In 52% (15) of the surviving patients (with a total of 55 ligated or detached collaterals), sufficient imaging of the thoracic aorta from CT (11) and/or MR (9) was available for evaluation. RESULTS: The median interval between unifocalization procedure and imaging was 15 years (interquartile range [IQR]: 9-19 years). In 93% (14) of the scanned patients, 18 blunt ends were detected at the location of a former collateral artery. No aneurysm formation of the descending aorta was observed. The median diameter of the ascending aorta was 35 mm (IQR: 31-40 mm). During follow-up, no aortic dissection or rupture occurred. CONCLUSIONS: Aortic imaging late after unifocalization showed abnormalities in 93% of the scanned patients. Abnormalities consisted mostly of blunt ends of the former collateral artery. We recommend to include routine imaging of the aorta during late follow-up to detect eventual future abnormalities and monitor aortic diameters. Ascending aortic diameters showed slight dilatation with no clinical implications so far.


Assuntos
Aorta Torácica/anormalidades , Procedimentos Cirúrgicos Cardíacos/métodos , Circulação Colateral/fisiologia , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/anormalidades , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Lactente , Ligadura , Imagem Cinética por Ressonância Magnética , Masculino , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia
2.
Medicine (Baltimore) ; 100(3): e24356, 2021 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-33546070

RESUMO

ABSTRACT: To investigate the characteristics of pulmonary artery distensibility (PAD) in patients with acute pulmonary embolism (APE) and to assess the correlation of PAD with APE severity and right ventricular function. A total of 33 patients who underwent retrospective electrocardiogram (ECG)-gated computed tomography pulmonary angiography (CTPA) with a definite diagnosis of APE were included in the study. According to APE severity, the patients were divided into severe (SPE) and non-severe (NSPE) groups. Data from a control group without APE matching the basic demographics of the APE patients were collected. Pulmonary artery distensibility (PAD) and right ventricular function parameters were compared among the 3 groups, their relationships were investigated, and receiver operating characteristic (ROC) curves were used to determine the sensitivity and specificity of the above parameters for the diagnosis of APE severity. The PAD values of the control, NSPE, and SPE groups were (7.877 ±â€Š2.637) × 10-3 mm/Hg, (6.050 ±â€Š2.011) × 10-3 mm/Hg, (4.321 ±â€Š1.717) × 10-3 mm/Hg, respectively (P < .01). There were statistically significant differences in right ventricular function parameters among the 3 groups (P < .05). The correlation analysis between PAD and right ventricular function parameters showed a weak negative correlation (r = -0.281--0.392). The area under the ROC curve of PAD was 0.743, the critical value was 4.200, and the sensitivity and specificity were 62.5% and 94.1%, respectively. The PAD obtained by retrospective ECG-gated CTPA could accurately evaluate APE severity and right ventricular function. As the severity of APE increases, PAD decreases, which is helpful to identify patients at high risk of APE.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Artéria Pulmonar/anormalidades , Embolia Pulmonar/fisiopatologia , Função Ventricular Direita/fisiologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar/patologia , Curva ROC , Estudos Retrospectivos
3.
Medicine (Baltimore) ; 100(3): e24021, 2021 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-33545997

RESUMO

RATIONALE: Pulmonary artery sling (PAS) is a rare congenital anomaly. Associated airway anomalies and/or those of the cardiovascular system are present in about half the patients. Situs inversus is a rare disease in which organs of the chest and/or abdomen are arranged in a mirror image reversal of their normal position. Herein, we report a rare case of pulmonary artery sling and situs inversus incompletus, which has not yet been reported. PATIENT CONCERNS: A 10-year-old girl was admitted because of heart murmur for more than 9 years. On physical examination, the second heart sound was prominent, and a grade 2/6 systolic murmur was heard at the left mid-sternal border. Echocardiography revealed PAS and atrial septal defect (8.6 mm). A chest computer tomography angiograph demonstrated that she had lung inversus, right aortic arch, and right lung hypoplasia in addition to PAS, with a normal positioning of the heart. The PAS intersected and twisted across the bronchus, which was obviously narrowed. The PAS was type II B, since the carina was at the T6 level without a separate right upper lobe bronchus. DIAGNOSES: Her final diagnosis was that of PAS, tracheal stenosis, situs inversus incompletus, right lung hypoplasia, right aortic arch, ASD and PDA. INTERVENTIONS: She underwent one-stage total correction for her initial cardiovascular defects through median sternotomy under cardiopulmonary bypass support. OUTCOMES: She had an uneventful recovery and completely healthy following the procedure. LESSONS: A thorough examination before PAS surgery was essential in discovering and carefully evaluating complicated heart and lung anomalies.


Assuntos
Aorta Torácica/anormalidades , Cardiopatias Congênitas/complicações , Pulmão/anormalidades , Artéria Pulmonar/anormalidades , Situs Inversus/complicações , Estenose Traqueal/congênito , Anormalidades Múltiplas/cirurgia , Criança , Feminino , Humanos
4.
BMC Surg ; 21(1): 101, 2021 Feb 23.
Artigo em Inglês | MEDLINE | ID: mdl-33622276

RESUMO

BACKGROUND: A pulmonary arteriovenous fistula (PAVF) is a rare condition that is associated with pulmonary arteriovenous malformation (PAVM). Few reports have described managing PAVMs using uniportal video-assisted thoracoscopic surgery (VATS). CASE PRESENTATION: A 13-year-old child with PAVF in the left inferior pulmonary artery was treated by uniportal VATS with left lower lobectomy. After surgery, hemoptysis did not recur and there were no postoperative complications. Six months after the operation, postoperative review of computerized tomography showed no recrudescence of PAVF. CONCLUSIONS: PAVF is a rare case that should be diagnosed and treated early. 3D- computerized tomography (CT) reconstruction is useful for diagnosis and preoperative assessment. The case shows that PAVF can be managed with uniportal VATS.


Assuntos
Fístula Arteriovenosa , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Cirurgia Torácica Vídeoassistida , Adolescente , Fístula Arteriovenosa/cirurgia , Humanos , Artéria Pulmonar/cirurgia , Veias Pulmonares/cirurgia , Cirurgia Torácica Vídeoassistida/métodos
5.
Pediatr Cardiol ; 42(3): 533-542, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33394118

RESUMO

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Cateterismo Cardíaco/métodos , Permeabilidade do Canal Arterial/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Cuidados Paliativos/métodos , Reoperação/métodos , Estudos Retrospectivos , Stents , Resultado do Tratamento
6.
BMJ Case Rep ; 14(1)2021 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-33419752

RESUMO

Hereditary haemorrhagic telangiectasia (HHT) also known as Osler-Weber-Rendu syndrome is an autosomal dominant disorder affecting 1 in 8000 individuals. The eponym recognises the 19th-century physicians William Osler, Henri Jules Louis Marie Rendu and Frederick Parkes Weber who each independently described the disease. It is characterised by epistaxis, telangiectasia and visceral arteriovenous malformations. Individuals with HHT have been found to have abnormal plasma concentrations of transforming growth factor beta and vascular endothelial growth factor secondary to mutations in ENG, ACVRL1 and MADH4. Pulmonary artery malformations (PAVMs) are abnormal communications between pulmonary arteries and veins and are found in up to 50% of individuals with HHT. The clinical features suggestive of PAVMs are stigmata of right to left shunting such as dyspnoea, hypoxaemia, cyanosis, cerebral embolism and unexplained haemoptysis or haemothorax. The authors present the case of a 33-year-old woman presenting with progressive dyspnoea during the COVID-19 pandemic. She had a typical presentation of HHT with recurrent epistaxis, telangiectasia and pulmonary arteriovenous malformations. Although rare, PAVM should be considered in individuals presenting to the emergency department with dyspnoea and hypoxaemia. Delayed diagnosis can result in fatal embolic and haemorrhagic complications.


Assuntos
Malformações Arteriovenosas/diagnóstico , Dispneia/fisiopatologia , Epistaxe/fisiopatologia , Hipóxia/fisiopatologia , Telangiectasia Hemorrágica Hereditária/diagnóstico , Receptores de Activinas Tipo II/genética , Adulto , Antitireóideos/uso terapêutico , Malformações Arteriovenosas/fisiopatologia , Gasometria , Carbimazol/uso terapêutico , Diagnóstico Diferencial , Feminino , Doença de Graves/complicações , Doença de Graves/tratamento farmacológico , Humanos , Transtornos de Enxaqueca/complicações , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Telangiectasia Hemorrágica Hereditária/complicações , Telangiectasia Hemorrágica Hereditária/fisiopatologia , Tomografia Computadorizada por Raios X
7.
J Cardiothorac Surg ; 15(1): 290, 2020 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-33008483

RESUMO

BACKGROUND: Pulmonary arteriovenous malformations are uncommon conditions of abnormal communications between pulmonary arteries and veins, which are most commonly congenital in nature. Although such condition is not extremely rare, it is a challenge to the differential diagnosis of pulmonary problems such as hypoxemia and pulmonary lesions. CASE PRESENTATION: We report a meaningful case of a 23-year-old male presented with elevated hemoglobin (23.0 g/dl) on admission. Physical examination revealed cyanosis, digital clubbing and low oxygen saturation on room air. The patient was initially diagnosed as polycythemia vera while the subsequent result of bone marrow aspiration was negative. During further assessment, pulmonary arteriovenous malformations were detected by CT pulmonary angiography. Lobectomy was successfully performed with significant increase in oxygen saturation from 86 to 98%. The hemoglobin decreased to almost normal level of 14.9 g/dl 3 months after surgery and the patient had been followed up for nearly 5 years. CONCLUSIONS: Pulmonary arteriovenous malformations should be suspected in patients with central cyanosis, digital clubbing, polycythemia, pulmonary lesion and without cardiac malformations. Embolization or surgery is strongly recommended to reduce the risks caused by pulmonary arteriovenous malformations.


Assuntos
Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/cirurgia , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Veias Pulmonares/anormalidades , Tuberculose/complicações , Fístula Arteriovenosa/complicações , Angiografia por Tomografia Computadorizada , Progressão da Doença , Humanos , Masculino , Pneumonectomia , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Adulto Jovem
9.
BMC Neurol ; 20(1): 316, 2020 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-32847536

RESUMO

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a rare disorder characterized by recurrent epistaxis, telangiectasias and systemic arteriovenous malformations (AVMs). HHT is associated with mutations in genes encoding for proteins involved in endothelial homeostasis such as ENG (endoglin) and ACVRL1 (activin receptor-like kinase-1). CASE PRESENTATION: Here we describe a 22-year-old male presenting with a transient episode of slurred speech and left arm paresis. Brain MRI displayed polymicrogyria. A right-to-left shunt in absence of an atrial septum defect was noted. Chest CT revealed multiple pulmonary AVMs, likely causing paradoxical embolism manifesting as a transient ischemic attack. The heterozygous ENG variant, c.3G > A (p.Met1lle), was detected in the patient. This variant was also found in patient's mother and in his younger brother who displayed cortical dysplasia type 2. CONCLUSIONS: The detection of cortical development malformations in multiple subjects from the same pedigree may expand the phenotypic features of ENG-related HHT patients. We suggest considering HHT in young patients presenting with acute cerebral ischemic events of unknown origin.


Assuntos
Endoglina/genética , Malformações do Desenvolvimento Cortical/genética , Telangiectasia Hemorrágica Hereditária/diagnóstico , Receptores de Activinas Tipo II/genética , Fístula Arteriovenosa/diagnóstico , Malformações Arteriovenosas/genética , Heterozigoto , Humanos , Masculino , Mutação , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Telangiectasia Hemorrágica Hereditária/genética , Tomografia Computadorizada por Raios X , Adulto Jovem
10.
Asian Cardiovasc Thorac Ann ; 28(8): 463-469, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32659103

RESUMO

BACKGROUND: Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions. METHODS: Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3-9). Surgical approaches included pulmonary artery sling alone (n = 10), pulmonary artery sling with tracheoplasty (n = 41), and pulmonary artery sling with both intracardiac and tracheal surgery (n = 28). RESULTS: There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2-24.8) and 17.9 (interquartile range 4.3-19.8) days, and considerably longer when associated tracheal surgery (p = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality. CONCLUSION: Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.


Assuntos
Anormalidades Múltiplas , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/cirurgia , Reimplante , Estenose Traqueal/cirurgia , Malformações Vasculares/cirurgia , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Humanos , Masculino , Complicações Pós-Operatórias/mortalidade , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Reimplante/efeitos adversos , Reimplante/mortalidade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Estenose Traqueal/diagnóstico por imagem , Estenose Traqueal/mortalidade , Resultado do Tratamento , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/mortalidade
11.
Pediatr Cardiol ; 41(7): 1376-1385, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32494877

RESUMO

We performed this meta-analysis to assess the safety and efficacy of tracheoplasty for patients with pulmonary artery sling (PAS) and tracheal stenosis. Published studies that included surgical treatment of PAS and tracheal stenosis with and without tracheoplasty were identified by searching the PubMed, EMBASE, and Cochrane Library databases until May 2020. The outcomes assessed included postoperative ventilation time, early and late mortality, and follow-up respiratory symptoms. The mean difference (MD)/risk ratio (RR) with 95% confidence intervals (CI) was estimated with a random-effects/fixed-effects model. Subgroup analysis was performed stratified by percentage of patients with tracheal rings. A total of eight studies comprising 219 patients with PAS accompanied by tracheal stenosis were included. The pooled estimates of postoperative ventilation time (MD 17.68, 95% CI 6.38 to 28.98, p < 0.01) and early mortality (RR 3.93, 95% CI 1.55 to 9.95, p < 0.01) favored the repair-only group. Late mortality (RR 1.33, 95% CI 0.48 to 3.68, p = 0.58) and respiratory symptoms (RR 1.51, 95% CI 0.50 to 4.57, p = 0.47) at follow-up showed no significant differences between the groups with repair-only and repair with tracheoplasty. The same results were found in subgroup analyses. For the surgical treatment of PAS with tracheal stenosis, repair without tracheoplasty appears to result in shorter postoperative ventilation time and lower early mortality, with no increase in late mortality or respiratory symptoms at follow-up, compared with concomitant tracheoplasty.


Assuntos
Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Traqueia/cirurgia , Estenose Traqueal/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Razão de Chances , Artéria Pulmonar/anormalidades , Procedimentos Cirúrgicos Reconstrutivos/efeitos adversos , Respiração Artificial , Resultado do Tratamento
12.
BMC Infect Dis ; 20(1): 370, 2020 May 24.
Artigo em Inglês | MEDLINE | ID: mdl-32448130

RESUMO

BACKGROUND: Brain abscesses, a severe infectious disease of the CNS, are usually caused by a variety of different pathogens, which include Streptococcus intermedius (S. intermedius). Pulmonary arteriovenous fistulas (PAVFs), characterized by abnormal direct communication between pulmonary artery and vein, are a rare underlying cause of brain abscesses. CASE PRESENTATION: The patient was a previous healthy 55-year-old man who presented with 5 days of headache and fever. Cerebral magnetic resonance imaging (MRI) suggested a brain abscess. Thoracic CT scan and angiography demonstrated PAVFs. Aiding by metagenomic next-generation sequencing (mNGS) of the cerebrospinal fluid (CSF) sample which identified S. intermedius as the causative pathogen, the patient was switched to the single therapy of large dose of penicillin G and was cured precisely and economically. CONCLUSIONS: It is an alternative way to perform mNGS to identify causative pathogens in patients with brain abscesses especially when the results of traditional bacterial culture were negative. Further thoracic CT or pulmonary angiography should also be undertaken to rule out PAVFs as the potential cause of brain abscess if the patient without any known premorbid history.


Assuntos
Fístula Arteriovenosa/diagnóstico por imagem , Abscesso Encefálico/diagnóstico por imagem , Abscesso Encefálico/tratamento farmacológico , Penicilina G/uso terapêutico , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/tratamento farmacológico , Streptococcus intermedius/genética , Fístula Arteriovenosa/complicações , Abscesso Encefálico/líquido cefalorraquidiano , Abscesso Encefálico/microbiologia , Angiografia por Tomografia Computadorizada , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/diagnóstico por imagem , Veias Pulmonares/diagnóstico por imagem , Infecções Estreptocócicas/líquido cefalorraquidiano , Infecções Estreptocócicas/microbiologia , Streptococcus intermedius/isolamento & purificação , Resultado do Tratamento
13.
Clin Imaging ; 66: 23-25, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32442856

RESUMO

A 50-year-old female with a 20-year history of multifocal pulmonary benign metastasizing leiomyoma (BML), and asthma presented with subacute worsening of chronic dyspnea. A contrast-enhanced computerized tomography of the chest showed a single 1.4 × 1.5-cm contrast-enhancing mass in the right lower lobe among numerous non-enhancing bilateral pulmonary BML lesions. Pulmonary angiogram was not performed at that time due to clinical improvement. Four years later, the patient presented with refractory subacute worsening of her chronic dyspnea and was referred for embolization of the pulmonary arteriovenous malformation (PAVM). Two feeder arteries to the PAVM were embolized; each with a 6-mm Amplatzer-IV vascular plug and a 4-mm Nester coil. Follow-up angiograms demonstrated no flow through the PAVM. The patient's dyspnea resolved and she remained asymptomatic at one-year follow-up.


Assuntos
Fístula Arteriovenosa/diagnóstico por imagem , Malformações Arteriovenosas/diagnóstico por imagem , Leiomioma/diagnóstico por imagem , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Angiografia , Malformações Arteriovenosas/terapia , Embolização Terapêutica/métodos , Feminino , Humanos , Leiomioma/terapia , Pessoa de Meia-Idade , Artéria Pulmonar/diagnóstico por imagem , Veias Pulmonares/diagnóstico por imagem , Tomografia Computadorizada por Raios X
14.
J Cardiothorac Surg ; 15(1): 90, 2020 May 12.
Artigo em Inglês | MEDLINE | ID: mdl-32398101

RESUMO

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death. By the development or lack of coronary collateral, it can be classified as infantile or adult type. However, even with the compensatory mechanism in adult patients, there is an estimated 80 to 90% incidence of sudden death at the mean age of 35 years. METHODS: We enrolled 9 patients with ALCAPA within the age group 5 to 16 years. RESULTS: Only one patient developed symptoms (apsychia), whereas other patients were asymptomatic, and there was no evident left ventricular dysfunction found in any of the cases. CONCLUSION: With the development of imaging techniques, asymptomatic adult-type ALCAPA patients could be identified and diagnosed in childhood or adolescence. As a potential cause of sudden death, ALCAPA should be surgically repaired soon after the diagnosis.


Assuntos
Síndrome de Bland-White-Garland/diagnóstico por imagem , Anomalias dos Vasos Coronários/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Adolescente , Doenças Assintomáticas , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Artéria Pulmonar/anormalidades
16.
Medicine (Baltimore) ; 99(15): e19507, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32282700

RESUMO

RATIONALE: Paradoxical embolism (PE) is an important cause of cryptogenic stroke, particularly in young patients, which usually have a relation with an unexpected route in circulation. Here we report a rare case of cryptogenic stroke carried 2 uncommon malformations. PATIENT CONCERNS: A 48-year-old female experienced double neurological events in just 2 months. DIAGNOSIS: Patent foramen ovale was diagnosed with transesophageal echocardiography and successfully occluded in the first admission due to stroke. In the second admission, chest tomographic angiography found a chordae shadow in the right middle lobe, was the first clue for pulmonary arteriovenous fistula (PAVF), thereafter further confirmed by the enhanced pulmonary computed tomographic angiography. INTERVENTIONS: This patient then received intervention occlusion therapy with coils for PAVF under the help of microcatheter. Given the possible native origin of the thrombus in PAVF due to the spiral morphology, dual antiplatelet therapy was prescribed for this patient for the first 3 months to prevent device-related embolism after discharge, and the following single antiplatelet therapy was mandated. OUTCOMES: No recanalization was detected on the follow-up enhanced pulmonary computed tomographic angiography (PCTA), no neurological defect event recurred in the 16 months of follow-up. LESSONS: Computed tomograph (CT) deserved more value in screening and depicting the morphology of the PAVF, particular in young adults with no apparent arteriosclerotic risk factor. Microcatheter would be helpful for intervention treatment. Antiplatelet therapy might be adequate in specific patients, yet definitely need more evidence to verify.


Assuntos
Fístula Arteriovenosa/complicações , Embolia Paradoxal/etiologia , Forame Oval Patente/complicações , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Acidente Vascular Cerebral/etiologia , Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/terapia , Angiografia por Tomografia Computadorizada , Feminino , Forame Oval Patente/terapia , Humanos , Pessoa de Meia-Idade , Acidente Vascular Cerebral/diagnóstico por imagem
20.
Arch. argent. pediatr ; 118(2): e170-e173, abr. 2020. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1100428

RESUMO

Las fístulas arteriovenosas pulmonares son malformaciones congénitas dadas por la comunicación directa anómala entre arterias y venas, con una incidencia mundial de 2-3 : 100 000 habitantes. La presentación es, en general, única, asintomática, y aparecen en forma incidental como hallazgo imagenológico en la adultez, y su tratamiento de elección es la embolización endovascular.Se describe la inusual presentación clínica en una paciente de 10 años, que ingresó por disnea, tos, cianosis central y cefalea. Se encontró hipoxemia persistente, hipocratismo digital, nódulos parahiliares pulmonares, gases arteriales con gradiente alvéolo-arterial aumentado. La tomografía axial computarizada de tórax de alta resolución confirmó la presencia de una malformación arteriovenosa pulmonar en la región parahiliar derecha, la cual no se asociaba con la enfermedad de Rendu-Osler-Weber. La paciente fue tratada con embolización endovascular transcutánea. Tras 1,5 años de seguimiento, no hubo recaídas. Son pocos los casos reportados de estas fístulas en la edad pediátrica


Pulmonary arteriovenous fistulas are congenital malformations due to anomalous direct communication between arteries and veins; the incidence is 2-3 : 100,000 inhabitants. This condition is usually asymptomatic and incidentally appearing in adult imaging findings. Transcutaneous endovascular embolization is the technique of choice for treatment. The unusual presentation in a 10-year-old patient is described; she was presented to the Emergency Department with dyspnea, cough, central cyanosis and headache. The examination revealed persistent hypoxemia and digital clubbing; chest X-ray with images suggestive of parahilar nodules, arterial blood gases with increased alveolar arterial gradient. The high resolution computed tomography of the thorax revealed pulmonary arteriovenous malformation in the right parahilar region not associated with Rendu-Osler-Weber disease. The patient was treated with transcutaneous endovascular embolization, and after a year and a half of follow-up there were no relapses. There are few reported cases of pulmonary arteriovenous fistulas in the pediatric age.


Assuntos
Humanos , Feminino , Criança , Artéria Pulmonar/anormalidades , Fístula Arteriovenosa/diagnóstico por imagem , Embolização Terapêutica , Artéria Pulmonar/diagnóstico por imagem , Fístula Arteriovenosa/terapia
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