Cause Of Pulmonary Hypertension Research Articles

Right ventricular dysfunction secondary to hyperthyroidism with total reversal after thyroidectomy: case report

Thyrotoxicosis is a condition caused by an increase in thyroid hormone, commonly associated with Graves' disease and toxic multinodular goiter. Hyperthyroidism is known to increase the risk of pulmonary hypertension and right heart failure, although the underlying mechanisms are still unclear. The identification of these conditions is crucial, as they are potentially reversible with the achievement of euthyroidism. This case report describes a 40-year-old woman with right ventricular dysfunction and pulmonary hypertension due to hyperthyroidism, which was successfully treated with total thyroidectomy. The case highlights the importance of considering thyrotoxicosis as a potential cause of pulmonary hypertension and the need to rule out other underlying causes. Echocardiography is a valuable tool in assessing right ventricular dysfunction, and achieving euthyroidism can result in the reversal of structural and functional cardiac alterations.

Residual pulmonary hypertension after left to right shunt closure in common pediatric congenital heart diseases: rate and risk factors

Introduction: Left to right shunt in congenital heart disease is the common causes of pulmonary hypertension (PH) in children, and the pulmonary pressure sometime isn’t recovered to normal value after the shunts closed. Residual PH after correcting these shunts may cause deaths. The purpose of this study was to describe the rate of residual PH after surgical closure the left to right shunts in pediatric congenital heart disease and determine its risk factors.
 Patients and methods: A retrospective study was performed on the medical recorded documents of 59 children who were diagnosed pulmonary artery hypertension associated with common left to right shunt congenital heart diseases and performed shunt closure surgically at Cho Ray hospital between January 2020 and May 2022. Systolic pulmonary artery pressure (PAPs) was measured via tricuspid regurgitation jet on transthoracic echocardiography (mode: continuous wave doppler, plane: apical 4-chamber). PH was defined as PAPs ≥ 40mmHg. Postoperative PAPs was measured 1 day before discharge.
 Results: The median age and weight at surgery were 7 months (2 – 215) and 5.9 kg (3.6 – 35), 59.3% was female. Preoperatively, 66.1% were mild PH, 33.9% moderate. Postoperatively, 10 patients (17%) diagnosed residual PH and all was mild. Among 10 patients with residual PH: 7 were combined defects (5 VSD-PDA, 2 VSD-ASD), 3 simple defects (2 VSD, 1 ASD). Preoperative high pulmonary pressure was associated with residual PH (correlation coefficient 0.116, p = 0.001). Conclusions: The proportion of patients with postoperatiovely residual PH is significant and most of these cases are combined shunt. Preoperative high pulmonary pressure is related to residual PH.

Think out of the box: association of left congenital diaphragmatic hernia and abnormal origin of the right pulmonary artery

BackgroundWe report the occurrence of a severe pulmonary hypertension (PH) in a neonate affected by a left congenital diaphragmatic hernia (CDH). PH in this patient was associated with an abnormal origin of the right pulmonary artery from the right brachiocephalic artery. This malformation, sometimes named hemitruncus arteriosus, has to the best of our knowledge never been reported in association with a CDH.Case presentationA male newborn was hospitalized from birth in the neonatal intensive care unit after prenatal diagnosis of a left CDH. Ultrasound examination at 34 weeks of gestational age evaluated the observed-to-expected lung-to-head ratio at 49%. Birth occurred at 38+ 5 weeks of gestational age. Soon after admission, severe hypoxemia, i.e., preductal pulse oximetry oxygen saturation (SpO2) < 80%, prompted therapeutic escalation including the use of high frequency oscillatory ventilation with fraction of inspired oxygen (FiO2) 100% and inhaled nitric oxide (iNO). Echocardiography assessment revealed signs of severe PH and normal right ventricle function. Despite administration of epoprostenolol, milrinone, norepinephrine, and fluid loadings with albumin and 0.9% saline, hypoxemia remained severe, preductal SpO2 inconsistently greater than or equal to 80-85% and post ductal SpO2 lower on average by 15 points. This clinical status remained unchanged during the first 7 days of life. The infant’s clinical instability was incompatible with surgical intervention, while chest X-ray showed a relatively preserved lung volume, especially on the right side. This prompted an additional echocardiography, aimed at searching an explanation of this unusual evolution and found an abnormal origin of the right pulmonary artery, which was confirmed on computed tomography angiography subsequently. A change in the medical strategy was decided, with the suspension of pulmonary vasodilator treatments, the administration of diuretics, and the decrease in norepinephrine dose to decrease the systemic-to-pulmonary shunt. Progressive improvement in the infant respiratory and hemodynamic status enabled to perform CDH surgical repair 2 weeks after birth.ConclusionsThis case recalls the interest of systematic analysis of all potential causes of PH in a neonate with CDH, a condition frequently associated with various congenital malformations.

Pulmonary hypertension in ANCA-associated vasculitis: a retrospective analysis.

Little is known about pulmonary hypertension (PH) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The aims of this retrospective study, in which echocardiography was used for detection of PH, were to identify the potential causes of PH in AAV and to analyze the risk factors for mortality. We performed a retrospective descriptive review of 97 patients who had AAV with PH at our institution from January 1, 1997, through December 31, 2015. These patients with PH were compared with 558 patients who had AAV without PH. Demographic and clinical data were abstracted from electronic health records. Among the patients who had PH, 61% were men; mean (SD) age was 70.5 (14.1) years at the time of PH diagnosis. The majority of patients with PH (73.2%) had more than 1 potential cause of PH, with left heart disease and chronic lung disease being the most common causes. Older age, male sex, smoking history, and kidney involvement were associated with the presence of PH. PH was associated with an increased risk of death (hazard ratio, 3.15; 95% CI, 2.37-4.18). On multivariate analysis, PH, age, smoking status, and kidney involvement were independent risk factors for death. Median survival after the diagnosis of PH was 25.9 months (95% CI, 12.2-49.9). PH in AAV is often multifactorial, is commonly associated with left heart disease, and is associated with a poor prognosis.

Pulmonary capillary hemangiomatosis: an unusual cause of primary pulmonary hypertension in pediatric age group

Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. PCH’s main clinical presentations are progressive dyspnea, fatigue, hemoptysis, palpitations and later irreversible pulmonary hypertension and right-sid­ed heart failure. It is characterized by severe hypoxemia, centrilobular ground-glass opacities on computed tomography and pulmonary congestion triggered by pulmonary vasodilating therapy. The imaging features include diffuse centrilobular ground‑glass opacities with features of pulmonary hypertension. We present a case of PCH in a 4‑year‑old boy who was diagnosed with post-partum hemorrhage (PPH) in echocardiography and computed tomography of the thorax.

Left atrial remodeling and right ventricular-pulmonary arterial coupling after MitraClip implantation

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Transcatheter edge-to-edge mitral valve repair (TEER) with the MitraClip system has emerged as a therapeutic alternative to surgical solutions in high risk patients with severe mitral regurgitation (MR). MR is characterized by left ventricular volume overload, which leads to left ventricular dilatation. Furthermore, MR is a common cause of pulmonary hypertension. Right ventricular (RV) intrinsic contractility and its response to afterload is referred to as RV-pulmonary arterial (PA) coupling. Effect of TEER in patients with MR on left atrial (LA) function and RV-PA coupling is less investigated. Purpose The aim of our study was to assess clinical characteristics of patients undergoing MitraClip implantation including LA function and RV-PA coupling. Methods Eighty-eight consecutive patients with chronic severe MR were enrolled in the single-center observational study (mean age 71.5±7.9 years, male 54.5%, ischemic cardiomyopathy 54.5%). Primary MR (PMR) was present in 22.7%, secondary MR (SMR) in 73.9 % and mixed (MxMR) etiology in 3.4%. Conventional and advanced echocardiography examination was performed using speckle tracking echocardiography before TEER and at 6 month post-TEER follow-up. Left atrial maximal (LAV max) and minimal (LAV min) volumes were measured indexed for body surface area. Furthermore, we analysed left atrial functional parameters, such as reservoir strain, conduit strain and contraction strain. Results The technical success of MitraClip implantation was 95,5%. Mortality rate at 6 month follow-up time was 10.8% (1/88 PMR, 7/88 SMR, 1/88 MxMR). Severe residual MR developed during follow-up in 4.2% and New York Heart Association class III-IV functional status was concluded in 6.8% at 6 month post-TEER. Significant decrease in left ventricular end-diastolic median diameter (mm) was observed at follow-up compared to pre-TEER: 57.5 (IQR: 51.25 – 63.75) vs 60.5 (IQR: 52.5 – 66.75), respectively, p = 0.01. Similarly, the median value of pulmonary artery systolic pressure (PASP) substantially decreased (mmHg) after TEER: 42 (IQR: 32 – 52) vs 49.5 (IQR: 41 – 57), respectively, p = 0.003. Tricuspid annulus systolic excursion/PASP median value, representing RV-PA coupling, improved remarkably: 0.45 (IQR: 0.31 – 0.69) vs 0.38 (IQR: 0.29 – 0.52), respectively, p = 0.02. Compared to pre-TEER, median LA volumes and strain parameters did not change significantly at 6 month post-TEER: reservoir strain (%) 12.88 (IQR: 8.18–18.78) vs 11.74 (IQR: 7.48–20.36), p = 0.41; LAV max (ml) 48.70 (IQR: 39.81–62.29) vs 48.17 (IQR: 42.08–58.7), p = 0.31; LAV min (ml) 34.33 (IQR: 24.5–46.32) vs 33.89 (IQR: 26.02–44.53), p = 0.69. Conclusions The function of the enlarged LA was severely reduced, which did not change after MitraClip implantation in our population. However, positive left ventricular remodeling and improvement in RV-PA coupling was observed 6 month after MitraClip procedure.

MicroRNA and lncRNA as the Future of Pulmonary Arterial Hypertension Treatment.

Pulmonary hypertension (PH) is characterized by a progressive increase in pulmonary arterial pressure and pulmonary vascular resistance. In a short time, it leads to right ventricular failure and, consequently, to death. The most common causes of PH include left heart disease and lung disease. Despite the significant development of medicine and related sciences observed in recent years, we still suffer from a lack of effective treatment that would significantly influence the prognosis and prolong life expectancy of patients with PH. One type of PH is pulmonary arterial hypertension (PAH). The pathophysiology of PAH is based on increased cell proliferation and resistance to apoptosis in the small pulmonary arteries, leading to pulmonary vascular remodeling. However, studies conducted in recent years have shown that epigenetic changes may also lie behind the pathogenesis of PAH. Epigenetics is the study of changes in gene expression that are not related to changes in the sequence of nucleotides in DNA. In addition to DNA methylation or histone modification, epigenetic research focuses on non-coding RNAs, which include microRNAs (miRNAs) and long non-coding RNAs (lncRNAs). Preliminary research results give hope that targeting epigenetic regulators may lead to new, potential therapeutic possibilities in the treatment of PAH.

Pulmonary hypertension: modern methods of treatment and ways of their long-term development.

Introduction: The purpose of this work is to review the main pathogenetic mechanisms of the development of PH, as well as modern established methods of treatment of various types of PH and to consider new, most relevant and promising possibilities of therapy for this pathology. Etiology, pathogenesis and epidemiology: In the modern structure of diseases, pulmonary hypertension plays a significant role, affecting about 1% of the population. However, each of its five main groups has its own characteristics. The causes of pulmonary hypertension can be a polyetiological violation of the processes of endotheliocyte apoptosis and angiogenesis, passive transmission of increased pressure from the left heart to the small circulatory system, chronic hypoxia, leading to vascular remodeling, and many others. In general, it is worth noting that, despite the multiplicity of etiological factors, the main pathogenetic link in the development of pulmonary hypertension is endothelial dysfunction. Treatment and promising areas of research: This article discusses the main established approaches to the treatment of pulmonary hypertension: general recommendations for patients, drugs used as supportive nonspecific therapy, as well as specific conservative and radical methods of treatment. In addition, the main modern directions of scientific research in the field of treatment of pulmonary hypertension are described. Such promising methods as the use of stem cells, gene therapy and epigenetic drugs are considered. Conclusion: Despite active research and many different drugs intended for the treatment of pulmonary hypertension, this pathological condition remains an urgent health problem. Thus, the search for new points of application of therapy and fundamentally new methods of treatment of pulmonary hypertension remains relevant to this day.

Pulmonary vascular disease as a complication of pediatric congenital heart diseases.

Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile. Cardiac catheterization remains the gold standard exam for PH diagnosis. Treatment of pulmonary arterial hypertension (PAH) associated with CHD (PAH-CHD) can then be started according to the recent guidelines recommendations, although most of the evidence is extrapolated from studies on other causes of PAH. PH in pediatric heart disease is often multifactorial, and sometimes unclassifiable, making the management of these patients complicated. The operability of patients with a prevalent left-to-right shunt and increase of PVR, the management of children with PH associated with left-sided heart disease, the challenges of pulmonary vascular disorders in children with univentricular heart physiology and the role of vasodilator therapy in failing Fontan patients are some of the hot topics discussed in this review.

A rare cause of pulmonary hypertension in an elderly woman.

A rare cause of pulmonary hypertension in an elderly woman.

A Case of Fulminant Right Heart Failure Owing to Tumoral Pulmonary Hypertension

Tumoral pulmonary hypertension is a rare cause of pulmonary hypertension. We report a patient who was thought to have idiopathic pulmonary arterial hypertension, but later developed fulminant right heart failure ultimately leading to death. Autopsy revealed substantial pulmonary tumor embolism burden originating from liver adenocarcinoma. (Level of Difficulty: Advanced.)

Evaluation of pulmonary artery stiffness and right ventricle functions in polycythemia vera patients by transthoracic echocardiography.

Polycythemia vera (PV) is known to be a subgroup of chronic myeloproliferative neoplasms and is recognized as a cause of pulmonary hypertension (PH). Pulmonary artery stiffness (PAS) is a relatively new noninvasive echocardiographic index developed to evaluate the structural features and functions of the pulmonary vascular bed. In this study, we aimed to evaluate right ventricular (RV) functions and PAS in PV patients and healthy controls. A group of 65 consecutive PV patients and 40 healthy controls were included in the study. RV global longitudinal strain (RVGLS) and RV free wall longitudinal strain were (RVFwLS) evaluated using two-dimensional (2D) strain echocardiography. RV volume, systolic and diastolic functions were evaluated with three-dimensional (3D) echocardiography. PAS was calculated using the maximum frequency shift (MFS) and acceleration time of the pulmonary artery flow trace. PAS values were significantly higher in the PV group than in the control group (25.2 ± 5.2 vs. 18.2 ± 4.2, p < .001). We found that tricuspid annular plane systolic excursion (TAPSE) (p < .001), RV fractional area change (p < .001) and RV ejection fraction (p < .001) measurements evaluated by 3D echocardiography were significantly lower in the PV group. In our study, PAS values were higher in PV patients than in the healthy control group. Patients with PV may have subclinical RV dysfunction, and PAS value can be used in the early diagnosis of PH and RV dysfunction in this patient group.

PS-C32-2: PRECAPILLARY PULMONARY HYPERTENSION IN ENUGU, NIGERIA AETIOLOGY, CLINICAL FEATURES AND RISK FACTORS

Objective: Pulmonary hypertension is characterized by a progressive and sustained increase in pulmonary vascular resistance which eventually may lead to right heart failure. Echocardiography can be used to diagnose pulmonary hypertension, and CT pulmonary angiography can be used to confirm the cause in most patients. Precapillary pulmonary hypertension includes WHO Clinical Groups 1, 3, 4 and 5. Data on the causes of pulmonary hypertension are lacking in our environment, and we set out to identify the common causes, clinical features and the risk factors. Design and Method: Consecutive patients who had moderate to severe pulmonary hypertension on transthoracic echocardiography in the absence of left heart disease were recruited for the study from July 2015 to April 2022. All the patients were expected to do CT pulmonary angiography to determine the cause of the pulmonary hypertension. The case notes of patients who had done CT pulmonary angiography were retrieved and retrospectively reviewed, and demographic data, presenting complaints, physical signs, and associated clinical conditions extracted. Results: A total of 82 patients met the inclusion criteria, but only 46 did CT pulmonary angiography. The age range of the latter group was from 24 to 80 years with a mean age of 50.35 ± 12.43 years. Chronic thromboembolic pulmonary hypertension (CTEPH) was noted in 30 patients (65%), out of which 24 (80%) are females. Idiopathic cases and VSD Eisenmenger were the next common causes. Breathlessness on exertion, cough and syncopal attacks were the commonest presenting features. The presence of uterine fibroids was a frequent association with CTEPH. Conclusion: Chronic thromboembolic pulmonary hypertension is the commonest cause of pre capillary pulmonary hypertension, and is basically a disease of middle aged women in Nigeria. Breathlessness on exertion and syncopal attacks are common symptoms, and the presence of uterine fibroids is strongly associated with its presence.

Second International Pulmonary Hypertension/Heart Failure Symposium—Structural heart disease, right ventricular dysfunction, and stem cell therapy: The European Pediatric Pulmonary Vascular Disease Network

Second International Pulmonary Hypertension/Heart Failure Symposium—Structural heart disease, right ventricular dysfunction, and stem cell therapy: The European Pediatric Pulmonary Vascular Disease Network

EVALUATION OF PATIENTS WITH COVID-19 IN THE EARLY HYPOXEMIC STAGE AND PATIENTS WITH VIRAL RESPIRATORY TRACT INFECTION IN TERMS OF PULMONARY HYPERTENSION.

Arterial hypoxemia occurs in many COVID-19 patients. Hypoxemia is one of the causes of pulmonary hypertension (PH). Main pulmonary artery dilatation and the main pulmonary artery diameter (mPAD) to ascending aorta diameter (AAD) ratio of ≥1 are significant findings regarding PH. In this study, COVID-19 patients and non-COVID-19 patients with viral respiratory tract infection were evaluated retrospectively in terms of PH. A total of 124 patients (71 male and 53 female), age range 18-85 years, were included in the study as case group and control group. Thoracic computed tomography (CT) images, blood and biochemical parameters, and demographic information were compared between the case group and control group. The normality of numerical variables was examined with Kolmogorov-Smirnov test and homogeneity of the variances with Levene's test. This is the first study researching the effect of early hypoxemic stage COVID-19 infection on development of PH. As a result, it was specified that COVID-19 infection had no effects on mPAD, whereas it had a positive effect on AAD and thus led to a decrease in the mPAD/AAD ratio. Through these values, which could be easily calculated from thoracic CT images, the changes caused by COVID-19 infection on vessel diameters were put forward.

The prevalence of pulmonary hypertension after successful tuberculosis treatment in a community sample of adult patients

There are an estimated 155 million survivors of tuberculosis (TB). Clinical experience suggests that post tuberculosis lung disease (PTLD) is an important cause of Group 3 pulmonary hypertension (PH). However, TB is not listed as a cause of PH in most guidelines. A cross-sectional, community-based study was conducted in nonhealthcare seeking adults who had successfully completed TB treatment. Subjects underwent questionnaires, spirometry, a 6-min walk distance test (6MWD) and transthoracic echocardiography (TTE). Screen probable PH was defined on TTE as an estimated pulmonary artery peak systolic pressure (PASP) of ≥40 mmHg. One hundred adults (71 males) were enrolled, with a mean age of 42 years (SD 13.8 years) and a median of one TB episode (interquartile range: 1-2). Co-morbidities included hypertension (21%), diabetes (16%), human immunodeficiency virus (10%) and asthma/COPD (5%). Only 25% had no residual symptoms after TB. Probable PH was found in 9%, while 7% had borderline raised PASP values (PASP 35-40 mmHg). An association was found between PH and the number of previous TB episodes, with each additional episode of TB increasing the odds of PH-postTB 2.13-fold (confidence interval [CI]: 1.17-3.88; p = 0.013). All of those found to have PH were smokers or ex-smokers yielding an unadjusted odds ratio for PH-postTB of 3.67 (95% CI: 0.77-17.46). There was no statistical difference in spirometry or 6MWD, between those with and without PH. Neither symptoms nor co-morbidities demonstrated significant association with PH. PH after TB was a common finding in this community-based population. Further research is needed to confirm and determine the significance of these findings.

Cancer Therapy-Related Pulmonary Hypertension: A Review of Mechanisms and Implications for Clinical Practice.

Cancer therapy-related pulmonary hypertension is a rare yet potentially fatal cardiotoxicity. However, it is a reversible cause of pulmonary hypertension if detected in its early stages. Cancer therapy-related pulmonary hypertension has been encountered in patients using tyrosine kinase inhibitors, particularly dasatinib. However, it is also well known that many agents used in cancer treatment such as alkylating agents, proteasome inhibitors, thoracic radiation exposure, and immune checkpoint inhibitors are particularly associated with pulmonary hypertension evolution. In case that history, symptoms, and clinical findings suggest a potential cancer therapy-related pulmonary hypertension, echocardiography is considered as the initial tool to detect pulmonary hypertension. If the possibility of pulmonary hypertension is high based on echocardiographic data, cancer treatment, as the initial step, should be discontinued due to its potential risks and other causes for pulmonary hypertension should be investigated thoroughly. Right heart catheterization should be the next step to establish the final diagnosis, and medical management, where appropriate, should be started without delay in these patients according to their pulmonary hypertension subgroup. There exists limited information regarding the diagnostic and management strategies of cancer therapy-related pulmonary hypertension in the current guidelines. In this review article, we aim to present current literature data on the mechanisms and management of cancer therapy-related pulmonary hypertension along with its follow-up algorithm in the setting of cardio-oncology practice.

Thiamine responsive Pulmonary Hypertension among exclusively breastfed babies –A Hospital based prospective study from southern India

Pulmonary hypertension remains leading cause of mortality despite many advances in its management. Thiaminedeficiency is identified as a reversible cause of pulmonary hypertension in exclusively breastfed infants whosemothers are thiamine defecient. The present study is planned to find out the reversible pulmonary hypertensionin association with thiamine deficiency.Objectives: To study the pattern of pulmonary hypertension and its associated factors due to thiamine deficiency.Methodology: It is hospital based study in a tertiary care hospital of tumkur. Pretested and pre designedquestionnaire was used to collect patients socio demographic data, clinical data and the data regardinginvestigations and treatment and prognosis.Results: Total of 300 babies were included in the present study after taking into consideration of all inclusionand exclusion criteria. All the babies, 300 (100) belonged to Hindhu community. 234 (79.3%) belonged to class3 socio economic status according to modified kuppuswamy classification. Majority 212 (70.6%) had onlyprimary education and 32 (10.6%) were illiterates. 88(29.3%) had come with severe gasping. 287(95.6%) of babieshad hepatomegaly and oliguria was present in 144 (48%) of babies. The majority of the infants were presentedwith shock 256 (85.3%), which was managed. Repeat echo was done serially and the difference in reduction ofpulmonary pressure after administration of thiamine was statistically significant (p value &lt;0.005) Conclusion:Thiamine responsive pulmonary hypertension presents as an acute condition with severe respiratory distress,vomiting in a previously well and exclusively breast fed baby. Hence clinical suspicion to recognise the symptomsearly and diagnose and mere thiamine administration is life saving and which reverses the fatal condition. Healtheducation to mothers and family members about food taboos related to use of polished rice to prevent deficiencyof thiamine is also important and supplementation of thiamine as prophylaxis can also be considered in theprogrammes related to antenatal and postnatal care.

Improving Quality of Life in Pulmonary Hypertension with Exercise Training Therapy

Pulmonary hypertension is an increase in pulmonary artery pressure &gt;25 mmHg. This causes some disturbing clinical complaints. The most common causes of pulmonary hypertension are idiopathic and congenital heart disease. ASD (Arterial Septal Defect) is a congenital heart disease that occurs in 1.6 per 1000 live births, with a fairly high life expectancy. However, most people with ASD are not aware of a heart problem before the appearance of a clinically significant disorder. Most patients with ASD with complications of pulmonary hypertension will complain of being easily tired, fainting easily and having difficulty carrying out daily activities, even light activities. Most ASD cases apply shunt closure therapy as the main therapy, but not a few ASD cases cannot be shunted closure because it is a contraindication that can lead to more severe complications, especially with complications of chronic pulmonary hypertension. One of the non-medical therapies used to improve the quality of life of patients with pulmonary hypertension is exercise therapy. This therapy provides a variety of physical exercises that aim to increase the strength of respiratory muscles and extremities. From this case, exercise therapy is able to provide a significant improvement in the quality of life of the patient with an increase in the patient's ability to perform physical activities, as evidenced by the increase in distance traveled when doing the six-minute walk test (SMWT).

Clinical Presentations and Multimodal Imaging Diagnosis in Chronic Thromboembolic Pulmonary Hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening pulmonary vascular disease caused by the presence of a prolonged thrombus in the pulmonary artery. CTEPH is a distinct disease entity classified as group 4 pulmonary hypertension according to the World Symposium on Pulmonary Hypertension. It is the only potentially curable cause of pulmonary hypertension. However, timely diagnosis and treatment are often hampered by nonspecific symptoms and signs and a lack of physician awareness regarding the condition. Thus, it is important to be familiar with the clinical features of CTEPH and the associated diagnostic processes. Herein, we cover the diagnostic approach for CTEPH using multimodal imaging tools in a clinical setting.