Mild Pulmonary Arterial Hypertension Research Articles

Left Atrial Myxoma with Mild Left Ventricular Dysfunction— A Case Report

Atrial Myxoma is the most common primary cardiac tumors accounting for about 50% of benign primary cardiac tumors, with the majority located in the left atrium. This is a case of large left atrial (LA) myxoma presented with features of mitral stenosis associated with moderate left ventricular failure (LVF) and mild pulmonary artery hypertension (PAH) The patient improved markedly after tumor excision.Pulse Vol.10 January-December 2017 p.29-33

Pulmonary arterial stiffness assessed by cardiovascular magnetic resonance imaging is a predictor of mild pulmonary arterial hypertension

Early detection of mild pulmonary arterial hypertension (PAH) based on clinical evaluation and echocardiography remains quite challenging. In addition to enhanced right ventricular (RV) assessment, cardiac magnetic resonance (CMR) imaging may accurately reflect deleterious remodeling and increased stiffness of the central pulmonary arteries based on pulsatility, or percent change of the PA during the cardiac cycle. The purpose of this study is to assess the utility of measuring PA pulsatility by CMR as a potential early maker in PAH. We hypothesize that pulsatility may help discriminate mild PAH from normal control subjects. Consecutive patients with PAH (n = 51) were prospectively enrolled to receive same day CMR and right heart catheterization (RHC). PA stiffness indices including pulsatility, distensibility, compliance, and capacitance were calculated. Comparisons were made between patients with varying severities of PAH and normal controls (n = 18). Of the 51 subjects, 20 had mild PAH, and 31 moderate-severe based on hemodynamic criteria. PA pulsatility demonstrated a progressive decline from normal controls (53%), mild PAH (22%), to moderate-severe PAH (17%; p < 0.001). There was no difference in RV size, function or mass between mild PAH and normal controls. PA pulsatility below 40% had an excellent ability to discriminate between mild PAH and normal controls with a sensitivity of 95% and specificity of 94%. CMR assessment of PA stiffness may noninvasively detect adverse pulmonary vascular remodeling and mild PAH, and thus be a valuable tool for early detection of PAH. Trial Registration: ClinicalTrials.gov Identifier: NCT01451255; https://clinicaltrials.gov/ct2/show/NCT01451255 .

A rare case of Ebstein’s anomaly scheduled for elective caesarean section

Ebstein’s anomaly is a rare congenital heart defect comprising less than 1% of patients with congen-ital heart disease. Among the congenital heart lesions, Ebstein’s anomaly is one of the most diverse in presentation, severity and management. It involves the septal cusp of the tricuspid valve wherein, due to anomalous attachment of the leaflets of the valve the cusp is elongated causing downward displacement of the valve into right ventricle. This results in tricuspid regurgitation. The abnormally situated valve produces atrialization of the right ventricle. It may be associated with atrial septal defect and supraventricular ar-rhythmias. Here, we report a case of second gravida patient with Ebstein’s anomaly. She was diagnosed with it a year back and was not on any treatment. Her echocardiogram showed grossly dilated right atrium grade III tricuspid regurgitation with mild pulmonary artery hypertension. Patient was taken up for elective cae-sarean section under spinal anaesthesia. Key-words: caesarean section, spinal anaesthesia, bupivacaine

Impact of preoperative pulmonary arterial hypertension on early and late outcomes in patients undergoing valve surgery for rheumatic heart disease.

Background and Aims:There is conflicting evidence on adverse effect of Pulmonary Arterial Hypertension (PAH) on outcomes after cardiac surgery for rheumatic heart disease (RHD). The authors studied Indian patients with RHD and preoperative PAH, who undergo cardiac surgery with a hypothesis that they have poor short and long-term outcomes.Methods:This was a retrospective observational study of 407 patients. The patients were divided in three groups based on PAH estimated on echocardiograph as; no or mild PAH (pulmonary artery systolic pressure (PASP) <30 mm of Hg); moderate PAH (PASP 31-55 mm of Hg) and severe PAH (PASP >55 mm of Hg). The primary endpoint was in-hospital mortality and major morbidities; while secondary endpoint was long-term survival.Results:In-hospital mortality was 24 (5.9%); and was not different in patients with severe, (9.1%), moderate (4.5%) or mild PAH (2.8%) (P = 0.09). Patients with severe PAH had higher incidence of prolonged ventilation (P = 0.007). Factors independently associated with mortality were; >2-packed cell transfusion, prolonged ventilation and acute kidney injury but not moderate and severe PAH. Patients with mitral stenosis (MS) and severe PAH had significantly higher mortality as compared to no or mild PAH (P = 0.03) on long-term follow-up [81.37% (mean duration 19.40 ± 14.10 months)], mortality was 8% and not statistically different (P = 0.25) across PAH categories.Conclusion:Moderate and severe PAH does not affect short and long term outcomes of patients undergoing valve surgery for RHD. Patients with MS with severe PAH had higher mortality compared to those with no PAH.

Case Report on Dressler’s Syndrome

Introduction: Dressler’s syndrome (delayed pericarditis) is considered as a secondary form of pericarditis resulting in the inflammation of the sac surrounding heart (pericardium). Case Presentation: A 56-year-old male was admitted to the cardiology department due to left sided chest pain associated with breathlessness, palpitation and sweating. patient had a past history of CAD-AWMI, moderate left ventricular(LV) dysfunction (diagnosed 2 months back). Percutaneous transluminal coronary angioplasty (PTCA) with stent to CAD done 2 months back. ECHO shows mild to moderate pericardial effusion, mild pulmonary arterial hypertension(PAH), moderate mitral regurgitation (MR), moderate LV dysfunction. Conclusion: This reveals that the patient is diagnosed with Dressler’s syndrome, a rare disease in the age of reperfusion therapy.

Echocardiographic Evaluation of the Right Atrial Area Index in Dogs with Pulmonary Hypertension

BackgroundRight atrial area (RAA) is a prognostic factor in human patients with pulmonary arterial hypertension (PAH). Reference intervals for RAA have been described in healthy dogs.ObjectivesTo evaluate RAA indexed to the body surface area in dogs with PAH as an indicator of right atrial size, PAH severity and right‐sided congestive heart failure (R‐CHF).AnimalsA total of 119 client‐owned dogs, 48 dogs with PAH and 71 control dogs.MethodsProspective observational study. Pulmonary arterial hypertension was classified according to the tricuspid regurgitation pressure gradient (TRPG) as mild (36–50 mmHg), moderate (51–75 mmHg), or severe (>75 mmHg). The RAA index was calculated as the RAA divided by body surface area.ResultsThe RAA index was higher in dogs with moderate PAH (13.3 cm2/m2; range, 3.4–24.7 cm2/m2) and severe PAH (12.1 cm2/m2; range, 5.4–21.8 cm2/m2) than in those with mild PAH (6.7 cm2/m2; range, 4.8–10.7 cm2/m2) or in controls (7.3 cm2/m2; range, 4.2–10.2 cm2/m2; P < 0.001). The RAA index was higher (P < 0.0001) in dogs with R‐CHF (17.5 cm2/m2; range, 12.7–24.7 cm2/m2) compared to those without R‐CHF (7.6 cm2/m2; range, 4.4–19.4 cm2/m2). The most accurate cutoff value of the RAA index to identify R‐CHF was >12.3 cm2/m2 (sensitivity, 100%; specificity, 89.5%). In dogs with PAH, severity of tricuspid regurgitation (TR) was the only independent predictor of RAA index based on multivariate analysis (P < 0.02).Conclusions and Clinical ImportanceThe RAA index can be used to evaluate right atrial size in dogs and may be more effective than TRPG in predicting R‐CHF in dogs with PAH. The severity of TR is the main determinant of the RAA index in dogs with PAH.

Assessment of Diastolic Dysfunction Parameters and Cardiac Chamber Size in Smokers with COPD: A Case Control Study

Introduction: Chronic Obstructive Pulmonary Disease (COPD) is a critical health care burden across the globe. Pulmonary Hypertension (PH) is a widespread feature of advanced COPD and is estimated to affect 20% of individuals with advanced COPD.Aim: To compare the variables of cardiac function, chamber size and diastolic parameters across various stages of COPD.Materials and Methods: A prospective case control study was conducted among 50 subjects and 50 controls (convenient sampling) with COPD over a period of two years in kasturba Medical college, Manipal (July 2012-July 2014). Chronic smokers who met the criteria for COPD according to GOLD criteria were included in the study. All patients underwent Pulmonary Function Test (PFT) using spirometer.Results: Twenty seven out of 50 studied patients (54%) had Pulmonary Artery Hypertension (PAH). Nineteen patients had mild PAH (38%). Dimension of cardiac chamber across various stages of COPD were found to have a significant decrease in Left Atrial (LA) area. Our study found an increase in Isovolumic Relaxation Time (IVRT), Pulmonary Artery Pressure (PAP) and TEI index (myocardial performance index) across stages of COPD however it was not statistically significant.Conclusion: Majority of the patients with COPD had PH, with increasing frequency of PAH across the GOLD stages. A decrease in cardiac chamber size was demonstrated with increasing severity of COPD.

Prevalence of pulmonary artery hypertension in patients of chronic obstructive pulmonary disease and its correlation with stages of chronic obstructive pulmonary disease, exercising capacity, and quality of life

Background:Pulmonary arterial hypertension (PAH) is a complication of chronic obstructive pulmonary disease (COPD) in advance stages, and its presence indicates poor prognosis.Aims and Objective:The present study was design to know the prevalence of PAH in patients with COPD and its correlation with stages of COPD, exercising capacity, and quality of life.Materials and Methods:It is a cross-sectional prevalence study over a period of 1 year from August 2015 to July 2016. The study included 109 COPD patients, diagnosed by spirometry, and severity was determined according Global Initiative for Chronic Obstructive Lung Disease (GOLD) classification criteria. Screening two-dimensional echocardiography was done to determine pulmonary arterial hypertension and exercising capacity assessed by 6 min walk test (6MWT) while the quality of life was assessed by St George respiratory Questionnaire for COPD (SGRQ-C) Questionnaires.Results:Out of 109 patients, PAH was present in 68 (62.4%) cases consisting of mild grade 41 (37.6%), moderate grade 11 (10.1%), and severe grade 16 (14.7%). In GOLD A stage, there were 20 cases of mild PAH and Stage B included 18 cases of mild and 3 cases of moderate PAH. Stage C had 3 cases of mild and 8 cases of moderate PAH while Stage D had 16 cases of severe PAH. In 6MWT, patients with severe grade PAH fail to perform the test while patients with mild to moderate PAH walked short distance. In SGRQ-C Questionnaires symptom, activity, impact, and total score were high with the severity of PAH.Conclusion:The prevalence of PAH in COPD was significant. Therefore, every COPD patient should be evaluated for PAH.

Long-term outcome and prognostic factors in pregnant women with pulmonary arterial hypertension associated with congenital heart disease

Objective: To investigate the perinatal outcome, risk factors and long-term outcome of pregnancy complicated with pulmonary arterial hypertension(PAH) and congenital heart diseases (CHD). Methods: Clinical data of 110 pregnant women who were diagnosed as PAH-CHD were retrospectively analyzed in the Department of Obstetrics and Gynecology and Surgical Intensive Care Unit at Beijing Anzhen Hospital from 2004 to 2013. The survival and treatment status were followed up. Results: 110 subjects consisted of 11 mild PAH, 33 moderate and 66 severe ones. The incidences of deterioration in New York Heart Association (NYHA) classes (≥2) during pregnancy, respiratory failure, pulmonary hypertension crisis and arrhythmia were 25.5% (28/110), 7.3% (8/110), 10.0% (11/110), 10.0% (11/110) respectively. Among them, the difference of deterioration in NYHA classes (≥2) during pregnancy among the three groups was statistically significant. A total of 8 (7.3%) maternal deaths occurred during hospitalization, all of whom were severe PAH cases. Multivariate analysis showed that pulmonary artery systolic pressure was a risk factor of perioperative death (OR=1.042, P=0.005). There were 55 cases (50.0%) of term delivery, and 35 cases (31.8%) of iatrogenic abortion. The proportion of term delivery in the severe PAH group was significantly lower. The proportion of iatrogenic abortion and small for gestational age infant (SGA) were higher in severe group. The incidence of neonatal malformations was 8.0% (6/75). The follow-up rate was 61.8% (63/102). Sudden death was reported in a parturient a few days after discharge. The remaining 62 patients survived during follow-up, while 53 patients (85.5%) were functional class (FC) Ⅰ-Ⅱ, 9 (14.5%) were FC Ⅲ-Ⅳ at follow-up. The cardiac function deterioration during pregnancy was not significantly correlated with long-term deterioration (P=0.767). Conclusions: Perinatal mortality and the incidence of maternal and fetal adverse events were high in pregnancy with PAH-CHD. Pulmonary artery systolic pressure is a major risk factor for perioperative mortality in pregnant women. PAH-CHD woman had good overall outcome after puerperium.

High Intensity Interval Training in a Rat Model of Severe, Angioproliferative Pulmonary Arterial Hypertension

PURPOSE: We previously demonstrated superior benefit of high intensity interval training (HIIT) over continuous exercise training (CET) in a monocrotaline rat model of mild pulmonary arterial hypertension (PAH). Here we investigate HIIT in a model that elicits a more severe, angioproliferative PAH. METHODS: SD rats (~200g, male) received Sugen5416 (20mg/kg), followed by 3 wks of hypoxia (Patm=362 mmHg) and 4 wks of room air to induce PAH (SuHx, n=33). Subgroups of SuHx then underwent 6 wks of treadmill training performed as either HIIT (2 min at ~80-90%VO2reserve [VO2R] + 3 min at 30%VO2R, for 4-5 cycles, n=12), or low intensity CET (45-60 min at 50%VO2R, n=11), with the remainder untrained (SED, n=10). Values are mean±SE. RESULTS: Mortality in SuHx was unexpectedly worse for HIIT (4 deaths at 56, 60, 68, and 71 days), vs. CET (2 deaths at 60, and 73 days), and SED (2 deaths at 66 days). While all animals had similar baseline echocardiographic measures of cardiac output (CO, in uL) and stroke volume (SV, in mL/min), SuHx that died prematurely (n=8) had greater impairment in CO (141±19) and SV (424±52) following PAH induction (at pre-training) compared to surviving SuHx (n=25, 235±26, 598±38). Final CO and SV were higher for both HIIT (239±49, 540±58) and CET (207±41, 529±51) vs. SED (122±12, 405±35) SuHx rats, and were similar to untrained healthy controls (CON, n=6, 268±59, 548±71). SuHx-induced elevation in right ventricular (RV) systolic pressure (mmHg) and RV hypertrophy (as RV mass/LV+septum mass, and as RV thickness on echo in mm) were not improved by training with HIIT (61±7; 0.60±0.07, 2.2±0.2) or CET (60±8; 0.47±0.04, 2.0±0.2), vs. SED (55±8; 0.52±0.06, 2.2±0.2), and were higher than CON (28±3; 0.24±0.01, 1.3±0.1). Final VO2max (ml/kg/min) in SuHx was also not improved for either HIIT (44±1.9) or CET (46±1.7) vs. SED (46±3.8). CONCLUSION: Both HIIT and CET promoted better RV function in SuHx rats despite no amelioration of RV hypertrophy, PAH, or exercise impairment. However, in contrast to previous findings in a mild PAH rat model, exercise training, particularly with HIIT, increased mortality for animals with poorer cardiac function prior to training onset and suggests that further investigation is needed to optimize training approach for patients with more severe RV dysfunction. Funding: NIH-NHLBI R-15 (to MB Brown).

Utility of Hepatocyte Growth Factor as a Biomarker for Early Diagnosis of Pulmonary Artery Hypertension.

The goal of this study was to determine plasma levels of hepatocyte growth factor (HGF) in patients with pulmonary artery hypertension (PAH), and to explore the diagnostic value of plasma HGF for PAH. Sixty subjects were divided into a control group of healthy individuals (N=15) and a PAH group (N=45). The PAH group was divided into three groups (N=15 each) according to disease severity: mild PAH (groupL), moderate PAH (groupM), and severe PAH (groupH). Plasma HGF levels in PAH patients were collected on the morning after admission to the hospital. Mean pulmonary arterial pressure was measured by right heart catheterization. Plasma HGF levels were significantly higher in the PAH group than in the control group (P<0.001), and significantly higher in groupH than in groupM (P<0.001) and groupL (P<0.001). There was no statistically significant difference in plasma HGF levels between patients with PAH of idiopathic etiology and those with PAH of secondary etiology (P=0.595). The HGF level was positively correlated with mean pulmonary arterial pressure (Pearson correlation coefficient 0.967, P<0.001). Plasma levels of HGF in PAH patients with mild disease were significantly higher than those in healthy controls, suggesting that plasma HGF has potential utility as a diagnostic biomarker for early PAH.

High Intensity Interval Training is Superior to Continuous Training in a Pulmonary Hypertension Rat Model

Exercise appears to have overall benefit in pulmonary arterial hypertension (PAH); however, studies to date indicate little effect on the elevated pulmonary artery pressure (PAP) or RV hypertrophy (RVH) and dysfunction associated with the disease. We investigated if the greater endothelial stimulation of high intensity interval training (HIIT) would provide superior benefit over the more customary prolonged continuous exercise training (CExT) protocol in a rat model of PAH. We further sought to characterize acute PAP responses to HIIT and CExT in a PAH rat. METHODS: Six wks of treadmill training was performed 5x/wk in male Sprague-Dawley rats (250-300g) with monocrotaline- (MCT, 40 mg/kg) induced mild PAH. Training sessions were either HIIT (2 min at ~90% VO2 reserve [VO2R] + 3 min at 30% VO2R, for 5 cycles; n=8), or low intensity CExT (60 min at 50% VO2R; n=7). In an additional rat, telemetric recording of simultaneous PAP and systemic pressures were obtained during HIIT and CExT running, serially at pre- and 2, 4, 6, and 8 wks post-MCT. Values are means±SE. RESULTS: MCT-induced decrement in VO2max was ameliorated by both HIIT and CExT (p≤0.01 vs. sedentary MCT rats, MCT-SED, n=6), and were similar to healthy sedentary controls (CON, n=6). Most importantly, RV systolic pressure (in mmHg) and RVH (ratio of RV to LV+S mass) were lowered (p<0.05) only by HIIT (28.7±2, and 0.32±0.02) and not by CExT (44.1±3, and 0.43±0.01) vs. MCT-SED (40.2±3.2, and 0.41±0.02). Echocardiography performed pre- and post-HIIT also indicated reduced RVH ([INCREMENT] wall thickness 0.11±8 mm) and improved RV function ([INCREMENT] cardiac output 117±28 μl) vs. MCT-SED (0.79±8 mm, and 6±42 μl, p<0.05). RV fibrosis was less only for HIIT (p<0.05 vs. MCT-SED), and no training effect was observed on RV inflammation and apoptosis or pulmonary vascular remodeling. Pulmonary endothelial nitric oxide synthase was increased (p<0.05) only with HIIT, consistent with enhanced endothelial stimulation. Implantable telemetry revealed repeated ‘surging’ of PAP during HIIT running, followed by more pronounced PAP reduction during recovery. CONCLUSIONS: HIIT may be superior to CExT for improving hemodynamics and RV dysfunction in PAH and may be explained by greater sheer-stress mediated vascular endothelial adaptation to HIIT stimulus. Funding: Amer. Heart Assoc. SDG to Brown.

Abdominal lymphadenopathy: An interesting and rare case diagnosed on endoscopic ultrasound-guided fine needle aspiration cytology.

A 68-year-old woman presented to a gastroenterologist with abdominal pain, loss of appetite, and significant weight loss for last 5–6 months. Her history revealed that she had been having recurrent episodes of abdominal pain for last 15 years with postprandial distension and vomiting. She had taken two courses of anti-tubercular therapy (ATT) in the past. These were probably given empirically as no tissue diagnosis was available. She had been investigated extensively in another hospital for her present complaints; however, no diagnosis could be made. Computed tomography scan done outside revealed multiple calcified and noncalcified lymph nodes in the axilla, chest, and abdomen, and healed calcified foci in liver and spleen. There were fibro-calcified lesions in bilateral upper lobes of the lung. There was also mild ascites and bilateral pleural effusions. Physical examination revealed pallor, pedal edema, basal crepitation, and hepatomegaly. She had albuminuria, and 24 h urinary protein levels were raised. Her echocardiography revealed mild pulmonary arterial hypertension. She underwent endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) of the abdominal lymph nodes with a clinical suspicion of tuberculosis. The EUS-FNA was performed using 22 G needle from the peri-pancreatic lymph nodes [Figures ​[Figures11 and ​and22].

Abstract 15949: High Intensity Interval Training is Superior to Continuous Training and Reverses RV Remodeling and Dysfunction in a Rat Model of Pulmonary Hypertension

Exercise appears to have overall benefit in pulmonary arterial hypertension (PAH); however, studies to date indicate little effect on the elevated pulmonary pressure or RV hypertrophy (RVH) and dysfunction associated with the disease. High intensity interval training (HIIT) is reported to be superior to the more customary prolonged continuous exercise training (CExT) protocol for chronic heart failure but has not been tested for PAH. Therefore, we investigated impact of a 6 wk HIIT vs. CExT treadmill program in a monocrotaline rat model of mild PAH (MCT, 40 mg/kg) on indicators of disease progression. Methods: Treadmill training was performed 5x/wk in male Sprague-Dawley MCT rats (250-300g), following a protocol of either HIIT (5 cycles of 2 min at ~90% VO 2 reserve [VO 2 R] + 3 min at 30% VO 2 R; n=8), or low intensity CExT (60 min at 50% VO 2 R; n=7). Statistical analysis was performed by one-way ANOVA. Results: MCT-induced decrements in VO 2 max were ameliorated by both HIIT and CExT (p &lt; 0.01 vs. sedentary MCT rats, MCT-SED, n=6), and were similar to healthy controls (CON, n=6). Most importantly, RV systolic pressure (RVSP, in mmHg; via Millar catheter) and RVH (ratio of RV to LV+S mass) were lowered (p&lt;0.05) only by HIIT (28.7±2, and 0.32±0.02) and not by CExT (44.1±3, and 0.43±0.01) vs. MCT-SED (40.2±3.2, and 0.41±0.02). Cardiac output (Δ from baseline in μl, via RV echocardiography) was also improved by HIIT (117±28) vs. MCT-SED (6±42, p=0.04). Additional hemodynamic recordings during running, via novel implantable telemetry (DSI), were obtained serially at pre- and 2, 4, 6, and 8 wks post-MCT, and revealed ‘surges’ in RVSP during HIIT, vs. a steady RVSP pattern during CExT. Pulmonary eNOS (per immunoblotting) was increased (p&lt;0.05) with HIIT, consistent with greater endothelial stimulation. Conclusions: HIIT is superior to CExT for improving hemodynamics and RV remodeling and dysfunction in MCT rats and warrants further investigation in other models and in patients. More favorable outcomes may be explained by greater sheer-stress mediated vascular endothelial adaptation to HIIT stimulus, or lower cumulative training-induced RV wall stress with the briefer HIIT session duration.

EVALUATION OF STRUCTURAL CHANGES IN THE PULMONARY ARTERIES BY OPTICAL COHERENCE TOMOGRAPHY IN PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION

day following the procedure. The primary outcome was a composite of: sheath insertion outside the common femoral artery limits, transfixing arterial puncture, 2 puncture attempts, hemoglobin drop 30 g/L or red blood cell transfusion, pseudo-aneurysm formation, retroperitoneal haemorrhage, arteriovenous fistula, arterial dissection, and thrombosis. Group comparisons were made using the chi-square test. A total of 80 patients, 73% male, aged 66 10 years, were randomized to the anatomical technique (n 1⁄4 40) or the ultrasound-guided technique (n 1⁄4 40). Indication for coronary angiography was stable angina in 30 (38%) patients and acute coronary syndrome in 50 (63%) patients. The primary composite outcome occurred in 34 (85%) patients randomized to the anatomical technique and in 23 (58%) patients randomized to the ultrasound-guided technique (p1⁄40.007). For every individual outcome, there was no difference in complication rates between anatomical and ultrasound-guided techniques, including specifically sheath insertion outside of the common femoral artery limits (18% vs 13%, respectively; p1⁄40.531). However, there was a trend towards more 2 puncture attempts (53% vs 35%; p1⁄40.115) with the anatomical technique. Significantly more venous punctures were made with the anatomical technique compared to the ultrasound-guided technique (68% vs 43% ; p1⁄40.045). When the sheath was not inserted in the common femoral artery, the most frequent sites of insertion were: superficial femoral artery (6 patients), external iliac artery (4 patients), and deep femoral artery (2 patients). CONCLUSION: In patients undergoing cardiac catheterization via the femoral artery, the ultrasound-guided technique is associated with less minor vascular complications and venous punctures. A trend towards less puncture attempts was also observed.

A Valve in Valve: Mitral Valve Replacement with Tricuspid Annuloplasty in a Case of Mitral Annular Calcification

A 79 year old female presented with history of dyspnoea on exertion (New York Hear Association class III) and palpitation at rest for last 3 years and has developed paroxysmal nocturnal dyspnoea during the last 1 year. On examination she had irregular pulse. She had a hyper dynamic precordium with the apex displaced to the left 6th intercoastal space 1 cm lateral to the midclavicular line. A pan systolic murmur was heard at the apex. Chest X-ray showed annular calcification of the mitral valve. Echocardiogram revealed a dilated left ventricle (LVEDD 69 mm). There was bi-leaflet prolapse causing severe mitral regurgitation. Tricuspid annulus was dilated (43 mm) causing moderate regurgitation (Figure 1). There was a mild pulmonary artery hypertension. She was planned for surgery and under moderate hypothermic cardiac arrest; left atrium was approached through the Sondergaard’s groove. We can appreciate calcification of the mitral annulus extending on the posterior annulus across both commisures and partly into the anterior annulus. It was also extending into the ventricular mass. Mitral valve was replaced with #25 bioprosthetic valve with complete preservation of both the leaflets. Plegetted sutures taken from atrium to ventricle through the leaflets in a way to plicate them were used to fix the prosthetic valve. Tricuspid annuloplasty ring was placed. She had a normal postoperative recovery. Her echocardiogram at discharge and 6 month follow up showed no paravalvular leak and good function of the prosthetic valve (Figure 2).

Agenesia pulmonar: reporte de doscasos

Antecedentes: la agenesia pulmonar es una anomalía poco común (1 por cada 15 000 nacidos vivos) que consiste en la ausencia total o en hipoplasia severa de uno o ambos pulmones. El espectro clínico de la agenesia unilateral puede ir desde dificultad respiratoria temprana y grave, neumonías de repetición, hasta ser un hallazgo incidental. El pronóstico se basa en alteraciones congénitas asociadas. Materiales y métodos: se informan dos casos clínicos de agenesia pulmonar unilateral en pacientes del Hospital Infantil de Tlaxcala durante el 2012. Resultados: se reporta el caso de un niño de un mes de vida con agenesia pulmonar izquierda con comunicación interauricular e hipertensión arterial pulmonar leve. Presentó dos cuadros de neumonía con su resolución. El otro caso fue una niña de un mes de vida con agenesia pulmonar derecha asociada con malformaciones cardiacas múltiples con evolución hacia insuficiencias respiratoria y cardiaca con desenlace de muerte. Conclusiones: la agenesia pulmonar es una anomalía poco común cuya evolución y pronóstico varían de acuerdo con la repercusión hemodinámica relacionada a las malformaciones asociadas.

Circulating Collagen Biomarkers as Indicators of Disease Severity in Pulmonary Arterial Hypertension

ObjectivesThe goal of this study was to determine if biomarkers of collagen metabolism in PAH identify patients with worse disease and higher risk of death. BackgroundThe relationship between circulating markers of collagen metabolism, degree of disease severity, and outcome in pulmonary arterial hypertension (PAH) is unknown. MethodsPatients with stable idiopathic, anorexigen-associated, and hereditary PAH were prospectively enrolled. Levels of the following collagen biomarkers were measured: N-terminal pro-peptide of type III procollagen (PIIINP), C-terminal telopeptide of collagen type I (CITP), matrix metalloproteinase (MMP)-9, and tissue inhibitor of metalloproteinase (TIMP)-1. Patients were divided into mild, moderate, and severe PAH groups. Data were compared between tertiles of each biomarker. Pearson correlation and Spearman rank coefficient analyses were performed. Data on time to death or transplantation were examined by Kaplan-Meier survival curves. ResultsCirculating levels of PIIINP, CITP, MMP-9, and TIMP-1 were higher in the PAH group (n = 68) as compared with age- and sex-matched healthy controls (n = 37) (p < 0.001 for each). PIIINP levels increased with the severity of disease (p = 0.002). PIIINP tertile data indicated that with increasing levels, 6-min walk distance and cardiac index decreased, World Health Organization functional classification worsened, and resting heart rate increased. A significant correlation existed between PIIINP levels and worsening World Health Organization functional classification (rs = 0.320; p < 0.01), and there was a negative correlation between cardiac index and 6-min walk distance (r = −0.304 and r = −0.362, respectively; p < 0.05). PIIINP tertiles showed a trend toward worse outcome in patients with higher tertiles (lung transplant or death) (p = 0.07; log-rank test). ConclusionsMarkers of collagen metabolism were associated with worse disease in patients with PAH.

Analysis of ET-1 system in mild and severe pulmonary arterial hypertension in mice

Analysis of ET-1 system in mild and severe pulmonary arterial hypertension in mice

Pregnancy outcomes of 103 women with pulmonary arterial hypertension

To evaluate the pregnancy outcome of women with pulmonary arterial hypertension (PAH). The medical records of 103 pregnant women with PAH admitted to Beijing Anzhen Hospital from January 2007 to March 2011 were studied retrospectively. (1) Degree of PAH and cardiac function. Among the 103 PAH, 92 cases were patients with congenital heart disease and 13 cases were with rheumatic heart disease. They were divided by color doppler ultrasound into mild PAH group (34 cases), moderate PAH group (22 cases) and severe PAH group (47 cases). Per heart function classification, 21 cases (20.4%, 21/103) were class I, 44 cases (42.7%, 44/103) were class II, 27 cases (26.2%, 27/103) were class III and 11 cases (10.7%, 11/103) were class IV. More patients were class III and IV cardiac function in the severe PAH group than in the mild and moderate PAH groups, with statistically significant difference (P < 0.05). (2) Delivery mode. There were 44 term delivery and 23 preterm birth in the 103 PAH patients.Sixty-three cases (94%, 63/67) received cesarean section and 4 cases had vaginal delivery. There were 36 iatrogenic abortion (35.0%, 36/103). The iatrogenic abortion rate in the severe PAH group was significantly higher than those in the mild and moderate PAH groups (P < 0.05).(3) Perinatal outcomes. The full-term delivery rates in the mild and moderate PAH groups [80% (20/25) and 14/17] were significantly higher than the severe PAH group (40%, 10/25), respectively (P < 0.05). And the premature birth rate of the severe PAH group(60%, 15/25) was significantly higher than the mild and moderate PAH group [20% (5/25) and 3/17, P < 0.05]. There were 4 neonatal asphyxia. The birth weight of mild, moderate and severe PAH groups were (3071 ± 443), (2910 ± 619) and (2381 ± 589) g, respectively. The birth weight in the severe PAH group was significantly lower than the mild and moderate PAH groups (P < 0.05). (4) Mortality. Nine cases maternal death happened, with a mortality of 8.7% (9/103). All of them were severe pulmonary hypertension patients. PAH is a serious complication during pregnancy.Women with mild PAH and normal cardiac function could continue pregnancy to full-term.Women with moderate PAH might continue pregnancy to 32 weeks under intensive care, cesarean delivery is preferable. Pregnant women with severe PAH should terminate pregnancy because of the high mortality.