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3.
Pediatr Cardiol ; 40(5): 958-964, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30982075

RESUMO

The purpose of this study was to evaluate the pathological changes of the pulmonary arterioles in patients ≥ 2 years of age who first underwent a pulmonary artery banding (PAB) procedure, followed by bidirectional Glenn or Fontan according to their specific conditions. This was a prospective study of 15 children diagnosed and treated with PAB at the Department of Cardiothoracic Surgery of Anzhen Hospital between January 2009 and December 2012. The percentage of media area (%MS) of pulmonary arteriole, the percentage of media thickness (%MT), and pulmonary arterial density (APSC) were calculated from lung tissue sections. Pulmonary artery pressure decreased significantly after PAB surgery (P < 0.01). Postoperative mean pulmonary artery pressure declined significantly (P < 0.01), the cardiothoracic ratio was reduced (P < 0.05), and percutaneous oxygen saturation (inhaled air) decreased to 80-85% (P < 0.01). %MT (from 35.1 ± 5.6% to 26.9 ± 4.3%, P < 0.01), %MS (from 51.4 ± 6.7% to 32.2 ± 7.4%, P < 0.01), and APSC (from 108.3 ± 38.5 to 83.6 ± 19.6, P < 0.01) were decreased significantly after PAB. Five patients underwent the bidirectional Glenn procedure and four underwent Fontan. In conclusion, the results suggest that PAB can reduce pulmonary artery pressure and that pulmonary arterial lesions can be reversed after PAB.


Assuntos
Hipertensão Pulmonar Primária Familiar/cirurgia , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/cirurgia , Arteríolas , Estudos de Casos e Controles , Criança , Pré-Escolar , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Feminino , Cardiopatias Congênitas/complicações , Humanos , Pulmão/patologia , Masculino , Cuidados Paliativos/métodos , Estudos Prospectivos , Artéria Pulmonar/patologia
4.
Cardiol Young ; 29(4): 462-466, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30957726

RESUMO

OBJECTIVES: The aim of this study was to evaluate the variant frequency of pulmonary arterial hypertension-related genes and provide theoretical basis for genetic screening of patients with pulmonary arterial hypertension further. METHODS: Ten genes associated with pulmonary arterial hypertension were sequenced in 7 cases of idiopathic pulmonary arterial hypertension and 34 cases of congenital heart disease (CHD) associated with pulmonary arterial hypertension by next-generation high-throughput sequencing. Function prediction and gene variant amino acid conservation were carried out by bioinformatics software. Family study was performed on the patients with the variant. RESULTS: A new bone morphogenetic protein receptor type 2(BMPR2) variant (c.344T>C, p. F115S) was discovered in a girl who was diagnosed with idiopathic pulmonary arterial hypertension. Her second aunt and third aunt carried the same variant and were confirmed as patients with pulmonary arterial hypertension as well. No variants or single nucleotide polymorphisms were found in other pulmonary arterial hypertension-associated genes. CONCLUSIONS: BMPR2 variant is the most common variant of pulmonary arterial hypertension. Genetic screening of BMPR2 variant and family survey in patients with pulmonary arterial hypertension is suggested for the sake of definite cause and better treatment.


Assuntos
Receptores de Proteínas Morfogenéticas Ósseas Tipo II/genética , Hipertensão Pulmonar Primária Familiar/genética , Cardiopatias Congênitas/genética , Adolescente , Criança , Pré-Escolar , Hipertensão Pulmonar Primária Familiar/complicações , Feminino , Predisposição Genética para Doença , Testes Genéticos , Cardiopatias Congênitas/complicações , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lactente , Masculino
5.
Heart Lung ; 48(1): 34-38, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30301549

RESUMO

BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder with a high mortality. Clinical experience and small case series suggest thrombocytopenia may be frequent in this population and associated with a poor prognosis. We sought to estimate the prevalence of thrombocytopenia in patients with PAH and characterize its association with disease characteristics and patient outcome. METHODS: Single center cohort study of 714 incident adult patients with Group 1 PH who were evaluated for baseline platelet count at the time of diagnosis. Pts were stratified into three groups: normal platelet count (>150 × 109/L), Grade 1 thrombocytopenia (75-149 × 109/L) and Grade 2-4 thrombocytopenia (<75 × 109/L). RESULTS: The median platelet count was 209 × 109/L (IQR 163, 264). There were 572 (80%) pts without thrombocytopenia, 107 (15%) with Grade 1 and 35 (5%) with Grade 2-4 thrombocytopenia. The median pt age was 55 years (IQR 44-65) with no difference between platelet groups (p = 0.85). Men were more likely to have thrombocytopenia (62, 34%) than women (80, 15%, p < 0.0001). Thrombocytopenia was frequent with portopulmonary PAH (84%) as opposed to idiopathic PAH (iPAH; 14%) or connective tissue disease associated PAH (12%). Platelet counts were not associated with functional class symptoms, the degree of right ventricular enlargement or dysfunction or tricuspid regurgitation by echocardiography. Invasive hemodynamics of right atrial pressure, mean pulmonary artery pressure and pulmonary vascular resistance were also similar between platelet groups. Thrombocytopenia was associated with higher mortality in iPAH patients (age- and sex-adjusted 5 year mortality [HR 1.95 (1.20, 3.08) p = 0.008] but not in other etiology groups. In a multivariate model of iPAH patients (adjusted for age, sex, DLCO, PVR, creatinine and 6MW distance) thrombocytopenia was most predictive of 5-year mortality [HR 1.68 (1.32, 2.12), p < 0.0001]. CONCLUSION: Thrombocytopenia in the context of iPAH portends a poor prognosis and is a simple independent factor to consider in judging severity of disease.


Assuntos
Hipertensão Pulmonar Primária Familiar/mortalidade , Hemodinâmica/fisiologia , Trombocitopenia/complicações , Adulto , Causas de Morte/tendências , Ecocardiografia , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Prevalência , Prognóstico , Taxa de Sobrevida/tendências , Trombocitopenia/sangue , Trombocitopenia/epidemiologia , Estados Unidos/epidemiologia
6.
Paediatr Anaesth ; 29(5): 441-456, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30414333

RESUMO

Pediatric pulmonary hypertension is a complex disease with multiple, diverse etiologies affecting the premature neonate to the young adult. Pediatric pulmonary arterial hypertension, whether idiopathic or associated with congenital heart disease, is the most commonly discussed form of pediatric pulmonary hypertension, as it is progressive and lethal. However, neonatal forms of pulmonary hypertension are vastly more frequent, and while most cases are transient, the risk of morbidity and mortality in this group deserves recognition. Pulmonary hypertension due to left heart disease is another subset increasingly recognized as an important cause of pediatric pulmonary hypertension. One aspect of pediatric pulmonary hypertension is very clear: anesthetizing the child with pulmonary hypertension is associated with a significantly heightened risk of morbidity and mortality. It is therefore imperative that anesthesiologists who care for children with pulmonary hypertension have a firm understanding of the pathophysiology of the various forms of pediatric pulmonary hypertension, the impact of anesthesia and sedation in the setting of pulmonary hypertension, and anesthesiologists' role as perioperative experts from preoperative planning to postoperative disposition. This review summarizes the current understanding of pediatric pulmonary hypertension physiology, preoperative risk stratification, anesthetic risk, and intraoperative considerations relevant to the underlying pathophysiology of various forms of pediatric pulmonary hypertension.


Assuntos
Hipertensão Pulmonar/complicações , Assistência Perioperatória/métodos , Anestesia , Anestésicos/uso terapêutico , Criança , Pré-Escolar , Hipertensão Pulmonar Primária Familiar/complicações , Cardiopatias Congênitas/complicações , Humanos , Hipertensão Pulmonar/fisiopatologia , Lactente , Recém-Nascido , Hipertensão Arterial Pulmonar/complicações , Medição de Risco
7.
J Pediatr ; 205: 29-34.e1, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30396684

RESUMO

OBJECTIVE: To assess the demographics, treatment algorithm, and outcomes in a large cohort of children treated with sildenafil. STUDY DESIGN: A retrospective cohort study of children with pulmonary hypertension (PH) treated with sildenafil at a single institution between 2004 and 2015. Baseline and follow-up data collected by chart review. RESULTS: There were 269 children included in this study: 47 with idiopathic pulmonary arterial hypertension, 53 with congenital heart disease, 135 with bronchopulmonary dysplasia, 24 with congenital diaphragmatic hernia, and 7 with other causes. Sildenafil was initial monotherapy in 84.8% and add-on therapy in 15.2%. Median follow-up time was 3.1 years (2 weeks-12.4 years). On follow-up, 99 (37%) remained on sildenafil or transitioned to tadalafil, 93 (35%) stopped sildenafil for improvement in PH, 54 (20%) died, and 20 (7%) were lost to follow-up. PH was most likely to improve in those with bronchopulmonary dysplasia, allowing for the discontinuation of sildenafil in 45%. Eighteen deaths were related to PH and 36 from other systemic causes. Two patients stopped sildenafil owing to airway spasm with desaturation. Overall survival was significantly lower in World Health Organization group 3 PH (bronchopulmonary dysplasia and congenital diaphragmatic hernia) vs group 1 (idiopathic pulmonary arterial hypertension and congenital heart disease), P = .02. CONCLUSIONS: In this retrospective experience in children with mainly World Health Organization groups 1 and 3 PH, low-dose sildenafil was well-tolerated, safe, and had an acceptable side effect profile. Although patients with group 3 PH have high mortality, survivors have a high likelihood of PH improving.


Assuntos
Hipertensão Pulmonar/tratamento farmacológico , Citrato de Sildenafila/administração & dosagem , Tadalafila/administração & dosagem , Vasodilatadores/administração & dosagem , Adolescente , Displasia Broncopulmonar/complicações , Criança , Pré-Escolar , Hipertensão Pulmonar Primária Familiar/complicações , Feminino , Cardiopatias Congênitas/complicações , Hérnias Diafragmáticas Congênitas/complicações , Humanos , Hipertensão Pulmonar/classificação , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Lactente , Recém-Nascido , Masculino , Resultado do Tratamento
9.
Int J Mol Sci ; 19(10)2018 Oct 17.
Artigo em Inglês | MEDLINE | ID: mdl-30336550

RESUMO

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disease characterised by multisystemic vascular dysplasia. Heritable pulmonary arterial hypertension (HPAH) is a rare but severe complication of HHT. Both diseases can be the result of genetic mutations in ACVLR1 and ENG encoding for proteins involved in the transforming growth factor-beta (TGF-ß) superfamily, a signalling pathway that is essential for angiogenesis. Changes within this pathway can lead to both the proliferative vasculopathy of HPAH and arteriovenous malformations seen in HHT. Clinical signs of the disease combination may not be specific but early diagnosis is important for appropriate treatment. This review describes the molecular mechanism and management of HPAH and HHT.


Assuntos
Hipertensão Pulmonar Primária Familiar/complicações , Telangiectasia Hemorrágica Hereditária/complicações , Hipertensão Pulmonar Primária Familiar/diagnóstico , Hipertensão Pulmonar Primária Familiar/genética , Hipertensão Pulmonar Primária Familiar/terapia , Hemodinâmica , Humanos , Padrões de Herança/genética , Telangiectasia Hemorrágica Hereditária/genética
10.
Br J Haematol ; 183(2): 267-275, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30141279

RESUMO

Acquired von Willebrand syndrome (AVWS) is reported in high-flow high-shear congenital cardiac disorders. We hypothesized that the narrowed pulmonary vasculature in idiopathic pulmonary arterial hypertension (IPAH) may induce AVWS. We conducted a cross-sectional evaluation of children with IPAH. Patients with bleeding symptoms and/or laboratory abnormalities (thrombocytopenia, anomalies in coagulation screening tests) were tested in-depth for haemostatic defects. Fourteen children were followed with IPAH of which 8 were eligible. Four children exhibited abnormal bleeding scores (International Society on Thrombosis and Haemostasis Bleeding Assessment Tool: 3-5). All 8 patients showed very prolonged platelet function analyser (PFA)-100 closure times. Six children demonstrated either mild thrombocytopenia or low-normal von Willebrand factor (VWF) antigen (VWF:Ag) or VWF activity [mean (range), in iu/dl: VWF:Ag: 70 (61-91); VWF activity: 57 (34-70)]. Average VWF collagen binding capacity (VWF:CB) was 64 iu/dl (range: 53-123 iu/dl), with low-normal VWF activity/VWF:Ag or VWF:CB/VWF:Ag ratios occurring in five patients. All children had normal multimers distribution patterns. One patient underwent a lung transplantation, with normalization of haemostatic abnormalities post-surgery. Overall, 8 out of 14 children with IPAH had mild to moderate bleeding symptoms and/or laboratory abnormalities in keeping with AVWS. Normalization of the haemostatic defects following lung transplantation and lack of family history of bleeding attests to the acquired nature of their defects.


Assuntos
Hipertensão Pulmonar Primária Familiar/complicações , Doenças de von Willebrand/etiologia , Adolescente , Criança , Estudos Transversais , Hipertensão Pulmonar Primária Familiar/sangue , Hipertensão Pulmonar Primária Familiar/cirurgia , Feminino , Hemorreologia , Humanos , Transplante de Pulmão , Masculino , Contagem de Plaquetas , Trombocitopenia/sangue , Trombocitopenia/etiologia , Doenças de von Willebrand/sangue , Fator de von Willebrand/metabolismo
11.
Am J Emerg Med ; 36(11): 2130.e3-2130.e5, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30033134

RESUMO

Chest pain and shortness of breath are chief complaints frequently evaluated in the emergency department. ACS, pulmonary embolism, and disorders involving the lung parenchyma are some of the disease processes commonly screened for. Occasionally, patients presenting with histories and clinical exams consistent with these common illnesses may end up having more rare pathology. We present the case of a young patient who presented with chest pain and dyspnea with ECG changes and history concerning for pulmonary embolism who was ultimately diagnosed with idiopathic primary pulmonary hypertension. The importance of a prompt diagnosis of this condition along with emergency department management of complications related to the disease is discussed in this report.


Assuntos
Dispneia/etiologia , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/diagnóstico , Adulto , Dor no Peito/etiologia , Angiografia por Tomografia Computadorizada , Diagnóstico Diferencial , Eletrocardiografia , Serviço Hospitalar de Emergência , Hipertensão Pulmonar Primária Familiar/tratamento farmacológico , Furosemida/uso terapêutico , Humanos , Masculino , Citrato de Sildenafila/uso terapêutico , Espironolactona/uso terapêutico
12.
Zhonghua Er Ke Za Zhi ; 56(6): 451-456, 2018 Jun 02.
Artigo em Chinês | MEDLINE | ID: mdl-29886609

RESUMO

Objective: To investigate the risk factors, clinical features, treatments, and prevention of pulmonary hypertensive crisis (PHC) in children with idiopathic pulmonary arterial hypertension (IPAH) undergoing cardiac catheterization. Methods: This retrospective study included 67 children who were diagnosed with IPAH and underwent cardiac catheterization between April 2009 and June 2017 in Beijing Anzhen Hospital. The medical histories, clinical manifestations, treatments, and outcomes were characterized. Statistical analyses were performed using t test, χ(2) test and a multiple Logistic regression analysis. Results: During cardiac catheterization, five children developed PHC who presented with markedly elevated pulmonary artery pressure and central venous pressure, decline in systemic arterial pressure and oxygen saturation. Heart rate decreased in 4 cases and increased in the remaining one. After the treatments including cardiopulmonary resuscitation, pulmonary vasodilator therapy, improving cardiac output and blood pressure, and correction of acidosis, 4 of the 5 cases recovered, while 1 died of severe right heart failure with irreversible PHC 3 days after operation. Potential PHC was considered in 7 other patients, whose pulmonary artery pressure increased and exceeded systemic arterial pressure, oxygen saturation decreased, and central venous pressure and vital signs were relatively stable. Univariate analysis showed that the risk factors of PHC in children with IPAH undergoing cardiac catheterization were younger age (t=3.160, P=0.004), low weight (t=4.004, P<0.001), general anesthesia (χ(2)=4.970, P=0.026), history of syncope (χ(2)=4.948, P=0.026), and WHO cardiac functional class Ⅲ or Ⅳ (χ(2)=19.013, P<0.001). Multivariate Logistic regression analysis revealed that worse WHO cardiac functional class (Wald=13.128, P<0.001, OR=15.076, 95% CI: 3.475-65.418) was the independent risk factor of PHC. Conclusions: PHC is a severe and extremely dangerous complication in children with IPAH during cardiac catheterization. WHO cardiac functional class may be associated with PHC. Integrated treatment is required for these patients. Reducing risk factors, early identification, and active treatment may help to prevent the occurrence and progression of PHC.


Assuntos
Cateterismo Cardíaco , Hipertensão Pulmonar Primária Familiar , Hipertensão Pulmonar , Cateterismo Cardíaco/efeitos adversos , Criança , Hipertensão Pulmonar Primária Familiar/complicações , Humanos , Hipertensão Pulmonar/etiologia , Estudos Retrospectivos , Fatores de Risco
13.
Can J Cardiol ; 34(6): 812.e9-812.e11, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29801749

RESUMO

The coexistence of idiopathic pulmonary artery hypertension with pulmonary arteriovenous malformation (PAVM) is challenging because although the PAVM causes hypoxia and polycythemia with potential thrombotic complications, closing the PAVM increases pulmonary artery pressure. We report on a lady with PAVM and idiopathic pulmonary artery hypertension who, within 2 years of diagnosis, had an ischemic stroke, PAVM enlargement from 20 × 20 × 30 mm to 30x × 40 × 40 mm and oximetry decrease to 90%. Transcatheter occlusion of PAVM with a vascular plug was successful. A year later, she had no flow via the PAVM and systemic oximetry improved to 97%, but pulmonary vascular resistance increased to 1.5-fold of baseline.


Assuntos
Fístula Arteriovenosa , Isquemia Encefálica , Embolização Terapêutica , Hipertensão Pulmonar Primária Familiar , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Acidente Vascular Cerebral , Adulto , Fístula Arteriovenosa/complicações , Fístula Arteriovenosa/diagnóstico , Fístula Arteriovenosa/fisiopatologia , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/etiologia , Embolização Terapêutica/efeitos adversos , Embolização Terapêutica/instrumentação , Embolização Terapêutica/métodos , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/diagnóstico , Hipertensão Pulmonar Primária Familiar/etiologia , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Feminino , Humanos , Achados Incidentais , Imagem por Ressonância Magnética/métodos , Administração dos Cuidados ao Paciente/métodos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Veias Pulmonares/fisiopatologia , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/etiologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Dispositivos de Oclusão Vascular , Resistência Vascular
14.
Chest ; 154(4): 872-881, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29800550

RESUMO

BACKGROUND: An obesity paradox, wherein patients who are obese have lower mortality, has been described in cardiopulmonary diseases, including pulmonary arterial hypertension (PAH). Our objective was to determine whether obesity and BMI are associated with mortality in patients with PAH. METHODS: We assessed incident patients with idiopathic, drug-induced, and heritable PAH from the French Pulmonary Hypertension Network registry. Cox regression and Kaplan-Meier analysis were used to assess the association between BMI and obesity with all-cause mortality. RESULTS: Of 1,255 patients included, 30% were obese. A higher proportion of women (65.1% vs 53.4%, P < .01), drug-induced PAH (28.9% vs 9.2%, P < .01), systemic hypertension, diabetes, and hypothyroidism were present in the obese group. More obese patients were in New York Heart Association class III (66.4% vs 57.1%), fewer were class IV (11.8% vs 16.9%, P < .01), and 6-min walk distance was lower (276 ± 121 vs 324 ± 146, P < .01). Right atrial pressure, pulmonary wedge pressure, and cardiac index were higher, whereas pulmonary vascular resistance was lower in patients who were obese. Neither BMI (hazard ratio [HR], 0.99; 95% CI, 0.97-1.01; P = .41) nor obesity (HR, 1.0; 95% CI, 0.99-1.01; P = .46) were associated with mortality in multivariable analyses. There was a significant interaction between age and obesity such that mortality increased among patients < 65 years of age who were morbidly obese (HR, 3.01; 95% CI, 1.56-5.79; P = .001). CONCLUSIONS: Obesity was not associated with mortality in the overall population, but there was an age-obesity interaction with increased mortality among young patients who were morbidly obese. These results have implications for active weight management in younger patients who are morbidly obese who are otherwise candidates for lung transplantation.


Assuntos
Hipertensão Pulmonar/mortalidade , Obesidade Mórbida/mortalidade , Idoso , Índice de Massa Corporal , Métodos Epidemiológicos , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/mortalidade , Feminino , França/epidemiologia , Humanos , Hipertensão Pulmonar/complicações , Masculino , Pessoa de Meia-Idade , Obesidade Mórbida/complicações
15.
Medicine (Baltimore) ; 97(10): e0075, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29517668

RESUMO

BACKGROUND: Oral bosentan has been widely applied in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). A systemic review and meta-analysis was conducted for a therapeutic evaluation of oral bosentan in both adult and pediatric patients with PAH-CHD. The acute responses and a long-term effect were respectively assessed in a comparison with baseline characteristics, and the improvement of exercise tolerance was analyzed. METHODS: PubMed, Medline, Embase, and Cochrane Central Register of clinical controlled trails or observational studies have been searched for a recording of bosentan effects on the PAH-CHD participants. For mortality and rate of adverse events (AEs), it was described in detail. Randomized-effects model or fixed-effects model was used to calculate different effective values with a sensitivity analysis. RESULTS: Seventeen studies were pooled in this review, and 3 studies enrolled the pediatric patients. Among all studies, 456 patients were diagnosed with PAH-CHD, and 91.7% were treated with oral bosentan. With a term less than 6 months of bosentan therapy, there existed a significant improvement in 6-minute walk distance (6MWD) and the World Health Organization functional class (WHO-FC), but no such differences in Borg dyspnea index scores (BDIs) and the resting oxygen saturation (SpO2). Although with a prolonged treatment, not only 6MWD and FC, but also the resting SpO2 and heart rate were changed for a better exercise capability. Additionally, compared with the basic cardiopulmonary hemodynamics, it showed a statistically significant difference in mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance index (PVRi). Although a limitation of pooled studies with comparative outcomes of different terms, outcomes presented a lower WHO-FC which contributes to a success in a prolonged treatment. CONCLUSIONS: Bosentan in PAH-CHD is well established and still requires clinical trials for an identification of its efficiency on CHD patients for an optimized period lessening a serious complication and the common AEs.


Assuntos
Antagonistas dos Receptores de Endotelina/uso terapêutico , Hipertensão Pulmonar Primária Familiar/tratamento farmacológico , Cardiopatias Congênitas/complicações , Sulfonamidas/uso terapêutico , Adolescente , Adulto , Bosentana , Criança , Pré-Escolar , Hipertensão Pulmonar Primária Familiar/complicações , Humanos , Lactente , Pessoa de Meia-Idade , Resultado do Tratamento , Adulto Jovem
16.
Pediatr Cardiol ; 39(6): 1115-1122, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29541815

RESUMO

Right ventricular (RV) hypertrophy is regarded as the adaptation on chronic RV pressure load in pulmonary hypertension. As the RV Sokolow-Lyon index (RVSLI) is an electrocardiographic marker of RV hypertrophy, we hypothesized that RVSLI might be able to reflect RV pressure load. Therefore, the purpose of this study was to characterize the diagnostic impact of the RVSLI in children with idiopathic pulmonary arterial hypertension (IPAH) in order to assess disease severity and to evaluate its value for the prediction of worse outcome. Forty-two children (aged 3-17 years) with IPAH were included in this retrospective study. The follow-up after baseline examination was 59 ± 17 months. Receiver-operating characteristic (ROC) curves and Kaplan-Meier analysis were used to discriminate a cut-off value of RVSLI and to assess its predictive value regarding morbidity and mortality. In 12/42 patients (29%) severe cardiovascular events (defined as death, lung transplantation, or Potts shunt) were observed (time to event 20 ± 22 months). Patients with an event showed higher RVSLI values (3.6 ± 1.2 mV vs. 2.6 ± 1.6 mV; p < 0.05). ROC analysis discriminated an RVSLI of 2.1 as the best cut-off value (area under the ROC curve: 0.79, sensitivity: 0.91, specificity: 0.70, p < 0.05) to detect patients with high-risk PAH (mPAP/mSAP ratio > 0.75). Relative risk for a severe event with an index > 2.1 mV was 1.76 (95% CI 1.21-3.20). Relative risk for death with RVSLI > 2.1 mV was 2.01 (95% CI 1.61-4.80). Our study demonstrates a strong relationship between RVSLI and disease severity in children with IPAH. An RVSLI > 2.1 mV at the time of first diagnosis is a predictor for patients at risk for cardiac events. As an adjunct to the usual diagnostic assessment this parameter may therefore contribute to the initial prognostic estimation.


Assuntos
Eletrocardiografia , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Ventrículos do Coração/fisiopatologia , Adolescente , Criança , Pré-Escolar , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/diagnóstico , Feminino , Humanos , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/fisiopatologia , Estimativa de Kaplan-Meier , Masculino , Prognóstico , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade , Índice de Gravidade de Doença
17.
Rheumatology (Oxford) ; 57(5): 831-834, 2018 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-29447389

RESUMO

Objectives: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in adults with systemic autoimmune rheumatic diseases (ARDs). The aim of this study was to determine whether adults with ARDs and PAH on right-sided heart catheterization (ARD-PAH) have increased mortality following lung transplantation compared with those with PAH not due to an ARD. Methods: We conducted a retrospective cohort study of 93 adults with ARD-PAH and 222 adults with PAH who underwent lung transplantation in the USA between 4 May 2005 and 9 March 2015 using data from the United Network for Organ Sharing. We examined associations between diagnosis and survival after lung transplantation using stratified Cox models adjusted for potential confounding recipient factors. Results: Among adults undergoing lung transplantation in the USA, we did not detect a difference in the multivariable-adjusted mortality rate between those with ARD-PAH and those with PAH [hazard ratio 0.75 (95% CI 0.47, 1.19)]. Conclusion: The presence of an ARD was not associated with increased mortality after lung transplantation in adults with PAH.


Assuntos
Doenças Autoimunes/mortalidade , Hipertensão Pulmonar Primária Familiar/mortalidade , Transplante de Pulmão , Doenças Reumáticas/mortalidade , Adulto , Doenças Autoimunes/complicações , Doenças Autoimunes/imunologia , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/cirurgia , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Período Pós-Operatório , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Doenças Reumáticas/complicações , Doenças Reumáticas/imunologia , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologia
18.
Int Ophthalmol ; 38(1): 375-379, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28108900

RESUMO

PURPOSE: To introduce a case of ciliary detachment with primary pulmonary hypertension (PPH). METHODS: The clinical manifestations of a case of ciliary detachment with PPH were addressed by comprehensive examination including ultrasound biological microscope (UBM), intraocular pressure, color fundus photographs, fluorescence fundus angiography (FFA). In addition, echocardiography is used to measure primary pulmonary pressure. RESULTS: When the echocardiography displayed a systolic pulmonary arterial pressure of 106 mmHg, UBM exhibited ciliochoroidal detachment, as well as peripheral retinal effusion and non-perfusion areas in FFA. After well controlled of PPH, UBM showed normal ciliary body. FFA confirmed that retinal effusion disappeared. CONCLUSIONS: The elevated venous pressure in PPH is responsible for decreased choroidal backflow and reduced venous blood outflow from the eye. PPH would contribute to the clinical manifestations of severe choroidal detachment and peripheral retina effusion in this patient.


Assuntos
Corpo Ciliar , Hipertensão Pulmonar Primária Familiar/complicações , Angiofluoresceinografia/métodos , Retina/patologia , Descolamento Retiniano/etiologia , Adulto , Corioide/patologia , Diagnóstico Diferencial , Ecocardiografia , Hipertensão Pulmonar Primária Familiar/diagnóstico , Feminino , Fundo de Olho , Humanos , Pressão Propulsora Pulmonar , Descolamento Retiniano/diagnóstico , Acuidade Visual
20.
Respirology ; 23(2): 148-159, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-28845582

RESUMO

Pulmonary arterial hypertension (PAH) is a chronic disease with poor prognosis and important exercise limitation despite the proliferation of treatment options in the last decade. Chronically increased right ventricular (RV) afterload results in right heart failure and without treatment, rapid clinical deterioration is common. Exercise intolerance is the cardinal feature of the disease impacting upon quality of life and clinical outcome. The pathophysiological mechanisms that lead to reduced exercise capacity in this population are complex with ventriculoarterial uncoupling likely to be the predominant feature. The relative contributions of additional factors that contribute to exercise limitation beyond ventriculoarterial uncoupling have not been characterized. This review addresses these factors with a focus on recent developments and uncertainties. RV maladaptation and the intricate interplay between the heart, abnormal pulmonary vascular bed and peripheral factors such as dysfunction of the respiratory and peripheral muscles are discussed in detail.


Assuntos
Tolerância ao Exercício/fisiologia , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Qualidade de Vida , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia
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