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1.
Rev. colomb. anestesiol ; 49(4): e400, Oct.-Dec. 2021. graf
Article in English | LILACS, COLNAL | ID: biblio-1341243

ABSTRACT

The accompanying images demonstrate giant pulmonary artery aneurysms in a patient with idiopathic pulmonary arterial hypertension (Image 1). In addition to the main pulmonary artery, both the left and right pulmonary arteries are aneurysmal and are compressing the lung parenchyma (Image 2).


Las imágenes adjuntas muestran aneurismas gigantes de la arteria pulmonar en un paciente con hipertensión arterial pulmonar idiopática (Imagen 1). Además de la arteria pulmonar principal, tanto la arteria pulmonar izquierda como la derecha son aneurismáticas y están comprimiendo el parénquima pulmonar (Imagen 2).


Subject(s)
Humans , Pulmonary Artery , Radiology , Aneurysm , Familial Primary Pulmonary Hypertension , Parenchymal Tissue , Heart Defects, Congenital
2.
Rev. méd. Minas Gerais ; 31: 31406, 2021.
Article in Portuguese | LILACS | ID: biblio-1291372

ABSTRACT

A proteinose alveolar pulmonar (PAP) é rara e caracterizada por preenchimento alveolar com material lipoproteináceo. A proteinose alveolar é caracterizada por um alvéolo com material eosinofílico, acelular, finamente granular, com fendas de colesterol. Este relato de caso descreve um paciente do sexo masculino, 2 anos, portador de hipogamaglobulinemia. O paciente foi internado com quadro compatível com Stevens- Johnson após uso de amoxicilina e clavulanato para quadro de otite média aguda. Posteriormente, foi encaminhado à unidade de terapia intensiva devido à piora respiratória clínica e radiológica. Biópsia pulmonar: proteinose alveolar com alvéolos distendidos por material proteináceo, eosinofílico e grumoso com infiltrado linfo- histiocitário local. A proteinose alveolar pulmonar é rara e o diagnóstico correto deve ser realizado para que seja realizado tratamento adequado e acompanhamento da evolução. Deve-se atentar para complicações, especialmente infecções oportunistas.


Pulmonary alveolar proteinosis (PAP) is rare and it is characterized by alveolar filling with lipoproteinaceous material. Alveolar proteinosis is characterized by an alveolus with eosinophilic, acellular, finely granular material, with cholesterol cracks. This case report describes a 2-yearold male patient with hypogammaglobulinemia. The patient was hospitalized with Stevens-Johnson-compatible condition after use of amoxicillin and clavulanate for acute otitis media. Subsequently, he was referred to the intensive care unit due to worsening clinical and radiological breathing. Lung biopsy: alveolar proteinosis with alveoli distended by proteinaceous, eosinophilic and lumpy material with local lymphohistiocytic infiltrate. Pulmonary alveolar proteinosis is rare and the correct diagnosis must be made in order to carry out an appropriate treatment and follow-up of the evolution. Attention should be paid to complications, especially opportunistic infections.


Subject(s)
Male , Child, Preschool , Pulmonary Alveolar Proteinosis , Respiratory Distress Syndrome, Newborn , Opportunistic Infections , Dyspnea , Tachypnea , Familial Primary Pulmonary Hypertension , Amoxicillin
3.
Clinics ; 75: e1698, 2020. tab
Article in English | LILACS | ID: biblio-1101087

ABSTRACT

OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.


Subject(s)
Extracorporeal Membrane Oxygenation/methods , Lung Diseases/therapy , Postoperative Complications , Pulmonary Emphysema/therapy , Pulmonary Emphysema/epidemiology , Pulmonary Fibrosis/therapy , Pulmonary Fibrosis/epidemiology , Time Factors , Brazil/epidemiology , Bronchiectasis/therapy , Bronchiectasis/epidemiology , Prospective Studies , Retrospective Studies , Lung Transplantation/methods , Treatment Outcome , Familial Primary Pulmonary Hypertension/therapy , Familial Primary Pulmonary Hypertension/epidemiology , Intensive Care Units , Length of Stay , Lung Diseases/epidemiology
4.
Rev. pesqui. cuid. fundam. (Online) ; 10(3): 683-689, jul.-set. 2018. ilus, tab
Article in English, Portuguese | LILACS, BDENF | ID: biblio-906334

ABSTRACT

Objetivo: Realizar um estudo de caso de um jovem portador de tuberculose pulmonar (TP) e construir um plano de assistência de enfermagem, visando a melhoria da qualidade de vida do paciente. Métodos: Estudo de caso com abordagem qualitativa de um paciente portador de TP, hipertensão arterial pulmonar, desnutrição e alcoolismo, no Hospital Universitário Oswaldo Cruz (HUOC) em Recife-PE, no período de março a junho de 2016. A coleta de dados foi realizada mediante anamnese, exame físico, análise do prontuário, pesquisa literária e histórica de enfermagem. Resultados: Identificamos 13 diagnósticos de enfermagem: padrão respiratório ineficaz, nutrição/fadiga, manutenção ineficaz da saúde e do lar, distúrbios da identidade/autoestima, risco de solidão/suicídio e angustia espiritual. Conclusão: A enfermagem tem especial importância nas ações de controle da TP realizando a supervisão do tratamento, evitando o abandono e a tuberculose resistente, garantindo a adesão e um tratamento bem-sucedido


Objetivo: Realizar un estudio de caso de un paciente joven con tuberculosis pulmonar (TP) y construir un plan de atención de enfermería con el fin de mejorar la calidad de vida del paciente. Métodos: Un estudio de caso con enfoque cualitativo de un paciente con enfermedad de Parkinson, la hipertensión arterial pulmonar, la desnutrición y el alcoholismo, el Hospital Universitario Oswaldo Cruz (HUOC) en Recife, de marzo a junio de 2016. La recolección de datos fue realizado por la historia clínica, examen físico, análisis de la historia clínica, la investigación literaria y la historia de enfermería. Resultados: Se identificaron 13 diagnósticos de enfermería: Modelo ineficaz la respiración, la nutrición/fatiga, mantenimiento ineficaz de la salud y el hogar, trastornos de identidad/autoestima, la soledad de riesgo/suicidio y la angustia espiritual. Conclusión: La enfermería tiene especial importancia en las acciones de control TP mediante la supervisión del tratamiento, evitar el abandono y la tuberculosis resistente, asegurando la adherencia y un tratamiento exitoso


Objective: Performing a case study of a young patient having pulmonary tuberculosis disease (TB) and design a nursing care plan aiming to improve the patient life quality. Methods: Case study with a qualitative approach of a patient having TB, pulmonary arterial hypertension, malnutrition state and also alcoholism. The study was done at the Oswaldo Cruz University Hospital (HUOC) in Recife, Brazil, from March to June 2016. Data collection was performed through anamnesis, physical examination, medical records analysis, literary research and nursing history. Results: We were able to identify 13 nursing diagnoses, as follows: ineffective respiratory pattern, nutrition/fatigue, ineffective maintenance of health and home, identity/self-esteem disorders, solitude/ suicide risk and spiritual distress. Conclusion: The nursing professionals have especial importance regarding the actions of TB control, supervising the treatment, avoiding abandonment and resistant tuberculosis, as well as, guaranteeing adherence and a successful treatment


Subject(s)
Humans , Male , Adult , Nursing Care , Treatment Adherence and Compliance , Tuberculosis, Pulmonary/complications , Tuberculosis, Pulmonary/nursing , Alcoholism/nursing , Familial Primary Pulmonary Hypertension/nursing , Malnutrition/nursing
5.
Laboratory Medicine Online ; : 119-124, 2018.
Article in English | WPRIM | ID: wpr-715907

ABSTRACT

Pathogenic variants of bone morphogenic protein receptor type 2 gene (BMPR2) are related to the majority of cases of heritable pulmonary arterial hypertension (PAH). Over 400 pathogenic variants have been identified. However, clinical characterization of PAH is still incomplete. We present a case of heritable PAH in a Korean family showing serious clinical presentation with high penetrance. Genetic sequencing revealed a known heterozygous BMPR2 pathogenic variant, c.418+5G>A, at a splice site of intron 3. Serious clinical presentation with high penetrance suggested that the interplay of other factors with pathologic variants might be in genotype-phenotype correlation. Further studies are needed to clarify these issues for the development of personalized medicine approaches for PAH.


Subject(s)
Familial Primary Pulmonary Hypertension , Genetic Association Studies , Humans , Hypertension , Hypertension, Pulmonary , Introns , Penetrance , Precision Medicine , Pulmonary Artery
6.
Chinese Medical Journal ; (24): 382-391, 2017.
Article in English | WPRIM | ID: wpr-303142

ABSTRACT

<p><b>BACKGROUND</b>The clinical significance of acute vasoreactivity testing (AVT) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains unclear. We analyzed changes in hemodynamics and oxygenation dynamics indices after AVT in patients with CTEPH using patients with pulmonary arterial hypertension (PAH) as controls.</p><p><b>METHODS</b>We analyzed retrospectively the results of AVT in 80 patients with PAH and 175 patients with CTEPH registered in the research database of Beijing Chao-Yang Hospital between October 2005 and August 2014. Demographic variables, cardiopulmonary indicators, and laboratory findings were compared in these two subgroups. A long-term follow-up was conducted in patients with CTEPH. Between-group comparisons were performed using the independent-sample t-test or the rank sum test, within-group comparisons were conducted using the paired t-test or the Wilcoxon signed-rank test, and count data were analyzed using the Chi-squared test. Survival was estimated using the Kaplan-Meier method and log-rank test.</p><p><b>RESULTS</b>The rates of positive response to AVT were similar in the CTEPH (25/175, 14.3%) and PAH (9/80, 11.3%) groups (P > 0.05). Factors significantly associated a positive response to AVT in the CTEPH group were level of N-terminal pro-brain natriuretic peptide (≤1131.000 ng/L), mean pulmonary arterial pressure (mPAP, ≤44.500 mmHg), pulmonary vascular resistance (PVR, ≤846.500 dyn·s-1·m-5), cardiac output (CO, ≥3.475 L/min), and mixed venous oxygen partial pressure (PvO2, ≥35.150 mmHg). Inhalation of iloprost resulted in similar changes in mean blood pressure, mPAP, PVR, systemic vascular resistance, CO, arterial oxygen saturation (SaO2), mixed venous oxygen saturation, partial pressure of oxygen in arterial blood (PaO2), PvO2, and intrapulmonary shunt (Qs/Qt) in the PAH and CTEPH groups (all P > 0.05). The survival time in patients with CTEPH with a negative response to AVT was somewhat shorter than that in AVT-responders although the difference was not statistically significant (χ2 =3.613, P = 0.057). The survival time of patients with CTEPH who received calcium channel blockers (CCBs) was longer than that in the group with only basic treatment and not shorter than that of patients who receiving targeted drugs or underwent pulmonary endarterectomy (PEA) although there was no significant difference between the four different treatment regimens (χ2 =3.069, P = 0.381).</p><p><b>CONCLUSIONS</b>The rates of positive response to AVT were similar in the CTEPH and PAH groups, and iloprost inhalation induced similar changes in hemodynamics and oxygenation dynamics indices. A positive response to AVT in the CTEPH group was significantly correlated with milder disease and better survival. Patients with CTEPH who cannot undergo PEA or receive targeted therapy but have a positive response to AVT might benefit from CCB treatment.</p>


Subject(s)
Administration, Inhalation , Adult , Aged , Arterial Pressure , Atrial Natriuretic Factor , Metabolism , Calcium Channel Blockers , Therapeutic Uses , Endarterectomy , Familial Primary Pulmonary Hypertension , Drug Therapy , Female , Hemodynamics , Humans , Hypertension, Pulmonary , Drug Therapy , Iloprost , Therapeutic Uses , Male , Middle Aged , Protein Precursors , Metabolism , Retrospective Studies , Software , Vasodilator Agents , Therapeutic Uses
7.
Arq. bras. med. vet. zootec ; 68(4): 887-893, jul.-ago. 2016. tab
Article in Portuguese | ID: lil-792470

ABSTRACT

A hipertensão arterial pulmonar (HAP) é uma doença complexa, caracterizada por disfunção endotelial, que resulta em remodelamento vascular pulmonar e elevação da pressão arterial pulmonar, com consequente insuficiência cardíaca direita. O speckle tracking bidimensional (2D-STE) é uma das mais recentes ferramentas da ecocardiografia, o qual tem sido empregado para avaliação mais precoce da função ventricular e do efeito da HAP sobre a função dos ventrículos esquerdo e direito. O objetivo deste estudo foi avaliar a deformação (St e StR) miocárdica radial do VE em modelo experimental de suínos com HAP induzida e tratados com angiotensina-(1-7), a fim de verificar as possibilidades desse novo fármaco nas respostas clínica e hemodinâmica, pois apresenta efeitos anti-inflamatório e vasodilatador, bem como ações antiproliferativas no sistema cardiovascular. Neste estudo, foi possível observar que os animais tratados com Ang-(1-7) apresentaram St e StR radiais maiores que o grupo placebo aos 60 dias de experimento, demonstrando uma melhora na função sistólica do miocárdio pelo aumento da deformação miocárdica (16,06±7,50 - placebo; 25,14±14,91 - Ang-(1-7)) e StR (1,28±0,51 - placebo; 1,51±0,58 - Ang-(1-7)). Essa melhora na função sistólica pode ser atribuída aos efeitos do fármaco, que reduziram também o desenvolvimento da hipertensão pulmonar. Diante dos resultados, acredita-se que a Ang-(1-7) possa ser um medicamento promissor para tratamento da HAP.(AU)


Pulmonary arterial hypertension (PAH) is a complex disease characterized by endothelial dysfunction resulting in pulmonary vascular remodeling, increased pulmonary arterial pressure, with subsequent right heart failure. The two-dimensional speckle tracking (2D-STE) is one of the newer tools used for early assessment of ventricular function. The aim of this study was to evaluate the radial myocardial deformation (St and StR) of LV in experimental pigs with induced PAH and treated with Ang- (1-7), checking the possibilities of this new drug in clinical and hemodynamic response, since it has anti-inflammatory effects and antiproliferative actions on the cardiovascular system. In this study we observed that animals treated with Ang (1-7) had radial St and StR higher than the placebo group at 60 days of the experiment demonstrating an improvement in systolic function of the myocardium by increased myocardial deformation (16.06±7.50 - placebo; 25.14±14.91 - Ang (1-7)) and StR (1.28±0.51 - placebo; 1.51±0.58 - Ang (1-7)). This improvement in systolic function can be attributed to drug effects while also reducing the development of pulmonary hypertension. Based on the results it is believed that Ang (1-7) may be a promising drug for the treatment of PAH.(AU)


Subject(s)
Animals , Angiotensins/therapeutic use , Familial Primary Pulmonary Hypertension/veterinary , Swine , Ventricular Dysfunction/veterinary , Echocardiography/veterinary
8.
Bogotá; IETS; mayo 2016. tab, ilus.
Monography in Spanish | LILACS, BRISA | ID: biblio-847224

ABSTRACT

Introducción: la hipertensión arterial pulmonar es una enfermedad grave y progresiva. Si la enfermedad no es tratada, la sobrecarga de presión del ventrículo derecho lleva a dilatación, hipertrofia, falla cardíaca en estado final entre dos a tres años y a muerte. El sildenafilo, un inhibidor de la Fosfodiesterasa tipo 5, es una alternativa para su tratamiento actualmente en uso clínico. Esta evaluación de tecnología se desarrolló para informar la toma de decisiones en el marco de la actualización integral del Plan Obligatorio de Salud para Colombia. Objetivo: examinar la efectividad y seguridad comparativas del sildenafilo para el tratamiento de pacientes con hipertensión arterial pulmonar. Metodología: se realizó una búsqueda sistemática en MEDLINE, EMBASE, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects y LILACS. La tamización de referencias se realizó por dos revisores de forma independiente y la selección de estudios fue hecha por un revisor, aplicando los criterios de elegibilidad predefinidos en el protocolo de la evaluación. La calidad de las revisiones sistemáticas se valoró con la herramienta AMSTAR. Se realizó una síntesis narrativa de las estimaciones del efecto para las comparaciones y desenlaces de interés. Resultados: los hallazgos de efectividad y seguridad de la presente evaluación se basan en cuatro revisiones sistemáticas, dos de calidad alta y dos de calidad media, y cuatro ensayos controlados aleatorizados cabeza a cabeza, en general de buena calidad, para un total aproximado de 658 pacientes. Se identificó evidencia de los efectos del sildenafilo en monoterapia comparado con bosentán, y en terapia combinada más bosentán o epoprostenol, para una variedad de desenlaces incluyendo el tiempo al primer evento de morbilidad/mortalidad, capacidad de ejercicio, mortalidad, hospitalización, cambio en la clase funcional (OMS), empeoramiento de la hipertensión, trasplante de pulmón, disnea, parámetros hemodinámicos, calidad de vida, eventos adversos globales, serios y específicos. La evidencia disponible corresponde a adultos con hipertensión arterial pulmonar, de clase funcional (OMS) I, II, III y IV. También se presentan los eventos adversos reportados en la etapa post-clínica con el uso del sildenafilo. Conclusiones: la evidencia identificada en esta evaluación de tecnología, muestra efectos mixtos en la efectividad y seguridad del sildenafilo para el tratamiento de adultos con hipertensión arterial pulmonar: en cuanto a la monoterapia con sildenafilo, los resultados de efectividad demuestran que este medicamento es similar a sus comparadores; respecto a su seguridad, hay incertidumbre. En terapia combinada, algunos hallazgos de efectividad muestran superioridad del sildenafilo frente a sus comparadores, otros datos indican que esta tecnología es similar respecto a sus alternativas y para algunos desenlaces existe incertidumbre. La seguridad comparada del sildenafilo en terapia combinada puede ser mayor, similar, menor o incierta. A juicio de los expertos clínicos y representantes de los pacientes, el sildenafilo en monoterapia y en terapia combinada, tiene una relación favorable entre los beneficios y riesgos, esto sugiere que los efectos deseables con el uso de esta tecnología superan a los efectos indeseables.(AU)


Subject(s)
Humans , Familial Primary Pulmonary Hypertension/drug therapy , Colombia , Reproducibility of Results , Sildenafil Citrate/administration & dosage , Technology Assessment, Biomedical , Treatment Outcome
9.
Rev. Soc. Cardiol. Estado de Säo Paulo ; 25(3): 147-155, jul.-set. 2015. tab
Article in Portuguese | LILACS | ID: lil-788856

ABSTRACT

O avanço no tratamento das cardiopatias tem permitido um progressivo número de mulheres com doenças cardíacas congênitas alcançarem a idade reprodutiva e a gravidez. Assim, o conhecimento sobre as modificações fisiológicas do ciclo gravídico-puerperal e sua interface com as lesões cardiacas estruturais é fundamental para a adequada conduta em portadoras de cardiopatia congênita durante a gestação. Neste artigo, discutiremos os fatores de prognóstico materno-fetal, as principais complicações, os cuidados obstétricos durante a gestação, parto e puerpério e o planejamento familiar da mulher com cardiopatia congênita.


Advances in the treatment of congenital heart defects has led to a gradually increase in the number of women with congenital heart defects reaching reproductive age and pregnancy. Thus, a knowledge of the physiological changes of the pregnant-puerperal cycle, and their interface with structural heart defects, is essential for adequate conduct in patients with congenital heart disease during pregnancy. In this article, we discusses thefactors of maternal-fetal prognosis, the main complications, the obstetric care provided during pregnancy, labor and puerperium, and family planning for women with congenital heart defects.


Subject(s)
Humans , Female , Adult , Heart Defects, Congenital/mortality , Heart Defects, Congenital/therapy , Prenatal Care/methods , Risk Factors , Pregnancy , Parturition , Fetal Development , Heredity , Familial Primary Pulmonary Hypertension , Maternal Mortality , Women , Fontan Procedure/methods
10.
Article in English | WPRIM | ID: wpr-183078

ABSTRACT

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 +/- 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.


Subject(s)
Adult , Aged , Connective Tissue Diseases/complications , Data Collection , Databases, Factual , Familial Primary Pulmonary Hypertension/epidemiology , Female , Heart Defects, Congenital/complications , Humans , Internet , Male , Middle Aged , Prognosis , Prospective Studies , Pulmonary Artery/physiopathology , Registries , Republic of Korea/epidemiology , Survival Rate , Young Adult
11.
Article in English | WPRIM | ID: wpr-195235

ABSTRACT

BACKGROUND/AIMS: Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for IPAH, the benefits are uncertain, particularly in Korea, where it has not been studied. The purpose of this study was to evaluate survival outcomes of Korean patients with IPAH treated with warfarin. METHODS: We performed a retrospective cohort study of patients diagnosed previously with pulmonary arterial hypertension (PAH) at the Asan Medical Center in Korea, between January 1994 and February 2013. We excluded patients with associated PAH, patients who did not undergo right heart catheterization (RHC), and patients with a positive vasoreactivity test. Patients in the study cohort were classified into a "warfarin group" and a "non-warfarin group," according to the treatment they received during the first year after diagnosis. RESULTS: We identified 31 patients with IPAH and a negative vasoreactivity test on RHC. Median patient age was 36.0 years, and 23 patients (74.2%) were female. The median time from the onset of symptoms to diagnosis was 19.0 months, and the most common presenting symptom was dyspnea. Survival rates of the patients at 1, 3, 5, and 10 years were 90.2%, 79.5%, 62.7%, and 34.8%, respectively. The mean survival period was 12.0 years in the warfarin group and 6.1 years in the non-warfarin group. Warfarin treatment had significant survival benefits in patients with IPAH (p = 0.023). CONCLUSIONS: Warfarin treatment substantially improved survival outcomes in Korean cases of IPAH.


Subject(s)
Adult , Anticoagulants/therapeutic use , Asian Continental Ancestry Group , Familial Primary Pulmonary Hypertension/diagnosis , Female , Humans , Kaplan-Meier Estimate , Male , Proportional Hazards Models , Protective Factors , Republic of Korea , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome , Warfarin/therapeutic use
12.
Chinese Journal of Cardiology ; (12): 319-322, 2015.
Article in Chinese | WPRIM | ID: wpr-328804

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the efficacy of atrial septostomy in idiopathic pulmonary arterial hypertension (IPAH) patients complicating right ventricular failure.</p><p><b>METHODS</b>This retrospective analysis included 5 IPAH patients (3 males, (29.3±15.2) years old) with right ventricular failure which were refractory to conventional and target-specific medication in Shanghai Chest Hospital from March to July 2014. Graded balloon dilation septostomy procedures were performed in all 5 patients.</p><p><b>RESULTS</b>Successful atrial septostomy was achieved in 5 attempts with no procedure-related complications. Immediately post procedure, the mean systemic oxygen saturation decreased from (98.0±1.8)% to (86.4±3.2)% (P = 0.002), while the mean right atrial pressure decreased from (18.9±1.7) mmHg (1 mmHg = 0.133 kPa) to (16.0±1.3) mmHg (P = 0.039) and the mean cardiac index increased from (2.1±0.3) L · min(-1) · m(-2) to (2.7±0.5) L · min(-1) · m(-2) (P = 0.029). Mean follow-up was (6.2 ±1.8) months. Cardiac functional class (WHO) was 3 in 3 patients and 4 in 2 patients before the procedure, and increased 1 class in all patients during follow-up (P = 0.062). Exercise endurance (6-min walk test) also improved from (289.2±16.9) m to (320.4±19.6) m (P = 0.019), while B-type natriuretic peptide (BNP) level declined from (550.0±35.7) ng/L to (218.0±36.2) ng/L (P < 0.001). Except one patient developed spontaneous closure of created defect, right to left shunt at atrial septal level was evidenced by echocardiography during follow-up in the rest 4 patients.</p><p><b>CONCLUSIONS</b>Atrial septostomy is safe and can improve hemodynamics and heart function in selected IPAH patients with right heart failure. Atrial septostomy can be used as a palliative treatment for IPAH and further study is warranted to evaluate the long-term efficacy of this procedure.</p>


Subject(s)
Adult , Catheterization , Familial Primary Pulmonary Hypertension , General Surgery , Female , Heart Failure , Heart Septum , General Surgery , Hemodynamics , Humans , Male , Palliative Care , Retrospective Studies , Ventricular Dysfunction, Right , General Surgery , Young Adult
13.
Bogotá; IETS; dic. 2014. tab, ilus.
Monography in Spanish | LILACS, BRISA | ID: biblio-847169

ABSTRACT

Introducción: la Hipertensión pulmonar (HTP) es una enfermedad progresiva caracterizada por el aumento de la presión arterial pulmonar (por encima de 25 mm HG) debido al aumento de la resistencia vascular pulmonar. La enfermedad lleva a falla cardíaca ventricular derecha y muerte prematura. Actualmente es causante de alta morbilidad y mortalidad entre los pacientes que la sufren, presentando una tasa de supervivencia media una vez diagnosticada la enfermedad de alrededor de 3,6 años. Esta evaluación tecnológica se desarrolló en el marco de la actualización integral del Plan Obligatorio de Salud para el año 2015. Objetivo: evaluar la efectividad y seguridad del uso de ambrisentan para el tratamiento de pacientes con hipertensión arterial pulmonar idiopática y asociada. Metodología: la evaluación fue realizada de acuerdo con un protocolo definido a priori. Se realizó una búsqueda sistemática hasta noviembre de 2014 en MEDLINE, EMBASE, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects y LILACS, sin restricciones de idioma ni fecha de publicación. Se identificaron las RSL y ECA que cumplieran los criterios de elegibilidad. La calidad de los estudios fue valorada con la herramienta AMSTAR. Las características de los estudios fueron extraídas a partir de las publicaciones originales.Se actualizó la búsqueda de la RSL para identificar nuevos ECA. Los resultados se presentan de acuerdo al reporte de la RSL. Se realizó un meta-análisis de novo con los ECA que compararon solo ambrisentan. Resultados: se identificó una RSL de calidad alta por AMSTAR y moderada por GRADE que incluye dos ECA (393 pacientes). El ambrisentan disminuye el riesgo de muerte (OR=0,24 (IC 95% 0,06-0,99)) comparado contra placebo. No existe evidencia a favor para la mejoría de clase funcional (OR=1,08 (IC 95%0,72-1,62)). La mejoría en la prueba de la caminata de 6 minutos, fue en promedio de 43,02 metros (IC95% 25,68 a 60,36) en comparación con el placebo. La calidad de vida, mejoró estadísticamente significativa en la función física del SF-36 (3.41,+/-6.96) comparada con placebo (-0.20, +/-7.14). La hospitalización fue menor en el grupo de ambrisentan (OR=0,39 (IC95% 0,16-0,97)) comparado contra placebo. Conclusiones: de acuerdo con los hallazgos de una RSL de alta calidad el ambrisentan es una tecnología segura, con efectos a favor, ya que disminuye la mortalidad de los pacientes con HTP, mejora la distancia recorrida en la prueba de 6 minutos, mejora la prueba de disnea de Borg, disminuye la hospitalización y mejora la CVRS en el constructo de función física. No se encontró evidencia para el desenlace de atrioseptostomía. En general el ambrisentan es bien tolerado, comparado con el placebo, el perfil de seguridad de la tecnología en cuanto a hepatoxicidad es a favor del ambrisentan. Los eventos adversos más frecuentes reportados en el grupo de ambrisentan fueron edema periférico, cefalea, sinusitis, flushing y congestión nasal comparados contra el placebo.


Subject(s)
Humans , Endothelin A Receptor Antagonists/therapeutic use , Familial Primary Pulmonary Hypertension/drug therapy , Biomedical Technology , Colombia , Treatment Outcome
14.
Article in English | IMSEAR | ID: sea-163476

ABSTRACT

Severe pulmonary Arterial Hypertension with Pulmonary Edema with Sepsis in a postnatal mother with Atrial Septal Defect (ASD) followed by LSCS is uncommon. Atrial Septal Defect (ASD) is the commonest adult congenital heart defect (CHD). 15 % of these patients will eventually develop pulmonary hypertension if left untreated. ASD closure is not recommended when pulmonary hypertension is irreversible. Congenital heart disease should be considered in the evaluation of dyspnoea in a young adult. The management of ASD with associated pulmonary hypertension is difficult. It is pertinent that a detailed hemodynamic assessment be undertaken. The present case report focusses on a patient with severe ASD with pulmonary hypertension with pulmonary edema and sepsis who was with 35 weeks of gestation and the control of symptoms during Caesarean section.


Subject(s)
Adult , Cesarean Section/methods , Familial Primary Pulmonary Hypertension/complications , Familial Primary Pulmonary Hypertension/drug therapy , Female , Heart Septal Defects, Atrial/complications , Heart Septal Defects, Atrial/drug therapy , Humans , Pregnancy , Pulmonary Edema/drug therapy , Pulmonary Edema/etiology , Sepsis/etiology , Sepsis/drug therapy
15.
Chinese Journal of Pediatrics ; (12): 468-472, 2014.
Article in Chinese | WPRIM | ID: wpr-345764

ABSTRACT

<p><b>OBJECTIVE</b>As an important method of hemodynamic assessment in idiopathic pulmonary arterial hypertension (IPAH), cardiac catheterization combined with pulmonary vasoreactivity testing remains with limited experience in children, and the acute pulmonary vasodilator agents as well as response criteria for vasoreactivity testing remain controversial. The aim of this study was to investigate the clinical importance, agent selection, and responder definition of cardiac catheterization combined with pulmonary vasoreactivity testing in pediatric IPAH.</p><p><b>METHOD</b>The patients admitted to Department of Pediatric Cardiology of Beijing Anzhen Hospital between April 2009 and September 2013 with suspected IPAH, under 18 years of age, with WHO functional class II or III, were enrolled. All the patients were arranged to receive left and right heart catheterization and pulmonary vasoreactivity testing with inhalation of pure oxygen and iloprost (PGI2) respectively. Hemodynamic changes were analyzed, and two criteria, the European Society of Cardiology recommendation criteria (Sitbon criteria) and traditional application criteria (Barst criteria), were used to evaluate the test results.</p><p><b>RESULT</b>Thirty-nine cases of children with suspected IPAH underwent cardiac catheterization. In 4 patients IPAH was excluded; 4 patients developed pulmonary hypertension crisis. The other 31 patients received standard cardiac catheterization and pulmonary vasoreactivity testing. Baseline mean pulmonary artery pressure (mPAP) was (66 ± 16) mmHg (1 mmHg = 0.133 kPa), and pulmonary vascular resistance index (PVRI) (17 ± 8) Wood U · m². After inhalation of pure oxygen, mPAP fell to (59 ± 16) mmHg, and PVRI to (14 ± 8) Wood U · m² (t = 4.88 and 4.56, both P < 0.001) . After inhalation of PGI2, mPAP fell to (49 ± 21) mmHg, and PVRI to (12 ± 9) Wood U · m² (t = 7.04 and 6.33, both P < 0.001). According to the Sitbon criteria, the proportion of pure oxygen responders was 6.5% (3/31) , while PGI2 responders was 35.5%, and the difference was significant (P = 0.004). According to the Barst criteria, the proportion of pure oxygen responders was 16.1% (5/31), while PGI2 responders was 51.6% (16/31), and the difference was significant (χ² = 0.09, P = 0.001).</p><p><b>CONCLUSION</b>For children with IPAH, cardiac catheterization combined with pulmonary vasoreactivity testing has important value in differential diagnosis, severity estimation, and treatment (including the emergency treatment) choices. Pulmonary hypertension crisis is an important complication of cardiac catheterization in pediatric IPAH. Younger age, general anesthesia, crisis history, and poor heart function are important risk factors for pulmonary hypertension crisis. PGI2 is a relatively ideal agent for vasoreactivity testing in children with IPAH, which has more responders than traditionally used pure oxygen.</p><p><b>RESULTS</b>of responders are not completely consistent using different criteria, and comprehensive evaluation should be done according to the goals of treatment in clinical practice.</p>


Subject(s)
Administration, Inhalation , Adolescent , Anesthesia, General , Cardiac Catheterization , Child , Child, Preschool , Familial Primary Pulmonary Hypertension , Diagnosis , Female , Hemodynamics , Humans , Iloprost , Infant , Male , Pulmonary Artery , Pulmonary Circulation , Pulmonary Wedge Pressure , Severity of Illness Index , Vascular Resistance , Vasodilator Agents
16.
Chinese Journal of Pediatrics ; (12): 625-629, 2014.
Article in Chinese | WPRIM | ID: wpr-345727

ABSTRACT

<p><b>OBJECTIVE</b>To determine the relationship between the serum sulfur dioxide, homocysteine and the pulmonary arterial pressure in children with congenital heart defects who generated a pulmonary arterial hypertension syndrome (PAH-CHD), and analyze their role in the pathological process of the disease.</p><p><b>METHOD</b>This was a prospective cohort study, children with systemic pulmonary shunt CHD were selected. The patients were divided into three groups: the CHD with no PAH group:n = 20, 10 males, 10 females, 5 with ventricular septal defect (VSD), 8 with atrial septal defect (ASD) and 7 with patent ductus arteriosus (PDA), mean age (1.9 ± 1.8) years; the CHD with mild PAH group:n = 20, 10 males, 10 females, 12 with VSD, 6 with ASD, and 2 with PDA, mean age (1.0 ± 0.8) year; the CHD with moderate or severe PAH group:n = 20, 8 males, 12 females, 12 with VSD, 6 with ASD, and 1 with PDA, 1 with ASD+VSD, mean age (1.8 ± 1.6) year. Twenty healthy children were enrolled from outpatient department as the control group [included 8 males, 12 females, mean age (1.9 ± 1.5) years]. The homocysteine and SO2 concentrations in the serum samples were detected by a modified high performance liquid chromatographic method with fluorescence detection (HPLC-FD), then, multiple comparisons among the groups were performed with analysis of variance, and the pearson correlation.</p><p><b>RESULT</b>The serum homocysteine concentrations were respectively (11.0 ± 2.7) , (11.7 ± 2.5), (12.0 ± 2.1), (14.3 ± 3.2) µmol/L in the control group, CHD with no PAH group, CHD with mild PAH group, and CHD with moderate or severe PAH group. According to the multiple comparisons, the CHD with moderate or severe PAH group had the highest level (P all < 0.05) .While the comparison within the control group, CHD with none PAH group, and CHD with mild PAH group, the differences were not significant (P all > 0.05). The serum sulfur dioxide strength (concentrated as SO3(2-)) were respectively (10.6 ± 2.4), (8.9 ± 2.3), (7.3 ± 2.9), (4.3 ± 2.1) µmol/L in the control group, CHD with none PAH group, CHD with mild PAH group, and CHD with moderate or severe PAH group. CHD with moderate or severe PAH group had the highest level of serum sulfur dioxide (P < 0.05) . The pearson correlation analysis indicated that in the CHD children, the serum homocysteine were positively correlated with the pulmonary arterial pressure (r = 0.481, P < 0.01), while, the sulfur dioxide were negatively correlated with pulmonary arterial pressure (r = -0.553, P < 0.01).In all children, the serum homocysteine levels were negatively correlated with the sulfur dioxide (r = -0.231, P = 0.039).</p><p><b>CONCLUSION</b>The PAH-CHD children had higher homocysteine levels and lower sulfur dioxide levelsl, which demonstrated the disturbance of homocysteine-sulfur dioxide pathway in the sulfur containing amino acids metabolish in the disease. The homocysteine may become a biological marker which reflecting the severities of the PAH-CHD, while the sulfur dioxide can be a new target for the therapy of PAH-CHD.</p>


Subject(s)
Biomarkers , Blood , Case-Control Studies , Child, Preschool , Ductus Arteriosus, Patent , Blood , Familial Primary Pulmonary Hypertension , Blood , Female , Heart Defects, Congenital , Blood , Heart Septal Defects , Blood , Hemodynamics , Homocysteine , Blood , Humans , Infant , Male , Sulfur Dioxide , Blood
17.
Yonsei Medical Journal ; : 1526-1532, 2014.
Article in English | WPRIM | ID: wpr-221610

ABSTRACT

PURPOSE: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. MATERIALS AND METHODS: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9+/-14.5 years, 80% of female) were enrolled. RESULTS: Causes were congenital heart disease in 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6+/-45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, median survival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). CONCLUSION: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore, early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.


Subject(s)
Adult , Antihypertensive Agents/therapeutic use , Disease Management , Familial Primary Pulmonary Hypertension , Female , Heart Defects, Congenital/complications , Humans , Hypertension/complications , Hypertension, Pulmonary/classification , Kaplan-Meier Estimate , Male , Middle Aged , Molecular Targeted Therapy/methods , Prognosis , Retrospective Studies , Survival Rate
18.
Chinese Medical Journal ; (24): 3798-3802, 2014.
Article in English | WPRIM | ID: wpr-240684

ABSTRACT

<p><b>OBJECTIVE</b>The objective of this study is to review the research on the prognostic markers of idiopathic pulmonary arterial hypertension (IPAH).</p><p><b>DATE SOURCES</b>We searched literature from PubMed and CNKI databases both in English and Chinese up to 2013.</p><p><b>STUDY SELECTION</b>Data about mortality and cut-off value are from clinical trials and identified by analysis.</p><p><b>RESULTS</b>IPAH is an unexplained, progressive, and rare disease characterized by increased pulmonary artery pressure and pulmonary vascular resistance. The diagnosis is difficult, mortality of IPAH is high, and the survival periods are only 2-3 years after diagnosis. Investigations in recent years have identified a range of prognostic markers for IPAH, including the 6-minute walking test, red blood cell distribution width, and platelet levels, as well as imaging findings. Changes in these markers are important sources of information to predict the prognosis of patients with IPAH, which carries significant benefits for treatment planning.</p><p><b>CONCLUSION</b>Even though the prognosis of IPAH has been investigated, the mortality is also high. More accurate and meaningful assessment for the prognosis of IPAH is required.</p>


Subject(s)
Biomarkers , Blood , Familial Primary Pulmonary Hypertension , Blood , Metabolism , Pathology , Humans , Prognosis
19.
Article in Chinese | WPRIM | ID: wpr-254209

ABSTRACT

<p><b>OBJECTIVE</b>To observe the clinical efficacy and safety of sildenafil in the treatment of high altitude heart disease associated with severe pulmonary arterial hypertension (PAH) in children.</p><p><b>METHODS</b>Fifty children (aged 2 months to 2 years) with high altitude heart disease associated with severe PAH, who were continuously transferred to the Intensive Care Unit between January 2011 and October 2013, were randomly assigned to observation and control groups. The control group was given conventional treatment, while the observation group received oral sildenafil [1 mg/(kg . d)] three times daily for 7-10 days in addition to the conventional treatment. Before and after treatment, hemodynamics, blood gas, routine blood parameters, and blood biochemical parameters were recorded.</p><p><b>RESULTS</b>After treatment, the observation group had a significantly higher decrease in mean pulmonary artery pressure and significantly higher increases in arterial partial pressure of oxygen, cardiac output, cardiac index, and oxygenation index compared with the control group (P<0.05). In the observation group, there were no significant changes in mean arterial pressure, routine blood parameters and blood biochemical parameters (P>0.05), and no obvious adverse reactions were found.</p><p><b>CONCLUSIONS</b>For children with high altitude heart disease associated with severe PAH, sildenafil can effectively reduce pulmonary artery pressure and improve cardiac function and does not cause adverse reactions. This therapy has good safety according to the preliminary evaluation.</p>


Subject(s)
Altitude , Familial Primary Pulmonary Hypertension , Female , Heart Diseases , Drug Therapy , Humans , Hypertension, Pulmonary , Infant , Male , Piperazines , Therapeutic Uses , Purines , Therapeutic Uses , Sildenafil Citrate , Sulfones , Therapeutic Uses , Vasodilator Agents , Therapeutic Uses
20.
Chinese Journal of Cardiology ; (12): 1001-1005, 2014.
Article in Chinese | WPRIM | ID: wpr-303789

ABSTRACT

<p><b>OBJECTIVE</b>To analyze the clinical characteristics of WHO Class I pulmonary hypertension (PAH) in central China.</p><p><b>METHODS</b>Data was collected as a part of prospective registry of PAH through Jan. 2009 to Oct. 2013 in Wuhan Asia Heart Hospital. A total of 195 patients were recruited including 144 cases with congenital heart disease with pulmonary hypertension (CHD-PAH) and 51 cases with idiopathic pulmonary hypertension (IPAH).</p><p><b>RESULTS</b>The age of all patients ranged from 1 to 68 years (mean (27.5 ± 13.2) years), 129 cases were female (66.2%). WHO Class I PAH accounted for 91.1%, CHD-PAH 67.3%, IPAH 23.8%, and other 8.9%.WHO function class III/IV in newly diagnosed PAH accounted for 32.3%, the mean 6MWD was (397 ± 74) m. For patients with IPAH, the median time period between onset of symptoms and diagnosis by right heart catheterization was 38 months. The mean pulmonary pressure, pulmonary vascular resistance index, cardiac index of patients with IPAH and CHD-PAH were measured by the right heart catheterization and there was no difference between the two groups. Acute pulmonary vasodilator testing was negative in all patients in this cohort. Cardiac function was improved in the 121 cases who received the targeted drug treatment and 1 patient died out of these 121 patients while 5 cases died out of patients receiving conventional therapy.</p><p><b>CONCLUSION</b>In Central China, Class I pulmonary hypertension is the most predominant type of PAH, the cardiac function and hemodynamic indexes of these patients were significantly impaired at the time of first PAH diagnosis. Most of the patients accepted targeted drug treatment of pulmonary hypertension, but the drug dose used for the targeted drug treatment is not effective enough in these patients.</p>


Subject(s)
Adolescent , Adult , Aged , Cardiac Catheterization , Child , Child, Preschool , China , Familial Primary Pulmonary Hypertension , Pathology , Female , Heart Defects, Congenital , Hemodynamics , Humans , Hypertension , Hypertension, Pulmonary , Pathology , Infant , Male , Middle Aged , Prospective Studies , Registries , Vasodilator Agents
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