Pulmonary Hypertension – ABC CARDIOL

ABCIC

Review Article

My Approach to a Standardized Assessment of the Tricuspid Valve: A Contemporary Analysis

Halsted Alarcão Gomes Pereira da Silva et al. Alexandre Costa Souza Adenalva Beck

Abstract

Tricuspid regurgitation (TR) remains one of the most frequent valvular diseases encountered in echocardiographic practice. Mild cases generally do not require further investigation or specific treatment. Moderate and severe valvular disease, however, require more detailed anatomical and etiological assessment to better understand the underlying pathophysiological mechanism.

Two-dimensional echocardiographic analysis is the initial examination of choice and should be performed in a standardized manner, with individual identification of the leaflets using the main transthoracic echocardiographic windows. The description of complementary parameters – such as right ventricular (RV) systolic function, myocardial deformation assessed by longitudinal strain, right-sided chamber diameters and volumes, and estimation of pulmonary pressures – guides the clinician regarding potential therapeutic strategies and provides relevant prognostic information.

In this context, the 2025 European Society of Cardiology (ESC) guideline reinforces the importance of a standardized and comprehensive evaluation of the tricuspid valve and its hemodynamic repercussions. Most of this assessment can be performed using widely available two-dimensional transthoracic echocardiography.

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ABC

Research Letter

Never Stop Looking – the Heart is Not Always the Answer

Daniel Inácio Cazeiro et al. Diogo Ferreira Lia Bruno Ana Isabel Ferreira … João Leitão Fausto J. Pinto Rui Plácido

Case presentation

A 63-year-old woman presented with a two-year history of worsening fatigue, dyspnea (World Health Organization functional class III), and palpitations. The echocardiogram had demonstrated a high probability of pulmonary hypertension (PH), and she was subsequently referred to a PH expert center. Her past medical history included systemic arterial hypertension, iron deficiency anemia secondary to gastrointestinal bleeding from duodenal telangiectasias (endoscopically treated), and a previous extraforaminal L3-L4 discectomy due to lumbar herniated disk with associated radiculopathy, performed at another hospital two years before the first appointment. The post-operative period was complicated by severe acute anemia of unclear etiology, which required nine red blood cell transfusions.

At the time of referral, the patient was taking furosemide 40 mg once daily (OD), candesartan 16 mg + hydrochlorothiazide 12.5 mg OD, simvastatin 20 mg OD, and alprazolam 0.25 mg OD. She had a history of smoking but no significant occupational exposures, and her family history was unremarkable.

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ABC

Case Report

Systemic Sclerosis Associated Pulmonary Arterial Hypertension with Venous Involvement – a Novel ABCC8 Pathogenic Variant

Daniel Inácio Cazeiro et al. João Cravo Mariana Pereira Silva João R. Inácio … Oana Moldovan Catarina Sousa Catarina Lopes Resende João Eurico Fonseca Fausto J. Pinto Rui Plácido

Discussion

PAH with venous/capillary involvement (formerly referred to as pulmonary veno-occlusive disease [PVOD] /capillary hemangiomatosis) is a rare, severe form of PH, with an incidence of 0.1-0.5 cases/million/year. This condition comprises a fibroproliferative process of pulmonary venous and capillary compartments, with small vein occlusion caused by smooth muscle hypertrophy and collagen deposition.

The diagnosis of PVOD is challenging due to its non-specific presentation, often mimicking PAH. Moreover, histological confirmation via lung biopsy is contraindicated due to the significant risk of hemorrhage. Certain clinical and imaging features can facilitate a presumptive diagnosis, which include typical findings on CCTA – centrilobular ground-glass opacities, septal lines, and mediastinal lymphadenopathy, severely reduced DLCO on PFTs, and low oxygen saturation nadir during the 6MWT. PVOD manifests in two forms: hereditary, which is associated with autosomal recessive biallelic mutations in the EIF2AK4 gene, and sporadic, which presents later in life and more frequently in males. Several risk factors are linked to sporadic PVOD, including prior exposure to chemotherapy (particularly alkylating agents),^, tobacco, and organic solvents such as trichloroethylene.^,

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ABCIC

Case Report

Lutembacher Syndrome Associated With Pulmonary Hypertension: The Importance of Early Diagnosis for Enabling Surgical Treatment

Ana Luiza Caldeira Lopes et al. Maria Estefânia Bosco Otto Bianca Corrêa Rocha de Mello Michelle Bruna da Silva Sena Wagner Luis Gali

Introduction

Lutembacher syndrome (LS) is defined by the simultaneous presence of an atrial septal defect (ASD) and mitral stenosis (MS). Although it is a rare condition, its clinical significance lies in the considerable hemodynamic impact and the potential for early progression to irreversible pulmonary hypertension (PH).

The exact prevalence of LS remains unknown. However, for estimation purposes, MS is considered the most frequent manifestation of rheumatic fever (RF) in young women, and the prevalence of RF in developing countries ranges from 5 to 10 cases per 1,000 children. Furthermore, the incidence of congenital ASD in patients with MS is estimated at 0.6% to 0.7%.

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ABCHF

Review Article

Advanced Management of Right Heart Failure: An Illustrative Case

Pedro Castilhos de Freitas Crivelaro et al. Guilherme Pinheiro Machado Marcelo Basso Gazzana William Lorenzi … Igor Gorski Benedetto Felipe Homem Valle Rodrigo Vugman Wainstein

Abstract

Right heart failure (RHF) is a serious condition characterized by the inability of the right ventricle (RV) to generate sufficient output for pulmonary circulation, leading to systemic congestion and hypoperfusion. Proper management of RHF is essential to improve prognosis and quality of life, requiring individualized treatment based on hemodynamic assessment.

This article reviews pathophysiology, diagnosis, and therapeutic options for RHF, including strategies for preload optimization, afterload reduction, and advanced circulatory support. Additionally, it presents a clinical case of a patient with severe RHF secondary to chronic pulmonary thromboembolism and primary tricuspid regurgitation, treated with a hybrid approach involving balloon pulmonary angioplasty and pulmonary thromboendarterectomy combined with surgical valve repair, resulting in significant functional improvement. This review highlights the importance of personalized strategies and combined therapies in the management of advanced RHF.

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Editorial

Coronary Compression in Pulmonary Hypertension: A Treatable Threat Hidden in Plain Sight

Allan K. N. Alencar

Pulmonary hypertension (PH) represents a severe, progressive condition associated with right ventricular failure, impaired functional capacity, and increased mortality.^, Although angina is not a hallmark symptom of PH, it can affect up to 30% of patients and is frequently overlooked in clinical practice. The study published in this issue of Arquivos Brasileiros de Cardiologia addresses a rare yet critical cause of chest pain in PH: extrinsic compression of the left main coronary artery (LMCA) by a dilated pulmonary artery. The authors present an observational case series of 12 patients undergoing percutaneous coronary intervention (PCI) with stenting for LMCA compression, reporting both feasibility and sustained symptom relief.

While LMCA atherosclerosis is well recognized for its prognostic impact, compression of this vessel due to pulmonary artery dilation in PH remains underdiagnosed. The authors highlight that up to 76% of patients with advanced PH exhibit pulmonary artery enlargement, which may result in significant extrinsic compression of the LMCA, even in the absence of coronary atherosclerosis.^, Importantly, LMCA compression has been associated with sudden cardiac death in PH cohorts, emphasizing the need for high clinical suspicion.^–

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Original Article

Left Main Coronary Artery Angioplasty for the Treatment of Extrinsic Compression in Patients with Pulmonary Hypertension

Luciana Dornfeld Bichuette et al. Daniela Calderaro Pedro Alves Lemos Neto Eduardo Kaiser Ururahy Nunes Fonseca … Luiza Sarmento Tatagiba Tulio Martins Vieira Yally Priscila Pessoa Nascimento Marcela Araujo Castro Taysa Antônia Félix Silva Yuri de Deus Montalverne Parente Caio Julio Fernandes Francisco Akira Malta Cardozo Cesar Higa Nomura Pedro Henrique Almeida Marins Rogerio Souza Carlos Vianna Poyares Jardim

Abstract

Background

The most common clinical presentation of pulmonary hypertension (PH) includes exertional dyspnea, signs of systemic congestion, and syncope. Angina pectoris can also be a relevant manifestation, especially in cases where the left main coronary artery (LMCA) is externally compressed by a dilated pulmonary artery. However, significant gaps remain regarding the most appropriate diagnostic and therapeutic strategies for coronary obstruction in this clinical scenario.

Objectives

To assess the feasibility and impact of coronary angioplasty with stent implantation on symptom relief in patients with PH and extrinsic compression of LMCA.

Methods

This descriptive study included 12 patients with PH who were followed at the Pulmonary Circulation Outpatient Clinic of the Instituto do Coração, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. All patients underwent coronary angioplasty with stent implantation to treat extrinsic compression of LMCA.

Results

A total of 12 patients were analyzed, with a mean age of 47.9 years, predominantly with group 1 PH and under specific therapy. All procedures achieved excellent immediate results, with angina relief observed at 30 days. During a mean follow-up of 33 months, no procedure-related complications were reported, and angina symptoms remained controlled. Four patients died due to progressive heart failure.

Conclusion

The findings support the feasibility of coronary angioplasty as a strategy for symptomatic relief of angina in patients with LMCA compression associated with PH. Further studies are needed to evaluate the impact of this intervention on hard clinical outcomes, as well as the role of screening in asymptomatic patients.

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ABCIC

Review Article

My Approach to Estimate Pulmonary Pressures: Practical Aspects

Halsted Alarcão Gomes Pereira da Silva et al. Helder Moura Gomes

Abstract

Pulmonary hypertension (PH) is a global disease and is increasingly present in echocardiography laboratories, given the progressive rise in its prevalence with population aging. Previously diagnosed only through invasive methods, echocardiographic assessment of pulmonary pressures has become fundamental in screening and monitoring patients with PH. This topic was revisited by the American Society of Echocardiography in a new guideline published in March 2025, reinforcing the importance of echocardiography in clinical stratification, considering recent updates in the definition of PH. Among the main assessment parameters, systolic pulmonary artery pressure, mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), and pulmonary capillary pressure (PCP) are described, along with a series of precautions for the correct estimation and interpretation of these values. The classification of PH levels has also been revised in a new framework. As an accessible and reproducible method, the assessment of pulmonary pressures through echocardiography stands out as an essential tool in the evaluation and follow-up of PH.

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ABCHF

Case Report

Reversal of Type 2 Pulmonary Hypertension after Levosimendan: A Challenging Approach Bringing New Insights

Helena Garcia Betinardi Bernardi et al. Deborah de Sá Pereira Belfort Bruno Biselli Paulo Roberto Chizzola … Robinson Tadeu Munhoz Silvia M. Ayub-Ferreira Edimar Alcides Bocchi

Abstract

Among the inotropic agents used in the management of heart failure, levosimendan, in particular, has a selective venodilator property that appears to explain its clinical benefit during pulmonary hypertension (PH). Most of the evidence refers to patients with group 2 PH and consequent right ventricular (RV) dysfunction. In this scenario, the observed benefits may be due to the effect of levosimendan on RV contractility and pulmonary vessel dilation, or to the improvement of left ventricular function and consequent reduction of pulmonary congestion. What we have noticed is that more and more studies are being done on this action and applicability of the drug, appearing in small studies, reviews and formal indications.

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Original Article

In Vivo Anti-Inflammatory Activity of D-Limonene in a Rat Model of Monocrotaline-Induced Pulmonary Hypertension: Implications to the Heart Function

Jorge Lucas Teixeira-Fonseca et al. Diego Jose Belato y Orts Polyana Leal da Silva Michael Ramon de Lima Conceição … Hernan Hermes Carlos R. Prudencio Danilo Roman-Campos

Abstract

Background

D-limonene (D-L) is the major monocyclic monoterpene in citrus plants with anti-inflammatory properties. Pulmonary hypertension (PH) can cause right heart dysfunction and increases the risk of death, partially due to inflammatory response in the heart.

Objective

To evaluate the possible protective effect of D-L on cardiac function in a rat model of monocrotaline-induced PH (MCT-PH).

Methods

Electrocardiogram was monitored in vivo. Masson Trichrome technique was deployed to verify fibrosis in the heart. Contractility function of isolated atrial tissue was studied using organ bath chamber. Real-time quantitative PCR was applied to quantify inflammation in the right ventricle.

Results

The MCT-PH group showed electrical and structural heart remodeling, with the presence of fibrosis in the cardiac tissue and in vivo electrocardiographic changes. Treatment with D-L partially prevented the development of tissue fibrosis and the increase in P wave duration in the MCT-PH group. The contraction and relaxation velocity of isolated right and left atrium were accelerated in CTR and MCT-PH animals treated with D-L. Finally, D-L was able to prevent the abnormal expression of the key inflammatory cytokines (interleukin 1-β, interleukin 6 and tumor necrosis factor-α) in the right ventricle of MCT-PH animals. D-L was able to enhance the production of the anti-inflammatory cytokine Interleukin-10.

Conclusion

Our results showed that in vivo administration of D-L partially prevented the molecular, structural and functional remodeling of the heart in the MCT-PH model with attenuation of the inflammatory response in the heart.

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Original Article

Electrocardiographic Prognostic Marker in Pulmonary Arterial Hypertension: RS Time

Emin Koyun et al. Anil Sahin Ahmet Yilmaz Ferhat Dindas … Idris Bugra Cerik Gorkem Berna Koyun

Abstract

Background:

Pulmonary hypertension is a condition that involves the remodeling of the right ventricle. Ongoing remodeling is also associated with disease prognosis. During the restructuring process, complex changes such as hypertrophy and dilatation may also be reflected in electrocardiographic parameters.

Objectives:

Our study aimed to investigate the relationship between prognosis and electrocardiographic parameters in patients with pulmonary arterial hypertension.

Methods:

The study was designed retrospectively and included patients diagnosed with pulmonary arterial hypertension between 2010 and 2022. The patients were divided into two groups based on their survival outcome. Various parameters, including electrocardiographic, demographic, echocardiographic, catheter, and blood parameters, were compared between the two groups. A p-value of <0.05 was considered statistically significant.

Results:

In the multivariate Cox analyses, the parameters that were found to be independently associated with survival were the 6-minute walk test, mean pulmonary artery pressure, presence of pericardial effusion, and time between the beginning of the QRS and the peak of the S wave (RS time) (p<0.05 for each). Of all the parameters, RS time demonstrated the best diagnostic performance (AUC:0.832). In the survival analysis, a significant correlation was found between RS time and survival when using a cut-off value of 59.5 ms (HR: 0.06 [0.02-0.17], p < 0.001).

Conclusions:

According to the results of our study, a longer RS time is associated with poor prognosis in patients with pulmonary arterial hypertension. We can obtain information about the course of the disease with a simple, non-invasive parameter.

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Case Report

Unveiling Arrhythmogenic Right Ventricular Cardiomyopathy in Scleroderma

Mehmet Rasih Sonsöz et al. Uğur Ozan Demirhan Cemal Bes

Introduction

Systemic sclerosis (SSc) patients commonly report exertional dyspnea and pulmonary hypertension should be first suspected, as the prevalence in these patients is reported to be 5-19% and it is associated with worse clinical outcomes. Additional differential diagnosis includes congenital left-to-right shunt and arrhythmogenic right ventricular cardiomyopathy (ARVC) when right ventricular dilatation and systolic dysfunction are present. Our case concerns an elderly lady with SSc who was initially diagnosed with precapillary pulmonary hypertension.

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Original Article

New Incremental Model for Predicting Mortality in Pre-Capillary Pulmonary Hypertension

Andressa Alves de Carvalho et al. Wanessa Alves de Carvalho Eliauria Rosa Martins Agostinho Hermes de Medeiros Neto … Fernando Bacal Marcelo Dantas Tavares de Melo

Abstract

Background:

In pulmonary hypertension (PH), the identification of easily obtainable prognostic markers associated with right ventricular (RV) dysfunction and survival is needed.

Objective:

To evaluate the association of red cell distribution width (RDW) with clinical, echocardiographic parameters and survival in patients with pre-capillary PH, with the development of a mortality prediction model.

Methods:

Observational, longitudinal, and prospective study conducted from May 2019 to December 2022. Thirty-four patients with pre-capillary PH underwent two-dimensional echocardiography and complete blood count. A cutoff point of 14.5% was considered to define RDW as altered (≥14.5%) or normal (<14.5%). P values <0.05 were considered significant.

Results:

The median RDW was 14.4%. There was a significant difference in peripheral arterial oxygen saturation (SpO₂) (p=0.028), RV strain (p=0.047), and pericardial effusion (p=0.002) between the normal and elevated RDW groups. During a median follow-up of 15 months, 20.6% died. Patients with increased RDW had a shorter overall survival (44.7%, log-rank p=0.019), which was a predictor of mortality in univariate Cox regression (HR 8.55, p=0.048). The addition of RV strain <16% and SpO₂ ≤93% to the model including RDW alone showed incremental value in predicting mortality (χ²=8.2, p=0.049; χ²=12.4, p=0.041), with increased area under the receiver operating characteristic curve (0.729 vs. 0.837 vs. 0.909) and decreased probability of survival (44.7% vs. 35.6% vs. 25%, log-rank p=0.019).

Conclusions:

RDW provides information on the severity of pre-capillary PH by correlating with echocardiographic parameters of RV dysfunction and mortality, which is best predicted by a model including RDW, RV strain and SpO₂.

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Original Article

Echocardiographic Evaluation of Right Atrial Function in Patients with Precapillary Pulmonary Hypertension

Wanessa Alves de Carvalho et al. Andressa Alves de Carvalho Eliauria Rosa Martins Agostinho Hermes de Medeiros Neto … Fernando Bacal Marcelo Dantas Tavares de Melo

Abstract

Background:

Considering that the effect of right ventricular (RV) failure on the right atrium (RA) is an essential part of the pathophysiology of pulmonary hypertension (PH), the assessment of RA function seems to have prognostic value in the course of the disease, but there are still few studies on this subject.

Objective:

To evaluate myocardial strain of the RA in patients with precapillary PH.

Methods:

This is an observational, longitudinal, prospective study involving 36 patients with precapillary PH, in whom two-dimensional Doppler echocardiography was performed. A control group consisting of 26 healthy individuals was used.

Results:

There was a statistically significant difference in mean RA strain between the group with PH (26.3%) and the control group (40.7%). Regarding RA strain of the patients, there was a negative and moderate correlation with RA pressure, RV diameter, and RV hypokinesia; negative and weak correlation with pulmonary artery systolic pressure (PASP); negative and strong correlation with RA area; and positive and moderate correlation with, tricuspid annular plane systolic excursion (TAPSE), TAPSE/PASP, and RV strain. Patients with RA strain ≤ 23 had significantly reduced overall survival compared with patients with RA strain > 23, but this value was not a predictor of mortality.

Conclusion:

It was possible to demonstrate that the analysis of myocardial strain of the RA provides more accurate information about RA function and can be used as an additional parameter in the follow-up of patients with PH, given the early evaluation of atrial dysfunction.

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Editorial

The Role of Atrial Fibrosis for Atrial Fibrillation: Not Always Essential?

Rodrigo Miguel-dos-Santos

The most common cardiac arrhythmia, atrial fibrillation (AF), is estimated to affect ∼2% of the worldwide population and is expected to impact more people as the population ages.^– This scenario is not different in Brazil, where ∼2.5% of the population is projected to be affected by AF.^, The situation is more alarming among pulmonary hypertension patients, in which ∼30% may present AF episodes and ∼70% atrial flutter.^, Hence, a better understanding of the mechanisms and substrates of AF is constantly needed in order to tailor patients’ treatment better.

In a recent publication of the Arquivos Brasileiros de Cardiologia, Teixeira-Fonseca et al. investigated the effects of the left atrial morphofunctional changes induced by pulmonary hypertension in the arrhythmogenesis in isolated left atria. Monocrotaline-induced pulmonary hypertension led to left atria hypertrophy, tissue fibrosis, and electrophysiological remodeling. Curiously, ex vivo electrical burst pacing of the left atrial tissue did not elicit increased atrial arrhythmias in the tissue from pulmonary hypertension animals. As pointed out by the authors, some factors may have contributed to the absence of arrhythmias ex vivo. Here, I am going to focus on the trigger and substrate relationship in AF and discuss the potential cause for the lack of arrhythmias inducibility.

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Original Article

Isolated Left Atrium Morphofunctional Study of an Experimental Pulmonary Hypertension Model in Rats

Jorge Lucas Teixeira-Fonseca et al. Julliane Vasconcelos Joviano-Santos Fabiana da Silva Alcântara Polyana Leal da Silva … Michael Ramon Lima Conceição Danilo Roman-Campos

Abstract

Background

The high incidence of atrial arrhythmias in pulmonary hypertension (PH) might be associated with poor prognosis, and the left atrium (LA) may play a role in this. An important finding in PH studies is that LA remodeling is underestimated.

Objective

This study investigated LA morphology and mechanical function, as well as the susceptibility to develop arrhythmias in a monocrotaline-induced PH (MCT-PH) model.

Methods

Wistar rats aged 4 weeks received 50 mg/kg of MCT. Electrocardiography and histology analysis were performed to evaluate the establishment of the MCT-PH model. The tissue was mounted in an isolated organ bath to characterize the LA mechanical function

Results

Compared with the control group (CTRL), the MCT-PH model presented LA hypertrophy and changes in cardiac electrical activity, as evidenced by increased P wave duration, PR and QT interval in MCT-PH rats. In LA isolated from MCT-PH rats, no alteration in inotropism was observed; however, the time to peak contraction was delayed in the experimental MCT-PH group. Finally, there was no difference in arrhythmia susceptibility of LA from MCT-PH animals after the burst pacing protocol.

Conclusion

The morphofunctional remodeling of the LA did not lead to increased susceptibility to ex vivo arrhythmia after application of the burst pacing protocol.

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ABCIC

Review Article

My Approach to Echocardiographic Assessment of the Premature Newborn

Karen Saori Shiraishi Sawamura et al. Márcio Miranda Brito

Abstract

Echocardiographic assessment of preterm infants, why do it? The incidence of premature births has been increasing worldwide and already affects about 10% of live births. Extremely preterm newborns (PTNB) are at increased risk of developing pulmonary immaturity, leading to pulmonary hypertension (PH), in addition to cardiovascular immaturity and patent ductus arteriosus (PDA).Hemodynamic monitoring of this population is challenging and differs from critically ill pediatric patients due to hemodynamic lability. Thus, minimal manipulation is essential to avoid complications. In this context, non-invasive monitoring methods such as transthoracic echocardiography (TTE) gain importance for hemodynamic assessment. It is a low-cost, radiation-free test that offers good anatomical and functional visualization in pediatric patients, making it a useful tool in the clinical management of severe preterm newborns.What you will find in this article:We will take an objective, didactic, and at times humorous approach to the use of echocardiography in premature newborns in the neonatal intensive care unit (NICU), and we will discuss the echocardiographic particularities of premature infants, especially regarding equipment adjustments, PH, and persistence of the ductus arteriosus (DA).

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ABCIC

Editorial

How to Proceed in a Multimodality Analysis for the Diagnosis and Risk Stratification of Pulmonary Hypertension?

Pedro Gutiérrez-Fajardo

Transthoracic echocardiography

TTE is considered a highly useful, inexpensive, safe, and available imaging modality for initial approach to differential diagnosis of PH. Although a normal TTE does not rule out diagonsis, it decreases the likelihood of PH. Neither CT nor CMRI are part of the initial line of PH; however, in selected patients they can provide additional information before proceeding to right heart catheterization. ^,

It is important to be familiar with normal ranges of RV size and function. ^, TTE showing right heart structure and function abnormalities increases suspicion of PH. It also allows detection of left heart (systolic and diastolic dysfunction, valvular heart disease) and congenital heart disease as potential causes of PH. RV function surrogates such as RV fractional area change, right atrial size, pericardial effusion, tricuspid annular plane systolic excursion (TAPSE), systolic pulmonary artery pressure (SPAP)/TAPSE ratio, and eccentricity index are recommended in follow-up and assessment of therapy response. However, it is important to note that these surrogates should be interpreted in the context of other clinical and imaging parameters, as they are not standalone diagnostic tools. Doppler can be used to estimate the possibility of PH by measuring tricuspid regurgitant jet velocity (TR) and calculating SPAP using the modified Bernoulli equation. When no TR is present or the signal is inadequate, pulmonary acceleration time can be used to calculate mPAP obtained from pulse wave Doppler signal of pulmonary flow. Pulmonary artery diastolic pressure can be estimated if pulmonary regurgitation is present. The velocity at end-diastole is converted into a pressure gradient, adding the estimated right atrial pressure to the resulting value. Finally, both left ventricle and RV output can be calculated. In clinical practice, differentiating acute, chronic, and chronic acute PH can be challenging ( ).

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ABCIC

Original Article

Long-Term Evolution of Patients with Important Pulmonary Hypertension due to Schistosomiasis

José Maria Del Castillo et al. Katarina Barros de Oliveira Rafael Ricardo de Oliveira Travassos Ângela Maria Pontes Bandeira … Carlos Antônio da Mota Silveira Djair Brindeiro Filho

Abstract

Introduction

Hepatosplenic schistosomiasis mansoni, associated with pulmonary arterial hypertension (PAH), causes cardiac chamber remodeling. Little is known about its long-term evolution.

Objective

To evaluate the alterations caused by PAH in patients with schistosomiasis and analyze the clinical and echocardiographic evolution over a period of 10 years.

Methods

The study included 60 patients with PAH due to schistosomiasis and 50 healthy control patients. Clinical and echocardiographic data were evaluated, including dimensions, parietal thickness, function of the right and left chambers, and parameters of myocardial strain. Patients in the group with PAH due to schistosomiasis were followed for 10 years. Data were compared using Student’s t test for unpaired samples with significance level of < 5%.

Results

Patients with PAH had smaller left ventricular dimension without alteration in the ejection fraction, but with decreased left ventricular global longitudinal strain and left atrial reservoir strain. The right ventricular dimensions and parietal thickness were increased, and the parameters of systolic function (tricuspid annular plane systolic excursion, fractional area change, tricuspid s’ wave, and the right ventricular global longitudinal strain) were significantly reduced. During the follow-up period, 18 patients (32%) died, and death was associated with higher functional class, decreased right ventricular longitudinal strain, larger right ventricular size, decreased fractional area change, and decreased tricuspid annular s’ wave velocity.

Conclusion

PAH related to schistosomiasis causes remodeling of the right chambers, with decreased parameters of systolic function and myocardial strain. The long-term evolution, with elevated mortality, presents with larger right ventricular dimensions, lower fractional area change, lower tricuspid annular systolic velocity, lower right ventricular global longitudinal strain, and more advanced functional class.

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Review Article

My Approach to Imaging in Sickle Cell Anemia

João Carlos Moron Saes Braga et al. Fábio Villaça Guimarães Filho Alexandre Rodrigues Raphael Aparecido Barreto

Abstract

Sickle cell disease (SCD) is recognized as a global problem in public health, characterized by the alteration in the red blood cells to the sickle form. Moreover, chronic anemia can also be observed through the change in the rheology of the red blood cells, leading to a scenario of inflammation and oxidative stress, making SCD a multisystem disease.

Cardiac output (CO) proved to be high, leading to an overall increase in the heart chambers and an eccentric myocardial hypertrophy. These heart alterations were attributed only to adaptive reactions to chronic anemia. Recent studies have more clearly recognized an association with pulmonary hypertension (PH), left ventricular diastolic dysfunction, arrhythmias, and sudden death. Moreover, what has also arisen in this context is the hypothesis of the existence of a sickle-cell cardiomyopathy, characterized by diastolic dysfunction and restrictive physiology. The echocardiogram represents a key tool in determining cavitary volumes, diastolic dysfunction, and the estimation of pulmonary pressure, as well as constitutes a valuable resource in the diagnosis and therapeutic treatment of acute chest syndrome. The myocardial strain, rotational variables, myocardial work, and 3D echocardiography can be applied in an attempt to aid in the early detection of patients who are at a higher risk of developing complications and evolving to death related to SCD.

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ABCHF

Review Article

Pathophysiological Bases of Pulmonary Hypertension in Heart Failure with Preserved Ejection Fraction and Implications for Management

Aguinaldo F. Freitas Jr. et al. Daniela do Carmo Rassi Frota Salvador Rassi

Prevalence, definition, and diagnosis

The prevalence of PH in patients with HFpEF is widely heterogeneous, with frequencies ranging from 36% to 81%, and this is due to the diagnostic means used, to the time of progression of the underlying disease, and to the different ejection fraction cut-off points used to define HFpEF. For example, in a subanalysis of the PARAGON study, the prevalence of PH in patients with HFpEF was 31%. Moreover, the different phenotypes of HFpEF may present with a lower or higher degree of PH, depending on the increase in pulmonary vascular resistance, the increase in right atrial pressure, and associated concomitant chronic kidney disease.

Group 2 PH is defined as mean pulmonary artery pressure > 20 mmHg and pulmonary capillary pressure > 15 mmHg. Right heart catheterization is considered the gold standard for accurate measurement of pulmonary pressures, and it is valuable in evaluation of the hemodynamic response to physical exertion, providing important information for differentiating between group 2 PH and other etiologies.

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Original Article

Resistance Exercise Training Mitigates Left Ventricular Dysfunctions in Pulmonary Artery Hypertension Model

Leôncio Lopes Soares et al. Luciano Bernardes Leite Luiz Otávio Guimarães Ervilha Bruna Aparecida Fonseca da Silva … Maíra Oliveira de Freitas Alexandre Martins Oliveira Portes Leonardo Mateus Teixeira Rezende Filipe Rios Drummond Miguel Araújo Carneiro-Júnior Mariana Machado Neves Emily Correna Carlo Reis Antônio José Natali

Abstract

Background

The right ventricular hypertrophy and dilation observed in pulmonary artery hypertension (PAH) damages the left ventricle (LV) dynamics by flattening the interventricular septum.

Objective

To investigate whether low- to moderate-intensity resistance exercise training (RT) is beneficial to LV and cardiomyocyte contractile functions in rats during the development of monocrotaline (MCT)-induced PAH.

Methods

Male Wistar rats (Body weight: ~ 200 g) were used. To assess the time to potential heart failure onset (i.e., end point), rats were divided into sedentary hypertension until failure (SHF, n=6) and exercise hypertension until failure (EHF, n=6) groups. To test RT effects, rats were divided into sedentary control (SC, n = 7), sedentary hypertension (SH, n=7), and exercise hypertension (EH, n=7) groups. PAH was induced by two MCT injections (20 mg/kg, with 7 days interval). Exercise groups were submitted to an RT protocol (Ladder climbing; 55-65% of carrying maximal load), 5 times/week. Statistical significance was assumed at P < 0.05.

Results

RT prolonged the end point (~25 %), enhanced the physical effort tolerance (~ 55%), and mitigated the LV and cardiomyocyte contractility dysfunctions promoted by MCT by preserving the ejection fraction and fractional shortening, the amplitude of shortening, and the velocities of contraction and relaxation in cardiomyocytes. RT also prevented increases in left ventricle fibrosis and type I collagen caused by MCT, and maintained the type III collagen and myocyte dimensions reduced by MCT.

Conclusion

Low- to moderate-intensity RT benefits LV and cardiomyocyte contractile functions in rats during the development of MCT-induced PAH.

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ABCHF

Review Article

Invasive Cardiopulmonary Hemodynamic Assessment in Patients with Advanced Heart Failure: How to Interpret?

Bruno Biselli et al. Luis Fernando Bernal da Costa Seguro

Abstract

Invasive cardiopulmonary hemodynamic assessment by means of a pulmonary artery catheter is an important tool for evaluating patients with advanced heart failure. It makes it possible to definitively diagnose pulmonary hypertension and, when pulmonary hypertension is present, to classify it as isolated post-capillary or combined pre- and post-capillary. Its use is mandatory in evaluation for heart transplantation and mechanical circulatory assist device implantation. Furthermore, it can be very useful in the management of cardiogenic shock.

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Editorial

Should we Consider the Stimulation of Soluble Guanylyl Cyclase as Beneficial for Treating Pre-Capillary Pulmonary Hypertension?

Allan Kardec Nogueira de Alencar

One of the rarest and most complex group of diseases that affects the cardiopulmonary system is known as pulmonary hypertension (PH), a life-threatening clinical condition that in advanced stages eventually results in irreversible dysfunction of the right heart chamber and sudden cardiac death. Pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH) are two different groups within the PH clinical classification system, in which loss and obstructive remodeling of the lung vessels is responsible for a significant rise in pulmonary arterial (PA) pressure and pulmonary vascular resistance (PVR), resulting in a functional decline of the heart performance and progressive right ventricle (RV) failure.

PAH is a pre-capillary-type PH (Group 1), hemodynamically defined by a mean pulmonary arterial pressure (mPAP) >20 mmHg, PA wedge pressure ≤15 mmHg, and PVR ≥3 Wood units. Remodeling of pulmonary vessels in PAH is depicted by the accumulation of pulmonary artery smooth muscle (PASMCs) and endothelial cells (PAECs), fibroblasts, myofibroblasts, and pericytes in the PA walls. In addition, this remodeling process results in a loss of pre-capillary arteries and exacerbates perivascular inflammation. The excessive loss of PAECs is a key pathobiological feature of PAH. This phenomenon triggers the development of an apoptosis-resistant and hyperproliferative phenotype of PAECs. Subsequently, an intense proliferation of PAECs induces the formation of plexogenic lesions in the lung vessels, a histopathologic hallmark of PAH.

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