Severe Pulmonary Hypertension Secondary to Concomitant Mitral Stenosis with Veno-occlusive Disease in the Context of Systemic Sclerosis: Importance of Careful and Comprehensive Assessment

Pulmonary Arterial Hypertension (PAH) is a clinical syndrome consisting of physiologic/ hemodynamic criteria that are a consequence of several etiologies. Confirmation of pulmonary hypertension is based on right heart catheterization. Pulmonary hypertension is a devastating condition that can lead to considerable morbidity and premature mortality. In the last few decades, significant advancement in the pharmacotherapy of pulmonary hypertension has resulted from better understanding of the complex pathogenesis and pathophysiology of this dreaded disease. Despite these accomplishments, pharmacotherapy of pulmonary hypertension is still far from perfect, and the mortality in this modern treatment era is still unacceptably high. We report a complex clinical presentation characterized by severe pulmonary hypertension secondary to concomitant mitral stenosis with veno-occlusive disease in the context of systemic sclerosis. Our case highlights the importance of a systematic and comprehensive diagnostic approach to avoid missing an underlying pathology.

Clinical features of POEMS syndrome initially diagnosed as pulmonary hypertension / 中华全科医师杂志

Objective:To analyze the clinical characteristics, diagnosis and treatments of patients with POEMS syndrome initially diagnosed as pulmonary hypertension (PH).Methods:Clinical data of 7 patients who were initially diagnosed as PH and finally diagnosed as POEMS syndrome in Shanghai Pulmonary Hospital from May 2013 to November 2021 were retrospectively reviewed. Clinical manifestations, laboratory tests, echocardiography, hemodynamic findings, treatment and prognosis of patients were analyzed.Results:Seven patients, including 4 males and 3 female, aged (55±9) (44-62) years were presented with elevated pulmonary artery pressure by echocardiography at admission. Chest tightness and shortness of breath (7/7), fatigue (6/7) and lower limb edema (4/7) were the most common symptoms in the first-episode. Meanwhile, patients also presented symptoms associated with POEMS syndrome, including multiple peripheral neuropathy (7/7), multiserosal cavity effusion (6/7), organomegaly (5/7), skin changes (5/7), and endocrine lesions (4/7). Serum levels of vascular endothelial growth factor (VEGF) were significantly increased in all patients. The pulmonary arterial systolic blood pressure was (66±21)mmHg (1 mmHg=0.133 kPa) estimated by echocardiography. Six patients underwent right heart catheterization and significantly increased mean pulmonary artery pressure((35±9) mmHg) was confirmed; and their pulmonary vascular resistance was (4.00±2.10) Wood U. All patients received corresponding treatment for POEMS syndrome. The excise tolerance was improved in 5 patients after successful treatment with stable or reversed WHO functional class. One patient received hemodialysis treatment for uncontrolled POEMS. One patient died during follow-up. The echocardiography was followed up in 4 patients, and 2 of whom had a complete reversal of PH, 1 had a partial reversal, and 1 had not yet reversed.Conclusions:In patients with PH who have multisystem manifestations, such as multiple peripheral neuropathy, multiserosal cavity effusion, organomegaly and skin changes, POEMS syndrome should be considered, and proper and active treatment of POEMS may reverse PH and improve the prognosis of patients.

Revisión Sistemática Exploratoria sobre Uso de Óxido Nítrico Inhalado para Evaluación de Reactividad Vascular Pulmonar en Laboratorio de Hemodinamia en Sujetos Pediátricos con Hipertensión Pulmonar / Exploratory Systematic Review on the Use of Inhaled Nitric Oxide for Evaluation of Pulmonary Vascular Reactivity in Hemodynamics Laboratory in Pediatric Subjects with Pulmonary Hypertension

Introducción. La hipertensión arterial pulmonar (HAP) es una enfermedad que presenta un elevado índice de mortalidad en la población pediátrica. Para su diagnóstico, el gold standard es la prueba de reactividad vascular pulmonar (PRVP), debido a que permite medir la respuesta vasodilatadora del lecho vascular pulmonar frente a la administración de moléculas con acción terapéutica, como el óxido nítrico inhalado (iNO). Esta prueba al ser positiva se asocia a un mejor pronóstico. En la actualidad existe incertidumbre y falta de consenso sobre la indicación y administración de iNO durante la PRVP. Objetivo. Describir el uso reportado en la literatura sobre iNO en PRVP en sujetos pediátricos con HAP. Métodos. Revisión sistemática exploratoria sensible en bases de datos PubMed, Epistemonikos, Cochrane, Scopus, Lilacs y Scielo, que describen el uso de iNO durante la PRVP en sujetos pediátricos con HAP. Resultados. se identificaron 8.906 artículos, de los cuales se seleccionaron 5 para la revisión cualitativa. La PRVP se realizó durante el cateterismo cardiaco derecho (CCD) en sujetosentre 2 semanas y 18 años de edad. Los diagnósticos fueron HAP primaria, idiopática y asociada a patología cardiaca congénita, cardiomiopatía y enfermedad pulmonar. Esta prueba fue realizada en sujetos sólo con soporte de oxígeno o con sedación profunda en ventilación mecánica invasiva, con dosis variables de oxígeno (21 y 100%) e iNO (3 y 80 ppm), o asociado a otras moléculas como iloprostol®, dilitiazem, sildenafil y/o epoprostenol. La administración de iNO disminuyó presión de arteria pulmonar y la resistencia vascular pulmonar, con mantención de presión arterial sistémica y gasto cardiaco y sin complicaciones asociadas a su uso. Conclusiones. Existen escasos estudios sobre iNO en PRVP pediátrica y con calidad metodológica limitada. El iNO se utiliza como método diagnóstico de vaso reactividad en sujetos pediátricos con HAP asociada a cardiopatía congénita, primaria o secundaria. Los protocolos para su uso son variables con dosis entre 20 y 40 ppm, con o sin uso de oxigeno adicional, con tiempos poco precisos y sin consenso en equipos de administración.

Background. Pulmonary arterial hypertension (PAH) is a disease that has a high mortality rate among the pediatric population. For its diagnosis, the pulmonary vascular reactivity test (PVRT) is considered the "Gold Standard", because it allows to measure the vasodilator response of pulmonary vascular circulation with the administration of molecules with therapeutic action, such as inhaled nitric oxide (iNO). This test, when positive, is associated with a better prognosis of the disease. Currently, there's uncertainty and lack of consensus on the indication and administration of iNO during the PVRT. Objetives. to describe use of iNO in PVRT in pediatric users with PAH reported in the literature. Methods. Scoping review of studies published between 1992 and 2021 in PubMed, Epistemonikos, Cochrane, Scopus, Lilacs and Scielo databases, which describe the use of iNO during PVRT in pediatric users with PAH, in English and Spanish. Primary and secondary studies with a sensitive search strategy were considered. Results. 8,906 articles were identified, 40 were selected by title, 8 by full text, and 5 for final qualitative review. Of the total of articles selected, 3 were primary and 2 secondary studies. PVRT was performed during right heart catheterization (RHC) in a population between 2 weeks and 18 years old. Diagnoses were primary PAH, idiopathic PAH and PAH associated with congenital heart disease, cardiomyopathy and pulmonary disease. This test was carried out in subjects on spontaneous ventilation with oxygen support or with deep sedation in invasive mechanical ventilation, with variable oxygen doses between 21 and 100%, with exclusive use of iNO between 3 and 80 ppm, being more used between 20 and 40 ppm, or associated with other molecules such as iloprostol®, dilithiazim, sildenafil and / or epoprostenol. In all selected studies, administration of iNO decreased PAP (pulmonary artery pressure) and PVR (pulmonary vascular resistance), with maintenance of SBP (systemic arterial blood pressure) and cardiac output. The primary studies were made up of pre and post-test of serial or parallel interventions. The selected studies of iNO in PVRT did not report complications associated with its use. Conclusions. studies on iNO in pediatric PVRT are scarce in number of publications and methodological quality. iNO is used as a diagnostic method of vasoreactivity in pediatric users with PAH associated with congenital, primary, or secondary heart disease. The protocols for its use are variable with recommended doses between 20 and 40 ppm, with or without the use of additional O2, with imprecise times and without consensus in administration equipment. The response to PVRT serves as a guide for the treatment and prognosis of pediatric users with PAH.

Efficacy of transcatheter pulmonary valve perforation by micro-guidewire and balloon dilation in neonates with pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objective@#To investigate the safety and efficacy of transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS).@*Methods@#The retrospective study included 21 neonates (14 males and 7 females) with PA-IVS who underwent transcatheter micro-guidewire pulmonary valve perforation and balloon dilation in Xinhua Hospital from January 2012 to December 2018. All patients underwent the pulmonary valve perforation by micro-guidewire through the Simmons catheter. During the follow-up period at 1, 3, 6, 12 months postoperatively and annually thereafter, the operative efficacy and the development of the right ventricle (RV) were evaluated by echocardiography. Statistical analyses were performed using t test.@*Results@#A total of 21 neonates with PA-IVS were enrolled, and 13 cases were diagnosed prenatally. The median age of surgery was 6 days, the weight was (3.2±0.5) kg. The balloon/valve ratio was 1.19±0.12, and the RV pressure measured by catheter was (121±33) mmHg (1 mmHg=0.133 kPa) . The immediate postoperative RV pressure was (47±13) mmHg. The median follow-up time was 30 months. All the cases enrolled achieved biventricular circulation without death and serious complications. According to the last follow-up data including 16 cases who were followed up for 1 year or longer, the pulmonary artery transvalvular pressure was (29±15) mmHg. The postoperation ratio of right to left ventricular transverse diameter was significantly higher than that before operation (0.86+0.10 vs. 0.73+0.13, t=-2.96, P=0.006). Compared with preoperative data, the postoperation pulmonary valvular diameter Z-score was significantly higher (-1.41±0.89 vs. -2.83±1.06, t=-3.65, P=0.001) and the tricuspid valvular diameter Z-score was significantly higher (-0.52±0.29 vs. -1.34±0.81, t=-3.55, P=0.001).@*Conclusion@#Transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation is a safe and effective initial therapy for neonatal PA-IVS.

Valoración de ecuaciones derivadas del flujo pulmonar y regurgitación tricuspídea: utilidad en el test de vasoreactividad pulmonar / Valuation of equations derived from Pulmonary Flow and Tricuspid Regurgitation: usefulness in Pulmonary Vasoreactivity Testing

RESUMEN: Antecedentes: La presión media arterial pulmonar (PMAP) es una variable hemodinámica indispensable para el diagnóstico, clasificación y pronóstico de la Hipertensión Pulmonar (HP). Su cuantificación se realiza en forma invasiva por cateterismo cardíaco derecho (CCD) y no invasivamente por ecocardiografía Doppler. Masuyama propuso su medición mediante el gradiente transvalvular pulmonar diastólico derivado de la velocidad máxima inicial de la regurgitación pulmonar (∆RPi2) correspondiendo cercanamente a la medición invasiva. Objetivos: Revalidar 3 métodos ecocardiográficos que estiman la PMAP y valorar la utilidad del método de Chemla en el Test de Reactividad Vascular Pulmonar (TRVP). Métodos: Estudio prospectivo, observacional, doble ciego divido en dos etapas. A) o I) 30 pacientes se realizó ecocardiografía Doppler diagnóstica en nuestro centro. Se midieron regurgitación tricuspídea (RT) y tiempo de aceleración pulmonar (TAP) para derivar las siguientes ecuaciones: 1) 0.61xPSAP+1.95 (Chemla) 2) Gradiente presión media RT (∆PmRT) +PAD (presión-aurícula derecha) (Aduen) y 3) 79-0.45xTAP o 90-0.60xTAP, según sea el valor del TAP. B) o II) 10 pacientes enrolados para realizar el TRVP comparando la medición ecocardiográfica (Chemla) con CCD. Resultados: En la primera parte del estudio se encontró alta correlación entre las 3 ecuaciones: ChemlaAduen, R2=0.91; Chemla-Kitabatake, R2=0.87; Aduen-Kitabatake, R2=0,91. En la segunda parte comparando la PMAP-Chemla y Cateterismo derecho (CD) obtuvimos alta correlación: en tiempo 0, 30 min y recuperación:(R2=0.87, 0.99, 0.98, respectivamente). Ambas partes del estudio mostraron límites de concordancia satisfactoria con valor medio de la diferencia entre los métodos cercano a 1 en el t30 y tR del TRVP. Conclusión: los métodos dependientes de la medición de la RT son efectivos y confiables para estimar la PMAP. El método de Chemla es útil en el TRVP.

ABSTRACTS: Background: Mean Pulmonary arterial pressure (PMAP)is an indispensable hemodynamic variable for the diagnosis, classification and prognosis of Pulmonary Hypertension (PH). Its quantification is performed invasively by cardiac catheterization and non-invasively by Doppler echocardiography. Masuyama proposed its measurement by the transvalvular diastolic pulmonary gradient derived from the initial maximum velocity of pulmonary regurgitation(ΔPRi2) corresponding closely to the invasive measurement. Objectives: to compare 3 known echocardiographic methods to estimate MPAP and demonstrate the usefulness of the Chemla method in the Pulmonary Vascular Reactivity Test (PVRT). Methods: prospective, observational, double-blind study divided into two stages. A) 30 patients underwent diagnostic Doppler echocardiography. Tricuspid regurgitation (TR) and pulmonary acceleration time (PAT) were measured to derive the equations: 1) 0.61xSPAP + 1.95 (Chemla) 2) Gradient mean pressure TR (ΔPmTR) + RAP (right atrium pressure) (Aduen).3) 79-0.45xPAT o 90-0.60xPAT depending on the value of PAT. B) 10 patients enrolled to PVRT comparing the echocardiographic measurement (Chemla) with right catheterization. Results: in the first part of the study a high correlation between the 3 equations was found : ChemlaAduen, R2 = 0.91; Chemla-Kitabatake, R2=0.87; Aduen-Kitabatake, R2=0.91. In the second part comparing the MPAP-Chemla and RHC we obtained a high correlation in time 0, 30 min and recovery: (R2=0.87,0,99,0.98, respectively). Both parts of the study showed satisfactory limits concordance with mean value of the difference between the methods close to 1 in the t30 and tR of the TRVP. Conclusion: the methods dependent on the measurement of the TR are effective and reliable for estimating MPAP. The Chemla method is useful in the PVRT.

Pulmonary hypertensive crisis in children with idiopathic pulmonary arterial hypertension undergoing cardiac catheterization: the risk factors and clinical aspects / 中华儿科杂志

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, χ2 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 (χ2=4.970, P=0.026), history of syncope (χ2=4.948, P=0.026), and WHO cardiac functional class Ⅲ or Ⅳ (χ2=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.

Comparative Study Between Complete Ultrasound Guidance and Radiation Guidance in Percutaneous Balloon Pulmonary Valvuloplasty / 中国循环杂志

Objective:To evaluate the safety and efficacy of ultrasound guidance for percutaneous balloon pulmonary valvuloplasty (PBPV) in comparison with conventional X-ray guidance.Methods:Our research included in 2 groups:Ultrasound group,n=102 patients with PBPV under ultrasound guidance in our hospital from 2013-03 to 2016-08 and X-ray group,n=280 patients with PBPV under traditional X-ray guidance in our hospital at the same period of time.Post-operative effect was evaluated by echocardiography and compared between 2 groups.Results:The patients' age,body weight,pulmonary artery diameter,immediate post-operative pulmonary transvalvular pressure gradient (PTPG),the in-hospital time and cost were similar between 2 groups,P＞0.05.The success rate of operation in Ultrasound group and X-ray group was 99.0％ vs 100％,P=0.267.In the ultrasound group,1 patient was converted to a conventional surgery due to right ventricular outflow tract muscle spasm after dilation.The operation time in X-ray group was longer than Ultrasound group,(38.9±9.2) min vs (34.6±10.0) min,P＜0.001.The X-ray exposure time was (3.9±1.2) min in X-ray group.The mean follow-up time was (25.5±13.2) months and PTPG in Ultrasound group and X-ray group were (16.2±4.3) mmHg and (15.3±4.5) mmHg,P=0.120.No serious complications as death,peripheral vascular injury,cardiac perforation and pericardial effusion occurred in either group.Conclusion:PBPV under complete ultrasound guidance may not only avoid radiation and contrast agent,but also keep the safety and efficacy of minimally invasive conventional percutaneous interventional treatment.

Comparative Study Between Complete Ultrasound Guidance and Radiation Guidance in Percutaneous Balloon Pulmonary Valvuloplasty / 中国循环杂志

Objective:To evaluate the safety and efficacy of ultrasound guidance for percutaneous balloon pulmonary valvuloplasty (PBPV) in comparison with conventional X-ray guidance.Methods:Our research included in 2 groups:Ultrasound group,n=102 patients with PBPV under ultrasound guidance in our hospital from 2013-03 to 2016-08 and X-ray group,n=280 patients with PBPV under traditional X-ray guidance in our hospital at the same period of time.Post-operative effect was evaluated by echocardiography and compared between 2 groups.Results:The patients' age,body weight,pulmonary artery diameter,immediate post-operative pulmonary transvalvular pressure gradient (PTPG),the in-hospital time and cost were similar between 2 groups,P＞0.05.The success rate of operation in Ultrasound group and X-ray group was 99.0％ vs 100％,P=0.267.In the ultrasound group,1 patient was converted to a conventional surgery due to right ventricular outflow tract muscle spasm after dilation.The operation time in X-ray group was longer than Ultrasound group,(38.9±9.2) min vs (34.6±10.0) min,P＜0.001.The X-ray exposure time was (3.9±1.2) min in X-ray group.The mean follow-up time was (25.5±13.2) months and PTPG in Ultrasound group and X-ray group were (16.2±4.3) mmHg and (15.3±4.5) mmHg,P=0.120.No serious complications as death,peripheral vascular injury,cardiac perforation and pericardial effusion occurred in either group.Conclusion:PBPV under complete ultrasound guidance may not only avoid radiation and contrast agent,but also keep the safety and efficacy of minimally invasive conventional percutaneous interventional treatment.

Evaluation of Pulmonary Hypertension Using Tricuspid Regurgitation Spectrum / 中国医学影像学杂志

Purpose The mean pulmonary artery (MPAP) has been widely used as an important parameter to diagnose and evaluate pulmonary hypertension (PH).The purpose of this paper is to compare the efficacy of two methods in evaluating PH,including estimating pulmonary artery systolic pressure (PASP) using Doppler ultrasonography to measure tricuspid regurgitation (TR) velocity,and directly using the peak velocity of TR.Materiasl and Methods From January 2012 to June 2013,eighty patients with left-to-right shunt congenital heart diseases (CHD) planned for closure procedure in Tangdu Hospital of the Fourth Military Medical University and the General Hospital of Shenyang Military region were included in this prospective study,who underwent right heart catheterization to measure pulmonary artery pressure,and underwent Doppler ultrasonography to measure the peak velocity ofTR.Results Using catheter-measured MPAP of≥ 25 mmHg as diagnostic reference,the false positive rate was 62.96％,and the false negative rate 0％ when the estimated PASP of ＞30 mmHg determined by TR method was used to diagnose PH.There was high diagnostic agreement when peak velocity of TR was used to diagnose PH.When 320 cm/s and 340 crn/s were used as diagnostic cutoff values,false positive rates were 14.81％ and 7.41％,and false negative rates were 15.91％ and 20.45％,respectively.Conclusion In patients with left-to-right shunt CHD,peak velocity of TR measured on echocardiography can be used to diagnose PH which overcomes the high false positive rate in estimation method.It is more suitable to diagnose PH when the MPAP is used as the diagnostic criterion.

Safety and efficacy of percutaneous patent ductus arteriosus closure via femoral vein under transesophageal echocardiography guidance / 中华实用儿科临床杂志

Objective To investigate the efficacy and safety of percutaneous patent ductus arteriosus (PDA) closure via femoral vein solely under transesophageal echocardiography guidance.Methods From May 2014 to May 2015,28 patients(13 boys,15 girls) were selected in Dalian Children's Hospital Affiliated to Dalian Medical University with PDA closure via the femoral vein under transesophageal echocardiography guidance,with mean age (3.5 ± 2.6)years and mean body weight (16.0 ± 6.5) kg.The mean diameter of PDA was (7.1 ± 3.9) mm.Patients were all treated by percutaneous PDA closure solely by transesophageal echocardiography via the femoral vein.The effect of the procedures was evaluated by echocardiography.The transthoracic echocardiography,chest X-ray film,cardiogram at 1 month,3 months and 6 months after procedure were followed up.Results Twenty-seven cases were successfully treated with percutaneous PDA closure via the femoral vein solely under transesophageal echocardiography guidance,while 1 patient was closed by surgical closure with on-pump beating-heart because PDA occluder strayed into the left pulmonary artery on 1-month follow-up.The procedural time was (48.5 ±8.7) min.The mean diameter of PDA occluder was (8.2 ± 4.1) mm.Twenty-seven patients survived without peripheral vascular injury or complications such as residual shunt,arrhythmia and cardiac perforation.One patient was transformed to surgical closure.Hospitalization time was (2.5 ± 0.5) days.At one month follow-up,no complications such as residual shunt or pericardial effusion occurred.Conclusion Transesophageal echocardiography guided percutaneous PDA closure via the femoral vein approach is safe and effective without the damage from radiation and contrast agents,and aviods the use of femoral artery puncture.

Cardiac Functional Changes in 8 Patients After Percutaneous Pulmonary Valve Implantation / 中国循环杂志

Objective: To evaluate the changes of left and right ventricular function in patients after percutaneous pulmonary valve implantation (PPVI). Methods: A total of 8 patients with successful PPVI in our hospital from 2014-05-27 to 2015-03-25 were studied. The patients received pre-operative evaluation including clinical symptoms, plasma BNP levels, ECG, chest X-ray, echocardiography, CT and MRI examinations. Post-operative follow-up study was conducted at 6 months after PPVI to compare the ventricular functional changes. Results: The average age in 8 patients was 14-42 (25.4±8.1) years including 7 with tetralogy of Fallot correction and 1 with pulmonary stenosis plasty for 24 years. Echocardiography found that 3 patients with mid to large pulmonary regurgitation and 5 with large regurgitation. Compared with pre-operation, at 6 months after PPIV, all patients had decreased right ventricular end-diastolic diameter (RVEDD) as (44.0±4.8) mm vs (33.6±7.1) mm, right ventricular end-diastolic volume index (RVEDVI) (150.1±25.7) ml/m2 vs (111.4±39.1) ml/m2, RVESVI (107.8±21.5) ml/m2 vs (80.7±22.2) ml/m2 and right ventricular cardiac output (RV-CO) (6.8±1.3) L/min vs (4.9±0.8) L/min, but right ventricular ejection fraction (RVEF) was similar (40.5±6.2) % vs (39.5±9.9) %, P>0.05; while increased LVEDD (42.9±4.4) mm vs (46.1±3.0) mm, P0.05. Conclusion: PPVI may decrease right ventricular preload, improve its reverse remodeling and maintain systolic function at normal level in relevant patients; while the impacts on left ventricular function and geometry should be further studied.

Prevalence of Pulmonary Arterial Hypertension in Korean Adult Patients with Systemic Sclerosis: Result of a Pilot Echocardiographic Screening Study

BACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with systemic sclerosis (SSc). Early detection and prompt treatment of PAH associated with SSc (SSc-PAH) result in better prognosis. We conducted echocardiographic study to presume the prevalence of PAH in Korean adult SSc patients and to diagnose SSc-PAH in their early stages with right heart catheterization (RHC). METHODS: We performed free of charge echocardiographic study including 37 adult SSc patients at the Chungnam National University Hospital. The possibility of PAH is determined by the estimation of pulmonary arterial pressure by peak tricuspid regurgitation velocity of > 3.0 m/s. Patients with possible PAH were recommended to undergo RHC to confirm the diagnosis. RESULTS: In 37 patients, 8 patients were suspected with PAH. Among them, 6 patients agreed to be examined with RHC, and 4 were confirmed with PAH. The prevalence of possible PAH was 21.6% (8 of 37 patients), and that of confirmed PAH was 10.8% (4 of 37 patients). Four patients who were confirmed with SSc-PAH through RHC have been treated with specific pulmonary vasodilators and maintained stable. CONCLUSION: Eight patients (21.6%) were possible PAH and 4 (10.8%) were diagnosed as SSc-PAH by RHC after the echocardiographic screening study of 37 adult SSc patients.

Resultados imediatos e de médio prazo da intervenção com stent ductal em recém-nascidos e lactentes / Immediate and medium-term outcomes of ductal stenting in neonates and infants

O implante de stents para manter o ducto arterial patente na cardiopatia congênita cianótica éuma alternativa à cirurgia de Blalock-Taussig modificada (BTm) em pacientes de alto risco. Descrevemos osresultados imediatos e de médio prazo do implante de stent em neonatos e lactentes com circulação pulmonarducto-dependente. Métodos: Trata-se de estudo descritivo e prospectivo, que incluiu diferentes cardiopatias congênitas cianóticas tratadas entre 2014 e 2015.Resultados: Avaliamos 14 pacientes, com média de idade de 46 dias e pesando 4,5 kg, sendo a atresia pulmonar associada à comunicação interventricular a cardiopatia mais tratada. A abordagem pela artéria femoral ocorreu em 70% dos procedimentos e, nos demais, por via carotídea. Stents de 3,5 × 12 mm foram usados na maioria dos casos, e o sucesso do implante foi obtido em 78% das intervenções (11/14). Os casos de insucesso foram encaminhados para cirurgia − uma delas em situação de urgência, que resultou em óbito. Ocorreu espasmo ductal < 48 horas em três pacientes que necessitaram de BTm, com evolução favorável. Complicações após a alta e nos primeiros 30 dias incluíram trombose de stent (2/11), uma delas controlada com redilatação e outra que evoluiu para óbito, e uma morte súbita (1/11). A mortalidade total foi de 21,4% (3/14). A patência do ducto arterial nos primeiros 6 meses foi obtida em 5 casos que foram submetidos à cirurgia paliativa.Conclusões: A experiência inicial de implante de stent ductal mostrou resultados imediatos favoráveis, e, emmédio prazo, mais de um terço dos pacientes com circulação pulmonar ducto-dependente manteve seus canaispatentes...

The implantation of stents to keep the ductus arteriosus patent in cyanotic congenital heart disease is an alternative to the modified Blalock-Taussig surgery (mBT) in high-risk patients. This study describes theimmediate and medium-term outcomes of stent implantation in neonates and infants with duct-dependentpulmonary circulation. Methods: This was a descriptive and prospective study including different cyanotic congenital heart diseases treated between 2014 and 2015. Results: Fourteen patients with a mean age of 46 days, and mean weight of 4.5 kg were assessed, andpulmonary artresia with interventricular communication was the most treated condition. The femoral artery approach was used in 70% of procedures; carotid approach was used in the remaining cases. Stents of 3.5 x 12 mm were used in most cases, and implant success was achieved in 78% of interventions (11/14). The failed cases were referred to surgery – one of them was an emergency, which resulted in death. Ductal spasm occurred in < 48 hours in three patients who required mBT, with favorable outcome. Complicationsafter discharge and within the first 30 days included stent thrombosis (2/11), one of which was controlled with redilation, another progressed to death, and one sudden death (1/11). The overall mortality was 21.4% (3/14). A patent ductus arteriosus in the first 6 months was present in five cases, which underwent palliative surgery.Conclusions: The initial experience of ductal stenting showed favorable immediate outcomes, but in the mediumterm, little more than a third of the cases maintained a patent ductus arteriosus within 6 months...

Transesophageal echocardiography guided occlusion of ventricular septal defect via small chest incision: a report of 142 cases / 中国医师杂志

Objective To investigate the experience of microinvasive surgical occlusion of ventricular septal defect (VSD).Methods A total of 142 children with VSD was given microinvasive surgical occlusion from March 2009 to December 2013 at our hospital.There were 90 males and 52 females,the age ranged from 8 months to 11 years,and body weight from 7 kg to 35 kg,and ventricular septal defects were divided into membranous type,film cycle headquarter type,pulmonary valve type,and muscle type.The diameter of VSD was 2 ～ 10 mm.Under general anesthesia,an incision was made in the lower part of sternum or intercostals space,and a special occluder was inserted to close the ostium via right ventricle puncture under the guidance of transesophageal echocardiography.Results A total of 139 cases had successful occluded with a 97.8％ of successful rate,using blocking umbrella 4 ～ 12#,including 25 eccentric umbrella.Two cases were operated under extracorporeal circulation because of aggravated aortic valve insufficiency.One case without handled muscular ventricular septal defect combined atrial septal defect for guide wire pass muscular defect failed.Full set did not have death and third degree A-V block.Conclusions Microinvasive surgical occlusion is easy to handle,operation-time short,and relative broad for the closure of ventricular septal defect.It has a fast recovery and good effectiveness with a beautiful outlook and safety.

Optimization of detection method of pulmonary arterial pressure / 中国药理学通报

Aim To optimize the traditional method of right catheterization in rats and establish a rapid , stable and reliable method of the right heart catheter guided intubation to measure pulmonary artery pressure. Methods Nighty male wistar rats were used to optimize the method of detection of pulmonary arte-rial pressure. Three catheter namely PE50, PU I, and PU II were used for choosing the best intubation. The new technology of right catheterization was established and used for the research of pulmonary arterial hypertension. Results The PU I catheter was obviously better than PE50 and PU II catheter in the success rate and measurement time ( P <0. 05 ) . The method of right heart catheter guided intubation was significantly superior to the traditional right heart direct intubation (P<0. 05 or P<0. 01). After improving the right catheterization, the detection of hemo-dynamic indexes in PAH-model rat was successful with regular pressure curve and reliable experimental data. Conclusions The right heart catheter guided intubation method has a high suc-cess rate and it can detect the pulmonary artery pressure quick-ly, easily, and can help other researchers to complete experi-ment as efficiently as possible.

Visão geral do diagnóstico e identificação de pacientes de risco / Overview of the diagnosis and identification of at-risk populations

A hipertensão pulmonar é um estado hemodinâmico e fisiopatológico que pode ser encontrado em diversas condições clínicas e quando presente, está associada ao aumento da morbimortalidade. Somados à etiologia diversa estão os achados clínicos inespecíficos. Esses dois aspectos contribuem para o atraso no diagnóstico e, por conseguinte, no início do tratamento. Daí a importância da sistematização da avaliação diagnóstica aliada à suspeição clínica na investigação de dispneia, sobretudo nas populações de risco, visando o diagnóstico etiológico correto e a instituição de medidas terapêuticas adequadas. O seguimento desses pacientes deverá ser feito também de forma sistemática, associado a ferramentas complementares que permitam a avaliação adequada da gravidade da doença...

Pulmonary hypertension is a hemodynamic and pathophysiological state that can be find in various clinical conditions and is associated with increased morbidity and mortality. As symptoms are nonspecific and the condition is uncommon, usually the diagnosis is delayed and, consequently, the start of treatment.Hence, the importance of systematization of diagnostic evaluation combined with clinical suspicion in dyspnea research, especially in at-risk populations, in order to achieve the correct etiologic diagnosis and institution of appropriate therapeutic measures. The follow-up of these patients also should be do systematically, together with complementary tools that allow the proper assessment of disease severity...

Changes features of middle hepatic vein flow velocity waveform in patients with pulmonary hypertension caused by congenital heart disease / 中华医学超声杂志（电子版）

were measured at the end of inspiratory.ResultsThe differences of following parameters were statistically significant between the healthy control group, mild PH group, moderate PH group and severe PH group: velocity time integral of ventricular systolic wave (SVTI), velocity time integral of ventricular diastolic wave (DVTI), AR, AR/S, ARVTI/SVTI, AR/(S+D), ARVTI/(SVTI+DVTI,F=2.940, 2.838, 3.373, 7.681, 3.478, 12.052, 4.806;P<0.05,<0.05,<0.05,<0.01,<0.05,<0.01,<0.01). Correlation analysis showed that ratio of AR/(S+D) was positively correlated with pulmonary artery mean pressure (r= 0.575,P<0.01). ConclusionsThe ratio of AR/(S+D) of MHV Doppler flow spectra was correlated with pulmonary artery mean pressure. The ratio increased along with the increasing of pulmonary pressure in CHD patient. The Doppler echocardiographic detection of MHV flow had a certain reference value for the diagnosis of PH. The ratio of AR/(S+D) could be a new non-invasive parameter for evaluating PH.

Follow-up results of heart conduct block after transcatheter closure of ventricular septal defect surgery / 实用医学杂志

Objective To study the prevalence and risk factors of heart conduct block after transcatheter closure of ventricular septal defect (VSD)surgery. Methods A total of 1 069 cases underwent transcatheter closure VSD were retrospectively analyzed. The risk factors were assessed by multivariable logistical analysis. Results The median follow-up time was 2.2 (1 to 4.16) years. The early post-procedure heart conduct block was 20.5 %(219 cases), and 35 cases underwent severe conduct block (3.3%). During the follow-up, there were 43 late onset heart conduct block (4.0%), including 4 (0.4%) complete atrioventricular block. Multivariable logistic analysis showed that implanted of asymmetrical occluder from foreign company was the risks factors for early onset severe conduct block, with longer procedure time. Placement of thin-waist-big-side occluder were risk factor for the late onset conduct block. Conclusions Heart conduct block after transcatheter closure VSD is common , light and recovery. The late onset severe conduct block is minor. Symmetrical occluder should be chosen in transcatheter closure VSD if possible.

Eight Hundred and ninety-one cases with perimembranous ventricular septal defect performed minimally invasive transthoracic device closure / 中国医师杂志

Objective To investigate retrospectively the therapeutic effect and safety of minimally invasive transthoracic device closure of isolated perimembranous ventricular septal defect (PVSD).Methods Eight hundred and ninety-one consecutive patients with PVSD from December 2008 to October 2014 in our hospital were enrolled,which performed minimally invasive transthoracic device closure of ventricular septal defect (VSD),with a 4 ～ 5 cm incision made on the inferior sternum.Under the guide of transthoracic echocardiography (TEE),right ventricular free wall was punctured followed by a delivery sheath threaded into the VSD and the occluder was released thereafter.Whether were there residual shunt and the occluder anchored firmly or not,the position of occluder and the influence on heart valve were re-evaluated intraoperatively by TEE.All patients were followed up prudently post-operation.Echocardiography and electrocardiogram were scheduled according to the protocol.Results Eight hundred and thirty-four cases were successfully occluded.Fifty-seven cases were performed VSD repair operation with cardiopulmonary bypass.The amounts of concentric occluder and eccentric occluder were 703 and 133,respectively.The patients performed minimally invasive transthoracic device closure of VSD had no severe complications after operation.Conclusions Although the short-term and mid-term therapeutic effect of minimally invasive transthoracic device closure of PVSD seem plausible to be definitive,with a favorable prospective application,the long-term follow-up remains to be studied.

Nine cases of children with ventricular septal defect performed super minimally invasive trans intercostal device closure / 中国医师杂志

Objective To introduce the super minimally invasive transintercostal device closure of ventricular septal defect (VSD) and summarize it's therapeutic effect.Methods Nine patiens with VSD from August 2014 to December 2014 in our hospital were enrolled,which performed super minimally invasive transintercostal device closure of VSD.All patients were followed up prudently post-operation.Echocardiography was scheduled according to the protocol.Results All cases were successfully occluded.There was no death case in the peri-operation period and severe complications after operation.Conclusions The super minimally invasive transintercostal device closure of VSD is safe and reliable.