Moderate Pulmonary Arterial Hypertension Research Articles

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Diagnosed in Adulthood

Introduction: An anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare heart malformation, with 90% of patients dying during the first year of life. If the right coronary artery compensation and multiple collateral circulation are sufficiently established, the patient's myocardial ischemia symptoms are mild and appear later, which is called the adult type ALCAPA. Case Description: A 42-year-old woman presented to our hospital with one-month history of the aggravation of active shortness of breath which gradually progressed to nocturnal paroxysmal shortness of breath and cough. Admission physical examination suggested mild edema of both lower limbs. Transthoracic echocardiography (TTE) showed that a small vessel shadow was abnormally connected to the pulmonary artery (PA), and moderate pulmonary artery hypertension. Coronary computed tomography angiography (CTA) showed an anomalous origin of the left main coronary artery (LMCA) dividing into the left anterior descending (LAD) and left circumflex (LCX) artery from the PA, with no clear connection to the left coronary sinus. The right coronary artery (RCA) was significantly dilated and originated from the normal Valsalva sinus. It was accompanied by multiple collateral circulations, most of which traveled anterior to the right ventricular free wall and anterior interventricular sulcus, and some emanated from the posterior descending branch of the posterior interventricular sulcus and walked toward the posterolateral wall of the left ventricle. Conclusion: Coronary computed tomography angiography (CTA) can be used to visualize the abnormal origin and distribution of the coronary artery's course and may be the first choice in the diagnosis of ALCAPA.

Cardiopulmonary Involvement in Patients with Systemic Sclerosis: A Cross-sectional Study

Introduction: One of the most challenging aspects of systemic sclerosis is the potential involvement of the cardiopulmonary system, which can lead to significant morbidity and mortality if not detected and managed promptly. Aim: To assess the clinical characteristics and prevalence of specific cardiopulmonary involvement and to investigate its association with disease duration in patients with systemic sclerosis from South India. Materials and Methods: This cross-sectional, observational study was conducted at the Department of General Medicine, Government Medical College, Thrissur, India, between January 1, 2020, and December 31, 2020. Forty patients with systemic sclerosis were included according to the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology classification criteria. Skin thickening was evaluated using the modified Rodnan Skin Score (mRSS). Investigations such as chest X-ray, echocardiogram, and antibody profiling were conducted to evaluate cardiopulmonary involvement. The data were analysed using Statistical Package for Social Sciences (SPSS) version 23.0, and the Chi-square test was used to compare qualitative variables between groups. Results: Sixteen patients (40%) had an mRSS score between 11 and 20, followed by 14 patients (35%) with mRSS scores between 21 and 30. The prevalence of Interstitial Lung Disease (ILD) was 50%. The echocardiogram findings showed mitral regurgitation and moderate Pulmonary Arterial Hypertension (PAH) in 30% (n=12) and 25% (n=10) of patients, respectively. High-Resolution Computed Tomography (HRCT) and pulmonary function tests were normal in 10 patients (25%). The duration of the disease was three to eight years in 14 patients and eight to 12 years in 12 patients. Both ILD and PAH were significantly associated with the duration of the disease (p<0.05). Antiscleroderma-70 and anti-centromere were the most common antibodies present in patients with ILD and PAH, respectively. Conclusion: The study indicated a high prevalence of PAH and ILD in patients with systemic sclerosis. Moreover, both ILD and PAH were significantly associated with the duration of the underlying disease, with longer disease duration being associated with a higher likelihood of these conditions.

Abstract 17599: Sotatercept for the Treatment of Pulmonary Arterial Hypertension: A Systematic Review and Single-Arm Meta-Analysis

Introduction: Pulmonary arterial hypertension (PAH) is associated with progressive clinical worsening and a 50% 5-year survival rate with current PAH therapies. Sotatercept is a first-in-class fusion protein with promising results in preclinical trials, but clinical evidence on its role in treating PAH remains limited. Hypothesis: Sotatercept may provide clinical benefits for patients with symptomatic PAH. Aims: To assess the efficacy of sotatercept in addition to background PAH therapy in this population. Methods: We searched PubMed, Embase, Cochrane and Clinicaltrials.gov in April 2023 for clinical trials assessing efficacy outcomes of sotatercept in patients with PAH. Using a random-effects model, we performed a single-arm meta-analysis of proportions for binary outcomes and applied generic inverse variance weighting formulas to pool arithmetic means and least squares means for continuous outcomes. Statistical analyses were performed using Review Manager 5.4.1 and R software 4.2.2. Results: We included three clinical trials comprising 254 patients with WHO functional class II or III PAH. At 24 weeks, only 2% of patients on sotatercept experienced symptoms of clinical worsening (95% CI 1 to 5; Fig. 1A). Sotatercept was also associated with a reduction in pulmonary vascular resistance (mean change from baseline -199.92 dyn·sec·cm -5 ; 95% CI -256.83 to -143.0; Fig. 1B), while improving 6-minute walk distance (mean change from baseline 45.86 m; 95% CI 28.54 to 63.18; Fig. 1C). Conclusions: These findings suggest that sotatercept may improve exercise capacity while decelerating clinical worsening and attenuating pulmonary vascular resistance in patients with mild to moderate PAH.

Effects of maternal pulmonary arterial hypertension on fetal hemodynamics and maternal-fetal outcome in late pregnancy.

The aim of this study was to investigate the effects of maternal pulmonary arterial hypertension (PAH) on fetal hemodynamics in the third trimester and to identify hemodynamic indicators associated with adverse maternal and fetal outcomes. We recruited 48 pregnant women with PAH in the third trimester and 32 women with normal pregnancies as controls matched for age and gestational week. Fetal growth and hemodynamic parameters were assessed by two-dimensional and color Doppler. All cases were followed up until delivery and maternal and fetal outcomes were collected. High throughput sequencing method was used to determine differential miRNA patterns in plasma exposed to pulmonary arterial hypertension (PAH) in pregnant women. We then performed the validated of key differentially expressed miRNAs by real-time PCR. Compared with the normal and mild PAH groups, resistance index (RI), pulsatility index (PI) of the fetal umbilical artery (UA) and quantitative ductus venosus (QDV) blood flow were increased in subjects with moderate to severe PAH, while PI and the ratio of peak systolic velocity (PSV) to end-diastolic velocity (EDV) (S/D) of the middle cerebral artery (MCA) were decreased. Compared with the normal group, subjects in the mild and moderate PAH groups had lower neonatal weight, shorter neonatal height, and higher preterm birth rates. In addition, miRNA sequencing data showed that PAH affected the levels of 23 miRNAs in plasma. At the same time, we showed that PAH significantly decreased the level of miR-1255a and increased the level of miR-548ar-3p by real-time PCR. In the group of pregnant women with moderate to severe pulmonary hypertension, there was a higher proportion of preterm births and low birth weight babies. Hemodynamic changes in the fetal UA, MCA, and ductus venosus (DV) during late pregnancy were associated with adverse fetal outcomes. At the same time, miRNA sequencing results showed that miR-1255a and miR-548ar-3p may play an important role in the development of PAH.

Echocardiographic right ventricular evaluation in cardiac surgery patients undergoing mitral valve reconstruction: a single center prospective observational study.

Mitral valve (MV) regurgitation (MR) is the second most frequent indication for valvular surgery in Europe. Right ventricular (RV) dysfunction is a common finding after cardiac surgery and might persist for years. The RV-function after MV surgery has been controversially discussed. We therefore aimed to evaluate early RV-performance in patients undergoing MV surgery. Between 09/2020 and 06/2022, ninety-two patients presenting with MR undergoing MV surgery were consented and prospectively included for evaluation. Echocardiographic evaluation was performed one day before surgery, one week after surgery and three months later. Primary endpoints reported RV-function changes including tricuspid annular plane systolic excursion (TAPSE), RV systolic prime (S') and fractional area change (FAC). Secondary endpoints included stability of MV repair, changes in left ventricular functions and early mortality. Mean patients' age was 59.1±11.4 years. Fifty-five (59.7%) patients were male. Most of patients presented with severe (n=88; 95.7%) MR. Mean systolic pulmonary artery pressure was 35.6±15.7 mmHg. Moderate or severe pulmonary arterial hypertension (PAH) was present in 60 (65.2%) patients. Patients underwent either isolated MV surgery (n=67; 72.8%) or combined with tricuspid valve surgery (n=25; 27.2%). Minimal invasive surgery was performed in 26.1% (n=24) of the patients. Postoperative short-term follow-up at 3 months reported RV-dysfunction in 44.5% (n=41) of the patients as indicated by reductions in TAPSE & RV S' from 21.2±4.7 to 14±3.3 mm (P<0.001) and from 14.7±4.3 to 9.7±2.8 cm/s (P<0.001) respectively. The FAC reduction from 42.9%±9.6% to 42.2%±9.9% was non-significant (P=0.593) and no need for redo mitral or tricuspid valve surgery was reported. Finally, the presence and severity of preoperative PAH played significant roles for the incidence of RV dysfunction, P=0.021 and P=0.047, respectively. Minimal invasive surgical procedure significantly reduced the incidence of postoperative RV-dysfunction (P=0.013). Study early results report a significant reduction of RV-function after MV surgery as measured by TAPSE, & RV S', even when the FAC remains unchanged. Even though, this finding has limited prognostic implications during an uneventful surgical course.

587 LEFT ATRIAL APPENDAGE OCCLUSION: A REAL SOLUTION TO CARDIOEMBOLIC RISK OF ATRIAL FIBRILLATION?

Abstract 82-year-old woman comes to our clinical observation for worsening exertional dyspnea, with ECG evidence of atrial fibrillation (AF) with high ventricular response. Past medical history: previous right breast carcinoma treated in 1998 with quadrantectomy, radiotherapy and hormone therapy. Previous ischemic transitory attack. Hypertension. Dyslipidemia. The patient reports the first episode of paroxysmal AF in 2016, for which anticoagulant therapy with new oral anticoagulants NOACs was started for CHA2DS2VASc=6. During anticoagulant therapy episode of ocular haemorrhage for which the patient discontinued therapy with NOACs and used low-molecular-weight heparin LMWH "as needed" in the course of arrhythmic relapses. In July and August 2019 relapses of symptomatic paroxysmal AF. Multiple attempts to take different anticoagulants (Rivaroxaban, Edoxaban) poorly tolerated. The patient undergoes catheter ablation by isolation of the pulmonary veins (considered first line according to ESC guidelines for the control of symptoms in patients with paroxysmal AF) and percutaneous left atrial appendage occlusion (LAAO), with indication of anticoagulation therapy for at least 4 weeks after the procedure (Apixaban 2.5 mg bid, reduced dose due to the presence of age&amp;gt;80 years and weight&amp;lt;60 kg). At the entrance to our hospital, an ECG is performed with a finding of total arrhythmia from AF with a high ventricular response (HR 130 bpm), conducted with left bundle branch block (LBBB); cardiac echo-Doppler ultrasound is performed with a slight reduction in left ventricular systolic function (EF 45%), second degree diastolic dysfunction with increased filling pressures, moderate mitral valve insufficiency, severe left atrial dilatation (LAVi 125 ml/m2), moderate tricuspid valve insufficiency with moderate pulmonary arterial hypertension (PAPS 55 mmHg), right atrial dilatation, circumferential pericardial detachment, dilated inferior vena cava hyporeactive with breath acts. At blood tests: BNP 1022 pg/ml, sodium 145 mmol/l, potassium 3.5 mmol/l, creatinine 1.17 mg/dl with eGFR 43 ml/min/1.73mq. Rate control therapy with ß-blockers and Digoxin and diuretic therapy with Furosemide and Canrenone is set, with improvement of symptoms and clinical conditions. Anticoagulant therapy with LMWH is also undertaken but the patient shows poor adherence. After home discharge, the patient goes back to the emergency room for dyspnea. Further cardiac echo-Doppler ultrasound is performed with evidence of thrombotic formation in the left atrium of the size of 30×25 mm. Therapeutic dosage LMWH therapy is started with subsequent Warfarin embrication according to INR, obtaining complete resolution of the thrombotic formation. A systematic review of device-related thrombosis (DRT) after left atrial appendage occlusion (LAAO) proved that the overall incidence of DRT was 3.9% and the median time from procedure to diagnosis of DRT was 1.5 months. According to currently reported cases, most DRTs occur within one year after LAAO. This particular clinical case of DRT three years after LAAO underlines the importance of accurate assessment of the patient's risk profile: LAAO is a valid alternative to anticoagulant therapy in patients at high risk of bleeding but with low embolic risk profile. It's known that high CHA2DS2VASc values ​​and reduced cardiac function are recognized risk factors for thrombus formation, such as patient compliance with antithrombotic therapy. Surely additional studies are needed to determine if the current practice of antithrombotic regimens and duration after LAAO is sufficient.

A Case Study on Telmisartan Induced Acute Kidney Injury

Abstract: Telmisartan is a widely used antihypertensive drug that effectively lowers blood pressure. In this report, we present the case of a patient who developed acute kidney injury after taking an ARB inhibitor, Telmisartan. A 40-year old, male patient who is known case of Congestive heart failure and dilated cardiomyopathy got admitted to the emergency department with breathlessness and cough. He was diabetic for the past four years and was on Insulin. He was also hypertensive for the past 11 years and was on Telmisartan. All his blood parameters except renal function tests (blood urea, creatinine, potassium, sodium) were normal during hospitalization. Cardiac markers (d-dimer and troponins) were within normal limits, but the ECG report revealed sinus tachycardia and left ventricular hypertrophy. Echocardiography report showed Global left ventricular hypokinesia, dysfunction of the left ventricle, moderate pulmonary arterial hypertension, moderate mitral regurgitation, dilated left arterial and ventricular enlargement. Chest X-ray showed cardiomegaly. High Blood pressure was controlled with ARB Inhibitor- Telmisartan. Uncontrolled hypertension caused a change in dose during admission, which could explain the sudden kidney injury. The drug was discontinued abruptly and switched over to an alpha-blocker- Prazosin. A decrease in serum creatinine levels indicated improvement in the patient’s condition in the following days. Hence, we bring your attention to the possibility that all aged patients with or without a compromised cardiac function must undergo careful monitoring to prevent such adverse events due to the drug (Telmisartan). Also, it is prudent to consider the possibility of cardio-renal syndrome for fewer unwanted events. Key words: Angiotensin receptor blockers, Telmisartan, Acute kidney injury, Angiotensin receptor blockers, Kidney function.

Transcatheter closure of ventricular septal defects: preliminary results in children weighing 10 kg or less.

Ventricular septal defect is one of the commonest heart defect in children and closure of this defect with devices has seen a rapid progression over a period of time. The availability of new and safer devices has made the transcatheter closure of ventricular septal defect a suitable option even in young children. The study was done to evaluate the feasibility and complications of device closure of ventricular septal defect in children weighing 10 kg or less with different types of devices. The present study was undertaken in a newly established dedicated Paediatric Cardiac Unit at a Tertiary Care Hospital. Relevant data were obtained retrospectively from the case files and the catheterisation records and data were analysed for first 50 patients with ventricular septal defect weighing 10 kg or less between March 2018 and March 2021. Among these 50 patients selected, device closure was successfully done in 45 (90%) cases while 5 (10%) attempts were unsuccessful for various reasons. The mean weight in this study was 7.46 ± 1.89 kg (2.3-10 kg), 21 (42%) cases were females while 29 (58%) were males; mean age was 19.4 ± 11.88 months (4-48 months). Right heart catheterisation study showed 21 (42%) patients with normal pulmonary artery pressures (no pulmonary artery hypertension). Among 29 patients with pulmonary arterial hypertension, 13 patients (22%) were having mild pulmonary arterial hypertension, 4 (8%) were with moderate pulmonary arterial hypertension, and 12 (24%) were with severe pulmonary arterial hypertension. Mean Qp/Qs was 2.73 ± 0.72 (2.5-4.5) and mean pulmonary vascular resistance was 1.5 ± 1.04 (0.6-4.6 WU). Amplatzer Duct Occluder (ADOI) was used in 15 (30%) cases, 27 (52%) cases were closed with Amplatzer Duct Occluder (ADOII), and the 3 (6%) cases closure was done with Amplatzer muscular ventricular septal defect occluder. Transcatheter closure of ventricular septal defect in children 10 kg or less is feasible and safe alternative to surgical ventricular septal defect closure. The immediate and short-term outcomes have proven this method to be safe and valid.

Pregnancy outcome analysis of 94 patients with pulmonary arterial hypertension during pregnancy.

The aim of the study was to analyze the pregnancy outcomes of patients with pulmonary arterial hypertension to provide a reference for clinical diagnosis and treatment. Clinical data of 94 patients with a pregnancy complicated by pulmonary hypertension were retrospectively analyzed. The means and percentages of the pregnancy outcomes were calculated, and the analysis of variance, Chi-square test, and Fisher's exact test were applied for comparison. The pregnancy outcomes were less favorable in the severe pulmonary arterial hypertension group compared to the mild and moderate groups. The more severe the pulmonary arterial hypertension, the worse the heart function. A poorer heart function was associated with a poorer prognosis across different pregnancy outcomes. A pregnancy with more severe pulmonary arterial hypertension and worse cardiac function has a poorer maternal and infant prognosis and pregnancy outcome. Cesarean section is the preferred delivery method for patients with severe pulmonary arterial hypertension, whereas vaginal delivery is preferred for patients with mild or moderate pulmonary arterial hypertension and good cardiac function.

Prevalence of pulmonary arterial hypertension in severe chronic obstructive pulmonary disease patients attending tertiary care centre Ernakulam

Background: Chronic obstructive pulmonary disease (COPD) is an irreversible bronchial inflammation of lung airways and parenchyma. Various factors play an important role in occurrence and severity of pulmonary arterial hypertension (PAH), a common in severe COPD. The objective of the study is to know the proportion of PAH in patients with severe COPD and to find the association between various factors.Methods: 180 cases of severe COPD patients admitted in Government Medical College Ernakulum from January 2019-December 2019, were enrolled into this cross-sectional descriptive study. Subject’s history, clinical examination, anthropometric measurements, vitals, Arterial Blood Gas (ABG) analysis were done.Results: Among 180 subjects, 148 (82.22%) had mild PAH, 22 (12.22%) subjects had moderate PAH and 10 (5.56%) had severe PAH. Use of accessory muscle was the most elicited sign in the study with 174 (96.67%). 170 (94.44%) had cough and 169 (93.89%) had breathlessness which were the most reported symptoms. Demographic variables and clinical features had no significant mean pulmonary artery pressure (mPAP) association. Grade 3 PAH groups were elder than others, which was statistically significant (p&lt;0.047). FEV1, Oxygen Saturation and ejection fraction were lowest in grade 3 PAH subjects. Respiratory rate, hemoglobin, PCV, tricuspid velocity, PaCO2 were highest in grade 3 PAH subjects being statistically significant (p&lt;0.0001).Conclusions: In our study, majority of severe COPD patients had mild PAH. There was an independent correlation between respiratory rate, hemoglobin, PCV, tricuspid regurgitant velocity and PaCO2 with severity of PAH.

Non-Hodgkin's lymphoma presenting with pulmonary involvement

A 25-year-old woman presented with a history of episodes of fever with night sweats, multiple joint pains and breathlessness for 5 months. Eight months before the present episode, she had developed a generalised rash and transient swellings in the groin on both sides. Physical examination revealed pallor, pedal oedema and a hyperpigmented rash over the chest, back and thighs. Pulse was 126 beats per minute and respirations 38/min; while breathing ambient air, the oxygen saturation by pulse oximetry was 83%. On auscultation of the chest, breath sounds were diminished bilaterally and crepitations were heard in both the lung bases. Neurological examination revealed weakness of proximal muscles of both upper and lower limbs on both sides with a power of grade 3/5. Laboratory investigations revealed elevated total leucocyte count with neutrophilic leucocytosis; band forms and metamyelocytes were seen in the peripheral blood smear. Oxygen inhalation and broad-spectrum intravenous antibiotics were initiated. Echocardiogram showed severe tricuspid regurgitation with moderate pulmonary arterial hypertension. Computed tomography (CT) of the chest revealed patchy consolidation in both the lungs, bilateral pleural effusions and pericardial effusion. Bone marrow biopsy showed normocellular normoblastic marrow with erythroid hyperplasia. 18F-fluorodeoxyglucose positron emission tomography-CT showed metabolically active lymphadenopathy both above and below the diaphragm and bilateral patchy lung infiltrates. Biopsy of the inguinal lymph node along with immunohistochemistry staining confirmed the diagnosis as T-cell lymphoma.

Pulmonary Arteriovenous Pressure Gradient and Time-Averaged Mean Velocity of Small Pulmonary Arteries Can Serve as Sensitive Biomarkers in the Diagnosis of Pulmonary Arterial Hypertension: A Preclinical Study by 4D-Flow MRI.

(1) Background: Pulmonary arterial hypertension (PAH) is a serious condition that is associated with many cardiopulmonary diseases. Invasive right heart catheterization (RHC) is currently the only method for the definitive diagnosis and follow-up of PAH. In this study, we sought a non-invasive hemodynamic biomarker for the diagnosis of PAH. (2) Methods: We applied prospectively respiratory and cardiac gated 4D-flow MRI at a 9.4T preclinical scanner on three different groups of Sprague Dawley rats: baseline (n = 11), moderate PAH (n = 8), and severe PAH (n = 8). The pressure gradients as well as the velocity values were analyzed from 4D-flow data and correlated with lung histology. (3) Results: The pressure gradient between the pulmonary artery and vein on the unilateral side as well as the time-averaged mean velocity values of the small pulmonary arteries were capable of distinguishing not only between baseline and severe PAH, but also between the moderate and severe stages of the disease. (4) Conclusions: The current preclinical study suggests the pulmonary arteriovenous pressure gradient and the time-averaged mean velocity as potential biomarkers to diagnose PAH.

Pulmonary arterial hypertension and renal impairment in patients from the National Institute of Cardiology Dr. Ignacio Chávez, Mexico

The objective of the study was to describe the clinical characteristics and the evolution of the severity of pulmonary arterial hypertension (PAH) and the degree of renal failure. A retrospective observational study was carried out in which the physical and electronic medical records of 60 patients older than 18 years with a diagnosis of pulmonary arterial hypertension were analyzed. In our study, 11.4% of the severe PAH group worsened renal function at six months, and 13.6% of the participants worsened it at one year. In contrast, in the group with moderate PAH, 18.8% worsened at six months, and 12.5% worsened at one year. Also, the GFR at one year was 54.15 mL/min/1.73 m2 in the moderate PAH group and in the severe PAH group was 73.55 mL/min/1.73 m2. The results of this research suggest that the deterioration of kidney function is related to the severity of pulmonary arterial hypertension.

Post-interventional Evaluation and Follow-Up in Children With Patent Ductus Arteriosus Complicated With Moderate to Severe Pulmonary Arterial Hypertension: A Retrospective Study.

Background: Transcatheter closure is an important treatment for patent ductus arteriosus (PDA) complicated with moderate and severe pulmonary arterial hypertension (PAH). This report presents our experience with transcatheter closure of PDA complicated with moderate and severe PAH.Methods: The 49 cases of PDA complicated with moderate and severe PAH were collected in the Second Affiliated Hospital and Yuying Children's Hospital from January 2014 to December 2019 with transcatheter closure of PDA and follow-up. All patients were invited for transthoracic echocardiography, electrocardiogram, and thoracic radiography check-up.Results: Device implantation was successful in 48 of 49 patients (98.0%). Among them, 30 cases were in the PAH after defect correction (CD) group, and 19 examples were in the Non-PAH after defect correction (NCD) group. Pulmonary systolic pressure, left atrial diameter, and left ventricular end-diastolic diameter immediately after interventional therapy and 6 months later were lower than the pre-operative levels (p < 0.05). The incidence of the immediate residual shunt (RS) in this study was 34.9%, most of which were minimal amount shunt. RS disappeared in all patients within 1 year of therapy. Four patients had thrombocytopenia and one patient had left pulmonary artery stenosis. No other serious adverse event occurred during the follow-up period. The pressure gradient tricuspid valve regurgitation (PGTI) and the right heart catheterization (RHC) consistency points were 93.75% (15/16) and were within the 95% consistency limit by the Bland-Altman method. The Logistic regression analysis concluded that the pre-operative Pp/Ps and the narrowest diameter of PDA are risk factors for post-operative PAH (p < 0.05). The cut-off point of the pre-operative Pp/Ps and the narrowest diameter of PDA were calculated to be 0.595 and 4.75 mm, respectively.Conclusion: Interventional occlusion in children with PDA complicated with moderate and severe PAH is safe, effective, and has few complications. Targeted drug therapy has a good clinical effect. The narrowest diameter of PDA and the pre-operative Pp/Ps may be one of the risk factors of residual PAH after interventional therapy.

The conundrum of Thiamine Responsive Acute Pulmonary Hypertension (TRAPH) syndrome in the Emergency Department.

Management of acute pulmonary hypertension in the Emergency Department(ED) can be challenging. The treatment is specialised, requires rapid identification and correction of the precipitating cause; failing which the patient enters a vortex of deterioration. We describe a lesser-known cause for the same, Thiamine responsive acute pulmonary hypertension (TRAPH) syndrome where timely appropriate treatment can result in dramatic improvement. Medical records with ICD code E51.12 (Wet Beriberi) from Mar 2018 to Mar 2020 were screened. The data regarding presenting symptoms, initial vitals, lab and radiological investigations, and treatment received were retrieved from patient files and the Hospital Informatics System, entered into an MS Excel sheet and compared. The study includes eight cases, which we believe to be TRAPH syndrome. Majority were young adult males, ethanol users. All patients presented with acute shortness of breath with tachypnea and shock index more than 0.9. Gross right atrioventricular dilatation, tricuspid regurgitation and mild to moderate pulmonary arterial hypertension was identified in echocardiography. The initial blood gas revealed median pH 6.98 (IQR 6.81-7.09), Bicarbonate 3.4 meq/L (IQR 2.5-5) and lactate 172 mg/dL (IQR 132-200) which improved within 12-16 h of admission. Patients received median 400 mg IV Thiamine. The mean duration of ICU stay was 2.5 days and total hospital stay was 7 days. Thiamine Responsive Acute Pulmonary Hypertension (TRAPH) Syndrome is an under-recognised entity which should be included in differentials for acute right ventricular dysfunction in the ED. Early diagnosis and rapid protocolised management of the same can cause quick recovery of patients.

Intraoperative Management of Atrial Fibrillation Secondary to Hypercalcemia in a Patient with Parathyroid Adenoma

Introduction: Atrial fibrillation is one of the most common arrhythmias occuring in 0.45% of adult population and usually are not assoicated with any cardiac disease. Atrial fibrillation may be seen preoperatively in patients posted for anesthesia. Case Report: A 48 Vyshya male patient with swelling in the neck, palpitations since two years, hypercalcemia and chronic kidney disease with irregularly irregular pulse (88 per min), BP of 110/60 mmHg and respiratory rate of 20/min. Electrocardiogram showed atrial fibrillation. Echocardiography showed non obstructive hypertrophic cardiomyopathy with dilated left atrium and right atrium , moderate pulmonary artery hypertension with ejection fraction of 60%. Patient was diagnosed to have left parathyroid adenoma and was posted for parathyroidectomy. Serum parathyroid hormone levels was 756 pg/ml. Patient was on Inj. Enoxaparin 40 mg that was stopped twelve hours before day of surgery and Amiodarone 100mg and Metoprolol 25mg which was continued on day of surgery. Continuous ECG, invasive arterial blood pressure, pulse oximetry, capnography, neuromuscular monitoring was done. After preoxygenation, induced with propofol and fentanyl.Intubation done with injection vecuronium. Anaesthesia was maintained with Isoflurane ,oxygen, nitrous oxide and vecuronium. Arterial line was established. Left inferior parathyroidectomy was done in 4 hours. Inj. Amiodarone 3mg/kg diluted in 100 ml normal saline was started before skin incision. Patient was in persistent atrial fibrillation throughout the surgery, extubation was done and the patient was sent to intensive care unit. Conclusion: Patients with atrial fibrillation secondary to hypercalcemia undergoing a crucial non cardiac surgery needs thorough understanding of hemodynamic changes, vigilant intraoperative monitoring with proper preoperative and intraoperative cardiac medication.

Effects of exercise training on heart rate variability in children and adolescents with pulmonary arterial hypertension: a pilot study.

Pulmonary arterial hypertension (PAH) is often associated with cardiac autonomic dysfunction, and heart rate variability (HRV) as marker of cardiac autonomic function is even related to disease severity. Knowledge about the effects of physical activity on HRV is limited in these patients. We aimed to assess whether HRV parameters can be influenced by a supervised exercise training program and whether respective changes are related to levels of activity. Six children and adolescents with moderate PAH (3 female, mean age 15.0±4.4 years; mean pulmonary to systemic arterial pressure ratio 0.5±0.2) performed supervised endurance and resistance training for 16 weeks. PAH-specific targeted medication remained unchanged during the study period. HRV was assessed before training and after 16 weeks of training by the use of ECG Move accelerometers. HRV indices included: standard deviation of normal-to-normal (NN) intervals (SDNN), square root of the mean of the sum of the squares of differences between successive NN-intervals (RMSSD), proportion of the number of pairs of successive normal-to-normal intervals that differ by more than 50 ms divided by total number of normal-to-normal interval (pNN50), and the Baevsky stress index (BSI) calculated by the histogram method. Before and after the training program, SDNN, RMSSD and pNN50 correlated with the level of physical activity. Mean values of SDNN, RMSSD and pNN50 did not change significantly due to the training program. Notably, activity level depending SDNN increased markedly after the exercise program (during activity +12.4%, at rest +6.6% and reclining +8.1%, Hedge's g of 0.28, 0.14 and 0.27, respectively). BSI decreased during activity due to the training program reflecting a reduction of stress level (Hedge's g -0.87 indicating an effect of clinical relevance). According to the experience of this pilot study a workout program of 16 weeks revealed an activity level dependent effect on parameters of autonomic cardiac function in children and adolescents with PAH. This mechanism might contribute to the positive effects of exercise training in patients with PAH.

Assessment of pulmonary artery hypertension by Doppler echocardiography and its correlation with right heart catheterization

Background: Definitive diagnosis of pulmonary artery hypertension (PH) requires an elevated mean pulmonary arterial pressure (MPAP) of 25 mmHg at rest measured by right heart catheterization (RHC). As it is invasive mode of investigation, it is declined by many patients, echocardiography was thought to be an acceptable substitute to assess pulmonary arterial pressures. Whether there is a correlation between these measurements is controversial. The aim of this study was to assess PH by echocardiography and its correlation with RHC.Methods: Twenty-six patients aged ≥18 years with pulmonary artery hypertension with or without tricuspid regurgitation (TR) were included in this cross-sectional study. All the patients underwent a transthoracic echocardiography evaluation and were taken for RHC study within an hour.Results: The correlation between pulmonary artery acceleration time (PAAT) and pulmonary artery systolic pressure (PASP) and PAAT and MPAP was significant in all degrees of PH. In contrast, correlation between TR jet maximum velocity (TR Vmax) derived estimated pulmonary artery systolic pressure (EPASP) and PASP was significant in moderate and severe PH, while it did not correlate in mild PH.Conclusions: PAAT is easily measurable parameter and strongly correlates with the values of PASP and the MPAP obtained by right heart catheterization. Implementation of a novel method of determining EPASP from PAAT shall increase significantly the number of patients in whom TTE can be used for the assessment of pulmonary hemodynamic non-invasively.

Evaluation of maternal and fetal outcomes in pregnancy complicated with pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is an uncommon type of pulmonary hypertension (PH) disease characterized by progressive remodeling of distal pulmonary arteries. It could inevitably lead to pulmonary vascular resistance and even right ventricular failure. Biologists have explored the basic pathobiology of PAH, but its functional mechanism and effect in pregnant people remain unknown. This study was designed to investigate the maternal and fetal outcomes of pregnancy-related PAH. Clinical data of 59 pregnant women with PAH who were admitted to Peking Union Medical College Hospital from Jan. 2000 to Dec. 2018 were retrospectively reviewed and analyzed. Multiple parameters, including age, gestational week, the New York Heart Association (NYHA) cardiac functional classification, ultrasonic cardiogram (UCG), blood test, pregnancy complications, pulmonary arterial systolic pressure, maternal and fetal outcomes, were comprehensively investigated and analyzed. According to the pulmonary arterial systolic pressure, all 59 pregnant women were divided into mild PAH (30-49 mmHg, n=18), moderate PAH (50-79 mmHg, n=17) and severe PAH (>79 mmHg, n=24). Five patients died, and the mortality rate was 8.5%. Compared with the mild and moderate groups, the mean gestational week, age of the pregnancy, and NYHA cardiac functional classification grade in the severe PAH group were dramatically different (all P<0.05). The incidence of pregnancy-related complications in the severe PAH group was significantly higher than those in the mild and moderate PAH groups (both P<0.05). The blood parameters, PAH, and NYHA cardiac functional classification grade were significantly changed before and after surgery. We found that the severity of PAH was a major factor of maternal and fetal outcomes. Strengthening the nursing care for pregnant women with PAH is of great clinical significance.

NATIONAL REGISTRY OF INTERSTITIAL LUNG DISEASE FROM PAKISTAN

SESSION TITLE: Diffuse Lung Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: The aim of this study was to determine the epidemiology and characteristics of interstitial lung disease from Pakistan. METHODS: This web-based registry which is the first multicenter registry of ILD from Pakistan recruited patients from 10 centers of 5 major cities between January 2016 and March 2019.The ILD diagnosis was based on high-resolution computed tomography (HRCT) and histopathology in cases where biopsy was performed. The opinion of rheumatologist was also sought in cases of CTD related ILDs. The purpose of this multidisciplinary discussion (MDD) resource group was to maintain uniformity in the diagnostic criteria. RESULTS: A total of 744 patients were enrolled in the registry. The final ILD diagnoses were as follows: IPF 256 (34.4%), HP 132 (17.7%), iNSIP 125 (16.8%), CTD-ILD 121 (16.3%), sarcoidosis 68 (9.1%) and 42 (5.7%) were other ILDs. The median age was 57.5 years with interquartile range 46–65 years and gender was predominantly female (65.6%). A mean age greater than 60 years and history of smoking was most significant in IPF patients (P<0.001). The female gender was significant in all ILD's except IPF (P<0.001). The most common clinical symptoms were cough in 674 (90.6%) and dyspnea in 519 (69.75%). On physical examination, crepitations were audible in 674 (90.6%) and rhonchi in 83 (11.2%) while clubbing was seen in 247 (33.2%) which were mostly IPF. Gastroesophageal reflux disease (GERD) was present in 195 (26.2%) patients and was statistically significant with iNSIP and IPF (P=0.021). Echocardiography was done in 432 (58.1%) patients, of which 206 (47.7%) showed pulmonary arterial hypertension (PAH); 44.7% had mild PAH, 30.1% had moderate PAH and 25.2% had severe PAH. The overall predicted forced vital capacity (FVC) was 58.2 ±18.7%. FVC % of predicted was presented as box-and-whiskers plot showing the median, interquartile range and outliers (if any) for ILD patients. The median FVC for IPF was 59 (range: 25-99.99), for iNSIP was 54 (range: 25-99.99), for CTD-ILD was 60.5 (range: 26-99.99), for sarcoidosis it was 67 (range: 26-99.99). The HP patients had the lowest FVC % of predicted median that was 45.5 (range: 25-82) where four cases had ≥85 FVC and considered as outliers (Figure 2). An avian exposure was reported in 105 (79.5%) HP patients and out of which 86.8% were females. CONCLUSIONS: IPF is the most prevalent ILD in Pakistan, followed by HP and iNSIP. An ongoing prospective registry with longitudinal follow-up will help us further elaborate the clinical characteristics, treatment and survival outcome of patients with ILD. CLINICAL IMPLICATIONS: An expansion of the ILD registry to other cities and rural areas of the country with longitudinal follow-up of currently enrolled patients and more stringent validation of data by MDD in tertiary care and National Coordinating Centers could further expand the network and refine disease information. DISCLOSURES: no disclosure on file for Mosavir Ansarie; No relevant relationships by Ali Zubairi, source=Web Response